Solitary Brainstem Abscess: MR Imaging Features of an Unusual Condition
Ruchika Jain,(Associate) Department of Internal Medicine, Sinai Grace Hospital, Rajan Jain, Division of Neuroradiology, Department of Radiology, Henry Ford Health System, Detroit, MI
Background: Solitary brainstem abscess is an uncommon condition associated with high mortality. Its differentiation from a neoplasm can be done with MR imaging as an abscess usually has characteristic features. We present MR imaging features of a case of large brain-stem abscess, treated successfully by surgical drainage. Case Report: We present here a 59-year-old male who initially presented with about two weeks of headaches, nausea, and vomiting. He developed left arm weakness and left facial pain. MRI of the brain revealed a large peripheral ring enhancing lesion involving the medulla oblongata extending inferiorly up to the cervico-medullary junction. The walls of the lesion were very smooth and central cystic/necrotic part showed restriction of water diffusivity on diffusion-weighted images, suggesting an abscess. The patient underwent a suboccipital craniectomy for evacuation of the brain abscess. Cultures from the pus showed staphylococcus hominis. Post-operatively he continued to have residual left side weakness along with nystagmus and diplopia. He was treated with vancomycin and Flagyl intravenously for four weeks. Follow up MRI done after 6 weeks showed complete resolution of the brainstem abscess. Discussion: Brainstem is a rare site of an abscess formation. MR imaging, especially diffusion-weighted imaging (DWI) can be diagnostic and can help differentiate it from a neoplasm. Neoplastic lesions usually show solid, irregular, heterogeneous enhancement in case of a ring enhancing lesion as compared to an abscess which usually shows smooth, thin, enhancing wall. Diffusion-weighted images show restriction of water diffusivity, seen as bright signal on DWI in the central necrotic part, diagnostic of an abscess. Neoplasms usually don't show bright signal on DWI in the central necrotic part of the lesion. Surgical drainage of a brain-stem abscess is indicated when medical therapy fails. Proper anatomical knowledge of the brain-stem and the selection of appropriate surgical approach is important for safe drainage of the abscess. Conclusion: MR imaging can help differentiate an abscess from a neoplastic lesion in the brainstem which is an unusual site for an abscess formation. DWI shows restriction of water in the central necrotic part of the abscess; with thin, smooth enhancing walls seen on post-contrast images.
A Case Report on Idiopathic Esophageal Ulceration in Acquired Immunodeficiency Syndrome
Chadi Saad, MD, Associate;
Shaik Mohammed Abu Fazal, MD, Associate. Department of Medicine,
Introduction: Esophageal ulceration is a common and important cause of morbidity in patients with acquired immunodeficiency syndrome (AIDS). After known causes are excluded, a subgroup remains with unexplained esophageal ulceration, known as idiopathic esophageal ulceration (IEU). Case Report A 44 year old woman with a past medical history of HIV diagnosed 12 years ago, was evaluated for progressive odynophagia and dysphagia. The patient was not compliant with her medications, and had stopped HAART therapy one year ago. She complained of a fever, epigastric pain and difficulty in swallowing, which was progressing over the past week. On examination she had diffused oral trash up to the back of her throat involving the tongue. Her lab results revealed that her CD4 count was 45, viral load was more than 100,000. The patient was treated for oral candidiasis with fluconazol and nystatin, but she didn't show any improvement with this treatment. Upper GI endsocopy revealed multiple punched out giant ulcers involving the distal half of the esophageous with normal study for stomach and proximal part of small bowels. The Histopathologystudy showed acute inflamation with fibropurulent exudate. CMV and HSV were negative on immunostain and also no fungi were detected on GMS stain. The patient was then started on oral prednison. After the start of steroid therapy the patient showed significant improvement in her symptoms and at the time of her discharge she was able to tolerate oral diet. She was sent on a 4 week course of steroid therapy. Discussion: The pathogenesis of Idiopathic Esophageal Ulcers is still unclear. It has been found in some patients that electron microscopy demonstrated retroviral-like particles at the ulcer site. Further studies documented HIV-1 glycoprotein (gp41) antigen within mononuclear cells of esophageal ulcers in AIDS and also showed that squamous epithelial cells in the esophagus in patients with IEUs undergo apoptosis. Apoptotic cells were absent in control tissues and in tissue samples from ulcerations of known etiology, but abundant in the edges and bases of lesions and sloughed-off tissues in IEUs. Thus, they proposed a role for apoptosis in the pathogenesis of IEUs by a mechanism involving HIV-associated bystander killing of uninfected mucosal cells. Potential factors in development of these ulcers include: high-titer viremia, inflammatory cytokine stimulation, and altered GI immunopathophysiology. These ulcers do not respond to empirical antiviral or antifungal therapy, but do respond to immunosuppressive drugs, suggesting that that they are immune mediated. The current therapy of IEU includes corticosteroids or, less frequently, thalidomide, although no placebo-controlled trials have been reported. Some other retrospective studies showed some effect with the treatment of misoprostol and viscous lidocaine.
Is CA-125 A Reliable Screening Tool For Ovarian Cancer?
Elena Jdanova (Associate), Department of Medicine, Sinai-Grace Hospital, Detroit Medical Center, Wayne State University, Detroit, Michigan.
Is CA-125 A Reliable Screening Tool For Ovarian Cancer? Introduction: Ovarian cancer is the leading cause of gynecological cancer deaths and the fifth leading cause of cancer deaths in women. Epithelial ovarian cancer is usually described as the "silent killer" because 70% of cases present with stage III-IV disease. With the discovery of CA125 monoclonal antibody, there is an increased interest in screening for the early stages of ovarian cancer where survival rate is 90%. Methods: Using the search terms, "CA 125 in ovarian cancer screening", "screening for ovarian cancer" the Cochrane library and Medline were searched for English- language publications that provided information regarding positive predictive value, specificity and sensitivity of the CA 125 test. Only Randomized, Prospective cohort, Retrospective note audit and Case-control studies were selected. Search Results: 16 articles were found that met the selection criteria. Study Results: The apparent sensitivity of the CA 125 test ranged from 53% to 89%, with the specificity ranging from 72% to 100%. Positive predictive values were too low to justify routine screening with CA 125 (3.7%-4.1%) alone. No randomized controlled trials exist to show whether screening with CA 125 decreases mortality from ovarian cancer or not. Conclusion: CA 125 can not be used as a screening test alone but shows promise as a screening test that may be used in conjunction with other screening tests such as transvaginal US or ovarian palpation. Epidemiologic studies have identified age and a family history of ovarian cancer as the most important risk factors. These risk factors could be used to identify subpopulations of patients who would receive the greatest potential benefit from screening.
Is pharmacologic therapy for elevated triglycerides beneficial in reducing the risk of developing coronary artery disease?
Saimabanu Mansuri, MD,
Associate, Sinai-Grace Hospital, Detroit, MI
Introduction: Hypertriglyceridemia may be part of hyperlipidemia syndromes, or secondary to certain metabolic disorders, medications, alcohol, etc. Studies have previously proven that elevated triglycerides are an independent risk factor for coronary artery disease (CAD). Objective: To understand if treating elevated triglycerides (TG) reduces the risk of CAD. Study Methods: An Ovid and Pubmed search of the medical literature was conducted using the key terms hypertriglyceridemia, triglyceride, coronary artery disease, coronary atherosclerosis. All double blind randomized controlled trails published in English were reviewed and relevant studies were identified. Most of these studies had used fibric acid derivatives (gemfibrozil, and bezafibrate) for intervention. Results: One study showed the sub-group of patients with the highest risk of CAD were patients with an LDL:HDL ratio of >5:1 with TG >204. These patients had a decrease in coronary events by 70% when treated with gemfibrozil. Another study showed a 40% reduction in CAD events in patients with TG >200 when taking bezafibrate. Two other studies showed reductions in TG levels and a decrease in progression of atherosclerosis studied by angiography. Conclusion: It is evident that elevated TG co-exists with other conditions that elevate CAD risk, such as decreased HDL-C and diabetes. Due to lack of a drug that only affects TG, alterations in other lipid profiles were also reported. The studies were able to show a decrease in CAD risk, however, if that was attributable to decreasing TG levels is yet to be determined. Thus, hypertriglyceridemia alone is probably not an appropriate therapeutic target because we have no trials directly addressing the question of weather lowering TG level reduces the number of CAD events. Evidence for the benefit of treating this lipid disturbance remains less clear than that for treating elevated LDL-C. Further studies are required to evaluate the benefit of reducing elevated TG.
THROMBOCYTOPENIA AND RINGED SIDEROBLASTS IN A PATIENT WITH 5Q DELETION
Sonali Sud MD, Associate; Preeti Misra MD, Associate; Leopoldo Eisenberg MD FACP, Clinical Associate Professor of Medicine, Wayne State University; Department of Internal Medicine (Hematology/Oncology), Sinai Grace Hospital, Detroit, MI
Introduction 5q- Syndrome is a myelodysplastic syndrome characterized by refractory macrocytic anemia, normal to high platelet counts and preserved WBC counts. Neither thrombocytopenia nor ringed sideroblasts are usually found in association with it. A case series presented by Mathew et al of 43 patients with 5q- reported 7% had refractory anemia with ringed sideroblasts (RARS), and 18% had platelets <150,000. Thrombocytopenia with 5q- has been reported in 6 children by Pitman et al. Here we report a previously unreported association of 5q-, RARS and thrombocytopenia. Case Report A 76 year old female with history of breast cancer 14 years back treated with combination chemotherapy (Adriamycin/Cytoxan), CHF, and atrial fibrillation was referred for new onset thrombocytopenia. At that time, blood work revealed a platelet count of 63,000. Past records showed a platelet count of 156,000. Physical examination revealed no pallor, icterus, ecchymosis, petechiae or splenomegaly. CBC revealed WBC count of 3900, hemoglobin 8.4, MCV 88.9 and platelet count 55,000. Peripheral smear showed decreased WBCs, anisopoikilocytosis, decreased platelets and giant platelets. A bone marrow aspiration and biopsy was done to evaluate this evolving pancytopenia. Bone marrow was hypercellular, with dyserythro, dysmegakaryopoeisis, 13% ringed sideroblasts, and no increase in blasts. Cytogenetics revealed a predominant 5q-, along with some other consistent chromosomal abnormalities. Discussion This case brings to our attention many new facts in association with what is known about MDS. First, the presence of thrombocytopenia and ringed sideroblasts with 5q-syndrome does not fit with the current classification of MDS. Also, treatment with alkylating agents, which this patient underwent, can predispose to developing 5q-. The question arises of whether such treatment may impact the blood picture in patients that do develop 5q-. Another thought is do patients who have complex chromosomal abnormalities vs. those with an isolated 5q- have a predisposition to present with such a picture? Finally, the question arises how these factors impact response to treatment with lenolidomide, and the prognosis. Further research in this complex field of myelodysplastic syndrome is definitely warranted, and therefore recommended.
Eustachian valve endocarditis: An unusual presentation of right sided endocarditis
Naga VA Kommuri, MD, Associate, MALINI VENKATRAM, MD, Associate, R.Rajagopal, MD, Member, RAJIKA MUNASINGHE, MD, Department of Internal Medicine, Sinaigrace HOSPITAL/Detroit Medical Center, Detroit, MI
Introduction: Endocarditis of the right side of the heart is commonly associated with intravenous drug use, congenital heart disease or indwelling central venous lines. Eustachian valve [EV] is an endocardial fold localized between the inferior vena cava and the right atrium to direct the blood flow in the fetal circulation from the vena cava through the foramen ovale into the left atrium. Eustachian valve endocarditis is extremely rare and only 17 cases are reported in literature so far. There are no prior reports of EV endocarditis following AICD placement. Case report: A 42 year old African American Male presented two months after Coronary artery bypass surgery and AICD placement with lethargy, fever, abdominal pain and diarrhea. On examination, he had a fluctuant swelling without signs of inflammation around the AICD site suggestive of seroma. Initial work up revealed leucocytosis with neutrophilia and the serial blood cultures showed methicillin sensitive staphylococcus aureus (MSSA). Endocarditis was suspected and a trans- esophageal echocardiogram was obtained which showed vegetation on the Eustachian valve. Bacteremia persisted despite suitable antibiotic treatment. The source was suspected to be AICD which was removed and subsequent culture from the tip of AICD leads showed MSSA with similar sensitivities confirming the source for endocarditis. After treating with antibiotics he improved clinically and the blood cultures became negative. Discussion: The EV is an embryological remnant of the sinus venosus. EV endocarditis is characterized by clinical signs and symptoms of right-sided infective endocarditis in association with infective vegetations. The duration and the choice of antibiotics are similar to tricuspid endocarditis. The role of antibiotic prophylaxis and surgery in EV endocarditis is unknown. Conclusion: Endocarditis of the EV is a rare and probably under diagnosed entity. With the increase in the use AICD devices, clinicians should be aware of the possibility of EV endocarditis following AICD lead infection and consider TEE early to evaluate for this complication.
Contrast Induced Nephropathy: Incidence and Preventive Measures; An evidence Based Review.
Abhijit Saste MD. Department of Medicine, Wayne State University/Detroit Medical Center-Sinai Grace Hospital
Introduction: Contrast Induced Nephropathy (CIN) is the 3rd most common cause of hospital-acquired ARF (11% Incidence & 14% Mortality). Identifying patients at risk and implementing preventive measures remain the most important factors in treatment of CIN. In this study, we performed an evidence based review to identify the incidence of CIN in patients with pre-existing renal insufficiency (baseline Createnine>1.5mg%) as well as the proven measures to prevent CIN. Methods: A Pubmed and Cochranre review identified more than 300 trials dealing with incidence and prevention of CIN. After applying limits for prospective, randomized, clinical trials between 2001 and 2007 with sample size>200, 90 trials were seleced. Results: A)Serum creatinine is not a reliable predictor of CIN.A 24-hour Creatine clearance pre-contrast is more accurate where a value of < 80 mL / min has 81% sensitivity for predicting CIN. Cystatin C peaks at 24hrs and serves as an early marker of impending CIN. B) A dose< 8cc for LOCM (Low Osmolar Contrast Media) was found to be safe in CKD. C) Intravenous contrast was safer than Intra-arterial contrast. D) In case of multiple contrast procedures a 72hr interval was recommended between procedures. E) Different hydration solutions have variable efficacies in prevention of CIN: Na-bicarb (more efficacious)> 0.9%Saline> 0.45%Saline.When combined with NAC(N-acetyl cysteine), Na Bicarb withNAC did not offer significant benefit over NAC+0.9%Saline.Recommended dose for Na-Bicarb was 154 mEq /L in D5W (3 mL/kg/hr for 1 hr before & 1 mL/kg/hr for 6 hrs after the procedure) & for 0.9% NS was 1 mL /kg/hr for six to 12 hours before and after the procedure. D) NAC use has conflicting results. Most trials advocate its use. Dose recommended is 600mg p.o.BID a day before & on contrast administration. E)In emergency contrast studies, an IV bolus of 5 ml/kg/h of 154 mEq/l of Na-bicarb in D5W plus 2,400 mg of NAC in the same solution over 1 hr pre-contrast was useful. Theophylline 200mg IV 30 min pre-procedure and Vitamin C 3g -2 hrs pre- procedure and 2 g in the night and the morning post- procedure were also found to be beneficial, but theophylline is underused due to fears of toxicity. F) Statin pre treated patients had a lower incidence of CIN. G) Peri-procedural Hemofiltration reduces CIN and dialysis requirment in severe CKD (S Creatinine > 5 mg %). Conclusion: CIN is overlooked in most patients. There is enough evidence based on our review to support recommendations regarding type and dose of contrast, pre-procedure hydration and antioxidants in the case of emergency procedure.
Recurrent Pneumothorax associated with Invasive Pumonary Aspergillosis in HIV patient
Ruchika Jain, MD (Associate) Wasif Hafeez (Member) Department of Medicine, Husain Saleh M.D Department of Pathology, Sinai Grace Hospital Detroit, Michigan.
Introduction : Invasive Aspergillosis is a significant cause of morbidity and mortality in severely immuno-compromised hosts. Isolated cases of invasive pulmonary aspergillosis have been reported in patients with AIDS. Although it is not an AIDS-defining infection any more, It is an important and commonly fatal complication of advanced HIV disease. We report a case of invasive aspergillosis presenting as recurrent pneumothorax in an AIDS patient with history of COPD. Case Report: A forty-eight year old male with history of hypertension, COPD, HIV and recent pneumonia presented to the emergency department with progressive dyspnea, and left sided pleuritic chest pain . Chest x-ray revealed left tension pneumothorax which was treated with chest tube insertion. However due to persistent chest tube air leak and worsening pneumothorax, he underwent left thoracotomy and stapling of apical bullae and wedge resection of the lingular segment. GMS staining for fungus revealed a focus of fungal hyphae present in an area of intense inflammation and morphologically resembling aspergillus species. He was started on Voriconazole and discharged to home. One week later he presented again to the hospital with similar complaints. Chest X-ray revealed recurrent left-sided tension pneumothorax. He again underwent chest tube insertion and subsequent left pneumonectomy. GMS stains performed on multiple sections showed no evidence of fungal organisms or PCJ infection. Discussion: Spontaneous pneumothorax in HIV patients is usually associated with Pneumocystis jeroveci pneumonia , pulmonary tuberculosis or bacterial pneumonia. Invasive aspergillosis also remains an enigmatic disease that occurs predominantly among immuno-suppressed patients including those with HIV infection and can rarely present with pneumothorax, pneumo-mediastinum and pneumo-pericardium. Concurrent predisposing factors for Aspergillus infection among HIV patients include granulocytopenia, PCJ, CMV, bacterial and /or other fungal pneumonia, underlying chronic lung conditions, use of broad spectrum antibiotics , chemotherapy and steroids . .Diagnosis of invasive pulmonary aspergillosis is usually done by BAL cytology and or culture, Tracheal, trans -bronchial or open lung biopsy, sputum examination, thoracentesis or serum antigen detection .Early diagnosis and aggressive antifungal treatment is recommended Surgical intervention can be tried in certain patients However mortality rate remains high despite recent advances in therapy.
Efficacy of MRI for breast cancer screening in high risk women
Ramchandani H, Munasinghe R, Khalid N, Sinai Grace Hospital, Wayne State University, Detroit
Introduction- Almost one third (32%) of all cancers diagnosed in women is breast cancer. Breast magnetic resonance imaging (MRI) has been proposed as an additional screening test for young women at high risk of breast cancer in whom mammography alone has poor sensitivity. Method- We conducted the review of literature to assess the effectiveness of adding MRI to mammography in screening this population. Results - We found consistent evidence in 6 studies that adding MRI provides a highly sensitive screening strategy (sensitivity range: 77-100%) compared to mammography (16-40%). This leads to incremental cancer yield that varies from 8.2 - 24.7 cancers/1000 screening rounds in different studies. Specificity with MRI (range: 81-99%) in 6 studies was less than mammography (range: 93-99%) and suggested a 3-5 fold higher risk of patient recall for investigation of false positive results. 1 study compared the characteristic of tumor detected by MRI with 2 age matched control group screened by mammography. Tumors < 10 mm in diameter were significantly greater in surveillance group (43.2 percent) than in either control group (14.0 percent [P<0.001] and 12.5 percent [P=0.04], respectively. No studies assessed as to whether adding MRI reduces patient mortality. Conclusions about the effectiveness of MRI therefore depend on assumptions about the benefits of early detection from trials of mammographic screening in older average risk populations. The extent to which high risk younger women receive the same benefits from early detection and treatment of MRI detected cancers has not yet been established
UNUSUAL PRESENTATION OF LARYNGEAL CARCINOMA.
Naushaba Khalid,MD
Associate,Naga V Kommuri,MD, Associate,
Head and neck tumors presenting as neck abscesses is quite rare. Because head and neck cancers accompanied with a neck abscess are rare, it may be difficult to consider the possibility that cancer lies behind the neck abscess at the time of first examination. We report a case of 37 yr old AAM who presented with a history of hoarseness, dysphagia and left sided neck swelling. On examination he had diffuse, tender, warm swelling in the anterior part of neck with left side being involved more than right. Initial investigations revealed that the thyroid functions tests are normal, and he was treated as subacute thyroiditis but patient returned with increased swelling . A soft tissue radiograph of the neck showed deviation of trachea to the right and also the presence of soft tissue cystic mass in the neck anterior to and involving the region of the thyroid cartilage predominantly below the level of the hyoid. CT of the neck shows mixed cystic and solid mass involving the isthmus and left thyroid gland and continuous with the laryngeal mass that is involving the left vocal cord and supraglottic region. The patient had a panendoscopy showing a large exophytic, infiltrative lesion in the supraglottic larynx primarily on the left side extending over the anterior commissure to the right blocking the true cords. The biopsy of vocal cords and anterior neck mass was performed which revealed Supraglottic squamous cell carcinoma and the neck mass was found to have acute inflammatory infiltrate [abscess] without any evidence of malignancy. Staging showed that it is a T3 N2A M0. I & D of abscess was performed and then he received chemotherapy and radiotherapy This case highlights the need to be aware of unusual lesions that may arise in the region of the thyroid. The presentation of this lesion as a thyroid mass is an unusual occurrence that serves to remind the clinicians that in the differential diagnosis of thyroid masses one must include the possibilities of abscess or direct extension of a primary neoplasm from another organ.
Risk Factors for Burnout and Frustration During Residency Training
Niraj Parikh, MD, Associate,
Melhem Solh, MD, Associate, Saimabanu Mansuri, MD, Associate, Rashed Khazi Syed,
MD, Associate,
Introduction: Studies have found burnout to be associated with decreased job performance, low career satisfaction, and stress-related health problems. Increased burnout has been linked to work hours in a previous study by Wayne State University. We hypothesize that work hours is not the sole factor for burnout in residency. Hence, the purpose of this study was to assess the frustration and burnout level amongst residents in the department of Internal Medicine at Sinai-Grace Hospital, and if present, to find potential risk factors. Methods: A Standardized survey was distributed among 38 residents in internal medicine in different stages of training. The burnout survey was adopted from Maslach Burnout Inventory invented by Dr Christina Maslach. The survey had 15 questions that assess three factors: depersonalization, emotional exhaustion and lack of personal accomplishment. Participants were asked to rate the frequency of such feelings on a scale ranging from "not at all" to "very often". Data was also gathered concerning age, marital status, training level, previous training and satisfaction with education received. Statistical analysis was done using SPSS. Results: 38 residents participated in the study, of which 62% reported not receiving enough teaching by the supervising faculty which was inversely related to the burnout score. 67% of the residents were at significant risk of burn out. Prior residency training, marital status, and number of children were not related to the burnout risk. Conducting research and sports correlated to a lower burnout risk and private attendings caused the most frustration compared to teaching faculty, or chief residents. Discussion: The risk of burnout was significantly high, especially amongst the PGY2 level and lowest amongst the PGY1 level. This contradicts other published data where the risk of frustration was highest among interns; however, most of the studies were not specific to internal medicine residents. A similar study was carried out at the University of Washington where they reported that 76% of their residents were at risk of burn out. Unfortunately none of the factors analysed gained statistical significance and further surveys, based on feedback by residents, will be used to assess other risk factors.
Tranformation of a long standing Essential thrombocythemia to Polycythemia Rubra Vera.
Dr Abhijit Saste MD, Associate. Dr Preeti Misra MD, Associate. Dr Leopoldo Eisenberg FACP, Clinical Associate Professor. Dept. of Medicine: Sinai Grace Hospital, Wayne State University, MI.
Case Report: A 63 year old male on routine physical and laboratory evaluation in 1997 was found to have a platelet count of 535,000/cmm. His WBC at that time was 10,800/cmm and RBC 5.32 million/cmm. Reactive thrombocytosis was ruled out. A bone marrow biopsy confirmed the diagnosis of essential thrombocythemia and the patient was started on Aspirin. The patient's clinical and hematologic course remained stable over the next 10 years.In 2005 with the arrival of JAK-2 analysis the patient was found to exhibit a mutation in the same. A PRV-1 gene analysis was not done then. In 2007 on routine physical and laboratory evaluation the patient was found to have a WBC of 23100/cmm, RBC of 6.38 million/cmm, Platelets of 667,000/cmm & Hematocrit of 54.1%.His serum EPO level was 2units(low). All secondary causes of polycythemia were ruled out.His physical exam revealed a marked splenomegaly.A BMB showed trilinear hyper proliferation, low reticulin fibrosis and exhausted marrow iron, all consistent with a diagnosis of PRV. Conclusion: ET usually runs an indolent course. Rarely, (5.3%cases) ET with JAK 2 V617 + subtype and/or PRV-1 + has been shown to later develop into a PRV. Thus JAK 2 V617 + ET should be monitored with careful laboratory and clinical follow-up for the development of PRV. Whether we can imply that JAK 2 V617+ ET thus has a poorer prognosis than the JAK 2 V617 counterpart calls for further study into the long term course of the JAK 2 V 617(-) subtype.
Purpura Fulminans in Septicemia-An Association with underlying Protein C &S Deficiency.
Ruchika Jain (Associate), Rashad Syed Khazi (Associate), Wasif Hafeez (Member) Department of Internal Medicine, Sinai Grace Hospital Detroit Michigan.
Introduction : Presenting with skin discoloration, fever, and septic shock, purpura fulminans is a rare syndrome of hemorrhagic infarction of the skin, extremity loss, and intravascular thrombosis that can progress rapidly to disseminated intravascular coagulation, vascular collapse and death . The majority of the patients are children, but adults can be affected . The most common infectious agents associated with purpura fulminans are Neisseria meningitides, Hemophilus influenzae, Streptococcus pneumoniae and Varicella zoster . We present a case of Purpura fulminans in association with severe sepsis due to poly microbial infection with Enteroccus faecalis and E coli . Case report: An eighty-three year old female with history of Squamous cell carcinoma of right lower extremity, Oglivie syndrome, and stroke was admitted with change in mental status ,fever ,hypotension, and tachycardia. Blood cultures were positive for Vancomycin resistant Enterococcus faecalis and ESBL producing Escherichia coli. Pt was started on Meropenam and Linezolid . On fifth day of admission patient developed extensive irregular areas of skin necrosis with rim of surrounding erythema with mild tenderness. Protein C activity was found to be 41% and protein S activity was 58%.D-dimer levels were 24.40 and fibrin monomers were also present Pt was diagnosed to have Purpura fulminans with underlying protein C&S deficiency. Discussion: Purpura fulminans is an uncommon disorder first described by Guelliot in 1884.It can be identified in three clinical conditions.1)In patients with pre existent abnormality of protein C or protein S anticoagulant pathway; 2)In patients with acute severe bacterial infections called acute infectious purpura fulminans; and 3) in patients without known abnormalities of protein C pathway or acute infections ,termed idiopathic purpura fulminans. Sepsis induced purpura fulminans is associated with high mortality due to multiple organ dysfunction. Necrotic lesions usually progress to distal ischemia, and skin grafting and limb amputation are often required .Early antibiotic administration and intensive care management are crucial to patients survival. Adjuvant therapies against inflammatory and coagulation cascades and promoting fibrinolysis are still controversial .Since Purpura fulminans is a syndrome, not a specific disease; therefore treatment should be aimed towards underlying infection with ongoing supportive management.
Response to imatinib mesylate in a patient with idiopathic hypereosinophilic syndrome
Ramchandani Harsha MD, Associate; Solh Melhem MD, Associate; Robin Adam Medical student, Department of medicine, Sinai Grace Hospital, Wayne State University, Detroit, MI
INTRODUCTION Hyper Eosinophilic syndrome (HES) is a heterogeneous group of rare disorders characterized by sustained and otherwise unexplained overproduction of eosinophils with organ involvement and consecutive dysfunction. Differential diagnosis between the HES and the related chronic eosinophilic leukemia (CEL) relied on the identification of signs of clonality that allowed, when present, the reclassification of patients as CEL. Treatment for CEL and a subset of HES, positive for the FIP1L1/PDGFRA mutation remains the same, however the prognosis varies. When either one is complicated by eosinophilic lung deposits at presentation, it becomes more challenging to differentiate the diagnosis from chronic eosinophilic pneumonia which has a different treatment. CASE REPORT A 50 yr old African American female with multiple admissions to the hospital for shortness of breath, cough and urticaria in the last 7 years was readmitted for the same symptoms. Cough was nonproductive, not associated with fever or chills. No weight loss or night sweats reported by the patient who has a non-significant past medical and past surgical history. Blood labs showed absolute eosinophil count of 21.0 K/ cumm which was not in response to any allergen as a complete allergy workup failed to identify any precipitating factor. She had an elevated alkaline phosphatase, tryptase and IL-6 consistent with a myeloproliferative process. Chest X Ray showed interstitial markings which may relate to interstitial pneumonitis and focal consolidation in the posterior right lower lobe likely representing pneumonia. Bronchoscopy showed bilateral nodular infiltrates with eosinophilia. Flow cytometry and FISH provided CD3-/CD4+ predominance cells. Molecular testing was positive for the FIP1L1/PDGFRA mutation.Based on the above work up, patient was diagnosed as Hypereosinophilic syndrome with myeloproliferative features and was started on imatinib. Patient responded to the treatment with Imatinab at dose of 100mg/ day. Eosinophil count dropped to 10.2k/cumm.The cough and other disease features were subsequently suppressed by the tyrosine kinase inhibitor, imatinib. DISCUSSION Idiopathic hypereosinophilic syndrome remains a serious condition with a poor prognosis for most patients, unless treated. Multiple case series have demonstrated efficacy of Imatinab in treating patients with FIP1L1/PDFGRA-associated HES with clinical, hematological, and molecular remission documented in the majority of patients. Response rates are very high, with rapid resolution of clinical symptoms and normalization of eosinophil counts generally occurring within one to two weeks. Because of the marked sensitivity of this condition to imatinib therapy, identifying these patients is also now of considerable clinical importance as this group of patients are not responsive to steroids, the treatment of choice in HES without myeloproliferative features.
Can beta-blockers be prescribed to cocaine abusers in acute coronary syndromes?
Niraj Parikh, MD, Associate, Department of Internal Medicine, Sinai-Grace Hospital, Detroit, Michigan
Introduction: The 2005 National Survey on Drug use and health reported approximately 33.7 million Americans aged 12 and older have used cocaine at least once. Cocaine is well known for its association with acute coronary syndromes. The use of beta blockers in these instances has been debatable for fear of unopposed alpha agonist actions of cocaine. Objective: To determine if beta-blockers should be prescribed to cocaine abusers with acute coronary syndromes. Study Methods: An Ovid and Pubmed search of medical literature was conducted using the key terms Adrenergic Beta-Antagonists, Propranolol, Metoprolol, Atenolol, Esmolol, Carvedilol, Coronary Artery Disease, Myocardial Infarction, Hypertension, Congestive Heart Failure, Cocaine, Cocaine related disorders, and Crack Cocaine. All studies published in English including case reports were reviewed. The drugs studied were propranolol, labetalol, esmolol, and carvedilol. Results: Two similar randomized control studies evaluated the effect of propranolol and labetalol with cocaine use. They not only measured variables such as heart rate, blood pressure, coronary sinus blood flow but also conducted cineangiography of the left coronary artery. Both studies showed potentiation of vasoconstriction by beta blockade. A case series of 7 patients was also conducted and did not recommend using esmolol for cocaine induced cardiotoxicity. A retrospective cohort study was conducted on patients who were given beta-blockers, but subsequently had positive urine drug screens to cocaine and concluded, administration of beta-blockers may offset the risk of coronary artery vasospasm. Other case reports showed varied results. Conclusions: The theoretical risk of unopposed alpha agonist actions of cocaine when beta blockers are used in cocaine abusers is well recognized. From the evidence that is available, the use of beta-blockers in cocaine induced acute coronary syndromes remains unclear. Further observational and subsequent clinical trials are needed to clarify if beta-blockers are warranted in this situation.
RENAL TUBULAR ACIDOSIS TYPE-1 AND NEPHROLITHIASIS IN A PATIENT WITH SARCOIDOSIS
Rao Ali Hashim Khan, MD, Associate; Rama Nadella, MD, Associate; Irfan Omar, MD, Member, Department of Medicine, Sinai Grace Hospital, Detroit Medical Center, Detroit, MI.
Introduction: Sarcoidosis is a chronic, multisystem disorder of unknown cause. All parts of the body can be involved, but the organ most frequently affected is the lung. Involvement of the skin, eye, liver, and lymph nodes is also common. Nephrolithiasis occurs in approximately 1 to 14 % of patients with sarcoidosis, but association of renal tubular acidosis (RTA) with sarcoidosis is unusual. Case Report: This is a 45 years old african american female with past medical history of sarcoidosis diagnosed 17 years ago. Over the course it was complicated by blindness, ventilator dependant respiratory failure and nephrolithiasis. This time patient presented with shortness of breath of two days duration, left flank pain radiating to groin and was found to have left renal calculi and diagnosis of renal tubular acidosis (RTA) type-1 was made based on following laboratory values: serum bicarbonate of 15 mMol/L, serum chloride 115 mMol/L, serum calcium 9.5 mMol/L, serum anion gap of 16, urine pH of 5.5 to 6.0, urine anion gap of +44, urine calcium of 20.3 mg/dl. Patient's vital signs and physical examination were unremarkable. Chest X-ray was negative. Both ultrasound and CT-scan of kidneys were negative for nephrocalcinosis. Discussion: Sarcoidosis is known to be associated with both nephrolithiasis and nephrocalcinosis but only nephrocalcinosis is a known etiology of RTA. Hence, in our patient we suspect association of sarcoidosis with RTA type-1, in the absence of nephrocalcinosis. Nephrolithiasis in this case could be secondary to both sarcoidosis and distal RTA. This case suggests that it is important to identify and treat distal RTA to prevent recurrence of renal calculi in patients with sarcoidosis. This patient was treated with sodium bicarbonate and her acidosis improved with serum bicarbonate reaching above 20 mMol/L. We expect that treatment of RTA type-1 will certainly slow if not halt the progression of nephrolithiasis in this patient with active sarcoidosis.
A rare case of spinal, pelvic and long bone involvement in osseous sarcoidosis
Olobatoke A.O, Associate,Venkatram M. Associate, Department of Medicine, DMC/Sinai Grace Hospital, Detroit Michigan.
INTRODUCTION Sarcoidosis is a multisystemic non caseating granulomatous disease. Documented osseous involvement is about 5%. This typically affects the phalanges, ribs and skull in a lytic pattern. Sclerotic lesions however are rare and have predilection for the spine when they occur. CASE REPORT A previously healthy 38yr old African American man presented with a one year history of weight loss, night sweats, radicular back pain, limping and no loss of rectal sphincteric tone. A differential diagnosis of myeloma, lymphoma and tuberculosis were considered after physical examination. Radiological work up included X-rays of the spine and extremities, CT scan of the chest and MRI of the spine. Increased density of lumbar spine as well as diffuse signal abnormality in lumbar, sacrum, paraspinal and presacral structures with epidural extension was noted. Mediastinal and paratracheal lymph nodes were biopsied and returned negative for malignant cells. Serum protein electrophoresis was negative for monoclonal immunoglobulin and HIV ELISA was negative. Interestingly, whole body bone scan revealed increased activity in both right and left tibia appearing as bilateral cortical lytic lesions on MRI and prompted a tibia biopsy which showed a non caseating granulomatous inflammation, negative for malignant cells, acid fast bacilli and fungi. DISCUSSION Published literature has described dactylitis particularly in the middle and distal phalanges as the commonest manifestation of bone sarcoidosis. This pattern was however absent in this patient who presented with sclerotic lesions of the spine, pelvic bone and lytic lesions of the long bones mimicking multiple myeloma. Vertebral and long bone sarcoidosis is rare and diagnostically challenging entity since it may mimic either lytic or blastic neoplastic lesions, particularly metastases from primary sites like lung or breast cancer. CONCLUSION This case reiterates the diagnostic challenge posed by sarcoidosis due to its variable presentation. A combination of radiological and tissue biopsy is highly recommended to arrive at a diagnosis since radiographic findings alone are non specific. Despite the fact that osseous sarcoidosis is rare, a high index of suspicion is needed for timely detection since it portends an aggressive and poor prognosis in such patients.
VALUE OF PROPHYLACTIC ANTIBIOTICS IN PROSTATE BIOPSY
Sonali Sud MD, Associate, Sinai Grace Hospital, Detroit Medical Center, Detroit, Michigan
Background: Prostate cancer is the second most common cancer in the United States. The lifetime chance of being diagnosed with prostate cancer is 1 in 6. Diagnosis currently entails a prostate biopsy, with the most common method being TransRectal Prostate Biopsy (TRPB). The overall risk of infectious complications of TRPB is reported to be less than 1% and can range from asymptomatic bacteruria to urinary tract infection and sepsis. There are currently no guidelines on the use of antibiotic prophylaxis prior to biopsy. This review was conducted to evaluate the effectiveness of antibiotic prophylaxis in reducing the risk of infectious complications following TRPB. Materials and Methods: A literature search using Medline (1950 to August week 1, 2007) was performed using the search terms: Prostate biopsy, Prostatic biopsy, Antibiotic Prophylaxis and Prophylactic Antibiotics to find all Randomized Controlled Trials that compared prophylactic antibiotic use to placebo or no treatment. Results: We found three studies that evaluated the role of prophylactic antibiotic therapy prior to TRPB, but different endpoints and antibiotics were used in each study. All of the studies demonstrated a significant reduction in the incidence of bacteruria following TRPB, with the number needed to treat (NNT) ranging from 4.7 to 20. There were no significant differences in the efficacy of the different antibiotics. Conclusions: This review suggests that the use of antibiotics, either fluoroquinolones such as ciprofloxacin/ofloxacin, or TMP-SMX before prostate biopsy may help prevent infectious complications. However, keeping in mind the overall low rate of infectious complications associated with the procedure, the benefits need to be weighed against the risk of increasing antimicrobial resistance before recommending this practice.
CLINICAL IMPLICATIONS OF INCIDENTAL LEFT BUNDLE BRANCH BLOCK (LBBB)
Naga VA KOMMURI MD,ASSOCIATE;
Introduction: Electrocardiogram has been one of the most common diagnostic tests in routine clinical practice. As a result incidental LBBB in asymptomatic patients is becoming more frequent, raising clinical questions and concerns about the significance of this finding. Clinical Question: What is the risk of cardiovascular related problems in asymptomatic patient with incidental LBBB on routine electrocardiogram? Methods: Data Source:Literature search was performed using the following search engines: Ovid, Pubmed, and Cochrane. Only case control and cohort studies were included since there are no randomized controlled studies to date. Key search words were LBBB, asymptomatic, incidence, prevalence, complications, clinical approach. Results: We found fifteen studies relevant to the clinical question. Based upon these studies the incidence of LBBB is between 0.16%-1%. Incidence increases with age [Imanshi et al]. Incidental LBBB is a marker of slowly progressive degenerative disease of heart [Errikson etal]. There is increased risk of developing cardiovascular disease [21 % in isolated LBBB vs. 11% in controls, p=0.04] and mortality [p=0.001] related to cardiac causes in this group of people [Fahy et al]. LBBB leads to abnormal ventricular activation causing asynchrony and global cardiac abnormalities [Grines etal]. People with incidental LBBB have a compromised long term follow up comparable with conventional cardiac risk factors [Miller etal]. In this subset of population there is increased risk of AV block and coronary death. The clinical consensus in the management of incidental LBBB is further evaluation using Holter ECG and echocardiogram. Based upon these results patients can either be followed up or further evaluated using EP study and Myocardial Perfusion imaging. Interventional options based upon these results being Coronary angiography, Permanent pacing [Francia etal]. Conclusion: Incidental LBBB is not a benign finding. Presence of isolated LBBB denotes a high risk patient for cardiovascular complications. Asymptomatic LBBB is underestimated and patients have a compromised follow up similar to the patients who have conventional cardiovascular risk factors. LBBB is associated with increased mortality due to cardiac causes..