This protocol addresses a specific sub-population of autosomal dominant tubulointerstitial kidney disease: patients in whom the disease is driven by an HNF1B gene mutation and who also have diabetes mellitus and kidney failure. The concurrent endocrine and renal burden defines the clinical decision-making in this setting.
ADTKD in the context of an HNF1B mutation is associated with both progressive kidney failure and diabetes mellitus arising from pancreatic dysfunction. When these conditions coexist, treatment planning must account for both organ systems together.
In this specific clinical scenario, a combined organ transplantation strategy is among the approaches to be considered. The full protocol details the precise indications, patient selection criteria, and the complete decision framework — access it below.
DOI: 10.1038/ki.2015.28
In HNF1B patients with diabetes mellitus, combined kidney/pancreas transplantation should be considered.
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