Friday, August 10, 2012

Cystic Fibrosis




CYSTIC FIBROSIS (CF): The major cause of chronic debilitating pulmonary disease and pancreatic exocrine deficiency in the first three decades of life. It is characterized by the triad of chronic obstructive pulmonary disease, pancreatic exocrine deficiency, and abnormally high sweat electrolyte concentrations.
Characteristic pancreatic changes give the disease its name.

➤ Cystic fibrosis (CF) involves a defect in mucus secretion and eccrine sweat gland function, resulting in various visceral lumina obstructions and excessive electrolyte secretion.

➤ Extrapulmonary signs and symptoms, such as digital clubbing, recurrent sinusitis, growth retardation, and fat malabsorption, are clues to the diagnosis of cystic fibrosis.

➤ A negative sweat chloride test result does not preclude cystic fibrosis.

➤ Meconium ileus in the newborn period is nearly pathognomonic for cystic fibrosis

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