Problem questions (ie ones I can't remember) Flashcards
Characteristics of the Parietal Pleura
Consists of a single layer of flat, cuboidal mesothelial cells, supported by loose connective tissue.
The arterial supply = intercostal and internal mammary arteries.
Venous blood drains to the systemic circulation.
Innervation = sensory branches of the intercostal and phrenic nerves.
Has direct connection to the lymphatic vessels.
- Stomas: increase with inspiration
Anterior parietal pleura drains to the internal intercostal lymph nodes
Posterior parietal pleura drains to the lymph nodes located along the internal thoracic artery.
Characteristics of the Visceral Pleura
Tightly adherent to lung surface and interlobar fissures
Microvilli, are evident on the apical surfaces - homeostasis of the pleural fluid and contribute to transmembrane solute and fluid movement.
Vesicles trap particles and glycoproteins, thereby reducing friction between the visceral and parietal pleurae.
Arterial supply = the bronchial arteries, with a minor contribution from the pulmonary circulation.
The lymphatic glands drain to the mediastinal nodes, following the course set by the pulmonary veins and arteries.
The visceral pleura has no sensory innervation, but it is supplied by branches of the vagus and sympathetic trunks.
Blood supply to the lung?
Lung gets blood from 2 separate systems:
1) Bronchial
○ High pressure/low volume
○ Bronchial arteries come form aorta or a branch from that
○ Provides blood to conducting airways -> mainstem bronchi to terminal bronchioles
○ Bleeding -> profuse, can result in massive hemoptysis
2) Pulmonary ○ Low pressure/high capacitance ○ Comes from RV ○ Supplies acinar units (gas exchange) ○ Alveolar hemorrhage -> low grade, chronic, diffuse
2 pathways for pulmonary fluid clearance
1) Lymphatics (most important)
2) Venular end of the microvascular bed
Causes of pulmonary hemorrhage in children
Infection Abscess Pneumonia Trauma Vascular disorders Coagulopathy Congenital Lung Malformations Miscellaneous: Catamenial, Factitious, Malignancy DAH syndromes
Causes of DAH syndromes
1) Immune mediated
- Idiopathic pulmonary capillaritis
- GPA
- MPA
- Anti-GBM disease
- SLE
- HSP
- Behcets
- Cryoglobulinemia vasculitis
- JIA
- COPA syndrome
2) Non-immune mediated
- IPH
- Acute idiopathic pulmonary hemorrhage of infancy
- Heiner’s syndrome
- Asphyxiation/abuse
- CV causes
- Pulm vein atresia/stenosis
- TAPVR
- Mitral stenosis
- Left sided failure
- PCH
- Pulm telangiectasia
6 classes of CF mutations
Class I: protein synthesis defect - affects CFTR transcription
Class II: maturation defect - misfolded protein, early degradatiob
Class III: gating defect - reduced ATP binding, resulting in abnormal gating characterized by a reduced open probability
Class IV: conductance defect-alter the channel conductance by impeding the ion conduction pore, leading to a reduced unitary conductance
Class V: reduced quantity- reduced amount of normal functioning protein
Class VI: Reduced stability - conformational stability reduced
Pseudomonas mechanisms of inhibition of lung defenses
1) Induced damage in immune cells
2) Virulence traits and secretion of virulence factors
3) Epithelial injury - loss of alveolar barrier - biofilm formation
4) resistant to neutrophils
Pseudomonas biofilm properties
1) Mucous provides anaerobic environment for bacterial growth
2) Decreased secretion of bactericidal compounds into CF airways
3) increased DNA and actin
Pseudomonas mechanisms of resistance
1) Amp C beta-lactamase
2) Extended spectrum beta-lactamase
3) Downregulation of Opr-D
4) Multidrug efflux pumps
5) Biofilm formation
Measurement of nasal potential difference
- Difference of electric potential measured between a reference electrode and silver/silver chloride electrode
- Inferior turbinate
- Recordings - continuous flow of salt solutions
- Measured in: saline, amiloride, chloride free amiloride, isoproteronol added to activate CFTR
- Sum = index of CFTR function
- <40 = abnormal ( 2 separate days)
Components of MAC complex
- M. Avium
- M. Intracellulare
- M. Chimaera
- M. Kansasii
“slow growers”
Treatment of MAC complex
1) No CF or cavitations: macrolide, rifampin, ethambutol 3x/week PO
2) CF: daily as above
3) CF with resistance or cavitations: 1-3 mos IV amikacin plus above
Treatment of M. Kansasii
**Think of TB
Isoniazid, Rifampin, Ethambutol x 12 mos after 1st culture negative
Components of M. Abscessus species complex
- M. Abscessus
- M. Massiliense
- M. Balleti
“rapid growers” *Younger and more severe disease
Treatment of M. Abscessus
Intensive and Continuation
I = 3-12 weeks of IV Amikacin + 1 of: Cefoxitin, Imipenum, Tigecycline
C = Inhaled Amikacin with 2-3 of: Moxifloxacin, Minocycline, Clofazamine, Linezolid
Dx criteria for NTM disease
Clinical (both are required):
- Pulmonary symptoms, nodular or cavitary opacities on CXR or CT showing multifocal bronchiectasis with multiple small nodules
- Appropriate exclusion of other diagnoses
Micro criteria (need one)
- Positive culture results from at least 2 separate expectorated sputum samples
- Postive culture results from at least one bronchial wash or lavage
- Transbronchial or other lung bx with mycobacterial histopath features and postive cx for NTM
General treatment guidelines for NTM pulmonary disease
1) Treat 12 mos beyond culture conversion (after first negative culture, need 3 negative in total)
2) No macrolide monotherapy
3) Monthly AFB smears + cultures while on treatment
4) Should show clinical improvement in 3-6 mos
5) Treatment failure = no response after 6 mos of appropriate treatment or no conversion to AFB negative culture after 12 mos
Conditions that may be associated with an increase incidence of CFTR mutations but insufficient evidence to fulfill a CF diagnosis
- Pancreatitis: acute or recurrent
- Disseminated bronchiectasis
- Isolated obstructive azoospermia
- ABPA
- Diffuse panbronchiolitis
- Sclerosing cholangitis
- Neonatal hypertrypsinogenemia
- Rhinosinusitis
- Heat exhaustion
Minimum criteria for ABPA (6)
1) Asthma or CF
2) Worsening lung function without another etiology
3) + skin prick with Aspergillus
4) IgE ≥ 1000
5) Increased Aspergillus species specific IgE and IgG
6) New or recent abnormal CXR or CT findings not improved with abx or physio
Additional criteria for ABPA (4)
1) Increased blood eosinophilia ≥ 400 when not on steroids
2) Aspergillus species -specific precipitating antibodies
3) Central bronchiectasis (central varicose)
4) Aspergillus species-specific containing mucous plugs
Characteristics of congenital bilateral absence of vas deferens (CFTR-RD)
1) Absence of palpable vas deferens
2) normal or increased FSH
3) Absence of intra-abdominal tract of VD and hypoplasia of seminal vesicles
4) pH <7.2, negative fructose + alpha 1-4 glucosidase
5) no developmental anomaly
How does intestinal current measurement work to assess CFTR function?
- record transepithelial short-circuit current or transepithelial voltage in rectal biopsies as measure of ion transport after stim with chloride secretagogues
- stimulates potassium channels - increasing driving force for luminal chloride
- in CF - activation fails to induce apical chloride secretion and results in inverse response
+ response = potassium secretion with no chloride
Assessment of Pancreatic Function
Pancreatic fxn should be objective assessed at time of diagnosis
Pts who are PS should be monitored regularly for evidence of progression ot PI
Function can be assessed several ways:
- 72h fecal fat collection (+record fat intake)
- PI defined by fat loss >15% of intake in infants <6 mos; >7% of intake in older infants - serum trypsinogen
- at birth serum levels usually elevated, then decline to low-undetectable leves in PI pts by age 5-7
- PS pts show fluctuating levels w/I and above N range at all ages
- in pts who are PS at diagnosis and progress to PI, there’s a delayed decline in serum trypsinogen - fecal elastase -low in pts with PI
Pulmonary complications of IBD
Bronchiectasis (most common) Tracheal stenosis Ileobronchial colobronchial fistula COP (Cryptogenic organizing pneumonia) granulomatous and necrobiotic nodules ILD Pulmonary Vasculitis Drug induced disease (Sulfasalazine and Mesalamine can cause HP) Opportunistic infection Malignancy Pulmonary thromboembolism
Major criteria for HP (6)
Need 4
- Symptoms compatible with HP
- Evidence of exposure to antigen
- Radiographic characteristics consistent with HP
- BAL fluid lymphocytosis (CD8 > CD4)
- Lung biopsy demonstrates histology consistent with HP
- Positive natural challenge that produces symptoms and objective abnormalities after reexposure to the offending antigen
Pathology findings in sub-acute hypersensitivity pneumonitis
The classic triad of
- Interstitial lymphocytic-histiocytic cell infiltrate.
- Bronchiolitis obliterans.
- Scattered poorly formed non-necrotizing granulomas
Pathology findings in chronic hypersensitivity pneumonitis
- Giant cells, granulomas, or Schaumann bodies
- Interstitial pneumonia (UIP)–like Pattern.
- A nonspecific interstitial pneumonia (NSIP)–like pattern or a bronchiolitis obliterans organizing pneumonia like disease can also be seen in chronic HP
Pathology findings in acute hypersensitivity pneumonitis
- Interstitial mononuclear cell infiltrate.
2. Granulomas and macrophages with foamy cytoplasm have also been reported.
Causes of central bronchiectasis
- ABPA
- Congenital tracheobronchomegaly
- CF
- Williams Campbell syndrome
Causes of upper lobe predominant bronchiectasis
- CF
- TB (NTM can be middle too)
- ABPA (mid and upper)
- Chronic HP (mid, upper)
Causes of lower lobe predominant bronchiectasis
Most common location
- post infectious
- aspiration
- immunodeficiency
- PCD
- with BO post transplant
Pathology lesions characteristic of cryptogenic organizing pneumonia
Excessive proliferation of granulation tissue -> consists of loose collagen-embedded fibroblasts and myofibroblasts involving alveolar ducts + alveoli +/- bronchiolar intraluminal polyps
Treatment of cryptogenic organizing pneumonia
Steroids (IV or PO)
Macrolides
BAL findings in:
1) Sarcoidosis
2) LCH
3) Hypersensitivity pneumonitis
4) Pulmonary hemorrhage
5) Aspiration
1) Lymphocytosis (increased CD4:CD8 ratio)
2) no eosinophils, stain S-100, CD1a, langerin
3) Acute = neutrophilia, >48hrs = lymphocytosis, increased CD8
4) Gross blood, increasing with each sample
5) Lipid laden macrophages
2 pathology features of desquamative interstitial pneumonitis
Foamy alveolar macrophages
Type 2 alveolar cell hyperplasia
Components of the Starling equation
Qf = Kf[(Pc − Pis) − σ(πpl − πis)]
where Qf = net flow of fluid
Kf = capillary filtration coefficient; this describes the permeability characteristics of the membrane to fluids and the surface area of the alveolar-capillary barrier
Pc = capillary hydrostatic pressure
Pis = hydrostatic pressure of the interstitial fluid
σ = reflection coefficient; this describes the ability of the membrane to prevent extravasation of solute particles such as plasma proteins
πpl = colloid osmotic (oncotic) pressure of the plasma
πis = colloid osmotic pressure of the interstitial fluid
Note that the surface area of the alveolar-capillary barrier is included in the Kf.
The Starling equation is very useful in understanding the potential causes of pulmonary edema, even though only the plasma colloid osmotic pressure (πpl) can be measured clinically.
Five PARDS diagnostic criteria.
- Exclude patients with perinatal lung disease
- Within 7 days of known clinical insult
- Respiratory failure not fully explained by cardiac failure or fluid overload
- Chest imaging findings of new infiltrate(s) consistent with acute pulmonary parenchymal disease
- Oxygenation
CPAP ≥ 5, P/F ≤ 300, SpO2/FiO2 ≤ 264
Mild = 4 ≤ OI ≤8
Moderate = 8 ≤ OI ≤ 16
Severe = OI ≥ 16
Pathophysiology of ARDS
Consequence of inflammatory process at alveolar-capillary interface
Increased alveolar capillary permeability à flooding of alveoli with protein rich fluid, leading to:
- Impaired gas exchange
- Impaired surfactant function
Kf in the Starling equation
filtration constant = measure of membrane permeability to water
σ in the Starling equation
reflection coefficient; this describes the ability of the membrane to prevent extravasation of solute particles such as plasma proteins
4 stages of ARDS
- Triggered by direct or indirect injury
- Acute exudative phase with pulmonary edema, cytokine release, activated neutrophils
- Fibroproliferative phase (may lead to fibrosing alveolitis)
- Recovery Stage
3 functional lung units in ARDS
1) fully aerated, normal “baby lung”
2) Poorly aerated (recruitable)
3) Non-aerated (collapsed, atelectatic)
Most common cause of ARDS
Direct (pulmonary) causes
Mechanism of injury for direct (pulmonary) causes of ARDS
Characterized by a primary injury to the alveolar epithelium
Results in intra-alveolar edema, reduced lung compliance, with preservation of chest wall compliance
Common causes for direct (pulmonary) ARDS
Pneumonia (viral, bacterial) Aspiration pneumonia Bronchiolitis Near drowning Lung contusion Toxic inhalation
Common causes for indirect (extra-pulmonary) ARDS
Sepsis
Nonthoracic trauma
Transfusion of blood products
Pancreatitis
Pathology of direct (pulmonary) causes of ARDS
Predominantly consolidation
Differences in mechanism of injury and pathology may explain why pulmonary causes tend to have more refractory hypoxemia, with resistance to recruitment maneuvers and prone posturing, but respond better to surfactant
Mechanism of injury for indirect (extra-pulmonary) causes of ARDS
Primary insult is systemic, with the major injury occurring to the capillary endothelium
Results in interstitial edema, with greater reduction of compliance in the chest wall
Responds better to recruitment
Pathology of indirect (extra-pulmonary) causes of ARDS
Predominantly atelectasis
Characteristics of Pulmonary Capillaritis
Also referred to as alveolar capillaritis, is characterized by neutrophilic infiltration of the alveolar septa (lung interstitium) -> necrosis of these structures, loss of capillary structural integrity and spilling of RBCs into the alveolar space and interstitium
Causes of Pulmonary Capillaritis
The systemic vasculitides Anti-glomerular basement membrane (anti-GBM, Goodpasture’s) disease Rheumatic diseases Certain drugs Idiopathic pulmonary hemosiderosis, Idiopathic pulmonary capillaritis
Idiopathic pulmonary capillaritis, also known as pauci-immune pulmonary capillaritis, is characterized by the histopathologic findings of pulmonary capillaritis, but without clinical or serologic evidence of an associated systemic illness
Characteristics of Isolated Pulmonary Capillaritis
Present with signs and symptoms of alveolar hemorrhage without renal or other systemic manifestation
Diffuse alveolar opacities on imaging
Low hemoglobin
Hemosiderin-laden macrophages on BAL
Normal kidney function
Associated with lower hemoglobin and higher ESR (compared to IPH)
Usually positive ANCA - if not, need biopsy
Characteristics of Pulmonary veno-occlusiove disease and pulmonary capillary hemangiomatosis (PVOD/PCH)
Subtype of group 1 (PAH)
PVOD may represent a common aberrant response to an inciting event of endothelial injury that leads to widespread fibrosis of pulmonary venules
Many patients with PVOD additionally have findings of capillary congestion and lymphadenopathy, but with a normal-sized left atrium and normal-sized major pulmonary veins
The presence of venous congestion and lymphadenopathy with a normal-sized left atrium and normal-sized major pulmonary veins are features that may help distinguish PH due to PVOD from other causes of post-capillary PH, namely that due to left-sided heart disease
These features are likely due to chronic pulmonary capillary hypertension, transudation of fluid into the interstitium, and enlargement of pulmonary lymphatic channels.
Biopsy findings of PVOD/PCH
PVOD is a fibroproliferative disease primarily affecting the small pulmonary veins with relative sparing of the larger veins.
The pathologic hallmark of PVOD is extensive and diffuse occlusion of the pulmonary veins due to smooth muscle hypertrophy and collagen matrix deposition (ie, fibrous tissue).
Characteristic triad of IPH
- Iron deficiency Anemia
- Hemoptysis
- Diffuse infiltrates on imaging
List 3 features of the primary complex in TB. Where does this occur in the lungs?
The triad of: 1) primary focus 2) local tuberculous lymphangitis (basically inflammation of surrounding lymphatic channels) 3) enlarged regional lymph nodes = the primary complex.
Ghon focus = happens when cellular infiltrates continue to the site of infection, the center for the granuloma because caseous (or necrotizing) and you can see a fibrocalcific residua on imaging
Ghon complex = Ghon focus + calcified granulomatous focus in a draining lymph node
Child with CT finding of cavitating nodules diffusely. List four non-infectious etiologies
Inflammatory, such as granulomatous with polyangitis, rheumatoid arthritis, sarcoid
Fat emboli
Malignancy, such as squamous cell carincoma
Langerhans cell histiocytosis
Differential for cavitary lung lesions
CAVITY is the acronym to remember
C = cancer, such as squamous cell carcinoma
A = autoimmune, such as GPA, rheumatoid arthritis (rheumatoid nodules)
V = vascular, such as septic pulmonary emboli or
I = infection, such as bacterial or fungal, such as pulmonary abscess, pulmonary TB, NTAM, aspergillus
T = trauma, such as pneumatoceles
Characteristics of Intralobar Pulmonary Sequestration
- Shares visceral pleura
- More common with infections
- Doesn’t usually have associated anomalies
- Blood supply = Aorta, Venous usually to left atrium but can also be to right side
usu posterior basal LLL. More than 50% diagnosed as adult - often asymptomatic.
- Usually drain into pulmonary system (=shunt)
Characteristics of Extralobar Pulmonary Sequestration
- Own visceral pleura
- Usually detected incidentally on imaging
- More common to have associated anomalies
- Blood supply = Aberrant vessel from Aorta, Venous drainage to right atrium
usu beneath LLL, 15% abdominal. More often detected in neonates - assoc abn
- Usu. drain into azygous
Hydatid lung disease. What are 5 features on CT.
Location: periphery, centre or hilum Pulmonary cyst Regular shaped cyst Thin walled cyst, Water lily sign Meniscus, crescent sign Bronchial displacement Pericentric emphysema Air fluid level
List four non-infectious pulmonary complications of AIDS
Lymphoid interstitial pneumonitis Pulmonary tumours Bronchiolitis obliterans Bronchiectasis Immune reconstitution inflammatory syndrome (IRIS) Airway hyperreactivity/asthma Aspiration pneumonitis Pulmonary HTN (limited data in children) Upper airway disease
Respiratory disease is the most common complication occurring in human immunodeficiency virus (HIV)-infected children.
Acute disease = most likely to be infectious
Alternatives to Septra
Pentamidine
Dapsone
Atovoquone
Stages of lung development
“Every Pulmonologist Can See Alveoli”
1) Embryonic 4-7 wks
2) Pseudoglandular 5-17 wks
3) Canalicular 16-26 wks
4) Saccular 24 wks- term
5) Alveolarization 36 wks to 21 years
Structural events that happen during the Embryonic stage of lung development (3-7 weeks)
Lung buds; trachea, main stem, lobar, and segmental bronchi; trachea and esophagus separate
Structural events that happen during the Pseudoglandular stage of lung development (6–17weeks)
Subsegmental bronchi, terminal bronchioles, and acinar tubules; mucous glands, cartilage, and smooth muscle
Structural events that happen during the Cannalicular stage of lung development (16–26 weeks)
Respiratory bronchioles, acinus formation and vascularization; type I and II AEC differentiation
Structural events that happen during the Saccular stage of lung development (26-36 weeks)
Dilation and subdivision of alveolar saccules, increase of gas-exchange surface area, and surfactant synthesis
Structural events that happen during the Alveolar stage of lung development (36 weeks on)
Further growth and alveolarization of lung; increase of gas-exchange area and maturation of alveolar capillary network; increased surfactant synthesis
Causes of pleural fluid eosinophilia
Drugs Idiopathic Infection (bacteria, fungi, mycobacteria, parasites, virus) Inflammation (acute/chronic eosinophilic pneumonia, Churg-strauss, Rheumatoid effusion) Malignancy (lymphoma) PE Toxicity Trauma
Characteristics in Lymphoid Interstitial Pneumonia (LIP)
in AIDS
Diffuse infiltration of lymphocytes and scattered nodules of mononuclear cells
Unclear etiology → ?lymphoproliferaton with response to the HIV alone or co-infection with another virus
EBV = common co-infection
Insidious course and slowly progressive
Median age = 2.5-3yrs
Dx = lung biopsy
Tx = non-specific → bronchodilators, oxygen, steroids
Some may progress to lymphoproliferative disease with polyclonal, polymorphic B-cell content with extranodal systemic and prominent pulmonary involvement or to malignant lymphoma
What is the interaction concern with some of the CFTR modulators?
Ivacaftor is metabolized by cytochrome CYP3A. (don’t use a strong inducer with Ivacaftor. If it’s a strong inhibitor, you can still use the drug, but you need to do a dose adjustment).
strong cyp3a inducers = Rifampin, Rifabutin, Phenytoin Phenobarb, herbal supplements (St Johns wort) is not recommended for use with Ivacaftor.
strong cyp3a inhibitors -azoles,erythro clarithro telithromycin- needs dose adjustment
seville orange, grapefruit-to avoid
caution when co administering other cyp3a substrates -tacrolimus, cyclosporine ,digoxin
Diurnal Variation
Highest PEF - Lowest PEF/Mean of both
> 13 = significant, take the highest of 3 values for each PEF
PEF lowest in the early morning and highest in the afternoon
Nitric oxide in pulmonary hypertension - mechanism of action?
Inhaled nitric oxide selectively dilates pulmonary vasculature in ventilated areas of the lung.
Mechanism of action:
NO activates soluble guanylyl cyclase (sGC) to produce cyclic guanosine monophosphate (cGMP) leading to decrease vascular smooth muscle tone (ie, vasodilation)
Additional effects of NO include suppression of both smooth muscle proliferation and platelet aggregation
MoA phosphodiesterase inhibitors
Phosphodiesterase inhibitors (eg. Sildenafil): Prevent degradation of cyclic GMP → potentiates the effect of NO activity by inhibiting PDE 5
MoA Endothelin Receptor Anatagonists
Endothelin Receptor Antagonists (eg. Bosentan-dual receptor): Block ET receptors (found in smooth muscle and endothelial cells) thus promoting vasodilation and prevents proliferation
MoA Prostacyclin Analogs
Prostacyclin Analogs/receptor agonists (eg. Epoprostenol, Iloprost, Trepostinil): Acts as a pulmonary vasodilator, inhibits vascular smooth muscle proliferation, inhibits platelet aggregation, improves endothelial dysfunction and can also act as a possible cardiac inotrope
3 criteria in the cath lab for pulmonary HTN diagnosis
Mean Pulmonary arterial pressure at rest ≥ 20 mmHg
Pulmonary artery wedge pressure (<15mmHg)
Elevated Pulmonary Vascular Resistance > 3 woods units x m2
Stain for TB (acid fast bacilli)
Ziehl-Neelsen
Stain for hemosiderin laden macrophages
Prussian blue
Stain for NEHI
Bombesin staining
Stain for surfactant
Periodic Acid Shiff stain
IGRA or TST - better sensitivity?
TST
IGRA of TST - better specificity?
IGRA
Flow loop characteristics for Spirometry
Rapid increase
Sharp peak
Linear curve
Inspiratory loop
FRC (gas dilution)
Cinitial x Vsystem = Cfinal (Vsystem + FRC)
MoA for Azithromycin for decreasing bacterial virulence
1) Inhibits pseudomonas growth, protein synthesis and biofilm formation
2) Decreased bacterial virulence factors for P
3) Most benefit in those + with P
Key points ALPINE trial for Pseudomonas eradication in CF
“Aztreonam Lysine for Pseudomonas Infection Eradication”: 28 days of AZLI treatment in pediatric patients 3 months to b18 years of age is both effective and well tolerated in the treatment of early Pa pulmonary infection associated with CF
Results from ALPINE, ELITE, and EPIC support a short treatment course (28 days) as effective for initial eradication of Pa for most CF patients.
Key points re: ELITE trial for Pseudomonas eradication in CF
28 days of inhaled tobramycin was as beneficial as 56 days in treating first of early pseudomonas
Key points re: EPIC trial for Pseudomonas eradication in CF
EPIC: Inhaled tobramycin 300mg BID for 28 days and oral ciprofloxacin 14 days –>addition of cirpo didn’t make a difference to rate of eradication
Lung malformations in Pseudoglandular phase
Tracheomalacia and bronchomalacia
Intralobar bronchopulmonary sequestration
CPAM
Acinar aplasia or dysplasia
ACD-MPV
Congenital pulmonary lymphangiectasia, and other pulmonary vascular malformations
Hypoplasia with CDH
Lung malformations in Canalicular phase
Congenital alveolar dysplasia
Alveolar capillary dysplasia
Pulmonary hypoplasia
Lung malformations in Canalicular phase
Congenital alveolar dysplasia
Alveolar capillary dysplasia
Pulmonary hypoplasia
Clinical definition PBB
- Chronic wet cough >4 weeks
– Absence of symptoms or signs (i.e. specific cough pointers) of other causes of wet or productive cough
– Resolution of cough within 2 weeks of an appropriate oral antibiotic (usually amoxicillin-clavulanate)
In a minority of children 4 weeks of antibiotics are required
RSV prophylaxis
1) Children with hemodynamically significant CHD or CLD (defined as a need for oxygen at 36 weeks’ GA) who require ongoing diuretics, bronchodilators, steroids or supplemental oxygen, should receive palivizumab if they are <12 months of age at the start of RSV season.
2) In preterm infants without CLD born before 30 + 0 weeks’ GA who are <6 months of age at the start of RSV season, it is reasonable (but not essential) to offer palivizumab.
3) Infants in remote communities who would require air transportation for hospitalization born before 36 + 0 weeks’ GA and <6 months of age at the start of RSV season should be offered palivizumab.
