It is a rare syndrome initially described in type 1 diabetic children, in. Mauriac syndrome still exists endocrinologia y nutricion english. Mauriac syndrome ms is a rare complication of type 1 diabetes mellitus dm1. We have a problem find, read and cite all the research you need on researchgate. The incidence of this syndrome has decreased significantly with introduction of longacting insulin and better control of sugars.
Cuidados intensivos, hospital dona estefania, chlisboa central. Constantino, c farela neves, j marta, r pereira, g barata, d lopes, l. Hepatic glycogenosis, previously called mauriac syndrome, is one of them. Mauriac syndrome is a rare complication of type 1 diabetes characterized by extreme liver enlargement due to glycogen deposition, along with growth failure and delayed puberty.
Franzese a, iorio r, buono p, mascolo m, mozzillo e, valerio g. Mauriac syndrome is a rare cause of severe growth failure in. Mauriac syndrome ms is a glycogenic hepatopathy, initially described in 1930, characterized by growth failure, delayed puberty, cushingoid appearance, hepatomegaly with abnormal liver enzymes. The clinical features consist of growth retardation, hepatomegaly, and cushingoid features. It occurs in some children and adolescents with type 1 diabetes irrespective of their glycemic control. Pdf growth failure in type 1 diabetes mellitus t1dm can occur for several reasons. Mauriac syndrome ms is a rare complication of type 1 diabetes. Hepatic glycogenosis hg is a complication of poorly controlled type 1 diabetes mellitus t1dm, characterized by. Mauriac syndrome is an uncommon condition characterized by growth retardation, hepatomegaly and obesity in children with type 1 diabetes. However, there are common features noted in these patients. Mauriac syndrome, initially described by mauriac in 1930, is one of the causes of hepatomegaly and elevated liver enzymes in poorly controlled diabetic patients. Roberts syndrome is a genetic disorder characterized by limb and facial.
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