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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 .
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.
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 ...
Katz syndrome is a rare congenital disorder, presenting as a polymalformative syndrome characterized by enlarged viscera, hepatomegaly, diabetes, and skeletal anomalies that result in a short stature, cranial hyperostosis, and typical facial features.
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.
Like hepatic adenomas, they are diagnosed with imaging and biopsies as needed. Treatment of liver cell adenomatosis is difficult due to the multiple, widespread lesions. Liver imaging should be reviewed to see if it is possible to surgically remove the tumors. [7] Liver transplantation is a treatment option for some patients. [7]
The act authorized diabetes research and training centers, and an intergovernmental diabetes coordinating committee that included representatives from the NIAMDD and six other NIH Institutes. January 1975 —The National Arthritis Act of 1974 (P.L. 93—640) was signed into law to further research, education, and training in the field of ...
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