End-stage renal disease due to ARPKD in the first months of life: transplantation or dialysis?--two case reports.
ARPKD with renal insufficiency during the first months of life is a clinical challenge. We report on two children with ARPKD with massively enlarged kidneys requiring renal replacement therapy in early infancy. Patient 1 developed pulmonary insufficiency due to massively enlarged kidneys. At the age of six months the girl was listed for KT as "high urgency" on the Eurotransplant waiting list. A kidney from a deceased donor was pre-emptively transplanted and simultaneous nephrectomy performed. No postoperative complications were observed, and the patient was discharged from in-patient care 42 days after transplantation. Unexpectedly, she died at the age of one yr due to cerebral vascular spasms of unknown origin. Patient 2 was transferred at the age of three months to our clinic with life-threatening pulmonary insufficiency. Pre-emptive KT was not possible; therefore, bilateral nephrectomy was performed and PD begun. The boy is still doing well on PD one yr later. Pre-emptive KT and bilateral nephrectomy followed by PD are two options for infants with ARPKD and excessive kidney enlargement. PD could be complicated and in some cases become impossible by peritoneal damage during nephrectomy. On the other hand, KT covers a high risk of infections caused by immunosuppression. The decision, which method to choose, should be driven by the individual situation of the patient and the expertise of the center.
Department of Pediatric Nephrology, Hepatology and Metabolic Diseases, Hannover Medical School, Hannover, Germany. foerster.stefanie@mh-hannover.de
Stefanie
S
Beil
Jens
J
Drube
Sylvia
S
Gluer
Frank
F
Lehner
Jochen H H
JH
Ehrich
Lars
L
Pape
D004200:Diseases in Twins / Q000601:surgery; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007223:Infant; D016030:Kidney Transplantation; D008297:Male; D010530:Peritoneal Dialysis; D017044:Polycystic Kidney, Autosomal Recessive / Q000503:physiopathology / Q000601:surgery*; D006435:Renal Dialysis