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Omendra Narayan,1 Siobhan Davies,1 Carly Tibbins,2 JH Martyn Rees,3 Warren Lenney,1,4 Francis J Gilchrist1,4 1Academic Department of Child Health, Royal Stoke University Hospital, Stoke-on-Trent, 2West Midlands Medicines for Children Research Network, Royal Stoke University Hospital, Stoke-on-Trent, 3Department of Paediatrics, Royal Shrewsbury Hospital, Shrewsbury, 4Institute for Science and Technology in Medicine, Keele University, Guy Hilton Research Centre, Stoke-on-Trent, UK Abstract: Patient handheld records (PHHRs) promote self-management and empower the holder to take a more active role in the management of their disease. They have been used successfully in improving preventative care for children and have contributed to improved adherence in a number of chronic illnesses. Despite the potential advantages, there are no standard PHHRs for patients with cystic fibrosis (CF). We report the consultation process that led to the development of a CF PHHR, describe the final document, and analyze the feedback from their use at our center. We have made the CF PHHR freely available online.Keywords: cystic fibrosis, pediatrics, patient and public involvement, patient handheld records

Background: Chronic infection with Burkholderia cepacia complex (BCC) has a detrimental effect on morbidity and mortality for patients with cystic fibrosis (CF). It is therefore logical to attempt to eradicate new isolates however there is a paucity of information to guide treatment. We report the successful eradication of new isolates of BCC in two children with CF. Case presentation: Burkholderia cepacia was successfully eradicated in a 14 year old boy with CF and Burkholderia gladioli was successfully eradicated in a six year old girl with CF. In both children two weeks of intravenous (IV) tobramycin, ceftazidime and temocillin were used followed by three months of inhaled tobramycin. Bronchoalveolar lavage samples taken during flexible bronchoscopy were used prior to treatment to exclude spontaneous clearance as well as after treatment to confirm eradication. Conclusions: New isolates of BCC can be successfully eradicated in children with CF. More research is urgently required in this area to identify the best treatment regimen for BCC eradication. [ABSTRACT FROM AUTHOR]

Carroll WD, Lenney W, Child F, Strange RC, Jones PW, Fryer AA. Maternal glutathione S-transferaseGSTP1genotype is a specific predictor of phenotype in children with asthma.Pediatr Allergy Immunol 2005: 16: 32–39.© 2005 Blackwell MunksgaardMaternal factors are known to influence the heritability and expression of asthma and atopy. We report the association of maternal, paternal and probandGSTP1genotype with lung function in 145 Caucasian children with asthma.GSTP1Val105/Val105 and Ala114/Val114 genotypes in the child were associated with non-significant increases in lung function (forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC) and the FEV1/FVC ratio). Paternal genotype had no influence on lung function in the child. In contrast, maternalGSTP1Val105/Val105 genotype was significantly associated with offspring lung function and was strongly predictive of FEV1/FVC (Val105/Val105 105.2%, Ile105/Val105 and Ile105/Ile105 97.9% p = 0.006) and maternalGSTP1Ala114/Val114 genotype was associated with significantly higher FEV1 (Ala114/Val114 109.0%, Ala114/Ala114 99.0% p = 0.008), and FEV1/FVC ratios (Ala114/Val114 104.1%, Ala114/Ala114 98.2% p = 0.04). The associations between maternalGSTP1Val105/Val105 genotype and FEV1/FVC and maternalGSTP1Ala114/Val114 genotype and FEV1 remained significant (p = 0.003 and p = 0.007) after correction for child and maternal atopic status, passive smoke exposure, smoking during pregnancy, individual and paternalGSTP1genotype and was independent of transmission to the child. These data support the hypothesis that maternalGSTP1genotype can act as a specific risk factor which hasex uteroconsequences for children with asthma. As a child's genotype is not independent of maternal genotype, effects seen in candidate gene studies may be due at least in part to this phenomenon. [ABSTRACT FROM AUTHOR]

The use of oral prednisolone (2 mg/kg) to treat children admitted to hospital with acute asthma was assessed in a placebo controlled study. Children were further randomised to receive either 0.15 mg/kg salbutamol every 30 minutes for the first three hours of admission, or 5 mg salbutamol every one to four hours as needed. Treatment was double blind and the assessor was unaware of the nebuliser regimen given. Children were examined before and after treatment with salbutamol on arrival and reassessed four hours after admission. Seventy children completed the study. Seventeen (46%) of 37 children receiving prednisolone and six (9%) of 33 receiving placebo were fit for discharge after four hours of treatment. There was no significant difference between the two nebuliser regimens. Clinical parameters indicative of asthma severity were improved in all groups. Between group comparisons at reassessment showed higher peak flows in those receiving prednisolone and nebulisers every 30 minutes but differences were not significant for other parameters. Objective parameters indicating steroid efficacy over placebo were minimal. Despite this, those receiving prednisolone were more readily identifiable as being fit for discharge within four hours of treatment. [ABSTRACT FROM AUTHOR]

The treatment of severe asthmatics aged 1-3 years with budesonide administered via Nebuhaler and Laerdal facemask was assessed in a six month, double blind, placebo controlled study. Patients were randomised to budesonide (n = 20) or placebo (n = 20) treatment. Daily symptom scores were recorded by parents and clinic visits occurred every six weeks. Cough scores improved significantly with budesonide: nocturnal and daytime cough scores (0-2) fell by a mean (SEM) of 0.4 (0.2) and 0.5 (0.2); while with placebo the nocturnal score increased by 0.1 (0.2) and daytime score was unchanged at 0.0 (0.2). All other data showed improvements for active over placebo treatment. Thirteen children in each group completed six months' treatment. Parents of 25 of these found the delivery system convenient and easy to use. While blinded to treatment it was assessed to be beneficial in nine of 13 receiving budesonide and four of 13 receiving placebo. The Nebuhaler and facemask is an acceptable delivery system for long term asthma treatment. Symptoms are significantly reduced when it is used to give 400-800 micrograms budesonide/day to children aged 1-3 years who cannot use other delivery systems. [ABSTRACT FROM AUTHOR]

An eightfold rise in hospital admissions for acute asthma from 1971-85 prompted two studies to audit the admissions policy at the Royal Alexandra Hospital. In the first study the on call senior house officer (SHO) was replaced by an experienced registrar and over a four month period 53 children out of 158 were sent home from the receiving room compared with six out of 39 seen by the SHOs. In the second study an SHO training programme was established together with a home treatment package. Over a 12 month period the on call SHOs assessed 687 children with acute asthma; 229 (43.5%) were deemed fit to be sent home. Only seven of these were readmitted within one week. Diary symptom score cards filled in by parents indicated that children sent home without admission fared no worse at home than those admitted and then discharged for the two weeks after leaving hospital. The development of strategies to improve assessment and immediate management in the hospital receiving room can reduce hospital admissions for acute asthma, allowing more children to be safely managed in the community. [ABSTRACT FROM AUTHOR]

The admission rate for asthma at a children's hospital was studied over an 11 year period. Admissions varied unpredictably over periods of a few days, but there was a repeated yearly pattern of peaks and troughs with an interval of several weeks. The short term variation could be attributed to chance effects alone, excluding any important role for short term influences--for example, weather changes--in precipitating asthma admissions. There was a definite association between the longer term variation and school holidays. The admission rate fell during holidays and there were two or more peaks during terms. The pattern is consistent with a largely viral aetiology for asthmatic attacks throughout the year. We postulate that school holidays disrupt the spread of viral infections in a community, with synchronisation of subsequent attacks. Travel during holidays may facilitate acquisition of new viral strains by the community. [ABSTRACT FROM AUTHOR]

An eight fold rise in asthma admissions to the Royal Alexandra Hospital for Sick Children, Brighton occurred over the 15 year period 1971-85. The cause was an increase in the number of children seen, rather than increased readmission. The initial rise was associated with the introduction of nebulised salbutamol in 1976. The children concerned were mainly over 5 years old. Three years later came a larger influx of younger children. Circumstances leading to admissions were examined in a prospective study over a one year period. There were 605 admissions, 437 (72%) were self referrals. The commonest reason parents gave for preferring hospital treatment was the availability of nebuliser treatment. [ABSTRACT FROM AUTHOR]

Fifteen infants with birth weights below 1500 g, who required intravenous feeding from birth, were given one of two regimens differing only in their calcium and phosphate content. The duration of intravenous feeding varied from 26 to 75 days, and infants were studied after the age of 10 days when growth could be expected to have started. Five infants were given regimen A, which provided calcium and phosphate intakes of 0.55 and 0.44 mmol/kg/day, and 10 received regimen B, which provided intakes of 1.08 and 0.89 mmol/kg/day, respectively. Infants given regimen A had lower plasma and urine phosphate but similar urine calcium excretion to those given regimen B. Plasma calcium concentrations were higher in infants on regimen A than regimen B. In infants given regimen B plasma phosphate concentration was inversely related to weight gain, and urine phosphate and calcium concentrations were significantly correlated with urine sodium results. [ABSTRACT FROM AUTHOR]

Twenty nine young children with severe recurrent asthma were given nebulised beclomethasone dipropionate or normal saline in a double blind manner over a six month period. Progress was monitored using diary score cards. Those receiving beclomethasone had lower symptom scores, had more symptom free days, and required less additional treatment with bronchodilator agents. The code needed to be broken more frequently if normal saline was used. Over the study period height and weight increases in the two groups were similar, and no serious side effects were noted. [ABSTRACT FROM AUTHOR]

In this study on 31 asthmatic children, salbutamol powder was as effective as salbutamol aerosol in the older asthmatic child studied daily during a 6-week period. The rotahaler can be used successfully from 3 years and, as measured by the peak expiratory flow rate, salbutamol powder is effective in this young age group. [ABSTRACT FROM AUTHOR]

Using a modification of the forced oscillation technique we have measured respiratory resistance before and after nebulised salbutamol therapy in 32 wheezing children aged 7 months to 3 years 7 monts. 12 of the children were also studied in a total body plethysmograph. No child under 18 months of aged showed a greater than 5% fall in resistance or any fall in thoracic gas volume. In contrast, 18 of 20 children over 20 months of age showed a fall in resistance greater than 20%. [ABSTRACT FROM AUTHOR]

The bronchoconstrictor response to exercise was measured in 29 healthy children and in 23 children with recurrent wheezing attacks, using the Wright low-range peak flow meter and a modification of the forced oscillation technique. None of the healthy children showed significant change in airflow obstruction after a 6-minute run, whereas 20 of the 23 wheezy children showed a mean rise in total respiratory resistance of 65-5%. All 23 children showed a fall in airflow obstruction after inhaling salbutamol solution. [ABSTRACT FROM AUTHOR]

Comments on several issues related to health services in Great Britain. Importance of eye protectors on reducing facial injuries; Use of home nebulizers by children with asthma; Effects of hormone therapy in general practice.