title: Nutritional outcomes in children with epidermolysis bullosa: the experiences of two centers in Korea.
abstract: OBJECTIVE
  Epidermolysis 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.
  
  
  METHODS
  Thirty 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.
  
  
  RESULTS
  Among 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.
  
  
  CONCLUSIONS
  Postnatal 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.
authors: Department of Pediatrics, Gangnam Severance Hospital, Yonsei University College of Medicine, 211 Eonju-ro, Gangnam-gu, Seoul 135-720, Korea. smlee@yuhs.ac.
authors: Kyu-Yeun
authors: KY
authors: Kim
authors: Ran
authors: R
authors: Namgung
authors: Soon Min
authors: SM
authors: Lee
authors: Soo Chan
authors: SC
authors: Kim
authors: Ho Sun
authors: HS
authors: Eun
authors: Min Soo
authors: MS
authors: Park
authors: Kook In
authors: KI
authors: Park
authors: Chul
authors: C
authors: Lee
mesh_terms: D001724:Birth Weight / Q000502:physiology; D004820:Epidermolysis Bullosa / Q000503:physiopathology*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007723:Korea; D008297:Male; D011247:Pregnancy; D056910:Republic of Korea; D012189:Retrospective Studies
keywords: Epidermolysis bullosa; growth failure; nutritional outcome