Red Cell Disorders

@article{Hamilton2004RedCD,
  title={Red Cell Disorders},
  author={J W Hamilton and Frank G C Jones and Mary Frances Frances McMullin},
  journal={Hematology},
  year={2004},
  volume={9},
  pages={307 - 309}
}
Abstract Glucose-6-phosphate dehydrogenase (G6PD) is an enzyme of the pentose phosphate shunt pathway a major function of which is to prevent cellular oxidative damage. Deficiency in red blood cells is associated with a number of varied clinical manifestations. Chronic non-spherocytic haemolytic anaemia is uncommon but is usually characterized by chronic haemolysis, often with severe anaemia. In the past splenectomy in this condition has been thought to be of questionable benefit. We report a… 
12 Citations
Red Blood Cell Enzyme Disorders.
Recommendations regarding splenectomy in hereditary hemolytic anemias
TLDR
Recommendations for hereditary hemolytic anemias were based on expert opinion and were subsequently critically revised and modified by the Splenectomy in Rare Anemias Study Group, which includes hematologists caring for both adults and children.
A case of G6PD Utrecht associated with β‐thalassemia responding to splenectomy
TLDR
Because IVS‐Ⅱ‐654 C>T on its own does not cause CNSHA, it is believed that the clinical manifestations in this patient are essentially due to the G6PD c.1225C>T mutation.
Evaluation of the Relationship Between Hb F Levels and Nucleated Red Blood Cells with Morbidity in Non Transfusion-Dependent Thalassemia Patients
TLDR
It is shown that Hb F level and developmental stage of normoblasts does not effect the incidence of morbidities in non transfusion-dependent thalassemia patients but sex and splenectomy were effective factors in development of morbidity.
Comparison for functional aberration of G-6-PD deficiency variants with exon 10 mutations
TLDR
A functional analysis on some alpha haemoglobinopathies using a novel bioinformatic tool, Polyphen, indicates that the functional aberration in the G-6-PD deficiency variant is based on complex pathogenesis.
Frequency of Anemia and Blood Transfusion in Critically Ill Patients
TLDR
Anemia is very frequent and multifactorial in critically ill patients and blood transfusion has become an important component in the critical care units for the management of worsening anemia during ICU stay, the study has highlightened.
Cell rigidity and shape override CD47's "self"-signaling in phagocytosis by hyperactivating myosin-II.
TLDR
It is demonstrated that rigidity of a phagocytosed cell also hyperactivates myosin-II, which locally overwhelms self-signaling at a phAGocytic synapse, and physical properties of phagocytes regulate self signaling, as is relevant to erythropoiesis.
Sometimes, It's Just Black and White: Dark Urine and Pallor in a 2-Year-Old Boy.
TLDR
A 2-year-old Chinese boy was admitted to the hospital after 2 days of pallor and fatigue and 1 day of dark urine, and on examination, the patient was afebrile and tachycardic, with an oxygen saturation of 88% on room air.
Splenektomie bei Kindern und Jugendlichen mit hämatologischen Erkrankungen
TLDR
In diesem Kapitel werden zunachst die potenziellen, mit einer Splenektomie assoziierten Risiken und Komplikationen dargestellt and Empfehlungen zur Prophylaxe der Postsplenek tomie-Infektionen diskutiert.
Glucose-6-phosphate Dehydrogenase Deficiency
TLDR
The prevalence of G6PD deficiency in Korea is about 0.9%.
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TLDR
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TLDR
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TLDR
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TLDR
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TLDR
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TLDR
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TLDR
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