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Townes-Brocks syndrome 2(TBS2)

MedGen UID:
1381939
Concept ID:
C4479534
Disease or Syndrome
Synonym: TBS2
 
Gene (location): DACT1 (14q23.1)
 
Monarch Initiative: MONDO:0054582
OMIM®: 617466

Clinical features

From HPO
Vesicoureteral reflux
MedGen UID:
21852
Concept ID:
C0042580
Disease or Syndrome
Vesicoureteral reflux (VUR) is characterized by the reflux of urine from the bladder into the ureters and sometimes into the kidneys. It is a risk factor for urinary tract infections. Primary VUR results from a developmental defect of the ureterovesical junction (UVJ). In combination with intrarenal reflux, the resulting inflammatory reaction may result in renal injury or scarring, also called reflux nephropathy (RN). Extensive renal scarring impairs renal function and may predispose patients to hypertension, proteinuria, and renal insufficiency (summary by Lu et al., 2007). Genetic Heterogeneity of Vesicoureteral Reflux A locus designated VUR1 maps to chromosome 1p13. VUR2 (610878) is caused by mutation in the ROBO2 gene (602431) on chromosome 3p12; VUR3 (613674) is caused by mutation in the SOX17 gene (610928) on chromosome 8q11; VUR4 (614317) maps to chromosome 5; VUR5 (614318) maps to chromosome 13; VUR6 (614319) maps to chromosome 18; VUR7 (615390) maps to chromosome 12; and VUR8 (615963) is caused by mutation in the TNXB gene (600985) on chromosome 6p21. A possible X-linked form has been reported (VURX; 314550).
Hypospadias
MedGen UID:
163083
Concept ID:
C0848558
Congenital Abnormality
Abnormal position of urethral meatus on the ventral penile shaft (underside) characterized by displacement of the urethral meatus from the tip of the glans penis to the ventral surface of the penis, scrotum, or perineum.
Crossed fused renal ectopia
MedGen UID:
372130
Concept ID:
C1835796
Congenital Abnormality
A developmental anomaly in which the kidneys are fused and localized on the same side of the midline. This anomaly is thought to result from disruption of the normal embryologic migration of the kidneys.
Bifid uterus
MedGen UID:
342474
Concept ID:
C1850327
Finding
The presence of a bifid uterus.
Imperforate anus
MedGen UID:
1997
Concept ID:
C0003466
Congenital Abnormality
Congenital absence of the anus, i.e., the opening at the bottom end of the intestinal tract.
Rectovaginal fistula
MedGen UID:
11152
Concept ID:
C0034895
Finding
The presence of a fistula between the vagina and the rectum.
Microtia
MedGen UID:
57535
Concept ID:
C0152423
Congenital Abnormality
Underdevelopment of the external ear.
Overfolded helix
MedGen UID:
325239
Concept ID:
C1837731
Finding
A condition in which the helix is folded over to a greater degree than normal. That is, excessive curling of the helix edge, whereby the free edge is parallel to the plane of the ear.
Cupped ear
MedGen UID:
335186
Concept ID:
C1845447
Congenital Abnormality
Laterally protruding ear that lacks antihelical folding (including absence of inferior and superior crura).
Spina bifida occulta
MedGen UID:
36380
Concept ID:
C0080174
Congenital Abnormality
The closed form of spina bifida with incomplete closure of a vertebral body with intact overlying skin.
Scoliosis
MedGen UID:
11348
Concept ID:
C0036439
Disease or Syndrome
The presence of an abnormal lateral curvature of the spine.

Professional guidelines

PubMed

Bozal-Basterra L, Martín-Ruíz I, Pirone L, Liang Y, Sigurðsson JO, Gonzalez-Santamarta M, Giordano I, Gabicagogeascoa E, de Luca A, Rodríguez JA, Wilkie AOM, Kohlhase J, Eastwood D, Yale C, Olsen JV, Rauchman M, Anderson KV, Sutherland JD, Barrio R
Am J Hum Genet 2018 Feb 1;102(2):249-265. doi: 10.1016/j.ajhg.2017.12.017. PMID: 29395072Free PMC Article

Recent clinical studies

Etiology

Innoceta AM, Olivucci G, Parmeggiani G, Scarano E, Pragliola A, Graziano C
Genes (Basel) 2023 Jan 19;14(2) doi: 10.3390/genes14020258. PMID: 36833185Free PMC Article
Amirhassankhani S, Lloyd MS
J Craniofac Surg 2018 Mar;29(2):372-375. doi: 10.1097/SCS.0000000000004201. PMID: 29239919
Liang Y, Shen D, Cai W
J Pediatr Surg 2008 Feb;43(2):391-3. doi: 10.1016/j.jpedsurg.2007.09.079. PMID: 18280297
Wang RY, Earl DL, Ruder RO, Graham JM Jr
Pediatrics 2001 Aug;108(2):E32. doi: 10.1542/peds.108.2.e32. PMID: 11483842
König R, Schick U, Fuchs S
Eur J Pediatr 1990 Dec;150(2):100-3. doi: 10.1007/BF02072048. PMID: 2279502

Diagnosis

Beaudoux O, Lebre AS, Doco Fenzy M, Spodenkiewicz M, Canivet E, Colosio C, Poirsier C
Am J Med Genet A 2021 Mar;185(3):937-944. Epub 2021 Jan 13 doi: 10.1002/ajmg.a.62050. PMID: 33438842
Amirhassankhani S, Lloyd MS
J Craniofac Surg 2018 Mar;29(2):372-375. doi: 10.1097/SCS.0000000000004201. PMID: 29239919
Lawrence C, Hong-McAtee I, Hall B, Hartsfield J, Rutherford A, Bonilla T, Bay C
Am J Med Genet A 2013 Sep;161A(9):2266-73. Epub 2013 Jul 25 doi: 10.1002/ajmg.a.36104. PMID: 23894113Free PMC Article
Solomon BD
Orphanet J Rare Dis 2011 Aug 16;6:56. doi: 10.1186/1750-1172-6-56. PMID: 21846383Free PMC Article
Powell CM, Michaelis RC
J Med Genet 1999 Feb;36(2):89-93. PMID: 10051003Free PMC Article

Prognosis

Chi Y, Yao Y, Sun F, Zhang W, Zhang Z, Wang Y, Hao W
Ital J Pediatr 2024 Jun 24;50(1):121. doi: 10.1186/s13052-024-01691-0. PMID: 38915054Free PMC Article
Beaudoux O, Lebre AS, Doco Fenzy M, Spodenkiewicz M, Canivet E, Colosio C, Poirsier C
Am J Med Genet A 2021 Mar;185(3):937-944. Epub 2021 Jan 13 doi: 10.1002/ajmg.a.62050. PMID: 33438842
Solomon BD
Orphanet J Rare Dis 2011 Aug 16;6:56. doi: 10.1186/1750-1172-6-56. PMID: 21846383Free PMC Article
Liang Y, Shen D, Cai W
J Pediatr Surg 2008 Feb;43(2):391-3. doi: 10.1016/j.jpedsurg.2007.09.079. PMID: 18280297
Kohlhase J, Taschner PE, Burfeind P, Pasche B, Newman B, Blanck C, Breuning MH, ten Kate LP, Maaswinkel-Mooy P, Mitulla B, Seidel J, Kirkpatrick SJ, Pauli RM, Wargowski DS, Devriendt K, Proesmans W, Gabrielli O, Coppa GV, Wesby-van Swaay E, Trembath RC, Schinzel AA, Reardon W, Seemanova E, Engel W
Am J Hum Genet 1999 Feb;64(2):435-45. doi: 10.1086/302238. PMID: 9973281Free PMC Article

Clinical prediction guides

Chi Y, Yao Y, Sun F, Zhang W, Zhang Z, Wang Y, Hao W
Ital J Pediatr 2024 Jun 24;50(1):121. doi: 10.1186/s13052-024-01691-0. PMID: 38915054Free PMC Article
Liu X, Wang H, Zhang Y, Zhang R, Zhang R, Shi X, Pan F, Qiao D, Xin Q, Liu Z, Zhang Y, Li C, Lang Y, Shao L
Nephrology (Carlton) 2024 Aug;29(8):541-546. Epub 2024 Apr 7 doi: 10.1111/nep.14300. PMID: 38584358
Kohlhase J, Heinrich M, Schubert L, Liebers M, Kispert A, Laccone F, Turnpenny P, Winter RM, Reardon W
Hum Mol Genet 2002 Nov 1;11(23):2979-87. doi: 10.1093/hmg/11.23.2979. PMID: 12393809
Doray B, Langer B, Stoll C
Genet Couns 1999;10(4):359-67. PMID: 10631923
Kohlhase J, Taschner PE, Burfeind P, Pasche B, Newman B, Blanck C, Breuning MH, ten Kate LP, Maaswinkel-Mooy P, Mitulla B, Seidel J, Kirkpatrick SJ, Pauli RM, Wargowski DS, Devriendt K, Proesmans W, Gabrielli O, Coppa GV, Wesby-van Swaay E, Trembath RC, Schinzel AA, Reardon W, Seemanova E, Engel W
Am J Hum Genet 1999 Feb;64(2):435-45. doi: 10.1086/302238. PMID: 9973281Free PMC Article

Recent systematic reviews

Amirhassankhani S, Lloyd MS
J Craniofac Surg 2018 Mar;29(2):372-375. doi: 10.1097/SCS.0000000000004201. PMID: 29239919

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