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)

As a clinical entity, sickle cell anemia (SCA) is known to be relatively rarer in Africans than in the African-American population of the United States. Paradoxically, sickle cell trait (SCT), the non-anemic, heterozygous condition, is about three times more common among indigenous Africans than in African-Americans. The ratio of SCA to SCT is 1:50 for African-Americans, and less than 1:1,000 for tropical Africans. This etiological disparity is attributed to an anti-sickling agent, thiocyanate, (SCN-) found abundantly in staple African foods, such as the African yam (Dioscorea sp) and cassava (Manihot utilissima). Staple American foods have negligible SCN-concentrations. Nonstaple foods in the American diet, such as carrots, cabbage, and radishes, have SCN- levels far below the African yam and cassava. This finding explains the high incidence of SCA among African-Americans and its rarity in Africans.The author concludes that SCA is a congenital deficiency anemia, ameliorable by prophylactic diets of foods with high SCN- contents. Thus, "thiocyanate deficiency anemia" is nutritionally a more correct clinical status for those born with the homozygous sickle hemoglobin genome. Just as any iron undernourished person can suffer from iron deficiency anemia, sickle hemoglobin homozygotes suffer from "thiocyanate deficiency anemia" when they subsist on SCN-deficient foods. This article reviews the role of dietary SCN- in SCA control.
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PMID:Anti-sickling effect of dietary thiocyanate in prophylactic control of sickle cell anemia. 379 84

About 25% of children with sickle cell disease (SCD) have statural deficit and retarded skeletal maturation and pubertal development. These disorders have been attributed to low serum concentrations of zinc. We report the study of serum zinc in 34 patients with SCD, of whom 17 had statural deficit (SCS group) and 17 without growth deficiency (SCN group). They were compared with 20 normal children (N group). GH secretion and thyroid function were investigated in the group with short stature (SCS) in order to rule out GH deficiency and hypothyroidism. The mean serum zinc concentration was 108.45+/-22.888 microg/100 ml in the N group, 87.529+/-30.069 microg/100 ml in the SCN group and 82.765+/-34.766 microg/100 ml in the SCS group. There was a statistically significant difference between the mean of the N group and the mean of the SCN group (p<0.05) but no difference was found between the two groups of patients (N>SCN=SCS). Although there is a well-established zinc deficiency in patients with SCD, the statural retardation found in the SCS group could not be attributed only to zinc deficiency. Many other severe metabolic disturbances are also involved.
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PMID:Study of zinc and growth hormone in sickle cell disease. 1145 28

Sickle cell disease (SCD), the most common hemoglobinopathy, is an increasing cause of chronic kidney disease. In the last decade, we have witnessed a better understanding in the characterization of clinical manifestations and pathogenesis of sickle cell nephropathy. The spectrum of renal diseases during SCD includes various renal manifestations such as impairment of urinary concentrating ability, defect in urine acidification, renal papillary necrosis and proteinuria related to glomerular injury leading to progressive end-stage renal disease. Endothelial dysfunction related to chronic hemolysis and the relative renal hypoxia caused by vaso-occlusive sickle red blood cells are probably two key factors for SCN development. Optimal therapeutic management (including the use of blockers of the renin-angiotensin system) of patients with proteinuria remains to be determined. Renal replacement therapy with dialysis is required in SCD patients with end-stage renal disease but these patients should probably undergo kidney transplantation that requires careful management.
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PMID:[Spectrum of renal manifestations in sickle cell disease]. 2411 2