Salem Aida M S, AbdEltwwab Takwa Mohamed, Moawad Hanan Hosni, Elgendy Marwa O, Al-Fakharany Reham S, Khames Ahmed, Meabed Mohamed Hussein
Department of Pediatrics, Faculty of Medicine, Beni-Suef University, Beni-Suef 62521, Egypt.
Department of Pediatrics, General Authority for Health Insurance, Beni-Suef 62511, Egypt.
Hematol Rep. 2024 Nov 26;16(4):742-751. doi: 10.3390/hematolrep16040071.
: 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. : 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. : Serum vitamin D levels were significantly lower in hemophilia A patients than in controls ( < 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, = 0.038) and serum vitamin D level (R = -0.974, < 0.001) while also positively correlated with alkaline phosphatase (R = 0.834, < 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, < 0.001) and negatively correlated with serum vitamin D level (R = -0.924, -value lower than 0.001), alkaline phosphatase (R = 0.842, < 0.001), and severity of hemophilia (R = 0.67, < 0.001). : 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.
甲型血友病是一种由于凝血因子 VIII 缺乏而导致的 X 连锁隐性疾病。本研究旨在评估血友病儿童患者的血清维生素 D 水平及其与关节健康和生活质量的相关性。:本病例对照研究针对 90 名 18 岁以下儿童进行,分为两组:在贝尼苏韦夫大学医院的儿科血液门诊,45 名甲型血友病儿童组成研究组,45 名健康儿童组成对照组。:甲型血友病患者的血清维生素 D 水平显著低于对照组(<0.001)。研究组中血清维生素 D 水平缺乏的有 38 人(84.4%),不足的有 4 人(8.8%),充足的有 3 人(6.6%);而对照组中缺乏的有 8 人(17.7%),不足的有 16 人(35.5%),充足的有 21 人(46.6%)。血友病关节健康总分(HJHS)与血清总钙(R = -0.31,= 0.038)和血清维生素 D 水平(R = -0.974,<0.001)呈显著负相关,同时与碱性磷酸酶呈正相关(R = 0.834,<0.001)。血友病总体特异性生活质量指数(Haemo-Qol/Haem-A-QoL)与血友病关节健康总分(HJHS)呈显著正相关(R = 0.934,<0.001),与血清维生素 D 水平(R = -0.924,P 值低于 0.001)、碱性磷酸酶(R = 0.842,<0.001)以及血友病严重程度(R = 0.67,<0.001)呈负相关。:甲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 缺乏的严重程度与血友病关节健康总分(HJHS)以及血友病患者的生活质量呈正相关。