作者对15例(男5例,女10例,年龄1～8岁)临床诊断、皮损活检、免疫组化及电镜证实为营养不良性大疱性表皮松解症(EB)的儿童进行了营养状况的评定,并评价了营养性食谱的优点。参加研究的儿童中11例有口腔溃疡,7例分别有咽下困难及龋齿,9例便秘,12例行钡餐检查,4例有吞咽困难的儿童发现异常,其中3例在胸食道或颈胸交界处有5处狭窄,但实际只1例有梗阻。2例有裂孔疝伴胃食道返流及食道蠕动功能减 The authors performed clinical diagnosis, skin biopsy, immunohistochemistry, and electron microscopy in 15 children (5 males, 10 females and 1 to 8 years of age) with malnutritional epidermolysis bullosa (EB) Assessment of nutritional status, and evaluated the benefits of nutritional recipes. Eleven children with oral ulcer, seven with dysphagia and caries respectively, nine with constipation, 12 with barium meal, and four with dysphagia were found to have abnormalities, of which three were found in the thorax or neck At the junction of 5 narrow, but in fact only 1 case of obstruction. There were 2 cases of hiatal hernia with gastroesophageal reflux and esophageal motility reduction