Gardner氏症候群2例报告

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1950~1954年,Gardner分析了一个51名人口的家族,有6人同时患有大肠多发性腺癌、骨瘤和多发性软组织肿瘤三组病变,并指出主要是遗传因素的缺陷所致。该病已被命名为Gardner氏症候群。本文报告2例。例1 朱××,男性,28岁,玻璃厂工人。因便频带血,肛门有肿块脱出4年余入院。4年来排便习惯改变、便频,一日2~3次,伴有粘液。每于疲劳或排便时肛门口有肿块脱出并有便血,量不等,便纸上有时擦到肉样小块。近半年来发作频繁。患者自幼有多处肿瘤。2年前曾切除面部、左臂及左下肢表皮样囊肿4个。父母、三弟、一妹及二女均健在,无便血、便频史。体检:前额、左臂、左下肢有手术疤痕4处,愈合良好,无纤维组织增生或疤痕疙痞。左额、右肩、左腰骶部及右下肢见大小肿瘤多个,和皮肤不粘连,与深组织固定,质软,边缘清晰。直肠指检及镜检:距肛门5厘米处以上 From 1950 to 1954, Gardner analyzed a family of 51 people. Six people also had three groups of colorectal multiple adenocarcinomas, osteomas, and multiple soft tissue tumors, and pointed out that they were mainly due to defects in genetic factors. The disease has been named Gardner's syndrome. This article reports 2 cases. Example 1 Zhu X, male, 28 years old, glass factory worker. Because of the banded blood, the anus had a lump that had been removed from the hospital for more than four years. For 4 years, the habit of bowel movement changed, it was frequent, 2 or 3 times a day, accompanied by mucus. Every time when fatigue or defecation occurs, there is a mass out of the anus and there is blood in the stool, the amount is not equal, it sometimes rubs to pieces of meat. Attacks have been frequent in the past six months. The patient had multiple tumors since childhood. Two years ago, four epidermoid cysts were removed from the face, left arm, and left lower limb. Parents, three younger brothers, one sister and two daughters are all alive. Physical examination: There were 4 surgical scars on the forehead, left arm, and left lower limb, which healed well without fibrosis or keloids. The left frontal, right shoulder, left lumbosacral and right lower limbs have multiple tumors of large and small size. They do not adhere to the skin and are fixed with deep tissues. They are soft and clear. Rectal examination and microscopic examination: 5 cm above the anus
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