1/257. Autoimmune sclerosing cholangitis: an overlap syndrome in a child. We report an overlap syndrome of autoimmune chronic liver disease and primary sclerosing cholangitis in a young girl. This could be the first such report from india. ( info) |
2/257. Successful treatment of acquired perforating dermatosis with rifampicin in an Asian patient with sclerosing cholangitis. Acquired perforating dermatosis (APD) is a very rare disorder which has been described in association with systemic diseases such as diabetes mellitus, hiv infection or lymphoma. In this report we describe a patient with APD associated with sclerosing cholangitis and diabetes mellitus who was successfully treated with rifampicin. A 33-year-old Indian woman with a history of extensive pancreatic surgery, sclerosing cholangitis and insulin dependent diabetes mellitus was referred to our unit with intractable pruritus. She was treated with cholestyramine, ursodeoxycholic acid, several analgesics, UVB therapy, topical steroids, sedative antihistamines and plasmapheresis without significant improvement. Increasingly severe itching was associated with papular skin changes limited initially to the lower limbs but which later involved her entire body. biopsy of a representative lesion showed the changes of APD. She was subsequently treated with rifampicin which produced a dramatic resolution of pruritus within 3 weeks and the skin changes progressively resolved over subsequent months. In this newly described association of APD with sclerosing cholangitis, rifampicin treatment appeared to be efficient in ameliorating pruritus and the papular skin changes typical of APD. ( info) |
3/257. Primary sclerosing cholangitis mimicking choledocal cyst type 1 in a young patient. A choledochal cyst type I was diagnosed in a 12-year-old boy in 1984. The diagnosis was made using ultrasound and confirmed using computed tomography (CT) and endoscopic retrograde cholangiopancreatography (ERCP). Instead of the usual surgical treatment, endoscopic balloon dilatation of the sphincter of oddi and the distal common bile duct was carried out using an endoscopic procedure. The patient experienced relief of symptoms, gained weight and felt healthy again. An ERCP performed in 1990, because of increasing levels of liver enzymes and clinical features of abdominal pain and fatigue, revealed typical cholangiographic findings associated with primary sclerosing cholangitis, including bile duct irregularities with diffuse narrowing and twisting of the bile ducts with localized ectatic and strictured areas. Percutaneous needle liver biopsy confirmed the diagnosis. We conclude that primary sclerosing cholangitis should be considered when interpreting ERCP films from patients who are supposed to have choledochal cysts type I. ( info) |
4/257. Primary sclerosing cholangitis in a child. Primary sclerosing cholangitis (PSC) is a rare disease in taiwan and has not been described in Taiwanese children previously. We report a 4-year-old girl who presented with prolonged fever, eosinophilia (11%), hepatomegaly, and markedly elevated serum levels of alkaline phosphatase (3,318 IU/L) and gamma-glutamyl transpeptidase (475 IU/L). Subsequent investigations including endoscopic retrograde cholangiopancreatography and liver histology confirmed the diagnosis fo PSC. Treatment with a low dose of prednisolone for 2 months and ursodeoxycholic acid during 32 months of follow-up resulted in clinical remission and halted disease progression. A high index of suspicion is necessary for physicians to diagnose this disorder in children with chronic liver disease. Our experience in this case indicates that therapy with prednisolone and ursodeoxycholic acid may be helpful for the treatment of PSC in children, and suggests the need for more trials of combined therapy. ( info) |
5/257. Recurring fibro-obliterative venopathy in liver allografts. Recurrent diseases in liver allografts are not uncommon. These occur most frequently in those transplanted for viral hepatitis b and C. We report an unusual case of recurrent process in two consecutive liver allografts received by a 37-year-old woman, who previously had an unremarkable past medical history but developed a rapidly progressive cholestatic liver failure. Histopathologic examination of the native liver showed fibroocclusive lesions of both terminal hepatic venules and portal vein branches. The exuberant fibroobliterative process created dense fibrosis with whorled appearance, and broad fibrous septa connecting adjacent central areas, and sometimes bridging portal to central areas. Dense portal fibrosis resulted in compression atrophy and loss of bile ducts. The first allograft, which failed within 3 months, showed histopathologic findings similar to that of the native liver. A liver biopsy that was performed 20 months after the second liver transplant again showed similar histopathology. The histopathologic features and clinical presentation of this patient suggest an unusual form of recurring progressive fibroobliterative venopathy causing liver failure. ( info) |
6/257. Massive T wave changes following a combined kidney and liver transplant in a young female with cirrhosis. We report the case of a young female with PSC-associated cirrhosis and chronic renal failure who developed clinical and electrocardiographic signs consistent with acute myocardial infarction after a combined kidney and liver transplant. Cardiac investigations at that time were negative and she is currently asymptomatic one year post-transplant with resolution of most of her ECG abnormalities. Although the cause of her symptoms and ECG abnormalities is not immediately apparent, this case illustrates the difficulties in interpreting abnormal cardiac investigations in transplanted patients with liver cirrhosis who may have a background of subclinical cardiac disease. ( info) |
| We present the case of a 44-year-old male patient with a history of primary sclerosing cholangitis who developed a cholangiocarcinoma with pulmonary metastasis. The cholangiocarcinoma was identified in the PET scan by its enhanced 18F-FDG-uptake. prospective studies should be performed to demonstrate whether PET will be suited to detect small and early cholangiocarcinomas, particular in young patients, who could be treated immediately and curatively by liver transplantation. ( info) |
| In countries where a living donor is the only source of the graft, the limited size of the graft is of serious concern when considering extending the procedure to adult recipients. In order to overcome this problem, auxiliary partial orthotopic liver transplantation (APOLT) was applied to the concept that the residual native liver would support the graft function until the graft expanded enough to work by itself. We herein report on a 20-year-old woman with primary sclerosing cholangitis (PSC), who received a small-size liver graft by APOLT. Computed tomography and scintigraphy showed that the graft had regenerated sufficiently 1 month after the operation. The diseased residual native liver is potentially carcinogenetic. Therefore, second-stage native hepatectomy was done 35 days after the first operation. Histopathologic examination of the resected native liver revealed biliary cirrhosis with PSC but no evidence of cholangiocarcinoma. Second-stage native hepatectomy after APOLT seems to be a curative treatment for chronic end-stage liver disease with graft size mismatch that may be as good as orthotopic liver transplantation. ( info) |
9/257. Living-related auxiliary partial orthotopic liver transplantation for primary sclerosing chonangitis--subsequent removal of the native liver. In japan, living-related auxiliary partial orthotopic liver transplantation (APOLT) is mainly indicated for small-for-size grafts. We present the case of a 24 year-old patient with primary sclerosing cholangitis (PSC) who underwent a living-related auxiliary partial orthotopic liver transplantation for a small-for-size graft, that was subsequently excised. During the transplantation procedure, the native liver was freed from the surrounding tissues, and was only connected to the body by the right hepatic artery and right hepatic vein. The auxiliary extended left lobe graft, corresponding to 22% of the estimated recipient liver volume, was orthotopically transplanted after extended left lobectomy of the recipient native liver. Post-transplant CT-volumetry showed rapid increase of the graft volume with atrophy of the native liver, and GSA scintigraphy showed dominant function of the graft. Although hyper-bilirubinemia was prolonged by the removal of the native liver on the 18th post-transplantation day, it gradually subsided after plasmapheresis was performed twice, and the patient was discharged on the 77th post-transplantation day. We conclude, based on this case, that subsequent removal of the native liver is necessary in APOLT for patients with potential hepatic malignancies. The optimal timing of the removal of the remnant native liver should be determined based on CT-volumetry, GSA scintigraphy, and the liver biopsy specimen of the graft. ( info) |
10/257. Sclerosing cholangitis associated to cryptosporidiosis in liver-transplanted children. Three children of a series of 461 pediatric liver transplant recipients developed diffuse cholangitis associated with intestinal cryptosporidium carriage. All three received immunosuppression consisting of tacrolimus and prednisone. Cryprosporidium carriage was treated with paramomycin, while immunosuppression was decreased according to graft tolerance. No other infectious pathogens were found, and no vascular problems were detected. Bile duct anastomosis was reoperated in all three, but biliary cirrhosis developed in one patient, requiring retransplantation. All three patients are alive and well, and free of intestinal parasites on follow-up. CONCLUSION: cryptosporidium intestinal infection may play a role in some cases of otherwise unexplained cholangiopathies in pediatric liver transplant recipients. This may lead to significant morbidity, including need for retransplantation. ( info) |