지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2014.1
- 수록면
- 264 - 269 (6page)
이용수
초록· 키워드
오류제보하기
Purpose: 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. Materials and 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. Conclusion: 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.
목차
등록된 정보가 없습니다.
참고문헌 (23)
참고문헌 신청
Boeira VL / 2013 / Inherited epidermolysis bullosa: clinical and therapeutic aspects / An Bras Dermatol 88 : 185 ~ 198
Fewtrell MS / 2006 / Bone mineralization in children with epidermolysis bullosa / Br J Dermatol 154 : 959 ~ 962
Fine JD / 1986 / Epidermolysis bullosa. Clinical aspects, pathology, and recent advances in research / Int J Dermatol 25 : 143 ~ 157
Fine JD / 2005 / Pseudosyndactyly and musculoskeletal contractures in inherited epidermolysis bullosa: experience of the National Epidermolysis Bullosa Registry, 1986-2002 /
Fine JD / 2008 / Cause-specific risks of childhood death in inherited epidermolysis bullosa / J Pediatr 152 : 276 ~ 280
Fewtrell MS / 2006 / Bone mineralization in children with epidermolysis bullosa / Br J Dermatol 154 : 959 ~ 962
Fine JD / 1986 / Epidermolysis bullosa. Clinical aspects, pathology, and recent advances in research / Int J Dermatol 25 : 143 ~ 157
Fine JD / 2005 / Pseudosyndactyly and musculoskeletal contractures in inherited epidermolysis bullosa: experience of the National Epidermolysis Bullosa Registry, 1986-2002 /
Fine JD / 2008 / Cause-specific risks of childhood death in inherited epidermolysis bullosa / J Pediatr 152 : 276 ~ 280
이 논문의 저자 정보
최근 본 자료
댓글(0)
0