Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019079 (hemoptysis)
 6,129 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In the last years, the understanding of HHT has greatly progressed. The two genes for most on cromosomes 9 and 12 have been discovered and the existence of a third involved gene has been predicted. Recent progress in the field of genetics has allowed the identification of many gene mutation thus facilitating the characterisation of the at risk members of the same family. Complications from bleeding or shunting (pulmonary AVMs) may be sudden and life-threatening (hemothorax, haemoptysis, stroke and brain abscess). Catastrophic events are preventable by early diagnosis and treatment. Appropriate screening programmes are mandatory and multi-specialistic cooperation is needed. Special centers have been developed in the world, where physicians, who are specialised and trained in all aspects of HHT, are working to develop better therapeutic approaches for the disease and to locate new genes in view of the future potential of gene therapy for this condition.
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PMID:The need for an interdisciplinary network of investigations on HHT. 1661 Nov 7

Pulmonary arteriovenous malformation (PAVM) consists of abnormal communications between the pulmonary arteries and veins. Because PAVM may cause neurological defects (such as stroke and brain abscess) or hemoptysis and hemothorax, embolization therapy or surgery is required. Resection using video-assisted thoracic surgery (VATS) has recently been performed for small peripheral PAVMs. For large or hilar PAVM, surgical resection with thoracotomy is required. We report herein a case of PAVM (4.0 x 3.5cm) located in the left S6 segment near the lung hilum that was resected successfully using simultaneously stapled S6 segmentectomy (simultaneous stapling of anomalous vessels of the PAVM and hillar structures of S6) with VATS.
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PMID:Video-assisted simultaneously stapled segmentectomy for pulmonary arteriovenous malformation located in the pulmonary hilum. 1723 59

Hereditary hemorrhagic telangiectasia (HHT) or Rendu-Osler-Weber disease is a genetic disorder with autosomal dominance and variable penetrance, characterized by epistaxis, telangiectasia and visceral manifestations of the disease. The estimated minimal prevalence is 1/10,000 inhabitants. The diagnosis is established on clinical criteria, and may be further confirmed by the identification of causative mutations in either the ENG or the ACVRL1 gene coding for endoglin and ALK1, respectively. Pulmonary vascular manifestations of HHT include pulmonary arteriovenous malformations (PAVMs; especially in patients with ENG mutations) and less frequently pulmonary hypertension (especially in patients with ACVRL1 mutations). In 15-33% of patients with HHT, PAVMs consist of abnormal communications between pulmonary arteries and pulmonary veins, causing right-to-left shunting, and thus, frequently hypoxemia and dyspnea on exertion, although PAVMs may remain asymptomatic and frequently undiagnosed unless complications occur. PAVMs result in severe and frequent complications often at a young age, which may reveal the diagnosis, e.g. transient ischemic attack and cerebral stroke (10-19% of patients), systemic severe infections and abscesses (including cerebral abscess in 5-19% of patients), and rarely massive hemoptysis or hemothorax. Infections in HHT are related to the right-to-left shunting that bypasses the pulmonary capillaries and facilitates the passage of septic or aseptic emboli into the systemic and especially cerebral circulation, and potentially to minor defects in innate immunity. Treatment of PAVMs based on transcatheter coil vaso-occlusion of the feeding artery significantly decreases right-to-left shunting, hypoxemia and dyspnea on exertion, and reduces the risk of systemic complications. Long-term follow-up is warranted after transcatheter vaso-occlusion of PAVMs due to frequent recanalization of treated PAVMs and development or growth of untreated PAVMs. Patients with HHT should be informed of the risk of PAVM and potentially severe complications occurring in heretofore asymptomatic subjects. All adult patients with HHT should be proposed systematic screening for PAVM, by contrast echocardiography (preceded by anteroposterior chest radiograph) or computed tomography of the chest. Pulmonary hypertension is rare in HHT, and may be due either to systemic arteriovenous shunting in the liver increasing cardiac output or be clinically and histologically indistinguishable from idiopathic pulmonary arterial hypertension. Pulmonary hypertension is detected by systematic examination of right cardiac cavities and tricuspid regurgitation flow at echocardiography, and the diagnosis is established by right heart catheterization.
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PMID:Pulmonary vascular manifestations of hereditary hemorrhagic telangiectasia (rendu-osler disease). 1764 82

We report a case of a 32-year-old man who presented with invasive pulmonary aspergillosis and bronchial ulcerations that resulted in massive hemoptysis and concomitant thrombotic microangiopathy shortly after cadaveric renal transplantation. Despite vigorous antifungal therapy the patient succumbed due to apoplexy of brain fungal mass lesion.
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PMID:Aspergillosis after renal transplantation. 1764 1

Pulmonary arteriovenous malformations (PAVM) are high-flow, low-pressure shunts, consisting of a single feeding artery connecting via an aneurysmal sac to a draining vein. The aneurysmal connection is referred to as an aneurysmal sac. The "filter capacity" of the pulmonary capillaries is lost and results in predisposition to brain abscess, stroke, and transient ischemic attack and, when multiple, dyspnea, because of right-to-left shunting and hypoxemia. PAVM are markers of hereditary hemorrhagic telangiectasia (HHT). Up to 30% of patients with HHT have PAVM complicating their disorder. Left untreated, 50% of patients with PAVM will develop disabling or fatal complications. In addition to stroke and transient ischemic attack syndromes due to passage of paradoxical emboli through the PAVM, rupture of the aneurysmal sac, particularly in the third trimester of pregnancy, can lead to fatal hemoptysis or hemothorax. Finally, brain abscess or more obscure musculoskeletal or spinal infections may be secondary to PAVM. Since detachable silicone balloons are no longer available, we have developed precise techniques for closing pulmonary malformations using pushable fibered coils. It is not the coil that is so important, but it is the use of coaxial or triaxial catheters that allow for precise placement of the coil. Cross-sectional occlusion is essential for embolization of PAVM and this is achieved using the "anchor" or "scaffold" technique. Our recent results indicate permanent involution of treated malformations with a 3% recurrence rate. All patients should be assessed for other manifestations of HHT before treatment and they are best followed in one of the 20 HHT Centers worldwide (www.hht.org).
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PMID:Pulmonary arteriovenous malformations: how do I embolize? 1857 43

Pulmonary arteriovenous malformations (PAVMs) can cause stroke, brain abscess or life-threatening haemorrhage. Most PAVMs are associated with hereditary haemorrhagic telangiectasia (HHT). The aim of the present study was to describe the clinical presentation and treatment outcomes of those with idiopathic PAVMs, which has not previously been described in the literature. Patients with idiopathic PAVMs were identified at our HHT centre. Retrospective review of charts and imaging were performed. 20 patients were identified with idiopathic PAVMs. The most common symptoms reported were dyspnoea and migraines (50 and 30% of patients, respectively). Previous complications of PAVMs included haemoptysis (20%), stroke (20%) and brain abscess (5%). A total of 28 focal PAVMs were identified. Most patients (80%) had a solitary PAVM. 13 out of 28 PAVMs (46%) were located in the lower lobes. Most were simple and fistulous rather than complex and plexiform. Transcatheter embolotherapy was performed in 17 patients and was successful in improving oxygenation in all cases. The clinical manifestations and complications of idiopathic PAVMs are similar to those associated with HHT. Idiopathic PAVMs are anatomically similar to HHT-related PAVMs except for a greater number of solitary PAVMs and a lack of lower lobe predominance. Transcatheter embolotherapy is a safe and effective method for treating idiopathic PAVMs.
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PMID:Idiopathic pulmonary arteriovenous malformations: clinical and imaging characteristics. 2117 36

A 52-year-old man with massive hemoptysis underwent a left upper lobectomy. He suddenly reported a severe headache and lost consciousness on postoperative day 4. Computed tomography of the brain demonstrated a subarachnoid hemorrhage due to right vertebral artery dissection. Fortunately, he recovered well with intensive neurosurgical treatment. Vertebral artery dissection sometimes develops following systemic vascular disease, infection, and even minor neck trauma such as hyperextension or torsion of the neck. We should be aware that vertebral artery dissection may cause postoperative stroke and subarachnoid hemorrhage.
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PMID:Subarachnoid hemorrhage due to vertebral artery dissection after lung resection. 2257 27

Hereditary hemorrhagic telangiectasia (HHT) or Rendu-Osler-Weber disease is an autosomic dominant disorder, which is characterized by the development of multiple arteriovenous malformations in either the skin, mucous membranes, and/or visceral organs. Pulmonary arteriovenous malformations (PAVMs) may either rupture, and lead to life-threatening hemoptysis/hemothorax or be responsible for a right-to-left shunting leading to paradoxical embolism, causing stroke or cerebral abscess. PAVMs patients should systematically be screened as the spontaneous complication rate is high, by reaching almost 50%. Neurological complications rate is considerably higher in patients presenting with diffuse pulmonary involvement. PAVM diagnosis is mainly based upon transthoracic contrast echocardiography and CT scanner examination. The latter also allows the planification of treatments to adopt, which consists of percutaneous embolization, having replaced surgery in most of the cases. The anchor technique consists of percutaneous coil embolization of the afferent pulmonary arteries of the PAVM, by firstly placing a coil into a small afferent arterial branch closely upstream the PAVM. Enhanced contrast CT scanner is the key follow-up examination that depicts the PAVM enlargement, indicating the various mechanisms of PAVM reperfusion. When performed by experienced operators as the prime treatment, percutaneous embolization of PAVMs, is a safe, efficient and sustained therapy in the great majority of HHT patients.
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PMID:Diagnosis and treatment of pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: An overview. 2376 87

Computed tomography (CT)-guided transthoracic lung biopsy is a common procedure for the diagnosis of pulmonary lesion. Pneumothorax, pulmonary hemorrhage and hemoptysis are the most common complications of the procedure. Air embolism is a rare serious complication. We reported a case with air embolism related acute ischemic stroke and non-ST elevation myocardial infarction (NSTEMI) simultaneously after percutaneous transthoracic lung biopsy.
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PMID:Systemic air embolism causing acute stroke and myocardial infarction after percutaneous transthoracic lung biopsy-a case report. 2637 39

Iron supplements such as ferrous sulfate tablets are usually used to treat iron-deficiency anemia in some elderly patients with primary neurologic disorders or decreased gag reflexes due to stroke, senile dementia, or parkinsonism. While the aspiration of ferrous sulfate is rarely reported, it is a potentially life-threatening condition that can lead to airway necrosis and bronchial stenosis. A detailed history and high suspicion of aspiration are required to avoid delays in diagnosis and treatment. The diagnosis can be confirmed by bronchoscopic examination and a tissue biopsy. Early removal of the aspirated tablet prevents acute complications, such as bronchial necrosis, hemoptysis, and lobar consolidation. Tablet removal is also necessary to prevent late bronchial stenosis. We presented the first case in Korea of a ferrous sulfate tablet aspiration that induced severe endobronchial inflammation.
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PMID:Severe Endobronchial Inflammation Induced by Aspiration of a Ferrous Sulfate Tablet. 2677 Feb 33


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