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hemoglobinopathies/fièvre
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Approach to management of fever and infection in patients with primary bone marrow failure and hemoglobinopathies.
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The characteristic spectrum of infections in patients with aplastic anemia, chronic neutropenic diseases, sickle cell disease, thalassemia, and other hemoglobinopathies are described. The major risk factor for infection in patients with bone marrow failure is the degree of neutropenia and
Fever in children with sickle cell hemoglobinopathies.
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To estimate the frequency of bacteremia in children with sickle cell hemoglobinopathies (SCHs) and to evaluate the usefulness of various findings in predicting septicemia, we reviewed the charts of 153 patients with SCH who made 326 emergency department visits in a one-year period. Ninety children
Preventable sudden death in children with sickle hemoglobinopathies and fever: the need for a protocolized approach.
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Emergency department visits by children with sickle hemoglobinopathies: factors associated with hospital admission.
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Children with sickle cell disease frequently present to the emergency department (ED) for evaluation of fever or management of pain. We retrospectively analyzed all ED visits by children with sickle hemoglobinopathies during 1990, excluding those for trauma. Of 146 visits by 56 children, 73 (50%)
Pregnancy with a severe hemoglobinopathy: unintended consequences of transfusions.
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We report a case of a pregnant woman with a complex hemoglobinopathy who developed a symptomatic anemia at 28 weeks of gestation and was treated with multiple transfusions of type-specific packed red blood cells. Shortly thereafter, she developed a fever and joint pains, along with laboratory values
An etiologic study of hemoglobinuria and blackwater fever in the Kivu Mountains, Zaire.
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Between January 1985 and March 1986, in the high altitude area of Kivu, Eastern Zaïre, 38 patients presenting with hemoglobinuria as main manifestation were investigated. Profound glucose-6-phosphate dehydrogenase deficiency was detected in 4 patients, leptospirosis in 2 and Hantaan virus infection
Bilateral simultaneous macular infarction with spontaneous visual recovery in genotype ss hemoglobinopathy patient.
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To report the rare and dramatic event of bilateral macular infarction in a sickle cell hemoglobinopathy (SS genotype) patient, resulting in bilateral severe reduction in visual acuity. Without any intervention, the patient's vision gradually improved over the follow-up period. Central visual field
A Study on the Efficacy of Empirical Antibiotic Therapy for Splenectomized Children with Fever
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Thalassemia represents a heterogeneous group of inherited diseases characterized by the lack or reduced production of hemoglobin β-chains. Many patients with thalassemia require splenectomy. What should be considered in the evaluation and management of candidates for splenectomy is to cover
Fat Embolism Syndrome Secondary to Bone Marrow Necrosis in Patients with Hemoglobinopathies.
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Bone marrow necrosis with subsequent embolization of the fat and necrotic tissues into the systemic circulation causing fat embolism syndrome and multiorgan failure is a rare complication of patients with hemoglobinopathies. The exact etiology of this condition is not known. Because it occurs more
DENGUE FEVER AND DENGUE HEMORRHAGIC FEVER IN ADULTS.
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Dengue fever and dengue hemorrhagic fever are re-emerging diseases that are endemic in the Tropics. The global prevalence of dengue cases has increased in South-East Asia, Africa, the Western Pacific, and the Americas. The increasingly widespread distribution and the rising incidence of dengue virus
Clinical spectrum of serious bacterial infections among splenectomized patients with hemoglobinopathies in Israel: a 37-year follow-up study.
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OBJECTIVE
Patients with hemoglobinopathies who undergo splenectomy are at risk for invasive infections. The aim of this investigation was to present the clinical spectrum of infections in splenectomized patients.
METHODS
The study cohort comprised 54 splenectomized patients with beta-thalassemia
[Blackwater fever in adults with sickle cell anemia. Two fatal cases].
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Blackwater fever is characterized by severe intravascular hemolysis with renal failure caused by recurrent use of quinine for prophylaxis. Once described in European patients, sporadic cases have been reported more and more often in autochthonous Africans and Asians. Newer antimalarials including
Bacteremia in children with sickle hemoglobinopathies.
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BACKGROUND
Bacteremia is one of the most feared infectious complications of sickle cell disease, and it is associated with a high mortality rate in children. The objective of our study was to investigate the proportion of bacteremia among febrile children with sickle hemoglobinopathies and the
[Neonatal screening for hemoglobinopathies in Rio de Janeiro, Brazil].
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OBJECTIVE
To describe the main results obtained in the first 15 months of neonatal screening for sickle cell disease in the state of Rio de Janeiro, Brazil, from August 2000 to November 2001.
METHODS
Starting in August 2000, blood samples began to be collected for sickle cell disease screening from
[Hemoglobinopathy Newcastle: use of chromatography and first case reported in Spain].
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OBJECTIVE
Unstable hemoglobins (Hb) show amino acid substitutions in critical places that produce a decrease of molecular solubility facilitating its denaturalization and precipitation. We describe the first case of Hb Newcastle in Spain.
METHODS
5 year-old girl who came to visit due to fever over 4
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