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Intellectual developmental disorder, x-linked, syndromic 37

MedGen UID:
1052758
Concept ID:
CN377268
Disease or Syndrome
Synonym: intellectual developmental disorder, x-linked, syndromic 37
 
Gene (location): ZFX (Xp22.11)
 
Monarch Initiative: MONDO:0958322
OMIM®: 301118

Definition

X-linked syndromic intellectual developmental disorder-37 (MRXS37) is a developmental disorder showing phenotypic variability and variable severity. Male mutation carriers tend to be more severely affected than female mutation carriers, some of whom may even be asymptomatic. In general, the disorder is characterized by global developmental delay with delayed walking, speech delay, impaired intellectual development that ranges from borderline low to moderate, and behavioral abnormalities, such as autism and sleeping difficulties. Many patients are able to attend mainstream schools with assistance and work under supervision. Additional more variable features include sensorineural hearing loss, ocular anomalies, feeding difficulties, dysmorphic facial features, inguinal and umbilical hernia, genitourinary defects, congenital heart defects, musculoskeletal anomalies, and endocrine dysfunction, such as hypogonadism or hyperparathyroidism (Shepherdson et al., 2024). [from OMIM]

Professional guidelines

PubMed

Refeat MM, El Saied MM, Abdel Raouf ER
Ir J Med Sci 2023 Oct;192(5):2265-2272. Epub 2022 Nov 21 doi: 10.1007/s11845-022-03166-9. PMID: 36409419Free PMC Article
Berry-Kravis E
J Child Neurol 2022 Oct;37(10-11):797-812. Epub 2022 Jul 5 doi: 10.1177/08830738221089740. PMID: 35791522
Haham LM, Avrahami I, Domniz N, Ries-Levavi L, Berkenstadt M, Orvieto R, Cohen Y, Elizur SE
J Assist Reprod Genet 2018 Nov;35(11):2071-2075. Epub 2018 Aug 22 doi: 10.1007/s10815-018-1293-3. PMID: 30136016Free PMC Article

Recent clinical studies

Etiology

Famula J, Ferrer E, Hagerman RJ, Tassone F, Schneider A, Rivera SM, Hessl D
J Neurodev Disord 2022 Mar 23;14(1):23. doi: 10.1186/s11689-022-09436-y. PMID: 35321639Free PMC Article
Mierlo PV, Braakman H, Vandenbussche N, Schelhaas HJ, Pillen S
Neuropediatrics 2022 Apr;53(2):109-114. Epub 2021 Dec 21 doi: 10.1055/s-0041-1740551. PMID: 34933379
Will EA, Roberts JE
Am J Intellect Dev Disabil 2021 Nov 1;126(6):460-476. doi: 10.1352/1944-7558-126.6.460. PMID: 34700347Free PMC Article
Glaze DG, Neul JL, Percy A, Feyma T, Beisang A, Yaroshinsky A, Stoms G, Zuchero D, Horrigan J, Glass L, Jones NE
Pediatr Neurol 2017 Nov;76:37-46. Epub 2017 Jul 8 doi: 10.1016/j.pediatrneurol.2017.07.002. PMID: 28964591
Leonard H, Cobb S, Downs J
Nat Rev Neurol 2017 Jan;13(1):37-51. Epub 2016 Dec 9 doi: 10.1038/nrneurol.2016.186. PMID: 27934853

Diagnosis

West AE
Genes Dev 2023 Oct 1;37(19-20):863-864. Epub 2023 Nov 1 doi: 10.1101/gad.351285.123. PMID: 37914350Free PMC Article
Mierlo PV, Braakman H, Vandenbussche N, Schelhaas HJ, Pillen S
Neuropediatrics 2022 Apr;53(2):109-114. Epub 2021 Dec 21 doi: 10.1055/s-0041-1740551. PMID: 34933379
Hatch HAM, O'Neil MH, Marion RW, Secombe J, Shulman LH
Am J Med Genet A 2021 Oct;185(10):2951-2958. Epub 2021 Jun 4 doi: 10.1002/ajmg.a.62381. PMID: 34089235Free PMC Article
Leonard H, Cobb S, Downs J
Nat Rev Neurol 2017 Jan;13(1):37-51. Epub 2016 Dec 9 doi: 10.1038/nrneurol.2016.186. PMID: 27934853
Low Kapalu CM, Gartstein MA
J Intellect Disabil Res 2016 Sep;60(9):891-900. Epub 2016 Jun 19 doi: 10.1111/jir.12304. PMID: 27321588

Therapy

Neul JL, Percy AK, Benke TA, Berry-Kravis EM, Glaze DG, Marsh ED, Lin T, Stankovic S, Bishop KM, Youakim JM
Nat Med 2023 Jun;29(6):1468-1475. Epub 2023 Jun 8 doi: 10.1038/s41591-023-02398-1. PMID: 37291210Free PMC Article
Berry-Kravis E
J Child Neurol 2022 Oct;37(10-11):797-812. Epub 2022 Jul 5 doi: 10.1177/08830738221089740. PMID: 35791522
Glaze DG, Neul JL, Percy A, Feyma T, Beisang A, Yaroshinsky A, Stoms G, Zuchero D, Horrigan J, Glass L, Jones NE
Pediatr Neurol 2017 Nov;76:37-46. Epub 2017 Jul 8 doi: 10.1016/j.pediatrneurol.2017.07.002. PMID: 28964591
Reddy DS, Estes WA
Trends Pharmacol Sci 2016 Jul;37(7):543-561. Epub 2016 May 5 doi: 10.1016/j.tips.2016.04.003. PMID: 27156439Free PMC Article
da Silva EM, Strufaldi MW, Andriolo RB, Silva LA
Cochrane Database Syst Rev 2016 Feb 5;2(2):CD008185. doi: 10.1002/14651858.CD008185.pub4. PMID: 26845288Free PMC Article

Prognosis

Crivellari I, Pecorelli A, Cordone V, Marchi S, Pinton P, Hayek J, Cervellati C, Valacchi G
Arch Biochem Biophys 2021 Mar 30;700:108790. Epub 2021 Feb 4 doi: 10.1016/j.abb.2021.108790. PMID: 33549528
Saby JN, Benke TA, Peters SU, Standridge SM, Matsuzaki J, Cutri-French C, Swanson LC, Lieberman DN, Key AP, Percy AK, Neul JL, Nelson CA, Roberts TPL, Marsh ED
Ann Neurol 2021 Apr;89(4):790-802. Epub 2021 Feb 4 doi: 10.1002/ana.26029. PMID: 33480039Free PMC Article
López-Sainz Á, Salazar-Mendiguchía J, García-Álvarez A, Campuzano Larrea O, López-Garrido MÁ, García-Guereta L, Fuentes Cañamero ME, Climent Payá V, Peña-Peña ML, Zorio-Grima E, Jordá-Burgos P, Díez-López C, Brugada R, García-Pinilla JM, García-Pavía P
Rev Esp Cardiol (Engl Ed) 2019 Jun;72(6):479-486. Epub 2018 Aug 11 doi: 10.1016/j.rec.2018.04.035. PMID: 30108015
da Silva EM, Strufaldi MW, Andriolo RB, Silva LA
Cochrane Database Syst Rev 2016 Feb 5;2(2):CD008185. doi: 10.1002/14651858.CD008185.pub4. PMID: 26845288Free PMC Article
da Silva EM, Strufaldi MW, Andriolo RB, Silva LA
Cochrane Database Syst Rev 2014 Jan 8;(1):CD008185. doi: 10.1002/14651858.CD008185.pub3. PMID: 24399699

Clinical prediction guides

Neul JL, Percy AK, Benke TA, Berry-Kravis EM, Glaze DG, Marsh ED, Lin T, Stankovic S, Bishop KM, Youakim JM
Nat Med 2023 Jun;29(6):1468-1475. Epub 2023 Jun 8 doi: 10.1038/s41591-023-02398-1. PMID: 37291210Free PMC Article
Will EA, Roberts JE
Am J Intellect Dev Disabil 2021 Nov 1;126(6):460-476. doi: 10.1352/1944-7558-126.6.460. PMID: 34700347Free PMC Article
Glaze DG, Neul JL, Percy A, Feyma T, Beisang A, Yaroshinsky A, Stoms G, Zuchero D, Horrigan J, Glass L, Jones NE
Pediatr Neurol 2017 Nov;76:37-46. Epub 2017 Jul 8 doi: 10.1016/j.pediatrneurol.2017.07.002. PMID: 28964591
Leonard H, Cobb S, Downs J
Nat Rev Neurol 2017 Jan;13(1):37-51. Epub 2016 Dec 9 doi: 10.1038/nrneurol.2016.186. PMID: 27934853
da Silva EM, Strufaldi MW, Andriolo RB, Silva LA
Cochrane Database Syst Rev 2016 Feb 5;2(2):CD008185. doi: 10.1002/14651858.CD008185.pub4. PMID: 26845288Free PMC Article

Recent systematic reviews

Deng H, Xiong BT, Wu Y, Wang W
Neurosurg Rev 2023 Jan 25;46(1):40. doi: 10.1007/s10143-023-01950-4. PMID: 36694014
da Silva EM, Strufaldi MW, Andriolo RB, Silva LA
Cochrane Database Syst Rev 2016 Feb 5;2(2):CD008185. doi: 10.1002/14651858.CD008185.pub4. PMID: 26845288Free PMC Article
da Silva EM, Strufaldi MW, Andriolo RB, Silva LA
Cochrane Database Syst Rev 2014 Jan 8;(1):CD008185. doi: 10.1002/14651858.CD008185.pub3. PMID: 24399699
da Silva EM, Strufaldi MW, Andriolo RB, Silva LA
Cochrane Database Syst Rev 2011 Nov 9;(11):CD008185. doi: 10.1002/14651858.CD008185.pub2. PMID: 22071845

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