Nocturnal home hemodialysis for a patient with type 1 hyperoxaluria.
Type 1 primary hyperoxaluria is a genetic disorder caused by deficiency of the liver-specific peroxisomal enzyme alanine-glyoxylate aminotransferase. This enzyme deficiency leads to excess oxalate production and deposition of calcium oxalate salts, resulting in kidney failure and systemic oxalosis. Aside from combined liver/kidney transplantation, no curative treatment exists. Various strategies for optimizing dialysis treatment have been evaluated, but neither conventional hemodialysis nor peritoneal dialysis can keep pace with oxalate production in this patient population. In this report, we describe a patient with end-stage renal disease from type 1 primary hyperoxaluria managed with nocturnal home hemodialysis. Performing hemodialysis 8-10 hours each night with blood flow of 350 mL/min and total dialysate volume of 60 L, she has maintained pre- and postdialysis serum oxalate levels at or below the level of supersaturation. We also review published literature regarding oxalate removal in various modalities of dialysis in patients with type 1 primary hyperoxaluria. In our patient, nocturnal hemodialysis has controlled serum oxalate levels better than conventional hemodialysis therapies. Home nocturnal hemodialysis should be considered an option for management of patients with end-stage renal disease from type 1 hyperoxaluria who are awaiting transplantation.
['Plumb|Troy J|TJ|', 'Swee|Melissa L|ML|', 'Fillaus|Jennifer A|JA|']
D000328:Adult D019943:Amino Acid Substitution D001120:Arginine D002940:Circadian Rhythm D003131:Combined Modality Therapy D004252:DNA Mutational Analysis D005091:Exons D005260:Female D005998:Glycine D006437:Hemodialysis, Home D006801:Humans D006960:Hyperoxaluria, Primary D007676:Kidney Failure, Chronic D010070:Oxalates D011736:Pyridoxine D000637:Transaminases
home hemodialysis Primary hyperoxaluria oxalate dialysis