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本症又称多发性肠道息肉综合征,至目前国内仅见2例报告。其特点为外胚层异常伴吸收不良综合征和蛋白渗出性肠病。临床表现为秃发、指(趾)甲萎缩、皮肤色素沉着和胃-空肠-回肠-结肠-直肠多发性息肉。病程后期可出现严重腹泻和低蛋白血症.本病发病机理尚不清楚。我院于1988年10月诊治1例,现报告如下. 女患,58岁。始于20年前头发逐渐脱落,指、趾甲萎缩,近三年来感脐周围隐痛,常解稀水样便.经当地卫生院按“肠炎”给予抗生素治疗后症状好转。近20天腹痛明显,每天稀水样便6～10次。于1988年10月17日住院治疗。查体:T正常,P 98次/分,Bp 10.4/6.7kPa.发育正常,营养稍差,精神萎糜,全身皮肤褐色素沉着。头发稀疏,心肺无
This disease, also known as multiple intestinal polyposis syndrome, up to now only 2 cases of domestic reports. It is characterized by ectodermal abnormalities with malabsorption syndrome and protein exudative enteropathy. Clinical manifestations are alopecia, nail atrophy, pigmentation and stomach - jejunum - ileum - colon - rectal polyps. Severe diarrhea and hypoproteinemia may occur late in the course of the disease pathogenesis is unclear. Our hospital in October 1988 diagnosis and treatment of a case, the report is as follows. Female patient, 58 years old. Beginning 20 years ago, the hair gradually shedding, fingernail, toenail atrophy, the past three years, feeling pain around the umbilical cord, often solution of watery stools by the local hospitals according to “enteritis” antibiotics after treatment to improve the symptoms. Obvious pain in the past 20 days, every day watery urine will be 6 to 10 times. In October 17, 1988 hospitalization. Physical examination: T normal, P 98 beats / min, Bp 10.4 / 6.7kPa. Normal development, slightly malnutrition, spiritual wilt, brown skin pigmentation. Thinning hair, no heart and lungs