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Infantile glycine encephalopathy

MedGen UID:
1781124
Concept ID:
C5548209
Disease or Syndrome
Synonyms: glycine encephalopathy of infancy; infantile glycine encephalopathy; Infantile NKH; infantile NKH; Infantile non-ketotic hyperglycinemia; infantile non-ketotic hyperglycinemia; infantile onset glycine encephalopathy
SNOMED CT: Infantile glycine encephalopathy (1156826003); Infantile non-ketotic hyperglycinemia (1156826003)
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
 
Monarch Initiative: MONDO:0017354
Orphanet: ORPHA289860

Definition

Infantile glycine encephalopathy is a mild to severe form of glycine encephalopathy (GE; see this term), characterized by early hypotonia, developmental delay and seizures. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVInfantile glycine encephalopathy

Professional guidelines

PubMed

Zou W, Li M, Wang X, Lu H, Hao Y, Chen D, Zhu S, Ji D, Zhang Z, Zhou P, Cao Y
J Assist Reprod Genet 2024 May;41(5):1245-1259. Epub 2024 Mar 12 doi: 10.1007/s10815-024-03057-1. PMID: 38470552Free PMC Article
Mercimek-Mahmutoglu S, Patel J, Cordeiro D, Hewson S, Callen D, Donner EJ, Hahn CD, Kannu P, Kobayashi J, Minassian BA, Moharir M, Siriwardena K, Weiss SK, Weksberg R, Snead OC 3rd
Epilepsia 2015 May;56(5):707-16. Epub 2015 Mar 25 doi: 10.1111/epi.12954. PMID: 25818041
Wang PJ, Lee WT, Hwu WL, Young C, Yau KI, Shen YZ
Brain Dev 1998 Oct;20(7):530-5. doi: 10.1016/s0387-7604(98)00042-4. PMID: 9840674

Recent clinical studies

Etiology

Zou W, Li M, Wang X, Lu H, Hao Y, Chen D, Zhu S, Ji D, Zhang Z, Zhou P, Cao Y
J Assist Reprod Genet 2024 May;41(5):1245-1259. Epub 2024 Mar 12 doi: 10.1007/s10815-024-03057-1. PMID: 38470552Free PMC Article
Leyne E, Anselem O, Jordan P, Vivanti AJ, Benachi A, Salomon L, Jacquier M, Jouannic JM, Dhombres F, Cambier T, Rosenblatt J, Pannier E, Goffinet F, Tsatsaris V, Athiel Y
Acta Obstet Gynecol Scand 2024 Jan;103(1):51-58. Epub 2023 Nov 9 doi: 10.1111/aogs.14716. PMID: 37942915Free PMC Article
Kuseyri Hübschmann O, Juliá-Palacios NA, Olivella M, Guder P, Zafeiriou DI, Horvath G, Kulhánek J, Pearson TS, Kuster A, Cortès-Saladelafont E, Ibáñez S, García-Jiménez MC, Honzík T, Santer R, Jeltsch K, Garbade SF, Hoffmann GF, Opladen T, García-Cazorla Á
Ann Neurol 2022 Aug;92(2):292-303. Epub 2022 Jun 16 doi: 10.1002/ana.26423. PMID: 35616651
Bayrak H, Yıldız Y, Olgaç A, Kasapkara ÇS, Küçükcongar A, Zenciroğlu A, Yüksel D, Ceylaner S, Kılıç M
Metab Brain Dis 2021 Aug;36(6):1213-1222. Epub 2021 Apr 1 doi: 10.1007/s11011-021-00718-3. PMID: 33791923
Mercimek-Mahmutoglu S, Patel J, Cordeiro D, Hewson S, Callen D, Donner EJ, Hahn CD, Kannu P, Kobayashi J, Minassian BA, Moharir M, Siriwardena K, Weiss SK, Weksberg R, Snead OC 3rd
Epilepsia 2015 May;56(5):707-16. Epub 2015 Mar 25 doi: 10.1111/epi.12954. PMID: 25818041

Diagnosis

Leyne E, Anselem O, Jordan P, Vivanti AJ, Benachi A, Salomon L, Jacquier M, Jouannic JM, Dhombres F, Cambier T, Rosenblatt J, Pannier E, Goffinet F, Tsatsaris V, Athiel Y
Acta Obstet Gynecol Scand 2024 Jan;103(1):51-58. Epub 2023 Nov 9 doi: 10.1111/aogs.14716. PMID: 37942915Free PMC Article
Kuseyri Hübschmann O, Juliá-Palacios NA, Olivella M, Guder P, Zafeiriou DI, Horvath G, Kulhánek J, Pearson TS, Kuster A, Cortès-Saladelafont E, Ibáñez S, García-Jiménez MC, Honzík T, Santer R, Jeltsch K, Garbade SF, Hoffmann GF, Opladen T, García-Cazorla Á
Ann Neurol 2022 Aug;92(2):292-303. Epub 2022 Jun 16 doi: 10.1002/ana.26423. PMID: 35616651
Bayrak H, Yıldız Y, Olgaç A, Kasapkara ÇS, Küçükcongar A, Zenciroğlu A, Yüksel D, Ceylaner S, Kılıç M
Metab Brain Dis 2021 Aug;36(6):1213-1222. Epub 2021 Apr 1 doi: 10.1007/s11011-021-00718-3. PMID: 33791923
Nasrallah F, Hadj-Taieb S, Chehida AB, Jelassi A, Ben Massoued S, Charfi M, Zidi W, Amri F, Helel KB, Mejaoual H, Seboui H, Mahdhaoui N, Gargouri A, Monastiri K, Turki I, Cheour M, Sanhaji H, Tebib N, Feki M, Kaabachi N
Neuropediatrics 2020 Oct;51(5):349-353. Epub 2020 Aug 20 doi: 10.1055/s-0040-1712489. PMID: 32818969
Dinopoulos A, Matsubara Y, Kure S
Mol Genet Metab 2005 Sep-Oct;86(1-2):61-9. doi: 10.1016/j.ymgme.2005.07.016. PMID: 16157495

Therapy

Mullier B, Wolff C, Sands ZA, Ghisdal P, Muglia P, Kaminski RM, André VM
Neuropharmacology 2017 Sep 1;123:322-331. Epub 2017 May 19 doi: 10.1016/j.neuropharm.2017.05.017. PMID: 28533163
Devinsky O, Cilio MR, Cross H, Fernandez-Ruiz J, French J, Hill C, Katz R, Di Marzo V, Jutras-Aswad D, Notcutt WG, Martinez-Orgado J, Robson PJ, Rohrback BG, Thiele E, Whalley B, Friedman D
Epilepsia 2014 Jun;55(6):791-802. Epub 2014 May 22 doi: 10.1111/epi.12631. PMID: 24854329Free PMC Article
Matsumoto S, Hidaka S, Goto K, Hagiwara S, Shingu C, Iwasaka H, Noguchi T
J Anesth 2009;23(2):288-91. Epub 2009 May 15 doi: 10.1007/s00540-008-0735-y. PMID: 19444574
Chen PT, Young C, Lee WT, Wang PJ, Peng SS, Shen YZ
Brain Dev 2001 Nov;23(7):715-20. doi: 10.1016/s0387-7604(01)00285-6. PMID: 11701284
Melançon SB, Dallaire L, Vincelette P, Potier M, Geoffroy G
Prog Clin Biol Res 1979;34:217-29. PMID: 531053

Prognosis

Kuseyri Hübschmann O, Juliá-Palacios NA, Olivella M, Guder P, Zafeiriou DI, Horvath G, Kulhánek J, Pearson TS, Kuster A, Cortès-Saladelafont E, Ibáñez S, García-Jiménez MC, Honzík T, Santer R, Jeltsch K, Garbade SF, Hoffmann GF, Opladen T, García-Cazorla Á
Ann Neurol 2022 Aug;92(2):292-303. Epub 2022 Jun 16 doi: 10.1002/ana.26423. PMID: 35616651
Bayrak H, Yıldız Y, Olgaç A, Kasapkara ÇS, Küçükcongar A, Zenciroğlu A, Yüksel D, Ceylaner S, Kılıç M
Metab Brain Dis 2021 Aug;36(6):1213-1222. Epub 2021 Apr 1 doi: 10.1007/s11011-021-00718-3. PMID: 33791923
Nasrallah F, Hadj-Taieb S, Chehida AB, Jelassi A, Ben Massoued S, Charfi M, Zidi W, Amri F, Helel KB, Mejaoual H, Seboui H, Mahdhaoui N, Gargouri A, Monastiri K, Turki I, Cheour M, Sanhaji H, Tebib N, Feki M, Kaabachi N
Neuropediatrics 2020 Oct;51(5):349-353. Epub 2020 Aug 20 doi: 10.1055/s-0040-1712489. PMID: 32818969
Dinopoulos A, Matsubara Y, Kure S
Mol Genet Metab 2005 Sep-Oct;86(1-2):61-9. doi: 10.1016/j.ymgme.2005.07.016. PMID: 16157495
Schiffmann R, Kaye EM, Willis JK 3rd, Africk D, Ampola M
Ann Neurol 1989 Feb;25(2):201-3. doi: 10.1002/ana.410250218. PMID: 2919871

Clinical prediction guides

Leyne E, Anselem O, Jordan P, Vivanti AJ, Benachi A, Salomon L, Jacquier M, Jouannic JM, Dhombres F, Cambier T, Rosenblatt J, Pannier E, Goffinet F, Tsatsaris V, Athiel Y
Acta Obstet Gynecol Scand 2024 Jan;103(1):51-58. Epub 2023 Nov 9 doi: 10.1111/aogs.14716. PMID: 37942915Free PMC Article
Kuseyri Hübschmann O, Juliá-Palacios NA, Olivella M, Guder P, Zafeiriou DI, Horvath G, Kulhánek J, Pearson TS, Kuster A, Cortès-Saladelafont E, Ibáñez S, García-Jiménez MC, Honzík T, Santer R, Jeltsch K, Garbade SF, Hoffmann GF, Opladen T, García-Cazorla Á
Ann Neurol 2022 Aug;92(2):292-303. Epub 2022 Jun 16 doi: 10.1002/ana.26423. PMID: 35616651
Khraim W, Abu-Libdeh B, Ayesh S, Dweikat I
Brain Dev 2017 Aug;39(7):601-605. Epub 2017 Mar 18 doi: 10.1016/j.braindev.2017.03.005. PMID: 28325525
Navarro-Sastre A, Tort F, Stehling O, Uzarska MA, Arranz JA, Del Toro M, Labayru MT, Landa J, Font A, Garcia-Villoria J, Merinero B, Ugarte M, Gutierrez-Solana LG, Campistol J, Garcia-Cazorla A, Vaquerizo J, Riudor E, Briones P, Elpeleg O, Ribes A, Lill R
Am J Hum Genet 2011 Nov 11;89(5):656-67. doi: 10.1016/j.ajhg.2011.10.005. PMID: 22077971Free PMC Article
Dinopoulos A, Matsubara Y, Kure S
Mol Genet Metab 2005 Sep-Oct;86(1-2):61-9. doi: 10.1016/j.ymgme.2005.07.016. PMID: 16157495

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