Sindrome de mauriac pdf free

Constantino c, neves jf, marta r, pereira g, barata d, lopes l. The incidence of this syndrome has decreased significantly with introduction of longacting insulin and better control of sugars. Therese desqueroux 2 seigneur, ayez pitie, ayez pitie des fous et des folles. Mauriac syndrome is a rare cause of severe growth failure in t1dm. Roberts syndrome genetic and rare diseases information. It is a rare syndrome firstly described by mauriac in 1930 and has become even less common after the emergence of advances on diabetes treatment. A rare hepatic glycogenosis in poorly controlled type 1 diabetes. Pediatria, hospital clinico universitario lozano blesa. 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.

Artigo tipo case report mestrado integrado em medicina. After two years of satisfactory control she began to have several bouts of hospitalization with hyperglycaemic ketoacidosis, and developed tender hepatomegaly, which persisted to age 11 years. An insulindependent diabetic was diagnosed at the age of 7 years. It occurs in some children and adolescents with type 1 diabetes irrespective of their glycemic control.

Nov 11, 2015 mauriac s syndrome ms may occur in children with poor controlled type 1 diabetes mellitus dm1 and is associated with growth impairment, puberty delay and hepatomegaly with elevated transaminases. The clinical features consist of growth retardation, hepatomegaly, and cushingoid. Feb 11, 2011 roberts syndrome is a genetic disorder characterized by limb and facial abnormalities. A 17yearold male with type 1 diabetes mellitus t1dm presented to clinic with elevated transaminases and a positive antinuclear antibody ana screen. However, there are common features noted in these patients. Mauriac syndrome is associated with poor metabolic control of. Mauriac syndrome, diabetes mellitus tipo 1, undernutrition, malnutrition, dwarfism, hypogonadism.