Background and Aims Common variable immunodeficiency (CVID) can affect quality of life (QoL) which can be better assessed with validated scales. Our goal was to validate the Turkish version of the Italian CVID-QoL questionnaire. Methods International recommendations for cultural adaptation and translation process of original scale was followed. CVID patients completed Turkish CVID-QoL questionnaire between October 2019 and January 2020. The Short Form Health Survey (SF-36) was used as a comparative questionnaire. Reliability, reproducibility, factor analysis, content validity, convergent validity and discriminant validity were analysed. Results 50 CVID patients were included in the study. 64 % of patients (n=32), the mean age of the patients was 36.68 ± 13.2 years, the median duration of disease was 52.5 months. The instrument had good internal consistency in 50 patients [Cronbach’s alpha: 0.92, emotional functioning (EF): 0.91, relational functioning (RF): 0.77]. It also revealed high reproducibility in 26 patients QoL global, intraclass correlation coefficient (ICC)= 0.80 (95 % CI 0.56 - 0.91); EF, ICC = 0.78 (95 % CI 0.51- 0.90); RF, ICC = 0.82 (95 % CI 0.59-0.92); Gastrointestinal and skin symptoms (GSS), ICC = 0.89 (95 % CI 0.76-0.95); (p <0.001, p <0.001, p <0.001, p <0.001). QoL global, EF and RF scores showed good convergent validity with similar subscales of SF-36. The number of infections within last 3 months had a significant impact on QoL global, EF and RF (p=0.038, p=0.045, p=0.028). Conclusions The Turkish version of CVID QoL scale has appropriate validity and reliability among Turkish patients with CVID.

Background: The success of subcutaneous immunotherapy (SCIT) mostly depends on regular injections. Our aim was to investigate adherence to SCIT with aeroallergens during the COVID-19 pandemic and demonstrate clinical consequences of treatment disruptions in real-life. Methods: Visual analogue scale for quality of life (VAS-QoL), VAS for symptom scores (VAS-symptom), medication scores (MSs) and total symptom scores (TSS-6) were recorded during the pandemic in 327 adult allergic rhinitis and/or asthmatic patients receiving maintenance SCIT and these scores were compared with the pre-pandemic data. Patients were grouped according to SCIT administration intervals; no delay (Group 1), <2 months (Group 2), and ≥2 month intervals (Group 3). Results: 104 (31.8%) patients (Group 3) were considered as non-adherent which was mostly related to receiving SCIT with HDMs and using public transportation for reaching the hospital. Median MS, VAS-symptom and TSS-6 scores of Group 3 patients during the pandemic were higher than the pre-pandemic scores (p=0.005, p<0.001, p<0.001, respectively) whereas median VAS-QoL scores of Group 3 during the pandemic were lower than the pre-pandemic scores (p<0.001). Median TSS-6 and VAS-symptom scores were the highest in Group 3 compared to other groups (p<0.001 for each comparison). Median VAS-QoL scores were the lowest in Group 3 compared to Group 1 and Group 2 (p<0.001, p=0.043, respectively). Conclusion: When precautions in allergy clinics are carefully applied, adherence to SCIT can be high during a pandemic. Patients must be warned about adhering to SCIT injections since delays in SCIT administration can deteriorate clinical symptoms.

Background: Knowledge on endothelial dysfunction and its relation to atherosclerosis in mastocytosis is limited. Aim: To investigate the endothelial function in mastocytosis by flow mediated dilatation (FMD) and biomarkers related to vascular endothelia, the presence of subclinical atherosclerosis by carotid intima media thickness (CIMT). Method: Forty-nine patients with mastocytosis and 25 healthy controls (HCs) were included. FMD and CIMT during transthoracic echocardiography, biomarkers including endocan, endothelin-1 (ET-1), vascular endothelial growth factor (VEGF) were measured in sera of participants. Tumor necrosis factor-alpha (TNF-α), interleukine-6 (IL-6) and high sensitive c-reactive protein (hsCRP) were determined as inflammatory biomarkers. Result: The mean FMD% was lower in the patients than HCs (11.26±5.85% vs 17.84±5.27% p<0.001) and was the lowest in the advSM and SSM group among the patients (p=0.03). The median value of VEGF was significantly higher in patients than HCs. [73.30 pg/mL; min-max (32.46-295.29) pg/mL vs (46.64 pg/mL; min-max 11.09-99.86 pg/mL; p:0.001] and it was the highest in the advSM and SSM group (p:0.01). FMD was inversely correlated with endocan (r:-.390, p:0.006), ET-1 (r:-.363, p:0.01) and VEGF (r:-.402, p:0.004) but there were no correlations between FMD and TNF-α, IL-6, and hsCRP. No differences in CIMT values between patients and HCs and no correlation between CIMT and the biomarkers were observed. Conclusion: Endothelial dysfunction in mastocytosis becomes evident with decreased FMD and elevated serum VEGF, in the absence of atherosclerosis or systemic inflammation and is related to disease severity. Keywords: CIMT, Endocan, Endothelial function, Endothelin-1, FMD, VEGF