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马某,男,6个月。因腹大五月于1989年元月入院。足月顺产,第二胎,生后一月开始腹大,进行性肿大伴面色苍白,大便稀,次数频,胃纳好。无发烧、惊厥、咳嗽和出血倾向。小便正常。生后以母乳喂养,现仍不会坐,不会抓玩具,父母体健,系近亲结婚,其姐因肝脾大于11/2岁死亡。体查:神清,贫血貌,表情呆板,运动及发育迟缓,智商87。头围44cm,身长66cm,体重8.5kg,皮肤未见色素沉着,各组表浅淋巴结无肿大,咽充血,肋骨串珠(+),两肺呈肺泡型呼吸音粗糙。心脏无异常。腹膨隆,腹壁静脉曲张明显,无脐周静脉曲张,肝大肋下10cm,质硬,表面光滑。脾肋下8cm,质硬。腹水征(-),右侧有腹股沟斜疝。四肢肌张力尚正常。腱反射不亢进。眼底见黄斑区有约视乳头之1/3大小的樱桃红斑。实验室检查:Hb 8.5g/dl,RBC270万/μl,WBC 12700/μl,N 36％,L 64％,单核及淋巴细胞胞浆内有空泡。血小板80000/μl,肝功:TTT9~μ,ZnTT8~μ,GPT 40~μ(赖氏法),HBsAg(-),空腹血糖
Ma Mou, male, 6 months. Due to bulky May admission in January 1989. Full-term natural delivery, the second child, born after January belly, progressive enlargement with pale complexion, thin stools, the frequency of frequency, good appetite. No fever, convulsions, cough and bleeding tendency. Normal urine. Breastfeeding after birth, is still not sitting, will not grasp the toys, physical health parents, close relatives get married, the sister died of liver and spleen larger than 11/2 years old. Physical examination: Shen Qing, anemia appearance, dull expression, exercise and stunting, IQ 87. Head circumference 44cm, body length 66cm, weight 8.5kg, the skin no pigmentation, no superficial lymph nodes in each group, pharyngeal congestion, ribs beaded (+), both lungs were alveolar type of breathing sound rough. No abnormal heart. Abdominal bulge, abdominal varicose veins obvious, no umbilical varicose veins, liver ribs 10cm, hard, smooth surface. Spleen ribs 8cm, hard. Ascites sign (-), the right side of the inguinal hernia. Limb muscle tension is still normal. Tendon reflex is not hyperthyroidism. See the macular area of about 1/3 of the size of the cherry papules. Laboratory tests: Hb 8.5g / dl, RBC 270 million / l, WBC 12700 / l, N 36%, L 64%, mononuclear and lymphocytes cytoplasm vacuoles. Platelet 80000 / μl, liver function: TTT9 ~ μ, ZnTT8 ~ μ, GPT 40 ~ μ (Lai’s method), HBsAg (-), fasting blood glucose