2005 National ACP Abstract Submissions

 

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.

COPAXONE INDUCED PLEURAL EFFUSION AND HILAR LYMPHADENOPATHY

V. Nayak, MD, Associate, D. Obeid, MD, Associate, L. MacDonald, MD,

Sinai Grace Hospital, Detroit Medical Center, Wayne State University,

Detroit, Michigan

 

Copaxone or Glatiramer Acetate is an immunomodulatory agent used in the treatment of Multiple Sclerosis (MS).  The drug has been shown in controlled clinical trials to reduce the frequency of relapses in MRI-defined disease activity and burden in relapsing –remitting MS. Most common side effects in patients on copaxone include injection site reactions, vasodilatation, depression, dizziness, dyspnea, and urticaria. This case report is to demonstrate the manifestation of pleural effusion and hilar lymphadenopathy secondary to copaxone.

 

 

We report  a 28 year old African-american female who is a nursing home resident with history of MS maintained on copaxone 20 mg SC once daily since March 2003 who was admitted to the hospital in August 2003 with a one week history of progressively worsening shortness of breath. She was found to have a right pleural effusion as well as hilar lymphadenopathy.  Additionally, soft tissue fullness was now evident in the subcarinal region that may have represented additional lymph nodes. The pleural effusion was found to be exudative with a predominance of macrophages. The possibilities of Tuberculosis, malignancy, granulomatous diseases and infections were considered. A thoracoscopic biopsy was done which showed focal perivascular chronic inflammation mainly composed of lymphocytes. No atypical cells or granulomata were identified. Therefore, we concluded that these findings are drug-induced secondary to Copaxone.

 

 

Copaxone has been shown to cause lymphadenopathy, mainly localized to draining lymph nodes. Biopsy findings on reported nodes in the literature were similar to that found in our patient. However, we present this case to demonstrate unilateral pleural effusion and hilar lymphadenopthy as a hitherto undocumented manifestation of copaxone.

 

SYNDROME OF INAPPROPRIATE SECRETION OF ANTI-DIURETIC HORMONE (SIADH) CAUSED BY ADENOCARCINOMA OF THE COLON

R Kotihal, MD, Associate,  M Ghaffari, MD, Associate, and O Alzohaili, MD,

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

 

SIADH has many etiologies including ectopic production of anti-diuretic hormone (ADH) by lung cancer or other neoplasms, eutopic release by various diseases or drugs, and exogenous administration of ADH or its analogues. Among neoplastic producers of ectopic ADH, we know pulmonary tumors, sarcomas, breast tumors and brain tumors are common.

 

We report a rare case of adenocarcinoma of the colon with the classic features of SIADH. A 76-year-old-man who complained of abdominal pain and constipation, had guaiac positive stools. At the time of admission, he was found to be hyponatremic with a normal blood pressure and euvolemia. Laboratory examination showed serum sodium of 124 mEq/L, plasma osmolality 257 mosm/kg, urine sodium 83 mEq/L and urine osmolality of 268 mosm/kg. The liver function tests, cortisol levels, TSH and imaging studies of the head and the lungs were found to be normal. The hyponatremia was too early to be caused by the facial trauma, which occurred one hour prior to the admission. Colonoscopy revealed a mass at the rectosigmoid junction with more than 90% obstruction. Partial colectomy was performed and pathologic analysis of the mass revealed a moderately differentiated adenocarcinoma. Three days following the surgery, an initially difficult to correct hyponatremia was found to be within the normal range.

 

A review of literature revealed only two other cases of adenocarcinoma of the colon presenting with SIADH. We report the third such case. 

 

SIADH can be a paraneoplastic manifestation of colon carcinoma and this case underlines hyponatremia as one of the presenting symptoms of a colonic malignancy. After having ruled out the obvious causes of SIADH, a diagnostic colonoscopy could be included in the search for ectopic causes.

 

 

CASE REPORT OF ISOLATED HYPOPARATHYROIDISM

Jayasree Grandhi, MD, Associate, Fazal Omar, MD, Associate, O Alzohaili, MD,  Dept. Of Medicine, Sinai-Grace Hospital, Wayne State University, Detroit, Michigan.

 

Introduction: Hypoparathyroidism is a state of inadequate parathyroid hormone (PTH) secretion where ionized calcium in the extra cellular fluid decreases. The causes of primary hypoparathyroidism are diverse representing disruptions of one or more steps in development and maintenance of PTH secretion.

 

Case Report: We describe a case of 68 yr old middle-eastern female who came in with tingling of her fingers and toes, hand tremors, palpitations and muscle cramps of her legs for the past 3 years. Past medical history was strikingly negative for any neck surgery or irradiation of neck. Physical examination was significantly positive for chvostek’s and trousseau’s sign. Labs: Ca 4.5, P 6.5, Mg l.4. Albumin, activated vita D3, 24 hr urine Ca , TSH, prolactin and cortisol levels were all within normal limits but PTH was inappropriately low <5pg/ml (Normal PTH 11-65 pg/ml ).  Patient was diagnosed with primary isolated hypoparathyroidism.  Patient was started on calcium and vitamin D replacement therapy and was told to wear an alert bracelet. She follows up regularly and continues to do well.

 

Discussion: Primary hypoparathyroidism can be due to wide variety of causes. It is most commonly due to damage or removal of parathyroid glands during neck surgery like thyroidectomy or following removal of parathyroid adenoma for primary hyperparathyroidism or following neck irradiation. It can be seen as a part of autoimmune polyglandular syndrome, which is characterized by the presence of at least two of the following: candidiasis, hypoparathyroidism and Addison’s disease. Parathyroid deficiency can occur form developmental defects like DiGeorge syndrome or as a result of damage from heavy metals, granulomatous diseases. A very rare cause of hypoparathyroidism is primary isolated hypoparathyroidism where it is seen as isolated entity and not due to any of the causes as mentioned above the cause of which is exactly not known. Our patient was also thus diagnosed with isolated hypoparathyroidism.

 

Conclusion: The most common cause of hypoparathyroidism is acquired like post surgical. Isolated hypoparathyroidism is a diagnosis of exclusion and is a rare entity. It should be as a part of differential diagnosis in a patient coming with hypocalcaemia secondary to hypoparathyroidism. The index of suspicion should be high in patients who have not had any neck surgery or irradiation. In our literary search we could not find any case reports in USA in last 10 years.

 

 

 

NON-FUNCTIONING ANAPLASTIC ADRENOCORTICAL CARCINOMA PRESENTING AS CARCINOMATOSIS.

Raghukumar D. Thirumala M.D, Associate; Apurva Motivala M.D, Associate; and Manesh Kottapuram M.D, Member.

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

 

Adrenocortical carcinoma is a rare, aggressive tumor usually affecting young children and adults. Approximately 60% of tumors are hormone producing on presentation. Non-functioning anaplastic tumors are heralded by symptoms of local invasion by tumor or by metastasis. Most common sites of metastasis are peritoneum, lung, liver and bone.

 

We present a rare case of Non-functioning adrenocortical carcinoma presenting as carcinomatosis. The patient is a 34-year-old female, who presented with headache, neck pain, difficulty walking and tingling left arm of 2 days duration. No signs of neurological deficits or excessive steroid secretion were present. Medical history was significant for SLE, seizures secondary to lupus cerebritis, and asthma. Examination was significant for orthostasis, guaiac positive stools, and staggering gait. Labs were significant for a hemoglobin level of 2.5gm/ dl. Upper GI endoscopy revealed two bleeding gastric masses. A work up for ataxia with a non-contrast CT scan revealed a 2-3cm mass in the left occipital region. Subsequently, MRI brain revealed mass occupying lesions in both the cerebellar hemispheres with a 2cm lesion in the posterior left parietal area. CT thorax revealed a large mass in the right paratracheal region with mass effect on the airways and heart. CT abdomen revealed heterogeneous mass in right adrenal gland with lymphadenopathy in the porta and left paraaortic region. USG of pelvis demonstrated enlargement of the ovaries with cystic features. Autopsy revealed a large adrenal tumor with a large retroperitoneal hematoma and disseminated metastasis to the lungs, liver, pancreas, ileum, gastric wall, peritoneum, ovaries, and brain. Immunohistochemical stains were negative for vimentin, S100, synaptophysin, CK20 but positive for CK7, keratin, EMA and focally positive for HCG and PLAP, consistent with epithelial tumors.

 

At present, there is no pathognomic immunohistochemical profile for adrenocortical carcinoma. Negative neuroendocrine tumor markers may rule out the possibility of a malignant pheochromocytoma. This case highlights certain key features in the diagnosis of adrenocortical carcinoma. First, lack of specific immunohistochemical profile fails to localize the primary site of the tumor. Second, carcinomatosis secondary to adrenocortical carcinoma has rarely been reported.

 

 

SIGNET RING CELL CARCINOMA OF UNKNOWN PRIMARY WITH DIC: A CASE REVIEW

Amer Tfaili MD, Associate, Darshana Tawde MD, Associate, Geetha Krishnamoorthy MD, Member, Sinai-Grace Hospital, Wayne State University, Detroit, Michigan.

 

A 62-year-old African American female was hospitalized with a 1-month history of weight loss and a recent history of abdominal pain. Physical examination revealed a distended tender abdomen with ascites but without any jaundice. There was cyanosis of the 3rd and the 4th toes bilaterally. Laboratory evaluation revealed the presence of DIC. Alkaline phosphatase, CEA and CA 19-9 levels were significantly elevated.

 

Paracentesis revealed signet cell adenocarcinoma. CT of the abdomen and pelvis showed dilatation of the intra hepatic ducts, common bile duct and the pancreatic duct without any evidence of cholelithiasis. An ERCP done 1 year earlier for pancreatitis had shown the same dilatation, which was considered a normal variant then. MRI of the abdomen with MRCP failed to show any hepatobiliary or pancreatic mass. EGD and colonoscopy were negative for malignancy and a bone scan did not show bony metastasis.

The patient refused chemotherapy and died a month later after a cardiopulmonary arrest. The family refused an autopsy.

 

Our literature review revealed 2 cases of signet ring cell carcinoma of unknown primary, one of which was found to have a 0.8 cm carcinoma of the Ampulla of Vater on autopsy. Treatment with chemotherapy has been shown to improve DIC, decrease tumor burden and prolong survival in these patients. Hence chemotherapy should be offered to such patients. The diagnosis of signet ring cell carcinoma of the gastrointestinal tract by endoscopy remains a diagnostic challenge.

 

Our patient presented such a challenge. This is a rare case of signet ring cell carcinoma of unknown primary presenting with DIC and a greatly elevated alkaline phosphatase level with no evidence of obstructive hepatobiliary or pancreatic disease.

 

RUPTURE OF ABDOMINAL AORTA ANEURYSM IN A 24 YEAR OLD FEMALE WITH CYSTIC MEDIAL NECROSIS.

K Potluri, MD (Associate), S Marur, MD (Associate), A Alabbadi* MD, L Lackey, MD, (Member)

Department of Medicine and Department of Pathology*, Sinai-Grace Hospital, Wayne State University, Detroit, Michigan.

 

Abdominal Aortic Aneurysm is a disease that is rarely manifested before the age of fifty-five. The most common cause is atherosclerosis.

 

We present a 24 year old African American female, who came to the emergency room with sudden onset of abdominal pain, low back pain and vomiting. Physical examination revealed diffuse abdominal tenderness.  CT Scan of the abdomen reported, a large infrarenal pseudoaneurysm and rupture of the abdominal aorta with extensive retroperitoneal hemorrhage. Patient was in shock and underwent an emergency resection of the abdominal aorta aneurysm with a bypass graft. Intra-operative findings revealed a nine centimeter infrarenal aortic “blow out” rupture. Although the post-operative period was complicated, the patient survived this event. The pathology of the aorta revealed extensive myxoid degeneration and cystic medial necrosis.

 

Aortic aneurysms in patients younger than 40 years are most often associated with cystic medial necrosis. The thoracic aorta is more commonly involved.

Very few cases of the abdominal aorta aneurysm rupture secondary to cystic medial necrosis have been reported.  Cystic medial necrosis may occur as an isolated abnormality or as part of a systemic connective tissue disease such as Marfans syndrome or Ehlers Danlos syndrome. This patient had some features of Marfans but failed to meet all the criteria of Marfans. There was no family history of aneurysms. Our patient could have idiopathic cystic medial necrosis or a partial expression of the Marfan syndrome with a possibility of a new missense mutation.

 

Regardless of the diagnosis, prophylactic treatment and prevention of further complications associated with cystic medial necrosis  is important. Awareness of the broad spectrum of manifestations in myxoid degeneration disorders needs to be increased among practitioners, to lower the threshold of suspicion necessary for referral to a specialist center.

 

 

MIXED SMALL /LARGE CELL CARCINOMAS OF THE LUNG, THERAPEUTIC AND PROGNOSTIC IMPLICATIONS. Bazzi, Kaled, MD. Associate, Marius Vidinas, MD, Associate and Lawrence Macdonald, MD, Fellow, Sinai-Grace Hospital/Wayne State University, Detroit, Michigan

 

Lung cancer is currently the most common cause of cancer mortality in the United States responsible for 157,200 deaths annually. Primary lung cancer can be generally categorized into four groups: these include squamous cell carcinoma (20-30%), adenocarcinoma (30-40%), large cell (10%) and small cell carcinoma (20%).  Rarely, lung tumors may present with a mixed histological pattern.

 

We report the case of a 67 year-old Hispanic female with a 5 pack-year history of smoking who presented with a dry cough of 4 months that failed to respond to several courses of oral antibiotics. The patient also reported a weight loss of 15 pounds over the same time period associated with dysphagia to solid foods and a progressive weakness of the lower extremities. Three days prior to presentation the patient had developed urinary and fecal incontinence. Diagnostic evaluation showed a lung mass on chest X-ray and MRI of the spine demonstrated multiple metatstatic foci involving the vertebral bodies at various levels with signs of spinal cord compression. Bronchoscopy with biopsy of the lung revealed a mixed small/large cell lung cancer. Palliative treatment with radiation therapy was initiated but the patient deceased two weeks later from complications of her cancer and septic shock.

 

Mixed histology small cell/large cell carcinoma constitutes about 4-6% of all small cell cancers and is considered a distinct variant with clinical, therapeutic and prognostic implications. The distribution of the mixed elements can be heterogeneous and the definitive pathological features evident only after resection of the tumor or at autopsy. Although this case illustrates a small cell/large cell mixed cancer, mixed histology of small cell with squamous and/or adenocarcinoma have also been described. Interestingly, metastases from these tumors may consist of mixed elements or limited to the small cell or non-small cell component alone. When treated with aggressive chemotherapy protocols for small cell cancer, patients with mixed small cell/large cell cancer have lower partial or complete response rates (58% vs 91%), and shorter survival rates (median 6 vs. 10.5 months) than patients with pure small cell cancer.

 

 

Cryptococcal Pneumonia Mimicking as Pneumocystis Carnii Pneumonia in a patient with AIDS. Jayasree Grandhi, MD, Associate, Apuva Motivala, MD, Associate, Wasif Hafeez, MD, Fellow, Sinai-Grace Hospital, Wayne State University, Detroit, Michigan.

 

Introduction: The most common pulmonary complication of AIDS is pneumocystis carnii pneumonia (PCP). However, other opportunistic infections like cryptococcus neoformans and mycobacterium avium intracellulare (MAI) can present with diffuse pulmonary involvement and mimic PCP. We report a patient with disseminated cryptococcosis whose initial clinical presentation was indistinguishable from PCP.

 

Case Report: A 30 yr old  homosexual male with AIDS and with multiple previous admissions for PCP came with difficulty in breathing, pleuritic chest pain and diarrhea of 1 week duration. He was on highly active antiretroviral therapy (HAART), azithromycin for MAI prophylaxis but was noncompliant with bactrim for PCP prophylaxis. He was slightly tachypneic with inspiratory rhonchi in bilateral lung fields with oxygen saturation of 92-95% on 2 liters of oxygen. Initial labs were WBC 21.9, CD4 count 0, HIV viral load 175,000 and chest X-ray showed bilateral perihilar interstitial infiltrates. He was treated for presumed PCP pneumonia. On day 2 patient became very tachypneic, desaturated and was intubated for impending respiratory failure. Cardiac arrest ensued and despite vigorous attempts at resuscitation, the patient died. Meanwhile, bronchoscopic alveolar lavage (BAL) was performed which showed budding yeasts with halos around them, suggestive of C. neoformans. Autopsy revealed disseminated cryptococcosis.

 

Conclusion: C. neoformans must be high in the differential diagnosis of diffuse interstitial pneumonia in patients with AIDS. Since cryptococcal infection is potentially treatable with antifungal agents, an early microbiological diagnosis is essential. Early aggressive investigations aimed at definitive diagnosis like serum cryptococcal antigen titer and identification of Cryptococcus in BAL washings will give strong support in planning therapy. 

 

 

Neurologic symptoms as the initial manifestation of Legionella pneumonia, a case series. Irfan Hameed, MD, Associate, Wasif Hafeez, MD fellow, Sinai-Grace Hosptial, Wayne State University, Detroit, Michigan.

 

Infection with Legionella pneumophilia manifests as pneumonia in 90% of cases and can also manifest with neurologic findings ranging from headache to lethargy to frank encepholopathy. We describe a series of three cases of Legionella pneumonia that presented with prominent neurologic symptoms at our institution over a span of just five months. In all three patients, Legionella antigen was present in their urine.

 

Case 1: A 55 year-old man presented with a seizure, productive cough, RUL infiltrate, acute renal failure, temperature of 105o, WBC = 22,000 and CPK=32,000. Septic shock and respiratory failure ensued requiring ICU admission. He was treated with azithromycin and rifampin and eventually discharged to the rehabilitation service after a 42 day stay in the ICU.

 

Case 2: A 44 year-old man presented with delirium and had been having influenza-like symptoms for seven days. LUL infiltrate, acute renal failure, CPK = 15,000 and leukopenia were also present on admission. Septic shock with respiratory failure ensued. He was discharged to the rehabilitation service after a 57-day stay in the ICU.

 

Case 3:A 32 year-old man presented with obtundation progressing to coma. Bilateral infiltrates, WBC = 31,000 and coagulapathy were also present. Septic shock and respiratory failure ensued. Blood culture yielded pneumoccoccus. Despite aggressive antibiotic treatment the patient died on the 5th hospital day.

 

These three cases illustrate the propensity of Legionella infection to cause severe neurologic symptoms as compared to other causes of pneumonia. Patients who do present initially with predominantly CNS symptoms, in general, have more complications and are more likely to develop multiorgan involvement. The neurologic involvement may precede the pulmonary involvement and has been reported to develop in the absence of pulmonary disease. Encephalopathy, delirium, profound coma, hallucinations, cerebellar dysfunction, hemiparesis, quadraplegia, chorea, seizures and cranial nerve palsies have all been reported. Therefore, Legionella infection should be considered in all patients presenting with neurologic symptoms in the setting of pneumonia. Timely testing to confirm the diagnosis and appropriate treatment may be life saving.

 

A RARE CASE OF POTT’S PUFFY TUMOR DUE TO GROUP C STREPTOCOCCAL INFECTION. Jayasree Grandhi, MD, Associate, Adeel Ijaz, MD, Associate, Sinai-Grace Hospital, Wayne State University, Detroit, Michigan.

 

Introduction: Pott’s puffy tumor is a subperiosteal abscess of the frontal bone associated with underlying frontal bone osteomyelitis, causing swelling and edema over the forehead. We describe a 38-year-old man with a Pott’s puffy tumor due to streptococcus C that was successfully treated with antibiotics and surgery.

 

Case Report: A 38-year-old male with HIV came with pain and swelling over the forehead and both eyes. Patient had similar complaints one month ago and was diagnosed with preseptal orbital cellulitis and treated with ampicillin-sulbactam. He gave a history of chronic sinusitis for 2 years without any regular treatment. On examination, there was an eythematous, tender, fluctuant swelling over the forehead and eyes. He had bilateral frontal, maxillary, ethmoid sinus tenderness, intact visual acuity and no evidence of opthalmoplegia. His CD4 count was 650 and CT and MRI imaging showed preseptal orbital cellulites, extensive pan sinusitis, frontal bone osteomyelitis, destruction of anterior wall of the right frontal sinus and a right frontal scalp abscess. Aspiration of the fluid grew streptococcus C. The patient was treated with ampicillin-sulbactam and underwent early surgical drainage with bilateral ethmoidectomy and revision of the frontal bone.

 

Discussion: Pott’s puffy tumor is usually a complication of chronic sinusitis rarely seen at present time due to the widespread availability of antibiotics. Suppurative complications such as epidural, subdural and intracerebral abscesses are common, therefore early recognition is very important. A review of 23 cases showed that it is mostly seen in children, with only 8 cases reported in adults, none of them were caused by streptococcus C.

 

Conclusion: Pott’s puffy tumor is most common in children; it should be included in the differential diagnosis of a swelling on the forehead in adults. Surgical drainage and antibiotic therapy remains standard of therapy.

 

 

ATYPICAL CLINICAL COURSE OF LOCALIZED REACTIVE LYMPHOID HYPERPLASIA OF THE SPLEEN (SPLENIC LYMPHOID HAMARTOMA): A CASE REPORT.

Yadav Suresh, MD, Associate, Tawde Darshana, MD, Associate, Eisenberg Leopold MD, FACP. Sinai-Grace Hospital, Wayne State University, Detroit, Michigan.

 

Introduction: Localized reactive lymphoid hyperplasia (lymphoid hamartoma) is a rare lesion of the spleen. Most of the patients are asymptomatic but a small group of patients present with a wide range of clinical manifestations.

 

Case report: A 24-year-old Caucasian male of Italian descent presented with severe microcytic anemia and thrombocytosis of a chronic nature with symptoms of tiredness, fatigue and syncope eventually requiring transfusions. The patient admitted to using anabolic steroids for body building. Diagnostic studies demonstrated a normal bone marrow with adequate iron stores, elevated ESR, fibrinogen, and CRP levels and an unexpected IgA deficiency. Familial hemoglobinopathy was excluded by hemoglobin electrophoresis of available family members. Panendoscopy of the gastrointestinal tract was negative for bleeding but revealed esophagitis due to C. Albicans and gastritis due to H pylori. CT scan demonstrated splenomegaly with a 3.6 x 3.8 cm mass that increased in size over a 2-month interval. Diagnostic and therapeutic splenectomy was performed revealing localized reactive lymphoid hyperplasia of the spleen consistent with a lymphoid hamartoma with negative cytogenetics and polyclonal flow cytometry. Follow up surprisingly demonstrated normalization of all hematological parameters with no additional therapy.

 

Conclusion: In our literature review of splenic lymphoid hamartoma, we did not find a previously described association with severe microcytic anemia. Additionally, no case reports of elevated ESR, fibrinogen or C-reactive protein were found except in association with Hodgkin’s disease. Lymphoid harmartomas are usually seen in children with most patients being asymptomatic although a few present with hypersplenism. Histopathology is important in differentiating splenic lymphoid hamartoma from lymphoma, inflammatory psuedotumor of the spleen and red-pulp hamartomas (splenic hamartoma due to malformed vascular elements). This case reports illustrates several important clinical features of lymphoid splenic harmatomas that have not been reported previously and the potential for a complete and sustained response to splenectomy.

 

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.

 

DECREASING RENAL FUNCTION AS A PREDICTOR OF INCIDENT CARDIOVASCULAR DISEASE IN DIABETICS 

 

Oronde White, MD, Errol Crook, MD, Sinai-Grace Hospital, Wayne State University School of Medicine and John D. Dingel VAMC, Detroit, MI.

 

Objective:  Chronic kidney disease (CKD) has been recognized as a cardiovascular disease (CVD) risk factor.  However, whether progression of established CKD further increases risk for CVD is not clear.  In this study we examined the effects of declining renal function in diabetics as a predictor of incident CVD.

 

Methods:  The charts of all diabetic patients seen in the Wayne State University Nephrology clinic in 2001 and 2002 were reviewed and data on basic demographics, blood pressure, renal function, medications, and both prevalent and incident cardiovascular disease was extracted.  Combined hear disease (CHD) was defined as CHF and Coronary Heart disease, and CVD was defined as CHD & stroke.  Stable renal function was defined as remaining in the same CKD stage (according to the classification of the National Kidney Foundation) over the course of follow-up, and unstable kidney function was defined as deterioration by 1 or more stages.  Estimated GFR (eGFR) was calculated using the abbreviated Modification of Diet in Renal Disease formula.  Statistical analysis was performed using the StatView program.  Differences between continuous variables were determined by t-tests, and between nominal variables by Chi-Square analysis.  The initial relationship regarding progression of kidney disease and incident CVD was done by Chi-Square analysis.  Logistic Regression was used to adjust for the effects of other factors on incident CVD risk.

 

Results:  337 patients were included (85% African American, 216 women, 217 stable CKD).  Patients with unstable CKD had significantly higher rates of incident CVD than stable patients (19.2% vs. 29.7%, stable vs. unstable, respectively (chi 2, p = 0.039)).  Similar results were seen for CHD.  Logistic regression revealed 57.4 %  and 71% increased risk of CVD (p = 0.034) & CHD (p = 0.015) respectively. Prevalent CAD, CHF, diabetic extrarenal microvascular complications, follow-up time, # BP meds on follow-up, and smoking status were significantly related to incident CHD & CVD.  When controlling for these factors in stepwise logistic regression, unstable CKD was still significantly associated with increased risk of incident of CHD (p = 0.0126) but not CVD.  However, the continous variable of change in eGFR was significantly related to incident CHD and CVD after adjustment for the above factors.

 

Conclusions:  Diabetic patients with progression of kidney disease are at higher risk for CVD events.  Any patient with progression of CKD, especially diabetics, should be closely monitored for cardiovascular complications.

 

Myelodysplastic Syndrome (MDS) and Pregnancy- Still an Enigma

Tannu Sahay,MD (associate), Saad Usmani,MD (associate), Leopoldo Eisenberg, MD (member), 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 medical and obstetric history was significant only for a spontaneous vaginal delivery at age 24 with no family history of any hematological disorder. Physical examination showed significant pallor, 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. The peripheral smear showed polychromasia, teardrop cells, macro-ovalocytes, pseudo-Peulger-Heut anomaly, occasional Dohle bodies, thrombocytopenia with few large platelets and spherocytes. Bone marrow examination that 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 in pregnant females. With extensive search of English literature, we could only find 6 reported such cases. All were Caucasian females in the ages between 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 diagnoses. 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. But we did come across a Japanese case (not available for review) of successful treatment with steroids.

Conclusion

   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.