Abstractresolved during or shortly after treatment. Significant changes were seen in mean Background -Patients with cystic fibrosis serum urea levels in both groups, but in have received more intravenous antibiotic only four patients to a level above the norcourses as median survival has steadily mal range, and in creatinine clearance in increased. A number of centres have adthe dual therapy group. At 24 month follow opted a policy of regular (three monthly) up no long term adverse consequences rather than on demand intravenous antifrom intravenous colistin were found in pseudomonal antibiotics. More widepatients who completed the study. spread bacterial antibiotic resistance has Conclusions -Intravenous colistin is an resulted from this increased antibiotic use.effective treatment for Pseudomonas Most Pseudomonas aeruginosa strains reaeruginosa associated pulmonary exmain fully sensitive to colistin but its use acerbations in patients with cystic fibrosis. has been resisted owing to concerns about Assessment of the individual effect of each neurotoxicity and nephrotoxicity. A study treatment regimen suggests a greater was carried out to assess the safety and efficacy when colistin is combined with a efficacy of intravenous colistin in the treatsecond antibiotic to which the pseudoment of acute respiratory exacerbations in monas shows in vitro sensitivity. Changes adult patients with cystic fibrosis.in renal function should be monitored. Methods -Patients with chronic Pseudo- (Thorax 1997;52:987-993) monas aeruginosa colonisation who presented with protocol defined respiratory Keywords: colistin, cystic fibrosis, Pseudomonas aerutract exacerbations were randomised to ginosa, nephrotoxicity, neurotoxicity. receive treatment for 12 days with either colistin (2 MU tds intravenously) alone or with a second anti-pseudomonal anti-Cohort survival curves from 1968 for children biotic. Comparisons of the absolute values born with cystic fibrosis show an increasing life of respiratory function tests on days 1, 5, expectancy with a median survival presently and 12 and of overnight oxygen saturation of about 29 years 1 2 and an expected median on days 1 and 12 were the primary outcome survival for today's children of 40 years.3 This measures. Patient's weight, clinical and success directly reflects better patient nutrition, chest radiographic scores, and peripheral better and individualised physiotherapy, and blood markers of inflammation were also the advent of effective anti-pseudomonal antidocumented. The effect of each treatment biotics. 4 More frequent antibiotic usage inregimen individually was assessed by the evitably has resulted in a greater prevalence change in clinical measurements from of bacterial antibiotic resistance and patient baseline values. Adverse renal effects were hypersensitivity reactions. 5-9Resistance of monitored by measurement of serum Pseudomonas aeruginosa to colistin is still unlevels of urea and electrolytes, creatinine usual 7 but cystic fibrosis specialist physicians clearance, and war...
Aim-To assess the potential for administering catch up and scheduled immunisations during hospital admission. Methods-Immunisation status according to the child's principal carer was checked against oYcial records for 1000 consecutively admitted preschool age children. Junior doctors were instructed to oVer appropriate vaccination before discharge, and consultants were asked to reinforce this proactive policy on ward rounds. Results-Excluding those children who were not fully immunised against pertussis through parental choice, 142 children (14.2%) had missed an age appropriate immunisation and 41 were due a scheduled immunisation. None had a valid contraindication. Only 43 children were oVered vaccination on the ward but uptake was 65% in this group. Conclusions-Admission to hospital provides opportunities for catch up and routine immunisations and can contribute to the health care of an often disadvantaged group of children. These opportunities are frequently missed. Junior doctors must be encouraged to see opportunistic immunisation as an important part of their routine work. (Arch Dis Child 1999;81:422-425)
Respiratory tract infection with eventual respiratory failure is the major cause of morbidity and mortality in cystic fibrosis (CF). Infective exacerbations need to be treated promptly and effectively to minimize potentially accelerated attrition of lung function. The choice of antibiotic depends on in vitro sensitivity patterns. However, physicians treating patients with CF are increasingly faced with infection with multidrug-resistant isolates of Pseudomonas aeruginosa. In addition, innately resistant organisms such as Burkholderia cepacia complex, Stenotrophomonas maltophilia and Achromobacter (Alcaligenes) xylosoxidans are becoming more prevalent. Infection with methicillin-resistant Staphylococcus aureus (MRSA) is also a problem. These changing patterns probably result from greater patient longevity and increased antibiotic use for acute exacerbations and maintenance care. Multidrug-resistant P. aeruginosa infection may be treated successfully by using two antibiotics with different mechanisms of action. In practice antibiotic choices have usually been made on a best-guess basis, but recent research suggests that more directed therapy can be achieved through the application of multiple-combination bactericidal testing (MCBT). Aerosol delivery of tobramycin for inhalation solution achieves high endobronchial concentrations that may overcome bacterial resistance as defined by standard laboratory protocols. Resistance to colistin is rare and this antibiotic should be seen as a valuable second-line drug to be reserved for multidrug-resistant P. aeruginosa. The efficacy of new antibiotic groups such as the macrolides needs to be evaluated.CF units should adopt strict segregation policies to interrupt person-to-person spread of B. cepacia complex. Treatment of panresistant strains is difficult and often arbitrary. Combination antibiotic therapy is recommended, usually tobramycin and high-dose meropenem and/or ceftazidime, but the choice of treatment regimen should always be guided by the clinical response.The clinical significance of S. maltophilia, A. xylosoxidans and MRSA infection in CF lung disease remains uncertain. If patients show clinical decline and are chronically colonized/infected with either of the former two pathogens, treatment is recommended but efficacy data are lacking. There are defined microbiological reasons for attempting eradication of MRSA but there are no proven deleterious effects of this infection on lung function in patients with CF. Various treatment protocols exist but none has been subject to a randomized, controlled trial. Multidrug-resistant microorganisms are an important and growing issue in the care of patients with CF. Each patient infected with such strains should be assessed individually and antibiotic treatment planned according to in vitro sensitivity, patient drug tolerance, and results of in vitro studies which may direct the physician to antibiotic combinations most likely to succeed.
We report late diagnoses of cystic fibrosis (CF) in two men aged 61 and 65 years. At the time of presentation, both patients had significant pulmonary disease. In each case two CFTR gene mutations were identified, including R117H on a background of a poly T genotype of 7T/9T. Patients with two identified CFTR mutations which include the R117H/7T anomaly should be followed up routinely as they remain susceptible to severe lung disease.
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