Nutritional Supplementation in Treatment of Cystic Fibrosis of the Pancreas

Allan, J. D.; Mason, Allen; Moss, Alexander

doi:10.1001/archpedi.1973.02110190018004

Cited by 17 publications

(19 citation statements)

References 11 publications

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“…As treatment standards for CF patients improved over the years, the average mean survival of CF patients increased dramatically. Most notable among such advances was the refinement of nutritional regimens (4,19,290) and the advent of antibiotic chemotherapy (122,193,197,228). While essentially all patients prior to the 1950s died by the age of 10 years, reports published in the 1950s (192) and 1960s (297) described a considerable proportion of CF patients surviving well beyond this age.…”

Section: Historical Framework For the Study Of Cystic Fibrosismentioning

confidence: 99%

Lung Infections Associated with Cystic Fibrosis

2002

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While originally characterized as a collection of related syndromes, cystic fibrosis (CF) is now recognized as a single disease whose diverse symptoms stem from the wide tissue distribution of the gene product that is defective in CF, the ion channel and regulator, cystic fibrosis transmembrane conductance regulator (CFTR). Defective CFTR protein impacts the function of the pancreas and alters the consistency of mucosal secretions. The latter of these effects probably plays an important role in the defective resistance of CF patients to many pathogens. As the modalities of CF research have changed over the decades from empirical histological studies to include biophysical measurements of CFTR function, the clinical management of this disease has similarly evolved to effectively address the ever-changing spectrum of CF-related infectious diseases. These factors have led to the successful management of many CF-related infections with the notable exception of chronic lung infection with the gram-negative bacterium Pseudomonas aeruginosa. The virulence of P. aeruginosa stems from multiple bacterial attributes, including antibiotic resistance, the ability to utilize quorum-sensing signals to form biofilms, the destructive potential of a multitude of its microbial toxins, and the ability to acquire a mucoid phenotype, which renders this microbe resistant to both the innate and acquired immunologic defenses of the host

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Section: Historical Framework For the Study Of Cystic Fibrosismentioning

confidence: 99%

Lung Infections Associated with Cystic Fibrosis

2002

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“…Functional changes (assessed in fifty-nine studies) following ONS varied according to the disease or condition of patients, and were typically observed more commonly in those patients with a BMI < 20 kg/m 2 than in those with BMI > 20 kg/m 2 . Functional benefits included improved muscle strength, walking distance and well-being in patients with COPD (Wilson et al 1986;Efthimiou et al 1988;Donahoe et al 1989;Rogers et al 1992), improved growth performance in children with cystic fibrosis (Allan et al 1973;Berry et al 1975;Yassa et al 1978;Kirschner et al 1981;Parsons et al 1983;Shepherd et al 1983;Skypala et al 1998), reduced falls (Gray-Donald et al 1995) and increased activities of daily living (Volkert et al 1996) in the elderly, and improvements in immune function in patients with human immunodeficiency virus (Hellerstein et al 1994). It is possible that detriments due to ONS may occur in some overweight patients with certain diseases (e.g.…”

Section: Outcome Measuresmentioning

confidence: 99%

Summary of a systematic review on oral nutritional supplement use in the community

Stratton

2000

Proc. Nutr. Soc.

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Despite a marked increase in the prescription of oral nutritional supplements (ONS) in the community (Department of Health, 1991–7), there is still uncertainty about the value of their use in patients with different diseases. To answer questions about the effects on ONS on body weight and structure, spontaneous food intake and body function, a critical systematic review was undertaken (Stratton & Elia, 1999a). Eighty-four trials were reviewed (forty-five randomized, thirty-nine non-randomized; 2570 patients; diagnoses including chronic obstructive pulmonary disease, Crohn’s disease, cystic fibrosis, human immunodeficiency virus and acquired immune deficiency syndrome and cancer). Most studies (83 %) were conducted in patients living at home. The supplements were typically mixed macronutrients in liquid form, providing < 0.42–10.5 MJ/d for 1 week–2 years. The studies reviewed in patients with predominantly chronic conditions living in the community suggested that: (1) ONS produce demonstrable clinical (including functional) benefits, but the nature and extent of these benefits varies with the underlying chronic condition; (2) ONS increase total energy intake with > 50 % of the energy from ONS typically additional to that from habitual food intake; (3) improvements in body weight, total energy intake and body function following ONS appear to occur more frequently in individuals with a BMI < 20 kg/m2 than in those with a BMI > 20 kg/m2.

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“…As 70 % of patients with CF can now be expected to live to age 17 (Robinson and Norman, 1975), and many survive to adulthood, the ultimate achievement of normal stature and weight has become important. Recent reports have claimed that growth may be improved by an artificial diet (Allan et al, 1973;Berry et al, 1975). We have examined the effects of this diet on growth in a controlled trial.…”

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confidence: 99%