CMPP -- Bronchiectasis and Cystic Fibrosis

obstructive lung disease

classification of bronchiectasis

airflow limitation preventing adequate exhalation

common feature of obstructive lung diseases

Bronchiectasis

destruction of bronchial walls which leads to lumen dilatation and excessive secretions

lobe, lung segment, or subsegment (focal process)

Bronchiectasis typically involves...

inflamed and early collapsible airways -- results in airflow obstruction

#1 abnormality associated with Bronchiectasis

irreversible

airway damage associated with Bronchiectasis is considered...

chronic daily cough and viscid sputum

presentation of Bronchiectasis

muscular and elastic components of bronchial wall --> permanent bronchial dilatation --> impaired clearance of secretions

bronchiectasis results from destruction of...

pulmonary infections (may result from a single episode fo severe pneumonia)

what is the #1 inciting event for bronchiectasis?

recurrent lower respiratory tract infections (vicious cycle)

the development of bronchiectasis then results in...

infections, TB, foreign body aspiration, COPD, AAT deficiency, tumors (can also be congenital, not acquired)

what are some causes of acquired bronchiectasis?

daily cough with tenacious sputum, fever, weight loss, hemoptysis, pneumonias

what is commonly seen in a history of one with bronchiectasis?

RHF secondary to increased pulmonary artery pressure (JVD, hepatomegaly, edema)

cyanosis

weight loss

clubbing

wheezes, rales, rhonchi

what is commonly seen on PE for those with bronchiectasis?

no

in bronchiectasis, is airway obstruction reversed with bronchodilators?

High-resolution CT (excellent sensitivity and specificity for identifying bronchial lumen dilation)

what is the study of choice for bronchiectasis?

increased markings, atelectasis, honeycombing

what is a common finding on CXR for those with bronchiectasis?

vicious, mucopurulent, blood streaked

how can one describe the sputum associated with bronchiectasis?

Pseudomonas spp., E. coli, may also see atypical mycobacteria and fungi

what kinds of bacteria are found in sputum studies for those with bronchiectasis?

bronchodilators for acute exacerbations, prophylactic/nebuluzed antibiotics, chest physiotherapy, lung resection, manage underlying causes

treatment options for bronchiectasis

therapeutic adjunct in advanced or complicated disease -- last resort for control of massive hemoptysis

when should one perform lung resection on a pt with bronchiectasis?

Cystic fibrosis

exocrine gland disease affecting the bronchi, pancreatic ducts, intestines, reproductive tract, and sweat glands; genetic disorder

autosomal recessive (1 in 4 chance if both parents are carriers)

CF has an ________ inheritance pattern

standard newborn screening

how are most cases of CF diagnosed?

CF transmembrane conductance regulator

CF is caused by a defect in...

defects in chloride transport

defects in the CF gene (chromosome 7) can result in...

reduced height of epithelial lining and decreased hydration of mucus (viscous, tenacious mucus)

also affects secretions in pancreas, liver, intestines, sweat glands, and reproductive tract

how can defects in chloride transport affect a CF pt?

bacteria adhere to viscous mucus, which can result in obstruction, recurrent infection, and airway injury (can lad to bronchiectasis)

how can tenacious mucus lead to complications?

immunoreactive trypsinogen

DNA analysis for mutations in CFTR (CF transmembrane conductance regulator)

what are the screening assays for CF in newborns?

sweat chloride testing -- is diagnostic

if any of the screening assays for CF are positive, what's the next step?

positive newborn screening

suggestive symptoms

sibling with disease (1 in 4 risk)

what are the indications for sweat chloride testing?

failure to thrive, cyanosis, clubbing

general signs and symptoms of CF

severe sinus disease

chest pain

DOE

purulent, viscous sputum

atypical "asthma"

tachypnea, retractions

wheezes, rales, rhonchi

respiratory manifestations of CF

delayed meconium passage/meconium ileus

increased stool frequency

steatorrhea

abdominal distention and intestinal obstruction

hepatomegaly

rectal prolapse -- BIG RED FLAG IN KIDS

GI manifestations of CF

undescended testicles

hydrocele

delayed secondary sexual development

high incidence of infertility in males

amenorrhea

GU manifestations of CF

no

do women with CF typically have fertility issues?

Chest PT -- vibratory vest promotes secretion clearance

what is the primary tx for CF?

nebulized, oral, and IV antibiotics (TRYING TO PREVENT VIRULENT PULM INFXNS SECONDARY TO PSEUDOMONAS and STAPH)

nebulized bronchidilators

inhaled mucolytic enzymes and hypertonic saline

what are other treatments for CF other than vibratory vest?

oral abx

mild CF exacerbations are treated with...

highly virulent bugs such as Pseudomonas and Staph aureus

(require broad spectrum abx)

severe exacerbations of CF are secondary to...

nutritional support -- fat soluble vitamin supplementation, pancreatic enzyme supplementation, pancreatic endocrine function support

what are some therapeutic options that aren't "medical" (not medications per se)

ADEK

fat soluble vitamins

respiratory failure and RHF

primary causes of death of those with CF

a tertiary care center

all patients with CF should be evaluated at...