Describe the underlying pathology of cystic fibrosis including any and all mechanisms affected by the disease.  Be sure to include all channels/transporters involved and the clinical consequences due to the underlying pathology.

Human Anatomy & Physiology (11th Edition)
11th Edition
ISBN:9780134580999
Author:Elaine N. Marieb, Katja N. Hoehn
Publisher:Elaine N. Marieb, Katja N. Hoehn
Chapter1: The Human Body: An Orientation
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Describe the underlying pathology of cystic fibrosis including any and all mechanisms affected by the disease.  Be sure to include all channels/transporters involved and the clinical consequences due to the underlying pathology.

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Step 1: Introduction:

Cystic fibrosis is a progressive hereditary disorder that affects the lungs and stomach related framework. The body creates thick and clingy bodily fluid that can clog up the lungs and block the pancreas. It is an autosomal recessive disorder.

Cystic fibrosis (CF) can be perilous, and individuals with the condition will in general have a shorter than-typical life expectancy. It is a chronic disease and can last for life-long.

There is presently no remedy for CF. It influences exactly 30,000 individuals in the US with around 1,000 new cases analyzed every year. Of these new diagnoses, 75 percent are made in kids younger than 2 years. In the past years kids with CF did not survive. But with advancement of medicine, they now survive upto the age of 30-40 years.

Symptoms include breathing distress, shortness of breath, intermittent coughing, sneezing and wheezing, chest pain, loss of weight, fatty stool, pneumonia, pale face with outgrowth of flesh near nasal area etc.

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