CCP 345 Respiratory Emergencies

Question 1

Retropharyngeal Abscess in Pediatric patients

Answer 1

1. This is a potentially life-threatening emergency in young children with signs of upper airway obstruction or meningismus
2. Retropharyngeal abscess is often related to oral trauma.
3. Retropharyngeal abscess is most frequently caused by: Staphylococcus aureus, group A streptococci, and anaerobes
4. Treatment is admission, IV antibiotics and, for more severe cases, surgical drainage

Question 2

pediatric respiratory distress vs respiratory failure

Answer 2

Respiratory failure is identified by the presence of extreme distress, hypoventilation or hyperventilation, altered mental status, pale, mottled or cyanotic skin color, and/or hypotonia

Question 3

define Stridor

Answer 3

1. sound associated with upper airway obstruction
2. harsh vibratory sound of variable pitch caused by partial airway obstruction or collapse → turbulent airflow through upper airway
3. Stridor is described by timing in the respiratory cycle (inspiratory, expiratory, biphasic) and quality (coarse or high-pitched).

Question 4

define Inspiratory stridor

Answer 4

stridor d/t pathology above the glottis

Question 5

define biphasic stridor

Answer 5

stridor d/t pathology at the glottis

Question 6

define expiratory stridor

Answer 6

stridor d/t pathology below the glottis

Question 7

What the typical pathogens in epiglottitis?

Answer 7

1. Epiglottitis may be caused by many bacteria or local injury.
2. In the post–H. influenzae type b vaccine era, the typical profile of epiglottitis has changed to include older patients

Haemophilus influenza B, A, F, nontypeable)
Strep
Staph
Pseudomonas
Candida
Non-infectious causes – thermal burns, chemical burns, allergic rxn, foreign bodies

Question 8

describe the management of epiglottitis

Answer 8

Stable patient:

1. Infants and children: A stable patient who is maintaining a patent airway and adequate oxygenation should not be moved or repositioned for examination, laboratory tests, or radiography. Such patients should be carefully transported to a setting where definitive airway management can be achieved in a controlled fashion, generally the operating room
2. Adolescents and adults = have more real estate and rarely require airway management. They should be observed in the ICU and given IV abx

Unstable patient (respiratory failure):

1. Get help! (anesthesia, ENT, gensx)
2. Start with BVM and prepare for intubation
3. Have a backup plan – needle ventilation / cut to air

Patients often remain intubated for 3 to 5 days in order for antibiotic therapy to reduce inflammation and surrounding tissue edema

Question 9

define Mild Croup

Answer 9

Stridor at rest or only when agitated
No tachypnea
No retractions
No Mental Status Changes

Question 10

define Moderate Croup

Answer 10

Stridor at rest
Mild tachypnea
Mild retractions
No mental status changes

Question 11

define Severe Croup

Answer 11

Stridor at rest
Respiratory distress
Severe retractions
± Mental status changes present

Question 12

Define TTN and identify the population most at risk for it

Answer 12

1. benign, self-limited condition that can present in infants of any gestational age shortly after birth
2. caused by a delay in the clearance of fetal lung fluid after birth → ineffective gas exchange, respiratory distress, tachypnea
3. Maternal risk factors: delivery before completion of 39 weeks gestation, a cesarean section without labor, gestational diabetes, and maternal asthma
4. Fetal risk factors: male gender, perinatal asphyxia, prematurity, small for gestational age, and large for gestational age infants
5. Incidence is inversely proportional to gestation delivery age: 10% of infants 33-34 weeks, 5% 35-36 weeks, <1% in term infants

Question 13

Detail the pathophysiology of TTN

Answer 13

1. Caused by ineffective absorption of fetal lung fluid
2. Passive movement of sodium through epithelial sodium channels (ENaC) is believed to be the principle mechanism of reabsorption of fetal lung fluid
3. With the onset of labor, maternal epinephrine and glucocorticoids activate the ENaC on the apical membranes of type II pneumocytes
4. Failure or delay in clearance of intra-alveolar fluid in patients with TTN is due to:
   - Lack of ENaC expression or activity
   - Lack of active labor and its associated hormonal changes
   - Ineffective lung distention and lack of alveolar air interface
   - Immaturity of ENaC
   - Especially relevant in the late preterm infant

Ok so this all sounds like a bunch of complicated bullshit, and it is, but basically what it boils down to is when the baby is floating around in the womb it has a bunch of fluid in its lungs. When the baby gets born normally, there’s a bunch of biochemical shit that happens that causes the lung fluid to get re-absorbed/washed out. If the kid gets born premature/c-section/whatever there isn’t the trigger to wash out the fluid in the lungs and he gets this shitty lung water that causes respiratory distress

Question 14

Summarize & describe the clinical manifestations of TTN

Answer 14

1. Clinical presentation is reflection of decreased lung compliance associated with pulmonary edema and ineffective fetal lung fluid clearance
2. Onset: Present very early after birth
3. Duration: 2- 3 days

Tachypnea (RR >60)
Nasal flaring
Grunting
Intercostal/subcostal/suprasternal retractions
Crackles on auscultation
Mild degrees of hypoxia

Question 15

Define neonatal RDS and identify the population most at risk for it

Answer 15

1. common cause of respiratory distress in a newborn
2. presents within hours after birth, most often immediately after delivery
3. occurs from a deficiency of surfactant, d/t either inadequate surfactant production, or surfactant inactivation (MAS)
4. inversely proportional to the gestational age of the infant, with more severe disease in the smaller and more premature neonates
5. The most important risk factors are prematurity and low birth weight

Question 16

Detail the pathophysiology of Neonatal RDS (Respiratory Distress Syndrome)

Answer 16

1. RDS affects premature neonates, born before the lungs start producing adequate surfactant
2. RDS commonly occurs below 32 weeks
3. CXR shows a “ground-glass” appearance
4. Inadequate surfactant → high surface tension within alveoli → atelectasis → inadequate gas exchange → hypoxia, hypercapnia and respiratory failure

Question 17

Explain surfactant, its chemical composition and how it helps with lung compliance and the factors that affect its production (Gestational age, Meconium aspiration syndrome, Steroid administration prior to delivery)

Answer 17

1. mixture of lipids and proteins which is essential for gas exchange at the fluid−air interface of the internal lung surface
2. hydrophobic and hydrophilic properties cause ↓ surface tension, thus keeping the alveoli open during the respiratory cycle
3. begins to be produced in the fetus at about 24 to 28 weeks of pregnancy. By ~35 weeks gestation, most babies have adequate amounts of surfactant
4. Pulmonary surfactant gets inactivated in babies who have MAS
5. surfactant after birth prevents/treats neonatal RDS in preterm delivery
6. Antenatal steroids for preterm labour ↑ the production of surfactant

Question 18

Summarize and describe the clinical manifestations of RDS

Answer 18

1. Signs of RDS appear within 4hr of birth
2. tachypnea (>60 breaths/min), intercostal + subcostal retractions, nasal flaring, grunting, and cyanosis
3. Tachypnea is d/t an attempt to ↑ Ve to compensate for a ↓ Vt and ↑ dead space
4. Retractions occur as the infant is forced to generate a ↑ intrathoracic pressure to expand the poorly compliant lungs.
5. The typical CXR shows low lung volumes and a bilateral, reticular granular pattern (ground glass appearance) with superimposed air bronchograms. In more severe cases, there is complete “white out” of the lung fields.

Question 19

General management for RDS

Answer 19

1. Avoid hypoxemia and acidosis
2. Optimize fluid management: avoid fluid overload and resultant body and pulmonary edema while averting hypovolemia and hypotension
3. Reduce metabolic demands and maximize nutrition
4. Minimize lung injury secondary due to volutrauma and oxygen toxicity

Antenatal corticosteroids (in an expected preterm birth)
Monitoring oxygenation and ventilation
Assisted ventilation of the neonate
Exogenous surfactant therapy
Supportive care, including thermoregulation, nutritional support, fluid and electrolyte management, antibiotic therapy, etc

Question 20

define and describe BLES surfactant

Answer 20

1. BLES = bovine lipid extract surfactant
2. natural extract of cow pulmonary surfactant
3. commonly used to treat RDS in premature infants in NICUs
4. studies show improved outcomes w/ animal‐derived surfactants vs synthetic products

Question 21

Which pathogens are associated with croup?

Answer 21

1. Human parainfluenza virus (HPIV) = 50% to 75% of cases

2. RSV, influenza A/B, and rhinovirus = remainder

Question 22

define MAS (Meconium Aspiration Syndrome) and identify the population most at risk for it

Answer 22

1. Meconium = 1st stool of newborn. sometimes meconium passed in uterus
2. MAS = neonatal respiratory distress w/ meconium-stained amniotic fluid (MASF)
3. can be mild distress to respiratory failure
4. MSAF is more common in POST-term newborns

Question 23

Detail the pathophysiology of MAS

💵💵💵MONEY SLIDE💵💵💵

Answer 23

1. Meconium passage
2. Aspiration (MSAF inhalation by the fetal airway)
3. Airway obstruction (meconium in the airways → meconium plugging → atelectasis)
4. Inflammation (meconium triggers inflammatory processes → chemical pneumonitis)
5. Surfactant inactivation: (Inflammation → Surfactant inactivation → increased surface tension w/ ↓ compliance, and ↓ oxygenation)

Question 24

Summarize and describe the clinical manifestations of MAS

Answer 24

1. Evidence of meconium-stained amniotic fluid (MSAF)
2. Perinatal asphyxia. These infants have neurologic and/or respiratory depression at birth typically due to hypoxia or shock
3. Fetal growth restriction and postmaturity – Affected infants are frequently small for gestational age and born postterm
4. Infants with MAS typically have respiratory distress with marked tachypnea and cyanosis immediately after birth → increased respiratory rate and use of accessory respiratory muscles of respiration demonstrated by intercostal and subxiphoid retractions and abdominal (paradoxical) breathing, often with grunting and nasal flaring
5. Affected infants typically have a barrel-shaped chest with an ↑ A/P diameter caused by overinflation. Auscultation reveals rales and rhonchi
6. In patients with severe MAS, pneumothorax and pneumomediastinum are common findings and other less common air leak syndromes can occur

Question 25

Define Early-onset neonatal pneumonia and identify the populations most at risk for it

Answer 25

1. Early-onset pneumonia (≤3 days), which is generally acquired from the mother during labor or delivery. 2. Early-onset bacterial pneumonia is most commonly caused by group B streptococcus. 3. Risk factors associated with early-onset pneumonia include prolonged rupture of the fetal membranes (>18 hours), maternal amnionitis, preterm delivery, fetal tachycardia, and maternal intrapartum fever 4. Early-onset pneumonia commonly presents with respiratory distress beginning at or soon after birth. 5. other clinical manifestations include temperature instability, apnea, lethargy, tachycardia, poor perfusion, tachypnea, and poor feeding.

Question 26

Define Late-onset neonatal pneumonia and identify the populations most at risk for it

Answer 26

1. Late-onset pneumonia (>3 days of age) is typically due to nosocomial organisms from previous colonization of the infant or transmission from care providers or contaminated equipment 2. Late-onset disease is associated with mechanical ventilation, airway anomalies, prolonged hospitalization, and aspiration of gastrointestinal contents due to neurologic impairment 3. clinical manifestations include temperature instability, apnea, lethargy, tachycardia, poor perfusion, tachypnea, and poor feeding

Question 27

why are neonates prone to developing pneumothorax?

Answer 27

1. Lack of surfactant makes premature lungs stiff and fragile. 2. The baby who requires BMV is at risk of having iatrogenic PTX (why we need to pay attention to the pressure that we are using)

Question 28

describe Tranillumination for pneumothorax

Answer 28

1. Place light source (otoscope, transilluminator) on infant’s chest. Ensure it isn’t hot. 2. A normal chest will have a small glowing “Halo” around the light source. Usually it extends less than 1 cm from the light source and is symmetric. 3. If the chest “lights up like a jack-o-lantern (large area of redness that is often asymmetric), then PTX should be HIGH on your DDx list. 4. You should compare to the other side if you are unsure.

Question 29

describe "Aspiration of pneumothorax" in the neonate

Answer 29

1. Traditionally used to relieve tension PTX. 2. In small children and neonates, may be the definitive procedure for simple PTX. 3. More compliant chest wall and elastic tissues in kids may allow for the hole to spontaneously seal after the procedure so that you don’t have to follow it up with a traditional chest tube. 4. If the PTX re-accumulates after aspiration, then a thoracostomy tube will need to be placed - Locate 2nd or 3rd intercostal space at the midclavicular line - Prep skin with antiseptic and provide analgesic and anesthetic - Advance needle perpendicularly over superior border of rib while aspirating. Having saline in syringe will allow you to detect when you’ve entered the ptx. - When bubbles are present or you have free movement of the syringe plunger, evacuate the pneumothorax. - A 3-way stopcock can be placed between syringe and butterfly needle, which will allow for controlled evacuation of pneumothorax.

Question 30

define Tracheoesophageal Fistula (TEF) + detail pathophysiology

Answer 30

1. Tracheoesophageal fistulas (TEFs) represent one of the most common congenital anomalies seen in major pediatric surgical centers 2. TEF occurs d/t abnormal septation of the caudal foregut during the fourth and fifth weeks of embryonic development 3. Under normal conditions, the trachea forms as a diverticulum of the foregut and develop a complete septum that separates it from the esophagus. 4. Fistula formation in conjunction with esophageal atresia (EA) occurs during an abnormal posterior positioning of the tracheoesophageal septum, resulting in a retained connection between trachea and esophagus.

Question 31

Summarize and describe the clinical manifestations of Tracheoesophageal Fistula (TEF)

Answer 31

1. clinical presentation of TEF depends upon the presence or absence of esophageal atresia (EA). 2. In cases w/ EA (95 percent), Infants w/ EA become symptomatic immediately after birth, with ↑ secretions → drooling, choking, respiratory distress, and inability to feed. 3. A fistula between trachea and distal esophagus → gastric distension. Reflux of gastric contents through the TEF → aspiration pneumonia and ↑ morbidity

Question 32

define Congenital Diaphragmatic Hernia (CDH)

Answer 32

1. Congenital diaphragmatic hernia (CDH) is a condition resulting from a developmental defect in the diaphragm → protrusion of abdominal contents into the thoracic cavity

Question 33

Detail the pathophysiology of Congenital Diaphragmatic Hernia (CDH)

Answer 33

1. The septum transversum and pleuroperitoneal membranes are the main components in the development of the diaphragm 2. Any disturbance in the formation of the pleuroperitoneal membranes can → diaphragmatic discontinuity and CDH 3. The associated herniation of the abdominal viscera into the thoracic cavity can interfere with normal lung development and forms the basis for the two main pathological findings noted in CDH - lung hypoplasia and abnormal pulmonary vascular development 4. Lung hypoplasia is reflected by a marked ↓ in the airway generations, terminal bronchioles, and alveoli. The hypoplasia is noted bilaterally with the lung on the same side of the defect affected more severely than the other lung. There is also an abnormal vascular remodeling of the pulmonary vasculature resulting in the thickening of the arterial medial walls with the potential to develop persistent pulmonary hypertension (PH) 5. Left ventricular hypoplasia and dysfunction, which can worsen the PH, are also noted in CDH TL;DR genetic shit causes the baby to have a fucked up diaphragm while in mom. D/t the fucked up diaphragm the abdominal contents get pushed up and fuck up lung development. These fuck up lungs → fucked up bronchioles, PH and infants with CDH most often present with respiratory distress in the first few hours or days of life

Question 34

Summarize & describe the clinical manifestations of CDH

Answer 34

1. respiratory distress in the first few hours or days of life. 2. usually acute respiratory distress at birth 3. degree of respiratory distress is dependent on the severity of lung hypoplasia and pulmonary hypertension (PH) 4. Adrenal insufficiency a common finding 5. Physical findings include a barrel-shaped chest, a scaphoid-appearing abdomen (d/t loss of the abdominal contents into the chest), and absence of breath sounds on the ipsilateral side

Question 35

Discuss the typical treatment plan for a patient with CDH

Answer 35

1. Intubation and ventilation 2. low PIP (PIP, goal <25 cm H2O) to minimize lung injury 3. NG tube w/ continuous suction to decompress abdominal contents and ↓ lung compression 4. UA line for frequent monitoring of ABG and blood pressure (BP), and UVC for administration of fluids + medications 6. BP support to maintain MAP ≥40 mmHg to minimize any right-to-left shunting

Question 36

Define BPD (Bronchopulmonary Dysplasia), the severities of BPD in the neonatal population born < 32 weeks and the neonatal population born > 32 weeks

Answer 36

1. The terms bronchopulmonary dysplasia and chronic lung disease of prematurity are used synonymously in practice and in the literature 2. Bronchopulmonary dysplasia is best thought of as a syndrome of lung immaturity, injury and inflammation, and a dysmature or dysregulated repair response, which → a persistent oxygen dependence and respiratory issues 3. Infants with birth weights (BW) <1250 g account for 97 percent of the cases of BPD. For extremely preterm (EPT) infants (gestational age [GA] <28 weeks), the incidence of BPD is approximately 40 percent, and the risk ↑ with decreasing GA

Question 37

define and detail BPD

Answer 37

disruption of fetal/neonatal lung development and injury d/t inflammation and damage from antenatal factors (intrauterine growth restriction, maternal smoking) and/or postnatal factors (eg, mechanical ventilation, oxygen toxicity, and infection)

Question 38

Summarize and describe the clinical manifestations of BPD

Answer 38

1. Infants with BPD usually are tachypneic 2. Depending upon the extent of pulmonary edema and/or atelectasis, they may have mild to severe retractions, and scattered rales may be audible. 3. Intermittent expiratory wheezing may be present in infants w/ airway narrowing from scar formation, constriction, mucus retention, collapse, and/or edema 4. As BPD evolves, the CXR also changes from clear lung fields to findings that include diffuse haziness and a coarse interstitial pattern, which reflect atelectasis, inflammation, and/or pulmonary edema 5. Patients with more severe BPD are hypoxemic and hypercapnic and typically require mechanical ventilation and oxygen supplementation. 6. They have abnormal pulmonary function including ↓ tidal volume, ↑ airway and vascular resistance, and ↓ dynamic lung compliance and uneven airway obstruction resulting in gas trapping and hyperinflation with abnormal distribution of ventilation

Question 39

Define bronchiolitis

Answer 39

1. bronchiolitis is a clinical syndrome of respiratory distress that occurs in children <2 years of age and is characterized by URI symptoms (eg, rhinorrhea) followed by lower respiratory (eg, small airway/bronchiole) infection with inflammation → wheezing and or crackles (rales) 2. Bronchiolitis is the most common lower respiratory tract infection under 2 years of age and the leading cause of hospital admission under 6 months of age.

Question 40

Detail the pathophysiology of bronchiolitis

Answer 40

1. Respiratory syncytial virus (RSV) is the culprit in up to 90% of cases of bronchiolitis. 2. RSV is nasty d/t the immune response to the virus: it binds to epithelial cells, replicates, and the submucosa becomes edematous and hypersecretes mucus. 3. RSV causes the host epithelia and lymphocytes to go into a frenzy – viral fusion proteins turn the membranes into a sticky goop – cells fuse into other cells, and you have a pile-on of multinucleated dysfunction. 4. This mucosal chaos → epithelial necrosis, destruction of cilia, mucus plugs, bronchiolar obstruction, air trapping, and lobar collapse

Question 41

Summarize and describe the clinical manifestations of bronchiolitis

Answer 41

1. typically affects kids <2yo during the fall and winter 2. Diagnosed clinically. viral URI prodrome followed by ↑ respiratory effort (eg, tachypnea, nasal flaring, chest retractions) and wheezing and/or crackles 3. Severe bronchiolitis is indicated by persistently ↑ respiratory effort (tachypnea; nasal flaring; intercostal, subcostal, or suprasternal retractions; accessory muscle use; grunting), hypoxemia, apnea, or acute respiratory failure

Question 42

Upper limit of normal respiratory rate by age (0mo, 6mo, 12mo)

Answer 42

Term Newborn → 50 breaths/minute 6 months → 40 breaths/minute 12 months → 30 breaths/minute

Question 43

List, explain and interpret the pertinent laboratory and diagnostic tests used to diagnose bronchiolitis

Answer 43

1. Clinical diagnosis. Recommend NO testing in the initial workup as per AAP guidelines. Wait and see how the child does with standard therapy for bronchiolitis. 2. CBC → Not sensitive or specific in kids for bacterial versus bacterial illness 3. Chem-7 → History is a better way to assess hydration status. If worried about significant hydration, consider getting a sodium. 4. Blood Cultures → If a patient is septic, very ill, or is premature (higher risk for group B strep), consider a blood culture 5. Chest X-ray → If a child looks profoundly sick, goes to the ICU, or is intubated, a CXR may be appropriate and you can expand your differential diagnosis

Question 44

Discuss your treatment plan for a patient with bronchiolitis

Answer 44

1. Oxygen goal → AAP guidelines say 90% 2. Ventolin → May consider it on a case-to-case basis, including if a child is getting worse or older/atopic infants 3. Steroids → Clinicians should not administer systemic corticosteroids to infants with a diagnosis of bronchiolitis in any setting (the only guide line with evidence quality A) 4. High Flow Nasal Cannula → Provides PEEP; Blows off CO2 from dead space; Improves pulmonary mechanics from hydration; Provides warmth 5. Hydration: NG Tube vs IV fluids? → Prefer NG tube

Question 45

Define pertussis

Answer 45

1. AKA "whooping cough," bacterial RTI caused by Bordetella pertussi (Gram-negative bacteria) 2. typically occurs in unvaccinated children. has three stages: a catarrhal stage, a paroxysmal and a convalescent stage

Question 46

Detail the pathophysiology of pertussis

Answer 46

1. Bordetella is a gram-negative bacteria that adheres to respiratory epithelium. 2. Local inflammation occurs in the mucosal lining of the respiratory tract. 3. Released toxins (pertussis toxin) act locally and systemically

Question 47

Summarize and describe the clinical manifestations of pertussis

Answer 47

1. incubation period of 1 to 3 weeks, then the infection has three distinct stages: catarrhal phase, paroxysmal phase, convalescent phase 2. The catarrhal is similar to other URI's, w/ fever, fatigue, rhinorrhea, and conjunctival injection. lasts 1 to 2 weeks 3. paroxysmal phase is characterized by whooping cough. patient typically coughs repeatedly, followed by forceful inspiration which creates the characteristic “whoop.” 4. during convalescent phase, a residual cough persists for weeks to months, usually triggered by exposure to irritants

Question 48

Discuss your treatment plan for a patient with pertussis

Answer 48

1. Treatment of pertussis is largely supportive, including oxygen, suctioning, hydration, and avoidance of respiratory irritants. Parenteral nutrition may be necessary as the disease tends to have a prolonged course. 2. Antibiotic effect on the duration or severity of the disease is minimal when started in the catarrhal phase, and not proven effective when started in the paroxysmal phase. Rather, the primary goal of antibiotic treatment is to decrease carriage and spread of disease 3. Corticosteroids have not shown definite benefit in reducing severity and course of illness, but are sometimes given to critically-ill infants. 4. Beta2-agonists, pertussis immune globulin, cough suppressants, and antihistamines are not effective

Question 49

croup mimics

Answer 49

Foreign body aspiration Bacterial tracheitis Epiglottitis Retropharyngeal abscess.

Question 50

Peak age for croup

Answer 50

6 mo–3 yr

Question 51

Peak age for epiglottitis

Answer 51

5–7 yr, but can be seen throughout childhood

Question 52

main organisms for croup

Answer 52

RSV, Parainfluenza virus, adenovirus, influenza

Question 53

Key Clinical features for epiglottitis

Answer 53

Rapid progression of high fever, toxicity, drooling, stridor

Question 54

Key Clinical features for croup

Answer 54

Onset follows URI prodrome consisting of croupy cough, hoarse voice, low-grade fever, inspiratory stridor

Question 55

Laboratory and radiographic findings for epiglottitis

Answer 55

CXR Thumbprint sign on lateral aspect of the neck, thickened aryepiglottic folds, loss of air in the vallecula

Question 56

Laboratory and radiographic findings for croup

Answer 56

Steeple sign on PA view of the neck or normal

Question 57

List 5 Xray findings of epiglottitis

Answer 57

``` Thumbprint sign (big epiG!) Thickened aryepiglottic folds Lack of air in the vallecula Dilated hypopharynx NORMAL (70% of xrays) ```

Question 58

What is the culprit pathogen in whooping cough (pertussis) ?

Answer 58

Bordetella pertussis

Question 59

What are the stages of whooping cough?

Answer 59

Catarrhal stage paroxysmal stage convalescent stage.

Question 60

What are three clinical complications of cystic fibrosis?

Answer 60

Progressive lung tissue disease – scarring, etc. Recurrent infections By age 18 – 80% of patients with CF are colonized with pseudomonas Pancreatic insufficiency / recurrent pancreatitis Cystic fibrosis-related diabetes (CFRD) Infertility Vitamin deficiencies, osteoporosis

Question 61

What is the pathophysiology of CF?

Answer 61

Autosomal recessive disease: 1. Defects in chloride transport across the airway epithelium → reduced ciliary clearance of thickened mucus, ↓ antimicrobial effect of the airway surface, ↑ bacterial adherence, and innate secretion of inflammatory cytokines. 2. Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Deranged chloride transport leads to thick, viscous secretions in the lungs, pancreas, liver, intestine, and reproductive tract.

Question 62

Westley Croup Score

Answer 62

Quantifies croup severity

Question 63

PRAM scoring tool for asthma

Answer 63

1. Measures severity of airway obstruction in pediatric patients using clinical observations 2. When to use: Pediatric patients (3-17 years old) with asthma exacerbations

Question 64

mL/kg/min for pediatric patient mech vent

Answer 64

100-200mL/kg/min

Question 65

mL/kg/min for neonatal patient mech vent 💵💵💵MONEY SLIDE💵💵💵

Answer 65

200-300mL/kg/min

Question 66

steps to calculating vent parameters in kids 💎💎💎MEGA PEARL💎💎💎

Answer 66

1. calculate IBW 2. determine VE by multiplying IBW by ideal VE in mL/kg/min 2. divide VE by determined Vt (in mL/kg). this will give you your Resp Rate 3. divide 60 (seconds) / RR to give you your Tct (total cycle time) 4. determine your desired I:E via your Tct, this will give you your Ti 5. throw in FiO2 and PEEP and voila! Sooooo easy

Question 67

lung protective volumes in paediatrics (ml/Kg)

Answer 67

6-8 mL/kg

Question 68

lung protective volumes in neonates (ml/Kg)

Answer 68

4-6 mL/kg

Question 69

key features for TTN

Answer 69

1. Delay in fluid clearance from the lungs. Increased in C-Section or precipitous delivery. 2. Usually mild to moderate WOB with FiO2 less than 40% 3. Fluid apparent in the fissures on x-ray 4. Gradually improves over hours to days

Question 70

key features for neonatal Respiratory Distress Syndrome

Answer 70

1. D/t lack of surfactant 2. Primary disease of preterm babies 3. Present w/ ↑ WOB and ↑ FiO2 requirement 4. Will worsen for the first 72 hours then improve if no surfactant administered

Question 71

key features for Meconium Aspiration Syndrome

Answer 71

1. Perinatal Aspiration of meconium leading to small airway obstruction, pneumonitis, surfactant inactivation and V:Q mismatching. 2. A disease of the post term infant 3. May present with variable degree of respiratory distress 4. May disrupt transition leading to PPHN

Question 72

key features for neonatal Pneumothorax

Answer 72

1. Air leak within the pleural space 2. Acute increased in respiratory distress 3. Primarily in babies requiring respiratory support but can occur spontaneously

Question 73

key features for neonatal Pneumonia

Answer 73

1. Inefctious infiltrate of the lungs 2. More likely to occur in the presence of risk factors 3. May not present as systemically ill at onset but can progress to fulminant quickly 4. Due to an inability to rule out via clinical or radiographic it is recommended for all babies with RDS receive ABx

Question 74

key features for neonatal PPHN 💵💵💵 MONEY SLIDE 💵💵💵

Answer 74

1. PVR>SVR leading to a right to left shunt 2, Can be associated with MAS, RDS, CDH, Pneumonia or a disturbance of transition 3. Presents as hypoxic respiratory failure 4. Preductal SpO2 greater than Postductal 5. Right to Left Shunts through PDA, PFO and a component of tricuspid insufficiency due to right ventricular dysfunction

Question 75

who do we ventilate at Minute Ventilation of 200mL/min/kg

Answer 75

Newborn <1mo

Question 76

who do we ventilate at Minute Ventilation of 175mL/min/kg

Answer 76

Infant 1mo to 1yo

Question 77

who do we ventilate at Minute Ventilation of 150mL/min/kg

Answer 77

Toddler 1 to 3yo

Question 78

who do we ventilate at Minute Ventilation of 125mL/min/kg

Answer 78

child 3yo to 10yo

Question 79

who do we ventilate at Minute Ventilation of 100mL/min/kg

Answer 79

adolescent >10yo

Question 80

Describe persistent pulmonary hypertension of the newborn

Answer 80

1. “Unproportionally sick compared to the CXR”. 2. PVR is normally high in early gestation. Pressure falls gradually beyond the 2nd trimester, with a dramatic drop with the initiation of ventilation after birth. 3. PPHN is characterized by elevated PVR w/ "failure to transition", resulting in right-to-left shunting of blood and hypoxemia

Question 81

What are the contributing factors to the fall of PVR immediately post birth?

Answer 81

1. ↑ in alveolar and arterial O2 tension. Fetal pulmonary circulation becomes more responsive to the vasodilator effect of oxygen after 31 weeks. 2. ↑ in production of vasodilators 3. Rhythmic distension of the lungs

Question 82

Explain the regulation of pulmonary tone 🌿🌿🌿DANK PATHO🌿🌿🌿

Answer 82

1. NO is produced by the endothelium and is metabolized into cGMP, which produces vasodilation. 2. cGMP is degraded into GMP by the enzyme PDE5. cGMP degradation can be ↓ by PDE5 inhibitors (such as sildenafil). 3. PGI2 is metabolized into cAMP, which produces vasodilation. 4. cAMP is degraded into AMP by the enzyme PDE3. cAMP degradation can be ↓ by PDE3 inhibitors (such as milrinone)

Question 83

Outline the management of PPHN

Answer 83

1. Sedation to facilitate MV 2. PGE 3. NO 4. Milrinone

Question 84

List some of the respiratory differences seen in children

Answer 84

1. Cone-shaped narrow trachea (narrowest at cricoid cartilage). 2. Larger tongue. 3. Softer pallate. 4. Lower FRC (contributing to quicker desaturation, along with ↑ basal metabolic rate). 5. Immature sinuses 6. Toothless, but at risk of tooth bud injury 7. Obligate nose breathers. 8. ↑ Compliant chest wall

Question 85

What is Curosurf derived from?

Answer 85

Pig surfactant

Question 86

What is a histogram?

Answer 86

A graph that is used to determine time at specific oxygen saturations

Question 87

Describe transient tachypnea of the newborn (TTN)

Answer 87

1. Respiratory distress associated to inadequate fetal lung fluid clearance. 2. Transient in nature, generally resolves within 24hr

Question 88

Describe neonatal respiratory distress syndrome (RDS)

Answer 88

Respiratory distress caused by surfactant deficiency

Question 89

Describe persistent pulmonary HTN of the newborn (PPHN)

Answer 89

1. Pulmonary HTN caused by abnormal presence of ↑ PVR → right-to-left shunting of deoxygenated blood through the PFO and PDA. 2. PPHN is often caused by underdevelopment of the pulmonary vascular bed, and can also be caused by fetal distress, such as in MAS

Question 90

Define meconium aspiration syndrome (MAS)

Answer 90

1. Fetal distress → meconium voiding w/ subsequent aspiration 2. Meconium layers the respiratory tract → chemical irritation, inflammation, infection, and surfactant inactivation. 3. MAS → bilateral diffuse grossly patchy opacities. The areas of the CXR are patchy because of areas of total atelectasis, as well as areas of air trapping within the alveoli.

Question 91

Why is meconium voided during fetal distress?

Answer 91

↑ vagal outflow from umbilical cord compression or ↑ sympathetic outflow from hypoxia → peristalsis and relaxation of the anal sphincter

Question 92

Define congenital diaphragmatic hernia (CDH)

Answer 92

1. Herniation of the abdominal contents into the thorax. | 2. CDH compresses the lung parenchyma → maldevelopment and PPHN

Question 93

What are congenital pulmonary airway malformations (CPAM)?

Answer 93

Fistula development between the airways and surrounding structures, particularly affecting the trachea and esophagus

Question 94

What are the equations for calculating ETT size in paediatrics? (cuffed and uncuffed)

Answer 94

Age/4 + 3 (cuffed) Age/4 + 4 (uncuffed)

Question 95

What is the equation for calculation ETT depth?

Answer 95

ETT size x 3