1/184. Idiopathic thrombocytopenic purpura after a living-related liver transplantation.BACKGROUND: Idiopathic thrombocytopenic purpura (ITP) is a rare complication after liver transplantation. We describe three cases of ITP in pediatric patients after a living-related liver transplantation (LRLT). methods: Of 266 patients who underwent an LRLT between June 1990 and June 1996, severe thrombocytopenia developed in three pediatric patients after transplantation, and ITP was also diagnosed. The original disease was biliary atresia in all cases, and the patients were given a partial liver graft from a living-related mother and subsequently treated with tacrolimus and low-dose steroids as an immunosuppressive regimen. RESULTS: The duration until the onset of ITP after transplantation in the three cases was 1 day, 3 months, and 13 months, respectively. The platelet-associated IgG levels increased in all cases. A preceding viral infection was suspected in two of the three cases. All patients were treated with intravenous gamma globulin with a transient recovery of thrombocytopenia in two cases and a sustained recovery in another. CONCLUSIONS: Transplant clinicians need to be aware of the possibility of ITP complication because a sudden onset of severe thrombocytopenia can occur even in patients who are apparently doing well after undergoing an LRLT.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
2/184. Extrahepatic biliary atresia associated with trisomy 18.A case of extrahepatic biliary atresia (EBA) associated with trisomy 18 is presented. A 1-month-old boy was suspected to have alagille syndrome with obstructive jaundice, a systolic heart murmur, growth retardation, and a small, pointed chin. However, surgery and chromosomal analysis revealed EBA associated with trisomy 18. Chromosomal examination must be performed in patients with jaundice and congenital anomalies. It is possible that EBA in trisomy 18 syndrome is due to a chromosomal disorder.- - - - - - - - - - ranking = 5keywords = atresia (Clic here for more details about this article) |
3/184. liver transplantation in patients with situs inversus.Two patients with situs inversus and biliary atresia were treated with hepatic transplantation, one with an auxiliary liver and the other with an orthotopic graft which was placed using a piggy-back technique. Both transplants functioned well initially. The auxiliary liver was rejected after 1 1/2 months, and the patient died after an attempt at retransplantation many months later. The recipient of the orthotopic liver has perfect liver function 10 months postoperatively.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
4/184. Antenatal diagnosis of biliary atresia (noncorrectable cyst type): a case report.At 32 weeks of gestation a cystic mass was identified in the hepatic hilum of a fetus by maternal sonography. laparotomy was performed at 39 days of life after a diagnosis of correctable type of biliary atresia (Type I). A cystically dilated extrahepatic duct, in which the proximal and distal sides of the common bile duct were occluded (Type III with cyst, noncorrectable type), was identified by operative cholangiography. A standard Kasai operation was performed, and 1 year after operation the patient was doing well and was jaundice-free. From this experience in routine maternal sonography, a cystic mass in the hepatic hilum may also suggest Type III biliary atresia with a cyst.- - - - - - - - - - ranking = 6keywords = atresia (Clic here for more details about this article) |
5/184. Endoscopic injection sclerotherapy for esophagogastric variceal bleeding in children with biliary atresia.BACKGROUND/AIMS: To determine the safety and effectiveness of endoscopic injection sclerotherapy (EIS) for children with biliary atresia. METHODOLOGY: Subjects were 7 patients with biliary atresia with esophagogastric varices and variceal bleeding. Intravariceal injection using 5% ethanolamine oleate was performed under fluoroscopy until varices were eradicated. RESULTS: Endoscopic examination revealed that bleeding occurred in the junctional gastric varices in most of the cases. The mean number of EIS sessions required for obliteration of the varices was 2.3. In the observation period (mean: 21 months), recurrent esophagogastric varices occurred in 2 patients. One had variceal bleeding that was treated successfully by additional EIS. There were no severe complications associated with EIS. CONCLUSIONS: EIS under fluoroscopy was safe and effective for variceal bleeding in children with biliary atresia.- - - - - - - - - - ranking = 7keywords = atresia (Clic here for more details about this article) |
6/184. Pulmonary hypertension associated with postoperative biliary atresia: report of two cases.The authors report on 2 patients with biliary atresia in whom pulmonary hypertension (PH) developed in the long-term follow-up after hepatoportoenterostomy. Both had portal hypertension and had undergone distal splenorenal shunt. dyspnea developed around 14 to 15 years of age. cardiac catheterization showed pulmonary artery pressure (PAP) of 99/37 (58) and 67/32 (48) mm Hg, respectively, which did not respond to vasodilators. One patient suffered from respiratory tract infection followed by right heart failure and subsequent death at 20 years of age. Postmortum histological findings exhibited severe thickening of the pulmonary artery wall. PH may grow insidiously even after successful hepatoportoenterostomy. Careful monitoring of PAP and hemodynamic response of PAP to vasodilators is essential for evaluating the reversibility of PH and making treatment decisions.- - - - - - - - - - ranking = 5keywords = atresia (Clic here for more details about this article) |
7/184. Unusual biliary scan appearance in a child with a transplanted liver with hepatic arterial thrombosis: a case report.A 5-year-old girl with biliary atresia and a subsequent Kasai procedure is described. She had clinical symptoms suggestive of rejection after a recent orthotopic liver transplant A hepatobiliary scan showed partial hepatic infarction and a biloma in the infarcted area.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
8/184. biliary atresia with hyperandrogenic amenorrhea: case report.The etiology of hyperandrogenic amenorrhea in a 16 year-old girl after a successful Kasai operation for biliary atresia was unclear. Delayed puberty and menarche were observed. There was no luteinizing hormone-follicular stimulating hormone surge. A provocative luteinizing hormone-releasing hormone test showed a normal response. Peripheral aromatization of androgens appeared to function normally. However, plasma levels of sex hormone-binding globulin and total testosterone were high and the free testosterone level was normal. The anovulatory menstrual cycle continued after menarche at 18 years of age. A combination of estrogen and progesterone therapy was effective. The etiology might be associated with delayed metabolic clearance of testosterone accompanied by the compensatory cirrhosis and portosystemic shunt.- - - - - - - - - - ranking = 5keywords = atresia (Clic here for more details about this article) |
9/184. Combined pure esophageal atresia, duodenal atresia, biliary atresia, and pancreatic ductal atresia: prenatal diagnostic features and review of the literature.The authors recently managed a case of combined pure esophageal, duodenal, biliary, and pancreatic ductal atresia in a fetus and newborn with trisomy 21. The authors present a case report and review of the literature, emphasizing the prenatal radiographic features of the combined lesions and the high incidence of associated anomalies including Down's syndrome. Prenatal suspicion of these anomalies warrants karyotype analysis as well as careful pre- and postnatal screening for other anomalies.- - - - - - - - - - ranking = 17keywords = atresia (Clic here for more details about this article) |
10/184. Branchial cleft anomaly, congenital heart disease, and biliary atresia: Goldenhar complex or Lambert syndrome?The features of Goldenhar complex have been well-described and classically include branchial arch abnormalities, epibulbar dermoid and vertebral abnormalities. We have identified an infant with these features in association with complex congenital heart disease and intrahepatic biliary atresia. Although Lambert described an autosomal recessive disorder with an association of biliary atresia and branchial arch abnormalities, none of those cases had epibulbar dermoid. Diagnostic considerations in this case include inclusion of biliary atresia as a new feature in the expanding spectrum of the Goldenhar complex, versus Lambert syndrome with epibulbar dermoid.- - - - - - - - - - ranking = 7keywords = atresia (Clic here for more details about this article) |
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