Resistance to activated protein C: arterial thrombosis associated with autoimmune features.

A 27 year old woman presented with recurrent cerebrovascular strokes in the setting of an ill defined auto-immune disease responsive to corticosteroid therapy. Investigation for a hypercoagulable state revealed activated protein C resistance in the absence of protein C, protein S, or antithrombin III deficiency or anticardiolipin antibodies. Her parents and sibling did not demonstrate APC resistance.

Legionella feeleii pneumonia and pericarditis.

Legionella feeleii pneumonia has been described in seven cases, three of them being immunocompromised. We describe a case of L. feeleii pneumonia and pericarditis in a healthy man.

Renal failure, paraproteinemia, and lung squamous cell carcinoma.

A 66-year-old man presented with gastrointestinal symptoms and acute renal failure. He had paraproteinemia and tested positive for antinuclear antibodies. There was no evidence for autoimmune disorder or amyloidosis, and bone marrow biopsy was not consistent with multiple myeloma.

Ischemic hepatitis associated with toxic epidermal necrolysis in a cirrhotic patient treated with cefuroxime.

Ischemic hepatitis is rare in patients with liver cirrhosis and is usually precipitated by hypotension due to gastrointestinal bleeding. We describe a cirrhotic patient who developed ischemic hepatitis as a consequence of toxic epidermal necrolysis (TEN) caused by cefuroxime.

Hepatitis C-associated cryoglobulinemia presenting with pericardial effusion.

A 54-yr-old man underwent orthotopic liver transplantation for cirrhosis caused by hepatitis C virus and associated with cryoglobulinemia and proteinuria. Four months after transplantation, the patient was reinfected with hepatitis C virus and cryoglobulinemia recurred, accompanied by increasing proteinuria, ascites, and pleural and pericardial effusion. Serum cryoglobulins and pleural and pericardial effusion disappeared rapidly following treatment with ribavirin, whereas proteinuria remain unchanged.

CT-guided biopsy of peripheral lung lesions associated with BOOP.

Diagnosis of Bronchitis Obliterans with Organizing Pneumonia (BOOP), has been carried out thus far by open lung biopsy, or by transbronchial biopsy. We report a patient who presented with fever, cough and dyspnea. A transthoracic CT-guided biopsy of a peripheral lesion resulted in the diagnosis of BOOP.

A late retroperitoneal hematoma mimicking acute appendicitis--an unusual complication of coronary angioplasty.

Peripheral vascular complications following coronary angiography and angioplasty are well established. They consist of arterial bleeding, occlusion, false aneurysm, and a-v fistula. Bleeding and thrombosis are usually evident within the twelve hours after the procedure.

Salmonella infection and pneumonia in a patient with kyphoscoliosis.

Pulmonary involvement is an uncommon extraintestinal manifestation of salmonellosis. We describe a 30 year old man with mental retardation, presenting with salmonella gastroenteritis and bacteremia. An early pneumonia evolving in the clinical setting of severe kyphoscoliosis, suggests that hematogenous spread to the lungs may occur as a result of abnormalities of the chest wall.

Molecular genetics of familial hypercholesterolemia in Israel.

Familial hypercholesterolemia (FH) is an autosomal dominant disease caused by a multitude of low density lipoprotein receptor (LDL-R) mutations. The purpose of the current investigation was to define the spectrum of mutations causing FH in Israel and determine their relative distribution among diverse origin groups. A total of 193 FH families were recruited in Israel, 54 of them through the MED PED (Make Early Diagnosis Prevent Early Death) FH program.

Gallbladder varices: a case report and review of the literature.

We present an 18 year old woman suffering from cavernous transformation of the portal vein, hypersplenism and bleeding esophageal varices. Color flow Doppler sonography showed gallbladder wall varices. A review of the literature, revealed 76 cases of gallbladder varices.

Mononeuritis multiplex: a rare complication of acute hepatitis A.

A 28-year-old man suffered from acute severe hepatitis A virus infection, documented by serologic diagnosis. During the clinical and enzymatic recovery phase, mononeuritis multiplex that involved the left ulnar and right lateral cutaneous nerves developed. It presented by sensorimotor disturbances in left arm and right thigh.

Beneficial effect of ribavirin on hepatitis C-associated cryoglobulinemia after liver transplantation.

Mixed cryoglobulinemia is a well-known complication after hepatitis C virus (HCV) infection. We report five cases in which cryoglobulinemia appeared or grossly exacerbated following orthotopic liver transplantation (OLT). Cryoglobulinemia and the associated clinical symptoms resolved or improved in two patients treated with ribavirin after liver transplantation, while plasmapheresis was ineffective in another patient.

Conservative therapy in an ERCP-induced abdominal abscess.

We describe a 30 year-old man who presented with an abdominal abscess as an unusual complication of endoscopic retrograde cholangiopancreatography with papillotomy. His presenting symptom was recurrent vomiting, while fever, abdominal pain, and leukocytosis were not significant. The abscess was observed with repeated computerized tomographic scans and completely regressed with intravenous antibiotic treatment over a three week period, leading to complete remission.

Manometric responses of human duodenum during infusion of HCl, hyperosmolar saline, bile and oleic acid.

Duodenal motor activity is incompletely understood. The purpose of this study was to define the contractile patterns of the duodenum that occur in response to rate controlled injection of various solutions. In nine healthy volunteers we placed a six channel perfused catheter, and recorded pressure activity in the antrum, pylorus and duodenum.

A patient with hypoparathyroidism, dysmorphic features and mental retardation.

There have been various reports in the medical literature concerning children with syndromes of congenital hypoparathyroidism, seizures, dysmorphic features and mental retardation. We describe a patient with hypoparathyroidism, mental retardation, micrognathia, deep-set eyes and pes cavus in a 31-year-old man. This combination of abnormalities in an adult is unique.

Merkel cell tumor in a woman with chronic lymphocytic leukemia.

We describe a 69-year-old woman with basal cell carcinoma, and chronic lymphocytic leukemia who developed Merkel cell tumor. This latter malignancy first appeared as enlarged lymph nodes in the axilla and elbow regions and responded initially to radiotherapy. Later, the patient developed obstructive jaundice which was due to pancreatic metastases of the Merkel cell tumor, documented by post-mortem examination.

Metformin-induced lactic acidosis associated with acute renal failure.

A 69-year-old diabetic woman with diffuse atherosclerosis presented with acute renal failure due to contrast nephropathy and severe metformin-induced lactic acidosis. There was a discrepancy between the patient's elevated serum creatinine level, indicative of severe renal insufficiency, and her very low blood urea nitrogen content. It is postulated that the patient's extremely severe acidosis interfered with her hepatic urea generation, contributing to this unusual biochemical abnormality.