One hundred percent @entity1 and kidney allograft survival with simultaneous liver and kidney transplantation in @entity1 with primary XXXX : a single-center experience.

@entity1 @entity8 @entity5201 @entity4295 @entity1009 @entity177 @entity42685 @entity1155 @entity1011 @entity12297 @entity454
BACKGROUND: Combined liver-kidney transplantation is the definitive treatment for @entity454 caused by @entity42685 ( @entity12297 ). The infantile form is characterized by @entity177 early in life, advanced systemic @entity5201 , and a formidable mortality rate. Although others have reported on overall results of transplantation for @entity12297 covering a wide age spectrum, none has specifically addressed the high-risk infantile form of the disease. METHODS: Six @entity1 with @entity12297 underwent simultaneous liver-kidney transplantation at our center between May 1994 and August 1998. Diagnosis was made at 5.2+/-3.3 months of age, they were on dialysis for 11.8+/-2.3 months, and they underwent transplantation at 14.8+/-3.0 months of age when they weighed 10.6+/-1.7 kg. RESULTS: At a mean follow-up of 6.4+/-1.7 years (range, 3.9-8.1 years), we report 100% @entity1 and kidney allograft survival. There were no cases of @entity4295 . Long-term kidney allograft function remained stable in all patients, with serum @entity1011 values of less than 1.1 mg/dL and a mean creatinine clearance of 99 mL/ @entity1009 .73 m2 at follow-up. Those who received combined hemodialysis and peritoneal dialysis pretransplant had lower posttransplant urinary oxalate values than those receiving peritoneal dialysis alone. There was improvement in growth and @entity1155 and mental developmental scores after transplantation. CONCLUSIONS: Combined liver-kidney transplantation for the infantile presentation of @entity12297 is associated with excellent outcome when the approach includes early diagnosis and early combined transplantation, aggressive pretransplant dialysis, and avoidance of posttransplant @entity8 .
