However, there are common features noted in these patients. It is shown a case of a 11 years old child examined in nino jesus hospital in barranquilla city with signs of mauriacs. Type 1 diabetes mellitus dm1 is one of the most important pediatric endocrinology diseases. Jan 12, 2009 mauriac syndrome is a severe form of growth retardation seen in patients with poorly controlled type 1 diabetes mellitus. It has different kinds of manifestations, such as systemic disturbances like hepatomegaly and dwarfism, caused by poor glycemic control. Hepatic glycogenosis hg is a complication of poorly.
There may be different forms and etiologies involved in mauriac syndrome. Pdf growth failure in type 1 diabetes mellitus t1dm can occur for several reasons. Mauriac syndrome ms is a rare complication of type 1. Mauriacs 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. Franzese a, iorio r, buono p, mascolo m, mozzillo e, valerio g. 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. Mauriac syndrome is a rare cause of severe growth failure in t1dm.
Clinical symptoms of onemonth duration were characterized by nausea, vomiting, and. Mauriac syndrome is a rare cause of severe growth failure in. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext. Laboratory tests were performed to rule out differential diagnoses. Mauriac syndrome, uncommon presentation of a common disease. 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.
Type 1 diabetes mellitus is a chronic metabolic disease whith increasing incidence. Francois mauriac et therese desqueyroux ou histoire dun. Pierre mauriac syndrome and uncontrolled type 1 diabetes mellitus. 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. Mauriac syndrome ms is a rare complication of type 1 diabetes mellitus dm1.
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