Fibrocalculous pancreatic diabetes

Area of Science:

• Endocrinology
• Gastroenterology
• Epidemiology

Background:

• Fibrocalculous pancreatic diabetes (FCPD) is a distinct diabetes mellitus subtype.
• It is strongly associated with chronic calcific pancreatitis, particularly in lean, young adults from tropical, developing countries.
• Etiology is unclear, often linked to malnutrition, with no clear diagnostic criteria or features.

Purpose of the Study:

• To review the literature on the epidemiology, pathophysiology, natural history, clinical features, and treatment of FCPD.
• To consolidate current knowledge on this uncommon diabetes phenotype.

Main Methods:

• Systematic review of English-language literature published from 1956 to 2014.
• Analysis of studies focusing on FCPD's characteristics and associations.

Main Results:

• FCPD typically affects young, lean adults in tropical regions, presenting with chronic abdominal pain and progressive pancreatic dysfunction.
• Common findings include pancreatic calcifications, duct dilation, fibrosis, and atrophy, with alcohol abuse usually absent.
• Microvascular complications are frequent, but diabetic ketoacidosis is rare; pancreatic cancer is an infrequent long-term complication.

Conclusions:

• FCPD is characterized by early-onset, non-alcoholic chronic pancreatitis with hyperglycemia, insulin deficiency, and ketosis resistance.
• Further research is needed to elucidate its etiology and establish standardized diagnostic criteria and management strategies.