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Intellectual disability, X-linked 46(XLID46)

MedGen UID:
337255
Concept ID:
C1845526
Mental or Behavioral Dysfunction
Synonym: XLID46
 
Monarch Initiative: MONDO:0010326
OMIM®: 300436

Definition

Any non-syndromic X-linked intellectual disability in which the cause of the disease is a mutation in the ARHGEF6 gene. [from MONDO]

Clinical features

From HPO
Sensorineural hearing loss disorder
MedGen UID:
9164
Concept ID:
C0018784
Disease or Syndrome
A type of hearing impairment in one or both ears related to an abnormal functionality of the cochlear nerve.
Intellectual disability
MedGen UID:
811461
Concept ID:
C3714756
Mental or Behavioral Dysfunction
Intellectual disability, previously referred to as mental retardation, is characterized by subnormal intellectual functioning that occurs during the developmental period. It is defined by an IQ score below 70.

Professional guidelines

PubMed

Pierpont EI, Isaia AR, McCoy E, Brown SJ, Gupta AO, Eisengart JB
J Inherit Metab Dis 2023 Mar;46(2):174-193. Epub 2022 Dec 26 doi: 10.1002/jimd.12581. PMID: 36527290Free PMC Article
Albersen M, van der Beek SL, Dijkstra IME, Alders M, Barendsen RW, Bliek J, Boelen A, Ebberink MS, Ferdinandusse S, Goorden SMI, Heijboer AC, Jansen M, Jaspers YRJ, Metgod I, Salomons GS, Vaz FM, Verschoof-Puite RK, Visser WF, Dekkers E, Engelen M, Kemp S
J Inherit Metab Dis 2023 Jan;46(1):116-128. Epub 2022 Oct 26 doi: 10.1002/jimd.12571. PMID: 36256460Free PMC Article
Moser AB, Moser HW
Prenat Diagn 1999 Jan;19(1):46-8. doi: 10.1002/(sici)1097-0223(199901)19:1<46::aid-pd501>3.0.co;2-e. PMID: 10073906

Recent clinical studies

Etiology

De Feyter S, Beyens A, Callewaert B
J Inherit Metab Dis 2023 Mar;46(2):163-173. Epub 2023 Feb 3 doi: 10.1002/jimd.12590. PMID: 36692329
Albersen M, van der Beek SL, Dijkstra IME, Alders M, Barendsen RW, Bliek J, Boelen A, Ebberink MS, Ferdinandusse S, Goorden SMI, Heijboer AC, Jansen M, Jaspers YRJ, Metgod I, Salomons GS, Vaz FM, Verschoof-Puite RK, Visser WF, Dekkers E, Engelen M, Kemp S
J Inherit Metab Dis 2023 Jan;46(1):116-128. Epub 2022 Oct 26 doi: 10.1002/jimd.12571. PMID: 36256460Free PMC Article
Iskandar K, Triono A, Sunartini, Dwianingsih EK, Indraswari BW, Kirana IR, Ivana G, Sutomo R, Patria SY, Herini ES, Gunadi
PLoS One 2022;17(10):e0276640. Epub 2022 Oct 31 doi: 10.1371/journal.pone.0276640. PMID: 36315559Free PMC Article
van Geest FS, Groeneweg S, van den Akker ELT, Bacos I, Barca D, van den Berg SAA, Bertini E, Brunner D, Brunetti-Pierri N, Cappa M, Cappuccio G, Chatterjee K, Chesover AD, Christian P, Coutant R, Craiu D, Crock P, Dewey C, Dica A, Dimitri P, Dubey R, Enderli A, Fairchild J, Gallichan J, Garibaldi LR, George B, Hackenberg A, Heinrich B, Huynh T, Kłosowska A, Lawson-Yuen A, Linder-Lucht M, Lyons G, Monti Lora F, Moran C, Müller KE, Paone L, Paul PG, Polak M, Porta F, Reinauer C, de Rijke YB, Seckold R, Menevşe TS, Simm P, Simon A, Spada M, Stoupa A, Szeifert L, Tonduti D, van Toor H, Turan S, Vanderniet J, de Waart M, van der Wal R, van der Walt A, van Wermeskerken AM, Wierzba J, Zibordi F, Zung A, Peeters RP, Visser WE
J Clin Endocrinol Metab 2022 Feb 17;107(3):e1136-e1147. doi: 10.1210/clinem/dgab750. PMID: 34679181Free PMC Article
Liberato AP, Mallack EJ, Aziz-Bose R, Hayden D, Lauer A, Caruso PA, Musolino PL, Eichler FS
Neurology 2019 Apr 9;92(15):e1698-e1708. Epub 2019 Mar 22 doi: 10.1212/WNL.0000000000007294. PMID: 30902905Free PMC Article

Diagnosis

De Feyter S, Beyens A, Callewaert B
J Inherit Metab Dis 2023 Mar;46(2):163-173. Epub 2023 Feb 3 doi: 10.1002/jimd.12590. PMID: 36692329
Albersen M, van der Beek SL, Dijkstra IME, Alders M, Barendsen RW, Bliek J, Boelen A, Ebberink MS, Ferdinandusse S, Goorden SMI, Heijboer AC, Jansen M, Jaspers YRJ, Metgod I, Salomons GS, Vaz FM, Verschoof-Puite RK, Visser WF, Dekkers E, Engelen M, Kemp S
J Inherit Metab Dis 2023 Jan;46(1):116-128. Epub 2022 Oct 26 doi: 10.1002/jimd.12571. PMID: 36256460Free PMC Article
Liberato AP, Mallack EJ, Aziz-Bose R, Hayden D, Lauer A, Caruso PA, Musolino PL, Eichler FS
Neurology 2019 Apr 9;92(15):e1698-e1708. Epub 2019 Mar 22 doi: 10.1212/WNL.0000000000007294. PMID: 30902905Free PMC Article
Reuter MS, Tawamie H, Buchert R, Hosny Gebril O, Froukh T, Thiel C, Uebe S, Ekici AB, Krumbiegel M, Zweier C, Hoyer J, Eberlein K, Bauer J, Scheller U, Strom TM, Hoffjan S, Abdelraouf ER, Meguid NA, Abboud A, Al Khateeb MA, Fakher M, Hamdan S, Ismael A, Muhammad S, Abdallah E, Sticht H, Wieczorek D, Reis A, Abou Jamra R
JAMA Psychiatry 2017 Mar 1;74(3):293-299. doi: 10.1001/jamapsychiatry.2016.3798. PMID: 28097321
Moser AB, Moser HW
Prenat Diagn 1999 Jan;19(1):46-8. doi: 10.1002/(sici)1097-0223(199901)19:1<46::aid-pd501>3.0.co;2-e. PMID: 10073906

Therapy

van Geest FS, Groeneweg S, van den Akker ELT, Bacos I, Barca D, van den Berg SAA, Bertini E, Brunner D, Brunetti-Pierri N, Cappa M, Cappuccio G, Chatterjee K, Chesover AD, Christian P, Coutant R, Craiu D, Crock P, Dewey C, Dica A, Dimitri P, Dubey R, Enderli A, Fairchild J, Gallichan J, Garibaldi LR, George B, Hackenberg A, Heinrich B, Huynh T, Kłosowska A, Lawson-Yuen A, Linder-Lucht M, Lyons G, Monti Lora F, Moran C, Müller KE, Paone L, Paul PG, Polak M, Porta F, Reinauer C, de Rijke YB, Seckold R, Menevşe TS, Simm P, Simon A, Spada M, Stoupa A, Szeifert L, Tonduti D, van Toor H, Turan S, Vanderniet J, de Waart M, van der Wal R, van der Walt A, van Wermeskerken AM, Wierzba J, Zibordi F, Zung A, Peeters RP, Visser WE
J Clin Endocrinol Metab 2022 Feb 17;107(3):e1136-e1147. doi: 10.1210/clinem/dgab750. PMID: 34679181Free PMC Article
Semyachkina AN, Voskoboeva EY, Zakharova EY, Nikolaeva EA, Kanivets IV, Kolotii AD, Baydakova GV, Kharabadze MN, Kuramagomedova RG, Melnikova NV
BMC Med Genet 2019 May 2;20(1):66. doi: 10.1186/s12881-019-0807-x. PMID: 31046699Free PMC Article
Bauer M, Krüger R, Kölsch U, Unterwalder N, Meisel C, Wahn V, von Bernuth H
Pediatr Infect Dis J 2018 May;37(5):466-468. doi: 10.1097/INF.0000000000001799. PMID: 28938254
Kaler SG, Liew CJ, Donsante A, Hicks JD, Sato S, Greenfield JC
J Inherit Metab Dis 2010 Oct;33(5):583-9. Epub 2010 Jul 21 doi: 10.1007/s10545-010-9118-2. PMID: 20652413Free PMC Article
Watkins PA, Naidu S, Moser HW
J Inherit Metab Dis 1987;10 Suppl 1:46-53. doi: 10.1007/BF01812846. PMID: 3119941

Prognosis

Jiang W, Jin W, Zhao H, Huang D, Wu L
Acta Neurol Belg 2023 Dec;123(6):2259-2268. Epub 2023 May 29 doi: 10.1007/s13760-023-02295-x. PMID: 37247117
De Feyter S, Beyens A, Callewaert B
J Inherit Metab Dis 2023 Mar;46(2):163-173. Epub 2023 Feb 3 doi: 10.1002/jimd.12590. PMID: 36692329
Pierpont EI, Isaia AR, McCoy E, Brown SJ, Gupta AO, Eisengart JB
J Inherit Metab Dis 2023 Mar;46(2):174-193. Epub 2022 Dec 26 doi: 10.1002/jimd.12581. PMID: 36527290Free PMC Article
van Geest FS, Groeneweg S, van den Akker ELT, Bacos I, Barca D, van den Berg SAA, Bertini E, Brunner D, Brunetti-Pierri N, Cappa M, Cappuccio G, Chatterjee K, Chesover AD, Christian P, Coutant R, Craiu D, Crock P, Dewey C, Dica A, Dimitri P, Dubey R, Enderli A, Fairchild J, Gallichan J, Garibaldi LR, George B, Hackenberg A, Heinrich B, Huynh T, Kłosowska A, Lawson-Yuen A, Linder-Lucht M, Lyons G, Monti Lora F, Moran C, Müller KE, Paone L, Paul PG, Polak M, Porta F, Reinauer C, de Rijke YB, Seckold R, Menevşe TS, Simm P, Simon A, Spada M, Stoupa A, Szeifert L, Tonduti D, van Toor H, Turan S, Vanderniet J, de Waart M, van der Wal R, van der Walt A, van Wermeskerken AM, Wierzba J, Zibordi F, Zung A, Peeters RP, Visser WE
J Clin Endocrinol Metab 2022 Feb 17;107(3):e1136-e1147. doi: 10.1210/clinem/dgab750. PMID: 34679181Free PMC Article
Cenacchi G, Papa V, Pegoraro V, Marozzo R, Fanin M, Angelini C
Neuropathol Appl Neurobiol 2020 Jun;46(4):303-322. Epub 2019 Nov 25 doi: 10.1111/nan.12587. PMID: 31698507

Clinical prediction guides

Xu F, Huang H, Shen Q, Bao Y, Zhang D, Liu L, Xu Y
Neurol Sci 2025 Jan;46(1):437-444. Epub 2024 Jul 6 doi: 10.1007/s10072-024-07676-5. PMID: 38969962
De Feyter S, Beyens A, Callewaert B
J Inherit Metab Dis 2023 Mar;46(2):163-173. Epub 2023 Feb 3 doi: 10.1002/jimd.12590. PMID: 36692329
Pierpont EI, Isaia AR, McCoy E, Brown SJ, Gupta AO, Eisengart JB
J Inherit Metab Dis 2023 Mar;46(2):174-193. Epub 2022 Dec 26 doi: 10.1002/jimd.12581. PMID: 36527290Free PMC Article
Iskandar K, Triono A, Sunartini, Dwianingsih EK, Indraswari BW, Kirana IR, Ivana G, Sutomo R, Patria SY, Herini ES, Gunadi
PLoS One 2022;17(10):e0276640. Epub 2022 Oct 31 doi: 10.1371/journal.pone.0276640. PMID: 36315559Free PMC Article
Liberato AP, Mallack EJ, Aziz-Bose R, Hayden D, Lauer A, Caruso PA, Musolino PL, Eichler FS
Neurology 2019 Apr 9;92(15):e1698-e1708. Epub 2019 Mar 22 doi: 10.1212/WNL.0000000000007294. PMID: 30902905Free PMC Article

Recent systematic reviews

De Feyter S, Beyens A, Callewaert B
J Inherit Metab Dis 2023 Mar;46(2):163-173. Epub 2023 Feb 3 doi: 10.1002/jimd.12590. PMID: 36692329

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