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Cerebral vasculopathy in pediatric sickle-cell anemia.
Abstract In children with sickle-cell anemia, cerebral vasculopathy is a frequent and severe complication. It is attributed not only to erythrocyte sickling but also to multiple physiological modifications associated with sickle-cell anemia: platelet and leukocyte activation, endothelial injury and remodeling, coagulation activation, hemolysis and subsequent chronic inflammation, impaired vasomotricity, etc. Intracranial large-vessel remodeling leads to clinical cerebral infarction, whereas microvascular injury and impaired vasoreactivity lead to so-called silent infarcts, which are actually associated with impair...
Source: Archives de Pediatrie - March 12, 2014 Category: Pediatrics Authors: Kossorotoff M, Grevent D, de Montalembert M Tags: Arch Pediatr Source Type: research

Cerebral vasculopathy in children with sickle cell disease: Key issues and the latest data.
Abstract Cerebral vasculopathy is a common and severe complication of sickle cell disease in children. The pathophysiology consists of progressive damage to the basal intracranial arteries and cerebral microcirculation, while chronic anemia worsens exposure to cerebral hypoxia. It results in stroke and subclinical or poorly symptomatic ischemic lesions. Many clinical, biological, and radiological risk factors have been identified. The prevention strategy through systematic transcranial Doppler screening of large-vessel vasculopathy has revolutionized the management of this disease and has greatly decreased the ris...
Source: Archives de Pediatrie - December 15, 2017 Category: Pediatrics Authors: Corvest V, Blais S, Dahmani B, De Tersant M, Etienney AC, Maroni A, Ormières C, Roussel A, Pondarré C Tags: Arch Pediatr Source Type: research

Pediatric Sickle Cell Disease - Past Successes and Future Challenges.
Abstract Once a fatal disease of childhood, more than 95% of patients born today with sickle cell disease (SCD) in developed countries are expected to survive into adulthood, largely because of improvements in supportive and preventive care (newborn screening, penicillin prophylaxis, transcranial Doppler (TCD) screening). Hydroxyurea (HU) therapy, the only oral medication currently available to prevent SCD complications, has become more widespread over the past 20 years. The NHLBI recommends that HU be offered to all patients with HbSS beginning at nine months of age, and the recently published Abnormal TCD with T...
Source: Pediatric Research - October 4, 2016 Category: Pediatrics Authors: Meier ER, Rampersad A Tags: Pediatr Res Source Type: research