Liver transplantation in patients with situs inversus.
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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. (+info)
Screening of newborn infants for cholestatic hepatobiliary disease with tandem mass spectrometry.
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OBJECTIVE: To assess the feasibility of screening for cholestatic hepatobiliary disease and extrahepatic biliary atresia by using tandem mass spectrometry to measure conjugated bile acids in dried blood spots obtained from newborn infants at 7-10 days of age for the Guthrie test. SETTING: Three tertiary referral clinics and regional neonatal screening laboratories. DESIGN: Unused blood spots from the Guthrie test were retrieved for infants presenting with cholestatic hepatobiliary disease and from the two cards stored on either side of each card from an index child. Concentrations of conjugated bile acids measured by tandem mass spectrometry in the two groups were compared. MAIN OUTCOME MEASURES: Concentrations of glycodihydroxycholanoates, glycotrihydroxycholanoates, taurodihydroxycholanoates, and taurotrihydroxycholanoates. Receiver operator curves were plotted to determine which parameter (or combination of parameters) would best predict the cases of cholestatic hepatobiliary disease and extrahepatic biliary atresia. The sensitivity and specificity at a selection of cut off values for each bile acid species and for total bile acid concentrations for the detection of the two conditions were calculated. RESULTS: 218 children with cholestatic hepatobiliary disease were eligible for inclusion in the study. Two children without a final diagnosis and five who presented at <14 days of age were excluded. Usable blood spots were obtained from 177 index children and 708 comparison children. Mean concentrations of all four bile acid species were significantly raised in children with cholestatic hepatobiliary disease and extrahepatic biliary atresia compared with the unaffected children (P<0.0001). Of 177 children with cholestatic hepatobiliary disease, 104 (59%) had a total bile acid concentration >33 micromol/l (97.5th centile value for comparison group). Of the 61 with extrahepatic biliary atresia, 47 (77%) had total bile acid concentrations >33 micromol/l. Taurotrihydroxycholanoate and total bile acid concentrations were the best predictors of both conditions. For all cholestatic hepatobiliary disease, a cut off level of total bile acid concentration of 30 micromol/l gave a sensitivity of 62% and a specificity of 96%, while the corresponding values for extrahepatic biliary atresia were 79% and 96%. CONCLUSION: Most children who present with extrahepatic biliary atresia and other forms of cholestatic hepatobiliary disease have significantly raised concentrations of conjugated bile acids as measured by tandem mass spectrometry at the time when samples are taken for the Guthrie test. Unfortunately the separation between the concentrations in these infants and those in the general population is not sufficient to make mass screening for cholestatic hepatobiliary disease a feasible option with this method alone. (+info)
Partial left lateral segment transplant from a living donor.
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A shortage of liver donors for low-weight transplant recipients has prompted the development of procedures for liver-reduction, split-liver, and living related donor transplantations. For pediatric recipients weighing less than 10 kg, the left lateral segment is often still too large. We describe the procedure of monosegmental transplantation using segment II after segment III was resected in situ from a living related donor. Successful monosegmental transplantation is technically feasible and is a valid alternative to be considered for cases of size discrepancy between the recipient's volume and the donor's left lateral segment. (+info)
Extrahepatic metabolism of sevoflurane in children undergoing orthotopic liver transplantation.
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BACKGROUND: Sevoflurane is metabolized by cytochrome P450 and produces inorganic fluoride. The anhepatic phase of liver transplantation provides a useful tool to study the extrahepatic metabolism of drugs. The authors therefore studied the extrahepatic metabolism of sevoflurane by measuring the fluoride production in children receiving sevoflurane solely during the anhepatic phase of orthotopic liver transplantation. METHODS: Children with end-stage liver disease undergoing orthotopic liver transplantation were studied. Anesthesia was provided with isoflurane, sufentanil, and pancuronium. In one group, isoflurane was replaced by sevoflurane as soon as the liver was removed from the patient and maintained until reperfusion of the new liver. Arterial blood samples were drawn at induction, before removal of the liver, 15 min and 30 min after the beginning of the anhepatic phase, at the unclamping of the new liver, and finally 60 and 120 min after the unclamping. Plasma fluoride concentrations were determined by ion-selective electrode. RESULTS: No differences between the two groups (n = 10) regarding age, weight, duration of the anhepatic phase, or basal level of inorganic fluoride were found. The fluoride concentration increased significantly as soon as sevoflurane was introduced; it remained stable in the group receiving isoflurane. The peak fluoride concentration was also significantly higher in the first group (mean +/- SD: 5.5 +/- 0.8 microM (sevoflurane group) versus 1.4 +/- 0.5 microM (isoflurane group) P < 0.05). CONCLUSIONS: These results demonstrate the existence of an extrahepatic metabolism of sevoflurane at least in children with end-stage liver disease. (+info)
Health-related quality of life in long-term survivors of pediatric liver transplantation.
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The purpose of this study is to measure the health-related quality of life (HRQOL) in children who are long-term survivors of liver transplantation and to pilot the Liver Transplant Disability Scale (LTDS), a newly developed 12-point scale that quantifies chronic medical disability related to liver transplantation. This study is a cross-sectional survey of 51 children surviving liver transplantation by at least 2 years, with a median age of 4.94 years. Functional capacity and utility scores were measured by the Health Utilities Index Mark II (HUI2), and chronic disease-specific medical disability was measured by the LTDS. HUI2 results were compared with a reference population. LTDS scores were compared with utility scores and patient survival 3 years later. Ninety percent of the study patients had functional deficits compared with 50% of controls. Functional impairment was typically mild. The resulting mean utility score, 0.86 +/- 0.13 (0 = dead, 1 = perfect health), was significantly less than that of the reference population, 0.95 +/- 0. 07 (P <.001). LTDS scores ranged from 0 (no disability) to 6 (moderate disability). Seventy-one percent of the children had mild disability (scores 0 to 3), and 29% had moderate disability (scores 4 to 6). LTDS scores did not correlate with utility scores but were predictive of survival. The majority of pediatric liver transplant recipients have mild functional deficits. Their utility scores reflected a high level of HRQOL but were significantly less than those of a reference population. The majority also had mild medical disability, predominantly delayed growth. Medical disability did not correlate with HRQOL but predicted survival 3 years later. (+info)
The significance of functioning gallbladder visualization on hepatobiliary scintigraphy in infants with persistent jaundice.
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The purpose of this study was to determine whether gallbladder visualization can help exclude biliary atresia in hepatobiliary scintigraphic studies of infants with persistent jaundice. METHODS: One hundred fifty-two infants with persistent jaundice (49 patients with a final diagnosis of biliary atresia and 103 with biliary patency) were studied using both hepatobiliary scintigraphy and abdominal sonography. Food was withheld for 4 h before the examination, and the infants were fed nothing but glucose until 6 h after the initial injection of (99m)Tc-disofenin or until the gallbladder was seen. If the gallbladder was seen, the infants were fed milk, and imaging was continued to observe gallbladder contractility. RESULTS: In none of the 49 patients with biliary atresia could the gallbladder be seen with hepatobiliary scintigraphy, but abdominal sonography revealed 9 normal-sized gallbladders. Of the 103 patients with biliary patency, hepatobiliary scintigraphy detected the gallbladder more frequently (74%, 76/103) than did abdominal sonography (63%, 65/103). All visualized gallbladders contracted after the infants were fed milk. If we include visualization of both the gallbladder and bowel radioactivity as criteria, the specificity of biliary atresia on hepatobiliary scintigraphy increases to 86% (89/103). CONCLUSION: Gallbladders were usually visible on hepatobiliary scintigraphy of fasting patients with biliary patency. A functioning gallbladder, with or without visualization of bowel radioactivity, indicated biliary patency. (+info)
Characteristics of Malaysian infants with biliary atresia and neonatal hepatitis.
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Cholestatic disorders of infancy (viz neonatal hepatitis and biliary atresia) have not been well studied in Malaysia. In a retrospective study in the Department of Paediatrics, University Hospital, Kuala Lumpur from January 1982 through December 1991, a total of ninety-three infants with such conditions were identified: 35 (38%) had biliary atresia, 58 (62%) neonatal hepatitis. There was a statistically significant male preponderance in the neonatal hepatitis group (P = 0.020). There was no significant difference in the racial distribution and in the proportions of low birthweight infants between the two groups of disorders. When the biliary atresia group was compared with the neonatal hepatitis group, significant differences were observed in the age of presentation (mean +/- SD) 9.8 +/- 6.8 VS 20 +/- 17.3 weeks (P < 0.001), proportion of infants with prolonged jaundice (> seven weeks) 28/35 (80%) VS 20/58 (34.5%) (P < 0.00001), occurrence of alcoholic stools 26/35 (74.3%) VS 27/58 (46.6%) (P = 0.020), liver size (mean +/- SD): 4.3 (1.6 cm VS 3.3 +/- 1.8 cm (P < 0.01) and splenic size: 2.5 (1.8 cm VS 1.4 (1.2 cm (P < 0.001). There was however considerable overlap between the two groups in these features at presentation, making clinical differentiation between the two conditions difficult. Infants with cholestasis tended to present late, compromising the chance of survival. In order to improve the medical care of these patients, these conditions must be emphasised during the training of medical practitioners, and efforts to increase public awareness of these conditions must be created. (+info)
Paediatric liver transplantation: Queen Mary Hospital experience.
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OBJECTIVE: To assess the results of paediatric liver transplantation in our institution. METHODS: From September 1993 to November 1996, 10 living-related liver transplants (LRLT) and 3 reduced-size liver transplants (RSLT) were performed on 12 children at our hospital. The medical records of the patients were reviewed. All patients suffered from end-stage liver disease resulting from biliary atresia with failed Kasai's operations. Their ages at initial transplantation ranged from 8 months to 11 years. Excluding the 2 older children aged 7.5 and 11 years, the remaining patients were aged 10.5 months on the average and weighed 6 to 9.5 kg (mean: 6.8 kg) at the time of initial transplantation. RESULTS: All living donors were discharged on postoperative day 4 to 8 and resumed their previous normal activities. All recipients were alive with normal liver function and growing after a follow-up period of 3-40 months (mean: 21 months). The patient survival rate was 100%. One patient with RSLT had hepatitis of undetermined aetiology and underwent retransplant with a graft from her mother. The graft survival rate was 92%. Postoperative complications included: postoperative bleeding (n = 3), hepatic vein stenosis (n = I), biliary-enteric anastomotic stenosis (n = 3), intestinal perforation (n = I) and portal vein thrombosis (n = I). They were all treated promptly. In all patients, the hepatic artery (diameter ranged from 1.5 to 2.5 mm) anastomosis was achieved by microvascular technique. There was no hepatic artery thrombosis in our patients. CONCLUSION: With technical refinements, early detection and prompt treatment of complications, and advances in immunotherapy, excellent results can be achieved in paediatric liver transplantation. (+info)