Serum Vitamin D in Children with Hemophilia A and Its Association with Joint Health and Quality of Life

<b>Background/Objectives</b>: 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. <b>Methods</b...

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Main Authors: Aida M. S. Salem, Takwa Mohamed AbdEltwwab, Hanan Hosni Moawad, Marwa O. Elgendy, Reham S. Al-Fakharany, Ahmed Khames, Mohamed Hussein Meabed
Format: Article
Language:English
Published: MDPI AG 2024-11-01
Series:Hematology Reports
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Online Access:https://www.mdpi.com/2038-8330/16/4/71
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author Aida M. S. Salem
Takwa Mohamed AbdEltwwab
Hanan Hosni Moawad
Marwa O. Elgendy
Reham S. Al-Fakharany
Ahmed Khames
Mohamed Hussein Meabed
author_facet Aida M. S. Salem
Takwa Mohamed AbdEltwwab
Hanan Hosni Moawad
Marwa O. Elgendy
Reham S. Al-Fakharany
Ahmed Khames
Mohamed Hussein Meabed
author_sort Aida M. S. Salem
collection DOAJ
description <b>Background/Objectives</b>: 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. <b>Methods</b>: 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. <b>Results</b>: Serum vitamin D levels were significantly lower in hemophilia A patients than in controls (<i>p</i> < 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, <i>p</i> = 0.038) and serum vitamin D level (R = −0.974, <i>p</i> < 0.001) while also positively correlated with alkaline phosphatase (R = 0.834, <i>p</i> < 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, <i>p</i> < 0.001) and negatively correlated with serum vitamin D level (R = −0.924, <i>p</i>-value lower than 0.001), alkaline phosphatase (R = 0.842, <i>p</i> < 0.001), and severity of hemophilia (R = 0.67, <i>p</i> < 0.001). <b>Conclusions</b>: 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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spelling doaj-art-01a109f7d16749a9b4c2a8918a0c39c92024-12-27T14:28:43ZengMDPI AGHematology Reports2038-83302024-11-0116474275110.3390/hematolrep16040071Serum Vitamin D in Children with Hemophilia A and Its Association with Joint Health and Quality of LifeAida M. S. Salem0Takwa Mohamed AbdEltwwab1Hanan Hosni Moawad2Marwa O. Elgendy3Reham S. Al-Fakharany4Ahmed Khames5Mohamed Hussein Meabed6Department of Pediatrics, Faculty of Medicine, Beni-Suef University, Beni-Suef 62521, EgyptDepartment of Pediatrics, General Authority for Health Insurance, Beni-Suef 62511, EgyptDepartment of Clinical and Chemical Pathology, Faculty of Medicine, Beni-Suef University, Beni-Suef 62521, EgyptDepartment of Clinical Pharmacy, Beni-Suef University Hospitals, Faculty of Medicine, Beni-Suef University, Beni-Suef 62521, EgyptDepartment of Medical Physiology, Faculty of Medicine, Beni-Suef University, Beni-Suef 62521, EgyptDepartment of Pharmaceutics and Industrial Pharmacy, College of Pharmacy, Taif University, Taif 21944, Saudi ArabiaDepartment of Pediatrics, Faculty of Medicine, Beni-Suef University, Beni-Suef 62521, Egypt<b>Background/Objectives</b>: 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. <b>Methods</b>: 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. <b>Results</b>: Serum vitamin D levels were significantly lower in hemophilia A patients than in controls (<i>p</i> < 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, <i>p</i> = 0.038) and serum vitamin D level (R = −0.974, <i>p</i> < 0.001) while also positively correlated with alkaline phosphatase (R = 0.834, <i>p</i> < 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, <i>p</i> < 0.001) and negatively correlated with serum vitamin D level (R = −0.924, <i>p</i>-value lower than 0.001), alkaline phosphatase (R = 0.842, <i>p</i> < 0.001), and severity of hemophilia (R = 0.67, <i>p</i> < 0.001). <b>Conclusions</b>: 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.https://www.mdpi.com/2038-8330/16/4/71hemophilia Avitamin DHJHSHaemo-Qol
spellingShingle Aida M. S. Salem
Takwa Mohamed AbdEltwwab
Hanan Hosni Moawad
Marwa O. Elgendy
Reham S. Al-Fakharany
Ahmed Khames
Mohamed Hussein Meabed
Serum Vitamin D in Children with Hemophilia A and Its Association with Joint Health and Quality of Life
Hematology Reports
hemophilia A
vitamin D
HJHS
Haemo-Qol
title Serum Vitamin D in Children with Hemophilia A and Its Association with Joint Health and Quality of Life
title_full Serum Vitamin D in Children with Hemophilia A and Its Association with Joint Health and Quality of Life
title_fullStr Serum Vitamin D in Children with Hemophilia A and Its Association with Joint Health and Quality of Life
title_full_unstemmed Serum Vitamin D in Children with Hemophilia A and Its Association with Joint Health and Quality of Life
title_short Serum Vitamin D in Children with Hemophilia A and Its Association with Joint Health and Quality of Life
title_sort serum vitamin d in children with hemophilia a and its association with joint health and quality of life
topic hemophilia A
vitamin D
HJHS
Haemo-Qol
url https://www.mdpi.com/2038-8330/16/4/71
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