Bone status in cystic fibrosis.

AIM: To investigate bone mineral status of children with cystic fibrosis (CF). METHODS: In 29 children with CF and 49 matched controls, bone mineral content (BMC), projected bone area (BA), and areal bone mineral density (BMD) of the whole body, total hip, and lumbar spine (L1-L4) were measured using dual energy x ray absorptiometry. The BMC values at each site were adjusted for BA, height, and weight. At the lumbar spine, the bone mineral apparent density (BMAD) was calculated by dividing the BMC by the estimated volume, derived from BA. Vertebral (T12-L3) trabecular bone mineral density (vTBMD) was measured using quantitative computed tomography in children with CF. Calcaneal broadband ultrasound attenuation (BUA) was measured in CF patients and controls using quantitative ultrasound. The disease severity of CF children was evaluated by the Shwachman-Kulczycki (SK) score. RESULTS: The mean BUA, whole body and regional BA, adjusted BMC, and areal BMD of children with CF were not different from those of controls. The mean BMAD of the lumbar spine was reduced in CF patients compared with controls, whereas the mean vTBMD standard deviation scores were significantly higher in CF patients. The median SK score of the CF group was 81 (range 42-100), indicating that as a group our CF patient population had relatively mild disease. CONCLUSION: The normal vertebral BMC, decreased BMAD, and higher vTBMD suggests that the vertebral cortical thickness or density might be reduced in CF subjects. The overall bone mineral status of CF children with relatively mild disease was not different from size matched controls.

Bone density in asthmatic children treated with inhaled corticosteroids.

Volumetric trabecular bone mineral density of the lumbar spine (vTBMD) and distal radius (rTBMD) were measured in 20 prepubertal white asthmatic children treated with moderate to high doses of inhaled corticosteroids. The median standard deviation score for vTBMD (0.20, -0.56 to 2.09) and rTBMD (-0.04, -0.82 to 1.39) were within the normal range.

The use of human glutathione S-transferase A1 in the detection of cystic fibrosis liver disease.

OBJECTIVE: To determine the value of serum human glutathione S-transferase A1 (hGST A1) in the detection of cystic fibrosis liver disease (CFLD). METHODS: Sixty-three children (aged 0.5-16 years) with cystic fibrosis (CF) were screened prospectively for evidence of hepatobiliary abnormalities between February 1993 and February 1996. Comparison was made between clinical examination, abdominal ultrasonic scan, measurement of conventional liver enzymes (LFTs) and serum hGST A1 concentration in the detection of hepatobiliary abnormalities in children with CF. RESULTS: The 5-95% concentration of serum hGST A1 was 1.7-4.27 micrograms L-1 for the control group. The hGST A1 levels in the CF patients were significantly higher than in the non-CF group. Thirty-eight (60%) children had detectable hepatobiliary abnormalities. Ultrasound scanning detected the highest number of abnormalities (41%), followed by hGST A1 (30%). The presence of clinical liver disease was found in 19% of the children. The estimated sensitivities of detecting CFLD by clinical method, ultrasound scan, serum hGST A1, and LFTs would be 32%, 68%, 50% and 16%, respectively. CONCLUSIONS: Serum hGST A1 measurement increases the sensitivity of detecting hepatic abnormalities when included with clinical and ultrasound evaluation although, in some cases with advanced liver disease, serum hGST A1 may be normal. Conventional liver enzyme tests add little information in the detection of CF liver disease.

Cystic fibrosis mutations in Romania.

UNLABELLED: We have investigated the genotype in 32 children with cystic fibrosis from Romania. The diagnosis of cystic fibrosis was made on the basis of typical clinical findings and sweat electrolyte levels using the pilocarpine iontophoresis method. CONCLUSION: Genetic analysis of 32 children with cystic fibrosis from Romania showed a 25% incidence of DeltaF508 mutation and a 64.5% incidence of unknown mutations, 5 other known mutations and 1 new mutation 1,717-2(A > G).

Evaluation of two commercial jet nebulisers and three compressors for the nebulisation of antibiotics.

Nebulised antibiotics have been shown to be beneficial in the treatment of lung infections in cystic fibrosis. Studies on the efficiency of nebuliser systems are constantly required in view of the large number of compressor/drug/nebuliser combinations which are possible and the development of new systems and drugs. Six combinations of three commercially available compressors were compared (PortaNeb 50 (Medic-Aid; 5.4-6.1 l/min), Turboneb (Medix; 8.3-9.1 l/min), and CR 60 (Medic-Aid; 7.3-7.8 l/min)) and two jet nebulisers (Microneb III (Lifecare) and System 22 Acorn (Medic-Aid)) for the nebulisation of colomycin, gentamicin, and ciprofloxacin. Aerosol droplet size, nebulisation time, and aerosol output were determined. Turboneb and CR 60 reduced the nebulisation time and produced higher proportions of 'respirable' (< 5 microns diameter) antibiotic aerosols. The residual volume of the Microneb III was lower than that of the System 22 Acorn. It was found that the Turboneb and CR 60, when coupled with either Microneb III or System 22 Acorn, were suitable for the nebulisation of all three antibiotics. Of the equipment tested, Turboneb coupled with Microneb III was the most efficient combination. Even with this combination, only around 50% of the nominal dose was released as respirable aerosol.

The chest radiograph in cystic fibrosis: a new scoring system compared with the Chrispin-Norman and Brasfield scores.

BACKGROUND: Scoring systems for the chest radiograph in cystic fibrosis are used to compare patients and different treatment regimens, and to monitor respiratory disease in individual patients. The Northern chest radiograph score was designed to allow one person to assess the radiological features of lung involvement in cystic fibrosis in as simple, rapid and equally reproducible manner as the established Chrispin and Norman, and Brasfield scoring systems. METHODS: Forty five chest radiographs were scored by 10 physicians with a special interest in cystic fibrosis according to the Brasfield and Northern methods, and by five pairs of physicians according to the Chrispin-Norman criteria. Three individuals and two pairs rescored the radiographs after an interval of 3-5 months. The Northern score was performed with and without a lateral view, using the original posteroanterior radiograph. RESULTS: The Northern score showed a better agreement between observers for the ranking of the radiographs. It was equally well related to respiratory function tests, the Shwachman-Kulczycki score of overall clinical status, and in its discrimination between different radiographs as the other two scoring systems. The Northern score performed equally well with or without a lateral film. CONCLUSIONS: The Northern system fulfils the requirements of a chest radiograph score more successfully than the Chrispin-Norman or Brasfield systems, and does not require a lateral film.

Delta F508 testing of the DNA bank of the Royal Manchester Children's Hospital.

Details of haplotype and delta F508 status from various populations represented in the cystic fibrosis (CF) DNA bank of the Royal Manchester Children's Hospital are provided, together with information on the association of genotype and clinical status. Clinical details and DNA analyses from native English in the North-West and South-West of England (Bath), from Lancashire Pakistani families and from Afrikaans Namibian families are compared. A 78.5% incidence of delta F508 has been found in English families. Compound heterozygotes with CF and only one delta F508 gene have an increased likelihood of having milder disease, with less Pseudomonas isolated from sputum and relatively more showing either no regular respiratory pathogens or colonisation with Staphylococcus. There is also a relative increase in meconium ileus in these compound heterozygotes. The diagnosis of CF may be in doubt in some subjects negative for delta F508. Some of the Bath families have unusual haplotypes for an English population and a compound heterozygote delta F508/delta I507 has been found. There is evidence from metD analysis of the founder effect in the Afrikaans Namibian families, who have a high delta F508 incidence.

The distribution of lung damage in children with cystic fibrosis and its relationship to colonisation with Pseudomonas aeruginosa.

Ventilation-perfusion lung scans were performed with 81mKr inhalation and 99mTc-albumin injection in 33 children with cystic fibrosis. It was found that both ventilation and perfusion scans yielded more information than radiographs of the chest in assessing lung damage. Using a scoring system, it was demonstrated by statistical methods that in the worst affected patients the disease was worse in the upper zones of the lungs, which changes were not detectable on chest radiographs. Pseudomonas was present in the sputum of these patients.

Is the combination inhaler of salbutamol and beclomethasone dipropionate as effective as the same agents from separate inhalers in the management of childhood asthma?

A double-blind crossover study lasting 16 weeks was carried out in children with chronic extrinsic asthma to compare the clinical effects of regular inhalations of salbutamol and beclomethasone dipropionate from a combination inhaler and regular inhalations of the same two drugs used sequentially from separate inhalers. Eighteen patients entered the study and 16 completed both 8-week treatment periods. However, there was a large amount of missing data, with only 9 complete sets of paired data; efficacy analyses were performed on data from 10 patients on separate inhaler therapy and from 14 patients on combination inhaler therapy. There were no significant differences between daily peak flow rate measurements, symptom scores, additional symptomatic bronchodilator therapy, acute exacerbations of asthma or incidence of adverse events, demonstrating that both treatments were similarly effective in controlling the patient's asthmatic symptoms. There was, however, a trend for higher peak flow values and lower symptom scores on separate inhaler therapy in this small group of asthmatic children. Although the results suggest that regular therapy using a combination inhaler is as effective as sequential administration of salbutamol and beclomethasone dipropionate from separate inhalers, it is concluded that the study should be extended to a larger group of patients to determine whether there might be any statistically significant differences between the two regimens.

Use or abuse of phototherapy for physiological jaundice of newborn infants.

To investigate the need for and effects of phototherapy in full-term otherwise healthy babies with physiological jaundice, 40 consecutive babies with serum bilirubin levels of 250 mumol/l or more were assigned at random to two treatment groups. Phototherapy was started in the early group (n = 20) when serum bilirubin was 250 mumol/l and in the late group (n = 20) when serum bilirubin reached 320 mumol/l; however, only 3 of the late group required treatment. Phototherapy prevented a further rise in bilirubin in almost all treated babies, but the difference in peak bilirubin level between early and late treatment groups was not significant. Early phototherapy produced a more rapid decline in bilirubin; levels fell to below 250 mumol/l in a median of 28 h with early treatment and 54 h with late treatment. In each group the ratio of boys to girls was 2/1 and boys remained jaundiced for significantly longer. Phototherapy therefore curtailed the rise and duration of hyperbilirubinaemia, but the effect was small. Jaundice subsided spontaneously in most of these mature infants, especially girls. Phototherapy can separate mother from baby, and it is physiologically stressful. Treatment may be safely withheld until serum bilirubin exceeds 320 mumol/l.

Comparison of IV salbutamol with IV aminophylline in the treatment of severe, acute asthma in childhood.

A double-blind test to compare IV aminophylline with salbutamol in the treatment of acute, uncontrolled asthma showed that both drugs were equally effective during the first 24 hours. Salbutamol caused a relative tachycardia. Hydrocortisone was given after 2 hours, but did not appear to affect the rate of recovery.