Hereditary Haemorrhagic Telangiectasia, also known as Osler-Rendu-Weber disease is a rare autosomal dominant disorder affecting small vessels of multiple systems whose main pathological change is the presence of abnormal arteriovenous communications. Usually presents as skin and mucosal telangiectasias, epistaxis, gastrointestinal bleeding and visceral arteriovenous malformations. Although the ...

Background: Congenital hepatic fibrosis (CHF) is an autosomal hereditary disorder affecting the porto-biliary system. It is a rare hereditary disorder often presenting in childhood or adolescence with hepatomegaly, splenomegaly, and gastrointestinal bleeding. A timely diagnosis of organomegalies by sonography can prevent esophageal varices. Liver transplantation is now the only...

The article represents a review of recent data about the therapy of von Willebrand disease in children and adolescents (hereditary as well as acquired forms of the disease). The treatment of bleeding events in these patients, the indications in different subtypes, and the future lines of research are mentioned.

Assisted reproductive technology (ART) requires transvaginal oocyte retrieval (TVOR), and ovarian bleeding after TVOR rarely occurs. We present a case of 37-year-old woman (0-gravida) who was diagnosed with possible hemorrhagic telangiectasia (HHT) had history three laparotomies for an inclusion cyst adjacent to the right ovary third operation. HHT is hereditary disease characterized by spontan...

Background: Coagulation plays an important role in haemostasis. Bleeding disorders caused by deficiency of certain coagulation factors such as hemophilia and Von Willebrand disease can affect haemostasis and may endanger life. Aim: To put a focus on the aetiology, pathogenesis, methods of diagnosis and lines of management of bleeding disorders. Conclusion: Bleeding disorders usually result from...

Whereas many healthy people consider their bleeding and bruising excessive, patients with underlying von Willebrand disease, the most common hereditary bleeding disorder, often fail to identify their bleeding symptoms. Therefore, it is necessary to ask for specific information from patients about bleeding and bruising (Is the patient easily bruised? What is the size of the bruises? If the patie...

Objective: This study aims to investigate the presence of acquired and hereditary coagulation in adolescent girls presenting with increased menstrual bleeding. Method: The consisted 63 female patients (15.4±1.5 years) who applied pediatric clinic our hospital due bleeding, did not have any acute or chronic disease, use medication last 14 days, were same age gender as group. 74 healthy adolescen...

Data from 98 patients with hereditary haemorrhagic telangiectasia (HHT) are presented. All were symptomatic by 40 years of age and 62% by 16 years. Nose bleeding was the first symptom of disease in 90% of cases with mucocutaneous telangiectases appearing 5 to 20 years later. Complications of HHT are discussed and an age of onset curve given.

Five female members of a family manifesting a dominantly inherited bleeding disorder were investigated for coagulation and platelet abnormalities. A long bleeding time, mild thrombocytopenia, large platelets, low platelet factor-3 activity (PF-3), and low factor VIII levels were found in the proband. Long bleeding times, low PF-3, and low factor VIII levels were found in various combinations in...