Pulmonary function in infants with cystic fibrosis: the effect of antibiotic treatment.

Since 1982 all infants born within the East Anglian Regional Health Authority have been screened for cystic fibrosis. Between April 1985 and April 1992 infants identified in this way have been entered into a randomised prospective controlled trial of antibiotic prophylaxis. Approximately half the infants received continuous oral flucloxacillin and the remainder received antibiotics when clinically indicated. Infants underwent tests of respiratory function at 3-4 months and at 1 year of age. Measurements of thoracic gas volume and airway conductance were made with an infant whole body plethysmograph, and maximum expiratory flow by the 'squeeze' technique. A total of 73 tests was performed of 42 infants. To facilitate comparisons, measurements were expressed as scores. The mean values of the scores for the two groups of infants fell within normal limits. There was no difference between the treatment groups at either age. A reduction in airways conductance was observed between the two tests.     

Home intravenous antibiotic treatment of patients with cystic fibrosis

We report one-year's experience of home iv antibiotic treatment in 31 patients with cystic fibrosis chronically colonized with Pseudomonas aeruginosa. The patients were aged 4-67 years and had a mild to severe disease as indicated by a Shwachman score of 46-95 (mean 77). Ninety-two courses of iv antibiotic therapy were given (mean 3.0 per patient). The mean duration of the courses was 15.4 days. The entire antibiotic course, except for the first dose, was administered at home in 70% of the courses. Most patients (94%) were given a combined treatment of a beta-lactam and an aminoglycoside, administered by the patients themselves or their parents. One inserted venous cannula could be used for the whole treatment period in 30% of the courses. There were no complications. The clinical and bacteriological outcome was good to excellent in 89% of the courses, with temporary eradication or semi-quantitative decrease of Pseudomonas growth in sputum. Lung function (forced expiratory volume at 1 s) and blood gases improved significantly (p less than 0.001) and p less than 0.01, respectively). Most patients were able to attend work or school as usual, and 96% of the patients preferred this type of treatment to hospitalization. Apart from the psychosocial advantages, the economical savings were substantial. In comparison to traditional treatments in hospital (21 patients, 41 courses) home iv antibiotic treatment was safe and effective.     

Home intravenous antibiotic treatment in cystic fibrosis.

The prognosis for patients with cystic fibrosis who are colonised with Pseudomonas aeruginosa has improved as a result of the regular use of intravenous antibiotics; however, this necessitates long periods of hospitalisation. Home intravenous antibiotic treatment has potential advantages over hospital treatment. We describe our experience during the first 20 months of using a system of home intravenous antibiotic treatment in which a cystic fibrosis liaison sister has an essential role. Thirteen patients have received 40 courses of treatment. There were highly significant improvements in weight, respiratory function, and white cell count during home treatment. There was no significant difference in weight and forced expiratory volume in one second between the end of home treatment and the end of hospital treatment while forced vital capacity was better after home treatment. All patients preferred home treatment. The advantages of home visits by the cystic fibrosis liaison sister during treatment are emphasised.     

Energy metabolism in infants with cystic fibrosis

OBJECTIVE: To determine whether a defect in energy metabolism exists in infants with cystic fibrosis (CF). DESIGN: Unselected, newly-diagnosed subjects with CF (n = 46) and 24 healthy infants aged <20 weeks had measurements of resting energy expenditure (REE), total energy expenditure (TEE) (n = 25), and body composition. Metabolizable energy intake (MEI) was calculated. Genotype, energy intake, and pancreatic status was determined in all subjects with CF, and 24 underwent bronchial lavage. RESULTS: At diagnosis, infants with CF detected by newborn screening had significant anthropometric deficits (mean [SD] z-weight = 0.5 [1.0], z-length = 0.7 [1.3]) associated with pancreatic insufficiency. Their REE, TEE, or MEI (absolute measurements, per unit body weight or fat-free mass) were not increased. No relationship between REE, TEE, or MEI and Delta F(508) genotype, and no proportional differences in individual components of MEI between subjects with CF and controls, or between subjects with CF who were homozygotes or compound heterozygotes for Delta F(508) were observed. REE and TEE were not correlated with bronchial infection or inflammation. CONCLUSION: Growth impairment during the first weeks of life in infants with CF is associated with pancreatic insufficiency. However, there is no evidence for a defect of energy metabolism related to Delta F(508), and in infants with CF, minimal lung disease is unaccompanied by increased energy expenditure.     

Pulmonary function of the reserpine and isoproterenol models of cystic fibrosis

Two experimental animal models exhibiting functional and morphologic changes of exocrine glands similar to those seen in patients with cystic fibrosis (CF) have been reported in the rat: chronic stimulation with reserpine (Martinez et al. 1973 Pediatr. Res. 9:463, 470) and chronic stimulation with isoprenaline (Sturgess and Reid 1973 Br. J. Exp. Pathol. 54:388). We have studied the pulmonary function of these models induced by injecting rats subcutaneously with reserpine (RES, 0.5 mg/kg/day), isoproterenol (ISO, 25 mg/kg/day), or saline (Con, 1.0 ml/kg/day) for 6 days. Plethysmographic measurements were made for functional residual capacity (FRC), airways resistance (Raw), specific airways conductance (sGaw), phase difference between air flow rate and mean alveolar pressure (PD), frequency of breathing (f), and tidal volume (VT) of the anesthetized rats. In the RES and ISO rats, the FRC, Raw and f were not different from Con values. The PD was greater and the VT was less than Con values (p less than 0.05). The results of both studies indicate uneven ventilation (increased PD) and penduluft (decreased VT) consistent with maldistribution of resistance and/or compliances of the peripheral airways and/or alveolar compartments. These physiologic effects can be related to the morphologic changes reported for the airways of rats under chronic adrenergic stimulation (ISO) and expected for rats under chronic catecholamines depletion (RES). Since peripheral airways involvement is usually the earliest pulmonary lesion found in CF, these studies indicate that the RES and ISO models may be representative of the early pulmonary involvement of CF.     

Nocturnal oximetry in infants with cystic fibrosis

In a prospective study, 60% of admissions to an academic hospital were infected with HIV. HIV infected children were younger, less likely to have been referred, more likely to have pneumonia and candidiasis, and had more health service attendances. This impact may be alleviated by appropriate primary and secondary level health care.

     

Pulmonary surgery in cystic fibrosis

Cystic fibrosis (CF) has a variety of pulmonary manifestations that include pneumonia, pulmonary abscess, bronchiectasis, hemoptysis, and pneumothorax. Although newer therapies have greatly improved survival of patients with CF, surgical interventions for definitive treatment of these sequelae are often required. Several reports have illustrated that, with the current advances in the perioperative treatment and care of CF patients, surgical interventions for these pulmonary manifestations may be performed safely, resulting in a greatly improved quality of life. Also, although improvements in lung transplantation offer new hope for definitive treatment of those patients with cystic fibrosis, special considerations regarding other surgical issues, such as the prevalence of gastroesophageal reflux, need to be addressed.     

Pulmonary hemosiderosis in a child with cystic fibrosis

Two episodes of acute iron deficiency anemia with blood-stained sputum and symptoms of severe acute pulmonary exacerbation were observed in a child with cystic fibrosis (CF). Hemosiderin laden macrophages (siderophages) were repeatedly found in sputum and gastric juice, suggesting the coexistence of pulmonary hemosiderosis (PH). The possibility that pulmonary immune-mediated mechanisms characteristic of CF may have played a role in the development of PH is considered.