An overview of nutritional issues for the adult with cystic fibrosis

Dowsett, Julie

doi:10.1016/s0899-9007(00)00351-8

Cited by 17 publications

(8 citation statements)

References 42 publications

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“…Most reports on nutrition in cystic fibrosis have been reviews, or include data collected in children (16)(17)(18)(19)(20)(21)(22)(23). In this study, anthropometric, nutritional, and lifestyle variables were examined to determine their impact on health parameters in an outpatient sample of adult patients with cystic fibrosis, with the hypothesis that nutritional status would be compromised as reflected by a decreased intake of energy and specific nutrients, and low BMI and fat mass.…”

Section: Introductionmentioning

confidence: 99%

Nutrient Status of Adults with Cystic Fibrosis

Gordon¹,

Anderson²,

Herlyn³

et al. 2007

Journal of the American Dietetic Association

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Nutrition is thought to influence disease status in patients with cystic fibrosis (CF). This crosssectional study sought to evaluate nutrient intake and anthropometric data from 64 adult outpatients with cystic fibrosis. Nutrient intake from food and supplements was compared with the Dietary Reference Intakes for 16 nutrients and outcomes influenced by nutritional status. Attention was given to vitamin D and calcium given potential skeletal implications due to cystic fibrosis. Measurements included weight, height, body composition, pulmonary function, and serum metabolic parameters. Participants were interviewed about dietary intake, supplement use, pulmonary function, sunlight exposure, and pain. The participants' mean body mass index (±standard deviation) was 21.8±4.9 and pulmonary function tests were normal. Seventy-eight percent used pancreatic enzyme replacement for malabsorption. Vitamin D deficiency [25-hydroxyvitamin D (25OHD)<37.5 nmol/L] was common: 25 (39%) were deficient despite adequate vitamin D intake. Lipid profiles were normal in the majority, even though total and saturated fat consumption represented 33.0% and 16.8% of energy intake, respectively. Reported protein intake represented 16.9% of total energy intake (range 10%-25%). For several nutrients, including vitamin D and calcium, intake from food and supplements in many participants exceeded recommended Tolerable Upper Intake Levels. Among adults with cystic fibrosis, vitamin D deficiency was common despite reported adequate intake, and lipid profiles were Copyright © 2007 Significant advances in the management of cystic fibrosis (CF) have increased the lifespan for patients with this common disease (1 in 3,900 white live births) (1). Previous research has suggested that patients with cystic fibrosis who have a good nutritional status have the best chance of maintaining and improving health (1-3). Nutritional therapy itself may also contribute to a better pulmonary status (4,5). A 1992 consensus report (1) emphasized the importance of detecting malnutrition in these patients to ensure optimal therapeutic management. A 2003 expert report on bone disease in cystic fibrosis cited both malnutrition and physical inactivity as risk factors for poor bone health (6).Osteoporosis is a common health problem as patients with cystic fibrosis survive longer (6-11), and one study of patients with this disease showed that decreased bone density was more prevalent in the most malnourished patients (8). A subnormal body mass index (BMI), among other factors, can compromise bone density (6,11,12). A high fracture risk and kyphosis are also documented in these patients (12). Serum vitamin D concentrations may be an important predictor of skeletal health, but the relationship between vitamin D status and bone density remains unclear in this disease (6,9-15).Most reports on nutrition in cystic fibrosis have been reviews, or include data collected in children (16)(17)(18)(19)(20)(21)(22)(23). In this study, anthropometric, nutritional, and lifestyle va...

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Section: Introductionmentioning

confidence: 99%

Nutrient Status of Adults with Cystic Fibrosis

Gordon¹,

Anderson²,

Herlyn³

et al. 2007

Journal of the American Dietetic Association

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“…Mesmo sendo considerado um método ouro para o diagnóstico da fibrose cística, é aconselhável realizar outros testes para confirmar a doença, mesmo quando encontrados níveis normais ou limítrofes de níveis de cloro no suor 21 .…”

Section: Diagnósticounclassified

“…Os microorganismos, na maioria das vezes, aparecem nas vias aéreas na seguinte ordem: Staphilococcus aureus, Haemophilus influenzae, Pseudomonas aeruginosa, Pseudomonas aeruginosa mucóide, Pseudomonas cepacea e membros do complexo Burkholderia cepacia 5,21,25 . Nos últimos anos, entretanto, outros microrganismos, especialmente gram-negativos não fermentadores de glicose, vêm sendo apontados como capazes de colonizar a árvore respiratória de portadores de fibrose cística 25 .…”

Section: Manifestações Respiratóriasunclassified

Fibrose cística: uma abordagem clínica e nutricional

et al. 2008

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A fibrose cística é a doença genética letal mais comum em populações caucasianas e é caracterizada por infecções crônicas e recorrentes do pulmão, insuficiência pancreática e elevados níveis de cloro no suor. É uma doença de herança autossômica recessiva causada pela mutação no gene do Regulador da Condutância Transmembrana da Fibrose Cística, que induz o organismo a produzir secreções espessas e viscosas que obstruem os pulmões, o pâncreas e o ducto biliar. Muitos pacientes apresentam insuficiência pancreática, que leva a má-absorção de nutrientes especialmente de proteínas e lipídeos e a complicações gastrintestinais tais como prolapso retal, síndrome da obstrução intestinal, constipação e cirrose hepática. A fibrose cística normalmente é diagnosticada na infância, pelos programas de triagem neonatal ou pelo teste do suor. Devido aos vários sistemas envolvidos e à variabilidade e cronicidade da doença, uma abordagem multidisciplinar é essencial para auxiliar o paciente e sua família a compreenderem a doença e aderirem ao tratamento. A terapia atual da fibrose cística inclui a manutenção do estado nutricional, a remoção das secreções das vias aéreas com fisioterapia e mucolíticos, o uso de antibióticos para prevenção e tratamento de infecções, a prescrição de suplementos energéticos, dietas hiperlípidicas e hiperprotéicas, bem como a suplementação de minerais e vitaminas lipossolúveis. O objetivo deste trabalho foi realizar breve revisão de literatura sobre os aspectos clínicos e nutricionais da fibrose cística.

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“…Vitamin B 12 deficiency should be assessed in patients with a history of ileal resection for meconium ileus [19]. • Routine iron supplementation is not recommended and theoretically may enhance the growth of Pseudomonas spp [20]. • Empiric zinc supplementation for a period of 6 months can be considered for CF patients who are failing to thrive or who have short stature or low vitamin A levels.…”

Section: Other Vitamins and Mineralsmentioning

confidence: 99%

Liver involvement in cystic fibrosis

Brigman

Feranchak

2006

Curr Treat Options Gastro

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The hepatobiliary manifestations of cystic fibrosis (CF) encompass a broad clinical spectrum, from mild steatosis, associated with poor nutrition, to multilobular cirrhosis and the complications of portal hypertension. The factor(s) responsible for the development and progression of liver disease in a subset of patients with CF are unknown. Liver disease can be silent and progressive, manifesting only with complications associated with cirrhosis and portal hypertension. Clinical evaluation for detecting and monitoring the progression of liver disease includes the following: physical examination of the liver, biochemical tests of liver function and injury, and radiological imaging with abdominal ultrasonography. Careful monitoring should take place in all patients with CF, as currently, there are no sensitive and/or specific historical or biochemical markers to predict who is at risk for the development of liver disease. Current treatment options for CF-associated liver disease are very limited. The bile acid ursodeoxycholic acid may improve biochemical parameters of liver disease, but its long-term efficacy in preventing the progression of liver disease in CF is unproven. Treatment therefore rests on optimizing nutritional status; correcting fat-soluble vitamin, essential fatty acid, and other mineral deficiencies; and treating complications of end-stage liver disease, such as pruritus, ascites, and portal hypertension.

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An overview of nutritional issues for the adult with cystic fibrosis

Cited by 17 publications

References 42 publications

Nutrient Status of Adults with Cystic Fibrosis

Nutrient Status of Adults with Cystic Fibrosis

Fibrose cística: uma abordagem clínica e nutricional

Liver involvement in cystic fibrosis

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