Teunissen Myra, Hijmans Channa T, Cnossen Marjon H, Bronner Madelon B, Grootenhuis Martha A, Peters Marjolein
Psychosocial Department, Emma Children's Hospital, Academic Medical Center, Amsterdam, The Netherlands.
Eur J Pediatr. 2014 Sep;173(9):1217-23. doi: 10.1007/s00431-014-2299-1. Epub 2014 Apr 16.
The objective of this study was to evaluate health-related quality of life (HRQoL) and behavioral functioning in pediatric patients with hereditary spherocytosis (HS). A cross-sectional study was conducted in 132 Dutch children and adolescents with HS and aged 8-18 years of whom 48 underwent splenectomy prior to the study. HRQoL was assessed using the KIDSCREEN-27, and behavioral functioning was evaluated using the strength and difficulties questionnaire (SDQ). Scores of pediatric patients with HS were compared to a Dutch norm population. Additionally, the effects of three factors were assessed: fatigue, self-image, and parents' perceived vulnerability (measured with the checklist individual strength, the self-perception profile for children and adolescents, and the child vulnerability scale). Both unsplenectomised and splenectomised pediatric patients reported lower HRQoL on the domain physical well-being (KIDSCREEN-27) compared to Dutch peers. For behavioral functioning, parents of both groups reported more emotional problems (SDQ) compared to the norm population. Pediatric patients with lower scores on physical well-being experienced more fatigue. The patients' perceived social acceptance and parents' perceived vulnerability appeared as determinants of emotional problems.
Pediatric patients in the current study generally report few complaints, and the results suggest that these patients overall have a strong ability to cope with HS. Despite these few complaints, fatigue and parents' perceived vulnerability seem to be important determinants for lower HRQoL and more emotional problems. Therefore, screening on these factors could serve as an addition to the treatment of HS, to help pediatric patients who are at risk for lower HRQoL or more emotional problems.
本研究的目的是评估遗传性球形红细胞增多症（HS）患儿的健康相关生活质量（HRQoL）和行为功能。对132名8至18岁的荷兰HS患儿及青少年进行了一项横断面研究，其中48名在研究前接受了脾切除术。使用儿童生活质量量表（KIDSCREEN - 27）评估HRQoL，并使用长处与困难问卷（SDQ）评估行为功能。将HS患儿的得分与荷兰正常人群进行比较。此外，评估了三个因素的影响：疲劳、自我形象和父母感知到的易损性（分别用个人优势清单、儿童及青少年自我认知量表和儿童易损性量表进行测量）。与荷兰同龄人相比，未接受脾切除术和接受脾切除术的患儿在身体健康领域（KIDSCREEN - 27）的HRQoL均较低。在行为功能方面，两组患儿的父母报告的情绪问题（SDQ）均多于正常人群。身体健康得分较低的患儿经历了更多疲劳。患儿感知到的社会接纳度和父母感知到的易损性似乎是情绪问题的决定因素。
本研究中的患儿总体上报告的不适较少，结果表明这些患儿总体上有很强的应对HS的能力。尽管不适较少，但疲劳和父母感知到的易损性似乎是HRQoL较低和情绪问题较多的重要决定因素。因此，对这些因素进行筛查可作为HS治疗的补充，以帮助有HRQoL较低或情绪问题风险的患儿。
