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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.
Mice and dogs with MPS I have been successfully treated with gene therapy. Most vectors can correct the disease in the liver and spleen, and can correct brain effects with a high dosage. Gene therapy has improved survival, neurological, and physical symptoms; however, some animals have developed unexplained liver tumors.
Autopsy: Cardiomegaly has been suggested when the heart weighs more than >399 grams in women and >449 grams in men. [8] Large intestine: Dilation Upper limit of normal range of diameter: [9] Cecum: 10.5 cm; Ascending colon: 7.0 cm; Transverse colon: 6.5 cm; descending colon and sigmoid colon: 6.8 cm; Rectum near rectal/sigmoid junction: 7.5 cm ...
Splenomegaly is an enlargement of the spleen. [1] The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen.Splenomegaly is one of the four cardinal signs of hypersplenism which include: some reduction in number of circulating blood cells affecting granulocytes, erythrocytes or platelets in any combination; a compensatory proliferative response in the bone marrow; and the ...
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]
A male who inherits a defective X chromosome, though, usually does not have another X chromosome to compensate for the mutant gene. Thus, a female would need to inherit two mutant genes to develop MPS II, while a male patient only needs to inherit one mutant gene. A female carrier can be affected due to X-inactivation, which is a random process.
Portal vein thrombosis causes upper abdominal pain, possibly accompanied by nausea and an enlarged liver and/or spleen; the abdomen may be filled with fluid . [3] A persistent fever may result from the generalized inflammation. [1]
Males and females appear to be affected in equal proportions. [7] It has also become more common since the 1950s. [7] [9] Together, ulcerative colitis and Crohn's disease affect about a million people in the United States. [177] With appropriate treatment the risk of death appears the same as that of the general population. [3]