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血色病又称细胞色素沉着症,是一种先天代谢缺陷的疾病,临床上罕见,发病多在中年。我科曾收治一例。男孩,13岁,因上腹包块,进行性增大,并全身皮肤逐渐变黑3年,于1991年3月入本院。查体:慢性病容,全身皮肤青灰色,以颜面、颈部明显,腹中度膨隆,可见腹壁静脉怒张;肝大于剑下7cm、右锁骨中线肋下3cm,质韧、边钝、表面结节状、轻压痛;脾肋下2cm,质软、边钝、表面光滑、无压痛,腹水征阴性。实验室检查:血清总蛋白52.8g/L、白蛋白31.5g/L、球蛋白21.3g/L,肝功能正常,血清胎甲球蛋白阴
Hemochromatosis, also known as cytochromes, is a disease of inherent metabolic defects, clinically rare, the incidence in middle age. My department had admitted a case. The boy, aged 13, had progressive enlargement due to the mass in the abdomen and a gradual blackening of the whole body skin for 3 years. She was admitted to our hospital in March 1991. Physical examination: chronic disease, systemic skin bluish gray, with the face, the neck was obvious, the abdomen of the degree of bulging, visible abdominal wall vein engorgement; liver greater than the sword 7cm, right subclavian midline rib 3cm, quality tough, blunt edge, surface knot Arthralgia, mild tenderness; spleen ribs 2cm, soft, blunt, smooth surface, no tenderness, ascites sign negative. Laboratory tests: serum total protein 52.8g / L, albumin 31.5g / L, globulin 21.3g / L, normal liver function, serum fetal nail globulin