Prediction of Mortality in Patients with Cystic Fibrosis

Kerem, Eitan; Reisman, Joseph; Corey, Mary; Canny, Gerard J.; Levison, Henry

doi:10.1056/nejm199204303261804

Cited by 923 publications

(627 citation statements)

References 16 publications

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“…Nevertheless, our study highlights the major importance of pulmonary disease in malnutrition, but also the implication of PI and gastroesophageal reflux. Longitudinal studies demonstrated that the decline of lung function and undernutrition are related with each other (Corey et al, 1988;Kerem et al, 1992;Schoni & CasaultaAebischer, 2000) and that malnutrition may be expected to increase with advancing age (Nir et al, 1996;Steinkamp & Wiedemann, 2002). However, we observed that age and BMI were positively correlated and that patients with a severe malnutrition were significantly younger than the other patients.…”

Section: Abstract: Cystic Fibrosis; Malnutritionmentioning

confidence: 43%

Malnutrition in adults with cystic fibrosis

et al. 2004

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Section: Abstract: Cystic Fibrosis; Malnutritionmentioning

confidence: 43%

Malnutrition in adults with cystic fibrosis

et al. 2004

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“…There have been previous studies that have employed proportional hazards modelling in order to identify measurements that are of prognostic value in these patients, with particular emphasis on the use of lung function measurements to determine life expectancy. KEREM et al [2] studied the survival of 673 patients (children and adults) from the Hospital for Sick Children, Toronto, over a 13 yr period (1977±1989). Twenty-eight per cent of the patients died during this time.…”

Section: Discussionmentioning

confidence: 99%

A model for predicting life expectancy of children with cystic fibrosis

Aurora

Wade²,

Whitmore

et al. 2000

Eur Respir J

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In this study the authors aimed to produce a model for predicting the life expectancy of children with severe cystic fibrosis (CF) lung disease.The survival of 181 children with severe CF lung disease referred for transplantation assessment 1988±1998 (mean age 11.5 yrs, median survival without transplant 1.9 yrs from date of assessment) were studied. Proportional hazards modelling was used to identify assessment measurements that are of value in predicting longevity.The resultant model included low height predicted forced expiratory volume in one second (FEV1), low minimum oxygen saturation (Sa,O 2 min) during a 12-min walk, high age adjusted resting heart rate, young age, female sex, low plasma albumin, and low blood haemoglobin as predictors for poor prognosis. Extrapolation from the model suggests that a 12-yr old male child with an FEV1 of 30% pred and a Sa,O 2 min of 85% has a 44% risk of death within 2 yrs (95% confidence interval (CI) 35±54%), whilst a female child with the same measurements has a 63% risk of death (95% CI 52±73%) within the same period.The model produced may be of value in predicting the life expectancy of children with severe cystic fibrosis lung disease and in optimizing the timing of lung transplantation.

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“…During this period, the earlier referral of females and younger patients was encouraged as these patient categories have been shown to have a poorer prognosis [5]. All patients were from the UK or Eire.…”

Section: Study Subjectsmentioning

confidence: 99%

“…The first question has been addressed by previous studies [4,5]. 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.…”

mentioning

confidence: 99%

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

Aurora¹,

Gassas²,

Ehtisham³

et al. 2000

Eur Respir J

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The aim of this study was to determine whether transplanting paediatric cystic fibrosis (CF) patients later in the course of their disease was detrimental to their post‐transplant survival. Data was collected from 51 children with CF undergoing lung or heart‐lung transplantation May 1988–March 1999. The following risk factors were tested by Cox proportional hazards modelling: age at transplant; sex; donor/recipient sex mismatch; donor/recipient cytomegalovirus (CMV) mismatch; cold and warm graft ischaemic times; and donor age. Pretran‐splant forced expiratory volume in one second (FEV1), minimum oxygen saturation obtained during 12 min walk (Sa,O2min), and a survival probability score (SP) calculated from FEV1, age adjusted resting heart rate, age, sex, blood haemoglobin (Hb), and serum albumin were then added to the model. None of the risk factors were significantly correlated with death during the study period. No evidence that clinical status prior to transplant has any effect upon the post‐transplant survival of children with cystic fibrosis was found.

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Prediction of Mortality in Patients with Cystic Fibrosis

Abstract: Patients with cystic fibrosis should be considered candidates for lung transplantation when the FEV1 falls below 30 percent of the predicted value. Female patients and younger patients may need to be considered for transplantation at an earlier stage.

Cited by 923 publications

References 16 publications

Malnutrition in adults with cystic fibrosis

Malnutrition in adults with cystic fibrosis

A model for predicting life expectancy of children with cystic fibrosis

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

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