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Noonan syndrome-like disorder with loose anagen hair(NS/LAH)

MedGen UID:
334697
Concept ID:
C1843181
Disease or Syndrome
Synonyms: Noonan-like syndrome with loose anagen hair; NS/LAH
SNOMED CT: Noonan syndrome-like disorder with loose anagen hair (723444009)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
 
Monarch Initiative: MONDO:0011899
OMIM® Phenotypic series: PS607721
Orphanet: ORPHA2701

Definition

A Noonan-related syndrome with characteristics of facial anomalies suggestive of Noonan syndrome, a distinctive hair anomaly described as loose anagen hair syndrome, frequent congenital heart defects, distinctive skin features with darkly pigmented skin, keratosis pilaris, eczema or occasional neonatal ichthyosis and short stature, often associated with a growth hormone deficiency and psychomotor delay. There is evidence that this syndrome is caused by heterozygous mutation in the SHOC2 gene on chromosome 10q25. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNoonan syndrome-like disorder with loose anagen hair
Follow this link to review classifications for Noonan syndrome-like disorder with loose anagen hair in Orphanet.

Recent clinical studies

Etiology

Zenker M
Am J Med Genet C Semin Med Genet 2022 Dec;190(4):414-424. Epub 2022 Nov 25 doi: 10.1002/ajmg.c.32015. PMID: 36428239
Akgun-Dogan O, Simsek-Kiper PO, Taskiran E, Lissewski C, Brinkmann J, Schanze D, Göçmen R, Cagdas D, Bilginer Y, Utine GE, Zenker M, Ozen S, Tezcan İ, Alikasifoglu M, Boduroğlu K
Am J Med Genet A 2019 Dec;179(12):2474-2480. Epub 2019 Oct 4 doi: 10.1002/ajmg.a.61363. PMID: 31584751

Diagnosis

Maruwaka K, Nakajima Y, Yamada T, Tanaka T, Kosaki R, Inagaki H, Kosaki K, Kurahashi H
Am J Med Genet A 2022 Jul;188(7):2246-2250. Epub 2022 Mar 25 doi: 10.1002/ajmg.a.62733. PMID: 35338599
Zhou P, Zhu L, Fan Q, Liu Y, Zhang T, Yang T, Chen J, Cheng Q, Li T, Chen L
Am J Med Genet A 2020 Aug;182(8):1967-1971. Epub 2020 Jun 1 doi: 10.1002/ajmg.a.61638. PMID: 32476286
Akgun-Dogan O, Simsek-Kiper PO, Taskiran E, Lissewski C, Brinkmann J, Schanze D, Göçmen R, Cagdas D, Bilginer Y, Utine GE, Zenker M, Ozen S, Tezcan İ, Alikasifoglu M, Boduroğlu K
Am J Med Genet A 2019 Dec;179(12):2474-2480. Epub 2019 Oct 4 doi: 10.1002/ajmg.a.61363. PMID: 31584751

Therapy

Zhou P, Zhu L, Fan Q, Liu Y, Zhang T, Yang T, Chen J, Cheng Q, Li T, Chen L
Am J Med Genet A 2020 Aug;182(8):1967-1971. Epub 2020 Jun 1 doi: 10.1002/ajmg.a.61638. PMID: 32476286

Clinical prediction guides

Zhou P, Zhu L, Fan Q, Liu Y, Zhang T, Yang T, Chen J, Cheng Q, Li T, Chen L
Am J Med Genet A 2020 Aug;182(8):1967-1971. Epub 2020 Jun 1 doi: 10.1002/ajmg.a.61638. PMID: 32476286
Akgun-Dogan O, Simsek-Kiper PO, Taskiran E, Lissewski C, Brinkmann J, Schanze D, Göçmen R, Cagdas D, Bilginer Y, Utine GE, Zenker M, Ozen S, Tezcan İ, Alikasifoglu M, Boduroğlu K
Am J Med Genet A 2019 Dec;179(12):2474-2480. Epub 2019 Oct 4 doi: 10.1002/ajmg.a.61363. PMID: 31584751

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