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Query: UMLS:C0019045 (hemoglobinopathies)
 2,704 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The frequency and etiology of anemia were investigated in 2,913 children between six and 16 years of age in Sanliurfa, in the southeast region of Turkey. Anemia was found in 142 (7.8%) children in the 6-11 years of age group, and in 16 (1.5%) in the 12-16 years of age group; in total, in 158 (5.4%) children. Causes were iron deficiency in 93 (58.9%) children, beta-thalassemia heterozygosity in 10 (6.3%) children, chronic disease that causes anemia of inflammation in 30 (19.0%) children, and intestinal parasitic infections in 17 (10.8%) children. In eight (5.1%) children, the cause of anemia could not be determined. The study's results showed that iron deficiency anemia and chronic and parasitic disease are important problems in schoolchildren of Sanliurfa, while beta-thalassemia and hemoglobinopathies have less importance.
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PMID:The frequency and etiology of anemia among children 6-16 years of age in the southeast region of Turkey. 1093 71

Foreign adopted children and children of asylum applicants and refugees, newly arrived in Denmark, often have lived under conditions that make the following diagnostic considerations relevant: scabies, lice, impetigo and fungal skin infections, nutritional iron deficiency or bleeding, anaemia caused by hook worms in the gastrointestinal tract, malaria, tuberculosis, hepatitis B, HIV infection and various intestinal parasites. Haemoglobinopathies including sickle cell anaemia and talassaemia should also be kept in mind in anaemia. Immigrant children are admitted to hospital approximately twice as frequently as Danish children but with the same diagnoses apart from some increased frequency of psychological and behavioural disturbances and talassaemia.
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PMID:[Diseases among refugee and immigrant children]. 1110 68

In sub-Saharan Africa, anaemia in pregnancy results from multiple causes including malaria, iron deficiency and haemoglobinopathies. In a cross-sectional study among 530 pregnant women in Ghana in November-December 1998, red blood cell indices were analysed with respect to malaria, serum concentrations of ferritin and C-reactive protein (CRP), and the haemoglobin and alpha-globin genotypes. Anaemia (haemoglobin [Hb] < 11 g/dL) was found in 54% of the women; 63% harboured malaria parasites at predominantly low numbers. Ferritin levels were considerably influenced by malaria and inflammatory processes (CRP > 0.6 mg/dL). Depending on the definition applied, the prevalence of iron deficiency ranged between 5% and 46%. The HbAS trait was observed in 14%, HbAC and elevated HbF in 7% each, and sickle cell disease in 1%. Heterozygous beta-thalassaemia was present in 1% of the women and alpha(+)-thalassaemia in 33% (29% heterozygous, 4% homozygous). Women with HbAS had higher malaria parasite densities than those with HbAA. In individuals with highly elevated HbF (> 10%), parasitaemia occurred in 27% only. Low gravidity, second trimester of pregnancy, malaria, raised CRP levels, and homozygous alpha(+)-thalassaemia were independent risk factors for anaemia in multivariate analysis. alpha(+)-Thalassaemia, however, was associated with a lesser degree of malarial anaemia when compared to non-thalassaemic women. Iron deficiency appears not to be a major health problem in this population. Haemoglobinopathies are common but, except for homozygous alpha(+)-thalassaemia, do not substantially contribute to anaemia in pregnancy. alpha(+)-Thalassaemia ameliorates malarial anaemia in pregnant women.
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PMID:Anaemia in pregnant Ghanaian women: importance of malaria, iron deficiency, and haemoglobinopathies. 1113 70

The continuing unresolved debate over the interaction of iron and infection indicates a need for quantitative review of clinical morbidity outcomes. Iron deficiency is associated with reversible abnormalities of immune function, but it is difficult to demonstrate the severity and relevance of these in observational studies. Iron treatment has been associated with acute exacerbations of infection, in particular, malaria. Oral iron has been associated with increased rates of clinical malaria (5 of 9 studies) and increased morbidity from other infectious disease (4 of 8 studies). In most instances, therapeutic doses of oral iron were used. No studies in malarial regions showed benefits. Knowledge of local prevalence of causes of anemia including iron deficiency, seasonal malarial endemicity, protective hemoglobinopathies and age-specific immunity is essential in planning interventions. A balance must be struck in dose of oral iron and the timing of intervention with respect to age and malaria transmission. Antimalarial intervention is important. No studies of oral iron supplementation clearly show deleterious effects in nonmalarious areas. Milk fortification reduced morbidity due to respiratory disease in two very early studies in nonmalarious regions, but this was not confirmed in three later fortification studies, and better morbidity rates could be achieved by breast-feeding alone. One study in a nonmalarious area of Indonesia showed reduced infectious outcome after oral iron supplementation of anemic schoolchildren. No systematic studies report oral iron supplementation and infectious morbidity in breast-fed infants in nonmalarious regions.
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PMID:Iron and its relation to immunity and infectious disease. 1116 May 94

In 1996, The Ministry of Health and Inciensa conducted the latest National Nutrition Survey, to provide support and guidance to the national policies, plans and programs in the field of food and nutrition. The present paper reports the results for the prevalence of anemia estimated in a total of 884 women of reproductive age, in three areas: metropolitan area, other urban areas and rural areas. Anemia was determined through measurements of hemoglobin, plasma ferritin and plasma folates. In addition, hemoglobin patterns were determined by electrophoresis. The cutt-off points used were those recommended by the WHO. Anemia was present in 18.6% of the women. Severe to moderate deficiency of iron (< 12 ng/dl) and of folates (< 6 ng/dl) were found in 43.2 and 24.7% of women respectively, with statistically significant differences by area of residence. The magnitude of the problem for anemia can be classified as mild, for iron deficiency as severe, and for folate deficiency as moderate. In conclusion, Anemia represents a public health problem for Costa Rica that has remained constant throughout the last decade. In women of reproductive age, iron deficiency is the main cause of Anemia, followed by folates deficiency, and in a small percentage hemoglobinopathies. Intestinal parasites are not longer a mayor cause of Anemia. Prevalence of Anemia is influenced by place of residence, but not by age. In summary, despite the favorable health conditions present in Costa Rica, the prevalence of Anemia and of iron deficiency are similar to those of the Latin-American region. To improve this situation, public health interventions are necessary.
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PMID:[Prevalence of nutritional anemia in women of reproductive age. Costa Rica. National nutrition survey, 1996]. 1151 28

The American Academy of Pediatrics (AAP) recommends screening for anemia between the ages of 9 to 12 months with additional screening between the ages of 1 and 5 years for patients at risk. The screening may be universal or selective depending on the prevalence of iron deficiency anemia in the population. Improved infant rearing practices-including wider availability, acceptance, and use of iron-fortified formulas; iron fortification of foods; and increased awareness of the importance of dietary iron supplementation especially early in life-have lead to significant decline in the incidence of anemia in the first year of life. However, incidence of iron deficiency and ensuing anemia in children between 1 and 2 years continues to be significant and an important issue. Although iron deficiency may develop soon after cessation of or inadequate iron intake, anemia secondary to iron deficiency develops gradually over a period of several weeks to months. For children who have received/are receiving iron-fortified infant formulas and foods, hemoglobin screening at 9 to 12 months of age is inappropriate as there may not have been sufficient time to develop anemia, despite the rapid growth rate at this age. Widespread implementation of hemoglobin electrophoresis included in the neonatal metabolic screening programs in many states in the United States now has resulted in earlier diagnosis of hemoglobinopathies. Screening children at 9 to 12 months of age for hemoglobinopathies is somewhat redundant now. Screening for anemia before or around 1 year of age should continue to be important for communities and children at risk. Universal screening of toddlers at a later time allows sufficient time for nutritional anemia to become evident after the child has been weaned off iron-fortified formulas, for the influence of toddler dietary fads to manifest, and for evaluation of tolerance of cow's milk protein. This may be addressed via 2 approaches. The first involves postponing the currently recommended screening or an additional screening for anemia between 15 to 18 months of age. Determination of hemoglobin (or hematocrit) is not the optimal way to identify children at risk from effects of iron deficiency as it fails to identify patients who are iron-deficient but are not anemic. Long-term psychomotor, behavioral, and developmental effects secondary to iron deficiency anemia are known but sufficient data are lacking regarding the role of iron deficiency without anemia. Development and evaluation of sensitive, specific, and cost-effective screening tools to identify children at risk for iron deficiency is important. Until such methods are instituted, the AAP should emphasize and recommend universal screening for anemia during the second year of life.
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PMID:Screening for anemia in children: AAP recommendations--a critique. 1153 74

Anemia in children is commonly encountered by the family physician. Multiple causes exist, but with a thorough history, a physical examination and limited laboratory evaluation a specific diagnosis can usually be established. The use of the mean corpuscular volume to classify the anemia as microcytic, normocytic or macrocytic is a standard diagnostic approach. The most common form of microcytic anemia is iron deficiency caused by reduced dietary intake. It is easily treatable with supplemental iron and early intervention may prevent later loss of cognitive function. Less common causes of microcytosis are thalassemia and lead poisoning. Normocytic anemia has many causes, making the diagnosis more difficult. The reticulocyte count will help narrow the differential diagnosis; however, additional testing may be necessary to rule out hemolysis, hemoglobinopathies, membrane defects and enzymopathies. Macrocytic anemia may be caused by a deficiency of folic acid and/or vitamin B12, hypothyroidism and liver disease. This form of anemia is uncommon in children.
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PMID:Anemia in children. 1168 80

Most patients receiving anemia therapy respond well, with a significant rise in hemoglobin concentration. However, approximately 5%-10% of patients fail to show a satisfactory response despite high doses of erythropoietin. The definition of hyporesponsiveness to anemia therapy is somewhat arbitrary, but it is generally regarded as a failure to achieve a hemoglobin concentration of 10-11 g/dL despite a dose of erythropoietin in excess of 200 U/kg weekly. The condition has many causes, the most important ones being iron deficiency, infection or inflammation, and underdialysis. Investigating a patient's poor response to erythropoietin should begin with a check for compliance, followed by screening for iron deficiency. If doubt exists about the presence of iron deficiency, then a trial of intravenous iron may be given. A reticulocyte count may be helpful. A significantly elevated count suggests the presence of blood loss or hemolysis. The level of C-reactive protein (CRP) may be useful as an indicator of underlying inflammation, and underdialysis may be corrected by increasing the dialysis prescription. If other, minor causes of hyporesponsiveness to erythropoietin have been excluded, then a bone marrow biopsy should be performed. Some patients may require higher doses of erythropoietin, and it is not unreasonable to increase the dose to 10,000 U thrice weekly. Some causes of hyporesponsiveness to erythropoietin, such as iron deficiency and underdialysis, are easily corrected; but others, such as primary bone marrow disorders and hemoglobinopathies, are not possible to overcome.
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PMID:Hyporesponsiveness to anemia therapy--what are we doing wrong? 1188 26

In developing countries, multiple comorbidities such as malnutrition, parasitoses, and hemoglobinopathies contribute to the aggravation of anemia observed in patients with end-stage renal diseases. We analyze here the results of a retrospective evaluation of red-cells indices and iron parameters conducted at the end of December 2000 in 304 prevalent Tunisian patients (sex ratio, 1.05; mean age, 53.7 years) receiving chronic hemodialysis for a median duration of 49.6 months (range, 1.6 to 278). Anemia, observed in 87.8% of patients, was normochromic and normocytic in 73% of cases. Only 2% of patients had microcytic and hypochromic anemia. Iron deficiency was observed in 21.6% of anemic patients. The mean rate of hemoglobin was significantly higher in men and in patients with polycystic kidney disease as the cause of renal failure. There was a positive correlation between hemoglobin values and the quality of dialysis. Only 10.8% of patients were on recombinant human erythropoietin (rHuEPO) and 38% required regular transfusions. We conclude that anemia observed in our patients had, in most cases, the characteristics of renal anemia and could be attributed to a deficit of renal production of erythropoietin. However, for financial reasons, prescription of rHuEPO is rather restrictive and blood transfusion remains largely used. The nephrology community and dialysis providers should increase their efforts to improve the anemia care of dialyzed patients in developing countries.
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PMID:Anemia and end-stage renal disease in the developing world. 1219 29

We describe, in Ethiopia, a third successful pattern of human adaptation to high-altitude hypoxia that contrasts with both the Andean "classic" (erythrocytosis with arterial hypoxemia) and the more recently identified Tibetan (normal venous hemoglobin concentration with arterial hypoxemia) patterns. A field survey of 236 Ethiopian native residents at 3,530 m (11,650 feet), 14-86 years of age, without evidence of iron deficiency, hemoglobinopathy, or chronic inflammation, found an average hemoglobin concentration of 15.9 and 15.0 gdl for males and females, respectively, and an average oxygen saturation of hemoglobin of 95.3%. Thus, Ethiopian highlanders maintain venous hemoglobin concentrations and arterial oxygen saturation within the ranges of sea level populations, despite the unavoidable, universal decrease in the ambient oxygen tension at high altitude.
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PMID:An Ethiopian pattern of human adaptation to high-altitude hypoxia. 1247 Nov 59


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