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MSN Exam for Cystic Fibrosis

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1) Association systems fictitious by cystic fibrosis embody:

  1. Pancreas
  2. Sweat glands
  3. Lungs
  4. All of these
2) Which of the forthcoming clients is at elevated betray for fruital total?
  1. A toddler succeeding a era intelligent Glomerulonephritis on antihypertensive and antibiotics
  2. A 5-year-old succeeding a era asthma on cromolyn sodium
  3. A preschooler succeeding a era tonsillitis
  4. A 2 1/2 –year old boy succeeding a era cystic fibrosis
3) The recommended nourishment for someone succeeding a era cystic fibrosis is:
  1. High calorie and elevated fat nourishment
  2. High fat nourishment
  3. Low fat nourishment
  4. High calorie nourishment
4) If one of your constructors enjoy the disdirect does that balance you enjoy a casualty of getting the contagion?
  1. True
  2. False
5) An 8-year-old client succeeding a era cystic fibrosis is admitted to the hospital and allure endure a chest physiotherapy composition. The therapy should be expendly coordinated by the promote succeeding a era the respiratory therapy portion so that compositions befall during:
  1. After macerations
  2. Between macerations
  3. After medication
  4. Around the slip’s state schedule
6) Which of the forthcoming is the near preponderance appurtenancy for cystic fibrosis?
  1. 1: 25,000
  2. 1: 5,000
  3. 1: 2,000
  4. 1: 800
7) Cystic fibrosis is treated by:
  1. Airway colliquation techniques that aim to propel the dull mucus from the lungs
  2. Taking extraordinary vitamins
  3. Taking pancreatic enzymes to acceleration direct fixation
  4. All of these
8)  The dowager of a slip succeeding a era cystic fibrosis mentions the promote that her slip makes “snoring” sounds when lively. The promote is assured that divers slipren succeeding a era cystic fibrosis enjoy:
  1. Choanal atresia
  2. Nasal polyps
  3. Septal deviations
  4.  Enlarged adenoids
9) Cystic fibrosis is accountd by:
  1. A insufficient gene that accounts irregularities in the brain
  2. It is not unreserved what the account is
  3. Someone who eats too abundantly salt
  4. A insufficient gene that leads to the making of an irregular protein
10) The promote is providing a bloom education to the dowager of an 8-year-old slip succeeding a era cystic fibrosis. Which of the forthcoming announcement if made by the dowager would evince to the promote the scarcity for raise education encircling the medication sustenance of the slip?
  1. “My slip jurisdiction scarcity an extra capsule if the maceration is elevated in fat”
  2. “I’ll yield the enzyme capsule anteriorly total snack”
  3. “I’ll yield the enzyme capsule anteriorly total maceration”
  4. “My slip hates to admit pills, so I’ll mix the capsule into a cup of hot chocolate
11) At what age is this disdirect diagnosed?
  1. Early years [0-5]
  2. Childhood years [5-12]
  3. Teenage years [12-17]
  4. Adult years [18-50]
  5. 50+
12) A slip succeeding a era cystic fibrosis is entity treated succeeding a era life therapy succeeding a era Pulmozyme (dornase alfa). A party result of the medication is:
  1. Weight gain
  2. Hair loss
  3. Sore throat
  4. Brittle nails
13) A 6-year-old succeeding a era cystic fibrosis has an direct for Creon. The promote knows that the medication allure be fond:
  1. At bedtime
  2. With macerations and snacks
  3. Twice daily
  4. Daily in the morning
14) The promote in accuse is reviewing a resigned’s prenatal fact. Which judgment evinces a genetic betray content?
  1. The resigned is 25 years old
  2. The resigned has a slip succeeding a era cystic fibrosis
  3. The resigned was unguarded to rubella at 36 weeks’ gestation
  4. The resigned has a fact of preterm work at 32 weeks’ gestation
15) A slip who has of-late been diagnosed succeeding a era cystic fibrosis is in a pediatric clinic wless a promote is enacting an tribute. Which behind judgment of this disdirect would the promote not rely-on to see at this span?
  1. Positive perspirationinginging proof
  2. Bulky greasy stools
  3. Moist, efficient cough
  4. Meconium ileus
16) A client succeeding a era cystic fibrosis is commencement pancreatic enzymes. The promote should carry this medication:
  1. Once per day in the morning
  2. Three spans per day succeeding a era macerations
  3. Once per day at bedtime
  4. Four spans per day
17) Is tless a heal for Cystic Fibrosis?
  1. True
  2. False
18) The foul-smelling, frothy singularity of the stool in cystic fibrosis results from the influence of big totals of which of the forthcoming:
  1. sodium and chloride
  2. discursive fat
  3. semi-digested carbohydrates
  4. lipase, trypsin and amylase
19) A courageous adult client succeeding a era cystic fibrosis is admitted to an intelligent anxiety adroitness succeeding a era an intelligent respiratory contagion. Prescribed respiratory comlie embodys chest physiotherapy. When should the promote enact this mode?
  1. Immediately anteriorly a maceration
  2. At moderationest 2 hours behind a maceration
  3. When bronchospasms befall
  4. When secretions enjoy mobilized
20) A 2-year-old client succeeding a era cystic fibrosis is intolerant to bed and is not unreserved to go to the stateroom. Which of the forthcoming is an expend toy would the promote laudable for the slip:
  1. Puzzle
  2. Musical automobile
  3. Arranging stickers in the album
  4. Pounding consultation and hold
21) Cystic fibrosis is diagnosed by:
  1. Echocardiogram
  2. Complete lineage panel
  3. Chest X-ray
  4. Sweat proof
22) Cystic fibrosis is too determined:
  1. Bronchiectasis
  2. Sticky mucus empiricism
  3. Mucovicidosis
  4. Irritating cough empiricism
23) One of the most tokenificant pulmonary compositions in cystic fibrosis is:
  1. inhaled beta agonists.
  2. inhaled corticosteroids.
  3. chest physiotherapy.
  4. vocal enzymes.
24) When carrying pancrelipase (Pancreases capsules) to slip succeeding a era cystic fibrosis, promote Faith knows they should be fond:
  1. With macerations and snacks
  2. Every three hours era awake
  3. On awakening, forthcoming macerations, and at bedtime
  4. After each bowel propelment and behind postural drainage
25) The promote is education the dowager of a slip succeeding a era cystic fibrosis how to do postural drainage. The promote should mention the dowager to:
  1. Use the heel of her operative during collision
  2. Change the slip’s lie total 20 minutes
  3. Do collision behind the slip eats and at bedtime
  4. Use cupped operatives during collision
Answers and Rationales
  1. D. All of these . Cystic fibrosis chiefly affects the glands that conceal. An irregular protein is resulted making secretions viscous. These dullened secretions are fix in the association’s perspirationinging, the lungs’ mucus, the pancreas’ output, liver output, and reefficient organs.
  2. D. A 2 1/2 –year old boy succeeding a era cystic fibrosis . It is the fruital work of an 18-month-old toddler to ponder and imbibe encircling the environment. The respiratory complications associated succeeding a era cystic fibrosis (which are yield in nearly all slipren succeeding a era cystic fibrosis) could obstruct this fruit work from befallring.
  3. A. Elevated calorie and elevated fat nourishment . The vulgar advice is 120-150% of the recommended daily equivalent of calories for slipren, and 40% fat. Adults succeeding a era cystic fibrosis too scarcity to eat elevated calorie fixation, succeeding a era increased fat. This is consequently a individual succeeding a era cystic fibrosis doesn’t assume nutrients as polite from their fixation, chiefly fat. A incontrovertible total of fat is scarcityed in your nourishment to conceal you hale.
  4. B. False . Both your constructors must enjoy the gene in direct for you to get Cystic Fibrosis
  5. B. Betwixt macerations . Chest physiotherapy compositions are scheduled betwixt macerations to obstruct appetition of stomach interruption, consequently the slip is settled in a medley of lies during the comlie arrangement.
  6. C. 1: 2,000 
  7. D. All of these . All of these are compositions for cystic fibrosis. Airway colliquation techniques acceleration mob succeeding a era cystic fibrosis propel dullened mucus from the lungs. Pancreatic enzymes are captured to acceleration direct the fixation. Extraordinary vitamins are scarcityed to acceleration re-establish vitamins A, D, and K which may not be fascinated polite by the CF resigned. Tless are past compositions, of race, but these are the deep ones to acceleration conceal the individual succeeding a era cystic fibrosis hale.
  8. B. Nasal polyps. Children succeeding a era cystic fibrosis are tender to continuous sinusitis and nasal polyps, which jurisdiction claim surgical dissolution. Retort A is inemend consequently it is a probable mood in which tless is a lean obstacle betwixt the nares and the pharynx. Answers C and D are not peculiar to the slip succeeding a era cystic fibrosis; accordingly, they are insufficient.
  9. D. A insufficient gene that leads to the making of an irregular protein . Usually, the gene concerned makes a protein determined CFTR (Cystic Fibrosis Conductance Transmembrane Regulator). This protein regulates the propelment of sodium and chloride in the association’s cells. Disruption of this protein leads to duller secretions due to the imbalance of salt and breathe-into in the cells. Too dwarf salt and breathe-into are beyond the cells, making totalthing duller
  10. D. “My slip hates to admit pills, so I’ll mix the capsule into a cup of hot chocolate. The pancreatic capsules hold pancreatic enzyme that should be carryed in a self-possessed, not a hot, balance (example: chilled applesauce versus hot chocolate) to deeptain the medication’s uprightness.
  11. A. Early years [0-5] 
  12. C. Pungent throat. Side possessions of Pulmozyme emmass pungent throat, hoarseness, and laryngitis. Answers A, B, and C are not associated succeeding a era Pulmozyme; accordingly, they are insufficient.
  13. B. Succeeding a era macerations and snacks. Pancreatic enzyme re-establishment is fond succeeding a era each maceration and each snack.
  14. B. The resigned has a slip succeeding a era cystic fibrosis . Cystic fibrosis is a recessive stroke; each manifestation has a one in foul-mouthed casualty of having the stroke or the empiricism. Maternal age is not a betray content until age 35, when the stroke of chromosomal defects increases. Maternal inhospitableness to rubella during the primeval trimester may account probable defects. Although a fact or preterm work may settle the resigned at betray for preterm work, it does not correlate succeeding a era genetic defects.
  15. C. Moist, efficient cough . Moist, efficient cough Discretion c is a behind prefiguration. Noisy respirations and a dry non-efficient cough are regularly the primeval of the respiratory prefigurations to reply in a newly diagnosed client succeeding a era cystic fibrosis (CF). The other discretions are the leading judgments. CF is an ancestral (genetic) mood tokenificant the cells that property mucus, perspirationinging, saliva and directive juices. Normally, these secretions are flimsy and unsafe, but in CF, a insufficient gene accounts the secretions to beseem dull and viscous. Instead of acting as a lubricant, the secretions arrest up tubes, ducts and passageways, chiefly in the pancreas and lungs. Respiratory deficiency is the most hazardous outcome of CF.
  16. B. Three spans per day succeeding a era macerations. Pancreatic enzymes should be fond succeeding a era macerations for optimal possessions. These enzymes succor the association in directing scarcityed nutrients. Answers A, C, and D are inemend methods of carrying pancreatic enzymes.
  17. B. False 
  18. B. discursive fat . The client succeeding a era cystic fibrosis assumes fat out-of-sorts consequently of the reflect secretions blocking the pancreatic duct. The closing of eventual pancreatic enzyme leads to weak parching of predominantly fats in the duodenum. Foul-smelling, frothy stool is termed steatorrhea.
  19. B. At moderationest 2 hours behind a maceration. The promote should enact chest physiotherapy at moderationest 2 hours behind a maceration to attenuate the betray of vomiting and appetition. Performing it straightway anteriorly a maceration may dispirit the client and diminish the power to eat. Collision and oscillation, ingredients of chest physiotherapy, may worsen bronchospasms; accordingly, the mode is contraindicated in clients succeeding a era bronchospasms. Secretions that enjoy mobilized (chiefly when suction equipment isn’t serviceable) are a contraindication for postural drainage, another ingredient of chest physiotherapy.
  20. D. Pounding consultation and hold . The autonomous toddler would be frustrated by entity intolerant to be. The pounding consultation and hold is fruitally expend and an laudable way for the toddler to free inhospitableness.
  21. D. Sweating proof . A inferior electrode is settled on the husk, which stimulates the perspirationinginging glands. Sweating is serene for a determination of span and then analyzed. Mob succeeding a era cystic fibrosis enjoy an increased total of chloride in their perspirationinging, due to the irregular protein false by the cystic fibrosis gene.
  22. C. Mucovicidosis . Cystic fibrosis is repeatedly determined mucovicidosis, chiefly in other regions of the globe. This refers to the dull, viscous mucus conceald by the exocrine glands of someone fictitious by cystic fibrosis. Cystic fibrosis was determined that in 1938 by Dr. Dorothy Anderson, a pathologist in New York City. She picturesquely changes in the pancreas as cystic, or fluid-filled, and fibrotic, or scarred.
  23. C. chest physiotherapy. The deep pulmonary total succeeding a era CF is dull pertinacious secretions. CPT propels the secretions from the inferior airways to the big wless they can be coughed out. Options a and b are used but are undignified to discretion c; the vocal enzymes that CF resigneds admit are for direction, not pulmonary reasons.
  24. A. Succeeding a era macerations and snacks. Pancreases capsules must be captured succeeding a era fixation and snacks consequently it acts on the nutrients and readies them for parching.
  25. D. Use cupped operatives during collision . The promote or constructor should use a cupped operative when enacting chest collision. Retort A is inemend consequently the operative should be cupped. Retort B is inemend consequently the slip’s lie should be radical total 5–10 minutes and the total assemblage should be boundaryed to 20 minutes. Retort D is inemend consequently chest collision should be resulted anteriorly macerations.


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