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Pulmonary Capillary Hemangiomatosis
Pulmonary capillary hemangiomatosis (PCH) is a disease affecting the blood vessels of the lungs, where abnormal capillary proliferation and venous fibrous intimal thickening result in progressive increase in vascular resistance. It is a rare cause of pulmonary hypertension, and occurs predominantly in young adults. Together with Pulmonary venoocclusive disease, pulmonary veno-occlusive disease, PCH comprises WHO Group I' causes for pulmonary hypertension. Indeed, there is some evidence to suggest that PCH and pulmonary veno-occlusive disease are different forms of a similar disease process. Presentation These are non specific. Typical symptoms include dyspnea, cough, chest pain and fatigue.Chaisson NF, Dodson MW, Elliott CG (2016) Pulmonary Capillary Hemangiomatosis and Pulmonary Veno-occlusive Disease. Clin Chest Med 37(3):523-534 Genetics At least some cases appear to be due to mutations in the eukaryotic translation initiation factor 2-alpha kinase 4 (EIF2AK4) gene.Best DH, Su ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Pulmonary Venoocclusive Disease
Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension caused by progressive blockage of the small veins in the lungs. The blockage leads to high blood pressures in the arteries of the lungs, which, in turn, leads to heart failure. The disease is progressive and fatal, with median survival of about 2 years from the time of diagnosis to death. The definitive therapy is lung transplantation. Signs and symptoms The symptoms for pulmonary veno-occlusive disease are the following: Cause The genetic cause of pulmonary veno-occlusive disease is mutations in EIF2AK4 gene. Though this does not mean other possible causes do not exist, such as viral infection and risk of toxic chemicals (chemotherapy drugs). Pathophysiology Pulmonary veno-occlusive disease may have a genetic basis. Published reports have indicated fatal occurrences that appeared to possess a familial pattern, more to the point, a germline mutation. The pathophysiology of veno-occlusive disease ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Dyspnea
Shortness of breath (SOB), also medically known as dyspnea (in AmE) or dyspnoea (in BrE), is an uncomfortable feeling of not being able to breathing, breathe well enough. The American Thoracic Society defines it as "a subjective experience of breathing discomfort that consists of qualitatively distinct sensations that vary in intensity", and recommends evaluating dyspnea by assessing the intensity of its distinct sensations, the degree of distress and discomfort involved, and its burden or impact on the patient's activities of daily living. Distinct sensations include effort/work to breathe, chest tightness or pain, and "air hunger" (the feeling of not enough oxygen). The tripod position is often assumed to be a sign. Dyspnea is a normal symptom of heavy physical exertion but becomes disease, pathological if it occurs in unexpected situations, when resting or during light exertion. In 85% of cases it is due to asthma, pneumonia, cardiac ischemia, interstitial lung disease, congesti ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Cough
A cough is a sudden expulsion of air through the large breathing passages that can help clear them of fluids, irritants, foreign particles and microbes. As a protective reflex, coughing can be repetitive with the cough reflex following three phases: an inhalation, a forced exhalation against a closed glottis, and a violent release of air from the lungs following opening of the glottis, usually accompanied by a distinctive sound. Frequent coughing usually indicates the presence of a disease. Many viruses and bacteria benefit, from an evolutionary perspective, by causing the host to cough, which helps to spread the disease to new hosts. Most of the time, irregular coughing is caused by a respiratory tract infection but can also be triggered by choking, smoking, air pollution, asthma, gastroesophageal reflux disease, post-nasal drip, chronic bronchitis, lung tumors, heart failure and medications such as angiotensin-converting-enzyme inhibitors (ACE inhibitors). Treatment should t ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
EIF2AK4
Eukaryotic translation initiation factor 2-alpha kinase 4 is an enzyme that in humans is encoded by the ''EIF2AK4'' gene. EIF2AK4 belongs to a family of kinases that phosphorylate the alpha subunit of eukaryotic translation initiation factor-2 (EIF2S1; MIM 603907) to downregulate protein synthesis in response to varied cellular stresses (Berlanga et al., 1999). upplied by OMIMref name="entrez"/> See also * Gcn2 GCN2 (general control nonderepressible 2) is a serine/threonine-protein kinase that senses amino acid deficiency through binding to uncharged transfer RNA (tRNA). It plays a key role in modulating amino acid metabolism as a response to nutrient d ... References Further reading * * * * * * * * External links * EC 2.7.11 {{gene-15-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Echocardiography
An echocardiography, echocardiogram, cardiac echo or simply an echo, is an ultrasound of the heart. It is a type of medical imaging of the heart, using standard ultrasound or Doppler ultrasound. Echocardiography has become routinely used in the diagnosis, management, and follow-up of patients with any suspected or known heart diseases. It is one of the most widely used diagnostic imaging modalities in cardiology. It can provide a wealth of helpful information, including the size and shape of the heart (internal chamber size quantification), pumping capacity, location and extent of any tissue damage, and assessment of valves. An echocardiogram can also give physicians other estimates of heart function, such as a calculation of the cardiac output, ejection fraction, and diastolic function (how well the heart relaxes). Echocardiography is an important tool in assessing wall motion abnormality in patients with suspected cardiac disease. It is a tool which helps in reaching an ear ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Pulmonary Angiography
Pulmonary angiography (or pulmonary arteriography,conventional pulmonary angiography, selective pulmonary angiography) is a medical fluoroscopic procedure used to visualize the pulmonary arteries and much less frequently, the pulmonary veins. It is a minimally invasive procedure performed most frequently by an interventional radiologist or interventional cardiologist to visualise the arteries of the lungs. Uses Pulmonary angiography is useful as the confirmation test where the non-invasive imaging such as CT pulmonary angiography is inconclusive on determining the presence of pulmonary embolism. The accuracy of pulmonary angiography may be higher than clinical examination, arterial blood gas results, and ventilation/perfusion scan. Pulmonary angiography is also used to confirm chronic thromboembolic pulmonary hypertension (CTEPH) and provides a platform for balloon pulmonary angioplasty to treat the disease. CT pulmonary angiography has nearly entirely replaced conventional pul ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Pulmonary Veno-occlusive Disease
Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension caused by progressive blockage of the small veins in the lungs. The blockage leads to high blood pressures in the arteries of the lungs, which, in turn, leads to heart failure. The disease is progressive and fatal, with median survival of about 2 years from the time of diagnosis to death. The definitive therapy is lung transplantation. Signs and symptoms The symptoms for pulmonary veno-occlusive disease are the following: Cause The genetic cause of pulmonary veno-occlusive disease is mutations in EIF2AK4 gene. Though this does not mean other possible causes do not exist, such as viral infection and risk of toxic chemicals (chemotherapy drugs). Pathophysiology Pulmonary veno-occlusive disease may have a genetic basis. Published reports have indicated fatal occurrences that appeared to possess a familial pattern, more to the point, a germline mutation. The pathophysiology of veno-occlusive disease ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Ehlers Danlos Syndrome
Ehlers may refer to: * Ehlers–Danlos syndrome * Ehlers (surname) Ehlers is a German surname. Notable people with the surname include: * Arthur Ehlers, executive in minor and Major League Baseball * Beth Ehlers (born 1968), American actress * Brian Ehlers (born 1978), American professional basketball player * B ... {{disambig ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
CREST Syndrome
CREST syndrome, also known as the limited cutaneous form of systemic sclerosis (lcSSc), is a multisystem connective tissue disorder. The acronym "CREST" refers to the five main features: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia. CREST syndrome is associated with detectable antibodies against centromeres (a component of the cell nucleus), and usually spares the kidneys (a feature more common in the related condition systemic scleroderma). If the lungs are involved, it is usually in the form of pulmonary arterial hypertension. Signs and symptoms Calcinosis CREST causes thickening and tightening of the skin with deposition of calcific nodules ("calcinosis"). Raynaud's phenomenon Raynaud's phenomenon is frequently the first manifestation of CREST/lcSSc, preceding other symptoms by years. Stress and cold temperature induce an exaggerated vasoconstriction of the small arteries, arterioles, and thermoregulatory vessels of the skin ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Scimitar Syndrome
Scimitar syndrome, or congenital pulmonary venolobar syndrome, is a rare congenital heart defect characterized by anomalous venous return from the right lung (to the systemic venous drainage, rather than directly to the left atrium). This anomalous pulmonary venous return can be either partial (PAPVR) or total (TAPVR). The syndrome associated with PAPVR is more commonly known as ''Scimitar syndrome'' after the curvilinear pattern created on a chest radiograph by the pulmonary veins that drain to the inferior vena cava. This radiographic density often has the shape of a scimitar, a type of curved sword. The syndrome was first described by Catherine Neill in 1960. Presentation The anomalous venous return forms a curved shadow on chest x-ray such that it resembles a scimitar. This is called the Scimitar Sign. Associated abnormalities include right lung hypoplasia with associated dextroposition of the heart, pulmonary artery hypoplasia and pulmonary sequestration. Incidence is around 1 ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Lung Transplantation
Lung transplantation, or pulmonary transplantation, is a surgical procedure in which one or both lungs are replaced by lungs from a donor. Donor lungs can be retrieved from a living or deceased donor. A living donor can only donate one lung lobe. With some lung diseases, a recipient may only need to receive a single lung. With other lung diseases such as cystic fibrosis, it is imperative that a recipient receive two lungs. While lung transplants carry certain associated risks, they can also extend life expectancy and enhance the quality of life for those with end stage pulmonary disease. Qualifying conditions Lung transplantation is the therapeutic measure of last resort for patients with end-stage lung disease who have exhausted all other available treatments without improvement. A variety of conditions may make such surgery necessary. As of 2005, the most common reasons for lung transplantation in the United States were: * 27% chronic obstructive pulmonary disease (COPD), inclu ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
