title: Long term results of liver-kidney transplantation in children with primary hyperoxaluria.
abstract: From 1990 to 2000, we performed eight liver-kidney transplants in eight children, aged 1-16 years, with end-stage renal failure (ESRF) due to primary hyperoxaluria (PH1). The duration of dialysis before transplantation ranged from 2 to 42 months (mean 14 months) and was <1 year in four patients. Only the first patient underwent postoperative hemodialysis; in the other five, we chose to induce maximal diuresis from the first hours with intravenous and intragastric hyperhydration (> or =3 l/m2 per day). High water intake with nocturnal tube hydration was maintained for 6 months to 5 years, as long as oxaluria exceeded 0.5 mmol/day. A quadruple sequential immunosuppressive regimen was used. Two patients died during liver graft surgery. The other six patients are alive and well, with a mean follow-up of 7.4 years (range 5-11 years). Patient and graft survival is 75% at 5 years. At latest follow-up, liver tests were normal in all six patients; creatinine clearance ranged from 55 to 95 ml/min per 1.73 m2 (mean=74). Oxaluria was lower than 0.4 mmol/day in all patients (mean=0.22). The six patients underwent 15 renal biopsies, 1-11 years after transplantation. Chronic transplant nephropathy was present in four patients and mild cyclosporin nephrotoxicity in another. No oxalate crystals were seen and repeat ultrasonography has been consistently normal in all patients. The three patients with bone oxalosis showed progressive complete healing of bone lesions. All six children or adolescents now live a normal life. From this series, we conclude that early combined liver-kidney transplantation is the treatment of choice for children with ESRF due to primary hyperoxaluria.
authors.affiliation: Department of Pediatric Nephrology, Hôpital Necker-Enfants Malades, 149 rue de Sèvres, 75743 Paris Cedex 15, France.
authors.forename: M F
authors.initials: MF
authors.lastname: Gagnadoux
authors.forename: F
authors.initials: F
authors.lastname: Lacaille
authors.forename: P
authors.initials: P
authors.lastname: Niaudet
authors.forename: Y
authors.initials: Y
authors.lastname: Revillon
authors.forename: P
authors.initials: P
authors.lastname: Jouvet
authors.forename: D
authors.initials: D
authors.lastname: Jan
authors.forename: G
authors.initials: G
authors.lastname: Guest
authors.forename: M
authors.initials: M
authors.lastname: Charbit
authors.forename: M
authors.initials: M
authors.lastname: Broyer
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D016572:Cyclosporine / Q000009:adverse effects; D005260:Female; D006085:Graft Survival; D006621:Hip Joint / Q000000981:diagnostic imaging; D006801:Humans; D006959:Hyperoxaluria / Q000150:complications* / Q000000981:diagnostic imaging; D007166:Immunosuppressive Agents / Q000009:adverse effects; D007223:Infant; D007674:Kidney Diseases / Q000139:chemically induced / Q000209:etiology; D007676:Kidney Failure, Chronic / Q000209:etiology* / Q000601:surgery*; D016030:Kidney Transplantation / Q000009:adverse effects; D016031:Liver Transplantation / Q000009:adverse effects / Q000401:mortality; D008137:Longitudinal Studies; D008297:Male; D011184:Postoperative Period; D011859:Radiography; D016019:Survival Analysis
chemical_list: D007166:Immunosuppressive Agents; D016572:Cyclosporine