Abstract
An overview of the general pediatric pulmonary topics necessary for ward knowledge, clinical acumen, and board examination preparation is presented in an easy-to-study question-and-answer format.
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What is the most common chronic pediatric disorder? | Asthma |
Is the incidence & prevalence of asthma been increasing or decreasing? | Both increasing |
Is the morbidity & mortality from asthma increasing or decreasing? | Both increasing (some studies show that M&M is leveling out, but for the boards use increasing) |
Which children are most likely to experience serious morbidity & mortality from asthma? (demographic factors) | • Poor children • Inner-city children • African-American |
What type of asthma puts children at highest risk of death from an acute asthma episode? | Mild asthma (>80 % of deaths were in children with mild asthma histories) |
What proportion of children who have asthma should be on chronic medication for the disorder? | ¾ |
What is the relationship between household pets and asthma? | More pets = less asthma risk (it’s best to have at least two – it has to do with a bacterial endotoxin on dogs & cats) |
What is the “hygiene hypothesis” for the etiology of asthma? | Lack of microbial exposure in developed countries prevents a normal shift from fetal Type 2 Helper T-Cell dominance to Type 1 Helper T-Cell dominance Type 2 Helper T’s promote allergy |
What is the prevalence of asthma in developing countries? | <2 % |
What is the role of obesity in asthma? | Seems to be pro-inflammatory |
If you have two dogs in the household, how much lower is your kid’s risk of developing asthma, compared to kids with no pets? | About 18 % on the average |
PFTs can’t be done properly on very young children. How is asthma diagnosed in these very young children, if they seem to have symptoms? | Clinically – Repeated episodes of wheezing, coughing, retractions/use of accessory muscles, tachypnea without other explanation & Response to typical asthma medications |
Which PFTs should be used in older children to document asthma? | FEV 1 & FEV 1 /FVC |
Why is it important to begin appropriate treatment of asthma, for children requiring long-term medication, as soon as possible? | Because permanent damage (airway remodeling) occurs in the first few years |
After permanent changes occur in the lung of an asthma patient, can you still improve pulmonary symptoms? | Yes – symptoms will improve but lung function will not return to normal |
What is the most important part of asthma medication treatment – inhaled steroids or inhaled beta-agonists? | Steroids – inflammation seems to be the main issue |
Do children with mild asthma develop structural changes in the lung? | Yes – thickening of basement membranes |
“Transient wheezing” that does not develop into asthma usually occurs in what setting? | Young children with viral illnesses |
Which kids are at biggest risk for “transient wheezing” episodes? | • Boys • h/o low birth weight • Maternal smoking during & after pregnancy |
What anatomic difference has been observed in children with transient wheezing? | Smaller than average airways and lungs |
If a child wheezes before age 3, what major risk criteria indicate a high risk of chronic asthma? | • Parental asthma • Eczema • Sensitivity to inhaled allergens |
If a child wheezes before age 3, what minor risk criteria indicate a high risk of chronic asthma (if the child has two of the risk criteria)? | • Wheezing without URI • Food sensitivities • Eosinophilia ≥4 % |
The risk factors just mentioned are the criteria for what index, developed by following 1,000 children to identify likely risk factors for asthma after age 5? | The Asthma Predictive Index |
Which children does the index just mentioned apply to? | Children <3 years old with four or more episodes of wheezing over a 1-year period (The Asthma Predictive Index) |
What is the expected percentage of normal FEV1 for children with moderate persistent asthma? | 60–80 % (severe asthma is <60 %, naturally) |
What is the expected percentage of predicted FEV 1 for children with mild forms of asthma (persistent & intermittent)? | ≥80 % |
Many children “grow out of” asthma. When does that usually happen? | At puberty |
If a child’s asthma is not controlled when he or she reaches the critical age for “growing out of” asthma, is it likely to go away? | No |
If a child with asthma is obese when he/she reaches puberty, is the asthma likely to remit? | Less likely |
In addition to obesity and poor control of asthma, what other factors are associated with continuing asthma through adolescence? | Sinusitis & environmental allergies not under good control at puberty |
Which paromyxovirus causes an RSV-like infection in late winter and early spring? | Human metapneumovirus |
Which cardiac problem often presents like RSV, especially in very young infants & children? | Congestive heart failure |
An infant is brought in for an apnea. A coughing adolescent sibling accompanies mom and baby. What are these two clues supposed to tell you? | That the infant caught pertussis from the adolescent (could also be a coughing mom) – Apnea – or seizure – is a fairly common pertussis presentation for infants |
What is a common, and worrisome, presentation for pertussis in infants? | Apnea! |
What is the mortality for infants presenting with apnea due to pertussis? | 50 % |
Pertussis is most severe in which age group? | Infants |
Bilateral empyemas and a scarlatiniform rash strongly suggest what diagnosis? | Group A strep pneumonia |
A pneumonia that begins within hours of birth, and has a fulminant course, is likely due to what organism? | Grp B strep |
If you are treating a bacterial pneumonia in a child between a month of age and 10 years, what organisms must you cover? | Staph aureus & Strep pneumo |
For patients older than 10 years, which organisms will you mainly cover, if you believe the patient has a bacterial pneumonia? | Strep pneumo & atypicals |
What is the most common (immediate) cause of cardiac arrest in kids? | Respiratory failure |
In infants, a blocked nose can cause respiratory distress. In older patients, how much of the total airway resistance is determined by the nose? | 50 % (!) |
How much of the total airway resistance comes from the peripheral airways in a child? Why is this important? | • 50 % (vs. 20 % in an adult) • A little edema in the airways can cause a big problem |
Generally speaking, what ABG values indicate that your patient is in respiratory failure? | pH < 7.3 PaO2 and PaCO2 of about 50 each |
What kind of a cardiac shunt produces cyanosis? | Right to left (bypasses the lungs) |
Asthma can increase CO2 for a variety of reasons. What are the main two? | 1. Lack of ventilation when the episode is very bad 2. High intrathoracic pressure squishes the alveolar capillaries, increasing the effective dead space |
If the board wants to tell you that a patient is hypoxemic, what are they likely to put in the vignette? | Change in level of consciousness |
If the board wants to tell you that a patient is hypercarbic, what are they likely to put in the vignette? | Change in level of consciousness |
If the board wants to tell you that there is an airway obstruction issue, what are they likely to put in the vignette? | Stridor |
If a patient is described with gray skin coloration and tachycardia, what is the vignette usually trying to tell you? | Patient is hypoxic |
What do patients with pulmonary hypertension, cor pulmonale, and polycythemia all have in common, in terms of respiratory function? | Chronic respiratory inadequacy/insufficiency |
If a patient lives with chronic respiratory insufficiency, what must you be careful of when treating them with oxygen? | Suppression of respiratory drive if too much oxygen is given |
How can a respiratory drive be oxygen dependent? I thought it was based on carbon dioxide levels? | Normally, it is based on CO2 level – with chronic CO2 retention, though, it switches to O2 |
When a boards question lists “Circulation, Airway, Breathing” as one of the answer choices, what should that mean to you? | It is almost always the right answer! |
If a patient has a respiratory acidosis , what must the CO 2 on the ABG show? | High CO 2 (Just think of CO 2 as acid) |
If a patient has a respiratory alkalosis , what must the CO 2 on the ABG show? | Low CO 2 |
In a metabolic alkalosis , what must be on the ABG? | High bicarb |
In a metabolic acidosis , what must be on the ABG? | Low bicarb |
Which pediatric patients are likely to have a metabolic alkalosis? | Those on chronic diuretics, such as BPD patients |
Which pediatric patient group is most likely to develop a metabolic acidosis? | Shock (any of the types) or toxin ingestion |
When do you see respiratory alkalosis? | Most often, with simple hyperventilation – Also seen with ↑ ICP and encephalopathies, early portion of asthma exacerbation, and salicylate overdose |
If you have a patient with acidosis and a low bicarb, which type of metabolic derangement is that? | Metabolic acidosis |
If you have a patient with acidosis and a high CO 2 , which type of metabolic derangement is that? | Respiratory acidosis |
If bicarb is the base that’s supposed to take care of (buffer) any extra acid in the body, what do you think will happen to the bicarb level, if the body acids increase? | It should go up to take care of (buffer) the acid (it takes a little while to change the bicarb level, though) |
If you have a patient with acidosis and a high CO 2 , who also has a high bicarb, what does that mean then? | Partially compensated respiratory acidosis (If it’s acidosis with high CO 2 , it’s still respiratory acidosis – the bicarb went up to try to fix the problem. That’s why it’s partially compensated.) |
If CO 2 is acid for the body, what do you think will happen to the CO 2 level if the body gets alkalotic? | It will go up, to try to take care of (buffer) the excess of base/bicarb (this process happens pretty quickly, because it mainly depends on how fast or slow we’re breathing) |
If you have a patient with alkalosis, and a high bicarb, who also has a high CO 2 , what does that mean then? | Partially compensated metabolic alkalosis (If it’s alkalosis with a high bicarb, it’s still metabolic alkalosis – the CO2 went up to try to fix the problem. That’s why it’s partially compensated.) |
What is the trend for morbidity and mortality from asthma? | Both increasing |
What is the probability that a child will have problems with recurrent wheezing, if the child develops RSV bronchiolitis in the first 3 years of life? | 50 % (may not become asthma, necessarily) |
If a patient is having an asthma exacerbation, and the wheezes eventually disappear so that the chest is much quieter, what should you conclude? | Two possibilities – Either the patient has improved, or the patient is worse (not moving enough air to produce a wheeze) |
Early in an asthma exacerbation, what kind of blood gas changes do you expect? | Low CO 2 (due to rapid breathing) & sometimes low O 2 |
What is the name for the metabolic derangement early in an asthma episode? | Respiratory alkalosis |
With impending respiratory failure, what happens to the blood gas values? | CO 2 goes up (poor ventilation) and O 2 goes down |
What sort of metabolic derangement do you expect late in a bad asthma exacerbation? | Respiratory acidosis (very little compensation, because the kidney can’t adjust the bicarb so quickly) |
How can you tell that an asthma patient is heading for respiratory failure, by the way he/she is speaking? | Can’t speak or can’t speak more than a few words at a time |
When should you worry about cyanosis in an asthma patient? | If it’s central (not really a good thing, though, in any part of the patient!) |
If you see oral/perioral cyanosis, is that considered to be central cyanosis? | No |
If an asthma patient is heading for respiratory failure, what hint can you get from their body positioning? | Won’t lie down (not very specific, though – most patients with a significant asthma exacerbation are anxious, and anxious people don’t like to lie down) |
Patients with impending respiratory failure often have what skin finding, in addition to cyanosis? | Diaphoresis (especially if the patient is conscious) |
What three sorts of risk factors determine a patient’s risk for a fatal asthma exacerbation? | 1. Medical 2. Psychosocial 3. Ethnicity |
How do you know that a child is at increased risk for a fatal asthma attack, based on ethnicity? | Non-whites are at greater risk |
What characteristics of previous asthma attacks mean that your patient is at increased risk for a fatal exacerbation? | 1. Rapid decline or severe compromise 2. Respiratory failure 3. Seizure 4. Loss of consciousness |
Most of the psychosocial factors that impact a patient’s risk for a fatal asthma exacerbation have to do with how likely they are to follow their asthma regimen. What are they? | 1. Psychiatric disorder (including depression) 2. Dysfunctional family 3. Denial of disease with (direct) noncompliance |
There is a final psychosocial factor that impacts risk for fatal asthma attacks. It is really more of a demographic characteristic. What is it? | Resides in the inner city |
Which cardiovascular finding is wildly popular on boards exams, rarely performed in real life, and a sign of impending respiratory failure in an asthmatic? | Pulsus paradoxus |
For those of you who don’t measure it on a daily basis, what is pulsus paradoxus? | When the difference between the systolic BP in inspiration vs. expiration is more than 10 mmHg (it’s normally <5 mmHg) |
Why does pulsus paradoxus happen? | The variation in systolic BP is due to the change in intrathoracic pressure – it drops with inspiration. That reduces the systolic blood pressure a little |
Pulsus paradoxus seems like a mainly cardiovascular thing. Why is it affected by a pulmonary problem like asthma? | If intrathoracic pressure drops a whole lot due to making a huge inspiratory effort, the systolic BP is lowered that much further |
Can’t pulsus paradoxus happen with cardiac problems, too? | Yes – for example, pericardial tamponades are usually worsened by inspiration, lowering cardiac output, and reducing the systolic BP (but the mechanism is different than in asthma) |
Should you base your decision to intubate a patient on lab values or on clinical impression? | Clinical impression |
Why should you be careful to avoid overhydration in patients with chronic lung disease? | Tendency to SIADH (could make the extra fluid very hard to get rid of) |
Do you need to worry about SIADH in your asthma patients? | Yes |
Do you need to worry about suppressing the ventilatory drive when you give O2 to asthma patients? | No (they don’t have chronic hypercarbia, so their drive should still be CO2 dependent) |
What type of peak expiratory flow suggests that an asthma patient can be discharged to home? | ≥70 % of expected peak flow |
After you’ve treated an asthmatic patient, what level of peak expiratory flow indicates that he/she needs to be admitted to the PICU? | <50 % |
If your asthmatic patient’s response to treatment is between 50 and 70 %, what disposition is recommended? | Admit to floor |
Should you routinely give antibiotics to asthma patients who develop an exacerbation in response to a respiratory infection? | No – most of the infectious triggers are viral |
If you need to give a systemic beta-agonist for asthma, what is the preferred agent? | Terbutaline |
When is it reasonable to give a systemic beta-agonist? | Response to inhaled treatment is not adequate |
Why do beta-agonists improve asthma? | Stimulate the Beta-2 receptors on the smooth muscle (increasing cAMP) which relaxes the smooth muscle |
Which is more effective for children with a severe asthma exacerbation, continuous or intermittent nebulization? | Continuous (it may be a more cost-effective way to deliver nebulization for asthma, in general, also) |
Should inhaled ipratropium bromide (Atrovent®) be used for children with asthma exacerbations? | Yes! Every time. (Especially beneficial for the worst exacerbations) |
If an asthma patient is acidotic, should he/she be intubated? | Not automatically – evaluate clinically |
There are three absolute indications to intubate an asthmatic. What are they? | 1. Arrest (I think you know that) 2. Severe hypoxia 3. Rapid deterioration in mental state |
A lot of doctors feel happier with a controlled airway. What are the “downsides” to intubating asthmatics? (List 1 mechanical problem, 1 correlation, 2 ventilation issues) | 1. Sticking the laryngoscope in worsens bronchospasm 2. >50 % of morbidity/mortality happens during and immediately after intubation 3. Ventilation increases the risk of hypotension 4. Ventilation increases the risk of barotrauma |
Why would you want to consider using ketamine when intubating an asthma patient? | Relaxes bronchoconstriction |
Why would you want to consider using a cuffed tube after intubating an asthmatic? | Improves ability to ventilate |
Is it a good idea to intubate an asthma patient without neuromuscular blockade? | No – Big increases in pleural pressure and increased bronchoconstriction |
You have just finished intubating an asthmatic patient, and now the patient has suddenly become hypotensive. What is the best initial management of the hypotension? | Support pressure with fluid – not pressors (also consider whether intrathoracic pressure is too high due to a tension pneumo or not allowing enough time for expiration) |
Are secretions a worry after you’ve intubated an asthma patient? | Yes – can obstruct the tube, and are another cause of hypotension post-intubation |
If you set the ventilator to allow for a long expiratory phase, your patient will often continue to be hypercarbic while ventilated. What should you do about this? | Usually nothing – it’s called permissive hypercapnia, and is okay as long as the pH and oxygen level are alright |
If you are seeing a near-drowning patient in the ER, and they look fine and have a good chest x-ray, is it safe to discharge them home? | No – ARDS often develops hours after the incident. Best to observe in a monitored setting for first 24 h |
What is the main problem for the patient with ARDS? | Not enough oxygen (hard to find a way to get O 2 into the blood stream) |
The best way to match ventilation and perfusion in (ventilated) patients with cardiogenic pulmonary edema is __________? | PEEP (Positive End Expiratory Pressure) |
An infant is admitted to the hospital with meningococcemia. She was resuscitated successfully and is receiving IV antibiotics. Twelve hours after admission she becomes tachypneic and hypoxemic. What will her x-ray most likely show? | Diffuse reticular infiltrate (ARDS s/p fluid resuscitation in an ill patient) |
Upper airway obstruction typically produces what symptom? | Stridor |
What are the three typical signs of lower airway obstruction or constriction? | 1. Cough 2. Wheeze 3. Prolonged expiratory phase (earliest sign) |
What are normal adult arterial blood gas (ABG) values? (pH/PaO2/PaCO2) | 7.4/100/40 |
At what age should children’s blood gases look approximately the same as those of adults? | 7 years |
The younger the child, the more the ABG differs from adult values. In what three ways is the ABG of a child normally different from that of an adult? | 1. O2 is lower 2. CO2 is lower 3. Bicarb is lower |
What is a normal PaO2 for children <24 months old? | About 90 |
What is a normal PaCO2 for children <24 months old? | About 34 |
The bicarb (HCO3 −) in an ABG is calculated, not measured. What bicarb values are normal for adults vs. children younger than 2 years? | Adult 24 Older infant/toddler 20 |
At birth, ABGs are wildly different from those of older children. What is a typical birth ABG? (pH/PaO2/PaCO2/HCO3 −) | 7.27/60/55/19 (These are not typos! Newborns really have these scary ABG values.) |
Neonates >24 h old have an ABG between that of the birth ABG and toddler ABG. What are the typical values? | 7.37/70/33/20 |
Can a VBG (venous blood gas) be used to evaluate pH if an arterial sample is not available? | Yes – Venous blood may have a slightly lower pH, but it’s very similar |
In most children with respiratory problems, how high is your oxygen saturation goal? | >95 % |
The mainstay of treatment for reactive airway disease exacerbations is what medication (and dose)? | Nebulized albuterol (0.1 mg/kg/dose – just FYI) |
In moderate to severe reactive airway disease (RAD) exacerbations, what other nebulized medication is helpful, in additional to albuterol? | Ipratropium bromide (Atrovent®) (0.25–0.5 mg FYI) |
If an RAD patient ordinarily takes steroids, and presents to you with an exacerbation, what medication is definitely indicated? | Oral or IV/IM steroids – 2 mg/kg loading dose |
Are parenteral steroids “proven” to have a greater or more rapid effect on RAD exacerbations than oral steroids? | No – Not for mild to moderate exacerbations (If exacerbation is severe, give IV or IM) |
In a severe RAD exacerbation, or if a patient cannot take nebulizer treatments, what other medications may provide rapid relief? (Name 2 medications) | 1. Epinephrine SQ (0.01 cc/kg of 1:1,000 – maximum dose 0.3 cc’s) 2. Terbutaline 0.01 mg/kg SQ (maximum dose 0.4 cc’s per dose) |
If you are treating RAD with epinephrine or terbutaline, how many times may you repeat the dose? | Three times total, 15 min apart |
If an RAD patient does not have significant improvement with the first albuterol treatment, what medication should be added? | Steroids (even if the patient does not take them regularly) |
For severe RAD episodes, responsive to terbutaline given SQ, what medication should be started? | Terbutaline infusion, of course! |
In addition to epinephrine, steroids, and terbutaline, what other injectable medication may aid in relaxing the smooth muscle of the pulmonary tree? | Magnesium sulfate |
If available, what special gas mixture can be used to decrease the work of breathing in either upper airway obstruction or RAD? | Heliox (helium/oxygen mix – studies are mixed as to whether it helps) |
When should heliox definitely not be used? | When the patient is severely hypoxic – (It is mixed with helium so the FiO2 is less than a rebreather mask or similar device) |
In addition to treatment with medications, asthma patients need to identify triggers for their exacerbations. What are four common ones? | 1. Cold 2. Exertion 3. Upper respiratory infections 4. Irritants (pollen, dander, smoke, etc.) |
How is childhood asthma categorized? (4) | 1. Mild intermittent 2. Mild persistent (<1 episode per day, but more than 2 per week) 3. Moderate persistent 4. Severe persistent |
What is the only level of chronic asthma that can be treated without daily medication? | Mild intermittent |
In general, what is the guiding principle in RAD treatment? | Be aggressive – (When you have gained good control of the problem, you can gradually decrease treatment as tolerated) |
What is the most common cause of bronchiolitis? | RSV (other viruses & mycoplasma also cause it) |
What percentage of infants with bronchiolitis will have RAD later? | 50 % (It often resolves after age 3) |
What age group is mainly affected by bronchiolitis? | <12 months & especially <3 months |
How is RSV spread? (Name 2 mechanisms) | 1. Mainly direct secretion contact 2. Droplets |
Do bronchiolitis patients usually have rhinorrhea & thick nasal secretions? | Yes (That’s how it spreads so easily via secretions) |
What are two important environmental or demographic risk factors for the development of bronchiolitis? | 1. Low socioeconomic status (due to crowding, more frequent delay in immunizations, etc.) 2. Exposure to smoke – especially cigarette smoke |
Rapid viral test are available for diagnosis of RSV. If negative, what follow-up test should be sent? | Nasopharyngeal culture |
Bronchiolitis is an annoying but benign disease for most infants. In very general terms, which patients are likely to have serious complications? | Those with comorbidities (including prematurity) |
In addition to viral tests, what labs or diagnostics should you order for a bronchiolitis patient? (Name 4) | 1. CBC with differential 2. Pulse ox 3. ABG (depending on severity of illness) 4. Chest x-ray |
What complications are seen in bronchiolitis? | 1. Pneumonia 2. Apnea 3. Respiratory distress & failure 4. Pneumothorax (with coughing or mechanical ventilation) |
Which airways are affected in bronchiolitis? | Bronchioles, silly! (They’re the small airways before the alveolar sacs) |
Why do the airways become swollen in bronchiolitis? | The virus attacks the respiratory epithelium yielding inflammation |
Why do the small airways become obstructed in bronchiolitis? | Normal respiratory epithelium dies – The replacement cells do not initially have cilia (so secretions don’t move!) |
Why might an infant with bronchiolitis seem to have hepato-splenomegaly? | The hyperinflated lungs move these organs down |
A history of apnea, cyanosis, or respiratory distress in an infant with bronchiolitis suggests what complication is likely? | Impending respiratory failure |
Do patients with bronchiolitis usually have a fever? | Yes – Low grade |
When might theophylline be useful in bronchiolitis? | Apneic patients – It stimulates the respiratory drive |
What are the typical findings of bronchiolitis on chest x-ray? (Name 3) | 1. Hyperinflation 2. Atelectasis 3. Diffuse interstitial infiltrates |
Do infants with bronchiolitis improve when treated with aerosolized β-adrenergic agents? (in other words, albuterol) | Sometimes (Infants with a personal or family history of wheezing are most likely to benefit) |
In addition to supportive care and β-adrenergic agents, are antibiotics, steroids, or antiviral agents helpful in the treatment of RSV bronchiolitis? | Antibiotics – no Steroids – probably not Antivirals – no (Aerosolized ribavirin no longer considered helpful for the most severely ill infants as of 2004) |
Although the use of ribavirin is controversial in RSV treatment, what three beneficial effects has it been shown to have? | 1. Improved O2 saturation 2. Shortened illness duration 3. Shortened period of viral shedding |
If a child with RSV requires mechanical ventilation, weaning & extubation is often difficult. Why? | Copious secretions & atelectasis |
What are typical signs of impending respiratory failure? (Name 4 signs) | 1. ↓ O2 2. ↑ CO2 3. Retractions 4. Lethargy (or sometimes agitation) |
Does RSV bronchiolitis require isolation? | If admitted, yes (Meaning don’t mix them with non-RSV patients) |
If a child has recovered from RSV bronchiolitis, can they become reinfected? | Yes – Even in the same season! |
How long is the typical course of RSV? | Usually improves in <5 days, but cough may persist for weeks |
Chlamydial organisms can cause pneumonia in children & adults – which chlamydial organism affects neonates and which affects older children/adults? | Neonates/infants – Chlamydia trachomatis Children/adults – Chlamydia pneumoniae |
I have a nagging feeling there’s another sort of chlamydia that causes pneumonia. Is there? | Yes, Chlamydia psittaci – Comes from birds Much less common |
What triad of symptoms goes with croup? | Barking cough Stridor Hoarseness ( All are due to subglottic stenosis ) |
What is the other (more official) name for croup? | Laryngotracheobronchitis Mnemonic: LTB stands for Laryngo-Tracheo-Bronchitis or Long-Term Bark! |
In general terms, what causes croup? | Many viruses (mainly parainfluenza, but also RSV, adenovirus, etc.) |
What is spasmodic croup? | Sudden onset of inspiratory stridor for a several-hour period – Recurs for several days – Usually happens at night |
What is the typical age for croup? | 6 months–36 months (most common between 1 and 2 years) |
Is croup more common in males or females? | Males (3:2) |
What is the natural course of croup? | Complete recovery |
What are the main complications of croup? (Name 3 complications) | 1. Respiratory failure (rare) 2. Airway obstruction 3. Hypoxia |
Why does airway obstruction sometimes develop in croup infection? | First, the immune response creates surplus sections, then – It causes erythema & edema of cords and upper airway |
Why are subglottic areas most affected by the swelling of croup? | The cricoid cartilage sits there, and it limits the diameter of the airway |
If a child seems to have croup, but doesn’t have a fever, and the onset of symptoms was while awake & playing – what other diagnosis should be considered? | Foreign body aspiration |
If you suspect a diagnosis of croup, what x-rays might you want? (Name 2) | Chest x-ray (rule-out foreign body, pneumonia) & AP & lateral soft tissue neck x-rays (rule-out epiglottitis, pharyngeal abscess) |
What are the typical x-ray findings of croup? (Name 2 findings) | “Steeple sign” (on AP neck) & Chest x-ray may show atelectasis |
What is the typical duration of croup infections? | 3–5 days |
Why should you wait at least 3–4 h after treatment with racemic epinephrine before discharging a croup patient? | Croup patients often have a rebound as much as 2 h after the treatment |
How can croup be treated (if treatment is necessary)? (Name 3 treatments) | 1. Cool, humified air 2. Nebulized racemic epinephrine (0.5 mL of 2.25 %) 3. Dexamethasone IM |
If a croup patient is thought to have impending respiratory failure, what is the next step in management? | Intubation in the OR (do not delay for x-ray or other tests) |
When is racemic epinephrine indicated for croup? (Name 2) | Extreme respiratory distress Or Minimal response to humidified air |
What five factors are evaluated in the croup severity score? | 1. Inspiratory stridor 2. Retractions 3. Air entry (how much it is decreased) 4. Cyanosis 5. Level of consciousness |
If a child suffers from recurrent croup, what should be investigated? | 1. Anatomic abnormalities 2. Congenital anomalies 3. GE reflux |
Why is IM dexamethasone preferred to oral prednisone in croup treatment? (Name 4 reasons) | 1. Efficacy of oral steroid is not clear 2. Croup patients often have poor oral intake 3. Less GI distress 4. Longer half-life (about 48 h vs. 24 h) |
What is the most common cause of cor pulmonale? | Parenchymal lung disease (sometimes other types of lung disease also cause it) |
What causes cor pulmonale? | Chronic hypoxia → Pulmonary vasoconstriction and pulmonary hypertension → High RV afterload |
When does cor pulmonale usually start? | In teens of 20s – Especially with pregnancy (can be found at any age, though) |
Why does the right ventricle fail in cor pulmonale? | The afterload of the pulmonary vasculature is too much for it |
What are the typical complications of cor pulmonale? (Name 3 complications) | 1. Anemia or polycythemia (can go either way) 2. RV failure 3. Sudden death |
Why are cor pulmonale patients at risk for sudden death? | They cannot augment cardiac output with exercise |
What physical findings are expected in cor pulmonale? (4 – do not give auscultation findings) | 1. Parasternal heave 2. JVD/hepatomegaly/peripheral edema 3. Tachycardia 4. Cyanosis |
What do cor pulmonale patients usually complain of? (Name 5 complaints) | Fatigue Syncope Palpitations Chest pain Poor exercise tolerance |
How can upper airway disease cause cor pulmonale? | The hypoxia leads to pulmonary vasoconstriction |
Why do newborn infants have less trouble with RV failure secondary to pulmonary hypertension? | Both ventricles have a similar structure at birth (so the RV is stronger then than it will be later) |
If the right ventricle is strong enough to “stand up” to the pressure of pulmonary hypertension, why do neonates develop heart failure with cor pulmonale? | Secondary to the hypoxemia & acidosis of the condition |
Is nocturnal O2 useful for cor pulmonale patients, in general? | No – But it may delay progression in individuals with obstructive sleep hypoxia producing cor pulmonale |
What diagnostic procedure is indicated for all cor pulmonale patients? | Cardiac catheterization |
What are the main invasive measurements needed in cardiac catheterization for cor pulmonale? | Pulmonary artery pressure & Reaction of pulmonary vasculature to oxygen & various medications |
What are the two most common causes of eosinophilic pneumonia? | 1. Drug reactions (therapeutic or recreational drugs) 2. Parasites |
What proportion of eosinophilic pneumonias has no known etiology? | 1/3 |
How is eosinophilic pneumonia diagnosed? (Name 3) | 1. Infiltrates + peripheral eosinophilia 2. Infiltrates + >5 % eosinophils in lavage fluid 3. Eosinophilic infiltrate on biopsy |
What is “Loffler’s syndrome?” | The other name for “simple pulmonary eosinophilia” |
How severe are the pulmonary symptoms in Loffler’s syndrome? | They are mild |
Can eosinophilic pneumonia become chronic? | Yes – Unknown etiology |
Which patient groups might develop “allergic bronchopulmonary aspergillosis?” | Those with asthma or cystic fibrosis |
What occurs in allergic bronchopulmonary aspergillosis? (Name 3 steps) | 1. Aspergillosis colonizes the bronchi 2. ↑ IgE and eosinophils 3. Worsens control of CF, asthma, and often causes bronchiectasis |
“Acute” eosinophilic pneumonia is different from Loffler’s syndrome. How? (Name 4 ways) | 1. Acute febrile illness (it has acute in the name, after all) 2. Progresses rapidly 3. Severe – often leads to respiratory failure 4. Lavage has eosinophils, but blood often doesn’t |
What is “Churg-Strauss” syndrome? (great distractor item!) | Asthma + vasculitis (multiorgan) + eosinophilia |
What causes “Churg-Strauss?” | Unknown (patients often have other allergic tendencies) |
What is the mainstay of treatment for Churg-Strauss syndrome? | Steroids |
With the exceptions of Churg-Strauss and allergic aspergillosis, what is the prognosis for the various types of eosinophilic pneumonias? | Excellent with prompt treatment when needed |
What is the main complication of allergic bronchopulmonary aspergillosis (a form of eosinophilic pneumonia)? | Pulmonary fibrosis/ Severe bronchiectasis |
If eosinophilic pneumonia is caused by parasites, will you be able to locate them in the stool? | Sometimes – Several common causes are often not (e.g., toxocara, ancylostoma, early ascaris) |
If the differential from the CBC does not indicate eosinophilia, is eosinophilic pneumonia still a possibility? | Yes – There may be pulmonary infiltration without peripheral eosinophilia |
Steroid therapy for acute & chronic eosinophilic pneumonia is very important – What aspect requires the most careful approach? | The steroid taper must be very gradual to prevent relapse |
In pediatrics, hemoptysis is most common in what age group? | Older children & Adolescents |
What are the main things in your differential for a patient with hemoptysis? (Name 6 general categories) | 1. Bronchiectasis/pneumonia 2. Cavitary infections 3. Tumors 4. Congenital heart disease 5. Foreign body 6. Pulmonary embolus |
What are the main dangers associated with significant hemoptysis? | 1. Respiratory insufficiency 2. Hypovolemic shock |
Why does Hanta virus cause respiratory problems? | Profound pulmonary edema (Alveolar capillaries leak) |
How do humans acquire Hantavirus? | Contact with rodent urine or feces (wet or dry) |
What are the main presenting complaints of Hantavirus infection? | High fever, headache, GI complaints (The famous “flu-like syndrome” so many odd diseases start with) |
When does cough develop for a Hantavirus patient? | When cardiac depression & pulmonary edema begin |
Does a Hantavirus patient have URI complaints? | No! |
Why are serial CBCs performed while awaiting confirmation of Hanta infection (which takes days)? | The platelet count falls in the prodromal phase – supporting the diagnosis |
When pertussis infection is fatal, how does it kill? | Pneumonia (90 % of pertussis deaths in young children are due to pneumonia) |
In general terms, why do children with pertussis develop pneumonia? | Bacterial superinfection (It is possible for pertussis to do it, directly, though) |
The highest mortality for pertussis is in what age range? | <6 months (about 1 % mortality) |
How is pertussis spread? (2 modes) | Aerosol & Contact with secretions |
What is the common name for pertussis? | Whooping cough |
Do children <6 months “whoop” with pertussis? | Usually not – Apnea is common, though (and terribly silent) |
What cells does pertussis primarily attack? | Ciliated epithelium |
Why does bronchiectasis develop with pertussis? | Secretions & sloughed epithelium block the bronchioles (as in RSV) |
In the US, children who acquire pertussis usually encounter it in what reservoir? | Nonimmune adolescents & adults |
What are the stages of B. pertussis infection? | 1. Catarrhal (URI) 2. Paroxysmal (cough) 3. Convalescent (cough may continue) |
What causes epiglottitis? | Various bacteria (e.g., Hemophilus influenza B, Staph aureus, Strep pneumo, Strep pyogenes) |
How much has HiB vaccine decreased the incidence of epiglottitis and related HiB infections? | 98 % |
Strep pyogenes as a cause of epiglottitis is usually seen in what population? | School-aged children in winter/spring |
Classic physical diagnostic findings for epiglottitis are? (Name 3) | 1. Drooling 2. Tripod posture 3. “Thumb sign” on lateral neck x-ray (The epiglottis looks like a thumb sticking into the airway) |
What must never be included in the physical exam of a child suspected of having epiglottitis? | Direct visualization of the oropharynx – Closure of the airway can result !!! |
Should you try to send lab work on a child suspected to have epiglottitis? | Not unless the airway is secure (Upsetting the child could cause the glottis to close) |
What is the main cause of death for plague victims? | Pneumonia |
How is pneumonic plague acquired? | Contact with saliva or droplet respiratory secretions (of other humans) |
Most cases of plague occur in children & adolescents. Why? | They are more likely to have contact with rodents/small animals |
Chronic, intermittent pulmonary hemorrhage can result from two autoimmune diseases. What are they? | SLE & Idiopathic pulmonary hemosiderosis |
Idiopathic pulmonary hemosiderosis is very similar to Goodpasture’s disease. How is it different? | No effect on the kidney |
Where is the hemosiderin in idiopathic pulmonary hemosiderosis? | In the macrophages (where hemosiderin is usually found!) |
Do patients lose enough iron in idiopathic pulmonary hemosiderosis to become anemic? | Yes |
What is the course of idiopathic pulmonary hemosiderosis? | Kids – May have spontaneous remission Adults – Chronic (Doesn’t go away) |
Which rheumatologic lab will be positive for Wegener’s granulomatosis patients? | c-ANCA (p-ANCA is for polyarteritis nodosa) Mnemonic: Think of an anchor (ANCA) swinging up and sinking one end into the lungs and the other into the kidneys to remember what Wegener’s affects, and that it is c-ANCA positive |
What three parts of the body does Wegener’s affects? | 1. Lungs 2. Kidneys 3. Upper respiratory tract/sinuses (that’s why they get nosebleeds) |
What is the new term for Wegener’s granulomatosis? | Granulomatosis with polyangiitis |
What is the buzzword for the histologic changes in Wegener’s granulomatosis (Granulomatosis with polyangiitis)? | Necrotizing granulomas/granulo-matous vasculitis |
How is a Wegener’s diagnosis confirmed? | Nasal or lung biopsy (not kidney biopsy – too invasive) |
How do Wegener’s patients usually present? (Name 3 symptoms) | • Hematuria/glomerulo-nephritis • Cough/hemoptysis • Epistaxis |
Should a Wegener’s patient present with dyspnea? | No!!! |
If a patient presentation sounds like Wegener’s, but Goodpasture’s is also an option, how can you differentiate them? (List 4 ways – 3 are clinical 1 is lab based) | Nose stuff – Wegener’s Dyspnea – Goodpasture’s Anemia – Goodpasture’s ANCA negative – Goodpasture’s |
How is Wegener’s granulomatosis treated? | Cyclophosphamide ± steroids |
The primary process creating the problems in Wegener’s granulomatosis is __________? | Vasculitis (granulomatous type & mainly small vessel) |
What is the main problem in Goodpasture’s syndrome? | Antibodies to glomerular basement membrane, which deposit in both lung & kidney ( Then complement attacks it !) |
What is the buzzword for the histology in Goodpasture’s? | “Linear” deposits of IgG along the basement membrane Mnemonic: Think of a one-lane road winding through a “good pasture” with a few cows & horses. This reminds you the deposits are linear in “Goodpasture’s”!) |
How is Goodpasture’s treated? | Immunosuppressive medications & Plasmapheresis (if necessary) |
How does Goodpasture’s syndrome present? | Dyspnea Hemoptysis Iron deficiency anemia Glomerulonephritis |
SIDS is most common in the first 6 months of life. In which of these 6 months is it very uncommon? | The first month! |
Where does SIDS rank, as a cause of death in infants? | Third |
What pulmonary issue is a risk factor for SIDS? | Smoking – Both during pregnancy & passive smoke exposure after delivery |
What intervention has had the biggest impact on the incidence of SIDS? | The “back to sleep” program (Putting infants to sleep on their backs, and keeping them that way) |
What two factors are most important to preventing bronchopulmonary dysplasia (BPD)? | 1. Preventing birth until after 30 weeks gestation 2. Using prenatal steroids to enhance lung development |
Which diuretic has been shown to be helpful for BPD infants? | Furosemide (it improves lung function – others do not) |
After birth, are steroids helpful in the management of BPD? | No |
What is the mainstay of treatment for BPD, and how do you know whether it is effective? | • Oxygen (low flow) • Weight gain (20–40 g per day) |
Infants with pulmonary conditions like BPD are sometimes given RSV immunoglobulin. If RSV-IVIG is given, how does this alter the infant’s immunization schedule? | MMR & Varicella vaccines cannot be given until 9 months after the last IVIG |
What is the difference in onset for broncho-pulmonary dysplasia vs. bronchiolitis obliterans? | • BPD must begin within roughly 1 month of birth • Bronchiolitis obliterans usually occurs between 6 months and 2 years, and follows an infection |
What is bronchiolitis obliterans? | Small airways close up → poor gas exchange |
Which ethnic group is at special risk to develop bronchiolitis obliterans? | Native Americans |
Which infection is especially likely to produce bronchiolitis obliterans? | Adenovirus lower respiratory infection (Especially types 3, 7 & 21 Mnemonic: 3 × 7 = 21) |
In addition to gas exchange problems, producing hypoxemia & hypercarbia, what other respiratory problem often develops for bronchiolitis obliterans patients? | Pulmonary edema |
How is bronchiolitis obliterans diagnosed definitively? | Lung biopsy |
If a child has adenovirus pneumonia, what is the probability that he or she will develop bronchiolitis obliterans? | 1/3!!! (For Native Americans, about 2/3) |
In addition to adenovirus infection, what other situations increase the risk of bronchiolitis obliterans? | Lung transplant Or Bone marrow transplantation with Graft vs. Host Disease (GVHD) |
Is bronchiolitis obliterans related to BOOP (bronchiolitis obliterans organizing pneumonia)? | No |
Which patient age group is at highest risk to develop BOOP? | Adults – Although it does occur in kids |
How is the pathology different in the two bronchiolitis obliterans disorders? | BO – Loss of patency in the smallest airways leads to reduced surfaces for gas exchange BOOP – Hyperplasia & inflammatory infiltrates swell the respiratory septa, blocking the small airways |
How do BOOP patients present clinically? | Multiple bouts of bronchitis that do respond to antibiotics |
Which cells are hyperplastic in BOOP? | Type II pneumocytes (The ones that make surfactant, remember?) |
What is the best known disorder involving respiratory cilia dysfunction? | Kartagener’s |
What is the nature of the problem with the cilia in Kartagener’s disorder? | One or both of the “dynein arms” that make the cilium move are missing |
Is Kartagener’s syndrome common or rare? | Rare |
What are the presentations of Kartagener’s syndrome? (Name 4) | Sinusitis Bronchiectasis Male infertility Situs inversus (!) |
Are antibiotics indicated in most cases of foreign body aspiration? | No – Just get it out! |
What is the probability that an aspirated foreign body won’t be diagnosed for a month after the incident (in pediatrics)? | 1 in 5 (Yikes!) |
Which objects are the most popular ones for aspiration, among children? | Seeds & nuts (including peanuts) |
If most aspirated foreign bodies are radiolucent, why is a chest x-ray helpful for diagnosis? | Most (about 2/3) will have localized hyperinflation noticeable on x-ray |
If a child has aspirated an object, and you’re not sure what it is, should you try a blind finger sweep? | No! (You might push the object further in.) |
What is bronchiectasis, when it occurs as a chronic condition? | Dilatation or distortion of the bronchi |
How do patients with chronic bronchiectasis present? | • Recurrent pulmonary infections • Chronic productive cough • Wheezing • Clubbing (very common) |
How would a child develop bronchiectasis? | Infection or inflammation |
Define “periodic breathing?” (Name 3 components) | Pause of ≥3 s At least three times Less than 20 s of respiration between each episode |
No breathing for how many seconds definitely constitutes apnea? | 20 s |
When does failure to breathe for less than 20 s still constitute apnea? | If cyanosis or bradycardia occurs along with the pause |
Periodic breathing is most normal & common in which infant group? | Preemies |
Is prematurity a risk factor for SIDS? | Yes |
Is apnea of prematurity a risk factor for SIDS? | No!!! |
If an asthma patient has an abnormal sinus x-ray, is it safe to assume that antibiotic therapy is indicated? | No – Most kids with asthma have abnormal sinus x-rays |
Which asthma medication decreases lower esophageal sphincter tone, possibly worsening GE reflux and increasing the possibility of asthma exacerbations? | Theophylline |
What treatment is most recommended for children with exercise-induced asthma? | Short acting β-adrenergic med just before exercise |
If asthma symptoms are well controlled on the current regimen, when should the patient have follow-up, and what is the goal of follow-up? | • Follow-up in 1–6 months after good control is established • Evaluate for possible “step-down” in treatment |
What is the most critical, simple, measure for the long-term management of asthma? | A peak flow meter (& proper knowledge of how to use it) |
Do inhaled steroids have the same long-term side effects as the systemic steroids? | Generally, no |
What class of agents are salmeterol and formoterol? | Long-acting β2-agonists (12 h action) |
What is the very important limitation to the use of salmeterol and formoterol? | Not useful in acute asthma (long acting and slow onset ) |
Which common CNS drugs will decrease theophylline levels? | Phenobarbital & Phenytoin |
Ciprofloxacin elevates theophylline levels. Which two common pediatric medications will also elevate theophylline levels? | Erythromycin & Cimetidine |
Recurrent or prolonged croup could be a manifestation of what congenital tracheal malformation? | Tracheal stenosis (usually segmental) |
If an infant has significant tracheal stenosis at birth, how will he or she present? | Severe retractions, stridor & dyspnea |
Webs can sometimes partly or completely obstruct the laryngeal inlet. What genetic syndrome are they associated with? | Velocardiofacial syndrome |
What genetic problem should you test for in an infant with glottic webs? | 22q11 |
What are laryngeal cysts? | Pouches of mucus-secreting epithelium, usually supraglottic |
What causes laryngeal cysts? | Usually result from trauma or prolonged intubation (removed via laser) |
How does subglottic stenosis present? | Congenital – stridor in first few months Acquired – follows multiple intubations/procedures, often asymptomatic |
How can you evaluate whether your patient has subglottic stenosis? | Bronchoscopy is best, but direct laryngoscopy can provide diagnosis & some limited information |
How is congenital subglottic stenosis managed? | Nothing if asymptomatic Surgery as early as possible if symptomatic |
What is the most common cause of stridor in the newborn? | Laryngomalacia |
At what age is laryngomalacia most often symptomatic? | 2 weeks |
How is laryngomalacia managed? | Most kids outgrow it by about 1 year |
What is congenital lobar emphysema, and which part of the lung is most often affected? | • One or more lobes abnormally enlarged & filled with air or fluid • Left upper lobe |
When do infants with congenital lobar emphysema present? | Birth to 6 months old |
Which infants are most likely to have pulmonary hypoplasia, in terms of their birth history? | Premature due to premature rupture of membranes |
How is pulmonary hypoplasia managed? | Support oxygenation via mechanical ventilation or ECMO until lungs grow |
Is pulmonary hypoplasia a common reason for neonatal infant death? | Yes |
What is a pulmonary sequestration? | An area of nonfunctional lung-type tissue either within the lung or outside it. It has its own arterial supply & venous drainage. |
Will a bronchoscopy help you to diagnose a sequestration? | No – It is not part of the normal airway architecture, so you won’t see it |
What will help you to diagnose a pulmonary sequestration, in terms of radiology evaluations? | CT scan or Chest x-ray (Doppler US can sometimes be used due to the abnormal vessels serving the sequestration) |
What is scimitar syndrome? (Great distracter item!) | When right pulmonary venous blood returns to the IVC |
There are two forms of scimitar syndrome, labeled infantile & adult. What is the difference? | Adult: Appears later (childhood or adolescence) Has a better prognosis Involves just a small amount of right pulmonary blood returning to the IVC |
How does the infantile form of scimitar syndrome present? | Respiratory distress & Heart failure (Bad prognosis) |
How would a pulmonary sequestration patient present? | 1. Respiratory distress 2. Bad pleuritic chest pain 3. Hemoptysis 4. Recurrent pneumonia |
Patients with pulmonary A-V fistulas most likely have what autosomal dominant disorder? | Osler-Weber-Rendu (aka hereditary hemorrhagic telangiectasia) Mnemonic: Think of a gentleman all dressed up dancing the “rendu,” an old-fashioned dance. Unfortunately he gets a nosebleed and also bright red blood per rectum, because these patients have lots of hemorrhagic telangiectasias |
A child with asthma & nasal polyps is likely to have a medication sensitivity to what drug? | Aspirin (It’s called the “aspirin triad” & sometimes also called “Samter’s triad”) |
If a bronchogenic cyst is identified, is it okay to observe it as long as it is asymptomatic? | No – Excision is better due to the potential for malignancy & likely symptoms from local compression (eventually) or infection |
Why do bronchogenic cysts increase in size over time? | They are usually mucous filled & more is secreted over time |
In addition to compressing nearby structures, why else do bronchogenic cysts become symptomatic? | Infection → Chest pain, fever, & cough |
At what amount of curvature do you expect scoliosis to affect pulmonary function? | 50° (the problem is usually alveolar hypoventilation) |
What causes scoliosis, in most cases? | Idiopathic (we don’t know) Early bracing will fix it |
Cardiopulmonary compromise is expected when scoliosis reaches what level? | 90° |
Does pectus excavatum cause pulmonary problems? (Pectus excavatum means the chest goes inward) | No (Repair is cosmetic only) |
Pectus carinatum is when the sternum is unusually far out, and the lateral parts of the rib cage are flattened (pigeon chest). Does it cause pulmonary problems? | No (Repair is cosmetic only) |
Jeune syndrome alters the rib cage so that it is smaller than usual. Does this cause pulmonary problems? | Yes – Restrictive lung disease & frequent infections |
Jeune syndrome always causes short ribs, small rib cage, and what other problem? | Renal disease |
Jeune syndrome patients often have a variety of musculoskeletal issues and are always affected by a small rib cage & renal disease. How does Jeune syndrome develop? | Autosomal recessive disorder Mnemonic: “Recessive-renal-rib cage” on a young dwarf. (Jeune means young in French, and Jeune syndrome patients sometimes also have dwarfism. Visualize a kidney inside a “receding” rib cage on the poor young dwarf.) |
What is the other name for jeune syndrome (the descriptive name)? | Asphyxiating Thoracic Dystrophy |
What is the most common lethal recessive disorder? | Cystic Fibrosis |
What is the second most common lethal recessive disorder? | SMA (Spinal Muscular Atrophy) |
Why do the spinal muscles atrophy in SMA? | Degeneration of the anterior horn nuclei (sometimes also the bulbar nuclei) |
Where is the gene for spinal muscular atrophy? | Chromosome 5q |
Will patients with SMA develop sensory or cognitive problems? | No – The anterior horn cells (& sometimes bulbar nuclei) are the only ones affected, so it is motor only |
Which famous infectious disease also kills off the anterior horn cells of the spinal cord? | Polio (So SMA patients look much like polio patients!) |
If SMA is such a common and deadly disease, why haven’t I heard more about it? | There are three forms – The worst (& best known) one is Werdnig-Hoffman – You’ve probably heard of that |
How can you remember which gene causes the SMA group of disorders? | • Because they symmetrically weaken the muscles of “all 5” extremities – arms, legs, & head (if the bulbar nuclei are affected) • & it makes the patient “queer” (“q” part of the chromosome) in their motor function, not petite (p)! |
What are the three types of SMA? | • Werdnig-Hoffman is Type I (aka Severe Infantile) • Intermediate or Chronic Infantile is Type II • Mild SMA is Type III (aka Kugelberg-Welander) (The main info to know is that there are three types, and that Werdnig-Hoffman is Type I) |
Is CPK normal or elevated with SMA? | Normal – Muscle degeneration is secondary to nerve degeneration (CPK goes up when muscle damage is the primary problem) |
Are proximal or distal muscles more affected in Werdnig-Hoffman, and the other types of SMA? | Proximal (& legs are more affected than arms) |
How does Werdnig-Hoffman present? | • <6 months old • Hypotonia/weakness • Difficulty feeding • Tongue fasciculations ! |
How easy is it to diagnose SMA disorders with genetic screening for chromosome 5q mutations? | Pretty easy – 95 % will be found |
Biochemically, what causes Duchenne muscular dystrophy? | Missing or deficient dystrophin (a muscle protein) |
How is Duchenne muscular dystrophy inherited? | X-linked (recessive) |
What finding usually precedes significant motor function problems in Duchenne’s? | Scoliosis (worsens rapidly after the child is wheelchair bound) |
At what age will boys with muscular dystrophy begin to have problems? What are the usual problems? (Name 3 examples) | Ages 2–6 years (Think “frequent falling,” “toe-walking,” and “waddling gait”) |
Is CPK elevated in Duchenne’s? ( very frequent item ) | Yes – The muscle cells are having problems, so CPK is high |
What two buzzwords on physical exam & history go with Duchenne’s? ( very frequent item ) | 1. Calf pseudo-hypertrophy (they look big) 2. Gower’s sign – using the hands to rise or “pushing up” the legs with the arms to stand |
How is the diagnosis of Duchenne’s made? | Muscle biopsy |
Is the muscle dysfunction of Duchenne’s limited to the lower extremities? | No – All skeletal muscle is affected, & cardiomyopathy is also a big problem |
Long-term, what type of problem usually ends the lives of Duchenne’s patients? | Respiratory – Respiratory muscle weakness → respiratory failure + aspiration/ pulmonary infection is common due to weak bulbar muscles |
Is there any effective therapy for Duchenne’s? | No – Supportive only |
Neonatal myasthenia gravis can compromise the infant’s respirations. What is neonatal myasthenia? | Antibodies from the mom’s myasthenia are circulating in the baby → muscle weakness |
How do neonatal myasthenia patients present? | Same as usual – Ptosis, hypotonia, weak cry, difficulty feeding |
What is the course of neonatal myasthenia gravis? | It resolves during or after the neonatal period (as the antibodies are destroyed) |
Is congenital myasthenia the same as neonatal myasthenia? | No! ( Can you believe that?! ) |
How can you recognize congenital myasthenia gravis? | No antibodies (to the acetylcholine receptor) |
What is congenital myasthenia, then? | An autosomal recessive disorder with variable age of onset – Abnormal acetylcholine receptors cause a myasthenia-like syndrome |
“Regular” myasthenia, when it occurs in the pediatric population is called _____________? | Juvenile myasthenia gravis |
What diagnostic test can be used to confirm the diagnosis when myasthenia is suspected? | Edrophonium/the Tensilon Test (It is an anticholinesterase medication, so it boosts acetylcholine levels – function should improve briefly.) |
What is the problem in myasthenia gravis (neonatal, juvenile, or adult forms)? | Antibodies to the acetylcholine receptor decrease muscle strength/function |
Increasing use of muscles in myasthenic patients produces what result? | Increasing weakness |
How is myasthenia gravis treated medically? | Anticholinesterase medications (oral) & Immunosuppression |
What surgical management technique produces remission in about 50 % of myasthenia patients? | Thymectomy |
Is juvenile-onset myasthenia gravis more common in girls or boys? | Girls (like most autoimmune disorders) |
What is eventration? | Significant elevation of a hemidiaphragm |
What is the typical cause of eventration? | Usually congenital (can be acquired via phrenic nerve injury) |
Is eventration more common in boys or girls? | Boys (Usually on the left side) |
Accessory diaphragm (an extra diaphragm-like tissue in one hemithorax) usually causes what symptoms or signs? | • Respiratory distress due to lung hypoplasia in neonates • Recurrent infections in older kids |
If a disorder has “dystrophy” in the name, what does that mean about the disorder (e.g., muscular dystrophy)? | There is defective production of an important molecule – due to a nutrition or metabolism problem |
Pneumonia almost never occurs without what sign? | Fever |
When is it reasonable to get blood cultures in a child suspected or known to have pneumonia? | For patients requiring admission with very significant cases of pneumonia (Note: The federal government is now forcing us to send cultures for pneumonia patients – this is not medically indicated, however.) |
If you suspect a patient has pneumonia, is it a good idea to send an ESR and/or CRP? | No – not helpful |
Should serological tests, as well as cold agglutinins and viral studies, be ordered when pneumonia is suspected? | Generally no – Not unless it would change management |
If you’re not sure whether a pneumonia merits antibiotic therapy, what test(s) might help you decide? | CBC – Check for WBCs >15,000 (more likely to be bacterial) |
If you’re not sure whether the patient has a pneumonia, what test may help you decide? | CXR, of course (Does not tell you whether it’s bacterial or viral, though) |
When is it reasonable to order a chest x-ray in cases of suspected childhood pneumonia? (3) | 1. Pneumonia unresponsive to antibiotics 2. Pleural effusion is suspected 3. High fever & ↑ WBCs without a source in a child <5 years old |
In addition to fever, what other sign is a very important predictor of a (severe) pneumonia? | Cyanosis |
Signs of respiratory distress are most likely to indicate that a child has pneumonia when the child has more than one of them. What group of signs should you be looking for? (5) | Cough Tachypnea Crackles Decreased breath sounds Retractions |
Pneumonia is hardest to diagnose in which age group? | <2 years old |
What is the usual course for pleural effusions that develop in response to an infection? | Spontaneous resolution (although it may take weeks) |
Recurrence of fever or respiratory symptoms in a child with a recently resolved pneumonia should make you consider what diagnosis? | Empyema (An infected pleural effusion) |
How is an empyema treated? | Drain the fluid and give antibiotics |
Early in the course of streptococcal pneumonia, what are you supposed to hear on auscultatory exam? | Pleural friction rub (crackles come later) |
How are empyemas usually drained? | Initial thoracentesis, then closed suction drainage |
Strep pyogenes is Group A Strep. When it causes pneumonia, what will the patient’s history usually be? | A rash disease (e.g., rubeola, scarlet fever, or varicella), then pneumonia developed |
Which lung abnormality often develops with Strep pyogenes pneumonia? | Pneumatocele (resolves spontaneously) |
Which two types of pneumonia often produce pneumatoceles? | Strep pyogenes & Staph aureus |
Klebsiella is most likely to affect which pediatric patient populations? (2) | Prolonged intubation & Immunocompromised |
Staph aureus pneumonia usually develops with what history? | Recent viral URI or influenza |
Which patients are at greatest risk for anaerobic pneumonias? | Patients who aspirate (rare otherwise) |
What is the usual drug of choice for anaerobic pneumonias? | Clindamycin |
Histoplasmosis is common in which parts of the US? | Mississippi & Ohio River Valleys (In other words, midwestern and mid-southern US) |
Histoplasmosis is associated with which creatures? | Bats & Birds (& soil with bat or bird droppings) |
What is the usual course for histoplasmosis infection? | Most are asymptomatic |
Bad cases of histoplasmosis require what treatment? | Amphotericin B (It’s a fungus! Bad fungus gets Ampho B) |
A patient presents with erythema multiforme, history of travel to the southwest US, and chest x-ray shows “thin-walled” blebs. What’s the diagnosis? | Coccidioidomycosis |
Coccidioidomycosis + worsening chest x-ray or hemoptysis = | Treatment – Use Ampho B or Fluconazole (most cases don’t require treatment, though) |
When coccidioidomycosis occurs in immuno-compromised or HIV patients, how does it often manifest? | Fulminant & often fatal – Bone, skin, & meningeal involvement |
Multiple “masses” on chest x-ray, and large yeast with single buds on sputum sample, mean your patient has what infectious disease? | Blastomycosis Mnemonic: Single Bud Blasto – Just use the sounds or imagine a single guy named “Bud” who has no success with the ladies. They always tell him to “blast-off!” |
In children, blastomycosis sometimes disseminates to what two areas (from the lungs)? | Bone & Skin Mnemonic: Blasto goes to Bone & KidS SKin |
Where is blastomycosis usually found in the United States? | Midwest & Midatlantic/Southeastern (Think of a stripe from Connecticut southward, and Chicago southward) |
If a blastomycosis pneumonia patient is having a mild course, what should you do in terms of treatment? | Observe (also okay to give oral itraconazole) |
Which fungal pneumonia is also known as San Joaquin Valley Fever? | Coccidioidomycosis Mnemonic: The San Joaquin Valley is in California. “C” is for Coccidioides! |
How is allergic bronchopulmonary aspergillosis treated? | Corticosteroids |
What unusual chest x-ray appearance is sometimes noted with allergic bronchopulmonary aspergillosis? | Not only do infiltrates migrate, sometimes there is also an infiltrate that looks like “fingers in a glove” in the central lung ( creepy! ) |
Do atypical pneumonia patients usually have a productive cough? | No |
If a patient develops an atypical pneumonia – and the vignette mentions that the patient participated in hunting or skinning animals – which atypical pneumonia does he or she have? | Tularemia |
Your patient has an atypical pneumonia , and the vignette mentions that they’ve been around cattle or sheep . What’s the diagnosis? | Q fever (caused by Coxiella burnetii ) |
Is Coxiella burnetii pulmonary infection contagious between people? | No |
What is the most common cause of community acquired pneumonia for kids >5 years old? | Mycoplasma |
Is atypical pneumonia common in pre-school children? | No – Generally seen ages 6 years & up |
Is a positive cold agglutinin test diagnostic for mycoplasma? | Not diagnostic – It is suggestive, though |
What lab test is definitive for mycoplasma infection, when positive? | IgM serology |
What problems outside the lung sometimes develop in ENT systems with mycoplasma infection? | Pharyngitis Tonsillitis Bullous myringitis (vesicles on the tympanic membrane) |
Mycoplasma infection sometimes causes neurological problems. What neurological sign is especially associated with mycoplasma infection? | Confusion |
What rheumatological issues can mycoplasma infection induce? | Arthritis (of course) & Erythema multiforme (& occasionally the more serious Stevens-Johnson syndrome with lesions on mucous membranes) |
What effects does Mycoplasma sometimes have on the hematological system? | Hemolytic anemia & Splenomegaly |
If you can’t use a macrolide to treat mycoplasma (due to allergy, etc.), what inexpensive alternative could you use? | Doxycycline! (Fine for Chlamydia & many other atypicals, too – avoid in children <7 years due to tooth issues) |
Chlamydia pneumonia has another name – what is it? | TWAR pneumonia Or TWAR pathogen (TW & AR designate the labs that originally found the organism. That’s how it got the name “TWAR”) |
Is an epidemic of atypical pneumonia likely to be caused by Chlamydia or Mycoplasma? | Chlamydia |
What unusual pattern does chlamydial pneumonia often follow? | Sore throat that resolves, then, Pneumonia 2–3 weeks later |
How rapidly does Mycoplasma infection spread? | Slowly (2–3-week incubation period) |
What is the preferred regimen for mild persistent asthma? (Episodes >2× per week, but not every day) | Inhaled corticosteroid (daily) |
In moderate persistent asthma, how frequent are the asthma problems? | Daily ( That’s why they call it persistent , it happens every day ) |
What is the preferred treatment regimen for patients with moderate persistent asthma? (Name 2 options) | Medium-dose inhaled steroids Or Low-dose inhaled steroids & long-acting inhaled β-agonist |
If your patient has moderate persistent asthma, but the episodes are worse than average, or the patient develops severe exacerbations regularly, what slightly stronger than usual regimen may be required? | Medium-dose inhaled steroids & Long-acting inhaled β-agonist |
How would you identify a patient with severe persistent asthma? | Continuous day-time difficulties with asthma (& frequent problems at night) |
What is the recommended treatment for severe persistent asthma? (minimum required) | High-dose inhaled steroids & Long-acting inhaled β-agonist |
In addition to the inhaled medication regimen for severe persistent asthma, some patients may also need what treatment? | Oral steroids (2 mg/kg/day up to 60 mg) |
Which asthma patients are candidates for leukotriene inhibitors? | Mild & moderate asthma patients |
If you choose to use a leukotriene inhibitor for your patient with moderate asthma, what other medication must be part of the regimen? | Inhaled corticosteroids ( A leukotriene can be used by itself can be used in mild persistent disease ) |
Which type of asthma has an “intermittent” form? | Only mild!!! (otherwise, it’s all some sort of persistent asthma) |
Stridor that is both inspiratory & expiratory usually indicates that the patient has what disorder? | Subglottic stenosis (congenital or acquired) |
If a patient has both inspiratory & expiratory stridor, which component is usually louder? | Inspiratory |
What causes expiratory stridor? | Problems below the thoracic inlet (trachea or bronchi) |
If something is compressing the trachea, expiratory stridor often results. What other pulmonary exam finding is common? | Wheezing |
Tracheomalacia means “soft trachea.” What is the practical consequence of tracheomalacia during breathing? | The trachea collapses during expiration |
Stridor just during inspiration, with a quiet expiration in an otherwise well infant, is most often due to __________? | Laryngomalacia |
If a child is presented who has expiratory stridor and feeding problems, what diagnosis should leap to mind? | Extrinsic tracheal & esophageal compression due to vascular ring (It encircles both) |
If you suspect that your expiratory stridor patient with feeding difficulties has a vascular ring problem, how can you evaluate that possibility? | Barium swallow study |
A child is presented with expiratory stridor, and the vignette mentions that a tracheo-esophageal fistula repair was done in infancy. What is the likely cause of the expiratory stridor? | Tracheomalacia – It is a common long-term complication of TE fistula repair |
When might a tracheomalacia patient present with “biphasic” stridor? (Biphasic means both inspiratory & expiratory) | If the problem is very high (near the larynx) |
Can pulsus paradoxus be a sign of respiratory failure? | Yes (Inspiration vs. expiration SBP difference >10 mmHg) |
If a vignette asks you to make a decision about whether intubation is needed, what is the “first thing” you need to do? | Assess respiratory effort/assess the airway |
Is a normal respiratory rate reassuring when evaluating for possible respiratory failure? | Not by itself – Can mean that a tachypneic patient has fatigued to a normal rate |
If you are evaluating for possible impending respiratory failure, how reliable are sweating & tachycardia? | Not great, because they are also signs of anxiety |
What is the earliest sign of impending respiratory failure? | Tachypnea |
In addition to tachypnea, when it is present, what are other good signs of impending respiratory failure? (Name 2 signs) | Retractions & Pulsus paradoxus (SBP drop >10 mmHg with inspiration) |
Headache, joint pain, unexpected clot formation & hemoptysis should make you think of what pulmonary-related disorder? | Polycythemia due to chronic hypoxemia |
In addition to the increase in hematocrit, what other change in the CBC accompanies polycythemia? | Platelets are destroyed more rapidly – ↑ bleeding risk & low platelets |
Are cough suppressants a good idea? | No – None have been shown to be better than placebo |
Which patients often do not cough well enough to clear their respiratory passages? (Name 3 categories) | 1. Nerve & muscle disorder patients (vocal cord paralysis, CP, etc.) 2. Those in pain 3. Thoracic deformities |
On the boards, a parent asks whether it would be alright to give a 6-year-old a cough suppressant to help him sleep. What is the correct response? | No – Not effective as it s a newly popular to test item “& risks medication side effects!” |
Appropriate tests at the initial evaluation of a kid with a chronic cough are _________? (Name 3 tests) | 1. TB test 2. Chest x-ray 3. Sweat test |
A cough that disappears during sleep is usually due to what? | Psychogenic (often loud & brassy cough when kid is awake) |
What is the “buzz phrase” that often goes with psychogenic cough? | Can be “produced on command” |
What is the current trend for mortality from asthma? | Increasing |
If a child has mild asthma, what is the likelihood that he or she will outgrow it with age? | 60 % |
For children with severe asthma, what is the likelihood of outgrowing the asthma? | 30 % (Still pretty good, considering…) |
Which is more effective – MDI with spacer or nebulizer? (MDI = metered-dose inhaler) | Equal in children old enough to use them properly |
Which gender has more asthma? | Boys before puberty – Girls after |
In an asthma patient having an asthma episode, is a normal CO 2 a good thing? | No – Should be low due to rapid breathing |
Chronic nighttime cough without associated symptoms or history is likely to be what disorder? | Asthma |
If a nighttime cough is productive, but without other symptoms or history, is asthma still likely? | Yes |
In addition to asthma, what other causes of nighttime cough should you consider? (Name 2 causes) | GE reflux Sinusitis |
Infants that wheeze may have asthma (assuming it’s not due to an infection), but what other important structural & environmental problems should you consider? (Name 3 problems) | Aspiration of something Vascular ring BPD (Bronchopulmonary dysplasia) |
If a child has allergies, how much more likely is he/she to develop asthma than kids without allergies? | 3× |
If a vignette tells you that a child is “having trouble exercising,” what differential should you run through? | C ardiac problems A nemia M uscle disorder P sychology/ P ulmonary factors (e.g., depression) Mnemonic: Think of a kid having trouble exercising at CAMPP! |
What is the main concern with giving steroids to children long-term? | Growth problems (other concerns: hypertension, osteoporosis, & cataracts) |
Why are inhaled steroids the preferred method for preventing asthma exacerbations, in terms of their actions on the pulmonary system? | Decreases inflammation & Bronchial hyperreactivity |
What about the onset of reactive airway disease can help you to predict whether it will continue or spontaneously resolve? | Very early onset (<3 years) is more likely to resolve |
A family history of asthma in which parent makes persistent asthma more likely? | Mother |
What lab abnormalities suggest that your patient will probably keep asthma into adulthood? | ↑ IgE & Eosinophilia |
On a CF sweat test, what number is a positive test? | 60 mEq (Sometimes normal values are included in boards vignettes, so you need to know!) |
What lab abnormalities should make you suspicious that a child might have CF? (two main ones) | Low albumin Low sodium |
Can CF carriers sometimes have mild manifestations of CF, due to their carrier state? | No! Don’t be fooled by this one! Popular item |
Has DNA testing replaced sweat testing as the gold standard for CF diagnosis? | No |
In infancy, which symptoms of CF are more prominent – gut or lung symptoms? | Gut |
If your patient has CF, what are the chances that his/her healthy sibling is a carrier? | 2/3 (Remember it’s a healthy sibling, so she/he can only be a carrier or have two completely normal genes. That’s why it’s 2 out of 3.) |
What is the CF carrier rate in the general Caucasian US population? | 1 in 25 |
If the healthy sibling of a CF patient marries someone from the general population, what is the probability that their first child will have CF? | 1 in 150 (Easier to memorize than to calculate, for most of us. 2/3 probability carrier x 1/25 probability carrier in population x 1/4 probability child gets both bad genes) |
Which vitamin supplement is especially important for children with CF? | Vitamin E |
Are infections in CF patients eliminated with antibiotic treatment, or just controlled? | Controlled |
What neuro problems sometimes develop in CF patients as a consequence of gut problems/vitamin malabsorption? | Ptosis Truncal ataxia Problems with proprioception |
Vitamin absorption issues can lead to what hematologic problem for CF patients? | Bleeding – Vitamin K deficiency |
If cor pulmonale develops due to partial airway obstruction, will it reverse if you fix the airway problem? | Yes |
If cor pulmonale develops due to pulmonary hypertension, can it be reversed? | Generally not |
Lower extremity edema, hepatomegaly, gallop heart rhythm, and sometimes clubbing, suggest what diagnosis? | Cor pulmonale |
Hypoproteinemia, anemia, and steatorrhea in an infant often indicates what diagnosis? | CF |
If a pneumonia patient improves with treatment initially, then stays sick or worsens, suspect what diagnosis? | Empyema |
A vignette patient is described as “ cyanotic with a depressed sensorium .” What conclusion are you expected to draw that explains both findings? (Don’t think too deeply!) | Patient is hypoxic |
If a vignette patient has “ headaches ,” and is described as “ flushed and agitated ,” the boards may be trying to indicate that the patient has what pulmonary-related problem? | Hypercapnia (elevated CO 2 will vasodilate intracerebral vessels → headache) |
Does sarcoidosis always create respiratory symptoms? | No |
Weight loss, fatigue, and hilar adenopathy is probably a description of which disorder? | Sarcoid |
Sarcoid & tuberculosis have some similarities. Both can affect the heart. How does each affect the heart? | TB – pericarditis Sarcoid – conduction changes (blocks or widening of components) |
A child who refuses to lie down, prefers to sit leaning forward, has dysphonia, dysphagia, drooling, & stridor has what diagnosis? | Epiglottitis (more likely is child is not H. flu immunized) |
Do epiglottitis patients have a cough? | No |
What kind of stridor do epiglottitis patients tend to have, if they have stridor? | Biphasic (the swelling is just supraglottic, so it can cause noise in both directions) |
If you suspect epiglottitis, and you’d like to get pre-op labs, should you order them (after all, the patient is going to the OR for intubation)? | No – Let them draw labs after intubation – the airway sometimes closes off when the child cries |
Chylothorax (lymphatic fluid leaking into the thorax) most often happens in what setting? | Post-surgical (especially following cardiovascular and scoliosis surgery) |
Which two values are high in the lab analysis of chylous fluid? | Triglycerides (>110) Protein (>3) |
If a pleural effusion is a “transudate,” what does that mean, in general terms? | The fluid developed due to a problem that was not directly a lung problem |
What are some typical causes of a transudate? (Name 3 causes) | 1. CHF 2. Nephrotic syndrome 3. Cirrhosis |
How high do you expect triglycerides to be if the fluid is a transudate? | Low (<50) |
If an adolescent develops a spontaneous pneumothorax, what predisposing factors should you be thinking about? | 1. Connective tissue disorders (although tall think male adolescents have a higher chance of spontaneous pneumothorax even if they are perfectly healthy) 2. Marijuana use – The huge inhalation, and attempting to hold the smoke in, can lead to pneumothorax |
Is there a correlation between the amount of pain a patient feels and the severity of a pneumothorax? | No |
In some cases, a small but significant pneumothorax can be treated with what minimally invasive treatment? (pneumothorax of about 15 %) | Needle aspiration |
What are the three most common causes of croup? | 1. Parainfluenza 2. Influenza 3. RSV |
If an infant has an ALTE (apparent life-threatening event), what is the correct disposition for the patient? (The infant is fine at the time of exam.) | Admit for observation & evaluation |
An ALTE always involves what history? | 1. Stopped breathing 2. Turned pale or blue & unresponsive 3. Got better (resuscitates with stimulation, mouth-to-mouth, etc.) |
What are the main causes to evaluate in an ALTE patient? | Trauma/abuse Pulmonary Neuro Reflux/aspiration Electrolyte Infection Mnemonic: Think of an infant on a camping trip in the wilderness with a problem. If the infant has an apparent life-threatening event, she/he could need a lot of support – like TPN from REI, the wilderness outfitters! |
Is pulse oximetry a reliable measure of oxygenation for a patient in shock? | Not really – Peripheral vasculature is “clamped down” so blood flow is limited |
If a patient’s blood is described as being “chocolate colored,” what diagnosis should you suspect? | Methemoglobinemia |
If a patient’s blood is described as being “cherry red,” what diagnosis should you suspect? | Carbon monoxide poisoning |
Can the pulse oximeter produce a reliable estimate of oxygenation with carboxyhemoglobin circulating? | No (It will just read that the hemoglobin is bound, but not whether it is bound to oxygen vs. carbon monoxide.) |
Will anemic patients have reliable pulse ox measurements? | Not if the hemoglobin is very low (<6) |
RSV is the most common cause of bronchiolitis. What is the second most common cause? | Parainfluenza |
What is the best way to prevent RSV transmission to other patients? A – put a mask on the infected patient B – put a mask on the other patients C – wash hands frequently | C – Wash hands (the board exam likes hand washing) |
A mentally retarded child presents with sudden onset of a nonproductive cough . The child has a history of asthma, and is wheezing on the right side only . Likely diagnosis? | Foreign body aspiration (The exam often throws in some red herring pulmonary stuff like this.) |
In what proportion of foreign body aspirations do the parents or child report an aspiration event? | ½ |
Recurrent lower respiratory tract infections with atelectasis developing in the same area each time suggests what diagnosis? | Bronchiectasis |
What clue to the bronchiectasis diagnosis can you often find in the history of the cough? | Cough symptoms vary with position |
What is the best way to make the bronchiectasis diagnosis? | Chest CT |
Most children who catch TB are asymptomatic. How helpful is a chest x-ray for diagnosing TB in kids? | When it’s positive, it’s helpful, but it’s often negative in children (The CXR is often as asymptomatic as the child) |
A child is brought in for low-grade fever & cough for 6 weeks. On exam, the child seems well, but has rales at the bases. What is the likely diagnosis? | TB – Active (Start triple therapy until sensitivities are available) |
If you are treating TB meningitis, should you use steroids? | Yes |
If a patient is diagnosed with TB meningitis, what treatment should you start? | Triples (Rifampin, INH, & Pyrazinamide) + Streptomycin Streptomycin is discontinued when INH sensitivity is confirmed |
What is disseminated TB called (when it spreads all over, and especially to the lungs, as many tiny foci of TB)? | Miliary TB Mnemonic: “Miliary” refers to millet seeds – so think of TB sprinkled like seeds throughout the body |
If a child has symptoms of what seems to be a bacterial pneumonia, but also has an effusion & is an immigrant, what will you need to consider? | Pleural effusion can be primary TB |
If a child has had chest trauma, and is tachycardic with signs of respiratory distress, what is the most important procedure to perform? | Physical exam comes first (at least a brief exam) |
If the answer choices for a board exam question have “perform physical exam” as an option, why is that important? | It is very often the right answer! |
What is the initial chest x-ray appearance of ARDS? | “Fine, diffuse, reticular infiltrate” |
What is the main problem in ARDS? | Wet alveoli – The alveolar capillaries are too leaky → pulmonary edema |
What two ventilator parameters are important for improving the respiratory status of your ARDS patient? | • PEEP • Low tidal volume |
Assuming the pulmonary situation can be handled in ARDS, why is the mortality for these patients still relatively high? | Often develops into multi-system organ failure (liver, kidneys, etc., deteriorate) |
If a male patient is noted to have atresia or absence of the vas deferens, what is the most likely underlying cause? | CF |
What nasal test is sometimes used to aid in the diagnosis of CF? | Nasal potential difference measurement (as in electrical potential – it is altered due to the unusual sodium concentration in CF) |
What happens chemically to make the secretions of CF patients so thick? (Name 2) | • Overactive sodium pumps • Chloride channels that are blocked in epithelial tissue |
Which factor correlates most strongly to lifespan in CF patients? | Fitness level |
When we think of CF, we automatically think of lung & pancreatic problems. Which other body tissues are significantly affected by the disorder? (Name 3 tissues) | Liver (!) Reproductive tract Sweat glands |
How is CF inherited? | Autosomal recessive |
Which gene mutation is most important as a cause of CF? | Delta F508 – A three base-pair deletion that eliminates a phenylalanine |
Where is the cystic fibrosis gene located in the genome? | Chromosome 7 – Long arm (same as “q”) |
The gene involved in CF is officially known as ______________? | CFTR (Cystic Fibrosis Transmembrane Receptor – at least this makes sense!) |
Nasal polyps in a child <12 years old should make you consider what diagnosis? | CF – ¼ of all CF patients will have it! |
CF patients very reliably have what ENT problem? | Pansinusitis |
Lung structure & function are normal at birth for CF patients. Over time, what general category of pulmonary problem develops for them? | Obstructive pulmonary disease |
What pattern of pulmonary function test (PFT) abnormalities is expected for CF patients? (Name 2 items) | • Decreased FEV1 & peak expiratory flow • Increased residual volume |
Early in the course of CF, what infectious organisms most often bother the respiratory tract? | Staph aureus & Klebsiella (Pseudomonas/Burkholderia infection comes later) |
What proportion of CF babies have pancreatic insufficiency at birth ? | ½ |
Only about 15 % of kids with CF have meconium ileus. What percentage will develop “meconium ileus equivalent” in childhood? | 25 % (Same as “distal intestinal obstruction syndrome”) |
What is abnormal about the sweat produced by CF patients? | Very high sodium (& chloride) concentration |
Other than providing a convenient way to test for CF, does the abnormality in sweat production have any clinical significance? | In infants, it can sometimes produce hyponatremia |
What unusual joint finding suggests that your patient has CF? | Hypertrophic pulmonary osteoarthropathy Mnemonic: Thick secretions, Thick joints! |
What joint & bone changes sometimes occur in CF, known as hypertrophic pulmonary osteo-arthropathy? | Periosteal thickening of long bones & their joints |
In addition to meconium ileus & distal intestinal obstruction syndrome, what other GI problems can indicate a CF diagnosis? | Rectal prolapse Or Intussusception in kids >1 year old |
What impact does CF have on pubertal development? | Often delayed for both males & females (due to nutrition/chronic illness issues) |
What impact does CF have on male fertility? | The vas deferens is atretic – in vitro fertilization is possible, but there are no sperm in ejaculate |
What impact does CF have on female fertility? | Decreased fertility due to mucous abnormalities |
How many mutations that produce CF have been identified so far? | >1,000!!! (Less than 100 mutations produce 95 % of clinical disease, though) |
When can a sweat test for CF be considered reliable? | If it is done by a CF center |
Diagnosing CF requires that at least two criteria are met. There are two groups of criteria. What are they? (Name 3 items for each group) (1st item – standard tests) (2nd item – genetic factors) (3rd item – clinical/unusual tests) | Group 1 1. + Newborn screen 2. CF in a sibling 3. Typical CF problems Group 2 1. + Sweat test 2. + For two known CF mutations 3. + Nasal potential difference test |
How is the newborn screening for CF done? | Blood test for IRT (immunoreactive trypsinogen) |
If a newborn screen for CF is positive, what does that mean? | Not much – >90 % are false positives, so more tests are needed |
If you suspect CF, and genetic testing reveals one CF mutation, how should you interpret that? | You can’t – 4 % of the population carries CF genes (meaning has a single gene without disease) |
Children with CF have the best chance for long-term survival when their care is provided by _________? | A CF center |
What infectious diseases should prompt you to consider sweat testing, even though most of these kids will not turn out to have CF? (Name 2 diseases) | • Pseudomonas or Burkholderia infection (other than otitis externa) • Staph aureus pneumonia (Klebsiella is also concerning) |
What ENT/respiratory issues should prompt you to consider sweat testing? (Name 4 issues) | • Chronic cough or recurrent wheezing • Pansinusitis • Nasal polyps (<12 years old) • Digital clubbing |
In addition to meconium ileus & rectal prolapse, what other GI issues should make you question whether CF might be the correct diagnosis? (Name 4 issues) | 1. Steatorrhea 2. Chronic diarrhea 3. Prolonged neonatal jaundice 4. Intussusception in kids older than 1 year |
Which types of exercise are most beneficial in CF? | Swimming & jogging |
What are the mainstays of pulmonary treatment for CF? (Name 2 main modalities) | Antibiotics (various regimens) & Chest PT/postural drainage (one to four times daily) |
If a CF patient has a pulmonary exacerbation, infection is usually involved. For moderate to severe exacerbations, what sort of treatment is usually required? | 2–3 weeks IV antibiotics |
If your CF patient is diagnosed with pseudomonal disease, which popular antibiotics must you avoid using? | Ceftriaxone – Doesn’t treat it ! |
A CF patient develops chest pain. She is afebrile, and has had the same problem before. What is the problem? | Pneumothorax (10 % of CF patients develop this, and it often recurs) |
How should hemoptysis be dealt with, in a CF patient? (Name 2 ways) | 1. Consider vitamin K if bleeding is brisk 2. Most CF bleeding is the result of infection – treat with antibiotics & withhold airway clearance during the acute phase |
What cardiac complication often develops late in the course of CF? | Pulmonary hypertension/ Cor pulmonale |
If a CF patient develops clinical (right-sided) heart failure, what does this tell you about prognosis? | Survival <8 months is likely (standard treatment is used – salt restriction, O2, diuretics) |
Is digitalis helpful, when treating the cardiac complications of CF? | No – Not unless left-sided dysfunction is present (not usually the case) |
What medications are necessary to support gut absorptive function for CF patients? Why is absorption important to the pulmonary system? | • Pancreatic enzymes & H2 blockers • Normal height-to-weight ratios correlate with better pulmonary function |
What other nutritional support is necessary for CF patients, if they are to maintain their height/weight ratio? (Name 2 items) | Extra vitamins A & E High-fat diet (Some kids also need nighttime enteral feeding) |
CF patients are famous for having difficulties passing stool around the time of birth. What sort of difficulties do they tend to have with passing stool as they get older? | Diarrhea – Usually foul-smelling & bulky |
If CF patients often have diarrhea, why are they sometimes given Miralax® or other anti-constipation agents? | Constipation is even more common! |
Which genotype is worst for α-1-antitrypsin deficiency? | PiZZ |
Although α-1-antitrypsin doesn’t usually result in much pulmonary trouble in childhood, what body system is significantly affected, even in the young? | Liver – Cirrhosis & hepatomas |
When α-1-antitrypsin pulmonary problems present, what sort of problems do they have? | Emphysema (without smoking) in a young person – Often with bullous disease at the bases |
Patients who are heterozygous for α-1-antitrypsin will have what sort of pulmonary problems in their future? | None – If they don’t smoke (If they smoke, they develop early disease!!!) |
What sort of transplant will prevent α-1-antitrypsin pulmonary disease? | Liver transplant |
How are α-1-antitrypsin patients treated for pulmonary problems? | Monthly infusions of the missing molecule only when pulmonary symptoms develop (Note: There is no evidence that this helps) |
What is alveolar proteinosis? | Overproduction of surfactant by confused alveolar macrophages! |
How can alveolar proteinosis be treated? | Lavage the extra surfactant out & Give GM-CSF (sometimes gets the alveolar macrophages back on track) |
Why does scleroderma cause pulmonary hypertension out of proportion to the pulmonary disease it causes (which is interstitial fibrosis)? | It causes proliferation of the intimal layer of the pulmonary artery → pulmonary hypertension |
What are the main pulmonary effects of systemic lupus erythematosus? (Name 3 effects) | Effusion Pleuritis (painful) Hemoptysis |
What is the buzzword “noncaseating granuloma” associated with? | Sarcoid |
What chest x-ray findings are expected in sarcoidosis? | Bilateral hilar lymphadenopathy (sometimes mediastinal, also) |
What sort of PFT findings are expected with sarcoidosis? | Restrictive (& sometimes obstructive) |
Which metabolic derangement is common with sarcoidosis? | Hypercalcemia (& hypercalciuria) |
How is sarcoidosis definitively diagnosed? | Biopsy showing noncaseating granuloma |
What dermatological finding is a good sign in sarcoidosis? | Erythema nodosum (purplish nodules, sometimes tender, on the shins) |
Sarcoidosis clearly involves some derangement of the immune system. What are the typical abnormalities? (Name 2 abnormalities) | 1. Hypergamma-globulinemia 2. >4:1 ratio of helper:suppressor T cells in fluid from BAL (bronchoalveolar lavage) |
What is the typical course for sarcoidosis in children? | 75 % spontaneously recover |
If sarcoidosis treatment is required, what is typically used? | Steroids (improves symptoms, but doesn’t induce remission) |
Hypersensitivity pneumonitis also causes granulomas. What is the expected immune cell profile from BAL in this disorder? | <1:1 for helper:suppressor ratio (Suppressors are the bigger group, in other words) |
What is the most basic way to check that the pulse oximeter is functioning properly? | Check whether it is correlating correctly with the pulse |
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Houser, C.M. (2014). General Pulmonary Question and Answer Items. In: Pediatric Cardiology and Pulmonology. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-9481-2_3
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