Comparison of refugee patients with cystic fibrosis and their counterpart children from Turkey during the war.

Since the outbreak of the Syrian civil war in 2011, the population of Arab refugees in Turkey has rapidly increased. While cystic fibrosis (CF) is believed to be rare among Arabs, recent studies suggest it is underdiagnosed. This study aims to present the demographic, clinical, and genetic characteristics of CF patients among Arab refugees in Turkey. Additionally, a comparison is made between the findings in the National CF Registry 2021 in Turkey (NCFRT) and the refugee CF patient group. The study included refugee patients between the ages of 0 and 18 years who were diagnosed with CF and received ongoing care at pediatric pulmonology centers from March 2011 to March 2021. The study examined demographic information, age at diagnosis, age of diagnosis of patients through CF newborn screening (NBS), presenting symptoms, CF transmembrane conductance regulator (CFTR) mutation test results, sputum culture results, weight, height, and body mass index (BMI) z score. Their results were compared with the NCFRT results. The study included 14 pediatric pulmonology centers and 87 patients, consisting of 46 (52.9%) boys and 41 (47.1%) girls. All of the patients were Arab refugees, with 80 (92%) being Syrian. All the patients were diagnosed in Turkey. The median age at diagnosis of patients was 22.33 (interquartile range, 1-258) months. The median age of diagnosis of patients through NBS was 4.2 (interquartile range, 1-12) months. The median age of older patients, who were unable to be included in the NBS program, was 32.3 (interquartile range, 3-258) months. Parental consanguinity was observed in 52 (59.7%) patients. The mutation that was most frequently found was F508del, which accounted for 22.2% of the cases. It was present in 20 patients, constituting 32 out of the total 144 alleles. There was a large number of genetic variations. CFTR genotyping could not be conducted for 12 patients. These patients had high sweat tests, and their genetic mutations could not be determined due to a lack of data. Compared to NCFRT, refugee patients were diagnosed later, and long-term follow-up of refugee CF patients had significantly worse nutritional status and pseudomonas colonization.  Conclusion: Although refugee CF patients have equal access to NBS programs and CF medications as well as Turkish patients, the median age at diagnosis of patients, the median age of diagnosis of patients through NBS, their nutritional status, and Pseudomonas colonization were significantly worse than Turkish patients, which may be related to the difficulties of living in another country and poor living conditions. The high genetic heterogeneity and rare mutations detected in the refugee patient group compared to Turkish patients. Well-programmed NBS programs, thorough genetic studies, and the enhancement of living conditions for refugee patients in the countries they relocate to can have several advantages such as early detection and improved prognosis. What is Known: • Children who have chronic diseases are the group that is most affected by wars. • The outcome gets better with early diagnosis and treatment in patients with Cystic Fibrosis (CF). What is New: • Through the implementation of a newborn screening program, which has never been done in Syria previously, refugee patients, the majority of whom are Syrians were diagnosed with cystic fibrosis within a duration of 4 months. • Despite equal access to the newborn screening program and CF medications for both Turkish patients and refugee patients, the challenges of living in a foreign country have an impact on refugees.

Childhood interstitial lung disease in Turkey: first data from the national registry.

The childhood interstitial lung diseases (chILD) Turkey registry (chILD-TR) was established in November 2021 to increase awareness of disease, and in collaboration with the centers to improve the diagnostic and treatment standards. Here, the first results of the chILD registry system were presented. In this prospective cohort study, data were collected using a data-entry software system. The demographic characteristics, clinical, laboratory, radiologic findings, diagnoses, and treatment characteristics of the patients were evaluated. Clinical characteristics were compared between two main chILD groups ((A) diffuse parenchymal lung diseases (DPLD) disorders manifesting primarily in infancy [group1] and (B) DPLD disorders occurring at all ages [group 2]). There were 416 patients registered from 19 centers. Forty-six patients were excluded due to missing information. The median age of diagnosis of the patients was 6.05 (1.3-11.6) years. Across the study population (n = 370), 81 (21.8%) were in group 1, and 289 (78.1%) were in group 2. The median weight z-score was significantly lower in group 1 (- 2.0 [- 3.36 to - 0.81]) than in group 2 (- 0.80 [- 1.7 to 0.20]) (p < 0.001). When we compared the groups according to chest CT findings, ground-glass opacities were significantly more common in group 1, and nodular opacities, bronchiectasis, mosaic perfusion, and mediastinal lymphadenopathy were significantly more common in group 2. Out of the overall study population, 67.8% were undergoing some form of treatment. The use of oral steroids was significantly higher in group 2 than in group 1 (40.6% vs. 23.3%, respectively; p = 0.040).   Conclusion: This study showed that national registry allowed to obtain information about the frequency, types, and treatment methods of chILD in Turkey and helped to see the difficulties in the diagnosis and management of these patients. What is Known: • Childhood interstitial lung diseases comprise many diverse entities which are challenging to diagnose and manage. What is New: • This study showed that national registry allowed to obtain information about the frequency, types and treatment methods of chILD in Turkey and helped to see the difficulties in the diagnosis and management of these patients. Also, our findings reveal that nutrition should be considered in all patients with chILD, especially in A-DPLD disorders manifesting primarily in infancy.

Therapeutic insights: Use of ceftazidime-avibactam in pediatric patients.

BACKGROUND: The increasing worldwide prevalence of multidrug-resistant (MDR) bacteria underscores the pressing demand for innovative therapeutic solutions. Ceftazidime-avibactam (CAZ-AVI) represents a promising new drug combination that has received approval for specific infection types. However, there is limited information regarding its application in pediatric patients. METHODS: This study investigates the effectiveness and adverse reactions associated with CAZ-AVI treatment in pediatric patients with life-threatening infections caused by MDR pathogens. The study was conducted at a tertiary children's hospital between December, 2021 and July, 2023. RESULTS: A total of 21 patients with life-threatening infections caused by MDR pathogens were enrolled in the study. All patients had underlying medical conditions: 10 had cerebral palsy, four had congenital neurometabolic disease, two had Nieman-Pick disease, two had cystic fibrosis, two had primary immunodeficiency, and one had leukemia. Among these, 12 patients had tracheostomies. Eight patients received CAZ- AVI monotherapy, and 13 patients received combination therapy. Microbiological eradication was achieved in 18 patients (85.7%), and a clinical response was observed in 20 patients (95.2%). Two patients (9.5%) experienced relapse with the same bacteria. One patient developed anaphylaxis, and one patient had elevated creatine phosphokinase levels that normalized following discontinuation of treatment. One patient died during the study period due to gastrointestinal bleeding. CONCLUSIONS: Ceftazidime-avibactam may be a promising new drug option for the treatment of life-threatening infections caused by MDR Gram-negative microorganisms in pediatric patients. However, further studies with larger case series are needed to further evaluate the efficacy and safety of CAZ-AVI in this population.

Evaluation of specificity and sensitivity of IRT/IRT protocol in the cystic fibrosis newborn screening program: 6-year experience of three tertiary centers.

We aimed to evaluate cutoff values of immunoreactive trypsinogen (IRT)/IRT and determine relationship between IRT values and clinical characteristics of children with cystic fibrosis (CF). This study is cross-sectional study. Data of children with positive newborn screening (NBS) between 2015 and 2021 were evaluated in three pediatric pulmonology centers. Age at admission, sex, gestational age, presence of history of meconium ileus, parental consanguinity, sibling with CF, and doll-like face appearance, first and second IRT values, sweat chloride test, fecal elastase, fecal fat, biochemistry results, and age at CF diagnosis were recorded. Sensitivity and specificity of IRT cutoff values were evaluated. Of 815 children with positive NBS, 58 (7.1%) children were diagnosed with CF. Median values of first and second IRT were 157.2 (103.7-247.6) and 113.0 (84.0-201.5) µg/L. IRT values used in current protocol, sensitivity was determined as 96.6%, specificity as 17.2% for first IRT, and 96.6% sensitivity, 20.5% specificity for second IRT. Positive predictive value (PPV) was determined as 7.1%. When cutoff value for first IRT was estimated as 116.7 µg/L, sensitivity was 69.0% and specificity was 69.6%, and when cutoff value was set to 88.7 µg/L for second IRT, sensitivity was 69.0% and specificity was 69.0%. Area under curve was 0.757 for first and 0.763 for second IRT (p < 0.001, p < 0.001, respectively). PPV was calculated as 4.3%.    Conclusion: Although sensitivity of CF NBS is high in our country, its PPV is significantly lower than expected from CF NBS programs. False-positive NBS results could have been overcome by revising NBS strategy. What is Known: • Although immunoreactive trypsinogen elevation is a sensitive test used in cystic fibrosis newborn screening, its specificity is low. • In countries although different algorithms are used, all strategies begin with the measurement of immunoreactive trypsinogen in dried blood spots. What is New: • In our study, it was shown that use of the IRT/IRT protocol for cystic fibrosis newborn screening is not sufficient for the cut-off values determined by the high number of patients. • Newborn screening strategy should be reviewed to reduce false positive newborn screening results.

The associations between the clinical course of children with tracheostomy and their mothers' depression, burnout, burden, and self-esteem.

AIMS AND OBJECTIVES: The aim of this study was to evaluate the clinical features of children with tracheostomy and the predictors of psychological status of their primary caregivers in order to determine the associations between the children's clinical course with their caregivers' psychological status. BACKGROUND: The caregivers of children with tracheostomy are responsible for providing basic tracheostomy care at home. All these responsibilities may be associated with significant changes in family members' lifestyles, daily routines and family dynamics. DESIGN: This study is a cross sectional study. METHODS: Data of the family's socioeconomic status and clinical status of children with tracheostomy were noted in four paediatric pulmonology centers. The Beck Depression Inventory, Maslach Burnout Inventory, Zarit Caregiver Burden Scale, and Rosenberg Self-esteem Scale were used for psychological measurements of the caregivers. The STROBE checklist was used for this study. RESULTS: Eighty-five children and their primary caregivers were enrolled in the study. The children's median age was 4.1 years. Thirty-eight of them were dependent on home ventilators. Twenty-one had bacterial colonisation. All children's primary caregivers were their mothers. Beck Depression Inventory scores of mothers of children with colonisation were higher. Number of hospitalizations in previous 6 months was related to mothers' emotional exhaustion and depersonalization scores. Duration of children's hospitalizations in previous 6 months was positively correlated to mothers' emotional exhaustion, depersonalization, and Beck Depression Inventory scores. CONCLUSIONS: Mothers of children with tracheostomy may experience psychological conditions, such as high levels of depression, burnout, burden and low levels of self-esteem. Frequent and long-term hospitalizations of children correlated with mother's depression and burnout. Therefore, primary caregivers should be evaluated and supported psychologically. RELEVANCE TO CLINICAL PRACTICE: Preventing mothers of children with tracheostomy from experiencing psychological conditions such as depression, burnout, burden and low self-esteem can also increase the quality of care for children.

Caspase-1 and interleukin-18 in children with post infectious bronchiolitis obliterans: a case-control study.

The exact immunological mechanisms of post infectious bronchiolitis obliterans (PIBO) in childhood are not fully known. It has been shown that the inflammasome and IL-18 pathway play important roles in the pathogenesis of lung fibrosis. We aimed to investigate the role of caspase-1, IL-18, and IL-18 components in PIBO. From January to May 2020, children with PIBO, children with history of influenza infection without PIBO, and healthy children were asked to participate in the study in three pediatric pulmonology centers. Serum caspase-1, IL-18, IL-18BP, IL-18R, and INF-γ levels were measured by ELISA and compared between the 3 groups. There were 21 children in the PIBO group, 16 children in the influenza group, and 39 children in the healthy control group. No differences in terms of age and gender between the 3 groups were found. IL-18 and IL-18BP levels were higher in the healthy control group (p = 0.018, p = 0.005, respectively). IL-18R was higher in the PIBO group (p = 0.001) and caspase-1 was higher in the PIBO and influenza group than the healthy control group (p = 0.002). IFN-γ levels did not differ between the 3 groups. IL-18BP/IL-18 was higher in the influenza group than the PIBO group and the healthy control group (p = 0.003). CONCLUSIONS: Caspase-1 level was increased in patients with PIBO which suggests that inflammasome activation may have a role in fibrosis; however, IL-18 level was found to be low. Mediators other than IL-18 may be involved in the inflammatory pathway in PIBO. Further immunological studies investigating inflammasome pathway are needed for PIBO with chronic inflammation. WHAT IS KNOWN: • Post infectious bronchiolitis obliterans (PIBO) is a rare, severe chronic lung disease during childhood which is associated with inflammation and fibrosis which lead to partial or complete luminal obstruction especially in small airways. • The exact immunological mechanisms of PIBO in childhood are not fully known. WHAT IS NEW: • Inflammasome activation persists even years after acute infection and may play a role in fibrosis in PIBO. • Mediators other than IL-18 may be involved in these inflammatory pathway.

Clinical findings of patients with cystic fibrosis according to newborn screening results.

BACKGROUND: Cystic fibrosis (CF) is a lethal recessive genetic disease caused by loss of function associated with mutations in the CF trans-membrane conductance regulator. It is highly prevalent (approximately 1 in 3,500) in Caucasians. The aim of this study was to compare demographic and clinical features, diagnostic tests, treatments, and complications of patients with CF whose newborn screening (NBS) with twice-repeated immune reactive trypsinogen testing was positive, normal, and not performed. METHODS: In this study, 359 of all 1,488 CF patients recorded in the CF Registry of Turkey in 2018, who had been born through the process of NBS, were evaluated. Demographic and clinical features were compared in patients diagnosed with positive NBS (Group 1), normal (Group 2), or without NBS (Group 3). RESULTS: In Group 1, there were 299 patients, in Group 2, there were 40 patients, and in Group 3, there were 20 patients. Among all patients, the median age at diagnosis was 0.17 years. The median age at diagnosis was higher in Groups 2 and 3 than in Group 1 (P = 0.001). Fecal elastase results were higher in Group 2 (P = 0.033). The weight z-score was lower and chronic Staphylococcus aureus infection was more common in Group 3 (P = 0.017, P = 0.004, respectively). CONCLUSIONS: Frequency of growth retardation and chronic S. aureus infection can be reduced with an early diagnosis using NBS. In the presence of clinical suspicion in patients with normal NBS, further analyses such as genetic testing should be performed, especially to prevent missing patients with severe mutations.

Assessment of smoking in adolescents - a sample from vocational education centre.

OBJECTIVE: The aim of this study was to analyse the knowledge and attitudes about smoking in young people between 16-20 years of age, who were both working and attending the Vocational Education Centre. METHODS: This study was conducted with high school students at the Vocational Education Centre. The socio-demographic questionnaire and the Fagerström Test of Nicotine Dependence (FTND) were applied to the students using a face-to-face interview technique. Secondly, interactive educations on smoking-free life and smoking-related diseases were given. At last, CO (carbon monoxide) levels and respiratory functions were evaluated. RESULTS: Of the students 92.9% were males, 37.4% were 16 years, 35.8% were 17 years and 26.9% were 18-20 years old. Among 46.9% of smokers, 75.8% started smoking before 15 years of age, 86.1% were living with smokers. While 70.5% of smokers smoked more than 10 cigarettes daily, 61.3% smoked their first cigarette in the first half hour after waking up. Of the smokers, 28.8% were highly dependent, 13.0% were very highly dependent. CO levels were significantly higher in those who smoked in the workplace, who smoked mostly in the morning time, and those who started smoking at 7 years of age and younger (p < 0.05). FEV1, FVC, FEF25-75 values of morning smokers were significantly lower (p < 0.05). CONCLUSION: Unfortunately, the first smoking age was very low, the first cigarette of the day was lit in a short time after waking up, and family/friend's attitudes and behaviours were encouraging in this age group. More attention should be paid to this issue and especially new preventive projects should be implemented to protect young people from smoking.

Passive Smoking and Disease Severity in Childhood Pneumonia Under 5 Years of Age.

OBJECTIVE: To objectively investigate the effect of passive smoking on pneumonia and disease severity in children aged less than 5 years by using cotinine as an indicator of passive smoking. METHODS: Between December 2015 and April 2016, children aged less than 5 years with pneumonia and age-matched healthy controls were included in this study, which was conducted at three tertiary pediatric pulmonology centers. A questionnaire was given to the parents regarding demographic data and smoking status at home. Urinary cotinine/creatinine ratio (CCR) was measured. The data from the pneumonia and control groups, as well as children with mild and severe pneumonia within the pneumonia group, were compared. RESULTS: A total of 227 subjects were included in the study; there were 74 children in the pneumonia group and 153 in the control group. The mean age of all the children was 33.4 ± 1.28 months. Of all subjects, 140 were male and 102 were exposed to passive smoking by their parents at home. There were statistically significant differences in age, number of people in the home, and mother's and father's age between the control and pneumonia groups (p < 0.05). No difference was found in the CCR in the control and pneumonia group (p > 0.05). Age and urinary CCR were significantly different between children with mild and severe pneumonia (p < 0.05). CONCLUSION: We showed that passive smoking exposure was associated with the development of severe pneumonia in children. Further studies are needed to examine the underlying cause in detail.

Early severe anemia as the first sign of cystic fibrosis.

UNLABELLED: Severe anemia is reported to occur rarely in patients with cystic fibrosis (CF). This study aimed to determine the factors associated with early severe anemia in infants with CF. This study included 231 infants with CF from 3 pediatric CF centers ten year period that were retrospectively reviewed in terms of severe anemia as the first sign of CF. Factors that could affect anemia, such as age, pancreatic insufficiency, mutations, vitamin A and E, and albumin level were evaluated. Clinical and laboratory findings in CF patients that presented with severe anemia and no respiratory symptoms were compared to those in CF patients that did not present with severe anemia. Severe anemia as the first sign of CF was noted in 17 of 231 patients. Patient age, prolonged PT/INR and the albumin level differed significantly between the 2 groups of patients (P < 0.001). Feeding pattern, pancreatic insufficiency, vitamin E and A levels, and the types of genetic mutations did not differ between the 2 groups. The mean hemoglobin level was 5.59 ± 0.21 g/dL and respiratory symptoms began a mean 6.3 months after diagnosis of CF in the anemia group. CONCLUSION: In early infancy severe anemia in the absence of respiratory symptoms can be the first sign of CF. CF should be considered in the differential diagnosis of severe anemia in infants. Anemia can occur several months before respiratory symptoms in patients with CF and may be caused due to several reasons. WHAT IS KNOWN: • Severe anemia as a first sign is reported to occur rarely in patients with cystic fibrosis. • Although anemia is well known in cystic fibrosis, factors that cause severe anemia are not known clearly. What is New: • This study shows the importance of severe anemia as the first sign of cystic fibrosis. • Anemia can occur several months before respiratory symptoms in patients with CF.

8-year old patient with giant hydatid cyst consulted with chest wall asymmetry.

Cystic echinococcosis, a zoonotic disease, is common in Turkey and is caused by the Echinococcus granulosus tapeworm. We describe the case of an 8-year-old girl who was admitted to hospital with chest wall asymmetry after a fall from height, without any other symptoms. Cystic lesion was identified on radiology, and serology supported the diagnosis of hydatid cyst. The patient underwent thorax surgery. Postoperatively, she was treated with benzimidazoles for 2 months. Rarely, hydatid cysts can reach a size sufficiently large to cause chest deformity.

Total antioxidant and total oxidant states, and serum paraoxonase-1 in neonatal sepsis.

BACKGROUND: Paraoxonase-1 (PON-1) is an enzyme with a glycoprotein structure that depends on calcium and which is located in serum high-density lipoprotein (HDL). The aim of this study was to evaluate PON-1, and oxidant/antioxidant state, before and after treatment for neonatal sepsis, and to determine the usability of PON-1 in neonatal sepsis treatment. METHODS: A total of 35 neonatal sepsis patients and 35 healthy controls were included in the study. Activity of PON-1, total oxidant state (TOS) and total antioxidant state (TAS) were measured and oxidative stress index (OSI) was calculated. RESULTS: In the neonatal sepsis patients, pre-treatment TAS, TOS and OSI were significantly higher than the post-treatment levels (P < 0.0001, P < 0.0001 and P < 0.0001, respectively), and PON-1 was significantly lower (P < 0.0001). Similarly, pre-treatment TAS, TOS and OSI in the sepsis group were also significantly higher than in the control group (P < 0.0001, P < 0.0001 and P < 0.0001, respectively) and PON-1 was significantly lower (P < 0.0001). Post-treatment TAS in the sepsis group was significantly higher than in the control group (P = 0.009), whereas post-treatment TOS, OSI and PON-1 in the sepsis group were not significantly different to the control group (P = 0.078, P = 0.597 and P = 0.086, respectively). CONCLUSION: Low serum PON-1 was found in neonatal sepsis. Serum PON-1 is thought to be a useful biomarker to evaluate the effectiveness of treatment and recovery in neonatal sepsis.

A sample of paediatric residents' loneliness-anxiety-depression-burnout and job satisfaction with probable affecting factors.

OBJECTIVE: To assess levels of anxiety, depression, loneliness, burnout and job satisfaction among paediatric Residents, and how they influence each other. METHODS: The cross-sectional study was conducted at Necmettin Erbakan University, Meram, and Konya Meram Education and Research Hospital, Turkey from January to June 2011, and comprised paediatric Residents and their counterparts from other departments who formed the control group. While maintaining confidentiality, a questionnaire was used to collect data that had elements of the University of California, Los Angeles, Loneliness Scale, Hospital Anxiety and Depression Scale, Maslach Burnout Inventory and Minnesota Job Satisfaction Questionnaire. SPSS version 13 was used for statistical analysis. RESULTS: Overall there were 74 Residents in the study; 43 (58%) working with the paediatrics department, and 31 (42%) in the control group. Overall mean age was 27.60 ± 2.25 years. Residents who were not satisfied with the city they were living in, with their professional career and who would not choose the same career given a second chance were feeling more lonely and had higher loneliness scores (p < 0.05). In contrast, anxiety among female Residents who were unsatisfied with their professional career and working conditions was significantly high (p < 0.05). Positive correlation was detected between the burnout levels of Residents and their anxiety, depression and loneliness scores (r = 0.74; r = 0.65; r = 0.36). In terms of intrinsic, extrinsic and total job satisfaction, there was an obvious negative correlation (r = -0.57; r = -0.54; r = -0.61). CONCLUSION: Working conditions and professional liability were the main factors affecting the Residents. Informed decision and career willingness may help them feel better.

Spontaneous pneumomediastinum as a complication in human bocavirus infection.

The most common causes of spontaneous pneumomediastinum (SPM) in children are asthma attack and respiratory tract infection. Here, we describe a case of SPM in a human bocavirus-infected 2-year-old boy with bronchiolitis.

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.

Factors associated with pulmonary function decline of patients in the cystic fibrosis registry of Turkey: A retrospective cohort study.

BACKGROUND: The decline in pulmonary function is a predictor of disease progression in patients with cystic fibrosis (CF). This study aimed to determine the decline rate of percent predicted forced expiratory volume in 1 s (ppFEV1) based on the data of the CF Registry of Turkey. The secondary aim was to investigate the risk factors related to the decline in ppFEV1. METHODS: A retrospective cohort study of CF patients over 6 years old, with pulmonary function data over at least 2 years of follow-up was extracted from the national CF registry for years 2017-2019. Patients were classified according to disease severity and age groups. Multivariate analysis was used to predict the decline in ppFEV1 and to investigate the associated risk factors. RESULTS: A total of 1722 pulmonary function test results were available from 574 patients over the study period. Mean diagnostic age was older and weight for age, height for age, and body mass index z scores were significantly lower in the group of ppFEV1 < 40, while chronic Pseudomonas aeruginosa (p < .001) and mucoid P. aeruginosa colonization (p < .001) were significantly higher in this group (p < .001). Overall mean annual ppFEV1 decline was -0.97% (95% confidence interval [CI] = -0.02 to -1.92%). The mean change of ppFEV1 was significantly higher in the group with ppFEV1 ≥ 70 compared with the other (ppFEV1 < 40 and ppFEV1: 40-69) two groups (p = .004). Chronic P. aeruginosa colonization (odds ratio [OR] = 1.79 95% CI = 1.26-2.54; p = .01) and initial ppFEV1 ≥ 70 (OR = 2.98 95% CI = 1.06-8.36), p = .038) were associated with significant ppFEV1 decline in the whole cohort. CONCLUSIONS: This data analysis recommends close follow-up of patients with normal initial ppFEV1 levels at baseline; advocates for early interventions for P. aeruginosa; and underlines the importance of nutritional interventions to slow down lung disease progression.

Complex sleep apnea syndrome in a child with Chiari malformation type 1.

There are few reports of a patient presenting with symptoms of obstructive sleep apnea syndrome (OSAS) as the sole manifestation of Chiari malformation type 1 (CM1). In the literature, complex sleep apnea syndrome (CompSAS) was also reported as a rare condition related to CM1 patients. We report the case of a 13-year-old patient with the complaint of snoring and difficulty in breathing during sleep, but otherwise healthy. After an initial polysomnography, the patient was diagnosed with OSAS and nocturnal continuous hypoxemia. The child underwent titration to pressure of continuous positive airway pressure (CPAP); obstructive apnea improved but central apnea (i.e., CompSAS) and nocturnal continuous hypoxemia persisted. Magnetic resonance imaging led to diagnosis of CM1. Her central apnea and nocturnal hypoxemia resolved following bi-level positive airway pressure-spontaneous-timed (S/T) (BiPAPS/ T) treatment. We emphasize that the CM1 cases can admit with only breathing problems during sleep without concomitant neurological findings, and this malformation can cause central apnea resistant to CPAP.

The role of serum Pseudomonas aeruginosa antibodies in the diagnosis and follow-up of cystic fibrosis.

In cystic fibrosis (CF), if Pseudomonas aeruginosa (Pa) infection is not diagnosed and treated early, chronic colonization occurs, which causes rapid decline in pulmonary functions. The aim of this study was to evaluate Pa antibodies, compare them with Pa cultures and determine their role in early diagnosis and follow-up. Ninety CF patients were included; they were divided into chronic, intermittent, negative, and mucoid groups. They were evaluated every 3-6 months. In each visit, pulmonary function tests and sputum cultures were obtained, and Pa antibodies exotoxin A (ExoA), elastase (ELA) and alkaline protease (AP) were determined in the serum by enzyme-linked immunosorbent assay (ELISA). The most specific test that discriminated chronic colonized patients from noncolonized patients was Pa culture, and the presence of at least one antibody had the highest sensitivity. AP had the highest specificity, and ELA had the highest sensitivity. All antibodies were highest in the mucoid group. ELA was highest in chronic and lowest in the negative group. The presence of antibodies was much higher than positive Pa cultures in patients younger than five years of age. A negative correlation between forced expiratory volume in 1 second (FEV1) and AP was determined only in the mucoid group. In the two-year follow-up, antibody presence did not show a regular pattern. In CF, Pa antibodies can be early markers for diagnosis, especially in young children who cannot expectorate, but they should only be used together with sputum cultures for long-term follow-up and treatment.

Multicentric analysis of childhood tuberculosis in Turkey.

Only a few series of pediatric tuberculosis (TB) have been reported in the last 20 years. The purpose of this study was to evaluate the clinical, radiological, microbiological, and treatment characteristics of childhood TB. A total of 539 children with childhood TB diagnosed over a 12-year period (1994-2005) in 16 different centers in Turkey participated in the study. The medical records of all childhood TB patients were investigated. A total of 539 children (274 males, 265 females) with childhood TB aged 10 days-17 years participated in the study. Age distribution was nearly equal among all age groups. We detected the index case in 39.8% of the patients. More than one index case was detected in 17.3% of the patients. A minimum 15-mm induration is accepted on tuberculin skin test (TST) following Bacillus Calmette-Guérin (BCG) vaccination. The TST was positive in 55.3% of the patients. Acid-fast bacillus smear was positive in 133, and polymerase chain reaction for Mycobacterium tuberculosis was positive in 45 patients. In 75 patients (13.9%), cultures yielded M. tuberculosis. One hundred fifty-one patients (28%) did not present for followup, and families of 5 patients (0.9%) discontinued the treatment. Pulmonary TB (n=285) and meningeal TB (n=85) were the most frequent diseases. In 29% of the patients, there was poor adherence to treatment or patients were lost to follow-up. We have demonstrated that household contact screening procedures play a major and important role, especially considering the high ratio of cases with contact index cases. We also recommend that the positive TST values should be reviewed according to the local cut-off data and should be specified in as many countries as possible. In view of the considerably high percentages of patients lost to follow-up and treatment discontinuation observed in our study, we suggest that application of directly observed treatment short-course (DOTS) is preferable.