| We report two young men with clinical and laboratory evidence of macroscopic ulcerative colitis, sclerosing cholangitis, and insulin-dependent diabetes mellitus. The first patient presented at age 15 with vomiting, abdominal pain, weight loss, and abnormal liver function test results. Liver biopsy and endoscopic retrograde cholangiopancreatography (ERCP) demonstrated sclerosing cholangitis. colonoscopy with biopsy revealed ulcerative colitis which responded to sulfasalazine. Diabetes occurred at age 18 and insulin therapy was begun. The second patient was 19 at presentation with diarrhea, hematochezia, and weight loss. Proctosigmoidoscopy revealed ulcerative colitis, and sulfasalazine led to clinical remission. Three months later he developed diabetes requiring insulin therapy. At age 28, he developed elevated alkaline phosphatase, and ERCP revealed sclerosing cholangitis. At age 37 he expired from adenocarcinoma that metastasized to the liver. literature review revealed only one possible case report of this association with microscopic asymptomatic ulcerative colitis in that patient. Statistical analysis suggests that this association is real rather than a chance occurrence. An autoimmune process may be involved and a specific histocompatibility locus antigen (HLA) type may exert a regulatory influence. ( info) |
| The immunopathogenic importance of neutrophil cytoplasmic autoantibodies in ulcerative colitis and primary sclerosing cholangitis is unknown. These autoantibodies were investigated before and after liver transplantation in 9 patients with primary sclerosing cholangitis. Sera from 10 patients transplanted for metabolic disorders or hemangioma served as controls. Before liver transplantation neutrophil cytoplasmic autoantibodies, producing a perinuclear pattern by indirect immunofluorescence on ethanol fixed neutrophils, were present in all patients with primary sclerosing cholangitis. A decline in titer was noted in the first months after liver transplantation. During long-term follow up, the autoantibodies remained present and most often the titer did not differ from before transplantation. They were not directed against proteinase 3, myeloperoxidase, elastase or lactoferrin. All but one of the control patients were negative for the autoantibody. No relation was seen, before or after transplantation, with ulcerative colitis or proctocolectomy. There was no recurrence of primary sclerosing cholangitis in any of the patients as judged by liver histology. We conclude that neutrophil cytoplasmic autoantibodies remain present after liver transplantation for primary sclerosing cholangitis and that its synthesis is not related to the presence of the diseased organ(s). The primary disease process in primary sclerosing cholangitis and ulcerative colitis may well be a disturbance of the immune system. ( info) |
203/257. Localized sclerosing cholangitis in the intrapancreatic bile duct. Report of a case. A case of primary sclerosing cholangitis (PSC) localized in an isolated segment of the intrapancreatic bile duct was successfully treated by a pancreatico-duodenectomy. Although a progressively larger number of patients with PSC have recently been reported, there have been no other reports of a patient with this disease confined to the very distal part of the common bile duct. Since it is difficult to differentiate this rare disease from malignancy, surgical treatment as for a malignancy is sometimes required. ( info) |
204/257. The first liver transplant in saudi arabia and the arab world. The first liver transplant in saudi arabia and in the arab world was performed on the 30th of July, 1990, in the Armed Forces Hospital in Riyadh, on a 23-year-old male with end-stage liver disease due to sclerosing cholangitis. There were no major post-operative or post-transplantation complications. Now, sixteen months post-transplantation, the patient is doing well on immunosuppressive therapy with an almost normal liver function, and is already back at work and carrying out normal activities. This report shows that liver transplantation can be performed successfully in this part of the world. ( info) |
| A 63-year-old patient with sarcoidosis developed a clinical picture compatible with sclerosing cholangitis 20 years later. The uncommon association between these two rare diseases, and possible common pathophysiological-immunological mechanisms are discussed. ( info) |
206/257. Inflammatory pseudotumor of the liver in a patient with chronic sclerosing cholangitis. Inflammatory pseudotumor of the liver (IPL) is a rare fibro-inflammatory mass of unknown etiology, resembling a neoplasm. We report the occurrence of such a lesion in a young male Cambodian refugee to canada who had a history of chronic cholangitis. Imaging studies suggested an intrahepatic abscess. Our report suggests that the lesion in this patient may have resulted from chronic portal pyemia. ( info) |
207/257. Evaluation of AIDS-related diarrhea. A 31-year-old man was hospitalized for evaluation of chronic diarrhea accompanied by profound dehydration, abdominal pain, nausea, vomiting, and low-grade fever. He had been identified as hepatitis b surface antigen-positive in 1983 and hiv antibody-positive two years later. In 1987, after a diagnosis of pneumocystis carinii pneumonia, he had been placed on zidovudine and prophylactic pentamidine. Subsequently, thrush developed, which was treated with nystatin. The patient's gastrointestinal symptoms were of about six months' duration and originally had responded fairly well to diphenoxylate. More recently, however, he had been losing weight steadily and had required emergency room rehydration on two occasions. A search for stool ova and parasites and routine enteric pathogens, conducted by the outpatient department, had revealed cryptosporidium cysts. ( info) |
| A case of cholangitis Glandularis Proliferans (CAGP) in association with a cholangiocarcinoma of the common bile duct as described. This is the eighth case of CAGP described and the second association with cholangiocarcinoma. ( info) |
| Intra- and extrahepatic bile-duct strictures, papillary stenosis and acalculous cholecystitis have all been described in ill patients with acquired immunodeficiency syndrome (AIDS). acalculous cholecystitis associated with cytomegalovirus (CMV), cryptosporidium or campylobacter organisms has typically been described in critically ill or moribund patients. The authors report a case of acute acalculous CMV cholecystitis in a 28-year-old man who presented with abdominal pain. The patient was infected with the human immunodeficiency virus (hiv) but was ambulatory and had had no AIDS-defining illness. The patient did not have any well-recognized risk factors for acalculous cholecystitis, showing that this entity can occur in relatively healthy hiv-infected patients as well as in the terminal stages of AIDS. The diagnosis should be considered when such a patient presents with abdominal pain. Furthermore, this patient had sclerosing cholangitis of the intra- and extrahepatic bile ducts as well as papillary stenosis. The cause of the acalculous cholecystitis was presumed to be CMV, but the disease progressed despite therapy with foscarnet. ( info) |
| A patient with dermatitis herpetiformis (DH) developed abnormal liver function tests and was found to have histological features of primary sclerosing cholangitis (PSC) on liver biopsy. The association of DH and PSC has not been previously reported and the high incidence of phenotype HLA B8 and DR3 in these disorders suggests a common genetic predisposition may exist for both diseases. ( info) |