title: Quality of life and psychological distress in patients with Peutz-Jeghers syndrome.
abstract: 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.
authors.affiliation: Department of Gastroenterology and Hepatology, Erasmus University Medical Center, Rotterdam, The Netherlands. m.g.f.vanlier@erasmusmc.nl
authors.forename: M G F
authors.initials: MG
authors.lastname: van Lier
authors.forename: E M H
authors.initials: EM
authors.lastname: Mathus-Vliegen
authors.forename: M E
authors.initials: ME
authors.lastname: van Leerdam
authors.forename: E J
authors.initials: EJ
authors.lastname: Kuipers
authors.forename: C W N
authors.initials: CW
authors.lastname: Looman
authors.forename: A
authors.initials: A
authors.lastname: Wagner
authors.forename: K
authors.initials: K
authors.lastname: Vanheusden
mesh_terms: D000091162:AMP-Activated Protein Kinase Kinases; D000223:Adaptation, Psychological; D000293:Adolescent; D000328:Adult; D000368:Aged; D000704:Analysis of Variance; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D016014:Linear Models; D008297:Male; D008875:Middle Aged; D009154:Mutation; D010580:Peutz-Jeghers Syndrome / Q000523:psychology*; D017346:Protein Serine-Threonine Kinases / Q000235:genetics; D011788:Quality of Life / Q000523:psychology*; D013315:Stress, Psychological / Q000523:psychology*; D011795:Surveys and Questionnaires; D055815:Young Adult
chemical_list: D017346:Protein Serine-Threonine Kinases; C109790:STK11 protein, human; D000091162:AMP-Activated Protein Kinase Kinases