RESPIROLOGY M Flashcards
1
Q
A baby born vaginally to a poor controlled gestatioal diabetic mom had cyanosis, irregular work of breathing and decreased breath sounds on the right side. There is also decreased tone in the right arm. A CXR is normal. What is your next best test to figure out diagnosis?
A
Chest ultrasound to rule out phrenic nerve paralysis
2
Q
A patient presents with chronic cough and shortness of breath. After extensive investigations, you decide to do a bronchoscopy. On sputum sample, you see siderophages. What is your differential diagnosis? (6)
A
Hemosiderosis! -siderophages = macrophages with iron inside Differential diagnosis:
-
LUNG:
a. Heiner’s syndrome: CMPA with pulmonary hemorrhage
b. Idiopathic pulmonary hemosiderosis
c. Pulmonary capillary hemangiomatosis (proliferation of capillaries with alveolar hemorrhage) -
Vasculitis:
a. . Goodpasture syndrome
b. Wegner’s granulomatosis
3
Q
A patient with a pleural effusion receives a chest tube for drainage. The pleural fluid is chocolate colored. What is your diagnosis?
A
Amebiasis (entamoeba histolytica)
4
Q
A 10 yo girl with asthma is prescribed fluticasone 50 mcg 1 puff BID. She has no night time symptoms, has daytime symptoms of cough/wheezing 4x/wk while playing hockey.
Most appropriate next step?
a. Take salbutamol before exercise
b. Increase fluticasone to 125 mcg 1 puff BID
c. add montelukast
d. add LABA
A
B! This is a child 6-11 yo with poorly controlled asthma since she is needing beta agonist > 4x/wk! -she is currently on low dose ICS THUS next step is to increase to medium dose ICS!
5
Q
At what age should you start using the yellow aerochamber? Blue?
A
Yellow = pediatric = 1-5 yo
Blue = adult = >5 yo
6
Q
Asthma What are the stepwise approach for asthma control according to Canadian Asthma Guidelines < 5 yo?
A
for children < 5 yo:
a. start with low dose ICS
b. then EITHER increase to medium ICS
OR add LTRA
c. then use both
7
Q
What is the general management plan for asthma?
A
- Confirm diagnosis
-
Management plan:
a. Environmental controls- smoking, second hand smoke, allergens,
b. education and written action plan
c. need to improve adherence - Fast-acting bronchodilators prn
- Inhaled corticosteroids: (-low dose: 6-11 yo: 12 yo: 12 yo: 251-500 mcg/d) -
8
Q
What are risk factors for persistent asthma into adulthood? (5)
A
- Maternal history of asthma
- Asthma onset prior to 3 years of age
- Eosinophilia
- Elevated IgE levels
- Allergic rhinitis
9
Q
What are the 2 main types of childhood asthma?
A
- Recurrent wheezing in early childhood (ie. transient early wheezing): triggered by viruses, tends to resolve during preschool years without increased risk for asthma in later life
- Chronic asthma (persistent atopy-associated asthma): associated with allergy (usually persists into later childhood and often adulthood)
10
Q
What are the criteria for determining if a patient’s asthma is well-controlled? (7)
A
- Daytime symptoms: < 4 days/wk
- Need for salbutamol < 4 doses per week (INCLUDES the need for use prior to exercise)
- Night time symptoms: < 1 night/wk
- Physical activity: normal
- Exacerbations: mild, infrqeuent (ie. 2 or less steroid courses per year)
- No absence from work or school due to asthma
- FEV1 or PEF > 90% personal best
11
Q
What are the early childhood risk factors for persistent asthma? (10)
A
- Parental asthma
- Atopy
- Reduced lung function at birth
- Possible use of acetaminophen
- Exposure to chlorinated swimming pools
- Severe lower resp tract infection (pneumonia, bronchiolitis requiring hospitalization)
- Male gender
- Wheezing apart from colds
- Environmental tobacco smoke exposure
- Low birthweight
12
Q
Asthma What are the PFT features to Dx asthma?
A
- reversible airway obstruction
- peak expiratory flow (PEF) variability
- positive challenge test such as methacholine or exercise challenge.
13
Q
Asthma What are the stepwise approach for asthma control according to Canadian Asthma Guidelines > 12 yrs ?-
A
for 12 and older:
a. start with low dose ICS
b. then ADD LABA
c. THEN increase ICV to medium dose
OR add a LTRA
d. THEN use medium dose ICS + LTRA + LABA
14
Q
Asthma What are the stepwise approach for asthma control according to Canadian Asthma Guidelines 6-11: ?
A
for age 6-11:
a. start with low dose ICS
b. Then increase to medium dose ICS
c. Then add a LABA (for child with more exercise s/s)
OR LTRA (for more atopic child)
d. Then use medium dose ICS + LABA + LTRA -
15
Q
What are the two most common adverse effects of inhaled corticosteroids?
A
- Thrush
- Hoarse voice (dysphonia)
16
Q
What is the second line monotherapy for mild asthma?
A
Leukotriene receptor antagonists! (Montelukast)
17
Q
Asthma What is the Asthma PRAM score?
A
18
Q
A 16 yo patient with CF complains of chest pain and a cough that produced a rust-colored sputum. What is the most likely diagnosis?
A
ABPA
19
Q
A child presents to your clinic with a cough that has persisted for > 6 weeks. What is your next step in management?
A
Test for cystic fibrosis regardless of race or ethnicity
20
Q
How do you make a Dx of CF?
A
21
Q
CF How can CF present in infancy? (5)
A
(1) salt depletion syndrome, which results in a hyponatremic, hypokale- mic, and hypochloremic metabolic alkalosis;
(2) prolonged neonatal jaundice, resulting from intrahepatic biliary stasis or extrahepatic bile duct obstruction;
(3) edema, hypoproteinemia, and acrodermatitis enteropathica, resulting from malabsorption
(4) hemorrhagic disease of the newborn secondary to vitamin K deficiency
5. Meconium ileus bowel obstruction
22
Q
CF How do describe Pneumothorax in CF?
A
small (<5 cm)
large (5 cm)
23
Q
CF How do manage Pneumothorax in CF?
A
Small
- observation or small catheter
- continue measures for airway clearance NOT CPAP
Large -chest tube, Chemical or surgical pleurodesis
24
Q
CF How do you Dx Pneumothorax in CF?
A
chest radiography +/- CT chest
25
CF How do you grade hemoptysis in CF?
- **scant** (\<5 mL),
- **moderate** (5–240 mL)
- **massive** (\>240 mL)
26
CF How do you manage Distal Intestinal Obstruction Syndrome? (DIOS)
1. NGT if needed and electrolyte management
2. balanced intestinal lavage (such as GoLYTELY),
3. Pancreatic enzymes, hydration and dietary fibre
4. **Enema** if neded- bilious vomiting or other evidence of complete intestinal obstruction, or for those who do not respond to the above measures, a hyperosmolar gastrografin enema
5. **Surgery** if needed
27
CF How do you manage meconium ileus?
1. NGT, electrolyte replacement and iv fluids
2. **Gastrographin** enemas 20-40% success
3. **Surgery** if needed- simple enterotomy with lavage, double enterostomy, and/or resection of dilated, perforated, or atretic bowel, with diversion ileostomy
28
CF How do you manage the Gastrointestinal problems?
1. **Nutrition** is incredibly important – poor nutrition, means poor outcomes
•HIGH caloric requirements àbring on poutine
2. **Enzymes** are mainstay
•2500 Lipase Units/kg/meal (above 2,500 = fibrosing colonopathy)
3. **Fat soluble vitamin** replacement (ADEK)
4. **Liver**/GB stasis with _ursodiol_ (only 1% get **cirrrhosis and portal HTN**...Rx portal ven shunting and liver transplant
5. **pH of intestinal contents** +/- PPI
29
CF How do you manage the Sinopulmonary problems?
**1. Secretion/mucous mobilization**
•_Physio_+/- _mucolytics_ (aerosolised DNAse) and 7% hypertonic saline
**2. Aggressive treatment of pulmonary exacerbation**
a. _Physio_,
b. _acute antibiotics (inhaled Tobramycin)_
_c. long term_ antibiotics (Ie. **Tobramycin** and **aztreonam** nebs for Pseudo .Aerig.) and oral azithromycin 3 times a week
d. monitoring pulmonary flora (sputum/thraot culture)
**3. NUTRITION**
**4. Treat underlying resp diseases** (Ie. Asthma, nasal congestion, polyps)
30
CF How do you manage hemoptysis in CF?
1. **SCANT** **& MODERATE**- Abx, stop NSAID, stop clearing mucous with DNAse and physio
**2. MASSIVE-**
a. **ABC**- stabilization of cardiorespiratory status
b. **Limit the use of:** NSAIDS & airway clearance measures -
c. **Bronchial artery embolization i**s considered
31
How does the CFTR protein work?
**Explanation:**
CFTR protein = Cystic Fibrosis Transmembrane regulator
chloride channel on surface of epithelial cells responsible for movement of salt and water across cell membranes (airways, liver and pancreas)-essentially have overactive sodium pumps and impermeable chloride channels = thus, Na can get out of tubule structures and draw water out with it, leaving thick secretions left behind
32
CF How does the PFT pattern change with age in CF?
**PFTs**
–**Early disease** - peripheral airway disease - results in **airway obstruction,** gastrapping
–**Late disease** - chronic inflammation- ↑lung destruction and fibrosis- **restrictive pattern** with persistent gas trapping
33
CF How does the Prognosis differ between PCD and CF?
**IN PCD:**
1. -slower decline in PFT cf cystic fibrosis
2. - prognosis and long-term survival are better
3. -most patients - normal or near normal life span,
34
CF How does Ivacaftor work?
- oral p**harmacologic potentiator** that **activates defective CFTR** at the cell surface caused by the **class 3 gene mutation G551D.**
35
CF How is newborn screning performed?
- 2 IRT measurements or CFTR mutation testing if the IRT level is elevated.
- P**ositive screening** results indicate that **IRT levels remain persistently elevated** by the time the neonate is **ages 7 to 14 days** or that **at least one CFTR mutation** has been identified
36
What are 5 causes of a **false positive sweat** chloride test?
1. **Endocrine**:
- adrenal insufficiency (Addisons)
- hypothyroidism
3. **Skin**:
- Ectodermal dysplasia
- Atopic dermatitis
3. . **Age** \< 24 hrs of life
37
What are complications of Cystic Fibrosis by system?
1. **ENT**: Nasal polyps, Chronic sinusitis
2. **Resp**;
a. Clubbing, Chronic cough, (9%) Bronchiectasis & hemoptysis
b. **Lung**: Asthma, pulmonary hypertension (**Cor pulmonale** ), pneumothorax (3%)
c. **Chronic colonization** with staph, hemophilus, pseudomonas, burkholderia seppacia
3. **GIT**:
a. **infancy**- Meconium ileus (10%) & FTT
b. **Older kids:**
**-GERD**
**-Pancreatic insufficiency** (90%) - d F508 mutation
i) Fat soluble vitamin deficiency, steatorrhea, and
ii) protein malabsorbtion - hypoproteinemia and edema
- **Liver cirrhosis** - from thickened bile
- **Rectal prolapse** - from chronic diarrhea
- M**alnutrition** and weak musculature
4. **Endo**: eventual diabetes (glucose intolerance and eventally CF related DM)
5. **Genitourinary**:
- **Infertility** in both males and females
**Males** - -Congenital _absence of vas deferens_ in males and _azoospermia_ (nearly universal)
**Females**, thick cervical _secretions_ or secondary _amenorrhea_ from malnutrition (low BMI) and chronic illness
38
What are poor prognostic indicators in CF? (3)
1. Poor nutritional status
2. **Resp**: Pneumothorax , poor FEV1
3. **Infective**: Burkholderia cepacia (very deadly bug in CF)
39
CF what are Sx of sinusitis and nasal polyposis?
- **headache**, facial **pressure**, and
- **nasal obstruction** -broad nasal bridge and septal deformation.
40
What are the 3 most important interventions in CF that affects prognosis?
1. Nutrition
2. Chest physiotherapy
3. Antibiotics
41
CF What are the asocviations of CFRD?
| (CF related DM)
- more common in individuals with **pancreatic insufficiency**
- associated with a **higher mortality rate**
- early Dx and aggressive Gluc control may **prevent** this increase in mortality.
42
What are the causes of a False positive
sweat test in CysticFibrosis – groups
43
CF What are the causes of hemoptysis in CF?
1. **Infection**
2. **Coagulopthy**: fat malabsorbtion (**Vit K deficienc**y), liver disease and and hypersplenism,
3. **proliferation and hypertrophy of the bronchial arteries,**
44
CF What are the consequences of Vitamin A deficiency?
**ocular consequences**
-night blindness and conjunctival and corneal xerosis.
**Skin involvement -** follicular hyperkeratosis
45
CF What are the consequences of Vitamin D deficiency?
-nutritional rickets, osteopenia, and osteoporosis, pathologic fractures.
46
CF What are the consequences of Vitamin E deficiency?
1. peripheral **neuropathy**,
2. **myopathy**,
3. and **hemolysis**.
47
CF What are the consequences of Vitamin K deficiency?
-**coagulopathy** and can contribute to **bone** disease in CF.
48
CF What are the Cplxns of CFRD?
1. **Microvascular**, such as retinopathy, microalbuminuria, and autonomic neuropathy
2. Ketoacidosis is **uncommon** with CFRD.
49
What are the signs of a pulmonary exacerbation in CF?
1. Increased cough/sputum & deterioration in PFT's
2. Hemoptysis
3. Anorexia and weight loss
N.B. Fever is **NOT** a typical symptom of pulmonary exacerbations
50
CF What are the Sx of Pneumothorax in CF
chest pain and dyspnea.
51
CF What are the vitamin deficiencies in CF?
ADEK
**Replacement** of these vitamins begins at **diagnosis**
- **monitoring** of serum levels is performed **annually**.
52
CF What are the benefits of newborn screening
- early diagnosis
- slowing of lung disease progression
- prevention of malnutrition
- provision of psychosocial and extended medical support, such as genetic counseling, for individuals with CF and their families.
53
CF What is ABPA and what causes it?
A hypersensitivity reatcion to Aspergillus in CF airways
54
CF What is ABPA?
hypersensitivity reaction to aspergillus in lungs -occurs most commonly in CF pts, NOT responsive to antibiotics -
55
What is considered a positive sweat chloride test?
(positive, negative, inconclusive)
CF test **positive** if \> 60 mEQ/L
-**negative** if \<40 mEQ/L -40-60:
**inconclusive**, need gene testing
\*\* 99% Px have a +ve sweat test
56
What is considered delayed passage of meconium?
\>48 hrs after birth with no passage
57
What is the inheritance pattern of cystic fibrosis?
Autosomal recessive
58
CF What is the management of CFRD?
**Screening:**
1. annual with OGT \> 9 years. (HBA1C not effective)
**Management:**
1. Insulin (not oral hypoglycemic agents) .
2. Nutritional increase in caloric intake
3. avoidance of foods that are high in simple sugar
59
CF what is the management Sx of sinusitis and nasal polyposis?
1. nasal irrigation and topical steroids.
2. **Severe**: Functional endoscopic sinus surgery is the gold standard procedure to improve sinus drainage and remove inflamed and diseased mucosa.
60
What is the pattern of **colonization** in CF patients?
- by age group-
**Babies**
**Toddlers**
**School age children**
**Teenagers**
**Babies** - staph aureus
**Toddlers** - hemophilus b influenza
**School age children** - pseudomonas
**Teenagers**
- Burkholderia seppacia
- stenotrophomonas maltophilia
61
What is the role of azithromycin therapy in the treatment of CF?
Azithromycin = shown to reduce lung inflammation if given 3x weekly, thus decreasing pulmonary exacerbations
62
CF What is the Rx for ABPA?
1. 1. Systemic steroids - prednisone
2. Itraconazole
63
CF What it called when CFTR abnormality detected at at birth that does not immediately produce clinical manifestations?
Syndrome known as **CFTR-related metabolic syndrome.**
64
CF What problems does ABPA cause?
Airways infmation and obstruction and aggravates CF lung disease
65
CF When does CFRD present?
1. After \> 10 yrs
2. annual incidence increases
- 5% per year \> 10 yrs10% per year \> 20 years
66
CF When is Genetic analysis useful in CF?
helpful to confirm diagnosis of CF, particularly for cases that present with **indeterminate** sweat chloride measurements.
67
CF When should you consider CFLD?
at least 2 of the following:
(1) abnormal **physical examination findings** (hepatomegaly and/or splenomegaly);
(2) abnormalities of **liver function** test results above the reference range of normal on at least 3 consecutive determinations during a 12-month period;
(3) **ultrasonographic** evidence of abnormal liver echotexture or portal hypertension; and
(4) confirmation of cirrhosis by **tissue biopsy.**
68
Which chromosome has the gene for cystic fibrosis?
-most common mutation?
Chromosome 7 (encodes CFTR protein)
most common mutation deltaF508 mutation
69
CF Answer - Genes don’t really tell you prognosis….
70
CF How do you Dx ABPA? Dx and Rx
**Diagnosis**:
1. **Blood**: PERIPHERAL EOSINOPHILIA, Raised IGE,
2. **SPUTUM**
a. sputum culture with branching hyphae
b. RUST-COLORED SPUTUM,
3. Imaging:
a. CXR- **proximal** bronchiectasis on
b CT chest
4. **PFT's r**educed FeV1, and maybe mixed obstructive and restrictive pattern.
**Treatment**: steroids and anti-fungals (voriconazole) x 6 wks
71
CF Question – value of gene testing?
72
CF What are the causes FP in CF
Endocrine?
73
CF What are the causes FP
sweat test in CF – Other
74
CF What are the causes of a False positive
sweat test in Cystic Fibrosis – groups?
75
Cough What is initial work up?
guided by clues in history and physical; if no clues, then start with:
1. CXR
2. PFTs if possible
3. Sweat chloride
76
What is the differential diagnosis for chronic cough? (4)
Chronic cough
\> 3 wks:
1. **CNS**: Psychogenic cough
2. **Resp**:
a. -_Asthma_
b. _Allergy_/irritants: tobacco smoke, pollution, etc.
c. _Cystic fibrosis_
d. Recurrent _infections_
e. _Immunodeficiency_: IgA deficiency, hypogammaglobulinemia Primary ciliary dyskinesia (Kartagener syndrome, immotile cilia syndrome)
3. **GERD**
4. **Anatomic** lesions: foreign body, mediastinal tumor, congenital heart disease, TEF
77
Causes of **transudative** pleural effusions? (3)
1. **Organs:** Cirrhosis, CHF, Nephrotic syndrome
2. **Malignancies**
3. **Infections** (viral, Klebsiella, GAS, etc.)
78
How do you classify size of pleural effusions on CXR?
\<1/3 height of hemithorax = small
1/3 - 1/2 height of hemithorax = moderate
\> 1/2 height if hemithorax = large
79
What are the main differences between exudative and transudative effusions? **(ie. modified lights criteria)**
**Exudative**:
1. -**pH** \< 7.3 -
2. **glucose** \< 50% of serum -
3. pleural:serum **protein** \> 0.5 -
4. pleural:serum **LDH** \> 0.6 -
5. high polymorphonuclear leukocytes
**Transudative**: -
1. pH \> 7.3 -
2. glucose \> 50% of serum -
3. pleural:serum protein \< 0.5 -
4. pleural:serum LDH \< 0.6 -
5. low WBCs
80
How can you differentiate between epiglottitis vs. croup vs. bacterial tracheitis?
\*\*\*All have inspiratory stridor -
**Epiglottitis:**
Toxic, drooling, tripod position,
**_Cough_** no cough, high fever
**Croup:**
non toxic, no drooling appearance, responds to epi,
**_cough_**: hoarse barking cough
**bacterial tracheitis**: (secondary complication of croup)
toxic appearance, no drooling or dysphagia, NO response to epi,
**_Cough_** prominent brassy cough, high fever
81
How do you diagnose TEF? Name 2 tests.
1. _UGI series_ to start
2. rigid _bronchoscopy_ is **gold standard**
82
How does the presentation of alpha-1-antitrypsin deficiency differ between **children** and **adults**?
In **children**:
minimal pulmonary disease ..causes liver disease
**In adults:** early-onset pulmonary emphysema in 30s -
**Mecanism**: alpha-1-antitrypsin is a protein that _deactivates proteolytic enzymes_ released from **dead bacteria** or **leukocytes in the lung:** without this protein, these enyzmes destroy the **pulmonary tissue and cause emphysema**
83
In a child with congenital central hypoventilation syndrome presenting with constipation, what condition should be ruled out?
Hirschsprung disease: 20% of CCHS children -should undergo rectal biopsy to screen for absence of ganglion cells
84
In children with prolonged or high inhaled corticosteroid therapy, what are two things you should monitor for? -does the use of inhaled corticosteroids affect adult height?
1. Height velocity 2. Cataracts \*\*\*Does not affect adult height
85
In which infection does CXR look worse than clinical picture?
Mycoplasma chlamydia and mycoplasma pneumoniae
86
What are the long term complications of obstructive sleep apnea?
1. **Metabolic**: _insulin_ resistance, _dyslipidemia_
2. **Cardiovascular** disease + HTN
3. P**ulmonary hypertension** with cor pulmonale
4. **Neurocognitive** disturbances
87
What is the differential diagnosis for obstructive sleep apnea? (3)
1. **Head**:
a. Tonsilly/adenoid hypertrophy (most common)
b. Craniofacial anomalies
2. **Obesity**
3. **Neuro**:
a. _Neuromuscular_ disorders (ie. muscular dystrophy)
b. _Neurologic_ disorders involving incoordination of upper airway musculature
88
PCD What are clinical features of Kartagener syndrome?
1. Situs inversus (totalis)
2. chronic sinusitis
3. bronchiectasis
89
What are clinical features of PCD (4) -diagnosis? -treatment?
Primary ciliary dyskinesia!
1. **ENT**: Chronic sinusitis + otitis media, nasal polyps, hearing loss
2. **Cardiac**: Situs inversus in 50% of patients (25 % of all Px with situs inversus totalis have PCD)
3. **Resp**: Bronchitis and bronchiectasis, Pneumonia, Clubbing
4. **GUT**: Male and female infertility/decreased fertility -abnormal **sperm movement** -increased **ectopic pregnancy**
90
PCD What are the genetics of PCD?
Autosomal recessive patterns of inheritance,
though rare cases of autosomal dominant and X-linked inheritance have been reported.
The calculated frequency of PCD ranges from 1/12,000 to 1/20,000 live births,
91
PCD What bacteria colonise Px?
- Staphylococcus aureus
- Haemophilus influenzae
- Streptococcus pneumoniae
- Pseudomonas aeruginosa
92
What is the CLASSIC clinical feature on history for primary ciliary dyskinesia?
YEAR ROUND DAILY W**ET COUGH** AND **NASAL CONGESTION**!!!!
93
PCD What is the Inv work up of PCD?
**Diagnosis**:
1. CXR = dextrocardia, overinflation, lobar atelectasis.
2. CT scan: bronchiectasis
3. PFT: progressive intrathoracic airway obstruction.
4. **Gold standard**= look at cilia from nasal/bronchial brush by electron microscopy
5. Low NO (Nitric Oxide) , promising screen for PCD in patients \>5 yr of age, provided thatcystic fibrosis has been excluded
94
PCD What is the management of PCD?
1. **Respiratory**:
a. Pulmonary **toilet** to maximize mucous clearance(postural drainage, percussion vests, positive expiratory pressure devices)
b. Do NOT suppress cough and Encourage excercise
2. **Infection**:
**a. Antibiotics** to treat infection
b. **Prevention infection**:
a. Immunizationsagainst pertussis, influenza, and pneumococci
b. avoidance of cigarette smoke and other airway irritants.
3. **SURGERY**:
a. May need **lobectomy** if severe bronchiectasis (unclear whether surgical interventions provide any long-term or survival benefit.)
b. Adult patients have undergone successful **heart-lung**, **double lung**, or **living donor lobar lung** transplantation.
95
PCD What is the routine surveillance for PCD?
1. -regular spirometry to monitor PFT
2. -chest radiographs, and
3. -sputum or oropharyngeal cultures to assess airway flora.
96
PCD When do Px with PCD present?
Most -newborn period shortly after birth
-resp distress, RR ++, low O2, or even resp failure - IPPV
97
How do you determine if a PFT was of good quality?
1. Look at **flow-volume curve:** is it reproducible (aka straight exp line vs squiggly)?
2. Look at **effort curve:**
kids \< 12 should exhale for at least 6 seconds,
kids \> 12 should exhale for at least 10 seconds
98
PFT What are the 3 main patterns seen on PFT?
1. Obstruction?
2. Restriction?
3. Bronchodilator (BD) response?
99
-What do you see on PFTs of someone with **restrictive lung disease?**
-On PFT:
1. normal FEV1/FVC, decreased FEV1
2. decreased FVC
3. decreased TLC
4. decreased RV
-
100
PFT What does an FEV1/FVC ratio of \< 80% indicate?
obstruction
101
PFT What does this PFT show?
102
PFT What does this PFT show?
103
PFT What does this PFT show?
104
PFT What specifically do you look for in a PFT? FEV1
**1. FEV1: levels (drop) -** obstructive \< 80
mild (70-79),
moderate (60-69),
severe (50-59),
very severe (\<49) airflow limitation
105
PFT What specifically do you look for in a PFT? -FVC
Look at FVC (normal in obstructive, decreased in restrictive)
106
PFT What specifically do you look for in a PFT? FEF 25-75
Look at FEF 25-75 (N = 70-120):
\< 70 = evidence of small airway flow limitation
107
PFT What specifically do you look for in a PFT? FEV1 to last PFT
has it changed?
108
PFT What specifically do you look for in a PFT? **lung volumes**
Look at lung volumes if present
1. •TLC: \< 80 **restrictive**
2. •Suggestion of **gas trapping** if RV/TLC \> 30%
109
PFT What is a significant post bronchodilator response?
\>12% change is significant
110
PFT Ddx of restrictive lung disease? Lung
think from lungs and work outwards-
**_1. Lung:_**
a. R**esection/ Atelectasis**
b. **Interstitial lung disease (fibrosis)**= ex.
i) sarcoidosis, fibrosis from radiation, chemotherapy
ii) infections (adenovirus, CMV, PCP, aspergillus, mycoplasma), iii) langerhans histiocytosis, hemosiderosis
c. Pulmonary vascular congestion secondary to **CHF**
d. **Tumor**
e. **Pneumonia** /Effusion/empyema -
111
PFT Ddx of restrictive lung disease? Chest wall
**Chest wall:**
a. Scoliosis
b. Splinting due to pain
c. Ascites + Muscle:
d. Neuromuscular disease
e. Diaphragmatic paralysis
112
What are the 2 most sensitive physical findings in pneumonia?
1. Fever
2. Tachypnea \*\*\*unfortunately neither are very specific
113
What is the differential diagnosis for vocal cord paralysis -**bilateral?**
**Bilateral** vocal cord paralysis (hear inspiratory stridor)
**-congenital CNS lesion** (myelomeningocele, arnold-chiari malformation, hydrocephalus) = think of things that compress the brainstem (CN X)
114
Vocal cord How do you Dx and Tx Vocal cord paralysis?
**Dx**:
flexible awake laryngoscopy,
need MRI head if bilateral vocal cord palsy,
consider Neuro and Cardio consult
**Tx**:
- most spontaneously resolve within 6-12 mo
- some bilateral paralysis require tracheostomy temporarily
115
Vocal cord What is the Ddx diagnosis for vocal cord paralysis -unilateral?
**Sx**: choking, aspiration, coughing, weak cry) -
**Causes**:
1. usurgery
2. intubation to the recurrent laryngeal nerve
116
What age group do you commonly see the peak incidence of the following: -croup -epiglottitis -bacterial tracheitis
-**Croup**: 3 mo-4 yo -
**bacterial tracheitis:** 3 mo - 4 yo -
**epiglottitis**: 2 yo - 10 yo
\*\*\*overall, these can occur in any age really
117
What are 3 complications of bronchiectasis?
1. Increased risk of **infection** secondary to trapping of secretions
2. Increased risk of **pneumothorax**
3. Increased risk of **pulmonary hemorrhage** (and thus hemoptysis)
118
What are common risk factors for thromboembolic disease in children? (4) -what thrombophilia disorders cause hypercoagulability? (5)
1. **Malignancy** (solid tumors \> non solid)
2. **IATROGENIC:**
a. Presence of a c**entral venous catheter** (induces endothelial damage)
b. Recent surgery/immobilization
c. OCP
3. **Thrombophilia**
- deficiency of antithrombin 3,
- protein C or S;
- factor 5 leiden,
- hyperhomocysteinemia,
- antiphospholipid antibodies
4. **SYSTEMIC INFLAMATION:**
a. Nephrotic syndrome (losing protein C & S)
b. SLE (systemic inflammation)
**\*\*\*Remember virschow's triad:**
hypercoagulability,
endothelial injury,
stasis
119
What are the 3 most common causes of epiglottis in the post-HIB vaccine era?
1. Strep pneumo 2. GAS 3. Staph aureus
120
What are the 3 phases of ARDS?
1. **Exudative phase:** decreased pulmonary compliance, increased hypoxia, increased tachypnea -see inflammation, diffuse alveolar infiltrates, pulmonary edema
2. **Fibroproliferative phase:** increased alveolar dead space, pulmonary hypertension -scarring of lung, epithelial damage, surfactant deactivation
3. **Recovery phase:** restoration of pulmonary epithelial barrier
121
What are the 3 stages of symptoms from aspiration of an object into the airway?
1. **Initial event:** Violent paroxysms of coughing, choking, gagging, and airway obstruction
2. **Asymptomatic interval:** The foreign body becomes lodged, reflexes fatigue, and the immediate irritating symptoms subside. This stage is most treacherous and accounts for a large percentage of delayed diagnoses and overlooked foreign bodies. It is during this 2nd stage, when the child is first seen, that the possibility of a foreign body aspiration is minimized, the physician being reassured by the absence of symptoms that no foreign body is present.
3. **Complications:** Obstruction, erosion, or infection develops to direct attention again to the presence of a foreign body. In this 3rd stage, **complications** include fever, cough, hemoptysis, pneumonia, and atelectasis.
122
What are the 5 Ts of anterior mediastinal mass?
1. Thymoma
2. T cell leukemia
3. Terrible lymphoma
4. Thyroid tumors
5. Teratoma
123
What are the **2 main** causes of hypoxia?
1. Hypoventilation & Low FiO2
2. **Within lung:**
a. V/Q mismatch or Shunt
b. Diffusion barrier
c. Abnormal **Hgb saturation curve**
124
What are the abnormalities seen on spirometry in asthma?
1. Airflow limitation -**low FEV1 -FEV1/FVC ratio** \< 0.80
2. **Bronchodilator response: improvement** in FEV1 \> 12%
3. **Exercise challenge:** worsening in FEV1 \> 15%
125
What are the benefits of using a spacer with an MDI? (3) -
1. Decreases the coordination required to use MDIs
2. Improves delivery of drug to lower airways
3. Decreases risk of oral deposition (Minimizes risk of thrush)
126
What are the biologic risk factors for asthma morbidity and mortality? ( 3 categories)
1. Acute Care:
a. hx of **ICU admission** or **intubation-** (resp failure or arrest)
b. 2 or more **hospitalizations** for asthma in past **year**
c. **3** or more **ED** visits for asthma in past **year**
2. medciation:
a. Use of \> 2 cannisters of SABA per month
b. **Poor** response to **systemic corticosteroid** therapy
3. **Patient:**
**a.** Low birthweight
b. Non white ethnicity
127
What are the causes of ARDS? -two broad categories
**Direct lung injury**
1. Pneumonia (most common)
2. Aspiration
3. Pulmonary contusion
4. Submersion injury
5. Inhalational injury
**Indirect lung injury:**
1. Sepsis
2. Shock
3. Burns
4. Transfusion related
5. Trauma
128
What are the classic ECG findings in a patient with pulmonary embolism?
1. Most common:sinus **tachycardia**
2. Most characteristic: **S1-Q3-T3**
- large S wave in lead 1,
- large Q wave in lead 3,
- inverted T wave in lead 3
3. Also can have **RBBB and right axis deviation** since right heart is pumping heart against a blocked PA
129
What are the clinical features of **congenital central hypoventilation syndrome?**
1. Deficient **hypercarbia** and **hypoxia** sensitivity during wakefulness and sleep and thus do not respond with increased ventilation or arousal during sleep (or awake for some)
2. Present in first few hours after birth with **shallow respirations** and **apneas**
3. -can also present with **autonomic nervous system abnormalities:** like cardiac asystole,
**4. Congenital problems:** tumors of neural crest origin, Hirschsprung disease
130
OSA What are the clinical manifestations of OSA? -daytime
**Daytime**:
**CNS**: morning headaches, , mood/behaviour/learning difficulties, daytime sleepiness/frequent napping
**ENT:** mouth breathing and dry mouth, chronic nasal congestion, hyponasal speech,
**GIT:** difficulty swallowing, poor appetite/FTT,
**Renal:** secondary enuresis (due to disruption of normal nocturnal pattern of ADH)
131
What are the complications of chronic recurrent aspiration? (3)
1. **Granuloma formatio**n
2. Interstitial **fibrosis**
3. **Lipoid pneumonia** = through oral or nasal administration of animal or vegetable oils to treat childhood illnesses as a folk remedy
132
What are the components of pulmonary toileting? (3)
Overall goal: improve mucous clearance 1. Chest physio 2. Mucolytics 3. Bronchodilators
133
What are the differences between a barium swallow, swallowing study with video fluoroscopy, and a gastroesophageal scintigraphy (milk scan)?
\*\*\*All look for causes of recurrent aspiration
1. **Barium swallow:** Pt drinks barium and you take xrays to look at ANATOMIC ABNORMALITIES **(vascular ring, stricture, hiatal hernia, TEF**). Short viewing time so _insensitive_ and _nonspecific for GERD_
2. -**Swallowing study with video fluoroscopy:** conducted with OT feeding different consistencies and is the **gold standard for evaluating swallowing mechanism**. _More sensitive for demonstrating aspiration._
3. -**Milk scan:** pt swallows milk with radiolabel and multiple xrays are taken up to 60 minutes later to check gastric emptying
134
What are the treatment modalities of CF? (10)
1. Nutrtional support
2. Inhalation therapy
3. Chest PT 4.
Bronchodilators
5. Oxygen
6. Antibiotics
7. alpha-1-antitrypsin (inhibits neutrophil elastase which breaks down lung tissue)
8. amiloride (diuretic inhibiting sodium and water reabsorption from secretions)
9. DNAse = reacts with DNA released by dead leukocytes to reduce sputum viscosity
10. Gene therapy: alters CF gene
135
What can give falsely high O2 sat readings? -what can give falsely low O2 sat readings?
Pulse oximeter measures differential absorption of light and calculates ratio of oxyhemoglobin to deoxyhemoglobin -
**Falsely high O2 sat readings** (ie. monitor reads 100% sat but patient is actually hypoxic) = **carbon monoxide poisoning** due to high infinity of CO to hemoblogin and thus, looks like hemoglobin is saturated
**-Falsely low O2 sat readings** (ie. monitor reads 70% but patient is not hypoxic) = **methemoglobin**
136
What causes chylothorax? (3) -
**aggravating** factor? -
**treatment**? (2)
Chylothorax = disruption of thoracic duct leading to accumulation of **chyle** (high lipid and protein content)
**Causes:**
1. **Thoracic surgery** complication
2. **Birth injury:** thoracic duct leaks with stretch
3. **Obstruction** of central veins by **thrombosis or malignancy** -
**Worsened** by ingestion of fat -
**Treatment**:
1. **low fat diet** (portagen formula = medium-chain fatty acids, or TPN without lipids)
2. If severe, can **ligate thoracic duct**
137
What controls respiratory drive?
1. Central chemoreceptors in the **medulla** = sense **hypercarbia** (**MAJOR** role in respirations)
2. Peripheral chemoreceptors in the **carotid arteries/arch of aorta** = sense **hypoxia** (minor role in respirations)
138
What investigations should be ordered for a baby suspected to have central sleep apnea?
1. **Congenital central hypoventilation**: _PHOX2B genetic_ testing
2. **Airway obstruction:** consider bronchoscopy/CT chest
3. **Diaphragm dysfunction:** diaphragm fluoroscopy
4. C**ongenital cardiac** disease: consider ECHO
5. Structural h**indbrain or brainstem abnormality**: MRI head
6. **Metabolic disorders**
139
What is a clue on exam that can help you differentiate between laryngomalacia vs. tracheomalacia?
-Laryngomalacia = inspiratory stridor (above thoracic inlet) -tracheomalacia (weak tracheal rings collapse with expiration)= expiratory or biphasic stridor (below thoracic inlet
140
What is a possible resp complication of empyema?
Bronchopleural fistula
141
What is the best test to rule out laryngeal cleft? -clinical feature of laryngeal cleft?
**Clinical feature:** coughing/choking occurs DURING swallowing
**-best test:** rigid bronchoscopy is REQUIRED to rule it out!
142
What is the broad differential for bronchiectasis?
1. Cystic fibrosis
2. Aspergillus (proximal bronchiectasis)
3. Ciliary dyskinesia
4. Immunodeficiencies
143
What is the characteristic features of a habit (tic) cough? -treatment?
**Characteristic features**
1. Cough occurs only during the day and never during sleep
2. Cough is absent if physician listens outside the exam room but appears immediatly on direct attention to the child and the symptom
**Treatment**:
1. reassurance that pathologic lung condition is absent,
2. recommend that child resume school immediately,
3. speech therapy to allow child to increase awareness of the sensations that trigger cough,
4. self hypnosis
144
What is the classic finding on CXR for a lung abscess?
Parenchymal inflammation with a cavity containing an air fluid level
145
What is the definition of ARDS? (4)
1. **Acute onset**
2. **PaO2/FiO2 \< 200**
3. **Absence of congestive heart failure** (ie. absence of left atrial hypertension with pulmonary artery wedge pressure \< 18)
4. Diffuse **bilateral pulmonary infiltrates consistent with pulmonary edema**
146
What is the difference between a primary and secondary lung abscess?
**Primary**: occurs in previously healthy patient with no underlying medical conditions -more commonly found on **right** side
**Secondary**: occurs in patient with underlying medical conditions -more commonly on the **left** side
147
What is the differential diagnosis for middle mediastinal mass?
A+B
1. **Adenopathy:**
a. infectious (histoplasmosis most common),
b. neoplastic & metastatic,
c. sarcoidosis
2. **Bronchogenic cyst**
148
What is the differential diagnosis for posterior mediastinal mass?
1. **Neurogenic tumor** (3 N's): neuroblastoma, benign ganglioneuroma, ganglioneuroblastoma
2. **Neurofibroma**
3. **Esophageal** duplication cysts
4. **Pulmonary sequestration**
149
What is the differential diagnosis of wheeze? (10)
1. Asthma 2. Aspiration penumonitis: ask about coughing/choking with feeds, reflux symptoms, rule out TEF 3. BPD 4. Bronchiolitis obliterans: chronic wheezing after adenovirus infection 5. Congenital cardiac lesion: left to right shunts causing increased pulmonary blood flow 6. Cystic fibrosis: ask about recurrent diarrhea, failure to thrive 7. Foreign body aspiration 8. Vascular rings/slings 9. Airway malformations: tracheomalacia, lung cysts, mediastinal mass 10. Primary ciliary dyskinesia: ask about recurrent sinusitis, ear infections
150
What is the first step in evaluation for foreign body aspiration? -what might you see if there is a foreign body obstructing a bronchus?
**1st step:**
CXR! Expiratory PA chest film is most helpful
**Will see:** obstructive emphysema and air trapping with persistent inflation of the obstructed lung and shift of the mediastium towards the opposite side during expiration
151
What is the gene involved in congenital central hypoventilation syndrome?
**Gene:** PHOX2B gene: (An apneic fox - 2 be remembered!!)
**Mechanism**; essential to embryologic development of the **autonomic nervous system** from the **neural crest** -90-95% are de novo mutations and the rest inherit the mutation from **asymptomatic mosaic parent (autosomal dominant)**
**Inheritance**: -an individual with CCHS has 50% chance of passing on the mutation to their children
152
What is the gold standard diagnostic test for obstructive sleep apnea?
-What is the **first line** treatment for obstructive sleep apnea? **-2nd line** treatment**?**
Gold standard test: polysomnography (sleep study)
**treatment**
- 1st line : Adenotonsillectomy
- 2nd line: **CPAP** if surgery fails or pts who are not candidates for surgery
153
What is the gold standard for diagnosis for **interstitial lung disease**? -additional workup?
**Gold standard** - Lung biopsy
Workup needs to rule out conditions on the huge ddx:
1. **Infectious work-up:** mycoplasma PCR, CMV serologies, chlamydia culture, sputum samples
2. **Rheumatic** disease markers
3. **ACE level** (sarcoidosis)
4. UA (**urinalysis**) to rule out associated renal disease
154
What is the gold standard for diagnosis of PE? -what is the diagnostic test of choice? -what is the utility of a V/Q scan?
1. **Gold standard:** pulmonary angiography -not necessary except in unusual cases since spiral CT is almost always available
2. -**diagnostic test of choice:** spiral CT with IV contrast (specificity 90%)
3. -**V/Q scans** are noninvasive and sensitive: use if pre-test probability is low
155
What is the management of epiglottitis?
This is a MEDICAL EMERGENCY! Child looks toxic and can progress to complete airway obstruction within 24 hrs of presentation -clinical diagnosis = do NOT need xray to confirm if this will only agitate child (thumbprint sign on xray)
1. Secure airway
2. Secure iv line, Send CBC and blood culture and start Abx: (3rd generation cephalosporin IV +/- vancomycin)
156
What is the mechanism of action of theophylline? -why is it not a first-line agent for young children? -adverse effects?
1. **mechanism**: Bronchodilation and anti-inflammatory properties (phosphodiesterase inhibitor)
2. -**not first line agent** because of differences in **absorption** and **metabolism** of theophylline in different patients --\> ***narrow therapeutic window*** and need to closely monitor serum theophylline levels
3. -**adverse effects** with overdose: *headaches, vomiting, cardiac arrhythmias, seizures and death*
157
What is the most common bacterial infection associated with bronchiolitis?
AOM
158
What is the most common cause of recurrent or persistent cough in children?
Asthma
159
What is the most common foreign body aspirated by children?
Nuts! -must ask parents specifically about nuts; if there is any history of eating nuts, bronchoscopy should be carried out immediately
160
What is the most common underlying problem associated with recurrent pneumonias in hospitalized children? -
**Definition of recurrent pneumonia:**?
**Causes of recurrent pneumonias**
1. **Oropharyngal incoordination/dysphagia** leading to chronic recurrent aspiration
2. -**2nd most common is probably GERD** -
**Definition of recurrent pneumonia:**
2 or more pneumonias in 1 year OR 3 or more in a lifetime
161
What is the most important cause of a false negative on a sweat chloride test?
1. **Edema**! (ie. hypoalbuminemia)
2. -other causes:
a. **malnutrition**
b. **hyponatremia** (not enough sweat to collect)
162
What is the polysomnographic parameter most commonly used in evaluating sleep disordered breathing? -normal values for children \< 12 yo and \>12 yo?
**Apnea/**hypopnea **index (AHI):** tells us number of apneic and hypopneic events **per hour** of sleep
-Normal cutoff AHI value for OSA in kids
\< 12: \> 1.5
\> 12: \>5
\*\*\*consensus is that any child with an apnea index \> 5 should be treated
163
What is the treatment for a PE? -what is the mortality rate in children from PE?
**Treatment for a PE:**
1. **Heparin** infusion (aim for aPTT to be 1.5-2x normal) x several days, then begin PO warfarin x 3-6 mo
2. -**LMWH** can also be used
N.B. -thrombolytic agents should only be used in combo with anticoagulants in the sickest patients -
**Mortality rate:** 2.2%
164
What is the treatment for a pulmonary abscess? -empiric abx -treatment duration -option for patient who does not improve with IV abx
1. 2-3 wk course of IV abx followed by PO abx to complete 4-6 wk course
2. -choice of abx should be guided by results of gram stain and culture but should initially include agents with both aerobic and anaerobic coverage -**Ceftriaxone + clindamycin, add aminoglycoside** if gram negative bacteria are isolated
3. -for severely ill patients who do not improve after 7-10 d IV abx, consider **surgical** intervention (CT guided percutaneous drainage or thoractomy)
165
What is the treatment for acute vocal cord dysfunction exacerbations?
1. Relaxation breathing techniques 2. Inhalation of heliox to relieve vocal cord spasm
166
What is the treatment for alpha-1-antitrypsin deficiency?
IV enzyme replacement from pooled human plasma
167
What is the treatment for ARDS?
Overall: supportive management
1. **Medication**:
a. Nitric oxide and /or Oxygen
b. Surfactant (does not change mortality however)
c. Sedation (decrease pulm htn)
d. Neuromuscular blockade
e. Steroids (start within 72 hrs, can be used up to 30 d)
**2. Ventilation:**
a. **Prone position** (improves V/Q mismatch)
b. **Limit tidal** volumes to 6-8 ml/kg)
c. HFO (gentle ventilation)
d. Permissive hypercapnea
3. \*\*\*If all else fails, **ECMO**
8.
9.
10.
168
What is the treatment of a pneumothorax that is small (\<5%) or moderate sized in an otherwise normal child? -what is the treatment for recurrent pneumothoraces?
**1. Small (\<5%) oin an otherwise normal child**
No treatment needed: will self-resolve usually in 1 wk -
2. **recurrent** pneumothoraces: may need to induce formation of strong adhesions between the lung and chest wall (**sclerosing procedure** with ***talc*** or ***iodopovidone*** into pleural space, ***VATS*** with stripping of the pleura to leave inflamed pleura to heal with sealing adhesions)
169
CF: What is the prognostic importantance of the bacterial colonosiation by age group?
if a **baby** is already infected with **pseudomonas**, this means their CF is advanced;
if a teenager is only colonized with **h.flu,** doing well
170
What medication class should be avoided in individuals with history of obstructive or central sleep apnea?
OPIOIDS!!!! They are very sensitive to these medications and have worsening of apnea
171
What percentage of foreign bodies lodge in the right main bronchus in children? -what is the most important factor in determining the need for bronchoscopy in possible foreign body aspiration?
1. 60% of FB's lodge in the right main bronchus
2. most important factor in in determining the **need for bronchoscopy**: history! (imaging may be negative in up to 30% of cases)
172
What population do you most commonly see idiopathic pneumothorax in?
Tall, thin male with subpleural bleb
173
What radiological signs can be seen on a CXR in a patient with ABPA?
1. Proximal bronchiectasis
2. Bronchial wall thickening
3. Pulmonary infiltrates
174
What recreational drug is associated with pneumothorax?
Ecstasy!
175
Ddx for Localized wheezing?
1. Foreign body
2. Extrinsic compression by mass
3. Bronchomalacia
176
What is the differential for: -diffuse wheezing?
Diffuse wheezing:
1. CF
2. Asthma
3. Bronchiolitis
4. Anaphylaxis with resp involvement
5. GERD
177
Where is the acute airway obstruction seen in croup?
Subglottic narrowing at the cricoid cartilage!!
178
Which conditions predispose children to the development of pulmonary abscesses? (3)
1. **Esophageal/Airway conditions:**
a. Aspiration pneumonia
b. GERD
c. TEF
2. **Cystic fibrosis /**Immunodeficiencies
3. **Neurological** conditions
\*\*anything that can lead to aspiration of infected materials with oral organisms \*\*can also occur secondary to a pneumonia
179
Why should you never put O2 on a child with history of OSA and walk away?
Children with OSA are chronic CO2 retainers and thus their respiratory drive is dependent on hypoxia! If you give them O2, then there will be no more respiratory drive and they will become apneic
180
Asthma What is optimal technique for MDI?
1. MDI (no mask) for each puff, take 5 sec inhalation with 5-10 sec breath hold
2. MDI with mask-for young children, use spacer and mask and
after each puff, hold mask on face for 5-10 breaths (or 30 seconds)
181
OSA What are the clinical manifestations of OSA? nighttime
**ENT breathing:** snoring, apneas, choking or gasping arousals,
**Sleep;** restless sleep, adopt unusual sleeping positions to keep their necks hyperextended to open up airway
182
Asthma Guidelines \> 5 yrs
183
Asthma Is it controlled?
184
Asthma What is FEF 25-75?
185
CF What are the clinical Fx of CF - mnemonic?
