Hemofilik çocuklarda kas iskelet sistemi değişikliklerinin ve ilişkili faktörlerin değerlendirilmesi
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Date
2020-05-05
Authors
Demir, Büşra
Yildirim, Zuhal Keskin
Eren, Suat
Büyükavcı, Mustafa
Journal Title
Journal ISSN
Volume Title
Publisher
Bursa Uludağ Üniversitesi
Abstract
Bu çalışmada, hemofili tanısı ile takip edilen hastaların kas-iskelet sistemindeki hasarlanmayı ve bununla ilişkili olan faktörleri saptamayı amaçladık. Araştırmaya hemofili tanılı 35 hasta dahil edildi. Bunlardan hedef eklemi gelişmiş olan hastalar saptanarak fizik muayene, kanama ve radyolojik skorlamalar ile bu eklemlerin hasarlanma düzeyleri değerlendirildi. Ayrıca olguların tedavi şekilleri, kemik mineral yoğunluğu (KMY) ve serum D vitamini düzeylerinin hem hedef eklem gelişmesindeki rolü hem de eklemlerdeki hasarlanma düzeyiyle ilişkileri araştırıldı. Hastalarımızın 12’sinde (%34,2) hedef eklem gelişmiş olduğu gözlendi. Hedef eklemi olan ve olmayan gruplar arasında KMY, serum D vitamini düzeyi açısından anlamlı bir fark yoktu. Hedef eklemi olan hastaların %50’sinde serum D vitamini düzeyi normalden daha düşükken %17’sinde KMY Z-skoru -2’nin altında idi. KMY ile fizik muayene skorlaması, radyolojik skorlama ve D vitamini düzeyi arasında anlamlı bir ilişki tespit edilmedi. Aynı zamanda D vitamini düzeyi ile fizik muayene skorlaması ve radyolojik skorlama arasında da anlamlı bir ilişki yoktu. Ancak fizik muayene ve radyolojik skorlamalar birbiriyle anlamlı düzeyde ilişkiliydi. KMY ve serum D vitamini düzeyinin hastaların yaşı, hemofilinin türü ve hastalığın ağırlığından etkilenmediği tespit edildi. Hiçbir hastamızda demir eksikliği anemisi gözlemlenmedi. Hemofilik olgularımızın yaklaşık 1/3’ünde hedef eklem geliştiği gözlendi. Ayrıca bu eklemlerde farklı düzeylerde hasarlanmanın oluştuğu fiziksel ve radyolojik olarak saptandı. Hepsi ağır hemofili tanısıyla izlenen bu olgularda hedef eklem gelişmesi ve artropatinin derecesiyle KMY ve serum D vitamini düzeyinin bir ilişkisi olmadığı saptandı.
We aimed to investigate the musculoskeletal changes and related factors in children with hemophilia. Thirty-five children with hemophilia enrolled the study. Joint damage and its stage were evaluated by using physical examination, hemorrhage and radiological scoring in patients with target joints. In addition, the role and association of the treatment modalities, bone mineral density (BMD) and serum vitamin D levels of the patients in both target joint development and the stage of the damage were evaluated. Target joint was detected in 34.2% of the patients. BMD and serum vitamin D levels were similar in both groups with and without target joints. Vitamin D levels were lower than normal in 50% of the patients and BMD values were lower than normal (Z-score<-2) in 17% of the patients with target joint. No significant correlation was determined between BMD and physical examination scoring, radiological scoring or serum vitamin D levels. At the same time, there was no significant correlation between the serum vitamin D level and physical examination or radiological scoring. However, physical examination and radiological scorings were significantly correlated. BMD and vitamin D levels were not affected by patient’s age, type of hemophilia or severity of disease. No iron deficiency anemia was observed in patients. Target joint was detected in approximately 1/3 of the children with hemophilia. Different stages of the injuries were detected in these joints by physical and radiological examinations. BMD and serum vitamin D levels had no association in development of target joint and the stage of the damage in these patients with severe hemophilia.
We aimed to investigate the musculoskeletal changes and related factors in children with hemophilia. Thirty-five children with hemophilia enrolled the study. Joint damage and its stage were evaluated by using physical examination, hemorrhage and radiological scoring in patients with target joints. In addition, the role and association of the treatment modalities, bone mineral density (BMD) and serum vitamin D levels of the patients in both target joint development and the stage of the damage were evaluated. Target joint was detected in 34.2% of the patients. BMD and serum vitamin D levels were similar in both groups with and without target joints. Vitamin D levels were lower than normal in 50% of the patients and BMD values were lower than normal (Z-score<-2) in 17% of the patients with target joint. No significant correlation was determined between BMD and physical examination scoring, radiological scoring or serum vitamin D levels. At the same time, there was no significant correlation between the serum vitamin D level and physical examination or radiological scoring. However, physical examination and radiological scorings were significantly correlated. BMD and vitamin D levels were not affected by patient’s age, type of hemophilia or severity of disease. No iron deficiency anemia was observed in patients. Target joint was detected in approximately 1/3 of the children with hemophilia. Different stages of the injuries were detected in these joints by physical and radiological examinations. BMD and serum vitamin D levels had no association in development of target joint and the stage of the damage in these patients with severe hemophilia.
Description
Keywords
Hemofili, Hemophilia, Hedef eklem, Target joint, Bone mineral density, Vitamin D, Kemik mineral yoğunluğu, D vitamini
Citation
Demir, B. (2020). "Hemofilik çocuklarda kas iskelet sistemi değişikliklerinin ve ilişkili faktörlerin değerlendirilmesi". Güncel Pediatri Dergisi, 18(2),263-275.