[1]李冰,王晓敏,张丽萍,等.极长链酰基辅酶A脱氢酶缺乏症1例报告并文献复习[J].国际内分泌代谢杂志,2021,41(04):376-380.[doi:10.3760/cma.j.cn121383-20201215-12044]
 Li Bing,Wang Xiaomin,Zhang Liping,et al.One case report of very long-chain acyl-CoA dehydrogenase deficiency and literature review[J].International Journal of Endocrinology and Metabolism,2021,41(04):376-380.[doi:10.3760/cma.j.cn121383-20201215-12044]
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极长链酰基辅酶A脱氢酶缺乏症1例报告并文献复习()
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《国际内分泌代谢杂志》[ISSN:1673-4157/CN:12-1383/R]

卷:
41
期数:
2021年04期
页码:
376-380
栏目:
病例报告
出版日期:
2021-07-20

文章信息/Info

Title:
One case report of very long-chain acyl-CoA dehydrogenase deficiency and literature review
作者:
李冰1王晓敏1张丽萍1舒剑波23
1天津市儿童医院(天津大学儿童医院)重症医学科 300134; 2天津市儿科研究所 300134; 3天津市儿童出生缺陷防治重点实验室 300134
Author(s):
Li Bing1 Wang Xiaomin1 Zhang Liping1 Shu Jianbo23.
1Department of Intensive Care Unit, Tianjin Children's Hospital(Tianjin University Children's Hospital), Tianjin 300134, China; 2Tianjin Pediatric Research Institute, Tianjin 300134, China; 3Tianjin Key Laboratory of Birth Defects for Prevention and Treatment,Tianjin 300134, China
DOI:
10.3760/cma.j.cn121383-20201215-12044

参考文献/References:

[1] Strauss AW, Powell CK,Hale DE,et al.Molecular basis of human mitochondrial very-long-chain acyl-CoA dehydrogenase deficiency causing cardiomyopathy and sudden death in childhood[J].Proc Natl Acad Sci U S A,1995,92(23):10496-10500.DOI:10.1073/pnas.92.23.10496.
[2] Eminoglu TF,Tumer L,Okur I,et al.Very long-chain acyl CoA dehydrogenase deficiency which was accepted as infanticide[J].Forensic Sci Int,2011,210(1-3):el-3,DOI:10.1016/j.forsciint.2011.04.003.
[3] Marsden D,Bedrosian CL,Vockley J.Impact of newborn screening on the reported incidence and clinical outcomes associated with medium and long-chain fatty acid oxidation disorders[J].Genet Med,2021,23(5):816-829.DOI:10.1038/s41436-020-01070-0.
[4] 张超,胡真真,杨建滨,等,Region 4 Stork系统在串联质谱新生儿极长链酰基辅酶A脱氢酶缺乏症筛查中的应用[J].中华检验医学杂志,2020,43(10):978-983.DOI:10.3760/cma.j.cn114452-20200224-00116.
[5] Yamada K,Taketani T.Management and diagnosis of mitochondrial fatty acid oxidation disorders:focus on very-long-chain acyl-CoA dehydrogenase deficiency[J].J Hum Genet,2019,64(2):73-85.DOI:10.1038/s10038-018-0527-7.
[6] Fuseya Y,Sakurai T,Miyahara JI,et al.Adult-onset repeat rhabdomyolysis with a very long-chain Acyl-CoA dehydrogenase deficiency due to compound heterozygous ACADVL mutations[J].Intern Med,2020,59(21):2729-2732.DOI:10.2169/internalmedicine.4604-20.
[7] Burrage LC,Miller MJ,Wong LJ,et al.Elevations of C14:1 and C14:2 plasma acylcarnitines in fasted children:a diagnostic dilemma[J].J Pediatr,2016,169:208-213.e2.DOI:10.1016/j.jpeds.2015.10.045.
[8] Fukao T,Watannabe H,Orii K,et al.Myopathic form of very-long chain acyl-CoA dehydrogenase deficiency:evidence for temperature-sensitive mild mutations in both mutant alleles in a Hapanese girl[J].Pediatr Res,2001,49(2):227-231.DOI:10.1203/00006450-200102000-00016.
[9] Kang E,Kim YM,Kang M,et al.Clinical and genetic characteristics of patients with fatty acid oxidation disorders identified by newborn screening[J].BMC Pediatr,2018,18(1):103.DOI:10.1186/s12887-018-1069-z.
[10] Hesse J,Braun C,Behringer S,et al.The diagnostic challenge in very-long chain acyl-CoA dehydrogenase deficiency(VLCADD)[J].J Inherit Metab Dis,2018,41(6):1169-1178.DOI:10.1007/s10545-018-0245-5.
[11] Merinero B,Alcaide P,Martín-Hernández E,et al.Four years' experience in the diagnosis of very long-chain acyl-CoA dehydrogenase deficiency in infants detected in three Spanish newborn screening centers[J].JIMD Rep,2018,39:63-74.DOI:10.1007/8904_2017_40.
[12] Andresen BS,Bross P,Vianey-Saban C,et al.Cloning and characterization of human verylong-chain acyl-CoA dehydrogenase cDNA,chromosomal assignment of the gene and identification in four patients of nine different mutations within the VLCAD gene[J].Hum Mol Genet,1996,5(4):461-476.DOI:10.1093/hmg/5.4.461.
[13] 曹金俊,邱文娟,章瑞南,等.极长链酰基辅酶A脱氢酶缺乏症11例的临床和ACADVL基因突变谱分析[J].中华儿科杂志,2015,53(4):262-267.DOI:10.3760/cma.j.issn.0578-1310.2015.04.007.
[14] Li X,Ma R,Liu Y,et al.One potential hotspot ACADVL mutation in Chinese patients with very-long-chain acyl-coenzyme A dehydrogenase deficiency[J].Clin Chim Acta,2020,503:218-222.DOI:10.1016/j.cca.2019.11.034.
[15] 童凡,陈挺,蒋萍萍,等.极长链酰基辅酶A脱氢酶缺乏症新生儿的ACADVL基因变异分析[J].中华医学遗传杂志,2019,36(4):310-313.DOI:10.3760/cma.j.issn.1003-9406.2019.04.005.
[16] Pena LD,van Calcar SC,Hansen J,et al.Outcomes and genotype-phenotype correlations in 52 individuals with VLCAD deficiency diagnosed by NBS and enrolled in the IBEM-IS database[J].Mol Genet Metab,2016,118(4):272-281.DOI:10.1016/j.ymgme.2016.05.007.
[17] Shiraishi H,Yamada K,Egawa K,et al.Efficacy of bezafibrate for preventing myopathic attacks in patients with very long-chain acyl-CoA dehydrogenase deficiency[J].Brain Dev,2021,43(2):214-219.DOI:10.1016/j.braindev.2020.07.019.
[18] Watanabe K,Yamada K,Sameshima K,et al.Two siblings with very long-chain acyl-CoA dehydrogenase(VLCAD)deficiency suffered from rhabdomyolysis after l-carnitine supplementation[J].Mol Genet Metab Rep,2018,15:121-123.DOI:10,1016/j.ymgmr.2018,03,007.
[19] Janeiro P,Jotta R,Ramos R,et al.Follow-up of fatty acid β-oxidation disorders in expanded newborn screening era[J].Eur J Pediatr,2019,178(3):387-394.DOI:10.1007/s00431-018-03315-2.

备注/Memo

备注/Memo:
通信作者:李冰,Email:duaiyong@sina.cn
更新日期/Last Update: 1900-01-01