BACKGROUND: Asthma and sickle cell disease are common conditions that both may result in pulmonary complications. We hypothesized that children with sickle cell disease with concomitant asthma have an increased incidence of vaso-occlusive crises that are complicated by episodes of acute chest syndrome. METHODS: A 5-year retrospective chart analysis was performed investigating 48 children ages 3...

Patients with sickle cell disease have high morbidity and healthcare utilization due to repeated painful crises. Some coexisting conditions which cause pain similar to sickle cell disease may go undiagnosed in these patients. We report two adults with concurrent hyperparathyroidism who experienced significant improvement in sickle cell pain following parathyroidectomy thereby pointing to hyperp...

Sickle cell disease is one of the commonest single gene disorders in man and has a widespread distribution in different parts of the world with variable clinical manifestations. An important clinical issue requiring further clarification is the effect of this abnormal hemoglobin on the physical growth and development of children with sickle cell disease. Earlier reports have shown that American...

Cardiopulmonary bypass (CPB) in patients with sickle cell anemia can trigger lethal vaso-occlusive crises, especially cases of hypoxia, hypothermia, acidosis, or low-flow states. We described a patient who had bicuspid aortic valve stenosis and aneurysmal dilatation the ascending aorta complicated infective endocarditis. The history stroke. During routine workup, Hodgkin’s Lymphoma was diagnosed...

UNLABELLED Sickle cell anemia is the best known hereditary blood disorder; there are serious complications associated with the condition. Diagnosis and early intervention reduce morbidity and mortality. These benefits have resulted in the widespread use of newborn screening education programs. In Brazil, the National Neonatal Screening Program established by decree 822/01 included sickle cell d...

Sickle cell disease affects 70,000 Americans who experience an average of 0.8 painful episodes each year. The pathophysiology of sickle cell pain is not completely understood. The disease is characterized by both acute and chronic pain syndromes. Patients with sickle cell pain often encounter barriers to receiving appropriate care, including lack of continuity of care and perceived opiate addic...

Background: The sickle gene confers an increased vulnerability to infection, particularly to certain bacterial pathogens, and this has significantly contributed to morbidity and mortality in individuals with sickle cell disease. Immunization plays an important role in the prevention of infection in children with sickle cell disease; we sought to assess the pneumococcal vaccination rate of child...

OBJECTIVE Evaluate the Neonatal Screening Program of the Health Secretariat of the State of Santa Catarina for sickle-cell disease, from January 2003 to December 2012, regarding program coverage and disease frequency. METHODS Descriptive, observational, cross-sectional study with retrospective data collection. The variables analyzed were: number of live births in the State of Santa Catarina; ...

Sickel cell disease is common in urban areas of Britain and it is estimated that in London alone there are nearly 2000 patients. One hundred and eighty four patients with sickle cell disease are known to the Central Middlesex Hospital, and 155 of those attend the sickle cell clinic regularly. The commonest cause for admission to hospital is acute painful or vaso-occlusive crisis, which accounts...

BACKGROUND Reactive oxygen species have been shown to mediate inflammatory process and may be involved in lipid peroxidation. METHODS This study evaluates superoxide dismutase, glutathione peroxidase, catalase, malondialdehyde, C- reactive protein and fibrinogen in the serum of patients with sickle cell disease and their correlation with renal insufficiency. Superoxide dismutase, glutathione ...