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Lipoid Proteinosis (Urbach-Wiethe's Disease)
JAMA - Journal of the American Medical Association, 1963exaly +2 more sources
Journal of Cutaneous Pathology, 1980
A case of lipoid proteinosis was subjected to clinical, histologic and electron microscopic study. Palpebral biopsies showed a large dermal infiltration composed of closely interwoven granulofilamentous material in the superficial and deep dermis. Collagen fibers were normal.
G, Fabrizi +3 more
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A case of lipoid proteinosis was subjected to clinical, histologic and electron microscopic study. Palpebral biopsies showed a large dermal infiltration composed of closely interwoven granulofilamentous material in the superficial and deep dermis. Collagen fibers were normal.
G, Fabrizi +3 more
openaire +2 more sources
Translational neuroscience of basolateral amygdala lesions: Studies of urbach-wiethe disease [PDF]
Urbach-Wiethe disease (UWD) is an extremely rare autosomal recessive disorder characterized by mutations in the extracellular matrix protein 1 gene on chromosome 1.
Dan J. Stein, D J Stein
exaly +3 more sources
Spontaneous intracerebral hemorrhage in Urbach-Wiethe disease
Neurology, 2012A 39-year-old woman born from consanguineous parents was referred to our department for a second opinion regarding a left lenticular nucleus hemorrhage resulting in right hemiparesis, which occurred 1 year before in absence of history of hypertension, diabetes, smoking, or substance abuse ...
Maria Josè, Messina +7 more
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[Urbach-Wiethe disease/lipoidproteinosis].
Acta medica portuguesa, 1999The authors present a case of Urbach-Wiethe's disease (lipoid proteinosis), a rare autosomal recessive disorder, in a 49-year-old female patient with pathognomonic cranial radiological findings demonstrated by radiology and computed tomography. The rarity of this pathology made radiological diagnosis difficult.
R, Maia, L, Teixeira, J, Drago
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OC12 Lipoid proteinosis (Urbach‐Wiethe disease)
Oral Diseases, 2006Lipoid proteinosis (LP), also known as Urbach‐Wiethe disease is a rare, autosomal recessive disorder associated with deposition of acid‐Schiff (PAS)‐positive hyaline‐like material in various tissues including skin, mucosal membranes and internal organs. The aetiology of LP is currently unknown.
B Kurtuluş +4 more
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[Lipoid proteinosis (Urbach-Wiethe's Disease)].
Kulak burun bogaz ihtisas dergisi : KBB = Journal of ear, nose, and throat, 2016A 25-year-old female patient was admitted to our outpatient clinic with postpartum hoarseness. Punch biopsy specimens obtained from the larynx and sublingual region revealed multi-folded squamous epithelium with a hyperkeratosis pattern and amorphous hyaline material aggregation. This aggregation was also remarkable around the vessels.
Hatice, Karaman +3 more
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[Urbach-Wiethe disease. Apropos of 2 cases].
Annales d'oto-laryngologie et de chirurgie cervico faciale : bulletin de la Societe d'oto-laryngologie des hopitaux de Paris, 1986The Urbach-Wiethe disease is a rare condition, where a hyaline substance of an unknown biochemical nature is accumulated in both teguments and mucous membranes. It has an autosomic and recessive genetic transmission. Cutaneous and mucous lesions (especially on the upper digestive tract) are described, insisting about the laryngeal location and the ...
M, Wayoff +4 more
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Urbach-Wiethe Disease(Lipoid Proteinosis)
Pediatric Neurosurgery, 1998C C, Staut, T P, Naidich
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