hepatoportal sclerosis or idiopathic portal hypertension has a worldwide distribution with prevalence in developing and temperate countries. of 64 patients with this disease seen during a twelve year period, 36 underwent splenectomy or a shunt procedure. the indications for surgical intervention were severe hypersplenism and persistent left upper abdominal pain andlorhistory of frequent episode...

Introduction. Haemophilia A is the most common hereditary coagulation disturbance occurring due to lack of factor VIII. It widely accepted that people with haemophilia have a reduced incidence coronary artery disease, potentially because protective effect impaired against pathogenic mechanisms acute syndrome. Case report. 53-year-old man mild [FVIII 22% (mild form: more than 5%?40% normal)] was...

From a cohort of 35 patients with hereditary haemorrhagic telangiectasia (HHT), 12 patients have undergone closure of the one or both nasal cavities during the last three years for refractory epistaxis. All had failed other forms of treatment including hormone therapy, laser coagulation and septodermoplasty. All patients were available for follow-up at six months or longer. In all patients wher...

Hereditary haemorrhagic teleangiectasia (Rendu-Osler-Weber disease) is an inborn error of vascular structure with multiple manifestations. Its incidence is about 1-2:100 000 in the European population. The incidence of telangiectases and/or fistula formation was estimated to be 1 in 10 carriers of the Osler trait. The findings in the family reported herewith suggest a much higher incidence if a...

Here we report the case of a noncompliant 50-year-old female patient with high-grade, triple-negative breast cancer (TNBC) and strong family cancer history. She only agreed to start treatment after being admitted to the hospital with advanced stage disease and severe anaemia resulting from bulky, ulcerated, and actively bleeding tumor. Therapy was promptly started with platinum-based chemothera...

Montreal platelet syndrome (MPS), hitherto described in only one kindred, is a hereditary thrombocytopenia associated with mucocutaneous bleeding, giant platelets, and spontaneous platelet aggregation in vitro. These are features shared with some forms of type 2B von Willebrand disease (VWD); however, the MPS kindred had not been investigated for VWD. We found that all affected MPS family membe...

Hereditary Hemorrhagic Telangiectasia or Rendu-Osler-Weber Disease is a rare fibrovascular dysplasia that makes vascular walls vulnerable to trauma and rupture, causing skin and mucosa bleeding. It is of dominant autosomal inheritance, characterized by recurrent epistaxis and telangiectasia on the face, hands and oral cavity; visceral arteriovenous malformations and positive family history. Epi...

discussions as one ich patient whose pt and aptt suggest a coagulation disorder secondary to vitamin k deficiency or coagulation factor deficiency, unresponsiveness to vitamin k therapy should be useful to take fx deficiency into consideration. background inborn factor x deficiency (fxd) is a very rare (1: 500,000) hereditary coagulation disorder, which is characterized by clinical manifestatio...

In western European countries prostate cancer is one of the most common malignant disease among male population. Due to innovations in molecular genetics research technology over recent years genetic features etiology and pathogenesis have been discovered this helped distinguish people with high risk development. Hereditary forms tumors occupy a special position due association mutations BRCA1/...

Stuart Prower factor (Factor X) deficiency is a rare hereditary autosomal recessive coagulation disorder. We have come across three cases in the course of last 20 years at our institute. These patients presented with prolonged bleeding after minor trauma, epistaxis, subcutaneous bluish black nodules and two of them presented with history of consanguinity in parents. Hematological findings in co...