Analysis of predictive factors in children with high suspicion of pulmonary embolism.

BACKGROUND AND AIM: Since there is a limited number of predictive models designed for pediatric patients with pulmonary embolism (PE), healthcare providers have resorted to utilizing the Wells criteria and PE Rule-out Criteria (PERC) that have been validated for use in adults. The objective of the study is to identify the predictive factors for PE and assess the effectiveness of the PERC, Wells, and pediatric-specific PE (PPE) criteria. METHODS: The study included individuals between the ages of 0-18 years who underwent pulmonary CT angiography and/or ventilation-perfusion scintigraphy with suspicion of PE. Demographic characteristics, symptoms, physical examination findings, risk factors, and laboratory and imaging results were retrospectively analyzed, and PERC, Wells, and PPE criteria were applied to the study population. RESULTS: Of the 110 patients included in the study, 27 (24.5%) had PE. Saturation, albumin, cough, calf swelling, central catheter, and malignancy were found to significantly contribute to the model. The total weighted risk score, which represents the sum of all predictive scores, ranged from 0 to 16 with a mean of 5.41 ± 4.02. When the cut-off >6.5, the model had good discrimination power for positive PE (AUC 0.79, 77% sensitivity, and 70% specificity). In our study, the Wells criteria showed a sensitivity of 96% and a specificity of 24%. The PERC exhibited a sensitivity of 96% and a specificity of 21%, while the PPE demonstrated a sensitivity of 74% and a specificity of 30%. CONCLUSION: While the sensitivity of Wells, PERC, and PPE models was higher than our developed model, their specificities were considerably lower than our model.

Incidental pulmonary nodules in children: characteristic features and clinical course.

BACKGROUND: There exists insufficient information about the natural course of incidental pulmonary nodules (IPN) determined on tomography in children. The aim was to determine the characteristic features and factors affecting the course of IPN. METHODS: This retrospective study included patients who presented at the Pediatric Pulmonology, Allergy & Immunology Section of Akdeniz University Hospital between January 2014-2020, and were determined with pulmonary nodules on high-resolution computed tomography (HRCT). The patients were separated into two groups as those with a nodule decreased in size or which had disappeared on the follow-up HRCT (Group 1) and those with a nodule which had remained at the same size (Group 2). These two groups were compared in respect to demographic data, nodule size and characteristics, and accompanying findings on HRCT. RESULTS: A total of 177 nodules were determined in the 66 patients included in the study. A follow-up HRCT was taken within mean 16.29±11.38 months in 27 patients. In these patients, 78 nodules were determined on the initial HRCT. On the follow-up, twelve of the nodules were seen to have shrunk or disappeared compared to the initial images, 66 had remained the same size, and none had grown. The mean age of the patients in Group 1 was statistically significantly lower than that of patients in Group 2 (p < 0.001). The rates of an accompanying mosaic attenuation pattern (p < 0.001) on HRCT and subsolid density (p=0.011) of the nodules in Group 1 were statistically significantly higher compared to Group 2 and the rate of calcification content was statistically significantly lower (p=0.002). No suspicious or confirmed malignancy was observed in any case throughout the mean follow-up period of 38.33±16.5 months after the initial HRCT. CONCLUSIONS: The young age of patients, subsolid structure of nodules, calcification content and the presence of an accompanying mosaic attenuation pattern on HRCT, could be useful factors in the estimation of size in the follow-up of nodules.

Psychological profiles, quality of life, and parental attitudes in children with Sighing dyspnea.

BACKGROUND: There are a limited number of studies investigating the relationship of sighing dyspnea (SD) with psychiatric disorders and various mental states. METHODS: This cross-sectional study enrolled 39 pediatric patients with SD, but free from apparent cardiopulmonary diseases, who presented to a pediatric pulmonology policlinic between June and December 2019 and age-gender matched 34 healthy children were recruited for comparison. Patient characteristics, psychological or physical trauma history, and cigarette smoke exposure were recorded. All the participants completed the "NOSE scale," "Modified Borg Scale," "Children Depression Inventory," "Screen for Child Anxiety Related Disorders," "Pediatric Quality of Life Inventory," "Stress Coping Style Scale," "Family Assessment Device," and "Parents Attitude Scale." RESULTS: Compared to the control group, there was a statistically significantly higher rate in the SD group of physical or psychological trauma history (35.9% vs. 14.7%, p = .04) and passive cigarette smoking (60.5% vs.27.3%, p = .005). When the groups were compared in respect of depression and anxiety levels, the depression score, total anxiety level, somatic/panic, generalized anxiety and fear of school points were determined to be statistically significantly higher in the SD group. The QoL scores (total, physical, and psychosocial) in the SD group were found to be statistically significantly lower than those of the control group. CONCLUSION: To the best of our knowledge, this is the first study to have examined the psychological profiles of SD patients and their families in detail, and to have evaluated the effect of SD on quality of life.

The success of the Cystic Fibrosis Registry of Turkey for improvement of patient care.

BACKGROUND: Cystic fibrosis (CF) registries play an essential role in improving disease outcomes of people with CF. This study aimed to evaluate the association of newly established CF registry system in Turkey on follow-up, clinical, growth, treatment, and complications of people with this disease. METHODS: Age at diagnosis, current age, sex, z-scores of weight, height and body mass index (BMI), neonatal screening results, pulmonary function tests, history of meconium ileus, medications, presence of microorganisms, and follow-up were evaluated and compared to data of people with CF represented in both 2017 and 2019 registry data. RESULTS: There were 1170 people with CF in 2017 and 1637 in 2019 CF registry. Eight hundred and fourteen people were registered in both 2017 and 2019 of whom z-scores of heights and BMI were significantly higher in 2019 (p = 0.002, p =0.039, respectively). Inhaled hypertonic saline, bronchodilator, and azithromycin usages were significantly higher in 2019 (p =0.001, p = 0.001, p = 0.003, respectively). The percent predicted of forced expiratory volume in 1 sec and forced vital capacity were similar in 2017 and 2019 (88% and 89.5%, p = 0.248 and 84.5% and 87%, p =0.332, respectively). Liver diseases and osteoporosis were significantly higher, and pseudo-Bartter syndrome (PBS) was significantly lower in 2019 (p = 0.011, p = 0.001, p = 0.001, respectively). CONCLUSIONS: The z-scores of height and BMI were higher, the use of medications that protect and improve lung functions was higher and incidence of PBS was lower in 2019. It was predicted that registry system increased the care of people with CF regarding their follow-up. The widespread use of national CF registry system across the country may be beneficial for the follow-up of people with CF.

No Local Findings after Subclavian Catheter Removal. Is Everything Alright? Case Report.

A 10-year-old male patient was admitted to the paediatric intensive care unit due to septic shock and oliguric acute renal failure. A haemodialysis catheter (11.5 Fr) was inserted into left subclavian vein for haemodialysis and cytokine-adsorption therapy. Haemodialysis and cytokine adsorption filter was applied to the patient for a total of two days, and then haemodialysis catheter was not used. The catheter was removed from the patient who was decided to transfer to the service on the fifth day of his admission. Tachycardia and hypotension developed and general condition deteriorated immediately after removal of the catheter. With rapid interventions, shock findings were corrected and the patient was reintubated and followed up in mechanical ventilation. On chest X-ray and thorax ultrasonography, the left hemithorax was completely filled, and a total of 1,500 mL of blood was drained by inserting a thorax tube. The patient was transferred to the paediatric pulmonology clinic after nine days of intensive care stay. Haemothorax development after subclavian catheter removal is a rare but a life-threatening condition. For these reasons, we believe that cases with removed subclavian or internal jugular vein catheters should be followed up for a suitable period of time.

Association Between Cystic Fibrosis Severity Markers and CFTR Genotypes in Turkish Children.

OBJECTIVE: To compare class I/II cystic fibrosis transmembrane conductance regulator (CFTR) mutations to class III-V mutations with regards to cystic fibrosis disease severity markers in children. MATERIAL AND METHODS: This study was designed as a cross-sectional study in Antalya province, located on the south coast of Turkey. The study included 38 cystic fibrosis patients aged between 0.6 and 18 years. The CFTR genotype of the patients was categorized into 2 groups based on the presence or absence of class I or class II mutations in any of the alleles. Group I comprised 8 homozygous, 8 with unknown alleles, and 8 compound heterozygous patients, and group II comprised 11 homozygous and 3 compound heterozygous patients. The groups were analyzed in respect of cystic fibrosis disease severity markers, such as spirometry, ShwachmanKulczycki score, body mass index (BMI), sweat chloride concentration, chronic Pseudomonas aeruginosa infection, annual exacerbation frequency, and severe exacerbations requiring hospitalization during the previous year. RESULTS: In the comparison of group I and group II patients, a significant difference was observed in pancreas insufficiency (83.3% vs. 35.7%; P = .005), chronic P. aeruginosa infection (58.3% vs. 7.1%; P = .002), cough severity score (1.7 ± 1.1 vs. 0.9 ± 1.5; P = .029), number of severe exacerbations requiring hospitalization during the previous year (0.9 ± 1 vs. 0.3 ± 0.8; P = .03), and sweat chloride levels (76.7 ± 15.2 vs. 61 ± 22.3; P = .02). All these values were higher in group I patients. The mean BMI values (15.8 ± 2.2 vs. 17.6 ± 2.8; P = .03) were lower in group I patients. CONCLUSION: There seems to be a difference between class I/II CFTR mutations and class III-V mutations on the severity of the disease in cystic fibrosis patients.

Clinical features and accompanying findings of Pseudo-Bartter Syndrome in cystic fibrosis.

BACKGROUND: Pseudo-Bartter syndrome (PBS) is a rare complication of cystic fibrosis (CF) and there are limited data in the literature about it. We aimed to compare clinical features and accompanying findings of patients with PBS in a large patient population. METHODS: The data were collected from the Cystic Fibrosis Registry of Turkey where 1170 CF patients were recorded in 2017. Clinical features, diagnostic test results, colonization status, complications, and genetic test results were compared in patients with and without PBS. RESULTS: Totally 1170 patients were recorded into the registry in 2017 and 120 (10%) of them had PBS. The mean age of diagnosis and current age of patients were significantly younger and newborn screening positivity was lower in patients with PBS (P < .001). There were no differences between the groups in terms of colonization status, mean z-scores of weight, height, BMI, and mean FEV1 percentage. Types of genetic mutations did not differ between the two groups. Accompanying complications were more frequent in patients without PBS. CONCLUSION: PBS was detected as the most common complication in the registry. It could be due to warm weather conditions of our country. It is usually seen in younger ages regardless of mutation phenotype and it could be a clue for early diagnosis of CF.

Patients eligible for modulator drugs: Data from cystic fibrosis registry of Turkey.

BACKGROUND: A better understanding of cystic fibrosis transmembrane conductance regulator biology has led to the development of modulator drugs such as ivacaftor, lumacaftor-ivacaftor, tezacaftor-ivacaftor, and elexacaftor-tezacaftor-ivacaftor. This cross-sectional study evaluated cystic fibrosis (CF) patients eligible for modulator drugs. METHODS: Data for age and genetic mutations from the Cystic Fibrosis Registry of Turkey collected in 2018 were used to find out the number of patients who are eligible for modulator therapy. RESULTS: Of registered 1488 CF patients, genetic analysis was done for 1351. The numbers and percentages of patients and names of the drugs, that the patients are eligible for, are as follows: 122 (9.03%) for ivacaftor, 156 (11.54%) for lumacaftor-ivacaftor, 163 (11.23%) for tezacaftor-ivacaftor, and 57 (4.21%) for elexacaftor-tezacaftor-ivacaftor. Among 1351 genotyped patients total of 313 (23.16%) patients are eligible for currently licensed modulator therapies (55 patients were shared by ivacaftor and tezacaftor-ivacaftor, 108 patients were shared by lumacaftor-ivacaftor and tezacaftor-ivacaftor, and 22 patients were shared by tezacaftor-ivacaftor and elexacaftor-tezacaftor-ivacaftor groups). CONCLUSIONS: The present study shows that approximately one-fourth of the registered CF patients in Turkey are eligible for modulator drugs. As, frequent mutations that CF patients have in Turkey are different from North American and European CF patients, developing modulator drugs effective for those mutations is necessary. Furthermore, as modulator drugs are very expensive currently, financial support of the government in developing countries like Turkey is noteworthy.

Initial regional evaluation of the Cystic Fibrosis Newborn Screening Program: data from the Mediterranean coast of Turkey

Background/ aim: Since January 2015, the Cystic Fibrosis Newborn Screening (CFNS) program has been implemented in Turkey. We aimed to evaluate the demographic, clinical, and laboratory data of cases referred from the CFNS program and to determine the most suitable cut-off value for immunoreactive trypsinogen (IRT)-1 and immunoreactive trypsinogen (IRT-2) that are used in the CFNS program in Turkey. Materials and methods: A total of 156 Turkish Caucasian subjects were determined as positive cases during 3 years, from January 2015 to January 2018, and were referred to the pediatric pulmonology clinics of Akdeniz University Hospital, Antalya, Turkey, for the national CFNS program. The evaluation was made considering the IRT-1 and IRT-2 values, demographic characteristics, sweat test results, CFTR genotypes, and diagnoses. Results: Nine patients were diagnosed with cystic fibrosis (CF). Eight were diagnosed with CF-related metabolic syndromes and three were determined to be CF carriers. The ratio of CF to CF-related metabolic syndrome was determined as 1.1:1. Considering the limits of the present CFNS program and the IRT method, the positive predictive value (PPV) for the referred cases was determined as 5.8%. When a cut-off value of 105.6 ng/mL was taken for IRT-1, sensitivity was 100%, specificity was 59%, and PPV was 12.8%. For a cut-off value of 88.75 ng/mL for IRT-2, sensitivity was determined as 90%, specificity as 65%, and PPV as 15.2%. Conclusion: This is the first detailed clinical study to evaluate the data from the CFNS program along the Mediterranean coast of Turkey. As false positive results are extremely high in Turkey, there is an urgent need for revision of the IRT-1 and IRT-2 limits by evaluating the data of the whole country.

Evaluation of Noncystic Fibrosis Bronchiectasis Using Clinical and Radiological Scorings in Children.

OBJECTIVES: The aim of this study was to evaluate radiological, clinical, and demographic data of patients with noncystic fibrosis bronchiectasis and to compare high-resolution computed tomography (HRCT) scores based on the demographic and clinical characteristics. MATERIALS AND METHODS: A total of 34 patients (18 male, 16 female) were assessed in terms of age at symptom onset, age at diagnosis, annual attack frequency, cough severity score, physical examination findings, and pulmonary function test results. Modified Bhalla scoring system (B total) and anatomical prevalence degree score (D total) were used for HRCT examination. RESULTS: There was a strong negative correlation between forced expiratory volume at first second (FEV1) and bronchial dilatation degree (SBRDIL). There was a moderate negative correlation of FEV1, forced vital capacity (FVC), and maximum mid-expiratory flow rate (MEF; 25-75) with bronchiectasis degree (EXBRNC), bronchial wall thickness degree (SBWTHICK), and mucus accumulation in the major airways (PMPLA). The B total, D total, EXBRNC, and SBRDIL scores were significantly higher in patients with hemoptysis and sputum. In comparing B and D total scoring systems, B total provided better results in terms of rale, annual exacerbation frequency (AEF), cough severity score (CSS), and FEV1 values. CONCLUSION: As it is proved using HRCT, pulmonary function impairment, sputum production, hemoptysis, and increase in AEF strongly correlating with objective HRCT scoring can be accepted as markers for pathological changes due to bronchiectasis.

Normal sweat chloride test does not rule out cystic fibrosis.

Basaran AE, Karatas-Torun N, Maslak IC, Bingöl A, Alper ÖM. Normal sweat chloride test does not rule out cystic fibrosis. Turk J Pediatr 2017; 59: 68-70. A 5-month-old patient presented with complaints of fever and cough. He was hospitalized with the diagnosis of bronchopneumonia and pseudo-Bartter's syndrome. Patient was further investigated for diagnosis of cystic fibrosis. The chloride (Cl) level in sweat was determined within the normal range (25.1 mmol/L, 20.3 mmol/L). CFTR (Cystic Fibrosis Transmembrane Regulator gene; NM_000492.2) genotyping results were positive for p.E92K; p.F1052V mutations. The patient was diagnosed with cystic fibrosis. In our patient, with features of CF and normal sweat test, mutation analysis was helpful for the diagnosis of cystic fibrosis.