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<p>[QUOTE="kinghamada, post: 52712"]This article is to discuss Cystic fibrosis, which is a congenital disorder , also named Congenital polycystic lung or mucoviscidosis .We will discuss its definition, pathogenesis, clinical picture, investigation and management .</p><p>What is meant by Cystic fibrosis ?</p><p>Definition : it is a congenital disease that affects multiple systems ,mainly the lungs , digestive system, sweat glands, and male fertility .</p><p>The name "Cystic fibrosis" derives from the fibrous scar tissue that develops in the pancreas, one of the principal organs affected by the disease .</p><p>Cystic-fibrosisCystic-fibrosis-figure</p><p><br /></p><p><br /></p><p>Pathogenesis and causes of Cystic fibrosis </p><p>• There is a change in the viscosity and tenacity of mucous produced at epithelial surfaces.</p><p>• The disease includes mainly bronchopulmonary infection, pancreatic insufficiency and biliary cirrhosis with high sweat sodium and chloride levels.</p><p>• it is an autosomal recessive inherited disorder with gene mutation on the long arm of chromosome 7, producing abnormal membrane transport protein called cystic fibrosis transmembrane regulator (CFTR) leading to decreased chloride excretion into the airway lumen and increased reabrorption of sodium into the epithelial cells .</p><p>So, less excretion of salt leads to less of secretion of water ==> increased viscosity of secretions.</p><p>• Also, there a CFTR independent mechanism of chloride secretion in the sweat glands with impaired reabsorption of sodium chloride in the distal end of the duct</p><p>leading to increased salt content of sweat.</p><p>What are the organs affected in cystic fibrosis ?</p><p>cystic-fibrosis-organs-affected</p><p><br /></p><p>How to diagnose cystic fibrosis ?</p><p><font size="5"><span style="color: Red">Source</span></font> : <a href="http://www.med4al.com/2025/07/cystic-fibrosis.html" target="_blank" class="externalLink ProxyLink" data-proxy-href="http://www.med4al.com/2025/07/cystic-fibrosis.html" rel="nofollow">Cystic fibrosis (mucoviscidosis), Congenital polycystic lung diagnosis and treatment</a>[/QUOTE]</p><p><br /></p>
[QUOTE="kinghamada, post: 52712"]This article is to discuss Cystic fibrosis, which is a congenital disorder , also named Congenital polycystic lung or mucoviscidosis .We will discuss its definition, pathogenesis, clinical picture, investigation and management . What is meant by Cystic fibrosis ? Definition : it is a congenital disease that affects multiple systems ,mainly the lungs , digestive system, sweat glands, and male fertility . The name "Cystic fibrosis" derives from the fibrous scar tissue that develops in the pancreas, one of the principal organs affected by the disease . Cystic-fibrosisCystic-fibrosis-figure Pathogenesis and causes of Cystic fibrosis • There is a change in the viscosity and tenacity of mucous produced at epithelial surfaces. • The disease includes mainly bronchopulmonary infection, pancreatic insufficiency and biliary cirrhosis with high sweat sodium and chloride levels. • it is an autosomal recessive inherited disorder with gene mutation on the long arm of chromosome 7, producing abnormal membrane transport protein called cystic fibrosis transmembrane regulator (CFTR) leading to decreased chloride excretion into the airway lumen and increased reabrorption of sodium into the epithelial cells . So, less excretion of salt leads to less of secretion of water ==> increased viscosity of secretions. • Also, there a CFTR independent mechanism of chloride secretion in the sweat glands with impaired reabsorption of sodium chloride in the distal end of the duct leading to increased salt content of sweat. What are the organs affected in cystic fibrosis ? cystic-fibrosis-organs-affected How to diagnose cystic fibrosis ? [SIZE=5][COLOR=Red]Source[/COLOR][/SIZE] : [URL="http://www.med4al.com/2025/07/cystic-fibrosis.html"]Cystic fibrosis (mucoviscidosis), Congenital polycystic lung diagnosis and treatment[/URL][/QUOTE]
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