Cystic Fibrosis, Nutrition, and the Health Care Team

Creveling, Susan; Light, Michael J.; Gardner, Pamela; Greene, Linda

doi:10.1016/s0002-8223(97)00761-x

Cited by 9 publications

(11 citation statements)

References 3 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.…”

mentioning

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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mentioning

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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“…[29][30][31] How ever, predi cted w ei ght for hei ght i n CF i s affected by eati ng habi ts and l i festyl e, [32][33][34] type of and adherence to hi gh-cal ori e di et, [32][33][34] admi ni strati on of pancreati c enzymes and fat-sol ubl e vi tami ns to treat exocri ne pancreati c i nsuffi ci ency and mal digesti on of nutri ents 35 and frequency and severi ty of respi ratory i nfecti ons. 36 All investigated patient pai rs, w i th the excepti on of a few adul ts, shared homes, fami l y l i fe and CF physi ci an, and w ere thus exposed to the same nutri ti on and medi cal experti se.…”

Section: Discussionmentioning

confidence: 99%

Categories of ΔF508 homozygous cystic fibrosis twin and sibling pairs with distinct phenotypic characteristics

Mekus¹,

Ballmann

Bronsveld

et al. 2000

Twin res.

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Cysti c fi br osi s (CF), the most common sever e autosomal r ecessi ve tr ai t among Caucasi ans, i s caused by mol ecul ar l esi ons i n the cysti c fi br osi s tr ansmembr ane conductance r egul ator gene (CFTR). The cour se of the mul ti -or gan di sease CF i s hi ghl y var i abl e, suggesti ng the i nfl uence of envi r onmental factor s and/or modul ati ng genes other than CFTR on the di sease phenotype. To evaluate the cause of CF disease variability, the European CF Twin and Sibling Study col l ected data on tw o cl i ni cal par ameter s most sensi ti ve for the cour se and pr ognosi s of CF, i e w ei ght pr edi cted for hei ght (w fh)% (r epr esentati ve for the nutr i ti onal status) and FEVPer c (r epr esentati ve for the pul monar y status) for a cohor t of 277 si bl i ng pai r s, 12 pai r s of di zygous tw i ns and 29 pai r s of monozygous tw i ns. Of these 318 CF tw i n and si b pai r s, 114 w er e r epor ted to be homozygous for the most fr equent CF di sease-causi ng l esi on, ∆F508. I ntr a-pai r di scor dance w as assessed by the i ntr a-pai r di ffer ences w i th w fh% and FEVPer c and by DELTA , a composi te par ameter defi ned by l i near combi nati on of w fh% and FEVPer c i n or der to descr i be di scor dance w i th r espect to the overall disease severity. Monozygous twins had a significantly lower DELTA than dizygous twins (P = 0.05) i ndi cati ng that CF di sease sever i ty i s modul ated by an i nher i ted component i n addi ti on to the CFTR gene i tsel f. Extr eme phenotypes ar e consi der ed to be mor e i nfor mati ve for the anal ysi s of any quanti tati ve tr ai t. Thus, w e ai med to quanti fy di sease sever i ty and i ntr a-pai r di scor dance i n or der to sel ect pai r s w i th the extr eme phenotypes DI S (di scor dant pati ent pai r s), CON + (concor dant and mi l dy affected pati ent pai r s) and CON -(concor dant and sever el y affected pati ent pairs). The algorithm reliably discriminated between pairs DIS, CON + and CON -among the cohor t of ∆F508 homozygotes. The sel ected pai r s fr om these categor i es demonstr ated non-over l appi ng pr oper ti es for w fh%, FEVPer c and the i ntr a-pai r di ffer ence of both par ameter s. Twin Research (2000) 3, 277-293. Keyw or ds: cysti c fi brosi s di sease severi ty, affected rel ati ve pai r, tw i n and si bl i ng study, extreme phenotypes, al gori thm based sel ecti on I ntr oducti on Cysti c fi brosi s (CF) i s know n as the most common severe autosomal recessi ve di sease w i thi n the Caucasi an popul ati on, exhi bi ti ng an i nci dence of 1 i n 2500 bi rths.1 The symptoms of the di sorder are caused by an i mpai red functi on of exocri ne gl ands i n many organs, but major mani festati ons i nvol ve the respi ratory and the gastroi ntesti nal tracts.1 The di sease i s caused by mutati ons i n both chromosomal copi es of the cysti c fi brosi s transmembrane conductance regul ator (CFTR) gene. 2 The course of CF i s hi ghl y vari abl e w hen compari ng unrel ated pati ents w i th i denti cal CFTR mutati on genotypes, 3,4 o...

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“…Outra conseqüência desta doença no pâncreas é a diminuição da secreção de bicarbonato de sódio, que evitaria influxos de ácidos gástricos no duodeno, reduzindo a eficácia das enzimas pancreáticas e precipitação de sais biliares 29 . A baixa concentração de bicarbonato no suco pancreático faz com que o pH do duodeno seja ácido, e isso contribui para a má-absorção 11 .…”

Section: Manifestações Gastrintestinaisunclassified

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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Cystic Fibrosis, Nutrition, and the Health Care Team

Cited by 9 publications

References 3 publications

Nutrient Status of Adults with Cystic Fibrosis

Nutrient Status of Adults with Cystic Fibrosis

Categories of ΔF508 homozygous cystic fibrosis twin and sibling pairs with distinct phenotypic characteristics

Fibrose cística: uma abordagem clínica e nutricional

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