Klinefelter syndrome (KS) is the second-most prevalent chromosomal disorder in men, though late diagnosis is very common and 50-75% of men remain undiagnosed. Evidence suggests that men with KS have impaired Quality of Life (QoL) but research on how the diagnosis of KS is associated with different QoL domains and what factors influence patients’ QoL is limited. This study aimed to provide a systematic review of the published evidence on factors that influence QoL in men with KS. DESIGN: Systematic review and meta-analysis with narrative synthesis. METHODS: Medline, Cochrane, Embase, Psychinfo, CINAHL, BASE and relevant publication reference lists were searched in January 2021. Eligible studies included RCTs, cohort studies, cross-sectional studies and epidemiology studies on KS and its effect on QoL and all domains of WHOQOL-100. Clinical studies with no date restriction published in English were included. RESULTS: Thematic analysis was completed on thirteen studies, with a meta-analysis of intelligence quotient (IQ) completed on seven studies. Twelve out of 13 studies suggested that KS negatively affected QoL outcomes and KS was associated with impairments in physical, psychological, level independence and social relationship domains of WHOQOL-100. Meta-analysis suggested men with KS have significantly lower full-scale Intelligence Quotient versus controls (P <0.00001). CONCLUSIONS: This is the first evidence synthesis of QoL in men with KS. Current evidence suggests that combined physical and psychological impairments affect men with KS who also experience impairments in relationships and independence in society. Further research is needed to identify factors that influence QoL in men with KS.

☆ The Pediatric Endocrinology Nursing Society (PENS) is committed to the development and advancement o fnurses in the art and science of pediatric endocrinology nursing and to improve the care of all children with endocrine disorders through the education of the pediatric healthcare community. To aid in achieving that goal, the purpose of the PENS department is to provide up-to-date reviews of topics relevant to the PENS membership and to the general readership of the Journal of Pediatric Nursing

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Objective: To identify how Klinefelter’s Syndrome affects patients’ quality of life (QoL) and to determine which subsections of QoL are affected guided by the WHOQOL100-tool as an overarching framework for QoL. To determine the effects and impact KS has on patients’ QoL when compared to the QoL of healthy controls and general population. DESIGN - Systematic review of studies reporting QoL factors among patients with KS which included narrative synthesis and thematic analysis of 17 studies and a meta-analysis of intelligence quotient (IQ) completed in 7 studies. QoL factors were reviewed based on the parameters of the WHOQOL-100: physical health, psychological, level of independence, social relations, environment, and religion/spirituality/personal beliefs. DATA SOURCES - Medline, Cochrane, Embase, Psychinfo, CINAHL, BASE and grey search from the reference lists of key publications. Eligibility Criteria: RCT’s, Cohort studies, cross sectional studies and Epidemiology studies involving patients with KS and reporting on QoL parameters. Only human studies published in English were considered with no limits for publication date. Results: Out of all studies included (n=1266), (87.5%) had suggested KS negatively affected the outcomes measures tested, where recorded (91.1%) of studies had small/medium/large effect sizes (Cohen’s d). Narrative synthesis suggests all subgroups of QoL excluding ‘environment’ and ‘spirituality/faith/personal beliefs’ were negatively impacted for patients with KS, whilst meta-analysis showed statistical significance (P &lt;0.00001) which identified patients with KS having lower full-scale IQ compared to healthy controls. Psychological parameters were the most affected in this patient group, showing that patients with KS experienced greater social anxiety, distress during social interactions, self-esteem, self-injuries behaviours and symptoms or traits related to Autism spectrum. Conclusions: This review identifies the significant evidence supporting that QoL is reduced in patients with KS. There is a large spectrum of symptoms and no standard phenotype for KS suggesting that multiple facets of QoL are negatively impacted in these patients due to the complex nature of KS and the severity of symptoms and phenotype associated with KS. PROSPERO REGISTRATION NUMBER - CRD4202017343