Hereditary Gingival Fibrosis Study of a Family

Savara, Bhim Sen; Suher, Theodore; Everett, Frank G.; Burns, Albert G.

doi:10.1902/jop.1954.25.1.12

Cited by 23 publications

(6 citation statements)

References 2 publications

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“…In these cases the mode of inheritance seems to be autosomal recessive, as in the cases reported by Jorgensen and Cocker (1974), but in contrast to the autosomal dominant inheritance reported by Savara et al (1954), Rushton (1957) and Laband et al (1964).…”

Section: Discussionmentioning

confidence: 82%

Idiopathic Familial Gingival Fibromatosis Associated with Mental Retardation, Epilepsy and Hypertrichosis

Anavi¹,

Lerman²,

Mintz

et al. 1989

Develop Med Child Neuro

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SUMMARY Gingival fibromatosis, a rare but often familial condition, is described in two siblings, associated with mental retardation, epilepsy and hypertrichosis. In one child a maxillary giant‐cell tumour was found and excised. It is important to distinguish idiopathic gingival fibromatosis from phenytoin‐induced gingival hypertrophy. RÉSUMÉ Fibromatose gingivale familiale idiopathique associée à un retard mental, une épilepsie et une hypertrichose La fibromatose gingivale, une affection rare et souvent familiale, est décrite chez deux enfants de même fratrie, en association avec un retard mental, une épilepsie et une hypertrichose. Chez l'un des enfants, une tumeur maxillaire à cellules géantcs fut dépistée et excisée. II est important de distinguer la fibromatose gingivale idiopathique de l'hypertrophie gingivale liée au traitement par phénytoîne. ZUSAMMENFASSUNG Idiopathische, familiäre Gingivafibromatose mit geistiger Retardierung, Epilepsie und Hypertrichose Die Gingivafibromatose, eine seltene, aber häufig familiär auftretende Erkrankung, wird bei zwei Geschwistern in Verbindung mit geistiger Retardierung, Epilepsie und Hypertrichose beschrieben. Bei einem Kind wurde ein maxillärer Riesenzelltumor gefunden und exzidiert. Es ist wichtig, die idiopathische Gingivafibromatose von der Phenytoin bedingten Gingivahypertrophie zu unterscheiden. RESUMEN Fibromatosis gingival familiar idiopdtica asociada a retraso mental, epilepsia e hipertricosis Se describe en dos niños una gingivitis fibromatosa, alteración rara pero a menudo familiar, asociada a retraso mental, epilepsia e hipertricosis. En un niño se halló y extirpó un tumor maxilar de células gigantes. Es importante distinguir la fibromatosis gingival idiopática de la hipertrofia gingival debida a la fenitoina.

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Section: Discussionmentioning

confidence: 82%

Idiopathic Familial Gingival Fibromatosis Associated with Mental Retardation, Epilepsy and Hypertrichosis

Anavi¹,

Lerman²,

Mintz

et al. 1989

Develop Med Child Neuro

View full text Add to dashboard Cite

show abstract

“…Hereditary gingival fibromatosis (HGF) is a rare disorder manifesting clinically with normal coloured hyperplastic gingival tissue, with a firm consistency of the attached gingivae. The degree of hyperplasia is not related to the level of oral hygiene (Savara et al 1954, Fletcher 1966) and shows inter-and intra-familial variation (Fletcher 1966, Fig Raeste et al 1978). There may be minimal expression with focal sites of involvement, which are difficult to detect, or generalised gingival hyperplasia that partially or completely covers the dentition and may act as a physical barrier to the eruption of teeth (Savara et at.…”

Section: Discussionmentioning

confidence: 99%

“…The degree of hyperplasia is not related to the level of oral hygiene (Savara et al 1954, Fletcher 1966) and shows inter-and intra-familial variation (Fletcher 1966, Fig Raeste et al 1978). There may be minimal expression with focal sites of involvement, which are difficult to detect, or generalised gingival hyperplasia that partially or completely covers the dentition and may act as a physical barrier to the eruption of teeth (Savara et at. 1954, Fletcher 1966, Jorgenson & Cocker 1974.…”

Section: Discussionmentioning

confidence: 99%

Autosomal recessive gingival fibromatosis with distinctive facies

Goldblatt

Singer

1992

Clinical Genetics

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Hereditary gingival fibromatosis is a rare condition occurring as an isolated anomaly or as part of a genetic syndrome. The isolated or syndromic disorders are usually inherited in an autosomal dominant manner, but an autosomal recessive form has been suggested. We report on male and female siblings who have gingival fibromatosis in association with specific facial dysmorphism. Their phenotype is depicted and described to document this hitherto unreported autosomal recessive gingival fibromatosis syndrome.

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“…Gingival fibromatosis has been reported in association with a number of other abnormalities: the Rutherford Syndrome; 3, 4 the Laband Syndrome; 5,6 virginal hyper trophy of the breasts and hypertrichosis; 7,8 the Cowden Syndrome; 9 the Murray Syndrome; 10-12 cherubism, epi lepsy and oligophrenia; 13 hypothyroidism; 14-15 alveolar bone loss; 16,17 hypertrichosis; 18,19 hypertrichosis and oligophrenia; 20,21 hypertrichosis and epilepsy; 22 hyper trichosis, epilepsy and oligophrenia; 22 opligophrenia; 23 epilepsy and oligophrenia; 24 pituicytoma; 25 an acromegalic-like syndrome; 26 partial anonychia and osseous abnormalities; 27 or as an isolated trait. [28][29][30][31][32][33][34] Our patient does not fit any of the above described conditions and we propose that the group of anomalies presented by this patient are unique and as yet un reported.…”

Section: Discussionmentioning

confidence: 99%

Gingival Fibromatosis, Hypertelorism, Anti‐Mongoloid Obliquity, Multiple Telangiectases and Cafe Au Lait Pigmentation; A Unique Combination of Developmental Anomalies

Giansanti

McKenzie

Owens

1973

Journal of Periodontology

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DIFFUSE, IDIOPATHIC GINGIVAL ENLARGEMENT is knownby various terms: gingival fibromatosis, fibromatosis gingivae, diffuse gingival hyperplasia, hypertrophy of the gums, idiopathic gingival hyperplasia, elephantiasis gin givae, gigantism of the gingivae, hereditary gingival fibromatosis, multiple epulides, hypertrophic gingivae, and congenital macrogingivae. We will use the term gingival fibromatosis in keeping with a recent and ex cellent review of this subject. 1The most common conditions which could be con fused with gingival fibromatosis are inflammatory hyper plasias, leukemic infiltrations, and dilantin therapy. In most cases, the history, the duration of the condition, or a peripheral blood count easily serve to separate these.As with many syndromes and genetic diseases, welldocumented cases are needed. A case is therefore re ported of gingival fibromatosis with other, formerly un reported findings with a ten-year follow-up. Case Report:The patient is a 36-year-old white female. In 1961, at the age of 25, she was seen at Emory University School of Dentistry with the complaint of excessive gin givae. At that time she was treated by one of us (W.T.M.) by surgical gingivectomy. Preoperative and postoperative photos are shown in Figures IA and IB, respectively.In 1971 the patient was reevaluated and completely worked up. The gingival condition was excellent ( Figure 1C); there was a mild, generalized degree of recurrence. The patient has had no gingival treatment of any kind except for routine dental prophylaxis. The gingival en largement was dated to the age of about 8 years.A bone survey, blood chemistry and counts, a karyo type, and a complete physical examination, including pelvic, were all performed. Past medical records were available and studied. The family was investigated.The patient was relatively short (156.8 cms.) with proportionately short arms, legs and fingers.The bone survey showed a rather abrupt flaring of the metaphyses, but no other long bone abnormalities. The skull series showed cranio-facial disproportion with a tendency towards brachycephaly. There was marked hypertelorism* (38 m.m.; normal 26.1 mm. ±1.9 mm.). 2 Both the frontal and maxillary sinuses were quite large ( Figure 2).Clinically, there was frontal and parietal bossing (Fig ure 3A). The maxilla and zygoma were hypoplastic with a resultant facies of sunken cheeks and prognathism ( Figure 3B). The nose was thin and straight with small alae. As previously stated, there was true ocular hyper telorism, mild proptosis, and anti-mongoloid obliquity of the eyes.The ear meatus, the antitragus and the helical rim were large; the lobe was small.Distributed over most of the body were hundreds of telangiectases which had been present since birth. As a child, a larger vascular hamartoma had been removed from behind the right ear. Two spots of cafe au lait pigmentation were present on the right buttock and the right small of the back; these measured approximately three centimeters in greatest diameter. The mother of the patient had the same ...

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Hereditary Gingival Fibrosis Study of a Family

Cited by 23 publications

References 2 publications

Idiopathic Familial Gingival Fibromatosis Associated with Mental Retardation, Epilepsy and Hypertrichosis

Idiopathic Familial Gingival Fibromatosis Associated with Mental Retardation, Epilepsy and Hypertrichosis

Autosomal recessive gingival fibromatosis with distinctive facies

Gingival Fibromatosis, Hypertelorism, Anti‐Mongoloid Obliquity, Multiple Telangiectases and Cafe Au Lait Pigmentation; A Unique Combination of Developmental Anomalies

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