Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe a 52-year-old male manifesting severe insulin resistance associated with hypereosinophilic syndrome (HES). Diabetes mellitus was initially well-controlled by an oral hypoglycemic agent, and thereafter by human insulin. Due to the progression of hypereosinophilia, hepatosplenomegaly and peripheral lymphoadenopathy, severe insulin resistance associated with diabetic ketoacidosis occurred repeatedly, despite intravenous administration of over 1,000 U per day of human insulin. A high plasma insulin-binding capacity as determined by Scatchard analysis was consistent with insulin antibody-mediated resistance. The diagnosis of HES was made due to the persistent elevation of eosinophil count and associated liver and cardiac damage. Glucocorticoid therapy successfully achieved both reducing clinical symptoms and improving glycemic control.
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PMID:Severe insulin-resistant diabetes mellitus associated with hypereosinophilic syndrome. 782 82

Congenital rubella syndrome (CRS) usually occurs in a developing fetus of a pregnant woman who has contracted rubella, usually in the first trimester. With universal rubella vaccination, the incidence of CRS in developed countries has drastically reduced. However, in developing countries, CRS continues to be a public health menace in children. Sensorineural deafness, cataract, micropthalamia, patent ductus arteriosus and pulmonary stenosis are common clinical manifestations in a child affected with CRS. Other relatively rare manifestations are hepatosplenomegaly, thrombocytopenia and blueberry muffin spots. Although it is well known that CRS predisposes towards development of diabetes mellitus usually in adults, but in children only anecdotal case reports are available. Latent viral infection, immune mediated mechanism and HLA allele predisposition are various possible pathogenic mechanisms. Majority of affected persons require insulin therapy. Hereby we are demonstrating a case of seven year old boy with CRS, who presented with diabetic ketoacidosis and was successfully managed with insulin therapy. This report intends to aware clinicians regarding predisposition of children with CRS for developing diabetes, as timely diagnosing and instituting insulin therapy will help in avoiding complications and improving their quality of life.
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PMID:Diabetic ketoacidosis in a child with congenital rubella syndrome: A case report and review of literature. 3140 63