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Budd-Chiari氏综合征又称肝静脉阻塞综合征。系指多种病因致肝静脉狭窄或阻塞而引起的肝脏血液循环障碍的一组综合征。是成年人的少见病,儿童患者目前国内外罕见报道,现报告一例如下: 患儿男性,12岁,因间断呕血、便血伴脾肿大3年。于1993年5月10日入院。患儿5岁后经常感冒,腹部钝痛,食欲欠佳,日渐消瘦。近3年曾无何明显诱因呕血、便血3次,中等量。曾在当地医院就诊,诊断不清,经保肝、止血、输血等治疗,效果不佳。病后无发烧、咯血、骨痛等。否认家族及遗传病史。体格检查:体温:37.5℃,血压:16/11KPA,发育正常,略消瘦,中度贫血貌,精神萎靡、粘膜未见明显黄染。全身未见皮疹及出血点,未见蜘蛛痣,未见颈静脉怒张及颈动脉搏动。肝颈回流征阴性。心率112次/分,无震颤,心音无低钝及遥远,胸骨左缘3～4肋间闻及Ⅱ～Ⅲ级收缩期杂音,双肺呼吸音清晰。腹膨隆,未见腹壁静脉怒张,肝脏触不清,肝浊音界不清,脾中度大,1线9厘米,2线17厘米,3线8厘米,质硬。移动性浊音及搏动感阳性。双下肢未见明显浮肿,未见静脉曲张。实验室检查:血常规:白细胞2.8×10~9/L,红细胞2.5×10~(12)/L,血红蛋白54g/L,中性粒细胞0.62,淋巴细胞0.38、血小板:9.6×10~9/L。肝功:GOT<40~U/L,总蛋白64g/L,白蛋白37g/L球蛋白27g/L,白/球比值正常?
Budd-Chiari’s syndrome, also known as hepatic vein occlusion syndrome. Refers to a variety of causes caused by hepatic vein stenosis or obstruction caused by a group of liver blood circulation disorders syndrome. Is a rare disease in adults, children and patients rarely reported at home and abroad, the report is as follows: A male patient, aged 12, due to intermittent hematemesis, blood in the stool with splenomegaly 3 years. On May 10, 1993 admitted. Children after 5 years old often cold, abdominal dull pain, poor appetite, weight loss. Nearly 3 years had no obvious incentive vomiting blood, blood in the stool 3 times, moderate. Had a visit to a local hospital, diagnosis is not clear, the liver, hemostasis, blood transfusions and other treatment, the effect is not good. No fever after the illness, hemoptysis, bone pain and so on. Denied family and genetic history. Physical examination: body temperature: 37.5 ℃, blood pressure: 16 / 11KPA, normal development, a slight loss of weight, moderate anemia appearance, apathetic, mucosal no obvious yellow dye. No body rash and bleeding, no spider nevus, no jugular vein engorgement and carotid artery pulsation. Hepatic neck reflux sign negative. Heart rate 112 beats / min, no tremor, heart sound without low blunt and distant, sternal border 3 ~ 4 intercostal smell and Ⅱ ~ Ⅲ systolic murmur, lung breath sounds clear. Abdominal bulging, no abdominal wall dilated, liver palpable, unclear liver voiced, splenomegaly large, 1 line 9 cm, 2 lines 17 cm, 3 lines 8 cm, hard. Mobility dullness and positive sense of beat. No obvious lower extremity edema, no varicose veins. Laboratory tests: blood: 2.8 × 10 ~ 9 / L white blood cells, erythrocytes 2.5 × 10-12 / L, hemoglobin 54g / L, neutrophils 0.62, lymphocytes 0.38, platelets: 9.6 × 10 ~ 9 / L. Liver function: GOT <40 ~ U / L, total protein 64g / L, albumin 37g / L globulin 27g / L, white / ball ratio normal?