Respiratory Disease

Question 1

Pleural fluid > 1000 cells, >80% lymphocytes, TG > 100s.

Answer 1

Chylothorax

R>L
FA, TG and protein LOSS
Risk infection - also lose immunoglobulins

Question 2

Transudative vs exudative

Answer 2

Transudative -pH>7.4, wbc< 1000, LDH <200

Exudative pH < 7.4, wbc > 1000, LDH > 200

Question 3

Describe congenital pulmonary lymphangiectasia

• when does lymphatic system develop
• genetic associations (primary CPL)
• secondary CPL exs

Mgmt, Prognosis

Answer 3

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

Question 4

Review stages of lung devp and congenital lung abnormalities – 2 mistakes!

Answer 4

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

Answer 5

Answer 6

Question 7

Types 0 to 4 CPAM
- most common
- most severe / bad prognosis
- most associated with other anomalies

Answer 7

Question 8

intralobar vs extralobar BPS

Answer 8

Question 9

congenital pneumonia
- organisms by when it presents - perinatal, early (1 wk) or late (after 1 week)

Answer 9

Answer 10

Question 11

Choanal atresia

• R vs L
• Unilateral vs bilateral
• Bony vs mixed
• How often associated with other anomalies

Answer 11

• Right
• FEMALES > males
• 70% mixed
• 50% other anomalies

Question 12

Phrenic nerve injury

• which cranial nerves
• causes of injury
• sx

Answer 12

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)

Question 13

Pneumothorax

• when does it resolve

Answer 13

1 to 2 days

Question 14

Pneumomediastinum

• XR finding
• causes

Answer 14

• sail sign / angel wings
• can be associated with pharyngeal perf (see air around mediastinum, air around airway)

Question 15

Chylothorax

-what causes
- MOST COMMON PLEURAL EFFUSION IN NEONATES

• M vs F
• analysis of pleural fluid
• primary vs secondary (%)
• examples of primary

Answer 15

primary -
— congenital chylothorax eg trisomy 21, noonan, turner’s
— congenital pulm lymphangiectasia - abnormally wide or dilated lymphatics, can compress airway

M&raquo_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

Question 16

Congenital lobar emphysema

• pathophysiology
• primary area of lung

Answer 16

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

Question 17

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

Answer 17

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

Question 18

NEHI

Answer 18

CXR hyperexpansion and increased ground glass

ILD

initially asx, then sx within 1st 3 months

disorder of poorly understood or unknown etiology

Question 19

Pulmonary interstitial glycogenesis

Answer 19

Hypoxemic at birth, mild symptoms

may respond to steroids

disorder of poorly understood / unknown etiology

Question 20

Pulmonary hemorrhage

Answer 20

• 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

Question 21

CPAM

Answer 21

see by 18 weeks

tend to grow by 28 weeks

Question 22

Alveolar capillary dysplasia

Answer 22

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

Question 23

Cystic fibrosis

Answer 23

increase IRT

pancreatic insufficiency, FTT, fat malabsorption, mec ileus, bilateral agenesis vas deferencs, obstructive lung disease

Question 24

VV vs VA ECMO

• achievable PaO2
• high or low perfusion state required
• maintains pulm blood flow
• caridac support
• which lower risk of arterial emboli

Answer 24

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

Question 25

Acinar dysplasia

Answer 25

complete absence of alveolar development, ONLY airway structures Largely in FEMALES Term infant, severe PPHN, severe resp failure in 48 hours (like ACD)

Question 26