Gardner syndrome
Updates to Article Attributes
Body
was changed:
Gardner syndrome is one of the polyposis syndromes. It is characterised by:
- familial adenopolyposis
- multiple osteomas: especially of the mandible, skull, and long bones
- epidermal cysts
- fibromatoses
- desmoid tumours of mesentery and anterior abdominal wall
Other abnormalities include:
- supernumerary teeth and odontomas4
- duodenal tumours / ampullary carcinoma 2,3
- papillary thyroid carcinoma
Pathology
There is an autosomal dominant inheritance in the FAP gene (chromosome 5q) in a majority of patients but with 20% of cases resulting from new mutations. Extracolonic features often precede the diagnosis of colonic polyps.
History and etymology
First described in 1953 by Gardner and Richards 3.
-<li><a href="/articles/supernumerary-teeth">supernumerary teeth</a></li>- +<li>
- +<a href="/articles/supernumerary-teeth">supernumerary teeth</a> and <a title="Odontomas" href="/articles/odontoma">odontomas</a> <sup>4</sup>
- +</li>
-</ul><h4>Pathology</h4><p>There is an autosomal dominant inheritance in the <a title="Familial adenomatous polyposis (FAP)" href="/articles/familial-adenomatous-polyposis-syndrome">FAP</a> gene (chromosome 5q) in a majority of patients but with 20% of cases resulting from new mutations. Extracolonic features often precede the diagnosis of colonic polyps. </p><h4>History and etymology</h4><p>First described in 1953 by <strong>Gardner</strong> and <strong>Richards</strong> <sup>3</sup>.</p>- +</ul><h4>Pathology</h4><p>There is an autosomal dominant inheritance in the <a href="/articles/familial-adenomatous-polyposis-syndrome">FAP</a> gene (chromosome 5q) in a majority of patients but with 20% of cases resulting from new mutations. Extracolonic features often precede the diagnosis of colonic polyps. </p><h4>History and etymology</h4><p>First described in 1953 by <strong>Gardner</strong> and <strong>Richards</strong> <sup>3</sup>.</p>
References changed:
- 4. Wolf J, Järvinen HJ, Hietanen J. Gardner's dento-maxillary stigmas in patients with familial adenomatosis coli. (1986) The British journal of oral & maxillofacial surgery. 24 (6): 410-6. <a href="https://doi.org/10.1016/0266-4356(86)90054-9">doi:10.1016/0266-4356(86)90054-9</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/3024698">Pubmed</a> <span class="ref_v4"></span>