Pharmo Drugs Kim > Chapter 15 Patho > Flashcards

Chapter 15 Patho Flashcards

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1
Q
  1. Define genetic disorder
  2. Most common fatal inherited disorder in childhood
  3. Adult disease
A
  1. Most common fatal inherited disorder in childhood
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2
Q
  1. CF is autosomal recessive gene disorder of
A

mutation in a pair of genes located on Chromosome 7

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3
Q
  1. There are ? different mutations of CF in the gene that encodes CF
  2. 2400
  3. 2500
  4. 3400
A
  1. 2400
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4
Q
  1. CFTR define
A

cystic fibrosis transmembrane conductance regulator

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5
Q
  1. Genetic defect for CF involves absence of 3 pairs of codon 508, what percent of cases
  2. 70-75% of all CF pt tested
  3. 50-65% of all CF pt tested
A
  1. 70-75% of all CF pt tested
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6
Q
  1. Basis of what causes CF
  2. chlorine is not transported into cells, causes water to leave mucous (leaving behind thick mucous).
  3. cystic fibrosis transmembrane conductance regulator
A
  1. chlorine is not transported into cells, causes water to leave mucous (leaving behind thick mucous).
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7
Q
  1. anatomic alterations of the lung
A
  1. excessive production & accumulation of thick, tenacious mucus in the tracheobronchial tree
  2. mucous plugging, partial bronchial obstruction
  3. hyperinflation of alveoli
  4. total bronchial destruction (mucous plugging)
  5. altelectasis
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8
Q
  1. mucous blocks which organ causing the body to not be able to digest fats & proteins
  2. Pancreas
  3. Liver
  4. Heart
A
  1. Pancreas
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9
Q
  1. Blockage in the intestines (infants after birth) called?
  2. Meconium ileus.
  3. COPD.
  4. Empysema
A
  1. Meconium ileus.
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10
Q
  1. How CF affects fertility.
  2. Infertility in men, decreased fertility with women
  3. Infertility in women, decreased fertility with men
A
  1. Infertility in men, decreased fertility with women
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11
Q
  1. Clinical symptoms consistent with CF in at least one organ system?.

Has at least many diseases what is it?

A
  1. pulmonary system
  2. sinus disease
  3. pancreatic disease
  4. meconium ileus
  5. bilary disease
  6. male infertility
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12
Q
  1. Clinical evidence of CF, through test
A
  1. elevated sweat chlorine >60 mEq/L (on 2 occasions)
  2. Genetic testing presence of 2 disease causing mutation
  3. abnormal nasal potential difference
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13
Q
  1. Screening and dx
A
  1. newborn screening
  2. sweat test
  3. genetic testing
  4. nasal potential difference
  5. prenatal testing
  6. stool fecal fat test
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14
Q
  1. CF screening protocols will ID ?% of infants
A

90% at newborn

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15
Q
  1. Most common DNA protocol for screening newborn
A

for 32-85 of the most common CF mutations on the 2-5% of the samples with highest IRT

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16
Q
  1. A negative or normal sweat test ID
A

as a carrier of CF

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17
Q
  1. If both parents test positive for the CF mutation, the chances of a positive CF child is what %
A

25%, 50% unaffected, 25% unaffected

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18
Q
  1. When one parent is carrier and the other parent is CF parent, what are the chances of Child.
A

50% chance affected and 50% chance unaffected carrier

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19
Q
  1. Noncarrier parent and CF parent what is the chances of the child?
A

100% chance unaffected carrier

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20
Q
  1. Noncarrier parent and carrier parent what are the chances of the child?
A

50% chance unaffected and 50% chance unaffected

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21
Q
  1. define fecal fat test

test measures the amount of fat in the infants stool and the % of dietary fat not absorbed by the body

A

test measures the amount of fat in the infants stool and the % of dietary fat not absorbed by the body

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22
Q
  1. fecal fat test determines how which organs are functioning
A

liver, gallbladder, pancreas, intestines are functioning

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23
Q

normal results for fecal fat test

A

7 g of fat per 24 hours is normal for malabsorption, any number above 7 is associated with several disorders including CF

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24
Q

what are 3 clinical manifestation of CF

A
  1. atelectasis
  2. bronchospasm
  3. excessive bronchial secretions
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25
25. Vital signs for CF that show increased
respiratory rate tachypnea heart rate pulse blood pressure elevated
26
26. Physical observations
``` use of accessory muscles of inspiration use of accessory muscles of expiration pursed-lip breathing barrel chested cyanosis digital clubbing cough, sputum production & hemoptysis ```
27
27. chest assessment findings
``` decreased tactile & vocal fremitus hyperresonant percussion note diminished breath sounds diminished heart sounds bronchial breath sounds (over atelectasis) crackles wheezing ```
28
28. When CF pt has spontaneous pnuemothorax pt has ?% of reoccurence
50% chance
29
29. FEV1 (decreased or increased)
decreased, forced expiratory volume in 1 second
30
30. PEFR (decreased or increased)
decreased, peak expiratory
31
FRC & RV (decreased or increased)
increased, air trapping
32
32. ABG results typical when (mild to moderate)
``` pH increased PaCO2 decreased HCO-3 decreased PaO2 decreased SaO2/SpO2 decreased ```
33
33. ABG results when (severe)
``` pH Normal PaCO2 increased HCO-3 increased PaO2 decreased SaO2/SpO2 decreased ```
34
34. Oxygenation indices
Shunting(Qs/Qt) : increased
35
35. abnormal lab results: hematology
increased hematocrit & hemoglobin | increased white blood count (infections)
36
36. abnormal lab results: electrolytes
hypocholermia (chronic ventilatory failure)
37
37. abnormal lab results: sputum
gram positive bacteria | gram negative bacteria
38
38. radiologic findings
``` translucent (dark) lung fields depressed & flattened diaphragms right ventricular enlargement areas of fibrosis & atelectasis Tram - tracks (bronchiectasis) Bronchiectasis (2ndary complication) pnuemothorax (spontaneous) abscess formation (occasionally) ```
39
39. common non-respiratory clinical manifestations intestinal blockage malnutrition & poor body development
intestinal blockage malnutrition & poor body development
40
40. primary goals of management of CF
prevent pulmonary infections reduce thick bronchial secretions improve air flow provide adequate nutrition
41
41. Resp care protocols
O2 therapy Bronchopulmonary hygiene therapy (CPT & VEST) Lung expansion therapy ( IPPB, IS, PEP therapy) aerosolized meds
42
42. general therapies beyond resp care
``` antibiotics nutritional & vitamin supplements ibuprofen inhaled corticosteroids pancreatic enzymes psychosocial issues (quality of life, adherence to therapies, depression, end of life) ```
43
43. lung/heart transplant numbers
1/1700 year total transplants around 200 transplants for CF pt annually 1 year survival 90% 5 yr survival 80%
44
44. What organisms are commonly found in the tracheobronchial tree secretions of patients with CF? 1. Staphylococcus, Haemophilus influenza, Pseudomonas aeruginosa 2. Streptococcus, COPD
1. Staphylococcus, Haemophilus influenza, Pseudomonas aeruginosa
45
45. When 2 carriers of CF produce children, there is a...
25% chance that the baby will be completely normal | 25% chance the baby will have CF
46
46. The cystic fibrosis gene is located on which chromosome?
7
47
47. In CF the patient commonly demonstrates....
↓ MVV ↑ RV ↓ FEV₁/FVC ratio
48
48. During advance stages of CF, the patient generally demonstrates....
bronchial breath sounds diminished breath sounds hyperresonant percussion notes
49
49. About 80% of all CF patients demonstrate a deficiency in which vitamins?
A, D, E, K
50
50. What agent targets the underlying cause of CF, the faulty gene G551D, and its defective CFTR protein?
Ivacafor
51
51. Name possible mucolytic agents
``` ~ Dornase Alpha Pulmozyme ~ N-acetylcysteine Mucomyst ~ DNase ```
52
52. With regard to the secretion of sodium and chloride, the sweat glands of patients with CF secrete up to
4 times the normal amount
53
53. What clinical manifestations are associated with severe CF?
~ ↑ CVP ~ ↓ breath sounds ~ ↑ pulmonary vascular resistance
54
54. anatomic alterations
``` ~ ↑ production/ accumulation of thick, tenacious mucus in the tracheobronchial tree ~ partial/ total bronchial obstruction mucus plugging ~ hyperinflation of alveoli ~ atelectasis ```
55
55. etiology/ epidemiology
~ autosomal recessive gene disorder mutations of chromosome 7 ∆f508 defect ~most common fatal inherited disorder in childhood
56
56. 2 carrier parents
25% unaffected 50% unaffected (asymptomatic) carrier 25% affected
57
57. 1 carrier + 1 CF parent
50% affected | 50% unaffected (asymptomatic) carrier
58
58. 1 noncarrier + 1 CF parent
100% unaffected (asymptomatic) carrier
59
59. 1 noncarrier + 1 carrier parent
50% unaffected | 50% unaffected (asymptomatic) carrier
60
60. criteria for diagnosis
``` ~ ↑ sweat chloride >60 meq/L on 2 occasions ~ abnormal nasal potential difference ~ genetic testing ```
61
61. Newborn screening
~ trypsin-like | ~ serum trypsin
62
62. stool fecal fat test
measures amount of fat in infants stool percentage of dietary fat not absorbed by the body
63
63. organs affected by CF
~ lungs | ~ pancreas
64
64. meconium ileus
obstruction of small intestine of newborn (by meconium)
65
65. vital signs
↑ respiratory rate ↑ heart rate ↑ blood pressure
66
66. physical examination
``` ~ use of accessory muscles inspiration/ exporation ~ pursed lip breathing ~ barrel chest ~ cyanosis ~ digital clubbing ~ cough, sputum, hemoptysis ```
67
67. chest assessment
``` ~ ↓ tactile/ vocal fremitus ~ hyperresonance ~ diminished breath sounds ~ diminished heart sounds ~bronchial breath sounds over atelectasis ~ crackles/ wheezing ~ spontaneous pneumothorax ```
68
68. hematology
↑ HCT | ↑ Hb
69
69. electrolytes
hypochloremia | ↑ serum bicarbonate
70
70. CXR
``` ~ translucent lung fields ~ depressed/ flattened diaphragm ~ right ventricular enlargement ~ areas of atelectasis/ fibrosis ~ tram-tracks ~ bronchiectasis ~ spontaneous pneumonthorax ~ abscess formation ```
71
71. nonrespiratory complications
``` ~ meconium ileus ~ meconium ileus equivalent ~ malnutrition/ poor body development ↓ vitamins A, D, E, K infertility (males) nasal polyps/ sinusitis ```
72
72. general management
``` Primary Goals ~ prevent pulmonary infections ~ ↓ thick bronchial secretions ~ improve airflow ~ provide adequate nutrition Instruct patient and family about disease and its effects ```
73
73. respiratory treatment
``` ~ O₂ therapy ~ bronchopulmonary hygiene ~ lung expansion therapy ~ aerosolized medications bronchodilators mucolytics antibiotics ~ mechanical ventilation ```
74
74. other medication/ procedures
``` ~ antibiotics ~ Ibuprofen ~ ICS ~ systemic glucocorticoids ~ lung or heart-lung transplant ~ vest ~ pancreatic enzymes ```
Pharmo Drugs Kim
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