Cystic Fibrosis + Bronchiectasis + Bronchiolitis Flashcards
Cystic fibrosis
Etiology
*CFTR gene (Cl- transport, in all epithelial)
Delta F508
*auto recessive (both parents must be carrier)
*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
Cystic Fibrosis
Cystic Fibrosis
Path
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
Cystic fibrosis
Pulm effects
BRONCHIECTASIS Pneumonia *Pseudomonas/Staph Bronchitis Bronchiolitis Nasal polyps Chronic hypoxia
Child w/ rectal prolapse?
Cystic fibrosis
Child w/ pancreatitis?
Cystic fibrosis
Cystic fibrosis
GI effects
Meconium ileus Meconium plug Rectal prolapse Pancreatitis Steatorrhea
Cystic fibrosis
General Effects
- Failure to thrive
- Vitamin deficiency (lipase deficiency)
- Infertility (CBAVD) - crhonic bilateral absence of vas deferens)
Cystic Fibrosis
Dx
- newborn screening
- Sweat Cl test
- sibling dx w/ CF
- suggestive Hx
*Mutation analysis
*Nasal test
*CXR = bronchiectasis
USUALLY DURING INPATIENT
Cystic Fibrosis
Referring
*CF specialist referral
Managing:
- pulm dz
- acute exacerbations
- CF-related Diabetes (pancreatic destruction) = supplementation
- Complications
Cystic Fibrosis
Pulm Dz tx
- 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)
Cystic Fibroisis
Tx = Acute exacerbation MRSA
Vanc
OR
Linezolid
Cystic Fibroisis
Tx = Acute exacerbation
Pseudomonas
Severe = IV tobramycin + piperacillin
Cystic Fibroisis
Tx = Acute exacerbation
Pseudomonas
Tobramycin (inhaled) + Ceftazidime
Cystic Fibroisis
Tx = CF Diabetes
- > 30 y/o
- insulin
- pancreatic enzyme replacement therapy
Cystic Fibroisis
MC cause of death
Respiratory failure
Pulm dz biggest prognosis
Usually mid-20s to mid 30s
Cystic Fibroisis
Tx - no previous pseudomonas
Treat staph
Amoxi+clav
- 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)
Bronchiectasis
Bronchiectasis
Dx workup
- **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
Bronchiectasis
Tx
Treat underlying
Assume infxn- treat
Inflammation
Resection
Bronchiectasis
Acute exacerbation Tx
- antibio = H flu + Pseudo (fluoroquinolone)
- tx Failure? = sputum culture + sensitivity
- Prophylactic Abx = maybe
- CF = Tobra + aztreonam
Bronchiectasis
Reduce inflammation
Not helpful
Beta ag, anticholin, steroid
Bronchiectasis
TX - surgery
Better in non-CF cases
Bronchiolitis caused by
RSV
LRI = small airways
- infant/child
- small airway inflammed (+2mm dm)
- start = typical URI, then LRI
- normal CBC
RSV = Bronchiolitis
DX = History + PE
Bronchiolitis
Tx
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
Bronchiolitis
When to admit?
- <3 months/ 2 years Ipremature)
- SpO2 = <93
- RR >70-80
- Respiratory distress
- heart dz/lung dz
- poor feeding
Bronchiectasis
General Tx - Bronchial hygiene
- Chest physiotherapy (percussion, postural drainage)
* Self-Directed (Ultrasonic mechanical vibration, positive expiratory pressure, flutter valve)
Bronchiectasis
Increasing cough Dyspnea Increasing sputum Dark sputum Hemoptysis Chest pain
Acute exacerbation of Bronchiectasis
Bronchiectasis risk factors
Inherited lung diseases
- Ciliary fibrosis
- ABPA
- autoimmune/inflammatory
- marfan’s
Acquired
- HIV/AIDS
- Exposure/smoking
- aspiration/FB
- Infxn
Cystic Fibrosis - TX
- UP sputum, cough, RR,
- Crackles, wheeze
- dyspnea
- DOWN exercise tolerance, appetite, PFT, weight
- accessory muscles
ACUTE EXACERBATION
More chest physiotherapy + ABX
Cystic fibrosis progression
INFANT
*infxn
CHILD
- ABPA
- Sinusitis
- Polyposis
TEEN/ADULT
- ABPA
- Hemoptysis, pneumothorax
- Respiratory failure
- Sinusitis, polyposis, anosmia
Bronchiectasis
causes
*Recurrent infxn
NO CF = H flu
CF = Pseudomonas
- Hereditary (CF)
- Obstruction (FB, Tumor, severe mucus impaction)
Bronchiectasis
Causes
*recurrent infection
Bronchiolitis types
Acute
Bronchiolitis Obliterans
Cryptogenic Organizing Pneumonia
Obstructive
Increased volume
UP compliance
Hyperinflation *UP TLC/RV
Obstruction *DOWN FEV1/FVC
- Asthma
- COPD
- Bronchiectasis
- Cystic Fibrosis
- sometimes Coal Workers Pn
Restrictive Disorders
Decreased volume
DOWN compliance
*DOWN TLC, RV
*Normal FEV1/FVC (or UP)
- Sarcoidosis
- Pneumoconiosis
- Idiopathic Pulmonary Fibrosis
- scoliosis, mesothelioma
less muscular effort
