Arquivos de Asma, Alergia e Imunologia
https://www.aaai-asbai.org.br/article/doi/10.5935/2526-5393.20200033
Arquivos de Asma, Alergia e Imunologia
Clinical and Experimental Communication

Terapia nutricional de paciente com ataxia-telangiectasia: relato de caso

Nutrition therapy of a patient with ataxia-telangiectasia: case report

Talita Lemos Neves Barreto; Juliana Fernandez Santana Meneses; Roseli Oselka Saccardo Sarni

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Resumo

Neste artigo relatamos a terapia nutricional de um paciente com ataxia-telangiectasia (A-T) utilizando a gastrostomia (GTM) como via alternativa para alimentação. Paciente do sexo masculino, 13 anos de idade, com diagnóstico clínico de A-T aos 6 anos. Aos 8 anos e 7 meses o paciente foi identificado com risco nutricional (ZIMC/I: -1,67). Após 1 ano, evoluiu de forma desfavorável (ZIMC/I: -2,51) apesar da intervenção nutricional, sendo indicada a GTM aos 9 anos e 11 meses. No entanto, em decorrência da dificuldade de aceitação dos pais, o procedimento foi realizado somente quando o adolescente completou 11 anos e 7 meses. Inicialmente foi prescrita para oferta pela GTM dieta enteral normocalórica e normoproteica, correspondendo a 45,8% da necessidade energética diária. Após um mês, com estabilidade metabólica, houve a transição para uma dieta enteral hipercalórica e hiperproteica, fornecendo 91,6% da necessidade energética diária. Após 6 meses com a GTM, verificou-se ganho de peso total de 3,3 Kg (ZIMC/I -2,97), após 1 ano de 4,7 Kg (ZIMC/I -2,59), e após 1 ano e 9 meses de 6,7 Kg (ZIMC/I -2,63). Apesar da desnutrição nos pacientes com A-T ter origem multifatorial, o uso da GTM como via alternativa para alimentação por esse paciente resultou em uma evolução favorável dos seus indicadores antropométricos, sendo relatadas poucas intercorrências com a sua utilização. Portanto, sugere-se que pacientes com A-T devam ser monitorados periodicamente por equipe multiprofissional visando à identificação precoce de potenciais agravos.

Palavras-chave

Ataxia telangiectasia, terapia nutricional, gastrostomia.

Abstract

In this article we report the nutritional therapy of a patient with ataxia-telangiectasia (A-T) using percutaneous endoscopic gastrostomy (PEG) as an alternative way of feeding. The patient was a 13-year-old male diagnosed with A-T at the age of 6 years. At 8 years and 7 months, the patient was at nutritional risk (body mass index z-score [BMIZ]: -1.67). After 1 year, he had an unfavorable evolution (BMIZ: -2.51), despite nutritional intervention; then, a PEG was indicated when he was 9 years and 11 months. However, due to the difficulty of parental acceptance, the procedure was performed when the adolescent was 11 years and 7 months. At first, a standard energy and protein enteral formula was prescribed, reaching 45.8% of his daily energy requirement. After 1 month, with metabolic stability, there was a transition to a high-energy and protein enteral formula providing 91.6% of his daily energy requirement. After 6 months of PEG placement, the patient had a total body weight gain of 3.3 kg (BMIZ: -2.97); subsequently, body weight increased by 4.7 kg (BMIZ: -2.59) after 1 year, and by 6.7 kg (BMIZ: -2.63) after 1 year and 9 months. Despite the multifactorial origin of malnutrition in A-T patients, PEG placement as an alternative way of feeding for this patient resulted in favorable evolution of his anthropometric indicators, and only a few complications were reported with its use. Therefore, it is suggested that patients with A-T should be monitored periodically by a multidisciplinary team for early identification of potential damages.

Keywords

Ataxia telangiectasia, nutrition therapy, gastrostomy.

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Submitted date:
04/23/2020

Accepted date:
05/04/2020

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