Childhood onset diabetes posttransplant in a girl with TCF2 mutation



This article is corrected by:

  1. Errata: Corrigendum Volume 14, Issue 1, 80, Article first published online: 24 January 2013

  • [Correction added on 11th May 2012, after first online publication: The name and affiliation of Dr. Albert Bensman were added.]

Corresponding author:

Prof. Tim Ulinski,

Department of Pediatric Nephrology,

Armand-Trousseau Hospital, AP-HP,

26, Avenue du Docteur Arnold Netter,

Paris 75012, France.

Tel: +33-144736662;

fax: +33-144736663;



Heterozygous mutations of TCF2 (transcription factor 2) have been associated with maturity onset diabetes of the young, renal malformations, hyperuricemia, and occasionally internal genital malformations in female. We report a female patient with bilateral renal hypodysplasia and de novo heterozygous TCF2 gene mutation. At the age of 9 yr, she developed transient ketoacidosis immediately posttransplant, temporarily requiring insulin. During glucocorticoid tapering, impaired glucose tolerance persisted and overt insulin-dependent diabetes mellitus developed 1 yr later. Pathogenic factors which might have played a role in the acceleration of diabetes were (i) switch from cyclosporine to tacrolimus, (ii) weight excess, and (iii) cytomegalovirus infection. TCF2 analysis might, therefore, be of interest in patients with congenital abnormalities of the kidney and the urinary tract in order to improve posttransplant management in terms of steroid and tacrolimus exposure.