title: HNF1B-related diabetes triggered by renal transplantation.
abstract: BACKGROUND
  A 37-year-old man developed cholestasis-associated pruritus followed by overt hyperglycemia (blood glucose level 23 mmol/l), necessitating insulin treatment, within weeks of undergoing renal transplantation. He had a history of gout, but his fasting blood glucose and glycated hemoglobin concentrations had been normal before transplantation.
  
  
  METHODS
  Physical examination; laboratory tests, including assessment of glycated hemoglobin, anti-glutamic-acid-decarboxylase and anti-islet-antigen-2 antibodies, liver enzymes, renal function, tacrolimus blood trough level, exocrine (fecal elastase) and endocrine (C-peptide) pancreatic function; abdominal CT scan; liver biopsy; and screening of the hepatocyte nuclear factor 1 homeobox B (transcription factor 2) gene, HNF1B.
  
  
  METHODS
  New-onset diabetes after transplantation associated with a newly described deletion in HNF1B.
  
  
  RESULTS
  Minimization of tacrolimus exposure and withdrawal of steroids considerably reduced the patient's insulin requirement, and cholestasis-related pruritus was dramatically improved by administration of ursodeoxycholic acid. Renal ultrasonography and screening for the HNF1B molecular abnormality were offered to the patient's relatives.
authors.affiliation: Service de Transplantation Rénale Adulte, Université Paris Descartes, Paris cedex 15, France. julien.zuber@nck.aphp.fr
authors.forename: Julien
authors.initials: J
authors.lastname: Zuber
authors.forename: Christine
authors.initials: C
authors.lastname: Bellanné-Chantelot
authors.forename: Claire
authors.initials: C
authors.lastname: Carette
authors.forename: Guillaume
authors.initials: G
authors.lastname: Canaud
authors.forename: Sandrine
authors.initials: S
authors.lastname: Gobrecht
authors.forename: Khaled
authors.initials: K
authors.lastname: Gaha
authors.forename: Vincent
authors.initials: V
authors.lastname: Mallet
authors.forename: Frank
authors.initials: F
authors.lastname: Martinez
authors.forename: Eric
authors.initials: E
authors.lastname: Thervet
authors.forename: José
authors.initials: J
authors.lastname: Timsit
authors.forename: Christophe
authors.initials: C
authors.lastname: Legendre
authors.forename: Danièle
authors.initials: D
authors.lastname: Dubois-Laforgue
mesh_terms: D000328:Adult; D002779:Cholestasis / Q000276:immunology; D003924:Diabetes Mellitus, Type 2 / Q000175:diagnosis / Q000235:genetics* / Q000276:immunology; D003937:Diagnosis, Differential; D016368:Frameshift Mutation; D051539:Hepatocyte Nuclear Factor 1-beta / Q000235:genetics*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016030:Kidney Transplantation / Q000276:immunology; D008297:Male; D011537:Pruritus / Q000276:immunology
chemical_list: C496495:HNF1B protein, human; D007166:Immunosuppressive Agents; D051539:Hepatocyte Nuclear Factor 1-beta