U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Links from Gene

Steel syndrome(STLS)

MedGen UID:
767508
Concept ID:
C3554594
Disease or Syndrome
Synonym: STLS
SNOMED CT: Bilateral hip and radial head dislocations, short stature, scoliosis, carpal coalition, pes cavus, facial dysmorphism syndrome (1197589000); Steel syndrome (1197589000)
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
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 individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
 
Gene (location): COL27A1 (9q32)
 
Monarch Initiative: MONDO:0014061
OMIM®: 615155
Orphanet: ORPHA438117

Definition

Steel syndrome is characterized by characteristic facies, dislocated hips and radial heads, carpal coalition (fusion of carpal bones), short stature, scoliosis, and cervical spine anomalies. The dislocated hips are resistant to surgical intervention (summary by Flynn et al., 2010). [from OMIM]

Clinical features

From HPO
Pes planus
MedGen UID:
42034
Concept ID:
C0016202
Anatomical Abnormality
A foot where the longitudinal arch of the foot is in contact with the ground or floor when the individual is standing; or, in a patient lying supine, a foot where the arch is in contact with the surface of a flat board pressed against the sole of the foot by the examiner with a pressure similar to that expected from weight bearing; or, the height of the arch is reduced.
Dislocated radial head
MedGen UID:
488814
Concept ID:
C0265563
Congenital Abnormality
A dislocation of the head of the radius from its socket in the elbow joint.
Pes cavus
MedGen UID:
675590
Concept ID:
C0728829
Congenital Abnormality
An increase in height of the medial longitudinal arch of the foot that does not flatten on weight bearing (i.e., a distinctly hollow form of the sole of the foot when it is bearing weight).
Clinodactyly of the 5th finger
MedGen UID:
340456
Concept ID:
C1850049
Congenital Abnormality
Clinodactyly refers to a bending or curvature of the fifth finger in the radial direction (i.e., towards the 4th finger).
Limited elbow extension
MedGen UID:
401158
Concept ID:
C1867103
Finding
Limited ability to straighten the arm at the elbow joint.
Coxa vara
MedGen UID:
1790477
Concept ID:
C5551440
Anatomical Abnormality
Coxa vara includes all forms of decrease of the femoral neck shaft angle (the angle between the neck and the shaft of the femur) to less than 120 degrees.
Short stature
MedGen UID:
87607
Concept ID:
C0349588
Finding
A height below that which is expected according to age and gender norms. Although there is no universally accepted definition of short stature, many refer to "short stature" as height more than 2 standard deviations below the mean for age and gender (or below the 3rd percentile for age and gender dependent norms).
Sensorineural hearing loss disorder
MedGen UID:
9164
Concept ID:
C0018784
Disease or Syndrome
A type of hearing impairment in one or both ears related to an abnormal functionality of the cochlear nerve.
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.
Hip dislocation
MedGen UID:
42455
Concept ID:
C0019554
Injury or Poisoning
Displacement of the femur from its normal location in the hip joint.
Scoliosis
MedGen UID:
11348
Concept ID:
C0036439
Disease or Syndrome
The presence of an abnormal lateral curvature of the spine.
Carpal synostosis
MedGen UID:
98468
Concept ID:
C0431863
Congenital Abnormality
Synostosis (bony fusion) involving one or more bones of the carpus (scaphoid, lunate, triquetrum, trapezium, trapezoid, capitate, hamate, pisiform).
Lumbar hyperlordosis
MedGen UID:
263149
Concept ID:
C1184923
Finding
An abnormal accentuation of the inward curvature of the spine in the lumbar region.
Prominent forehead
MedGen UID:
373291
Concept ID:
C1837260
Finding
Forward prominence of the entire forehead, due to protrusion of the frontal bone.
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).
Wide nasal bridge
MedGen UID:
341441
Concept ID:
C1849367
Finding
Increased breadth of the nasal bridge (and with it, the nasal root).
Midface retrusion
MedGen UID:
339938
Concept ID:
C1853242
Anatomical Abnormality
Posterior positions and/or vertical shortening of the infraorbital and perialar regions, or increased concavity of the face and/or reduced nasolabial angle.
Hypertelorism
MedGen UID:
9373
Concept ID:
C0020534
Finding
Although hypertelorism means an excessive distance between any paired organs (e.g., the nipples), the use of the word has come to be confined to ocular hypertelorism. Hypertelorism occurs as an isolated feature and is also a feature of many syndromes, e.g., Opitz G syndrome (see 300000), Greig cephalopolysyndactyly (175700), and Noonan syndrome (163950) (summary by Cohen et al., 1995).

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSteel syndrome

Professional guidelines

PubMed

Tasaka S, Ohshimo S, Takeuchi M, Yasuda H, Ichikado K, Tsushima K, Egi M, Hashimoto S, Shime N, Saito O, Matsumoto S, Nango E, Okada Y, Hayashi K, Sakuraya M, Nakajima M, Okamori S, Miura S, Fukuda T, Ishihara T, Kamo T, Yatabe T, Norisue Y, Aoki Y, Iizuka Y, Kondo Y, Narita C, Kawakami D, Okano H, Takeshita J, Anan K, Okazaki SR, Taito S, Hayashi T, Mayumi T, Terayama T, Kubota Y, Abe Y, Iwasaki Y, Kishihara Y, Kataoka J, Nishimura T, Yonekura H, Ando K, Yoshida T, Masuyama T, Sanui M; ARDS Clinical Practice Guideline Committee 2021 from the Japanese Respiratory Society, the Japanese Society of Intensive Care Medicine, and the Japanese Society of Respiratory Care Medicine
Respir Investig 2022 Jul;60(4):446-495. Epub 2022 Jun 24 doi: 10.1016/j.resinv.2022.05.003. PMID: 35753956
Hamzah S, Fatimah A, Aswad I, Kurniaty
Gac Sanit 2021;35 Suppl 2:S588-S590. doi: 10.1016/j.gaceta.2021.10.089. PMID: 34929907Free PMC Article
Kelly JR, Sorenson HW, Turner EG
J Prosthet Dent 1978 May;39(5):554-60. doi: 10.1016/s0022-3913(78)80193-0. PMID: 274550

Recent clinical studies

Etiology

Gonzaga-Jauregui C, Gamble CN, Yuan B, Penney S, Jhangiani S, Muzny DM, Gibbs RA, Lupski JR, Hecht JT
Eur J Hum Genet 2015 Mar;23(3):342-6. Epub 2014 Jul 2 doi: 10.1038/ejhg.2014.107. PMID: 24986830Free PMC Article
Flynn JM, Ramirez N, Betz R, Mulcahey MJ, Pino F, Herrera-Soto JA, Carlo S, Cornier AS
J Pediatr Orthop 2010 Apr-May;30(3):282-8. doi: 10.1097/BPO.0b013e3181d3e464. PMID: 20357596

Diagnosis

Thakur S, Paliwal P, Saxena KK
J Postgrad Med 2023 Apr-Jun;69(2):99-101. doi: 10.4103/jpgm.jpgm_1153_21. PMID: 36695248Free PMC Article
Frigola G, Del Rincón OG, Florián VB, Fita AV, Campos B, Pauta M, Puimedon MS, Oliva R, Borrell A, Nadal A
Virchows Arch 2021 Aug;479(2):413-418. Epub 2021 Jan 7 doi: 10.1007/s00428-020-02979-2. PMID: 33411029
Amlie-Wolf L, Moyer-Harasink S, Carr AM, Giampietro P, Schneider A, Simon M
Am J Med Genet A 2020 Apr;182(4):798-803. Epub 2020 Jan 5 doi: 10.1002/ajmg.a.61465. PMID: 31903681
Gonzaga-Jauregui C, Gamble CN, Yuan B, Penney S, Jhangiani S, Muzny DM, Gibbs RA, Lupski JR, Hecht JT
Eur J Hum Genet 2015 Mar;23(3):342-6. Epub 2014 Jul 2 doi: 10.1038/ejhg.2014.107. PMID: 24986830Free PMC Article
Mirzaie M, Fatehpur S, Friedrich M, Sossalla S, Sohns C, Schoendube FA, Schmitto JD
Ann Thorac Cardiovasc Surg 2011;17(4):347-51. doi: 10.5761/atcs.oa.09.01432. PMID: 21881320

Therapy

Mirzaie M, Fatehpur S, Friedrich M, Sossalla S, Sohns C, Schoendube FA, Schmitto JD
Ann Thorac Cardiovasc Surg 2011;17(4):347-51. doi: 10.5761/atcs.oa.09.01432. PMID: 21881320

Prognosis

Pölsler L, Schatz UA, Simma B, Zschocke J, Rudnik-Schöneborn S
Am J Med Genet A 2020 Apr;182(4):730-734. Epub 2020 Jan 8 doi: 10.1002/ajmg.a.61478. PMID: 31913554Free PMC Article
Gonzaga-Jauregui C, Gamble CN, Yuan B, Penney S, Jhangiani S, Muzny DM, Gibbs RA, Lupski JR, Hecht JT
Eur J Hum Genet 2015 Mar;23(3):342-6. Epub 2014 Jul 2 doi: 10.1038/ejhg.2014.107. PMID: 24986830Free PMC Article
Flynn JM, Ramirez N, Betz R, Mulcahey MJ, Pino F, Herrera-Soto JA, Carlo S, Cornier AS
J Pediatr Orthop 2010 Apr-May;30(3):282-8. doi: 10.1097/BPO.0b013e3181d3e464. PMID: 20357596

Clinical prediction guides

Girisha KM, Jacob P, SriLakshmi Bhavani G, Shah H, Mortier GR
Eur J Med Genet 2022 Jun;65(6):104521. Epub 2022 May 11 doi: 10.1016/j.ejmg.2022.104521. PMID: 35568358

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

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...