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| __NOTOC__
| | #REDIRECT [[Bannayan-Riley-Ruvalcaba syndrome]] |
| {{SI}}
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| {{CMG}}; {{AE}} {{VKG}}
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| {{SK}} Ruvalcaba-Myhre syndrome, Ruvalcaba-Myhre-Smith syndrome, Riley-Smith syndrome, Bannayan syndrome, or Bannayan-Zonana syndrome
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| ==Overview==
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| Bannayan-Zonana syndrome is a rare [[hamartomatous]] disorder with occurrence of multiple subcutaneous [[lipoma]]s, [[macrocephaly]] and [[hemangioma]]s.
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| ==Historical Perspective==
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| ==Classification==
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| ==Pathophysiology==
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| The disease is inherited in an [[autosomal dominant]] form, but sporadic cases have been reported.
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| The disease belongs to a family of hamartomatous polyposis syndromes, which also includes [[Peutz-Jeghers syndrome]], [[juvenile polyposis]] and [[Cowden syndrome]].
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| * Bannayan-Riley-Ruvalcaba syndrome (BRRS) is transmitted in [[autosomal dominant]] pattern.
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| * It is understood that Bannayan-Riley-Ruvalcaba syndrome (BRRS) is the result caused by the following gene mutations:<ref name="pmid12938083">{{cite journal |vauthors=Eng C |title=PTEN: one gene, many syndromes |journal=Hum. Mutat. |volume=22 |issue=3 |pages=183–98 |date=September 2003 |pmid=12938083 |doi=10.1002/humu.10257 |url=}}</ref>
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| ** Germline phosphatase and
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| ** Tensin homolog (''[[PTEN (gene)|PTEN]]'') mutations.
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| **
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| ==Causes==
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| ==Differentiating {{PAGENAME}} from Other Diseases==
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| ==Epidemiology and Demographics==
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| ==Risk Factors==
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| ==Screening==
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| ==Natural History, Complications, and Prognosis==
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| Most lesions of BZS are slowly growing and easily resectable. Visceral as well as intracranial involvement may occur in rare cases, and can cause bleeding and symptomatic mechanical compression, especially of the spinal cord or spinal nerve roots.
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| This may require surgical resection.
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| ==Diagnosis==
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| ===Diagnostic Criteria===
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| ==History and Symptoms==
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| ===Physical Examination===
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| The [[macroencephaly]] of BZS is symmetrical, and does not cause widening of the ventricles or raised [[ICP]] ([[intracerebral pressure]]).
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| ==Laboratory Findings==
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| ==Imaging Findings==
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| ==Other Diagnostic Studies==
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| ==Treatment==
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| ===Medical Therapy===
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| ===Surgery===
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| ===Prevention===
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| [[Category:Endocrinology]]
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| [[Category:Disease]]
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| [[fr:Syndrome de Bannayan-Riley-Ruvalcaba]]
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| [[pl:Zespół Bannayana, Rileya i Ruvalcaby]]
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| {{WS}}
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| {{WH}}
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| <references />
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