ABSTRACT
BACKGROUND: The appropriate use of liver transplantation in children with type-1 primary hyperoxaluria (PH-1) is not well established. We reviewed our experience with 36 children with PH-1, including 12 who underwent liver transplantation. PATIENTS AND METHODS: From 1989-1998, 36 children from 10 families in northern Israel were diagnosed with PH-1. Eight children presented with renal failure; seven of these eight had the severe infantile form of the disease. One child was treated with kidney transplantation alone. Combined liver-kidney transplantation has been performed in nine children and preemptive liver transplantation in three children. A review of the patients' charts for the following parameters was performed: age, clinical signs, and renal sonographic findings at diagnosis, age at onset of dialysis, and current status. Type of transplant, pre- and posttransplant urine oxalate excretion, current renal function, survival, and complications were recorded in liver recipients. RESULTS: Of the 23 nontransplanted children, 9 died of complications related to severe systemic oxalosis and 14 are alive (mean follow-up, 7.4 years), including 2 who are candidates for transplantation. The child who underwent only kidney transplantation died of unrelated causes. Of the 12 liver recipients, 2 died within the first 3 months posttransplant and another child underwent retransplantation due to hepatic arterial thrombosis. At intervals after transplant ranging from 6-54 months, 10 recipients are alive (7 of the 9 recipients of combined liver-kidney transplants and all 3 recipients of preemptive liver transplants). Mean GFR in the 10 survivors is 77 ml/min/m2. In 9 of these 10, daily urinary oxalate excretion normalized. Renal function has improved (mean GFR 86 vs. 58 ml/min/m2) but renal oxalate deposits remain in the three recipients of isolated liver grafts. CONCLUSIONS: Our decade-long experience with children with PH-1 supports strategies for early diagnosis and timely liver transplantation. Preemptive isolated liver transplantation should be considered in children who develop the disease during infancy or in those with slowly progressive disease when significant symptoms develop. Combined liver-kidney transplantation is suggested for children with end-stage renal disease.
Subject(s)
Hyperoxaluria, Primary/surgery , Liver Transplantation , Adolescent , Adult , Child , Child, Preschool , Cohort Studies , Female , Glomerular Filtration Rate , Graft Survival , Humans , Hyperoxaluria, Primary/complications , Hyperoxaluria, Primary/mortality , Infant , Infant, Newborn , Kidney Failure, Chronic/complications , Kidney Failure, Chronic/mortality , Kidney Failure, Chronic/physiopathology , Kidney Failure, Chronic/surgery , Kidney Transplantation , Male , Survival AnalysisSubject(s)
Liver Transplantation , Adolescent , Child , Child, Preschool , Female , Follow-Up Studies , Graft Survival , Hospitals, Pediatric , Humans , Infant , Israel , Liver Transplantation/physiology , Male , Postoperative Complications/classification , Postoperative Complications/epidemiology , Reoperation , Retrospective Studies , Time FactorsABSTRACT
Our experience with living-related liver transplantation is described. In 2 boys and 1 girl, aged 4-4.5 years with acute, fulminating hepatitis A, the presence of very severe jaundice (bilirubin levels > 18 mg%) associated with severe coagulopathy (INR > 10) and encephalopathy indicated the need for urgent liver transplantation. In all 3 cases the left lateral hepatic segment of a matched blood type parent was transplanted. None of the donors suffered a serious complication postoperatively and all returned to full activity in 6-16 weeks. The post-transplantation course was uneventful in 1 child, but in the other 2 there was hepatic arterial thrombosis in 1 at 1 day and in the other at 8 days post-transplantation. Early detection of arterial thrombosis by Doppler sonography permitted salvage of the 2 hepatic grafts after thrombectomy and re-anastomosis. In 1 of these 2 children an anastomotic biliary stricture was found 2 months after transplantation. It was corrected at surgery and a percutaneous stent was inserted. All 3 children are alive with normal graft function at 2, 7 and 8 months post-transplantation, respectively. This initial experience indicates that living-related liver transplantation is feasible in Israel. The technique might help to solve our severe organ shortage for children awaiting liver transplantation.
Subject(s)
Living Donors , Adult , Child, Preschool , Female , Hepatectomy , Hepatic Encephalopathy/surgery , Hepatitis A/surgery , Humans , Male , Parents , Postoperative ComplicationsSubject(s)
Liver Diseases/therapy , Liver Transplantation , Tissue Donors , Child, Preschool , Female , Humans , Male , Organ PreservationABSTRACT
The effect of suboptimal folate nutrtion on the growing infant was studied in a population of infants fed a diet based on boiled, pasteurised cows' milk. One group of infants received a daily supplement of 1 mg folic acid from age 2 months, while the other group received a placebo. The infants were seen at bimonthly intervals. In the supplemented group the red cell folate level had increased to twice its pretreatment value by 4 months, and remained at this high level to the end of the first year. Hb concentration and incidence of anaemia were similar in both groups. The incidence of infection in the two groups did not differ. Weights and lengths attained at 6 months, and the rate of gain from 2 to 6 months were higher in infants whose folate levels were above the median value than in those below it. In the second half of the first year the differences between the two groups were no longer evident.
