Survival of childhood polycystic kidney disease following renal transplantation: The impact of advanced hepatobiliary disease
By Davis, I. D., Ho, M., Hupertz, V., and Avner, E. D.
Childhood PKD encompasses the diagnoses of AR and
ADPKD,glomerulocystic disease,and syndromes such as tuberous
sclerosis or Jeune’s syndrome. Given the fact that a majority of PKD
children with ESRD carry the diagnosis of ARPKD,natural history
studies assessing the long-term prognosis of PKD patients following
renal transplantation must focus on morbidity and mortality issues
related to complications from congenital hepatic fibrosis. Using the
NAPRTCS registry,we analyzed the patient and graft survival rates of
203 PKD patients and 7044 non-PKD patients undergoing renal
transplantation between 1987 and 2001. Deceased PKD patients,all
with a diagnosis of ARPKD,were further identified and characterized
using a special questionnaire submitted to the principal investigators.
Overall graft and patient survival rates were not significantly different
between PKD and non-PKD patients. No differences in rates of acute
rejection or time to first rejection were noted between PKD and
non-PKD patients. The relative risk of living longer than 3 yr in the
PKD patients was not significantly different from non-PKD patients
(RR ¼ 0.70,p ¼ 0.28). Sepsis was identified as a likely factor in the
cause of death in nine (64%) ARPKD patients and was comfirmed with
a positive blood culture in four patients. Despite similar graft and
patient survival rates among PKD and non-PKD children following
renal transplantation,our results suggest that ARPKD transplant
recipients appear to be at increased risk for sepsis that may be related to
hepatic fibrosis and ascending cholangitis. The utility of early liver
transplantation in ARPKD patients with significant hepatobiliary
disease is discussed.
