Ahmadabadi Farzad, Nemati Hamid, Abdolmohammadzadeh Amirmohammad, Ahadi Adel
Department of Pediatrics,Faculty of Medicine,Ardebil University of Medical Sciences, Ardebil, Iran.
Shiraz Neuroscience Research Center,Shiraz University of Medical Sciences, Shiraz, Iran.
Iran J Child Neurol. 2020 Fall;14(4):17-28.
Autism spectrum disorder (ASD) is a category of neurodevelopmental disorders characterized by social and communication impairment and restricted or repetitive behaviors. The pathogenesis of ASD is not well understood and it's proved that genetic is strongly associated with ASD in 5 to 25% of cases. Inborn errors of metabolism(IEMs), defined by a vast array of disorders that are caused by specific enzyme deficiencies or transport protein defects, is as frequent as in 1 in 800 births. IEMs can manifest several psychiatric or behavioral manifestations such as self-injuriesincreased activity and aggression, personality changes, paranoia, depression, catatonia, and psychosis. IEMs underlie autistic symptoms in less than 5% of cases. The literature on the association between ASD and respiratory chain abnormalities is growing, including complex III/IV deficiency and MELAS (mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes) syndrome, as well as glucose-6-phosphate dehydrogenase deficiency. Google Scholar, Pubmed, and SCOPUS databases were searched using a combination of the following keywords: "autism spectrum disorder", "autism spectrum", "autistic feature" and "inborn error of metabolism", " IEM", "congenital error of metabolism". Initially, 655 articles were found and our expert and methodologist altogether selected 187 articles based on the titles, relevance, and text language. After reading full texts, 37 studies were selected for review. We think it's best to consider IEMs in children with syndromic ASD and/or if there is a strong familial history of autism or parental consanguineous marriage.
自闭症谱系障碍(ASD)是一类神经发育障碍,其特征为社交和沟通障碍以及受限或重复行为。ASD的发病机制尚未完全明确,已证实5%至25%的病例中,遗传因素与ASD密切相关。先天性代谢缺陷(IEMs)由一系列因特定酶缺乏或转运蛋白缺陷引起的疾病所定义,其发病率高达每800例出生中就有1例。IEMs可表现出多种精神或行为症状,如自我伤害、活动增加和攻击行为、人格改变、妄想、抑郁、紧张症和精神病。在不到5%的病例中,IEMs是自闭症症状的潜在原因。关于ASD与呼吸链异常之间关联的文献不断增加,包括复合物III/IV缺乏和MELAS(线粒体脑病、乳酸酸中毒和卒中样发作)综合征,以及葡萄糖-6-磷酸脱氢酶缺乏。使用以下关键词组合在谷歌学术、PubMed和SCOPUS数据库中进行搜索:“自闭症谱系障碍”、“自闭症谱系”、“自闭症特征”以及“先天性代谢缺陷”、“IEM”、“先天性代谢错误”。最初,共找到655篇文章,我们的专家和方法学家根据文章标题、相关性和文本语言总共筛选出187篇文章。在阅读全文后,选取了37项研究进行综述。我们认为,对于患有综合征性ASD的儿童和/或如果有自闭症家族病史或父母近亲结婚的情况,最好考虑先天性代谢缺陷。