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Glycogenic hepatopathy [1] (also known as Mauriac syndrome [2]) is a rare complication of type 1 diabetes characterized by extreme liver enlargement due to glycogen deposition, along with growth failure and delayed puberty. It occurs in some children and adolescents with type 1 diabetes irrespective of their glycemic control.
Hepatomegaly is enlargement of the liver. [4] It is a non-specific medical sign, having many causes, which can broadly be broken down into infection, hepatic tumours, and metabolic disorder. Often, hepatomegaly presents as an abdominal mass. Depending on the cause, it may sometimes present along with jaundice. [1]
The principal treatment for this feature of GSD Ib is filgrastim; however, patients often still require treatment for frequent infections, and a chronically enlarged spleen is a common side effect. [4] GSD Ib patients often present with inflammatory bowel disease. [5] It is the most common of the glycogen storage diseases.
Hepatosplenomegaly (commonly abbreviated HSM) is the simultaneous enlargement of both the liver (hepatomegaly) and the spleen (splenomegaly). Hepatosplenomegaly can occur as the result of acute viral hepatitis, infectious mononucleosis, and histoplasmosis or it can be the sign of a serious and life-threatening lysosomal storage disease.
Treatment is directed largely to removing the cause, or, where that is impossible, to modifying effects of the heart failure. [5] Thus, therapy aimed at improving right heart function will also improve congestive hepatopathy.
Autopsy: Weight over upper limit of standard reference range, of 230 g (8.1 oz) [12] [13] Thoracic aorta: Dilation Diameters: >3.5 cm generally [30] Upper limits of standard reference range: Ascending aorta: 3.3 cm in small young individuals [31] 4.3 cm among large elderly individuals. [31] Descending thoracic aorta, 2.3 cm among small young ...
Physical symptoms generally include coarse or rough facial features (including a flat nasal bridge, thick lips, and enlarged mouth and tongue), short stature with disproportionately short trunk , dysplasia (abnormal bone size and/or shape) and other skeletal irregularities, thickened skin, enlarged organs such as liver (hepatomegaly) or spleen ...
The treatment of chronic liver disease depends on the cause. Specific conditions may be treated with medications including corticosteroids, interferon, antivirals, bile acids or other drugs. Supportive therapy for complications of cirrhosis include diuretics, albumin, vitamin K, blood products, antibiotics and nutritional therapy.