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Developmental delay with short stature, dysmorphic facial features, and sparse hair 1

MedGen UID:
1002069
Concept ID:
CN323360
Disease or Syndrome
Synonym: LOUCKS-INNES SYNDROME
 
Gene (location): DPH1 (17p13.3)
 
Monarch Initiative: MONDO:0800438
OMIM®: 616901

Definition

Developmental delay with short stature, dysmorphic facial features, and sparse hair-1 (DEDSSH1) is an autosomal recessive syndromic disorder characterized by the constellation of these features apparent from infancy. Craniosynostosis most often with a broad forehead, central nervous system malformations, cardiac defects, genital anomalies, or hypotonia may also be present (summary by Urreizti et al., 2020). Genetic Heterogeneity of Developmental Delay with Short Stature, Dysmorphic Facial Features, and Sparse Hair DEDSSH2 (620062) is caused by mutation in the DPH2 gene (603456) on chromosome 1p34. [from OMIM]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Recent clinical studies

Etiology

Gripp KW, Zand DJ, Demmer L, Anderson CE, Dobyns WB, Zackai EH, Denenberg E, Jenny K, Stabley DL, Sol-Church K
Am J Med Genet A 2013 Oct;161A(10):2420-30. Epub 2013 Aug 5 doi: 10.1002/ajmg.a.36098. PMID: 23918763Free PMC Article

Diagnosis

Lallar M, Bijarnia-Mahay S, Verma IC, Mandal K, Puri RD
Indian Pediatr 2021 Jan 15;58(1):30-33. PMID: 33452774
Sekiguchi F, Nasiri J, Sedghi M, Salehi M, Hosseinzadeh M, Okamoto N, Mizuguchi T, Nakashima M, Miyatake S, Takata A, Miyake N, Matsumoto N
J Hum Genet 2018 Apr;63(4):487-491. Epub 2018 Feb 6 doi: 10.1038/s10038-017-0404-9. PMID: 29410513
Leduc MS, Niu Z, Bi W, Zhu W, Miloslavskaya I, Chiang T, Streff H, Seavitt JR, Murray SA, Eng C, Chan A, Yang Y, Lalani SR
Am J Med Genet A 2016 Aug;170(8):2206-11. Epub 2016 Jun 2 doi: 10.1002/ajmg.a.37780. PMID: 27250922Free PMC Article
Zmolikova M, Puchmajerova A, Hecht P, Lebl J, Trkova M, Krepelova A
Am J Med Genet A 2014 May;164A(5):1218-21. Epub 2014 Jan 23 doi: 10.1002/ajmg.a.36404. PMID: 24458596
Gripp KW, Zand DJ, Demmer L, Anderson CE, Dobyns WB, Zackai EH, Denenberg E, Jenny K, Stabley DL, Sol-Church K
Am J Med Genet A 2013 Oct;161A(10):2420-30. Epub 2013 Aug 5 doi: 10.1002/ajmg.a.36098. PMID: 23918763Free PMC Article

Therapy

Zmolikova M, Puchmajerova A, Hecht P, Lebl J, Trkova M, Krepelova A
Am J Med Genet A 2014 May;164A(5):1218-21. Epub 2014 Jan 23 doi: 10.1002/ajmg.a.36404. PMID: 24458596

Prognosis

Zmolikova M, Puchmajerova A, Hecht P, Lebl J, Trkova M, Krepelova A
Am J Med Genet A 2014 May;164A(5):1218-21. Epub 2014 Jan 23 doi: 10.1002/ajmg.a.36404. PMID: 24458596
Gripp KW, Zand DJ, Demmer L, Anderson CE, Dobyns WB, Zackai EH, Denenberg E, Jenny K, Stabley DL, Sol-Church K
Am J Med Genet A 2013 Oct;161A(10):2420-30. Epub 2013 Aug 5 doi: 10.1002/ajmg.a.36098. PMID: 23918763Free PMC Article

Clinical prediction guides

Zmolikova M, Puchmajerova A, Hecht P, Lebl J, Trkova M, Krepelova A
Am J Med Genet A 2014 May;164A(5):1218-21. Epub 2014 Jan 23 doi: 10.1002/ajmg.a.36404. PMID: 24458596

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