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Warts-immunodeficiency-lymphedema-anogenital dysplasia syndrome

MedGen UID:
1799992
•Concept ID:
C5568569
•
Disease or Syndrome
Synonyms: Disseminated warts, impaired cell-mediated immunity, primary lymphedema, anogenital dysplasia syndrome; Disseminated warts-impaired cell-mediated immunity-primary lymphedema-anogenital dysplasia syndrome; disseminated warts-impaired cell-mediated immunity-primary lymphedema-anogenital dysplasia syndrome; Warts, immunodeficiency, lymphedema, anogenital dysplasia syndrome; warts-immunodeficiency-lymphedema-anogenital dysplasia syndrome; WILD (warts, immunodeficiency, lymphedema, anogenital dysplasia) syndrome; WILD syndrome
SNOMED CT: WILD syndrome (1186725001); Warts, immunodeficiency, lymphedema, anogenital dysplasia syndrome (1186725001); WILD (warts, immunodeficiency, lymphedema, anogenital dysplasia) syndrome (1186725001); Disseminated warts, impaired cell-mediated immunity, primary lymphedema, anogenital dysplasia syndrome (1186725001)
 
Monarch Initiative: MONDO:0035473
Orphanet: ORPHA568056

Definition

A rare primary lymphedema characterized by extensive multisegmental lymphedema associated with persistent, widespread infections with various genital high and low-risk human papillomaviruses, resulting in multifocal anogenital dysplasia. Laboratory examination shows abnormalities in lymphocyte subsets, in particular CD4+ T-cells. Epidermal nevi and capillary malformations have also been reported. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVWarts-immunodeficiency-lymphedema-anogenital dysplasia syndrome

Professional guidelines

PubMed

Myelodysplastic syndromes: 2023 update on diagnosis, risk-stratification, and management.
Garcia-Manero G
Am J Hematol 2023 Aug;98(8):1307-1325. Epub 2023 Jun 8 doi: 10.1002/ajh.26984. PMID: 37288607
Diagnosis and Treatment of Myelodysplastic Syndromes: A Review.
Sekeres MA, Taylor J
JAMA 2022 Sep 6;328(9):872-880. doi: 10.1001/jama.2022.14578. PMID: 36066514
Chronic myelomonocytic leukemia: 2022 update on diagnosis, risk stratification, and management.
Patnaik MM, Tefferi A
Am J Hematol 2022 Mar 1;97(3):352-372. Epub 2022 Jan 27 doi: 10.1002/ajh.26455. PMID: 34985762

Recent clinical studies

Etiology

Ectodermal dysplasias: Classification and organization by phenotype, genotype and molecular pathway.
Wright JT, Fete M, Schneider H, Zinser M, Koster MI, Clarke AJ, Hadj-Rabia S, Tadini G, Pagnan N, Visinoni AF, Bergendal B, Abbott B, Fete T, Stanford C, Butcher C, D'Souza RN, Sybert VP, Morasso MI
Am J Med Genet A 2019 Mar;179(3):442-447. Epub 2019 Jan 31 doi: 10.1002/ajmg.a.61045. PMID: 30703280Free PMC Article
The current approach to the diagnosis of myelodysplastic syndromes(☆).
Weinberg OK, Hasserjian RP
Semin Hematol 2019 Jan;56(1):15-21. Epub 2018 Jun 23 doi: 10.1053/j.seminhematol.2018.05.015. PMID: 30573039
Genetic short stature.
Grunauer M, Jorge AAL
Growth Horm IGF Res 2018 Feb;38:29-33. Epub 2017 Dec 6 doi: 10.1016/j.ghir.2017.12.003. PMID: 29249624
Ciliary disorder of the skeleton.
Huber C, Cormier-Daire V
Am J Med Genet C Semin Med Genet 2012 Aug 15;160C(3):165-74. Epub 2012 Jul 12 doi: 10.1002/ajmg.c.31336. PMID: 22791528
Fronto-facio-nasal dysplasia.
Suthers G, David D, Clark B
Clin Dysmorphol 1997 Jul;6(3):245-9. doi: 10.1097/00019605-199707000-00008. PMID: 9220195

Diagnosis

Hereditary Syndromes of Sudden Cardiac Death.
Berberian JG
Emerg Med Clin North Am 2022 Nov;40(4):651-662. Epub 2022 Oct 7 doi: 10.1016/j.emc.2022.06.005. PMID: 36396213
Septo-optic dysplasia.
Sataite I, Cudlip S, Jayamohan J, Ganau M
Handb Clin Neurol 2021;181:51-64. doi: 10.1016/B978-0-12-820683-6.00005-1. PMID: 34238479
Genetics of patella hypoplasia/agenesis.
Vanlerberghe C, Boutry N, Petit F
Clin Genet 2018 Jul;94(1):43-53. Epub 2018 Apr 11 doi: 10.1111/cge.13209. PMID: 29322497
Syndromes with supernumerary teeth.
Lubinsky M, Kantaputra PN
Am J Med Genet A 2016 Oct;170(10):2611-6. Epub 2016 Jun 2 doi: 10.1002/ajmg.a.37763. PMID: 27250821
Mazabraud syndrome.
Dreizin D, Glen C, Jose J
Am J Orthop (Belle Mead NJ) 2012 Jul;41(7):332-5. PMID: 22893885

Therapy

Myelodysplastic syndromes: 2023 update on diagnosis, risk-stratification, and management.
Garcia-Manero G
Am J Hematol 2023 Aug;98(8):1307-1325. Epub 2023 Jun 8 doi: 10.1002/ajh.26984. PMID: 37288607
Molecular Targeted Therapy and Immunotherapy for Myelodysplastic Syndrome.
Lee P, Yim R, Yung Y, Chu HT, Yip PK, Gill H
Int J Mol Sci 2021 Sep 23;22(19) doi: 10.3390/ijms221910232. PMID: 34638574Free PMC Article
Reactions Related to CAR-T Cell Therapy.
Miao L, Zhang Z, Ren Z, Li Y
Front Immunol 2021;12:663201. Epub 2021 Apr 28 doi: 10.3389/fimmu.2021.663201. PMID: 33995389Free PMC Article
Non-Invasive Ventilation in Neonatology.
Behnke J, Lemyre B, Czernik C, Zimmer KP, Ehrhardt H, Waitz M
Dtsch Arztebl Int 2019 Mar 8;116(11):177-183. doi: 10.3238/arztebl.2019.0177. PMID: 31014448Free PMC Article
MDS overlap disorders and diagnostic boundaries.
Tanaka TN, Bejar R
Blood 2019 Mar 7;133(10):1086-1095. Epub 2019 Jan 22 doi: 10.1182/blood-2018-10-844670. PMID: 30670443

Prognosis

Caudal duplication syndrome: 10-year experiences with a comprehensive literature review.
Wang K, Yang L, Peng C, Pang W, Wang Z, Zhang D, Wu D, Chen Y
Pediatr Surg Int 2022 Sep;38(9):1283-1289. Epub 2022 Jul 3 doi: 10.1007/s00383-022-05159-2. PMID: 35780394
Yunis-Varon Syndrome.
Siddique AW, Ahmed Z, Haider A, Khalid H, Karim T
J Ayub Med Coll Abbottabad 2019 Apr-Jun;31(2):290-292. PMID: 31094135
Autoimmune manifestations associated with myelodysplastic syndromes.
Grignano E, Jachiet V, Fenaux P, Ades L, Fain O, Mekinian A
Ann Hematol 2018 Nov;97(11):2015-2023. Epub 2018 Aug 8 doi: 10.1007/s00277-018-3472-9. PMID: 30091023
Glenoid Dysplasia.
Abboud JA, Bateman DK, Barlow J
J Am Acad Orthop Surg 2016 May;24(5):327-36. doi: 10.5435/JAAOS-D-15-00032. PMID: 27055054
Controversies surrounding Jarcho-Levin syndrome.
Cornier AS, Ramirez N, Carlo S, Reiss A
Curr Opin Pediatr 2003 Dec;15(6):614-20. doi: 10.1097/00008480-200312000-00012. PMID: 14631208

Clinical prediction guides

Lung Ultrasound Score Progress in Neonatal Respiratory Distress Syndrome.
Raimondi F, Migliaro F, Corsini I, Meneghin F, Dolce P, Pierri L, Perri A, Aversa S, Nobile S, Lama S, Varano S, Savoia M, Gatto S, Leonardi V, Capasso L, Carnielli VP, Mosca F, Dani C, Vento G, Lista G
Pediatrics 2021 Apr;147(4) Epub 2021 Mar 9 doi: 10.1542/peds.2020-030528. PMID: 33688032
Clinical Characteristics and Long-Term Outcomes of Midaortic Syndrome.
Patel RS, Nguyen S, Lee MT, Price MD, Krause H, Truong VTT, Sandhu HK, Charlton-Ouw KM, LeMaire SA, Coselli JS, Prakash SK
Ann Vasc Surg 2020 Jul;66:318-325. Epub 2020 Jan 7 doi: 10.1016/j.avsg.2019.12.039. PMID: 31923594
Genetics of patella hypoplasia/agenesis.
Vanlerberghe C, Boutry N, Petit F
Clin Genet 2018 Jul;94(1):43-53. Epub 2018 Apr 11 doi: 10.1111/cge.13209. PMID: 29322497
Advances in Skeletal Dysplasia Genetics.
Geister KA, Camper SA
Annu Rev Genomics Hum Genet 2015;16:199-227. Epub 2015 Apr 22 doi: 10.1146/annurev-genom-090314-045904. PMID: 25939055Free PMC Article
Ciliary disorder of the skeleton.
Huber C, Cormier-Daire V
Am J Med Genet C Semin Med Genet 2012 Aug 15;160C(3):165-74. Epub 2012 Jul 12 doi: 10.1002/ajmg.c.31336. PMID: 22791528

Recent systematic reviews

Orthodontic and dentofacial orthopedic treatments in patients with ectodermal dysplasia: a systematic review.
Cerezo-Cayuelas M, Pérez-Silva A, Serna-Muñoz C, Vicente A, Martínez-Beneyto Y, Cabello-Malagón I, Ortiz-Ruiz AJ
Orphanet J Rare Dis 2022 Oct 17;17(1):376. doi: 10.1186/s13023-022-02533-0. PMID: 36253866Free PMC Article
Selecting End Points for Disease-Modification Trials in Inflammatory Bowel Disease: the SPIRIT Consensus From the IOIBD.
Le Berre C, Peyrin-Biroulet L; SPIRIT-IOIBD study group
Gastroenterology 2021 Apr;160(5):1452-1460.e21. Epub 2021 Jan 6 doi: 10.1053/j.gastro.2020.10.065. PMID: 33421515
Continuous positive airway pressure (CPAP) for respiratory distress in preterm infants.
Ho JJ, Subramaniam P, Davis PG
Cochrane Database Syst Rev 2020 Oct 15;10(10):CD002271. doi: 10.1002/14651858.CD002271.pub3. PMID: 33058208Free PMC Article
Surgery for epilepsy.
West S, Nevitt SJ, Cotton J, Gandhi S, Weston J, Sudan A, Ramirez R, Newton R
Cochrane Database Syst Rev 2019 Jun 25;6(6):CD010541. doi: 10.1002/14651858.CD010541.pub3. PMID: 31237346Free PMC Article
Swaddling: a systematic review.
van Sleuwen BE, Engelberts AC, Boere-Boonekamp MM, Kuis W, Schulpen TW, L'Hoir MP
Pediatrics 2007 Oct;120(4):e1097-106. doi: 10.1542/peds.2006-2083. PMID: 17908730

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