Department of Gastroenterology and Hepatology, Erasmus University Medical Center, Rotterdam, The Netherlands.
Clin Genet. 2010 Sep;78(3):219-26. doi: 10.1111/j.1399-0004.2010.01469.x.
Little is known about psychological distress and quality of life (QoL) in patients with Peutz-Jeghers syndrome (PJS), a rare hereditary disorder. We aimed to assess QoL and psychological distress in PJS patients compared to the general population, and to evaluate determinants of QoL and psychological distress in a cross-sectional study. PJS patients completed a questionnaire on QoL, psychological distress, and illness perceptions. The questionnaire was returned by 52 patients (85% response rate, 56% females, median age 44.5 years). PJS patients reported similar anxiety (p = 0.57) and depression (p = 0.61) scores as the general population. They reported a lower general health perception (p = 0.003), more limitations due to emotional problems (p = 0.045) and a lower mental well-being (p = 0.036). Strong beliefs in negative consequences of PJS on daily life, a relapsing course of the disease, strong emotional reactions to PJS, and female gender were major determinants for a lower QoL. PJS patients experience a similar level of psychological distress as the general population, but a poorer general health perception, more limitations due to emotional problems, and a poorer mental QoL. Illness perceptions and female gender were major predictors for this lower QoL. These results may help to recognize PJS patients who might benefit from psychological support.
关于患有 Peutz-Jeghers 综合征（PJS）的患者的心理困扰和生活质量（QoL）知之甚少，PJS 是一种罕见的遗传性疾病。我们旨在评估 PJS 患者与普通人群相比的 QoL 和心理困扰，并在横断面研究中评估 QoL 和心理困扰的决定因素。PJS 患者完成了一份关于 QoL、心理困扰和疾病认知的问卷。该问卷由 52 名患者（85%的回复率，56%为女性，中位年龄为 44.5 岁）返回。PJS 患者报告的焦虑（p = 0.57）和抑郁（p = 0.61）评分与普通人群相似。他们报告了较低的总体健康感知（p = 0.003），更多因情绪问题导致的活动受限（p = 0.045）和较低的心理幸福感（p = 0.036）。对 PJS 对日常生活产生负面影响的强烈信念、疾病的反复发作、对 PJS 的强烈情绪反应以及女性性别是 QoL 较低的主要决定因素。PJS 患者经历的心理困扰与普通人群相似，但总体健康感知较差，因情绪问题导致的活动受限更多，心理健康状况较差。疾病认知和女性性别是这种较低 QoL 的主要预测因素。这些结果可能有助于识别可能受益于心理支持的 PJS 患者。
