عوز خميرة G6PD عند مرضى الداء المنجلي
Abstract
تم دراسة التداخل ما بين عوز خميرة نازعة هيدروجين الغلوكوز6-فوسفات و فقر الدم المنجلي. حيث تم إجراء معايرة كمية لفعالية خميرة G6PD عند50 مريضاً، مصاباً بالداء المنجلي و مقارنتها بعينة شاهد من
50 مريضاً ثم مقارنة التظاهرات السريرية و القيم المخبرية في مرضى الداء المنجلي الذين وجد لديهم عوز
في G6PD بأولئك المرضى دون عوز G6PD، وأشارت النتائج أن شيوع عوز G6PD في مجموعة المرضى المصابين بالداء المنجلي(20%) لا يختلف حقيقةً عن شيوعه في مجموعة الشاهد(16%), وكانت القيم الوسطية للخضاب:7.33 غ/دل وMCV: 78.2فمتولتر و الشبكيات:7.14% والبليروبين الكلي:2.28مغ/دل عند المرضى المنجليين مع عوزG6PD مماثلة عملياً لما هي عليه عند المرضى المنجليين دون عوز G6PD (الخضاب:7.7غ/دل ,:MCV80.5 فمتولتر ,الشبكيات:7.05% ,البيليروبين الكلي:2.48مغ/دل )، وكانت فعالية خميرةAST مرتفعة عند45% من المرضى المنجليين دون عوزG6PD وعند60%من المرضى مع عوز، كما لم يختلف تواتر النوب الانحلالية و الألمية في كلتا المجموعتين .
We studied the interaction of glucose 6-phosphate dehydrogenase deficiency and sickle cell anemia (HbSS). A total of 50 patients with HbSS had a quantitative assay of G6PD activity and were compared with a control group of 50 patients. We then compared clinical features and laboratory values in Hbss patients with G6PD deficiency with those without G6PD deficiency. We found that the prevalence of G6PD deficiency among HbSS patients (20%) does not really differ from its prevalence among control group (16%): and the average values of hemoglobin:7.33g/dl, MCV:78.2fl, reticulocyte count:7.14%, TB:2.28 mg/dl in HbSS patients with G6PD deficiency were close to those in HbSS patients without G6PD deficiency(Hb:7.7 g/dl, MCV:80.5 fl, ret:7.05%, TB: 2.48mg/dl). The AST activity was elevated in 45% of patients without G6PD deficiency and in 60% of patients with G6PD deficiency. The prevalence of hemolytic and panic crisis was similar in both groups.
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