Gorlin-Goltz syndrome, also known as the basal cell naevus syndrome, is a rare phakomatosis characterised by multiple odontogenic keratocysts (KOT), multiple . Gorlin and Goltz’s eponymous syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is an autosomal dominant disorder. The Gorlin-Goltz syndrome (GGS) (the nevoid basal cell carcinoma syndrome— NBCCS) is a rare autosomal dominant syndrome caused due.
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It is an autosomal dominant syndrome with complete penetrance and variable expressivity. It is related to a mutation in PTCH tumor suppressor gene on chromosome 9, which encodes for the “Sonic Hedgehog” receptor 3.
Gorlin-Goltz syndrome | Radiology Reference Article |
Individuals with the syndrome are sensitive to ionizing radiation, with one study showing accumulation of p53 in exposed cells 3. Patients with Gorlin syndrome may be hypersensitive to and contraindicated from receiving radiation therapy.
A clinical diagnosis can be made using major and minor criteria. To make the diagnosis, either two major or one major and two minor criteria must be met. You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. Unable to process the form.
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Synonyms or Alternate Spellings: Gorlin-goltz syndrome Gorlin syndrome Basal cell nevus syndrome Basal cell naevus syndrome Bifid rib syndrome Nevoid basal cell carcinoma Naevoid wyndrome cell carcinoma syndrome Nevoid basal cell carcinoma syndrome NBCCS.
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