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Asplenia. Specialty. Medical genetics. Asplenia refers to the absence of normal spleen function and is associated with some serious infection risks. Hyposplenism is used to describe reduced ('hypo-') splenic functioning, but not as severely affected as with asplenism. Functional asplenia occurs when splenic tissue is present but does not work ...
Prognosis. Almost invariably fatal without treatment [2] An overwhelming post-splenectomy infection (OPSI) is a rare but rapidly fatal infection occurring in individuals following removal (or permanent dysfunction) of the spleen. The infections are typically characterized by either meningitis or sepsis, and are caused by encapsulated organisms ...
Surgically removed spleen of a child with thalassemia. It is about 15 times larger than normal. A splenectomy is the surgical procedure that partially or completely removes the spleen. The spleen is an important organ in regard to immunological function due to its ability to efficiently destroy encapsulated bacteria.
Wandering spleen is most commonly diagnosed in young children [3] as well as women between the ages of 20 and 40. [6] Even so, the disease is very rare and fewer than 500 occurrences of the disease have been reported as of 2005, [3] of which around 148 (including both children and adult cases) were documented to have been from between 1960 and ...
The spleen, in healthy adult humans, is approximately 7 to 14 centimetres (3 to 5⁄ in) in length. An easy way to remember the anatomy of the spleen is the 1×3×5×7×9×10×11 rule. The spleen is 1 by 3 by 5 inches (3 by 8 by 13 cm), weighs approximately 7 oz (200 g), and lies between the 9th and 11th ribs on the left-hand side and along the ...
Frequency. 1 in 100,000. Hurler syndrome, also known as mucopolysaccharidosis Type IH (MPS-IH), Hurler's disease, and formerly gargoylism, is a genetic disorder that results in the buildup of large sugar molecules called glycosaminoglycans (GAGs) in lysosomes. The inability to break down these molecules results in a wide variety of symptoms ...
Splenic sequestration crisis is a life-threatening illness common in pediatric patients with homozygous sickle cell disease and beta thalassemia. Up to 30% of these children may develop splenic sequestration crisis with a mortality rate of up to 15%. This crisis occurs when splenic vaso-occlusion causes a large percentage of total blood volume ...
Autoimmune lymphoproliferative syndrome (ALPS) is a form of lymphoproliferative disorder (LPDs). It affects lymphocyte apoptosis. [2] It is a rare genetic disorder of abnormal lymphocyte survival caused by defective Fas mediated apoptosis. [3] Normally, after infectious insult, the immune system down-regulates by increasing Fas expression on ...