diffuse interstitial pulmonary fibrosis


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Related to diffuse interstitial pulmonary fibrosis: Idiopathic pulmonary fibrosis, Interstitial lung disease, ILD

fibrosis

 [fi-bro´sis]
formation of fibrous tissue; see also fibroid degeneration. adj., adj fibrot´ic.
congenital hepatic fibrosis a developmental disorder of the liver, marked by formation of irregular broad bands of fibrous tissue containing multiple cysts formed by disordered terminal bile ducts, resulting in vascular constriction and portal hypertension.
cystic fibrosis (cystic fibrosis of pancreas) see cystic fibrosis.
diffuse idiopathic interstitial fibrosis (diffuse interstitial pulmonary fibrosis) idiopathic pulmonary fibrosis.
endomyocardial fibrosis an idiopathic type of myocardiopathy that is endemic in various parts of Africa and rarely in other areas, characterized by cardiomegaly, marked thickening of the endocardium with dense white fibrous tissue that may extend to involve the inner myocardium, and by congestive heart failure.
idiopathic pulmonary fibrosis chronic inflammatory progressive fibrosis of the pulmonary alveolar walls, with steadily progressive dyspnea, resulting in death from oxygen lack or right heart failure. Most cases are of unknown origin, although some are thought to result from pneumoconiosis, hypersensitivity pneumonitis, scleroderma, and other diseases.
mediastinal fibrosis development of hard white fibrous tissue in the upper portion of the mediastinum, sometimes obstructing the air passages and large blood vessels; called also fibrosing or fibrous mediastinitis.
periureteral fibrosis retroperitoneal fibrosis.
pleural fibrosis fibrosis of the visceral pleura so that part or all of a lung becomes covered with a plaque or a thick layer of nonexpansible fibrous tissue. The more extensive form is called fibrothorax.
postfibrinous fibrosis that occurring in tissues in which fibrin has been deposited.
proliferative fibrosis that in which the fibrous elements continue to proliferate after the original causative factor has ceased to operate.
pulmonary fibrosis idiopathic pulmonary fibrosis.
retroperitoneal fibrosis deposition of fibrous tissue in the retroperitoneal space, producing vague abdominal discomfort, and often causing blockage of the ureters, with resultant hydronephrosis and impaired renal function, which may result in renal failure. Called also Ormond disease.
fibrosis u´teri a morbid condition characterized by overgrowth of the smooth muscle and increase in the collagenous fibrous tissue of the uterus, producing a thickened, coarse, tough myometrium.

fibrosis

(fi-bro'sis) [ fibro- + -sis]
The repair and replacement of inflamed tissues or organs by connective tissues. The process results in the replacement of normal cells by fibroblasts (and eventually, the replacement of normal organ tissue by scar tissue).

arteriocapillary fibrosis

Arteriolar and capillary fibroid degeneration.
Synonym: arteriofibrosis

cystic fibrosis

Abbreviation: CF
A potentially fatal autosomal recessive disease that manifests itself in multiple body systems, including the lungs, the pancreas, the urogenital system, the skeleton, and the skin. It causes chronic obstructive pulmonary disease, frequent lung infections, deficient elaboration of pancreatic enzymes, osteoporosis, and an abnormally high electrolyte concentration in the sweat. The name is derived from the characteristic histologic changes in the pancreas. CF usually begins in infancy and is the major cause of severe chronic lung disease in children. In the U.S., CF occurs in 1 in 2500 white live births and 1 in 17,000 black live births and is the most common fatal genetic disease in European-American children Synonym: fibrocystic disease of the pancreas; mucoviscidosis

Symptoms

A great variety of clinical manifestations may be present, including nasal polyposis; lung changes related to thick, tenacious secretions leading to bronchiectasis; bronchitis; pneumonia; atelectasis, emphysema and respiratory failure; gallbladder diseases; intussusception; meconium ileus; salt depletion; pancreatic exocrine deficiency causing intestinal malabsorption of fats, proteins, and, to a lesser extent, carbohydrates; pancreatitis; peptic ulcer; rectal prolapse; diabetes; nutritional deficiencies; arthritis; absent vas deferens with consequent aspermia and absence of fructose in the ejaculate; failure to thrive; and delayed puberty. The child exhibits a nonproductive, paroxysmal cough, barrel chest, cyanosis, clubbed fingers and toes, malabsorption leading to poor weight gain and growth, fat-soluble vitamin deficiency (A, D, E, K) leading to clotting abnormalities, and excretion of frequent pale stools that are bulky, foul-smelling and have a high fat content.

Treatment

Therapy must be individualized, carefully monitored, and continued throughout life. Pulmonary infection is controlled with antibiotics. It is essential that secretions be cleared from the airway by intermittent aerosol therapy. A mucolytic agent may be helpful as well as postural drainage, mist inhalation, and bronchodilator therapy. Bronchoalveolar lavage has been of use in some patients. In addition, bronchial drainage may be improved by use of aerosolized recombinant human DNase (rhDNase). Use of a Flutter device for airway mucus clearance is considerably more effective in increasing sputum expectoration than traditional postural drainage and clapping the chest. Lung transplantation may also be used to treat CF. High doses of ibuprofen taken consistently for years may slow progression of the disease by limiting airway inflammation. See: bronchoalveolar lavage; Flutter device

Prognosis

Median cumulative survival is approximately 30 years, with males surviving much longer than females for unknown reasons.

Genetic Screening

In 2001 The American College of Obstetricians and Gynecologists and the American College of Medical Genetics recommended that all prospective parents undergo screening to see if they are carriers of cystic fibrosis genes.

Patient care

Both patient and family are taught to perform pulmonary chest physiotherapy and postural drainage followed by deep breathing and coughing to help mobilize secretions. Fluid intake is encouraged to thin inspissated secretions. Humidified air, with intermittent positive-pressure breathing therapy if prescribed, is provided. Dornase alfa is also administered by nebulizer as prescribed. A DNA enzyme produced by recombinant gene therapy, the drug is used to reduce the frequency of respiratory infections, to decrease sputum thickness (viscosity), and to improve pulmonary functioning in patients with cystic fibrosis.

The patient should take precautions (e.g., annual influenza immunization and at least one pneumococcal vaccination) to prevent respiratory infections and should learn to recognize and report signs and symptoms and to initiate prescribed antibiotic prophylaxis promptly. Oral pancreatic enzymes are given with meals and snacks to replace deficiencies, and foods are well-salted or a sodium supplement prescribed to combat electrolyte losses in sweat. A well-balanced high-calorie, high-protein diet is recommended, including replacement of fat-soluble vitamins if laboratory analysis indicates any deficiencies. Aerobic exercise and physical activity within permitted limits are encouraged; breathing exercises should be performed during activity to improve ventilatory capacity and activity tolerance. The child is encouraged in age-appropriate developmental tasks, and acceptable activities are substituted for those in which the child is unable to participate.

Caregivers involve the child in care by offering valid choices and encouraging decision making. The family is encouraged to discuss their feelings and concerns. Genetic testing is explained. Realistic reassurance is offered regarding expectations after an exacerbation, and emotional support is provided to help both patient and family work through feelings of anticipatory grief. Referral is made to available local chapters of support groups such as the Cystic Fibrosis Foundation.

diffuse interstitial pulmonary fibrosis

Idiopathic pulmonary fibrosis.

idiopathic pulmonary fibrosis

The formation of scar tissue in the parenchyma of the lungs, following inflammation of the alveoli. The disease results in difficulty breathing caused by impaired gas exchange. Synonym: diffuse interstitial pulmonary fibrosis; pulmonary fibrosis; Hamman's syndrome

Symptoms

Dyspnea, cough, exertional fatigue, and generalized weakness are common. Signs of the illness include pulmonary crackles, finger clubbing, cyanosis, and evidence of right ventricular failure (such as lower-extremity swelling). The disease typically progresses to end-stage lung disease and death within 7 years of diagnosis.

Diagnosis

A biopsy of the lung is needed to make the diagnosis.

Treatment

Corticosteroids (such as prednisone) may be helpful in 10% to 20% of patients. Lung transplantation can be curative if a donor organ is available.

pleural fibrosis

A condition occurring in pulmonary tuberculosis, asbestosis, and other lung diseases in which the pleura becomes thickened and the pleural space may be obliterated.

postfibrinosis fibrosis

Development of fibrosis in a tissue in which fibrin has been deposited.

premacular fibrosis

Macular pucker.

proliferative fibrosis

Formation of new fibrous tissue from connective tissue cells.

pulmonary fibrosis

Idiopathic pulmonary fibrosis.

retroperitoneal fibrosis

Development of a mass of scar tissue in the retroperitoneal space. This may lead to physical compression of the ureters, vena cava, or aorta. This disease may be associated with taking methysergide for migraine, and with other drugs.
Synonym: Ormond's disease

fibrosis uteri

Diffuse growth of fibrous tissue throughout the uterus.

diffuse interstitial pulmonary fibrosis

see interstitial pneumonia.
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