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Pulmonology > Cystic Fibrosis + Bronchiectasis + Bronchiolitis > Flashcards

Cystic Fibrosis + Bronchiectasis + Bronchiolitis Flashcards

1
Q

Cystic fibrosis

Etiology

A

*CFTR gene (Cl- transport, in all epithelial)
Delta F508

*auto recessive (both parents must be carrier)

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2
Q

*white (most common auto-recessive disease among) (ireland/n. europe)
*Thick mucus in lungs, pancreas, liver, intestines
*failure to thrive
*Respiratory s/s OBSTRUCTIVE: bronchitis, bronchiolitis, Pneumonia
*Meconium ileus
*abnormal stools
*nfertility
*pancreatic insufficiency –>steatorrhea, DAEK def
OLDER: nasal polyps, rectal prolapse

A

Cystic Fibrosis

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3
Q

Cystic Fibrosis

Path

A

Low Airway Surface Liquid (ASL) hypothesis
*Mutated CFTR = Cl- can’t get into airway –> + net charge –> Na+ back into cell (ENaC) –> water follows Na into cell

  • HYPERVISCOUS MUCOUS
  • ALL EXOCRINE GLANDS
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4
Q

Cystic fibrosis

Pulm effects

A 
BRONCHIECTASIS
Pneumonia 
*Pseudomonas/Staph
Bronchitis
Bronchiolitis
Nasal polyps
Chronic hypoxia
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5
Q

Child w/ rectal prolapse?

A

Cystic fibrosis

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6
Q

Child w/ pancreatitis?

A

Cystic fibrosis

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7
Q

Cystic fibrosis

GI effects

A 
Meconium ileus
Meconium plug
Rectal prolapse
Pancreatitis
Steatorrhea
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8
Q

Cystic fibrosis

General Effects

A
  • Failure to thrive
  • Vitamin deficiency (lipase deficiency)
  • Infertility (CBAVD) - crhonic bilateral absence of vas deferens)
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9
Q

Cystic Fibrosis

Dx

A
    • newborn screening
  • Sweat Cl test
  • sibling dx w/ CF
  • suggestive Hx

*Mutation analysis
*Nasal test
*CXR = bronchiectasis
USUALLY DURING INPATIENT

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10
Q

Cystic Fibrosis

Referring

A

*CF specialist referral

Managing:

  • pulm dz
  • acute exacerbations
  • CF-related Diabetes (pancreatic destruction) = supplementation
  • Complications
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11
Q

Cystic Fibrosis

Pulm Dz tx

A
  • Chest physiotherapy (hitting at home by parents)
  • mucolytics = n-acetylcystein (mucomyst= dissolves mucus); Dornase alpha (Pulmozyme = DNAs)
  • bronchodilators
  • anti inflammatories (NO in kids 2-5)
  • antibios
  • CFTR Potentiator/Corrector = Ivacraftor (G551D, NO F508 = need Lumacaftor)
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12
Q

Cystic Fibroisis

Tx = Acute exacerbation MRSA

A

Vanc
OR
Linezolid

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13
Q

Cystic Fibroisis

Tx = Acute exacerbation
Pseudomonas

A

Severe = IV tobramycin + piperacillin

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14
Q

Cystic Fibroisis

Tx = Acute exacerbation
Pseudomonas

A

Tobramycin (inhaled) + Ceftazidime

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15
Q

Cystic Fibroisis

Tx = CF Diabetes

A
  • > 30 y/o
  • insulin
  • pancreatic enzyme replacement therapy
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16
Q

Cystic Fibroisis

MC cause of death

A

Respiratory failure

Pulm dz biggest prognosis

Usually mid-20s to mid 30s

17
Q

Cystic Fibroisis

Tx - no previous pseudomonas

A

Treat staph

Amoxi+clav

18
Q
  • chronic cough
  • productive = purulent, thick , sticky foul-smelling mucus (worse morning)
  • hemoptysis
  • Crackles at bases
  • looks like chronic bronchitis
  • cystic fibrosis MC cause in US
  • dilation + thick wall bronchi (permanent)
A

Bronchiectasis

19
Q

Bronchiectasis

Dx workup

A
  • **CT chest w/ contrast = big bronchus peripherally (tree in bud)
  • CXR= (tram track opacities, finger in Glove, singet ring)

*PFT = obstructive (DOWN FEV1 + FVC + FEV1/FVC <70%

  • Infxn : CBC (wbc, eosin), sputum= neutrophilia
  • Hypo-Ig: IgG/M/A
  • CF : sweat Cl test
  • ABPA panel
20
Q

Bronchiectasis

Tx

A

Treat underlying

Assume infxn- treat

Inflammation
Resection

21
Q

Bronchiectasis

Acute exacerbation Tx

A
  • antibio = H flu + Pseudo (fluoroquinolone)
  • tx Failure? = sputum culture + sensitivity
  • Prophylactic Abx = maybe
  • CF = Tobra + aztreonam
22
Q

Bronchiectasis

Reduce inflammation

A

Not helpful

Beta ag, anticholin, steroid

23
Q

Bronchiectasis

TX - surgery

A

Better in non-CF cases

24
Q

Bronchiolitis caused by

A

RSV

LRI = small airways

25
Q
  • infant/child
  • small airway inflammed (+2mm dm)
  • start = typical URI, then LRI
  • normal CBC
A

RSV = Bronchiolitis

DX = History + PE

26
Q

Bronchiolitis

Tx

A

SYMPTOMS + HYDRATION + O2

  • Low O2sat (<90%)? Give O2
  • Inpatient? Nebulized saline
  • <1 y/o? Hear/lung dz? premature? needed O2 at birth?—> #Ribavirin, #Prevention= Palivixumab

NO chest physiotherapy, albuterol, epi, ABX

27
Q

Bronchiolitis

When to admit?

A
  • <3 months/ 2 years Ipremature)
  • SpO2 = <93
  • RR >70-80
  • Respiratory distress
  • heart dz/lung dz
  • poor feeding
28
Q

Bronchiectasis

General Tx - Bronchial hygiene

A
  • Chest physiotherapy (percussion, postural drainage)

* Self-Directed (Ultrasonic mechanical vibration, positive expiratory pressure, flutter valve)

29
Q

Bronchiectasis

Increasing cough
Dyspnea
Increasing sputum
Dark sputum
Hemoptysis
Chest pain
A

Acute exacerbation of Bronchiectasis

30
Q

Bronchiectasis risk factors

A

Inherited lung diseases

  • Ciliary fibrosis
  • ABPA
  • autoimmune/inflammatory
  • marfan’s

Acquired

  • HIV/AIDS
  • Exposure/smoking
  • aspiration/FB
  • Infxn
31
Q

Cystic Fibrosis - TX

  • UP sputum, cough, RR,
  • Crackles, wheeze
  • dyspnea
  • DOWN exercise tolerance, appetite, PFT, weight
  • accessory muscles
A

ACUTE EXACERBATION

More chest physiotherapy + ABX

32
Q

Cystic fibrosis progression

A

INFANT
*infxn

CHILD

  • ABPA
  • Sinusitis
  • Polyposis

TEEN/ADULT

  • ABPA
  • Hemoptysis, pneumothorax
  • Respiratory failure
  • Sinusitis, polyposis, anosmia
33
Q

Bronchiectasis

causes

A

*Recurrent infxn
NO CF = H flu
CF = Pseudomonas

  • Hereditary (CF)
  • Obstruction (FB, Tumor, severe mucus impaction)
34
Q

Bronchiectasis

Causes

A

*recurrent infection

35
Q

Bronchiolitis types

A

Acute
Bronchiolitis Obliterans
Cryptogenic Organizing Pneumonia

36
Q

Obstructive

Increased volume
UP compliance
Hyperinflation *UP TLC/RV
Obstruction *DOWN FEV1/FVC

A
  • Asthma
  • COPD
  • Bronchiectasis
  • Cystic Fibrosis
  • sometimes Coal Workers Pn
37
Q

Restrictive Disorders

Decreased volume
DOWN compliance
*DOWN TLC, RV
*Normal FEV1/FVC (or UP)

A
  • Sarcoidosis
  • Pneumoconiosis
  • Idiopathic Pulmonary Fibrosis
  • scoliosis, mesothelioma

less muscular effort

Pulmonology (12 decks)

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