+ newborn screening Sweat Cl test sibling dx w/ CF suggestive Hx Mutation analysis Nasal test CXR = bronchiectasis USUALLY DURING INPATIENT

Cystic Fibrosis + Bronchiectasis + Bronchiolitis Flashcards by O lawformonde

Cystic fibrosis

Etiology

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

*auto recessive (both parents must be carrier)

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*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

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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

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Cystic fibrosis

Pulm effects

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

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Child w/ rectal prolapse?

Cystic fibrosis

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Child w/ pancreatitis?

Cystic fibrosis

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Cystic fibrosis

GI effects

Meconium ileus
Meconium plug
Rectal prolapse
Pancreatitis
Steatorrhea

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Cystic fibrosis

General Effects

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

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Cystic Fibrosis

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

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

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Cystic Fibrosis

Referring

*CF specialist referral

Managing:

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

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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)

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Cystic Fibroisis

Tx = Acute exacerbation MRSA

Vanc
OR
Linezolid

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Cystic Fibroisis

Tx = Acute exacerbation
Pseudomonas

Severe = IV tobramycin + piperacillin

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Cystic Fibroisis

Tx = Acute exacerbation
Pseudomonas

Tobramycin (inhaled) + Ceftazidime

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Cystic Fibroisis

Tx = CF Diabetes

> 30 y/o
insulin
pancreatic enzyme replacement therapy

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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

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

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