title: [Bezafibrate in an infant with congenital generalized lipodystrophy and severe hypertriglyceridemia].
abstract: Congenital generalized lipodystrophy (CGL) with severe hypertriglyceridemia in a children less than 1 year of age is associated with worse metabolic risk. We used data from patient records, as well as extensive literature research to write the manuscript. We report the case of an infant with typical phenotype of CGL and hypertriglyceridemia of 1,360 mg/dL who was treated with bezafibrate at a dose of 30 to 60 mg/day from age 11 months to 5.5 years old, with a measurement of nadir of triglycerides of 55 mg/dL. Clinical evolution and clinical laboratory tests before and after bezafibrate were carried out over 5 years and 6 months. Phenotype was classified as CGL type 2. Despite the efficient control of hypertriglyceridemia and absence of development of diabetes mellitus, the use of bezafibrate did not prevent the onset of hepatic steatosis during evolution. Hypolipidemic therapy with bezafibrate proved effective in maintaining the levels of triglycerides, cholesterol and its fractions at normal levels, and its use was not correlated with severe side effects during the described period.
authors.forename: Rogério Santiago
authors.initials: RS
authors.lastname: Araújo
authors.forename: André de Paula Silva
authors.initials: Ade P
authors.lastname: Ramos
authors.forename: Máriton de Araújo Sousa
authors.initials: Mde A
authors.lastname: Borges
mesh_terms: D001629:Bezafibrate / Q000627:therapeutic use*; D057238:Growth Charts; D006801:Humans; D015228:Hypertriglyceridemia / Q000188:drug therapy*; D000960:Hypolipidemic Agents / Q000627:therapeutic use*; D007223:Infant; D052497:Lipodystrophy, Congenital Generalized / Q000188:drug therapy*; D008297:Male
chemical_list: D000960:Hypolipidemic Agents; D001629:Bezafibrate