Aydin Köker Sultan, Kaygusuz Arife, Oymak Yeşim, Karapϊnar Tuba H, Çubukçu Duygu, Gözmen Salih, Ay Yilmaz, Vergϊn Raziye C
Departments of Pediatric Haematology.
Pediatric Psychology.
J Pediatr Hematol Oncol. 2020 Apr;42(3):e159-e163. doi: 10.1097/MPH.0000000000001664.
Hemophilia, which is a chronic illness associated with recurrent bleeding, may occur with psychosocial and behavioral problems.
The aim of this study was to evaluate the clinical characteristics and demographic features and changes in the self-image of adolescents with hemophilia.
Data about hemophilia type, the severity of hemophilia, secondary prophylaxis received, and annual bleeding rate (ABR) were recorded from patient files. Hemophilia Joint Health Score (HJHS) and the Offer Self-Image Questionnaire (OSIQ) (as a measure of self-esteem) were applied to hemophilia patients and a healthy control group.
Thirty-two hemophilia patients (mean age=16.2±3.06 y) and 35 healthy male individuals (mean age=16.02±1.4 y) were enrolled in the study. Hemophilia patients had lower total OSIQ score than their peers (P=0.007). There was no difference between patients who received and who did not receive secondary prophylaxis (P=0.408) in terms of total OSIQ score. The median total OSIQ score of patients with pathologic HJHS (>0 points) was lower than that of patients with normal HJHS (0 points) (P=0.010). The median of ABR was 6 (range: 0 to 20) in the whole hemophilia group. There were no differences between hemophilia patients with ABR≤4 and >4 (P=0.084). All of the subscale parameters of the OSIQ were lower for hemophilia patients compared with their peers, besides one. The subscale of sexuality attitudes was better for hemophilia patients than for the healthy control group (P=0.028).
Low self-esteem in hemophilia patients indicates the importance of lifelong psychosocial support. Patients with pathologic HJHS are at risk of low-esteem. Using OSIQ with HJHS during follow-up of hemophilia patients may be useful for management.
血友病是一种与反复出血相关的慢性疾病,可能伴有心理社会和行为问题。
本研究旨在评估血友病青少年的临床特征、人口统计学特征以及自我形象的变化。
从患者病历中记录血友病类型、血友病严重程度、接受的二级预防措施以及年出血率(ABR)。对血友病患者和健康对照组应用血友病关节健康评分(HJHS)和奥弗自我形象问卷(OSIQ)(作为自尊的衡量指标)。
32名血友病患者(平均年龄 = 16.2±3.06岁)和35名健康男性个体(平均年龄 = 16.02±1.4岁)纳入本研究。血友病患者的OSIQ总分低于同龄人(P = 0.007)。在OSIQ总分方面,接受和未接受二级预防措施的患者之间无差异(P = 0.408)。HJHS病理性(>0分)患者的OSIQ总分中位数低于HJHS正常(0分)患者(P = 0.010)。整个血友病组的ABR中位数为6(范围:0至20)。ABR≤4和>4的血友病患者之间无差异(P = 0.084)。除一项外,血友病患者的OSIQ所有子量表参数均低于同龄人。血友病患者的性态度子量表得分优于健康对照组(P = 0.028)。
血友病患者的低自尊表明终身心理社会支持的重要性。HJHS病理性患者存在自尊低下的风险。在血友病患者随访期间将OSIQ与HJHS结合使用可能有助于管理。