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Congenital Systemic Disorder

MedGen UID:
474891
Concept ID:
C3273258
Congenital Abnormality

Definition

An inherited disorder that presents with systemic manifestations. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital Systemic Disorder

Professional guidelines

PubMed

Wollenberg A, Werfel T, Ring J, Ott H, Gieler U, Weidinger S
Dtsch Arztebl Int 2023 Mar 31;120(13):224-234. doi: 10.3238/arztebl.m2023.0011. PMID: 36747484Free PMC Article
Konstam MA, Kiernan MS, Bernstein D, Bozkurt B, Jacob M, Kapur NK, Kociol RD, Lewis EF, Mehra MR, Pagani FD, Raval AN, Ward C; American Heart Association Council on Clinical Cardiology; Council on Cardiovascular Disease in the Young; and Council on Cardiovascular Surgery and Anesthesia
Circulation 2018 May 15;137(20):e578-e622. Epub 2018 Apr 12 doi: 10.1161/CIR.0000000000000560. PMID: 29650544
Couser WG
Clin J Am Soc Nephrol 2017 Jun 7;12(6):983-997. Epub 2017 May 26 doi: 10.2215/CJN.11761116. PMID: 28550082Free PMC Article

Recent clinical studies

Etiology

Atwell K, Michael W, Dubey J, James S, Martonffy A, Anderson S, Rudin N, Schrager S
J Am Board Fam Med 2021 Jul-Aug;34(4):838-848. doi: 10.3122/jabfm.2021.04.200374. PMID: 34312277
Lateef A, Petri M
Rheum Dis Clin North Am 2017 May;43(2):215-226. Epub 2017 Mar 14 doi: 10.1016/j.rdc.2016.12.009. PMID: 28390564
Miner JJ, Kim AH
Rheum Dis Clin North Am 2014 Feb;40(1):51-60. doi: 10.1016/j.rdc.2013.10.003. PMID: 24268009
Poh-Fitzpatrick MB
Dermatol Clin 1987 Jan;5(1):55-61. PMID: 3549079
Olson NY, Lindsley CB
Am J Dis Child 1987 Aug;141(8):908-10. doi: 10.1001/archpedi.1987.04460080094037. PMID: 3307387

Diagnosis

Castro Rodríguez J, Rodríguez Perálvarez ML, Montero-Álvarez JL
Rev Esp Enferm Dig 2024 Jan;116(1):1-6. doi: 10.17235/reed.2023.9781/2023. PMID: 37522317
Atwell K, Michael W, Dubey J, James S, Martonffy A, Anderson S, Rudin N, Schrager S
J Am Board Fam Med 2021 Jul-Aug;34(4):838-848. doi: 10.3122/jabfm.2021.04.200374. PMID: 34312277
Derdulska JM, Rudnicka L, Szykut-Badaczewska A, Mehrholz D, Nowicki RJ, Barańska-Rybak W, Wilkowska A
J Perinat Med 2021 Jun 25;49(5):529-538. Epub 2021 Jan 18 doi: 10.1515/jpm-2020-0543. PMID: 33470961
Izmirly PM, Rivera TL, Buyon JP
Rheum Dis Clin North Am 2007 May;33(2):267-85, vi. doi: 10.1016/j.rdc.2007.02.005. PMID: 17499707
Raiesdana A, Loscalzo J
Ann Med 2006;38(2):95-110. doi: 10.1080/07853890600622143. PMID: 16581695

Therapy

Téllez L, Rodríguez de Santiago E, Albillos A
Rev Esp Cardiol (Engl Ed) 2018 Mar;71(3):192-202. Epub 2018 Feb 9 doi: 10.1016/j.rec.2017.10.052. PMID: 29433942
Lateef A, Petri M
Rheum Dis Clin North Am 2017 May;43(2):215-226. Epub 2017 Mar 14 doi: 10.1016/j.rdc.2016.12.009. PMID: 28390564
Gutiérrez-González LA
Clin Rheumatol 2015 Dec;34(12):2011-9. Epub 2015 Jun 24 doi: 10.1007/s10067-015-2994-y. PMID: 26099604Free PMC Article
Izmirly PM, Rivera TL, Buyon JP
Rheum Dis Clin North Am 2007 May;33(2):267-85, vi. doi: 10.1016/j.rdc.2007.02.005. PMID: 17499707
Raiesdana A, Loscalzo J
Ann Med 2006;38(2):95-110. doi: 10.1080/07853890600622143. PMID: 16581695

Prognosis

Castro Rodríguez J, Rodríguez Perálvarez ML, Montero-Álvarez JL
Rev Esp Enferm Dig 2024 Jan;116(1):1-6. doi: 10.17235/reed.2023.9781/2023. PMID: 37522317
Broberg CS, van Dissel A, Minnier J, Aboulhosn J, Kauling RM, Ginde S, Krieger EV, Rodriguez F 3rd, Gupta T, Shah S, John AS, Cotts T, Kay WA, Kuo M, Dwight C, Woods P, Nicolarsen J, Sarubbi B, Fusco F, Antonova P, Fernandes S, Grewal J, Cramer J, Khairy P, Gallego P, O'Donnell C, Hannah J, Dellborg M, Rodriguez-Monserrate CP, Muhll IV, Pylypchuk S, Magalski A, Han F, Lubert AM, Kay J, Yeung E, Roos-Hesselink J, Baker D, Celermajer DS, Burchill LJ, Wilson WM, Wong J, Kutty S, Opotowsky AR
J Am Coll Cardiol 2022 Sep 6;80(10):951-963. doi: 10.1016/j.jacc.2022.06.020. PMID: 36049802
Opina AD, Franklin WJ
Prog Cardiovasc Dis 2018 Sep-Oct;61(3-4):308-313. Epub 2018 Aug 11 doi: 10.1016/j.pcad.2018.08.003. PMID: 30107184
Saw J, Humphries K, Aymong E, Sedlak T, Prakash R, Starovoytov A, Mancini GBJ
J Am Coll Cardiol 2017 Aug 29;70(9):1148-1158. doi: 10.1016/j.jacc.2017.06.053. PMID: 28838364
Shibata A, Akiyama M
Pediatr Int 2015 Aug;57(4):516-22. Epub 2015 Jul 14 doi: 10.1111/ped.12638. PMID: 25857373

Clinical prediction guides

Murthy AB, Palaniappan V, Karthikeyan K, Anbarasan V
Int J Dermatol 2023 Oct;62(10):1218-1227. Epub 2023 Aug 27 doi: 10.1111/ijd.16817. PMID: 37634201
Uriarte SM, Hajishengallis G
Immunol Rev 2023 Mar;314(1):93-110. Epub 2022 Oct 22 doi: 10.1111/imr.13152. PMID: 36271881Free PMC Article
Hayes SN, Kim ESH, Saw J, Adlam D, Arslanian-Engoren C, Economy KE, Ganesh SK, Gulati R, Lindsay ME, Mieres JH, Naderi S, Shah S, Thaler DE, Tweet MS, Wood MJ; American Heart Association Council on Peripheral Vascular Disease; Council on Clinical Cardiology; Council on Cardiovascular and Stroke Nursing; Council on Genomic and Precision Medicine; and Stroke Council
Circulation 2018 May 8;137(19):e523-e557. Epub 2018 Feb 22 doi: 10.1161/CIR.0000000000000564. PMID: 29472380Free PMC Article
Miner JJ, Kim AH
Rheum Dis Clin North Am 2014 Feb;40(1):51-60. doi: 10.1016/j.rdc.2013.10.003. PMID: 24268009
Izmirly PM, Rivera TL, Buyon JP
Rheum Dis Clin North Am 2007 May;33(2):267-85, vi. doi: 10.1016/j.rdc.2007.02.005. PMID: 17499707

Recent systematic reviews

Zou T, Wei A, Ma H, Lian H, Liu Y, Wang D, Zhao Y, Cui L, Li Z, Zhang R, Wang T
Pediatr Blood Cancer 2023 May;70(5):e30232. Epub 2023 Feb 13 doi: 10.1002/pbc.30232. PMID: 36779547
Elagib MFA, Alqaysi MAH, Almushayt MOS, Nagate RR, Gokhale ST, Chaturvedi S
Technol Health Care 2023;31(3):1051-1064. doi: 10.3233/THC-220581. PMID: 36502352
Zhang H, Shi G, Chen H
Interact Cardiovasc Thorac Surg 2022 Jul 9;35(2) doi: 10.1093/icvts/ivac162. PMID: 35713512Free PMC Article
Kaplan YC, Ozsarfati J, Nickel C, Koren G
Br J Clin Pharmacol 2016 May;81(5):835-48. Epub 2016 Feb 16 doi: 10.1111/bcp.12872. PMID: 26700396Free PMC Article
Chen CC, Lin KL, Chen CL, Wong AM, Huang JL
Lupus 2013 Dec;22(14):1484-8. Epub 2013 Oct 18 doi: 10.1177/0961203313509294. PMID: 24142583

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