Nutritional Supplementation in Patients with Cystic Fibrosis

Adde, Fabíola Villac; Dolce, Pasquale; Tanikawa, E; Uehara, D.; Cardoso, Ary L.; Rozov, Tatiana

doi:10.1203/00006450-199811000-00033

Cited by 3 publications

(3 citation statements)

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“…TTR also proved useful for monitoring the dietetic management of anorexia nervosa ( 59 ) and weight-reduction programs ( 60 ). Likewise, the use of TTR is recommended for the nutritional follow-up of a variety of genetic and metabolic disorders such as uncontrolled diabetes ( 61 ), cystic fibrosis ( 62 ), drepanocytosis ( 63 ), Reye syndrome ( 64 ), inborn errors of AA metabolism ( 65 ), and defects of the urea cycle ( 66 ).…”

Section: Importance Of Ttr In Protein-depleted Statesmentioning

confidence: 99%

Plasma Transthyretin as a Biomarker of Lean Body Mass and Catabolic States

Ingenbleek¹,

Bernstein

2015

Advances in Nutrition

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Plasma transthyretin (TTR) is a plasma protein secreted by the liver that circulates bound to retinol-binding protein 4 (RBP4) and its retinol ligand. TTR is the sole plasma protein that reveals from birth to old age evolutionary patterns that are closely superimposable to those of lean body mass (LBM) and thus works as the best surrogate analyte of LBM. Any alteration in energy-to-protein balance impairs the accretion of LBM reserves and causes early depression of TTR production. In acute inflammatory states, cytokines induce urinary leakage of nitrogenous catabolites, deplete LBM stores, and cause an abrupt decrease in TTR and RBP4 concentrations. As a result, thyroxine and retinol ligands are released in free form, creating a second frontline that strengthens that primarily initiated by cytokines. Malnutrition and inflammation thus keep in check TTR and RBP4 secretion by using distinct and unrelated physiologic pathways, but they operate in concert to downregulate LBM stores. The biomarker complex integrates these opposite mechanisms at any time and thereby constitutes an ideally suited tool to determine residual LBM resources still available for metabolic responses, hence predicting outcomes of the most interwoven disease conditions.

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Section: Importance Of Ttr In Protein-depleted Statesmentioning

confidence: 99%

Plasma Transthyretin as a Biomarker of Lean Body Mass and Catabolic States

Ingenbleek¹,

Bernstein

2015

Advances in Nutrition

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“…A study carried out on 102 children (51 malnourished and 51 well-nourished) in 1988 revealed that prealbumin is a sensitive and reliable marker to diagnose acute malnutrition when used along with anthropometric measurements. 12 Prealbumin is also recommended as nutritional status indicator in several early studies, in various chronic disease conditions such as uncontrolled diabetes mellitus, 13 Reye syndrome, 14 cystic fibrosis 15 and anorexia nervosa. 16 It was also found to be useful in monitoring protein-energy status in the metabolic conditions like inborn errors of metabolism 17 and urea cycle defects.…”

Section: Prealbumin As a Nutritional Markermentioning

confidence: 99%

Prealbumin: The clinical utility and analytical methodologies

2020

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Prealbumin is a small protein which has been widely evaluated as a nutritional and a prognostic marker. The small size and concentration of prealbumin in blood proposes challenges on measuring it with high sensitivity and specificity. Over the years, a number of analytical methodologies have been developed, which may help establish prealbumin as a useful biomarker in routine clinical practice. The aim of the short review was to explore the current literature on the clinical utility of prealbumin and the advances made in the analytical methodologies of prealbumin. We searched MEDLINE, EMBASE and the Cochrane Library for articles published between January 1980 and July 2019, with the general search terms of ‘prealbumin’, ‘prognostic marker’, ‘nutritional marker’, ‘analytical methodologies’ and ‘malnutrition’. Additionally, we selected relevant articles and comprehensive overviews from reference lists of identified studies. The routine use of prealbumin in clinical practice remains debatable; however; it can complement clinical history, anthropometric assessment and physical examination to assess malnutrition with more certainty. Consensus on the clinical applications of prealbumin in the management of malnutrition is warranted.

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“…Além da depressão emocional, os fibrocísticos apresentam diminuição do paladar pela deficiência de zinco, aumento da secreção pulmonar, dispneia, esofagite causada pelo refluxo gastroesofágico e iatrogenia, quando há restrição lipídica na alimentação. Outros fatores que também contribuem para esse déficit energético são: glicosúria, devido ao diabetes melito (DM) por falência do pâncreas endócrino, e os vômitos resultantes do refluxo gastroesofágico (RGE) (15) .…”

Section: Metabolismo Energético Na Fibrose Císticaunclassified

Avaliação e recomendações nutricionais para crianças e adolescentes com fibrose cística

Chaves¹,

Cunha²

2012

Rev. paul. pediatr.

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OBJETIVO: Revisar e discutir evidências sobre a avaliação do estado nutricional e as recomendações para o tratamento nutricional de crianças e adolescentes com fibrose cística. FONTES DE DADOS: Bancos de dados MEDLINE (versão PubMed) e Latin American and Caribbean Center on Health Sciences Information (LILACS), entre 1984 a 2009. Aplicou-se a combinação dos seguintes descritores: fibrose cística, estado nutricional, criança e adolescente - em inglês e português. SÍNTESE DOS DADOS: A fibrose cística é uma doença genética, de evolução crônica, progressiva e fatal. Resulta do defeito na proteína reguladora transmembrana que regula a condução de cloro e, consequentemente, o fluxo de sódio e água através da membrana apical das células epiteliais. Pacientes fibrocísticos são vulneráveis à desnutrição, que resulta do desequilíbrio entre ingestão alimentar, gasto e perdas energéticas. Novos conhecimentos sobre a fisiopatologia da doença, adquiridos nas últimas décadas, resultaram em mudanças significativas nas atuais recomendações energéticas e principalmente de lipídeos. A importância da nutrição no bem-estar e sobrevida dos fibrocísticos está bem estabelecida, assim como a associação entre a desnutrição e a deterioração da função pulmonar. Existem múltiplos fatores inter-relacionados que afetam o estado nutricional, tais como, mutação genética, insuficiência pancreática, ressecção intestinal, perda de sais e ácidos biliares, refluxo gastroesofágico, inflamação e infecções pulmonares, diabetes e condições emocionais. CONCLUSÕES: O monitoramento nutricional e o aconselhamento dietético são elementos chave no manejo de crianças e adolescentes com fibrose cística com o intuito de controlar a sintomatologia e a progressão da doença, proporcionando melhor qualidade de vida.

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Nutritional Supplementation in Patients with Cystic Fibrosis

Cited by 3 publications

References 0 publications

Plasma Transthyretin as a Biomarker of Lean Body Mass and Catabolic States

Plasma Transthyretin as a Biomarker of Lean Body Mass and Catabolic States

Prealbumin: The clinical utility and analytical methodologies

Avaliação e recomendações nutricionais para crianças e adolescentes com fibrose cística

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