Body Composition in Cystic Fibrosis

Gaskin, K. J.; Waters, Donna; Soutter, Velencia; Baur, Louise A.; Allen, Barry J.; Blagojevic, N.; Parsons, Dee S.

doi:10.1007/978-1-4613-1473-8_2

Cited by 9 publications

(4 citation statements)

References 10 publications

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“…Furthermore. the patients in the present study showed more marked depletion of body protein and, as previously demonstrated, an anomaly of body potassium content (13). Whatever the explanation, these preliminary data demonstrate a close relationship between weight gain and nitrogen deposition.…”

Section: Beforesupporting

confidence: 87%

Nutritional Status, Growth and Development in Children Undergoing Intensive Treatment for Cystic Fibrosis

Gaskin¹,

Waters²,

Baur³

et al. 1990

Acta Paediatrica

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Nutritional status, growth and development in children undergoing intensive treatment for cystic fibrosis. Acta Paediatr Scand [Suppl] 366: 106,1990.Dietary intakes were measured over a period of 5 days in 36 malnourished and 36 wellnourished patients with cystic fibrosis. Both energy and protein intakes were significantly less in the malnourished patients for the two age groups studied: 4-9.99 years ( p < 0.01 for both parameters), and 10-16 years ( p c 0.05 and p < 0.01, respectively). In both age groups and both patient groups, average protein intakes were well in excess of the recommended daily intake, but energy intake in the malnourished patients was below the recommended daily intake. Nutritional supplementation of 10 malnourished patients with a polymeric formula, infused overnight via a gastrostomy tube, resulted in a seven-fold increase in weight gain ( p < 0.001) and a doubling of linear growth velocity ( p < 0.01) over a period of 18 months, compared to the 18 months prior to gastrostomy feeding. Measurements of total body nitrogen in eight of these patients demonstrated a 38% increase in body nitrogen content over 12 months, indicating a replenishment of the protein deficit.

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Section: Beforesupporting

confidence: 87%

Nutritional Status, Growth and Development in Children Undergoing Intensive Treatment for Cystic Fibrosis

Gaskin¹,

Waters²,

Baur³

et al. 1990

Acta Paediatrica

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“…Weight for height ratio was calculated. Fat mass was assessed by anthropometry [8] and by dual-energy X-ray absorptiometry (DEXA) (Hologic QDR 2000, Waltham, MA, USA) [2,12,31]. Lean body mass (weight ± fat mass assessed by anthropometry) and fat-free mass (weight ± fat mass assessed by DEXA) were calculated.…”

Section: Body Compositionmentioning

confidence: 99%

Essential fatty acid deficiency in well nourished young cystic fibrosis patients

Roulet

Frascarolo

Rappaz

et al. 1997

European Journal of Pediatrics

135

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“…35 There is some support for this possibility in the observation that CF patients who are not pancreatic insufficient not only have better nutritional parameters but also a lower rate of pulmonary deterioration. 36 In addition, studies conducted in infants diagnosed through newborn screening indicate that growth failure still occurs in this patient population despite the early diagnosis, 23,37 and this seems to precede the decline in pulmonary function. 37 Thomson et al in a prospective evaluation of their CF patient population were able to identify important relationships between changes in growth and pulmonary function.…”

Section: Energy Expenditure In Cfmentioning

confidence: 99%

Nutrition in Cystic Fibrosis

Matel

Milla

2009

Semin Respir Crit Care Med

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Cystic fibrosis (CF) is mostly recognized for its pulmonary morbidity, but the earliest manifestations of the disease are related to its gastrointestinal and nutritional derangements. Destruction of acinar pancreatic tissue, pancreatic ductular obstruction, and lack of enzymatic activity lead to malabsorption (particularly of fats), diarrhea, and failure to thrive. A minority of CF patients carrying milder CF transmembrane conductance regulator (CFTR) mutations have preserved pancreatic secretory activity and are free from significant malabsorption early in life. However, these patients are at risk for losing pancreatic function over time. Nutritional status plays an important role in the progression of the pulmonary disease in CF. Further, CF patients with better nutritional status have a survival advantage. Several factors contribute to impaired nutritional status in CF (e.g., pancreatic insufficiency, chronic malabsorption, recurrent sinopulmonary infections, chronic inflammation, increased energy expenditure, suboptimal intake). Progressive lung disease further increases calorie requirements by increasing the work of breathing. Treatment programs that place an emphasis on higher caloric intake and more aggressive nutritional management in CF patients report better outcomes. Basic tenets of nutritional repletion in CF include the use of pancreatic enzyme replacement therapy and following a high calorie, high protein, unrestricted diet. At the Stanford Cystic Fibrosis Center, nutritional status is assessed on an ongoing basis through anthropometric parameters and annual assessment of body composition, bone density, glucose tolerance, and various biochemical and micronutrient levels. Based on the anthropometric data obtained on routine clinical encounters, patients are categorized as to their nutritional risk. This proactive approach for the early identification of nutritional risk has become a major theme within the network of US CF centers. Aggressive nutritional support with adequate pancreatic replacement management should lead to both normal growth and lung function preservation. In addition, nutritional status has to be monitored closely during routine encounters to allow for early intervention once derangements are noted. This will include increasing calories in the early stages of lung disease and being vigilant of gastrointestinal symptomatology and complications.

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Body Composition in Cystic Fibrosis

Cited by 9 publications

References 10 publications

Nutritional Status, Growth and Development in Children Undergoing Intensive Treatment for Cystic Fibrosis

Nutritional Status, Growth and Development in Children Undergoing Intensive Treatment for Cystic Fibrosis

Essential fatty acid deficiency in well nourished young cystic fibrosis patients

Nutrition in Cystic Fibrosis

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