Non-infectious disorders (Raf) Flashcards
Diagnostic criteria for PARDS?
- Age: exclude patients with perinatal lung disease
- Timing: within 7 days of known clinical insult
- Origin of edema: respiratory failure not fully explained by cardiac failure or fluid overload
- Imaging: new infiltrate on chest imaging, consistent with acute parenchymal lung disease
- Oxygenation: on at least CPAP+5, with PaO2/FIO2 ratio of <=300 or SpO2/FiO2<=264
Stratify severity of PARDS?
OI of 8-16 is moderate (100-200)
OI <8 is mild (201-300)
OI >=16 is severe (<=100)
Recommendation for ARDS management based on ARDS net?
- tidal volume of 4-8 mL/kg (lower than prior tidal volumes of 10-15 mL/kg)
- low plateau pressure of <=30 cm H2O (lower than previous standard of 50 cm H2)
Other recommendations for management:
- high PEEP
- minimize fiO2
- prone positioning –>this is the lowest priority recommendation, only for patients with refractory hypoxemia
- don’t forget to treat the underlying cause, eg. pneumonia
Pros/cons of heliox?
- Decreases airway resistance since flow is changed from turbulent flow to laminar flow
- Decreases work of breathing (since helium is less dense so less pressure gradient is required to drive flow)decreased work of breathing and patient is less likely to fatigue
- NOT curative
- NOT routinely recommended for asthma management in ED
- For the patient with respiratory distress who is tiring, it’s a bridge to more definitive management
- Disadvantage: less effective when helium is <70%, so not going to be helpful for patient needing significant amount of oxygen (since can only deliver about 20% oxygen)
- Conditions used for: croup, post-extubation stridor, asthma
Post HSCT, what are the different phases you think of to approach complications?
- Pre-engraftment: day 0-30 (early)
- Post-engraftment: day 30-100 (late)
- Late phase: >100
Post HSCT patient with respiratory symptoms within first 30 days, what is DDx?
Infectious complications:
- Bacteria: pseudomonas, G-, G+
- Fungi: Candida
- Viral: minimal
Non-infectious complications:
- pulmonary edema
- mucositis
- peri-engraftment respiratory distress syndrome
- diffuse alveolar hemorrhage, which is associated for bone marrow recovery
- idiopathic pneumonia syndrome
- veno-occlusive disease, which can be pulmonary or hepatic
What types of infections are seen in late (30-100 days) post transplant?
- Bacteria: staph aureus
- Fungi: PJP, Aspergillus
- Viral/protozoal (quite a few viral infections associated with this stage): EBV, CMV, VZV, adenovirus, Toxoplasma
- this is the phase where you seem to be at risk for most number of bugs
What types of infections are seen in the late phase (>100 days) post engraftment?
- Bacteria: H. influenza, Strep pneumo
- No predisposition for fungal infections
- Viral/protozoal: VZV
(don’t seem to be at risk for that many unusual bugs)
(It seems like just at risk for non-opportunistic organisms
What are late complications (>6 months) post lung transplant ?
- PTLD
- Chronic lung allograft dysfunction
If suspecting PTLD, what imaging investigation to order?
PET scan
What are late non-infectious complications post HSCT?
- these late complications start as early as 2- 3 months post transplant
- Bronchiolitis obliterans, which is often part of chronic GVHD
- ILD - often due to drugs or radiation
- Cryptogenic organizing pneumonia
- PTLD
Patient who is 12 months post HSCT, who has SOB and wheeze, top of differential?
- Bronchiolitis obliterans
- Investigations:
- PFT: non-reversible airflow obstruction
- CT: inspiratory and expiratory –>mosaic attenuation
- Treatment:
- Increased immunosuppression: calcineurin inhibitors, azathioprine, steroids
- Azithro + LTRA + ICS/LABA (FAM)
Patient who is >3 months post HSCT with dyspnea and PFT showing restriction and decreased DLCO?
- ILD
- COP
Gold standard for diagnosis of PE?
CT chest- pulmonary angiography
Risk factors for PE
- Oral contraceptive use
- Pregnancy
- Drugs - heavy cigarette smoking (>=20 packyears, probably difficult to achieve this level for a teenager), IV drug use (in particular injection into femoral veins, which casues direct trauma, irritation, infection), glucocorticoids, antidepressants
- Obesity
- Antiphospholipid antibody syndrome
General risk factors:
- Inherited thrombophilia: factor V Leidein, Prothrombin gene mutation, protein C or S deficiency, antithrombin deficiency
- Acquired:
- Malignancy
- Recent surgery, particularly orthopedic, major vascular, neurosurgery, and cancer surgery
- Major trauma
List 5 categories and provide 1 example of each for pulmonary hypertension
- Pulmonary arterial hypertension:
- idiopathic
- genetic
- drug and toxin
- associated with: HIV, portal hypertension, congenital heart disease, connective tissue disease
- PAH with overt signs of PVOD/PCH (pulmonary capillary hemangiomatosis)
- PPNH - PH due to left heart disease - left ventricular systolic dysfunction, diastolic dysfunction, valvular disease, congenital left sided cardiac disease
- Due to lung disease and/or hypoxia: ILD, COPD, any lung disease with restrictive/obstructive component, hypoxia, sleep disordered breathing, developmental lung disorders
- Due to pulmonary artery obstruction - CTEPH and other types of obstruction
- Unclear mechanisms:
- Hematologic - eg. chronic hemolytic anemia
- Systemic disorders - eg. sarcoid
- Metabolic - glycogen storage, gaucher, thyroid
Definition of pulmonary hypertension
mPAP >20 mmHg in child >3 months of age at sea level
Definition of pulmonary arterial hypertension
mPAP>20 in a child >3 months of age at sea level
pAWP or LVEDP <15 mmHg
Pvri >= 3 WU
categories or drugs and an example for each that are used for treatment of PH
NO pathway - eg. exogenous nitric oxide or phosphodiesterase type 5 inhibitor, like sildnafil
Endothelin pathway - eg. bosentan, endothelin receptor antagonist
Prostacyclin pathway - eg. prostacylin analogue like epoprostenol
Medications that can be used for sialorrhea and side effects?
Anticholinergic - glycopyrrlate, scopolamine, atropine. S/E: photophobia (blind as a bad),, behavioural change (mad as a hatter), flushing (red as a beet), full as a flask (urinary retention, constipation), difficulty with temeperature regulation (hot as a hare), thickening of secretions can lead to life threatening obstructions. Need to be very careful with use of these medications in patients with neuromuscular disease or small tracheostomy tube.
Botulinum toxin: pain, dry mouth, speech impairment, difficulty swallowing (basically if the dose is too high, then you get effects on surrounding tissues).
In general, the anticholinergics are NOT well tolerated due to side effects. Botulinum toxin seems to be better tolerated if appropraite dose.
1 example of benign and malignant mediastinal mass in each compartment
- Anterior mediastinum: thyroid goitre for benign, lymphoma for malignant
- Middle: lymph node enlargement from sarcoid (benign), bronchogenic cyst (benign), lymphoma (malignant)
- Posterior: neuroblastoma (malignant), meningocele
Light’s criteria for pleural effusion
Pleural fluid protein/serum protein >0.5
Pleural fluid LDH/serum LDH >0.6
Pleural fluid LDH >2/3 upper limit of normal
If one of these crtieria is met then the fluid is an exudate
What are the 4 clinical features considered in the PCD diagnosis algorithm?
- Year-round daily productive wet cough starting at <6 months
- Year round, daily, non-seasonal rhinosinusitis beginning at <6 months
- Above 2 are often present often immediately after birth
- Neonatal respiratory distress in term infant (needing oxygen or PPV x >24 hours with no clear explanation)
- Organ laterality defect
If 2 out of 4 present, then sensitivity and specifity for diagnosis of PCD is about 75%. (still not the most sensitive criteria) - Term newborn with unexplained respiratory distress and organ laterality is very likely to have PCD, even if they have not yet developed nasal congestion and cough.
How many genes are linked to PCD and how many are included in an extended genetic panel?
39 genes are linked to PCD
An extended genetic panel should test for >12 genes
What is a low nasal nitric oxide?
<77 nL/min
What is the first choice diagnostic test for PCD and what are the caveats?
- First choice is nasal nitric oxide since it is reliably low in PCD. If it’s normal then you can be pretty sure that PCD is excluded
- In contrast, for genetics–>even if no variant is found, you still go on to do electron microscopy. Even if electron microscopy is normal, PCD is still possible b/c 30% have normal EM.
- In a child >5 years of age, who has had CF excluded (negative sweat chloride), then nasal NO is the preferred first test. Since viral illness and sinusitis can also cause low nNO, the test has to be repeated on 2 separate occasions, at least 2 weeks apart
In a patient suspected of PCD, if nasal NO is low x 2, do you do any further investigations?
Genetic investigation as a next step +/- TEM to provide prognostic information
If child is <5 years of age, non-cooperative or no access to nNO, how do you proceed with PCD work up?
Extended genetic testing panel, which includes at least 12 genes
If PCD genetics are not positive for diagnosis of PCD (eg. 1 pathogenic variant, no pathogenic variants, variants of uncertain clinical significance), do you do any further investigations?
Yes, do TEM
Respiratory complications of Hunter syndrome?
Hunter’s syndrome is type II mucopolysaccharidosis, which is a type of lysosomal storage disorder
- Deposition of glycosaminoglycans in throat and trachea–>airway obstruction, OSA (they frequently need CPAP)
- Tracheobronchomalacia from deposition of GAG in tracheal/bronchial cartilage –>this can affect airway clearance and cause recurrent infection infection,
- Restrictive lung disease due abnormal shape of ribs, scoliosis, pectus carinatum, enlargement of abdominal organs which affects diaphragm excursion
- Recurrent pneumonia due to above mechanisms
why do lymphatic disorders cause chylothorax and protein losing enteropathy?
Lymphatic fluid leaks into pleural space.
Lymphatic fluid leaks into intestinal lumen–>protein losing enteropathy
(Chyle is lymphatic fluid that contains chylomicrons since it is coming from intestinal lymphatics)
In a patient with a lymphatic malformation, when should you suspect a complex lymphatic abnormality?
- Lytic bone lesions
- Presence of nonmalignant chylous effusion (pleural, pericardial, peritoneal)
- Evidence of protein losing enteropathy, with no other proven ethology
Examples of complex lymphatic abnormalities and distinguishing features?
Generalized lymphatic abnormality: chylothorax, lytic bone lesion
Gorham stout: lytic bone lesion, chylothorax
Kaposiform lymphangiomatosis: soft tissue mass, consumptive coagulopathy, thrombocytopenia
Long term complications of TEF?
- Esophageal stricture and abnormal motility–>reflux and aspiration
- Tracheomalacia
- Recurrent pneumonia
- Recurrent TEF
- Bronchospasm
