HNF1B-MODY (MODY-5): a rare form of diabetes with multisystemic features–two case reports - Takeaways - MDSpire

HNF1B-MODY (MODY-5): a rare form of diabetes with multisystemic features–two case reports

  • By

  • Tânia Carvalho

  • Mariana Lavrador

  • Joana Saraiva

  • Leonor Gomes

  • June 11, 2026

  • 0 min

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  • 1

    MODY-5 is a rare monogenic diabetes variant caused by HNF1B gene mutations, accounting for less than 5% of MODY cases.

  • 2

    Patients with MODY-5 often present with renal abnormalities and genitourinary malformations, alongside diabetes.

  • 3

    The first case involved a 29-year-old woman with diabetes, hypertension, renal cysts, and a complex Müllerian anomaly.

  • 4

    The second case featured a 40-year-old man with diabetes, chronic kidney disease, and multiple systemic complications.

  • 5

    Early recognition of HNF1B mutations is crucial for tailored management and surveillance in MODY-5 patients.

Original Source(s)

HNF1B-MODY (MODY-5): a rare form of diabetes with multisystemic features–two case reports

  • by Tânia Carvalho, Mariana Lavrador, Joana Saraiva, Leonor Gomes

  • June 11, 2026

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