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Titolo:
Outcome after surgical repair of junctional epidermolysis bullosa-pyloric atresia syndrome - A report of 3 cases and review of the literature
Autore:
Dank, JP; Kim, S; Parisi, MA; Brown, T; Smith, LT; Waldhausen, J; Sybert, VP;
Indirizzi:
Univ Washington, Sch Med,Med Ctr, Dept Med, Div Dermatol, Seattle, WA 98195 USA Univ Washington Seattle WA USA 98195 Div Dermatol, Seattle, WA 98195 USA Univ Washington, Sch Med,Med Ctr, Dept Med, Div Med Genet, Seattle, WA 98195 USA Univ Washington Seattle WA USA 98195 Div Med Genet, Seattle, WA 98195 USA Fred Hutchinson Canc Res Ctr, Div Basic Sci, Seattle, WA 98104 USA Fred Hutchinson Canc Res Ctr Seattle WA USA 98104 , Seattle, WA 98104 USA Childrens Hosp & Reg Med Ctr, Dept Surg, Seattle, WA USA Childrens Hosp & Reg Med Ctr Seattle WA USA , Dept Surg, Seattle, WA USA
Titolo Testata:
ARCHIVES OF DERMATOLOGY
fascicolo: 10, volume: 135, anno: 1999,
pagine: 1243 - 1247
SICI:
0003-987X(199910)135:10<1243:OASROJ>2.0.ZU;2-#
Fonte:
ISI
Lingua:
ENG
Soggetto:
GASTRIC OUTLET OBSTRUCTION; PRENATAL-DIAGNOSIS; ITGB4 MUTATIONS; INTEGRIN; LETALIS; SIBLINGS; PATIENT; LETHAL; ALPHA-6-BETA-4; DEFICIENCY;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
55
Recensione:
Indirizzi per estratti:
Indirizzo: Sybert, VP Univ Washington, Sch Med,Med Ctr, Dept Med, Div Dermatol, Box 356524, Seattle, WA 98195 USA Univ Washington Box 356524 Seattle WA USA 98195 e, WA 98195 USA
Citazione:
J.P. Dank et al., "Outcome after surgical repair of junctional epidermolysis bullosa-pyloric atresia syndrome - A report of 3 cases and review of the literature", ARCH DERMAT, 135(10), 1999, pp. 1243-1247

Abstract

Background: Junctional epidermolysis bullosapyloric atresia syndrome is recognized as a distinct autosomal recessive entity. Affected infants presentwith skin fragility and inability to feed due to intestinal obstruction. Despite successful surgical repair of the anatomical defect, the outcome is poor owing to poor feeding, malabsorption, failure to thrive, and sepsis. Observations: In 70 cases of intestinal obstruction and epidermolysis bullosa reported in the medical literature and the 3 reported here, surgical intervention was attempted 51 times. In all except 16 infants, death occurredbefore age 11 months (mean age, 70 days). Renal involvement and continued failure to thrive accompanied the skin disease in survivors, who ranged in age from 30 days to 16 years (mean age, 4.0 years). Conclusions: The poor prognosis of this condition must be considered when decisions are made regarding surgical correction. Attempting surgical correction may be warranted in individual circumstances, but withholding surgical intervention and providing palliative support is an acceptable alternative.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 27/11/20 alle ore 01:46:59