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 = 0.70, p = 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.