Fulminant hepatic failure caused by acute fatty liver of pregnancy treated by orthotopic liver transplantation.

A previously healthy 35-year-old woman was seen at 37 weeks' gestation with a 10-day history of fever, vomiting, diarrhea and malaise. Serum laboratory findings included elevation of serum bilirubin and AST, prolongation of serum prothrombin time and a positive monospot. A tentative diagnosis of acute fatty liver of pregnancy was made, and a healthy male infant was delivered by emergency cesarean section because of fetal distress. Over the subsequent 3 days, acute progressive oliguric renal failure, disseminated intravascular coagulation, hypoglycemia requiring intravenous dextrose infusion and pancreatitis developed; her mental status progressed to stage III encephalopathy. Quantitative computed tomography estimated the liver volume to be 770 cm3. The decision to proceed with orthotopic liver transplantation was made on the basis of progressive clinical deterioration despite aggressive support and because of her small liver size. After transplant, the patient's multisystem failure rapidly reversed. Histopathological examination of the native liver demonstrated predominantly zone 3 microvesicular steatosis with characteristic ultrastructural changes consistent with acute fatty liver of pregnancy. Southern blot analysis for Epstein-Barr virus DNA was negative. We conclude that orthotopic liver transplantation should be considered for the small group of patients with fulminant hepatic failure associated with acute fatty liver of pregnancy who manifest signs of irreversible liver failure despite delivery of the fetus and aggresive supportive care.

Chlamydia trachomatis perihepatitis with ascites.

Chlamydia trachomatis as an etiological agent in the Fitz-Hugh-Curtis syndrome has recently been reported; however, the condition has not been typically associated with ascites. We report a case of Chlamydia trachomatis perihepatitis in a young woman who had exudative ascites as a predominant clinical feature of her disease. Laparoscopy revealed characteristic "violin string" adhesions which were also identified on abdominal computerized tomographic scan.

Unusual cardiac complications of Wegener's granulomatosis.

Wegener's granulomatosis most commonly involves the sinuses, lungs and kidneys with necrotizing granulomatous vasculitis. In 12 percent of a large series of patients with Wegener's granulomatosis there was cardiac involvement, largely manifested by pericarditis and coronary arteritis. We present three patients with this disease who developed unusual cardiac complications. Patient 1 had renal failure requiring hemodialysis, pericardial tamponade requiring pericardiocentesis, and later developed constrictive pericarditis requiring pericardiectomy. Patient 2 developed pericarditis and high grade atrioventricular block, and patient 3 developed pericarditis and atrial tachycardia resistant to pharmacologic and transesophageal atrial pacing methods. All three patients greatly improved with cyclophosphamide therapy. The rhythm disturbances seen in patients 2 and 3 were attributed to coronary arteritis. The renal failure in patient 1 was due to Wegener's granulomatosis, but whether the constrictive pericarditis was due to uremic pericarditis or the pericarditis of Wegener's granulomatosis is uncertain. As patients with Wegener's granulomatosis live longer with cyclophosphamide therapy and because inpatient arrhythmia monitoring and recording has become more widespread, these uncommon manifestations of Wegener's granulomatosis may be seen more often.