Aim-To determine how early diagnosis of cystic fibrosis, using neonatal screening, aVects long term clinical outcome. Methods-Fifty seven children with cystic fibrosis born before neonatal screening was introduced (1978 to mid 1981) and a further 60 children born during the first three years of the programme (mid 1981 to 1984), were followed up to the age of 10. The cohorts were compared on measures of clinical outcome, including height, weight, lung function tests, chest x-ray picture and Shwachman score. Results-Age and sex adjusted standard deviation scores (SDS) for height and weight were consistently higher in children screened for cystic fibrosis than in those born before screening. At 10 years of age, average diVerences in SDS between groups were 0.4 (95% CI −0.1, 0.8) for weight and 0.3 (95% CI −0.1, 0.7) for height. This translates to an average diVerence of about 2.7 cm in height and 1.7 kg in weight. Mean FEV 1 and FVC (as percentage predicted) were significantly higher in the screened cohort at 5 and 10 years of age, with an average diVerence of 9.4% FEV 1 (95% CI 0.8, 17.9) and 8.4% FVC (95% CI 1.8, 15.0) at 10 years. Chest x-ray scores were not diVerent between the groups at any age, but by 10 years screened patients scored an average 5.3 (95% CI 1.2, 9.4) points higher on the Shwachman score. Conclusion-Although not a randomised trial, this long term observational study indicates that early treatment made possible by neonatal screening may be important in determining subsequent clinical outcomes for children with cystic fibrosis. For countries contemplating the introduction of neonatal screening for cystic fibrosis, its introduction to some areas in a cluster randomised design will permit validation of studies performed to date. (Arch Dis Child Fetal Neonatal Ed 1999;80:F1-F7)
In children with mild-to-moderate CF, whilst statistically significant improvement in both LCI and peak VO(2) were seen, heterogeneity of response was evident. The most consistent improvement was seen in CFCS. Correlation of LCI and peak VO(2) with change in clinical score (CFCS) was seen. The full clinical significance of these changes in LCI and peak VO(2) needs to be evaluated further with additional variability data. The CFCS may be useful in the assessment of response to treatment in CF but requires formal validation.
Cough is a common and distressing symptom that results in significant health care costs from medical consultations and medication use. Cough is a reflex activity with elements of voluntary control that forms part of the somatosensory system involving visceral sensation, a reflex motor response and associated behavioural responses. At the initial assessment for chronic cough, the clinician should elicit any alarm symptoms that might indicate a serious underlying disease and identify whether there is a specific disease present that is associated with chronic cough. If the examination, chest x‐ray and spirometry are normal, the most common diagnoses in ADULTS are asthma, rhinitis or gastro‐oesophageal reflux disease (GORD). The most common diagnoses in CHILDREN are asthma and protracted bronchitis. Management of chronic cough involves addressing the common issues of environmental exposures and patient or parental concerns, then instituting specific therapy. In ADULTS, conditions that are associated with removable causes or respond well to specific treatment include protracted bacterial bronchitis, angiotensin‐converting enzyme inhibitor use, asthma, GORD, obstructive sleep apnoea and eosinophilic bronchitis. In CHILDREN, diagnoses that are associated with removable causes or respond well to treatment are exposure to environmental tobacco smoke, protracted bronchitis, asthma, motor tic, habit and psychogenic cough. In ADULTS, refractory cough that persists after therapy is managed by empirical inhaled corticosteroid therapy and speech pathology techniques.
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