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32 Cards in this Set

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  • Back
1. Clubbing?
a. An increase in the angle between the nail and nail base of 180° or greater and softening of the nail base to palpation.
b. Although the condition can be familial, clubbing is uncommon in children, usually indicating chronic pulmonary, hepatic, cardiac, or GI disease.
2. Cystic Fibrosis?
major cause of chronic debilitating pulmonary disease and pancreatic exocrine deficiency, and abnormally high sweat electrolyte concentrations.
b. Characteristic pancreatic changes give the disease its name.
3. What lung consequence to most pts w/CF develop by 18 months?
a. Bronchiectasis.
b. Although some may not experience respiratory difficulty for several years.
4. What is Bacterial pneumonia caused by in Cystic Fibrosis?
a. Initially by staph aureus
b. Then by Pseudomonas aeruginosa.
c. Most pts w/advanced disease harbour heavy, slime-producing mucoid variants of P aeruginosa rarely found in other conditions. Once established, these bacteria are virtually impossible to eradicate.
5. Colonization with what bacteria may be associated with a rapid clinical deterioration in CF pts?
a. Burkholderia cepacia.
6. Bronchodilator response in CF?
a. Airway reactivity is present in 50% of pts, but bronchodilator response is unpredictable and varies.
7. Frequent complications of advanced disease CF?
1. Pneumothorax
2. Haemoptysis
3. Cor pulmonale.
b. Pulmonary problems ultimately cause respiratory and cardiac failure.
c. Chronic nasal congestion and sinus opacification is common, but acute sinusitis occurs infrequently.
8. Nasal issues in CF?
a. May develop nasal polyps, w/resultant nasal obstruction, HA, and mouth breathing.
9. Growth in CF children?
a. Grow poorly due to maldigestion from exocrine pancreatic insufficiency.
10. GI issues w/CF?
a. They may have abdominal distention, rectal prolapse, subQ fat and muscle tissue deficiency, and frequent passage of oily, malodorous stools.
b. These stools predispose to obstruction, volvulus, or intussusception.
11. Liver complications in CF?
a. Fatty liver infiltration or focal biliary cirrhosis.
b. Hepatomegaly, oesophageal varices, and hypersplenism caused by portal HTN develop in a small proportion of teens.
c. Gallbladder disease is common in adults.
d. Abdominal pain is relatively common.
12. CF liver complications in neonates?
a. May have prolonged jaundice.
13. Non-GI issues w/CF?
a. Azoospermia
b. Enlarged Submaxillary glands
c. Osteoarthropathy!!!!
d. Salty taste on skin (due to eccrine sweat gland dysfunction).
e. Pts and their families require extensive psychosocial support.
14. What is the diagnosis of CF based on?
a. A positive sweat test; in conjunction with one of the following:
b. Typical COPD
c. Documented exocrine pancreatic insufficiency
d. And/or a positive family history.
15. People w/CF have elevated sweat electrolyte concentration bc of abnormalities in the CF transmembrane conductance regulator (CFTR) protein.
15. People w/CF have elevated sweat electrolyte concentration bc of abnormalities in the CF transmembrane conductance regulator (CFTR) protein.
16. When is genetic CF testing used?
a. When CF is suspected but the result of sweat testing are negative or equivocal.
17. What gene mutation causes CF?
a. Long arm of chromosome 7. The most common being
b. Single phenylalanine deletion at amino acid 508 (Δ508).
18. What does routine screening for Cystic Fibrosis detect?
a. Blood sport screening test detects the pancreatic enzyme Immunoreactive Trypsinogen (IRT)
b. It is elevated in CF.
19. Second-tier testing if IRT is high?
a. A second IRT test or DNA testing.
20. What is the next step with for infants w/positive IRT results?
a. They undergo sweat chloride testing for definitive confirmation.
21. Note: False-negative results are possible w/the IRT newborn screen, so infants w/symptoms suggestive of CF (such as meconium ileus) should undergo further testing even if newborn screen results are negative.
21. Note: False-negative results are possible w/the IRT newborn screen, so infants w/symptoms suggestive of CF (such as meconium ileus) should undergo further testing even if newborn screen results are negative.
22. Note: (routine screening has been shown to improve nutritional status, growth, and reduce hospitalisations)
22. Note: (routine screening has been shown to improve nutritional status, growth, and reduce hospitalisations)
23. Who is best equipped (physician) for long-term management of CF pts?
a. Experienced Paediatric pulmonary specialists.
24. Long-term management of CF?
a. Minimizing airway reactivity and infections
b. Optimizing nutritional status
c. Providing ongoing psychosocial support.
25. Meconium Ileus?
a. Inspissated meconium obstructing the distal ileum.
b. Thought to be caused by deficiency or proteolytic enzymes.
c. Obstruction begins in utero, resulting in underdevelopment of distal lumina.
d. Essentially Pathognomonic for CF
e. Meconium Ileus is a surgical emergency.
26. S/S of meconium ileus?
a. Infant developing vomiting and abdominal distention in the first day of life.
b. No stool passage
c. Abdominal radiograph shows distended bowel loops and a “bubbly” pattern in a portion of the intestine.
d. The colon is narrow.
27. What 2 conditions cause similar symptoms to meconium ileus?
a. Intestinal atresia and Hirschsprung disease (congenital aganglionic megacolon).
28. Necrotizing enterocolitis (not a cf condition)?
a. Also causes emesis and abdominal distension but occurs primarily in extremely low-birth-weight infants (<1000 g).
b. The colon would be expected to be of normal size.
29. In what other condition is bronchiectasis and chronic sinusitis characteristic?
a. Kartagener disease.
30. Sx of vitamin A metabolism disorders?
a. Pseudotumour cerebri (↑’d ICP), which causes HA, vomiting, and neurologic abnormalities.
b. Thus, a bulging fontanelle may be the presenting sign of CF in an infant.
31. Extrapulmonary s/s of Cystic Fibrosis?
a. Digital clubbing
b. Recurrent sinusitis
c. Growth retardation
d. Fat malabsorption.
32. Does a negative sweat chloride test preclude CF?
a. No