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.
Investigations. 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.
Diagnosis. New-onset diabetes after transplantation associated with a newly described deletion in HNF1B.
Management. 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.
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Acknowledgements
Research by J. Zuber was funded by grants from the Fondation de Coopération Scientifique Centaure, which supports a French Transplantation Research Network, including the Necker, Lyon and Nantes transplant units. We are grateful to Dr. Pierre Turpin for his careful and critical reading of the manuscript. Désirée Lie, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the MedscapeCME-accredited continuing medical education activity associated with this article.
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Zuber, J., Bellanné-Chantelot, C., Carette, C. et al. HNF1B-related diabetes triggered by renal transplantation. Nat Rev Nephrol 5, 480–484 (2009). https://doi.org/10.1038/nrneph.2009.98
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DOI: https://doi.org/10.1038/nrneph.2009.98
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