CYCTIC FIBROSIS

PNEUMOCONIOSIS

   
   
   
   
   
   
 

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CYSTIC FIBROSIS
Cystic fibrosis is an inherited multisystem disease characterized chiefly by obstruction of airways and pancreatic duct by viscous secretions resulting in recurrent respiratory tract infections and maldigestion.

It is the major cause of severe chronic lung disease in children and is responsible for most of the exocrine pancreatic insufficiency cases during the early periods of life.

It is inherited as an autosomal recessive genetic trait. It is the most common life-limiting monogenetic disorder that presents among whites.

The prevalence is approximately 1 in 3000 live births in the North America and northern Europe which is far more frequent than the rest of the world.

The monogenetic disorder responsible for the disease is in the gene encoding CFTR protein ( Cystic fibrosis Transmembrane Regulator) . The CFTR protein appears to function as a cyclic AMP-regulated Chlorine channel ,which is expressed mainly in epithelial cells of airways, the gastrointestinal tract (including pancreas and biliary system), the sweat glands, and the genitourinary system.

Although there are many mutations that can result in the disease with variying intensities ranging from 'full-blown disease' to the asymptomatic cases, the most prevalent mutation is the deletion of a single phenylalanine residue at 508th amino acid ( F508 ).
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COAL WORKERS' PNEUMOCONIOSIS
Coal workers' pneumoconiosis is a parenchymal lung disease caused by inhaling coal mine dust. The basic pathophysiological mechanism responsible for the disease is simply, deposition of coal mine dust in the lung parenchyma and the reaction of tissue to its presence.

Although dust reduction measures in the coal mines have drastically reduced the incidence of coal dust induced disease, radiographically identified simple coal workers' pneumoconiosis is seen in 12% of all miners and in approximately 50% of anthracite miners with more than 20 years of underground mining history.

Although long term exposure to coal dust can also lead to Silicosis. Coal workers' pneumoconiosis is accepted as distinct from silicosis by means of epidemiological,pathological and prognostic differences.

The disease is customarily divided into simple and complicated pneumoconiosis, according to the radiographic features. The disease is termed "simple" if all radiographic opacities are less than 1 cm in diameter.
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Cystic fibrosis is an inherited multisystem disease characterized chiefly by obstruction of airways and pancreatic duct by viscous secretions resulting in recurrent respiratory tract infections and maldigestion.>>

 

 

 

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