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Pharmo Drugs Kim > Chapter 15 Patho > Flashcards

Chapter 15 Patho Flashcards

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
Q
  1. Vital signs for CF that show increased
A

respiratory rate tachypnea
heart rate pulse
blood pressure elevated

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26
Q
  1. Physical observations
A 
use of accessory muscles of inspiration
use of accessory muscles of expiration
pursed-lip breathing
barrel chested
cyanosis
digital clubbing
cough, sputum production & hemoptysis
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27
Q
  1. chest assessment findings
A 
decreased tactile & vocal fremitus
hyperresonant percussion note
diminished breath sounds
diminished heart sounds
bronchial breath sounds (over atelectasis)
crackles
wheezing
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28
Q
  1. When CF pt has spontaneous pnuemothorax pt has ?% of reoccurence
A

50% chance

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29
Q
  1. FEV1 (decreased or increased)
A

decreased, forced expiratory volume in 1 second

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30
Q
  1. PEFR (decreased or increased)
A

decreased, peak expiratory

31
Q

FRC & RV (decreased or increased)

A

increased, air trapping

32
Q
  1. ABG results typical when (mild to moderate)
A 
pH increased
PaCO2 decreased
HCO-3 decreased
PaO2 decreased
SaO2/SpO2 decreased
33
Q
  1. ABG results when (severe)
A 
pH Normal
PaCO2 increased
HCO-3 increased
PaO2 decreased
SaO2/SpO2 decreased
34
Q
  1. Oxygenation indices
A

Shunting(Qs/Qt) : increased

35
Q
  1. abnormal lab results: hematology
A

increased hematocrit & hemoglobin

increased white blood count (infections)

36
Q
  1. abnormal lab results: electrolytes
A

hypocholermia (chronic ventilatory failure)

37
Q
  1. abnormal lab results: sputum
A

gram positive bacteria

gram negative bacteria

38
Q
  1. radiologic findings
A 
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
Q
  1. common non-respiratory clinical manifestations
    intestinal blockage
    malnutrition & poor body development
A

intestinal blockage malnutrition & poor body development

40
Q
  1. primary goals of management of CF
A

prevent pulmonary infections
reduce thick bronchial secretions
improve air flow
provide adequate nutrition

41
Q
  1. Resp care protocols
A

O2 therapy
Bronchopulmonary hygiene therapy (CPT & VEST)
Lung expansion therapy ( IPPB, IS, PEP therapy)
aerosolized meds

42
Q
  1. general therapies beyond resp care
A 
antibiotics
nutritional & vitamin supplements
ibuprofen
inhaled corticosteroids
pancreatic enzymes
psychosocial issues (quality of life, adherence to therapies, depression, end of life)
43
Q
  1. lung/heart transplant numbers
A

1/1700 year total transplants
around 200 transplants for CF pt annually
1 year survival 90%
5 yr survival 80%

44
Q
  1. What organisms are commonly found in the tracheobronchial tree secretions of patients with CF?
  2. Staphylococcus, Haemophilus influenza, Pseudomonas aeruginosa
  3. Streptococcus, COPD
A
  1. Staphylococcus, Haemophilus influenza, Pseudomonas aeruginosa
45
Q
  1. When 2 carriers of CF produce children, there is a…
A

25% chance that the baby will be completely normal

25% chance the baby will have CF

46
Q
  1. The cystic fibrosis gene is located on which chromosome?
A

7

47
Q
  1. In CF the patient commonly demonstrates….
A

↓ MVV
↑ RV
↓ FEV₁/FVC ratio

48
Q
  1. During advance stages of CF, the patient generally demonstrates….
A

bronchial breath sounds
diminished breath sounds
hyperresonant percussion notes

49
Q
  1. About 80% of all CF patients demonstrate a deficiency in which vitamins?
A

A, D, E, K

50
Q
  1. What agent targets the underlying cause of CF, the faulty gene G551D, and its defective CFTR protein?
A

Ivacafor

51
Q
  1. Name possible mucolytic agents
A 
~ Dornase Alpha
Pulmozyme
~ N-acetylcysteine
Mucomyst
~ DNase
52
Q
  1. With regard to the secretion of sodium and chloride, the sweat glands of patients with CF secrete up to
A

4 times the normal amount

53
Q
  1. What clinical manifestations are associated with severe CF?
A

~ ↑ CVP
~ ↓ breath sounds
~ ↑ pulmonary vascular resistance

54
Q
  1. anatomic alterations
A 
~ ↑ production/ accumulation of thick, tenacious
mucus in the tracheobronchial tree
~ partial/ total bronchial obstruction
mucus plugging
~ hyperinflation of alveoli
~ atelectasis
55
Q
  1. etiology/ epidemiology
A

~ autosomal recessive gene disorder
mutations of chromosome 7
∆f508 defect
~most common fatal inherited disorder in childhood

56
Q
  1. 2 carrier parents
A

25% unaffected
50% unaffected (asymptomatic) carrier
25% affected

57
Q
  1. 1 carrier + 1 CF parent
A

50% affected

50% unaffected (asymptomatic) carrier

58
Q
  1. 1 noncarrier + 1 CF parent
A

100% unaffected (asymptomatic) carrier

59
Q
  1. 1 noncarrier + 1 carrier parent
A

50% unaffected

50% unaffected (asymptomatic) carrier

60
Q
  1. criteria for diagnosis
A 
~ ↑ sweat chloride
>60 meq/L
on 2 occasions
~ abnormal nasal potential difference
~ genetic testing
61
Q
  1. Newborn screening
A

~ trypsin-like

~ serum trypsin

62
Q
  1. stool fecal fat test
A

measures amount of fat in infants stool
percentage of dietary fat not absorbed by the
body

63
Q
  1. organs affected by CF
A

~ lungs

~ pancreas

64
Q
  1. meconium ileus
A

obstruction of small intestine of newborn (by meconium)

65
Q
  1. vital signs
A

↑ respiratory rate
↑ heart rate
↑ blood pressure

66
Q
  1. physical examination
A 
~ use of accessory muscles
inspiration/ exporation
~ pursed lip breathing
~ barrel chest
~ cyanosis
~ digital clubbing
~ cough, sputum, hemoptysis
67
Q
  1. chest assessment
A 
~ ↓ tactile/ vocal fremitus
~ hyperresonance
~ diminished breath sounds
~ diminished heart sounds
~bronchial breath sounds
over atelectasis
~ crackles/ wheezing
~ spontaneous pneumothorax
68
Q
  1. hematology
A

↑ HCT

↑ Hb

69
Q
  1. electrolytes
A

hypochloremia

↑ serum bicarbonate

70
Q
  1. CXR
A 
~ translucent lung fields
~ depressed/ flattened diaphragm
~ right ventricular enlargement
~ areas of atelectasis/ fibrosis
~ tram-tracks
~ bronchiectasis
~ spontaneous pneumonthorax
~ abscess formation
71
Q
  1. nonrespiratory complications
A 
~ meconium ileus
~ meconium ileus equivalent
~ malnutrition/ poor body development
↓ vitamins A, D, E, K
infertility (males)
nasal polyps/ sinusitis
72
Q
  1. general management
A 
Primary Goals
~ prevent pulmonary infections
~ ↓ thick bronchial secretions
~ improve airflow
~ provide adequate nutrition
Instruct patient and family about disease and its effects
73
Q
  1. respiratory treatment
A 
~ O₂ therapy
~ bronchopulmonary hygiene
~ lung expansion therapy
~ aerosolized medications
bronchodilators
mucolytics
antibiotics
~ mechanical ventilation
74
Q
  1. other medication/ procedures
A 
~ antibiotics
~ Ibuprofen
~ ICS
~ systemic glucocorticoids
~ lung or heart-lung transplant
~ vest
~ pancreatic enzymes

Pharmo Drugs Kim (23 decks)

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