Delayed adverse vascular events after splenectomy in hereditary spherocytosis

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Red cell life span after splenectomy in hereditary spherocytosis.

Despite the persistence of spherocytosis after splenectomy in hereditary spherocytosis, it has usually been assumed that red cell life span returns completely to normal after this treatment. Diisopropyl fluorophosphate. DF(32)P, a noneluting red cell label, was given intravenously to 11 patients in five unrelated families 2-27 yr after splenectomy for typical hereditary spherocytosis. Hemoglobi...

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Management of Anesthesia During Splenectomy and Cholecystectomy in a Pregnant Woman With Hereditary Spherocytosis

Hereditary spherocytosis (HS) is a familial hemolytic disorder with intracorpuscular mechanism that characterized by the production of red blood cells with sphere-like shape prone to hemolysis and can lead to hemolytic anemia, splenomegaly, jaundice and gallstones. One of the main reasons for referring these patients to the operating room is splenectomy and cholecystectomy to treat the complica...

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Hereditary spherocytosis in children: profile and post-splenectomy outcome.

OBJECTIVE To describe profile of 82 children with hereditary spherocytosis diagnosed over a period of 27 years (1985-2011) from a single center. METHODS Retrospective analyses of case records. RESULTS The mean (SD) age at diagnosis was 6.7 (2.8) years; 7 (8.5%) were diagnosed in infancy. Pallor (100%), icterus (67%), undocumented fever (28%), splenomegaly (96%) and hepatomegaly (73%) were t...

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Growth Failure in Hereditary Spherocytosis and the Effect of Splenectomy.

OBJECTIVE To analyze growth-failure and improvement, if any, following splenectomy in children with hereditary spherocytosis. METHODS Data collection from case-records (n=82) over 27-years (1985-2011). RESULTS Prevalence of stunting was 26%; 32% were underweight. Stunted children were older in age (P=0.006) and presented late (P=0.003). Splenectomy (n=26) improved anemia (P<0.001). However,...

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Hereditary spherocytosis: Consequences of delayed diagnosis

OBJECTIVE To determine whether patients with undiagnosed hereditary spherocytosis hospitalized for transfusions might have avoided hospitalization via earlier diagnosis. STUDY DESIGN Charts of all (N = 30) patients with hereditary spherocytosis seen in pediatric hematology at West Virginia University-Charleston were reviewed. Family and transfusion history and presence of neonatal jaundice we...

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ژورنال

عنوان ژورنال: Journal of Thrombosis and Haemostasis

سال: 2008

ISSN: 1538-7933,1538-7836

DOI: 10.1111/j.1538-7836.2008.03024.x