Clinical and Radiological Evaluation and Follow-Up of Patients with Noncardiac Plastic Bronchitis.

OBJECTIVE: Plastic bronchitis (PB) is a rare disease characterized by obstruction of the airway by fibrinous mucus plugs. The etiology can be idiopathic or secondary to systematic diseases such as congenital heart diseases. Definitive diagnosis is made by pathological examination of the sputum or bronchial sample taken by bronchoscopy. In this study, the clinical status and treatment status of patients with PB were evaluated. MATERIALS AND METHODS: Medical records of the patients diagnosed as PB were reviewed ret- rospectively. Age, gender, clinical symptoms, radiology, bronchoscopic findings, and pathology results were documented. RESULTS: Six patients with PB were included in this study (female:male, 2:4). The median age of the diagnosis was 45 months. The most common symptoms are persistent wet cough and short- ness of breath. The duration of symptoms ranged from 30 to 90 days. Atelectasis was the most common radiological finding. Diagnosis was made with pathological examination of the mucus in all patients. All of the patients were treated with bronchoscopic removal of the mucus, and 4 patients required oral prednisolone therapy. Symptoms and radiological findings resolved completely in all patients. CONCLUSION: Although PB is a rare disease, it should be kept in mind in relation to patients with persistent radiological and clinical respiratory symptoms.

Clinical characteristics of patients requiring lung transplantation referral in national cystic fibrosis registry data.

BACKGROUND: We aimed to determine the number of cystic fibrosis (CF) patients recorded in the Cystic Fibrosis Registry of Türkiye (CFRT) who were in need of lung transplantation (LT) referral and examine clinical differences between patients who were LT candidates due to rapid forced expiratory volume in one second (FEV1) decline and LT candidates without rapid FEV1 decline in the last year to identify a preventable cause in patients with such rapid FEV1 decline. METHODS: All CF patients recorded in the CFRT in 2018 were evaluated in terms of LT. Patients were divided into those with FEV1 below 50% and in need of LT due to a decrease of 20% or more in the previous year (Group 1) and those who did not have FEV1 decline of more than 20% in the previous year but had other indications for LT (Group 2). Demographic and clinical features were compared between the two groups. RESULTS: Of 1488 patients registered in CFRT, 58 had a need for LT. Twenty patients were included in Group 1 and others in Group 2. Our findings did not reveal any significant variations in treatment, chronic infection status, or complications between the two groups. The average weight z-score was significantly higher in Group 1. Positive correlations were detected between weight z-score and FEV1 in 2017 in Group 1 and between FEV1 values in 2017 and 2018 in Group 2. CONCLUSIONS: There appears to be a relationship between the nutritional status and weight z-scores of CF patients and pulmonary function, which may indirectly affect the need for lung transplantation referral.

Patients with cystic fibrosis who could not receive the CFTR modulator treatment: What did they lose in 1 year?

BACKGROUND: Cystic fibrosis (CF) is an autosomal recessive disorder caused by CF transmembrane conductance regulator (CFTR) genetic variants. CFTR modulators improve pulmonary function and reduce respiratory infections in CF. This study investigated the clinical and laboratory follow-up parameters over 1 year in patients with CF who could not receive this treatment. METHODS: This retrospective cohort study included 2018 and 2019 CF patient data from the CF registry of Turkey. Demographic and clinical characteristics of 294 patients were assessed, who had modulator treatment indications in 2018 but could not reach the treatment. RESULTS: In 2019, patients younger than 18 years had significantly lower BMI z-scores than in 2018. During the 1-year follow-up, forced expiratory volumes (FEV1) and FEV1 z-scores a trend toward a decrease. In 2019, chronic Staphylococcus aureus colonization, inhaled antipseudomonal antibiotic use for more than 3 months, oral nutritional supplement requirements, and oxygen support need increased. CONCLUSIONS: Patients who had indications for modulator treatments but were unable to obtain them worsened even after a year of follow-up. This study emphasized the importance of using modulator treatments for patients with CF in our country, as well as in many countries worldwide.

Evaluation of diagnostic components and management of childhood pulmonary tuberculosis: a prospective study from Istanbul, Turkey.

INTRODUCTION: The diagnosis of childhood tuberculosis is difficult and most of the patients are diagnosed clinically. The objective of this study is to reveal the diagnostic and therapeutic components of childhood pulmonary tuberculosis and to analyze the changes that occurred in our country over the years. METHODOLOGY: All patients diagnosed with tuberculosis between 2006 and 2016 were included. Demographic characteristics, diagnostic and treatment outcomes were recorded and patients were followed up prospectively. RESULTS: A total of 492 patients were included in the study. 97% had Bacillus Calmette-Guerin vaccine, 36% were diagnosed with microbiologically-confirmed tuberculosis and 64% were diagnosed with clinically-proven tuberculosis. 94% of the patients had symptoms consistent with tuberculosis, all patients had radiologic findings, 74% had a history of tuberculosis contact and 63% had tuberculin skin test positivity. The diagnoses included primary tuberculosis in 62%, secondary tuberculosis in 21%, progressive primary tuberculosis in 13% and miliary tuberculosis in 4%. 48% of the patients received a treatment regimen containing three drugs as the initial treatment, and drug-related side effects developed in 12%. Isoniazid resistance was detected in 13% of the patients and rifampicin resistance was detected in 8%. None of the patients died due to tuberculosis. In the last 50 years in Turkey, the rates of Bacillus Calmette-Guerin vaccination and diagnosis of tuberculosis cases have increased and the mortality rates have decreased over the years. CONCLUSIONS: Our study is one of the few prospective studies and revealed the differences between the recent data and the past 50 years in childhood tuberculosis in Turkey.

The ISPAT project: Implementation of a standardized training program for caregivers of children with tracheostomy.

BACKGROUND: Tracheostomy-related morbidity and mortality mainly occur due to decannulation, misplacement, or obstruction of the tube. A standardized training can improve the skills and confidence of the caregivers in tracheostomy care (TC). OBJECTIVE: Our primary aim was to evaluate the efficiency of standardized training program on the knowledge and skills (changing-suctioning the tracheostomy tube) of the participants regarding TC. MATERIALS AND METHODS: Sixty-five caregivers of children with tracheostomy were included. First, participants were evaluated with written test about TC and participated in the practical tests. Then, they were asked to participate in a standardized training session, including theoretical and practical parts. Baseline and postintervention assessments were compared through written and practical tests conducted on the same day. RESULTS: A significant improvement was observed in the written test score after the training. The median number of correct answers of the written test including 23 questions increased 26%, from 12 to 18 (p < .001). The median number of correct steps in tracheostomy tube change (from 9 to 16 correct steps out of 16 steps, 44% increase) and suctioning the tracheostomy tube (from 9 to 17 correct steps out of 18 steps, 44% increase) also improved significantly after the training (p < .001, for both). CONCLUSION: Theoretical courses and practical hands-on-training (HOT) courses are highly effective in improving the practices in TC. A standardized training program including HOT should be implemented before discharge from the hospital. Still there is a need to assess the impact of the program on tracheostomy-related complications, morbidity, and mortality in the long term.

Serum vitamin D levels in children with recurrent tonsillopharyngitis.

OBJECTIVE: In this study, we aimed to compare vitamin D levels of children with recurrent tonsillopharingitis and healthy controls, and investigate the relationship between sociodemographic characteristics and serum vitamin D levels. METHODS: Children with recurrent tonsillopharingitis and healthy controls aged between 2, and 12 years who consulted to the outpatient clinics of Zeynep Kamil Maternity and Children's Diseases Training and Research Hospital from January to October 2012 were included in this study. Serum 25 (OH) vitamin D levels were studied by tandem mass spectroscopy (tandem ms) method. Risk factors which might be associated with vitamin D levels were questioned. Ethical aproval was obtained from the Ethics Committee of Zeynep Kamil Maternity and Children's Diseases Training and Research Hospital and informed consent from the parents of the children. RESULTS: A total of 147 children; 74 (50.3%) patients and 73 (49.7%) controls were included in our study. Age, gender and demographic characteristics did not differ significantly between the two groups. Vitamin D levels in patients with recurrent tonsillopharingitis and controls were 19.7±8.7 ng/ml and 23.6±9.2 ng/ml, respectively (p<0.01). Although duration of vitamin D usage was shorter in children with recurrent tonsillopharingitis, this difference was not statistically significant (p>0.05). CONCLUSION: Vitamin D levels in children with ≥7 recurrent episodes of tonsillophargitis within the preceeding year were significantly lower compared to the control group. We believe that serum vitamin D levels should be checked in children with recurrent tonsillopharingitis and deficiencies should be treated.