Bannayan-Riley-Ruvalcaba syndrome

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Bannayan-Riley-Ruvalcaba syndrome

A rare autosomal dominant disorder (OMIM:153480) characterised by: excessive growth before and after birth; increased birth weight and length; macrocephaly often with scaphocephaly; normal intelligence or mild mental retardation; multiple subcutaneous hamartomas, lipomas, macrocephaly and hemangiomas; ocular defects (strabismus, ocular hypertelorism, exotropia and/or pseudopapilledema, hypotonia); drooling; delayed speech development and/or significant delay in developmental milestones (sitting, standing, walking); hamartomatous polyps in gastrointestinal tract and oropharynx; marbled  skin pigmentation (cutis marmorata and pigmented macules on the penis or vulva); and myopathy.

Molecular pathology
Like Peutz-Jeghers syndrome, juvenile polyposis, Cowden syndrome, Proteus syndrome and Proteus-like syndrome, Bannayan-Riley-Ruvalcaba syndrome is caused by mutations of PTEN, all of which are known as PTEN hamartoma-tumour syndromes.
References in periodicals archive ?
On the other hand, I'd be suspicious that BZS knows so much about the story and has so much interest in seeing it canned.
But I'd also want some assurance from Thornton that he would neither confirm nor deny to BZS that VCV was working on a story.
One additional assurance that should be given is that the BZS contention of a CIA investigation has been investigated - in this case by Dikes personally - and cannot be substantiated.
By that I mean this is a good test of our personal and professional relationship and a way for us to determine whether BZS was really prepared to be the owner of a responsible news organization.
The discussion with Wilder needs to acknowledge the pressure from BZS, but affirm that it is Dikes' decision to make.
BZS might learn they don't want to do business with a company that would air a story like that.
Stogsdill: Dikes should be honest with both his staff and with Wilder, explaining the concern about sourcing and the legal liability issues that BZS had with potential lawsuits from deep-pocket corporations.