Nutrition for Children with Epidermolysis Bullosa

Haynes, Lesley

doi:10.1016/j.det.2010.01.010

Cited by 51 publications

(58 citation statements)

References 40 publications

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“…Patients with RDEB have increased caloric and nutrient needs, likely because of the metabolic demands of chronic wound healing, and meeting these demands is challenging because oral and esophageal erosions and scarring make eating difficult and gastrointestinal absorption may be abnormal. 16,17 Caloric and nutrient requirements vary from patient to patient depending on disease extent and severity, and standards for adequate caloric and nutrient values in this population do not exist. Although we did not quantify daily caloric and nutrient intake for our patients, most were taking calorically dense liquid supplements fortified with vitamins and minerals orally or via gastrostomy tube.…”

Section: Discussionmentioning

confidence: 98%

Correlates of low bone mass in children with generalized forms of epidermolysis bullosa

Bruckner¹,

Bedocs²,

Keiser³

et al. 2011

Journal of the American Academy of Dermatology

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Section: Discussionmentioning

confidence: 98%

Correlates of low bone mass in children with generalized forms of epidermolysis bullosa

Bruckner¹,

Bedocs²,

Keiser³

et al. 2011

Journal of the American Academy of Dermatology

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“…Controlling infection, optimizing growth, wound healing, and providing the best possible overall quality of life are important for managing EB in children 20,21. However, these goals all require maintenance of optimal nutritional status.…”

Section: Discussionmentioning

confidence: 99%

Nutritional Outcomes in Children with Epidermolysis Bullosa: The Experiences of Two Centers in Korea

et al. 2014

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PurposeEpidermolysis bullosa (EB) is associated with variable risks of extracutaneous manifestations and death. Currently, there is limited information on the clinical course and prognosis of EB in Korea. This study analyzed the nutritional outcomes, clinical morbidity, and mortality of children with EB.Materials and MethodsThirty patients, admitted to Severance Hospital and Gangnam Severance Hospital, from January 2001 to December 2011, were retrospectively enrolled. All patients were diagnosed with EB classified by dermatologists.ResultsAmong the 30 patients, 5 patients were diagnosed with EB simplex, four with junctional EB, and 21 with dystrophic EB. Wound infection occurred in 47% of the patients, and blood culture-proven sepsis was noted in 10% of the patients. Two (9.2%) patients had esophageal stricture and 11 (52.4%) of the dystrophic EB patients received reconstructive surgery due to distal extremity contracture. There were five mortalities caused by sepsis, failure to thrive, and severe metabolic acidosis with dehydration. According to nutrition and growth status, most of the infants (97%) were born as appropriate for gestational age. However, at last follow-up, 56% of the children were below the 3rd percentile in weight, and 50% were below the 3rd percentile in weight for height. Sixty percent of the children had a thrive index below -3.ConclusionPostnatal growth failure is a serious problem in children with EB. Strategies to maximize nutritional support could alleviate growth failure in children with EB, and thus improve clinical outcomes.

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“…Recurrent blistering can result in scarring and esophageal strictures, requiring dilatation. In order to meet nutritional requirements, both supplementary drinks and enteral feed via a gastrostomy tube can be used to reduce the risk of malnutrition …”

Section: Introductionmentioning

confidence: 99%

Retrospective longitudinal study of osteoporosis in adults with recessive dystrophic epidermolysis bullosa

Hubbard

Mayre-Chilton

2018

Clinical Case Reports

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Nutrition for Children with Epidermolysis Bullosa

Cited by 51 publications

References 40 publications

Correlates of low bone mass in children with generalized forms of epidermolysis bullosa

Correlates of low bone mass in children with generalized forms of epidermolysis bullosa

Nutritional Outcomes in Children with Epidermolysis Bullosa: The Experiences of Two Centers in Korea

Retrospective longitudinal study of osteoporosis in adults with recessive dystrophic epidermolysis bullosa

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