血友病A患儿血清维生素D及其与关节健康和生活质量的关系

IF 1.1 Q4 HEMATOLOGY
Aida M S Salem, Takwa Mohamed AbdEltwwab, Hanan Hosni Moawad, Marwa O Elgendy, Reham S Al-Fakharany, Ahmed Khames, Mohamed Hussein Meabed
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引用次数: 0

摘要

背景/目的:血友病A是一种由凝血因子VIII缺乏引起的x连锁隐性疾病。本研究旨在评估血友病患儿血清维生素D水平及其与关节健康和生活质量的关系。方法:本病例对照研究对90例18岁以下儿童进行研究,分为两组:研究组45例A型血友病儿童和对照组45例健康儿童,分别在贝尼-苏夫大学附属医院儿科血液学门诊就诊。结果:血友病A患者血清维生素D水平明显低于对照组(p < 0.001)。研究组血清维生素D缺乏38例(84.4%)、不足4例(8.8%)、充足3例(6.6%);对照组血清维生素D缺乏8例(17.7%)、不足16例(35.5%)、充足21例(46.6%)。血友病关节健康总分(HJHS)与血清总钙(R = -0.31, p = 0.038)、血清维生素D水平(R = -0.974, p < 0.001)呈显著负相关,与碱性磷酸酶(R = 0.834, p < 0.001)呈正相关。血友病特异性生活质量指数(Haemo-Qol/ haema - qol)与血友病总关节健康评分(HJHS)呈显著正相关(R = 0.934, p < 0.001),与血清维生素D水平(R = -0.924, p < 0.001)、碱性磷酸酶(R = 0.842, p < 0.001)、血友病严重程度(R = 0.67, p < 0.001)呈显著负相关(R = 0.842, p < 0.001)。结论:A型血友病患者的维生素D水平低于健康对照组。根据Haemo-QoL,维生素D缺乏的严重程度与(HJHS)血友病和血友病患者的生活质量呈正相关。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Serum Vitamin D in Children with Hemophilia A and Its Association with Joint Health and Quality of Life.

Background/Objectives: Hemophilia A is an X-linked recessive illness produced by a deficiency of coagulation factor VIII. This study aimed to evaluate serum vitamin D in hemophilic pediatric patients and its correlation with joint health and quality of life. Methods: This case-control study was performed on ninety children under the age of 18 years old and separated into two groups: study group of 45 children with hemophilia A and control group of 45 healthy children at an outpatient pediatric hematology clinic at the Beni-Suef University hospitals. Results: Serum vitamin D levels were significantly lower in hemophilia A patients than in controls (p < 0.001). The level of serum vitamin D was deficient in 38 (84.4%), insufficient in 4 (8.8%) and sufficient in 3 (6.6%) in the study group while deficient in 8 (17.7%), insufficient in 16 (35.5%) and sufficient in 21 (46.6%) in the control group. Total hemophilia joint health score (HJHS) had a significant negative correlation with serum total calcium (R = -0.31, p = 0.038) and serum vitamin D level (R = -0.974, p < 0.001) while also positively correlated with alkaline phosphatase (R = 0.834, p < 0.001). A quality-of-life index that is specific to total hemophilia (Haemo-Qol/Haem-A-QoL) had a significant positive correlation with total hemophilia joint health score (HJHS) (R = 0.934, p < 0.001) and negatively correlated with serum vitamin D level (R = -0.924, p-value lower than 0.001), alkaline phosphatase (R = 0.842, p < 0.001), and severity of hemophilia (R = 0.67, p < 0.001). Conclusions: patients with hemophilia A had lower vitamin D levels than healthy controls. The severity of vitamin D deficiency is related positively to (HJHS) hemophilia and quality of life hemophilia cases according to Haemo-QoL.

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来源期刊
Hematology Reports
Hematology Reports HEMATOLOGY-
CiteScore
0.90
自引率
0.00%
发文量
47
审稿时长
10 weeks
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