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Short stature-wormian bones-dextrocardia syndrome

MedGen UID:
350025
Concept ID:
C1861448
Disease or Syndrome
Synonyms: GROWTH HORMONE DEFICIENCY WITH WORMIAN BONES, CARDIAC ANOMALY, AND BRACHYCAMPTODACTYLY; Stratton-Parker syndrome
SNOMED CT: Short stature, wormian bones, dextrocardia syndrome (763631006); Stratton Parker syndrome (763631006)
 
Monarch Initiative: MONDO:0008499
OMIM®: 185120
Orphanet: ORPHA2863

Definition

A multiple congenital anomalies syndrome with characteristics of wormian bones, dextrocardia and short stature due to a growth hormone deficiency. Additional manifestations that have been reported include brachycamptodactyly, kidney hypoplasia, bilateral cryptorchidism, midshaft hypospadias, imperforate anus/anorectal agenesis, body asymmetry, mild developmental delay, hemimegalencephaly and facial dysmorphism, such as hypotelorism, downslanting palpebral fissures, low-set and posteriorly angulated ears, depressed nasal bridge and microstomia. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVShort stature-wormian bones-dextrocardia syndrome
Follow this link to review classifications for Short stature-wormian bones-dextrocardia syndrome in Orphanet.

Professional guidelines

PubMed

Butler MG, Miller JL, Forster JL
Curr Pediatr Rev 2019;15(4):207-244. doi: 10.2174/1573396315666190716120925. PMID: 31333129Free PMC Article
Oliveira CB, Maher CG, Pinto RZ, Traeger AC, Lin CC, Chenot JF, van Tulder M, Koes BW
Eur Spine J 2018 Nov;27(11):2791-2803. Epub 2018 Jul 3 doi: 10.1007/s00586-018-5673-2. PMID: 29971708
Schiffman E, Ohrbach R, Truelove E, Look J, Anderson G, Goulet JP, List T, Svensson P, Gonzalez Y, Lobbezoo F, Michelotti A, Brooks SL, Ceusters W, Drangsholt M, Ettlin D, Gaul C, Goldberg LJ, Haythornthwaite JA, Hollender L, Jensen R, John MT, De Laat A, de Leeuw R, Maixner W, van der Meulen M, Murray GM, Nixdorf DR, Palla S, Petersson A, Pionchon P, Smith B, Visscher CM, Zakrzewska J, Dworkin SF; International RDC/TMD Consortium Network, International association for Dental Research; Orofacial Pain Special Interest Group, International Association for the Study of Pain
J Oral Facial Pain Headache 2014 Winter;28(1):6-27. doi: 10.11607/jop.1151. PMID: 24482784Free PMC Article

Recent clinical studies

Etiology

Fan X, Zhao S, Yu C, Wu D, Yan Z, Fan L, Song Y, Wang Y, Li C, Ming Y, Gui B, Niu Y, Li X, Yang X, Luo S, Zhang Q, Zhao X, Pan H, Li M, Xia W, Qiu G, Liu P, Zhang S, Zhang J, Wu Z; Deciphering Disorders Involving Scoliosis and COmorbidities (DISCO) study, Lupski JR, Posey JE, Chen S, Gong C, Wu N
J Genet Genomics 2021 May 20;48(5):396-402. Epub 2021 Mar 22 doi: 10.1016/j.jgg.2021.02.008. PMID: 34006472
Pereira AFJR, Coelho TOA
An Bras Dermatol 2020 May-Jun;95(3):271-277. Epub 2020 Mar 25 doi: 10.1016/j.abd.2020.02.001. PMID: 32317131Free PMC Article
Cereda A, Carey JC
Orphanet J Rare Dis 2012 Oct 23;7:81. doi: 10.1186/1750-1172-7-81. PMID: 23088440Free PMC Article
Hedley PL, Jørgensen P, Schlamowitz S, Wangari R, Moolman-Smook J, Brink PA, Kanters JK, Corfield VA, Christiansen M
Hum Mutat 2009 Nov;30(11):1486-511. doi: 10.1002/humu.21106. PMID: 19862833
Brugada P, Brugada J
J Am Coll Cardiol 1992 Nov 15;20(6):1391-6. doi: 10.1016/0735-1097(92)90253-j. PMID: 1309182

Diagnosis

Zizzo J, F Sávio L, Ramasamy R, F N Lima T
Eur Urol Focus 2023 Jan;9(1):22-24. Epub 2022 Oct 22 doi: 10.1016/j.euf.2022.09.016. PMID: 36283946
Kim JA, Chelu MG
Tex Heart Inst J 2021 Sep 1;48(4) doi: 10.14503/THIJ-20-7482. PMID: 34669951Free PMC Article
Fan X, Zhao S, Yu C, Wu D, Yan Z, Fan L, Song Y, Wang Y, Li C, Ming Y, Gui B, Niu Y, Li X, Yang X, Luo S, Zhang Q, Zhao X, Pan H, Li M, Xia W, Qiu G, Liu P, Zhang S, Zhang J, Wu Z; Deciphering Disorders Involving Scoliosis and COmorbidities (DISCO) study, Lupski JR, Posey JE, Chen S, Gong C, Wu N
J Genet Genomics 2021 May 20;48(5):396-402. Epub 2021 Mar 22 doi: 10.1016/j.jgg.2021.02.008. PMID: 34006472
Wennig R, Eyer F, Schaper A, Zilker T, Andresen-Streichert H
Dtsch Arztebl Int 2020 Oct 16;117(42):701-708. doi: 10.3238/arztebl.2020.0701. PMID: 33559585Free PMC Article
Cereda A, Carey JC
Orphanet J Rare Dis 2012 Oct 23;7:81. doi: 10.1186/1750-1172-7-81. PMID: 23088440Free PMC Article

Therapy

Cosci F, Chouinard G
Psychother Psychosom 2020;89(5):283-306. Epub 2020 Apr 7 doi: 10.1159/000506868. PMID: 32259826
Richards JR, Gordon BK, Danielson AR, Moulin AK
Pharmacotherapy 2017 Jun;37(6):725-734. Epub 2017 May 12 doi: 10.1002/phar.1931. PMID: 28370228
Pan Q, Angelina A, Marrone M, Stark WJ, Akpek EK
Cochrane Database Syst Rev 2017 Feb 28;2(2):CD009327. doi: 10.1002/14651858.CD009327.pub3. PMID: 28245347Free PMC Article
Smart KM, Wand BM, O'Connell NE
Cochrane Database Syst Rev 2016 Feb 24;2(2):CD010853. doi: 10.1002/14651858.CD010853.pub2. PMID: 26905470Free PMC Article
van der Heijden RA, Lankhorst NE, van Linschoten R, Bierma-Zeinstra SM, van Middelkoop M
Cochrane Database Syst Rev 2015 Jan 20;1(1):CD010387. doi: 10.1002/14651858.CD010387.pub2. PMID: 25603546Free PMC Article

Prognosis

Wennig R, Eyer F, Schaper A, Zilker T, Andresen-Streichert H
Dtsch Arztebl Int 2020 Oct 16;117(42):701-708. doi: 10.3238/arztebl.2020.0701. PMID: 33559585Free PMC Article
Coniglio AC, Mentz RJ
Heart Fail Clin 2020 Jan;16(1):45-51. Epub 2019 Oct 29 doi: 10.1016/j.hfc.2019.08.009. PMID: 31735314
Fabre A, Martinez-Vinson C, Goulet O, Badens C
Orphanet J Rare Dis 2013 Jan 9;8:5. doi: 10.1186/1750-1172-8-5. PMID: 23302111Free PMC Article
Cereda A, Carey JC
Orphanet J Rare Dis 2012 Oct 23;7:81. doi: 10.1186/1750-1172-7-81. PMID: 23088440Free PMC Article
Marshall JD, Beck S, Maffei P, Naggert JK
Eur J Hum Genet 2007 Dec;15(12):1193-202. Epub 2007 Oct 17 doi: 10.1038/sj.ejhg.5201933. PMID: 17940554

Clinical prediction guides

Shi Z, Tuomilehto J, Kronfeld-Schor N, Alberti G, Stern N, El-Osta A, Chai Z, Bilu C, Einat H, Zimmet P
Nutrients 2022 Dec 14;14(24) doi: 10.3390/nu14245317. PMID: 36558476Free PMC Article
Sanchez-Jimeno C, Blanco-Kelly F, López-Grondona F, Losada-Del Pozo R, Moreno B, Rodrigo-Moreno M, Martinez-Cayuelas E, Riveiro-Alvarez R, Fenollar-Cortés M, Ayuso C, Rodríguez de Alba M, Lorda-Sanchez I, Almoguera B
Genes (Basel) 2021 Aug 30;12(9) doi: 10.3390/genes12091360. PMID: 34573342Free PMC Article
Kattah JC
Stroke Vasc Neurol 2018 Dec;3(4):190-196. Epub 2018 Jun 23 doi: 10.1136/svn-2018-000160. PMID: 30637123Free PMC Article
Moortgat S, Berland S, Aukrust I, Maystadt I, Baker L, Benoit V, Caro-Llopis A, Cooper NS, Debray FG, Faivre L, Gardeitchik T, Haukanes BI, Houge G, Kivuva E, Martinez F, Mehta SG, Nassogne MC, Powell-Hamilton N, Pfundt R, Rosello M, Prescott T, Vasudevan P, van Loon B, Verellen-Dumoulin C, Verloes A, Lippe CV, Wakeling E, Wilkie AOM, Wilson L, Yuen A, Study D, Low KJ, Newbury-Ecob RA
Eur J Hum Genet 2018 Jan;26(1):64-74. Epub 2017 Nov 27 doi: 10.1038/s41431-017-0038-6. PMID: 29180823Free PMC Article
Fabre A, Martinez-Vinson C, Goulet O, Badens C
Orphanet J Rare Dis 2013 Jan 9;8:5. doi: 10.1186/1750-1172-8-5. PMID: 23302111Free PMC Article

Recent systematic reviews

Zhu Y, Su P, Xu T, Zhang L, Fu W
J Orthop Surg Res 2022 Jun 3;17(1):296. doi: 10.1186/s13018-022-03187-1. PMID: 35659016Free PMC Article
Tan HL, Smith JG, Hoffmann J, Renton T
Cephalalgia 2022 Feb;42(2):128-161. Epub 2021 Aug 18 doi: 10.1177/03331024211036152. PMID: 34404247Free PMC Article
Lew J, Kim J, Nair P
J Man Manip Ther 2021 Jun;29(3):136-146. Epub 2020 Sep 22 doi: 10.1080/10669817.2020.1822618. PMID: 32962567Free PMC Article
Lorenzo-López L, Maseda A, de Labra C, Regueiro-Folgueira L, Rodríguez-Villamil JL, Millán-Calenti JC
BMC Geriatr 2017 May 15;17(1):108. doi: 10.1186/s12877-017-0496-2. PMID: 28506216Free PMC Article
van der Heijden RA, Lankhorst NE, van Linschoten R, Bierma-Zeinstra SM, van Middelkoop M
Cochrane Database Syst Rev 2015 Jan 20;1(1):CD010387. doi: 10.1002/14651858.CD010387.pub2. PMID: 25603546Free PMC Article

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