Relative Underweight in Cystic Fibrosis and Its Prognostic Value

Kraemer, Richard; Rüdeberg, A; Hadorn, B; Rossi, E

doi:10.1111/j.1651-2227.1978.tb16273.x

Cited by 323 publications

(145 citation statements)

References 13 publications

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“…Cepacia colonization was defined as culture of Burkholderia cepacia from a sputum sample or cough swab within the preceding 2 yrs. The method for calculating percentage ideal weight has been recorded elsewhere [7]. Age, sex, blood haemoglobin concentration (Hb), blood leukocyte count (WCC), plasma immunoglobulin-G concentration (IgG), plasma albumin concentration, plasma aspartate transaminase concentration (AST), and number of courses of intravenous antibiotics administered to the child in the preceding 6 months (IV frequency) were also recorded.…”

Section: Methodsmentioning

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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Section: Methodsmentioning

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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“…Malnutrition also plays a role in the progressive lung disease that characterizes CF, although the mechanisms are not fully defined. Poor growth, a marker of malnutrition (Lai et al, 1998), has been associated with an increased morbidity and mortality rate for patients with CF (Kraemer et al, 1978;Erdman, 1999;Turck & Michaud, 1998).…”

Section: Introductionmentioning

confidence: 99%

Longitudinal investigation of energy expenditure in infants with cystic fibrosis

et al. 2002

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Objective: To determine when energy expenditure becomes elevated in infants with cystic fibrosis (CF). Design: Longitudinal studies of total energy expenditure (TEE) using doubly labeled water were conducted in infants identified with CF by newborn screening through the first year of life. Setting: Hospital and community based studies in Denver, Colorado, USA and Cambridge, UK. Results: Eight of the 12 infants enrolled had begun enzyme therapy but were clinically asymptomatic. Four of the 12 infants were heterozygous for the delta F508 mutation, however no difference was seen in TEE from the remaining homozygous infants. TEE was compared to control cohorts at 2, 6 and 12 months of age. There was no difference from the control groups in TEE=kg fat free mass (FFM)=day at 2 months. However, by 6 months of age TEE=kg FFM=day in infants with CF exceeded that of agematched controls by 25% (P < 0.001). This elevation in TEE continued at 12 months of age exceeding that of controls by 30% (P < 0.05). Conclusions: These results indicate that infants with CF have increased energy needs by 6 months of age and that early diagnosis alone does not prevent the development of increased caloric requirements. These findings emphasize the need for close nutritional monitoring to prevent suboptimal growth during infancy in this population.

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“…The connective tissue composition of the lung was found to be changed in rats during starvation, but reestablished after refeeding (9). However, the relevance for patients needs t o be established.…”

Section: Discussionmentioning

confidence: 99%

Influence of Respiratory Exacerbations on Lung Function Variables and Nutritional Status in CF Patients

et al. 1985

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The interaction between the severity of respiratory abnormalities and the nutritional status in cystic fibrosis (CF) patients is not fully elucidated as yet. Several studies indicate that a decline in lung function and a diminishment in the nutritional status are associated ( I , 2). The nutritional status of patients who have relatively low lung function values turns out t o have a disturbed nutritional status and vice versa. This association does, however, not resolve the problem which of both is the cause and which the result. It might well be that each of both, a decline in respiratory function or a decrease in the nutritional status, might affect the other one. Such a mutual interaction may easily lead to a viscious circle. Respiratory infections may deteriorate the nutritional status, which in turn may enhance the chance on infections, while break down of pulmonary tissue is the result. It is important to recognize these interactions and to counteract such a viscious circle as much as possible.In this study the relationship and interaction between respiratory infections and nutritional status are analysed in two ways. Firstly, we have compared lung function variables and either body weight o r height in a group of CF patients who are in steady state (group A, see methods). Secondly, we have compared the change in lung function variables and body weight as well as several other factors in the course of a respiratory exacerbation. The aim is to study the influence of the occurrence or increase in respiratory infections on the nutritional variables. These measurements are performed without and with an additional feeding regime during the night, in order to see if a high caloric supplementation can prevent a decline in nutritional status during the infection. METHODS

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Relative Underweight in Cystic Fibrosis and Its Prognostic Value

Cited by 323 publications

References 13 publications

A model for predicting life expectancy of children with cystic fibrosis

A model for predicting life expectancy of children with cystic fibrosis

Longitudinal investigation of energy expenditure in infants with cystic fibrosis

Influence of Respiratory Exacerbations on Lung Function Variables and Nutritional Status in CF Patients

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