Pneumo Flashcards by Sergio Perez

CF is a (recessive/dominant) genetic trait

Recessive; most common among whites

How well did you know this?

Not at all

Perfectly

Responsible for most cases of exocrine pancreatic insufficiency in early life

Cystic fibrosis

How well did you know this?

Not at all

Perfectly

The CF gene codes for the:

CFTR protein; Cystic Fybrosis Transmembrane conductance Regulator

How well did you know this?

Not at all

Perfectly

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

Severe mutations; complete or near complete absence of function

How well did you know this?

Not at all

Perfectly

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

Milder forms of CF; retain some function

How well did you know this?

Not at all

Perfectly

CF intestinal epithelial cells with the F508del mutation are:

Unresponsive to the secretory effects of cholera toxin

How well did you know this?

Not at all

Perfectly

Cite 4 pathologic characteristics of CF:

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?

Not at all

Perfectly

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

Chloride; Bicarbonate

How well did you know this?

Not at all

Perfectly

Earliest observable abnormality of the resp sys. in CF

Aiflow obstuction at the level of small airways

How well did you know this?

Not at all

Perfectly

Sweat in CF is saltier because:

Inability of glands to retrieve chloride from “isotonic” sweat

How well did you know this?

Not at all

Perfectly

Initial lung manifestations in CF: (2)

Chronic bronchiolitis and bronchitis

How well did you know this?

Not at all

Perfectly

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

S. aureus; P. aeruginosa; Burkholderia cepacia complex

How well did you know this?

Not at all

Perfectly

Antimicrobial activity is _____ in CF secretions

Diminished; may be due to hyperacidic surface

How well did you know this?

Not at all

Perfectly

Females have a ____ prognosis

poorer; estrogen may influence disease exacerbations

How well did you know this?

Not at all

Perfectly

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

Globet cell hyperplasia; Submucosal gland hypertrophy

How well did you know this?

Not at all

Perfectly

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

False; organisms appear to be confined to the endobronchial space

How well did you know this?

Not at all

Perfectly

Which lung lobes tend to be most affected in CF:

Upper lobes

How well did you know this?

Not at all

Perfectly

Pancreas in CF is usually ___

Small; hard to find in post-mortem

How well did you know this?

Not at all

Perfectly

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

False; intestinal tract shows minimal changes

How well did you know this?

Not at all

Perfectly

Blochage og intrahepatic bile ducts may cause…

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

How well did you know this?

Not at all

Perfectly

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

95%; most are infertile

How well did you know this?

Not at all

Perfectly

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

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

How well did you know this?

Not at all

Perfectly

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

cough; treated with ATB

How well did you know this?

Not at all

Perfectly

Pulmonary exacerbation is defined as:

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

How well did you know this?

Not at all

Perfectly

Rapid pulmonary deterioration is associated with which patogen?

B. cepacia; also other multidrug-resistant organisms

Common pulmonary complications in CF (4)

Pneumothorax; atelectasias; cor pulmonale; hemoptysis

In CF; paranasal sinuses are always ____ radiographically

Opacified; acute sinusitis is "infrequent"

In CF; nasal polyps are:

Frequent; most troublesome between 5-20 yr

In CF; meconium ileus appears in ____ of NBs

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

In CF; meconium ileus often requires ___

Surgical intervention

Distal intestinal obstruction syndrom (DIOS) is:

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

Exocrine pancreatic insuficiency is pressent in ____ of CF patients

85%; protein and fat malabsorption

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

they are prone to pancreatitis as adolescents

Liver disfunction is pressent during early infancy T/F

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

In CF; Endocrine pancreatic insufficiency develops _____

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

Ketoacidosis is common in CF related diabetes T/F

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

All males are "azoospermic in CF

True; sexual function is generally unimpaired

In CF; pregnancy is ____ tolerated but may ____

Well; lead to pulmonary deteriorment

Excessive loss of salt in the sweat may cause…

salt depletion episodes; especially during gastroenteritis and warm weather

In CF; most common type of alkalosis:

"Hypo"chloremic

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

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

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

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

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

pilocarpine iontophoresis; chloride; standard approach to CF

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

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

In CF; the first possitive sweat test should

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

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

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

In CF; pancreatic malabsorption Dx is done by…

Quantification of "elastase-1 activity" in fresh stool

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

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

In Cf; thorax Rx is indicated with what frequency?

Annually

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

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

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

pulmonary exacerbation that should be treated with ATBs

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

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

In CF; The cepacia syndrome refers to:

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

In CF; screening is done with:

Immunoreactive trypsinogen; 95% sensitiviy

In CF; most common nonrespiratory manifestation in males:

Absence of the vas deferens

In CF; importance of thorough pulmonary history and evaluation:

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

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

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

In CF; what is the object of pulmonary therapy?

Clear secretions from airways and to control infection

In CF; _____ enzymatically dissolves extracellular DNA

Human recombinant Dnase (2.5mg)

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

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

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

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

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

True; no one technique can be shown to be superior

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

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

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

2 wks at max dosage

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

Azithromycin 3x wk; improves lung function

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

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

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

Consider complications; viruses; fungus ; micobacterias or unusual orgs

In CF; ___ is a potentiator of the CFTR mutation

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

In CF; Ivacaftor is aproved for patients….

older than 2yrs; with class III and IV mutations

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

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

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

intensification of ATB therapy and Chest PT

Define massive hemoptysis

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

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

Allergic Bronchopulmonaty Aspergillosis

Allergic Bronchopulm Aspergillosis Dx: (6)

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

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

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

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

False; it is important to differenciate colonization from invasive infection

Hypertropic osteoarthropathy is closely related to lung infection in CF

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

In CF; Chronic respiratory failure is most frequent in…

adult patients

In CF; Chronic dyspneia may be ameliorated with nebulized…

Fentanyl

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

hypoxemia; 50; Right

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

apetite

In CF; Appetite stimulants are not recommended T/F

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

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

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

In CF; Wich vitamins should be suplemented?

DEKA; fat-soluble vits;

in CF; Vit D should be suplemented as…

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

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

Cystic fibrosis

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

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

Name 4 hepatic complications in CF

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

In CF; Diabetes occurs after ___

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

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

15-20%; local CTC; nasal decongestants

In CF; Surgical indication for nasal polyps (2)

complete obstruction of nasal airway; widening of the nasal bridge

In CF; Salt depletion can be managed by….

Free access to salt; salt supplements