Red cell life span 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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Red cell survival studies in hereditary spherocytosis.

The in vivo survival of hereditary spherocytes has been investigated with the (51)Cr red cell survival technique. Spherocytes invariably showed a shorter survival in a normal recipient than in the donor's own circulation. Moreover transfusion of spherocytes from a mother to her son, both with hereditary spherocytosis, showed that "foreign" spherocytes have a shorter survival than the "self" sph...

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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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ژورنال

عنوان ژورنال: Journal of Clinical Investigation

سال: 1968

ISSN: 0021-9738

DOI: 10.1172/jci105911