Wilson Disease (Hepatolenticular Degeneration)
General: Lesion in the putamen and lenticular 939f58j nucleus; familial; occurs in first decade of life; reduced liver function; increase in copper content in tissue.
Ocular: Night blindness; golden, grayish-green, or ruby-red peripheral posterior corneal stromal deposit (Kayser-Fleischer ring); sunflower cataract (same as with retention of copper foreign body); nystagmus; extraocular muscle palsies.
Clinical: Liver cirrhosis; jaundice (early); difficulties in speaking (early); difficulties in swallowing and mastication; extensive muscular rigidity; mouth usually open with salivation ('fixed grin'); coarse tremor.
Tso MOM, et al. Kayser-Fleischer ring and associated cataract in Wilson's disease. Am J Ophthalmol 1975; 79:479.
Walshe JM. The eye in Wilson's disease. Birth Defects 1976; 12:187.
Wilson SAK. Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver. Brain 1912; 34:295.
Spanlang-Tappeiner Syndrome (Keratosis Palmotaris and Corneal Dystrophy Syndrome) General: Autosomal dominant; etiology unknown; occurs in [...] |
Hepatic Failure [...] |
Sunrise Syndrome 1 [...] |
Copyright © 2010 - 2024
: eSanatos.com - Reproducerea, chiar si partiala, a materialelor de pe acest site este interzisa!
Informatiile medicale au scop informativ si educational. Ele nu pot inlocui consultul medicului si nici diagnosticul stabilit in urma investigatiilor si analizelor medicale la un medic specialist.
Termeni si conditii - Confidentialitatea datelor - Contact
Despre diseases |
| Alte sectiuni |
|
Ai o problema medicala? |
