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本病自五十年代初国内就有报道,今将我院收治的经病理检查确诊的二例介绍如下。例1:女,29岁,住院号16273,1078年6月21日入院。患者自幼发现尿色深黄。1972年2月因尿路感染去医院检查时发现巩膜黄染,无发热及明显的乏力、纳差、上腹部胀痛等症状,化验黄疸指数21单位,血清絮浊试验及谷丙转氨酶均正常,诊为胆道疾患。此后黄疸一直持续不退,大便黄色,有时色深,无皮肤搔痒。1974年2月有阵发性右上腹痛,无放射,无畏寒发热,当时作口服胆囊造影,示胆囊显影不佳。1976年4月又类似右上腹痛发作,不伴有发热及其他症状,黄疸始终存在。1978年6月以“黄疸待查”入院。家族中其父、姐有类似持续性黄疸存在。查体:体温37.5℃,脉搏80次/分,呼吸20次/分,血压110/70毫米汞柱。皮肤粘膜黄染,无出血点,未见肝掌及蜘蛛痣。面色黝黑,巩膜黄染,浅表淋巴结不肿大。颈软。心肺正常。腹平软,肝肋下二指,剑突下四指,质中,轻压痛,脾未扪及。腹部无移动性浊音肠呜音正常。脊柱四肢活动自如。下肢不肿。
The disease since the early 1950s have been reported in China, this hospital admitted to the pathological examination confirmed the two cases are as follows. Example 1: Female, 29 years old, hospital number 16273, June 21, 1078 admission. Patients found urine dark yellow. February 1972 due to urinary tract infection to the hospital scoliosis sclerosis found no fever and significant fatigue, anorexia, abdominal pain and other symptoms, test the index of 21 units of jaundice, serum flocculation test and alanine aminotransferase were normal , Diagnosed as biliary disorders. Jaundice has been sustained since then, stool yellow, and sometimes color depth, no skin itching. February 1974 with paroxysmal right upper quadrant pain, no radiation, no chills fever, then for oral cholecystography, showed poor development of gallbladder. April 1976 similar to the right upper quadrant pain episodes, not accompanied by fever and other symptoms, jaundice always exists. June 1978 to “jaundice pending” admission. Family father, sister similar to persistent jaundice exists. Physical examination: body temperature 37.5 ℃, pulse 80 beats / min, breathing 20 beats / min, blood pressure 110/70 mm Hg. Skin mucosa yellow dye, no bleeding point, no liver palms and spider nevus. Dark complexion, scleral yellow dye, superficial lymph nodes are not swollen. Neck soft. Cardiopulmonary normal. Abdomen soft, liver ribs under the two fingers, the xiphoid four fingers, quality, light tenderness, the spleen is not palpable. No movement of the abdomen Voiced bowel sounds normal. Spine limbs freely. Lower extremity not swollen.