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A history usually reveals gradual onset of shortness of breath, fatigue, angina pectoris, syncope (fainting) and peripheral edema.
In order to establish the cause, the physician will generally conduct a thorough medical history and physical examination. A detailed family history is taken to determine whether the disease might be familial.
Normal pulmonary arterial pressure in a person living at sea level has a mean value of 12-16mmHg. Definite pulmonary hypertension is present when mean pressures at rest exceed 25 mmHg. Although pulmonary arterial pressure can be estimated on the basis of echocardiography, pressure sampling with a Swan-Ganz catheter provides the most definite measurement.
Diagnostic tests generally involve blood tests, electrocardiography, arterial blood gas measurements, X-rays of the chest (generally followed by high-resolution CT scanning). A biopsyA biopsy is a medical test involving the removal of cells or tissues for examination. The tissue is often examined under a microscope and can also be analyzed chemically (for example, using PCR techniques). When only a sample of tissue is removed, the pro of lung tissue, angiography with endoluminal biopsy of the pulmonary artery, or biopsyA biopsy is a medical test involving the removal of cells or tissues for examination. The tissue is often examined under a microscope and can also be analyzed chemically (for example, using PCR techniques). When only a sample of tissue is removed, the pro of any associated skin lesions, is often attempted to obtain tissue for histopathological investigation.
Clinical improvement is often measured in a "six-minute walking test", i.e. the distance a patient can walk in six minutes.
Pulmonary hypertension can be primary (occurring without an obvious cause) or secondary (a result of other disease processes.)
Primary pulmonary hypertension (PPH) is considered a genetic disorderA genetic disorder or genetic disease is a disease caused, at least in part, by the genes of the person with the disease. There are a number of possible causes for genetic defects: They may be caused by an unwelcome mutation, as are most cancers. There ar. It has been linked to mutations in the BMPR2 gene, which encodes a receptorIn biochemistry, a receptor is a protein on the cell membrane or within the cytoplasm that binds to a specific factor (a ligand), such as a neurotransmitter, hormone, or other substance, and initiates the cellular response to the ligand. As all receptors for bone morphogenic proteins (Deng et al, 2000This page is about the year 2000. See 2000 AD for the UK comic book, Number 2000 for other uses. 2000 is a leap year starting on Saturday (see link for calendar), and also the International Year for a Culture of Peace''. Events Y2K passes without the seri), as well as the 5-HT(2B) gene, which codes for a serotoninSerotonin Chemical name5-Hydroxy-tryptamine or3-(2-aminoethyl)-1''H indol-5-ol Chemical formulaCHNO Molecular mass176. 22 g/mol CAS number50-67-9 SMILESNCCC1 CNC2 C1C C(O)C C2 Serotonin 5-hydroxytryptamine or 5-HT is a monoamine neurotransmitter synthesis receptor (Blanpain et al, 2003). Recently, characteristic proteins of human herpesvirus 8 (also known for causing Kaposi sarcoma) were identified in vascular lesions of PPH patients (Cool et al, 2003). However, it is not understood what roles these genes and viral particles play in PPH. PPH has also been associated to the use of appetite suppressants (e.g. Fen-phen, see Abenhaim et al, 1996). While genetic susceptibility to adverse drug reactions is suspected, the cause of the disease is still largely unknown.
PPH is very rare but often fatal. Patients usually have no symptoms until they reach their late twenties or early thirties. It is characterized by elevated pulmonary vascular resistance attributable to the abnormal thickening of the vessel wall and narrowing of the lumen of arterioles in the lungs.
Secondary pulmonary hypertension (SPH) is often due to chronic obstructive pulmonary disease (COPD). Other factors that have been linked to secondary pulmonary hypertension are:
A common consequence of chronic pulmonary hypertension is cor pulmonale (right sided heart failure) believed to be caused by the increased load on the right ventricle and atrium of the heart. A chest radiograph (X-ray) will often reveal an enlarged right atrium and ventricle, and prominent pulmonary arteries. An ECG will often demonstrate right ventricular hypertrophy or strain. Oedema and fluid retention follow.
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