Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A microchromatographic procedure (Isolab Fast Hb Test System) which was developed for the quantitation of Hb AI (10) has been found useful for the quantitation of Hb F in samples that contain Hb S and/or Hb C but no Hb A (% Fmicro). This method has been evaluated through analyses of known mixtures of Hb F and Hb S. The Hb Fmicro levels in patients with sickle cell anemia and related conditions were compared with results obtained by alkali denaturation (% FAD) and conventional DEAE-cellulose chromatography (% FDE). This microchromatographic technique is a fast, simple, and sensitive method for Hb F quantitation in patients with Sickle cell disease.
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PMID:Microchromatographic quantitation of fetal hemoglobin in patients with sickle cell disease. 50 Mar 76

The effect of riboflavin supplementation (5mg twice daily for 8 weeks) on reduced blood glutathione (GSH) and iron status was assessed in 18 patients with sickle cell disease (SCD-HbSS). Twelve SCD patients and 13 normal (Hb-AA) subjects served as the control. The total iron binding capacity (TIBC) and serum ferritin (SF) were significantly higher (p < 0.01), but GSH level, haemoglobin and transferrin saturation (TS) were significantly lower (p < 0.001) in SCD patients than in normal subjects. The administration of riboflavin elicited a significant increase (p < 0.01) in serum iron and TS but a non significant increase in SF and circulating Hb. The GSH level varied little in riboflavin supplemented but decreased significantly in unsupplemented SCD. The disparity in GSH concentration might reflect availability of FAD for regeneration of GSH from glutathione. Likewise, the haematological improvement in the supplemented group supports the assertion that riboflavin enhances erythropoiesis. For an effective management of SCD in Africa, a closer attention should be directed to the riboflavin status in haemolytic disorders.
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PMID:Clinical trial of riboflavin in sickle cell disease. 829