Analysis of neurological complications in children transplanted due to fulminant liver failure.

Treatment of patients with fulminant liver failure is a challenge of contemporary medicine. Liver transplantation, in this group, is presently the only reasonable alternative, but in many patients the disastrous condition of the patient results in serious life-threatening complications, including neurological sequelae, which may influence the quality of life after transplantation, and in some cases even cause death. From 1990 to 2004, we performed 241 liver transplantations in children, including 20 transplanted due to fulminant liver failure (8.2%). Serious neurological complications followed liver transplantation in five cases (20%), three of which were fatal. The analysis revealed that the duration of pretransplant coma (grade III or IV) strongly correlated with the incidence of neurological complications (P < .05). Also a suboptimal quality of the donor liver and poor early graft function may contribute to these posttransplant complications.

Further delineation of the classical Smith-Lemli-Opitz syndrome phenotype at different patient ages: clinical and biochemical studies.

We present here extensive clinical and biochemical data on thirteen SLOS (type I) patients with proven defect in cholesterol biosynthesis for further delineation of the classical SLOS phenotype at different patient ages.