2005 ACP Associates Meeting Abstract Submissions

 

Blebitis causing red eye in a patient with glaucoma

Vindya Achuthan MD (Associate), Tom Obertynski MD, Anju Gupta MD, James Puklin MD, Apurva Motivala MD (Member)

Sinai-Grace Hospital and Detroit Medical Center/ Wayne State University, Detroit, MI.

 

Background: Red eye is a frequently encountered condition by primary care doctors both in the in-patient and ambulatory settings. While most cases can be easily managed, it is important that the astute physician recognizes cases which require immediate referral to a specialist to prevent vision loss.

Case Report: We report a 78 year old female who presented with a red eye since 3 days. She denied any vision changes. Past medical history included Glaucoma, HTN, DM, CVA, and hypothyroidism. She had undergone bilateral extra-capsular cataract extraction, glaucoma filtration procedure in the left eye and pan-retinal photocoagulation for proliferative diabetes mellitus. On evaluation in the affected eye she was noted to have 20/60 vision and hypotony with an IOP (intra-ocular pressure) of 5 mm Hg. The slit lamp exam revealed a turbid, thin avascular bleb with surrounding injection of the conjunctiva.  The corneal exam was unremarkable. Fundoscopic exam showed choroidal folds with serous retinal detachment. A subsequent vitreal tap was negative for any infection. Her vision eventually worsened to 20/400. Upon treatment with oral and intra-ocular antibiotics, her bleb showed increasing translucency with a decrease in inflammation. Her hypotony resolved and subsequently her choroidal folds resolved as well. At last check her vision was 20/80 and intraocular pressure was 11 mm Hg. 

Discussion:  In a patient with known glaucoma filtration surgery, primary care physicians should entertain the possibility of a bleb infection (blebitis) as a cause of red eye. The filtering bleb is a rather thin walled structure. In some cases the wall is extremely thin and intermittently even leaks fluid through it. This is not unusual in a person who has had a very successful glaucoma operation in the past. This requires immediate attention as, if blebitis occurs, it quickly can lead to endophthalmitis. Endophthalmitis commonly can cause severe loss of vision. Blebitis in our case, resulted in overfiltration, leading to hypotony and resulting in choroidal folds and retinal detachment.

 

 

 

 

 


Malignant Mesothelioma in an Adult Patient.

Achuthan, Vindya MD (Associate); Yadav, Suresh, MD, (Associate), Ghabsha, Ahmed MD (Associate), Mohammed Asadulla (Member) Sinai-Grace Hospital, Wayne State University, Detroit, Michigan. Detroit, MI.

Introduction:  Malignant mesothelioma (MM) is a rare malignant tumor which arises from the mesothelial surfaces of pleura and peritoneum. The tumor is significant in view of its recognized association with occupational exposure to asbestos. Prolonged asbestos exposure is considered more significant than heavy exposure.

Case Report:  A 54 year old retired assembly line worker with a history of COPD and 20 pack year smoking history presented with progressively worsening dyspnea of 2 weeks.  He was tachypneic, tachycardic and hypoxic using accessory muscles, had decreased breath sounds on right lower lung field and diffuse crackles bilaterally. Patient had to be intubated in the ER for worsening dyspnea and hypoxia. Chest X-ray showed right sided pleural effusion. CT guided thoracentesis revealed bloody pleural fluid with lymphocytosis and mesothelial cells. Pleural biopsy showed areas of poorly differentiated malignant neoplasm with focal areas of necrosis and mitosis, epitheloid cells with  pleomorphic nuclei intermixed with neoplastic spindle cells with elongated nuclei. This kind of mixed pattern is characteristic for mesothelioma. Staining was positive for cytokeratin and calretinin, which favored a diagnosis of mesothelioma.  The patient is currently oxygen dependent and followed up as out patient, awaiting  chemotherapy with cisplatin and alimta.

Discussion:  We report a unique case of localized malignant mesothelioma presenting with dyspnea and unilateral pleural effusion in a patient with a risk of occupational exposure to asbestos. Lifetime risk of developing mesothelioma is 8-13 % in workers exposed to asbestos. The incidence of mesothelioma is expected to increase till 2020 and then decrease due to environmental legislation in place since 1990. There are almost 2500 new cases every year. Average survival time is one year. There are new emerging therapies that improve survival and provide palliation.   It is therefore important that this serious condition be recognized as a differential diagnosis in patients presenting like ours.

           

 

 

 

 

 


Cardiac Sarcoidosis Presenting as Acute Non-ST Elevation Myocardial Infarction

Ashraf Ahmed, M.D; Kamal Nasser, MD; Shazia Essani, MD

Department of Internal Medicine, Sinai-Grace Hospital, Detroit Medical Center, Detroit, MI

 

Background: Significant cardiac involvement in sarcoidosis occurs in 5% of patients, manifesting as sudden death, arrhythmia, conduction disorder, heart failure or cardiomyopathy. To date, this is the first reported case of cardiac sarcoidosis presenting with acute non-ST elevation myocardial infarction (MI).

Case Report: 68 years old African American female nonsmoker with a history of paroxysmal atrial fibrillation and first degree atrioventricular (AV) block, had severe shortness of breath at rest, wheezing, and dry cough for one day. She denied chest pain or history of bronchial asthma. On exam, she was in severe respiratory distress, using the accessory muscles of respiration, with bilateral decreased air entry, wheezes and prolonged expiratory phase. Cardiovascular exam was normal. EKG showed first-degree AV block without ischemic changes. Chest x-ray showed slight haziness and congestion. Patient initially improved with breathing treatments, but later she developed chest pain and troponins turned to be positive. She deteriorated significantly, requiring intraaortic balloon and a cardiac catheterization that showed occlusion of left anterior descending artery (LAD 75%), right coronary artery (RCA 100%), and circumflex artery (80%), with ejection fraction of 20% (decreased from 60%, four month prior to presentation). Emergency coronary bypass surgery revealed widespread epicardial involvement, including LAD, RCA, and circumflex artery, with noncaseating granuloma. Patient received oral steroids and an implantable defibrillator was placed.

Discussion:  Sarcoidosis is a multisystem granulomatous disorder of unknown etiology. It has 5% mortality rate secondary to respiratory failure and cardiac involvement. Cardiac sarcoidosis commonly presents as conduction abnormalities, intractable arrhythmias (which can lead to sudden death), congestive heart failure and progressive cardiomyopathy which might require cardiopulmonary transplants. Corticosteroids remain the main therapy. The survival rate varies between 44 to 75 % at 5 years, among steroid treated patients.

Conclusion:   It is very unusual for cardiac sarcoidosis to present as non-ST elevation MI. The mortality in cardiac sarcoidosis with infiltration of the coronaries is not established, as there are no similar cases reported in the literature.

 

 

 

 

 


Multi-System Organ Failure In Fifty Year Old African American Male With Non-Typhoid Salmonella Serotype Dublin.

Glenn P. Cipullo, M.D. (Associate), Wasif Hafeez, M.D. (Member), Department of Medicine, Sinai Grace Hospital, Detroit, Michigan.

Background: Salmonella are widely dispersed in nature, including gastrointestinal tracts of domesticated and wild animals, reptiles, birds and insects. Salmonella paratyphoid and sendai are highly adopted to humans.  Some Salmonella, such as Dublin (cattle) and Arizona (reptiles) are most adapted to animal species, but rarely  infect humans.

Case Report: A fifty (50) year old, African American Male with a history of alcoholism and hepatitis c infection and hypertension presented to Sinai Grace hospital Emergency Room with the chief complaint of three (3) to six (6) months of increasing “yellowing” of the eyes and skin, two (2) to three (3) days of generalized weakness, light-headedness, and decrease in oral intake, secondary to nausea and vomiting.  The initial presentation revealed an ill appearing, jaundiced male in septic shock with renal and liver failure.  Patient was intubated and  placed on mechanical ventilation.  He was transfused with fresh frozen plasma, packed red blood cells and given vitamin K.  Additional therapeutics included aggressive hydration, broad spectrum antibiotics and steroids. Antibiotics were changed to cover positive blood cultures that grew Salmonella Dublin.

Discussion: The clinical manifestations of non-typhoid Salmonella infection are varied. These variations include asymptomatic infection to clinically manifested disease, which can present as enterocolitis, vasculitides resulting in aneurysm, bacteremia, sepsis, localized infections or a chronic carrier state.  The majority of these infections are associated with the food service establishments. This case is unique in that there are no reports of Salmonella Dublin sepsis and multiorgan involvement from 1966 to the present. World wide infections relating to nontyphi Salmonella are increasing, with most infections secondary to under cooked poultry and eggs.

Conclusion: Although the exact nature of acquisition of infection for our patient was not identified, it is imperative that high clinical suspicion of bacteremia, early pathogen identification and aggressive therapeutics must be utilized in patients who present with sepsis and underlying co-morbidity.

 

 

 

 

 


Determinants of Central Apneas in Patients with Sleep-Disordered Breathing

B Dalal, MD; Ghabsha A, MD; Bdar MS, MD; Rowley James, MD

Introduction: Research has shown that men are more susceptible to the development of central apneas compared to women under research conditions, suggesting that gender differences in the control of ventilation may explain the increased prevalence of sleep-disordered breathing (SDB) in men.  It is unclear if this physiologic finding translates into a clinical finding of increased central apneas in men presenting for evaluation of SDB. Hypothesis: men are more likely than women to have an increased central apnea index on polysomnography.

Methods: Database review of 773 patients diagnosed with sleep apnea (AHI &#8805;5) between 8/2001 and 7/2004.  Records were reviewed for gender, age, body mass index (BMI), neck circumference (NC), co-morbidities including hypertension, heart disease, diabetes mellitus and tobacco use, apnea-hypopnea index (AHI), central apnea index (CAI), and obstructive apnea index (OAI). Patients were divided into two groups: Group 1, CAI&#8805;5/hr (n=91), Group 2, CAI<5 (n=682). Demographic and comorbid parameters and indices of SDB were compared between the groups. Multivariate logistic regression was performed to determine the independent predictors of the presence of a CAI &#8805;5/hr.

Results: Group 1 patients were more likely to be male (60.4% v. 37.0%). NC was larger in Group 1 patients (44.1±5.1 cm v. 41.9±5.1 cm, Group 2, p<0.001) but there was no difference in either age (48.0±14.9 yrs v. 50.1±13.0 yrs) or BMI (41.3±10.2 kg/m2 v. 41.1±11.5 kg/m2).  Both AHI (111.3±71.9 v. 51.4±49.8, p<0.001) and OAI (68.1±61.3 v. 36.7±45.9, p<0.001) were increased in Group 1 compared to Group 2. The prevalence of comorbidities did not differ between the two groups. After multiple logistic regression, gender (OR 2.00, p=0.012), AHI (OR 1.04, p<0.001) and OAI (OR 0.97, p<0.001) but not NC were found to be independent predictors of a CAI&#8805;5/hr.

Conclusions: Men are twice as likely to demonstrate central apneas on polysomnography than women, consistent with the research finding of an increased susceptibility to the development of central apnea. Central apneas are also more likely in patients with increased overall AHI, suggesting that all types of SDB are more common in patients with severe disease.

 

 

 

 

 


Multipe Hydatid Cysts in Heart

Dalal B, MD, Associate; Dalal I, MD; Shah U, MD; Dalal P, MD; Nasser K, MD; Dalal SB, MD

V. S. General Hospital, India

Introduction

Despite the rise in occurrence, echinococcosis remains a very rare disease (<1 case per 1 million inhabitants) in the continental United States. Northern Alaska has endemic areas of E granulosus.

60% of the hydatid cysts are found in liver, 30% in lungs, 2.5% in kidney, 2.5% in heart and pericardium and 5% in other areas. We are reporting a case report of multiple hydatid cysts in heart.

Case report

A 30 year old male came to the out patient clinic with complaints of intermittent chest pain & palpitations. Physical examination was normal except PMI was displaced laterally. EKG was showing RVH with RBBB. 2D echo showed multiple well defined cystic lesions with presence of daughter cysts, the appearance compatible with Hydatid Cyst. Doppler showed mild to moderate MR. USG of the abdomen was normal. Patient underwent for open heart surgery and all the cysts were removed. Patient was also prescribed Albendazole (400 mg twice a day) for period of 12 weeks. Postoperative 2D Echo didn’t reveal any cyst. Subsequently patient didn’t have complains of chest pain or palpitation.

Discussion

Cardiac involvement of hydatid cyst is rare though, solitary hydatid cyst of heart is well documented. In our patient there were multiple cysts in heart with predominance in RV and IVS. In spite of that he was minimally symptomatic and such symptoms are always overlooked by the patient as well as physicians. Diagnosis of hydatid cyst in heart can be easily made by 2D echo. CT scan or MRI may help for lesions with mixed echogenic pattern on echocardiography. Casoni’s test, complement fixation, indirect haemagglutination, latex agglutination and indirect fluorescent antibody tests are used to make diagnosis. Cysts excision is the mainstay of treatment. If untreated, hydaitd cyst of heart can cause arrhythmia, syncope, rupture of cyst in heart. Albendazole or Mabendazole is given to sterilize the cyst and to prevent recurrence if spillage occurred during surgery. Public education is essential to prevent echinococcosis.

 

 

 

 

 


Morgagni’s Hernia as a Cause of Hemodynamic Instability – A Rare Entity

Dalal B MD

Department of Internal Medicine, Sinai Grace Hospital, Detroit Medical Center, Detroit, MI;

Dhar S MD, Dalal S MD, Desai N MD

Temple University Hospital, Philadelphia, PA

 

INTRODUCTION: The liver, bowel, or mesenteric fat may herniate directly through small congenital defects in the anterior diaphragm (i.e., Morgagni's foramina). We are reporting a case in which Morgagni’s hernia was responsible for hemodynamic instability and surgical emergency.

CASE REPORT: A 63 year old man was transferred to the hospital with two episodes of hematemesis at boarding home. EGD revealed erosive esophagitis and a healing duodenal ulcer without an active source of bleeding.  On the evening of the second day, patient was restless and 2 mg of lorazepam was given intravenously. Subsequently, he became lethargic and a blood gas analysis revealed a PCO2 of 84. He was intubated for hypercapnic failure. On the third day, patient dropped his blood pressure to 70/30 mm Hg with pulse rate of 105. He was aggressively hydrated with 4 liters of normal saline without any significant response. Patient was started on dopamine drip with which he maintained his mean arterial pressure. A thoracic radiograph revealed increased cardiac silhouette and some gaseous shadows in mediastinum. A stat echocardiogram was performed which was technically limited with no visualization of heart anteriorly. Subsequently a stat chest CT scan was performed which revealed a huge Morgagni’s hernia compressing the heart anteriorly and causing atelectasis of left lower lobe of lung. Surgical team was consulted and patient was taken to operating room, where laparoscopic reduction of Morgagni’s hernia was performed with closure of defect. Patient was off pressors post surgery, and he was discharged four days later in stable condition.

DISCUSSION: Morgagni’s hernia accounts for 2-4% of all diaphragmatic hernias. The majorities of hernias occurs on the right side and are asymptomatic, although occasionally there is non specific epigastric discomfort. In our patient, Morgagni’s hernia caused compression of heart from anterior side mimicking signs of cardiac tamponade. Surgical closure is required in all symptomatic patients. We report this case to emphasize the significance of this rare complication of Morgagni’s hernia. To our knowledge, no similar presentation has been reported in the literature.

 

 

 

 

 


Alpha Fetoprotein Producing Lung Adenocarcinoma

Ahmed Ghabsha, MD; Kamal Nasser, MD; Husain Saleh, MD

Department of Internal Medicine, Sinai-Grace Hospital, Detroit Medical Center, Detroit, MI.

 

INTRODUCTION: The elevation of serum AFP level has been used as a diagnostic tool for hepatocellular carcinoma and yolk sac tumors.  Rarely, AFP can be produced by other tumors such as lung and gastric carcinomas. We are presenting a rare case of AFP producing adenocarcinoma of the lung

CASE REPORT: A 68 year old African American woman with history of smoking, presented to the hospital with right sided weakness and bloody sputum. Physical exam was significant for right upper and lower extremities weakness and dullness in the left chest posteriorly. Chest X-ray showed a large mass (8 cm) in the region of superior segment of the left lower lobe. CT of the brain showed several lesions in the left hemisphere. Blood tests were significant for markedly elevated serum alpha fetoprotein (AFP) level to 26,800 ng/ml. No evidence of liver metastasis or liver disease was found.  Esophagogastroduodenoscopy revealed no abnormalities. Pathologic examination of the brain metastasis and the lung mass revealed poorly differentiated adenocarcinoma. Immunohistchemistry revealed AFP positive tumor cells, so the diagnosis of AFP producing lung adenocarcinoma was made. The patient died in 18 days after diagnosis.

DISCUSSION: AFP producing lung cancer is a rare disease with less than 30 cases reported thus far. More than 20 cases were reported in Japan. In most cases, the tumor size was large and patients’ prognosis was poor. However, two cases were reported at early stages and had long time survival. Changes in serum AFP level correlates well with the disease status and with prognosis. The higher the level, the more advanced the cancer. Our patient, who had a normal chest x-ray one year prior to presentation, was found to be in stage IV lung carcinoma. She had a large lung mass and a very high serum AFP level, the highest reported in literature so far. Her prognosis was very poor at diagnosis and she passed away soon afterwards. This is believed to be secondary to the high proliferation rate of the AFP producing tumors, as reflected by various histological studies, which revealed high degree of tumor proliferation. Due to the aggressive nature of this kind of tumors, early diagnosis when serum AFP levels are low might affect prognosis, and thus prolong survival

 

 

 

 

 


Solitary Plasmacytoma presenting as Spinal compressions syndrome

Jeevan C. Gowda M.D.Assoc.,  Lourin Chahin M.D. Assoc., Joel Appel M.D. Member, FACP

Introduction

We report a case of extramedulary plasmacytoma presenting as painless cord compression. 

Case Presentation

Our patient is an 85-year-old African American male with a history of hypertension, congestive heart failure, and a prior history of a pituitary adenoma, which was surgically resected.  He presents with a history of progressive weakness over the past three days prior to admission. Patient was using his wife’s old walker to ambulate when he lost all function of his lower extremities. He also had urinary and bowel disturbances. Patient denied any back pain or trauma. His neurological exam showed absence of all sensation up to the T8 level. The motor examination revealed paraplegia and loss of DTR. There was also loss of rectal sphincter tone.  A Stat MRI of the patients back showed a mass of the spinal cord at the level of L5-L6.  The patient underwent surgical decompression and complete resection of the tumor. Histological examination of the tumor showed sheets of plasma cells, consistent with a plasma cell tumor with Kappa cell restriction.

Discussion and conclusion

Our patient had an uncommon presentation of painless cord compression secondary to solitary extramedullary plasmacytoma.  Solitary Plasmacytomas are rare and account for only 3 % of all plasma cell disorders. Only a minority of these are soft tissue plasmacytomas. Although soft tissue plasmacytoma can occur in any site, 90% of tumors develop in the head and neck area, especially in the aerodigestive tract Case reports of CNS involvement exist, however pain is commonly the presenting complaint.  In a study by Posnea et al from Memorial Sloan-Kettering Cancer Center, it was suggested that 90% of malignant spinal cord tumor have some back pain on presentation. Painless presentation, uncommon location and the rarity of the tumor is what makes this case unique.

 

 

 

 

 


Sedative use in the elderly

 

Jeevan C. Gowda M.C. Assoc., Lourin Chahin M.D. Assoc., Surendar Marur M.D. Member.

 

Clinical Question:   Which sleep aids can be safely prescribed in the elderly

Methods:

Data Sources

Pubmed

OVID Medline

Cochran Library

Sources were searched for studies of sedative hypnotics from 1990 to present. 

Results

            Current literature consists of meta analysis of Papers from previous studies of incidence of fall in elderly.  Studies were designed to exam the long term use of sedatives as it relates to falls and traffic accidents.

                        Neutal study 1995-96

                        Tromp study 1995-96

                        Ray Study 1993-96

                        Passaro study 1991-1993

                       

Discussion

Current literature defines an ideal sedative as one that induces sleep  within 30 minutes, and maintains normal REM for 6-8 hours with little or no residual sleepiness during the daytime.  Current literature shows an increase in the odds ratio on average of 1.2 to 2.3 for Benzodiazepam and 1.3 for polydrug use

Conclusion

Use of hypnotics have been associated with increase falls and fractures during chronic use.  As of yet no large studies have been done on single use of sedatives in elderly hospitalized patients.  Such a study would be useful to house officers.  Especially during night shifts when many patients request sleeping aids.

 

 

 

 

 


ACQUIRED C1 INHIBITOR DEFICIENCY SECONDARY TO HODGKIN'S LYMPHOMA CAUSING RECURRENT ANGIOEDEMA

Jayasree Grandhi,  MD, Associate, Tannu Sahay,  MD, Associate, Geetha  Krishnamoorthy,  MD, Member, Joel Appel, MD, FACP, Member

Department Of Medicine, Sinai-Grace Hospital, Wayne State University, Detroit, MI.

 

 

Introduction:  Angioedema is characterized by extravasation of fluid into interstitial tissues. It can be mast cell mediated or complement mediated. The complement mediated can be hereditary or acquired.  Acquired angioedema subtype is usually seen in the second or third decade of life and is usually due to an underlying disease. We report a case of acquired angioedema in a patient with Hodgkin’s disease.

Case Report: A 35-year-old African American female came with multiple episodes of angioedema and weight loss of 50 lbs for last four months. No previous documented cases of angioedema in her family were reported. Physical examination was normal except for angioedema of the upper lip. There was no evidence of lymphadenopathy. Chest x-ray and CT scan showed anterior mediastinal mass. Patient underwent resection of the mass. Pathology showed classic Hodgkin’s lymphoma, syncytial variant of nodular sclerosis type. Patient’s CT scan of abdomen, pelvis and bone marrow biopsy were negative. PET scan showed increased uptake in mediastinum. She was diagnosed to have stage 2B Hodgkin’s disease. Serum levels of C1q, C3, C4, and C1-esterase inhibitor were lowered consistent with acquired angioedema. Pt is being treated with ABVD chemotherapy.

Discussion: Acquired C1-inhibitor deficiency is well recognized in B-cell lymphoproliferative disorders with a prevalence of approximately 0.5%. The typical association of acquired angioedema is with non-Hodgkin’s lymphoma. Upon review of English literature, there were no reported cases of angioedema secondary to Hodgkin’s lymphoma. 

Conclusion: Patients with angioedema are often referred to an allergist to rule out an allergic cause. In most cases no allergic cause is identified and the cases are labeled as “idiopathic.” An adult onset angioedema without family history should raise the suspicion of other underlying diseases. The clinician should be aware of the possibility of Hodgkin’s disease as a cause of this paraneoplastic phenomenon.

 

 

 

 

 


Hemodynamic and Clinical Changes in Patients Following Alcohol Septal Ablation for Hypertrophic Obstructive Cardiomyopathy

Irfan Hameed, MD, Amr Abbas, MD, Stacy Brewington, MD, Judy Boura, William O’Neil, MD, Sinai Grace Hospital and William Beaumont Hospital, Michigan

Background:

Dynamic left ventricular outflow tract obstruction is an important determinant of patient’s symptoms and prognosis. Relief of outflow obstruction leads to positive clinical and hemodynamic effects. Alcohol Septal Ablation (ASA) is a reliable alternative to surgical myomectomy in symptomatic patients with Hypertrophic Cardiomyopathy (HCM) who fail medical therapy. We sought to determine the clinical and hemodynamic benefits following the procedure.

Methods:

A retrospective analysis of 30 patients who underwent ASA for HCM from 1999 to 2004 was conducted. Baseline clinical and echocardiographic findings were reviewed and compared at follow up. The changes from baseline to follow-up were compared using a chi-square or fisher's exact test. The differences were tested using a paired t-test. A p<0.05 was considered significant.

Results:

The mean age was 52 with 63% male. Median clinical follow up was 80 days and echocardiographic follow up was 46 days.

Clinically, a sustained improvement was noted in NYHA class, chest pain, palpitation, syncope, and use of medications. The majority of patients were NYHA class 3-4 at baseline (86%) and improved to class 1 (96%) at follow up. Similarly, by echo an improvement in septal thickness (from 2.2 ± 0.6 to 1.71 ± 0.4 cm, P<0.0001), mitral regurgitation (from 100% to 70%), and outflow gradient (from 78.0 ± 44 to 30.8 ± 31 mm Hg, P<0.0006) was noted.

Conclusion:

ASA leads to a hemodynamic and clinical improvement in patients with HCM.

 

 

 

 

 


Soft Tissue Amyloidoma in a Patient with Multiple Myeloma

Wahed Ishaqsei, MD; Kamal Nasser, MD; Leopoldo Eisenberg, MD, FACP

Department of Internal Medicine, Sinai Grace Hospital, Detroit Medical Center, Detroit, MI.

 

Introduction: Amyloidosis represents a heterogeneous group of disorders characterized by abnormal extra cellular deposition of insoluble fibrillar proteinacious material. Amyloidoma, which is an uncommon finding, is defined as localized nodular mass of amyloid deposit with or without systemic amyloidosis.

Case Report: We report a rare case of a 66 year-old woman, presented with right shoulder pain and limping due to left hip pain of few months’ duration and 7-pound weight loss over 6 weeks prior to presentation. On exam there was tenderness to slight percussion over the left scapula, minimal tenderness to the chest wall, and slight discomfort to the right shoulder, both humeri proximally, and the left groin.

X-rays showed punched-out lesion of the proximal humerus and pelvis consistent with multiple myeloma. Her hemoglobin was 10.2, creatinine 1.6 and 24-hour urine revealed 1226mg bence jones type protein. Bone marrow revealed markedly increased number of atypical kappa restricted plasma cells, with 47% plasma cells of total nucleated cells with normal karyotype. Patient was treated on Zometa, Decadron, Thalidomide and procrit with clinical improvement, but with persistence of left groin pain. An MRI of the groin revealed a 9cm x 6cm x 5cm mass in the left inguinal region with intercapsular extension in the left hip joint with synovial thickening and no bony erosion. A CT-guided biopsy revealed hypocellular lesion in which bland spindled cells were deposited in an amorphous eosinophilic matrix. A Congo red stain revealed an apple-green birefringence under polarized light, consistent with soft tissue amyloidoma. A successful surgical resection was done with good result.

Discussion: Only three reported cases of kappa-light chain Amyloidoma presenting simultaneously with multiple myeloma were found in the English literature. It is important to establish a differential diagnosis of amyloidoma in soft tissue tumor. The clinical presentation mimics other aggressive pathologic processes. The prognosis of amyloidoma is excellent, and cure can be expected by local resection when occurring primarily in a localized anatomic area and no recurrence has been described yet. The therapy of multiple myeloma as in our case, does not affect the course of amyloidoma.

 

 

 

 

 


LOW BONE MASS DUE TO HYPOESTROGENISM IN A PATIENT WITH CARBOHYDRATE DEFICIENT GLYCOPROTEIN SYNDROME

Cortney V. Jones, MD, Associate; Kamal A. Nasser, MD, Associate; Gary W. Edelson, MD, Member, Department of Medicine, Sinai Grace Hospital, Detroit, Michigan.

 

Background: Carbohydrate Deficient Glycoprotein Syndrome (CDGS) is a rare autosomal recessive condition caused by incomplete glycosylation of plasma proteins. There are less than 200 documented cases of CDGS, with four described types, each caused by a specific enzyme deficiency.

 

Case Report: An eighteen-year-old female with a history of CDGS was referred to an endocrinologist regarding osteoporosis. She had mental retardation, cerebellar hypoplasia, ataxia, pectus deformity, antithrombin III deficiency, and primary ovarian failure.  Serum estradiol, FSH, and LH, done in February 2002, were consistent with postmenopausal values; TSH was normal. In August 2002, a bone density by DXA technique revealed a lumbar spine density of 0.465 gm/cm2 (T score –5.29, Z score -5.0).  Femoral neck density was 0.498 gm/cm2 (T score –3.16, Z score –3.7).  Teriparatide, and weight bearing exercises were implemented. In May 2004, repeat DXA technique revealed a lumbar spine density of 0.561 gm/cm2 (T score – 4.4, Z score –4.8).  Femoral neck density was 0.509 gm/cm2 (T score –3.1, Z score –3.9). An improvement of 20.6%, 2.2%, and 6.4% in spine, femoral neck, and total hip density respectively was noted with teriparatide therapy, so she was advised to continue it for 24 months and have a repeat DXA scan in 12 months.

 

Discussion: Skeletal abnormalities present in CDGS include: Kyphoscoliosis, pectus carinatum, inverted nipples, ankle and hip contractures, and short stature.  In our patient, primary ovarian failure led to decreased attained peak bone mass. The etiology of low bone mass was hypoestrogenism, lack of weight bearing exercise, and inadequate glycosylation of bone proteins. The only reasonable option to increase bone mass was human recombinant parathyroid hormone. Aledronate is indicated in patients with accelerated bone loss, and a hypercogulable state is a contraindication to estrogen therapy. Literature review confirmed the use of DXA technique for bone density assessment in CDGS; however, the use of teraperatide to increase bone mass was novel.             

 

Conclusion: The inadequate glycosylation of plasma proteins has systemic effects. Until enzyme supplementation proves to be beneficial, the mainstay of therapy is supportive care.  This entails a meticulous investigation of each organ system and should include bone density assessment.

 

 

 

 

 


Rarest of the Rare Tumor: Primary Leiomyosarcoma of the Sacrum

Rashad Khazi Syed, MD; Kaled Bazzi, MD; Kamal Nasser, MD; Susan Harold, MD

Department of Internal Medicine, Sinai-Grace Hospital, Detroit Medical Center, Detroit, MI.

INTRODUCTION: Primary leiomyosarcoma of the bone is regarded as a rare form of bone sarcoma and thus, few cases have been reported in literature. To our knowledge, this is the second case report of primary leiomyosarcoma involving the spine.

CASE REPORT: A 52 yr old African American female with history of leiomyosarcoma of the sacrum diagnosed and treated with chemotherapy and radiotherapy in 1999, presented to the hospital with complaints of  back pain, weakness in her legs, saddle anesthesia, bladder and bowel incontinence. In the lower limb, there was decreased sensation to pinprick, decreased motor power, and positive babinski bilaterally. She was found to have recurrence few months prior to this admission and was started on chemotherapy. Cord compression was ruled out. Her bone scan did not show any other bone involvement and tissue was negative for C-Kit. Patient was started on gemcitabine, and analgesics. She received in-patient rehabilitation and was given the following options upon discharge: photon therapy, combined radiotherapy and chemotherapy, and sacrectomy.

DISCUSSION: Primary leiomyosarcoma of the bone (PLB) is a rare condition. The origin of the leiomyosarcoma can be one of the following: (1) Primary soft tissue tumor invading the bone, (2) Metastases to bone from leiomyosarcoma, either gastrointestinal tract or uterus, and (3) Primary tumor arising from the bone. Most PLBs are located in the metaphysis of the long bones of the lower extremities. Rarely, it involves the axial skeleton. The origin of the leiomyosarcoma is thought to be from the smooth muscles cells in the intraosseous blood vessels, although an origin from other cells like perivascular, pleuripotent or mesenchymal cannot be ruled out.  Diagnosis of leiomyosarcoma still remains tissue based.  These tumors are usually osteolytic and can be aggressive with interpenetration of the cortical bone.  The lesions have atypical radiographic appearance and can mimic various other primary and secondary malignant tumors.  We are presenting this case report to emphasize that primary leiomyosarcoma of the sacrum, though rare, is associated with mortality as high as 50% with a median survival of 3.4 years after diagnosis. Therefore, clinicians should take an aggressive approach in diagnosing as well as treating this condition.

 

 

 

 

 


Effects of Free Fatty Acid on Glomerular Mesangial Cell Viability and Extracellular Matrix Protein Expression

Aamir Memon, MD, Lalit P. Singh, PhD, and Errol D. Crook, MD. Wayne State University School of Medicine and John D. Dingell VA Medical Center, Detroit, Michigan.

 

Diabetes and insulin resistance are marked by the presence of hyperglycemia and high serum free fatty acids (FFA). High glucose condition has been shown to cause changes in mesangial cells of the kidneys via different pathways including the Hexosamine Biosynthesis Pathway (HBP). There are limited studies on the effects of high free fatty acids on mesangial cell survival and matrix protein expression under normal or high glucose conditions. To address these questions, we employed tissue culture techniques using a mouse mesangial cell line (MES-13), and exposed them to different concentrations of free fatty acids. Different techniques such as MTT (absorbance) and CyQuant (Fluorescent) assays were used to measure cell viability and proliferation rates. SDS-polyacrylamide gel electrophoresis and Western blots were also performed to detect laminin, a glycoprotein component of ECM, on MES-13 extracts. We used Palmitate (saturated fatty acid) and Oleate (unsaturated fatty acid) bound to bovine serum albumin (BSA) used as a carrier protein, and used BSA as our standard. MTT assay was performed in a 96 plate well, after incubating MES-13 cells with FFAs for 48 h using, in a Gemini absorbance microplate reader. Similar technique was used was used to calculate nucleic acid content of the cells exposed to FFA by CyQuant assay in 24, 48 and 72 hours. The addition of 0.25 mM Palmitate to mesangial cells in culture showed decreased cell viability and proliferation when compared to the control either at normal (5.5 mM) and high glucose (25 mM) conditions. However, the nucleic acid content was marginally increased though the effect was not significant. The effect of Oleate on either MTT or CyQuant was similar to that of the palmitate. Palmitate enhanced the production of laminin-g1 subunit pointing to increase matrix production in glomerular mesangial cells. These results show, though preliminary, that free fatty acids alter some functions of mesangial cells contributing to the development of diabetic nephropathy.

 

 

 

 


EFFICACY OF NON-PHARMACOLOGICAL MODALITIES TO TREAT DEMENTIA

Rama Nadella, MD, Associate, Dept of Internal Medicine, Sinai Grace Hospital, Wayne State University, Detroit, MI

BACKGROUND :Gingko Biloba (Gingko) has been used medicinally for over one thousand years. Ginkgo represents one of the most studied and commonly used herbal remedies for Dementia. Vitamin E has been tried in the treatment of Dementia as it is an anti-oxidant scavenging toxic free radicals.

DATA SOURCES : Medline database from 1985 – 2004 using PubMed and Ovid.

SEARCH TERMS : Non-pharmacological, Dementia, Vitamin E, Gingko, Treatment of Dementia

STUDY SELECTION : Only Randomized controlled trials (RCT) selected.

Gingko: RCT by Le Bars (N-309) and Metaanalysis conducted by Le Bars using only well conducted RCT(s) since 1989 (Eg: Le Bars, Kanowski, Maurer and Hofferberth analyses) were studied to see whether Gingko works over Placebo in cognitive disorders, and if it works, how safe is it in treatment of Dementia.

Vitamin E: Sano 1996 is a double blind randomized trial (N-341), which showed effect of a-tocopherol compared with Placebo in the treatment of Alzheimers disease.

OUTCOME MEASURES:     

Gingko => Outcome evaluation was done at 12, 26 and 52 weeks, wherein higher the score poorer the function.

1.         ADAS – Cog (to assess memory, language and orientation)

2.         GERRI (to assess daily living and social behavior)

3.         CGIC (to assess general psychopathology)

Vitamin E => Survival time to the first of four end points

1.         Death

2.         Institutionalization

3.         Severity of Dementia

4.         Loss of 2 out of 3 basic activities of daily living.

RESULTS:      

Gingko: The primary outcome measures were analyzed using intent-to-treat analysis showing a significant improvement in ADAS-Cog score and GERRI score with a p-value < 0.05. No difference was seen in CGIC score.

Vitamin E: There appeared some benefit from Vitamin E with fewer participants reaching end point (only 58%), showing a Peto odds ratio of 0.49 (95% CI 0.25 – 0.96). Moreover, there were excess of falls in Vitamin E group compared to Placebo.

CONCLUSION: Gingko is safe and appears capable of improving cognitive performance and social functioning of Demented patients for 6 months to 1 Year.There is insufficient evidence of efficacy of Vitamin E in the treatment of dementia.

 

 

 

 


SULPHASALAZINE INDUCED PULMONARY TOXICITY

Rama Nadella, MD, Associate, Jayasree Grandhi, MD, Associate, Ahmed Ghabsha, MD, Associate, and , Rajika Munasinghe, MD, Fellow, Piskorowski Thomas, DO, Member, Sinai-Grace Hospital/Wayne State University, Detroit, Michigan.

INTRODUCTION: Sulphasalazine is widely used in the treatment of Inflammatory Bowel disease (IBD) and Rheumatoid arthritis (RA). Pulmonary toxicity due to Sulphasalazine is rare. The most common Pulmonary complication is hypersensitivity pneumonitis with or without eosinophilia. Fibrosing alveolitis, bronchiolitis obliterans and non-cardiogenic pulmonary edema are also reported. Differentiating Sulphasalazine induced lung toxicity from pulmonary manifestations of IBD can be difficult.

CASE REPORT: A 60 year old female with past medical history of ulcerative colitis, was admitted to the hospital with progressive difficulty in breathing, chest pain, fever, 13 pound weight loss over one month and failure to respond to multiple trials of antibiotics. She was on Sulphasalazine 500mg four times a day.  She had bilateral inspiratory crackles and chest X-ray showed lower lobe infiltrates. The possibilities of tuberculosis, malignancy and interstitial pneumonitis secondary to active IBD were considered and excluded by transbronchial biopsy, bronchio-alveolar lavage and colonoscopy with biopsy. Lung biopsy results showed non-specific interstitial pneumonitis. Discontinuation of Sulphasalazine resulted in a relapse of her IBD and treatment with steroids and 5-amino salicylic acid (5-ASA) was initiated. Her symptoms and infiltrates on chest X-ray resolved in 6-8 weeks.

DISCUSSION: Pulmonary toxicity of Sulphasalazine is attributed to Sulphapyridine and can occur within one to six months of exposure and even up to six years later. IBD can itself cause lung manifestations such as pulmonary vasculitis, chronic bronchitis, bronchiectasis and bronchiolitis obliterans. The rapid resolution of symptoms in this patient after Sulphasalazine withdrawal and treatment with steroids is consistent with the diagnosis of delayed hypersensitivity reaction due to Sulphasalazine. The definite way to establish causality is to re-challenge with Sulphasalazine. However, due to prior case reports of fatal pulmonary toxicity associated with Sulphasalazine and the availability of safer 5-ASA agents as alternative therapy for IBD, re-challenge with Sulphasalazine was not attempted.

 

 

 

 


SEVERE SYMPTOMATIC HYPOCALCEMIA DUE TO VITAMIN D DEFICIENCY PRESENTING AS TETANY

Padmaja Naidu, MD, Associate, Wahed Ishaqsei, MD, Associate, Rajika Munasinghe, MD, Fellow, Dept. of Medicine, Sinai-Grace Hospital, Wayne State University, Detroit, Michigan

Introduction: Vitamin D deficiency is increasingly recognized as an important cause of osteomalacia, osteoporosis and fracture in adult patients with limited exposure to sunlight and reduced dietary intake of Vitamin D. Most patients are asymptomatic except for an increased risk of fracture. Severe symptomatic hypocalcemia is rare. Patients with Vitamin D deficiency should be evaluated for additional predisposing conditions when they present with severe hypocalcemia.

Case Report: We present a 54 year old female with who presented to our emergency department with involuntary jerks in all four extremities. She had a past medical history of chronic renal failure secondary to obstructive nephropathy from bladder cancer treated by cystectomy and urostomy, history of partial gastrectomy for peptic ulcer disease, diabetes mellitus, breast cancer and osteoporosis. She was significantly debilitated from her multiple medical conditions and reported only very limited exposure to sunlight. She was found to have severe hypocalcemia ( calcium 4.3 mg/dL) and  renal insufficiency (creatinine 2.9 mg/dL). She also had elevated serum parathyroid hormone levels (PTH 187 pg/ml) along with low 25 OH Vitamin D levels(<5ng/ml). The serum, 1,25(OH) Vitamin D was normal (25 pg/ml).

Discussion: Severe hypocalcemia with tetany is a rare complication of Vitamin D deficiency and is usually associated with the concurrent presence of other risk factors. Our patient had previously undergone a partial gastrectomy, which contributes to malabsorption of fat and fat solvable Vitamins such as Vitamin D. She also had renal insufficiency although the normal levels of serum 1,25(OH) Vitamin D, suggest that renal conversion of 25(OH) Vitamin D to 1,25(OH) Vitamin D was probably not affected to a significant extent. The combined presence of Vitamin D malabsorption and inadequate sunlight exposure contributed to the severity of hypovitaminosis D and resulting hypocalcemia in our patient. It is important to recognize patients at risk of Vitamin D deficiency and institute adequate dietary supplement of calcium and Vitamin D to prevent serious skeletal and metabolic complications.

 

 

 

 

 


IMPACT OF LONG TERM USE OF INHALED CORTICOSTEROIDS ON BONE MINERAL DENSITY

Padmaja Naidu, MD, Department of Medicine, Sinai Grace Hospital, Wayne State University, Detroit, MI

Research Question:

Does long term use of inhaled corticosteroid decrease bone mineral density?

Data Source:

Pubmed, Ovid Medline 1966 to 2004 July 01

Study Selection: Randomised trials were identified by Medline search term “Inhalation Steroids” and “Bone mineral density”. One Meta-analysis and two randomized controlled trials were selected for  study,  out of 143 search results found. Out of these three, only the meta-analysis was accepted for present study as other two had incomplete follow up of patients. In the meta analysis selected, published and unpublished articles were identified using Medline and Embase databases.

Data Extraction: Studies reporting BMD among adult asthma and COPD patients using Inhaled steroids(ICS)  and non-ICS controls were identified. Studies selected for review included at least 1 year of follow up. Two independent reviewers evaluated studies, data from those meeting specified inclusion criteria were abstracted for inclusion  in the meta-analysis.

Results: In the meta-analysis, 266 studies were reviewed, out of which fourteen(5.3%)  met specified inclusion criteria. Sufficient data was available to perform meta-analysis on 3 measures for ICS using patients (lumbar, femoral neck and major trochanter BMD)  and one measure(lumbar BMD)  for non-ICS using controls. Using current National Asthma Education and Prevention Program definitions, the majority of studies (12 of 14) included patients receiving moderate to high doses of ICSs. Among ICS users, annual changes from baseline in lumbar, femoral neck and major trochanter BMD(-0.23%, -0.17%, and +1.46% respectively) were not statistically significant for subgroups of patients with asthma or COPD. Mean change in lumbar BMD were also not significantly different from controls(-0.02%). Further annual changes in lumbar BMD were not significantly  different for subgroups of patients with asthma or COPD.

Conclusions: Long term use of ICSs in patients with asthma or COPD was not associated with statistically significant changes in BMD.

 

 

 

 

 


Recurrent Torsades de Pointes Caused by Levofloxacin

Kamal Nasser, MD; Amer Tfaili, MD; Jennifer Maples, PharmD; Lawrence MacDonald,  MD, FACP

Department of Internal Medicine, Sinai-Grace Hospital, Detroit Medical Center, Detroit, MI.

 

INTRODUCTION: Torsades de pointes (Tdp) represents a type of polymorphic ventricular tachycardia associated with the long QT syndrome. Antiarrythmics, antihistamines, macrolide antibiotics, and psychotropics are the major drug classes associated with Tdp. We are reporting a case of levofloxacin-induced recurrent episodes of Tdp in a young female with ESRD and CHF.

CASE REPORT: A 37 year old Caucasian female came with an infected indwelling hemodialysis catheter. She received one dose of levofloxacin 500mg intravenously (IV) in ED and another dose two days later. Because of sepsis and respiratory failure, the patient was transferred to the intensive care unit on mechanical ventilation. Within nine hours of the last levofloxacin dose, she had recurrent episodes of Tdp in association with prolongation of the QTc interval from 395 ms at presentation to 525 ms at onset of the first Tdp. Each episode required immediate nonsynchronized electric defibrillation. IV magnesium sulfate was given and levofloxacin was discontinued, but these episodes persisted. Serum levofloxacin concentration was 3 then 1.2 mcg/ml at 6 and 12 days respectively (therapeutic range 0.6 – 6.4 mcg/ml). After the addition of isoproterenol, Tdp episodes became self terminating and less frequent with normalization of the QTc interval. Patient was discharged 25 days later in stable condition with a levofloxacin level of < 0.5 mcg/ml, and QTc of 398 ms.

DISCUSSION: Levofloxacin is renally cleared, with 80-90% of the dose excreted unchanged in the urine. Its half-life extends from approximately 6 hours in patients with normal renal function to greater than 35 - 72 hours in patients with ESRD, and it is not removed to any significant extent by hemodialysis. Only two cases of levofloxacin-induced Tdp have been reported in the literature none of which was recurrent. The persistently elevated concentration of the drug 6 and 12 days following the last dose of levofloxacin might explain the recurrence of Tdp.

CONCLUSION: Caution has been advised with the use of levofloxacin in patients with risk factors for Tdp such as heart disease, electrolyte disturbances, and concomitant use of other QT-prolonging drugs. This case calls for more caution to be exercised when these patients also have ESRD due to the longer levofloxacin half-life which could render a possible Tdp recurrent. 

 

 

 

 

 


Posttransplant Glomerulonephritis (GN) In Hepatitis C Positive (HEP C+) Kidney Transplant (KT) Recipients.

Vibha Nayak, MD, Jose-Marie El-Amm, MD, Abdolreza Haririan, MD.

Department of Medicine; Sinai Grace Hospital, Detroit Medical Center, Wayne State University, Detroit, MI, United States.

 

Introduction: Little is known about risk factors for development of posttransplant GN in Hep C+ KT recipients.

Methods: The records of 41 Hep C + patients (pts) transplanted between 9/98 and 5/04 and followed for 2 yrs (median) were reviewed. The occurrence of GN was associated with the use of steroids, Interleukin 2 receptor antibody agents (IL-2R Ab), Calcineurin Inhibitors (CI), Sirolimus (SRL), and number of acute rejection episodes (AR) along with other patient characteristics like age, sex, race, delayed graft function, donor hep C status and total number of ESRD years. Association between GN and different variables were examined using  X2 or t-test, as appropriate. P<0.05 was considered as significant.

Results: Five pts (12%) developed membranoproliferative  / membranous GN (MPGN/MGN) and 9 (22%) focal segmental glomerulosclerosis (FSGS). 14 pts (34%) experienced AR. Donor Hep C status, delayed graft function and CI use was not associated with GN. There was an association between the development of FSGS and the use of maintenance steroid (P<0.03) and IL-2R Ab induction (P<0.04). Also, there was an association between the development of MPGN/MGN and AR (p<0.02) and SRL use (P< 0.05).

Conclusion: We observed that posttransplant GN occurs in 1/3rd of Hep C+ KT recipients, with FSGS accounting for 2/3rd of these cases in our predominantly African-American population. There was an association between use of maintenance steroid and IL-2R Ab induction with development of FSGS, and between SRL use and AR with development of MPGN/MGN.

 

 

 

 

 


The Impact of Inflammation on Cox-2 and Expression in Patients with and without Barretts Esophagus.

Fazal Omar, Omar Al-Subee, Luis Meija, Violeta Yordanova, Yi Xu, James Hatfield, Nabiha Khoury, Martin Tobi.Departments of Medicine and Pathology,  Sinai-Grace Hospital and John D.Dingell VAMC, Detroit. M.I.

 

Cox-2 is over expressed in Barretts Esophagus (BE) a premalignant condition.  However, it also modulates inflammation (INF) that is not necessarily a concomitant of premalignancy.

 Objectives:

1.         To assess for established and unique markers of chronic inflammation in patients with Barretts Esophagus (BE)

2.         To explore the likely pathway for carcinogenesis in patients with Barrett’s esophagus specifically related to inflammation

Hypothesis:

Reflux esophagitis is associated with Barrett’s esophagus that is in turn is associated with adenocarcinoma arising after prolonged inflammation. Barrett’s epithelium, unlike colon cancer does not progress through the adenoma-carcinoma sequence or the de novo pathway seen in the IBD- affected colon, and that the putative pathway is definable by its relationship to chronic inflammation.

Methods: We studied Cox-2 expression in 55 patients with BE, BE & IFN, IFN only and normal controls and correlated staining with neoplastic and IFN markers.   Endoscopic biopsy sections from esophagus, BE and fundus were and section stained with Cox-2 monoclonal (Cayman).  Sections were also stained with a markers of the adenoma carcinoma pathway (Adnab-9), de novo pathway (p53-Dako), vasculogenic (VEGF-PharMingen), inflammatory bowel disease biomarkers (Tn-Dako and Das-1-Dr. K. Das).  The slides were pre-graded for INF and BE by a pathologist blinded to results and intake of NSAIDs/esophageal irritant drugs (EIDs) noted. 

Results:  Patient mean age 59.3+12.9 years, 91% male.  29% were taking NSAID/EID, 5 had BE, 10 BE&IFN, 24 IFN and 16 were controls.  Overall, 32% of sections were Cox-2 positive and are summarized by proportion positive and drug intake in the table and for biomarker correlations and differences in intensity below.

Category(BE,Sq,Fun, GEJ)      Barrett’s Esophagus      Non-Barrett’s Esophagus

Inflammation [%NSAIDs]         14/21 (67%) [20]         10/43(23%) [25]

Non-Inflammation[%NSAIDs] 0/14 (0%) [20] 5/21(24%) [17]

In contrast to Cox-2, Adnab-9 was positive in 26%, p53 in 5%(p<0.001); VEGF in 44%; Tn 33% and Das-1 23%.  There was a correlation of intensity between Cox-2 and p53 (r=0.53;p<0.05) and Adnab-9 (r=0.36;p<0.05) but none with VEGF, Tn, or Das-1.   There was no difference of NSAID/EID usage with pathology category.  Mean stain intensity in BE&IFN was significantly (SS) > BE patient sections (p=0.04) and IFN sections SS>controls (p=0.02); combined IFN, BE&IFN sections were SS> BE and controls (p=0.003). Cox-2 stain from all BE/BE&IFN tended to be more intense compared to IFN and control patient sections (p=0.07). 

Conclusions:  Cox-2 is significantly increased in Fundic and BE compared to squamous epithelia; is suppressible and appears to be allied more with markers of premalignancy than IFN in glandular epithelia than IBD biomarkers, providing a mechanism for causation and possible suppression of esophageal adenocarcinoma by Cox-2 inhibitors in this subset of patients. Cox-2 does appear to be associated with IFN when considering intensity alone but the weight of evidence supports our contention that Cox-2 is a premalignant marker only for BE patients with inflammation.

 

 

 

 

 


Surgical management of a lung abscess: A review.

Asmita Patel, MD, Department of Internal Medicine, Sinai Grace Hospital, Wayne State University, Detroit, Michigan.

 

 

Question: After failure of optimal antibiotic therapy in patients with a lung abscess, what are the benefits of surgical intervention?

Data Sources: Data was collected by searching Medline, Pubmed, and Cochrane Library from 1980 – 2004, using the search terms lung abscess and pulmonary abscess. Different modes of surgical interventions available were reviewed.

 

Methods: A retrospective analysis of the patients who underwent lobectomy was studied. A review of literature also revealed four case series, which studied various modes and efficacy of surgical management of lung abscess. The outcome measures studied in these case series included the cure rate, rapidity of recovery, and residual defects. No randomized control trials were found.

Results: The cure rate with lobectomy was 89% and mortality was reduced to 11%. Patients with percutaneous mode of drainage also had comparable cure rates, but had slower resolution requiring up to 30 days. The mean duration of hospital stay was 10 days with percutaneous drainage, compared to 21 days with lobectomy.

Conclusion: Although lobectomy resulted in complete resolution of the abscess with minimal complications, it was only suitable for a limited number of patients due to underlying co-morbid conditions. Percutaneous drainage was associated with shorter hospital stay, but with slower resolution and some residual defects.  Bronchoscopic-guided pigtail catheter insertion provided access to otherwise unreachable abscesses, but has limited practical applications due to lack of clinical experiences.

 

 

 

 


Scleroderma Renal Crisis in Pregnancy

Asmita Patel, MD, Amer Tfaili, MD, Kotihal Ramesh, MD, Kamal Nasser, MD, Department of Internal Medicine, Sinai-Grace Hospital, Wayne State University, Detroit, Michigan.

Introduction: Scleroderma is an uncommon systemic disease, which affects 1 to 2 per 100,000 individuals in the USA. Scleroderma renal crisis (SRC) develops in 10 to 15% of patients with diffuse cutaneous scleroderma. In the pregnant patients, however, it is difficult to distinguish hypertensive crisis due to SRC from that secondary to eclampsia.

Case Report: A 32 year-old pregnant African-American female with gestational age of 35 weeks, and past medical history of diffuse cutaneous scleroderma, diagnosed one year prior to presentation, presented to the ED with two witnessed tonic-clonic seizures and elevated blood pressure. Patient was diagnosed with eclampsia and managed with intravenous magnesium sulfate and hydralazine followed by an emergent cesarean section. Postpartum, her renal function progressively worsened and blood pressure remained uncontrolled. This was attributed to eclampsia, and the blood pressure was managed with esmolol and hydralazine. An ACE inhibitor was also added to this treatment regimen. Patient’s renal function continued to deteriorate with a rise in creatinine levels from 1.8 to 3.3 mg/dl.. ACE inhibitor was withheld, which was followed by further elevation in creatinine levels to 9.8 mg/dl along with anuria. This deterioration in renal function was thought to be due to SRC and ACE inhibitor was reintroduced at that time. Hemodialysis was eventually initiated for signs of volume overload. Patient was discharged home in a stable condition.

Discussion: In pregnant patients, it is difficult to distinguish hypertensive crisis due to SRC from eclampsia. The cutaneous manifestations of scleroderma help this distinction to a certain extent. Liver function tests, which are usually normal in SRC and elevated in ecclampsia, and the fast deterioration of renal function in SRC could also be helpful clues. In patients with scleroderma who develop eclampsia, therapy with ACE inhibitors, particularly captopril, has been shown to improve survival and shorten the duration of dialysis (1-year survival: 15% without and 76% with ACE inhibitors). ACE inhibitors should be promptly initiated postpartum and maintained. Despite initiation of ACE inhibitor therapy, poor outcome is associated with an uncontrolled blood pressure, congestive heart failure, and initial serum creatinine more than 3.0 mg/dl.

 

 

 

 

 


PERIPHERAL BUT NOT CENTRAL CHEMOREFLEX RESPONSIVENESS IS ENHANCED IN AFRICAN AMERICANS COMPARED TO CAUCASIANS DURING WAKEFULNESS.

 

Tabarak Qureshi, MD, Associate, Jason Mateika, PhD, Ismail Bobat, MD, 

Safwan Badr, MD.

Sinai Grace Hospital, Detroit Medical Center, Wayne State University and John D. Dingell VA Medical Center, Detroit, MI.

 

Introduction: The present study was designed to determine if the ventilatory response to carbon dioxide (CO2) in the presence of high or low oxygen (O2) levels are significantly different in African Americans (AA) as compared to Caucasians (C) during wakefulness.

Methods: Ten healthy African American (AA) and 9 healthy Caucasian (C) subjects completed 4 CO2 rebreathing trials. Hyperventilation was completed before each rebreathing trial to attain a partial pressure of CO2 (PETCO2) between 22-25 mmHg. Thereafter, subjects were switched into a re-breathing bag that contained an initial PETCO2 of 42 mmHg and a partial pressure of O2 (PETO2) of either 50 mmHg (CP - central + peripheral chemoreflex activation) (2 trials) or 140 mmHg (CC – central chemoreflex activation) (2 trials). During the rebreathing trials O2 levels were maintained while PETCO2 increased in a linear fashion.

Results: Age (AA vs. C - 38.9 ± 1.73 vs. 38.7 ± 2.5) and body mass index (BMI) (AA vs. C - 27.3 ± 0.9 vs. 28.6 ± 1) was not significantly different between the groups. The slope of the ventilatory response to increases in CO2 (i.e. responsiveness) was greater in the AA as compared to the C group during the CP (8.9 ± 1.3 vs. 6.2 ± 1.1 L/min/mmHg; p < 0.03), but not the CC rebreathing trials. The ventilatory recruitment threshold (i.e. when ventilation begins to rise in a linear fashion with increases in carbon dioxide) was not significantly different between the groups during both the CP (AA vs. C - 40.7 ± 0.7 vs. 42.1 ± 0.8 mmHg) and CC (AA vs. C - 46.8 ± 0.7 vs. 46.0 ± 0.8 mmHg) rebreathing trials.

Conclusions: Activation of the peripheral chemoreflex leads to a greater ventilatory response in AA as compared to C during wakefulness. This increase is due solely to an increase in chemoreflex responsiveness and not due to changes in the recruitment threshold.

 

 

 

 

 


Orthostatic Hypotension; is it a sign of post concussion syndrome?

S. Rajaguru, M.D (Associate), A. Reddy M.D (Associate), S. Marur MD (Member), Department of Medicine, Sinai Grace Hospital/Wayne State University, Detroit, Michigan.

Background: Patients sustaining mild traumatic brain injuries often report physical, cognitive, and emotional or behavioral symptoms referred to as post concussion syndrome (PCS).

Case report: A 35-year-old African American male with no significant past medical history presented to Sinai Grace emergency department for new onset seizures after sustaining a closed head injury from “pistol whipping”. He developed seizure activity in the emergency department as well. Initial presentation revealed a 2.5 cm laceration to the border of his parieto-occipital area with hematoma on the inferior aspect of his left occipital area. During the hospital stay, the patient continued to have seizure, headache, anxiety, and symptomatic orthostatic hypotension, which did not respond to IV fluid boluses. Further, the orthostasis did resolved with fludrocortisone treatment.

Discussion: The most commonly reported post concussion symptoms are headache, dizziness, decreased concentration, memory impairments, irritability, fatigue, visual disturbances, sensitivity to noise, judgment impairments, depression, and anxiety. However the occurrence of autonomic dysfunctions like orthostatic hypotension following head trauma in the absence of other causes such as hypovolemia is not documented thus far.

Conclusion: Here we would like to announce the first reported case of autonomic dysfunction presenting as orthostatic hypotension with post concussion syndrome.

 

 

 

 

 


Efficacy of Trnscutaneous Pacing in Asystole

S. Rajaguru MD (Associate), S. Marur MD (Member) Department of Medicine, Sinai Grace Hospital/ Wayne state University, Detroit, MI

Background: Asystole is the absence of electrical activity in the myocardium. Confirmation of the rhythm is crucial in the management of Asystole. Transcutaneous pacing (TCP) is the electrical stimulation from pads placed on the body to stimulate heart contraction. This is indicated as an emergency temporary solution to improve asystole, a slow heart rate with inadequate cardiac output. The aim of this study is to identify the efficacy of transcutaneous pacing in Asystole management.

Method: Extensive literature search was carried out on the studies done on asystole from 1980 onwards. Ovid and Pub med search were done using asystole and transcutaneous pacing as keywords. Selection criteria were randomized controlled prospective studies with reasonable number of population. Six studies were selected based on the selection criteria.

Results: Three out of these studies were done in the emergency department and the other three studies were carried out in pre-hospital settings. All the studies done in pre-hospital setting came to a similar conclusion.

Conclusion: Out of hospital studies concluded that transcutaneous pacing does not improve the survival of the patient with asystolyic cardiac arrest. In-hospital studies basically concluded that TCP should be done early enough to have a satisfactory out come.

Discussion: Asystole occurs due to several underlying conditions. However, the studies done so far have not taken etiology in to consideration. Here we suggest designing a retrospective study conducting on inpatients that develop asystolic cardiac arrest and received TCP as a study group. The control group will be the patients who develop asystolic cardiac arrest but did not receive TCP. Further more this study will analyse the out come considering underlying condition which can be obtained from the medical records.

 

 

 

 

 


ROLE OF DIET IN GOUT

M Reddy, MD

Sinai-Grace Hospital, Wayne State University, Detroit, Michigan.

 

Background: Gout is a chronic crystal induced arthropathy with an incidence of 20-35/100,000. Hyperuricemia leading to the gouty attacks is controlled by the use of drugs to decrease the production or increase excretion of uric acid. A 21 percent increase in the risk of having gout has been associated with the intake of food having high purine content.

Methods: Several studies were analyzed from OVID Medline. Two trials were selected for this study as they were comparing clinical outcomes versus measuring metabolite levels. The first is a Prospective clinical trial involving 13 patients, the second is a Prospective cohort study involving 47,150 patients in the Health Professionals Follow-up study. Pearson’s Correlation analyses and Relative Risks were used for analysis of the data.

Results: In the first study there was a significant (p=0.002) reduction in number of Gouty attacks from 2.1 attacks per month to 0.6 in patients on a low purine diet for 16 weeks. In the second study it was shown that there was a 21% increased risk of gout with each additional serving of meat a day, and a 7% increased risk with one additional weekly serving of seafood. In this study they also showed that there was no increased risk with purine rich vegetable intake. In both these studies there was a significant decrease in incidence of gouty attacks with reduction of BMI/ body weight and alcohol consumption.

Conclusions: There is convincing evidence about the reduction in the incidence of gouty attacks with reduced consumption of alcohol and a reduction in BMI/ body weight. There is some evidence to show that meat, and seafood intake can cause an increase in number of gouty attacks. However there is no convincing evidence to prove that a reduction in purine rich vegetable intake has any effect on the incidence.

 

 

 

 

 


Uremic Enteropathy

M. Reddy, MD, K. Majekodunmi, MD, M. Sklar, MD

Sinai-Grace Hospital, Wayne State University, Detroit, MI

 

Background: Since the advent of hemodialysis, the long-term complications of uremia have been reduced. But there are rare instances of complications even on patients undergoing Hemodialysis. Among the many organ systems affected by uremia, gastrointestinal tract (GI) tract is most profoundly affected. Some of the clinical manifestations include anorexia, nausea, vomiting, metallic taste in mouth and some times bloody diarrhea1, 2. Studies done prior to the start of dialysis had shown changes in mucosa and submucosa including hemorrhage, ulceration, and pseudomembranous lesions in End Stage Renal Disease (ESRD) patients3, 4. 

Clinical Vignette: We report a rare case of a 33 yr old female with ESRD on hemodialysis for 9 yrs who was not shown to have any changes in the GI tract on Esophagogastroduedunoscopy (EGD) and colonoscopy did show remarkable changes in the small intestine on capsule endoscopy. The patient came to the hospital with complaints of nausea, vomiting and severe diarrhea associated with hematochezia for 24-hrs. Physical exam was unremarkable except for the presence of vascular grafts. The patient was scheduled for a capsule endoscopy, which showed multiple sites of hemorrhagic, polypoid folds. The mucosa had a mosaic pattern with absence of villi. These findings were suggestive of enteropathy most probably related to uremia.

Conclusions: There has been evidence of motility disorders of the esophagus as a result of ESRD induced neuropathy5, and hemorrhagic gastritis and hemorrhagic duodenitis6. There have not been enough studies to look at the remainder of the small intestine to note chronic changes as a result of ESRD. Our case here represents changes occurring in the small intestine as a result of ESRD. In ESRD patients with iron deficiency anemia with negative EGD and colonoscopy need further investigations by other means such as capsule endoscopy. Wireless capsule endoscopy offers an optimal approach for evaluating patients with obscure gastrointestinal bleed. Its main advantages are that it is noninvasive and permits examination of the entire small bowel, which is not always possible with other available methods like enteroscopy.

 

 

 

 

 


Hypothyroidism: A Risk Factor for Hepatocellular Carcinoma (HCC) in Patients with No Known Underlying Cause of Liver Disease

Arvind Reddy, MD, MPH Department of Medicine, Sinai Grace Hospital / Wayne State University

Chiranjeev Dash, Apinya Leerapun, Teresa A. Mettler, Linda M. Stadheim, Lewis R. Roberts

Mayo Clinic, Rochester, Minnesota

 

Background:

 Up to 25% of HCCs seen in US centers occur in patients with no known cause of liver disease. Most of these are thought to be due to non-alcoholic steatohepatitis (NASH). Hypothyroidism may lead to obesity, hyperlipidemia, insulin resistance and lipid peroxidation, which contribute to the pathogenesis of NASH. We conducted a case-control study to evaluate the relationship between hypothyroidism and HCC.

 Methods:

 Cases (n = 54) were HCC patients seen at the Mayo Clinic Rochester between Jan 1, 1999 and March 31, 2004 in whom no underlying etiology for chronic liver disease (alcohol, infectious, metabolic or autoimmune) could be determined. Two groups of controls were selected; HCC patients with hepatitis C (HCV)(n=57) and HCC patients with alcoholic liver disease (ALD)(n=49). Hypothyroidism was defined as a TSH level 5.0, a history of hypothyroidism prior to HCC diagnosis, or a history of being on thyroid hormone replacement at the time of HCC diagnosis. We used multivariate logistic regression to model the relationship between hypothyroidism and etiology of HCC.

 Results:

Compared to HCC patients with HCV or ALD, patients with HCC of unknown etiology were more likely to be females, non-smokers, have larger tumors, have advanced HCC at diagnosis, be symptomatic, and have a history of hypertension; and less likely to have cirrhosis. Of the 160 patients, 16 (10%) had a history of hypothyroidism. Of the 16 patients, 12 (67%) had no known etiology for HCC, 2 (11%) had HCV and 4 (22%) had ALD. As compared to HCC patients with HCV, patients with HCC of unknown etiology were more likely to have a history of hypothyroidism (OR: 6.4 , 95% CI: 1.3, 31.1). Patients with HCC of unknown etiology were also more likely to have a history of hypothyroidism than HCC patients with ALD, but the result was not statistically significant (OR: 2.4, 95% CI: 0.7, 8.5). When the two control groups were pooled, patients with no known etiology were 3.6 times more likely to have a history of hypothyroidism (95% CI: 1.2, 10.9). The results were adjusted for age, ge!

nder, BMI, smoking status, platelet count, tumor size, TNM stage, presence/absence of cirrhosis, surveillance for HCC, diabetes and hypertension.

 Conclusion:

 Hypothyroidism may play a role in liver carcinogenesis and should be further investigated as a potential risk factor. Early detection and treatment of hypothyroidism may reduce the incidence of HCC in patients with no other underlying cause of liver disease.

 

 

 

 

 


MDS and Pregnancy-Still an enigma

Tannu Sahay,MD(associate), Saad Usmani,MD (associate),Eisenberg Leopoldo, M.D., F.A.C.P.  Sinai-Grace Hospital/Wayne State University, Detroit, Michigan.

Introduction

   MDS is a hematological disorder with ineffective hematopoesis commonly seen in elderly patients with a median age at onset in the seventh decade. There are only rare cases of MDS reported amongst young pregnant patients. We report a case of such a patient.

Case Report

   Our patient is a 27-year-old G2 P1 Caucasian female who was referred at 30th week of gestation for further work-up of a macrocytic anemia and thrombocytopenia. Her past history was significant only for a spontaneous vaginal delivery at age 24 with no family history of any hematological disorder. She had significant pallor but no icterus, petechiae or splenomegaly. Her CBC revealed an Hb-9.5; Hct-28.1; MCV-110; RBC count-2.92; RDW-15.4; platelets-59,000. Bone marrow examination showed a hypocellular marrow (20-30% cellularity), macronormoblastic erythrocytogenesis with few dysplastic forms, 31% sideroblasts, decreased storage iron, decreased myeloid leukogenesis with orderly maturation and 0.9%myeloblasts. The M:E ratio was 1.5:1. No karyotypic abnormality was noted.

Discussion

MDS is an extremely rare condition to present during pregnancy. With extensive search of English literature, we could only find 6 reported cases. All were Caucasian females between the ages 30 to 42 years. Only two were in FAB class RAEB and the rest in RA. 4 of the 6 cases underwent leukemic transformations following diagnosis. Karyotypic abnormality was noted in only one patient with Iso (17) q abnormality. The association of MDS with pregnancy was thought to be coincidental by most authors. One of the patients underwent therapeutic abortion and no other adverse outcomes were reported. Survivals amongst these patients were from 1 month to as long as 5 years. There was no consensus regarding the treatment of MDS in these studies.Association of MDS during pregnancy should be recognized. Issues related to multiple transfusions, pregnancy outcome, severe anemia and IUGR might present with potential difficulties in the management of these patients. There are reasons be believe that there may be an increasing incidence of MDS in pregnant females especially with an ongoing trend towards childbirth in older females.

 

 

 

 

 


MYELODYSPLASTIC SYNDROME(MDS)AND PREGNANCY-STILL AN ENIGMA

Tannu Sahay,MD(associate), Saad Usmani,MD (associate),Eisenberg Leopoldo, M.D., F.A.C.P.  Sinai-Grace Hospital/Wayne State University, Detroit, Michigan.

 

Introduction

   MDS is a hematological disorder with ineffective hematopoesis commonly seen in elderly patients with a median age at onset in the seventh decade. There are only rare cases of MDS reported amongst young pregnant patients. We report a case of such a patient.

Case Report

   Our patient is a 27-year-old G2 P1 Caucasian female who was referred at 30th week of gestation for further work-up of a macrocytic anemia and thrombocytopenia. Her past history was significant only for a spontaneous vaginal delivery at age 24 with no family history of any hematological disorder. She had significant pallor but no icterus, petechiae or splenomegaly. Her CBC revealed an Hb-9.5; Hct-28.1; MCV-110; RBC count-2.92; RDW-15.4; platelets-59,000. Bone marrow examination showed a hypocellular marrow (20-30% cellularity), macronormoblastic erythrocytogenesis with few dysplastic forms, 31% sideroblasts, decreased storage iron, decreased myeloid leukogenesis with orderly maturation and 0.9%myeloblasts. The M:E ratio was 1.5:1. No karyotypic abnormality was noted.

Discussion

MDS is an extremely rare condition to present during pregnancy. With extensive search of English literature, we could only find 6 reported cases. All were Caucasian females between the ages 30 to 42 years. Only two were in FAB class RAEB and the rest in RA. 4 of the 6 cases underwent leukemic transformations following diagnosis. Karyotypic abnormality was noted in only one patient with Iso (17) q abnormality. The association of MDS with pregnancy was thought to be coincidental by most authors. One of the patients underwent therapeutic abortion and no other adverse outcomes were reported. Survivals amongst these patients were from 1 month to as long as 5 years. There was no consensus regarding the treatment of MDS in these studies.Association of MDS during pregnancy should be recognized. Issues related to multiple transfusions, pregnancy outcome, severe anemia and IUGR might present with potential difficulties in the management of these patients. There are reasons be believe that there may be an increasing incidence of MDS in pregnant females especially with an ongoing trend towards childbirth in older females.

 

 

 

 

 


Inpatient GI Prophylaxis and Comparison between different Pharmacological Agents

Zarnab Sajjad, M.D, Department of Medicine, Sinai Grace Hospital, Wayne State University, Detroit, MI

Research Question:

Like many physicians, we prescribe a PPI for Inpatient GI Prophylaxis more frequently then Sucrulfate or H2 blocker. Do PPI’S work better then H2 blockers or Sucrulfate in inpatient GI Prophylaxis?

Data Sources:

Study were identified by searching Medline (1998-2004), Journal reviews and

The following sources.

            Medline

            Pubmed

            Cochrane Library

Study Selection:

Randomized controlled trial and prospective, multi center, cohort study were identified by a Medline search termed “GI Prophylaxis”. Studies chosen focused to determine the risk factors associated with clinically important GI bleed and their prevalence in a heterogeneous group of population. 

Outcome Measures:

The data points chosen as out come measures in these studies were the overt and clinically important bleeding.

Results:

A randomized controlled trial was done on 287 patients, assigned to 4 groups (72 in omeprazole, 71 in famotidine, 69 in sucrulfate and 75 in control group). Number of days on ventilator, length of ICU stay or mortality played no significant role in statistics of bleeding. According to this study 3 out of 31 patients with coagulopathy bled (10%) compared to the patients without the coagulopathy, 4 out of 250 developed bleeding (2%), suggesting that presence or absence of coagulopathy had a more significant effect on bleeding rather than GI Prophylaxis. Another prospective, multicenter cohort study done by Cook (2002) on 2252 patients at four university affiliated hospitals (ICU) showed that 100 out of 674 patients on GI prophylaxis developed overt bleeding. Clinically significant bleeding occurred in 33 patients, 22 of which had a clinically important source of bleeding.

Conclusion:

Patients who are at low risk may not necessarily benefit from GI Prophylaxis. Among high risk individuals, patients with gastric and duodenal ulcers, gastric and esophageal erosion may benefit by routine prophylaxis. Studies suggested that gastric PH increasing medication like PPI would increase the risk of nosocomial pneumonia. All in all there was not enough data to support that PPI’s work better then any other medications for GI Prophylaxis.

 

 

 

 

 


Bleeding: An Unusual Presentation of Jejunal Diverticula

Zarnab Sajjad, M.D; Fazal Omar, MD; Kamal Nasser, MD; Manuel Sklar, MD

Department of Internal Medicine, Sinai-Grace Hospital, Detroit Medical Center, Detroit, MI

INTRODUCTION: Jejuno-ileal diverticula occur in only 1% of the general population. They are usually multiple, often asymptomatic, localized to the proximal jejunum. If symptomatic they are frequently associated with disorders of intestinal motility. We are presenting a case of bleeding jejunal diverticula.

CASE REPORT: An 82-year-old female presented to the hospital with melena of few days duration. Patient was hemodynamically unstable requiring aggressive fluid resuscitation. Her hemoglobin upon arrival to ER was 7.9 grams, baseline hemoglobin being 11 grams, so she received blood transfusions and was admitted to MICU. She had no history of similar prior episodes, no history of peptic ulcer disease (PUD), chronic liver disease or alcohol abuse. Patient continued to have frequent episodes of melena and was still hemodynamically unstable, so she underwent urgent Esophagogastroduodenoscopy (EGD) and colonoscopy. No source of bleeding was identified. Video Capsule endoscopy (VCE) was performed for evaluation of the source of obscure GI bleed.  VCE revealed multiple, actively bleeding diverticula in the jejunum. Due to continued blood loss, patient underwent laparotomy with bowel resection for actively bleeding jejunal diverticula. Patient recovered from surgery without any complications. Her bleeding stopped, and following postoperative recovery, she was discharged home in stable condition. Upon outpatient follow up, she was doing well without any further episode of bleeding and with stable hemoglobin.

DISCUSSION:  Jejunal diverticula are usually multiple, localized to the proximal jejunum. More than 80% are asymptomatic. Whenever symptomatic, the most frequent presentation is chronic abdominal pain with malabsorption due to bacterial overgrowth. Our patient denied any past medical history suggestive of malabsorption. Very rarely do patients have emergent complications, which include diverticulitis, perforation or bleeding. Inflammation, perforation and bleeding are much more commonly seen in colonic diverticular disease. We are reporting this case because of the very unusual presentation of jejunal diverticula with bleeding as a first manifestation without other complications. Review of the English medical literature did not reveal any other similar case report.

 

 

 

 

 


HEMOLYTIC ANEMIA AFTER MITRAL VALVE REPAIR

Shahid Z, MD, Associate, Gupta A, MD, Associate, Sahay T, MD, Associate, Appel J, DO, FACP

INTRODUCTION

Microangiopathic hemolytic anemia is commonly seen after mitral valve replacement. But it is an uncommon complication of mitral valve repair and when it happens it is considered a failure of the valve repair. We present a case of hemolysis after mitral valve repair.

           

CASE REPORT

A 54-year-old male with past medical history of HTN, CHF, Hodgkins disease in remission, Prostate cancer s/p radiation therapy, presents with progressive exertional dyspnea, chest pain and dark colored urine. Patient had undergone mitral valve repair approximately a month prior to this presentation for severe Mitral regurgitation with jet velocity of 4 cm. The surgery was done using 30 mm Carpantier-Edward physio ring as well as posterior valve resection for redundancy. Lab results showed Hemoglobin-6.3g/dl, Hematocrit-17.5%, Platelets of 346K/cumm, total Bilirubin-4.0mg/dl, indirect bilirubin 3.5mg/dl, LDH-2296u/l, haptoglobin<6mg/dl, creatinine of 3 mg/dl, negative Hemosiderin, negative coombs test, reticulocytes count of 221,840/cumm, 3+blood and 2-5 RBC in urine. The peripheral smear showed schistocytes and polychromasia. Transthoracic echocardiography showed moderate to severe mitral regurgitation .No other cause of hemolysis was identified. Patient underwent reoperation, approximately 3 weeks after presentation, showing dehiscence of the annuloplasty ring and paravalvular leak. The valve was replaced by St.Jude’s prosthetic valve and patient recovered from the surgery without complication. There was complete resolution of the hemolytic process after the surgery.

           

DISCUSSION AND CONCLUSION

This case is a unique presentation of early mitral valve repair failure. In the English literature 65 such cases have been reported showing development of hemolysis within 3-4 months. Yeo and associates observed that hemolysis after valve repair is due to hydrodynamic patterns associated with high shear stresses (fragmentation, collision, rapid acceleration) and low shear stress (free jet and slow deceleration). The mechanism in this instance was attributed to fragmentation of RBC due to regurgitant jets against dehisced annuloplasty ring. In patients with signs and symptoms of worsening CHF with low hemoglobin who have recently undergone mitral valve repair, the etiology may be undergoing hemolysis and warrant reoperation.

Reference: Hemolysis after mitral valve repair: mechanisms and treatment.

Ann Thorac Surg. 2004 Jan;77(1):191-5.

 

 

 

 

 


Role of Oral Hypoglycemic agents in Diabetes Mellitus type 2 patients after Diabetic ketoacidosis

Shahid Z, MD(Associate); Usmani SZ, MD(Associate).

Sinai-Grace Hospital/Wayne State University, Detroit, Michigan

 

Introduction: It is a general practice to send patients home on insulin regimen after an episode of diabetic ketoacidosis with eventual switchover to oral hypoglycemics.

Clinical Question: What is the evidence for the use of Oral hypoglycemics in

Diabetes mellitus type 2 status post-diabetic ketoacidosis?

Methods: Literature search was conducted utilizing Medline Ovid(1966-2004), Medline PubMed and Cochrane library with DKA, oral hypoglycemics and type 2 diabetes mellitus used as key words. Only double-blinded randomized controlled trials were to be selected.

Results: Only one relevant study meeting the above criteria observed use of oral hypoglycemics in African American patients who presented with severe hyperglycemia and DKA.  The study revealed that over a period of sixteen months, use of low dose sulfonylurea decreased the recurrence of hyperglycemia signifinicantly (p= 0.03), in patients post DKA from 60% to 14% when comparing with patients on diet alone. Assessment of beta-cell function showed improvement in basal and stimulated C peptide levels in patients with history of DKA and became comparable to patients with severe hyperglycemia (P<0.01).

Conclusions: This study was conducted on a select subset of the population and provided evidence that oral hypoglycemics decrease the incidence of hyperglycemic episodes post DKA. . It should be remembered that DKA occurs because of transient and reversible glucotoxicity of islets cell in patients with type two DM. Experts practices reveal a pattern of gradual transition of insulin to oral hypoglycemics , starting with secretogouges than adding other hypoglycemic agents with tapering of insulin over a period of time. Clear guidelines are strongly required. More randomized control studies are recommended to study the efficacy of different oral hypoglycemics agents against insulin in patients post DKA.

 

 

 

 

 


UPPER EXTREMITY DEEP VEIN THROMBOSIS: A REVIEW OF MANIFESTATIONS, COMPLICATIONS AND TREATMENT OPTIONS.

Srikanth Tamma, MD, Associate, Department of Internal Medicine, Sinai-Grace Hospital/Wayne State University, Detroit, Michigan.

Introduction: Upper extremity deep vein thrombosis (UEDVT) is defined as thrombosis of the subclavian, axillary, brachial or internal jugular veins. It accounts for nearly 2% of all cases of DVT. There has been a steady increase in the incidence of UEDVT with increasing use of central venous catheters and better imaging techniques.

Data Source: Observational studies and review articles were identified using Medline, Ovid and Pubmed databases.

Study Selection: Multiple studies were selected to look at the complications and complication rates of UEDVT. All were retrospective study designs. Review articles were selected to look at the treatment options available based on expert opinion. No randomized controlled trials were found.

Results: Edema or swelling of the involved upper extremity was the predominant manifestation of UEDVT in majority of the subjects. This was followed by pain, erythema, tenderness and palpable cord in decreasing order of frequency. More than 70% cases were associated with the use of central venous catheters. Nearly half of the cases were associated with some kind of malignancy. About a fourth of patients diagnosed ended up with complications of pulmonary embolism or post-thrombotic sequelae. Anticoagulation, limb elevation and use of compression arm sleeve is the mainstay of therapy for UEDVT. Mechanical thrombolytic therapy is considered if the patient is severely symptomatic. Other treatment options such as catheter directed thrombolysis, balloon angioplasty, suction thrombectomy, vein stenting, thoracic outlet decompression and use of superior vena caval filters have also been cited in the literature.

Conclusions: Edema of the upper extremity in a patient with central venous catheter should prompt a physician to exclude UEDVT with a venous duplex.

Further Research: Prospective studies of a larger number of patients with central venous catheters are needed. A meta-analysis of such studies would better characterize the problem. After taking ethical aspects into consideration, randomized controlled trials should be conducted to come up with a good treatment algorithm for patients diagnosed with UEDVT.

 

 

 

 


A RARE CASE OF ISOLATED GRANULOCYTIC SARCOMA PRESENTING AS CERVICAL ADENOPATHY

Srikanth Tamma MD (Associate), Arvind Reddy MD (Associate), Lawrence Lackey MD (Member). Department of Internal Medicine, Sinai-Grace Hospital/Wayne State University, Detroit, Michigan.

Introduction: Granulocytic Sarcoma, also known as Chloroma, is a rare extramedullary solid tumor composed of malignant immature cells of the granulocytic series. It has unique cytologic features independent of the site of the tumor.

Case Report:  Our patient is a 55-year-old African American gentleman with a known history of metastatic prostate cancer and pancreatitis, who presented with abdominal pain, nausea and vomiting. On neck exam, he was found to have 5 masses which were hard, mobile, painless, each measuring approximately 3x4 cm. There was no axillary lymphadenopathy and lungs were clear. Abdominal exam revealed distension, positive bowel sounds, diffuse tenderness without guarding or rebound. CBC and chemistry was within normal limits except for elevated alkaline phosphatase and AST. Peripheral smear was normal. Chest and Abdominal x-rays revealed increase in blastic disease of the dorsal spine and pelvis. A fine needle aspirate of a neck mass revealed numerous cells with increased nuclear/cytoplasmic ratio. The differential diagnosis was malignant lymphoma, poorly differentiated carcinoma or granulocytic sarcoma. Further analysis showed sheets of cells composed of myeloblasts, promyelocytes, myelocytes, mature neutrophils, numerous mitosis and occasional monocytes. Immunologic staining was positive for myeloperoxidase, lysozyme, CD68, synaptophysin and chromogranin. These findings strongly suggest a diagnosis of granulocytic sarcoma. The following stains were negative: pancytokeratin, leukocyte common antigen, vimentin, S100, CD3, CD10, CD20, CD34, and CD 43. These findings make lymphoma unlikely.

Discussion and Conclusion: Granulocytic sarcoma, also known as chloroma, is a rare extramedullary solid tumor composed of malignant immature cells of the granulocytic series. It usually occurs in patients with acute myelogenous leukemia. Isolated chloromas are rare. The diagnosis of granulocytic sarcoma can be very difficult when there is no demonstrable abnormality in the peripheral blood or bone marrow. Therefore histopathologic findings should be evaluated before a diagnosis of malignant lymphoma is pronounced. Granulocytic sarcoma has poor prognosis. In review of medical literature, we could find very few cases of isolated chloromas. One study showed that administration of intensive chemotherapy similar to that used to treat acute nonlymphoblastic leukemia, is associated with a significantly lower probability of developing acute myelogenous leukemia and prolongs survival.

 

 

 

 

 


Predictive Ability of Lymphocytic Bronchitis (LB) in Lung Transplant Recipients

Vibha Lama M.D, Member (1); Raghukumar Thirumala M.D, Associate (2); J. Mumford (1), S. Murray (1), A. Chang (1), G. Toews (1), M. Peters-Golden (1) and F.J. Martinez (1). (1) University of Michigan, Ann Arbor, MI and (2) Sinai-Grace Hospital, Wayne State University, Detroit, MI.

LB is considered a probable risk factor for development of bronchiolitis obliterans syndrome (BOS). The temporal relationship between LB diagnosis and fall in pulmonary function remains unclear. In 231 lung transplant recipients we examined the risk of BOS associated with a diagnosis and frequency of LB. In patients with LB (n=81), we examined the the positive predictive value (PPV) of this diagnosis over time.

Methods: Transbronchial biopsies (TBBx) were used to diagnose LB. BOS stages were determined on the basis of fall in FEV1 as defined by International society for heart and lung transplantation. Final diagnosis of BOS was made after excluding confounding variables including CMV infection, time to follow up.

Results: The median follow-up time was 5.4 years (95% CI, 4.6, 6.9). The median time from transplantation to stage 1 BOS was 2.27 years (95% CI, 1.84, 2.71). In uni- and multi-variate cox analysis, diagnosis of LB was found to be a significant predictor for development of BOS (hazard ratio [HR], 1.58; 95% CI, 1.06 2.35; p=0.02). Each additional TBBx with LB increased the hazard by 0.26 (HR 1.26; 95% CI, 1.11 1.43; p=0.0005). 53 patients were in BOS stage 0 and 12 patients were in BOS stage 0-p at the time of first episode of LB. The probability of meeting BOS1 PFT criteria within 1 and 2 years of diagnosis of LB was 0.34 and 0.64 respectively. The PPV was significantly higher in late onset LB (time from transplant to LB >90 days). Median time to meeting BOS1 PFT criteria in this group was 1 year (95% CI 0.82, 1.46) compared to 1.88 year (95% CI 1.48, 6.23) in the cases with early LB. 80% of cases with late and 53% of cases with early LB met BOS1 PFT criteria at 2 years after diagnosis of LB.

Conclusion: LB is a significant risk factor for development of BOS and the risk increases with number of biopsies positive for LB. Late onset LB is associated with shorter duration to decline in pulmonary function and portends a poorer prognosis.

 

 

 

 

 


Primary Cardiac Lymphoma Presenting as Pulmonary Embolism

 Anna Trostinskaia MD Associate, Joel Appel DO FACP, Manish Kesliker MS MD Associate,Geetha Krishnamoorthy MD Member

Department of Internal Medicine, Sinai-Grace Hospital, Wayne State University, Detroit

 

 

  Primary Cardiac Lymphoma (PCL) is non-Hodgkin’s Lymphoma involving only the heart and/or pericardium. It is extremely rare in immunocompetent patients. While secondary involvement of the heart is found in 16% to 28% of patients with lymphoma, PCL accounts for 1.3% of all cardiac tumors and 0.5% of all extranodal lymphomas. In most previously reported cases, clinical manifestations of PCL were nonspecific and included congestive heart failure, tamponade, arrhythmias, pericardial effusions, and nonspecific ECG abnormalities.

  We report case of a 65-year-old African American male who presented with complains of shortness of breath, easy fatigability, and intense right scapular pain of sudden onset. His previous medical history was remarkable for hypertension, smoking for 40 years, and 40 pounds weight loss during the past 6 months. He appeared well nourished, in no acute distress, HR 108, BP 151/73, and RR 20. He had bilateral crackles in middle to lower lung fields, muffled heart sounds, systolic murmur on left sternal border in 3-4 intercostal spaces, pedal edema without organomegaly or lymphadenopathy.  Lab findings were unremarkable. Patient’s symptoms prompted chest CT with contrast which gave initial impression of pulmonary embolus but on further examination appeared to be a large intracardiac mass confirmed with echocardiography as well. 

  Open heart biopsy showed right atrial and ventricular tumor with cardiomegaly, tricuspid valve involvement, and constrictive pericarditis. Further workup also revealed ascitis, splenomegaly, renal cyst, cholelithiasis. Immunohistochemistry demonstrated a high grade diffuse large B -cell lymphoma. Bone marrow biopsy indicated no lymphoma cells. The diagnosis of Primary heart non-Hodgkin’s Lymphoma Stage II B was entertained.  After 2 courses of chemotherapy CHOP and Rituximab patient achieved marked reduction of the tumor mass and is in a good health without signs of heart failure.

  To our knowledge this is the first case of PCL initially presenting as pulmonary embolism. This is a rare case as diagnosis was made antemortem. PCL remains a difficult diagnosis because of non specific presentation, as in our case with picture of CHF and PE. We believe that PCL should be a differential in any case presenting with signs of intrathoracic disease.

 

 

 

 

 


What is the Risk for Thromboembolic Phenomenon in a Patient with Dilated Cardiomyopathy?

 Anna Trostinskaia MD Associate, Department of Internal Medicine, Sinai-Grace Hospital, Wayne State University, Detroit

 

  Patients with Dilated Cardiomyopathy (DC) have multiple factors that predispose them to thromboembolic (TE) events.

     What is the risk of TE phenomenon in patients with DC? Medline/Pubmed/Ovid search of published studies was performed to find statistical data to analyze TE in DC patients. Seven small studies (5 retrospective and 2 prospective) and secondary analysis of 4 large prospective randomized trials were identified. None of randomized controlled studies evaluated TE events as a primary end point.

     Prevalence and incidence of TE events (stroke, systemic and pulmonary emboli) in patients with CHF due to DC varied widely from study to study with  the prevalence range 3 – 50% and incidence range 1.5 -3.5 / 100pt-years. One of the most significant confounders for TE risk evaluation in DC patients appeared to be nonrheumatic atrial fibrillation (AFib) (Crawford et all, 2004). One small study showed similar prevalence of TE events and mortality in patients with and without AFib (Polic et all, 1997).

      Another large study which included only patients in normal sinus rhythm has shown significantly increased risk for TE events in women compared with men (2.4/100 patient-years and 1.8/100 patient-years respectively) (Koniaris et all,1998). Moreover, risk of stroke appeared to be left ventricular ejection fraction (LVEF) dependent in women but not in men and each 10% decline in LVEF was significantly associated with 53% increased risk of TE in women. 

     Other large studies demonstrated inversed correlation between TE events risk and LVEF without gender differences (Sirajuddin et all, 2002).

     Conclusions: 1. The risk of TE events in patients with DC is poorly defined.

2. Studies of TE events risk in DC imply most important question to answer: Should all the patients with DC/CHF be anticoagulated?

3. Assuming insufficient data about TE events risk in patients with DC long term warfarin therapy can not be recommended until data are available from new upcoming trials. Presently, the only supported indications for anticoagulation in patients with DC are Afib, prior TE event, or Left Ventricular thrombus documented on imaging studies.

 

 

 

 

 


Warfarin Anticoagulant Therapy for Vascular Access Patency in Hemodialysis

Usmani SZ, MD (Associate), Shahid Z, MD (Associate)

Sinai-Grace Hospital/Wayne State University, Detroit, Michigan

 

Introduction:   Unlike other established indications for its use, warfarin to prevent AV graft clotting in hemodialysis patients is controversial with no clear guidelines.

Clinical Question:  What is the evidence and efficacy of Warfarin in preventing AV graft clotting in patients with ESRD on HD with no known hypercoagulable states?

Methods:   A literature search was done looking for double-blinded randomized controlled trials using keywords AV grafts, hemodialysis and warfarin. The search was conducted on Medline Ovid, Medline PubMed and Cochrane library.

Results:   We came across only one study on use of low-intensity warfarin for vascular patency in ESRD patients which showed that 73% patients allocated to Warfarin and 61% in the placebo group experienced graft loss (p=0.21; odds ratio in favor of placebo 1.76; 95% confidence interval 0.72 to 4.34). 5 patients in the Warfarin group had 6 major bleeding events as compared to none in the placebo (one-tailed Fisher exact test, p=0.03)

Conclusions:  Based on the evidence provided by this study, it can be said that the use of warfarin anticoagulation in end-stage renal disease population for prevention of thrombotic complications of AV access should be avoided due to increased risk of major bleeding events. There is a need for further studies on this topic to reach a decision on which agent(s) to use for anticoagulation purposes in these patients.

 

 

 

 

 


Angio-immunoblastic T-cell lymphoma in evolution- a Case Report

Saad Z.Usmani, MD (associate); T.Sahay, MD (associate) L.Eisenberg, MD, FACP.

Sinai-Grace Hospital/Wayne State University

Introduction

Angio-immunoblastic T-cell lymphoma (AITL), a rare disease which constitutes of 1-2% of the Non-Hodgkin’s Lymphomas, presents in middle-aged and elderly individuals. Evidence indicates that an abnormal immune reaction to an unknown antigen is a possible etiology. This creates an oligoclonal phase leading eventually to malignant monoclonal proliferation. The infectious agents associated with AITL primarily include EBV. Such a case is being presented.

Case Report

59-year-old Caucasian female presented with high-grade fever and diffuse lymphadenoapthy. Initial extensive in-hospital work-up for fever of unknown origin yielded negative results. Lymph node biopsy was suspicious for angio-immunoblastic T cell lymphoma with a non-diagnostic flowcytometry. Further follow-up revealed a pattern suggestive of Pel-ebstein fever but the adenopathy faded. Patient remained stable for 4-6 weeks but then the symptoms recurred in a more pronounced lymphadenoapthy. Lab results showed normocytic normochromic anemia, thrombocytosis and normal WBC count with variable eosinophilia. Serological studies showed CMV IgM-0.50, EBV VCA IgG-1.80, EBV VCA IgM-negative, EBV antinuclear antigen is more than 1:320.Tests for Toxoplasma, West Nile, Lyme, ANA were negative. CT scan documented recurrent diffuse lymphadenoapthy. At this point a second lymph node biopsy was done which revealed florid changes strongly suggesting AITL. Flowcytometry confirmed the cytogenetic abnormality.

Discussion and Conclusion

EBV exposure and latency of expression in AITL is well documented. This case is unique due to the waxing and waning pattern of lymphadenoapthy. Review of the current literature does not dictate any definite therapy. The prognosis and natural course of this disease is poor with classic chemotherapy protocol. The patient was therefore treated as Mantle cell lymphoma with HyperCVAD, achieving complete response at six weeks of therapy. Physicians should be wary of aggressive lymphomas presenting with diffuse lymphadenoapthy and fever when infections have been ruled out.

 

 

 

 

 


Gemcitabine and Taxotere in successful treatment of Primary pulmonary angiosarcomas-a case report

Usmani SZ, MD (Associate), Reisman D, MD (Member)

Sinai-Grace Hospital/Wayne State University; University of Michigan School of Medicine, Ann Arbor.

 

Introduction:

Angiosarcomas account for about 1% of all soft tissue sarcomas. Metastatic involvement of the lung is by far more common that primary involvement. Primary pulmonary angiosarcomas are aggressive tumors having high local recurrence as well as metastases with most patients dying within months of diagnosis. We present a case of primary pulmonary angiosarcoma that was treated successfully with chemotherapy.

Case Report:

A 56-year-old Caucasian man presented with hemoptysis. CT scan showed a left upper lobe mass and defined as Stage II non-small cell lung cancer. Biopsy via bronchoscopy showed undifferentiated high-grade sarcoma. Immunostaining was negative. The patient underwent left upper lobe resection and was found to have N2 nodal involvement, making him Stage IIIA. The histopathology of the resected mass was the same. Radiotherapy was planned after 2 months, but CT and PET scan showed interval development of bone metastases in left and right scapula, pelvis, lower thoracic and upper lumbar spine. The left scapula was biopsied and pathology of the tumor was consistent with an epitheliod angiosarcoma. The patient then received 2 weeks of radiotherapy to the lower back followed by chemotherapy with 6 cycles of Gemcitabine + Docetaxel therapy 2 out of 3 weeks. Follow-up CT scan and bone scan done afterwards showed no evidence of disease. FDG-PET scan was used for follow-up every 3 months. Recent PET scan has been negative.

Discussion & Conclusion:

Primary pulmonary angiosarcomas are very rare tumors, with only 9 reported cases of primary lung angiosarcoma in the English literature since 1966. Currently there are no treatment protocols for this tumor due to few cases. A recently published study* on soft tissue sarcomas has quoted a 43% response rate to gemcitabine and docetaxel combination. The present case is the first instance of primary pulmonary angiosarcoma responding to this combination with a sustained complete response for over 9 months. Repeated FDG-PET scanning has shown to impact the management of patients with soft-tissue and bone sarcomas with early detection of local recurrence.

References:

*Leu KM et al. Laboratory and clinical evidence of synergistic cytotoxicity of sequential treatment with gemcitabine followed by docetaxel in treatment of sarcoma. J Clin Oncol 2004; 22(9): 1706-12

 

 

 

 


Can Pneumcoccus Cause Post-Infectious Glomerulonephritis?

Saad Usmani, MD, Ramesh Kotihal, MD, Kamal Nasser, MD 

Department of Internal Medicine, Sinai Grace Hospital, Detroit Medical Center, Detroit, MI.

 

INTRODUCTION: Post-infectious glomerulonephritis is commonly seen as a complication of nephritogenic strains of Group A Streptococci, in particular, Streptococcus Pyogenes infection. Most commonly it is seen in pediatric population after acute pharyngitis, but it can present following skin infections as well as with organisms other than streptococci. Presentation in adult population is declining in developed countries. We present the second case of post-infectious glomerulonephritis caused by streptococcus pneumonia in an adult.

CASE REPORT: A 55-year male presented with a one week history of progressively worsening leg swelling, decreased urinary output and hematuria. Prior to this presentation, patient had recovered from pneumoccocal pneumonia with positive blood cultures. Initial lab results showed normocytic anemia and acute renal failure (creatinine rising from 1.1 to 3.5 mg/dl). Urine analysis showed hematuria and proteinuria (1200 mg /day). Patient’s renal function deteriorated and he became anuric requiring hemodialysis. Work-up for acute nephritic syndrome was negative for Anti-neutrophil cytoplasmic antibodies (ANCA) and glomerular basement membrane antibodies (anti-GBM). Complement studies revealed C3 = 109 (90-200); C4 = 29 (16-50); CH50 = 59 (100-300). Renal biopsy showed immune-complex mediated crescenteric glomerulonephritis with granular capillary loop florescence for IgG, IgA, C1, C3, C1q, kappa and lambda. Patient received pulse dose steroids for 5 days, which was followed by oral steroids. This therapy led to improvement of urinary output, and he was off dialysis on discharge.

DISCUSSION: This is a unique presentation of post-infectious glomerulonephritis secondary to pneumococcal pneumonia. Only one adult case and few pediatric cases have been reported in English literature. In the present case, other possible etiologies of nephritic syndrome were ruled out, with negative ANCA, Anti-GBM, Antistreptolysin O (ASO), Antinuclear Antibody (ANA), HIV and viral hepatitis profile. The low CH50 indicates a complement mediated injury. The histopathology is characteristic of post-infectious etiology. We are presenting this case to alert physicians about the possibility of post-infectious glomerulonephritis following pneumococcal pneumonia.

 

 

 

 

 


Kallmann syndrome in a female – an approach to treatment.

Gaurav Vashishta, MD; Zainab Shahid, MD; Gary W Edelson, MD; Kamal Nasser, MD

Kallmann syndrome is characterized by hypogonadotropic hypogonadism manifesting as delayed puberty associated with anosmia. It usually goes undiagnosed till late teens and is more common in males. Management of this disorder in females presents unique challenges.

Our patient is an 18-year-old caucasian lady who was diagnosed with Kallmann syndrome at age 15 and followed as an outpatient. She was seen initially for delayed puberty and short stature, found to have hyposmia. She had low FSH and LH levels, with other pituitary hormones as well as IGF 1, IGF 2 and IGF BP 3 levels being normal and a head MRI suggestive of empty sella. Radiological bone age lagged chronological age by 2-3 years. She was started on estradiol, which was switched to oral contraceptives a year later. At this visit she did not report any eating disorder, ran daily for exercise, denied any skin or hair changes, had no breast discomfort or discharge and was not sexually active. She was the shortest member of the family. Physical exam revealed a healthy looking well nourished female 5’2.5” tall, weighing 115 lbs with no visual field defects or thyromegaly. She had Tanner IV stage breast development. Axillary and pubic hair were normal. Cardiovascular, respiratory and abdominal exam were unremarkable. Patient expressed desire to conceive in the near future.

Kallmann syndrome is a rare disorder with a strong predilection for the male sex (5:1). Sporadic cases form the majority of cases, but X linked, autosomal dominant and recessive forms have been described. Treatment modalities for male patients include testosterone to attain sexual maturity, and hCG, with FSH if required, to induce spermatogenesis. In female patients, estrogens alone initially ensure proper breast and secondary sexual characteristics development, which are then followed by estrogen-progesterone combinations that bring about uterine maturity and establish menstrual cycles. Using exogenous gonadotropins or pulsatile GnRH therapy with adequate monitoring induces ovulation. Both options have been shown to be equally efficacious with 6 months of therapy resulting in reported pregnancy rates of 45%. Pulsatile GnRH has been argued to be a more physiological approach. This case presents the intricacies involved in treating female patients with Kallmann syndrome, with end points being to induce and maintain pubertal development and eventual fertility.

 

 

 

 

 


A Rare Case of Peritoneal Dialysis Associated Peritonitis Caused By Alcaligenes Xylosoxidans.

A.Virk, MD; K.Potluri, MD; K.Nasser, MD.

Department of Internal Medicine, Sinai-Grace Hospital, Detroit Medical Center, Detroit, MI.

 

INTRODUCTION: Peritonitis is one of the main reasons for morbidity and treatment failure in chronic Peritoneal Dialysis (PD) patients, aerobic gram-positive and gram-negative being the most common organisms. The incidence of peritonitis has progressively declined since the 1980’s and during the past decade, approximately 1 episode every 24 patient-treatment-months was routinely observed.

CASE REPORT: A 57 year old female with past medical history of ESRD, on PD for almost 4 yrs, presented in March, 2004 with abdominal pain, nausea and vomiting of one day duration. She was on temporary hemodialysis (HD) for one week prior to admission as her PD catheter was replaced due to malfunction. On examination, she was febrile with diffuse abdominal tenderness and the PD catheter site looked clean. Peritoneal fluid analysis showed 19,100 nucleated cells with cultures growing few Alcaligenes Xylosoxidans, sensitive to multiple antibiotics. The patient was started back on PD in hospital and discharged home on antibiotics. Few weeks later, she presented with similar complaints of fever and abdominal pain. Repeat fluid analysis revealed 1,830 nucleated cells with cultures positive for numerous Alcaligenes, sensitive to only a few antibiotics. She was started on temporary HD and PD catheter was removed. In September 2004, another attempt to start the patient back on PD failed due to recurrent peritonitis.

DISCUSSION: Our literature review revealed only 5 reported cases of PD associated peritonitis caused solely by Alcaligenes xylosoxidans. Alcaligenes is a motile, gram negative rod, easily confused with Pseudomonas and causes serious infections in immunocompromised hosts. It is associated with high mortality rate because of its virulence and resistance to multiple antibiotics. In addition to appropriate antimicrobial therapy, removal of the catheter is almost mandatory as this organism is adherent to plastic material. One aspect of our literature review and case report deals with the recurrent nature of peritonitis caused by Alcaligenes and whether patients should be allowed to return to PD after recovery.

 

 

 

 

 


Role of IV Steroids in Adults with Bacterial Meningitis

A.Virk, MD.

Department of Internal Medicine, Sinai-Grace Hospital, Detroit Medical Center, Detroit, MI.

 

Introduction: In childhood bacterial meningitis, adjuvant corticosteroids reduce risk of severe hearing loss by 58% and have been shown to help in reducing mortality and neurological sequelae. The aim of this project is to study the role of IV steroids in adult bacterial meningitis.

Methods: A comprehensive literature review was done using Ovid Medline and Cochrane Library databases. Keywords used were meningitis, bacterial meningitis, adult meningitis, dexamethasone, steroids. The results of relevant articles were then compared.

Results: Of the few relevant studies, this project reviews mainly two, one of which is a metaanalysis and the other is a multicenter randomized conrolled trial. The metaanalysis showed an absolute risk reduction in mortality of 10% in the steroid group when compared to the placebo group. Subanalysis for causative organism showed a statistically significant favorable relative risk of mortality in steroid group compared to placebo group in pneumococcal meningitis (RR=0.5;p=0.001). Even though the beneficial effect of steroids on neurological sequelae showed borderline statistical significance, including the subanalysis for causative organisms, the point estimates were below one in all groups. In the prospective multicenter randomized controlled trial, adjunctive treatment with dexamethasone reduced the risks of both an unfavorable outcome (RR=0.59;p=0.03) and death (RR=0.48;p=0.04) significantly. Overall treatment with dexamethasone did not result in an increased risk of adverse events.

Conclusion: IV dexamethasone has beneficial effect on mortality and unfavorable outcomes in adults with bacterial meningitis. A dose of 10mg IV dexamethasone Q6hrs can be given before or along with the antibiotics. Even though it does not increase adverse events, further studies on congnitive evaluation are needed. 

 

 

 

 

 


Methylobacterium Mesophilicum Causing Infectious Endocarditis

Durga Yerasuri, MD; Amer Tfaili, MD; Kamal Nasser, MD; Mark Wolf, MD

Department of Internal Medicine, Sinai-Grace Hospital, Detroit Medical Center, Detroit, MI.

 

INTRODUCTION: Methylobacterium mesophilicum is a methylotropic gram-negative rod with natural habitat in vegetation, sewage and soil. This bacterium has been increasingly reported as a cause of sepsis and line infections in immunocompromised patients. We report a case of sepsis and infective endocarditis with methylobacterium mesophilicum in a 68 year old woman with ESRD. To date this is the first case of infectious endocarditis caused by methylobacterium mesophilicum.

CASE REPORT: A 68 year old women with history of Hepatitis C and ESRD, receiving dialysis through a permacatheter presented with fever and chills. Patient had multiple admissions in the past with infected vascular accesses due to klebsiella and stenotrophomonas maltophila. On this admission, patient had a temperature of 101.4 and a WBC count of 12000. She was started on vancomycin and tobramycin empirically pending blood cultures. Blood cultures grew methylobacterum mesophilicum susceptible to tobramycin and ciprofloxacin. Echocardiogram showed mitral valve vegetations. The permacatheter was removed and vancomycin was replaced by ciprofloxacin. Patient later became afebrile and subsequent blood cultures showed no growth.

DISCUSSION: Methylobacterium mesophilicum is increasingly reported as a cause of

bacteremia in immunocompromised patients. Most of the reported infections were nosocomial. Exposure to soil and vegetation was reported in some cases. Infections included infected hemodialysis access catheters, pneumonias, septic arthritis, osteomyelitis, keratitis, uveitis, encephalitis, and peritonitis. The bacteria were successfully isolated from blood, sputum, bronchial collections, synovial fluid, bone marrow, corneal scrapings, CNS, spleen, and liver. The only reported outbreak of infection in a hospital was linked to contamination of bronchoscopes, endoscopes, and endoscopy washers. The bacterium is resistant to UV rays, Gamma radiation, dessication, and hydrogen peroxide. Our patient represents the first case of infective endocarditis complicating a Methylobacterium mesophilicum  bacteremia.

CONCLUSION: With the newer effective treatments for cancers and AIDS and with the increased use of central veins as vascular access in chronic debilitated patients, the incidence of rare opportunistic infections has increased. The increased use of endoscopic procedures and contamination of these instruments with resistant organisms is also a concern. This presents a challenge to the physicians and diagnostic laboratories to look for unusual opportunistic infections.

 

 

 

 

 


EFFICACY OF NON-PHARMACOLOGICAL MODALITIES TO TREAT DEMENTIA

Rama Nadella, MD, Associate, Dept of Internal Medicine, Sinai Grace Hospital, Wayne State University, Detroit, MI

BACKGROUND :Gingko Biloba (Gingko) has been used medicinally for over one thousand years. Ginkgo represents one of the most studied and commonly used herbal remedies for Dementia. Vitamin E has been tried in the treatment of Dementia as it is an anti-oxidant scavenging toxic free radicals.

DATA SOURCES : Medline database from 1985 – 2004 using PubMed and Ovid.

SEARCH TERMS : Non-pharmacological, Dementia, Vitamin E, Gingko, Treatment of Dementia

STUDY SELECTION : Only Randomized controlled trials (RCT) selected.

Gingko: RCT by Le Bars (N-309) and Metaanalysis conducted by Le Bars using only well conducted RCT(s) since 1989 (Eg: Le Bars, Kanowski, Maurer and Hofferberth analyses) were studied to see whether Gingko works over Placebo in cognitive disorders, and if it works, how safe is it in treatment of Dementia.

Vitamin E: Sano 1996 is a double blind randomized trial (N-341), which showed effect of a-tocopherol compared with Placebo in the treatment of Alzheimers disease.

OUTCOME MEASURES:     

Gingko => Outcome evaluation was done at 12, 26 and 52 weeks, wherein higher the score poorer the function.

1.         ADAS – Cog (to assess memory, language and orientation)

2.         GERRI (to assess daily living and social behavior)

3.         CGIC (to assess general psychopathology)

Vitamin E => Survival time to the first of four end points

1.         Death

2.         Institutionalization

3.         Severity of Dementia

4.         Loss of 2 out of 3 basic activities of daily living.

RESULTS:      

Gingko: The primary outcome measures were analyzed using intent-to-treat analysis showing a significant improvement in ADAS-Cog score and GERRI score with a p-value < 0.05. No difference was seen in CGIC score.

Vitamin E: There appeared some benefit from Vitamin E with fewer participants reaching end point (only 58%), showing a Peto odds ratio of 0.49 (95% CI 0.25 – 0.96). Moreover, there were excess of falls in Vitamin E group compared to Placebo.

CONCLUSION: Gingko is safe and appears capable of improving cognitive performance and social functioning of Demented patients for 6 months to 1 Year.There is insufficient evidence of efficacy of Vitamin E in the treatment of dementia.

 

 

 

 


MDS and Pregnancy-Still an enigma

Tannu Sahay,MD(associate), Saad Usmani,MD (associate),Eisenberg Leopoldo, M.D., F.A.C.P.  Sinai-Grace Hospital/Wayne State University, Detroit, Michigan.

Introduction

   MDS is a hematological disorder with ineffective hematopoesis commonly seen in elderly patients with a median age at onset in the seventh decade. There are only rare cases of MDS reported amongst young pregnant patients. We report a case of such a patient.

Case Report

   Our patient is a 27-year-old G2 P1 Caucasian female who was referred at 30th week of gestation for further work-up of a macrocytic anemia and thrombocytopenia. Her past history was significant only for a spontaneous vaginal delivery at age 24 with no family history of any hematological disorder. She had significant pallor but no icterus, petechiae or splenomegaly. Her CBC revealed an Hb-9.5; Hct-28.1; MCV-110; RBC count-2.92; RDW-15.4; platelets-59,000. Bone marrow examination showed a hypocellular marrow (20-30% cellularity), macronormoblastic erythrocytogenesis with few dysplastic forms, 31% sideroblasts, decreased storage iron, decreased myeloid leukogenesis with orderly maturation and 0.9%myeloblasts. The M:E ratio was 1.5:1. No karyotypic abnormality was noted.

Discussion

MDS is an extremely rare condition to present during pregnancy. With extensive search of English literature, we could only find 6 reported cases. All were Caucasian females between the ages 30 to 42 years. Only two were in FAB class RAEB and the rest in RA. 4 of the 6 cases underwent leukemic transformations following diagnosis. Karyotypic abnormality was noted in only one patient with Iso (17) q abnormality. The association of MDS with pregnancy was thought to be coincidental by most authors. One of the patients underwent therapeutic abortion and no other adverse outcomes were reported. Survivals amongst these patients were from 1 month to as long as 5 years. There was no consensus regarding the treatment of MDS in these studies.Association of MDS during pregnancy should be recognized. Issues related to multiple transfusions, pregnancy outcome, severe anemia and IUGR might present with potential difficulties in the management of these patients. There are reasons be believe that there may be an increasing incidence of MDS in pregnant females especially with an ongoing trend towards childbirth in older females.