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Coffin-Siris syndrome 10(CSS10; IDDSDF)

MedGen UID:
1683634
Concept ID:
C4760583
Disease or Syndrome
Synonym: INTELLECTUAL DEVELOPMENTAL DISORDER WITH SPEECH DELAY AND DYSMORPHIC FACIES
 
Gene (location): SOX4 (6p22.3)
 
Monarch Initiative: MONDO:0032791
OMIM®: 618506

Disease characteristics

Excerpted from the GeneReview: Coffin-Siris Syndrome
Coffin-Siris syndrome (CSS) is classically characterized by aplasia or hypoplasia of the distal phalanx or nail of the fifth and additional digits, developmental or cognitive delay of varying degree, distinctive facial features, hypotonia, hirsutism/hypertrichosis, and sparse scalp hair. Congenital anomalies can include malformations of the cardiac, gastrointestinal, genitourinary, and/or central nervous systems. Other findings commonly include feeding difficulties, slow growth, ophthalmologic abnormalities, and hearing impairment. [from GeneReviews]
Authors:
Samantha Schrier Vergano  |  Gijs Santen  |  Dagmar Wieczorek, et. al.   view full author information

Additional description

From OMIM
Intellectual developmental disorder with speech delay and dysmorphic facies (IDDSDF) is characterized by mildly to severely impaired intellectual development, global developmental delay, mild but distinct facial dysmorphism, fifth finger clinodactyly, and small stature. Hypotonia, ventricular septal defect, and spastic quadriparesis may also be present (Zawerton et al., 2019).  http://www.omim.org/entry/618506

Clinical features

From HPO
Clinodactyly
MedGen UID:
1644094
Concept ID:
C4551485
Congenital Abnormality
An angulation of a digit at an interphalangeal joint in the plane of the palm (finger) or sole (toe).
Ventricular septal defect
MedGen UID:
42366
Concept ID:
C0018818
Congenital Abnormality
A hole between the two bottom chambers (ventricles) of the heart. The defect is centered around the most superior aspect of the ventricular septum.
Low-set ears
MedGen UID:
65980
Concept ID:
C0239234
Congenital Abnormality
Upper insertion of the ear to the scalp below an imaginary horizontal line drawn between the inner canthi of the eye and extending posteriorly to the ear.
Posteriorly rotated ears
MedGen UID:
96566
Concept ID:
C0431478
Congenital Abnormality
A type of abnormal location of the ears in which the position of the ears is characterized by posterior rotation (the superior part of the ears is rotated towards the back of the head, and the inferior part of the ears towards the front).
Spastic tetraparesis
MedGen UID:
658719
Concept ID:
C0575059
Disease or Syndrome
Spastic weakness affecting all four limbs.
Intellectual disability
MedGen UID:
811461
Concept ID:
C3714756
Mental or Behavioral Dysfunction
Subnormal intellectual functioning which originates during the developmental period. Intellectual disability, previously referred to as mental retardation, has been defined as an IQ score below 70.
Generalized hypotonia
MedGen UID:
346841
Concept ID:
C1858120
Finding
Generalized muscular hypotonia (abnormally low muscle tone).
Microcephaly
MedGen UID:
1644158
Concept ID:
C4551563
Finding
Head circumference below 2 standard deviations below the mean for age and gender.
Congenital laryngomalacia
MedGen UID:
120500
Concept ID:
C0264303
Anatomical Abnormality
Laryngomalacia is a congenital abnormality of the laryngeal cartilage in which the cartilage is floppy and prolapses over the larynx during inspiration.
Wide mouth
MedGen UID:
44238
Concept ID:
C0024433
Congenital Abnormality
Distance between the oral commissures more than 2 SD above the mean. Alternatively, an apparently increased width of the oral aperture (subjective).
Persistence of primary teeth
MedGen UID:
75597
Concept ID:
C0266050
Disease or Syndrome
Persistence of the primary teeth beyond the age by which they normally are shed and replaced by the permanent teeth.
Epicanthus
MedGen UID:
151862
Concept ID:
C0678230
Congenital Abnormality
Epicanthus is a condition in which a fold of skin stretches from the upper to the lower eyelid, partially covering the inner canthus. Usher (1935) noted that epicanthus is a normal finding in the fetus of all races. Epicanthus also occurs in association with hereditary ptosis (110100).
Anteverted nares
MedGen UID:
326648
Concept ID:
C1840077
Finding
Anteriorly-facing nostrils viewed with the head in the Frankfurt horizontal and the eyes of the observer level with the eyes of the subject. This gives the appearance of an upturned nose (upturned nasal tip).

Professional guidelines

PubMed

Campeau PM, Hennekam RC; DOORS syndrome collaborative group
Am J Med Genet C Semin Med Genet 2014 Sep;166C(3):327-32. Epub 2014 Aug 28 doi: 10.1002/ajmg.c.31412. PMID: 25169651

Recent clinical studies

Etiology

Campeau PM, Hennekam RC; DOORS syndrome collaborative group
Am J Med Genet C Semin Med Genet 2014 Sep;166C(3):327-32. Epub 2014 Aug 28 doi: 10.1002/ajmg.c.31412. PMID: 25169651

Diagnosis

Wang Q, Wu J, Yang J, Huang S, Yuan Y, Dai P
Am J Med Genet A 2023 Jan;191(1):183-189. Epub 2022 Nov 11 doi: 10.1002/ajmg.a.63011. PMID: 36369738Free PMC Article
Leventoğlu E, Döğer E, Büyükkaragöz B, Nalçacı S, Öner G, Alpman BN, Fidan K, Söylemezoğlu O, Bakkaloğlu SA
Pediatr Nephrol 2022 Oct;37(10):2341-2345. Epub 2022 Mar 14 doi: 10.1007/s00467-022-05510-8. PMID: 35288793
Lee Y, Choi Y, Seo GH, Kim GH, Keum C, Kim YM, Do HS, Choi J, Choi IH, Yoo HW, Lee BH
BMC Med Genomics 2021 Oct 27;14(1):254. doi: 10.1186/s12920-021-01104-9. PMID: 34706719Free PMC Article
Miyake N, Abdel-Salam G, Yamagata T, Eid MM, Osaka H, Okamoto N, Mohamed AM, Ikeda T, Afifi HH, Piard J, van Maldergem L, Mizuguchi T, Miyatake S, Tsurusaki Y, Matsumoto N
Am J Med Genet A 2016 Oct;170(10):2662-70. Epub 2016 Jun 5 doi: 10.1002/ajmg.a.37778. PMID: 27264538
Campeau PM, Hennekam RC; DOORS syndrome collaborative group
Am J Med Genet C Semin Med Genet 2014 Sep;166C(3):327-32. Epub 2014 Aug 28 doi: 10.1002/ajmg.c.31412. PMID: 25169651

Prognosis

Bina M
BMC Genomics 2020 May 31;21(1):378. doi: 10.1186/s12864-020-6688-8. PMID: 32475352Free PMC Article
Demily C, Duwime C, Lopez C, Hemimou C, Poisson A, Plasse J, Robert MP, Dénier C, Rossi M, Franck N, Besmond C, Barcia G, Boddaert N, Munnich A, Vaivre-Douret L
Psychiatr Genet 2019 Dec;29(6):237-242. doi: 10.1097/YPG.0000000000000225. PMID: 30933046
Vandeweyer G, Helsmoortel C, Van Dijck A, Vulto-van Silfhout AT, Coe BP, Bernier R, Gerdts J, Rooms L, van den Ende J, Bakshi M, Wilson M, Nordgren A, Hendon LG, Abdulrahman OA, Romano C, de Vries BB, Kleefstra T, Eichler EE, Van der Aa N, Kooy RF
Am J Med Genet C Semin Med Genet 2014 Sep;166C(3):315-26. Epub 2014 Aug 28 doi: 10.1002/ajmg.c.31413. PMID: 25169753Free PMC Article

Clinical prediction guides

Lee Y, Choi Y, Seo GH, Kim GH, Keum C, Kim YM, Do HS, Choi J, Choi IH, Yoo HW, Lee BH
BMC Med Genomics 2021 Oct 27;14(1):254. doi: 10.1186/s12920-021-01104-9. PMID: 34706719Free PMC Article
Bina M
BMC Genomics 2020 May 31;21(1):378. doi: 10.1186/s12864-020-6688-8. PMID: 32475352Free PMC Article
Demily C, Duwime C, Lopez C, Hemimou C, Poisson A, Plasse J, Robert MP, Dénier C, Rossi M, Franck N, Besmond C, Barcia G, Boddaert N, Munnich A, Vaivre-Douret L
Psychiatr Genet 2019 Dec;29(6):237-242. doi: 10.1097/YPG.0000000000000225. PMID: 30933046
Todd MA, Huh MS, Picketts DJ
Eur J Hum Genet 2016 Oct;24(10):1453-9. Epub 2016 May 11 doi: 10.1038/ejhg.2016.40. PMID: 27165002Free PMC Article
Vandeweyer G, Helsmoortel C, Van Dijck A, Vulto-van Silfhout AT, Coe BP, Bernier R, Gerdts J, Rooms L, van den Ende J, Bakshi M, Wilson M, Nordgren A, Hendon LG, Abdulrahman OA, Romano C, de Vries BB, Kleefstra T, Eichler EE, Van der Aa N, Kooy RF
Am J Med Genet C Semin Med Genet 2014 Sep;166C(3):315-26. Epub 2014 Aug 28 doi: 10.1002/ajmg.c.31413. PMID: 25169753Free PMC Article

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