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thalassemia/cefalea
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Transfusion reactions in thalassemia. A survey from the Cooleycare programme. The Cooleycare Cooperative Group.
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A survey on transfusion reactions in thalassemia was carried out within the COOLEYCARE Programme, a cooperative enterprise aimed at improving quantity and quality of life in thalassemia through a program of quality assurance of treatment delivered to patients. Reactions were reported in 1,225 of
Erythrocytosis in a child due to Hb Andrew-Minneapolis [β144(HC1)Lys→Asn (AAG>AAT or AAC)] associated with a Spanish (δβ)(0)-thalassemia.
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We report a rare association of δβ-thalassemia (δβ-thal) and a hemoglobin (Hb) variant with high oxygen affinity in a Spanish newborn. The proband had no Hb A and showed microcytosis and hypochromia; the peripheral blood smear was compatible with a thalassemia trait. Molecular studies revealed that
Prolonged Pyrexia: Kikuchi-Fujimoto Disease in a Patient With Hb H-Constant Spring Thalassemia
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Introduction: Haemoglobin H (Hb H) disease is an alpha thalassemia characterised by either 3 alpha-globin gene deletions (deletional type) or 2 alpha-globin gene deletions with 1-point mutation (nondeletional type). Haemoglobin H-Constant
Symptomatic Erythrocytosis Due to Homozygosity for Hb Luton [HBA2: c.269A>T (or HBA1)] and α-Thalassemia: A Clinical Update.
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A female proband homozygous for both Hb Luton [α89(FG1)His→Leu (CAC>CTC), HBA2: c.269A>T (or HBA1)], a high oxygen affinity hemoglobin (Hb), and for α(+)-thalassemia (α-thal), (-α(4.2), leftward deletion) was first described in 2012. This is a follow-up report of the same case. At the age of 18, the
Generic Direct Acting Antivirals in Treatment of Chronic Hepatitis C Infection in Patients of Thalassemia Major.
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BACKGROUND
There is no published data of treating hepatitis C in thalassemia major patients with any sofosbuvir based direct acting antivirals (DAAs). This study was performed to determine the efficacy and safety of these regimes using generic drugs in the thalassemia major population.
METHODS
In
Periodontal condition and orofacial changes in patients with thalassemia major: a clinical and radiographic overview.
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OBJECTIVE
To assess the prevalence of periodontal disease, orofacial changes and craniofacial abnormalities in patients with thalassemia major (TM). Dental management is discussed
METHODS
The sample consisted of 54 patients with TM, 31 males and 23 females aged 5.5 to 18.3 years, with the mean age
Hypoparathyroidism with extensive intracerebral calcification in patients with beta-thalassemia major.
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We report marked intracerebral calcification in eight thalassemic patients with hypoparathyroidism, followed regularly at the Haematology Research Center, Department of Pediatrics, Shiraz, Iran. Their mean age was 16.8 years (range 12-21 years). Six of the eight patients with thalassemia were
Growth hormone and adrenal response to intramuscular glucagon test and its relationship to IGF-1 production and left ventricular ejection fraction in adult B-thalassemia major patients.
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In patients with b-thalassemia major (TM), the anterior pituitary gland is particularly sensitive to free radical stresses. It has been reported that the GH deficiency (GHD) may be secondary to either pituitary or hypothalamic dysfunction. The duration of the disease, the patient's age and the
CsA-associated neurotoxicity and ineffective prophylaxis with clonazepam in patients transplanted for thalassemia major: analysis of risk factors.
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Cyclosporin A (CsA) has been shown to be useful in the prophylaxis of acute graft-versus-host-disease (GVHD). However, this immunosuppressive agent produces multiple side-effects including nephrotoxicity, hypertension, hypertricosis, gum hyperplasia, infections, and neurotoxicity. We report a
Two-year long safety and efficacy of deferasirox film-coated tablets in patients with thalassemia or lower/intermediate risk MDS: phase 3 results from a subset of patients previously treated with deferasirox in the ECLIPSE study
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Background: Despite the proven benefits of iron chelation therapy (ICT) in the management of chronic iron overload and related complications, compliance to long-term ICT is challenging. Results from the ECLIPSE study, an open-label,
Headache and migraine in children with sickle cell disease are associated with lower hemoglobin and higher pain event rates but not silent cerebral infarction.
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OBJECTIVE
To identify risk factors for headache and migraine in children with sickle cell disease and test the hypothesis that either or both are independently associated with silent cerebral infarcts.
METHODS
In this cross-sectional study, we evaluated the health history, laboratory values, and
[Neutropenia and/or thrombocytopenia due to acute parvovirus B19 infection].
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Erythema infectiosum (fifth disease) is the most common clinical presentation of acute parvovirus B19 infection in infancy. In healthy adults, most cases of infection are asymptomatic or accompanied by a flu-like syndrome like headaches and myalgia. Haematological manifestations are dominated by
[Infratentorial ischemic stroke in children: Three case reports].
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Ischemic stroke is rare in children, most of which occur in the supratentorial brain, and infratentorial infarcts are very rare. Some clinical manifestations may be similar but others such as ataxia and cranial nerve palsy are more specific. Vertebral artery dissection is the most frequent cause of
A case report of melioidosis in a diabetic patient in a union territory.
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Melioidosis is an emerging disease in India. Cases have also been reported from South East Asia, Australia and Japan. Major risk factors for melioidosis are diabetes mellitus, preexisting renal disease and thalassemia. Exposure to contaminated soil and water are also significant occupational hazards
Prevalence of intracardiac shunting in children with sickle cell disease and stroke.
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OBJECTIVE
To determine the prevalence of potential intracardiac shunts, including patent foramen ovale (PFO), in children with sickle cell disease (SCD) and stroke.
METHODS
We performed a transthoracic echocardiogram (TTE) on 40 children with SCD (39 with hemoglobin SS and 1 with sickle-beta0
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