Lung function from infancy to school age in cystic fibrosis.

The aim was to investigate pulmonary mechanics in patients with cystic fibrosis during infancy and again in early childhood to see whether infant tests predicted status at school age. Plethysmographic measurements of thoracic gas volume and airways resistance were made in 29 patients at 6 months and again at 5 years 10 months. Maximum flow at functional residual capacity was measured during infancy for comparison with maximum expiratory flow rates during childhood. While many patients had normal measurements during infancy, pulmonary function had deteriorated by school age. Thoracic gas volume at school age was significantly related to the values in infancy, but other measurements made during childhood were independent of infant values. The relations between measurements in infancy and early childhood described here provide a background against which the role of external factors on pulmonary function in young children with cystic fibrosis can be investigated.     

Physical activity and self treatment in cystic fibrosis.

A programme of increased physical activity and self treatment based on the forced expiration technique was introduced to 14 adolescent patients with cystic fibrosis, diagnosed since infancy or early childhood, who had gastrointestinal and pulmonary symptoms. Twelve patients who fulfilled the one year programme had unchanged clinical conditions, pulmonary function tests, and chest x ray films. There was a significant improvement in oxygen pressure correlated to the period of increased physical activity. Other blood gas variables, including pH, standard bicarbonate, and base excess, also indicated significant changes, mainly during exercise. The study shows that daily physical activity improves the pulmonary gas exchange and that self treatment combined with physical activity is as efficient as conventional physiotherapy.     

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.     

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.     

Respiratory function in childhood following repair of oesophageal atresia and tracheoesophageal fistula.

AIM: To determine the relation between respiratory function in infancy and at school age in children who have undergone oesophageal atresia and tracheoesophageal fistula repair, and assess the value of infant respiratory function testing; and to examine the effect of bronchodilators. METHOD: Fourteen children (6 girls, and 8 boys) who had undergone respiratory function testing in infancy were retested at school age (7-12 years). Measurements included lung volume, airways resistance, peak flow, and spirometry. Clinical problems were investigated by questionnaire. Twelve children had repeat measurements after taking salbutamol. RESULTS: Predominant complaints were non-productive cough and dysphagia, but even those children with major problems in infancy reported few restrictions at school or in sport or social activities. Respiratory function and clinical findings at school age appeared unrelated to status in infancy, such that even the patients with severe tracheomalacia requiring aortopexy did not have lung function testing suggestive of malacia at school age. Most patients showed a restrictive pattern of lung volume which would appear to result from reduced lung growth after surgery rather than being a concomitant feature of the primary congenital abnormality. Although six children reported wheeze and four had a diagnosis of asthma, only one responded to salbutamol. This suggests that a tendency to attribute all lower respiratory symptoms to asthma may have led to an overdiagnosis of this condition in this patient group. CONCLUSION: Respiratory function testing in infancy is of limited value in medium term prognosis, but may aid management of contemporary clinical signs. In children respiratory function testing is valuable in assessing suspected asthma and effects of bronchodilators.     

Serum immunoreactive trypsin and pancreatic lipase in cystic fibrosis

Serum immunoreactive trypsin (IRT) and pancreatic lipase have been measured in 59 patients with cystic fibrosis (age 1 month-27 years). Follow-up values were obtained from 49 patients. Their serum enzyme levels were compared to those of 120 healthy children of all age groups. Faecal fat excretion was determined in selected patients (n=23) to elucidate the relationship between serum enzyme levels and pancreatic exocrine function.In cystic fibrosis IRT and lipase showed a very similar agecorrelated pattern: in infancy levels were markedly elevated. During the following years the concentrations of both enzymes decreased rapidly and were found to be far below the normal range after the 10th year of life. Elevated enzyme levels in infancy as well as low levels in all age groups coincided with steatorrhaea. Older patients (11–27 years) without severe pancreatic insufficiency however, had IRT and lipase levels in or above the normal range.In healthy children there was no age dependency of IRT levels, whereas in the first 12 months of life lipase levels were significantly lower than in later childhood.Abbreviations IRT Serum immunoreactive trypsin - CF Cystic fibrosis     

Glutaric aciduria and suspected child abuse

AIM—To determine the relation between respiratory function in infancy and at school age in children who have undergone oesophageal atresia and tracheoesophageal fistula repair, and assess the value of infant respiratory function testing; and to examine the effect of bronchodilators.METHOD—Fourteen children (6 girls, and 8 boys) who had undergone respiratory function testing in infancy were retested at school age (7?12 years). Measurements included lung volume, airways resistance, peak flow, and spirometry. Clinical problems were investigated by questionnaire. Twelve children had repeat measurements after taking salbutamol.RESULTS—Predominant complaints were non-productive cough and dysphagia, but even those children with major problems in infancy reported few restrictions at school or in sport or social activities. Respiratory function and clinical findings at school age appeared unrelated to status in infancy, such that even the patients with severe tracheomalacia requiring aortopexy did not have lung function testing suggestive of malacia at school age. Most patients showed a restrictive pattern of lung volume which would appear to result from reduced lung growth after surgery rather than being a concomitant feature of the primary congenital abnormality. Although six children reported wheeze and four had a diagnosis of asthma, only one responded to salbutamol. This suggests that a tendency to attribute all lower respiratory symptoms to asthma may have led to an overdiagnosis of this condition in this patient group.CONCLUSION—Respiratory function testing in infancy is of limited value in medium term prognosis, but may aid management of contemporary clinical signs. In children respiratory function testing is valuable in assessing suspected asthma and effects of bronchodilators.     

Measurement of fat digestion in early life using a stable isotope breath test

13C breath tests are a safe, non-invasive way of assessing nutrient digestion and absorption that can be used repeatedly in infancy and childhood. The aim of this study was to assess their value for measuring fat digestion in infants and young children with cystic fibrosis, and healthy controls whose pancreatic exocrine function is immature, and to monitor pancreatic enzyme supplementation. Six infants with cystic fibrosis (aged 10-18 months) and nine healthy controls (aged 6-19 months) were studied. After an overnight fast each child ingested 7.5 mg/kg 13C trioctanoin (99 atom % excess) followed by a known volume of milk. Breath samples were collected before and at 30 minute intervals thereafter for five hours. The 13C enrichment of expired carbon dioxide was measured by gas isotope ratio mass spectrometry. The mean (SD) percentage dose recovery of 13C was 13.5 (5.3) for the cystic fibrosis group and 24.2 (6.7) for the healthy controls. When those with cystic fibrosis were studied after supplementary pancreatic enzymes, the mean percentage dose recovery rose to 17.1 (6.9). Total intraluminal lipolysis was diminished by 44% in young children with cystic fibrosis. Pancreatic enzyme supplements improved digestion by 27%. The 13C trioctanoin breath test was effective in detecting fat maldigestion and can be used to measure the benefits of enzyme supplements in early life.     

Measurement of fat digestion in early life using a stable isotope breath test.

13C breath tests are a safe, non-invasive way of assessing nutrient digestion and absorption that can be used repeatedly in infancy and childhood. The aim of this study was to assess their value for measuring fat digestion in infants and young children with cystic fibrosis, and healthy controls whose pancreatic exocrine function is immature, and to monitor pancreatic enzyme supplementation. Six infants with cystic fibrosis (aged 10-18 months) and nine healthy controls (aged 6-19 months) were studied. After an overnight fast each child ingested 7.5 mg/kg 13C trioctanoin (99 atom % excess) followed by a known volume of milk. Breath samples were collected before and at 30 minute intervals thereafter for five hours. The 13C enrichment of expired carbon dioxide was measured by gas isotope ratio mass spectrometry. The mean (SD) percentage dose recovery of 13C was 13.5 (5.3) for the cystic fibrosis group and 24.2 (6.7) for the healthy controls. When those with cystic fibrosis were studied after supplementary pancreatic enzymes, the mean percentage dose recovery rose to 17.1 (6.9). Total intraluminal lipolysis was diminished by 44% in young children with cystic fibrosis. Pancreatic enzyme supplements improved digestion by 27%. The 13C trioctanoin breath test was effective in detecting fat maldigestion and can be used to measure the benefits of enzyme supplements in early life.     

Retrospective review of cystic fibrosis presenting as infantile liver disease.

The mode of presentation, clinical course, and outcome of 12 infants with cystic fibrosis and liver disease referred over an 18 year period were investigated retrospectively. Median age at presentation was 6.5 weeks (range, 5-12). Two thirds were boys. Conjugated hyperbilirubinaemia was the presenting symptom in 11 patients, and hypoalbuminaemia in one. Jaundice was cleared over a median period of 7.36 months. Eight patients had bile duct proliferation on liver biopsy and one required cholangiography to exclude biliary atresia. Classic histological features of cystic fibrosis were only present in two children biopsied at 8 and 18 months. Three patients had meconium ileus, including one infant with concomitant alpha(1) antitrypsin deficiency, who required early liver transplantation. All other patients had no signs of significant chronic liver disease during a median follow up of 42 months (range, 10-205). Children with cystic fibrosis and infantile liver disease have a good short and medium term prognosis.     

Treatment and Prevention of Pulmonary Complications of Cystic Fibrosis in Infancy and Early Childhood

Seventy-six patients with cystic fibrosis seen between 1950 and 1964 were studied, all having been first seen before the age of 1 year. In retrospect, the year 1957 seemed to be a watershed in the treatment of this series of patients, and they were therefore divided into 2 groups. Group A, 30 patients seen before 1957; Group B, 46 patients seen after 1957. The clinical, radiological, and bacteriological findings in the 2 groups at entry were similar as to age of incidence, severity of the initial respiratory symptoms, abnormalities on chest radiographs, and bacteriology of the cultures of cough swabs. Treatment of Group B patients was generally more vigorous, with antibiotics used more efficiently to treat pulmonary infection.When the patients were followed up and their pulmonary status was assessed clinically and radiologically, at 1 year and at 5 years of age, Group B patients were found to be significantly less severely affected than Group A patients.It is concluded that vigorous treatment of cystic fibrosis can reduce mortality in infancy, and considerably improve the pulmonary status during childhood.     

Serial lung function studies in cystic fibrosis in the first 5 years of life.

Lung mechanics were studied in 8 infants with cystic fibrosis at 6 months of life and radiosotopic lung function was measured in 5 of them at 5 years of age. The children who were initially asymptomatic had normal lung mechanics in infancy but the 2 restudied later had abnormal radioisotopic lung function. The symptomatic children showed abnormalities in infancy and more marked changes later. It is concluded that the lungs in cystic fibrosis are probably normal initially and that damage occurs later even in the absence of symptoms.     

Serial lung function studies in cystic fibrosis in the first 5 years of life

Lung mechanics were studied in 8 infants with cystic fibrosis at 6 months of life and radiosotopic lung function was measured in 5 of them at 5 years of age. The children who were initially asymptomatic had normal lung mechanics in infancy but the 2 restudied later had abnormal radioisotopic lung function. The symptomatic children showed abnormalities in infancy and more marked changes later. It is concluded that the lungs in cystic fibrosis are probably normal initially and that damage occurs later even in the absence of symptoms.     

Wheezing in infants with cystic fibrosis: clinical course, pulmonary function, and survival analysis.

Wheezing is a common finding in infants with cystic fibrosis (CF). This study was undertaken to determine the prevalence of wheezing in infants with CF and to compare the clinical outcome of those who wheezed in infancy with that of those who did not. The study cohort included 229 CF patients born between 1965 and 1979 with CF diagnosed before 2 years of age. Fifty-seven (25%) had physician-documented wheezing during the first 2 years of life. Wheezing had resolved by the age of 2 years in 50% of the patients and by the age of 4 years in 75%. Although wheezing seemed to be linked to a family history of allergy and asthma, the frequency of the delta F508 mutation was similar to that of the non-wheezers. There was no significant difference in survival at the age of 13 years between the two groups. At the age of 7 years, patients who had wheezed had significantly lower forced expiratory flow rate at mid-expiratory phase (85 +/- 34% predicted) compared with those with no wheezing history (101 +/- 34% predicted). At the age of 13 years, forced expiratory volume in 1 second values was lower in the wheezing group (69 +/- 24% predicted vs 78 +/- 21% predicted), as was forced expiratory flow rate at mid-expiratory phase (56 +/- 33% predicted vs 69 +/- 30% predicted). In conclusion, although wheezing in infants with CF seems to have diminished with age, pulmonary function abnormalities were more evident at 7 and 13 years of age in the group that wheezed than in the group that did not.     

Prospective controlled study of home and hospital therapy of cystic fibrosis pulmonary disease

A controlled prospective study was undertaken to compare the efficacy and benefits of home and hospital treatment for patients with exacerbations of pulmonary disease caused by cystic fibrosis. A total of 41 home and 41 hospital treatments were analyzed. Home and hospital patients were matched according to sex, age, pulmonary function tests, and arterial blood gas values. Both home and hospital treatments resulted in statistically significant improvement in pulmonary function. A comparison of these values did not show any statistically significant difference between groups at admission or discharge. Furthermore, the mean number of treatment days for both groups, individually determined by the primary physician, was equivalent (home 17.7 +/- 1.1 days, hospital 18.1 +/- 4.1). The mean charge for a home treatment was approximately $10,000, and for a hospital treatment $18,000. Sixty-five percent of home care patients and 68% of hospital patients required retreatment for pulmonary exacerbations within the study period; the interval between pulmonary exacerbations for the two groups was not significantly different. In addition, 85% of patients receiving treatment at home were able to maintain at least some of their school or work activities. These data indicate that home therapy for cystic fibrosis patients with pulmonary exacerbations is less costly and is as effective as in-hospital therapy.     

Retrospective review of cystic fibrosis presenting as infantile liver disease

The mode of presentation, clinical course, and outcome of 12 infants with cystic fibrosis and liver disease referred over an 18 year period were investigated retrospectively. Median age at presentation was 6.5 weeks (range, 5-12). Two thirds were boys. Conjugated hyperbilirubinaemia was the presenting symptom in 11patients, and hypoalbuminaemia in one. Jaundice was cleared over a median period of 7.36 months. Eight patients had bile duct proliferation on liver biopsy and one required cholangiography to exclude biliary atresia. Classic histological features of cystic fibrosis were only present in two children biopsied at 8 and 18 months. Three patients had meconium ileus, including one infant with concomitant α₁ antitrypsin deficiency, who required early liver transplantation. All other patients had no signs of significant chronic liver disease during a median follow up of 42 months (range, 10-205). Children with cystic fibrosis and infantile liver disease have a good short and medium term prognosis.

     

Emerging developmental sequelae in the 'normal' extremely low birth weight infant.

Thirty-six extremely low birth weight (less than 1000 g birth weight) children received neurodevelopmental testing in infancy (mean age = 19.1 months), and again in early childhood (mean age = 46.5 months). Children were categorized into a high-risk group (n = 20) if bronchopulmonary dysplasia and/or Grades III or IV intracranial hemorrhage were present or a low-risk group (n = 16) if neither were present. Using standardized testing and neuromotor examination, 24 (67%) of 36 children showed normal infant development. Only 11 (31%) of 36 children (P less than .005) had normal development upon reassessment in early childhood. A decline in developmental status occurred in both groups. This indicates that for the extremely low birth weight population, normal infant development is poorly predictive of continued normal development. With or without major complications, extremely low birth weight places children at substantial risk for ongoing and emerging developmental problems with age.     

Benefits of breastfeeding in cystic fibrosis: a single-centre follow-up survey

AIM: To study the effect of breastfeeding (BF) on growth, lung function and number of infections during the first 3 years of life in children with cystic fibrosis (CF). MATERIAL AND METHODS: One hundred forty-six CF patients, 5-18 years old, were recruited at their annual care visit. Information about infant feeding, psychosocial and socioeconomic conditions and smoking exposure was obtained by interviews. Anthropometric parameters at 1 year of age and the number of infections and hospitalisations during the first 3 years of life were obtained from clinical charts. Anthropometrics and pulmonary function parameters were obtained at enrollment. RESULTS: In CF patients, particularly those with pancreatic insufficiency, the prevalence of BF was lower than the general Italian population. After multivariate analysis patients with prolonged BF showed higher values of CED expiratory volume in 1 sec (FEV-1) (p = 0.001) and a lower number of infections during the first 3 years of life (p = 0.098). CONCLUSION: Prolonged BF is beneficial in children with CF and may protect them against decline of pulmonary function. Particular attention should be paid to promote BF in infants with CF.     

Serum factor in cystic fibrosis: correlation with clinical parameters.

The relationship between the activity of the cystic fibrosis serum ciliary dyskinesia factor, detected by the rabbit tracheal bioassay, and clinical status of the patient has been investigated in children, 1-24 years old, with cystic fibrosis. No significant correlation was found between the amount of serum factor activity and age, clinical status assessed by the Schwachman score, pulmonary function (vital capacity, functional residual capacity, total lung capacity, residual volume, maximum breathing capacity, maximal midinspiratory flow, and maximal midexpiratory flow) or blood gas levels (pCO2, pO2). The activity showed no significant relationship to serum galactosyltransferase activity in children with cystic fibrosis.     

Study of lung involvement in patients with cystic fibrosis

OBJECTIVE: To characterize the involvement of the respiratory apparatus of patients with cystic fibrosis in order to obtain a comprehensive view of their pulmonary picture.METHODS: Data were obtained retrospectively from the medical records of 16 patients with cystic fibrosis; arterial gas and spirometry data were obtained prospectively for the same patients, who were not in an acute pulmonary situation. The patients were subjects of both sexes aged 6 years or older who were followed up at the Pediatrics Outpatient Clinic of the University Hospital, Faculty of Medicine of Ribeir?o Preto, USP.RESULTS: Median patient age was 114 months (9 years and 6 months) ranging between 72 - 360 months, and 68.75% were males. Productive cough was the most frequent symptom observed in 75% of the population studied. All patients had positive sputum culture obtained at least one year before, with Pseudomonas aeruginosa being detected in 81.25% of the cases. Arterial gases revealed some abnormalities in 81.25% of the patients and spirometry revealed abnormalities in 56.25%.CONCLUSION: All patients presented at least one type of pulmonary alteration. Measurement of arterial gases detected a larger number of patients with altered pulmonary function than did spirometry, but the two examinations complemented each other for a good evaluation of pulmonary function.