Cystic Fibrosis is caused by an alteration in what protein? What is its function?

Cystic fibrosis is caused by an alteration in the CFTR protein. CFTR allows chloride ions to move across the cell membane.

What is the primary mechanism of CF?

There is too much intracellular sodium, which pulls out the water from mucus.

CF affects what glands

exocrine glands, lungs, gi tract, pancreas, intestines, liver, reproductive organs.

Lack of digestive enzymes in CF causes

Chronic diarrhea and nutritional deficits, Failure to thrive, and impaired glucose levels

Frequent respiratory infections in CF causes

Fibrosis, or stiffening of the lungs.

What respiratory sx does CF cause?

Cough, sinusitis, periods of hypoxia, dyspnea, and clubbing of the fingers.

Liver impairment in CF causes

High billies high ALT and AST levels.

Treatment of CF includes

Abx, bronchopulmonary lavage, bronchodilators, mucolytics, CPT, lobectomy

Experimental treatment for CF includes

DNA, transplant, gene therapy, and exercise

What is a bronchoalveolar lavage?

They put a tube down your throat then they instill water and suck it back out

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Exam 1.6

Question	Answer
Cystic Fibrosis is caused by an alteration in what protein? What is its function?	Cystic fibrosis is caused by an alteration in the CFTR protein. CFTR allows chloride ions to move across the cell membane.
What is the primary mechanism of CF?	There is too much intracellular sodium, which pulls out the water from mucus.
Is CF a part of the new born screen at birth?	Ye
Inherited CF is	autosomal recessive,
CF affects what glands	exocrine glands, lungs, gi tract, pancreas, intestines, liver, reproductive organs.
Lack of digestive enzymes in CF causes	Chronic diarrhea and nutritional deficits, Failure to thrive, and impaired glucose levels
Frequent respiratory infections in CF causes	Fibrosis, or stiffening of the lungs.
What respiratory sx does CF cause?	Cough, sinusitis, periods of hypoxia, dyspnea, and clubbing of the fingers.
Liver impairment in CF causes	High billies high ALT and AST levels.
Treatment of CF includes	Abx, bronchopulmonary lavage, bronchodilators, mucolytics, CPT, lobectomy
Experimental treatment for CF includes	DNA, transplant, gene therapy, and exercise
What is a bronchoalveolar lavage?	They put a tube down your throat then they instill water and suck it back out
Should they take their enzyme replacements before or after eating?	Before
What causes the fatty greasy stools seen in CF?	Lack of digestive enzymes

Created by: pedsstudysets

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