Erişkin İmmün Yetmezlik Hastalarının İşlevsellik, Yetiyitimi ve Sağlığın Uluslararası Sınıflandırılması Kapsamında Değerlendirilmesi

Abstract

Primary immune deficiency is a group of diseases that vary clinically, genetically, and immunologically due to qualitative or quantitative deficiencies in the cellular and molecular components of the immune system. The International Classification of Functioning, Disability and Health (ICF) describes individuals' health conditions and daily life barriers from a biopsychosocial perspective. The aim of our study was to evaluate the body structure and function, activity, and participation limitations of adult patients with immune deficiency (ID) within the ICF framework and to compare these limitations with those of healthy individuals. Our secondary aim was to assess the relationships between body structure function, activity, and participation limitations in adult ID patients. The study included 20 adult patients diagnosed with ID and 20 healthy individuals. The ICF's b domain (body functions) was assessed using hand grip strength, the one-minute sit-to-stand test (1STS), Pittsburgh Sleep Quality Index (PSQI), Fatigue Severity Scale (FSS), and the Short-Form 36 Questionnaire (SF-36); the s domain (body structures) was evaluated with Corbin posture analysis assessment and body composition via bioelectrical impedance analysis; the d domain (activity and participation) was assessed using the International Physical Activity Questionnaire (IPAQ) and the Lawton Instrumental Activities of Daily Living Scale (IADL); and the e domain (environmental factors) was also evaluated. The results showed that compared to healthy individuals, adult ID patients had significantly lower 1STS scores and dominant hand grip strength (p<0.001), higher dyspnea during exercise (p=0.016), higher perceptions of general and leg fatigue (p<0.001), significantly higher FSS scores (p<0.001), and similar IPAQ-total scores (p=0.901). Additionally, while Corbin posture scores were similar between adult ID patients and healthy individuals (p=0.802), body fat percentage was lower (p<0.001), and visceral fat percentage (p=0.758) and muscle mass (p=0.865) were similar. The IADL scale scores were lower in adult ID patients compared to healthy individuals (p=0.007), and the SF-36 scores for physical (p<0.001) and emotional limitation (p=0.005), physical and social function, general health (p<0.001) were significantly lower, while the total PSQI score was higher (p=0.027). There was a positive correlation between 1STS score and IADL scale score (rho=0.576). The SF-36-social function score had a negative correlation with PSQI-sleep disturbance (rho=-0.520) and FSS score (rho=-0.746), and the SF-36-physical function score had a negative correlation with FSS score (rho=-0.502)(p<0.05). In conclusion, peripheral muscle strength and exercise capacity decrease, fatigue severity increases, and daily living activities and quality of life are negatively affected in adult ID patients. We believe that the use of the ICF framework in the evaluation and treatment planning of adult patients diagnosed with X is useful for all healthcare professionals.