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Diabetes of the Exocrine Pancreas Related to Hereditary Pancreatitis, an Update

  • Other Forms of Diabetes and Its Complications (JJ Nolan and H Thabit, Section Editors)
  • Published:
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Abstract

Purpose of Review

The aim was to review evidence about diabetes secondary to hereditary pancreatitis, seeking novel diagnostic and treatment features.

Recent Findings

Hereditary pancreatitis (HP) is an autosomal dominant condition, characterized by recurrent episodes of acute pancreatitis, progression to fibrosis, and chronic pancreatitis. Clinical presentation includes diabetes of the exocrine pancreas (DEP). HP prevalence ranges from 0.3 to 0.57 per 100,000 people, with up to 80% of these develop DEP. This condition often requires specific interventions: with regard to metabolic control, metformin is the first choice for those with mild DEP, and for those in advanced disease, insulin is considered the first-line therapy. Insulin analogues and insulin pump therapy are preferred due to the brittle glycemic pattern and risk of hypoglycemia. In case of exocrine insufficiency, pancreatic enzyme replacement therapy is recommended. Pancreatic polypeptide administration is a promising novel treatment feature.

Summary

DEP due to HP appears to be a misdiagnosed condition. The requirement of specific management demonstrates the importance of this matter; therefore, appropriate recognition and classification are important.

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Authors and Affiliations

  1. School of Medicine, Faculty of Education and Health Sciences, University Center of Brasilia (UniCEUB), Brasilia, Brazil

    Gabriel Xavier Ramalho & Marcio Garrison Dytz

  2. Endocrinology Division, Department of Intern Medicine, Sobradinho Regional Hospital, Brasilia, Brazil

    Marcio Garrison Dytz

  3. Endocrinology and Metabolism Medical Residency, Superior School of Health Sciences (ESCS), Brasilia, Brazil

    Marcio Garrison Dytz

  4. Institute of Diabetes and Endocrinology of Brasilia, SHS Qd. 6 Cj. A Bl. E Sl 1119, Brasilia, DF, 70316-902, Brazil

    Marcio Garrison Dytz

Authors
  1. Gabriel Xavier Ramalho
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Both authors wrote the manuscript and contributed to the design, drafting, and reviewing the paper.

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Correspondence to Marcio Garrison Dytz.

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Ramalho, G.X., Dytz, M.G. Diabetes of the Exocrine Pancreas Related to Hereditary Pancreatitis, an Update. Curr Diab Rep 20, 16 (2020). https://doi.org/10.1007/s11892-020-01299-8

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