Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hyperreactive malarious splenomegaly (HMS) represents an abnormal immune response to recurrent malaria, characterized by excessive production of both IgM and IgG antibodies. It has both a racial and a familial distribution in various parts of the world. Immune responses to many foreign antigens, including those of malaria, are under genetic control of the major histocompatibility locus (MHC), through the influence of HLA antigens on regulatory T-lymphocyte activity. It is therefore likely that this region also contains the genetic determinants for HMS, which would be reflected in associations between HMS and particular HLA antigens or haplotypes. Genetic studies of the Watut people of Papua New Guinea have not shown any association between HMS and a wide range of red cell and serum polymorphisms. However, HMS in this group is associated with the class II HLA antigen DR2, and with high levels of HLA heterozygosity. Formal genetic analysis of family data also points to a sex-linked gene as a further determinant of overresponsiveness to malaria in the Anga. These findings suggest that more than one genetic system may be involved in the development of HMS, and that the combined effects of several genetic determinants may be responsible for the extraordinarily high frequency of HMS found in the Upper Watut Valley.
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PMID:The genetic basis of hyperreactive malarious splenomegaly. 269 22

Visceral leishmaniasis is endemic in District Dir, NWFP. We evaluated 10 patients with visceral leishmaniasis at DHQ Hospital Timergara District Dir, N.W.F.P. All patients were in the age range 2 to 10 years. The predominant clinical features in these were chronic fever (10), splenomegaly (10), hepatomegaly (10), weight loss (10) and abdominal distention (5). Lymphadenopathy was absent. Common laboratory abnormalities included anaemia (10), leucopenia (7), thrombocytopenia (10) and hypergammaglobulinaemia (10). Formal Gel test was positive in all patients (100%) and all had positive bone marrow smears for Leishmania Donovani (L.D.) bodies (10). The response to stibogluconate (Glucantime Sodium) therapy was good with a 100 percent cure rate.
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PMID:Visceral leishmaniasis in District Dir, NWFP. 981 85

WE DESCRIBE FOUR PATIENTS WITH NIEMANN: Pick disease type C (NPC), in whom the presentation was isolated splenic enlargement; this remained the only abnormality for a number of years. Diagnosis can be suggested by either finding abnormal storage material in a tissue biopsy specimen or by showing a modest elevation in plasma chitotriosidase activity. In patients with suggestive abnormalities, filipin staining of a skin fibroblast sample should confirm the abnormality in cholesterol trafficking. Formal esterification studies and mutation analysis should also be performed, especially if prenatal testing is to be performed in subsequent pregnancies. If the diagnosis is not considered and established, the family are at risk of having further affected children. Investigation of patients with isolated splenomegaly is not complete until NPC has been excluded.
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PMID:Isolated splenomegaly as the presenting feature of Niemann-Pick disease type C. 1131 91

Based on the reports of 18 fever related deaths in Bhojpur Primary Health Centre (PHC) of Moradabad District (UP), a study was undertaken during September and October, 2000 to explore the possibility of outbreak of malaria in the area and the reasons of the outbreak. The slide positivity rate (SPR) and slide falciparum rate (SFR) in active fever surveys were found to be 84.7% and 82.2% respectively. Among children the average enlarged spleen and spleen rate were 1.9 and 27.4 per cent respectively. The mortality rate of Anopheles culicifacies in DDT, Malathion and Deltamethrin was 42.5, 86.7 and cent per cent respectively. The results of the study revealed that the outbreak was of malaria with predominance of Plasmodium falciparum causing several deaths in the affected villages. The investigation revealed that there was disruption of surveillance and supervision of malaria activities.
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PMID:Malaria outbreak in Bhojpur PHC of district Moradabad, Uttar Pradesh, India. 1476 29