Disordered bone metabolism in hereditary spherocytosis patients

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Erythrocyte energy metabolism in hereditary spherocytosis.

The incorporation of extracellular orthophosphate-(32)P into cellular ATP, 2,3-diphosphoglyceric acid, and inorganic phosphate has been measured over a period of 6 hours in vitro in red blood cells from normal subjects and from patients with hereditary spherocytosis who had undergone splenectomy. The pattern of labeling of the intracellular compounds was found to be the same in both types of re...

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Hereditary spherocytosis.

Hereditary spherocytosis is a common inherited disorder that is characterised by anaemia, jaundice, and splenomegaly. It is reported worldwide and is the most common inherited anaemia in individuals of northern European ancestry. Clinical severity is variable with most patients having a well-compensated haemolytic anaemia. Some individuals are asymptomatic, whereas others have severe haemolytic...

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Hereditary spherocytosis.

Hereditary spherocytosis is a congenital haemolytic anaemia due to defect in spectrin-a RBC membrane protein and is transmitted as autosomal dominant. Due to this defect there is presence of characteristic spherical cell in peripheral blood smear and osmotic fragility is increased. Haemolytic anaemia, reticulocytosis, jaundice and splenomegaly are present. This article reports a case of a 9 yea...

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Hereditary Spherocytosis

A 12-year-old girl was brought to the Dhaka Hospital of ICDDR,B with diarrhoea. Incidentally, the parents provided a history of repeated episodes of pallor and jaundice since she was two and half years old. Three of her family members had similar problems. History, clinical examination, and laboratory findings of the girl and her family members suggested a case of hereditary spherocytosis. To o...

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Natural History of Hereditary Spherocytosis Among Egyptian Pediatric Patients

Objective: This study aimed to better characterize short term and long term natural history of hereditary spherocytosis (HS) in Egyptian pediatric patients. Design and Methods: A retrospective record review was performed on 40 children with HS. Demographics, age at diagnosis, presenting features, history of complications, hospitalizations and medications were reviewed. Transfusion, neonatal and...

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ژورنال

عنوان ژورنال: Hematology

سال: 2019

ISSN: 1607-8454

DOI: 10.1080/16078454.2019.1565150