Cystic Fibrosis

Which statement explains the rationale as to why a person with cystic fibrosis develops gallstones?

Mucus plugs the biliary ducts, causing bile stasis.

Which is the probability that two parents who are carriers of the cystic fibrosis gene will give birth to a child with the disease?

25%

Which statistics is correct regarding the prevalence of cystic fibrosis?

30,000 people

Which is the probability of a child being a carrier of the cystic fibrosis gene when both parents are carriers?

50%

Which is the best response if an individual asks what causes cystic fibrosis?

A genetic flaw regarding chloride transportation into the cell membrane is present.

Which finding would cause the most concern for a parent of a newborn child with cystic fibrosis?

A salty taste to the skin

Which genetic defect causes cystic fibrosis?

CFTR gene Mutations to the CFTR gene lead to a protein deficiency that causes cystic fibrosis.

Which deficiencies are common in cystic fibrosis and are known to contribute to osteoporosis?

Calcium Calcium is deficient in people with cystic fibrosis due to malabsorption. Calcium deficiencies can lead to osteoporosis. Vitamin D Vitamin D deficiencies are found in people with cystic fibrosis due to an inability to absorb this nutrient. Vitamin D deficiency can cause osteoporosis.

The development of which complications would suggest cystic fibrosis (CF) has worsened in a person?

Cor pulmonale Pulmonary hypertension

Which factors contribute to difficulty breathing in individuals with cystic fibrosis?

Impaired gas exchange Decreased ciliary motility Increased bronchial mucus production

A male with CF is having difficulty conceiving a child with his spouse, who also has CF. Which complication may make this more difficult?

Incomplete vas deferens development

A baby is born to two parents who both carry the gene for cystic fibrosis. Which finding might indicate the newborn has the disease? Hypoxia Fatigue Activity intolerance Lack of meconium in 24 hours

Lack of meconium in 24 hours

A person with cystic fibrosis reports increased difficulties with constipation. Which statements explain why constipation occurs?

Opioid use from chronic pain causes constipation. Thickened, dried out stool can cause constipation. Chronic ingestion of mucus can lead to constipation. Dehydration occurs from protein mutation in the CFTR gene.

Parents of a 6-month-old infant with CF present to the emergency room because the infant is having difficulty breathing. Which infection would be most common in an infant with cystic fibrosis?

Staph aureus

Which clinical manifestation indicates a person with CF is experiencing fat malabsorption?

Steatorrhea Steatorrhea, or fatty stools, takes place as a result of inadequate pancreatic enzyme production, causing fat malabsorption from the gastrointestinal tract.

Which findings would be expected in a person with CF who has developed pancreatic insufficiency from the disease?

Steatorrhea Vitamin A deficiency Delayed growth and development

Which phrase explains the rationale for the development of diabetes mellitus in a person with cystic fibrosis?

Underdevelopment of islet cells in utero

Which clinical manifestations may be present in a person having a CF exacerbation?

Weight loss Increased sputum amount Decreased pulmonary function

Which complication may occur due to obstructed salivary glands?

Xerostomia