Mauriac syndrome is a very rare syndrome that occurs in poorly controlled type 1 diabetes mellitus with diabetic complications. Its cardinal features include delayed growth and puberty, hepatomegaly, and moon faces. These features were attributed mainly to insulin deficiency and sub-optimal diabetic management. Its incidence is decreasing due to the newer insulin formulation and intensive blood glucose control. Early recognition and management of this syndrome may improve the outcome of these patients. Recently, there are increasing reports of this syndrome. Here, we present the cases of two adolescent males with type 1 diabetes who presented with the classical features of Mauriac syndrome.
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| http://dx.doi.org/10.7759/cureus.14704 | DOI Listing |
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Almost 100 years ago, the French physician Pierre Mauriac described a syndrome named after him, resulting from poorly controlled type 1 diabetes, with growth retardation, delayed puberty, Cushingoid features and hepatomegaly. With modern diabetes care, this is very rare but does occur; however, despite the condition having important clinical implications and being easily treatable, this diabetes complication remains relatively unknown. We present here an authentic patient case in the form of a young man with glycemically poorly controlled type 1 diabetes who developed hepatomegaly, hyperlactatemia and histopathological changes in the liver consistent with glycogenosis, a state readily reversed by normalization of glycemia.
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Mauriac syndrome is a rare complication of longstanding, poorly controlled type 1 diabetes in pediatric patients. Mauriac syndrome is characterized by hepatomegaly and growth retardation. This case report discusses a 14-year-old girl with persistent, poorly controlled type 1 diabetes mellitus (T1DM) admitted to the pediatric intensive care unit (PICU), where she was ultimately diagnosed with Mauriac syndrome.
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Case Rep Endocrinol
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University of Calgary, Calgary, Alberta, Canada.
Mauriac syndrome is a rare disorder that occurs in patients with type 1 diabetes mellitus (T1DM) with glucose levels significantly above target, characterized by hepatomegaly, growth delay, and cushingoid features. Another distinguishing feature of Mauriac syndrome is persistent lactatemia during diabetic ketoacidosis (DKA) management. We present a case of an 18-year-old patient with T1DM who presented in DKA and then developed elevated lactate levels leading to a diagnosis of Mauriac syndrome.
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