Title : Should liver transplantation be performed before advanced renal insufficiency in primary hyperoxaluria type 1?

Pub. Date : 1993 Apr

PMID : 8476723






2 Functional Relationships(s)
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1 Patients with pyridoxine-resistant forms of PH1 usually require organ replacement therapy, i.e. liver transplantation to supply the deficient enzyme and/or kidney transplantation to replace the affected organ. Pyridoxine alanine--glyoxylate and serine--pyruvate aminotransferase Homo sapiens
2 Isolated liver transplantation should be considered in carefully selected children with severe forms of pyridoxine-resistance (PH1) before GFR has dropped to less than 30 ml/min per 1.73 m2; it seems to be indicated especially in the presence of a rapid decline of GFR in the preceding year. Pyridoxine alanine--glyoxylate and serine--pyruvate aminotransferase Homo sapiens