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Successful outcome after early combined liver and en bloc ‐kidney transplant in an infant with primary hyperoxaluria type 1: A case report
Author(s) -
Heffron Thomas G.,
Rodriguez John,
Fasola Carlos G.,
Casper Katherine,
Pillen Todd,
Smallwood Gregory,
Warshaw Barry,
Romero Rene,
Larsen Christian
Publication year - 2009
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/j.1399-3046.2009.01189.x
Subject(s) - primary hyperoxaluria , medicine , kidney , liver transplantation , transplantation , nephrology , surgery , congenital hepatic fibrosis , kidney transplantation , gastroenterology , urology , cirrhosis , portal hypertension
PH1 is a metabolic disorder characterized by urolithiasis and the accumulation of oxalate crystals in the kidneys and other organs. Although patients often first present with renal failure, PH1 results from a deficiency of the hepatic peroxisomal enzyme AGT. Ultimately only liver transplantation will cure the underlying metabolic defect. Herein, we report the case of a three‐month‐old male infant diagnosed with PH and treated using a combined liver and en bloc‐ kidney transplant from a single donor. At the time of transplant, the patient was 11 months old and weighed 7.9 kg. He received a full size liver graft and en bloc kidneys from a two‐yr‐old donor. At 36 months post‐transplant, the patient is steadily growing with normal renal and hepatic function. This is one of the first reports of successful liver and en bloc ‐kidney transplantation with abdominal compartment expansion by PTFE for the infantile form of PH1 in a high risk child before one yr of age. Prompt diagnosis and early referral to a specialized center for liver and kidney replacement offer the best chance for survival for infants with this otherwise fatal disease.