U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Developmental delay with dysmorphic facies and dental anomalies(DEFDA)

MedGen UID:
1785587
Concept ID:
C5543197
Disease or Syndrome
Synonyms: DEFDA; DEVELOPMENTAL DELAY WITH DYSMORPHIC FACIES AND DENTAL ANOMALIES
 
Gene (location): SATB1 (3p24.3)
 
Monarch Initiative: MONDO:0030988
OMIM®: 619228

Definition

Developmental delay with dysmorphic facies and dental anomalies (DEFDA) is characterized by generally mild global developmental delay with variably impaired intellectual development, walking by 2 to 3 years, and slow language acquisition. The severity of the disorder ranges from moderate cognitive deficits to mild learning difficulties or behavioral abnormalities. Most patients have dysmorphic facial features, often with abnormal dentition and nonspecific visual defects, such as myopia, astigmatism, and strabismus. Although rare, involvement of other systems, such as skeletal, cardiac, and gastrointestinal, may be present (summary by den Hoed et al., 2021). [from OMIM]

Clinical features

From HPO
Atypical behavior
MedGen UID:
14048
Concept ID:
C0004941
Sign or Symptom
Atypical behavior is an abnormality in a person's actions that can be controlled or modulated by the will of the individual. While abnormal behaviors can be difficult to control, they are distinct from other abnormal actions that cannot be affected by the individual's will.
Cerebellar ataxia
MedGen UID:
849
Concept ID:
C0007758
Disease or Syndrome
Cerebellar ataxia refers to ataxia due to dysfunction of the cerebellum. This causes a variety of elementary neurological deficits including asynergy (lack of coordination between muscles, limbs and joints), dysmetria (lack of ability to judge distances that can lead to under- or overshoot in grasping movements), and dysdiadochokinesia (inability to perform rapid movements requiring antagonizing muscle groups to be switched on and off repeatedly).
Dysarthria
MedGen UID:
8510
Concept ID:
C0013362
Mental or Behavioral Dysfunction
Dysarthric speech is a general description referring to a neurological speech disorder characterized by poor articulation. Depending on the involved neurological structures, dysarthria may be further classified as spastic, flaccid, ataxic, hyperkinetic and hypokinetic, or mixed.
Spasticity
MedGen UID:
7753
Concept ID:
C0026838
Sign or Symptom
A motor disorder characterized by a velocity-dependent increase in tonic stretch reflexes with increased muscle tone, exaggerated (hyperexcitable) tendon reflexes.
Seizure
MedGen UID:
20693
Concept ID:
C0036572
Sign or Symptom
A seizure is an intermittent abnormality of nervous system physiology characterized by a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain.
Sleep abnormality
MedGen UID:
52372
Concept ID:
C0037317
Finding
An abnormal pattern in the quality, quantity, or characteristics of sleep.
EEG abnormality
MedGen UID:
56235
Concept ID:
C0151611
Finding
Abnormality observed by electroencephalogram (EEG), which is used to record of the brain's spontaneous electrical activity from multiple electrodes placed on the scalp.
Delayed speech and language development
MedGen UID:
105318
Concept ID:
C0454644
Finding
A degree of language development that is significantly below the norm for a child of a specified age.
Global developmental delay
MedGen UID:
107838
Concept ID:
C0557874
Finding
A delay in the achievement of motor or mental milestones in the domains of development of a child, including motor skills, speech and language, cognitive skills, and social and emotional skills. This term should only be used to describe children younger than five years of age.
Developmental regression
MedGen UID:
324613
Concept ID:
C1836830
Disease or Syndrome
Loss of developmental skills, as manifested by loss of developmental milestones.
Motor delay
MedGen UID:
381392
Concept ID:
C1854301
Finding
A type of Developmental delay characterized by a delay in acquiring motor skills.
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.
Hypotonia
MedGen UID:
10133
Concept ID:
C0026827
Finding
Hypotonia is an abnormally low muscle tone (the amount of tension or resistance to movement in a muscle). Even when relaxed, muscles have a continuous and passive partial contraction which provides some resistance to passive stretching. Hypotonia thus manifests as diminished resistance to passive stretching. Hypotonia is not the same as muscle weakness, although the two conditions can co-exist.
Microcephaly
MedGen UID:
1644158
Concept ID:
C4551563
Finding
Head circumference below 2 standard deviations below the mean for age and gender.

Professional guidelines

PubMed

Mannino EA, Miyawaki H, Santen G, Schrier Vergano SA
Am J Med Genet A 2018 Nov;176(11):2250-2258. Epub 2018 Oct 1 doi: 10.1002/ajmg.a.40471. PMID: 30276971

Recent clinical studies

Etiology

Almashaqbeh SS, Aljammal DS, Alharahsheh HM, Alqudah SF, Alkrimeen RA
Med Arch 2022 Aug;76(4):301-304. doi: 10.5455/medarh.2022.76.301-304. PMID: 36313953Free PMC Article
Vasconcelos G, Stenehjem JS, Axelsson S, Saeves R
Orphanet J Rare Dis 2022 Feb 22;17(1):77. doi: 10.1186/s13023-022-02222-y. PMID: 35193626Free PMC Article
Lopez Martinolich M, Northrup H, Mancias P, Hillman P, Rao K, Mowrey K
Mol Genet Genomic Med 2022 Mar;10(3):e1879. Epub 2022 Feb 5 doi: 10.1002/mgg3.1879. PMID: 35122698Free PMC Article
Goodwin AF, Oberoi S, Landan M, Charles C, Massie JC, Fairley C, Rauen KA, Klein OD
Am J Med Genet A 2014 Jun;164A(6):1425-30. Epub 2014 Mar 25 doi: 10.1002/ajmg.a.36475. PMID: 24668879Free PMC Article
Moiseiwitsch JR
Crit Rev Oral Biol Med 2000;11(2):230-9. doi: 10.1177/10454411000110020601. PMID: 12002817

Diagnosis

Almashaqbeh SS, Aljammal DS, Alharahsheh HM, Alqudah SF, Alkrimeen RA
Med Arch 2022 Aug;76(4):301-304. doi: 10.5455/medarh.2022.76.301-304. PMID: 36313953Free PMC Article
Roelandt MA, Devriendt K, de Llano-Pérula MC, Raes M, Willems G, Verdonck A
Cleft Palate Craniofac J 2021 Apr;58(4):505-513. Epub 2020 Oct 16 doi: 10.1177/1055665620954090. PMID: 33063524
Lonardo F, Lonardo MS, Acquaviva F, Della Monica M, Scarano F, Scarano G
Clin Genet 2019 Feb;95(2):253-261. Epub 2018 Jan 25 doi: 10.1111/cge.13127. PMID: 28857140
Al-Tamimi B, Abela S, Jeremiah HG, Evans RD
Int J Paediatr Dent 2017 Nov;27(6):583-587. Epub 2017 Jun 21 doi: 10.1111/ipd.12309. PMID: 28635076
Almashraki N, Abdulnabee MZ, Sukalo M, Alrajoudi A, Sharafadeen I, Zenker M
World J Gastroenterol 2011 Oct 7;17(37):4247-50. doi: 10.3748/wjg.v17.i37.4247. PMID: 22072859Free PMC Article

Therapy

Moiseiwitsch JR
Crit Rev Oral Biol Med 2000;11(2):230-9. doi: 10.1177/10454411000110020601. PMID: 12002817
Burnett JW, Schwartz MF, Berberian BJ
J Am Acad Dermatol 1988 Feb;18(2 Pt 2):437-40. doi: 10.1016/s0190-9622(88)70065-1. PMID: 3343412

Prognosis

Stephen J, Maddirevula S, Nampoothiri S, Burke JD, Herzog M, Shukla A, Steindl K, Eskin A, Patil SJ, Joset P, Lee H, Garrett LJ, Yokoyama T, Balanda N, Bodine SP, Tolman NJ, Zerfas PM, Zheng A, Ramantani G, Girisha KM, Rivas C, Suresh PV, Elkahloun A, Alsaif HS, Wakil SM, Mahmoud L, Ali R, Prochazkova M; Undiagnosed Diseases Network members, Kulkarni AB, Ben-Omran T, Colak D, Morris HD, Rauch A, Martinez-Agosto JA, Nelson SF, Alkuraya FS, Gahl WA, Malicdan MCV
Am J Hum Genet 2018 Dec 6;103(6):948-967. doi: 10.1016/j.ajhg.2018.11.001. PMID: 30526868Free PMC Article
Al-Tamimi B, Abela S, Jeremiah HG, Evans RD
Int J Paediatr Dent 2017 Nov;27(6):583-587. Epub 2017 Jun 21 doi: 10.1111/ipd.12309. PMID: 28635076
Miyatake S, Okamoto N, Stark Z, Nabetani M, Tsurusaki Y, Nakashima M, Miyake N, Mizuguchi T, Ohtake A, Saitsu H, Matsumoto N
J Hum Genet 2017 Aug;62(8):741-746. Epub 2017 Mar 2 doi: 10.1038/jhg.2017.24. PMID: 28250421Free PMC Article
Robotta P, Schafer E
Quintessence Int 2011 Apr;42(4):331-8. PMID: 21516279
Walpole SM, Ronce N, Grayson C, Dessay B, Yates JR, Trump D, Toutain A
Hum Genet 1999 May;104(5):410-1. doi: 10.1007/s004390050976. PMID: 10394933

Clinical prediction guides

Sinthuwiwat T, Ittiwut C, Porntaveetus T, Shotelersuk V
Am J Med Genet A 2019 May;179(5):758-761. Epub 2019 Mar 3 doi: 10.1002/ajmg.a.61106. PMID: 30828969
Stephen J, Maddirevula S, Nampoothiri S, Burke JD, Herzog M, Shukla A, Steindl K, Eskin A, Patil SJ, Joset P, Lee H, Garrett LJ, Yokoyama T, Balanda N, Bodine SP, Tolman NJ, Zerfas PM, Zheng A, Ramantani G, Girisha KM, Rivas C, Suresh PV, Elkahloun A, Alsaif HS, Wakil SM, Mahmoud L, Ali R, Prochazkova M; Undiagnosed Diseases Network members, Kulkarni AB, Ben-Omran T, Colak D, Morris HD, Rauch A, Martinez-Agosto JA, Nelson SF, Alkuraya FS, Gahl WA, Malicdan MCV
Am J Hum Genet 2018 Dec 6;103(6):948-967. doi: 10.1016/j.ajhg.2018.11.001. PMID: 30526868Free PMC Article
Almashraki N, Abdulnabee MZ, Sukalo M, Alrajoudi A, Sharafadeen I, Zenker M
World J Gastroenterol 2011 Oct 7;17(37):4247-50. doi: 10.3748/wjg.v17.i37.4247. PMID: 22072859Free PMC Article
Stalin A, Varma BR; Jayanthi
J Indian Soc Pedod Prev Dent 2006 May;24 Suppl 1:S27-30. PMID: 16891747
Fujimoto A, Lipson M, Lacro RV, Shinno NW, Boelter WD, Jones KL, Wilson MG
Am J Med Genet 1987 Aug;27(4):943-51. doi: 10.1002/ajmg.1320270422. PMID: 3321995

Recent systematic reviews

Simmers R, Goodwin A, Al Saif H, Couser N
Ophthalmic Genet 2022 Oct;43(5):699-702. Epub 2022 Jun 27 doi: 10.1080/13816810.2022.2089358. PMID: 35762114

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...