Publications
204 results found
Shah PL, Hodson ME, 1997, The overuse or underuse of dornase alfa., Curr Opin Pulm Med, Vol: 3, Pages: 410-413, ISSN: 1070-5287
The poor clearance of airway secretions in patients with cystic fibrosis perpetuates the chronic bronchopulmonary sepsis that is predominant. In recent years, novel drugs have been developed to alter the rheologic properties of the secretions in an attempt to improve airway clearance. Dornase alfa reduces the viscoelasticity of sputum from patients with cystic fibrosis and may enhance the clearance of secretions. Current clinical knowledge suggests that it is a safe treatment that improves pulmonary function and reduces respiratory exacerbations. The response, however, is heterogeneous and unpredictable. Scientific studies support the therapeutic rationale for the use of dornase alfa in that treatment reduces the viscoelasticity of airway secretions. Its effect on bacterial persistence and airway inflammation, however, is marginal. The key piece of information that would influence the long-term use of dornase alfa is how it affects disease progression, and at present this is unknown.
Koch C, McKenzie SG, Kaplowitz H, et al., 1997, International practice patterns by age and severity of lung disease in cystic fibrosis: Data from the Epidemiologic Registry of Cystic Fibrosis (ERCF), Roche/Genentech Satellite Symposium on the Rationale for Early Intervention in Cystic Fibrosis, at the International Cystic Fibrosis Congress, Publisher: WILEY-LISS, Pages: 147-154, ISSN: 8755-6863
- Author Web Link
- Cite
- Citations: 49
Phillips GD, TrotmanDickenson B, Hodson ME, et al., 1997, Role of CT in the management of pneumothorax in patients with complex cystic lung disease, CHEST, Vol: 112, Pages: 275-278, ISSN: 0012-3692
- Author Web Link
- Cite
- Citations: 32
Shah PL, Scott SF, Geddes DM, et al., 1997, An evaluation of two aerosol delivery systems for rhDNase, EUROPEAN RESPIRATORY JOURNAL, Vol: 10, Pages: 1261-1266, ISSN: 0903-1936
- Author Web Link
- Cite
- Citations: 22
Hayllar KM, Williams SGJ, Wise AE, et al., 1997, A prognostic model for the prediction of survival in cystic fibrosis, THORAX, Vol: 52, Pages: 313-317, ISSN: 0040-6376
- Author Web Link
- Cite
- Citations: 72
Knight RA, Kollnberger S, Madden B, et al., 1997, Defective antigen presentation by lavage cells from terminal patients with cystic fibrosis, CLINICAL AND EXPERIMENTAL IMMUNOLOGY, Vol: 107, Pages: 542-547, ISSN: 0009-9104
- Author Web Link
- Cite
- Citations: 12
Hodson ME, 1997, Psychosocial aspects for the management of adults with cystic fibrosis, PEDIATRIC PULMONOLOGY, Pages: 113-114, ISSN: 8755-6863
- Author Web Link
- Cite
- Citations: 4
Shah PL, Hodson ME, 1997, Dornase alfa: A practical guide to patient selection and drug use in cystic fibrosis., Biodrugs, Vol: 8, Pages: 439-445
Williams SGJ, Samways J, Innes JA, et al., 1996, Systemic haemodynamic changes in patients with cystic fibrosis with and without chronic liver disease, JOURNAL OF HEPATOLOGY, Vol: 25, Pages: 900-908, ISSN: 0168-8278
- Author Web Link
- Cite
- Citations: 2
Congleton J, Hodson ME, Duncan-Skingle F, 1996, Quality of life in adults with cystic fibrosis., Thorax, Vol: 51, Pages: 936-940, ISSN: 0040-6376
BACKGROUND: Cystic fibrosis is an inherited condition with a high mortality and morbidity. The aims of this study were to assess quality of life in a population of adults with cystic fibrosis, to compare quality of life with published scores from a healthy population and other patient groups, and to examine the relation between quality of life and other measured clinical variables. METHODS: Patients over 16 years of age attending an adult cystic fibrosis outpatient clinic were surveyed at a time when they were clinically stable. A self-complete questionnaire was administered which comprised the Nottingham Health Profile (NHP) together with six additional questions related to cystic fibrosis. RESULTS: Completed questionnaires were obtained from 240 subjects (100 women) of median age 26 years (range 16-56). Mean (SD) forced expiratory volume in one second (FEV1) was 49 (26)% predicted, forced vital capacity (FVC) was 68 (26)% predicted, and the FEV1:FVC ratio was 59 (16)%. In this cross sectional study different patterns of perceived quality of life were seen in men and women. In part 1 of the NHP there was an age related trend compared with norms in men, with more distress/disability in the dimensions of emotion, sleep, and social isolation in the older age groups. In women there was no age related trend in the degree of distress/disability compared with norms. The mean score was different from norms in the dimensions of pain, emotion and sleep. For the patients with cystic fibrosis as a whole the scores in part 1 were comparable with published scores of patients with minor non-acute conditions. Scores in part 2 of the NHP for men were different from norms in six of the seven areas of daily living (all except home life). For women the scores were different from norms in the areas of looking after the home, social life, hobbies, and holidays. There were correlations between several of the quality of life dimensions and other measured variables such as FEV1, breathless
Hutchinson GR, Parker S, Pryor JA, et al., 1996, Home-use nebulizers: A potential primary source of Burkholderia cepacia and other colistin-resistant, gram-negative bacteria in patients with cystic fibrosis (vol 34, pg 584, 1996), JOURNAL OF CLINICAL MICROBIOLOGY, Vol: 34, Pages: 1601-1601, ISSN: 0095-1137
- Author Web Link
- Cite
- Citations: 1
Shah PL, Hodson ME, 1996, New treatment strategies in cystic fibrosis: rhDNase., Monaldi Arch Chest Dis, Vol: 51, Pages: 125-129, ISSN: 1122-0643
Cystic fibrosis (CF) is the commonest inherited disease of the Caucasian population, with a high morbidity and mortality from pulmonary disease. The high viscoelasticity of CF sputum is due, in part, to the high deoxyribonucleic acid (DNA) content. Recombinant human deoxyribonuclease I (rhDNase) has been developed and in vitro studies have shown that it reduces the viscoelasticity of CF sputum. This article reviews the in vivo clinical studies conducted to determine the safety and efficacy of rhDNase in the treatment of pulmonary disease in CF. Initial Phase I studies showed preliminary safety and some evidence of clinical benefit. Subsequently, two Phase II studies were conducted in the US and UK during which patients received rhDNase for 10 days. A Phase III study of 24 weeks duration involving 968 patients in 51 centres in North America is also reported in detail. Longer term open-label studies, the results of intermittent administration, administration to severely ill patients and the use of different delivery systems are reviewed. The Phase II study reported improvements in pulmonary function and had a good safety profile. The Phase III study showed improvement in forced expiratory volume in one second (FEV1) of 5.8 and 5.6% in patients treated once and twice daily, respectively. The risk of developing an exacerbation was reduced by 28% with once daily treatment and 37% with twice daily treatment compared to placebo. The drug was safe and there was some improvement in quality of life data. Recombinant human deoxyribonuclease is a new therapy for pulmonary disease in cystic fibrosis which has been shown to benefit patients when used in conjunction with conventional therapy.
Zhao MH, Jayne DRW, Ardiles LG, et al., 1996, Autoantibodies against bactericidal permeability-increasing protein in patients with cystic fibrosis, QJM-MONTHLY JOURNAL OF THE ASSOCIATION OF PHYSICIANS, Vol: 89, Pages: 259-265, ISSN: 0033-5622
- Author Web Link
- Cite
- Citations: 96
Hutchinson GR, Parker S, Pryor JA, et al., 1996, Home-use nebulizers: A potential primary source of Burkholderia cepacia and other colistin-resistant, gram-negative bacteria in patients with cystic fibrosis, JOURNAL OF CLINICAL MICROBIOLOGY, Vol: 34, Pages: 584-587, ISSN: 0095-1137
- Author Web Link
- Cite
- Citations: 107
Shah PL, Scott SF, Knight RA, et al., 1996, The effects of recombinant human DNase on neutrophil elastase activity and interleukin-8 levels in the sputum of patients with cystic fibrosis, EUROPEAN RESPIRATORY JOURNAL, Vol: 9, Pages: 531-534, ISSN: 0903-1936
- Author Web Link
- Cite
- Citations: 43
Shah PL, Scott SF, Knight RA, et al., 1996, In vivo effects of recombinant human DNase I on sputum in patients with cystic fibrosis, THORAX, Vol: 51, Pages: 119-125, ISSN: 0040-6376
- Author Web Link
- Cite
- Citations: 105
BRAMWELL EC, HALPIN DMG, DUNCANSKINGLE F, et al., 1995, HOME TREATMENT OF PATIENTS WITH CYSTIC-FIBROSIS USING THE INTERMATE - THE FIRST YEARS EXPERIENCE, JOURNAL OF ADVANCED NURSING, Vol: 22, Pages: 1063-1067, ISSN: 0309-2402
- Author Web Link
- Cite
- Citations: 20
HODSON ME, SHAH PL, 1995, DNASE TRIALS IN CYSTIC-FIBROSIS, EUROPEAN RESPIRATORY JOURNAL, Vol: 8, Pages: 1786-1791, ISSN: 0903-1936
- Author Web Link
- Cite
- Citations: 26
TOUW DJ, BRIMICOMBE RW, HODSON ME, et al., 1995, INHALATION OF ANTIBIOTICS IN CYSTIC-FIBROSIS, EUROPEAN RESPIRATORY JOURNAL, Vol: 8, Pages: 1594-1604, ISSN: 0903-1936
- Author Web Link
- Cite
- Citations: 123
SHAH PL, SCOTT SF, GEDDES DM, et al., 1995, 2 YEARS EXPERIENCE WITH RECOMBINANT HUMAN DNASE-I IN THE TREATMENT OF PULMONARY-DISEASE IN CYSTIC-FIBROSIS, RESPIRATORY MEDICINE, Vol: 89, Pages: 499-502, ISSN: 0954-6111
- Author Web Link
- Cite
- Citations: 55
HODSON ME, ROSENTHAL M, WALLIS C, et al., 1995, DORNASE ALFA FOR CYSTIC-FIBROSIS - PATIENTS SHOULD NOT BE DENIED A SAFE, EFFECTIVE TREATMENT, BRITISH MEDICAL JOURNAL, Vol: 310, Pages: 1533-1533, ISSN: 0959-8138
- Author Web Link
- Cite
- Citations: 4
HODSON ME, 1995, MAINTENANCE TREATMENT WITH ANTIBIOTICS IN CYSTIC-FIBROSIS PATIENTS SENSE OR NONSENSE, NETHERLANDS JOURNAL OF MEDICINE, Vol: 46, Pages: 288-292, ISSN: 0300-2977
- Author Web Link
- Cite
- Citations: 5
SHAH PI, BUSH A, CANNY GJ, et al., 1995, RECOMBINANT HUMAN DNASE-I IN CYSTIC-FIBROSIS PATIENTS WITH SEVERE PULMONARY-DISEASE - A SHORT-TERM, DOUBLE-BLIND-STUDY FOLLOWED BY 6 MONTHS OPEN-LABEL TREATMENT, EUROPEAN RESPIRATORY JOURNAL, Vol: 8, Pages: 954-958, ISSN: 0903-1936
- Author Web Link
- Cite
- Citations: 63
WILLIAMS SGJ, EVANSON JE, BARRETT N, et al., 1995, AN ULTRASOUND SCORING SYSTEM FOR THE DIAGNOSIS OF LIVER-DISEASE IN CYSTIC-FIBROSIS, JOURNAL OF HEPATOLOGY, Vol: 22, Pages: 513-521, ISSN: 0169-5185
- Author Web Link
- Cite
- Citations: 70
SHAH PL, SCOTT SF, FUCHS HJ, et al., 1995, MEDIUM-TERM TREATMENT OF STABLE STAGE CYSTIC-FIBROSIS WITH RECOMBINANT HUMAN DNASE-I, THORAX, Vol: 50, Pages: 333-338, ISSN: 0040-6376
- Author Web Link
- Cite
- Citations: 40
HODSON ME, 1995, AEROSOLIZED DORNASE ALFA (RHDNASE) FOR THERAPY OF CYSTIC-FIBROSIS, AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, Vol: 151, Pages: S70-S74, ISSN: 1073-449X
- Author Web Link
- Cite
- Citations: 34
CAPLEN NJ, ALTON E, MIDDLETON PG, et al., 1995, LIPOSOME-MEDIATED CFTR GENE-TRANSFER TO THE NASAL EPITHELIUM OF PATIENTS WITH CYSTIC-FIBROSIS, NATURE MEDICINE, Vol: 1, Pages: 39-46, ISSN: 1078-8956
- Author Web Link
- Cite
- Citations: 653
Hodson ME, 1995, Bacterial infection in cystic fibrosis (CF): special reference to mycobacteria and Burkholderia cepacia., Pediatr Pulmonol Suppl, Vol: 11, Pages: 66-67, ISSN: 1054-187X
Hodson ME, 1995, Clinical studies of rhDNase in moderately and severely affected patients with cystic fibrosis--an overview., Respiration, Vol: 62 Suppl 1, Pages: 29-32, ISSN: 0025-7931
Clinical data are now available on the use of recombinant human DNase (rhDNase) in the treatment of CF patients with mild, moderate and severe pulmonary disease. Phase I studies were conducted and indicated the initial safety of rhDNase in humans. In the US phase II study, 181 patients with FVC > or = 40% were randomly allocated to receive rhDNase 0.6, 2.5, 10 mg, or placebo twice daily for 10 days. All three doses of rhDNase significantly improved FEV1 10-14% and FVC 10-12% compared to placebo. There was no significant increase in serious intercurrent events but a slight increase in pharyngitis and voice alteration. A phase II study including 71 patients was carried out in the UK. FEV1 improved by 13% from baseline compared to placebo. There was an improvement in CF-related symptoms and no increase in serious adverse events. The phase III double-blind placebo-controlled study included 968 patients with FVC > or = 40% predicted. These patients were randomized to 2.5 mg rhDNase, once or twice daily, or placebo for 24 weeks. Compared to placebo, rhDNase-treated patients had a relative risk of protocol-defined respiratory tract infection reduced by 22 and 34% and improved FEV1 compared to baseline by 5.8% (p < 0.001) and 5.6% (p < 0.001), respectively, compared to placebo. A double-blind, short-term, placebo-controlled study in severely ill patients, FVC < 40% predicted, showed the drug to be safe but there was no significant improvement in lung function. After a further 6-month open-label treatment, patients showed improvement in FEV1 and FVC.(ABSTRACT TRUNCATED AT 250 WORDS)
HODSON ME, 1995, CLINICAL-STUDIES OF RHDNASE IN MODERATELY AND SEVERELY AFFECTED PATIENTS WITH CYSTIC-FIBROSIS - AN OVERVIEW, RESPIRATION, Vol: 62, Pages: 29-32, ISSN: 0025-7931
- Author Web Link
- Cite
- Citations: 8
This data is extracted from the Web of Science and reproduced under a licence from Thomson Reuters. You may not copy or re-distribute this data in whole or in part without the written consent of the Science business of Thomson Reuters.