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.

        · abstract in Spanish     · text in English     · English ( pdf )