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)

The paper reports the case of a previously healthy 4-year-old-girl who presented with pallor, fever and hepatosplenomegaly. Laboratory findings included pancytopenia, hypertriglyceridemia and hyperferritinemia. Initial diagnosis of kala-azar could not be confirmed because of the absence of clinical evidence, negativity of bone marrow aspiration or specific serology for visceral leishmaniasis. Repeated marrow aspiration, performed due lack of clinical response, revealed histiocytes showing haemophagocytosis consistent with haemophagocytic lymphohistocytosis (HLH) and appropriate treatment was started. She continued to have high-grade fever, and a third bone marrow aspiration ultimately revealed presence of Leishmania amastigotes with evidence of active haemophagocytosis. The girl was treated with liposomal amphotericin (AmBisome) for 5 days, following which she recovered rapidly with definitive remission.
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PMID:Visceral leishmaniasis and haemophagocytic syndrome in an Omani child. 2401 26

Visceral leishmaniasis (VL or kala-azar) is most endemic in Asia and Africa and commonly affects young children. It is usually caused by Leishmania donovani or Leishmania infantum that are transmitted by Phlebotomine sand flies. Transmission may be anthroponotic or zoonotic or both, depending on the endemic area. Clinical features include fever, hepatosplenomegaly, weight loss and pancytopenia. Younger age, malnutrition and immunosuppression (HIV infection, use of immunosuppressive drugs) are risk factors. Many infections remain asymptomatic. Diagnosis is made by demonstration of the Leishmania parasite in aspirates of lymph node, bone marrow or spleen. Serological tests such as rK39 strip test are widely used but the sensitivity varies. qPCR is useful to detect low numbers of parasites and to monitor treatment. Treatment is with AmBisome monotherapy in most areas but with drug combinations elsewhere. HIV co-infected patients are most difficult to treat and often relapse. Control efforts focus on case finding, availability of diagnostic tools, reservoir control and protection from sand flies (insecticides, bed nets). There is no human vaccine.
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PMID:Visceral leishmaniasis: a forgotten epidemic. 2689 6