Exam I, lungs, Gomez Flashcards
how many lobes of the lung are there? bronchopulm segments?
lobes-5
segments- 19
What size particle is most dangerous to inhale
0.5-5 micron
how big are terminal bronchi
<2mm
in lungs what type of specialized cells are only in trachea and bronchus
goblet cell, seromucous gland and cartiladge
in lungs what type of specialized cells are only found in bronchioles
clara cells
in lungs what type of specialized cells are only found in alveoli
type I pneumocyte and type II pneumocyte
Majority of surface alveolar cells ware what type
Type I non-div
What is a lobule? acinus?
lobule- cluster of terminal bronchioles with attached acini
acinus- respiratory bronchiole and all attached alveolar ducts and sacs
what is a common congenital anomalie found in 10% neonatal autopsies
pulmonary hypoplasia
What is the most common type of tracheoesophageal fistula
the dista part of esophagus comes off trachea, proximal part is a blind ending pouch
describe congenital foregut cysts
detached section of maldeveloped foregut mediastinal and hilar locations usually bronchogenic with resp epithelium some esophageal- squamous mucosa enteric- intestinal mucosa
What are congenital cystic adenomatoid malformations and the types?
hamartomatous lesion with abnormal bronchiolar tissue
type I- large cysts, good prognosis
type II- medium cysts with other malformations
what are bronchopulmonary sequestrations and types?
areas of lung without normal connection to airways
blood supply is from systemic aa
Extralobular- external to lung(have other anomalies)
intralobular- within lung have recurrent local infections, most likely an acquired lesion
What are causes of resp distress in newborn
excessive maternal sedation fetal head injury blood or amniotic fluid aspiration intrauterine hypoxia from nuchal cord hyaline membrane disease
What is most common cause of resp distress in newborn
hyaline membrane disease
the rate of neonatal resp distress syndrome is inversely proportional to what
gestational age
What factors are associ wtih neonatal resp distress syndrome
male, maternal DM, multiple gestation, C section before onset of labor
What induce surfactant secretion
glucocorticoids and thyroxine
At 20 weeks wat to alveoli look like? 30?
20-glandular
30- saccular
what type cells secrete surfatant
type II pneumocytes
If lecithin/sphingomyelin ratio is >2 what does this indicate in lungs?
<1?
> 2 lungs are mature
<1 lungs immature
How do you obtain the L/S ratio for lung development
thin layer chromotogrpahy
gluorescence polarization
foam stability index
lamellar body count
Atelectasis leads to what
uneven perfusion and hypoventilation
What is clinical presentation of respiratory distress syndrome?
preterm and AGA male sex, maternal DM, C section low 1 min Apgar score may need resuscitation become cyanotic fine pulm rales reticulonodular/groun glass CXR O2 Therapy death or recovery in 3-4 days
why is respiratory distress syndrome not seen in stillborns
need to exude protein rich fluid into alveolar space to make a hyaline membrane
what is risk of O2 therapy with infants with respiratory distress syndrome
retinopathy of prematurity
bronchopulmonary dysplasia
What criteria for O2 therapy in cases of bronchopulmonary dysplasia
> 28 days O2 therapy in infant>36 weeks post-menstrual age
What is deficit in infants with bronchopulmonary dysplasia
alveolar hypoplasia and thickened walls
developmental arrest at saccular stages
what is defect in cycstic fibrosis (mucoviscidosis
disorder of epithelial transport of cystic fibrosis transmembrane conductance regulator (epithelial Cl Ch)
affects fluid secretion in exocrine glands and epithelial linging of resp, GI and repro tracts
What gene is CFTR on
7q31.2
What is Dx criteria for cycstic fibrosis
one or more characteristic phenotypic features(or Hx of CF in sibling) (or +newborn screening test)
AND
Increased sweat Cl [ ] on 2+ occasions (or 2 CFTR mutations)(or abrnomral epithelial nasal ion transport)
How do you Tx pancreatic insufficiency from CF
oral pancrelipase
may uncommonly need to Tx DM if islets destroyed
How do you Tx vitamin deficiency from CF
oral fat soluble vitamins ADEK
parenterteal nutrition
how do you Tx pulmonary disease from CF
postural drainage and chest percussion bronchodilators (albuterol) mucolytic agents antibiotics hypertonic saline high dose ibuprofen
What is dangerous about infants who have had an apparent life threatening event
if they have been resuscitated they are now at increase risk of future respiratory death
have prolonged apnea, dminished responses to hypercarbia or hypoxia
often premature or have mechanical disorders leading to compromise
How can atelectasis occur in an adult
resportion (obstruction of airways and the mediastinum shifts toward involved lung)
compression from external Pressure(mediastinum shifts away from involved lung)
contraction (secondary to fibrosis of lung or pleura, irreversible)
All atelectasis are at risk for what
infection
What are 2 main types pulm edema
hemodynamic and microvascular
describe hemodynamic PE
most common increased hydrostatic P from Left HF affects basal lower lobes "ehart failure cells" can lead to alveolar fibrosis (brown induration of lungs)
describe microvascular PE
increased permeability from infection or toxic injury etc
if diffuse can lead to acute Resp distress syndrome
What is criteria for acute lung injury non-cardiogenic PE
acute onset dyspnea
hypoxemia
b/l infiltrates
absence of primary L HF !!
What type sof injuries can cause non-cardiogenic PE
congestion, surfactant disruption
atelectasis
What mediators are related to non-cardiogenic PE
cytokines, oxidants, TNF, ILs, TGF-beta
non cardiogenic PE can lead to what
ARDS (DAD diffuse alveolar damage) or AIP(acute interstitial pneumonia
what occurs in diffuse alveolar damange
damage to alveolar capillary walls(involvess neutrophilic migration)
have vascular injury and secondary loss of surfactant
can develop prothromnotic milieu (increased TF and dec Protein C)
what type of patients are more prone to diffuse alveolar damage
spesis, diffuse pulmonary infections, gastric aspiration, trauma
What infections can lead to aRDS
sepsis
diffuse pulm infection
gastric aspiration
what type of physical injuries can lead to ARDS
mechanical trauma and head injuries! pulm contusions near drowning fractures with fat embolism burns ionizing radiation
more then 50% ARDS are assoc with what 4 conditions
sepsis, diffuse pulmonary infections, gastric aspiration, mechanical trauma including head injuries
reperfusion after lung injury can lead to what
ARDS because increase ROS
What is clinical presentation of ARDS
already ill rapid onset, profound dyspnea and tachypnea cyanosis and resp failure diffuse b/l infiltrates on XR high moratlity
What would you seen histologically in alveoli healing from ARDS
resorption of hyaline membranes with thickened albeolar septa with fibroblasts, collagen and many atypical type II pneumocytes
Describe acute interstitial pneumonia AIP
Hamman-Rich syndrome
Sx similar to ARDS no associated causative disorder ~59 y.o M=F acute respi failure follwoing illness 33-74% mortalitiy rate within 2 mo
What are 2 types of chronic diffuse lung disease
obstructive and restrictive
describe obstructive airway disease characteristics, give examples
limit in rate of flow
FEV1/FVC reduced <0.7
R at any level
e.g: emphysema, chronic bronchitis, bronchiectasis, asthma
describe restrictive airway disease charactersitics, give examples
limit total lung capcity and residual volume
near normal flow rates (FEV1 may be mildy reduced due to dec TLC but FEV1/FVC near normal)
e.g: ches wall disorders, obesity, ARDS, interstitial fibrosis, penumoconioses
what are the major pathological changes in chronic bronchitis?
mucus gland hyperplasia, hypersecretion
what are the major pathological changes in emphysema
airspace enlargement, wall destruction
what are the major pathological changes in small-airway disease, bronchiolitis
inflammatory scarring/obliteration of bronchioles
What are the classifications of emphysema
centriacinar/centrilobular >95%
panacrinar/panlobular
distal acinar/paraseptal
irregular/paracicatrical
where is centriacinar emphysema found and what causes it
upper lobes and apices
SMOKING
where is panacinar epmphysema and what causes it
lower lobes/anterior
alpha 1 antitrypsin deficiency and SMOKING
where id distal acinar/paraseptal emphysema and wht caues it
subpleural and adjacent to septae
may be bullous and cause spontaneous pneumo in young adults
associated with previously damaged lung
which type of emphysema is usually asymptomatic
irregular/paracicatrical
how does panacinar emphysema progress
initial distention of albeolus and duct then involves entire pulmonary lobule
what is etiology of emphysema
proteolytic digestion of alveolar walls
neutrophil secreted elastase is primary villain (inhibited by alpha1 antitrypsin
What deficiency leads to early life emphysema
chrom 14 alpha 1 antitrypsin deficiency
When do Sx of emphysema develop
after 1/3 lung damaged
what are respiratory levels in emphysema
low FEV1, high TLC and RV
low FEV1 due to bronchiole collapse and fibrosis
what is Tx of emphysema
bronchodilators, steroids, bullectomy,transplant
what is obstructive overinflation
overexpansion by trapped air
1-ball valve obstruction by object
2-collaterals feeding around obstruction
3- congenital lobar overinflation from lack of bronchial cartilage
is there wall destruction in compensatory hyperinflation
no
ovvurs from loss of adjacent tissue
What is clinical definition of chronic bronchitis
3 mo productive cough/yr for 2 consecutive yeras
what are causes of chronic bronchitis
smoking
hypersecretion mucus from stimulation by protesases
and increased Reid index >0.4 is indicative of what
chronic bronchitis
What type of vasulities can occur in chronic bronchitis
bronchiolitis obliterans in small airways
who are the blue bloaters
chronic bronchitis
who are the pink puffers
COPD
Redi index is a measurement of what
thickness of glands/thickness of wall
What is age of onset with predominant bronchitis vs predominant emphysema
bronchitis- 40-45
emphysema 50-75
what are the characterisitics of bronchitis that do not occur in emphysema
cor pulmonale infections elastic revoil is normal increased airway resistance large heart blue bloater
what are characteristics of emphysema not seen with bronchitis
terminal resp insuficiency
low elastic recoil
small heart
pink puffer
What is asthma
chronic inflammarotyr disorder
reactive airspace disease with episodic partially reversible bronchoconstriction
most often at night and early morning
extrinsic is allergecns, exercise cold is intrinsic
what can asthma evolve into
acute severe asthma
What is atopic asthma
type I HS
IgE Antibodies to inhaled allergens
mast cells
stimulation of subepithelial vagal R
what are Dx testing for atopic asthma
skin allergen testing for confirmation RAST testing (radioallergosorbent test)
What molecules induce bronchoconstriction
Ach from nerves, Leukotrienes C4 D4 E4 from mast cells
histmine and PGD2
What is nonatopic asthma
pulmonary infections (viral) and air pollutants no allergic indicators
What is drug induced asthma
uncommon form of disease
aspirin is the classic cause because inhibit COX pathway
What is morphology of asthma
overinflated lungs
with airway remodeling like eosinophilia, subBM fibrosis, hypertrophy and hyperplasia of submucosal glands and goblet cell metaplasia
hypertrophy and hyperplasia of smooth muscle
increased vascularity
What are charco-leyden crystals
crystalloids with eosinphil lysophospholipase binding protein
what are curshcmann spirals in alveoli
from shed epithelium
What is bronchiectasis
permanent dilation of bronchi and bronchioles
tissue destruction secondary to infection
What are Sx of bronchiectasis
dyspnea, orthopnea, and rarley severe hemoptosis that is fould semlling
may develop brain abscesses and amyloidosis
What is bronchiectasis assoc with
CF, primary ciliary dyskinesia obstruction many infections autoimmune pulm sequestration allergic bronchopulmonary apsergiloosis
What syndrome is assoc with primary ciliary dyskinesia
kartagener syndrome:
male infertility, sinusitis, situs inverus of partial lateralizing abnormality
Xray shows reticulonodular or gournd glass appearance involeing intersitium and alveolar walls
patint has dec TLC RV
dyspnea tachypnea, cyanosis, end-inspiratory crackles
end-stage honeybomc lung
chronic diffuse interstitial lung disease
What are the fibrosing chronic intersitial lung diseases
idiopathic pulm fibrosis nonsepcific interstitial pneumonia cryptogenic organizing pneumonia CT disease associated pneumoconioses drug reactions radiation pneumonitis
what are the granulomatous chronic interstitial lung diseases
sarcoidosis
HS pneumonitis
eosinphilic pneumonias!!!
what are the smoking related chronic interstitial lung diseases
desqaumative interstitial pneumonia
respiratory bronchiolitis-assoc interstitial ung disease
what are the other types of chronic interstitial lung diseases
pulmonary langerhans cell histiocytosis
pulmonary albeolar proteinosis
lymphoid interstitial pneumonia
What occurs in idiopathic pulm fibrosis (usual interstitial pneumonia) “crytptogenic fibrosing aleolitis”
type I pneumocyt death
type II hyperplasia
inflammation with TH2 , cytokines, macrophages, fibroblasts driven by TGF-b, colagen deposition
What environmental factors contribute to idiopathic pulm fibrosis
smoking smoking
work related exposures
reflux esophagus
What are the effects of TGF-b on respiratory epithelium
inhibits caveolin in fibroblasts
ceveolin usually inhibits deposition of collagen and ECM so TGF-b promotes this
reduces telomerase and induces fibroblasts and myofibroblasts
What is clinical presentation of idiopathic pulm fibrosis
insidious unpredictable disease in middle aged >50
dyspnea, dry cough, hypoxemia with cyanosis, digital clubbing
mean survival is <3 yrs
describe patholody of idiopathic pulmonary fibrosis
repeated cycles of alveolitis
healing/scarring leads to patchy interstitial fibrosis
end stage looks like honeycomb lung
What is Tx for idiopathic pulm fibrosis
lung transplant
clinical presentaion non sepcific interstitial pneumonia
dyspnea and cough for months
middle age with milder Sx
Histo presentation of nonsepcific interstitial pneumonia
mild patchy or diffuse interstitial fibrosis
no fibroblastic foci or honeycombing
What does crytopgenic organizing pneumonia look like?
also called bronchiolitis obliterans organizing pneumonia (boop)
all CT of same ange and no interstitial fibrosis
does not progress to honeycomb lung
What is Tx for cryptogenic organizing pneumonia
oral steroids for > 6 mo
What are the collagen vascular disorder-related intersitial lung diseases
rheumatoid arthritis scleroderma SLE sjogren syndrome polymyositis/dermatomyositis mixed CT disorder
what is caplan syndrome
combination of rheumatoid arthritis and pneumoconiosis
What is most dangerous particle size and shwy
1-5 micron amount/volume size shape buyoncy solubility cytotoxicity concomitant irritants
what mineral inhalants can lead to caplan syndrome
coal dust
silica
asbestos
What chemical fumes can lead to asthma
NO, SO2, NH4, benzene