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.
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.
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.
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