title: Clinical aspects of pancreatogenic diabetes secondary to hereditary pancreatitis.
abstract: BACKGROUND
  Hereditary pancreatitis is a rare inherited form of pancreatitis, characterized by recurrent episodes of acute pancreatitis with early onset and/or chronic pancreatitis, and presenting brittle diabetes, composed of episodes of nonketotic hyperglycemia and severe hypoglycemia. The existing literature regarding this form of diabetes is scarce. In this report, clinical features of pancreatogenic diabetes secondary to hereditary pancreatitis are presented along with recommendations for appropriate medical treatment.
  
  
  RESULTS
  Clinical data from five patients of a family with pancreatogenic diabetes secondary to hereditary pancreatitis were analyzed. The average time between hereditary pancreatitis and diabetes diagnosis was 80 ± 24 months (range: 60-180 months) with a mean age of 25.6 ± 14.7 years (range: 8-42 years), four patients used antidiabetic agents for 46 ± 45 months and all progressed to insulin therapy with a mean dose of 0.71 ± 0.63 IU/kg (range: 0.3-1.76 IU/kg). The glycemic control had a high variability with average capillary blood glucose of 217.00 ± 69.44 mg/dl (range: 145-306 mg/dl) and the average HbA1c was 9.9 ± 1.9% (range: 7.6-11.6%). No ketoacidosis episodes occurred and there were several episodes of hospitalization for severe hypoglycemia.
  
  
  CONCLUSIONS
  Diabetes mellitus secondary to hereditary pancreatitis presents with early onset, diverse clinical presentation and with extremely labile glycemic control. Diabetes treatment varies according to the presentation and insulin is frequently necessary for glycemic control.
authors.affiliation: Endocrinology Section, Department of Internal Medicine, Medical School, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, Brazil ; Laboratory of Translational Endocrinology, Instituto de Biofísica Carlos Chagas Filho, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, Brazil ; Diabetes and Nutrology Section, Department of Internal Medicine, Medical School, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, Brazil ; Endocrinology Section, Hospital Universitário Clementino Fraga Filho, Rua Rodolpho Paulo Rocco 255, Ilha do Fundão, Rio de Janeiro, RJ 21941-913 Brazil.
authors.forename: Marcio Garrison
authors.initials: MG
authors.lastname: Dytz
authors.affiliation: Diabetes and Nutrology Section, Department of Internal Medicine, Medical School, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, Brazil.
authors.forename: Pedro Arthur Hamamoto
authors.initials: PA
authors.lastname: Marcelino
authors.affiliation: Endocrinology Section, Department of Internal Medicine, Medical School, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, Brazil.
authors.forename: Olga
authors.initials: O
authors.lastname: de Castro Santos
authors.affiliation: Diabetes and Nutrology Section, Department of Internal Medicine, Medical School, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, Brazil.
authors.forename: Lenita
authors.initials: L
authors.lastname: Zajdenverg
authors.affiliation: Endocrinology Section, Department of Internal Medicine, Medical School, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, Brazil.
authors.forename: Flavia Lucia
authors.initials: FL
authors.lastname: Conceição
authors.affiliation: Laboratory of Translational Endocrinology, Instituto de Biofísica Carlos Chagas Filho, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, Brazil.
authors.forename: Tânia Maria
authors.initials: TM
authors.lastname: Ortiga-Carvalho
authors.affiliation: Diabetes and Nutrology Section, Department of Internal Medicine, Medical School, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, Brazil.
authors.forename: Melanie
authors.initials: M
authors.lastname: Rodacki
keywords: Beta-cell; Cationic trypsinogen; Hereditary pancreatitis; Pancreatogenic diabetes