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黄脂瘤病亦称为Hand-Schueller-christi-ans病,为组织细胞增生症的一种少见类型。其特征为膜性骨缺损,尤其是颅骨,伴突眼及尿崩症。我院收治一例,通过手术切除,病理证实。现报告如下: 患儿男性,3岁。1980年10月13日入院。入院前1个半月开始多饮多尿,每日饮水1,500cc-2,000cc每夜尿量为1,500cc。精神尚好。无头痛、呕吐双下肢乏力,但步履正常。不烧,食欲正常。查体:发育正常,无肥胖。双眼视力正常,眼球不突出。左侧头顶部可触及颅骨缺损,范围约3×3cm~2大小。其余颅神经检查未见异常。化验:17-羟类固醇 6.6mg/24h 17-酮类固醇 3.85mg/24h X线平片侧位相:颅骨内外板结构正常。顶骨可见3.5×2cm~2大小骨质破坏区,边缘不光滑,周围无骨质硬化。蝶鞍大小形态正常。正位相:颅骨破坏区位于左侧顶骨,因与X线
Yellow lipoma, also known as Hand-Schueller-christi-ans disease, is a rare type of histiocytosis. It is characterized by membranous bone defects, especially the skull, with exophthalmos and diabetes insipidus. A hospital in our hospital, by surgical excision, pathology confirmed. The report is as follows: Children, 3 years old. October 13, 1980 admission. More than one and a half months before admission to drink polyuria, daily drinking water 1,500 cc-2,000cc night urine output of 1,500 cc. The spirit is good. No headache, vomiting, lower extremity fatigue, but normal walking. Not burning, normal appetite. Physical examination: normal development, no obesity. Eyesight normal, the eye is not prominent. Left head can reach the top of the skull defect, the size of about 3 × 3cm ~ 2 size. The remaining cranial nerve examination showed no abnormalities. Assay: 17-hydroxysteroid 6.6mg / 24h 17-ketosteroid 3.85mg / 24h X-ray film phase: internal and external skull structure is normal. The top bone visible 3.5 × 2cm ~ 2 size bone destruction area, the edge is not smooth, surrounded by bone sclerosis. Sella size normal shape. Orthographic phase: skull destruction area is located in the left parietal bone, because with the X-ray