Eldash Hanaa H, Atwa Zeze Th, Saad Mohammad A
aDepartment of Pediatrics bDepartment of Radiology, University Hospital, Fayoum, Egypt.
Blood Coagul Fibrinolysis. 2017 Jan;28(1):14-18. doi: 10.1097/MBC.0000000000000519.
The aim of this study is to investigate bone state and factors affecting it in children with hemophilia. This is a case-control study that included 37 children with hemophilia and 37 healthy controls. The patients were selected from the outpatient pediatric hematology clinic of Fayoum University Hospital, Egypt. Bone mineral density, serum vitamin D, parathormone, calcium, phosphorus, and calcium creatinine ratio levels were evaluated. Vitamin D level and bone mineral density were significantly lower in hemophiliacs than in control group (P < 0.0001). About 43.2% of cases had moderate vitamin D deficiency, whereas 35.1% had mild deficiency. Vitamin D positively correlated with bone mineral density Z-score, whereas it negatively correlated with total joint score. Positive correlation between bone mineral density and age was also found. Serum levels of urea, urinary calcium creatinine ratio, and parathormone were found to be higher in cases than in control. Also, serum calcium level was found to be lower in patients than in controls. We concluded that vitamin D deficiency is an essential cause of decreased bone mineral density in hemophilic children. Hemophilic arthropathy with consecutive immobilization plays an important role in vitamin D deficiency and decreased bone mineral density.
本研究旨在调查血友病患儿的骨骼状态及其影响因素。这是一项病例对照研究,纳入了37例血友病患儿和37名健康对照。患者选自埃及法尤姆大学医院儿科血液科门诊。评估了骨密度、血清维生素D、甲状旁腺激素、钙、磷和钙肌酐比值水平。血友病患者的维生素D水平和骨密度显著低于对照组(P<0.0001)。约43.2%的病例有中度维生素D缺乏,而35.1%有轻度缺乏。维生素D与骨密度Z评分呈正相关,而与总关节评分呈负相关。还发现骨密度与年龄呈正相关。病例组的血清尿素水平、尿钙肌酐比值和甲状旁腺激素水平高于对照组。此外,患者的血清钙水平低于对照组。我们得出结论,维生素D缺乏是血友病患儿骨密度降低的重要原因。伴有持续制动的血友病性关节病在维生素D缺乏和骨密度降低中起重要作用。