尼曼-匹克病(Niemann-Pick disease,NPD)系累及全身的代谢性疾病。国内少见,近十年有综合报道。肺部改变是该病的重要临床表现,具有诊断价值。我们报道1例随访12年的病例,并对其肺部病变作简要讨论。 临床资料 余××,女,13岁,学生。出生后第4个月因腹部膨隆、腹泻、废肤紫癜、鼻衄就诊。体检发现肝脾肿大,未明确诊断。2岁时复诊,检查其智力、发育正常,肝肋下4cm,脾肋下5cm,胸片两肺 Niemann-Pick disease (NPD) is a systemic metabolic disorder. Domestic rare, nearly a decade of comprehensive coverage. Lung changes is an important clinical manifestation of the disease, with diagnostic value. We report a case of 12 years of follow-up, and a brief discussion of its lung lesions. Clinical data Yu XX, female, 13 years old, student. The first 4 months after birth due to bulging belly, diarrhea, skin purpura, epistaxis treatment. Physical examination found that hepatosplenomegaly, no clear diagnosis. 2 years old referral, check their intelligence, normal development, liver ribs 4cm, 5cm under the spleen ribs, chest and lungs