Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

When performed by a team who are experienced in the use of embolization of the external carotid region, this procedure is nowadays a safe and extremely effective method for treating severe, massive, uncontrolled, recurrent epistaxis. Arteriography shows the site of bleeding, the nature of the lesion, and the arteries affected. When carried out by the femoral or common carotid route, this method was effective in 52 cases of severe epistaxis of various origins: essential epistaxis, or from hypertension, trauma, Rendu Osler's disease, vascular malformations, carotidocavernous fistulae, benign or malignant tumors, hematological affections, or hemostatic disorders. To avoid risk it is essential that a perfect technique be used and a certain number of principles respected. The method is effective in cases were surgical hemostasis is insufficient, and its rapidity of action allows removal of packs immediately after embolization. This clearly underlines the value of constantly available vascular radiology units for treatment in this region as well as in other parts of the body.
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PMID:[The value of embolization in severe epistaxis. Indications and methods (author's transl)]. 49 Apr 77

Hereditary hemorrhagic telangiectasia is a rare, hereditary fibrovascular dysplasia. We report a case associated with hepatolithiasis. Hepatolithiasis, relatively common in East Asia, is rare in the West. The association of the two conditions has not been previously reported. In this case, vascular malformations in the liver gave rise to arteriovenous and arterioportal fistulas, causing arteriovenous shunting and protal hypertension, respectively. Abnormal blood flow is the proposed mechanism for the hepatic fibrosis and nodular regeneration. Hepatic fibrosis, by causing stenosis of large intrahepatic bile ducts, bile stasis, and secondary infection, is the hypothesized mechanism for calculus formation. Hepatolithiasis ultimately caused death from acute bacterial cholangitis and septicemia.
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PMID:Hepatolithiasis in hereditary hemorrhagic telangiectasia. 232 2

We describe an elderly patient with an unusual presentation of hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease) involving the lung. He had recurrent "pneumonia" caused by massive hemorrhage from endobronchial telangiectases. When stable, he was normoxic, had no evidence of right-to-left shunting, and had mild pulmonary arterial hypertension. His pulmonary telangiectases may be isolated to the bronchial circulation. We report hemodynamic data and show the first photographs of endobronchial telangiectases.
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PMID:Pulmonary telangiectasia without hypoxemia. 335 30

Effect of furosemide at various concentrations (0.05-4 mM) was examined on the in vitro biosynthesis of prostaglandins in human platelets. At lower furosemide concentrations (0.05 and 0.1 mM) no effect was observed. At 1 and 2 mM concentrations, PGE2 significantly (P less than 0.01) increased. At 3 and 4 mM concentrations PGE2 increased though not significantly probably because of the small number of samples (n=5). A decrease in PGD2 formation was noted at 1-4 mM furosemide concentrations, though significantly only at 3 mM conc. At 1 and 2 mM concentrations, TxB2 and HHT increased whereas at 3 and 4 mM concentrations these metabolites were decreased. These effects were, however, not significant. No effect was observed on endoperoxide generation at 1 and 2 mM conc. Furosemide at 1 and 2 mM concentrations inhibited ADP- and arachidonic acid induced platelet aggregation. An increased platelet formation of PGE2 in the presence of furosemide may point to the fact that this drug shows its effect mainly on the formation of PGE2 which has been found to exert a profound effect on renal blood flow and thus ameliorates some forms of hypertension.
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PMID:Effect of furosemide on the in vitro prostaglandin biosynthesis in human platelets. 681 95

Rendu-Osler-Weber disease is an hereditary disorder characterized by cutaneo-mucous telangiectasis and vascular abnormalities in several organs. Bleeding, especially epistaxis, represents the most important clinical feature. Pulmonary arteriovenous fistulae can cause hypoxaemia, haemoptysis, polycythaemia and clubbing. Diagnosis is based on family and personal history, teleangiectasis, laboratory (haemochrome, fibrinogen, PT, PTT) and instrumental findings (endoscopy and/or roentgen). Therapy depends on symptoms. Embolization of pulmonary arteriovenous fistulae and laser treatment of intestinal vascular abnormalities have been successful. Danazol treatment yielded controversial results. We report the case of a patient admitted for arterial hypertension and recurrent epistaxis. Rendu-Osler-Weber disease diagnosis was made based on positivity at family and personal history, clinical examination, laboratory and instrumental findings. In conclusion we underline the pivotal role of anamnesis and clinical examination in the differential diagnosis of hereditary bleeding disorders and emphasize the importance of early diagnosis for the correct therapeutic approach.
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PMID:[Rendu-Osler-Weber disease. Report of a clinical case]. 899 9

Diagnostic angiography and super selective embolization of the internal maxillary artery were performed on 16 cases between 1990 and 1995. The patients (mean age, 49 years) were treated for severe posterior epistaxis, refractory to local haemostatic therapy (nasal packing alone for the most of them), hypertension was found to be the most frequent predisposing factor, 13 patients had their nose bleeding stopped. Among the 3 failures, were 2 cases of Osler-Weber-Rendu disease, which were subsequently significatively improved by this method. We conclude that angiography with super selective embolization is considered as a valuable diagnostic and therapeutic option in the management of patients with severe epistaxis.
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PMID:[Role of super selective arteriography with embolization in the treatment of severe epistaxis: our experience apropos of 16 cases]. 918 7

We encountered a case of acute cerebral hemorrhage secondary to capillary telangiectasia in a 10-year-old female. She was diagnosed as having Rendu-Osler-Weber disease (ROW). In this case, the cerebral hematoma did not result in neurologic damage and the final outcome was excellent. ROW is an autosomal dominant disorder characterized by the presence of vascular malformations of varying types in several tissues, including the brain, nasal mucosa, lungs, gastrointestinal tract, and liver. Neurological complications occur in 8 to 12 percent of patients with ROW. The pons is the most common site of capillary telangiectasia, but most of the malformations caused are clinically silent. Massive cerebral hematoma due to capillary telangiectasia is rare. Cerebral hematoma due to hypertension in a child is less than that found in an adult. So in a child it is important to investigate the origin of cerebral hematoma.
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PMID:[Rendu-Osler-Weber disease with cerebral hemorrhage due to a capillary telangiectasia: a case report]. 1036 62

Understanding the features of the various hereditary vascular pathologies allows consideration and confirmation of the diagnosis, and a search for treatable hidden disorders, avoiding harmful investigations, initiating follow up, performing family investigations and providing genetic counselling. Pseudoxanthoma elasticum must be considered in the presence of calcified distal arteriopathy of the lower limbs in a young subject without any other aetiological aspects. Cutaneous or mucosal lesions confirmed on histological examination, angioid streaks at the back of the eye and a family history support the diagnosis, which is confirmed by showing pathogenic mutations of the ABCC6 gene. It is then important to search for a peripheral disorder in other arterial territory, a low grade coronaropathy, hypertension and an endocardial disorder. Prescription of antithrombotics must be made carefully because of the risk of gastro-intestinal haemorrhage. Vascular Ehlers-Danlos syndrome is suspected in a subject less than 30 years old with diffuse aneurysmal disease, spontaneous arterial rupture or dissection, a carotido-cavernous fistula or early onset varices. Demonstrating an ecchymotic tendency or an acrogeric morphology, especially in a familial context, warrants cutaneous biopsy for anatomopathological examination and fibroblast culture for a study of the C0L3A1 gene. When the diagnosis is suggested, it is advisable to prohibit any arterial puncture, cold surgery or gastro-intestinal endoscopy. The search for aneurysmal lesions must be performed by non-invasive imaging. The therapeutic management requires specialised teams. The combination of repeated epistaxis, muco-cutaneous telangectasia and similar characteristics in a family suggests the diagnosis of Osler-Weber-Rendu disease. The search for iron deficiency, gastro-intestinal bleeds and pulmonary, hepatic or cerebral arterio-venous malformations is then necessary. Besides skilled endovascular management when indicated, it is important to advise every patient with a pulmonary arterio-venous malformation to take antibiotic prophylaxis against cerebral abscess in situations at risk of bacteraemia.
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PMID:[Mendelian arterial diseases. Pseudoxanthoma elasticum, Ehlers-Danlos vascular syndrome, Rendu-Osler disease]. 1469 86

Hereditary haemorrhagic telangiectasia (HHT) is a disorder of arteriovenous malformations and telangiectases. In rare cases affected individuals may develop typical pulmonary arterial hypertension (PAH). Vasodilator therapy has not been recommended because of a potential increase in arteriovenous shunt volume. This report is on two patients with severe HHT-associated PAH who were treated with bosentan, an oral endothelin ET(A)/ET(B) receptor antagonist. After 1 year, symptomatic and functional improvements were confirmed by haemodynamic evaluation demonstrating a significant decrease of mean pulmonary artery pressures and an increase in cardiac index, without evidence for an increase in effective shunt volume.
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PMID:Bosentan therapy for pulmonary arterial hypertension associated with hereditary haemorrhagic telangiectasia. 1691 15

Activin receptor-like kinase 1 (ALK1) is involved in the pathogenesis of hereditary hemorrhagic telangiectasia type II (HHT2) and pulmonary arterial hypertension. We have previously shown that Alk1 is predominantly expressed in the arterial endothelium and plays a pivotal role in the formation of embryonic blood vessels. At present, however, little is known about the precise expression pattern and function of ALK1 during extra-embryonic vascular development. Using previously generated lacZ reporter lines, we sought to examine the expression pattern and role of Alk1 during placental development in mice. Alk1 expression was restricted to endothelial cells of fetal vessels from the emergence of chorioallantoic fusion to the late gestational period, and no detectable Alk1 expression was observed in syncytiotrophoblasts or spongiotrophoblasts. Predominant arterial expression was observed in the umbilical and fetal placental vessels as well as in embryonic vessels. Morphological analysis of Alk1-null embryos indicates that Alk1 is essential for the development of distinct umbilical arteries and veins. The invasion of chorioallantoic mesoderm into the forming labyrinth layer was largely unaffected in the Alk1-null placenta, but chorioallantoic vessels appeared to be severely dilated and fused. Results from this study provide valuable information regarding the role of ALK1 in the development of placental vasculature as well as insights into the pathogenesis of HHT.
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PMID:Activin receptor-like kinase 1 is essential for placental vascular development in mice. 1753 30


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