Hereditary spherocytosis was diagnosed in five of six children in one family after a human parvovirus B19 (B19-virus) infection. The diagnosis was made on the basis of severe anaemia, demonstrable haemolysis, decreased osmotic fragility and an increased number of spherocytes. Since in the serum of the patients an increased level of IgG and IgM antibodies against B19 virus was detectable two weeks after the crisis it was concluded that the B19-virus infection caused the severe anaemia.
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Background: Clinical Hereditary Hemolytic Anemia (HAA) particularly Hereditary Spherocytosis (HS) encompasses diverse genetic disorders causing premature red blood cell destruction and intrinsic RBC defects. There's a pressing need for standardized diagnostic protocols tailored to the Asian population, particularly in Saudi Arabia, underscoring the significance of thorough blood biochemistry analysis.
Materials And Methods: A case-control prospective study was conducted at King Abdulaziz University, samples were obtained from King Fahad, hospital Jeddah, Saudi Arabia, serving a significant population, and blood samples from 27 patients meeting ethical criteria for HHA and HS.
The Prevalence of Peripheral Erythrophagocytosis in Pediatric Immune-Mediated Hemolytic Anemia.
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Background: Peripheral erythrophagocytosis appears to be a unique sign of acquired immune-mediated hemolytic anemia. It is said to be rare but its prevalence among patients with autoimmune hemolytic anemia has not been studied.
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Shape-Dependent Structural Order of Red Blood Cells.
Langmuir
January 2025
Department of Chemical Engineering, University of Michigan, Ann Arbor, Michigan 48109, United States.
In this work, we show how shape matters for the ordering of red blood cells (RBCs) at a water-air interface for both artificially rigidified and sphered cells as a model system for hereditary spherocytosis. We report enhanced long-range order for spherical RBCs over disk-shaped RBCs arising from the increased local ordering of spheres relative to disks. We show that rigidity has a greater effect on the radial distribution of spherical vs disk-shaped RBCs by slightly increasing the average distance between cells.
View Article and Find Full Text PDFMultigene Panel Testing Revealed Novel Variants in Hereditary Spherocytosis Patients in Türkiye.
Turk J Haematol
January 2025
Marmara University Faculty of Medicine, Department of Medical Genetics, İstanbul, Türkiye.
Objective: This study aims to determine the genotypic characteristics of Hereditary Spherocytosis (HS) patients in Turkiye and to examine the correlation between genotype and phenotype.
Materials And Methods: Herein we had 18 patients who were admitted to pediatric hematology outpatient clinic with hemolytic anemia, jaundice, cholelithiasis, and splenomegaly. According to the Eber's classification, the patients' clinical presentations were categorized as mild, moderate, and severe.
Evaluation of endocrine changes and insulin release in patients with hereditary spherocytosis.
J Paediatr Child Health
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Department of Physiology, Faculty of Medicine, Kahramanmaras Sutcu Imam University, Kahramanmaras, Turkey.
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