Alexithymia in primary ciliary dyskinesia.

OBJECTIVE: Recent evidence suggests that alexithymic deficits in emotional processing may also affect physical health, and alexithymia may also be associated with organic disorders. The emotional well-being of patients with primary ciliary dyskinesia (PCD) is often negatively affected by uncertainty about the prognosis, lack of ongoing medical care, and lack of symptom control. This study aims to evaluate the frequency of alexithymia and its possible impact on the management of childhood PCD. MATERIALS AND METHODS: Subjects were recruited from patients with PCD and healthy volunteers aged 8-18 years. The questionnaire included sociodemographic characteristics and self-report scales. Data were compared between patient and control groups. RESULTS: In the >14 years of age group, the total Toronto Alexithymia Scale (TAS-20) score was significantly higher in the patients (56.60 ± 13.01) compared to the control group (46.47 ± 7.50) (p = .007). There were 6 (30) patients with a TAS-20 score ≥61. There was a significant correlation between TAS-20 and Pediatric Quality of Life (PedsQL) score (child), but no correlation between TAS-20 and Kovacs Children Depression Inventory (CDI) and PedsQL score (parents). The TAS-20 score was significantly higher in patients with bronchiectasis (p = .035), nasal polyps (p = .045), and siblings with PCD (p = .001). Furthermore, the TAS-20 score had a significant negative correlation with pulmonary function tests. CONCLUSION: Although this study is based on limited data from a single center and cannot be generalized to all PCD patient communities, our results show that PCD patients are more likely to have alexithymia compared to healthy controls and highlight the need to evaluate for alexithymia in patients with PCD. It is important for pediatric pulmonologists to have a thorough knowledge of the alexithymic features associated with PCD and to refer patients to pediatric psychiatry when necessary, especially in patients who are noncompliant with treatment protocols.

Comparison of clinical features of cystic fibrosis patients eligible but not on CFTR modulators to ineligible for CFTR modulators.

INTRODUCTION: Cystic fibrosis transmembrane conductance regulator (CFTR) modulator drugs target the underlying defect and improve CFTR function. They are a part of standard care in many countries, but not all patients are eligible for these drugs due to age and genotype. Here, we aimed to determine the characteristics of non-eligible patients for CFTR modulators in the CF registry of Turkey (CFRT) to highlight their clinical needs. METHODS: This retrospective cohort study included CF patient data from the CFRT in 2021. The decision of eligibility for the CFTR modulator was determined according to the 'Vertex treatment-Finder' on the Vertex® website. Demographic and clinical characteristics of patients were compared between eligible (group 1) and ineligible (group 2) groups for CFTR modulators. RESULTS: Among the study population (N = 1527), 873 (57.2%) were in group 1 and 654 (42.8%) were in group 2. There was no statistical difference between groups regarding sex, meconium ileus history, diagnoses via newborn screening, FEV1 z-score, CF-associated complications, organ transplant history, and death. Patients in group 2 had a higher incidence of pancreatic insufficiency (87.7% vs. 83.2%, p = .010), lower median height z-scores (-0.87 vs. -0.55, p < .001), lower median body mass index z-scores (-0.65 vs. -0.50, p < .001), longer days receiving antibiotics due to pulmonary exacerbation (0 [interquartile range, IQR: 0-2] vs. 0 [IQR: 0-7], p = 0.001), and more non-invasive ventilation support (2.6% vs. 0.9%, p = 0.008) than patients in group 1. CONCLUSION: The ineligible group had worse clinical outcomes than the eligible group. This highlights their need for life-changing drugs to improve clinical outcomes.

The epidemiologic trend of respiratory syncytial virus has returned strongly to its origin after the pandemic: Five-year data from a single center.

OBJECTIVE: Only a few studies have investigated the frequency and severity of respiratory syncytial virus (RSV) infections after the end of the pandemic regulations. This study aims to investigate the frequency and severity of RSV infections before, during, and after the pandemic in Turkey. MATERIALS AND METHODS: Patients under 18 years of age and those who tested positive for RSV between April 2018 and March 2023 were retrospectively reviewed. All patients were divided into three groups (pre-COVID-19, COVID-19, and post-COVID-19) according to admission date. Among inpatients, data were compared between the three groups to determine the impact of the pandemic on RSV epidemiology and clinical outcomes. RESULTS: A total of 9567 patients were tested for RSV, of which 1073 (11.2%) were positive and included in the study. Hospitalization occurred in 447 (41.7%) patients. Inpatients were younger than outpatients (p < .000). Among the three inpatient pandemic groups, clinical outcomes were statistically significantly worse in the post-COVID-19 group than in the other two groups. SpO2 was lower (p < .000), inhaled salbutamol requirement was higher (p < .000), length of stay was longer (p = .031), and ICU admission was higher (p = .023). CONCLUSION: Although the RSV trend changed within 2 years after the COVID-19 outbreak, it returned to its usual seasonality last year. After the repeal of all COVID-19 measures and the normal life began, the number of RSV-positive patients and RSV-related hospitalizations increased, and the clinical outcomes of RSV worsened. This may be a result of decreased herd immunity due to a change in society's attitude toward epidemic diseases.

Successful treatment of postinfectious bronchiolitis obliterans with gamma globulin in a tertiary center: 10 years of experience.

INTRODUCTION: Bronchiolitis obliterans is characterized by partial or total occlusion of the bronchioles due to inflammation and fibrosis, and the most common form is postinfectious bronchiolitis obliterans (PIBO). This study aimed to retrospectively present our intravenous immunoglobulin (IVIG) treatment experience in PIBO patients with a clinically severe course despite receiving commonly used treatment protocols. MATERIALS AND METHODS: The study included patients aged 0-18 with subtle immunological abnormalities who were followed up in our center for PIBO between 2010 and 2021. Clinical evaluation, body mass index (BMI), computerized tomography (CT) image scoring, and immunological parameters were recorded before and after IVIG treatment. RESULTS: Of the 11 patients included in the study, 90% were male, the mean age at diagnosis was 27.1 months (range: 5-68 months) and the mean current age was 81.4 months (range: 15-188 months). The number of hospital visits due to infection and the frequency of hospitalizations decreased markedly in the patients who underwent IVIG therapy. Oxygen therapy was discontinued in all patients, and improvements in radiological severity scores were observed. BMI z-scores improved over the baseline values after IVIG therapy. CONCLUSION: Corticosteroids are considered the best first-line treatment to control inflammation in PIBO. In our study group, PIBO patients showed favorable clinical and radiological responses to regular IVIG treatment, possibly due to minor immune deficiency secondary to steroids or as a result of undetected adaptive and innate immune defects involved in the etiology of severe PIBO.

Evaluation of sarcopenia as a prognostic biomarker in locally advanced head and neck squamous cell carcinoma.

Background: We aimed to evaluate the effect of sarcopenia on survival in head and neck squamous cell carcinoma patients treated with chemoradiotherapy. Materials & methods: Disease-free survival and overall survival were compared according to cervical computed tomography for radiotherapy in 123 sarcopenic and non-sarcopenic patients with locally advanced head and neck squamous cell carcinoma treated with chemoradiotherapy with weekly cisplatin. Results: In multivariate analyses, pretreatment sarcopenia was associated with lower disease-free survival (hazard ratio: 2.60; 95% CI: 1.38-4.87; p = 0.003) and overall survival (hazard ratio: 2.86; 95% CI: 1.40-5.85; p = 0.004). Sarcopenic patients experienced more frequent radiotherapy-related toxicities and platinum-related side effects than non-sarcopenic patients. Conclusion: Sarcopenia could be a potential biomarker to predict prognosis and treatment toxicity in head and neck squamous cell carcinoma.

Head and neck cancer is one of the main causes of cancer-related death worldwide. Most patients are diagnosed in the advanced stage. Muscle wasting with significant weight loss occurs in nearly half of the patients at the initial diagnosis. In oncology research, sarcopenia has often been described as the loss of skeletal muscle mass. In this study, we evaluated the effect of sarcopenia on survival in head and neck cancer patients. Muscle mass was calculated using information from head and neck computed tomography before radiotherapy treatment in patients. We showed that patients with low muscle mass had significantly worse survival rates and were more susceptible to treatment-related side effects. Sarcopenia may function as a marker showing the course of disease in patients with head and neck cancer.

The association between pan-immune-inflammation value and survival in head and neck squamous cell carcinoma.

PURPOSE: A significant portion of patients with locally advanced head and neck squamous cell carcinoma (HNSCC) relapse despite multimodality treatment denoting the need for biomarkers. The pan-immune-inflammation value (PIV) is a recently developed blood count-based prognostic biomarker. We evaluated the relationship between PIV and survival in locally advanced HNSCC patients treated with chemoradiotherapy (CRT). METHODS: A total of 199 patients who underwent CRT at Hacettepe University Oncology Hospital were included. The relationship between clinical and laboratory parameters with overall survival (OS) and disease-free survival (DFS) was analyzed by multivariate analyses. RESULTS: The median age was 59 years and 90.5% of the patients were male. 66.8% of the patients had laryngeal primaries, and 78.9% had T3-T4 disease. 84.9% of the patients received CRT with cisplatin. The optimal PIV threshold value was calculated as 404 in ROC analyses. This PIV value had 75.8% sensitivity and 70.4% specificity for OS prediction (AUC 0.781; 95% CI 0.715-0.846; p < 0.001). In multivariate analyses, high PIV levels (≤ 404 vs. > 404, HR 2.862; 95% CI 1.553-5.276; p = 0.001), higher NLR (≤ 2.5 vs. > 2.5, HR 1.827; 95% CI 1.017-3.281; p = 0.044) levels and ECOG performance score of 2 (HR 2.267; 95% CI 1.385-3.711; p = 0.001) were associated with shorter OS. These factors were associated with shorter DFS also (HR for PIV 2.485, 95% CI 1.383-4.467, p = 0.002). CONCLUSIONS: We observed shorter OS and DFS in locally advanced HNSCC patients with high PIV levels. If prospective studies support our findings, the PIV score could be a prognostic biomarker in HNSCC.

Is Asymmetric Dimethylarginine a Useful Biomarker in Children With Carbon Monoxide Poisoning?

OBJECTIVE: Carbon monoxide poisoning (COP) is the leading cause of mortality and morbidity due to poisoning worldwide. Because children are affected more quick and severely from COP, they may require a longer treatment period, even if carboxyhemoglobin (CO-Hb) and/or lactate levels return to normal. Therefore, a new marker that predicts the duration of treatment and the final outcomes of COP is needed. METHODS: This case control study was conducted on 32 carbon monoxide-poisoned patients younger than 18 years who had been admitted to pediatric emergency department. The control group included age- and sex-matched 30 healthy children. Blood samples were obtained for analysis of arterial blood gases, CO-Hb percent, methemoglobine, lactate, and asymmetric dimethylarginine (ADMA). RESULTS: Asymmetric dimethylarginine levels were significantly increased (P < 0.05) in patients with COP on admission and after the treatment when compared with controls (1.36 [0.89-6.94], 1.69 [0.76-7.81], 1.21 [0.73-3.18] nmol/L, respectively). There was no positive correlation between CO-Hb and ADMA levels on admission and at 6 hours (P = 0.903, r = 0.218, P = 0.231, r = 0.022, respectively). Positive correlation was found between lactate and CO-Hb levels on admission (P = 0.018, r = 0.423). CONCLUSIONS: This study showed that ADMA levels were still high after 6 hours of 100% oxygen therapy in children with COP, even CO-Hb and/or lactate levels return to normal range. On the basis of these results, we consider that ADMA may be a useful biomarker in patient with COP.