O tema que me propus desenvolver possibilitou-me o acesso a bibliografia diversificada mas por vezes contraditória. A FQ é uma doença complexa com um diverso espectro de mutações e fenótipos clínicos.
Apesar dos avanços no conhecimento da função do gene CFTR o passo crucial pelo qual a disfunção CFTR conduz às características clínicas permanece por esclarecer (29).
Ainda não há uniformidade em muitos dos consentimentos Europeus, por exemplo, ainda não estão definidas as infra-estruturas necessárias para os centros de FQ, os métodos de avaliação e medição na rotina dos doentes e o tratamento das complicações (33). É urgente desenvolver e validar métodos sensitivos de composição corporal para aplicar na prática clínica.
A FQ permanece uma doença grave para a qual ainda não existe tratamento curativo, mas na qual a esperança média e a qualidade de vida melhoram, progressivamente, em razão de um melhor tratamento por equipas multidisciplinares, pela optimização da cinesoterapia respiratória, pela antibioterapia e pelo suporte nutricional (6).
A implementação de programas nutricionais de reabilitação como parte do tratamento clínico da FQ permitiu reduzir a prevalência de estados de deficiência nutricional, de malnutrição proteico-energética e atrasos de crescimento. A reabilitação nutricional melhora a função pulmonar, principalmente nos estágios precoces de doença pulmonar (27). Assim os doentes com FQ requerem PERT, uma dieta hipercalórica, hiperlipídica e suplementada com nutrientes essenciais, especialmente vitaminas lipossolúveis e AGE. A inflamação crónica e o défice de AG encontradas nos doentes com FQ são bons argumentos para utilizar
suplementos de PUFAs-w3. No entanto, não existe qualquer estudo conclusivo quanto à utilização de PUFAs-w3 nesta população (37, 115). Todas as crianças com FQ beneficiam de uma terapia nutricional agressiva.
O uso de suplementos proteico - energéticos é procurado, já que melhoram o estado nutricional dos doentes com FQ, mas é necessária vigilância para assegurar que não são usados como substitutos de alimentos (25, 50, 65). O próximo passo é estabelecer o efeito da suplementação na função pulmonar, qualidade e esperança de vida dos doentes com FQ (12).
Quando se verifica uma acentuada degradação do estado nutricional deve recorrer-se à nutrição artificial, principalmente a NE. A alimentação por gastrostomia e o recurso a NE nocturna tornaram-se intervenções comuns na terapêutica nutricional da FQ (41).
Novas terapêuticas para melhorar o estado nutricional na FQ estão a ser estudadas. Estimuladores do apetite e agentes anabólicos são terapêuticas promissoras para a FQ (24).
A importância do tratamento nutricional na FQ será certamente um assunto que continuará a merecer a minha atenção, em possíveis estudos futuros.
9. Conclusão
A FQ é uma doença autossómica recessiva, multissistémica que afecta o transporte epitelial de Cl". As manifestações gastrointestinais são diversas, com potencial disfunção do pâncreas, intestino, fígado e árvore biliar. Para além da doença pancreática, o envolvimento pulmonar e gastrointestinal, podem contribuir para o comprometimento do estado nutricional.
A malnutrição correlaciona-se com a severidade da doença pulmonar e com a esperança média de vida dos doentes com FQ. A avaliação do estado nutricional permite detectar precocemente a malnutrição, possibilitando que o suporte nutricional se torne mais eficaz. Este tem de ser individualizado, tendo em atenção a situação clínica, necessidades nutricionais e vias de administração possíveis.
O suporte nutricional pode ser feito pela via oral, entérica e parentérica. Cada método tem indicações precisas e adequadas. Deve insistir-se na via oral, modificando os alimentos e suas preparações, podendo mesmo enriquecer a alimentação oral com suplementos. Quando esta via é insuficiente deve recorrer- se à nutrição artificial, sendo a nutrição entérica preferível à nutrição parentérica.
É portanto de extrema importância a instituição de um suporte alimentar/nutricional adequado, de forma a evitar a malnutrição e a doença pulmonar precoce.
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