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thalassemia/hypoxia
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Hypoxemia in thalassemia.
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Data are reviewed describing hypoxemia, a newly identified feature in thalassemia. Evidence indicates platelet aggregation in the pulmonary circulation as being a key factor leading to hypoxemia and cor-pulmonale with right heart failure.
Hypoxia biomarkers, oxidative stress, and circulating microparticles in pediatric patients with thalassemia in Upper Egypt.
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This study aimed to investigate the oxidative stress, hypoxia biomarkers, and circulating microparticles (MPs) in β thalassemia major. The study included 56 children with thalassemia and 46 healthy controls. Hypoxia biomarkers, oxidative stress biomarkers, and total plasma fragmented DNA (fDNA) were
Low lung capacity and hypoxemia in children with thalassemia major.
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We evaluated lung function in 17 children with thalassemia major in stable condition receiving blood transfusions at regular intervals and subcutaneous desferoxamine daily. Total lung capacity (TLC) was below 2 SD of normal values for height in 7 of the 17 children and arterialized capillary PO2 was
[Thalassemia and pregnancy: concomitant problem of extreme resistance of the fetus to maternal anoxia].
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Hypoxemia in thalassemia.
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Alpha-thalassemia major presenting in a term neonate without hydrops.
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We describe the clinical and pathologic features of an unusual case of alpha-thalassemia major in a patient who survived to term and lived for 9 days. The neonate was nonhydropic and the clinical picture was dominated by severe hypoxia with pulmonary hypertension. The diagnosis was not suspected
The effect of transfusion on lung capacity, diffusing capacity, and arterial oxygen saturation in patients with thalassemia major.
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Our previous cross-sectional study of patients with thalassemia major suggested progressive lung changes characterized by low total lung capacity, hypoxemia, and elevated transfer factor for carbon monoxide. We reevaluated nine of the patients for three reasons: to determine the relationship of the
Peripheral retinal neovascularization in sarcoidosis with thalassemia.
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A black youth with peripheral retinal (sea fan) neovascularization had both sarcoidosis and alpha-thalassemia. The possibility that both of these disease entities were responsible for the observed pathology is explored. Most of the pathology present, the stasis, the hypoxia, and the
Abnormalities in lung function among multiply-transfused thalassemia patients: results from a thalassemia center in Malaysia.
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The aim of this study was to: (1) determine the prevalence and patterns of lung dysfunction among transfusion dependent thalassemics; (2) determine the associated factors that might contribute to this problem. This was a cross-sectional study involving 66 patients with transfusion dependent
Thyroid dysfunction in multi-transfused iron loaded thalassemia patients.
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Seventy-two transfusion-dependent iron loaded thalassemia patients were investigated for thyroid dysfunction by estimating circulating thyroid hormones (T4 and T3) and basal thyroid stimulating hormone (TSH). They were also evaluated for their liver function (biochemically) and iron overload by
Auditory involvement in thalassemia major.
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The auditory function of 75 children affected by homozygous beta0-thalassemia, managed with a low transfusion scheme and treated irregularly with low doses of desferrioxamine, and of 75 controls were examined. In 12 patients a mild bilateral conductive hearing impairment due to bony hypertrophy
Survival of homozygous alpha-thalassemia with aplasia/hypoplasia of phalanges and jejunal atresia.
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We report a case of survival of homozygous alpha-thalassemia with aplasia/hypoplasia of phalanges and jejunal atresia. The occurrence of these malformations is consistent with the postulation that intra-uterine hypoxia due to the presence of hemoglobin Bart's (Hb Bart's) is the causative factor for
Pulmonary function abnormalities in thalassemia major and the role of iron overload.
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To determine the predominant abnormality of pulmonary function in patients with thalassemia major (TM), we evaluated 29 patients with TM who were receiving hypertransfusion therapy and chelation with desferrioxamine (DFO), and who ranged in age from 6 to 40 yr (mean 19.8 +/- 8.5 yr). A reduction in
Relationship Between Serum Hepcidin and Ferritin Levels in Patients With Thalassemia Major and Intermedia in Southern Iran.
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BACKGROUND
Hepcidin is a key regulator of iron absorption in humans. It is mainly affected by hypoxia and iron stores.
OBJECTIVE
The current study aimed to determine the correlation between serum hepcidin and ferritin levels in patients with Thalassemia Major (TM) and Thalassemia Intermedia
FIRST IDENTIFICATION OF HEMOGLOBIN LANSINGRAMATHIBODI [α87(F8)His → Gln; CAC>CAG (HBA1: c.264C>G)] IN A THAI FAMILY WITH SPURIOUS HYPOXEMIA.
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We report, for the first time, hemoglobin (Hb) Lansing-Ramathibodi [α87(F8)His → Gln; CAC>CAG (HBA1: c.264C>G)] in four members of a Thai family presented with low measured oxygen saturation by pulse oximetry (SpO2), with discrepancy between low SpO2 and normal calculated oxygen saturation by
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