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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.
Hepatomegaly: Medical imaging: Longitudinal axis > 15.5 cm at the hepatic midline, or > 16.0 cm at the midclavicular line [14] Autopsy: Weight over upper limit of standard reference range, of 1,860 g (4.10 lb) in men [12] and 1,770 g (3.90 lb) in women. [13] Lymph nodes: Lymphadenopathy: Generally 10 mm [15] [16
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.
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 ...
To make it a full, satisfying dinner, serve over cooked brown rice. When shopping for simmer sauce, look for one with 400 mg of sodium or less and check the ingredient list for cream or fish sauce ...
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.