TY - JOUR
T1 - Gastroesophageal dysfunction in Brachmann-de Lange syndrome
AU - Rosenbach, Y.
AU - Zahavi, I.
AU - Dinari, G.
PY - 1992
Y1 - 1992
N2 - Two children with the Brachmann-de Lange syndrome and severe gastroesophageal reflux are described. Both had esophagitis, recurrent severe anemia, and one had recurrent episodes of aspiration pneumonia and clubbing. Medical treatment failed in both children. One child responded dramatically to surgery, but the other died before surgery could be attempted. Our experience and a review of the literature suggest that early recognition and surgical treatment of gastroesophageal reflux will reduce morbidity and mortality in children with this syndrome.
AB - Two children with the Brachmann-de Lange syndrome and severe gastroesophageal reflux are described. Both had esophagitis, recurrent severe anemia, and one had recurrent episodes of aspiration pneumonia and clubbing. Medical treatment failed in both children. One child responded dramatically to surgery, but the other died before surgery could be attempted. Our experience and a review of the literature suggest that early recognition and surgical treatment of gastroesophageal reflux will reduce morbidity and mortality in children with this syndrome.
KW - Brachmann-de Lange syndrome
KW - gastroesophageal reflux
KW - treatment
UR - http://www.scopus.com/inward/record.url?scp=0026517714&partnerID=8YFLogxK
U2 - 10.1002/ajmg.1320420327
DO - 10.1002/ajmg.1320420327
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AN - SCOPUS:0026517714
SN - 0148-7299
VL - 42
SP - 379
EP - 380
JO - American Journal of Medical Genetics
JF - American Journal of Medical Genetics
IS - 3
ER -