Causes of death in patients with Berardinelli-Seip congenital generalized lipodystrophy.
Josivan Gomes de LimaLucia Helena C NobregaNatalia Nobrega LimaMarcel Catão Ferreira Dos SantosPedro Henrique Dantas SilvaMaria de Fatima P BarachoDebora Nobrega LimaJulliane Tamara Araújo de Melo CamposLeonardo Capistrano FerreiraFrancisco Paulo Freire NetoCarolina de O Mendes-AguiarSelma Maria B JeronimoPublished in: PloS one (2018)
BSCL led to premature death, cutting the patients' lifespan by 30 or more years. The majority of these young patients died of liver disease or infection. Other studies are needed to understand better the mechanisms that predispose to infections, as well as to assess whether new therapies can alter the natural history of this disease.