A prognostic model for the prediction of survival in cystic fibrosis

Abstract

Abstractyears, predominantly as a result of improved respiratory care. 2 3 Death, however, continues Background -The treatment for endstage cystic fibrosis is, where appropriate, to be due, in most cases, to respiratory failure associated with cor pulmonale and pulmonary double-lung, heart-lung or, occasionally, heart-lung-liver transplantation. Op-hypertension. 3Transplantation (double-lung, heart-lung or, timising the timing of transplantation depends upon an accurate prediction of occasionally, heart-lung-liver) remains the cornerstone of treatment in the terminal phase of survival, but while current criteria give some guidance to this, they are not based the disease. 4 Results have been encouraging in both adults 5 and children 6 and include enupon statistically derived prognostic models.hanced survival and improvement in lung function. Methods -Data collected prospectively on 403 patients with cystic fibrosis, recruitedTo optimise the timing and results of transplantation, particularly in the presence of a between 1969 and 1987 (cohort A), were analysed by log rank and univariate Cox donor shortage, there is a need for an accurate prediction of patient survival. Current reregression analysis to determine variables that accurately predict survival. The sig-commendations for transplantation include those patients whose quality of life is severely nificant variables were then subject to time dependent multivariate Cox regression impaired and whose life expectancy is less than two years. 7 Current criteria suggested for reanalysis to generate a prognostic model. The model was validated, within the study commending transplantation include percentage predicted forced expiratory volume in population, using split sample testing, and was subsequently validated in a further one second (FEV 1 ) <30% and significant carbon dioxide retention.

A model for predicting life expectancy of children with cystic fibrosis

“…The authors also studied the value of percentage ideal weight as a predictor, but found it of little value, particularly in younger patients. HAYLLAR et al [11] studied 403 patients, predominantly adults, from the Royal Brompton Hospital, London, 1969±1989. This analysis identified FEV1, FVC, height, WCC, clinically detected hepatomegaly, serum albumin and alkaline phosphatase concentrations as the best predictors of survival.…”

“…The authors have also shown plasma albumin and Hb to be predictors for survival. Albumin was included in the model used by HAYLLAR et al [11], whereas Hb was suggested as a useful predictor by the initial analysis of their data, but was not included in the final model. The value of albumin and Hb may be as markers for nutritional status, if so, they appear to be more sensitive in this regard than percentage ideal weight.…”

“…The value of albumin and Hb may be as markers for nutritional status, if so, they appear to be more sensitive in this regard than percentage ideal weight. Two other measures previously suggested to be of value in adult CF patients are height and plasma alkaline phosphatase concentration (ALP) [11]. Height is less easy to analyse in a paediatric population, particularly in a population where delayed puberty is common, and ALP may be a marker for either bone or hepatic disease.…”

The effect of prelung transplant clinical status on post-transplant survival of children with cystic fibrosis

“…Current international consensus statements suggest that CF patients should be listed for transplantation when their forced expiratory volume in one second (FEV1) falls to 30% pred normal or below, as this appears to equate to a life expectancy of #2 yrs. Sophisticated multivariate modelling techniques are used with increasing frequency, which may prove to be more reliable in predicting life expectancy [6]. Previous studies have attempted to identify risk factors related to reduced survival following lung or heart-lung transplantation [7].…”

Evaluation of ventilation maldistribution as an early indicator of lung disease in children with cystic fibrosis

“…Parameters derived from maximal expiratory flow-volume loops (spirometry), such as the forced vital capacity (FVC), forced expiratory volume in one second (FEV1), and maximum expiratory flow at 25% of FVC (MEF25), have traditionally been used to monitor lung function in CF patients. Whilst FEV1 and FVC are still recognised to be good predictors of prognosis in subjects with moderate-to-severe CF lung disease [2][3][4], they are now of less value in monitoring children with CF. The majority (82%) of children aged 6-18 yrs attending the CF centre in Gothenburg, Sweden, have FEV1 results that are within the normal range.…”