Nutritional outcomes in children with epidermolysis bullosa: long-term follow-up.

BACKGROUND: some types of epidermolysis bullosa (EB) have extracutaneous manifestations. Manifestations that limit food intake and absorption may compromise nutritional status and increasing nutritional requirements. OBJECTIVES: to investigate the following nutritional status indicators: exclusive breastfeeding duration, problems caused by the introduction of complementary foods, birth weight and length, and growth curves of children with EB. METHODS: assessment was based on the World Health Organization (WHO) growth charts. The anthropometric data were stored in the WHO's programs Anthro and Anthro Plus. RESULTS: three and seven of the ten study children had EB simplex (EBS) and recessive dystrophic EB (RDEB), respectively. Four of the children with RDEB had problems when complementary foods were introduced. The difference between the chronological age and age-for-height at the 25th (A/H 25th) percentile (p) varied from four months to four years and two months. Most children with RDEB (85%) had weight-for-age (W/A) curve below p3 and low height-for-age (H/A), starting before age four years. One child with EBS had excess weight. CONCLUSIONS: anthropometric birth data, exclusive breastfeeding duration, and problems caused by the introduction of complementary foods are useful information for establishing the nutritional profile of children with EB. Supposedly, breastfeeding and no complementary feeding problems were not enough to prevent inadequate nutritional status, observed in the majority of the study children. The original presentation of the growth curves of children with EB may help to determine nutritional involvement and to establish how these children grow. The evaluation of growth curves with WHO as a standard suggests the need to establish growth curves adapted to the most serious type of EB and the need for permanent nutritional monitoring.

Nutritional outcomes in children with epidermolysis bullosa: long-term follow-up / Resultados nutricionales en niños con epidermólisis bullosa: seguimiento a largo plazo

Background: some types of epidermolysis bullosa (EB) have extracutaneous manifestations. Manifestations that limit food intake and absorption may compromise nutritional status and increasing nutritional requirements. Objectives: to investigate the following nutritional status indicators: exclusive breastfeeding duration, problems caused by the introduction of complementary foods, birth weight and length, and growth curves of children with EB. Methods: assessment was based on the World Health Organization (WHO) growth charts. The anthropometric data were stored in the WHO's programs Anthro and Anthro Plus. Results: three and seven of the ten study children had EB simplex (EBS) and recessive dystrophic EB (RDEB), respectively. Four of the children with RDEB had problems when complementary foods were introduced. The difference between the chronological age and age-for-height at the 25th (A/H 25th) percentile (p) varied from four months to four years and two months. Most children with RDEB (85%) had weight-for-age (W/A) curve below p3 and low height-for-age (H/A), starting before age four years. One child with EBS had excess weight. Conclusions: anthropometric birth data, exclusive breastfeeding duration, and problems caused by the introduction of complementary foods are useful information for establishing the nutritional profile of children with EB. Supposedly, breastfeeding and no complementary feeding problems were not enough to prevent inadequate nutritional status, observed in the majority of the study children. The original presentation of the growth curves of children with EB may help to determine nutritional involvement and to establish how these children grow. The evaluation of growth curves with WHO as a standard suggests the need to establish growth curves adapted to the most serious type of EB and the need for permanent nutritional monitoring

Introducción: algunos tipos de epidermólisis ampollosa (EB) presentan manifestaciones extracutáneas. Las manifestaciones que limitan la ingesta y absorción de alimentos pueden comprometer el estado nutricional y aumentar las necesidades nutricionales. Objetivos: investigar los siguientes indicadores de estado nutricional: duración exclusiva de la lactancia, problemas causados por la introducción de alimentos complementarios, peso y longitud al nacer y curvas de crecimiento de los niños con EB. Métodos: la evaluación se basó en los gráficos de crecimiento de la Organización Mundial de la Salud (OMS). Los datos antropométricos se almacenaron en los programas de la OMS Anthro y Anthro Plus. Resultados: tres y siete de los diez niños del estudio tenían EB simplex (EBS) y EB distrófica recesiva (RDEB), respectivamente. Cuatro de los niños con RDEB tuvieron problemas cuando se introdujeron alimentos complementarios. La diferencia entre la edad cronológica y la edad para la talla en el percentil 25 (A/H 25) (p) varió de cuatro meses a cuatro años y dos meses. La mayoría de los niños con RDEB (85%) tenían una curva de peso por edad (W/A) por debajo de p3 y baja altura por edad (H/A), comenzando antes de los cuatro años de edad. Un niño con EBS tenía exceso de peso. Conclusiones: los datos antropométricos de nacimiento, la duración de la lactancia exclusiva y los problemas causados por la introducción de alimentos complementarios son información útil para establecer el perfil nutricional de los niños con EB. La presentación original de las curvas de crecimiento de los niños con EB puede ayudar a determinar la implicación nutricional y establecer cómo estos niños crecen. La evaluación de las curvas de crecimiento con la OMS como patrón sugiere la necesidad de establecer curvas de crecimiento adaptadas al tipo más grave de EB y la necesidad de un seguimiento nutricional permanente

Nutritional aspects of children and adolescents with epidermolysis bullosa: literature review.

Epidermolysis Bullosa is a genetic disorder that affects mainly the skin, however, all others systems are influenced. The nutritional care of children and adolescents with Epidermolysis Bullosa is a key treatment strategy, since the energy needs are increased due to the disease's metabolism, burdening the immune system and cicatrization process, symptoms caused by the disease hinder the intake and adequate absorption of nutrients, which may result in inadequate growth and development. Because this is a rare disease, there are few professionals who know the characteristics of both the clinical evolution and nutritional and dietary treatments. This literature review discusses the latest knowledge on energy and specific nutrient requirements to the dietary treatment and monitoring of children and adolescents with Epidermolysis Bullosa.

Nutritional aspects of children and adolescents with epidermolysis bullosa: literature review

Epidermolysis Bullosa is a genetic disorder that affects mainly the skin, however, all others systems are influenced. The nutritional care of children and adolescents with Epidermolysis Bullosa is a key treatment strategy, since the energy needs are increased due to the disease's metabolism, burdening the immune system and cicatrization process, symptoms caused by the disease hinder the intake and adequate absorption of nutrients, which may result in inadequate growth and development. Because this is a rare disease, there are few professionals who know the characteristics of both the clinical evolution and nutritional and dietary treatments. This literature review discusses the latest knowledge on energy and specific nutrient requirements to the dietary treatment and monitoring of children and adolescents with Epidermolysis Bullosa.