1临床资料患儿,男,5岁,因进行性腹胀、纳差、消瘦、乏力入院。腹部超声提示:脾肿大。查体:慢性病容,贫血貌、精神差、体温36.8℃,脉搏100次/分,血压13.8/9.6Kpa,腹部膨隆,肝肋下4.0cm 脾下极达盆腔。血常规:血红蛋白51g/L,血小板48×10~9/L,白细胞3.1 × 10~9/L,白蛋白28g/L,球蛋白31g/L。骨穿诊断 Gauchen(高歇病)病。经术前充分准备后行脾切除术,切除脾脏重三千克,术后恢复良好,十三日后出院。 1 clinical data Children, male, 5 years old, due to progressive abdominal distension, anorexia, weight loss, fatigue, admission. Abdominal ultrasound tip: splenomegaly. Physical examination: chronic disease, anemia appearance, poor spirit, body temperature 36.8 ℃, pulse 100 beats / min, blood pressure 13.8 / 9.6Kpa, abdominal distension, 4.0cm under the spleen ribs pole reach the pelvis. Blood: hemoglobin 51g / L, platelet 48 × 10 ~ 9 / L, white blood cells 3.1 × 10 ~ 9 / L, albumin 28g / L, globulin 31g / L. Bone wear diagnosis of Gauchen disease. After preoperative preparation for splenectomy, resection of the spleen weight three kilograms, postoperative recovery was good, discharged after 13 days.