Khaldoon Alaswad, M.D., G. William Pogson, M.D., 
  William S. Harris, PhD., Kamran Sherwani, M.D., Hubert H. Bell, M.D. 

UNIVERSITY OF MISSOURI--KANSAS CITY

Effects of phenytoin, niacin, gemfibrozil and omega-3 fatty acids on HDL in patients with hypoalphalipoproteinemia

Background: Low levels of high density lipoprotein cholesterol (HDL-C) are a known risk factor for CAD. Several drugs are known to be able to raise HDL-C levels, but single-study comparison of several drugs has rarely been made. Here we evaluated the effects of niacin, phenytoin, omega-3 fatty acids, and gemfibrozil on levels of HDL-C and its subfractions (HDL2-C and HDL3-C) in patients with low HDL-C levels.

METHODS: Fifty-four patients with HDL-C of 35 mg/dl or less were randomly assigned to three months of blinded treatment with one of four different drugs or placebo.

RESULTS: The statistically significant changes (vs. placebo) in HDL-C and Trig were as follows: Niacin increased HDL-C by 28% (32± 6.3 to 40.8 ± 7.7 mg/dl; p=0.0001), HDL3-C and HDL2-C by 18%, 48%, respectively (p<0.0005), and decreased TG by 43.5%(203 ± 92 to 115 ± 36; p=0.002). Phenytoin increased HDL2-C by 16% (7.9 ± 2.2 to 9.2 ± 2.78; p=0.004). Omega-3 fatty acids increased HDL2-C by 19% (7.6 ± 1.6 to 9.0 ± 2.4; p=0.018), and lowered trig by 24% (p=0.02). Gemfibrozil raised both HDL-C by 17% (32.1 ± 3.7 to 37.4 ± 8.6; p=0.02) and HDL30C by 17% (24.4 ± 3.5 to 28.5 ± 5.2; p=0.02). Triglycerides was reduced by 49% (p=0.005).

Conclusion: The most effective drug for low HDL-C in this group of patients was niacin.

R-2

Samer Al-Kaade M.D., Francisco Alverez, M.D., Cesar Keller, M.D.                                                                                           

ST. LOUIS UNIVERSITY  

Spirometry at 6 months after lung transplant in patients monitored through the internet with daily home spirometry vs transbronchial biopsy (TBB)

Background: Several reports have demonstrated the usefulness of daily home spirometry for early detection of graft rejection and opportunistic infections after lung transplantation. Direct report of spirometry through the Internet may improve patient care and result in better preservation of lung function.

Methods: We retrospectively reviewed the spirometry of 16 patients for a period of six months after lung transplant. Eight patients were followed by routine clinic visits and bronchoscopy scheduled at regular intervals; the other eight patients were followed by home spirometry and the results were reported daily via modem to a central service (Datalog Inc.) where the data were converted into a spreadsheet format and reported to us via the World Wide Web for daily review.

Results: At 6 months post transplant the control group had a mean FVC%, FEV1% and FEF35-75% of predicted of 60%; 46%; and 27% respectively. The values for the group monitored with daily spirometry were 81%, 80% and 80% respectively. The percentage of change from the baseline at one month after transplant for FVC%, FEV1%, and FEF25-75% was 32%, 19% and –6% for the control group and 31%, 23% and 0% for the study group.

C-1

Ahmad Anjak M.D., Bibi Hasnain, M.D., Clifford Birge, M.D.,

St. Lukes Medical Center  

Diabetes insipidus in a patient with coexisting diagnosis of Sheehan's syndrome and Mollaret's Syndrome

We present the first case report of evolvement of Diabetes Insipidus in a patient, 18 years after the established diagnosis of Sheehan’s syndrome. Our patient also has documented occurrence of Mollaret’s syndrome 12 years prior to the recent development of Diabetes Insipidus.

Diabetes Insipidus has been reported as a rare complication of Sheehan’s syndrome. Our case report is unique, as the symptoms and the diagnosis of Diabetes Insipidus occurred about 18 years after the onset of Sheehan’s Syndrome.

An important fact which ws observed in our patient was the corticosteroids aggravated the underlying Diabetes Insipidus. As the patient was non-compliant with the steroid replacement in the past and was more compliant now, might explain the delayed appearance of the symptoms of Diabetes Insipidus.

Interestingly, Herpes Simplex virus has been linked with Mollaret’s syndrome as well as with development of Diabetes Insipidus. It is possible that Herpes Simplex virus was the etiologic agent of Mollaret’s syndrome and Diabetes Insipidus in this patient.

C-2

Frank Ashall M.D., Ph.D.  
    
Washington University

Ehrlichiosis due to Ehrlichia Ewingii in a liver transplant patient

Human ehrlichiosis is a tick-borne disease that occurs in two forms: human monocytic ehrlichiosis, which is caused by Ehrlichia chaffeensis, and human granulocytic ehrlichiosis. Recently, four cases of human granulocytic ehrlichiosis caused by Ehrlichia ewingii, a known cause of ehrlichiosis in dogs, were described in patients from Missouri. Three of these individuals were immunocompromised. Symptoms of monocytic ehrlichiosis are similar to those of granulocytic ehrlichiosis, and include fever, myalgia, headache, nausea, vomiting, diarrhea, arthralgias, rash and confusion. Prominent laboratory findings are thrombocytopenia, leukopenia and mild-to-moderate elevations of liver transaminases.

This poster describes the case-presentation of a 51-year-old man who presented with findings suggestive of ehrlichiosis and who had removed a tick from his body three days prior to hospital admission. Previously he received a liver transplant and was on chronic cyclosporine and steroid immunosuppression. He was diagnosed with ehrlichiosis by polymerase chain reaction (PCR), which showed that he was infected with Ehrlichia ewingii, and he acknowledged that he was exposed to dogs on his farm and that the dogs had ticks. He responded excellently to doxycycline therapy.

C-3

University of Missouri-- Columbia

Microangiopathic hemolytic anemia in a patient with diabetes mellitus

A 40 year old white male with a 16 year history of Type 1 Diabetes mellitus (DM) presented in Dec ’98 with generalized weakness and was found to have severe anemia (hematocrit 24%). Workup included an upper and lower GI endoscopy, a peripheral blood smear examination, serum B12 folate, iron studies, antinuclear antibody test, and viral serologies (Hepatitis B, Hepatitis C, CMV and EVB), all of which were normal. He remained transfusion dependent. Hypertension was detected a month later. He developed thrombocytopenia, high LDH (1639U/1), low haptoglobin (<8 mg/dl) and schistocytes in the peripheral smear. His creatinine increased from 1.3 to 1.8. Coomb’s test was negative. A diagnosis of hemolytic uremic syndrome/TTP was entertained. Plasmapheresis was initiated and following 42 units of plasma exchange, hemoglobin and platelets rose marginally from 9.3 g/dl and 65000 to 10.1 g/dl and 89000 respectively. LDH fell to 735U/1. His renal function continued to deteriorate.

The renal biopsy revealed changes consistent with diabetic nephropathy, which included Kimmelsteil Wilson nodules, capsular drop lesions and mesangial expansion. A crescentic lesion in one of the glomeruli was noted. No microthrombi or fibrin deposits were seen. A retinal exam had earlier revealed diabetic changes.

Microangiopathic hemolytic anemia (MAHA) persisted and plasmapheresis and transfusions were continued. Two cycles of vincristine were administered. Splenectomy was considered but not performed. A Von Willebrand factor multimer analysis was normal. The patient did not have valvular heart disease.

Review of literature yielded prior reports of 12 cases of diabetes associated MAHA without a recognizable underlying cause. Favorable response to platelet aggregation inhibitors was suggested. Diabetes induced cell membrane abnormality was postulated to cause MAHA. Accordingly, patient was given ticlopidine nearly two months after the symptoms began. Platelet count normalized and hemoglobin improved quickly and he has remained transfusion free for the last two months. He continues to be on ticlopidine. Serum creatinine and renal function remain abnormal but stable.

Summary: This case is an illustration of diabetes associated MAHA which responded to the platelet aggregation inhibitor ticlopidine. Literature review found 4 similar cases with poor prognosis. Renal death occurred in several of them.

Darin Brimhall D.O., Kim Carmichael, M.D., FACP                                                                    
Washington University

Acute Cushing's syndrome as a result of ectopic ACTH secretion

Learning Objectives: (1) Recognize the clinical features and establish the diagnosis of Cushing’s syndrome, (2) Localize ectopic ACTH-secreting tumors in Cushing’s syndrome with the use of In-Pentetreotide (octreotide) scintigraphy.

Case: A 39-year-old woman is admitted with generalized weakness and cramps, and a potassium level 1.7 mmol/L. The patient also complained of two months of facial fullness, hirsutism, acne, hair thinning, dysesthesia, irregular menstruation, hyperpigmentation of her face and upper trunk, and dizziness. The patient has no known past history of diabetes of hypertension, but was found to have a random blood sugar of 430 mg/dL and had developed hypertension.

Labs: morning plasma cortisol level 63 m /dL (normal 5-25 m g/dL), free urinary cortisol level 3100 m /24 hours (normal 20-100 m gh/24-4 and 3400 m g/24-4 after 2.0 mg and 8.0 mg dexamethasone respectively. Fasting morning ACTH level 181.1 pg/ml (normal <60 pg/ml), repeat level after 8.0 mg dexamethasone 303 pg/ml. Urinary 17-ketosteroids 47 mg/24-h (normal 5-15 mg/24-h).

MRI of the head and pituitary ruled out pituitary microadenoma. CT of the chest & abdomen were performed to evaluate the adrenal glands and rule out tumor. The results showed bilateral adrenal enlargement and multiple hepatic "hemangiomas." Tumor localization SPECT imaging using In-Pentetreotide (octreotide) scintigraphy was then obtained to evaluate for ectopic-ACTH secreting lesion. This revealed abnormal accumulation of tracer in a mass anterior to the aorta adjacent to the uncal process of the pancreas. Upon retrospective evaluation of the abdominal CT, a small mass was observed.

She underwent surgery which revealed unresectable islet cell carcinoma and multiple hepatic metastases. Bilateral adrenalectomy was performed to control the Cushing’s syndrome.

C-5

Angela M.  Bruno-Ryerson M.D., Daniel E.  Potts, M.D., FACP, 
   Jose L Manes, M.D.                                                      

St. Luke’s Medical Center

Recurrent endobronchial carcinoid treated with endoscopic laser therapy, brachytherapy, and external beam radiation

Carcinoid tumors, thought to arise from bronchopulmonary Kulchintsky cells, constitute less than 5% of all primary lung tumors. Overall bronchial carcinoids make up approximately 10% of all carcinoid tumors. Conventional treatment has been surgical resection and recurrence if rare.

A 72 year old male with hemoptysis underwent left lower lobe resection in 1982. After recurrence in 1991, completion pneumonectomy was performed. In 1993, the patient underwent bronchoscopy for recurrent hemoptysis, was again diagnosed with endobronchial carcinoid, now in the posterior segment of the right upper lobe. After treatment with endobronchial YAG laser photoresection and brachytherapy followed by external beam radiation to the right upper lung, the patient has been disease free for 7 years.

C-6

Yangming Cao M.D., Jay Marshall, M.D., Maura Pieretti, Ph.D., Jose Manes, M.D., Henry Lynch, M.D.                                                               

St. Luke’s Medical Center

Initial suspicion of familial adenomatous polyposis (FAP) led to the identification of hereditary nonpolyposis colorectal cancer: a family case report

This is to study a family who we initially suspected had familial adenomatous polyposis (FAP). The 62 year old proband presented with duodenal adenocarcinoma with 3 gastric adenomas. His personal history of subtotal colectomy for colon cancer at 45 years old, the rarity of duodenal adenocarcinoma in general population and his family history of colorectal cancer made us suspect that he might have familial adenomatous polyposis (FAP). With informed consent obtained, we started to investigate this family by obtaining medical records and gene tests. The proband had only about 10 colon polyps when he had subtotal colectomy for the cancer, based on which classic FAP was excluded. Three months after we started to investigate this family, the proband’s 32-year-old son developed rectal cancer. His family fulfilled the Amsterdam criteria for HNPCC, but we needed to exclude attenuated familial adenomatous polyposis (AFAP). The proband was negative for APC gene germline mutation, which made AFAP highly unlikely. On the other hand, he had high microsatellite instability (MDI) in the adenomas and cancer tissues. The fulfillment of Amsterdam criteria, the exclusion of AFAP, and the high MSI established the diagnosis of HNPCC in this family. The family members should be followed by the screening procedures recommended for HNPCC. In addition, only 17 cases of duodenal cancer associated with HNPCC can be identified in the literature, with our proband’s being the 18^th such case.

C-7

Kirk Chan-Tack M.D., Caroline Kerber, M.D.

University of Missouri-- Columbia

Subclavian steal syndrome: a rare but important cause of syncope

Subclavian steal syndrome is caused by occlusion of the proximal subclavian artery with subsequent retrograde filling of the subclavian artery via the vertebral artery. The decreased blood flow to the brain and upper extremity on the affected side can be manifested in a variety of symptoms due to (1) vertebrobasilar insufficiency or (2) ischemia of the affected extremity. Vertebrobasilar insufficiency may produce lightheadedness, dizziness, vertigo, ataxia, visual disturbances, motor deficits, focal seizures, confusion, aphasia, headache, or syncope. Symptoms due to ischemia of the affected extremity are less frequent and include weakness, paresthesias, or coldness on the affected side. Hypertension and vigorous exercise of the affected extremity are risk factors for subclavian steal syndrome.

A 79 year-old woman was admitted for evaluation of a syncopal episode. While climbing a flight of stairs, she turned her head to the left and abruptly passed out. She fell and sustained a left occipital laceration. The patient denied chest pain, palpitations, prodrome, visual changes or aura, tongue biting, bowel or bladder incontinence, and post-ictal state. She had no previous episodes of pre-syncope or syncope. Her past medical history was remarkable for type 2 diabetes and hyperlipidemia. Medications included prandin and lipitor. Temperature was 37.2°C, BP 141/65 (right arm) and 80/43 (left arm), heart rate 76 and regular, respiratory rate 16 breaths per minute. Positive physical findings included a 6cm left occipital laceration as well as non-palpable left radial and brachial pulses that were detectable only by Doppler. Complete blood count, chemistry panel (including cardiac enzymes and troponin), EKG, and chest x-ray were normal. Head CT was negative fore bleed, infarct, and mass effect. Carotid duplex study showed reverse flow in the left vertebral artery and abnormal, stenotic distal left subclavian artery. MRI angiography confirmed complete occlusion of the left subclavian artery with classic subclavian steal. The patient underwent a percutaneous tranSt. Louis University  minal angioplasty with stenting of the left subclavian artery. She tolerated the procedure without complications, was discharged on the following day, and has done well through 5 months of follow-up. This case underscores the importance of subclavian steal syndrome as well as it’s morbidity and potential for mortality if undiagnosed or misdiagnosed. Recognition is crucial since patients can be successfully treated by surgery.

C-8

Yugandhar Chimata M.D., Thomas Pohlman, M.D., Yangming Cao, M.D., 
   Motaz Alshaher, M.D., Jalal Abbas, M.D. 

St. Luke’s Medical Center

Calcific uremic arteriolopathy (CUA): a report of six cases

Calcific uremic arteriolopathy (CUA), also known as calciphylaxis, is a life threatening condition reported in chronic renal failure (CRF) patients. It is pathologically characterized by medial calcification and intimal hyperplasia of cutaneous and subcutaneous microvasculature, resulting in progressive necrosis of subcutaneous tissue and fat. Clinically it manifests as livedo reticularis and painful plaque-like subcutaneous nodules which invariably ulcerate and become infected, leading to sepsis and death. To date, about 200 cases of CUA have been reported in the literature. Here we report 6 cases diagnosed in our hospital over a two year period. They were all CRF patients on hemodialysis or peritoneal dialysis. Subcutaneous lesions were initially found on the lower extremities in all 6 patients and subsequently ulcerated in 5 patients. Transcutaneous oxymetry, done in 3 cases, over the nodules revealed moderate to severe hypoxia with no improvement even after 100% 02. Calcium x Phosphorus product and PTH were high in 3 patients and within normal range in the other 3 patients. The diagnosis was confirmed in 5 patients by biopsy. One patient underwent repeated debridement, skin grafts, and hyperbaric 02 therapy without success. She developed sepsis and multi-organ failure. Realizing the poor prognosis, she decided to enter the hospice program and died a few days later. Two patients died of comorbid conditions.

R-3

Heidi M. Crane, M.D.   
   
Washington University

Changing models of erectile dysfunction in men with diabetes

Introduction. Sexual dysfunction in general and impotence or erectile dysfunction in particular are devastating complications of diabetes. Despite the tremendous impact impotence has on the lives of those who suffer with it, little attention has been focused in the literature on this prevalent complication of diabetes as compared to many less common complications.

Methods. Literature was reviewed on the etiologics of importance in patients with diabetes with special attention to the interrelationships between impotence and depression.

Results. The etiology of impotence in men with diabetes has been the source of much debate. Early studies divided all causes of impotence into two groups – organic impotence, with lack of nocturnal penile tumescence, and psychogenic impotence, with intact nocturnal penile tumescence. Early studies stopped with an "organic" finding, neglecting psychological effects of impotence. Men with diabetes were classified as having an organic impotence pattern greater than 90% of the time versus the 90% psychogenic pattern found in the general population. Recent studies have found it difficult to distinguish between psychogenic and organic causes of impotence. Nocturnal penile tumescence results which originally were the gold standard for classifying impotence as organic have been found to be influenced by depression, and can return to normal after the resolution of a depression. A more appropriate model is one that recognized the contributions of physiologic, affective, and cognitive factors to erectile function. The correlation between impotence and neuropathy appears to be especially strong. There is also a suggestion of a role for vasculopathy. One study found that most men with diabetes and impotence have neurogenic abnormalities. Since many men without impotence did as well, a second factor was required. Either depression or vasculopathy combined with neuropathy resulted in impotence. Depression is both common and frequently unrecognized in patients with diabetes. Depressive symptoms are more common in men with sexual dysfunction. Depression in patients with impotence may be both contribution to and a consequence of impotence, which can lead to not only depression but self-depreciation, demoralization, and performance anxiety.

Conclusion. In considering impotence in patients with diabetes, not only do neurologic, vascular, endocrine, and pharmacological causes need to be considered but so do psychogenic causes. Even in patients where psychological factors may not be the initial cause of impotence, these factors often then become part of the sexual dysfunction and if not addressed, medical treatments are more likely to fail.

C-9

Heidi M. Crane M.D.                                                                

Washington University

Acute myocardial infarction as an unusual presentation of chronic myelogenous leukemia

Introduction: Chronic myelogenous leukemia is a rare hematological neoplasm ( less than 15% of leukemias). Patients typically present with fatigue, headache, weight loss or are diagnosed from routine leukocyte counts obtained for other reasons. This is an unusual case of a young woman with no cardiac risk factors who presented with an acute myocardial infarction.

Case: A 48-year-old female with no significant past medical history was admitted to the hospital after one week of chest pain. Pain typically began at night, usually at rest, often would wake patient from sleep. Initially pain did not radiate nor was it associated with nausea, vomiting, or diaphoresis. On night of admit pain was more severe than previously, it radiated to her left arm, and she vomited two times. At that point she went to the hospital. After receiving aspirin and nitrates patient had substantial reduction in pain. Initial laboratory studies showed a troponin level of 4.1, a white blood cell count of 88,000, a platelet count of 2.4 million, and a hematocrit of 36. An EKG revealed a sinus tachycardia with T wave inversions in V2 and V3, an echocardiogram showed apical akinesis and anteroseptal hypokinesis. A cardiac catheterization demonstrated a 90% LAD lesion which was treated with percutaneous tranSt. Louis University  minal coronary angioplasty as well as a stent, in addition, spasm was present at other sites, No reoccurrence of chest pain occurred after the cardiac catheterization. Patient’s treatment included platelet phoresis, aspirin, plavix, calcium channel blockers, and initially hydroxyurea myelogenous leukemia. Patient recovered uneventfully from the acute event.

Discussion: Acute myocardial infarctions can be due to a number of pathologic hematologic abnormalities including polycythemia vera, acute myelocytic leukemia, and others. An acute myocardial infarction as a presenting symptom for chronic myelogenous leukemia is exceeding rare especially in the setting of no cardiac risk factors. This case has value in not only its unusual presentation, but also the treatment questions that arise. As an example, one could suspect that this patient would have benefited from a glycoprotein IIb/IIIa inhibitor but there is no data. This leads to a number of other questions such as dosing (standard versus dosing until a percentage of platelets are affected), safety, etc. These issues then have implications about treatments for more standard acute myocardial infarctions.

R-4

Paul K. Crane,  M.D.                                             

Washington University

Evaluating guidelines for coronary heart disease in women

Introduction. Coronary heart disease is the leading cause of death in women. However, the prevalence of coronary heart disease is lower for women at every age than is the prevalence in men. This lower prevalence makes the predictive values of any diagnostic test for coronary artery disease lower in women than in men. This project evaluated the various guidelines that have been promulgated to determine how successfully they navigate the difficult issues of prevalence and predictive value.

Methods. Review of articles and textbooks that discussed the evaluation of coronary heart disease specifically in women were identified. References from these works were obtained. These references were then reviewed using pre-established criteria for methodologic adequacy and size of experimental groups, and each reference was graded on five point scale. Discussions of evaluation of coronary heart disease in women were then assessed based on the grade of evidence each utilized.

Results. A wide spectrum of references were identified, spanning from well conducted large trials with a clearly defined gold standard, to references to individual expert opinion. Guidelines on the evaluation of coronary disease in women varied widely in the strength of evidence they utilized to arrive at their recommendations.

Conclusions. A certain degree of skepticism is appropriate when approaching practice guidelines. Seemingly authoritative discussions of a topic may be based on less-than-optimal data. Complicated and intricate areas of the medical literature such as the evaluation of heart disease in women serve to highlight the rather varied statistical savvy and critical evaluation of the medical literature found in writers of guidelines.

C-10

Paul K. Crane, M.D.   

Washington University

Bacillus cereus food poisoning as a cause for sepsis: a case report

The patient is a 53 year old woman with a history of osteomyelitis of her right femoral head, leading to chronic intravenous antibiotics through a Hickman line. She did well until two nights prior to admission when she went with her mother to Chinese food. Both women ate the fried rice. Both women noted the onset of crampy diarrhea several hours later. The diarrhea was self-limited in the mother. The patient noted increasing malaise, fevers, chills, and weakness, and presented to the emergency department.

There, she was found to be in moderate distress, febrile to 38.4, hypotensive to 90/58, and tachycardic at 110. Respirations were normal. Exam was marked in the poor hygiene around the patient’s Hickman line; dressings were changed in the emergency department. The patient responded well to intravenous fluids. On the second hospital day, multiple sets of blood cultures grew a gram positive rod, which was identified on the third hospital day as Bacillus cereus. The patient responded well to antibiotics and was sent home. Three months following the admission there were no long-term sequelae.

Most cases of Bacillus cereus bacteremia have been linked to immunocompromised states or to intravenous drug use. No case of bacteremia had been described in patients who developed food poisoning initially. As patients are increasingly being cared for in outpatient settings with indwelling lines, cases similar to this one may be expected. Certainly hygiene of the line site is essential, and better education of patients will be required to prevent the risk of serious complications from sepsis.

C-11

W. Travis Dierenfeldt M.D., Prashant K. Pandya, M.D., 
          Gregory G. Tsiotos, M.D., Wendell K. Clarkston, M.D., FACP                                              

UNIVERSITY OF MISSOURI--KANSAS CITY

Index case of gastric volvulus secondary to incarcerated inguinal hernia

Gastric volvulus occurs when a congenital or acquired abnormality allows the stomach to twist upon itself. Some known causes of gastric volvulus include: abnormal ligamentous connections, congenital diaphragmatic defects, gastric tumors, ulcers, organomegaly, intra-abdominal masses, elevation of the left hemidiaphragm, phrenic nerve paralysis, and overeating. We report the first patient, to our knowledge, to develop a gastric volvulus secondary to incarcerated inguinal hernia.

An unresponsive 83-year-old white male presented with respiratory failure, hypotension, and marked abdominal distention. There was no history of vomiting. Abdominal exam revealed marked distention and hypoactive bowel sounds. Initial labs included a Hbg of 13.2 gm/dl, WBC 15.7/cmmm, platelets 149,000/cmm, pH 7.11, HCO3 10 mmol/L and lactic acid 10.0 mmol/L. Attempts to place a nasogastric tube were unsuccessful. An x-ray showed massive dilation of the stomach and multiple loops of small bowel. The nasogastric tube was coiled within the esophagus.

An emergent upper GI endoscopy revealed a dilated esophagus with a large amount of residual fluid. Near the GE junction, a complete obstruction due to esophageal torsion was noted. The endoscope was unable to pass the point of torsion.

An exploratory laparotomy revealed an ischemic, massively dilated stomach, twisted around its organoaxial axis and incarcerated jejunum through a right inguinal hernia. Six liters of feculent gastric fluid were aspirated through a gastrotomy, and the stomach was untwisted. The incarcerated jejunum was reduced, the hernia repaired, and a gastrostomy tube was placed. Postoperatively, the patient rapidly deteriorated and died 12 hours later due to multi-system organ failure and profound metabolic acidosis.

Many causes of gastric volvulus have been previously described. Incarcerated inguinal hernia should be included in the list of possible etiologies of gastric volvulus.

W. Travis Dierenfeldt, M.D., Prahant K. Pandya, D.O.,  
    Wendell K. Clarkston, M.D., FACP,  Ryan Taylor, M.S., 
   Willian Depond, M.D.  

UNIVERSITY OF MISSOURI--KANSAS CITY

Clinical and histologic significance of positive anti-smooth muscle antibody (ASMA) in patients chronically infected with hepatitis C virus (HCV)

Positive ASMA may occur in patients with chronic HCV and may indicate concomitant autoimmune liver disease. We anecdotally noted abnormal ASMA studies in a high percentage of our patients with chronic HCV who had no clinical or histologic evidence of autoimmune liver disease.

Methods: Patients who have HCV and a positive ASMA were compared retrospectively to patients with HCV and a negative ASMA. Baseline characteristics including HCV genotypes, initial viral load by RT-PCR, response rate to interferon therapy, histologic grade of disease activity and stage of fibrosis using the Knodell HAI were compared.

Results: Ninety-two consecutive patients with HCV were evaluated. Eighty-one patients were tested for ASMA and 30/81 patients (37%) had a positive ASMA. The ASMA titre was ‹1:160 in all but one patient. HCV genotypes 1,2,3 and 4 represented 63%, 20%,17% and 0% of ASMA positive patients and 71%,20%,6%, and 3% of ASMA negative patients (p=ns). The median viral load was 2.9 x 10 in patients with positive ASMA compared to 3.1 x 10 with a negative ASMA (p=ns). Review of 62 available liver biopsies showed no significant difference in the grade of disease activity (p-0.46) or the stage of fibrosis (p=0.59). Of the 51 patients who have completed monotherapy with alpha-interferon (INTRON A®) only 1/15 (7%) who were positive for ASMA responded to interferon compared to 6/36 patients (17%) who had a statistical significance (p+0.57). Discontinuation of therapy due to adverse events was similar in both groups.

Conclusion: A low titre of ASMA is common in patients chronically infected with HCV, but does not adversely affect the histologic factors or treatment outcome in patients with HCV.

C-12

Delair Gardi M.D., Greg C. Flaker, M.D.                                                              

University of Missouri-- Columbia

A case of aortic dissection which mimics an inferior myocardial infarction

A 72 year old hypertensive male came to the ER by ambulance after blacking out. He was confused, diaphoretic, and nauseated but did not complain of chest pain. His BP was 60/0 mm Hb in both arms. No cardiac murmurs were noted. An ECG showed 2mm ST segment elevation in the inferior leads. Right precordial ECG leads showed no RV infarction. An emergency cardiac catheterization study showed a 60% lesion in the right coronary artery (RCA) which was cannulated with difficulty. Minor lesions were noted in the left anterior descending and circumflex arteries. He received pressors and an intraaortic balloon pump but remained hypotensive. A right heart catheterization showed equilibration of diastolic pressures. An echocardiogram showed pericardial fluid with RV collapse consistent with pericardial tamponade. Despite resuscitative efforts the patient died. An autopsy demonstrated a proximal aortic dissection with a hematoma which compressed the ostium of the right coronary artery; blood was found in the pericardium.

Proximal aortic dissection rarely involves the ostium of the coronary arteries. When it does, the RCA is more often affected than the left coronary artery and signs of an inferior MI can obscure the diagnosis of aortic dissection. Angiographic findings include coronary artery dissection, or in this case, external compression of the coronary which can be missed if the catheter enters the ostium of the coronary artery. In this case the diagnosis was made by the finding of blood in the pericardium, the result of dissection from the aortic wall to the pericardium. Administration of thrombolytic therapy for an apparent acute MI in this situation would be disastrous.

C-13

Minxiang Gu M.D., Xiaoling, Wu, M.D., Austin F. Montgomery, M.D.,
     Mathew L. German, M.D.       

St. Luke’s Medical Center

Disseminated bacillus calmette-guerin (BCG) infection after BCG bladder instillation

Bacillus Calmette-Guerin (BCG) intravesical or intralesional injection has been the best available treatment for recurrent bladder transitional-cell carcinoma, and carcinoma in situ. Along with the 80% efficacy, fewer than 5% of patients will have adverse reactions range from minor cystitis to major, but rare, BCE sepsis. Because of the common misconception that BCG is a harmless organism, the diagnosis and treatment of BCG sepsis can be delayed. We report a patient with a disseminated BCG sepsis as a side effect of BCG bladder instillation.

Case study: 68 year old male developed remittent fever 10 days after second dose of BCG bladder instillation, with neutropenia, LFT abnormality, pulmonary infiltration, and had no response to broad-spectrum antibiotics. Liver biopsy revealed non-caseating granulomas and bacillus-like organism was identified with auramine-rhodamine stain. PCR of biopsy tissue was positive of bovus mycobacterium DNA and culture of the tissue grown mycobacterium tuberculosis. Patient responded to anti-tuberculosis treatment.

Disseminated BCG infection is a rare but serious complication associated with BCG bladder instillation. The early recognition and initiation of empirical anti-tuberculosis treatment is important when the patient developed a high fever after the procedure.

C-14

Rami Y. Haddad M.D., A. M. DiBisceglie, M.D., FACP                                                                     

ST. LOUIS UNIVERSITY  

Pericardial effusion: a rare complication of multiple myeloma

An 82 yr old man who was known to have multiple myeloma for more than 25 years presented with a 1 year history of shortness of breath and generalized weakness which was progressive. At the time of admission he was receiving dexamethasone but he had been treated previously with chemotherapeutic agents for myeloma. Subsequent examination of a bone marrow aspirate 3 yrs prior to admission revealed poorly differentiated multiple myeloma. Blood pressure was 120/67, pulse rate 97/min with palpable pulsus paradoxus of approximately 10 to 15 mmHg. The jugular venous pressure was not noted to be elevated and Kussmaul sign was negative. Auscultation of the heart revealed distant heart sounds with a grade II-III/VI pan-systolic murmur of mitral regurgitation. No peripheral signs of heart failure were noted (no ascites, edema). Plasma hemoglobin was 11.4 g/dL, total white cell count 6/3000/mm³ (differential count: 78% neutrophils, 6% lymphocytes, 13% monocytes) and serum creatinine 1.7 mg/dL. Total serum protein was 7.3 g/dL and albumin 2.8 g/dL. Serum protein electrophoresis showed gamma globulins 1.8 g/dL with no monoclonal peaks. Chest X ray showed bilateral pleural effusions, right greater than left and cardiomegaly. Echocardiogram revealed large pericardial effusion with notching of the right atrium consistent with cardiac tamponade. Pericardiocentesis was done and 750 ml of blood stained fluid was drained from the pericardium. Cytologic examination showed the presence of reactive mesothelial cells with a substantial proportion of plasma cells in addition to other inflammatory cells. Because of the patients age and advanced stage of disease, no additional specific therapy was given and he was offered hospice care. Over the course of the next 2 months, he was readmitted to hospital with pneumonia and respiratory distress due to a large pleural effusion. He subsequently developed renal failure and died soon thereafter. Review of the literature reveals less than 20 other cases of pericardial effusion related to myeloma. In some of these reports, the effusion was related to the presence of amyloidosis while in others it was due to malignant infiltration of the pericardium by plasma cells as appeared to be the case in our patient.

C-15

Bibi Hasnain M.D., J. Alex Marchosky, M.D., James Esther, M.D.,
       Charles Garvin, M.D., Ben Mayes, M.D.                                                             

St. Luke’s Medical Center

Ventricular atrial shunt, a novel and effective treatment of spontaneous CSF rhinorrhea

We present the first case report of a patient with intrasellar cisternal herniation and empty sella, who was successfully treated with ventricular atrial shunt for spontaneous and persistent CSF Rhinorrhea. One of the etiologies which has been hypothesized for intrasellar cisternal herniation is intermittent increase in the CSF pressure. Our patient presented with spontaneous CSF Rhinorrhea from a defect in the left cribriform plate. In addition the patient also had defects in the right cribriform plate and in the floor of the sella turcica. It was concluded that intrasellar cisternal herniation, the defects within the roof of paranasal sinuses and the spontaneous CSF leak was secondary to a rare complication of communicating hydrocephalus. It was decided that the best treatment of the CSF leak, halting further bony remodeling, preventing pituitary damage and resolving the pathophysiologic process was placement of a ventricular atrial shunt. The CSF leak resolved once the ventricular atrial shunt was placed and the valve pressure was adjusted.

Our case report not only reinforces increased intracranial pressure as an etiology of subarachnoid cisternal herniation but also furnishes a method for effective treatment of this unique condition.

Hatim A. Hassan M.D.,  B. Bastani, M.D.,  M. Gellens, M.D.

ST. LOUIS UNIVERSITY  

Successful treatment of normeperidine neurotoxicity by hemodialysis

Meperidine (demerol) is a potent widely used narcotic analgesic. Normeperidine, its major metabolite, is half as potent as an analgesic but 2-3 times more potent as a convulsant. The plasma half-life of normeperidine increased from 12-21 hrs in normal individuals to 35 hrs in those with significant renal impairment. Thus, normeperidine may significantly accumulate in patients with renal failure, and lead to serious complications. The intensity of the central nervous system (CNS) excitation is highly correlated with the plasma concentration of normeperidine. Moreover, normeperidine CNS toxicity is not reversed by naloxone which may, in fact, exacerbate it. We report a 72-year-old white female with end stage renal disease on peritoneal dialysis, and history of severe peripheral vascular disease, who had been receiving large doses of meperidine for pain control. The patient subsequently developed myoclonic contractions and a grand mal seizure. The patient was successfully treated with hemodialysis (F 8 dialyzer) for presumed normeperidine-induced CNS toxicity. During HD, normeperidine plasma clearance was 50 ml/min, percentage plasma extraction was 24%, and there ws 26% reduction in its plasma concentration over 3 hours of HD (average of measurements performed at times 0,1.5 and 3 hrs on HD). With approximate plasma clearance of 50 ml/min, about 2.4 mg of Normeperidine has been removed during the 4 hours of HD. This amount was greater than the normeperidine distributed in the extracellular fluid (ECF – 1.8 mg), and about half of the amount distributed in total body water (TBW – 5.2 mg), which in either case seems to be significant.

Conclusion: Our findings suggest that HD may be utilized effectively for treating patients with normeperidine-induced neurotoxicity. This is the first report of normeperidine neurotoxicity to be successfully reversed with HD.

Hatim A. Hassan M.D. E. A. Gonzalez, M.D., C.L. McConkey, M.D., 
   K. J. Martin, MB, Bch, FACP

ST. LOUIS UNIVERSITY  

Uremic plasma ultrafiltrate blunts the response to PTH in UMR-106-01 osteoblast-like cells

Secondary hyperparathyroidism is a frequent complication of chronic renal failure. Pathogenetic factors for secondary hyperparathyroidism are phosphate retention, decreased levels of calcitriol, hypocalcemia, abnormalities of parathyroid function and skeletal resistance to the actions of PTH. Homologous down regulation/desensitization of the PTH/PTHrP receptor-adenylate cyclase system could contribute to PTH resistance; however, recent studies have revealed that while the levels of PTH/PTHrP receptor mRNA were reduced in renal failure, this abnormality was not corrected by parathyroidectomy. These observations suggest that factors other than high levels of PTH contribute to the skeletal resistance. The present studies were designed to test the hypothesis that factors circulating in the uremic environment contribute to decreased response of target cells to PTH. To this end, ultrafiltrate of uremic plasma was collected at the initiation of hemodialysis, and the influence of this on PTH stimulated cAMP generation was evaluated in UMR-106-01 osteoblast-like cells. Experiments were performed by incubating confluent cultures of UMR 106-01 cells in medium containing up to 50% of the uremic ultrafiltrate for periods of up to 72 hours. Control experiments were performed using a buffered salt solution containing a comparable ionic composition to that of the uremic ultrafiltrate. Following these incubations the cultures were tested for PTH stimulated cAMP production. In control cultures, PTH stimulated cAMP averaged 1992 pmol of cAMP per culture. In contrast, following exposure to uremic ultrafiltrate, PTH stimulated cAMP generation averaged 1023 pmol of cAMP per culture. Thus, the response to PTH was blunted by 48.7% in the presence of uremic ultrafiltrate. The clearance of biologically active PTH peptides by hemodialysis is expected to be negligible due to their large molecular weight; therefore, the decreased response to PTH is unlikely to represent homologous desensitization/downregulation of the PTH/PTHrP receptor. These data suggest that factors circulating in the uremic environment which are present in the low-molecular weight ultrafiltrate decrease the response of the PTH/PTHrP receptor adenylate cyclase system, thus contributing in the skeletal resistance to PTH seen in chronic renal failure.

R-7

Khaled R. Hassan M.D., Ward Casscells, M.D., Said Siadaty, M.D.,
    Richard Kirkeeide, Ph.D.

Washington University

Angiographic predictors of plaque progression in mildly to moderately diseased coronary arteries

Progression of atherosclerotic plaques has not been predicted by angiography. We hypothesized that progression of plaques creating <50% stenosis is predicted by fresh mural thrombus, lesion location at a branch point, and plaque blush –delayed clearance of contract possibly due to angiogenesis or cap fissuring. Methods: Films of 200 patients undergoing repeat angiography for symptoms of ischemia, 5.6±4.8 (mean±sd) months apart, were viewed by two blinded observers. 123 patients were excluded due to prior PTCA or CABG, initial lesion severity >50% and a non-comparable paired angiograms. Presence of plaque blush, calcification, clot (mobile defect), eccentricity, and branch point location were compared in progressing (>20% stenosis increase) and non-progressing plaques. Results: 16 lesions in 15 patients progressed from 29±13% to 68±14% over 8.1±7.9 months. Patients with and without progression were similar in gender mix, age, CHD risks, medications, days between angiograms, clinical presentation and initial stenosis severity. Logistic regression identified plaque blush (p=.002), calcification (p=.024) and a branch point location (p=.001) as the predictor of plaque progression. Using these signs, the model predicted the odds ratio for plaque progression (Orp) as : Orp =e 2.5*BL+1.8*CA+2.6*BR. The model has a 81% sensitivity, 77% specificity and a overall accuracy of 78% when Orp of 1/3 was used to classify the groups. In other words, a moderate (<50%) stenosis with both "blush" and branch point signs had a 25% chance of progressing within 8 months if calcified as well. Such lesions had a 100% likelihood of progressing but only 40% progressing lesions had all 3 signs. Conclusion: In mild to moderate coronary stenoses, plaque blush (a novel sign) branch point location and calcification are predictive of plaque progression. If confirmed by prospective analysis, these criteria may be helpful in clinical decision making.

C-17

Muhammad Hussain M.D., Sara E. Walker, M.D., MACP, 
       Geetha Komatireddy, M.D., FACP

University of Missouri-- Columbia

Tolosa-Hunt syndrome mimicking temporal arteritis

A 72 year old caucasian male had onset of pain on the right side of his face 2 years ago. The pain was worse in damp weather and was not related to mastication. 1½ years ago, he felt numbness on right side of his face. One year ago, his ESR was 92 mm/hour. Right temporal artery biopsy was normal. Later, he was unable to feel food inside his right cheek or drink using a straw. He had drooling from the right side of his mouth. Two months ago, he experienced pain behind and around the right eye and diplopia on looking towards the right side. Past medical history includes multiple episodes of right otitis media and right tympanic membrane rupture. The last episode of ear infection was 2 ½ years ago. He had double vision 9 years ago, lasting for a few weeks. He had hypertension for 25 years, DM Type II for 19 years, CABG 13 years ago and squamous cell carcinoma of the right pinna 5 years ago. He smoked 2 ½ packs of cigarettes per day for 33 years and does not drink. His sister has DM and recurrent temporary diplopia.

Physical examination showed an age appropriate, alert, and oriented male. He weighed 94 Kg. Vitals signs, heart, lungs and abdominal examination were unremarkable. His pupils were equal and reactive. His right eye was deviated medially and he was unable to abduct it. He had impaired touch sensation on the right side of the face. He also had right facial palsy. His tongue was deviated to the right. Deep tendon reflexes were absent. He had an old scar from the right temporal biopsy. Left temporal pulse was normal. CT Scan and MRI showed moderate cortical atrophy. CBC, ESR, electrolytes and liver function tests were normal. His BUN 33 mg/dl, and creatinine was 1.8 mg/dl.

This patient initially presented with right sided facial pan and high ESR. He did not respond to treatment with Prednisone 40mg/day for one month. Temporal arteritis was ruled out by biopsy. He also had recurrent diplopia. Later on he developed right 4^th, 6^th, and 7^th nerve palsy. Over time, he also had right periorbital pain. At this time, more than 2 years after the initial presentation, it was possible to make the diagnosis of Tolosa-Hunt Syndrome, which consists of multiple cranial neuropathies, and painful ophthalmoplegia and has recurring and remitting course. Early in the disease course, this entity may be confused with Giant Cell Arteritis.

C-18

Ehab Kaiser M.D., Robert A. Wright, D.O.

Washington University

Paradoxic air embolism from a hemodialysis catheter

A 50-year-old man was admitted to the ICU after having hemopneumothorax secondary to cocaine abuse. The next day, he was found to have thrombocytopenia, hemolytic anemia with schistocytes on a peripheral blood smear and change in mental status. Thrombotic thrombocytopenic purpura was considered likely and therefore a hemodialysis catheter was inserted and plasmapheresis was started right away.

On the 10^th hospital day, the patient pulled out the hemodialysis catheter and immediately had a cardiopulmonary arrest. He was resuscitated within 4 minutes and a surface echo-cardiogram was done within 20 minutes after arrest demonstrated air in all four cardiac chambers as well as the aorta. He exhibited decerebrate posturing for approximately 4 hours without focal neurologic deficits. This was most likely secondary to air deposits in the CNS vs. hypoxic encephalopathy. By the next morning, his neurologic status returned to normal.

This case illustrates the potentially devastating consequences of venous air embolism if it gains access to the arterial system. The author will discuss the cause, clinical manifestations, mechanism (cardiac & non-cardiac) and treatment of venous air embolism. It is a good reminder to clinicians to exercise caution each time a central venous catheter is accessed or removed, especially if it has been in place for a long time.

C-19

Geeta Katwa M.D., Paul King, M.D., FACP, Sara E. Walker, M.D., MACP

University of Missouri—Kansas City

Episodic polyarthritis with high-titer rheumatoid factor: pseudo-rheumatoid arthritis associated with hepatitis C and cryoglobulinemia

A 37-year old construction worker had a past history of thermal injury with cold exposure in 1984, followed by purple discoloration of the fingers. In August 1998, he started to have episodic disabling pain in the shoulders, wrists, hands, and feet. Pain lasted for periods of 24 hours and subsided spontaneously with or without NSAIDs. Some episodes were associated with swelling of the right hand and the right foot. He presented to our hospital in February, 1999 with diffuse swelling of the right hand. A radiograph showed only soft tissue swelling. One month later the patient was re-evaluated. He complained of painful shoulders. Review of systems was negative for signs and symptoms of lupus, and for tick bite, morning stiffness, fatigue, conjunctivitis, urethritis, and diarrhea. Examination revealed purple discoloration of the fingers, widespread livedo reticularis, and remarkable limitation of shoulder range of motion. The patient was married and heterosexual. He denied extramarital sex, IV drug use, and blood transfusions. Laboratory investigation revealed very high rheumatoid factor (106 IU/ml; N=O-30) and a negative FANA test. The evaluation for purple fingers included testing for cryoglobulins and hepatitis. This patient had negative tests for hepatitis A and B. However, he had a positive test for anti-hepatitis C and > 1,000,000 HVC RNA copies/ml on quantitative PCR testing. Cryoglobulins were positive (1mm), and SGOT was minimally elevated (59 U/L; N=15-46). The combination of livedo reticularis and arthritis in this hepatitis C-positive patient led to the diagnosis of hepatitis C-associated arthritis, rather than rheumatoid arthritis. Inflammatory arthritis involving the shoulders, knees, wrists, hands, and hips has been described in 2 individuals with similar findings (J Am Acad Derm 37:659, 1997; Neth J Med 51:225, 1997). The high titer rheumatoid factor in our case is thought to result from the cryoglobulin, produced by virus stimulation of clonal expansion of B-cells. The cold-precipitable protein in hepatitis C-infected patients is typically mixed and has rheumatoid factor activity. He as been referred to a hepatologist for liver biopsy and further management. It is expected that interferon treatment will control his disease, including the skin lesions and arthritis.

C-20

Suruchi Kaul M.D., Ron Javdan, M.D., Thomas R. Pohlman, M.D.

St. Luke’s Medical Center

Spontaneous renal artery dissections

Spontaneous isolated renal artery dissection is a rare condition, and is usually related to abnormalities in the vessel wall, such as atherosclerosis in the older patient and fibromuscular dysplasia in younger patients. We report two 40 year old, male patients with spontaneous, non-traumatic renal artery dissections. The first patient, previously healthy, presented with severe flank pain and elevated blood pressure and creatinine. Urolithiasis/pyelonephritis was initially suspected but a diagnosis of bilateral renal infarcts due to bilateral dissections was established on arteriography. The second patient, with underlying hypertension, also had severe flank pain but a normal creatinine, and dissection of the right renal artery on arteriography. Both patients underwent successful surgical repair with saphenous vein patch angioplasty and have done well on follow-up. The lethality of renal artery dissections and the success of revascularization, which preserves renal function and ameliorates associated renovascular hypertension, emphasize the need for an aggressive approach to the recognition and treatment of this entity. We reviewed the literature on this uncommon condition and present an analysis of its pathology, clinical features, diagnosis, and therapeutic management options.

C-21

Mary M. Klix M.D., Burton Needles, M.D., FACP

St. John's Medical Center

Thrombophilia in women on oral contraceptives: we should not just blame the pill

A 31-year-old female with a 2-day history of a throbbing, occipital headache, presented to the emergency room. She developed nausea and vomiting several hours after the headache began. Her medical history included irritable bowel syndrome and polycystic ovary disease. Her only medication was oral contraceptives which she’d been taking for 3 years. Family history was negative for thrombophilia or related disorders. Physical exam was unremarkable, as was the neuro exam. The PT & PTT were normal. Cranial CT scan revealed a right transverse and sigmoid sinus thrombosis.

The patient was started on heparin, decadron and phenytoin and admitted to the hospital. On day 3, she became unresponsive with fixed, dilated pupils. She was intubated and repeat CT revealed acute cerebral edema obliterating all ventricles. The interventional radiologist was able to use intracranial urokinase to lyse the superior sagittal sinus and bilateral transverse sinus thromboses. Venous flow markedly improved. On hospital day eight, she was extubated, was able to speak and follow simple commands.

Lab investigation of the patient’s hypercoagulable state revealed: Normal, nonfasting homocysteine levels; low Protein C & S Activity, (she’d received one dose of warfarin on day 2); heterozygosity for the Factor V Leiden mutation; negative lupus anticoagulant and negative prothrombin promoter gene mutation.

There are many known predisposing factors for thrombophilia. In young women who wish to begin OCP therapy, a careful medical and family history should be obtained. If they later present with unusually located thromboses, underlying risk factors should be sought. Congenital heparin cofactor 2, Protein C & S deficiencies; activated protein C resistance, (some due to Factor V Leiden); prothrombin promoter gene mutation; antiphospholipid syndrome: lupus anticoagulant: sticky platelet syndrome; tissue plasminogen activator defects and others can cause patients to be more susceptible to thrombophilia. We can evaluate for many of these factors, but some values are altered by anticoagulants. Protein C and S are among these, and they are significantly reduced within 48 hours of beginning warfarin therapy. Factor V Leiden, conversely, is detected by DNA analysis, and is unchanged by drug therapies. Patients who are heterozygous for this mutation, which is believed to be part of the most common known risk factor for venous thrombosis, (present in as much s 15% of the population), increase their risk of a significant event by 5 to 10 times that of the general population.

C-22

Roshan Kothandaram M.D., Morey Gardner, M.D.

St. Mary's Medical Center

Blastomycosis presenting as lobar consolidation in an immunocompetent host

Blastomycosis is a rare cause of pneumonia in an immunocompetent host. A 33 year old, immunocompetent, Caucasian male developed consolidation of his right upper lobe. Patient failed to respond to multiple courses of antibiotics over a 3 week period. A repeat chest x-ray was unchanged. A CT Scan of the chest confirmed consolidation in the right upper lobe. PPD was negative, as was HIV serology. A CD4 T cell count was increased at 1,962. A CT guided biopsy of the lesion showed budding yeast which was shown on culture to be blastomyces. He was initially treated with Amphotericin B but developed rapid nephrotoxicity prompting a change to oral Itraconazole 400 mg po daily which he tolerated well. Subsequent testing of the isolate demonstrated susceptibility to both Amphotericin B and Itraconazole. He improved steadily and treatment was continued for 1 year. A chest x-ray at that time showed only RUL residual fibrosis. Fungal pneumonias may mimic bacterial pneumonias. Lobar consolidation unresponsive to antibacterial therapy should lead to an aggressive diagnostic approach.

C-23

Dhanunjaya Lakkireddy M.D., Manohart Gowda, M.D., 
     Scott Lerner, M.D., Vincent Lem, M.D., Schwartz Bruce, M.D.

University of Missouri--Kansas City

A 68 year old Caucasian female with 2 week history of upper respiratory tract symptoms, nasal congestion, cough-productive of yellow to green colored sputum and intermittent fevers (101° to 102° F) was evaluated and treated with Bactrim DS for four days and then Ceftin for one week with no improvement. After ten days of outpatient therapy, she was admitted for treatment with intravenous antibiotics with a diagnosis of community acquired pneumonia.

Physical exam revealed a tachycardia, fever (101°F), bronchial breath sounds and course inspiratory and expiratory wheezes with good oxygenation (98%) on room air. Chest x-ray showed extensive opacification of the left upper lobe with a patchy alveolar infiltrative process. Laboratory data showed leucocytosis with lymphopenia, neutrophilia and bandemia, elevated alkaline phosphatase and erythrocyte sedimentation rate. Routine cultures of blood, sputum, and urine were negative.

Hospital course: After two days intravenous Levofloxacin, the patient had no improvement clinically. Extensive fungal, viral, bacterial and connective tissue serologic studies were negative. CT scan of the chest showed extensive infiltrate with air bronchogram present of the left upper lobe, lingula and left lower lobe with perihilar calcification. Bronchoscopy revealed friable tissue obstructing the left upper lobe bronchus with unremarkable bronhcoavelolar lavage and transbronchial biopsy. Intravenous antibiotics were changed to Cefazolin and Primaxin with no further clinical improvement by day 15. At this point a video assisted thoracoscopic lung biopsy was performed. Histopathologic findings were consistent with bronchiolitis obliterans with organizing pneumonitis (BOOP). Patient was started on IV Solumedrol with substantial resolution of infiltrates on CXR within two weeks of starting therapy. Patient was continued on outpatient oral Prednisone for three months with near complete resolution of her initial left lung infiltrate.

BOOP traditionally presents as a bilateral pulmonary disease. This unusual case of unilateral BOOP is very interesting and should be entertained in the differential diagnosis of unresolving unilateral pulmonary infiltrates. It is very important to obtain a lung biopsy for confirmation and then treat aggressively with long term corticosteroid therapy.

C-24

Bahaeldeen A. Laz M.D., David W. Ortbals, M.D., FACP

ST. LUKE'S MEDICAL CENTER 

Infection of knee joint prosthesis with tularemia

Introduction: Prosthetic devices are particularly vulnerable to infection which can cause major morbidity and mortality. Francisella tularensis is the causative organism of Tularemia (Rabbit fever), which is an acute infectious disease usually characterized by a primary ulcerative local lesion, lymphadenopathy, atypical pneumonia, and a typhoid-like febrile illness. It is not described as a cause of prosthetic joint infection.

Abstract: 81 year-old male with degenerative joint disease. The patient underwent total right knee joint replacement in January ’98, and had been doing well until he presented in July ’98 with a two week history of increasing pain and swelling in his right knee associated with a low-grade fever and chills. The patient is a farmer from Missouri and he recalled skinning squirrels in June, and he has had two episodes of tick bites three weeks before presentation. Physical examination reveals no skin infection or lymphadenopathy. Examination of the right knee showed a tender, swollen joint with 2+ effusion and restriction of motion, the patient had knee joint fluid aspiration and the culture grew. Francisella tularensis, with a negative blood culture, the patient was admitted for intravenous Gentamicin and underwent surgical irrigation and debridement of his right knee. He recovered completely after three weeks of IV Gentamicin and oral ciprofloxacin.

R-8

Jamal Makhoul M.D., S. J. Birge, M.D.

Washington University

Early estrogen depravation: a risk factor for dementia

To test our hypothesis that estrogen deficiency plays a role in modifying dementia expression in elderly women, we compared the severity of dementia ascertained by the Short Blessed Test (SBT) and the Clinical Dementia Rating Scale (CDR) in two groups of women with surgical Vs natural menopause.

The surgical menopause group (SM) had a current age of 79.64±6.4 yr. and mean menopausal age of 44.67±5.11 yr. (n=28) and the natural menopause group (NM) had a current age of 80.41±6.41 and mean menopause age of 50.50±5.05 (n=64). The two groups did not differ with respect to education (SM, 10.7yr. Vs NM, 10.7yr.).

Comparison of Dementia Severity:

Measure SM NM P

CDR 1.77±0.84 1.12±0.58 0.000

SBT 19.82±7.17 14.13±8.44 0.001

The results indicate that despite comparable ages and education, women with a SM had more severe or advanced dementia than women with a NM and suggest that estrogen deficiency associated with a hysterectomy may modify the expression of Alzheimer’s disease.

C-25

Danijela Mataic M.D., Donald J. Kennedy, M.D.

ST. LOUIS UNIVERSITY  

Bilateral shoulder osteomyelitis due to an anaerobic microorganism

The patient is a 42 year old female with a history of intravenous drug abuse who presented with alcohol induced acute pancreatitis. She had a low grade fever, WBC of 11,300/mm3 and bibasilar pulmonary infiltrates. Blood cultures from the second day of hospitalization were positive for Streptococcus mitis and Clostridium sporogenes. The patient had no heart murmurs and transesophageal echocardiograms failed to demonstrate vegetation. Treatment with intravenous ceftriaxone and metronidazole for a possible right-sided endocarditis was begun. The patient continued to have fever, leukocytosis and increasing pain in both shoulders. Plain films and MRI were consistent with osteomyelitis and fasciitis. The patient underwent an open drainage procedure of shoulder joints. Purulent material was obtained, bone and synovial fluid cultures were positive for Clostridium sporogenes. The patient remained afebrile with normalization of her white blood cell count. She was discharged to a skilled nursing facility for 6 weeks of antibiotic therapy.

We have described an unusual case of bilateral, symmetric hematogenous osteomyelitis and arthritis with the anaerobe Clostridium sporogenes. The reported incidence of anaerobic osteomyelitis is less than 1% and may be the result of bacteremia. It is possible that IVDU was a portal of entry of bacteremia in this patient with subsequent seeding of shoulder joints. Treatment consists of a combination of surgical drainage and prolonged administration of high-dose antimicrobial therapy.

C-26

JingJuan Min M.D.. Patrick O"Donnell, M.D. Thomas Pohlman, M.D.

St. Luke’s Medical Center

Meningismus caused by spinal subarachnoid hemorrhage due to clear cell meningioma

Subarachnoid hemorrhage (SAH) is a catastrophic condition most commonly due to cerebral saccular aneurysm and arteriovenous malformation. We present a case of spinal SAH caused by a clear cell meningioma at L2 level.

A 18-year-old male presented clinically with symptoms mimicking meningitis or SAH with severe headache, neck stiffness, leukocytosis and low grade fever. The coexisting symptoms of low back pain and vague lower extremity paresthesia prompted MRI of spine, which disclosed an intradural extra-axial spinal tumor at L2 level with evidence of severe SAH. This finding deferred a lumbar puncture and cerebral arteriogram. Patient subsequently sent through decompressive laminectomy and tumor excision. Histological studies revealed a benign clear cell meningioma.

SAH is rarely caused by an intraspinal tumor. Spinal SAH should come under differential diagnosis when patient was suspected having SAH or meningitis, esp. with symptoms of back pain and only low grade fever. Our finding proved the value of MRI examination in tumor of the spine in the pertinent clinical setting.

R-9

Osama Mitri M.D.,  C. Shah, M.S.,  N. Winer, M.D.

UNIVERSITY OF MISSOURI--KANSAS CITY

Vascular compliance and risk of cardiovascular disease

Background: vascular compliance is probably reduced long time before the clinical evidence of cardiovascular disease, and it is considered by some a predictor of cardiovascular disease.

Objective: to detect factors that might affect vascular compliance in young healthy subjects, and to detect whether family history of cardiovascular disease affects vascular compliance at young age.

Results: 143 subjects ages 18-33 were enrolled, small artery elasticity was significantly reduced in females (P<0.0001), with increased age (P= 0.0011), lower BMI (P=0.0003), and a higher heart rate (P<0.0001), large artery elasticity was significantly reduced in females (P=0.004), and with lower BMI (P=0.015). There was no significant difference in vascular compliance in relation to family history of cardiovascular disease, but there was a trend towards decreased small artery elasticity in subjects with parental history of hyperlipidemia (P=0.062).

Conclusion: Gender and BMI significantly affected small and large artery elasticity, age and heart rate significantly affected only small artery elasticity. There was a trend towards a reduced small artery elasticity in the presence of parental history of hyperlipidemia.

C-27

Poombavai O. Nagappan M.D., Jill Ohar, M.D.

ST. LOUIS UNIVERSITY  

Acute renal failure due to acetaminophen toxicity

A 19 year old white female with a past medical history of depression was hospitalized for acetaminophen toxicity. The patient had consumed 60 gm of acetaminophen and presented to the emergency room 20 hours thereafter. She had 15 to 20 episodes of vomiting prior to arrival at the emergency room. Vitals: BP 128/80 PR 105 RR22 Temp 99.2 HT 5’4" Wt 58.9K. The physical examination was significant for asterixis. The acetaminophen level 20 hours after ingestion was 56 mcg/ml (toxic range). Admission labs were as follows; SGOT 168 U/L, SGOT 171 U/L, Bilirubin 1.9, Alk 123 U/L, BUN 10 mg/dl Cr 0.7 mg/dl PT/INR 22.5/2 NH 119 umol/L. On day #2 (Peak values) SGOT 15130, SGPT 9470 PT/INR 3.2. The patient was started on N-acetyl cysteine on day #1 after acetaminophen ingestion. On day #3 acetaminophen levels were undetectable. The patient was started on loop diuretics. On day #6 urine output had decreased to 30 ml;/24 hours and creatinine had gradually increased to 6.3, chest x-ray showed pulmonary vascular congestion and right pleural effusion. Urinalysis: specific gravity 1.014, pH 5, protein 30, glucose 250 mg/dl, ketones negative, blood large, nitrite: neg, leukocyte esterase: trace, RBC 2, WBC 2, bacteria occ, eosinophils: neg. On day #8 liver function test had normalized. On day #11 the creatinine had peaked at 14.4 mg/dl. And the urine output had gradually increased to 4800ml. The patient did not need hemodialysis and there was spontaneous remission of kidney function by day #13 when the creatinine level improved to 6.1 mg/dl.

Review of literature showed that acute renal failure secondary to acetaminophen poisoning occurs alone or in combination with hepatic necrosis. Acute renal failure occurs in less than 2% of all acetaminophen poisonings and manifests as acute tubular necrosis. Thus, although liver failure remains the major cause of death from acetaminophen overdose, this case report emphasizes that acute renal failure is a source of considerable morbidity in these patients.

C-28

Manisha S. Patwardhan M.D., Thomas R. Pohlman, M.D., 
     Alan R. Spivack, M.D.

St. Luke’s Medical Center

Exercise induced rhabdomyolysis (McArdle's Disease) - a case report

McArdle’s disease is a disorder of carbohydrate metabolism in which there is a deficiency of myophosphorylase enzyme. Clinical picture is characterized by exercise intolerance with muscle pain, stiffness and weakness during strenuous activity. Serum creatinine-phosphokinase at rest is variable increased and myoglobin may be found in urine. Diagnoses is confirmed by muscle biopsy with specific enzyme histochemistry showing absence of phosphorylase activity.

We present a 34 year old parole officer who presented to the E.R. with generalized myalgia and passing dark colored urine. Exam-non-focal. Lab data- BUN-119, creatinine-11.8, K-4.9, LDH-1473, total CK-21, 107. Urine analysis- Blood 3+, RBC-3-5. Renal ultrasound – bilaterally large kidneys with no focal mass lesion or hydronephrosis. Patient improved with vigorous I.V. hydration with his creatinine decreasing to 1.6, BUN – 27, LDH-301, total CK-2430 twelve days later. Patient had a muscle biopsy for repeated episodes of myalgia and myoglobinuria after vigorous activities which showed myophosphorylase deficiency consistent with the diagnosis of McArdle’s disease.

This disease has an autosomal recessive pattern of inheritance. Avoiding vigorous activity needs to be emphasized to the patients to prevent muscle necrosis following prolonged, severe exercise. I.V. infusion of glucose, fructose, glucagon or sublingual isoproterenol have been suggested but none are of therapeutic value in the long term management.

C-29

Motaz Alshaher M.D., Joseph Dooley M.D., Yugandhar Chimata, M.D.,
     Bahaeldeen Laz, M.D.

St. Luke’s Medical Center

Pituitary apoplexy presenting as aseptic meningitis, case report

Pituitary apoplexy is a rare disease resulting from hemorrhage into a pituitary tumor. It characteristically presents as a sudden onset of headache, partial ophthalmoplegia and blindness.

We present a 43 year-old female who came in with nausea, vomiting, generalized headache and neck stiffness for two days prior to admission. Lumbar puncture yielded a turbid CSF with 1360 WBCs (88% seg) and 210 RBCs, 73 glucose and 191 protein. The patient was treated initially with IV antibiotics for possible bacterial meningitis until her CSF culture came negative. She was feeling slightly better with improvement in her headache until the 5^th day of admission when she developed acute onset of bitemporal visual field defects. MRI of the head revealed a suprasellar mass. The patient underwent a left frontal craniotomy with subtotal removal of the mass. Pathology later showed an acute hemorrhagic infarction of a pituitary adenoma.

Conclusion: Pituitary apoplexy should be considered in the differential diagnosis of "aseptic meningitis".

R-10

Sonny Saggar, MBBS,  William Phillips, M.D.   

St. Luke’s Medical Center                                                                                                                      

Impact of cardiac catheterization on the integrity of the cardiac markers, myoglobin and troponin-I

Background: The ECG is non-diagnostic in more than 50% of cases of acute myocardial infarction (AMI). The importance of the clinical findings and, secondarily, cardiac marker results, cannot be underestimated. Guidelines currently exist for the use of ‘early’ and ‘definitive’ markers in ‘rule-out’ protocols, namely, myoglobin (MB) and troponin-I (cTnI). Elevations of such cardiac markers can occur after coronary artery angiography, but the range and duration of elevations have not been well-delineated. Whether these elevations are a result of AMI or lesser damage with release of myocardial proteins is not known. If this is the case, these elevations should not be attributed to AMI.

Goal: We wish to investigate the impact of coronary angiography on cardiac markers and thereby either champion or denounce their use in post-angiography presentations, such as chest pain.

Methods: Patients undergoing elective cardiac catheterization will be recruited for evaluation of the effect of the procedure on their baseline MB and cTnI levels. The standard for AMI diagnosis is as defined by World Health Organization criteria. Specific inclusion and exclusion criteria will apply to minimize confounding variables, Each patient will be consented, interviewed and examined: previous medical records will be reviewed. Pre- and post- catheterization tests will be done. Whole blood will be submitted and ECGs will be obtained at selected intervals in relation to the procedure. Specimens will be frozen and stored for analysis after patient discharge. There is no need for a comparison group, but previous stress tests may be taken into account.

Conclusions: In the event that cardiac marker elevations are noted in a significant number of electively catheterized patients, consideration will have to be given towards the application of a modified ‘rule-out MI’ protocol when faced with a recently –catheterized patient presenting with symptoms such as chest-pain.

C-30

Sanjay Sharma M.D.

Washington University

Arsenical keratosis, squamous cell carcinoma and pancreatic adenocarcinoma after 30-40 year ingestion of arsenic

This case describes complication of chronic arsenic ingestion in an 83-year-old white female who used arsenic insecticide on her potato plants for approximately 30-40 years. The classic dermatologic findings included palmar arsenical keratosis and squamous cell carcinoma of the hand. In addition, she had pancreatic adenocarcinoma which was complicated by duodenal invasion resulting in a fatal GI bleed. The association of this latter malignancy with arsenic exposure is unclear. After extensive review of the literature, it appears that ingestion has generally been limited to poisonings, industrial exposure, insecticide exposure, ingestion form contaminated well water, iatrogenic consumption from Fowler’s solution once widely used to tread psoriasis, and Chinese herbal medicines. The documented complications in chronic use include dermatologic changes as described in this patient, multiple internal malignancies, and hepatic dysfunction. There is a renewed interest in the use of arsenic for acute promyelocytic leukemia. Although it is doubtful that the internist will see a marked increase in toxicity, especially chronic manifestations, the findings as described in this patient are classic and thus, easy for the internist to identify.

Karan D. Singh M.D., Timothy S. Vaughn, M.D., Darta B. Hess, M.D.

University of Missouri--Columbia

A 73 years old man with a rash, rapidly progressive renal failure and abrupt onset of dyspnea

A 73 year old man was seen in general medicine clinic for a progressive rash on both lower extremities involving the extensor surface of knees, dorsum of feet and the toes. The rash was thought to be livedo reticularis. On routine laboratory evaluation, the blood urea nitrogen and serum creatinine concentrations were found to be 69 milligrams per dl and 5.7 milligrams per dl respectively. During the six months prior, the creatinine concentration had ranged between 1.0 and 1.4 milligrams per dl. He was admitted for the evaluation of rapidly progressive renal failure with the rash. He had a long-standing history of hypertension, hyperlipidemia dn chronic obstructive pulmonary disease. He was on treatment with an ACE inhibitor, a statin, inhaled beta-2 agonists and steroids. He had smoked one and a half packs per day of cigarettes for 50 years. Physical examination revealed a pale, averagely built man, Temperature was 3.69 degrees Celsius, pulse was 88, respiratory rate was 20 per minute, blood pressure was 146/80. JVP was 6cm above angle of louis. Inspiratory crackles were heard over the lower third of both lungs. A grade 2/6 ejection systolic murmur was maximal at the aortic area. No S3 or S4 was heard. There was no peripheral edema. Femoral pulses were 2+ and dorsalis pedis were 1+.

A proteinuria of one gram per day was noted. Urine sediment contained two WBCs and six RBCs per high-power field. Serum protein electrophoresis and urine protein electrophoresis were normal. A test for Bence Jones proteins was negative. Ultrasonographic examination of the kidneys was normal. CT scan of the abdomen revealed a 4.9cm infrarenal abdominal aortic aneurysm. An echocardiogram revealed left ventricle hypertrophy, normal left ventricular ejection fraction, mild mitral regurgitation, left atrial enlargement and aortic sclerosis. A recent lipid profile had revealed a cholesterol concentration of 216 milligrams per dl and a triglyceride concentration of 235 milligrams per dl. Other lab results were unremarkable. An electrocardiogram was unremarkable. X-ray of the chest was consistent with emphysema and bilateral small pleural effusions, Hepatitis serologies were negative, serum cryoglobulins were negative, and a test for anti-neutrophilic cytoplasmic antibody was negative.

While in the hospital, he became progressively dyspneic. There was no orthopnea. Repeat Chest x-ray showed bilateral scattered infiltrates. He was afebrile with no cough or sputum production. Renal function continued to deteriorate. Arterial blood gases revealed a p02 of 45 pC02 of 40 pH of 7.43 on room air. A procedure yielding the diagnosis was performed.

Over the ensuing days, Renal and respiratory function continued to deteriorate. Hemodialysis was instituted. He was also treated with IV Methylprednisone, empiric antibiotics and bronchodilators. None of the therapies halted the course of the disease. Patient and family declined intubation and mechanical ventilation. He died on hospital day 28. Autopsy was performed.

C-32

Kamala Tamirisa M.D., Ashish Singh, M.D., Cesar Keller, M.S.

ST. LOUIS UNIVERSITY  

Diagnosis of acute pulmonary embolism using pulmonary artery catheter

A 48 year old male firefighter was admitted due to respiratory failure following an explosive fire accident and acute lung injury. He was intubated and aggressively treated for carbon monoxide inhalation. Radiological imaging was negative for fractures. DVT prophylaxis was initiated with heparin and pneumatic compression stockings. The patient improved over the next 3 days, although he remained intubated for airway protection.

On the third day of hospitalization, he decompensated with severe hypoxia and hypotension which remained resistant to oxygen, fluids and vasopressors. Lab results: ABG – 7.37/46 pCO2/ 75 pO2/93% on 70% FIO2; Chest X-Ray, EKG and cardiac enzymes were negative; CBC and chemistry remained unchanged; Duplex scan of the lower extremities was negative for DVT. Simultaneously, a PS catheter was floated and the following tracing was obtained.

C-33

K. George Thampy Ph.D., M.D., M. Louay Omran, M.D.

ST. LOUIS UNIVERSITY  

Gentamicin-induced acute psychosis

The most common adverse effect of Gentamicin on the nervous system involves the eighth cranial nerve resulting in auditory (loss of hearing, ringing or buzzing, or a feeling of fullness in the ears) and or vestibular (clumsiness, dizziness, nausea, vomiting and unsteadiness) disturbances. Gentamicin is also known to cause encephalopathy and seizures. However, the neuropsychiatric effect of gentamicin are extremely rare. Here we describe an 80-year old woman with no previous history of psychiatric disorders who developed acute psychosis within 48 hours after starting gentamicin treatment for Enterobacter and Proteus cystitis. A number of potential causes, including electrolyte abnormalities, metabolic derangements, myocardial infarction, psychotropic drugs, intracranial bleed, stroke, intra-abdominal pathology, were ruled out. The patient had normal renal function and therapeutic serum levels of gentamicin during the episode. Upon withdrawal of gentamicin her psychosis resolved within 24-48 hours.

R-11

K. George Thampy, Ph.D., M.D., M.J. Haas, Ph.D., J-P Li, M.D., 
   A.D. Mooradian M.D.

ST. LOUIS UNIVERSITY  

Age-related changes in mRNA expression and activity of acetyl-coenzyme A carboxylase

Acetyl-CoA carboxylase (ACC) catalyzes the rate-limiting step in the synthesis of long chain fatty acids. Since aging influences adiposity we studied the activity of ACC and its mRNA expression in livers of 4mo, 12 mo and 27 mo-old F344 rats. The activity of ACC (mU/mg protein; Unit= m mole/min) in liver homogenates from 4 mo old rats was 1.01±0.14. There was an 80% increase in activity (1.83±0.27) in 12 mo-old rats (p=0.019). However, there was a significant decline in activity to 0.46±0.06 in livers of 24 mo-old rats (p=0.0018). The total activity of ACC (per g liver) followed the above trend. The enzyme from all age-groups was purified by avidin-affinity chromatography. The purified preparation migrated as a major protein band (Mr 262,000) on SDS-polyacrylamide gels. The specific activity of the purified preparation was 1.5, 1.8, and 1.8 U/mg for 4 mo, 12 mo and 24 mo old, respectively. The alkali labile phosphate content was 5.66±0.17, 5.64±0.21 and 6.21±0±.35 mols Pi/mole subunit for 4 mo, 12 mo, and 24 mo-old, respectively. Thus the 3 preparations had similar specific activity and similar phosphate content. The age-related changes in ACC mRNA-expression, as studies by RNAse protection assay, correlated with changes in total enzyme activity. In conclusion, the observed changes in the total activity and expression of ACC mRNA are in parallel with known age-related changes in adiposity which increases throughout early life to a peak level in late mid-life (12-18 mo-old) and then declines as the animal ages further.

Gentiana Voinescu M.D., C. Edward LaValle III, M.D.,  Leslie Hall, M.D.

University of Missouri--Columbia

Prothrombin 20210A gene mutation: a common abnormality causing an unusual abdominal venous thrombosis

A mutation in the prothrombin gene (GV A20210) has been associated with higher plasma prothrombin levels and an increased tendency for venous thrombosis. We present a 39-year-old healthy white female who was seen in a local emergency room seven days prior to admission to our hospital, complaining of gradual onset of midepigastric pain, mild nausea and low grade fever. At the 2-day follow up with her primary care physician, an ultrasound of the abdomen was diagnostic for extensive thrombosis of the portal and superior mesenteric vein. She was transferred to our facility after laboratory tests were drawn to assess her for hypercoagulable states. These included: protein C, protein S, anticardiolipin antibodies, activated protein C resistance and lupus anticoagulant.

On admission the patient denied abdominal trauma, previous clotting problems in her or her family, smoking, birth control pills usage, weight loss or previous pancreatic problems. The patient was on no medications. Physical examination revealed moderate epigastric tenderness, no rebound or guarding. The patient was started on intravenous heparin. A work-up for myeloproliferative disorders, tumors, and pancreatitis was negative. Findings included a normal complete blood count, amylase, lipase, chest X-ray, and mammogram. Computerized tomography of the abdomen showed occlusion of the superior mesenteric vein and portal vein. Since the hypercoagulable profile was negative to date, a polymerase chain reaction assay for prothrombin 20210A gene mutation was obtained. This revealed that the patient was heterozygous for the mutation. She began coumadin therapy. The computerized tomography of the abdomen repeated after six weeks of anticoagulation demonstrated interval reconstitution of blood flow through the portal vein and improvement in size of superior mesenteric vein thrombosis.

Laboratory tests to define the hypercoagulable state are continually being developed. This newly described mutation appears to be a frequent, although often overlooked cause of unexplained thrombosis. It is associated with at least a three-fold risk of developing venous thrombosis.

Keywords: thrombosis, prothrombin 20210A gene mutation, risk factors.