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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

Faundes V, Goh S, Akilapa R, Bezuidenhout H, Bjornsson HT, Bradley L, Brady AF, Brischoux-Boucher E, Brunner H, Bulk S, Canham N, Cody D, Dentici ML, Digilio MC, Elmslie F, Fry AE, Gill H, Hurst J, Johnson D, Julia S, Lachlan K, Lebel RR, Byler M, Gershon E, Lemire E, Gnazzo M, Lepri FR, Marchese A, McEntagart M, McGaughran J, Mizuno S, Okamoto N, Rieubland C, Rodgers J, Sasaki E, Scalais E, Scurr I, Suri M, van der Burgt I, Matsumoto N, Miyake N, Benoit V, Lederer D, Banka S
Genet Med 2021 Jul;23(7):1202-1210. Epub 2021 Mar 5 doi: 10.1038/s41436-021-01119-8. PMID: 33674768Free PMC Article
Cheng H, Du C, Zhang Y, James AF, Dempsey CE, Abdala AP, Hancox JC
J Mol Cell Cardiol 2019 Oct;135:22-30. Epub 2019 Jul 27 doi: 10.1016/j.yjmcc.2019.07.012. PMID: 31362019Free PMC Article
Fauth C, Toutain A
Prenat Diagn 2017 Oct;37(10):1055-1056. doi: 10.1002/pd.5137. PMID: 29057530

Recent clinical studies

Etiology

Shchubelka K, Turova L, Wolfsberger W, Kalanquin K, Williston K, Kurutsa O, Makovetska A, Hasynets Y, Mirutenko V, Vakerych M, Oleksyk TK
J Neurodev Disord 2024 Mar 27;16(1):13. doi: 10.1186/s11689-024-09528-x. PMID: 38539105Free PMC Article
Zhong L, Gao X, Wang Y, Qiu W, Han L, Gu X, Zhang H
Clin Genet 2023 Jun;103(6):655-662. Epub 2023 Mar 22 doi: 10.1111/cge.14329. PMID: 36945845
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
Salazar R, Beenders S, LaMarca NM, Thornburg O, Rubin-Thompson L, Snow A, Goldman S, Chung WK, Bain JM
Res Dev Disabil 2021 Dec;119:104110. Epub 2021 Nov 16 doi: 10.1016/j.ridd.2021.104110. PMID: 34794115
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

Diagnosis

Shchubelka K, Turova L, Wolfsberger W, Kalanquin K, Williston K, Kurutsa O, Makovetska A, Hasynets Y, Mirutenko V, Vakerych M, Oleksyk TK
J Neurodev Disord 2024 Mar 27;16(1):13. doi: 10.1186/s11689-024-09528-x. PMID: 38539105Free PMC Article
Zhong L, Gao X, Wang Y, Qiu W, Han L, Gu X, Zhang H
Clin Genet 2023 Jun;103(6):655-662. Epub 2023 Mar 22 doi: 10.1111/cge.14329. PMID: 36945845
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
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

Datta AN, Bahi-Buisson N, Bienvenu T, Buerki SE, Gardiner F, Cross JH, Heron B, Kaminska A, Korff CM, Lepine A, Lesca G, McTague A, Mefford HC, Mignot C, Milh M, Piton A, Pressler RM, Ruf S, Sadleir LG, de Saint Martin A, Van Gassen K, Verbeek NE, Ville D, Villeneuve N, Zacher P, Scheffer IE, Lemke JR
Epilepsia 2021 Feb;62(2):325-334. Epub 2021 Jan 7 doi: 10.1111/epi.16761. PMID: 33410528Free PMC Article
Saunier C, Støve SI, Popp B, Gérard B, Blenski M, AhMew N, de Bie C, Goldenberg P, Isidor B, Keren B, Leheup B, Lampert L, Mignot C, Tezcan K, Mancini GM, Nava C, Wasserstein M, Bruel AL, Thevenon J, Masurel A, Duffourd Y, Kuentz P, Huet F, Rivière JB, van Slegtenhorst M, Faivre L, Piton A, Reis A, Arnesen T, Thauvin-Robinet C, Zweier C
Hum Mutat 2016 Aug;37(8):755-64. Epub 2016 May 4 doi: 10.1002/humu.23001. PMID: 27094817Free 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
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

Prognosis

Shchubelka K, Turova L, Wolfsberger W, Kalanquin K, Williston K, Kurutsa O, Makovetska A, Hasynets Y, Mirutenko V, Vakerych M, Oleksyk TK
J Neurodev Disord 2024 Mar 27;16(1):13. doi: 10.1186/s11689-024-09528-x. PMID: 38539105Free PMC Article
Akahoshi K, Nakagawa E, Goto YI, Inoue K
BMC Med Genomics 2023 Mar 6;16(1):43. doi: 10.1186/s12920-023-01465-3. PMID: 36879246Free PMC Article
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
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

Shchubelka K, Turova L, Wolfsberger W, Kalanquin K, Williston K, Kurutsa O, Makovetska A, Hasynets Y, Mirutenko V, Vakerych M, Oleksyk TK
J Neurodev Disord 2024 Mar 27;16(1):13. doi: 10.1186/s11689-024-09528-x. PMID: 38539105Free PMC Article
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
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
Verot L, Alloisio N, Morlé L, Bozon M, Touraine R, Plauchu H, Edery P
Am J Med Genet A 2003 Sep 15;122A(1):37-41. doi: 10.1002/ajmg.a.20221. PMID: 12949969

Recent systematic reviews

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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