A 64-year-old female with a family history of hereditary hemorrhagic telangiectasia (HHT) was hospitalized due to complaints dyspnea during light physical exertion and leg edema. HHT diagnosed at 20 y.o., recurrent nasal bleeding started age 52, bleedings severity aggravated by not completely compensated hypertension. At the 60, after massive hemorrhage, she noted onset dyspnea, edema, ascites....

Osler–Weber–Rendu syndrome, also known as Hereditary Hemorrhagic Telangiectasia (HHT), is an autosomal dominant disorder. Telangiectasias and Arterio-Venous Malformations (AVMs) are vascular lesions present in HHT, most commonly causing epistaxis and gastrointestinal bleeding. While epistaxis presents as early as childhood, the gastrointestinal manifestations of HHT develop with increasing age.

Genetic bleeding disorders form an important presentation among various genetic disorders occurring in children. A prevalence of 6 per 100,000 population has been reported in India. Bleeding disorders constitute a heterogenous group of disorders with varying clinical presentations. The common bleeding disorders are Hemophilia A and B, von Willebrand disease, and inherited thrombocytopenias. In ...

Pediatric emergency visits with purpura fulminans should raise the suspicion of hereditary homozygous protein C deficiency even beyond the neonatal age. The absence of this classical finding does not role the diagnosis out as atypical presentation with isolated intraocular bleeding was observed. Premarital counseling should be offered when family history suggests.

BACKGROUND AND OBJECTIVES Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is an autosomal dominant disorder characterized by mucocutaneous and visceral vascular dysplasia associated with frequent episodes of epistaxis and gastrointestinal bleeding. The objective of this report was to describe the anesthesia of a patient with this syndrome. CASE REPORT A ...

Bevacizumab has become a frequently used therapeutic option for bleeding complications in hereditary hemorrhagic telangiectasia (HHT). Renal limited thrombotic microangiopathy is a welldocumented complication of Bevacizumab therapy. We present an unusual case of Bevacizumab induced thrombotic microangiopathy complicated by glomerular predominant renal hemosiderosis in a patient with HHT-associa...

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant inherited disease in which abnormal communications between arteries and veins, the so-called telangiectases, occur in the skin, mucosal surfaces, and solid organs [1]. Small telangiectases on the face may present an important cosmetic problem, but larger lesions can be a source of chronic blood loss, systemic emboli, hypoxemia...

OBJECT Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal-dominant vascular dysplasia with a high prevalence of cerebrovascular malformations (CVMs), mostly manifested as arteriovenous malformations (AVMs). The natural history and bleeding risk of these CVMs is unknown. The authors investigated the risk of bleeding in conjunction with clinical and radiological features in patients ...

BACKGROUND Hereditary hemorrhagic telangiectasia (HHT) is a hereditary disorder, leading to easily bleeding telangiectases on the skin and mucosal surfaces. The disease is associated with arteriovenous malformations in multiple organs. Potentially serious complications warrant an early diagnosis. Telangiectases are the hallmark of the disease, but may be difficult to distinguish. OBJECTIVES T...

Although non-melanoma skin cancers are the most predominant malignancies in the Caucasian population and hemophilia A is one of the most frequent hereditary bleeding disorders, medical literature data about the management of non-melanoma skin cancers in patients with hemophilia are surprisingly scarce. In this case report we describe the treatment of a patient with multiple recurrent non-melano...