[Familial melkersson rosenthal syndrome].
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Biomedical subjects
Publications and source records attributed to A Jacquier.
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The diagnosis of chronic sinusitis is based on clinical presentation, nasal endoscopy and CT scan. As a matter of fact, the CT scan of the paranasal sinuses is absolutely necessary to characterise the lesions, to visualise anatomic variations which are risk factors for the endoscopic surgery and to follow up treated and/or operated patients with recurrent symptoms. Nowadays, plain films of paranasal sinuses do not have any indication. MR Imaging may be indicated in selected cases of complicated sinusitis and of pseudotumors associated to sinusitis.
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The pathogenesis of the phacomatoses, developmental diseases of the embryonic plates, permits an understanding of the different manifestations which characterize these disorders. This pathogenesis also constitutes the best basis for a rational classification. The author sets out the main features of this pathogenesis and its practical applications, and then considers the principal vascular aspects of the phacomatoses, especially in Osler-Rendu disease, Blue Rubber Bleb Naevi, Mafussi's syndrome, the haemangioblastomatoses, Bailey's glomangiomatosis, the Louis-Bar syndrome, Struge-Weber angiomatosis, the syndrome of Bonnet-Dechaume and Blanc, Cobb's syndrome, the angio-osteo-hypertrophic syndromes, von Recklinghausen's neurofibromatosis, Bourneville's tuberous sclerosis, and the melanic phacomatoses.
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