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TRAF7-associated heart defect-digital anomalies-facial dysmorphism-motor and speech delay syndrome

MedGen UID:
1809140
Concept ID:
C5681633
Disease or Syndrome
Synonyms: TRAF7-associated heart defect, digital anomalies, facial dysmorphism, motor and speech delay syndrome; Tumor necrosis factor receptor associated factor 7-associated heart defect, digital anomalies, facial dysmorphism, motor and speech delay syndrome
SNOMED CT: Tumor necrosis factor receptor associated factor 7-associated heart defect, digital anomalies, facial dysmorphism, motor and speech delay syndrome (1208998007); TRAF7-associated heart defect, digital anomalies, facial dysmorphism, motor and speech delay syndrome (1208998007)
 
Monarch Initiative: MONDO:0035661
Orphanet: ORPHA592570

Definition

A rare multiple congenital anomalies/dysmorphic syndrome with characteristics of global developmental delay or regression, variable congenital heart defects (such as patent ductus arteriosus, atrial or ventricular septal defects, and double outlet right ventricle, among others), dysmorphic features (including ptosis, epicanthal folds, abnormally set/dysplastic ears, low hairline or excess nuchal skin, wide-spaced/inverted nipples, umbilical hernia or diastasis recti, and digital anomalies). Additional variable manifestations are hyper or hypotonia, seizures, hearing loss, cortical blindness and optic atrophy. Brain imaging may show cerebral and cerebellar atrophy and hydrocephalus. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • TRAF7-associated heart defect-digital anomalies-facial dysmorphism-motor and speech delay syndrome

Professional guidelines

PubMed

Shi P, Wang C, Zheng Y, Kong X
BMC Med Genomics 2021 Mar 23;14(1):88. doi: 10.1186/s12920-021-00940-z. PMID: 33757501Free PMC Article
Barry JC, Crowley TB, Jyonouchi S, Heimall J, Zackai EH, Sullivan KE, McDonald-McGinn DM
J Clin Immunol 2017 Jul;37(5):476-485. Epub 2017 May 24 doi: 10.1007/s10875-017-0403-9. PMID: 28540525Free PMC Article
Adam MP, Schelley S, Gallagher R, Brady AN, Barr K, Blumberg B, Shieh JT, Graham J, Slavotinek A, Martin M, Keppler-Noreuil K, Storm AL, Hudgins L
Am J Med Genet A 2006 Dec 15;140(24):2730-41. doi: 10.1002/ajmg.a.31530. PMID: 17103451

Recent clinical studies

Etiology

Haeuser E, Serfes AL, Cork MA, Yang M, Abbastabar H, Abhilash ES, Adabi M, Adebayo OM, Adekanmbi V, Adeyinka DA, Afzal S, Ahinkorah BO, Ahmadi K, Ahmed MB, Akalu Y, Akinyemi RO, Akunna CJ, Alahdab F, Alanezi FM, Alanzi TM, Alene KA, Alhassan RK, Alipour V, Almasi-Hashiani A, Alvis-Guzman N, Ameyaw EK, Amini S, Amugsi DA, Ancuceanu R, Anvari D, Appiah SCY, Arabloo J, Aremu O, Asemahagn MA, Jafarabadi MA, Awedew AF, Quintanilla BPA, Ayanore MA, Aynalem YA, Azari S, Azene ZN, Darshan BB, Babalola TK, Baig AA, Banach M, Bärnighausen TW, Bell AW, Bhagavathula AS, Bhardwaj N, Bhardwaj P, Bhattacharyya K, Bijani A, Bitew ZW, Bohlouli S, Bolarinwa OA, Boloor A, Bozicevic I, Butt ZA, Cárdenas R, Carvalho F, Charan J, Chattu VK, Chowdhury MAK, Chu DT, Cowden RG, Dahlawi SMA, Damiani G, Darteh EKM, Darwesh AM, das Neves J, Weaver ND, De Leo D, De Neve JW, Deribe K, Deuba K, Dharmaratne S, Dianatinasab M, Diaz D, Didarloo A, Djalalinia S, Dorostkar F, Dubljanin E, Duko B, El Tantawi M, El-Jaafary SI, Eshrati B, Eskandarieh S, Eyawo O, Ezeonwumelu IJ, Ezzikouri S, Farzadfar F, Fattahi N, Fauk NK, Fernandes E, Filip I, Fischer F, Foigt NA, Foroutan M, Fukumoto T, Gad MM, Gaidhane AM, Gebregiorgis BG, Gebremedhin KB, Getacher L, Ghadiri K, Ghashghaee A, Golechha M, Gubari MIM, Gugnani HC, Guimarães RA, Haider MR, Haj-Mirzaian A, Hamidi S, Hashi A, Hassanipour S, Hassankhani H, Hayat K, Herteliu C, Ho HC, Holla R, Hosseini M, Hosseinzadeh M, Hwang BF, Ibitoye SE, Ilesanmi OS, Ilic IM, Ilic MD, Islam RM, Iwu CCD, Jakovljevic M, Jha RP, Ji JS, Johnson KB, Joseph N, Joshua V, Joukar F, Jozwiak JJ, Kalankesh LR, Kalhor R, Kamyari N, Kanchan T, Matin BK, Karimi SE, Kayode GA, Karyani AK, Keramati M, Khan EA, Khan G, Khan MN, Khatab K, Khubchandani J, Kim YJ, Kisa A, Kisa S, Kopec JA, Kosen S, Laxminarayana SLK, Koyanagi A, Krishan K, Defo BK, Kugbey N, Kulkarni V, Kumar M, Kumar N, Kusuma D, La Vecchia C, Lal DK, Landires I, Larson HJ, Lasrado S, Lee PH, Li S, Liu X, Maleki A, Malik P, Mansournia MA, Martins-Melo FR, Mendoza W, Menezes RG, Mengesha EW, Meretoja TJ, Mestrovic T, Mirica A, Moazen B, Mohamad O, Mohammad Y, Mohammadian-Hafshejani A, Mohammadpourhodki R, Mohammed S, Mohammed S, Mokdad AH, Moradi M, Moraga P, Mubarik S, Mulu GBB, Mwanri L, Nagarajan AJ, Naimzada MD, Naveed M, Nazari J, Ndejjo R, Negoi I, Ngalesoni FN, Nguefack-Tsague G, Ngunjiri JW, Nguyen CT, Nguyen HLT, Nnaji CA, Noubiap JJ, Nuñez-Samudio V, Nwatah VE, Oancea B, Odukoya OO, Olagunju AT, Olakunde BO, Olusanya BO, Olusanya JO, Bali AO, Onwujekwe OE, Orisakwe OE, Otstavnov N, Otstavnov SS, Owolabi MO, Mahesh PA, Padubidri JR, Pana A, Pandey A, Pandi-Perumal SR, Kan FP, Patton GC, Pawar S, Peprah EK, Postma MJ, Preotescu L, Syed ZQ, Rabiee N, Radfar A, Rafiei A, Rahim F, Rahimi-Movaghar V, Rahmani AM, Ramezanzadeh K, Rana J, Ranabhat CL, Rao SJ, Rawaf DL, Rawaf S, Rawassizadeh R, Regassa LD, Rezaei N, Rezapour A, Riaz MA, Ribeiro AI, Ross JM, Rubagotti E, Rumisha SF, Rwegerera GM, Moghaddam SS, Sagar R, Sahiledengle B, Sahu M, Salem MR, Kafil HS, Samy AM, Sartorius B, Sathian B, Seidu AA, Shaheen AA, Shaikh MA, Shamsizadeh M, Shiferaw WS, Shin JI, Shrestha R, Singh JA, Skryabin VY, Skryabina AA, Soltani S, Sufiyan MB, Tabuchi T, Tadesse EG, Taveira N, Tesfay FH, Thapar R, Tovani-Palone MR, Tsegaye GW, Umeokonkwo CD, Unnikrishnan B, Villafañe JH, Violante FS, Vo B, Vu GT, Wado YD, Waheed Y, Wamai RG, Wang Y, Ward P, Wickramasinghe ND, Wilson K, Yaya S, Yip P, Yonemoto N, Yu C, Zastrozhin MS, Zhang Y, Zhang ZJ, Hay SI, Dwyer-Lindgren L; Local Burden of Disease sub-Saharan Africa HIV Prevalence Collaborators
BMC Med 2022 Dec 19;20(1):488. doi: 10.1186/s12916-022-02639-z. PMID: 36529768Free PMC Article
Noubiap JJ, Nansseu JR, Lontchi-Yimagou E, Nkeck JR, Nyaga UF, Ngouo AT, Tounouga DN, Tianyi FL, Foka AJ, Ndoadoumgue AL, Bigna JJ
Lancet Child Adolesc Health 2022 Mar;6(3):158-170. Epub 2022 Jan 17 doi: 10.1016/S2352-4642(21)00374-6. PMID: 35051409
Rots D, Chater-Diehl E, Dingemans AJM, Goodman SJ, Siu MT, Cytrynbaum C, Choufani S, Hoang N, Walker S, Awamleh Z, Charkow J, Meyn S, Pfundt R, Rinne T, Gardeitchik T, de Vries BBA, Deden AC, Leenders E, Kwint M, Stumpel CTRM, Stevens SJC, Vermeulen JR, van Harssel JVT, Bosch DGM, van Gassen KLI, van Binsbergen E, de Geus CM, Brackel H, Hempel M, Lessel D, Denecke J, Slavotinek A, Strober J, Crunk A, Folk L, Wentzensen IM, Yang H, Zou F, Millan F, Person R, Xie Y, Liu S, Ousager LB, Larsen M, Schultz-Rogers L, Morava E, Klee EW, Berry IR, Campbell J, Lindstrom K, Pruniski B, Neumeyer AM, Radley JA, Phornphutkul C, Schmidt B, Wilson WG, Õunap K, Reinson K, Pajusalu S, van Haeringen A, Ruivenkamp C, Cuperus R, Santos-Simarro F, Palomares-Bralo M, Pacio-Míguez M, Ritter A, Bhoj E, Tønne E, Tveten K, Cappuccio G, Brunetti-Pierri N, Rowe L, Bunn J, Saenz M, Platzer K, Mertens M, Caluseriu O, Nowaczyk MJM, Cohn RD, Kannu P, Alkhunaizi E, Chitayat D, Scherer SW, Brunner HG, Vissers LELM, Kleefstra T, Koolen DA, Weksberg R
Am J Hum Genet 2021 Jun 3;108(6):1053-1068. Epub 2021 Apr 27 doi: 10.1016/j.ajhg.2021.04.008. PMID: 33909990Free PMC Article
Van Dijck A, Vulto-van Silfhout AT, Cappuyns E, van der Werf IM, Mancini GM, Tzschach A, Bernier R, Gozes I, Eichler EE, Romano C, Lindstrand A, Nordgren A; ADNP Consortium, Kvarnung M, Kleefstra T, de Vries BBA, Küry S, Rosenfeld JA, Meuwissen ME, Vandeweyer G, Kooy RF
Biol Psychiatry 2019 Feb 15;85(4):287-297. Epub 2018 Mar 15 doi: 10.1016/j.biopsych.2018.02.1173. PMID: 29724491Free PMC Article
Benders MJ, Groenendaal F, De Vries LS
Semin Fetal Neonatal Med 2009 Oct;14(5):272-7. Epub 2009 Aug 4 doi: 10.1016/j.siny.2009.07.002. PMID: 19656748

Diagnosis

Szczawińska-Popłonyk A, Schwartzmann E, Chmara Z, Głukowska A, Krysa T, Majchrzycki M, Olejnicki M, Ostrowska P, Babik J
Int J Mol Sci 2023 May 5;24(9) doi: 10.3390/ijms24098317. PMID: 37176024Free PMC Article
Noubiap JJ, Nansseu JR, Lontchi-Yimagou E, Nkeck JR, Nyaga UF, Ngouo AT, Tounouga DN, Tianyi FL, Foka AJ, Ndoadoumgue AL, Bigna JJ
Lancet Child Adolesc Health 2022 Mar;6(3):158-170. Epub 2022 Jan 17 doi: 10.1016/S2352-4642(21)00374-6. PMID: 35051409
Paul R
Handb Clin Neurol 2020;174:21-35. doi: 10.1016/B978-0-444-64148-9.00002-8. PMID: 32977879
Cody JD, Sebold C, Heard P, Carter E, Soileau B, Hasi-Zogaj M, Hill A, Rupert D, Perry B, O'Donnell L, Gelfond J, Lancaster J, Fox PT, Hale DE
Am J Med Genet C Semin Med Genet 2015 Sep;169(3):265-80. Epub 2015 Aug 3 doi: 10.1002/ajmg.c.31446. PMID: 26235940
Benders MJ, Groenendaal F, De Vries LS
Semin Fetal Neonatal Med 2009 Oct;14(5):272-7. Epub 2009 Aug 4 doi: 10.1016/j.siny.2009.07.002. PMID: 19656748

Therapy

O'Byrne ML, Baxelbaum K, Tam V, Griffis H, Pennington ML, Hagerty A, Naim MY, Nicolson SC, Shillingford AJ, Sutherland TN, Hampton LE, Gebregiorgis NG, Nguyen T, Ramos E, Rossano JW
J Am Coll Cardiol 2024 Sep 10;84(11):1010-1021. doi: 10.1016/j.jacc.2024.06.033. PMID: 39232628
Gaynor JW, Moldenhauer JS, Zullo EE, Burnham NB, Gerdes M, Bernbaum JC, D'Agostino JA, Linn RL, Klepczynski B, Randazzo I, Gionet G, Choi GH, Karaj A, Russell WW, Zackai EH, Johnson MP, Gebb JS, Soni S, DeBari SE, Szwast AL, Ahrens-Nicklas RC, Drivas TG, Jacobwitz M, Licht DJ, Vossough A, Nicolson SC, Spray TL, Rychik J, Putt ME
JAMA Netw Open 2024 May 1;7(5):e2412291. doi: 10.1001/jamanetworkopen.2024.12291. PMID: 38805228Free PMC Article
Namazi N, Djalalinia S, Mahdavi-Gorabi A, Asayesh H, Mansourian M, Noroozi M, Qorbani M
Eat Weight Disord 2020 Feb;25(1):151-161. Epub 2018 Jul 3 doi: 10.1007/s40519-018-0534-x. PMID: 29971623
Homma TK, Freire BL, Honjo R, Dauber A, Funari MFA, Lerario AM, Albuquerque EVA, Vasques GA, Bertola DR, Kim CA, Malaquias AC, Jorge AAL
Horm Res Paediatr 2019;92(2):115-123. Epub 2019 Nov 12 doi: 10.1159/000503782. PMID: 31715605
Velayutham P, Irace AL, Kawai K, Dodrill P, Perez J, Londahl M, Mundy L, Dombrowski ND, Rahbar R
Laryngoscope 2018 Aug;128(8):1952-1957. Epub 2017 Dec 27 doi: 10.1002/lary.27070. PMID: 29280496

Prognosis

Nguyen TQ, Kerley CI, Key AP, Maxwell-Horn AC, Wells QS, Neul JL, Cutting LE, Landman BA
J Intellect Disabil Res 2024 May;68(5):491-511. Epub 2024 Feb 1 doi: 10.1111/jir.13124. PMID: 38303157Free PMC Article
Klaniewska M, Bolanowska-Tyszko A, Latos-Bielenska A, Jezela-Stanek A, Szczaluba K, Krajewska-Walasek M, Ciara E, Pelc M, Jurkiewicz D, Stawinski P, Zubkiewicz-Kucharska A, Rydzanicz M, Ploski R, Smigiel R
Mol Genet Genomic Med 2023 Dec;11(12):e2265. Epub 2023 Sep 1 doi: 10.1002/mgg3.2265. PMID: 37658610Free PMC Article
Cody JD, Sebold C, Heard P, Carter E, Soileau B, Hasi-Zogaj M, Hill A, Rupert D, Perry B, O'Donnell L, Gelfond J, Lancaster J, Fox PT, Hale DE
Am J Med Genet C Semin Med Genet 2015 Sep;169(3):265-80. Epub 2015 Aug 3 doi: 10.1002/ajmg.c.31446. PMID: 26235940
Swillen A, McDonald-McGinn D
Am J Med Genet C Semin Med Genet 2015 Jun;169(2):172-81. Epub 2015 May 18 doi: 10.1002/ajmg.c.31435. PMID: 25989227Free PMC Article
Benders MJ, Groenendaal F, De Vries LS
Semin Fetal Neonatal Med 2009 Oct;14(5):272-7. Epub 2009 Aug 4 doi: 10.1016/j.siny.2009.07.002. PMID: 19656748

Clinical prediction guides

Gaynor JW, Moldenhauer JS, Zullo EE, Burnham NB, Gerdes M, Bernbaum JC, D'Agostino JA, Linn RL, Klepczynski B, Randazzo I, Gionet G, Choi GH, Karaj A, Russell WW, Zackai EH, Johnson MP, Gebb JS, Soni S, DeBari SE, Szwast AL, Ahrens-Nicklas RC, Drivas TG, Jacobwitz M, Licht DJ, Vossough A, Nicolson SC, Spray TL, Rychik J, Putt ME
JAMA Netw Open 2024 May 1;7(5):e2412291. doi: 10.1001/jamanetworkopen.2024.12291. PMID: 38805228Free PMC Article
Giacomini T, Cordani R, Bagnasco I, Vercellino F, Giordano L, Milito G, Ferrero GB, Mandrile G, Scala M, Meli M, Falsaperla R, Luria G, De Grandis E, Canale E, Amadori E, Striano P, Nobili L, Siri L
Neuropediatrics 2023 Dec;54(6):433-438. Epub 2023 Oct 6 doi: 10.1055/s-0043-1775977. PMID: 37802085
Bishop A, Cartwright JE, Whitley GS
Hum Reprod Update 2021 Oct 18;27(6):1098-1114. doi: 10.1093/humupd/dmab028. PMID: 34432025Free PMC Article
Lim JA, Lee ST, Moon J, Jun JS, Kim TJ, Shin YW, Abdullah S, Byun JI, Sunwoo JS, Kim KT, Yang TW, Lee WJ, Moon HJ, Kim DW, Lim BC, Cho YW, Yang TH, Kim HJ, Kim YS, Koo YS, Park B, Jung KH, Kim M, Park KI, Jung KY, Chu K, Lee SK
Ann Neurol 2019 Mar;85(3):352-358. Epub 2019 Feb 10 doi: 10.1002/ana.25421. PMID: 30675918
Tenorio J, Arias P, Martínez-Glez V, Santos F, García-Miñaur S, Nevado J, Lapunzina P
Orphanet J Rare Dis 2014 Sep 20;9:138. doi: 10.1186/s13023-014-0138-0. PMID: 25238977Free PMC Article

Recent systematic reviews

Noubiap JJ, Nansseu JR, Lontchi-Yimagou E, Nkeck JR, Nyaga UF, Ngouo AT, Tounouga DN, Tianyi FL, Foka AJ, Ndoadoumgue AL, Bigna JJ
Lancet Child Adolesc Health 2022 Mar;6(3):158-170. Epub 2022 Jan 17 doi: 10.1016/S2352-4642(21)00374-6. PMID: 35051409
Namazi N, Djalalinia S, Mahdavi-Gorabi A, Asayesh H, Mansourian M, Noroozi M, Qorbani M
Eat Weight Disord 2020 Feb;25(1):151-161. Epub 2018 Jul 3 doi: 10.1007/s40519-018-0534-x. PMID: 29971623
Bass JL, Corwin M, Gozal D, Moore C, Nishida H, Parker S, Schonwald A, Wilker RE, Stehle S, Kinane TB
Pediatrics 2004 Sep;114(3):805-16. doi: 10.1542/peds.2004-0227. PMID: 15342857

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