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1.  Bone status in cystic fibrosis 
Archives of Disease in Childhood  2001;84(6):516-520.
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.


doi:10.1136/adc.84.6.516
PMCID: PMC1718803  PMID: 11369574
2.  Bone density in asthmatic children treated with inhaled corticosteroids 
Archives of Disease in Childhood  2001;84(2):183-184.
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.


doi:10.1136/adc.84.2.183
PMCID: PMC1718669  PMID: 11159303
3.  The chest radiograph in cystic fibrosis: a new scoring system compared with the Chrispin-Norman and Brasfield scores. 
Thorax  1994;49(9):860-862.
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.
PMCID: PMC475170  PMID: 7940422
4.  Nebulizers in cystic fibrosis. 
Journal of the Royal Society of Medicine  1996;89(Suppl 27):14-18.
PMCID: PMC1295620  PMID: 8778444
6.  Evaluation of two commercial jet nebulisers and three compressors for the nebulisation of antibiotics. 
Archives of Disease in Childhood  1994;71(4):335-338.
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.
PMCID: PMC1030014  PMID: 7979528
11.  Comparison of IV salbutamol with IV aminophylline in the treatment of severe, acute asthma in childhood. 
Archives of Disease in Childhood  1979;54(5):391-392.
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.
PMCID: PMC1545558  PMID: 383024
12.  Cutaneous and hepatic haemangiomata. 
Archives of Disease in Childhood  1977;52(2):155-157.
A 3-month-old infant presented with multiple cutaneous haemangiomata, and was found to have a cardiac murmur and hepatomegaly. The latter increased strikingly over the next 3 months, and was accompanied by cardiomegaly. Other usual signs of congestive cardiac failure were not present, however, and an arteriogram showed the presence of a grossly abnormal vascular pattern in the liver, confirming the clinical suspecions of hepatic haemangiomatosis. Two short courses of steroid therapy resulted in a marked but temporary decrease in liver size. After the age of one year, however, there was gradual regression of all abnormal clinical signs without further therapy.
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PMCID: PMC1546198  PMID: 138398
13.  Pulmonary eosinophilic gramuloma in a child. 
Archives of Disease in Childhood  1976;51(10):805-807.
The occurrence of pulmonary eosinophilic granuloma in a 3-year-old child is described. She presented with a pneumothorax and typical radiological changes and the diagnosis was confirmed by lung biopsy. There was no objective evidence of improvement after radiotherapy when lung function was assessed by gamma scans. She died suddenly while abroad.
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PMCID: PMC1546126  PMID: 1008587
14.  Origin of intraventricular haemorrhage in the preterm infant. 
Archives of Disease in Childhood  1976;51(9):651-659.
A technique has been developed for the injection and stereomicroscopic examination of blood vessels in the preterm newborn brain. Using this technique it can be seen that in the immature brain there is a rich capillary bed in the germinal layer region supplied mainly by Heubner's artery. Capillary channels drain directly into the terminal vein and its main branches. Study of 19 cases with spontaneous germinal layer haemorrhage (GLH) with or without intraventricular haemorrhage (IVH) failed to show rupture of the terminal vein or germinal layer infarction. In babies of up to 28 weeks' gestation GLH developed most frequently over the body of the caudate nucleus, whereas in babies of 29 weeks' gestation or more the haemorrhages were usually over the head of the caudate nucleus. Histological study of 10 cases of GLH failed to show rupture either of arteries or veins, though evidence of rupture at a capillary-vein junction was seen in one case and masses of fibrin adjacent to the vein wall in 2 others. Injection through the carotid artery caused prominent leaks of injection mass within the germinal layer capillary bed, often adjacent to the veins. Injection through the jugular veins in 2 cases failed to rupture the terminal vein but caused multiple vein ruptures at the junction of deep and cortical venous systems. Additional small ruptures in the germinal layer occurred in one of the cases only. It is suggested that the capillaries within the germinal layer may be ruptured by a rise in arterial pressure, particularly in conditions of hypercapnia and hypoxia.
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PMCID: PMC1546235  PMID: 999324
16.  Letter: Steroid aerosols and candidiasis. 
British Medical Journal  1974;2(5915):387.
PMCID: PMC1610211  PMID: 4835856
17.  Controlled trial of fresh frozen plasma in asphyxiated low birthweight infants 
A controlled trial of fresh frozen plasma in asphyxiated newborn infants weighing less than 2·5 kg is reported, together with coagulation and other data obtained from the infants. The findings based on 66 infants were as as follows. (1) There was no evidence that infusion of fresh frozen plasma prevented intraventricular haemorrhage. (2) Thrombotest did not appear to be a useful discriminant test for `at risk' infants. (3) Asphyxiated low birthweight infants do not respond to vitamin K1 in respect of thrombotest or prothrombin time. (4) The initial coagulation status of small-for-dates infants could not be distinguished from normal premature infants.
PMCID: PMC1647801  PMID: 4685591
19.  Evaluation of the Effects of Isoprenaline and Salbutamol Aerosols on Airways Obstruction and Pulse Rates of Children with Asthma* 
Archives of Disease in Childhood  1970;45(244):766-768.
Investigation of FEV1·0 and pulse rates in 10 asthmatic children who were given inhalations of isoprenaline, salbutamol, and placebo separately showed that both drugs had a similar effect on airways obstruction. Salbutamol has a longer duration of action. Neither drug caused a tachycardia at the dosage used.
PMCID: PMC1647441  PMID: 4923642
21.  Familial Lymphoma 
PMCID: PMC1810639  PMID: 5395225

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