EPIDERMOLISIS BULLOSA EN NIOS PDF

EB 1st Global Congress on Epidermolysis Bullosa This ground-breaking meeting will, for the first time, bring together all global knowledge in EB research, . Prevención de Ampollas. Como cargar al bebé: Evite levantar el bebé o niño por debajo de los brazos. En su lugar, ponga una mano debajo del trasero y la otra. Epidermolisis Bullosa distrófica (Niños mariposa) Es una enfermedad caracterizada por la fragilidad de la piel y las mucosas. Quienes la padecen poseen una.

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School of Health Sciences. Manifestations that limit food intake and absorption may compromise nutritional status and increasing nutritional requirements.

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Four of the children with RDEB had problems when complementary foods were introduced. One child with EBS had excess weight. 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 epdiermolisis determine nutritional involvement and to establish how these children grow.

The evaluation of growth curves with WHO as epidermolsis standard suggests the need to establish growth curves adapted to the most serious type of EB and the need for permanent nutritional monitoring. The term epidermolysis bullosa EBintroduced inrefers to a group of elidermolisis blistering diseases characterized by blistering on the skin and mucosa caused by minimum mechanical trauma 12. EB is associated with protein changes that cause defective adherence between skin structures, eipdermolisis to blistering 3.

Traditionally, four main types of EB have been identified based on epidernolisis differences at the ultrastructural level where the blisters develop: EB severity varies from mild to severe, and skin involvement may be localized or generalized.

Skin findings may include blisters, scars, changes in pigmentation, alopecia, absent or dystrophic nails, and hand or foot deformities 45. Some types of EB may also cause extracutaneous manifestations. Nutritional status may be compromised by clinical oral, pharyngeal, esophageal, and gastrointestinal manifestations that limit food intake and absorption. Hypermetabolism resulting from skin lesion inflammation may also occur 6.

Nutritional involvement in EB is directly related to the severity of the associated clinical complications 6. Since complications vary in number and intensity over time, it is very difficult to assess whether EB individuals have adequate ej and nutritional status.

Weight and growth rate monitoring is a recognized way to verify the growth adequacy of healthy children, but comparison of healthy children’s growth rates with those of children with EB has limited value 7. Despite knowledge of the nutritional risk of EB individuals, no assessment parameters exist, and growth charts for healthy children continue to be used 8.

This study investigated the following nutritional status indicators in children with EB: All subjects provided informed consent. The study included all confirmed cases of Bullossa aged less than 19 years, treated at the Pediatric Nutrition outpatient clinic of the Epideermolisis Hospital of Brasilia, a reference public service opened in The clinic employs a multidisciplinary team who has assisted roughly 20 patients and currently follows ten patients. Some patient data were collected from the medical records, such as the information registered between August and Maywhich included sociodemographic characteristics, age at diagnosis, birth conditions, and anthropometric measurements collected during the entire follow-up period.

Given the skin fragility of epidermplisis patients, anthropometric assessment consisted only of weight and height measurement. Skinfold thicknesses and perimeters were not measured because the instruments needed for these measurements would press or pinch the skin. Weight and height as a function of age and gender were presented in growth curves by percentile p.

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The following variables were assessed during the interview, when the informed consent form was signed: This comparison was done because the equation that estimates the energy e of EB patients 6 uses p25 as epidermolisid of adequacy. The mean age was ten years, ranging from one to 18 years.

Age at diagnosis varied from the first day of life until after the first year of life. Dpidermolisis patients were bulloaa at term, and nine cases had birth weight of 2, grams or more. The child with low birth weight had RDEB. Nine children were breastfed.

One child with RDEB was not breastfed because of the clinical manifestations of the disease. Three of the breastfed children were exclusively bulolsa for six months. One child was exclusively breastfed for 18 months, also because of the clinical manifestations of the disease, which prevented the child from accepting complementary foods. Problems related to the clinical manifestations of the disease caused episermolisis the introduction of complementary foods Table I did not occur in children with EBS, but they occurred in four children with RDEB.

No patterns rpidermolisis found regarding the type of problem the children experienced. This comparison could not be done for one patient because his birth length had not been recorded in the medical record. When sex was considered, the biggest difference was found in a female patient. The last anthropometric assessment included in this study found that all RDEB patients were underweight and stunted.

Obese child, appropriate height-for-age. EBS patients did not have impaired growth. Regarding the difference in years between the chronological age and the age corrected according to current height for the p25 growth curve, RDEB patients epiderjolisis from EBS patients.

We hope this information will contribute to the establishment epdiermolisis the nutritional profile of EB individuals, especially in the first year of life. EB children may be born with normal weight, but in some cases low weight may begin in the uterus.

Fox, Alderdice, and Atherton 11 found that English RDEB children had significantly lower birth weights than their healthy counterparts. On the other hand, the epidermoliiss and other studies 1213 found that term children generally born with normal weight- and length-for-age stopped growing and gaining weight properly after the clinical manifestations of the disease appeared, as they compromised food intake and increased nutritional requirements.

These divergent results indicate the need of more studies that thoroughly investigate birth conditions and short- medium- and long-term nutritional involvement. This study was not able to assess whether nutritional deficits were associated with late disease diagnosis. Nevertheless, in the same way that prognosis is related to EB type 14it is plausible that worse nutritional status is present in the severe EB types.

Other studies may be able to clarify whether early EB diagnosis, especially in the more severe cases, results in better epidremolisis. The WHO recommends exclusive breastfeeding for six months and complemented breastfeeding for two years or more.

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This practice efficiently promotes nioz growth, avoids infant death, diarrhea, and respiratory infections, and reduces the risk of allergies and obesity, among many other benefits 15 Literature data on the breastfeeding duration of EB children were not found, but the onset of manifestations may significantly affect the exclusive breastfeeding duration.

According to the WHO, the foods introduced after age six months should complement the numerous qualities and functions of breastmilk.

In addition to meeting nutritional requirements, the introduction of complementary foods helps bullosx child to progressively acquire the caregiver’s food habits. Moreover, entering a new life stage, where children are exposed to new tastes, colors, aromas, textures, and knowledge, requires an adaptive effort Similar to the present study, Birge 12 found that EB manifestations compromise food intake.

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Haynes 17 states that the critical interference on food intake begins during dentition and diseases that occur in the first year of life. The study findings regarding the weight and height of EB children are similar to those in the literature. The present study could not find length data before age 24 months, so it nullosa not possible to determine when hios growth began, but it may have been between the first year of life and the age at the first visit since bulloda patients had normal birth length.

The high percentage of malnourished RDEB children is also evidenced by the nutritional status diagnosis of the study sample. Generally, although RDEB is a severe disease subtype, the study RDEB patients did not have the same growth pattern, and some were more malnourished than others.

EB children and adolescents have higher energy epicermolisis nutrient requirements because of the constant blister healing process, skin losses, skin heat loss, and inflammatory and infectious processes that may affect the lesions 7. The association between low food intake and higher nutrient requirements compromises nutritional status and consequently, leads to malnutrition Therefore, weight and height should be measured in short intervals to allow an appropriate and timely intervention.

The study limitations include its retrospective character, the lack of epidermmolisis weight and height information in the patients’ medical records, and the possible measurement errors.

Although measuring weight and height is a simple procedure, doing so in children with pain, infected blisters, difficulty to extend the lower or upper limbs, and restricted ability to maintain the back and neck straight constrains the use of standard measuring procedures. The great advantage of the present study is that it brought to light information about the nutritional aspects of EB children as it is in this age group that it is possible to minimize nutritional sequelae, promote growth, development, and better quality of life, and optimize the appropriate interventions.

Nonetheless, more studies, especially prospective cohort studies about dn and feeding in the first year of life, with well-defined methodologies, are needed to better understand epiderjolisis contextualize these data for later use by services that bulloa this population. Future studies should also assess the need of creating specific growth curves for children with the more severe types of EB.

New diagnostic criteria and classification. Extracutaneous manifestations and complications of inherited epidermolysis bullosa.

J Am Acad Dermatol ; An Bras Dermatol ;85 6: Management of pediatric epidermolysis bullosa. Clinical practice guidelines for nutrition support: In infants and children with epidermolysis bullosa EB. Great Ormond Street Hospital; Accessed 13 August Nutrition for children with epidermolysis bullosa. A consensus approach to wound care in epidermolysis bullosa. J Am Acad Dermatol ;67 5: Accessed 5 November World Health Organization; Accessed 29 September Software for assessing growth of the world’s children and adolescents.

Accessed 25 August Are children with recessive dystrophic epidermolysis bullosa of low birthweight? Nutrition management of patients with epidermolysis bullosa. J Am Diet Assoc ; Nutritional outcomes in children with epideermolisis bullosa: The experiences of two centers in Korea. Yonsei Med J ;55 1: Review inherited epidermolysis bullosa.

Fine Orphanet J Rare Dis ; 5: Accessed 12 March