Transplantation procedures in primary hyperoxaluria type 1.
Standard
Transplantation procedures in primary hyperoxaluria type 1. / Latta, A; Müller-Wiefel, D E; Sturm, E; Kemper, Markus J.; Burdelski, M; Broelsch, C E.
In: CLIN NEPHROL, Vol. 46, No. 1, 1, 1996, p. 21-23.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
Harvard
APA
Vancouver
Bibtex
}
RIS
TY - JOUR
T1 - Transplantation procedures in primary hyperoxaluria type 1.
AU - Latta, A
AU - Müller-Wiefel, D E
AU - Sturm, E
AU - Kemper, Markus J.
AU - Burdelski, M
AU - Broelsch, C E
PY - 1996
Y1 - 1996
N2 - Primary hyperoxaluria type 1 (PH 1) is complicated by a high rate of early end-stage renal failure (ESRF). In ESRF combined liver kidney transplantation has emerged as treatment of choice for teenagers and adults. In chronic renal failure (CRF) and for small children the situation is less clear. We report on three isolated liver transplantations and show the data of young children from the European Registry for liver transplantation in PH 1. Patient #1 developed ESRF at 3 months of age. Deficiency of alanine:glyoxylate aminotransferase proved PH 1. Progressive bone disease developed and the boy received a living related liver graft (LRLTx) at age two. Due to recurrent cholangitis kidney transplantation (KTx) is currently not feasible. Plasma oxalate decreased after LRLTx indicating correction of the metabolic defect. Patient #2 was diagnosed at the age of 14 months. He had nephrocalcinosis and hyperglycolic hyperoxaluria. Two years later he developed ESRF. At 5 years of age isolated liver transplantation was performed as a first step of therapy. Due to prolonged warm ischemia time organ function was poor. A severe bleeding complicated the course. The child died four weeks after transplantation from untreatable CMV septicemia. Patient #3 was evaluated for failure to thrive at 6 months of age. Urinary oxalate/creatinine ratio was 705 mumol/mol and gave rise to the diagnosis of PH 1. Renal failure slowly progressed to a creatinine clearance of 20 ml/min/1.73 m2 at 8 years, when liver transplantation (LTx) was performed. Four months later, GFR has not changed. Liver function and urinary oxalate/creatinine ratio are normal. Slowly deteriorating chronic renal failure can be stabilized through isolated liver transplantation and thus the rapid need for KTx will at least be delayed. Even more important, normalization of the oxalate metabolism prevents extrarenal oxalate deposits during renal failure.
AB - Primary hyperoxaluria type 1 (PH 1) is complicated by a high rate of early end-stage renal failure (ESRF). In ESRF combined liver kidney transplantation has emerged as treatment of choice for teenagers and adults. In chronic renal failure (CRF) and for small children the situation is less clear. We report on three isolated liver transplantations and show the data of young children from the European Registry for liver transplantation in PH 1. Patient #1 developed ESRF at 3 months of age. Deficiency of alanine:glyoxylate aminotransferase proved PH 1. Progressive bone disease developed and the boy received a living related liver graft (LRLTx) at age two. Due to recurrent cholangitis kidney transplantation (KTx) is currently not feasible. Plasma oxalate decreased after LRLTx indicating correction of the metabolic defect. Patient #2 was diagnosed at the age of 14 months. He had nephrocalcinosis and hyperglycolic hyperoxaluria. Two years later he developed ESRF. At 5 years of age isolated liver transplantation was performed as a first step of therapy. Due to prolonged warm ischemia time organ function was poor. A severe bleeding complicated the course. The child died four weeks after transplantation from untreatable CMV septicemia. Patient #3 was evaluated for failure to thrive at 6 months of age. Urinary oxalate/creatinine ratio was 705 mumol/mol and gave rise to the diagnosis of PH 1. Renal failure slowly progressed to a creatinine clearance of 20 ml/min/1.73 m2 at 8 years, when liver transplantation (LTx) was performed. Four months later, GFR has not changed. Liver function and urinary oxalate/creatinine ratio are normal. Slowly deteriorating chronic renal failure can be stabilized through isolated liver transplantation and thus the rapid need for KTx will at least be delayed. Even more important, normalization of the oxalate metabolism prevents extrarenal oxalate deposits during renal failure.
KW - Humans
KW - Male
KW - Female
KW - Child
KW - Infant
KW - Retrospective Studies
KW - Fatal Outcome
KW - Liver Transplantation/methods
KW - Oxalates/metabolism
KW - Hyperoxaluria, Primary/complications/metabolism/surgery
KW - Kidney Failure, Chronic/etiology/metabolism/physiopathology
KW - Humans
KW - Male
KW - Female
KW - Child
KW - Infant
KW - Retrospective Studies
KW - Fatal Outcome
KW - Liver Transplantation/methods
KW - Oxalates/metabolism
KW - Hyperoxaluria, Primary/complications/metabolism/surgery
KW - Kidney Failure, Chronic/etiology/metabolism/physiopathology
M3 - SCORING: Journal article
VL - 46
SP - 21
EP - 23
JO - CLIN NEPHROL
JF - CLIN NEPHROL
SN - 0301-0430
IS - 1
M1 - 1
ER -