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作者报道1例6岁男孩,鼻炎、咳嗽和发热一周,伴左额肿胀。查体有严重发育障碍。左额有一3cm×3cm囊性肿胀,左手第四指及左足背肿胀。颈淋巴结肿大。双肺有捻发音和于性啰音。肝轻度肿大,脾尖可以触及。白细胞计数10.6×10~9/L,Hb5.2g/dl,MCV 65fl,除LDH为458 u/L(正常<350u/L)外,尿、电解质和肝酶均正常。Mantoux皮肤试验(一)。胸片示右下肺炎。骨骼X线片见多发溶骨性病变,伴有轻度硬化边缘,见于颅穹窿骨、肱骨、股骨、胫骨、桡骨、尺骨、掌骨、蹠骨和指(趾)
The authors report a 6-year-old boy with rhinitis, cough and fever for one week with left forehead swelling. Physical examination have serious developmental disorders. Left front has a 3cm × 3cm cystic swelling, left fourth and left foot dorsal swelling. Neck lymph nodes. Pulmonary twisting and sexual rales. Liver mild swelling, spleen tip can be touched. The white blood cell count was 10.6 × 10-9 / L, Hb5.2g / dl, MCV 65fl, urine, electrolytes and liver enzymes were all normal except LDH of 458u / L (normal <350u / L). Mantoux skin test (a). Chest radiograph shows right lower pneumonia. Skeletal radiographs see multiple osteolytic lesions, with mild sclerosis, found in cranial vault, humerus, femur, tibia, radius, ulna, metacarpal, metatarsal and phalanges