Pubertal growth and development in cystic fibrosis: a retrospective review.

AIM: Normal growth patterns are seen throughout the first decade in children with cystic fibrosis (CF). Growth in the second decade is, however, less satisfactory and may reflect pubertal delay. This study was performed to assess the extent of pubertal delay, to examine factors that influence the timing and magnitude of the pubertal growth spurt, and to establish whether the final height for most CF patients differed significantly from the normal population. METHODS: Thirty subjects (16 male) attending a single centre were studied. Peak height velocity (PHV), final height and ages when achieved were compared with population norms. Outcome data were correlated with disease severity using Shwachman and Chrispin-Norman scores and forced expiratory volume in 1 s. RESULTS: PHV was significantly later in both genders in this CF population compared with Tanner and Whitehouse standards: boys 14.6 y (95% confidence interval (95% CI) 12.4-16.8, p < 0.01) and girls 12.6 y (95% CI 10.5-14.7, p < 0.01). Mean PHV was also lower in both genders (boys 7.7 cm y(-1) and girls 6.4 cm y(-1), both p<0.001). However, final heights did not differ significantly from Freeman standards (height standard deviation scores: males--1.2, females--0.1); 52% of final heights equalled or exceeded the mid-parental centile. CONCLUSION: CF patients showed suboptimal PHVs with a later pubertal growth spurt influenced by disease severity, but eventually achieved a normal final height.

The pancreas in cystic fibrosis.

The pancreas secretes a bicarbonate-rich fluid containing digestive enzymes via the ampulla of Vater into the duodenum. Defective secretion leads to maldigestion of fat and protein with increased faecal losses. Cystic fibrosis (CF) is the major cause of pancreatic exocrine failure in childhood, whereas pancreatic insufficiency in adults is commonly associated with chronic pancreatitis and alcohol ingestion. In cystic fibrosis, pancreatic function correlates with genotype; pancreatic-sufficient (PS) patients have a milder course of respiratory disease, improved survival and lower mean sweat chloride concentrations than those with pancreatic insufficiency. Recent observations suggest that mutant CF alleles are over-represented in patients with chronic pancreatitis. Few show evidence of sino-pulmonary disease or high sweat electrolyte concentrations.

Elective versus symptomatic intravenous antibiotic therapy for cystic fibrosis.

BACKGROUND: Pseudomonas aeruginosa is the commonest micro-organism associated with respiratory infections in cystic fibrosis. Retrospective studies have suggested that survival is increased by using an aggressive policy of intravenous antipseudomonal antibiotics at regular intervals, irrespective of symptoms. OBJECTIVES: To determine whether there is evidence that an elective (regular) versus symptomatic intravenous antibiotic regime is associated with an improvement in clinical status and survival rates in patients with cystic fibrosis. To identify any adverse effects associated with the use of elective intravenous antibiotics, including an increase in the development of resistant organisms. SEARCH STRATEGY: The Cochrane Cystic Fibrosis and Genetics Disorders Group Specialist Trials Register was used. This comprises references identified from comprehensive electronic database searches, hand searching relevant journals and abstracts from conference proceedings. Date of the most recent search of the Group's specialised register: October 2000. SELECTION CRITERIA: All randomised or pseudo-randomised controlled trials describing the use of elective compared with symptomatic intravenous antibiotic policies for any duration or dose regimen. Elective versus symptomatic intravenous antibiotic regimes against any organisms were considered. Patients with cystic fibrosis were of any age or disease severity. DATA COLLECTION AND ANALYSIS: Trials were independently assessed for inclusion criteria, methodological quality and data extraction by the two reviewers. MAIN RESULTS: Three trials were identified by the initial search. Two trials reporting results from a total of 79 patients were included in the review. Differences in study design and objectives meant that data could not be pooled for meta-analysis. Neither trial demonstrated significant differences in outcome measures between intervention and comparison groups. REVIEWER'S CONCLUSIONS: Studies are insufficient to identify conclusive evidence favouring a policy of elective intravenous antibiotic administration, despite its widespread use. Neither are the potential risks adequately evaluated. The results should be viewed with caution as patient numbers are small. Clearly there is a need for a well-designed, adequately powered, multi-centred randomised controlled trial to evaluate these issues.