Cystic Fibrosis Questions & Answers

Updated: Jun 26, 2019
  • Author: Girish D Sharma, MD, FCCP, FAAP; Chief Editor: Kenan Haver, MD  more...
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Questions & Answers

Overview

What is cystic fibrosis (CF)?

What are the GI symptoms of cystic fibrosis (CF)?

What are respiratory symptoms of cystic fibrosis (CF)?

What are genitourinary symptoms of cystic fibrosis (CF)?

What are the possible physical findings of cystic fibrosis (CF)?

What are the diagnostic criteria for cystic fibrosis (CF)?

Which imaging studies may be performed in the workup of cystic fibrosis (CF)?

Which supplemental tests may be indicated in the workup of cystic fibrosis (CF)?

What are the primary treatment goals of cystic fibrosis (CF)?

What is the treatment for mild symptoms of cystic fibrosis (CF)?

Which medications are used to treat cystic fibrosis (CF)?

When is surgical therapy indicated for cystic fibrosis (CF)?

What is cystic fibrosis (CF)?

What is the cause of cystic fibrosis (CF)?

What is the role of CFTR mutations in the pathogenesis of cystic fibrosis (CF)?

What are manifestations of cystic fibrosis (CF)?

What causes sinus disease in the pathogenesis of cystic fibrosis (CF)?

How does lung disease originate in the pathogenesis of cystic fibrosis (CF)?

How does cystic fibrosis (CF) affect intestinal functioning?

What is the role of meconium ileus in the pathogenesis of cystic fibrosis (CF)?

How does cystic fibrosis (CF) affect pancreatic functioning?

How does cystic fibrosis (CF) affect liver functioning?

How does cystic fibrosis (CF) affect fertility?

Where is the genetic etiology of cystic fibrosis (CF)?

Which ethnicities carry genetic mutations that can cause cystic fibrosis (CF)?

How do CFTR mutations cause cystic fibrosis (CF)?

What is the role of genetic modifiers in the etiology of cystic fibrosis (CF)?

What is the prevalence of cystic fibrosis (CF) in the US?

How does the prevalence and clinical presentation of cystic fibrosis (CF) vary by race?

How does the prevalence of cystic fibrosis (CF) vary by sex?

What is the prognosis of cystic fibrosis (CF)?

Which complications can affect the prognosis of cystic fibrosis (CF)?

What are negative prognostic factors in cystic fibrosis (CF)?

What education about cystic fibrosis (CF) should patients and families receive?

Presentation

At what age is cystic fibrosis (CF) typically diagnosed?

What are GI symptoms of cystic fibrosis (CF)?

What are respiratory symptoms of cystic fibrosis (CF)?

What are urogenital symptoms of cystic fibrosis (CF)?

Which nasal findings suggest cystic fibrosis (CF)?

Which pulmonary findings suggest cystic fibrosis (CF)?

Which GI findings suggest cystic fibrosis (CF)?

What are possible physical findings suggestive of cystic fibrosis (CF)?

What cystic fibrosis (CF) etiology is suggested by a finding of aquagenic wrinkling of the palms (AWP)?

How is bone health affected by cystic fibrosis (CF)?

What are signs of peritonitis in cystic fibrosis (CF)?

What are atypical manifestations of cystic fibrosis (CF)?

What are potential complications of cystic fibrosis (CF)?

DDX

What are the differential diagnoses for Cystic Fibrosis?

Workup

What are the criteria for the diagnosis of cystic fibrosis (CF)?

How is prenatal diagnosis of cystic fibrosis (CF) made?

How are neonates screened for cystic fibrosis (CF)?

How is cystic fibrosis (CF) diagnosed?

Which sweat test is performed for diagnosis of cystic fibrosis (CF)?

How much sweat is needed for an accurate diagnosis of cystic fibrosis (CF)?

What sweat chloride reference value is required for a positive diagnosis of cystic fibrosis (CF)?

Which conditions that cause elevated levels of sweat chloride should be included in the differential diagnoses of cystic fibrosis (CF)?

What is the role of chest radiography in the workup of cystic fibrosis (CF)?

What is the role of sinus radiography in the workup of cystic fibrosis (CF)?

What is the role of abdominal radiography in the workup of cystic fibrosis (CF)?

What is the role of CT scanning in the workup of cystic fibrosis (CF)?

What is the role of ultrasonography in the workup of cystic fibrosis (CF)?

Which conditions that cause hyperechoic bowel should be considered in the differential diagnoses of cystic fibrosis (CF)?

Which GI conditions should be included in the differential diagnoses of cystic fibrosis (CF)?

What is the role of lung clearance index (LCI) in the workup of cystic fibrosis (CF)?

What is the role of ventilation MRI in the workup of cystic fibrosis (CF)?

When is genotype testing indicated in the workup of cystic fibrosis (CF)?

How is genotype testing performed in the workup of cystic fibrosis (CF)?

What is the role of nasal potential difference testing in the workup of cystic fibrosis (CF)?

Which pulmonary function tests are used in the workup of cystic fibrosis (CF)?

What is the role of pulmonary function testing in the workup of cystic fibrosis (CF)?

What is the role of BAL in the workup of cystic fibrosis (CF)?

What are the most common bacterial agents found in the sputum of patients with cystic fibrosis (CF)?

What is the role of immunoreactive trypsinogen (IRT) measurement in the workup of cystic fibrosis (CF)?

What is the role of contrast barium enema in the workup of cystic fibrosis (CF)?

Treatment

What are the initial steps in the management of cystic fibrosis?

What are the primary goals of treatment for cystic fibrosis (CF)?

How are mild pulmonary symptoms of cystic fibrosis (CF) treated?

What are medications used to treat cystic fibrosis (CF)?

What is the role of hypertonic saline inhalation in the treatment of cystic fibrosis (CF)?

What is the treatment for prenatally diagnosed meconium ileus in cystic fibrosis (CF)?

What is the role of ivacaftor (Kalydeco) in the treatment of cystic fibrosis (CF)?

What are the risks and benefits of corticosteroids for the treatment of cystic fibrosis (CF)?

What is the role of mannitol or tobramycin in the treatment of cystic fibrosis (CF)?

What is the treatment for bone mineral density (BMD) loss in cystic fibrosis (CF)?

What are the dietary restrictions for patients with cystic fibrosis (CF)?

What is the role of exercise in the management of cystic fibrosis (CF)?

Which respiratory complications of cystic fibrosis (CF) are treated surgically?

Which GI complications of cystic fibrosis (CF) are treated surgically?

When is a full lung transplant indicated in the treatment of cystic fibrosis (CF)?

How is cystic fibrosis (CF) treated during pregnancy?

What are the treatment options for cystic fibrosis (CF) comorbid with diabetes?

Which specialists should be consulted for the treatment of cystic fibrosis (CF)?

What monitoring is needed the management of cystic fibrosis (CF)?

What lab testing should be performed at each clinic visit for cystic fibrosis (CF)?

Which techniques are used for clearing airways in cystic fibrosis (CF)?

What is the role of immunizations in the management of cystic fibrosis (CF)?

Medications

What are common medications used for the treatment of cystic fibrosis (CF)?

Which medications in the drug class Antibiotics are used in the treatment of Cystic Fibrosis?

Which medications in the drug class CFTR Potentiators and Correctors are used in the treatment of Cystic Fibrosis?

Which medications in the drug class Mucolytic Agents are used in the treatment of Cystic Fibrosis?

Which medications in the drug class Bronchodilators are used in the treatment of Cystic Fibrosis?

Which medications in the drug class Vitamins are used in the treatment of Cystic Fibrosis?

Which medications in the drug class Enzymes, Pancreatic are used in the treatment of Cystic Fibrosis?