[The evaluation of asthma and COPD awareness in Turkey (GARD Turkey Project-National Control Program of Chronic Airway Diseases)]. / Türkiye'de astim ve KOAH farkindaliginin degerlendirilmesi (GARD Türkiye Projesi-Kronik Hava Yollari Hastaliklari Ulusal Kontrol Programi).

INTRODUCTION: Although chronic respiratory disorders are important causes of morbidity and mortality, health care workers, patients and caretakers are not well informed about these disorders. Therefore these problems are underdiagnosed and undertreated; also preventive measures are not widely taken. Our aim was to evaluate the knowledge of asthma and chronic obstructive pulmonary disease (COPD) in Turkey. This study was designed and performed as a Global Alliance Against Respiratory Disorders (GARD) project. MATERIALS AND METHODS: People greater than 15 years of age who lived in cities with a population of 200 or greater were eligible for the study. A questionnaire including demographic data and questions regarding asthma and COPD was used for the evalution of the participants. RESULTS: 12.000 people were selected (6000 in rural and 6000 in urban areas); 8527 people were reached. 8342 people who completed the questionnaire were included to the study. There were 4182 (50.1%) female and 4160 (%49.9) male subjects. 49.6% of the subjcets knew that COPD is a lung disease, 51.1% indicated that smoking is the most important risk factor for COPD and 48% identified quitting smoking as the most important preventive measure. Every other person had baseline knowledge on COPD. However only 25.2% knew that there are treatment options for COPD. 80% of subjects said astma can be seen in all age groups. 51.1% knew asthma is a genetic disease and 58% said it is not an infectious disease. However when whether asthma medications caused drug dependency only 27% answered as "No" while 55.2% said "They do not know". CONCLUSION: Awareness of COPD and asthma seem to be infsufficient among Turkish people. Since these disorders are important causes of morbidity and mortality and have high impact on work and economic loss, it is important to increase knowledge among public.

Objective monitoring of cough in children with cystic fibrosis.

Increased cough frequency is a common symptom associated with infective pulmonary exacerbations of cystic fibrosis (CF), but subjective assessment of cough is very unreliable. The aims of this study were: 1) to validate a modification of our previously described ambulatory cough recording device (LR 100); 2) to determine how accurately children with CF assess levels of cough; and 3) to assess the change in cough in children with CF when treated with intravenous antibiotics for a respiratory exacerbation, and whether the children themselves were able accurately to perceive any change. Fourteen CF children (aged 13.6 +/- 2.6 years) were included in the study. All 14 children were simultaneously recorded with the LR 100 cough recorder and a conventional tape recorder during a chest physiotherapy session on the first or second day of admission for an infective exacerbation diagnosed by standard criteria. The difference between the two was an underestimate of 0.5 epoch/session by the tape recorder. Ten children were recorded on admission with the LR 100 cough recorder for a complete cycle (17 hr and 40 min), and we also assessed their day and night-time cough with cough scores and visual analogue scores (VAS). In 8 of these children, the same assessments were repeated on discharge. There was no significant correlation between any of the admission or discharge cough scores, daytime or night-time cough scores, and daytime or night-time VAS scores, with the actual number of coughs recorded on the LR 100 cough recorder. For the 8 children who had cough monitoring on admission and on discharge, there was no significant improvement in daytime or night-time cough scores or VAS on discharge, despite significant improvements in spirometry. There was also no significant improvement on daytime and night-time cough counts with the cough monitor on discharge, and no significant correlation with changes in lung function. There were weak correlations only between change in daytime VAS scores and change in forced expired volume in 1 sec (r = -0.794, P = 0.019) and forced vital capacity (r = -0.723, P = 0.04). In conclusion, we describe a reliable and well-tolerated method for obtaining cough counts objectively. The use of this objective method showed that CF children did not assess their cough frequency well. In addition, treatment of respiratory exacerbation improved neither subjective nor objective measures of cough in CF children.

Diaphragmatic paralysis after pediatric heart surgery: usefulness of non-invasive ventilation.

Diaphragmatic paralysis after cardiac surgery is an important complication especially in infants. We report a child who developed diaphragmatic paralysis, atelectasis, bronchomalasia and respiratory failure following cardiac surgery. Ventilatory support alleviated respiratory distress in this child. This report illustrates the usefulness of invasive and non-invasive ventilatory support for a pediatric patient with diaphragmatic paralysis.

Mother-daughter transmission of congenital central hypoventilation syndrome.

The cause of congenital central hypoventilation syndrome (CCHS) is unknown, but a genetic etiology is strongly suspected. We report a 25-year-old woman with CCHS (no Hirschsprung's disease) who gave birth to a daughter who also has CCHS. This suggests a dominant mode of inheritance for CCHS in this family. Pregnancy can be associated with physiologic challenges in CCHS. The increase in endogenous progesterone may stimulate breathing and may possibly improve symptoms of hypoventilation. Although this patient did not have any worsening in symptoms, her hyperoxic hypercapnic rebreathing ventilatory response was not different when pregnant versus when not pregnant. Ventilatory support for the patient was successfully managed with diaphragm pacing throughout the pregnancy without the need to adjust settings, despite the enlarged abdomen during pregnancy. We conclude that CCHS may be an inherited disorder. Increased endogenous progesterone during pregnancy has no effect on the ventilatory response, and diaphragm pacing can successfully provide adequate ventilation throughout pregnancy.

Long-term pulmonary sequelae in children who were treated with extracorporeal membrane oxygenation for neonatal respiratory failure.

OBJECTIVE: Extracorporeal membrane oxygenation (ECMO) is a life-saving therapy for neonates with intractable respiratory failure, but the long-term pulmonary outcome is unknown. Our aim was to investigate the long-term pulmonary sequelae of these children. STUDY DESIGN: We studied 50 children at 11.1 +/- 1.1 years (mean +/- SD) who had been treated with neonatal ECMO for meconium aspiration syndrome (38%), sepsis (18%), sepsis with pneumonia (12%), congenital diaphragmatic hernia (12%), congenital heart disease (8%), persistent pulmonary hypertension of the newborn (6%), and respiratory distress syndrome (4%) and 27 healthy controls (10.8 +/- 1.6 years). All subjects completed a respiratory questionnaire and performed pulmonary function and graded cardiopulmonary exercise testing. RESULTS: Neonatal ECMO survivors had hyperinflation (median residual volume: 131%), airway obstruction (median forced expired volume in 1 second: 79%), lower oxygen saturation with exercise, and lower peak oxygen consumption than controls. The ECMO group achieved similar exercise minute ventilation to controls, with more rapid and shallow breathing. ECMO survivors had an increased frequency of exercise-induced bronchospasm. Those who required higher inspired oxygen tension and ventilator pressures after weaning from ECMO had lower forced expired volume in 1 second and oxygen saturation values. CONCLUSION: Neonatal ECMO survivors experience lung injury lasting into later childhood. Lung dysfunction correlates with the extent and duration of barotrauma and oxygen exposure as neonates.

Clinical findings and lung pathology in children with cystic fibrosis.

Cystic fibrosis pulmonary disease is assessed by pulmonary function tests, arterial blood gases, and chest X-rays, but the correlation with lung pathology is unknown. We reviewed the clinical findings and lung pathology of 21 cystic fibrosis patients who had lung transplant. Pulmonary function tests, Brasfield scores, arterial blood gases, and age were correlated with lung pathology. All patients had severe Brasfield scores (9.0 +/- 3.2), airways obstruction (FEV1 25.6 +/- 5.6% predicted, FEF(25-75%) 11.0 +/- 4.5% predicted), and hyperinflation (residual volume [RV] 341.8 +/- 75.8% predicted). All patients were hypoxemic (PO2 64.2 +/- 8.2 mm Hg), and 5 of 21 (24%) were hypercapneic (PCO2 > 50 mm Hg). Pulmonary function tests and Brasfield scores were within a narrow range, and did not allow correlation with lung pathology. Small airway density (airways < 2 mm/cm2) decreased with increasing age. There were no differences in small airways inflammation and fibrous narrowing between the hypercapneic and nonhypercapneic patients, but the percent of smallest airways (airways < 0.35 mm) was significantly lower in the hypercapneic group. We conclude that there is significant correlation between airway pathology and increased age and CO2 retention. We speculate that decreased small airway density in older patients and the decreased proportion of smallest airways in hypercapneic patients is caused by increased dilatation of small airways.