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Disease of Randyu — Oslera — Weber


Syndrome of Oslera-Rendyu-Vebera (Xing.; a hereditary teleangiectasia of skin and mucous membranes, a hereditary hemorrhagic angioma) W. Osler - the English therapist is described, Rendu and Weber is later.

Etiologies of Randyu — Oslera — Weber:

The syndrome is transferred by an autosomal and dominant way of inheritance. Population frequency 1:50 000.

The Bolezni Randyu symptoms — Oslera — Weber:

Dot and/or nodular teleangiectasias, vascular asterisks which are localized in language, a face, auricles, a conjunctiva, finger-tips, a nail bed to a mucous membrane of a nose are noted, nasal bleedings can be a consequence of what. Teleangiectasias can be in digestive tract, a bladder, a vagina, a uterus, lungs, a liver and a brain. Teleangiectasias arise at early age, and their number can increase.
Owing to emergence of the specified localizations can be noted:
• hematencephalons;
vomiting with blood;
posthemorrhagic anemia.
The hepatomegalia with the subsequent development of cirrhosis is quite often observed.
The diagnosis is not difficult in the presence of a teleangiectasia on visible sites of skin and mucous membranes. Identification them in the listed above internals demands, as a rule, carrying out endoscopic (a gastroduodenoskopiya, a tsistoskopiya, hysteroscopy) a research and ultrasonography.

Множественные телеангиоэктазии у пациента с болезнью Рандю-Ослера

Multiple telangiectasias at the patient with a disease Randyu-Osler

Телеангиоэктазии в области языка при болезни Рандю—Ослера—Вебера

Telangiectasias in the field of language at a disease of Randyu — Oslera — Weber

The Bolezni Randyu treatment — Oslera — Weber:

Teleangiectasias on the mucous membranes available to survey, can be exposed to a laser ablation if they are followed by frequent bleedings.

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