Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019045 (hemoglobinopathies)
 2,704 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pythium infection (pythiosis) in humans has not previously been described, even in areas endemic for animal pythiosis. We report five patients with a unique presentation of fungal arteritis. The medium- to large-sized arteries were involved, and in some cases this involvement led to gangrene of the limbs, aneurysm formation, and ultimately fatal arterial leakage. All five patients were farmers. All patients, with the possible exception of one who had hemoglobin typing performed after receiving a blood transfusion, had thalassemia hemoglobinopathy syndrome. Fungal isolation was difficult. Amphotericin B treatment seemed to be ineffective. Radical surgical removal of infected tissues and oral administration of a saturated solution of potassium iodide are proposed therapy. In the tropics, where Pythium is ubiquitous, one should actively look for this fungal infection in patients with unexplained arterial occlusion, especially in the case of patients with thalassemia hemoglobinopathy syndrome.
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PMID:Human pythiosis associated with thalassemia hemoglobinopathy syndrome. 264 70

Anemia and co-existing deficiencies of zinc, iron, iodine, and vitamin A occur among children in many developing countries including NE Thailand, probably contributing to impairments in growth, immune competence, and cognition. Sustainable strategies are urgently required to combat these deficiencies. We assessed the efficacy of a micronutrient-fortified seasoning powder served with a school lunch on reducing anemia and improving the micronutrient status of rural NE Thai children. Children (n = 569) aged 5.5-13.4y from 10 schools were randomly assigned to receive a seasoning powder either unfortified or fortified with zinc (5 mg), iron (5 mg), vitamin A (270 microg), and iodine (50 microg) (per serving) and incorporated into a school lunch prepared centrally and delivered 5 d/wk for 31 wk. Teachers monitored school lunch consumption. Baseline and final micronutrient status, hemoglobinopathies, and infection or inflammation were assessed from blood and urine samples. For the primary outcome, anemia (based on hemoglobin), no intervention effect was apparent (odds ratio: 1.02 95% CI: 0.69, 1.51) after adjustment for design strata. The odds of zinc (based on serum zinc) and urinary iodine deficiency in the fortified group were 0.63 (0.42, 0.94) and 0.52 (0.38, 0.71) times those in the unfortified group, respectively. Fortification had no effect on serum retinol (0.61: 0.25,1.51), ferritin (1.12: 0.43, 2.96), or mean red cell volume (1.16: 0.82, 1.64). Therefore, a micronutrient-fortified seasoning powder is a promising vehicle for improving zinc, iodine, and hemoglobin status, and its potential for incorporation into lunch programs in day care centers and schools in NE Thailand warrants investigation.
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PMID:A multimicronutrient-fortified seasoning powder enhances the hemoglobin, zinc, and iodine status of primary school children in North East Thailand: a randomized controlled trial of efficacy. 1670 30

Pythium insidiosum is a fungus that causes disease in both animals and humans. Human pythiosis is an emerging disease in the tropical, subtropical, and temperate regions of the world, occurring in localized and systemic or vascular forms. Most patients with arterial pythiosis have an underlying hemoglobinopathy, such as thalassemia. A case is presented of a thalassemic horse stable worker who developed an ulcerative cutaneous lesion on the lower left leg followed by progressive ascending involvement of the arteries of that extremity with a necrotizing arteritis with aneurysm formation. P. insidiosum was not isolated from the ulcer by culture or wet potassium hydroxide preparations but was diagnosed by histopathologic study of a biopsy. P. insidiosum infection was quickly confirmed by immunoblot method, aiding in preoperative decision making. Many systemic antibiotics or antimycotics have not been effective in the treatment of systemic pythiosis, and radical surgical removal of all infected tissue is the only method to ensure patient survival. An orally administered saturated solution of potassium iodide, amphotericin B-oral solution, and terbinafine has succeeded only in the cutaneous form but had no favorable effect on vascular pythiosis. It is likely that immunotherapy, successfully used in animal pythiosis, may be beneficial in the treatment of human vascular pythiosis.
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PMID:Vascular pythiosis in a thalassemic patient. 1969 7