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Query: UMLS:C0019079 (hemoptysis)
6,129 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hereditary familial telangiectasis is a vascular dysplasia characterized by a triple syndrome: hereditary character, telangiectasias and hemorrhages. Its association with systemic and/or pulmonary arterio-venous fistulae is frequent and can condition several complications: cerebral abscess, digestive hemorrhages, epistaxis, hemoptysis, hypoxemia, polyglobulia, and rarely the appearance of hemothorax. We describe a patient who was admitted in hospital with a hypovolemic shock secondary to a massive hemothorax; during emergency surgery, which was necessary to perform, a pulmonary A-V fistula was observed in the middle lobe. The studies afterwards performed demonstrated that it was a Rendu-Osler-Weber disease with duodenal, hepatic, and cerebral involvement.
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PMID:[Massive hemothorax: an exceptional complication of Rendu-Osler-Weber disease]. 178 May 31

Over a 10-year period, 276 pulmonary arteriovenous malformations (PAVMs) were occluded with balloon embolotherapy in 76 patients, 67 (88%) of whom had hereditary hemorrhagic telangiectasia. Eleven patients (14%) were discovered by means of family screening with measurement of arterial blood gases and chest radiography. Epistaxis, dyspnea, hemoptysis, and hemothorax occurred in 79%, 71%, 13%, and 9% of patients, respectively. Clinical histories of strokes and transient ischemic attacks were present in 18% and 37% of patients, respectively. Computed tomographic scans of 59 patients showed stroke in 36%. Sixty-five percent of PAVMs were located in the lower lobes, which correlated with the finding of more pronounced hypoxemia in the upright position. After embolotherapy, symptomatic hypoxemia was corrected, and serial values have remained constant for 5 years. Complications were minimal, and no patient required surgery. Balloon embolotherapy is effective long-term therapy for PAVMs, and family screening should be pursued because of the possibility of a higher frequency of paradoxical embolization (stroke) than previously recognized.
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PMID:Pulmonary arteriovenous malformations: techniques and long-term outcome of embolotherapy. 318 89

The occurrence of significant pulmonary hemorrhage associated with pulmonary arteriovenous malformations (PAVMs) and hereditary hemorrhagic telangiectasia (HHT) and the incidence of PAVMs in family members of patients with PAVMs and HHT are poorly defined. We reviewed our experience in 143 patients with PAVMs and HHT. Eleven (8 percent) of the 143 patients with HHT and PAVMs had a history of either massive hemoptysis or of hemothorax which required hospitalization. One patient died directly related to the pulmonary hemorrhage. There were four men and seven women. Three of the seven women experienced pulmonary hemorrhage during pregnancy. Seven of the 11 families participated in screening for PAVMs. Thirty-six (80 percent) of the 45 screened family members were found to have HHT. Thirteen (36 percent) of the 36 family members with HHT were proven to have PAVMs by pulmonary angiography. Pulmonary hemorrhage due to spontaneous rupture of the PAVM is a potentially life-threatening complication that should be treated aggressively with transcatheter embolotherapy. It occurs more frequently than previously recognized in patients with PAVMs and HHT. In addition, because of the increased incidence of PAVMs in family members of patients with HHT and PAVM, screening of family members with HHT is recommended especially in women of childbearing age.
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PMID:Life-threatening pulmonary hemorrhage with pulmonary arteriovenous malformations and hereditary hemorrhagic telangiectasia. 795 88

Pulmonary arteriovenous (AV) malformations occur sporadically and in the pediatric population are most commonly associated with hereditary hemorrhagic telangiectasia. Hemoptysis is an infrequent presentation in this age group. Pulmonary angiography is considered to be the definitive diagnostic modality. The authors describe an otherwise healthy eleven-year- old girl who presented with massive hemoptysis secondary to a large bronchial AV malformation unrelated to any congenital anomalies. The pulmonary angiogram appeared normal and the diagnosis was established by digital subtraction aortography. Successful embolotherapy was performed by selective injection of polyvinyl alcohol particles into the culprit bronchial artery.
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PMID:Bronchial arteriovenous malformation in a child with hemoptysis. A case report. 859 17

A pregnant woman presented with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) and a single pulmonary arteriovenous malformation (AVM) that had been embolized 5 years previously. Partly due to pregnancy, recanalization of the aneurysm occurred with subsequent hemoptysis. Despite successful therapeutic reembolization of the afferent pulmonary artery, hemoptysis recurred 5 days later. At this time, recanalization of the pulmonary artery was not demonstrated by pulmonary angiography, but a systemic angiogram revealed a bronchial arterial supply to the pulmonary AVM. A systemic supply should always be sought in cases of recurrent hemoptysis after technically successful embolization of the feeding pulmonary artery.
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PMID:Systemic supply to a pulmonary arteriovenous malformation: potential explanation for recurrence. 875 86

Causes of hemoptysis in children have not been well documented in the paediatric otolaryngology literature. The aim of this retrospective review is to determine the commonest causes of hemoptysis in the paediatric age group presenting to an otolaryngologist. We reviewed the charts of patients presenting to an otolaryngologist at The Hospital for Sick Children, Toronto, Ontario, over a 10-year period. A total of 37 inpatients beyond the neonatal period were referred for further assessment of hemoptysis. Thirty-two patients (86.5%) underwent bronchoscopy to determine the cause, the hemoptysis resolving spontaneously in the remaining five patients without a diagnosis. Four patients who had a bronchoscopy also had no identifiable pathology. Tracheobronchitis was the commonest diagnosis (19%), followed by tracheotomy-related problems (15.5%) Other causes included bronchiectasis, aspiration of blood, pulmonary hemorrhage, foreign-body aspiration, cystic fibrosis, A-V malformation, tracheobronchial hemangioma, hereditary telangiectasia, laceration of a vocal cord, and pneumonia. Otolaryngologists need to be aware of the etiology of hemoptysis in children. The commonest causes are infection and trauma, and not vascular anomalies or neoplasms as often perceived.
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PMID:Hemoptysis in children: the hospital for sick children experience. 881 10

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

Pulmonary arteriovenous malformations (PAVM) expand during pregnancy because of increases in blood volume, cardiac output, and venous distensibility. More than half of the cases reported during pregnancy are associated with hereditary telangiectasia. In this case a 36-year-old primigravida presented at 24 weeks of gestation with new onset hemoptysis and dyspnea. A PAVM was noted in the right lower lobe during angiography and was successfully treated with embolization. Recurrence of symptoms occurred at 36 weeks of gestation after recanalization of the PAVM. Cesarean delivery was performed because of both this recurrence and breech presentation. The patient's symptoms subsequently resolved after delivery. The patient underwent a segmentectomy without complication 6 months after delivery. Thus, women with known PAVM or a history of hereditary telangiectasia should be followed with serial chest roentgenograms and arterial blood gases to detect acute progression of the PAVM. Embolization can be used during pregnancy if the PAVM is symptomatic.
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PMID:Progression of pulmonary arteriovenous malformation during pregnancy: case report and review of the literature. 909 91

Neurologic manifestations occur in 8-12% of the patients with Rendu-Osler-Weber disease or hereditary hemorrhagic telangiectasia (HHT), principally infectious and hemorrhagic and, less frequently, ischemic ones. More than a half of these neurologic complications are associated with pulmonary arterio-venous malformations (PAVM). The diagnosis of HHT is based on the presence of telangiectases, hemorrhagic events and a family history with an autosomal dominant pattern. We report a case of a patient diagnosed as having HHT with transient ischemic attacks and a PAVM, which was occluded by the use of embolotherapy. Cerebral ischemia in HHT is related to the existence of a PAVM and results from three mechanisms: 1) secondary poliglobulia and hyperviscosity because of the hypoxemia due to a right-left shunt; 2) communication between the airway and the pulmonary circulation during cough access, which produces gas embolism and hemoptysis; 3) and, finally, paradoxical embolism trough the PAVM, the same mechanism proposed to the infectious neurologic manifestations of the disease. When the diagnosis of HHT is suspected, early search and treatment of PAVM, with embolotherapy or surgery, are necessary in order to avoid respiratory problems (hemoptysis, exertional dyspnea, cianosis, clubbing) and neurologic complications.
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PMID:[Cerebral ischemia in Rendu-Osler-Weber disease]. 1076 36

Hereditary hemorrhagic telangiectasia (HHT) is an angiodysplastic disorder transmitted in autosomal dominant fashion. This case report describes a 46-year-old woman with multiple arteriovenous malformations that eventually caused multiorgan failure. The diagnosis was made in the early childhood based on frequently recurring episodes of severe epistaxis. Telangiectases were found in the skin, the visible mucosal linings, gastrointestinal and bronchial mucosa and liver. Episodes of severe hemoptysis necessitated partial resection of the right lung middle lobe at the age of 22. Liver dysfunction associated with the primary disease was diagnosed by the characteristic clinical picture, laboratory findings and instrumental examinations including abdominal ultrasonography, upper endoscopy, liver CT venography.
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PMID:Pulmonary, gastrointestinal and liver vascular malformations in Rendu-Osler disease. 1134 41


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