Abstract: The clinical manifestations of inherited metabolic diseases are nonspecific，which depends on the early diagnosis by genetic or biochemical methods. Diet therapy plays a significant role. Timely clinical treatment and appropriate individualized nutritional intervention are of great significance for the disease outcome and prognosis of children with IEM.However，there are still many problems in the long-term nutritional management of IEM patients.

Key words: inherited metabolic diseases, nutrition manage-ment, multidisciplinary cooperation, hospitalized child

摘要： 遗传代谢病临床表现复杂，缺乏特异性，容易漏诊或误诊，有赖于遗传学或生化学等方法尽早确诊。饮食疗法是遗传代谢病的主要治疗手段之一，及时的临床诊断、治疗、适当的个体化营养干预及包括营养支持小组的多学科合作，对于改善遗传代谢病患儿的疾病转归及预后有重要意义。遗传代谢病患儿的长期营养管理过程中仍存在许多问题亟待解决。

关键词: 遗传代谢病, 营养管理, 多学科合作, 住院患儿