query: What is pulmonary fibrosis?

title: Pulmonary insufficiency

text: secondary pulmonary hypertension due to thromboembolism). Furthermore, pulmonary insufficiency is generally treated by addressing the underlying condition, in certain cases, the pulmonary valve may be surgically replaced. Pulmonary insufficiency Pulmonary insufficiency (or incompetence, or regurgitation) is a condition in which the pulmonary valve is incompetent and allows backflow from the pulmonary artery to the right ventricle of the heart during diastole. While a small amount of backflow may occur ordinarily, it is usually only shown on an echocardiogram and is harmless. More pronounced regurgitation that is noticed through a routine physical examination is a medical sign of disease and warrants

title: Pulmonary fibrosis

text: the "TERC" or "TERT" genes, which encode telomerase, have been identified in about 15 percent of pulmonary fibrosis patients. Diseases and conditions that may cause pulmonary fibrosis as a secondary effect include: Pulmonary fibrosis involves gradual exchange of normal lung parenchyma with fibrotic tissue. The replacement of normal lung with scar tissue causes irreversible decrease in oxygen diffusion capacity, and the resulting stiffness or decreased compliance makes pulmonary fibrosis a restrictive lung disease. Pulmonary fibrosis is perpetuated by aberrant wound healing, rather than chronic inflammation. It is the main cause of restrictive lung disease that is intrinsic to the lung

title: Pulmonary fibrosis

text: and fifties when diagnosed while the incidence of idiopathic pulmonary fibrosis increases dramatically after the age of fifty. However, loss of pulmonary function is commonly ascribed to old age, heart disease or to more common lung diseases. Pulmonary fibrosis Pulmonary fibrosis (literally "scarring of the lungs") is a respiratory disease in which scars are formed in the lung tissues, leading to serious breathing problems. Scar formation, the accumulation of excess fibrous connective tissue (the process called fibrosis), leads to thickening of the walls, and causes reduced oxygen supply in the blood. As a consequence patients suffer from perpetual shortness of

title: Pulmonary fibrosis

text: damage in the lung due to pulmonary fibrosis. Symptoms of pulmonary fibrosis are mainly: Pulmonary fibrosis is suggested by a history of progressive shortness of breath (dyspnea) with exertion. Sometimes fine inspiratory crackles can be heard at the lung bases on auscultation. A chest X-ray may or may not be abnormal, but high-resolution CT will frequently demonstrate abnormalities. Pulmonary fibrosis may be a secondary effect of other diseases. Most of these are classified as interstitial lung diseases. Examples include autoimmune disorders, viral infections and bacterial infection like tuberculosis which may cause fibrotic changes in both lung's upper or lower lobes

title: Pulmonary fibrosis

text: FVC (forced vital capacity) are reduced so the FEV1/FVC ratio is normal or even increased in contrast to obstructive lung disease where this ratio is reduced. The values for residual volume and total lung capacity are generally decreased in restrictive lung disease. Pulmonary fibrosis creates scar tissue. The scarring is permanent once it has developed. Slowing the progression and prevention depends on the underlying cause: Hypoxia caused by pulmonary fibrosis can lead to pulmonary hypertension, which, in turn, can lead to heart failure of the right ventricle. Hypoxia can be prevented with oxygen supplementation. Pulmonary fibrosis may also result in

title: Pulmonary fibrosis

text: Pulmonary fibrosis Pulmonary fibrosis (literally "scarring of the lungs") is a respiratory disease in which scars are formed in the lung tissues, leading to serious breathing problems. Scar formation, the accumulation of excess fibrous connective tissue (the process called fibrosis), leads to thickening of the walls, and causes reduced oxygen supply in the blood. As a consequence patients suffer from perpetual shortness of breath. In some patients the specific cause of the disease can be diagnosed, but in others the probable cause cannot be determined, a condition called idiopathic pulmonary fibrosis. There is no known cure for the scars and

title: Pulmonary fibrosis

text: and other microscopic injuries to the lung. However, pulmonary fibrosis can also appear without any known cause. In this case, it is termed "idiopathic". Most idiopathic cases are diagnosed as "idiopathic pulmonary fibrosis". This is a diagnosis of exclusion of a characteristic set of histologic/pathologic features known as usual interstitial pneumonia (UIP). In either case, there is a growing body of evidence which points to a genetic predisposition in a subset of patients. For example, a mutation in surfactant protein C (SP-C) has been found to exist in some families with a history of pulmonary fibrosis. Autosomal dominant mutations in

title: Pulmonary fibrosis

text: an increased risk for pulmonary emboli, which can be prevented by anticoagulants. Five million people worldwide are affected by pulmonary fibrosis. A wide range of incidence and prevalence rates have been reported for pulmonary fibrosis. The rates below are per 100,000 persons, and the ranges reflect narrow and broad inclusion criteria, respectively. Based on these rates, pulmonary fibrosis prevalence in the United States could range from more than 29,000 to almost 132,000, based on the population in 2000 that was 18 years or older. The actual numbers may be significantly higher due to misdiagnosis. Typically, patients are in their forties

title: Pulmonary fibrosis

text: of other features that may indicate a specific cause e.g. specific types of mineral dust or possible response to therapy e.g. a pattern of so-called non-specific interstitial fibrosis. Misdiagnosis is common because, while overall pulmonary fibrosis is not rare, each individual type of pulmonary fibrosis is uncommon and the evaluation of patients with these diseases is complex and requires a multidisciplinary approach. Terminology has been standardized but difficulties still exist in their application. Even experts may disagree with the classification of some cases. On spirometry, as a restrictive lung disease, both the FEV1 (forced expiratory volume in 1 second) and

title: Idiopathic pulmonary fibrosis

text: Idiopathic pulmonary fibrosis Idiopathic pulmonary fibrosis (IPF) is a type of chronic lung disease characterized by a progressive and irreversible decline in lung function. Symptoms typically include gradual onset of shortness of breath and a dry cough. Other changes may include feeling tired and nail clubbing. Complications may include pulmonary hypertension, heart failure, pneumonia, or pulmonary embolism. The cause is unknown. Risk factors include cigarette smoking, certain viral infections, and a family history of the condition. The underlying mechanism involves scarring of the lungs. Diagnosis requires ruling out other potential causes and may be supported by a CT scan or

title: Idiopathic pulmonary fibrosis

text: also ongoing. These molecules are directed against several growth factors and cytokines that are known to play a role in the proliferation, activation, differentiation or inappropriate survival of fibroblasts. mir-29 microRNA precursor investigations in mice have produced reversal of induced IPF. MRG-201 is currently being tested as-of 2016, but not in IPF patients yet, and no human trials for IPF use have been scheduled as-of January, 2016. Stem cell therapies for IPF are an active area of research. Idiopathic pulmonary fibrosis Idiopathic pulmonary fibrosis (IPF) is a type of chronic lung disease characterized by a progressive and irreversible decline in

title: Pulmonary fibrosis

text: parenchyma. In contrast, quadriplegia and kyphosis are examples of causes of restrictive lung disease that do not necessarily involve pulmonary fibrosis. The diagnosis can be confirmed by lung biopsy. A videoscopic assisted thoracoscopic wedge biopsy (VATS) under general anesthesia may be necessary to obtain enough tissue to make an accurate diagnosis. This kind of biopsy involves placement of several tubes through the chest wall, one of which is used to cut off a piece of lung to send for evaluation. The removed tissue is examined histopathologically by microscopy to confirm the presence and pattern of fibrosis as well as presence

title: Idiopathic pulmonary fibrosis

text: oxygen levels at rest. Although oxygen therapy has not been shown to improve survival in IPF, some data indicate an improvement in exercise capacity. Fatigue and loss of muscular mass are common and disabling problems for patients with IPF. Pulmonary rehabilitation may alleviate the overt symptoms of IPF and improve functional status by stabilizing and/or reversing the extrapulmonary features of the disease. The number of published studies on the role of pulmonary rehabilitation in idiopathic pulmonary fibrosis is small, but most of these studies have found significant short-term improvements in functional exercise tolerance, quality of life, and dyspnea on exertion.

title: Idiopathic pulmonary fibrosis

text: about twofold. Other environmental and occupation exposures such as exposure to metal dust, wood dust, coal dust, silica, stone dust, biologic dusts coming from hay dust or mold spores or other agricultural products, and occupations related to farming/livestock have also been shown to increase the risk for IPF. There is some evidence that viral infections may be associated with idiopathic pulmonary fibrosis and other fibrotic lung diseases. Despite extensive investigation, the cause of IPF remains unknown. The fibrosis in IPF has been linked to cigarette smoking, environmental factors (e.g. occupational exposure to gases, smoke, chemicals or dusts), other medical conditions

title: Idiopathic pulmonary fibrosis

text: with the diagnosis of IPF or whether the findings are due to another process. It has long been recognized that patients with ILD related to asbestos exposure, drugs (such as chemotherapeutic agents or nitrofurantoin), rheumatoid arthritis and scleroderma/systemic sclerosis may be difficult to distinguish from IPF. Other differential diagnostic considerations include interstitial lung disease related to mixed connective tissue disease, advanced sarcoidosis, chronic hypersensitivity pneumonitis, pulmonary Langerhan’s cell histiocytosis and radiation-induced lung injury. Idiopathic pulmonary fibrosis (IPF) belongs to a large group of more than 200 lung diseases known as interstitial lung diseases (ILDs), which are characterized by the involvement

title: Idiopathic pulmonary fibrosis

text: C (SFTPA1, SFTPA2B) and mucin (MUC5B). A remarkable aspect of the MUC5B variant is its high frequency of detection, as it is found in approximately 20% of individuals with Northern and Western European ancestry and in 19% of the Framingham Heart Study population. Mutations in human telomerase genes are also associated with familial pulmonary fibrosis and in some patients with sporadic IPF (e.g. the TERT, TERC genes). Recently an X-linked mutation in a third telomerase-associated gene, dyskerin (DKC1), has been described in a family with IPF. An earlier diagnosis of IPF is a prerequisite for earlier treatment and, potentially, improvement

title: Idiopathic pulmonary fibrosis

text: including gastroesophageal reflux disease (GERD), or to genetic predisposition (familial IPF). However, none of these is present in all people with IPF and therefore do not provide a completely satisfactory explanation for the disease. IPF is believed to be the result of an aberrant wound healing process including/involving abnormal and excessive deposition of collagen (fibrosis) in the pulmonary interstitium with minimal associated inflammation. It is hypothesized that the initial or repetitive injury in IPF occurs to the lung cells, called alveolar epithelial cells (AECs, pneumocytes), which line the majority of the alveolar surface. When type I AECs are damaged or

title: Idiopathic pulmonary fibrosis

text: pulmonary fibrosis, which leads to increased lung elastic recoil. Measurement of static lung volumes using body plethysmography or other techniques typically reveals reduced lung volumes (restriction). This reflects the difficulty encountered in inflating the fibrotic lungs. The diffusing capacity for carbon monoxide (DL) is invariably reduced in IPF and may be the only abnormality in mild or early disease. Its impairment underlies the propensity of patients with IPF to exhibit oxygen desaturation with exercise which can also be evaluated using the 6-minute walk test (6MWT). Terms such as ‘mild’, ‘moderate’, and ‘severe’ are sometimes used for staging disease and are

title: Idiopathic pulmonary fibrosis

text: the NIH announced in 2012 that the triple-therapy arm of the PANTHER-IPF study had been terminated early. This study also evaluated NAC alone and the results for this arm of the study were published in May 2014 in the New England Journal of Medicine, concluding that "as compared with placebo, acetylcysteine offered no significant benefit with respect to the preservation of FVC in patients with idiopathic pulmonary fibrosis with mild-to-moderate impairment in lung function". Nintedanib is an investigational orally-administered triple angiokinase inhibitor that targets receptor tyrosine kinases involved in the regulation of angiogenesis: fibroblast growth factor receptor (FGFR), platelet-derived growth

title: Pulmonary rehabilitation

text: is generally specific to the individual patient, with the objective of meeting the needs of the patient. It is a broad program and may benefit patients with lung diseases such as chronic obstructive pulmonary disease (COPD), sarcoidosis, idiopathic pulmonary fibrosis (IPF) and cystic fibrosis, among others. Although the process is focused on the rehabilitation of the patient him/herself, the family is also involved. The process typically does not begin until a medical exam of the patient has been performed by a licensed physician. The setting of pulmonary rehabilitation varies by patient; settings may include inpatient care, outpatient care, the office

title: Pulmonary function testing

text: (FIFs), and MVV. Measuring pulmonary mechanics assesses the ability of the lungs to move huge volumes of air quickly through the airways to identify airway obstruction. The measurements taken by the spirometry device are used to generate a pneumotachograph that can help to assess lung conditions such as: asthma, pulmonary fibrosis, cystic fibrosis, and chronic obstructive pulmonary disease. Physicians may also use the test results to diagnose bronchial hyperresponsiveness to exercise, cold air, or pharmaceutical agents. Spirometry is a safe procedure; however, there is cause for concern regarding untoward reactions. The value of the test data should be weighed against

title: Idiopathic pulmonary fibrosis

text: often corresponds to a clinical diagnosis of IPF, a UIP histologic pattern can be seen in other diseases as well, and fibrosis of known origin (rheumatic diseases for example). There are four key features of UIP including interstitial fibrosis in a ‘patchwork pattern’, interstitial scarring, honeycomb changes and fibroblast foci. Fibroblastic foci are dense collections of myofibroblasts and scar tissue and, together with honeycombing, are the main pathological findings that allow a diagnosis of UIP. Bronchoalveolar lavage (BAL) is a well-tolerated diagnostic procedure in ILD. BAL cytology analyses (differential cell counts) should be considered in the evaluation of patients with

title: Idiopathic pulmonary fibrosis

text: commonly based on resting pulmonary function test measurements. However, there is no clear consensus regarding the staging of IPF patients and what are the best criteria and values to use. Mild-to-moderate IPF has been characterized by the following functional criteria: The goals of treatment in IPF are essentially to reduce the symptoms, stop disease progression, prevent acute exacerbations, and prolong survival. Preventive care (e.g. vaccinations) and symptom-based treatment should be started early in every patient. In the 2011 IPF guidelines, oxygen therapy, or supplementary oxygen for home use, became a strong recommendation for use in those patients with significantly low

title: Cystic fibrosis

text: help push air into their lungs. These machines, known as bilevel positive airway pressure (BiPAP) ventilators, help prevent low blood oxygen levels during sleep. Non-invasive ventilators may be used during physical therapy to improve sputum clearance. It is not known if this type of therapy has an impact on pulmonary exacerbations or disease progression. It is not known what role non-invasive ventilation therapy has for improving exercise capacity in people with cystic fibrosis. During severe illness, a tube may be placed in the throat (a procedure known as a tracheostomy) to enable breathing supported by a ventilator. For children, preliminary

title: Pulmonary toxicity

text: lung fibrosis, organising pneumonia (bronchiolitis obliterans organising pneumonia, BOOP), ARDS (acute respiratory distress syndrome), solitary pulmonary mass (even including lung cancer in some cases, mainly in cases of asbestos-related lung disease, but today this is very rare, because asbestos is now completely prohibited by law in most countries), or pulmonary nodule. The diagnosis should be made by a specialist, if possible. Lung symptoms in a patient who is taking a medicinal drug that can cause pulmonary toxicity should not automatically lead to a diagnosis of "pulmonary toxicity due to the medicinal drug", because some patients can have another (i.e., simultaneous)

title: Idiopathic pulmonary fibrosis

text: ATS, ERS, together with the Japanese Respiratory Society (JRS) and Latin American Thoracic Association (ALAT). Currently, a diagnosis of IPF requires: In the right clinical setting, it is possible to make the diagnosis of IPF by HRCT alone, obviating the need for surgical lung biopsy.<ref name="ATS/ERS"></ref> Recognizing IPF in clinical practice can be challenging as symptoms often appear similar to those of more common diseases, such as asthma, chronic obstructive pulmonary disease (COPD) and congestive heart failure (www.diagnoseipf.com). The key issue facing clinicians is whether the presenting history, symptoms (or signs), radiology, and pulmonary function testing are collectively in keeping

title: Cystic fibrosis

text: would have been unlikely to live beyond their first year, infants today are likely to live well into adulthood. Recent advances in the treatment of cystic fibrosis have meant that individuals with cystic fibrosis can live a fuller life less encumbered by their condition. The cornerstones of management are the proactive treatment of airway infection, and encouragement of good nutrition and an active lifestyle. Pulmonary rehabilitation as a management of CF continues throughout a person's life, and is aimed at maximizing organ function, and therefore the quality of life. At best, current treatments delay the decline in organ function. Because

title: Cardiac fibrosis

text: (which in combination with phentermine was marketed as "Pondimin" and commonly referred to as fen-phen), chlorphentermine, and aminorex (along with its analogue 4-Methylaminorex which has seen sporadic use as a recreational drug) induce a similar pattern of cardiac fibrosis (and pulmonary hypertension), apparently by over-stimulating 5HT receptors on the cardiac fibroblast cells. These drugs consequently tend to cause increased risk of heart valve damage and subsequent heart failure, which eventually led to them being withdrawn from the market. Certain antimigraine drugs which are targeted at serotonin receptors as vasoconstrictive agents, have long been known to be associated with pulmonary hypertension

title: Pulmonary hygiene

text: acute respiratory distress syndrome after chest trauma. The need for bronchial hygiene is indicated in cases of COPD, pneumonia and cystic fibrosis as both interventional and prophylactic. Prophylactic indications also include pre and post thoracic surgery to prevent atelectasis and respiratory infections. The decision to use postural drainage therapy requires assessment of potential benefits versus potential risks. Therapy should be provided for no longer than necessary to obtain the desired therapeutic results. Some of the contraindications include an increased intracranial pressure (>20 mmHg), any spinal injury acute or otherwise, active hemoptysis, pulmonary embolism, pulmonary edema with congestive heart failure and

title: Idiopathic pulmonary fibrosis

text: or infection need to be excluded. Pulmonary infection have to be ruled out by endotracheal aspirate or BAL. Many patients experiencing acute deterioration require intensive care treatment, particularly when respiratory failure is associated with hemodynamic instability, significant comorbidities or severe hypoxemia. However, mortality during hospitalization is high. Mechanical ventilation should be introduced only after carefully weighing the patient’s long-term prognosis and, whenever possible, the patient’s wishes. However, current guidelines discourage the use of mechanical ventilation in patients with respiratory failure secondary to IPF. The clinical course of IPF can be unpredictable. IPF progression is associated with an estimated median survival

title: Idiopathic pulmonary fibrosis

text: factor receptor (PDGFR), and vascular endothelial growth factor receptor (VEGFR), which have also been implicated in the pathogenesis of fibrosis and IPF. In both phase III trials, nintedanib significantly reduced the decline in lung function by approximately 50% over one year. Lung transplantation may be suitable for those patients physically eligible to undergo a major transplant operation. In IPF patients, lung transplant has been shown to reduce the risk of death by 75% as compared with patients who remain on the waiting list. Since the introduction of the lung allocation score (LAS), which prioritizes transplant candidates based on survival probability,

title: Cardiovascular & pulmonary physiotherapy

text: Cardiovascular & pulmonary physiotherapy Physiotherapists treating patients following uncomplicated coronary artery bypass surgery (also called coronary artery bypass graft surgery, or CABG) surgery continue to use interventions such as deep breathing exercises that are not supported by best available evidence. Standardised guidelines may be required to better match clinical practice with current literature. Cystic fibrosis (CF), also known as mucoviscidosis, is a genetic disorder that affects mostly the lungs, but also the pancreas, liver, kidneys, and intestines. Major advances over the past few years in the management of cystic fibrosis (CF) have resulted in dramatic improvements in longevity and quality

title: Idiopathic pulmonary fibrosis

text: the accumulation of inflammatory cells and the consequent deposition of collagen. This pathogenetic model is indirectly supported by the clinical features of IPF, including an insidious onset, progression over several years, relatively infrequent acute exacerbations, and failure to respond to immunosuppressive therapy. A number of therapies that target fibroblast activation or the synthesis of extracellular matrix are currently in early testing or are being considered for development. Familial IPF accounts for less than 5% of the total of patients with IPF and is clinically and histologically indistinguishable from sporadic IPF. Genetic associations include mutations in pulmonary surfactant proteins A1, A2,

title: Idiopathic pulmonary fibrosis

text: lung biopsy. It is a type of interstitial lung disease (ILD). People often benefit from pulmonary rehabilitation and supplemental oxygen. Certain medications like pirfenidone or nintedanib may slow the progression of the disease. Lung transplantation may also be an option. About 5 million people are affected globally. The disease newly occurs in about 12 per 100,000 people per year. Those in their 60s and 70s are most commonly affected. Males are affected more often than females. Average life expectancy following diagnosis is about four years. In many people, symptoms are present for a considerable time before diagnosis. The most common

title: Pulmonary alveolar microlithiasis

text: Pulmonary alveolar microlithiasis Pulmonary alveolar microlithiasis (PAM) is a rare, inherited disorder of lung phosphate balance that is associated with small stone formation in the airspaces of the lung. Mutations in the gene "SLC34A2" result in loss of a key sodium, phosphate co-transporter (called Npt2b), known to be expressed in distal airway epithelial alveolar type II cells, as well as in the mammary gland, and to a lesser extent in intestine, kidney, skin, prostate and testes. As the disease progresses, the lung fields become progressively more dense (white) on the chest xray, and low oxygen level, lung inflammation and fibrosis,

title: Pulmonary function testing

text: good index of physical function and therapeutic response in patients with chronic lung disease, such as COPD or idiopathic pulmonary fibrosis. Arterial blood gases (ABGs) are a helpful measurement in pulmonary function testing in selected patients. The primary role of measuring ABGs in individuals that are healthy and stable is to confirm hypoventilation when it is suspected on the basis of medical history, such as respiratory muscle weakness or advanced COPD. ABGs also provide a more detailed assessment of the severity of hypoxemia in patients who have low normal oxyhemoglobin saturation. The helium dilution technique for measuring lung volumes uses

title: Idiopathic pulmonary fibrosis

text: conditions frequently associated with IPF requires a routinely evaluation of comorbidities, most of them simply reflecting concurrent diseases of aging, and medications with their interaction and side effects. Acute exacerbations of IPF (AE-IPF) are defined as an unexplained worsening or development of dyspnea within 30 days with new radiological infiltrates at HRCT abnormality often superimposed on a background consistent with UIP pattern. The yearly incidence of AE-IPF is between 10 and 15% of all patients. The prognosis of AE-IPF is poor, with mortality ranging from 78% to 96%. Other causes of AE-IPF such as pulmonary embolism, congestive heart failure, pneumothorax,

title: Idiopathic pulmonary fibrosis

text: clinical features of IPF include the following: Some of these features are due to chronic hypoxemia (oxygen deficiency in the blood), are not specific for IPF, and can occur in other pulmonary disorders. IPF should be considered in all patients with unexplained chronic exertional dyspnea who present with cough, inspiratory bibasilar crackles, or finger clubbing. Assessment of "velcro" crackles on lung auscultation is a practical way to improve the earlier diagnosis of IPF. Fine crackles are easily recognized by clinicians and are characteristic of IPF. If bilateral fine crackles are present throughout the inspiratory time and are persisting after several

title: Idiopathic pulmonary fibrosis

text: than treating the disease. This may include treatment of worsening symptoms with the use of chronic opioids for severe dyspnea and cough. Further, oxygen therapy may be useful for palliation of dyspnea in hypoxemic patients. Palliative care also includes relief of physical and emotional suffering and psychosocial support for patients and caregivers. With disease progression, patients may experience fear, anxiety and depression and psychological counseling should therefore be considered. In a recent study of outpatients with ILDs, including IPF, depression score, functional status (as assessed by walk test), as well as pulmonary function, all contributed to the severity of dyspnea.

title: Idiopathic pulmonary fibrosis

text: and desquamative interstitial pneumonia [DIP]); and acute/subacute IPs (i.e. cryptogenic organizing pneumonia [COP] and acute interstitial pneumonia [AIP]). The diagnosis of IIPs requires exclusion of known causes of ILD. Examples of ILD of known cause include hypersensitivity pneumonitis, pulmonary Langerhan’s cell histiocytosis, asbestosis, and collagen vascular disease. However, these disorders frequently affect not only the interstitium, but also the airspaces, peripheral airways, and blood vessels. Chest X-rays are useful in the follow up routine of IPF patients. Plain chest X-rays are unfortunately not diagnostic but may reveal decreased lung volumes, typically with prominent reticular interstitial markings near the lung bases.

title: Cardiac fibrosis

text: of the valve, with white tissue building up primarily on the tricuspid valve, but also occurring on the pulmonary valve. The thickening and loss of flexibility eventually may lead to valvular dysfunction and right-sided heart failure. Certain diseases such as gastrointestinal carcinoid tumors of the mid-gut, which sometimes release large amounts of 5-hydroxytryptamine, commonly known as 5-HT or serotonin into the blood, may produce a characteristic pattern of mostly right-sided cardiac fibrosis which can be identified at autopsy. This pathology has also been seen in certain East-African tribes who eat foods (Matoke —a green banana) containing excess amounts of serotonin.

title: Pulmonary hygiene

text: thoracic squeeze or abdominal thrust during the expiratory phase (exhale). An American Thoracic Society consensus statement in 2004 supported the use of mechanical insufflation-exsufflation physiotherapy for patients with Duchenne muscular dystrophy. The use of this technique for children with neuromuscular disease has gained widespread acceptance in the United States and internationally. Pulmonary bronchial hygiene is used for preventing infections such as pneumonia. It is also used in the management of conditions such as pneumonia and cystic fibrosis. For people with chronic lung diseases, bronchial hygiene is used to prevent infections and lung abscesses. Bronchial hygiene is also used to prevent

title: Fibrosis

text: Fibrosis Fibrosis is the formation of excess fibrous connective tissue in an organ or tissue in a reparative or reactive process. This can be a reactive, benign, or pathological state. In response to injury, this is called scarring, and if fibrosis arises from a single cell line, this is called a fibroma. Physiologically, fibrosis acts to deposit connective tissue, which can interfere with or totally inhibit the normal architecture and function of the underlying organ or tissue. Fibrosis can be used to describe the pathological state of excess deposition of fibrous tissue, as well as the process of connective tissue

title: Pulmonary toxicity

text: valid for all cases pulmonary toxicity due to medicinal drugs. As an example to illustrate what can be done, the use of the medicinal drug amiodarone: a) Prescribing the lowest possible dose of amiodarone leads to a lower incidence of pulmonary toxicity. b) Regular monitoring, in order to diagnose any possible pulmonary toxicity early. c) Discontinuation as soon as pulmonary toxicity is detected. One publication summarises the most important points about amiodarone-induced pulmonary toxicity (AIPT) as follows: "The most serious adverse reaction of amiodarone is pulmonary toxicity (AIPT). AIPT may manifest as chronic interstitial pneumonitis, organising pneumonia, acute respiratory distress

title: Pulmonary atresia

text: Consequently, blood flow due to the aforementioned structural abnormalities is affected, either by blocking or altering the flow of blood through the human cardiac muscle. There are two types of pulmonary atresia. The symptoms/signs of pulmonary atresia that will occur in babies are consistent with cyanosis, some fatigue and some shortness of breath (eating may be a problem as well). In the case of pulmonary atresia with ventricular septal defect, one finds that decreased pulmonary blood flow may cause associated defects such as: In terms of the cause of pulmonary atresia, there is uncertainty as to what instigates this congenital

title: Fibrosis

text: can occur in many tissues within the body, typically as a result of inflammation or damage, and examples include: Lungs Liver Heart Brain Other Fibrosis Fibrosis is the formation of excess fibrous connective tissue in an organ or tissue in a reparative or reactive process. This can be a reactive, benign, or pathological state. In response to injury, this is called scarring, and if fibrosis arises from a single cell line, this is called a fibroma. Physiologically, fibrosis acts to deposit connective tissue, which can interfere with or totally inhibit the normal architecture and function of the underlying organ or

title: Cystic Fibrosis Canada

text: Cystic Fibrosis Canada Cystic Fibrosis Canada, formerly the Canadian Cystic Fibrosis Foundation, is a Canada-wide health charity established in 1960, with volunteers in more than 50 chapters across Canada. In 2010, Cystic Fibrosis Canada celebrated its 50th Anniversary. Cystic Fibrosis Canada's mandate is to help individuals with cystic fibrosis, principally by funding cystic fibrosis research and care. The organization also provides educational materials for the cystic fibrosis community and the general public; undertakes advocacy initiatives with, and on behalf of Canadians with cystic fibrosis, and raises funds to supports its programs. Cystic Fibrosis Canada is one of the world's largest

title: Cystic Fibrosis Canada

text: such areas as research and training grants, clinical services and annual data on patients with cystic fibrosis. Read the Cystic Fibrosis Canada Annual Report 2014/2015 here Since 1964, Kin Canada, a Canadian service organisation, has supported Cystic Fibrosis Canada, raising over $42 million in support of cystic fibrosis research and care. Cystic Fibrosis Canada Cystic Fibrosis Canada, formerly the Canadian Cystic Fibrosis Foundation, is a Canada-wide health charity established in 1960, with volunteers in more than 50 chapters across Canada. In 2010, Cystic Fibrosis Canada celebrated its 50th Anniversary. Cystic Fibrosis Canada's mandate is to help individuals with cystic fibrosis,

title: Cystic Fibrosis Trust

text: Cystic Fibrosis Trust The Cystic Fibrosis Trust (stylised as Cystic Fibros"is"), is a UK-based national charity founded in 1964, dealing with all aspects of cystic fibrosis (CF). It funds research to treat and cure CF and aims to ensure appropriate clinical care and support for people with cystic fibrosis. Its objectives are: The aim of the Cystic Fibrosis Trust research is to understand, treat and cure cystic fibrosis. The Cystic Fibrosis Trust is the major funder of medical and scientific CF research in the UK. The Trust's research falls into two main categories: Cystic fibrosis is a complex disease requiring

title: Interstitial lung disease

text: pneumonia, drug-induced lung diseases, pulmonary alveolar proteinosis Acute: Pulmonary edema Chronic: Lymphangitic carcinomatosis, pulmonary alveolar proteinosis, sarcoidosis, pulmonary veno occlusive disease Pulmonary Langerhans cell histiocytosis, silicosis, coal workers pneumoconiosis, carmustine related pulmonary fibrosis, respiratory broncholitis associated with interstitial lung disease. Idiopathic pulmonary fibrosis, pulmonary fibrosis associated with connective tissue diseases, asbestosis, chronic aspiration Sarcoidosis, berylliosis Idiopathic pulmonary fibrosis, chronic eosinophilic pneumonia, cryptogenic organizing pneumonia Pulmonary edema, connective tissue diseases, asbestosis, lymphangitic carcinomatosis, lymphoma, lymphangioleiomyomatosis, drug-induced lung diseases Sarcoidosis, silicosis, berylliosis, lymphangitic carcinomatosis, lymphoma, lymphocytic interstitial pneumonia For some types of chILD and few forms adult ILD genetic causes have been

title: Cardiac fibrosis

text: approaches of countering cardiac fibrosis like microRNA inhibition (miR-21, for example) are being tested in animal models. Cardiac fibrosis Cardiac fibrosis may refer to an abnormal thickening of the heart valves due to inappropriate proliferation of cardiac fibroblasts but more commonly refers to the excess deposition of extracellular matrix in the cardiac muscle. Fibrotic cardiac muscle is stiffer and less compliant and is seen in the progression to heart failure. The description below focuses on a specific mechanism of valvular pathology but there are other causes of valve pathology and fibrosis of the cardiac muscle. Fibrocyte cells normally secrete collagen,

title: Cardiac fibrosis

text: Cardiac fibrosis Cardiac fibrosis may refer to an abnormal thickening of the heart valves due to inappropriate proliferation of cardiac fibroblasts but more commonly refers to the excess deposition of extracellular matrix in the cardiac muscle. Fibrotic cardiac muscle is stiffer and less compliant and is seen in the progression to heart failure. The description below focuses on a specific mechanism of valvular pathology but there are other causes of valve pathology and fibrosis of the cardiac muscle. Fibrocyte cells normally secrete collagen, and function to provide structural support for the heart. When over-activated this process causes thickening and fibrosis

title: Cystic Fibrosis Foundation

text: Cystic Fibrosis Foundation The Cystic Fibrosis Foundation (CFF) is a 501(c)(3) non-profit organization in the United States established to provide the means to cure and control cystic fibrosis (CF). The Foundation provides information about cystic fibrosis and finances CF research that aims to improve the quality of life for people with the disease. The Foundation also engages in legislative lobbying for cystic fibrosis. The Foundation was established in 1955 by a group of volunteers in Philadelphia, Pennsylvania. In addition to providing grants for research into cystic fibrosis and supporting clinical trials, the foundation promotes and accredits 115 specialized centers for

title: Congenital hepatic fibrosis

text: Congenital hepatic fibrosis Congenital hepatic fibrosis is an inherited fibrocystic liver disease associated with proliferation of interlobular bile ducts within the portal areas and fibrosis that do not alter hepatic lobular architecture. The fibrosis would affect resistance in portal veins leading to portal hypertension. The condition is usually congenital, but sporadic cases have also been reported. It may be associated with other congenital defects, commonly with autosomal recessive polycystic kidney disease, the most severe form of PKD. Some suggest that these two conditions are one disorder with different presentation. Embryogenically, congenital hepatic fibrosis is due to malformation of the duct

title: Cystic Fibrosis Trust

text: UK artist Jenny Wicks in 2009 with her photographic art exhibition, short documentary and book titled "Root Ginger". The exhibition describes the recessive gene inheritance pattern that causes ginger, or red, hair - the same inheritance pattern that causes cystic fibrosis. A portion of the proceeds from the exhibition and book sales will go to the Cystic Fibrosis Trust. Cystic Fibrosis Trust The Cystic Fibrosis Trust (stylised as Cystic Fibros"is"), is a UK-based national charity founded in 1964, dealing with all aspects of cystic fibrosis (CF). It funds research to treat and cure CF and aims to ensure appropriate clinical

title: Pirfenidone

text: In this model, bleomycin administration results in oxidative stress and acute inflammation, with the subsequent onset of pulmonary fibrosis in a number of animal species including the mouse and hamster. Numerous studies have demonstrated that pirfenidone attenuates bleomycin-induced pulmonary fibrosis. One study investigated the effect of pirfenidone over a 42-day period after repeated bleomycin administration. Administration of pirfenidone minimised early lung oedema and pulmonary fibrosis when treatment was initiated concurrently with lung damage. This study evaluated pulmonary protein expression and found pirfenidone treatment normalised expression of proinflammatory and fibrogenic proteins. Similar reductions in pulmonary fibrosis were observed when pirfenidone treatment

title: Exercise-induced pulmonary hemorrhage

text: the microscopic level in adjacent areas of lung tissue creates shear stress and capillary disruption. The theory predicts that hemorrhage would be more severe on hard track surfaces, but it does not explain why EIPH can occur in horses during swimming exercise. This theory proposes how high pulmonary venous pressures may lead to the capillary rupture and the tissue changes observed in EIPH. Regional veno-occlusive remodeling, especially within the caudodorsal lung fields, contributes to the pathogenesis of EIPH, with the venous remodeling leading to regional vascular congestion and hemorrhage, hemosiderin accumulation, fibrosis, and bronchial angiogenesis. While all horses undertaking intense

title: Oral submucous fibrosis

text: safe and effective treatment modality in oral sub mucosal fibrosis. It has been shown autologous bone marrow stem cell injections induces angiogenesis in the area of lesion which in turn decreases the extent of fibrosis thereby leading to significant increase in mouth opening. In 1952, T.Sheikh coined the term "distrophica idiopathica mucosa oris" to describe an oral fibrosing disease he discovered in five Indian women from Kenya. S.G. Joshi subsequently coined the termed oral submucous fibrosis (OSF) for the condition in 1953. Oral submucous fibrosis Oral submucous fibrosis (OSMF or OSF) is a chronic, complex, premalignant (1% transformation risk) condition

title: Cystic Fibrosis Foundation

text: treatment of individuals with cystic fibrosis. The Foundation has over 80 chapters and offices across the United States. Before it began using the current name, the organization was known as the "National Cystic Fibrosis Research Foundation". In 1989, scientists working for the Cystic Fibrosis Foundation discovered the gene that causes cystic fibrosis, considered the key to developing a cure for cystic fibrosis. From 1982 until 1999, sports journalist Frank Deford served as chairman of the Foundation. The Cystic Fibrosis Foundation has been a pioneer of cystic fibrosis treatment, having played a major role in the development and use of five

title: Cystic Fibrosis Canada

text: the country, Cystic Fibrosis Canada provides support to clinicians through grants, training awards and access to statistical information through the on-line Canadian Cystic Fibrosis (CF) Registry. Canadian researchers are viewed as leaders in the global effort to find a cure or control for cystic fibrosis. In 1989, Canadian researchers, funded by Cystic Fibrosis Canada, discovered the gene responsible for cystic fibrosis, and they continue to play a leading role in developing new treatments. As well as general information about cystic fibrosis in Canada and resources for teachers, parents, and health care professionals, Cystic Fibrosis Canada publishes newsletters and reports covering

title: Cystic Fibrosis Canada

text: non-governmental granting agencies in the field of cystic fibrosis research. Cystic Fibrosis Canada raises funds in order to promote public awareness and support research and high-quality cystic fibrosis care. Cystic Fibrosis Canada's primary objective is to fund CF research and care: the organization annually funds approximately 50 research projects, in addition to scholars, post-doctoral fellows, and students undertaking CF investigations. Each year, Cystic Fibrosis Canada awards more than $7 million in grants to CF researchers, and approximately $2 million in grants to the 42 CF clinics, and five transplant centres across the country. To help ensure consistent, high-quality care across

title: Cystic fibrosis

text: the risks and long-term effectiveness of this treatment. So far, no large-scale research involving the incidence of atherosclerosis and coronary heart disease in adults with cystic fibrosis has been conducted. This is likely because the vast majority of people with cystic fibrosis do not live long enough to develop clinically significant atherosclerosis or coronary heart disease. Diabetes is the most common nonpulmonary complication of CF. It mixes features of type 1 and type 2 diabetes, and is recognized as a distinct entity, cystic fibrosis-related diabetes. While oral antidiabetic drugs are sometimes used, the recommended treatment is the use of insulin

title: Cystic fibrosis

text: Cystic fibrosis Cystic fibrosis (CF) is a genetic disorder that affects mostly the lungs, but also the pancreas, liver, kidneys, and intestine. Long-term issues include difficulty breathing and coughing up mucus as a result of frequent lung infections. Other signs and symptoms may include sinus infections, poor growth, fatty stool, clubbing of the fingers and toes, and infertility in most males. Different people may have different degrees of symptoms. CF is inherited in an autosomal recessive manner. It is caused by the presence of mutations in both copies of the gene for the cystic fibrosis transmembrane conductance regulator (CFTR) protein.

title: High-altitude pulmonary edema

text: High-altitude pulmonary edema High-altitude pulmonary edema (HAPE) is a life-threatening form of non-cardiogenic pulmonary edema (fluid accumulation in the lungs) that occurs in otherwise healthy mountaineers at altitudes typically above . However, cases have also been reported at lower altitudes (between in highly vulnerable subjects), though what makes some people susceptible to HAPE is currently unknown. HAPE remains the major cause of death related to high-altitude exposure, with a high mortality rate in the absence of adequate emergency treatment. Physiological and symptomatic changes often vary according to the altitude involved. The Lake Louise Consensus Definition for high-altitude pulmonary edema has

title: Cardiac fibrosis

text: and Raynaud's phenomenon (both vasoconstrictive effects), as well as retroperitoneal fibrosis (a fibrotic cell/fibrocyte proliferation effect, thought to be similar to cardiac valve fibrosis). These drugs include ergotamine and methysergide and both drugs can also cause cardiac fibrosis. Certain antiparkinson drugs, although targeted at dopaminergic receptors, cross-react with serotoninergic 5-HT receptors as well, and have been reported to cause cardiac fibrosis. These drugs include pergolide and cabergoline. Guanfacine may be a 5-HT agonist, based on the results of theoretical modeling and high-throughput screening. Pergolide was an antiparkinson medications that was in decreasing use since reported in 2003 to be associated

title: Cardiac fibrosis

text: of 5-HTP to serotonin and so ensures that only brain serotonin levels are increased without producing peripheral side effects, however 5-HTP is also sold without carbidopa as a dietary supplement, and may have increased risks when taken by itself without carbidopa. The most obvious treatment for cardiac valve fibrosis or fibrosis in other locations, consists of stopping the stimulatory drug or production of serotonin. Surgical tricuspid valve replacement for severe stenosis (blockage of blood flow) has been necessary in some patients. A compound found in red wine, resveratrol has been found to slow the development of cardiac fibrosis. More sophisticated

title: Fibrosis

text: deposition in healing. Defined by the pathological accumulation of extracellular matrix (ECM) proteins, fibrosis results in scarring and thickening of the affected tissue, it is in essence an exaggerated wound healing response which interferes with normal organ function. Fibrosis is similar to the process of scarring, in that both involve stimulated fibroblasts laying down connective tissue, including collagen and glycosaminoglycans. The process is initiated when immune cells such as macrophages release soluble factors that stimulate fibroblasts. The most well characterized pro-fibrotic mediator is TGF beta, which is released by macrophages as well as any damaged tissue between surfaces called interstitium.

title: Pulmonary artery

text: Pulmonary artery A pulmonary artery is an artery in the pulmonary circulation that carries deoxygenated blood from the right side of the heart to the lungs. The largest pulmonary artery is the "main pulmonary artery." or "pulmonary trunk" from the heart, and the smallest ones are the arterioles, which lead to the capillaries that surround the pulmonary alveoli. In order of blood flow, the pulmonary arteries start as the pulmonary trunk or main pulmonary artery. The main pulmonary artery begins at the base of the right ventricle. It is short and wide—approximately in length and in diameter. The main pulmonary

title: Pulmonary artery

text: after a period of immobility. A pulmonary embolus is a common cause of death in patients with cancer and stroke. A large pulmonary embolus which becomes lodged in the bifurcation of the pulmonary trunk with extensions into both the left and right main pulmonary arteries is called a "saddle embolus". Pulmonary artery A pulmonary artery is an artery in the pulmonary circulation that carries deoxygenated blood from the right side of the heart to the lungs. The largest pulmonary artery is the "main pulmonary artery." or "pulmonary trunk" from the heart, and the smallest ones are the arterioles, which lead

title: Fibrosis

text: Other soluble mediators of fibrosis include CTGF, platelet-derived growth factor (PDGF), and Interleukin 4 (IL-4). These initiate signal transduction pathways such as the AKT/mTOR and SMAD pathways that ultimately lead to the proliferation and activation of fibroblasts, which deposit extracellular matrix into the surrounding connective tissue. This process of tissue repair is a complex one, with tight regulation of ECM synthesis and degradation ensuring maintenance of normal tissue architecture. However, the entire process, although necessary, can lead to a progressive irreversible fibrotic response if tissue injury is severe or repetitive, or if the wound healing response itself becomes deregulated. Fibrosis

title: Cystic fibrosis-related diabetes

text: Cystic fibrosis-related diabetes Cystic fibrosis-related diabetes (CFRD) is diabetes specifically caused by cystic fibrosis, a genetic condition. Cystic fibrosis related diabetes mellitus (CFRD) develops with age, and the median age at diagnosis is 21 years. CFRD shares features of both type 1 and type 2 diabetes. CFRD patients are typically young and are not obese, and lack metabolic syndrome features. On the other hand, the cause is not autoimmune, some insulin resistance is present, and ketosis is rare. The endocrine pancreatic function deterioration appears to be secondary to chronic pancreatitis and subsequent scarring associated with CF. CFRD occurs in some

title: Asbestosis

text: of asbestos bodies in association with pulmonary fibrosis establishes the diagnosis. Conversely, interstitial pulmonary fibrosis in the absence of asbestos bodies is most likely not asbestosis. Asbestos bodies in the absence of fibrosis indicate exposure, not disease. There is no cure available for asbestosis. Oxygen therapy at home is often necessary to relieve the shortness of breath and correct underlying low blood oxygen levels. Supportive treatment of symptoms includes respiratory physiotherapy to remove secretions from the lungs by postural drainage, chest percussion, and vibration. Nebulized medications may be prescribed in order to loosen secretions or treat underlying chronic obstructive pulmonary

title: Oral submucous fibrosis

text: very early stage, cessation of the habit is sufficient. Most patients with oral submucous fibrosis present with moderate-to-severe disease. Severe oral submucous fibrosis is irreversible. Moderate oral submucous fibrosis is reversible with cessation of habit and mouth opening exercise. Current modern day medical treatments can make the mouth opening to normal minimum levels of 30 mm mouth opening with proper treatment. The incidence of the disease is higher in people from certain parts of the world including South-East Asia, South Africa and the Middle East. Recently scientists have proven that intralesional injection of autologous bone marrow stem cells is a

title: Oral submucous fibrosis

text: Oral submucous fibrosis Oral submucous fibrosis (OSMF or OSF) is a chronic, complex, premalignant (1% transformation risk) condition of the oral cavity, characterized by juxta-epithelial inflammatory reaction and progressive fibrosis of the submucosal tissues (the lamina propria and deeper connective tissues). As the disease progresses, the jaws become rigid to the point that the person is unable to open the mouth. The condition is remotely linked to oral cancers and is associated with areca nut or betel quid chewing, a habit similar to tobacco chewing, is practiced predominantly in Southeast Asia and India, dating back thousands of years. In the

title: Nephrogenic systemic fibrosis

text: Nephrogenic systemic fibrosis Nephrogenic systemic fibrosis (NSF) is a rare syndrome that involves fibrosis of skin, joints, eyes, and internal organs. NSF is caused by exposure to gadolinium in gadolinium-based MRI contrast agents (GBCAs) in patients with impaired kidney function. Epidemiological studies suggest that the incidence of NSF is unrelated to gender or ethnicity and it is not thought to have a genetic basis. After GBCAs were identified as a cause of the disorder in 2006, and screening and prevention measures put in place, it is now considered rare. Clinical features of NSF develop within days to months following exposure

title: Cystic fibrosis-related diabetes

text: 20% of adolescents and 40-50% of adults affected by CF. Though rare in children, it has been described in CF patients of all ages, including infants. Beginning in the teenage years, CFRD has an annual incidence of ~3%, and may be more common in females. It is associated with more severe CF gene mutation types. As survival of CF patients has steadily increased in past decades, CFRD is an increasingly common - and currently the most common - complication of CF. Cystic fibrosis-related diabetes Cystic fibrosis-related diabetes (CFRD) is diabetes specifically caused by cystic fibrosis, a genetic condition. Cystic fibrosis

title: Nephrogenic systemic fibrosis

text: of NSF. The first cases of NSF were identified in 1997, but it was first described as an independent disease entity in 2000. In 2006, the link between NSF and gadolinium-based MRI contrast agents was made. As a result, restrictions on use of GBCAs in patients with an estimated glomerular filtration rate (a measure of renal function) under 60 and especially under 30 mL/min/1.73 m have been recommended and NFS is now considered rare. Nephrogenic systemic fibrosis Nephrogenic systemic fibrosis (NSF) is a rare syndrome that involves fibrosis of skin, joints, eyes, and internal organs. NSF is caused by exposure

title: Pulmonary angiography

text: Nevertheless, it is still used in cases where CT angiography is nondiagnostic. Conventional pulmonary angiography was first performed in 1931 by Portuguese angiography pioneers Lopo de Carvalho, Egas Moniz and colleagues. Robb and Steinberg described pulmonary angiography by infusion of peripheral radiocontrast. Pulmonary angiography Pulmonary angiography (or pulmonary arteriography) is medical fluoroscopic procedure used to visualize the pulmonary arteries and much less frequently, the pulmonary veins. Conventional pulmonary angiography is a minimally invasive procedure performed most frequently by an interventional radiologist or interventional cardiologist. This form of angiography has the added benefit of the ability to treat certain conditions. CT

title: Pulmonary hypertension

text: effusions. In the absence of pulmonary blood vessel narrowing, the increased back pressure is described as ‘isolated post-capillary pulmonary hypertension’ (older terms include ‘passive’ or ‘proportionate’ pulmonary hypertension or ‘pulmonary venous hypertension’). However, in some patients, the raised pressure in the pulmonary vessels triggers a superimposed component of vessel narrowing, which further increases the workload of the right side of the heart. This is referred to as ‘post-capillary pulmonary hypertension with a pre-capillary component’ or ‘combined post-capillary and pre-capillary pulmonary hypertension’ (older terms include ‘reactive’ or ‘out-of-proportion’ pulmonary hypertension). In pulmonary hypertension due to lung diseases and/or hypoxia (WHO Group

title: Physical therapy

text: practitioners and physical therapists offer therapy for a wide variety of cardiopulmonary disorders or pre and post cardiac or pulmonary surgery. An example of cardiac surgery is coronary bypass surgery. Primary goals of this specialty include increasing endurance and functional independence. Manual therapy is used in this field to assist in clearing lung secretions experienced with cystic fibrosis. Pulmonary disorders, heart attacks, post coronary bypass surgery, chronic obstructive pulmonary disease, and pulmonary fibrosis, treatments can benefit from cardiovascular and pulmonary specialized physical therapists. This specialty area includes electrotherapy/physical agents, electrophysiological evaluation (EMG/NCV), physical agents, and wound management. Geriatric physical therapy

title: Pulmonary vein

text: drain into the pulmonary circulation in whole or in part, this is known as a total anomalous pulmonary venous connection (or drainage), or partial anomalous pulmonary connection, respectively. Pulmonary vein The pulmonary veins are the veins that transfer oxygenated blood from the lungs to the heart. The largest pulmonary veins are the four "main pulmonary veins", two from each lung that drain into the left atrium of the heart. The pulmonary veins are part of the pulmonary circulation. Two main pulmonary veins emerge from each lung hilum, receiving blood from three or four bronchial veins apiece and draining into the

title: Pulmonary laceration

text: be scarred. Small pulmonary lacerations frequently heal by themselves if material is removed from the pleural space, but surgery may be required for larger lacerations that do not heal properly or that bleed. Pulmonary laceration A pulmonary laceration is a chest injury in which lung tissue is torn or cut. An injury that is potentially more serious than pulmonary contusion, pulmonary laceration involves disruption of the architecture of the lung, while pulmonary contusion does not. Pulmonary laceration is commonly caused by penetrating trauma but may also result from forces involved in blunt trauma such as shear stress. A cavity filled

title: Pulmonary scientist

text: the CPFT performs pulmonary function testing. Pulmonary scientist A Pulmonary Scientist is a health care occupation in the field of diagnostic studies related specifically to pulmonary function, the term Respiratory Scientist may also refer to a Pulmonologist who holds a medical degree and a doctoral degree (MD-PhD). Internationally pulmonary scientists have many different titles, such as: pulmonary function technologist, respiratory scientist, pulmonary scientist, cardiopulmonary specialist and in some places registered respiratory therapists are also used in the role of pulmonary scientists. A physician in respiratory science is generally referred to as a physician in their title (i.e. Respiratory Physician-Scientist). Respiratory

title: Pulmonary sequestration

text: but they include a persistent dry cough. Failure to have a pulmonary sequestration removed can lead to a number of complications. These include: There is still much debate to whether pulmonary sequestration is a congenital problem or acquired through recurrent pulmonary infection. It is widely believed that extralobar pulmonary sequestrations are a result of prenatal pulmonary malformation while intralobar pulmonary sequestrations can develop due to recurrent pulmonary infections in adolescents and young adults. Bronchopulmonary sequestration (BPS) is a rare congenital malformation of the lower respiratory tract. It consists of a nonfunctioning mass of normal lung tissue that lacks normal communication

title: Cystic Fibrosis Trust

text: influence and improve the care of those with CF across the UK. The Cystic Fibrosis Trust provides a confidential, 9-5 Monday to Friday helpline service for advice and support on all aspects of cystic fibrosis. The Trust also offers information and advice to those affected, along with their families and friends, schools and employers and anyone interested in cystic fibrosis. The Trust also advise families on benefits and, where appropriate, provides financial assistance and welfare grants. The Cystic Fibrosis Trust receives donations and support from local communities . In addition to the contributions, fund raising efforts have been undertaken by

title: Cystic fibrosis

text: In 1936, Guido Fanconi described a connection between celiac disease, cystic fibrosis of the pancreas, and bronchiectasis. In 1938, Dorothy Hansine Andersen published an article, "Cystic Fibrosis of the Pancreas and Its Relation to Celiac Disease: a Clinical and Pathological Study", in the "American Journal of Diseases of Children". She was the first to describe the characteristic cystic fibrosis of the pancreas and to correlate it with the lung and intestinal disease prominent in CF. She also first hypothesized that CF was a recessive disease and first used pancreatic enzyme replacement to treat affected children. In 1952, Paul di Sant'Agnese

title: Cystic fibrosis

text: in Africans and Asians. It was first recognized as a specific disease by Dorothy Andersen in 1938, with descriptions that fit the condition occurring at least as far back as 1595. The name "cystic fibrosis" refers to the characteristic fibrosis and cysts that form within the pancreas. The main signs and symptoms of cystic fibrosis are salty-tasting skin, poor growth, and poor weight gain despite normal food intake, accumulation of thick, sticky mucus, frequent chest infections, and coughing or shortness of breath. Males can be infertile due to congenital absence of the vas deferens. Symptoms often appear in infancy and

title: Pulmonary thromboendarterectomy

text: Pulmonary thromboendarterectomy In thoracic surgery, a pulmonary thromboendarterectomy (PTE) is an operation that removes organized clotted blood (thrombus) from the pulmonary arteries, which supply blood to the lungs. Surgery is indicated in patients with pulmonary artery emboli that are surgically accessible. Thrombi are typically the result of chronic thromboembolic pulmonary hypertension (CTEPH) (pulmonary hypertension induced by recurrent/chronic pulmonary emboli). Indeed, PTE is the only definitive treatment option currently available for CTEPH. Due to the nature of the procedure patients with significant hemodynamic or ventilation complications or impairments may be unable to undergo PTE. A PTE has significant risk; mortality for

title: Pulmonary atresia

text: of life. Many children with pulmonary atresia will go on to lead normal lives, though complications such as endocarditis, stroke and seizures are possible. Pulmonary atresia Pulmonary atresia is a congenital malformation of the pulmonary valve in which the valve orifice fails to develop. The valve is completely closed thereby obstructing the outflow of blood from the heart to the lungs. The pulmonary valve is located on the right side of the heart between the right ventricle and pulmonary artery. In a normal functioning heart, the opening to the pulmonary valve has three flaps that open and close In congenital

title: Pulmonary atresia

text: Pulmonary atresia Pulmonary atresia is a congenital malformation of the pulmonary valve in which the valve orifice fails to develop. The valve is completely closed thereby obstructing the outflow of blood from the heart to the lungs. The pulmonary valve is located on the right side of the heart between the right ventricle and pulmonary artery. In a normal functioning heart, the opening to the pulmonary valve has three flaps that open and close In congenital heart defects such as pulmonary atresia, structural abnormalities can include the valves of the heart, and the walls and arteries/veins near the heart muscle.

title: Pulmonary angiography

text: Pulmonary angiography Pulmonary angiography (or pulmonary arteriography) is medical fluoroscopic procedure used to visualize the pulmonary arteries and much less frequently, the pulmonary veins. Conventional pulmonary angiography is a minimally invasive procedure performed most frequently by an interventional radiologist or interventional cardiologist. This form of angiography has the added benefit of the ability to treat certain conditions. CT (computed tomography) angiography has nearly entirely replaced conventional pulmonary angiography in common practice as it is less invasive, faster, safer, and provides most of the same diagnostic information with the added benefit of visualizing the lung tissue as well as other structures.

title: Pulmonary scientist

text: Pulmonary scientist A Pulmonary Scientist is a health care occupation in the field of diagnostic studies related specifically to pulmonary function, the term Respiratory Scientist may also refer to a Pulmonologist who holds a medical degree and a doctoral degree (MD-PhD). Internationally pulmonary scientists have many different titles, such as: pulmonary function technologist, respiratory scientist, pulmonary scientist, cardiopulmonary specialist and in some places registered respiratory therapists are also used in the role of pulmonary scientists. A physician in respiratory science is generally referred to as a physician in their title (i.e. Respiratory Physician-Scientist). Respiratory science is a diminishing field of

title: Progressive massive fibrosis

text: the upper lobe of the lung - especially on the right, and its common occurrence in taller individuals. Progressive massive fibrosis increased during the period 1970-2016 among coal miners in central Appalachia who filed for black lung benefits. Occupational Lung Diseases, 3rd Edition, Morgan and Seaton https://web.archive.org/web/20060223051633/http://pim.medicine.dal.ca/il1.htm Progressive massive fibrosis Progressive Massive Fibrosis (PMF), characterized by the development of large conglomerate masses of dense fibrosis (usually in the upper lung zones), can complicate silicosis and coal worker's pneumoconiosis. Conglomerate masses may also occur in other pneumoconioses, such as talcosis, berylliosis (CBD), kaolin pneumoconiosis, and pneumoconiosis from carbon compounds, such as

title: Cystic Fibrosis Foundation

text: Great Strides walks in over 560 locations throughout the country.Great Strides has succeeded in raising over $180,000,000 for cystic fibrosis research. Some other signature events include the CF Cycle for Life, CF Climb, Xtreme Hike, and Team CF. The list of national events includes Ultimate Golf Experience, American Airlines Celebrity Ski, and Volunteer Leadership Conference. Individual chapters may host their own events which may include, but are not limited to, fishing events, dinner dance events, golf tournaments, and finest events. Cystic Fibrosis Foundation The Cystic Fibrosis Foundation (CFF) is a 501(c)(3) non-profit organization in the United States established to provide

title: Pulmonary circulation

text: Pulmonary circulation The pulmonary circulation is the portion of the circulatory system which carries deoxygenated blood away from the right ventricle of the heart, to the lungs, and returns oxygenated blood to the left atrium and ventricle of the heart. The term pulmonary circulation is readily paired and contrasted with the systemic circulation. The vessels of the pulmonary circulation are the pulmonary arteries and the pulmonary veins. A separate system known as the bronchial circulation supplies oxygenated blood to the tissue of the larger airways of the lung. The earliest human discussions of pulmonary circulation date back to Egyptian times.

title: Pulmonary valve

text: Pulmonary valve The pulmonary valve (sometimes referred to as the pulmonic valve) is the semilunar valve of the heart that lies between the right ventricle and the pulmonary artery and has three cusps. Similar to the aortic valve, the pulmonary valve opens in ventricular systole, when the pressure in the right ventricle rises above the pressure in the pulmonary artery. At the end of ventricular systole, when the pressure in the right ventricle falls rapidly, the pressure in the pulmonary artery will close the pulmonary valve. The closure of the pulmonary valve contributes the P2 component of the second heart

title: Retroperitoneal fibrosis

text: or certain infections. The diagnosis of retroperitoneal fibrosis cannot be made on the basis of results of laboratory studies. CT is the best diagnostic modality: a confluent mass surrounding the aorta can be seen on a CT scan. Although biopsy is not usually recommended, it is appropriate when malignancy or infection is suspected. Biopsy should also be done if the location of fibrosis is atypical or if there is an inadequate response to initial treatment. In the absence of severe urinary tract obstruction (which generally requires surgery with omental wrapping), treatment is generally with glucocorticoids initially, followed by DMARDs either

title: Pulmonary hemorrhage

text: edema, as the hematocrit is lower than normal blood (usually 15-20% less) and the concentration of small proteins is higher than in plasma. It is postulated that the infant suffers from asphyxia with resultant heart attack; this increases pulmonary microvascular pressure, resulting in pulmonary edema. Contributing factors include factors that favor increased filtration of fluid from pulmonary capillaries (e.g., low concentration of plasma proteins, high alveolar surface tension, lung damage, hypervolemia). Diffuse alveolar hemorrhage is bleeding from many alveoli throughout the lungs. Common causes include autoimmune diseases and connective tissue diseases. Pulmonary hemorrhage Pulmonary hemorrhage (or pulmonary haemorrhage) is an

title: Pulmonary laceration

text: Pulmonary laceration A pulmonary laceration is a chest injury in which lung tissue is torn or cut. An injury that is potentially more serious than pulmonary contusion, pulmonary laceration involves disruption of the architecture of the lung, while pulmonary contusion does not. Pulmonary laceration is commonly caused by penetrating trauma but may also result from forces involved in blunt trauma such as shear stress. A cavity filled with blood, air, or both can form. The injury is diagnosed when collections of air or fluid are found on a CT scan of the chest. Surgery may be required to stitch the

title: Pulmonary wedge pressure

text: Pulmonary wedge pressure The pulmonary wedge pressure or PWP, or cross-sectional pressure (also called the pulmonary arterial wedge pressure or PAWP, pulmonary capillary wedge pressure or PCWP, or pulmonary artery occlusion pressure or PAOP), is the pressure measured by wedging a pulmonary catheter with an inflated balloon into a small pulmonary arterial branch. It estimates the left atrial pressure. Pulmonary venous wedge pressure (PVWP) is not synonymous with the above; PVWP has been shown to correlate with pulmonary artery pressures in studies, albeit unreliably. Physiologically, distinctions can be drawn among pulmonary artery pressure, pulmonary capillary wedge pressure, pulmonary venous pressure