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Fibromatosis, gingival, 5(GINGF5; HGF5)

MedGen UID:
1624392
Concept ID:
C4539942
Disease or Syndrome
Synonym: FIBROMATOSIS, GINGIVAL, HEREDITARY, 5
 
Gene (location): REST (4q12)
 
Monarch Initiative: MONDO:0033493
OMIM®: 617626

Definition

Gingival fibromatosis-5 (GINGF5) is an autosomal dominant benign overgrowth disorder characterized by slowly progressive fibrous enlargement of the keratinized gingival tissues. Affected individuals may have diastema, malposition of the teeth, and prolonged retention of primary teeth. Onset is in the first decade. Treatment by surgical resection is generally followed by regrowth of the gingival tissues (summary by Pehlivan et al., 2009). For a general phenotypic description and a discussion of genetic heterogeneity of hereditary gingival fibromatosis, see GINGF1 (135300). [from OMIM]

Clinical features

From HPO
Gingival fibromatosis
MedGen UID:
42017
Concept ID:
C0016049
Finding
The presence of fibrosis of the gingiva.
Abnormal sternum morphology
MedGen UID:
349830
Concept ID:
C1860493
Anatomical Abnormality
An anomaly of the sternum, also known as the breastbone.

Professional guidelines

PubMed

Casas-Alba D, Martínez-Monseny A, Pino-Ramírez RM, Alsina L, Castejón E, Navarro-Vilarrubí S, Pérez-Dueñas B, Serrano M, Palau F, García-Alix A
Hum Mutat 2018 Dec;39(12):1752-1763. Epub 2018 Sep 17 doi: 10.1002/humu.23638. PMID: 30176098
Angelopoulos AP
Dent J 1975 May;41(5):275-7, 283. PMID: 1055114

Recent clinical studies

Etiology

Gawron K, Łazarz-Bartyzel K, Kowalska A, Bereta G, Nowakowska Z, Plakwicz P, Potempa J, Fertala A, Chomyszyn-Gajewska M
Connect Tissue Res 2019 Jan;60(1):29-39. Epub 2018 Sep 20 doi: 10.1080/03008207.2018.1517758. PMID: 30231645
Seki K, Sato S, Asano Y, Akutagawa H, Ito K
Quintessence Int 2010 Jul-Aug;41(7):543-5. PMID: 20614039
Kelekis-Cholakis A, Wiltshire WA, Birek C
J Can Dent Assoc 2002 May;68(5):290-4. PMID: 12019039
Wright HJ, Chapple IL, Matthews JB
J Oral Pathol Med 2001 May;30(5):281-9. doi: 10.1034/j.1600-0714.2001.300505.x. PMID: 11334464
Bittencourt LP, Campos V, Moliterno LF, Ribeiro DP, Sampaio RK
Quintessence Int 2000 Jun;31(6):415-8. PMID: 11203959

Diagnosis

Boutiou E, Ziogas IA, Giannis D, Doufexi AE
Clin Oral Investig 2021 Jun;25(6):3599-3607. Epub 2020 Nov 13 doi: 10.1007/s00784-020-03682-x. PMID: 33188467
Resende EP, Xavier MT, Matos S, Antunes AC, Silva HC
Spec Care Dentist 2020 May;40(3):320-328. Epub 2020 May 15 doi: 10.1111/scd.12458. PMID: 32413193
Rahvar M, Teng J, Kim J
Am J Dermatopathol 2016 May;38(5):e60-3. doi: 10.1097/DAD.0000000000000467. PMID: 26885603
Coletta RD, Graner E
J Periodontol 2006 May;77(5):753-64. doi: 10.1902/jop.2006.050379. PMID: 16671866
Angelopoulos AP
Dent J 1975 May;41(5):275-7, 283. PMID: 1055114

Therapy

Straka M, Danisovic L, Bzduch V, Polak S, Varga I
Neuro Endocrinol Lett 2016 Oct;37(5):353-360. PMID: 28171221
Wright HJ, Chapple IL, Matthews JB
J Oral Pathol Med 2001 May;30(5):281-9. doi: 10.1034/j.1600-0714.2001.300505.x. PMID: 11334464
Payne JB
Dent Teamwork 1992 Sep-Oct;5(5):22-4. PMID: 1291280
Cohen MM Sr
ASDC J Dent Child 1977 Sep-Oct;44(5):396-400. PMID: 332732
Angelopoulos AP
Dent J 1975 May;41(5):275-7, 283. PMID: 1055114

Prognosis

Casas-Alba D, Martínez-Monseny A, Pino-Ramírez RM, Alsina L, Castejón E, Navarro-Vilarrubí S, Pérez-Dueñas B, Serrano M, Palau F, García-Alix A
Hum Mutat 2018 Dec;39(12):1752-1763. Epub 2018 Sep 17 doi: 10.1002/humu.23638. PMID: 30176098
Rahvar M, Teng J, Kim J
Am J Dermatopathol 2016 May;38(5):e60-3. doi: 10.1097/DAD.0000000000000467. PMID: 26885603
Seki K, Sato S, Asano Y, Akutagawa H, Ito K
Quintessence Int 2010 Jul-Aug;41(7):543-5. PMID: 20614039
Kelekis-Cholakis A, Wiltshire WA, Birek C
J Can Dent Assoc 2002 May;68(5):290-4. PMID: 12019039
Bittencourt LP, Campos V, Moliterno LF, Ribeiro DP, Sampaio RK
Quintessence Int 2000 Jun;31(6):415-8. PMID: 11203959

Clinical prediction guides

Strzelec K, Dziedzic A, Łazarz-Bartyzel K, Grabiec AM, Gutmajster E, Kaczmarzyk T, Plakwicz P, Gawron K
Orphanet J Rare Dis 2021 Nov 24;16(1):492. doi: 10.1186/s13023-021-02104-9. PMID: 34819125Free PMC Article
Gawron K, Łazarz-Bartyzel K, Kowalska A, Bereta G, Nowakowska Z, Plakwicz P, Potempa J, Fertala A, Chomyszyn-Gajewska M
Connect Tissue Res 2019 Jan;60(1):29-39. Epub 2018 Sep 20 doi: 10.1080/03008207.2018.1517758. PMID: 30231645
Ramnarayan BK, Sowmya K, Rema J
Pediatr Dent 2011 Sep-Oct;33(5):431-6. PMID: 22104713
Xiao S, Bu L, Zhu L, Zheng G, Yang M, Qian M, Hu L, Liu J, Zhao G, Kong X
Genomics 2001 Jun 1;74(2):180-5. doi: 10.1006/geno.2001.6542. PMID: 11386754
Koch P, Wettstein A, Knauber J, Zaun H
Acta Derm Venereol 1992 Sep;72(5):376-9. PMID: 1361289

Recent systematic reviews

Boutiou E, Ziogas IA, Giannis D, Doufexi AE
Clin Oral Investig 2021 Jun;25(6):3599-3607. Epub 2020 Nov 13 doi: 10.1007/s00784-020-03682-x. PMID: 33188467
Coletta RD, Graner E
J Periodontol 2006 May;77(5):753-64. doi: 10.1902/jop.2006.050379. PMID: 16671866

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