Anticipation of age at renal death in autosomal dominant polycystic kidney disease (ADPKD)?
It has been claimed that anticipation occurs in ADPKD, i.e. endstage renal failure at progressively earlier age in successive generations. This observation might possibly point to unstable DNA as the molecular basis of ADPKD. We analysed 74 parent-offspring pairs of 148 families in Germany and Austria in whom (i) ADPKD was verified by appropriate imaging procedures and (ii) age at renal death was accurately known. The median difference for age at renal death between parent and offspring was 0 years, range -26.3 to +27.2 years. There was no deviation from normal (Gaussian) distribution according to the Shapiro-Wilk test (P = 0.75). We conclude that systematic anticipation cannot be demonstrated with this sample which had sufficient size for meaningful biostatistical analysis.
Geberth
S
S
Department of Internal Medicine, Ruperto Carola University, Heidelberg, Germany.
Ritz
E
E
Zeier
M
M
Stier
E
E
D000328:Adult; D017668:Age of Onset; D000368:Aged; D001317:Austria; D002648:Child; D004247:DNA / Q000235:genetics; D005260:Female; D005858:Germany; D006801:Humans; D007676:Kidney Failure, Chronic / Q000209:etiology* / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D010290:Parents; D016891:Polycystic Kidney, Autosomal Dominant / Q000150:complications* / Q000235:genetics*
D004247:DNA