Clinical features of diabetes mellitus in hereditary pancreatitis
© Dytz et al. 2015
Published: 11 November 2015
Hereditary pancreatitis (HP) is a rare autosomal dominant disease characterized by recurrent acute pancreatitis that progresses to chronic pancreatitis. Common clinical manifestations are abdominal pain, disabsorptive syndrome due to exocrine dysfunction and diabetes mellitus (DM) due to damage to islet cells.
To describe the characteristics of DM secondary to HP in a family with mutation in PRSS1 gene.
Materials and methods
Patients of one family with DM secondary to HP due to N29T mutation in exon 2 of the PRSS1 gene were evaluated by review of medical records and download of CG from Accucheck Active glucometer via software Accucheck 360º. Glycated hemoglobin (HbA1c) during the follow up and capillary blood glucose (CBG) in the previous month were analyzed.
DM secondary to HP may appear before or after the exocrine manifestations of pancreatitis. In this family with mutation in PRSS1 gene, glycemic control was poor and labile, with important glycemic fluctuations. Insulin was necessary in all cases, with a wide dosage variation between the different family members. These data indicate that DM secondary to HP has difficult management. Strategies to improve the glycemic control in affected patients should be pursued.
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