Peds/Geri – Cystic Fibrosis (CPC)

A 2 year old girl is being evaluated for cough, loose stools and failure to thrive. Which of the following tests has the highest sensitivity and specificity in establishing the diagnosis of cystic fibrosis in this child? A. DNA testing for CFTR genetic mutation B. Meconium trypsin concentration C. Nasal potential difference D. Immunoreactive trypsinogen in the blood E. Sweat chloride concentration

A 3 month old with failure to thrive has two sweat chloride tests of 72 and 68 mmol/L. Which of the following additional findings are necessary to confirm a diagnosis of cystic fibrosis? A. Bronchiectasis B. Family history of CF C. DNA mutational analysis D. Stool study documenting fat malabsorption E. No further information needed

Among the following the most frequent manifestation of CF in the neonatal period is A. Electrolyte abnormalities B. Meconium ileus C. Prolonged jaundice D. Rectal prolapse E. Respiratory distress syndrome

Which of the following is NOT a pulmonary complication of CF? A. Massive hemoptysis B. Atelectasis and acute respiratory failure C. Allergic bronchopulmonary aspergillosis D. Pneumothorax E. Chylothorax

Cor pulmonale occurs in conditions that cause pulmonary hypertension due to ventilation abnormalities. One of the most common causes of cor pulmonale in pediatric patients is cystic fibrosis. Of the following, the finding that is MOST consistent with a diagnosis of cor pulmonale is A. Apical systolic murmur B. Hepatomegaly C. Left ventricular enlargement D. Pulmonary ejection click E. Widely split S2

A 7 month old breastfed Caucasian girl has gained weight poorly since birth. The finding most strongly suggests a diagnosis of cystic fibrosis is: A. Frequent colds B. Excellent appetite C. Recurrent ear infections D. Hyperchloremic metabolic acidosis

A significantly elevated sweat chloride test is most likely to be found in A. An 8 month old who has failure to thrive and has no history of diarrhea B. A 10 month old infant who has Vitamin K deficiency who presents with bruising C. A 16 month old infant who has an episode of hypernatremic dehydration D. An 18 month old infant who presents with poor appetite, chronic diarrhea and malnutrition developing late in the first year E. A 2 year old child who has a history of 3 episodes of pneumonia in the right lower lobe

The life expectancy of a 15 year old teenage boy who has cystic fibrosis is best predicted by A. Congenital bilateral absence of the vas deferens B. History of distal intestinal obstruction syndrome C. Infection with Burkholderia cepacia D. Recent pulmonary hemorrhage E. Development of diabetes

A 15 year old female adolescent who has CF has had a viral URI for 3 days. She develops an increased cough productive of purulent sputum over the subsequent 5 days. On auscultation, moderate crackles are newly heard in the lower lung fields. Sputum culture is positive for Pseudomonas sp. Chest x ray is unchanged. The most appropriate management of the patient is A. Aerosol administration of Albuterol B. Increase in daily courses of chest PT 7-10 days prior to decision regarding antibiotic therapy C. Initiation of corticosteroid therapy to reduce airway inflammation D. Oral administration of cefaclor, a semisythetic cephalosporin E. Oral administration of ciprofloxacin, a fluoroquinolone

A 5-year-old girl who has cystic fibrosis (CF) is producing thick, green sputum and has developed fever and worsening cough. Her weight has decreased by 1 kg since her last visit 1 month ago. She is admitted to the hospital for management of a pulmonary exacerbation of CF. Of the following, the most appropriate INITIAL antibiotic combination to treat this infection is A. ampicillin/sulbactam plus gentamicin B. ceftazidime plus tobramycin C. ceftriaxone plus trimethoprim/sulfamethoxazole D. ceftriaxone plus vancomycin E. ciprofloxacin plus trimethoprim/sulfamethoxazole

Which of the following statements regarding the mechanism of action of Doranse alfa, recombinant human deoxyribonuclease (rhDNase) in CF is correct? A. Cleaves the extracellular DNA form the neutrophils in sputum to make it less viscous B. It has a potent antibacterial action that prevents the growth of Pseudomonas C. It has antibacterial, bronchodilator and mucolytic actions D. It corrects the defect in the CF gene on chromosome 7

A 6 year old girl has known cystic fibrosis. Currently she is asymptomatic. The most important component of her daily pulmonary management regimen is A. Aerosol treaments with N-acetyl-L cysteine B. Aerosol treatments with Albuterol C. Chest physiotherapy with percussion D. Oral prophylactic antibiotic

The management of pancreatic insufficiency in a child with cystic fibrosis is optimized by offering A. Dietary calories based on age B. Low fat diets C. High dose pancreatic enzyme replacement D. Proton pump inhibitors E. Supplemental vitamins B and C

What is two step approach to neonatal screen program for Cystic Fibrosis?