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

• Published in: Current diabetes reports 2020-06, Vol.20 (6), p.16-16, Article 16
• Main Authors: Ramalho, Gabriel Xavier, Dytz, Marcio Garrison
• Format: Article
• Language: English
• Subjects: Diabetes; Insulin; Medicine; Medicine & Public Health; Other Forms of Diabetes and Its Complications (JJ Nolan and H Thabit; Pancreatitis; Section Editors; Topical Collection on Other Forms of Diabetes and Its Complications
• ISSN: 1534-4827; 1539-0829
• DOI: 10.1007/s11892-020-01299-8

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.