Servicio de Crecimiento y Desarrollo, Hospital de Pediatría S.A.M.I.C. "Prof. Dr. Juan P. Garrahan", Ciudad Autónoma de Buenos Aires, Argentina.
Servicio de Cuidados Paliativos, Hospital de Pediatría S.A.M.I.C. "Prof. Dr. Juan P. Garrahan", Ciudad Autónoma de Buenos Aires, Argentina.
Arch Argent Pediatr. 2022 Jun;120(3):180-186. doi: 10.5546/aap.2022.eng.180.
Hereditary osteochondromatosis is an uncommon, autosomal, dominant condition characterized by the presence of multiple bone growths.
To analyze factors associated with health-related quality of life (HRQoL) among children > 2 years and adults receiving follow-up at a tertiary care children's hospital in Argentina.
Cross-sectional study of a follow-up cohort. HRQoL was measured using the Pediatric Quality of Life Inventory® (PedsQL) and the Short Form Health Survey (SF- 36). Sex, age, sociodemographic characteristics, height, radiology, axis alteration and limb function, presence of pain, and malignant change were recorded. Severity was classified as per Pedrini et al. Parametric and non-parametric tests and regression analysis were done.
A total of 66 cases (47 children and 19 adults) were included. Male/female ratio: 1.7/1. Median age: 13.4 years (r: 2.21-55.3). Pain was observed in 30/47 children and in 17/19 adults. Considering the adult bone age (or epiphyseal closure) as the cutoff point to define adult status, 11/37 children and 18/27 adults had a severe disease and 2/38 children and 9/27 adults had short stature. The average value of the physical component of HRQoL in children was 65.9 (SD: 22.5) and, in adults, 27.2 (IQR: 18.5- 34.7). The presence of pain and clinical severity were significantly associated with a lower HRQoL, both in children and adults.
This study found that pain and disease severity had a negative effect on HRQoL.
遗传性骨软骨瘤病是一种少见的常染色体显性遗传病，其特征为存在多处骨生长。
分析在阿根廷一家三级儿童保健医院接受随访的 2 岁以上儿童和成人的健康相关生活质量（HRQoL）相关因素。
这是一项随访队列的横断面研究。使用儿科生活质量量表（PedsQL）和简明健康调查问卷（SF-36）测量 HRQoL。记录性别、年龄、社会人口统计学特征、身高、影像学、轴改变和肢体功能、疼痛和恶变情况。根据 Pedrini 等人的标准对严重程度进行分类。进行了参数和非参数检验以及回归分析。
共纳入 66 例（47 名儿童和 19 名成人）。男女比例：1.7/1。中位年龄：13.4 岁（范围：2.21-55.3）。30/47 名儿童和 17/19 名成人有疼痛。根据成人骨龄（或骺板闭合）作为定义成人状态的截止点，11/37 名儿童和 18/27 名成人疾病严重，2/38 名儿童和 9/27 名成人身材矮小。儿童 HRQoL 生理成分的平均得分是 65.9（SD：22.5），而成人是 27.2（IQR：18.5-34.7）。疼痛和临床严重程度与儿童和成人的 HRQoL 降低显著相关。
本研究发现疼痛和疾病严重程度对 HRQoL 有负面影响。
