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Wendt于1902年首先认识到一组以皮肤损害、顽固性腹泻、部分或完全性秃发以及生长发育障碍为特征的临床综合征,曾称为非典型性连续性肢端皮炎、变异型大皰性表皮松解症。1942年Daubott根据本病的皮损好发于四肢末端以及与腹泻有密切关系,而定名为肠病性肢端皮炎(Acrodermatitis enteropathica,以下简称AE)。AE常发病于断奶期间的婴儿,病程迁延,往往以死亡而告终。自从1953年Dillaha使用双碘羟基奎宁(DIQ)治疗以来,本病的预后为之改观,许多患儿能较正常地生活到成年,但其病因、病理生理和DIQ为何能缓解症状等问题仍然无满意的解释。过去曾将白色念珠菌感染、肠道寄生虫感染或菌群失调、肠道毒素的
Wendt first recognized in 1902 a group of clinical syndromes characterized by skin lesions, refractory diarrhea, partial or complete alopecia, and growth and developmental disorders, known as atypical continuous acral dermatitis, variant bullae Epidermolysis disease. In 1942 Daubott based on the disease lesions occur in the extremities of the extremities and is closely related to diarrhea, and named acrodermatitis enteropathic (Acrodermatitis enteropathica, hereinafter referred to as AE). AE often affects babies during weaning, with prolonged course and often ending in death. Since Dillaha was treated with DIQ in 1953, the prognosis has been improved and many children live more normally than adults, but the etiology, pathophysiology, and the reasons why DIQ can relieve the symptoms remain No satisfactory explanation. In the past have Candida albicans infection, intestinal parasitic infection or flora, intestinal toxins