Respir Med

Clinical factors that affect blood gases in non-smoking women with chronic liver disease.

Year 1998
Fujimori K. Arakawa M.
Department of Medicine (II), Niigata University School of Medicine, Japan.
Chronic liver disease is often accompanied by hypoxaemia. We investigated the clinical factors that were related to the arterial oxygen tension (PaO2) in 40 women, all non-smokers with chronic liver disease. They were positive for hepatitis C virus (HCV) antibody and had no evidence of cardiopulmonary disease. Arterial blood was collected from patients at rest (> 15 min) for analysis of blood gases. We determined the correlation between blood gas tension and the clinical variables, i.e. the presence or absence of skin manifestations such as cutaneous spider nevi and palmar erythema, the presence or absence of splenomegaly, vital capacity, forced expiratory volume in one second, V25/body height, serum alanine aminotransferase (AST), serum asparate aminotransferase (ALT), serum cholinesterase, serum gamma-globulin/total protein, excretion of indocyanine green at 15 min (15-min retention rate, ICG level), blood level of ammonia, blood level of endotoxin, plasma level of glucagon and the serum level of type IV collagen-7S. The mean level of PaO2 was 78 +/- 11 (range: 43-95) torr. The mean alveolar-arterial oxygen tension gradient (A-aDO2) was 19 +/- 13 (range: 2-60) torr. Multiple regression analysis used PaO2 and A-aDO2 as objective variables, and the clinical findings as explanatory variables. The explanatory variables that were significantly correlated with blood gas values were ICG level, blood level of endotoxin and presence of skin manifestations. The ICG level showed a high correlation with blood gas values; the ICG level increased, the PaO2 decreased (r = -0.69), while the A-aDO2 showed a high positive correlation (r = +0.78, P < 0.001). Findings suggest that a reduction in hepatic blood flow and hepatocellular function interfere with the inactivation of vasoactive substances such as endotoxin by the liver, leading to the development of skin manifestations, the dilatation of intrapulmonary capillaries and the induction of hypoxaemia.

Transmission and prevalence of Burkholderia cepacia in Welsh cystic fibrosis patients.

Year 1998
Millar-Jones L. Ryley HC. Paull A. Goodchild MC.
Department of Child Health, University Hospital of Wales, Cardiff, U.K.
From 1987 to 1994, 16 of 162 cystic fibrosis (CF) patients attending CF clinics at three different hospitals in South Wales, U.K. were found to have respiratory secretions colonized with Burkholderia cepacia (B. cepacia). Bacteriological typing by polymerase chain reaction (PCR) ribotyping demonstrated seven strains of B. cepacia among these 16 CF patients. This typing confirmed that cross-infection was the mechanism of colonization in six of the nine patients who were colonized at the paediatric CF clinic at the University Hospital of Wales in Cardiff, and in three of the six patients who were colonized at the adult CF clinic at Llandough Hospital in Cardiff (cross-infection rate nine of 16 patients or 56%). A search was made for a nosocomial source, with screening of wards and clinics. Swabs from fomites produced four positive cultures for B. cepacia. Two isolates had the same PCR ribotype as that of the previous CF room occupant. To establish prevalence of B. cepacia among CF children living throughout Wales, respiratory secretions were cultured from 151 of 186 CF children (age < 16 years). This failed to demonstrate B. cepacia colonization other than in the CF patients already identified.

Epithelial cell damage is induced by neutrophil-derived, not pseudomonas-derived, proteases in cystic fibrosis sputum.

Year 1998
Venaille TJ. Ryan G. Robinson BW.
University Department of Medicine, QEII Medical Centre, Perth, Western Australia.
Airway histopathological changes in cystic fibrosis (CF) include damage to the epithelial tissue and accumulation of polymorphonuclear leukocytes (PMN). Airways of CF patients are usually colonized with bacteria such as mucoid Pseudomonas aeruginosa (PA). Bacteria and PMN can both release proteolytic enzymes capable of causing tissue damage. This study aims to clarify and compare the roles of these agents in epithelium damage. Epithelial cell (EC) damage and detachment induced by sputum samples from CF or non-CF patients, with and without lung infection, were assessed on amnionic EC in an in vitro model of airway epithelium. Protease activity was determined using inhibitor profiles, and compared to the proteolytic activity of isolated neutrophils and bacteria. Sputa from CF patients and infected non-CF patients induced high levels of detachment. PA also induced high levels of EC detachment but Staphylococcus aureus and Haemophilus influenzae, two other bacteria commonly isolated from CF sputa, induced no detachment. Antiprotease inhibition profiles were similar for PMN and sputa-induced EC detachment, but different for PA-induced detachment. These results suggest that PMN proteolytic enzymes, probably elastase and cathepsin G, are more likely to be the inducers of tissue damage in the airways of CF patients than PA proteolytic enzymes.

Do Nottingham Health Profile scores change over time in cystic fibrosis?

Year 1998
Congleton J. Hodson ME. Duncan-Skingle F.
Department of Cystic Fibrosis, Royal Brompton Hospital, London, U.K.
We have previously shown that the Nottingham Health Profile (NHP) shows good correlation with physiological scores in an adult cystic fibrosis (CF) population when assessing health status (HS). The aim of this study was to determine whether the NHP can detect change in HS over time. Patients attending an adult CF clinic were studied at the time of their 'annual review' appointment. HS was assessed by the NHP and an 'in house' CF-specific score was used. FEV1% predicted, FVC% predicted, FEV1/FVC ratio, and body mass index (BMI) were calculated. The patients were then studied at subsequent annual review appointments. Change over time was calculated for each parameter and the relationship between change in HS and change in physiological parameters was examined using Spearman's rank correlation. Initial (t1), NHP scores were obtained from 204 patients, median age 26 years (range 16-56 years). Follow-up scores were obtained from 152 patients (t2). The mean rate of change in FEV1 was -8.6 (261) ml yr-1 or -0.2 (6.9)% predicted yr-1. The only dimension of the NHP which showed a significant change over time was emotion, which showed a small improvement in score (score at t1 = 11.3, score at t2 = 8.1, P = 0.02). There was a significant deterioration in the CF-specific total score (t1 = 4.4, t2 = 4.7, P = 0.008). There were no significant correlations between change in physiology and change in NHP scores. There was no overall difference in change in NHP scores between the patients whose FEV1 declined at > 4% predicted yr-1 and those who did not. In conclusion, although the NHP correlates well with one-off physiological parameters, there is poor correlation between change in NHP scores and change in physiological parameters over time.