Respiratory Disease Flashcards
Pleural fluid > 1000 cells, >80% lymphocytes, TG > 100s.
Chylothorax
R>L
FA, TG and protein LOSS
Risk infection - also lose immunoglobulins
Transudative vs exudative
Transudative -pH>7.4, wbc< 1000, LDH <200
Exudative pH < 7.4, wbc > 1000, LDH > 200
Describe congenital pulmonary lymphangiectasia
- when does lymphatic system develop
- genetic associations (primary CPL)
- secondary CPL exs
Mgmt, Prognosis
Devp - starst 6 wks, usually complete by 20 wks
Genetics - Associated with Noonan’s, Ehlers-Danlos, Ullrich-Turner, Down’s syndrome
Secondary - TAPVR, HLH with restricted septum
Supportive care, poor prognosis, later dx has a better prognosis
Review stages of lung devp and congenital lung abnormalities – 2 mistakes!
cong lobar emph - LUL #1, increased risk CHD, disruption of brochopulm devp (cartilageous defect?)
CPAM - branchingabnormalitof lung at differentairway levels; imbalance cell proliferation and celldeath; cystic and adenomatous overgrowth, 5 types, #1 70%
0,2 with anomalies - poor prognosis, 3 vey large
bronchogenic cyst - anomalousbudding of foregut, mostly posterior trachea, sx dep on size, location
Cong pulm lymph - M>F increased risk pleural effusions, often need bx to dx, hyperinflated lungs, poor prognosis if severe
BPD - nonfunctioning lungtissue
bronchogenic cyst - embryonic stage
cong lobar emphysema - pseudoglandular stage
Types 0 to 4 CPAM
- most common
- most severe / bad prognosis
- most associated with other anomalies
intralobar vs extralobar BPS
congenital pneumonia
- organisms by when it presents - perinatal, early (1 wk) or late (after 1 week)
Choanal atresia
- R vs L
- Unilateral vs bilateral
- Bony vs mixed
- How often associated with other anomalies
- Right
- FEMALES > males
- 70% mixed
- 50% other anomalies
Phrenic nerve injury
- which cranial nerves
- causes of injury
- sx
C3 - C5
75% from associated injuries – neck hyperextension
innervates diaphragm - resp distress
could - rare - assocaited with spinal cord injury (VASCULAR or bony injury or fracture)
Pneumothorax
- when does it resolve
1 to 2 days
Pneumomediastinum
- XR finding
- causes
- sail sign / angel wings
- can be associated with pharyngeal perf (see air around mediastinum, air around airway)
Chylothorax
-what causes
- MOST COMMON PLEURAL EFFUSION IN NEONATES
- M vs F
- analysis of pleural fluid
- primary vs secondary (%)
- examples of primary
primary -
— congenital chylothorax eg trisomy 21, noonan, turner’s
— congenital pulm lymphangiectasia - abnormally wide or dilated lymphatics, can compress airway
M»_space; F
Fluid: lymph predominance > 80%, TG >100, LDH elevated
Can give MCT, fat free diet (MCT absorbed into vasculature not lymphatics)
Most are secondary / acquired
Congenital lobar emphysema
- pathophysiology
- primary area of lung
1 LUL
defective bronchial cartilage –> overexpansion of 1+ pulmonary LOBES which can compress others / airtrap
Non functional lobe
pseudoglandular stage?
about 1/4 present at birth
CDH
- most common location
- when dx (when develop)
- what LHR is poor prognosis; what LHR may be associated with high survival
- what O:E poor survival
1 - posterolateral
can dx around 24 weeks although develops around 16 wks (pseudoglandular stage)
LHR > 1.3 to 1.4 - good prognosis
LHR < 1 - poor
O:E < 25% - poor survival
NEHI
CXR hyperexpansion and increased ground glass
ILD
initially asx, then sx within 1st 3 months
disorder of poorly understood or unknown etiology
Pulmonary interstitial glycogenesis
Hypoxemic at birth, mild symptoms
may respond to steroids
disorder of poorly understood / unknown etiology
Pulmonary hemorrhage
- due to acute increase in capillary hydrostatic pressure — can result from L to R shunt from PDA OR vasoconstriction after perinatal depression or ACUTE pressures changes after surfactant
- capillary vessels break down and “leak” fluid
Mgmt - increase PEEP (distend against blood, decrease PBF), check coags
CPAM
see by 18 weeks
tend to grow by 28 weeks
Alveolar capillary dysplasia
LARGE pulm veins next to bronchovascs thick alveolar septa, capillary not close to alveoli
PPHN presentation at term, resp failure within 48 hours.
HUGE air leak risk
acinar dysplasia
80% associated with other anomalies
Cystic fibrosis
increase IRT
pancreatic insufficiency, FTT, fat malabsorption, mec ileus, bilateral agenesis vas deferencs, obstructive lung disease
VV vs VA ECMO
- achievable PaO2
- high or low perfusion state required
- maintains pulm blood flow
- caridac support
- which lower risk of arterial emboli
VV
- lower achievable PaO2
- as maintains PBF
- no cardiac support
- requires higher perfusion rates
- lower risk of arterial emboli
VA - higher achievable PaO2
- bypasses pulm circulation
- cardiac support
- requires lower perfusion rates
Acinar dysplasia
complete absence of alveolar development, ONLY airway structures
Largely in FEMALES
Term infant, severe PPHN, severe resp failure in 48 hours (like ACD)
