Carta- Maiese M, Contreras-Vivas L, Correa-Martínez CL, D’Ambruoso-Ruggiero A, Pérez J, Morales C

Language: Spanish
References: 12
Page: 48-50
PDF size: 122.95 Kb.

ABSTRACT

Familial hypertriglyceridemia is a dominant autosomal disorder that affects 1 of every 10000 people, with an unclear pathogenesis. Several hyperlipoproteinemias are the cause of 8% of all cases of acute pancreatitis among the pediatric population; the latter affects 0,006-0,008% of this age group. Thus, recurrent episodes of acute pancreatitis have been linked to levels of triglycerides that even higher than 1000 mg/dL. Destruction of pancreatic beta cells for other causes than autoimmune responses, determines the development of type 1 diabetes mellitus. The following is a case of a fourteen-year-old male patient, with diagnosis of type V familial hyperlipoproteinemia at the age of 6 years, reaching levels of triglycerides as high as 15780 mg/dl. The patient has presented 5 episodes of acute pancreatitis since the age of 7 years, caused by hyperlipoproteinemia. At the age of 8 years, he is diagnosed with type 1 diabetes mellitus, originated by the tissue damage caused by recurrent pancreatitis. Sustained study of the patient since the appearance of symptoms, along with an accurate clinical approach and the performance of adequate diagnostic tests, have all allowed to establish the chronological sequence of the pathologic processes involved in the present illness of the patient: severe familial hyperlipoproteinemia as the cause of recurrent pancreatitis, as well as the constant damage to the pancreatic parenchyma as the trigger of type 1 diabetes mellitus, thus ruling out autoimmune etiology in this case. All of the mentioned pathologies are of rare incidence among pediatric population.

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