Osler-Rendu syndrome

Osler-Rendu syndrome (Osler W., N. J.L. Rendu; synonym: Randy-Osler disease, Osler disease, Randy-Weber-Osler disease, hemorrhagic angiomatosis, telangiectasia hereditaria haemorrhagica, angioma haemorrhagicum hereditarum) - multiple inheritance telangiectasias, accompanied by repeated bleeding. The disease is rare; affects both sexes equally. The etiology is unknown. In most cases, the syndrome of Osler-Randu of a family nature, nasledujuci by dominant type. In the literature there are data about families where the disease is detected in four or five generations.
Histological study reveals a sine expansion vessels in the wall of which remains one of the endothelium and disappear elastic and muscular layer. Skin biopsy reveals loosening of the components of the connective tissue, perivascular accumulation of leukocytes and histiocytes; weak development of papillae and appendages: skin hair follicles little sweat glands expressed weakly. The inherent weakness of the mesenchyme poses telangiectasia followed by bleeding.
The main symptom is bleeding, the nature, frequency and intensity of which depends on the location, size and number of telangiectasia. Bleeding from the affected areas occur spontaneously or after a minor injury. Nasal bleeding is one of the most frequent types of hemorrhages in the syndrome of Osler-Randy. Bleeding from internal organs (stomach, intestines, lungs, kidneys) in bloody vomiting, melena, gemorroe, hematuria. With the frequent and copious bleeding anemia. Blood coagulation, the number of platelets, the retraction of the clot, the bleeding time and symptom harness syndrome of Osler-Randy normal.
The diagnosis is made on the basis of local vascular changes, data heredity, are no changes in the blood.
The prognosis is poor.
The treatment is symptomatic. Apply searing bleeding places, General hemostatic means (menadione, blood transfusion). If profuse bleedings have to resort to the ligation of vessels, in particular a. carotis externa during nasal bleeding. Lately abroad proposed treatment of sex hormones (large doses of estrogen). Women nominated levonorgestrel 0.25 mg per day, men - 0.5 mg to 5 mg methyltestosterone. ACTH and corticosteroids are ineffective. Sharply worsens the prognosis of General x-ray and radiation exposure. The short-focus x-ray treatments can be used for limited bleeding telangiectasia skin and mucous membrane of the lips.