Objectives: Congenital lung malformations (CLM) are rare, heterogeneous group of disorders that consist of abnormal lung development. Surgical resection is the definitive treatment for these lesions. ...After resection of abnormal congenital lesion, it is expected that lung volumes and functions will improve due to compensatory lung growth and regeneration. Our aim is to evaluate the lung functions of patients who underwent lobectomy for CLM in long term follow-up by comparison to normal healthy children, by using spirometry and Lung Clearance Index (LCI) Methods: 16 children, between the ages of 7-21, with CLM who had undergone surgical resection and 12 healthy children, between the ages of 7-18, as a control group were included in our study. All patients evaluated by their demographic data, physical examination findings and pulmonary function tests. Spirometric and LCI measurements performed in all participants and compared with each other. Results: Of these 16 patients, 9 (56.2%) were boys and 7 (43.8%) were girls, median age was 8.75 years (min:6.5, max 21.25). The median age at the time of operation was 14 months (min:1, max 168) and mean post-op follow-up duration was 80.06±31 months. 68.75% of patients had congenital pulmonary airway malformations, 12.5% had bronchogenic cyst, 6.25% had bronchopulmonary sequestration, 6.25% had congenital lobar emphysema and 6.25% had combine pulmonary airway malformations and sequestration. In the healthy control group, 4 (33.3%) of the children were boys and 8 (66.6%) children were girls, median age was 12 years (min:8.5, max 17.5). The pulmonary function measurements of the participants exhibited mean predicted FEV1 was 86,25±16,88% in post operative CLM patients and 101.75+11.5% in the control group. Both groups results were spirometrically normal. By means of FEV1%, post-operative CLM patients group had significantly lower values than healthy control grup (p:0.014). Comparing the two groups by assessing LCI, median LCI values in post –op CTM patients’ group were at 8.27 (min:6.39 max:11.50) and 7.55 (min:7.1 max:10.4) in healthy control group. Patients’ LCI values were not statistically significant when compared with healthy control group. (p:0.548). Also, there was a strong inverse correlation between LCI and FEV1 (p: 0.014) in patients who underwent surgery for CLM. Conclusion: Our study showed that, in long term follow –up’s, patients who underwent surgery for CLM will not occur airway disease and show no differences in pulmonary function tests by comparing healthy children and LCI was more accurate detecting airway diseases than spirometry.
Objectives: The prevalence of sleep disordered breathing (SDB) in children is 1-4% and OSA can cause significant morbidity in children. Overnight polisomnograpy (PSG) in the sleep laboratory is the ...gold standard for diagnosis. However, the availability of PSG is limited. Polygraphy (PG) is a simpler, cost effective method and can be used as an alternative to PSG in children. Our aim was to evaluate the clinical features and PG results of children with suspected SDB who underwent PG in the sleep laboratory of a tertiary center. Methods: 81 patients, who underwent PG between 2016 and 2018 were included. Demographic data, sleep symptoms and Pediatric Sleep Questionnaire (PSQ) scores were noted and polygraphy results were evaluated. OSAS was as an apnea-hypopnea index (AHI) more than 1. The severity classifications used were: mild (AHI:1-5), moderate (AHI:5-10) and severe (AHI >10). A cut off value of 0.33 in pediatric sleep questionnaire (PSQ) was used to identify pediatric sleep disordered breathing (SDB). Results: The mean age of the patients were 9.4±4.9 years (67.9% male). Thirty-five patients (43.2%) were referred to sleep laboratory for OSAS symptoms (snoring, mouth breathing, sleep apnea). Seventeen patients (21%) were referred because of high carbon-dioxide levels in blood gases, 20 (24.7%) were referred because of a genetic or neuromuscular disease or a syndrome that predisposes to OSAS. Forty percent of the children had observed apneas during sleep, 65.4% had habitual snoring. Median apnea-hypopnea index (AHI) was 4.6 (range:0-100.3). Mean AHI of the patients with observed apneas during sleep was not higher compared to children without apneas. Mean AHI of snorers were significantly higher than non-snorers (13.2 vs. 5.3, p=0.009). 73 patients completed PSQ and 33 (45.2%) had SDB. There was no significant correlation between PSQ scores and AHI (r=0.19, p=0.1). Mean AHI of the patients with abnormal and normal PSQ scores were 13.4±17.4 and 9.4±17.1, respectively (p: 0.33). Prevalence of OSAS was 80.3% in our population, 34.6% had mild, 14.8% had moderate and 30.9% had severe OSAS. Nocturnal hypoventilation and hypoxemia was diagnosed in 7.4% of the patients and only one PG was not interpretable. In 22 patients (27.2%) a follow up PSG was performed because PG results were incompatible with clinical symptoms or for titration. Conclusion: Polygrapy is a useful and relatively simple method for SDB diagnosis in children and can be an alternative to PSG to decrease the load of sleep laboratories.
Cystic fibrosis (CF) affects the musculoskeletal system via a multifactorial pathway that includes vitamin D deficiency and involvement of respiratory muscles such as intercostals due to recurrent ...upper and lower respiratory tract infections. Eventual result is the deterioration of musculoskeletal health and posture in CF patients. Postural stability is directly affected by posture and can be compromised in every musculoskeletal problem. The aim of this study is to evaluate musculoskeletal system and postural stability in patients with CF.
Patients with CF over six years of age and age and sex-matched control groups were included in the study. Cobb angle and thoracic kyphosis angles were measured on the spine radiographs. Both patients and control group were examined with pediatric gait, arms, legs and spine scale (pGALS). They also were evaluated with a NeuroCom Balance Master for their postural stability.
Fifty-one patients with CF and 94 healthy controls participated in the study. In results of the pGALS examination, CF group had significantly more pathological findings than the control group in lower extremity appearance and movement (p = 0.006 and p = 0.01) and spine appearance and movement (p = 0.001 and p = 0.022) domains. The tandem walking speed was significantly higher in controls with a mean of 24.45 ± 7.79 while it was 20.47 ± 6.95 in the CF group (p = 0.03). Various limits of stability parameters also showed significant differences. Medium correlations were found between musculoskeletal examination and postural stability parameters.
In patients with CF, a systematic but simple musculoskeletal examination can detect pathologies, which are more frequent than the normal population. These pathologies show a medium correlation with the involvement of postural stability.
Objectives: Nusinersen, promising treatment option for spinal muscular atrophy (SMA) patients was approved by the FDA in 2016. In this study, we aimed to evaluate respiratory support need in patients ...and to report their respiratory follow-up during the treatment period. Methods: The study included patients treated with 4 and more doses of Nusinersen. Nusinersen treatment was performed on the 1st-15th-29th-64th-183rd days. Patients were evaluated regarding respiratory findings before the initiation of Nusinersen treatment and after the 4.th doses in pediatric pulmonology department. Chop scores were evaluated in pediatric neurology department as well. Results: Twenty-seven patients who were followed up from our clinic were included in the study. 44% of the patients were female (n=12); The mean age was 47.81±6.27 months (7-120 months). The mean treatment duration of the patients was 36.1±7.57 months (2-111 months). Sixteen patients were on home ventilation via tracheostomy, 6 patients were in ICU and mechanically ventilated (5 of them were entubated and 1 of them was tracheostomized). One patient was on non-invasive ventilation and needed O2 support. Four patients breathing spontaneously in room air. 56.2% (n=9) of home ventilated patients and 50% (n=3) of patients staying in ICU had clinical improvement. One of the entubated patients was tracheostomized and home ventilation was initiated. Four patients breathing spontaneously did not require any respiratory support. The oxygen need of the non invasive ventilated patient was ended after the 4.th doses of Nusinersen. Home ventilation therapy was stopped by a family without the knowledge of the clinician after the third doses of Nusinersen theraphy but unfortunately the patient was passed away. Conclusion: Respiratory support need was decreased in nearly half of the patients after 4 doses of Nusinersen theraphy. Patients should be closely followed up by the neurology, pulmonology, physical therapy and rehabilitation departments with a multidisciplinary approach. Therapy should be discontinued by the decision of three disciplines in patients who do not get benefit. Long term studies including more patients treated with Nusinersen and specified respiratory scoring systems are needed to evalute the respiratory follow up.
Sleep disordered breathing is a common problem in childhood that encompasses a spectrum of disorders extending from primary snoring to obstructive sleep apnea. This study aims to investigate the ...results of children undergoing evaluation with polysomnography in the sleep laboratory of a tertiary care hospital.
Demographic and clinical features as well as sleep associated symptoms, scores of pediatric sleep questionnaire and Pittsburgh sleep quality index and polysomnography results are retrospectively evaluated.
Totally 131 patients were evaluated, of which 47.3% (n=62) were females and 52.7% (n=69) were males. Mean age was 101.85±59.15 months at the time of the study. Fifty percent (n=59) of patients complained of snoring and 43.7% (n=52) of patients complained of apnea during sleep. Mean obstructive hypopnea-apnea index was 5.12±11.72. Mean obstructive hypopnea-apnea index of snorers (6.93±13.53) was significantly higher than the mean obstructive hypopnea-apnea index of nonsnorers (2.32±5.43) (p=0.011). Mean obstructive hypopnea-apnea index of patients experiencing apnea during sleep (7.52±14.25) was significantly higher than the mean obstructive hypopnea-apnea index of the children who do not experience apnea (2.61±5.84) (p=0.008). No significant correlation was observed between obstructive hypopnea-apnea index and scores of pediatric sleep questionnaire and Pittsburgh sleep quality index. The prevalence of obstructive sleep apnea was 33.6% (n=44). Forty nine patients (39.8%) were treated after polysomnography. Frequently suggested treatment options were noninvasive mechanical ventilation (n=23, 46.9%), intranasal steroid (n=15, 30.6%), montelukast (n=11, 22.4%) and adenotonsillectomy (n=9, 18.4%).
Polysomnography is the gold standard in the diagnosis of sleep disordered breathing in children. Pediatricians should be able to recognize early signs and symptoms of sleep disordered breathing and refer the patients in risk to centers where evaluation with polysomnography is available.
Background: The objective of the study was to assess the diagnostic efficacy of the coulometric endpoint method and compare it with classic Gibson&Cooke and chloridometer methods.Methods: This study ...is a prospective clinical study comparing two conventional sweat testing methods with the coulometric endpoint method in previously diagnosed cystic fibrosis (CF) patients and a non-CF control group. All individuals underwent two simultaneous sweat collections. One sample of sweat, collected by the CFΔ collector coil system, was analyzed by two methods: the titrimetric Cl− measurement (Sherwood® Chloridometer 926S, Sherwood Scientific Ltd., Cambridge, UK) and the coulometric endpoint method (CF Δ Collection System®, UTSAT/Turkey); the second sample was collected from the other forearm by the Gibson&Cooke method and the collected sweat was analyzed by manual titration in accordance with the Schales&Schales method. Within-run and between-run imprecisions were evaluated via Cl− concentrations of 40, 70, and 130 mmol/L samples.Results: One hundred and seventy (60 CF and 110 controls) subjects were included in the study.All three sweat test methods discriminated CF subjects from the healthy individuals. The mean difference between the coulometric endpoint and titrimetric Cl− measurement methods was −1.5 mmol/L, (95% confidence limits of agreement, ranging from −8.9 to 15.9 mmol/L); the mean difference between manual titration vs. coulometric endpoint methods was 12.8 mmol/L, (95% confidence limits of agreement ranging from −9.7 to 45.3 mmol/L) and the mean difference between the manual titration and titrimetric Cl− measurement methods was 11.3 mmol/L, (95% confidence limits of agreement ranging from −7.8 to 40.5 mmol/L) based on a Bland-Altman analysis. In the Receiver operating characteristic (ROC) analysis, made on the basis that Cl− concentration values < 40 mmol/L exclude the CF diagnosis, the coulometric endpoint method resulted in 96.7% sensitivity and 100% specificity for a cut-off value of 58.5 mmol/L (AUC: 0.994; 95% CI = 0.986–1.000; p < 0.001).Conclusions: The coulometric endpoint method can be as reliable as quantitative sweat Cl− analysis and may be considered as a definitive diagnostic tool for CF.
Aim: Severe pulmonary disease is responsible for over 80% of deaths associated with cystic fibrosis. Our aim was to evaluate the other risk factors associated with mortality in patients with cystic ...fibrosis who have moderate and severe lung disease. Material and Method: Among 200 patients with cystic fibrosis who were followed up in our clinic, 35 patients with moderate and severe lung disease (%FEV1 predicted less than or equal to 60) were included in the study. Demographic data, pulmonary function tests, high resolution thorax tomography, modified Bhalla score, blood gas analysis and nutritional status of the patients were evaluated. Results: Requirement for respiratory support was 31.4% in moderate and severe lung disease. Lower pulmonary function, requirement for non invasive ventilation, presence of diabetes mellitus, a SaO.sub.2 value of < 95, a value of pCO.sub.2 >50 mm Hg and requirement for frequent IV antibiotics were significantly related with increased mortality. Conclusions: This is a single centre study conducted in patients with cystic fibrosis who had moderate and severe lung disease and who had not received lung transplantation. We evaluated the clinical outcome and other factors that were associated with mortality. (Turk Arch Ped 2012; 47:263-267) Key words: Child, cystic fibrosis, risk of mortality
Background
Health‐related quality of life (HRQoL) scales are now widely used in children with cystic fibrosis (cwCF) which reflects the course of the disease. In this cross‐sectional study, our ...primary aim was to compare the Pediatric Oral Health‐Related Quality of Life (POQL) and Oral Health Score (OHS) between cwCF and healthy group. Our secondary aim was to evaluate the association between Pseudomonas aeruginosa (PA) colonization, pulmonary function test, OHS and POQL in cwCF.
Methods
The study population (age ranging 6–14) included 55 cwCF followed at the Marmara University Division of Pediatric Pulmonology compared with 50 healthy peers. A survey consisted of general questions (age, sex, etc.) and the POQL instrument were filled by parents. The decayed, missing, and filled teeth for both primary (dft) and permanent dentition (DMFT) was detected according to WHO criteria. Data like current body mass index (BMI z score), colonization status with PA, predicted value for forced expiratory volume in 1 second (FEV1pp), and any hospitalizations during the previous year were obtained from their medical. Differences between the groups were evaluated using Chi‐square and Mann–Whitney U test with a significance level set at 0.05.
Results
There was no significant difference between PA‐colonized cwCF and healthy controls in DMFT (p = 0.916). For all domains of POQL (emotional function, social function, role function), scores of cwCF were significantly better than healthy controls (p < 0.05). There were no statistically significant differences between all domains of POQL scores in PA colonized and non‐PA colonized cwCF' POQL scores (p > 0.05).
Conclusion
Although POQL scores of cwCF were encouraging, dental caries prevention and regular follow‐ups should be taken into consideration.
Introduction
Polygraphy (PG) can be used as an alternative test for the diagnosis of obstructive sleep apnea syndrome (OSAS) in children. Night‐to‐night variability of PG in children is not known. ...Our aim was to determine whether a single night PG was reliable for OSAS diagnosis in children with symptoms of sleep‐disordered breathing (SDB).
Materials and Methods
Otherwise healthy children who had been evaluated for symptoms of SDB were included. Two nocturnal PGs were performed 2–7 days apart. Demographic and clinical characteristics, Pediatric Sleep Questionnaire, and modified Epworth Sleepiness Scale were recorded. OSAS was diagnosed if obstructive apnea–hypopnea index was (oAHI) ⩾ 1/h and classified as mild (oAHI: 1–4.9/h), moderate (oAHI: 5–9.9/h), and severe (oAHI ⩾ 10/h).
Results
Forty‐eight patients were included (37.5% female, age 10.8 ± 3.9 years) to the study. There were no significant differences in oAHI values and other respiratory parameters between the two PGs (p > 0.05). Thirty‐nine children were diagnosed with OSAS if the highest oAHI over any single night was used for diagnosis. Thirty‐three of the 39 children (84.6%) were diagnosed with OSAS with the first PG while 35 of 39 (89.7%) children were diagnosed with OSAS with the second PG. There was an agreement for identifying OSAS and its severity between the two PGs in our study even though there were few individual intra‐subject differences in oAHI.
Conclusion
There was no significant first‐night effect for PG in this study which suggests that a single night PG is adequate for diagnosis of OSAS in children with SDB‐ related symptoms.
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