Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Residência Pediatria > Pneumo > Flashcards

Pneumo Flashcards

Cystic fibrosis

You may prefer our related Brainscape-certified flashcards:
Medcards Residência Médica flashcards
1
Q

CF is a (recessive/dominant) genetic trait

A

Recessive; most common among whites

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Responsible for most cases of exocrine pancreatic insufficiency in early life

A

Cystic fibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

The CF gene codes for the:

A

CFTR protein; Cystic Fybrosis Transmembrane conductance Regulator

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

CF mutation classes I-II-III are generally considered to be:

A

Severe mutations; complete or near complete absence of function

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

CF mutation classes IV-V-VI are generally considered to be:

A

Milder forms of CF; retain some function

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

CF intestinal epithelial cells with the F508del mutation are:

A

Unresponsive to the secretory effects of cholera toxin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Cite 4 pathologic characteristics of CF:

A

Failure to clear mucous sec; paucity of water in mucous sec; eleveted salt content of sweat and other secretions; chronig respiratoy tract inf

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

CF epithelial cells are unable to secrete ____ or ____ in response to cyclic adenosine.

A

Chloride; Bicarbonate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Earliest observable abnormality of the resp sys. in CF

A

Aiflow obstuction at the level of small airways

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Sweat in CF is saltier because:

A

Inability of glands to retrieve chloride from “isotonic” sweat

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Initial lung manifestations in CF: (2)

A

Chronic bronchiolitis and bronchitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Main patogens in airways infections of CF patients: (3)

A

S. aureus; P. aeruginosa; Burkholderia cepacia complex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Antimicrobial activity is _____ in CF secretions

A

Diminished; may be due to hyperacidic surface

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Females have a ____ prognosis

A

poorer; estrogen may influence disease exacerbations

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Pahologic findings in response to chronic airway infection: (2)

A

Globet cell hyperplasia; Submucosal gland hypertrophy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Invasive bacterial infection is characteristic in CF (T/F)

A

False; organisms appear to be confined to the endobronchial space

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Which lung lobes tend to be most affected in CF:

A

Upper lobes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Pancreas in CF is usually ___

A

Small; hard to find in post-mortem

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

There is important hystologic changes in the GI of CF patients T/F

A

False; intestinal tract shows minimal changes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Blochage og intrahepatic bile ducts may cause…

A

Focal biliary cirrhosis; uncommon; may also cause prolonged neonatal jaundice

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

% of males with obliterated or atresic vas deferens; epididymis and seminal vesicles

A

95%; most are infertile

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Infection with S. aureus; P. aeruginosa or H influenza occurs within ____

A

the first year of life; CT scan may show air trapping by the first year too

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Earliest symptom ___; may begin with viral infection; persists unless____

A

cough; treated with ATB

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Pulmonary exacerbation is defined as:

A

Cough; chest congestion; sputum production; wheezing that improves with ATBs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Rapid pulmonary deterioration is associated with which patogen?

A

B. cepacia; also other multidrug-resistant organisms

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Common pulmonary complications in CF (4)

A

Pneumothorax; atelectasias; cor pulmonale; hemoptysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

In CF; paranasal sinuses are always ____ radiographically

A

Opacified; acute sinusitis is “infrequent”

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

In CF; nasal polyps are:

A

Frequent; most troublesome between 5-20 yr

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

In CF; meconium ileus appears in ____ of NBs

A

15-20%; abdominal distention; emesis; failure to pass meconium in the first 24-48h

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

In CF; meconium ileus often requires ___

A

Surgical intervention

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Distal intestinal obstruction syndrom (DIOS) is:

A

Ileal obstruction with fecal material; older children; initial tx is clinical

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Exocrine pancreatic insuficiency is pressent in ____ of CF patients

A

85%; protein and fat malabsorption

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

In CF; Class IV-V-VI are associated with pancreatic sufficiency; but…

A

they are prone to pancreatitis as adolescents

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Liver disfunction is pressent during early infancy T/F

A

False; usually detected in the first 15 yrs of life; up to 30%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

In CF; Endocrine pancreatic insufficiency develops _____

A

in the 2ndd decade; more common with family history of DM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Ketoacidosis is common in CF related diabetes T/F

A

False; more likely to develop cronic complications after 10yrs of dissease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

All males are “azoospermic in CF

A

True; sexual function is generally unimpaired

38
Q

In CF; pregnancy is ____ tolerated but may ____

A

Well; lead to pulmonary deteriorment

39
Q

Excessive loss of salt in the sweat may cause…

A

salt depletion episodes; especially during gastroenteritis and warm weather

40
Q

In CF; most common type of alkalosis:

A

“Hypo”chloremic

41
Q

Diagnostic criteria for CF includes 1 clinical + 1 laboratory; cite clinical criteria (3)

A

Presence of resp gasto or genitourinary features; CF in a sibling; Positive NB screening

42
Q

Diagnostic criteria for CF includes 1 clnical + 1 laboratory; cite lab criteria (3)

A

2 elevated sweat tests; CF mutation identified; abnormal nasal potential

43
Q

In CF; Sweat test uses ____ to colect sweat and analizes ___ content

A

pilocarpine iontophoresis; chloride; standard approach to CF

44
Q

In CF; possitive screening should have sweat tested after ___; weight of ___; age > ____

A

36wk corrected GA; 2kg; 10 days; to increase the likelihood of sufficient sweat

45
Q

In CF; the first possitive sweat test should

A

be confirmed by a second sweat test; a negative can be repeated if high suspicion

46
Q

In CF; ___ mmol/L of chloride in sweat is Dx if __

A

> 60; another criteria is present; +Screening and <30 makes CF unlikely; borderline (30-59) requires further testing

47
Q

In CF; pancreatic malabsorption Dx is done by…

A

Quantification of “elastase-1 activity” in fresh stool

48
Q

In CF; Oral glucose tolerance testing should be perform at…

A

age 10; 20% of adolescents suffer from CF related diabetes

49
Q

In Cf; thorax Rx is indicated with what frequency?

A

Annually

50
Q

In CF; FEV1 correlates most closely with ___ and shoes a gradual decline of ____ per year

A

mortality; 2-3%; routinely done by age 6

51
Q

In CF; clinical significant decrements in the espirometry are the most sensitive indicator of…

A

pulmonary exacerbation that should be treated with ATBs

52
Q

In CF; Which microbe develops a biofilm associated with a mucoid appearance in the microscope?

A

P. aeruginosa; mucoid photype makes it extremely difficult to eradicate

53
Q

In CF; The cepacia syndrome refers to:

A

Fulminant downhill course of lung disease associated with Burkholderia cepacia complex (G- rod)

54
Q

In CF; screening is done with:

A

Immunoreactive trypsinogen; 95% sensitiviy

55
Q

In CF; most common nonrespiratory manifestation in males:

A

Absence of the vas deferens

56
Q

In CF; importance of thorough pulmonary history and evaluation:

A

Irreversible loss of p. function from low-grade inf can occur gradually without acute symptoms

57
Q

If CF; the recommended multidis team is composed of: (6)

A

MD; nurse; physical therapist; respiratory therapist; social worker and dietician

58
Q

In CF; what is the object of pulmonary therapy?

A

Clear secretions from airways and to control infection

59
Q

In CF; _____ enzymatically dissolves extracellular DNA

A

Human recombinant Dnase (2.5mg)

60
Q

Human recombinant Dnase (2.5mg) improves pulmonary function T/F

A

True; decreases pulm exacerbations and promotes sense of well-beign

61
Q

In CF; hypertonic saline __%; ___ times daily; increases mucousclearence and ____ pulm exacerbation

A

7; 2-4; reduces; only a slight short-term improv in pulm function

62
Q

In CF: Airway clearance therapy is always recommended T/F

A

True; no one technique can be shown to be superior

63
Q

in CF; ATB for pulm exacerbations has the goal of…

A

reduce the intensity of endobronchial infection and to delay progressive lung damage

64
Q

in CF; the usual course of oral ATB lasts for…

A

2 wks at max dosage

65
Q

In CF; Chronic P. aeruginosa infection may benefit from…

A

Azithromycin 3x wk; improves lung function

66
Q

In CF; cite 2 aerosolized atbs for P.aeruginosa tx

A

Tobramycin and aztreonam; on 1mo-off 1mo; reduces symptoms and exacerbations; improves pulm function

67
Q

In CF; failure to improve after 7-14days of IV ATB…

A

Consider complications; viruses; fungus ; micobacterias or unusual orgs

68
Q

In CF; ___ is a potentiator of the CFTR mutation

A

Ivacaftor; improves FEV1; decreases exacerbations and sweat chloride concentration; increases weight gain

69
Q

In CF; Ivacaftor is aproved for patients….

A

older than 2yrs; with class III and IV mutations

70
Q

In CF; Atelectasis can be treated with ATBs and PT T/F

A

True; if no improvement after 5-7 days consider bronchoscopic examination

71
Q

In CF; Hemoptysis (<20ml) should prompt…

A

intensification of ATB therapy and Chest PT

72
Q

Define massive hemoptysis

A

Total blood loss of >/= 250ml in 24h

73
Q

CF patient with wheezing; increased cough; shortness of breath; hyperinflated lungs; reduced FEV1 unresponsive to ATBS; HDx:

A

Allergic Bronchopulmonaty Aspergillosis

74
Q

Allergic Bronchopulm Aspergillosis Dx: (6)

A

Elevated serum IgE>1000; rust-colored sputum; Aspergillus in sputum; + skin test for Asp; Asp specific IgE/G; eosinophils in sputum

75
Q

Tx of allergic bronchopulm asp includes antifungals as first line T/F

A

False; initial tx consists of CTC for inflammation control; antifungals if former fails; omalizumab for refractory cases may help

76
Q

In CF; infection with “nontubercolous” mycobacteria should be always treated. T/F

A

False; it is important to differenciate colonization from invasive infection

77
Q

Hypertropic osteoarthropathy is closely related to lung infection in CF

A

True; Control of infection usually reduces symptoms; acetaminophen or ibuprofen may provide relief

78
Q

In CF; Chronic respiratory failure is most frequent in…

A

adult patients

79
Q

In CF; Chronic dyspneia may be ameliorated with nebulized…

A

Fentanyl

80
Q

In CF; Severe ___ (PaO2<___) may leat to pulm hypertension and ___-side heart failure.

A

hypoxemia; 50; Right

81
Q

In CF; Poorly controlled lung disease increases metabolism but decreases…

A

apetite

82
Q

In CF; Appetite stimulants are not recommended T/F

A

False; liberal use of “cyproheptadine” is encouraged; nocturnal feeding via NG tube may be an option

83
Q

In CF; Pancreatic enzimes are used in doses up to ____

A

2500 lipase units/kg/meal; higher doses are linked to fibrosing colonopathy and colonic strictures

84
Q

In CF; Wich vitamins should be suplemented?

A

DEKA; fat-soluble vits;

85
Q

in CF; Vit D should be suplemented as…

A

Cholecalciferol (D3) instead of ergocalcifero (D2); 1000 UI/kg/wk

86
Q

Infants with meconuim ileus should be assumed to have ___ until proven otherwise

A

Cystic fibrosis

87
Q

In CF; Rectal prolapse can be manually reduced T/F

A

True; gentle pressure in knee-chest position; Sedation may help; Buttocks can be taped together

88
Q

Name 4 hepatic complications in CF

A

Portal HT; Esophageal varices; hypersplenism; ascites; up to 8% of CF children

89
Q

In CF; Diabetes occurs after ___

A

First decade; hyperglicemia favors acquisition of P. aeruginosa and B cepacia

90
Q

In CF; Nasal polyps occur in ___ of patients and can be treated with ___ and ___

A

15-20%; local CTC; nasal decongestants

91
Q

In CF; Surgical indication for nasal polyps (2)

A

complete obstruction of nasal airway; widening of the nasal bridge

92
Q

In CF; Salt depletion can be managed by….

A

Free access to salt; salt supplements

Residência Pediatria (12 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions