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MCC Part 1 > Pediatrics > Flashcards

Pediatrics Flashcards

1
Q

Timeline of newborn assessment in first 2yr of life?

A

Initial assessment within 24hr birth
Within 1 week post-d/c (if newborn exam at hospital)
2, 4, 6, 9, 12 mo
15, 18, 24 mo

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2
Q

Should all infants receive Vitamin D?

A

All exclusively breast-fed infants should have supplemental Vitamin D, minimum 400 IU/d

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3
Q

Apgar score

A

HR: 0 (absent), 1 (<100 bpm), 2 (>100 bpm)

Respiratory effort: 0 (absent), 1 (weak cry, hypoventilation), 2 (good effort, crying)

Muscle tone: 0 (flaccid), 1 (some flexion), 2 (well flexed, active)

Reflex irritability: 0 (no response), 1 (grimace), 2 (cry/ cough/ sneeze or withdrawal)

Color: 0 (blue/pale), 1 (acrocyanotic), 2 (completely pink)

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4
Q

How long to exclusively breastfeed?

A

6 mo of life

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5
Q

Will EtOH and recreational drugs enter breast milk?

A

Yes

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6
Q

Contraindications to breastfeeding?

A
  • HIV
  • Active TB (until 2wk of appropriate tx complete)
  • chemotherapy/nuclear medicine
  • high-dose metronidazole (12-24hr after dose)
  • illicit drug use
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7
Q

Ddx delayed passage of meconium?

A

> 24hr after birth

  • Hirschsprung disease
  • Meconium plug/ileus
  • CF
  • Anal stenosis
  • Anal atresia
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8
Q

Investigations jaundice in 24h life

A 
Inv
- bilirubin (total, direct, indirect)
- DAT (Coombs test)
- ABO group (if mother blood group O)
- minor group antigen screen
- Rh status (and if RhoGAM was given)
- assess for sepsis with consideration of FSWU
\+/- risk factors (G6PD deficiency)
- assess feeding and hydration 
- signs birth trauma (cephalohematoma, bruising)
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9
Q

If newborn has central cyanosis what kind of saturation’s do you want to get?

A

Pre and post-ductal saturations

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10
Q

What do you want to rule out in newborn with pallor?

A
  • anemia
  • asphyxia
  • shock
  • edema
  • blood loss into head from birth trauma
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11
Q

Define SGA and LGA babies

A 
SGA = BW <10th percentile
LGA = BW >90th percentile
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12
Q

Weight loss red flags in newborn?

A
  • loss >10% BW in first 7d life

- failure to regain BW by 10d of life

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13
Q

Define microcephaly and macrocephaly

A
  • microcephaly: >2 SD below mean (<3rd percentile) for gestational age
  • macrocephaly: >2 SD above mean (>97th percentile) for gestational age
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14
Q

Workup bulging fontanelle in newborn?

A
  • assess for signs ICP, fever, signs of meningitis, neurologic deficits
  • head US
  • CBC, blood/urine cultures, glucose, electrolytes, urea, Cr
  • consider transfer to tertiary centre
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15
Q

Types of head trauma in newborn?

A
  • caput succedaneum: diffuse edema (may cross midline and suture liens), may be ecchymotic
  • cephalohematoma: limited to surface of one cranial bone, no overlying discolouration, firm tense mass with palpable rim, presents after birth with gradual increased size
  • subgaleal hemorrhage: firm, diffuse, fluctuant mass that increases in size after birth (bleeding can be extensive into large potential space beneath epicranial aponeurosis)

-> monitor frequent vitals/assessments for perfusion, head circumference, hypotension, hyperbilirubinemia, blood loss (Act), or consumptive coagulopathy (DIC)

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16
Q

What is abnormal red reflex? Management?

A
  • white reflex (leukocoria), dark sports, absent reflex, asymmetric reflex
    Urgent referral to ophthalmologist to r/o tumor (e.g. retinoblastoma), cataracts, other pathology of lens, vitreous or retina
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17
Q

Is high arched palate normal in newborn?

A
  • may be syndromic - refer
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18
Q

What are normal neonatal murmurs?

A
  • persistent pulmonary flow murmur

- transient systolic murmur re: closing PDA

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19
Q

What are features consistent with pathologic murmur?

A
  • diastolic/pansystolic murmurs
  • harsh
  • radiation
  • no change with position
  • abnormal S2
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20
Q

Cardiac red flags in neonate?

A
  • murmur
  • cyanosis
  • FTT
  • tachypnea
  • diaphoresis with feeding
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21
Q

Workup murmur/ cardiac red flags in neonates?

A
  • 4 limb BPs
  • pre (right arm) and post-ductal O2 saturation
  • ECG
  • ECHO
  • referral to paediatric cardiologist
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22
Q

Workup increased RR without increased work of breathing (effortless tachypnea) in neonate?

A 
- r/o CHF, acidosis, sepsis
Inv
- VBG
- lactate
- CXR 
- look for clinical signs congenital heart disease and sepsis
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23
Q

Workup increased work of breathing and/or expiratory grunting in neonate?

A
  • *immediate
  • investigate signs of serious cardiopulmonary disease, sepsis
  • VBG
  • lactate
  • CXR
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24
Q

Workup palpable mass in neonate?

A

US to look for etiology

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25
Q

Normal palpable liver in neonate?

A
  • up to 2cm below costal margin

+/- spleen tip

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26
Q

Workup abdominal distention, and/or scaphoid abdomen and respiratory distress in neonate?

A
  • AXR to r/o obstruction (meconium ileus if shortly after birth), perforation
  • CXR to r/o congenital diaphragmatic hernia
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27
Q

What umbilical sign increases risk of occult renal abnormality?

A

2 vessel cord (1 artery + 1 vein)

- monitor and consider US

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28
Q

Management of ambiguous genitalia?

A

Urgent evaluation and referral -> can be due to life-threatening etiology

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29
Q

Management clitoromegaly in female neonates?

A
  • r/o congenital adrenal hyperplasia (can be life threatening)
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30
Q

Management/workup imperforate anus?

A
  • X-ray as initial investigation

- surgical management

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31
Q

Workup hip click on neonatal exam?

A

Ortolani and Barlow -> +
= US to look for DDH

  • note hip click can be normal, originating from fascia and/or tendons
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32
Q

Workup congenital scoliosis?

A

Investigations for associated pathologies

  • US for renal pathology
  • cardiac exam +/- ECHO
  • MRI for possible intraspinal pathology
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33
Q

Management hair tuft/dimple over lumbosacral spine?

A
  • investigate underlying pathology (occult spina bifida, tumor, sinus tract)
  • US
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34
Q

Name normal neonatal rashes

A
  • erythema toxicum
  • pustular melanosis
  • dermal melanocytosis (Mongolian spots)
  • harlequin color change
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35
Q

Management abnormal irrepressible movements, seizures in neonate?

A
  • EEG
  • paediatric neurologist assessment
    +/- genetics/ metabolic
  • consider MRI brain
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36
Q

Workup hyper/hypotonia in neonate?

A
  • paediatric neurology

- genetics/metabolics consults

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37
Q

Ddx asymmetric Moro reflex?

A
  • clavicle fracture
  • brachial plexus injury (birth trauma)
  • hamiparesis
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38
Q

Management absent Moro in term neonate?

Management obligatory ANTR (neonate becomes stuck in position)?

A

Suggests significant CNS dysfunction - full neurologic evaluation

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39
Q

What are the primitive neonatal reflexes?

A
  • grasp
  • suck
  • Moro
  • ANTR
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40
Q

Normal vitals in newborn (term)?

A

Wt 3-4kg
HR 90-170 bpm
RR 40-60 /min
sBP 70-90 mmHg

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41
Q

Define fever in neonate and management

A

Rectal 38.0
Axillary 37.3

FULL septic workup (CBC, blood C+S, urinalysis, urine C+S, lumbar puncture +/- CXR)

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42
Q

When do you do routine newborn screening?

A
  • within first 7d life (ideally first 2-3d)
  • varies by province -> inborn errors of metabolism, hemoglobinopathies, CF
    + additional screening re: risk factors

+ routine hearing screening
+ assessment of jaundice (+/- serum bilirubin) and feeding within first 24h of life

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43
Q

When does umbilical cord detach?

A
  • 1-3wk after birth -> keep clean and dry, wash with water only
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44
Q

Safe sleep re: SIDs prevention

A
  • sleep on back in empty crib first first year of life
    Back to sleep, front to play
  • room sharing reduces risk of SIDS
  • bed sharing and tobacco exposure increase risk of SIDS
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45
Q

Typical weight gain in infants

A

180g/wk until 4-5mo (1oz per day except Sundays)
2x BW by 4-5mo
3x BW by 1yr
4x BW by 2yr

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46
Q

What is omphalitis?

A

Umbilical cord infection

  • fever
  • purulent d/c
  • redness and swelling
  • foul odor
  • bleeding (more than few drops)
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47
Q

What is most important risk factor for infant mortality + significant determinant of infant and childhood morbidity?

A

Low birth weight

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48
Q

Complications of SGA?

A
  • difficult cardiopulmonary transition
  • complications of prematurity
  • impaired thermoregulation
  • hypoglycemia
  • polycythemia (hypoxia = increased EPO)
  • impaired immune function
  • perinatal mortality
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49
Q

List complications of prematurity

A
  • RDS (resp distress syndrome)
  • ROP (retinopathy of prematurity)
  • intraventrivular hemorrhage (IVH)
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50
Q

Complications of LGA

A
  • increased risk of cesarean delivery, severe postpartum hemorrhage, vaginal lacerations
  • birth injury (brachial plexus, shoulder dystocia, clavicular #)
  • respiratory distress (RDS if mom DM, TTM if born c/s, meconium aspiration)
  • perinatal asphyxia
  • hypoglycaemia
  • polycythemia (hyperinsulemia -> increased demands -> hypoxia -> increased EPO)
  • increased perinatal mortality
  • minor congenital anomalies
  • propensity adult obesity
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51
Q

Define IUGR

A
  • fetus hasn’t reached growth potential because genetic or environmental factors, resulting in SGA infant
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52
Q

What is Barker hypothesis?

A
  • adverse stimuli or events occurring in utero and during infancy can permanently change body’s structure, physiology and metabolism, which can influence occurrence of many diseases that will develop in adulthood
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53
Q

Rule to calculating fundal height after 12wk GA?

A
  • fundal height (cm) = week gestation +/- 2
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54
Q

Classification of IUGR?

A
  • symmetric: symmetric reduction in anthropometric measurements, usually due to early gestational insult
  • asymmetric: reduced body weight with relatively normal length and head growth, usually due to gestational insult affecting growth in late 2nd/ 3rd trimesters
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55
Q

How do you screen for and diagnose SGA/IUGR?

A

prenatal US estimation of fetal weight

- also assesses biometrics (e.g. biparietal diameter, femur length) and amniotic fluid volume

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56
Q

When is fetal karyotyping recommended for IUGR?

A
  • early (<32wk)
  • severe (<3rd percentile)
  • accompanied by polyhydramnios or structural anomalies
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57
Q

What maternal assessments are indicated if IUGR recurrent, early, severe, or postive FHx thrombophilia?

A
  • maternal thrombophilic disorders assessment
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58
Q

Abnormal weight in newborn investigations

A
  • CBC
  • blood gases (hypoxia -> acidosis)
  • glucose (hypoglycaemia)
  • chemistry (hyperbilirubinemia, hypoCa)
  • blood/urine/CSF cultures pro
  • drug screen
    +/- genetics, other testing
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59
Q

Ballard score (GA assessment)

A
  1. extent that sole of foot covered in creases
  2. presence and size of breast buds
  3. features of scalp hair
  4. formation of ear cartilage (pinna recoil)
  5. appearance of genitalia
  6. neurologic assessment of posture, active and passive tone, and reflexes
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60
Q

Use of Doppler of umbilical artery in utero?

A
  • identify small fetus at risk for adverse perinatal outcomes (preterm birth, NICU admission, asphyxia, etc)
  • > NOT useful for screening and dx of IUGR
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61
Q

Which is often pathologic - conjugated or unconjugated hyperbilirubinemia?

A
  • conjugated hyperbilirubinemia (>20% total bilirubin conjugated) is always pathologic
  • -> must investigate
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62
Q

How is bilirubin produced?

A
  • catabolism of hemoglobin
    hemoglobin -> unconjugated bilirubin (not water soluble)
    –> to liver –> UDP-GT conjugates bilirubin (water soluble)
  • conjugated bilirubin broken into urobilinogen and stercobilinogen -> excreted in stool and (lesser degree) urine
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63
Q

Define kernicterus + sx

A
  • neurologic outcome of bilirubin deposition in basal ganglia and brainstem nuclei
  • result of elevated unconjugated hyperbilirubinemia

early sx: lethargy, poor suck, hypotonia, high-pitched cry, seizures
late sx: irritability, hypertonia, opisthotonos, fever

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64
Q

Risk factors severe hyperbilirubinemia

A
  • jaundice within first 24h life
  • blood group incompatibility (DAT +)
  • late preterm infants (35-36 + 6wk GA)
  • cephalohematoma
  • sibling requiring phototherapy
  • exclusively breast-fed
  • east Asian race
  • G6PD deficiency
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65
Q

Risk factors neonatal sepsis

A
  • rupture membranes >18h before delivery
  • maternal fever (>38) during labor
  • chorioamnionitis
  • maternal GBS colonization (i.e. GBS UTI)
  • prior delivery of infant with GBS disease
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66
Q

Ddx unconjugated hyperbilirubinemia

A

Increased production

  • extravascular blood (cephalohematoma)
  • polycythemia
  • red cell instability (G6PD, spherocytosis, etc.)
  • Coombs positive (isoimmunization - Rh, ABO, minor antigens)

Decreased conjugation (UDP-GT deficiency)

  • premature
  • Gilbert syndrome
  • Crigler-Najjar syndrome
  • Congenital hypothyroidism

Increased reuptake (enterohepatic circulation)

  • breast-feeding jaundice (secondary to dehydration)
  • bowel obstruction (meconium ileus, etc)
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67
Q

Ddx conjugated hyperbilirubinemia

A 
Sepsis
- Intrauterine infection (TORCH)
= toxoplasmosis
= other: syphilis, EBV
= rubella
= cytomegalovirus
= herpes, HIV

Hepatic

  • biliary atresia
  • Alagille syndrome
  • disorders of bile acid metabolism
  • neonatal hepatitis
  • choledochal cyst
  • underlying metabolic condition (Galactosemia, tyrosinemia)
  • infiltrative (Wilsons, alpha 1 antitrypsin deficiency)
  • TPN-related cholestasis
  • CF
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68
Q

What is ABO isoimmunization?

A
  • ABO incompatibility (mother blood type O, baby blood type A or B) is important cause of hemolytic disease of newborn that must be ruled out
  • newborn with rapidly increasing bilirubin levels approaching levels for exchange transfusion, IVIG should be considered
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69
Q

Red flags for jaundice?

A
  • <24h or >2wk age
  • rate of rise of bilirubin >85 micro mol/24h
  • toxic appearance
  • risk factors for neonatal sepsis
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70
Q

Management of bilirubin level indicating treatment on nomogram?

A
  • phototherapy +/- IVIG, IV hydration
  • monitor serial serum bilirubin q6-8hr

nomogram re: risk factors and GA -> placed on low, intermediate or high risk zone

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71
Q

Routine jaundice investigations

A
  • CBC
  • blood smear
  • total and direct bilirubin (absolute values and rate of rise)
  • ABO blood type
  • Coombs test
  • electrolytes

+ specialized tests

  • G6PD
  • sickle cell screen
  • hemoglobinopathy screen
  • reticulocyte count
  • sepsis workup
  • metabolic evaluation (galactosemia screen, TSH, free T4)
  • abdo US
  • hepatobiliary imilodiacetic acid (HIDA) scan
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72
Q

Phototherapy treatment for jaundice

A
  • photoisomerization of unconjugated bilirubin to water soluble isomers
  • safe for mild to moderate hyperbilirubinemia

CI conjugated hyperbilirubinemia (bronze baby)

  • potential for burns, retinal damage
  • separation of infant and parents can be disadvantage
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73
Q

Pharmacologic tx jaundice

A
  • IVIG - for isoimmune hemolytic disease
  • heme-oxygenate inhibitors - for metalloporphyrins
  • may reduce need for exchange transfusion
  • unclear efficacy, long term effects
  • may have sedative effects
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74
Q

Exchange blood transfusion for jaundice

A
  • reserved for dangerously high levels or pt with sx kernicterus
  • removes and replaces partially hemolyzed and antibody-coated erythrocytes
  • most rapid method of tx

disadvantages

  • NEC
  • metabolic acidosis
  • thrombocytopenia
  • coagulopathy
  • arrhythmias
  • infection
  • death
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75
Q

3 types of vaccines

A
  • live attenuated: whole/weakened bacteria or virus; usually produce immunity with 1 dose
  • whole inactivated: contain whole or part-killed bacteria or virus; does not cause disease it is designed to prevent; usually require >1 dose
  • subunit: organism parts, protein/toxoid, polysaccharide +/- conjugate
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76
Q

What is active immunization?

A
  • complex biologic products designed to induce protective immune response
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77
Q

What is passive immunization?

A
  • prevent immunization: prevent infection and reduce severity of illness; used when vaccines for active immunization not available or contraindicated, unimmunized people exposed to agent, or in immunocompromised individuals
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78
Q

Do vaccinations work on individuals or population levels?

A

Both

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79
Q

Additives in vaccines?

A
  • adjuvant (aluminum salt) to enhance immune response
  • preservatives (thimerosal) to prevent serious secondary infections
  • others (egg, animal protein, glycerol, formaldehyde) support stability and growth of antigens
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80
Q

Side effects of vaccines?

A
  • common: fever, tenderness/swelling at injection site, tiredness, poor appetite, emesis
  • MMR/VZV: ‘pseudo-infection’ (measles-like rash, parotitis, lymphadenopathy, arthralgia/ arthritis, mild varicella-like papules/vesicles), 1/40000 thrombocytopenia, 1/25000 febrile seizure
  • DTaP: 1/14000 seizure, 1/1000 prolonged crying (>3h)
  • occasionally more serious reactions (anaphylaxis, allergy)
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81
Q

NACI vaccination schedule

A

2 mo

  • DTap-IPV-HiB
  • rotavirus
  • Men-C
  • Pneum-C-13

4mo

  • DTap-IPV-HiB
  • rotavirus
  • Men-C
  • Pneum-C-13

6mo

  • DTaP-IPV-HiB
  • rotavirus
  • Men-C
  • Pneum-C-13

6-59 mo
- influenza (seasonal)

12mo

  • MMR
  • Varicella
  • Men-C
  • Pneum-C-13

18mo

  • DTaP-IPV-HiB
  • MMR
  • varicella

4-6yr

  • DTaP- IPV
  • MMR
  • varicella

Preteen

  • Men-C-A, C, Y, W-135
  • hepatitis B
  • HPV

14-16yr
- Tdap or Tdap-IPV

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82
Q

Immunizations with asplenia/hyposplenic (congenital, surgical, functional)

A
  • no CI to vaccines
  • should receive influenza annually
  • coverage of encapsulated organisms (Hib, N. meningitidis, S. pneumonia)
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83
Q

BCG vaccine?

A
  • infants of parents with infectious TB at delivery

- high risk populations (First Nations)

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84
Q

Indication for RSV immune globulin?

A
  • children <24mo with prematurity (born <32wk GA), chronic lung disease, heart disease, or living in rural or remote location
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85
Q

Do you base timing of immunizations based on chronological or gestational age for premature infants?

A
  • chronological age
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86
Q

Contraindications to vaccinations?

A

Previous anaphylactic reaction to vaccine or component
- components: gelatin (MMR), egg (influenza), yeast (hep B), streptomycin (DTaP)

Live vaccines: severe asthma/wheezing, pregnancy, severe immunodeficiency or active immunosuppression therapy, TB (MMR, MMRV, univalent varicella, herpes zoster, BCG)

Rotavirus: congenital malformation of GI tract or intussusception

Influenza: not to pt who had ocluo-respiratory syndrome after prior influenza vaccine

Relative CI:

  • Guillain-Barre syndrome within 6wk vaccination
  • moderate/severe illness
  • rotavirus - moderate-severe gastroenteritis
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87
Q

How long do you delay live vaccination in patient who received antibody-containing blood products?

A

3-6mo (can interfere with endogenous antibody response)

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88
Q

Normal crying pattern baby?

A
  • average 3h/d by 6wk of age

- peak time between 3-11pm

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89
Q

How is infantile colic dx?

A

dx of exclusion

- benign, self-limited condition beginning in first weeks of life and peaking during 2nd and 3rd month of life

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90
Q

Rome criteria for infantile colic

A

occurs in infants <4mo of age with:

  1. paroxysmal without obvious cause
  2. no effect on infants growth and development (no FTT)
  3. lasts >3hr/d, >3d/wk (for >1wk)
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91
Q

Infant crying emergencies to rule out?

A
  • hair tourniquet: remove tourniquet immediately and ensure blood flow returns
  • testicular torsion: US doppler, urology referral
  • glaucoma: refer ophthalmologist
  • malrotation/volvulus: abdo plain films, gen surg
  • intussusception: abdo US, air enema, surgery
  • incarcerated/strangulated hernia: dx based on exam, inability to reduce hernia, consider US, gen surg
  • septic arthritis: consider joint aspiration if clinical exam suspicious, abx
  • abusive head trauma: head imaging, skeletal survey, involve child services and team for suspected cases of abuse
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92
Q

Management of colic

A
  • educate parents, support
    evidence conflicting
  • hypoallergenic diet in mother if breast fed
  • hypoallergenic formula if formula fed (e.g. hydrolyzed casein/ whey protein and amino acid based)
  • probiotics/ probiotics
  • alternative therapies
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93
Q

Full septic workup in neonate (<28d) with fever?

A

Yes

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94
Q

Measurement of temperature in peds

A

definitive

  • <5yr = rectal
  • > 5yr = oral

rectal temp >38 fever

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95
Q

Normal temperature ranges

A
  • rectal 36.6 - 38
  • tympanic 35.8 - 38
  • oral 35.5 - 37.5
  • axillary 34.7 - 37.3
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96
Q

What dx is considered in subacute or chronic fevers (>2wk duration)?

A

inflammatory or malignant aetiologies
vs. acute fever (<2wk) often infectious

inflammatory
- consider autoimmune: SLE, JIA (salmon-pink rash)

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97
Q

What is a pyrogen?

A
  • substance that produces fever
  • portion of viruses or bacteria (lipopolysaccharide) or components of innate and active immune system (complement, antigen-antibody complexes)
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98
Q

What is Reye syndrome?

A
  • encephalopathy and fatty degeneration of liver (significant morbidity and mortality)
  • ASA in kids with varicella or influenza (NO aspirin to kids with febrile illness)
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99
Q

Where is the thermoregulatory centre? What can change the target range?

A
  • hypothalamus -> maintains body temp in certain range

- cytokines and pyrogens can alter target range

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100
Q

What is caused by increased cellular metabolism, i.e. involuntary skeletal muscle shivering?

A

increased heat production

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101
Q

What mediates the increase in set point of the thermoregulatory centre (targeting by antipyretics - NSAIDs, acetaminophen)?

A

PGE2

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102
Q

Mechanism of antipyretics?

A

PGE2 synthesis depends on COX

  • antipyretics = COX inhibitors
  • -> NSAIDs = COX inhibitors
  • -> Acetaminophen oxidized in CNS to form inhibiting COX (poor peripheral tissue COX inhibitor)
  • -> Glucocorticoids reduced PGE2 synthesis by inhibiting activity of phospholipase A2 (prevent arachidonic acid release) and block transcription of mRNA pyrogenic cytokines
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103
Q

Clinical signs meningitis?

A
  • nuchal rigidity
  • Kernig sign - upper leg flexed at 90 and extension of leg painful
  • Brudzinki’s sign - forced flexion of head results in involuntary flexion of hips
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104
Q

Kawasaki disease criteria

A

Fever at least 5d, and presence of at least 4/5:

  • bilateral, non purulent conjunctivitis
  • mouth/oropharyngeal changes - strawberry tongue, dry/ cracked erythematous lips
  • polymorphous rash
  • extremity changes- edema of hands and feet, in later stages can see desquamation (peeling) or periungual skin
  • unilateral cervical adenopathy >1.5cm
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105
Q

What does full sceptic workup include?

A 
CSF culture (bacterial, viral, fungal meningitis)
- often LP in fever <90d
Blood culture (bacteremia)
Urine culture (UTI + pyelonephritis)
CXR (pneumonia + empyema)

consider nasopharyngeal swabs for viruses, throat swab, and stool for virology/ bacteriology/ parasitology

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106
Q

Rochester criteria (re: septic workup)

A

For infants at low risk of serious bacterial illness

  1. previously healthy term infant without perinatal complications and no previous antibiotic treatment
  2. normal physical exam findings
  3. WBC 5000-15000 cells/mm3
  4. band count <1500 cells/mm3
  5. urinalysis: <10WBC/HPF in centrifuged catheterized specimen
  • partial septic workup considered in febrile infants 1-3mo of age at low risk of having serious bacterial illness
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107
Q

Signs of sepsis on exam

A

early
- peripheral vasodilation (tachycardia, bounding pulses, warm extremities, adequate cap refill)

late
- poor distal perfusion (cool extremities, delayed cap refill, altered mental status, reduced u/o)

septic shock (inadequate organ perfusion/ function)

  • altered level of consciousness
  • hypoxemia
  • oliguria (<0.5 mL/kg/h)
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108
Q

Jones criteria

A 
For acute rheumatic fever
Dx: 2 major criteria OR 1 major + 2 minor + evidence of recent GAS infection (positive culture or rising tigers)
Major (SPACE)
- subcutaneous nodules
- polyarthritis
- arthritis
- carditis
- erythema marginatum

Minor (LEAF)

  • long PR interval
  • elevated acute phase reactants
  • arthralgia
  • fever
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109
Q

Dose paediatric acetaminophen

A

10-15mg/kg q4h (max 75mg/kg/d, or 4g)

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110
Q

Does paediatric ibuprofen

A

10mg/kd q6h (max 40 mg/kd/d)

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111
Q

GAS pharyngitis tx

A
  • Penicillin V or Amoxicillin
  • penicillin allergic = cephalosporins or macrocodes
  • > tx to prevent suppurative and nonsupprative complications
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112
Q

Common infectious organisms 0-28d and empiric tx

A
  • common: group B streptococcus, E. coli
  • other: S. aureus, Listeria monocytogens, Enterococcus, HSV, gram-negative orgamisms

empiric tx:
- ampicillin + cefotaxime OR
- ampicillin + amino glycoside
consider acyclovir

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113
Q

Common infectious organisms infant 29-90d and empiric tx

A
  • common: S. pneumonia, H. influenza, N. meningitidis
  • other: group B strep, E. coli, S. aureus, Enterococcus, Listeria monocytogenes, Pseudomonas sp., other gram negative orgamisms

empiric tx
- ampicillin + cefotaxime
consider vancomycin for suspected meningitis

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114
Q

Common infectious organisms if >3mo and empiric tx

A
  • S. pneumonia, H. influenza, N. meningitidis, S. aureus

empiric tx
- ceftriaxone +/- vancomycin

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115
Q

Meningitis: common pathogens and tx

A

N. meningitides, S. pneumonia, HiB (rare now)

third-generation cephalosporin (crosses BBB) + vancomycin

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116
Q

URTI: common pathogens and tx

A

pharyngitis: GABHS
acute otitis media: S. pneumonia, NTHI, M. catarrhalis

penicillin, amoxicillin

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117
Q

Pneumonia: common pathogens and tx

A

S. pneumonia, GABHS, atypicals

ampicillin/amoxicillin
OR cefuroxime, macrolides

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118
Q

UTI common pathogens and tx

A

E. coli (others: Klebsiella, Enterococcus, Proteus, Serratia)

TMP-SMX, cephalexin or cefixime

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119
Q

Septic arthritis common pathogens and tx

A

S. aureus, Strep

Cloxacillin

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120
Q

Endocarditis common pathogens and tx

A

S. viridians (native valve)

IV penicillin G or ceftriaxone + gentamicin

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121
Q

Pediatric bolus amount in resuscitation and next step

A

20mL/kg 0.9% NS (push as fast as possible) x3

- after 3 boluses consider vasopressors (dopamine, norepinephrine, epinephrine)

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122
Q

Abx contraindicated in peds?

A
  • fluoroquinolones: impair bone/ cartilage growth

- tetracyclines: stain teeth, damage growing cartilage

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123
Q

Antiviral medications for influenza?

A
  • if dx confirmed and pt at increased risk of severe or complicated influenza due to underlying chronic illness or kid has severe illness requiring hospitalization

Neuraminidase inhibitors - reduce release of influenza A and B from infected cells (Tamiflu)
Tricyclic amines - inhibit replication of influenza A (amantadine or rimantadine)

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124
Q

Centor/McIsaac score (GAS pharyngitis) and diagnosis/tx

A
  • fever >38 (+1)
  • no cough (+1)
  • tonsillar exudates/erythema (+1)
  • tender anterior cervical lymphadenopathy (+1)
  • age 3-14 (+1)
    0-1 = no culture
    2-3 = culture, only treat if +
    >=4 = treat with abx
  • rapid strep antigen test (70-90% sensitive)
  • throat culture (gold standard)

tx- 10d penicillin or amoxicillin (erythromycin if penicillin allergy)

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125
Q

Ddx pharyngitis

A
  • bacterial: GAS
  • viral: EBV, adenovirus, influenza, parainfluenza, coxsackie A
  • fungal: C. albicans
  • allergic
  • other, e.g. Kawasaki disease, foreign body
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126
Q

Croup: sx, dx, tx

A
  • URTI, hoarse voice + barking cough, fever, stridor
  • lateral neck X-ray: subglottic narrowing
  • frontal neck film: steeple sign in subglottic region

supportive tx, neublized racemic epinephrine for stridor, single-dose systemic steroid

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127
Q

Bacterial tracheitis sx, dx, tx

A
  • preceding URTI, high fever, stridor, retractions, dysphagia, muffled/suppressed cough, toxic
  • clinical suspicion
  • CXR: subglottic narrowing similar to croup
  • positive tracheal aspirate

emergent intubation
empiric tx, IV abx (cefuroxime)

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128
Q

Epiglottitis sx, dx, tx

A
  • rapid sx evolution: fever, sore throat, irritable, lethargy, drooling; dysphagia, severe stridor/ airway obstruction, toxic
  • clinical suspicion
  • direct visualization in OR
  • keep in comfortable position
  • intubate
  • IV abx: third/fourth generation cephalosporin
  • steroids not indicated
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129
Q

RPA sx, dx, tx

A
  • prodromal nasopharyngitis + abrupt onset high fever, dysphagia, respiratory distress; drooling, meningismus
  • lateral neck X-ray: widening of retropharyngeal space

IV abx: clindamycin, first or second generation cephalosporin and metronidazole
- emergent surgical drainage

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130
Q

Peritonsillar abscess

A
  • sore throat, ipsilateral ear pain, trismus, hot potato voice, fever
  • mass effect: deviated uvula; cervical adenopathy, fluctuance, WBC elevated, throat culture +

surgical drainage or tonsillar aspiration
IV abx

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131
Q

Tests for EBV

A
  • heterophil antibody test = mono spot test for EBV
  • EBV tigers
    + lymphocytosis on CBC
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132
Q

Complications GAS pharyngitis

A
  • rheumatic fever
  • post-streptococcal glomerulonephritis
  • retropharyngeal/ peritonsillar abscess
  • scarlet fever

–> tx prevents rheumatic fever

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133
Q

Management EBV pharyngitis

A
  • supportive
    +/- steroids if airway obstruction
  • no contact sports x4wk (protect spleen)
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134
Q

Normal RR by age (0->12)

A 
0-3 mo: 35-55
3-6 mo: 30-45
6-12 mo: 25-40
1-3 yr: 20-30
3-6 yr: 20-25
6-12 yr: 14-22
>12 yr: 12-16
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135
Q

Signs of increased work of breathing?

A
  • head bob
  • nasal flare
  • tracheal tug
  • substernal and intercostal retractions
  • subcostal recessions
  • paradoxical thoraco-abdo movement
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136
Q

Define respiratory failure and types

A
  • inadequate gas exchange (oxygenation or ventilation)
type I (hypoxemic): decreased oxygen exchange (PaO2 <60mmHg)
- often due to ventilation-perfusion (V/Q) mismatch; can be caused by anemia, poor blood flow to lungs (sepsis, cardiac failure), or toxins affecting utilization of O2 at tissue level (cyanide)
type II (hypercapnic): decreased removal of carbon dioxide (PaCO2 >50mmHg or pH <7.35)
- reduced RR (bradypnea) or reduced tidal volume (shallow breaths)
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137
Q

Paediatric respiratory assessment measure (PRAM)

- components

A 
Quantify level of respiratory distress
scored 0-3 per category (0 = no signs; 3 = severe)
- suprasternal indrawing
- scalene retractions
- wheezing
- air entry
- O2 saturation 
0-3 = low risk
4-7 = moderate risk
>8 = high risk
138
Q

What can a normal or increasing CO2 level in context of tachypnea and respiratory distress indicate?

A
  • fatigue –> impending respiratory failure
139
Q

Retropharyngeal abscess (RPA)

  • sx
  • X-ray findings
A
  • serious condition may present with fever, neck pain (or torticollis) and refusal to eat
  • X-ray neck: increased width of prevertebreal soft tissue (> half width of corresponding vertebral body then abnormal and r/o RPA)
140
Q

Management of bronchiolitis

A
  • supportive, can suction nares
  • O2 for sat >90%
  • trial bronchodilators option
  • high risk kids should receive RSV prophylaxis (palivizumab)
141
Q

Management acute asthma exacerbation

A

Mild (PRAM 0-3)

  • supplemental O2 to keep O2 >94%
  • short-acting bronchodilator (Salbutamol) q20 min x 1 -3 doses then q1h prn
  • consider systemic steroids

Moderate (PRAM 4-7)

  • O2 to keep O2 >94%
  • continuous O2 monitoring
  • Salbutamol +/- Ipratropium bromide q20min 3 doses then q30min prn
  • system sterioids

Severe (PRAM >8)

  • supplemental O2 for O2 >94%
  • continuous O2 monitoring
  • Salbutamol and Ipratropium bromide q2min x3 then salbutamol q30min prn
  • systemic steroids - IV if no PO intake
  • consider IV fluids containing K
  • if not improving, treat with magnesium sulfate
  • if resp distress persists, consider continuous salbutamol infusion and admission to ICU
  • be prepared for rapid sequence intubation

steroids: prednisone or dexamethasone for 3-5d

142
Q

Ddx child with stridor

A

croup
epiglottitis
foreign body aspiration

143
Q

Approach to croup

A
  • inflammation of larynx and trachea, often secondary to viral infection (parainfluenza accounts for 65%)
  • kids 1-6yr old
  • hx: preceding vital URTI (cough, coryza, fever)
  • sx: barking cough, inspiratory stridor
  • ix: steeple sign on PA radiograph of upper airway
  • tx: systemic steroids +/- nebulizer epinephrine
144
Q

Approach to epiglottitis

A
  • infection and inflammation of epiglottis, with high risk upper airway obstruction
  • most commonly due to HiB (important to ask about vaccination status of kid)
  • sx: toxic appearance, drooling, muffled voice, tripod positioning
  • ix: thumb print sign on lateral neck radiograph
  • tx: intubation, abx
145
Q

Approach to foreign body aspiration

A
  • must be considered in stridorous child regardless of hx
  • peanuts are most common aspirated foreign body in children
  • ix: AP radiographs can determine if there is a radiopaque object present; lateral decubitus radiographs may demonstrate air trapping on the side with foreign body present
  • tx: broncoscopy
146
Q

How to deliver salbutamol?

A
  • MDI via Aerochamber

- can be delivered by nebulizer

147
Q

Salbutamol effect on K

A
  • shifts K into cells lowering serum K

- monitor K closely if multiple salbutamol doses

148
Q

What layer of skin are melanocytes and Langerhans cells found in?

A

epidermis (outermost layer)

149
Q

What layer of skin contains collagen, elastic tissue and reticular fibres, hair follicles, sebaceous (oil) and eccrine (sweat) glands, blood vessels and nerves?

150
Q

What layer of skin is subcutaneous fat and connective tissue found in?

A

subcutis/hypodermis

151
Q

Is pruritus thought to be due to a higher number of sensory fibres or adrenergic autonomic nerve fibres?

A

higher around of sensory fibers

152
Q

What is erythema toxicum?

A
  • most common neonatal rash
  • noninfectious, benign papular rash that can evolve into vesicles; clustered lesions, often developing within days of brith but up to few weeks of age
  • self-limited, resolution within 1wk
  • recurrences may occur
153
Q

What is miliaria (different than milia)?

A
  • papule and pustules on erythematous base usually around nose (prickly heat rash)
  • secondary to eccrine sweat duct obstruction
  • onset after first wk life, predilection for areas with high heat production
  • self-limited though supportive tx; minimize excessive wrapping can facilitate improvement
154
Q

What is congenital dermal melanocytosis (Mongolian blue spot)?

A
  • benign blue-purple large, usually patchy skin macules
  • common in Aboriginal, Asian, African American, and other dark-toned infants
  • sites: often buttocks, legs, spine, but can occur anywhere
  • must ensure not non accidental injury
  • spontaneous resolution within 1yr, some may persist
155
Q

What is molluscum contagiosum: Poxvirus?

A
  • small pink-tan (flesh-coloured), dome shaped papoules with dimpled or umbilicated centre; can express curd-like material
  • usually asx, but can be associated with exzematous dermatitis and pruritis
  • rx: none - course self-limited in healthy kids, spontaneous regression within 2yr
  • -> options include destructive methods (curettage, cryotherapy, peeling agents, topical retinoids, cantharidin)
  • if persistent/widespread, screen for congenital or acquired immunodeficiency
  • complications: bacterial superinfection requiring abx
156
Q

What is a hemangioma?

A
  • newborn: pale macule with thin telangiectasia (thread-like)
  • older kids: vibrant, red, elevated, non compressible plaque sometimes with blue tinge indicating deeper components
  • only 10% present at birth; grow larger until 10-12mo age then natural regression over subsequent 3-5yr

complications: rare but can ulcerate with seondary infection, hemorrhage, and scarring

dx- clinical; US to differentiate vascular malformation or neoplastic process and visualize extent of deeper infiltration

rx- none

  • b-blocker (nadolol, propranolol) are first-line if face or requires tx
  • systemic steroids, laser ablation, interferon tx, embolization, or surgical excision depending on number, location, depth of lesions
157
Q

What is epidermolysis bullosa?

A
  • group of heterogenous mechanobullous diseases characterized by development of blisters after trauma to skin

types:

  • EB simplex (non scarring)
  • dystrophic EB (severe scarring, atrophy, multi system involvement)
  • junctional EB (often fatal or heals with atrophy, can present with airway compromise due to granulation tissue)

rx- education, symptomatic tx, monitor thermoregulation, fluid balance and airway potency, assess for secondary infections with extensive blistering; support groups; scarring can lead to internal and external deformities/strictures that have functional and cosmetic implications

158
Q

What is viral exanthem?

A
  • morbilliform red eruption, usually nonpruritic
  • extremely common; multitude of viral strains can cause
  • may be febrile, well-appearing child
  • usual sites: trunk +/- extremities
  • associated with measles, rubella, roseola, enteroviruses, mononucleosis
  • rx: self limiting; monitor sx
159
Q

What is varicella zoster virus infection?

A

Chicken pox

  • prodrome of mild fever and other systemic sx
  • generalized, polymorphous, pruritic, vesicular rash (dew drop on rose petal) of abrupt onset at varying stages of healing
  • severe infection less common with vaccine, often present atypically
  • latency in dorsal root ganglia during primary infection -> reactivation -> shingles
  • primary infection usually results in life-long immunity but recurrence can occur

transmission: airborne from respiratory secretions, direct contact of lesions, transplacental
- incubation period: 10-21d; contagious 1-2d prior to onset of rash and until crusting of all lesions complete

dx- clinical; can send vesicular scraping for direct fluorescent antigen/EM/PCR

rx- supportive
- if immunocompromised can give acyclovir, VZIG +/- vaccination

160
Q

Which infectious rash (common in peds before vaccination) can result in these complications: bacterial superinfection, thrombocytopenia, arthritis, hepatitis, cerebellar ataxia, encephalitis, meningitis, glomerulonephritis, pneumonia

A

Varicella zoster

161
Q

Perinatal/neonatal disease of varicella zoster?

A
  • high risk if onset of maternal skin lesions 5d before delivery or 2d after delivery
  • mortality rate 30% due to encephalitis
  • VZIG in immediate postpartum period to prevent
  • rx with IV acyclovir if baby develops varicella
162
Q

Coxsackie virus A16 is what?

A

Hand-foot-and-mouth disease
- red coloured macule, vesicular lesions on buccal mucosa, palate, tongue, hands (palms), and feet (soles)
- viral prodrome
- exquisitely tender lesions
transmission: direct contact
rx- supportive, PO fluid, lidocaine mouthwash

163
Q

Morbillivirus?

A

Measles
- fever, cough, coryza, conjunctivitis (3Cs)
- diffuse maculopapular rash
- transient enanthemas (Koplik spots - pathognomonic)
- rash starting on face and spreading caudally
- risk of death from resp and neuro complications
transmission: resp droplet
incubation period 8-12d
tx- supportive + vitamin A supplementation; consider post-exposure prophylaxis (vaccine, immunoglobulin)
- pubic health notification

164
Q

What are Koplik spots pathognomonic for?

165
Q

Parovirus B19 (erythema infectiousum)

A

Fifth disease
- prodrome fever, malaise, myalgia, approx 7d before rash
- red ‘slapped cheek’ rash with circumoral pallor
- maculopapular, lace-like, pruritic rash on trunk, moving peripherally
- other sx: polyarthropathies, anemia (immunocompromised), transient aplastic crisis (hemolytic anemia), hydrops fetalis (first trimester of pregnancy)
transmission: resp droplet
incubation period 4-14d
rx - supportive

166
Q

HSV 1&2

A
  • primary infection usually asx
  • gingivostomatitis: fever, irritability, tender lymphadenopathy, ulcerative enanthem in anterior oropharynx
  • genital herpes in adolescents
  • recurrence - grouped vesicles personally or genital, conjunctivitis, herpetic whitlow
  • transmission: direct contact with secretions from lesions, sexual intercourse
  • incubation period 2d - 2wk
    rx- supportive, ensure PO intake, lidocaine mouthwash
167
Q

Neonatal HSV

A
  • 1 in 3200 to 10000
    classification:
  • Localized skin, eye, mouth (SEM): coalescing/ clustering vesicular lesions; tearing, ocular pain, conjunctival deem +/- localized ulcerative lesions of mouth, palate, tongue -> eval all for CNS and disseminated disease
  • CNS +/- SEC: seizures (focal/generalized), lethargy, irritability, tremors, poor feeding, temp instability, full anterior fontanelle -> EEG often abnormal early when CSF and neuroimaging normal
  • Disseminated: multiple organs; often present in wk1 life with nonspecific signs of neonatal sepsis (temp dysregulation, apnea, irritability, lethargy, resp distress, abdo distention, ascites) -> mortality (untx) >8%

ddx- bacterial sepsis, meningitis, pneumonitis, hepatitis, other viral

ix - culture positive HSV, HSV DNA PCR, HSV antigens (rapid direct immunofluorescence/ enzyme immunoassays)
- if CNS neonatal HSV -> EEG, LP, neuroimaging
negative doesn’t exclude

rx- empirical tx with acyclovir

168
Q

What is roseola infantum: HHV6?

A 
Baby measles
- high fever then 3-5d later generalized maculopapular rash spreading from trunk to extremities, sparing face (note fever subsides by time rash begins)
transmission- likely droplet
peak incidence 6-24mo
incubation period: 9-10d
rx- supportive
169
Q

What are tinea infections?

A
  • can infect head (capitus), body (corporis), feet (pedis), face (faciei), groin (cruris)
  • many microorganisms - often Tinea family

5 clinical patterns tinea capitis

  • diffuse scaling
  • circumscribed alopecia with scale
  • black dots (broken hairs)
  • kerion (boggy mass)
  • pustular

dx -scraping for KOH and fungal culture

rx

  • tinea capitis: systemic tx with oral terbinafine x4wk
  • other types = topical bid with terbinafine, ciclopirox, clotrimazole, ketoconazole for up to 4wk
170
Q

Ddx diaper rash

A
  • irritant contact dermatitis (shiny red macules, patches)
  • seborrheic dermatitis (cradle cap) (yellow greasy plaques)
  • candida (beefy red patches, peripheral scales)
  • psoriasis (well demarcated papules/ plaques)
  • bullous impetigo (bullae on erythematous base)
  • Langerhans cell histiocytosis (hemorrhagic papule or plaques)
  • Acrodermatitis enteropathica (dermatitis , alopecia, diarrhea triad -> zinc deficiency)
171
Q

What is most emergent abdominal pain in peds?

A
  • midgut volvulus with malrotation
172
Q

Define visceral (splanchnic) pain

A
  • due to noxious stimuli (often stretch) stimulating receptors of visceral peritoneum, mesentery, or muscle or mucosa of hollow organs
  • dull, poorly localized, associated with midline (due to afferent autonomic innervation)
173
Q

Visceral pain is sensed in areas corresponding with what?
Epigastric pain?
Periumbillical pain?
Suprapubic/hypogastric pain?

A
  • corresponding to embryonic origin of affected structure
    epigastric -> foregut (esophagus to duodenum, liver, gallbladder, pancreas)
    periumbillical -> midgut (distal 2nd part duodenum to proximal two-third of transverse colon)
    suprapubic/hypogastric -> hindgut (distal third transverse colon to rectum)
174
Q

Define parietal (somatic) pain

A
  • noxious stimuli (stretch, inflammation/ irritation, tearing) stimulating receptors of parietal peritoneum, skin, or skeletal muscle
  • intense, localized, on same side and dermatomal region as origin of pain (somatic afferent nerves numerous, myelinated and transmit to specific dorsal root ganglion)
175
Q

Define referred pain

A
  • due to convergence/ shared projections of somatic and/or visceral pain pathways in CNS
176
Q

Red flags abdo pain for organic pathology

A
  • pain: progressive, localized (non umbilical), wake from sleep, radiates to shoulder/back/groin
  • GI sx: dysphagia/ odynophagia, anorexia, emesis (bilious, bloody, persistent), jaundice, no flatulus/bowel movement, diarrhea (chronic, nocturnal), hemtochezia/ melena
  • extraGI sx: unexplained fever, wt loss/ FTT, decelerated linear growth velocity, oral ulcers, perirectal disease, arthritis, cough/ dyspnea, dysuria/ hematuria, vaginal d/c or bleeding, scrotal/ pelvic pain
    fhx: IBD, celiac disease, peptic ulcer disease
177
Q

Diagnosis?

  • delayed meconium
  • FTT
  • bilious vomiting
  • chronic constipation since birth

o/e - abdo distention
DRE: tight anal sphincter, empty ampulla, blast sign (expulsion gas and stool after DRE)

–> further investigations?

A

Hirschsprung disease

Inv
- barium enema: narrow/ normal rectum, dilated colon proximal to ganglionic segment, with transition zone

definitive dx by rectal biopsy - absence of ganglion cells

178
Q

Diagnosis?

  • sx onset 3-8wk old
  • FTT, “hungry vomiter”
  • M>F
  • projectile, non bilious vomiting, immediately postprandial

o/e: +/- visible peristalsis
possible palpable epigastric mass (olive sign)

-> further investigations?

A

Pyloric stenosis

Inv

  • U/S: hypertrophied pylorus (increased muscle thickness and length)
  • lytes/blood gas: hypochloremic, hypokalemic metabolic alkalosis
179
Q

Diagnosis?
- bilious vomiting within hours of birth (later if stenosis)

o/e: epigastric distention, 1/4 have trisomy 21

-> investigations?

A

Duodenal atresia

Inv
- AXR: double bubble sign, absent distal abdo gas
Upper GI series: duodenal obstruction (UGI suggested preoperatively to r/o malrotation with midgut volvulus)

180
Q

Diagnosis?

  • unexplained paroxysms of irritability, fussiness, crying starting/stopping without obvious cause
  • <4mo old, no FTT
  • episodes >3hr/d, >3d/wk, for >1wk

o/e healthy infant

-> investigations?

A

Infantile colic

Inv
- clinical dx with Rome III criteria

181
Q

Rome III Criteria for functional chronic abdo pain disorders

A
  • all functional disorders -> no evidence of inflammatory, anatomic, metabolic, or neoplastic process to otherwise explain sx
  • functional dyspepsia: persistent/ recurrent upper abdo pain or discomfort >1x/wk for >2mo
  • IBS: abdo pain/discomfort with >2/3 (pain improved by defecation, associated with change in frequency of stool or form of stool) for >25% time, >1x/wk for >2mo
  • childhood functional abdo pain: episodic/ continuous abdo pain, >1x/wk for >2mo
  • childhood functional abdo pain syndrome: functional abdo pain >25% time with some loss of daily activity, headache, limb pain, or difficulty sleeping
  • abdo migraine: intense periumbillical pain for >1h, interferes with normal activities, associated with >2 of 6 (headache, photophobia, nausea, vomiting, pallor, anorexia), intervening usual health
  • recurrent abdo pain (Rome II): >3 episodes of pain severe enough to affect daily activities >3mo
182
Q

Diagnosis?

  • regurgitation/ emesis, feeding aversion, colicky baby, irritable
  • apnea, stridor, aspiration, wheeze
  • Sandifer syndrome

o/e +/- FTT
+/- hoarseness, stridor, wheeze

-> investigations

A

GER/ GERD

note: FTT in GERD

Inv
- empiric trial of acid suppressant
- endoscopy: esophagitis
24h pH probe/impedance study: acidic/ non-acidic reflux, strength of association with suspected signs/ symptoms
+/- upper GI series: r/o obstruction, stenosis, malrotation

183
Q

What is Sandifer syndrome?

A
  • back arching
  • chin lifting
  • neck contortions
  • > due to discomfort with GER(D)
184
Q

Diagnosis?

  • classic hx = swelling in inguinal area during crying/ straining
  • irritable, crying, vomiting, abdo distention, groin pain

o/e firm, tender, often oedematous inguinal mass
+/- surrounding erythema
+/- abdo distension/ tenderness
+/- scrotum appears blue (testicular venous congestion)

-> investigations?

A

Incarcerated inguinal hernia

Inv
- clinical dx
- AXR (may see lower GI obstruction or air bubble in groin)
U/S

185
Q

Diagnosis?
- 3mo - 3yr (up to 6yo)
- paroxysmal, severe, crampy abdo pain, inconsolable crying, drawing up legs
- episodes progress in frequency
+/- vomiting
- currant jelly stool (blood, mucous)
- initially comfortable/normal behaviour between episodes, progressive lethargy

o/e: abdo pain may be benign vs. variable abdo distension and tenderness
+/- palpable sausage-shaped RUQ or epigastric mass
+/- bloody mucus on DRE

-> investigations?

A

Intussusception

Inv
- air contrast enema: obstruction (filling defect), air fluid levels, absence of gas in RLQ
U/S: target sign (bowel within bowel), tubular mass

186
Q

Diagnosis?

  • sudden onset bilious emesis, abdo distension, melena
  • septic shock (necrotic bowel and 3rd spacing)
  • children may have episodic abdo pain with vomiting for weeks to years before detection

o/e: abdo distension/tenderness
+/- peritonitis/ shock

-> investigations?

A

Malrotation with mid-gut volvulus

Inv

  • AXR: upper GI obstruction (dilated proximal loops with fairly gasless abdo), pneumoperitoneum
  • Upper GI series: failure of duodenum to cross midline (malrotation), duodenal obstruction (volvulus)
187
Q

Diagnosis?

  • dysuria, urgency, frequency
  • irritability, fever, poor feeding
  • vomiting/diarrhea
  • flank/abdo pain

o/e: tend abdo (suprapubic)
+/- CVA tenderness - Murphy punch sign
+/- fever

-> investigations?

A

UTI
- Murphy punch sign with pyelonephritis

Inv

  • urine R&M (WBC, nitrites, leukocyte esterase), C&S
  • controversy in workup after 1st UTI: renal US +/- VCUG
188
Q 
Diagnosis?
- BMs 2 or less per week, large diameter, difficult/ incomplete evacuation
- colicky abdo pain, painful defacation
\+/- encopresis
\+/- retentive posturing

o/e: abdo palpation of hard fecal mass
- dilated rectum filled with stool
+/-anal fissure

-> investigations?

A

Constipation

Inv

  • clinical (Rome criteria for functional constipation)
  • AXR: fecal impaction (excessive stool in colon)
189
Q

Diagnosis?

  • fever, cramping abdo pain
  • diarrhea +/- BRBPR
  • vomiting, anorexia
  • H/A, myalgia

o/e soft, tender abdo
+/- fever

-> investigations?

A

Gastroenteritis

  • 70-80% viral
  • 10-20% bacterial
  • 5% parasitic

Inv
- stool C&S, stool O&P, stool C. difficile PCR
(only send if bloody or chronic diarrhea, immunosuppressed, recent abx use, exposures to bacterial/ parasitic disease)

190
Q

Diagnosis?

  • resp tract sx
  • n/v
  • chest and/or upper abdo pain

o/e: tachypnea, hypoxia, increased WOB, fever
- percussion dullness, increased tactile fremitus, decreased air entry, bronchial breath sounds

-> investigations?

A

Pneumonia

Inv
- CXR: focal consolidation, pleural effusion (atypical pneumonia with patchy diffuse opacifications)
+/- increased WBC

191
Q

Diagnosis?

  • preceding gastroenteritis, fever, bloody diarrhea (5-10d)
  • abrupt onset of irritability, lethargy, deem, pallor (usually without purpura)

o/e: toxic +/- uremic encephalopathy

  • tender abdo
  • HTN

-> investigations?

A

HUS
(E. coli O157:H7, Shigella)

Inv
- CBCD, blood smear, haptoglobin and bilirubin: MAHA, thrombocytopenia 
Cr: increased (ARF)
u/a: proteinuria, hematuria
stool C&amp;S: infection
192
Q

Diagnosis?

  • colicky abdo pain with vomiting (within 8d purpuric rash)
  • melena/ hematochezia
  • arthralgias
  • hematuria
  • rare GI complications: intussusception, pancreatitis, protein-losing enteropathy

o/e: palpable purpura on lower extremities/ gravity dependent areas
- oligoarthritis
+/- HTN

-> investigations?

A

Henoch-Schonlein Purpura (HSP)

Inv
- no lab findings dx
common: increased ESR, CRP, WBC, platelets; anemia
IgA: elevated 50-70%
INR/PTT: no coagulopathy
+/- hematuria, proteinuria, increased Cr
biopsy skin/kidney (rare); IgA deposition, leukocytoclastic vasculitis

193
Q

Diagnosis?

  • colicky abdo/flank pain, radiating to testes or labia
  • n/v, chills, ileus
  • gross hematuria, dysuria, urgency

o/e: fever
abdo +/- CVA tenderness

-> investigations?

A

Urolithiasis

Inv
- radiopaque stones on AXR
Non-contrast CT gold standard
- u/s: obstruction of GU system, hydronephrosis

194
Q

Diagnosis?

  • n/v, anorexia
  • dehydration, wt loss
  • fatigue
  • polyuria, polydipsia, polyphasic

o/e dehydration, diffuse abdo tenderness, Kussmaul breathing

-> investigations?

A

DKA

Inv
- increased blood glucose (>11.1 mM)
- ABG: WAG metabolic acidosis (HCO3 <15, pH <7.3)
- positive urine/serum ketones
+/- increased WBC, Cr, BUN (hemoconcentration)

195
Q

Diagnosis?

  • periumbilical pain migrating to RLQ (worse with cough, walking, jumping)
  • anorexia, n/v

o/e: fever
RLQ tenderness (percussion and palpation), often focal at McBurney point
+/- peritonitis: rebound tenderness, involuntary guarding, Rovsign sign, Obtruator sign, iliopsoas sign

-> investigations?

A

Appendicitis

Inv
- US: thick walled/dilated appendix +/- appendicolith
- CT: enlarged appendix, appendicolith, associated mesenteric fat stranding
+/- increased WBC, increased neutrophils

196
Q

Diagnosis?

  • severe persistent epigastric/ LUQ pain radiating to back, alleviated by bending forward
  • anorexia, vomiting
  • restlessness, agitation

o/e

  • fever, tachycardia
  • tender +/- distended abdo
  • periumbilical or flank bruising (hemorrhage) - rare in peds

-> investigations?

A

Pancreatitis

Inv

  • increased lipase, increased WBC
  • u/s: hypo echoic enlarged pancreas +/- gallstones
  • CT: deem, necrosis, hemorrhage, peripancreatic fat stranding, pseudocyst
197
Q

Diagnosis?

  • epigastric/ RUQ pain, steady, severe +/- radiation to shoulder or back, worse with motion
  • preceded by/ associated with fatty meal
  • anorexia, n/v

o/e: fever, tachycardia
- tender abdo +/- guarding +/- Murphy sign

-> investigations?

A

Cholecystitis

Inv

  • US: thick wall, gallstones, dilated gallbladder
  • increased WBC
  • bilirubin, ALP, GGT usually normal
  • HIDA: non visualized gallbladder
198
Q

Diagnosis?

  • FTT/wt loss, fatigue, growth failure, pubertal delay
  • red eyes, mouth ulcers
  • abdo pain
  • perianal disease
  • diarrhea +/- blood, mucous/pus, urgency, tenesmus
  • arthralgia, rashes

o/e - tender abdo +/- RLQ mass
- extraGI: fever, episcleritis, oral ulcers, pallor, digital clubbing, erythema nodosum, pyoderma gangrenous, arthritis/ scroilitis, perianal disease

-> investigations?

A

IBD

Inv

  • anemia (iron deficient and chronic inflammation)
  • increased WBC and platelets, increased ESR/CRP
  • low albumin, vit D
  • stool + blood, WBC
  • upper endoscopy/ colonoscopy, small bowel imaging
199
Q

Diagnosis?

  • severe sudden onset testicular or scrotal pain +/- inguinal/ lower abdo pain
  • n/v +/- fever

o/e: tender, swollen testicle

  • elevated +/- horizontal lie
  • absent ipsilateral cremasteric reflex

-> investigations?

A

Testicular torsion

Inv
- Scrotal doppler US: compromised testicular perfusion

200
Q

Diagnosis?

  • sexually active
  • missed period
  • vag bleeding, pelvic pain

o/e: painful internal pelvic exam +/- peritonitis

-> investigations?

A

Ectopic pregnancy

Inv

  • increased serum b-hCG (slow rise)
  • pelvic US: absent intrauterine fetus +/- visible ectopic pregnancy
201
Q 
Diagnosis?
- dysmenorrhea
\+/- acyclic pelvic pain
- abnormal vag bleeding
- urgency, dysuria, dyschezia, constipation

o/e: general pelvic tenderness
+/- tender uterosacral ligaments, posterior uterus
+/- fixed uterine retroversion

-> investigations?

A

Endometriosis

Inv

  • pregnancy test -
  • pelvic US: excludes other pathology, may see endometrioma
  • laparoscopy
202
Q

Diagnosis?
- sudden unilateral abdo/pelvic pain +/- n/v

o/e: painful unilateral adnexa +/- peritonitis

-> investigations?

A

Ovarian torsion

Inv

  • pregnancy test negative
  • pelvic US: ovarian torsion
203
Q

What is Pediatric Appendicitis Score (PAS)?

A

1 point:

  • anorexia
  • n/v
  • migration of pain
  • fever >38.5
  • WBC >10 x10^6/L
  • neutrophils + band forms >7.5 x 10^6 cells/L

2 points:

  • RLQ tenderness
  • pain with cough/percussion

PAS <2 = low risk
PAS 3 - 6 = intermediate risk -> consider surgical consult, serial exams
PAS >=7 = high risk -> surgical consult

204
Q

HUS triad

A
  • acute kidney injury
  • thrombocytopenia
  • microangiopathic hemolytic anemia
205
Q

DKA precipitants?

A
  • insulin deficiency (new dx or missed insulin dose)
  • infection
  • ischemia
  • intoxication
206
Q

Ladd procedure for what?

A

Malrotation with midgut volvulus

207
Q

Define diarrhea? Acute vs. chronic?

A
  • increased frequency, fluidity, volume of stools
    WHO - unusually loose or watery stools, at least 3x/d
  • acute = <2-3wk
  • chronic = >2-3wk
208
Q

How many L can colon absorb?

209
Q

Pathophysilogic mechanisms of diarrhea?

A

Osmotic - ingested, non absorbed solutes reach colon, creating osmotic gradient that allows water diffusion into lumen (e.g. lactase deficiency)

Secretory - excessive secretion of lytes (esp. Cl via CFTR) into lumen after noxious stimulus, with water following (e.g. bacterial toxin)

Altered motility - increased motility results in reduced transit time for water absorption (e.g. ileocecal valve resection)

Inflammation - multifactorial: damage/atrophy of vili, epithelial cell dysfunction (altered fluid/electrolyte transport) and increased motility and secretions (infection, IBD)

210
Q

Ddx acute abdo with diarrhea?

A
  • intussusception
  • appendicitis
  • toxic megacolon
  • evolving bowel obstruction
211
Q

Blood/ mucous diarrhea likely?

A

Inflammatory diarrhea

212
Q

Acholic diarrhea likely?

A

cholestasis

213
Q

Foaming/ floating/ greasy/ foul smell diarrhea likely?

A

Steatorrhea - 80% pancreatic insufficiency, 12-15% mucosal disease

214
Q

Watery diarrhea likely?

A

malabsorption, secretory

215
Q

Undigested particles in diarrhea likely?

A

toddler’s dirrhea

216
Q

Small volume leakage/ incontinence likely?

A

Consider constipation

217
Q

Risk of what GI condition with asthma/ allergic disease?

A

eosinophilic gastroenteritis

218
Q

Red flags diarrhea?

A
  • severe continuous or nocturnal diarrhea
  • systemic sx: fever, rash, arthritis
  • blood or mucous in stool
  • very acidic stools
  • weight loss/ FTT
  • petechiae or purpura
  • signs dehydration
  • change mental status
  • severe abdo pain or distension
219
Q

Stool assessment for inflammation?

A
  • fecal WBC +/- calprotectin
220
Q

When do you do stool pH and electrolytes?

A

Watery stools
- osmotic vs. secretory diarrhea
osmotic pH <5, osmotic gap >125 mM, Na <60 mM
secretory pH >5, osmotic gap <50 mM, Na >90 mM

221
Q

Workup suspected carbohydrate maldigestion/ malabsorption?

A
  • stool for reducing substances

- lactose hydrogen breath test

222
Q

Steatorrhea workup?

A
  • tests of malabsorption: stool microscopy for fat globules, quantitative 72h fecal fat collection
  • screen cholestasis: bilirubin (total/direct), GGT, ALP, vitamin A, D, E, INR, triglycerides, cholesterol
  • screen pancreatic insufficiency: fecal elastase, sweat chloride
223
Q

Workup suspected protein-losing enteropathy?

A
  • stool alpha-1-antitrypsin

- calcium, INR, albumin

224
Q

What etiologies of diarrhea increase immunoglobulin (IgE)?

A
  • suspected immunodeficiency or eosinophilic gastroenteritis
225
Q

Stool osmotic gap

A
  • estimates relative contributions of electrolytes and non electrolytes to retention of water in intestinal lumen
  • normal stool osmolarity is 290 mmol/L (isotonic to plasma)
  • stool Na and K in stool multiplied by 2 in equation to account for obligate anions
  • stool osmotic gap (mmol/L) = 290 (or measured stool osmolarity) - 2 (stool [Na] + stool [K])
226
Q

What supplementation can reduce duration and severity of diarrhea?

227
Q

Can antibiotics increase the risk of hemolytic ureic syndrome in patients with E. coli O157-H7?

228
Q

What is fecal impaction?

A
  • hard mass in lower abdo on exam, dilated rectum with large amount of stool on rectal exam, or excessive stool in distal colon on abdo xray
229
Q

What is encopresis?

A
  • recurrent, episodic fecal incontinence (involuntary loss of stool into child’s underwear, after reaching developmental age of 4yr) due to overflow of stool caused by fecal impaction
230
Q

Phases of normal defecation?

A
  • involuntary phase: normal colonic transit delivering fecal material to rectum
  • voluntary phase: leading to expulsion of stool
231
Q

What nervous system(s) input to control colonic and rectal motor function?

A
  • enteric NS

- autonomic NS

232
Q

What is vast majority of functional constipation secondary to?

A
  • purposeful/ subconscious stool withholding
  • > pushes stool higher into rectum (diminishing urge to defecate)
  • > colon absorbs water and electrolytes from retained stool, with increasingly larger stool volume with harder consistency
233
Q

Results of chronic rectal distension?

A
  • decreased rectal sensitivity and loss of normal urge to defecate
234
Q

Rome III diagnostic criteria for functional constipation

A

In absence of organic pathology and >=2 of:
Neonates/toddlers (<4yr)
- <=2 defections/wk
- >=1 episode fecal incontinence/wk after acquiring toileting skills
- excessive stool retention
- painful/hard bowel mvoements
- large diameter stools that may obstruct toilet
- large fecal mass in rectum
for >=1mo

Kids/adolescents (>=4yr)
- <= defecations in toilet/wk
- >=1 episode fecal incontinence/wk after toileting skills
- retentive posturing or excessive volitional stool retention
- painful/hard BM
- large diameter stools, may obstruct toilet
- large fecal mass in rectum
for >=1x/wk, >=2mo

235
Q

Anal fissures with functional constipation of HD?

A
  • more common in functional constipation
236
Q

Red flags on physical exam in child with constipation to suggest organic etiology?

A
  • severe abdo distention
  • abnormal abdo musculature
  • abnormal anal position (e.g. anteriorly displaced)
  • perianal fistula
  • anal scars, hematoma, or extreme fear during anal inspection
  • tight, empty rectum with palpable abdo fecal mass
  • explosive stool and air from rectum after withdrawal of examining finger (blast sign)
  • midline pigmentary abnormalities of lower spine
  • sacral dimple and/or overlying tuft of hair
  • gluteal cleft deviation, flat buttocks
  • absent anal reflex (anal wink) or cremasteric reflex
  • decreased strength/tone/deep tendon reflexes in lower extremities
237
Q

Lab tests requiring further evaluation in kid with constipation?

A
  • electrolytes, calcium, TSH, T4, celiac screen (+/- lead levels, sweat chloride)
238
Q

What needs to be ruled out with intractable constipation (no response to tx >3mo but normal lab tests)?

A

Hirschsprung disease

  • anorectal manometry
  • rectal biopsy (gold standard)

Anatomical malformations
- barium enema

+/- cows milk protein allergy

  • 2-4wk trial of cow milk protein avoidance
  • allergy testing not routinely done

+/- spinal cord malformation/anomalies
- MRI spine not routinely supported without neurologic or lumbosacral abnormalities

239
Q

First line tx for constipation (medication)?

A

PEG

maintenance PEG or lactulose

240
Q

Definition of FTT

A
  • weight that falls or remains below the third percentile for age; decreases and crosses 2 percentile lines or is less than 80% median weight for height
  • can be associated with short stature
241
Q

How long to do correct for prematurity in preterm infants?

A

Up to 24mo age

242
Q

Investigations FTT pre/perinatal causes

A
  • TORCH screen, HIV if appropriate
  • genetic consultation; chromosomal and molecular genetic testing
  • cranial US
243
Q

Investigations FTT feeding issues

A
  • three day dietary record
  • lactation consult (infants: latch, suck, swallow)
  • dietician and OT-observed feeds
244
Q

Investigations FTT malabsorption/ ultilization

A
  • CBCD
  • protein, albumin, bilirubin, LFTs, coagulation factors (INR)
  • iron, ferritin
  • inflammatory markers (ESR, CRP)
  • anti-TTG, IgA
  • stool for lipid globules, reducing subtances
  • sweat chloride, SaO2, sleep study
  • upper GI series (imaging)
245
Q

Investigations FTT increased demands

A
  • metabolic: metabolic screen, serum and urine amino acids, molecular genetic testing
  • renal failure: urinalysis, creatinine, electrolytes
  • infections: HIV/ other viral serology, stool cultures, immunoglobulins
  • congenital heart disease: associated clinical findings and cardiac imaging
  • respiratory: sweat chloride
  • endocrine: low TSH, high FT4, glucose, insulin tolerance test (for GH deficiency), bone age
246
Q

Pattern I growth parameters with FTT

A
  • HC: normal
  • Ht: normal or low
  • Wt: low, low for height
  • Appearance: malnourished
  • Etiologies - multiple
247
Q

Pattern II growth parameters with FTT

A
  • HC: normal
  • Ht: low
  • Wt: low, proportional to height
  • Appearance: healthy
  • Etiologies: endocrinopathy, dwarfism, constitutional deprivation, normal variant short stature (not true FTT)
248
Q

Pattern III growth parameters with FTT

A
  • HC: low
  • Ht: low
  • Wt: low, proportional height
  • Appearance: stunted
  • Etiologies: early onset = IUGR, chromosomal/ genetic abnormality, perinatal insults
249
Q

Define limp

A
  • uneven or jerky walk, due to pain, weakness or deformity (structural abnormality of bones, joints, soft tissues, or nerves of lower extremities, pelvis or spine)
250
Q

What is the inner tissue layer lining a joint, which secretes synovial fluid to lubricate the joint?

A

Synovial membrane

- fluid can become inflamed or infected and cause sx pain and swelling

251
Q

Diagnose: self-limiting, nonspecific inflammation of synovial membrane

A

Transient synovitis

- most common cause of paediatric non traumatic hip pain

252
Q

What dx must be ruled out with limp/ joint pain?

A
  • septic arthritis -> synovial fluid analysis and culture

often child with limp has fever and is toxic appearing

253
Q

What presents as recurrent, self-limited, and usually bilateral leg pain; common in preschool and school aged kids?

A

Growing pains

254
Q

Systems re: causal conditions of child with limp/ pain?

A
  • bones (osseous)
  • joints (articular)
  • soft tissue
  • neurologic
  • other
255
Q

Age group for DDH?

A
  • preschool age
256
Q

Age group for Legg-Calve-Perthes disease, leukaemia, osteosarcoma?

A
  • school aged
257
Q

Age group for Osgood-Schlatters disease, slipped capital femoral epiphysis, over-use injuries

A
  • adolescents
258
Q

Abnormal leg length discrepancy?

259
Q

First line investigations for kid with limp?

260
Q

What will U/S detect in kid with limp?

A

DDH <3mo
small joint effusions
US-guided joint aspiration

261
Q

Lab workup kid with limp?

A

CBC
ESR
CRP
blood cultures if sepsis suspected

  • synovial fluid analysis - for inflammatory and infectious conditions
262
Q

What is chronological delay in appearance of set developmental milestones achieved during infancy and early childhood?

A

Developmental delay

- causes: organic, psychological and environmental factors (cause of developmental lag often unknown)

263
Q

Define global developmental delay

A
  • developmental delay in >2 areas during infancy or preschool years
264
Q

What are the developmental milestones?

A
  • gross motor
  • fine motor
  • language
  • social
265
Q

Age of:
Gross motor: raises head slightly, hands fisted, primitive reflexes
Fine motor: tight grasp, follows to midline
Language: alerts to sound
Social: regards face, responds positively to feeding

266
Q

Age of:
Gross motor: holds head to midline, lift chest, supports on forearms
Fine motor: tracks past midline, waves at toys
Language: searches for sound with eyes, coos
Social: recognizes parents, social smile, moves arms actively to stimulus, anticipates feeds

267
Q

Age of:
Gross motor: rolls front-back/ back-front, sits when propped, supports on wrists
Fine motor: moves arms in unison to grasp objects
Language: orients to voice, makes razzing sounds
Social: hard regard, enjoys looking at environment, reaches out for toys, laughs

268
Q

Age of:
Gross motor: sits unsupported, feet to mouth while supine
Fine motor: reaches with one hand, transfers, raking grasp
Language: babbles, responds to name, indiscriminate dada/mama
Social: recognizes strangers, looks toward person talking to them

269
Q

Age of:
Gross motor: creeps, crawls, cruises, pulls to stand
Fine motor: pincer grasp, probes with forefinger, holds bottle
Language: understands ‘no’, waves, discriminant dada/ mama
Social: explores environment, object permanence

270
Q

Age of:
Gross motor: walks alone, creeps upstairs
Fine motor: throws objects, scribbles in imitation, builds 2-4 block tower
Language: uses 2 words, runs, unintelligible words together
Social: imitates, comes when called, cooperates with dressing, indicates wants

271
Q

Age of:
Gross motor: runs, throws toys, kicks balls, walks upstairs
Fine motor: turns 2 pages at a time, spoon feeds self, builds 4-6 block tower
Language: knows 8 body parts, uses intelligible words in margining, >20words
Social: copies tasks, plays along-side other kids, seeks approval

272
Q

Age of:
Gross motor: walks up + down stairs, overhand throw
Fine motor: turns pages one per time, removes clothes, holds pencil
Language: >50 words and 2word sentences, 25% intelligible
Social: parallel play, imaginary play, gender aware

273
Q

Age of:
Gross motor: pedals tricycle, alternates feet upstairs
Fine motor: dresses and undresses partially, copies circles
Language: 3-word sentences, minimum 250 words, knows own name, 75% intelligible
Social: group play, shares toys

274
Q

Age of:
Gross motor: hops, skips
Fine motor: buttons, catches ball, copies square
Language: colours, asks Q, prints first name
Social: tells tales, plays cooperatively

275
Q

Age of:
Gross motor: skips alternating feet, jumps over obstacles
Fine motor: ties shoes, copies triangle
Language: prints first name
Social: plays competitive games, follows rules

276
Q

Ddx developmental delay and short stature

A
  • malnutrition
  • Williams syndrome
  • Turner syndrome
277
Q

Ddx developmental delay and obesity

A
  • Prader-Willi syndrome
278
Q

Ddx developmental delay and microcephaly/ macrocephaly

A
  • micro: condition retarding brain growth

- macro: Sotos syndrome, hydrocephalus, mucopolysaccharidoses

279
Q

Ddx developmental delay and facial dysmorphism

A
  • fetal alcohol syndrome
  • trisomies
  • cri du chat
280
Q

Ddx developmental delay and skin lesions (cafe au last spots, Shagreen patches, port-wine stains)

A
  • neurofibromatosis
  • tuberous sclerosis
  • Sturge-Weber syndrome
281
Q

Ddx developmental delay and abnormal tone/ ataxia

A
  • hypertonic: cerebral palsy, neurodegenerative

- hypotonic: Prader-Willi, Down, Angelman syndromes

282
Q

Ddx developmental delay and joint contractors (sign of muscle imbalance around joints)

A
  • arthrogryposis cerebral palsy, muscular dystrophy
283
Q

Ddx developmental delay and size of genitalia

A
  • macro-orchidism= Fragile X syndrome

- hypogenatalism = Prader-Willi/ Klinefelter syndrome, CHARGE associated

284
Q

First line testing and lab investigations with developmental delay

A
  • full history/ physical
  • formal developmental assessment
  • formal hearing and vision assessment
  • if diagnosis not apparent, consider investigations

Inv

  • CBC, ferritin, vit B12
  • urea, electrolytes, creatine kinase
  • lead levels
  • TSH
  • urine metabolic screen
  • molecular karyotype
285
Q

What is a transient clinical expression of abnormal, excessive, synchronous discharges of cortical neurons?

286
Q

Define epilepsy

A
  • chronic disorder characterized by recurrent seizure episodes that are unprovoked in nature; may be due to syndromes or cryptogenic in nature
287
Q

What is status epilepticus?

A
  • ongoing seizure activity for >30 min or repetitive seizure activity without return of consciousness for >30min
288
Q

What can happen to neurons after 30min seizure activity?

A
  • ischemic and excitotoxic neuronal cell loss
289
Q

What is a psychogenic non epileptic seizure (pseudo- seizure)?

A
  • emotional or stress-related in origin; often involuntary
290
Q

Provoked seizure types?

A
  • benign suzire occurring in kids 6mo - 6yr, associated with temp >38
  • intercurrent illness
  • metabolic derangement/withdrawal
  • hemorrhagic/ ischemic stroke
291
Q

Classification of generalized seizures

A

Generalized (initial neuronal activation of both hemispheres)

  • generalized tonic-clonic (GTC) - most common; still limbs for 10-30sec then rapid jerking of limbs and trunk
  • tonic - sustained muscle contraction, high-pitched cry, <60 sec
  • clonic - rhythmic, symmetrical contractions of groups of muscles, prologned
  • atonic - sudden loss of muscle tone, <1-2sec
  • myoclonic - sudden, brief (<100 sec) involuntary muscle contractions; generalized or focal; single or repetitive; rhythmic or irregular
  • absence (staring spells) - abrupt cessation of activity with maintenance of tone, <30sec, no postictal state
292
Q

Classification of partial seizures

A

Partial (initial activation of neurons limited to part of one hemisphere)

  • simple motor - activation of cerebral cortex, may see Jacksonian march; tongue, lips, hands commonly involved
  • simple sensory - activation of sensory cortex; numbness or dysesthesias in any body part; abnormal proprioception
  • simple autonomic - activation of central autonomic network; epigastric sensation, sweating, dilated pupils
  • complex partial - any of above partial seizure subtypes + LOC
293
Q

What is atypical febrile seizure?

A
  • generalized seizure lasting >15min, focal feature or postictal paresis; occurring in series with total duration >30 min
294
Q

Features suggestive of seizure?

A
  • aura
  • identifiable triggers
  • altered breathing
  • cyanosis
  • incontinence
  • tongue-biting
  • prolonged postictal drowsiness
  • confusion amnesia
  • transient focal paralysis (Todds)
295
Q

Management seizure

A
  • ABCs, O2
  • O2 sat and cardiac monitor
  • establish IV
    rapid glucose, critical labs (Na, Ca, Mg, etc), CBC/ blood culture, toxicology screen, serum AED levels

5 min:

  • SL/PR/IV Lorazepam
  • or IV/ PR diazepam or IM midazolam
  • > repeat x1 if no control in 5min

give dextrose in first 10min (empirical or hypoglycaemic)

15min from start seizure:
- IV Fosphenytoin or IV phenytoin

20 min from start seizure:
- incubation prep
- IV/IM phenobarbital
+/- PR paradelehyde

30 min from start seizure:

  • rapid sequence intubation
  • PICU for continuous AED (anti-epileptic drug) infusion
296
Q

Rx carbamazepine or oxcarbazepine for what type seizure?

A

focal (partial)

297
Q

Rx valproate for what type of seizure?

A

generalized seizure

298
Q

Rx ethosuximide, valproic acid for what type of seizure?

A

childhood absence epilepsy

299
Q

Rx valproate, lamotrigine for what type of seizure?

A

juvenile myoclonic epilepsy

300
Q

Rx vigabatrin. ACTH for what type of seizure?

A

infantile spasms

301
Q

Rx valproate for what type of seizure?

A

unclassified epilepsy

302
Q

Define paediatric HTN

A
  • sustained elevation (on 3 separate occasions) of sBP or dBP >95th percentile for age, height, gender
303
Q

Equation for BP

A

BP = CO x SVR

304
Q

RAAS pathway

A
  • renin secreted by juxtaglomerular cells –> angiotensinogen to angiotensin I
  • -> angiotensin I –> angiotensin II via ACE
  • > angiotensin II = vasoconstriction of blood vessels (increase BP) and release of aldosterone (increase Na and H2O absorption)
305
Q

BP cuff

A
  • length = >80% circumference mid-upper arm

- width = >40% circumference mid-upper arm

306
Q

Classic triad pheochromocytoma

A
  • episode headache, diaphoresis, palpitations
307
Q

Assume child has primary HTN?

A

NO! Always do workup for secondary causes

308
Q

Investigations for kid with HTN

A
  • CBC, lytes, BUN, Cr
    +/- steroid levels, metanephrines
  • fasting labs: lipid levels, glucose
  • urine: urinalysis (R&M, C&S), b-hCG
    +/- urine toxicology if illicit drug use
  • imaging: abdo u/s re: kidneys, renal vasculature, genitourinary system
    +/- CXR, ECHO
  • sleep study re: OSA
  • ABPM x24h >5yr if considering white coat HTN
309
Q

When to treat paediatric HTN with pharmacological meds?

A
  • lifestyle x6mo but persistent HTN
  • ACEI if kid has DM and microalbuminuria or proteinuria renal disease
  • first line: ACEI, CCB, B-blockers, diuretics, ARBs
310
Q

Management HTN emergencies in kids?

A
  • acute symptomatic HTN
  • goal: initial reduction of sBP by 20%
  • > Nifedipine PO
  • continues HTN = hydrazine IV/IO
  • malignant HTN - consider ICU admission
  • > Labetalol IV; Nitroprusside infusion
311
Q

How does brain signal gonadal production of sex hormones?

A

HPG axis -> estrogen, progesterone, testosterone

312
Q

Gonadotropins?

313
Q

Is GnRH constant or pulsatile secretion? Starting when? Results?

A

Pulsatile during sleep

  • beginning 1-3yr prior to puberty (prepubertal stage)
  • > increases LH to mature and enlarge gonads resulting in increased sex hormones and secondary sex characteristics
314
Q

When does HPG axis assume normal activity?

A

Midpuberty = normal pulsatile activity and secondary sex characteristics further developed

315
Q

Female secondary sex characteristics

A
  • breast development (thelarche)
  • increased body hair: pubic, axillary (adrenarche)
  • widening hips, reduced waist-to-hip ratio
  • change in fat distribution - increase SC fat around buttocks, thighs, hips
316
Q

Male secondary sex characteristics

A
  • increased facial, axillary, chest, underarm, pubic hair
  • increased size of larynx and deepening of voice
  • increase stature and muscle mass
  • increased sweat glands and oil secretions causing acne and body odor
  • enlargement of penis
317
Q

Define precocious puberty

A
  • onset secondary sex characteristics before age 8 (females) or 9 (males)
  • females = 90% idiopathic
  • males = 75% CNS abnormality
318
Q

Ddx precocious puberty with increased FSH/LH levels

A

Gonadotropin dependent = central precocious puberty

  • idiopathic (dx of expulsion)
  • CNS tumor/lesion
319
Q

Ddx precocious puberty with normal/decreased FSH/LH levels

A

Gonadotropin independent = peripheral precocious puberty

  • adrenal
  • gonadal
  • normal variant
  • hCG secreting tumor
  • iatrogenic
320
Q

Ddx delayed puberty with decreased FSH/LH levels

A

Gonadotropin dependent = central etiology

  • constitutional delay
  • hypothalamic dysfunction
  • hypopituitarism
  • hypothyroidism
  • hyperprolactinemia
321
Q

Ddx delayed puberty with increased FSH/LH levels

A

Gonadotropin independent = peripheral etiology

  • Turner syndrome (XO)
  • Kleinfelter syndrome (47 XXY)
  • Bilateral gonadal failure
  • Hormonal
322
Q

What does bitemporal hemianopsia suggest?

A

pituitary tumor

323
Q

Male <=9yo with testicle volume >3mL vs. <3mL +/- precocious puberty

A
  • > 3mL = central precocious puberty -> investigate

- <3mL + features precocious puberty = adrenal or exogenous source of testosterone

324
Q

Define delayed puberty

A
  • sexual maturation not apparent by 13yr female or 14yr male
  • absence of menarche by 16yr or within 5yr pubertal onset (females)
  • m>f
  • female more likely to have underlying pathology
  • rule out overall growth failure
325
Q

Likely dx with webbed neck, low set ears and high arched palate; widely spaced nipples, shield chest?

A

Turner syndrome

326
Q

Cushing sx? Likely caused by?

A
  • moon facies, dorcocervical fat pad; striae

- > adrenal tumor

327
Q

How do you score age of development in male and female secondary sexual characteristics?

A

Tanner staging

328
Q

Investigations abnormal pubertal development

A
  • serum FSH, LH
    (increased in acquired gonadal failure by adolescence; if normal or decreased constitutional delay most common)
  • estradiol (female), total testosterone (male)
  • TSH
  • GnRH test (ability pituitary to respond to GnRH)
  • prolactin
  • 17-OH-P based on clinical features
  • CBC, ESR (chronic disease)
    central etiology suspected: increased ICP sx = MRI or CT head
  • bone age
  • pelvic u/s
  • adrenal u/s
  • karyotyping if peripheral delayed puberty and clinical feature suggest chromosomal abnormality
329
Q

What is bone age?

A
  • degree of bone maturation (increase with increased sex steroid hormones)
  • precocious puberty = bone age > chronological age
  • delayed puberty = bone age < chronological age
330
Q

Management central precocious puberty

A
  • tx for full adult height potential
  • GnRH analogs, GnRH agonist (Lupron) - turns off HPG by reducing GnRH receptors
  • medroxyprogesterone slows breast and genital development
331
Q

Management peripheral precocious puberty

A
  • tx underlying cause

- glucocorticoid replacement for CAH

332
Q

Management central delayed puberty

A

constitutional delay

  • reassurance
  • hormonal therapy (estrogen, testosterone) to initiate puberty in some pt

hypogonadism
- lifelong sex steroid replacement

333
Q

Management peripheral delayed puberty

A
  • tx underlying cause
334
Q

What is disorders of sex development (DSD)?

A
  • diagnostic criteria that refers to atypical development of chromosomal, gonadal, or phenotypic sex
335
Q

Normal sexual differentiation 5-9wk GA?

A

5wk GA = sexually indifferent

  • XY = paired gonadal ridges, Wolffian and Mullerian ducts
  • XX = paired gonadal ridges, Wolffian and Mullerian ducts

6wk

  • XY = begin expressing SRY (sex-determining region chrm Y) -> initiates testis formation
  • XX = SRY not expressed (no Y chrm)

7wk (biopotential gonadal formation)
- XY = testicular development begins; sertoli cells secrete MIS (Mullerian inhibiting substance, aka AMH or anti-Mullerian hormone) = Mullerian duct regression
and Leydig cells produce testosterone stabilizing Wolffian ducts and promoting development of epididymis, vas deferent, seminal vesicle
- XX = lack of MIS = Mullerian duct maturation into oviduct, uterus, cervix, upper vagina
and lack of testosterone = Wolffian duct regression

9 wk (sexually distinct external genitalia)
- XY = peripheral synthesis of DHT (dihydrotestosterone) –> differentiation and growth of male external genitalia
complete by 12-16wk
- XX = non-hormone dependent vaginal and urethral separation
complete by 12wk

336
Q

Most common cause of ambiguous genitalia?

A

CAH - congenital adrenal hyperplasia
- adrenal insufficiency can be life-threatening
- often 21-hydroxylase deficiency (autosomal recessive CYP21A2 gene mutation)
= defective conversion of 17-OH-P to 11- deoxycortisol
= reduced cortisol synthesis -> increased ACTH -> adrenal stimulation

337
Q

Classic CAH presentation?

A
  • salt losing vs. non-salt losing
    females = genital ambiguity
    males = no genital ambiguity
    -> FTT, dehydration, electrolyte abnormalities
    = adrenal insufficiency in early infancy

vs. late onset = signs androgen excess
- childhood: non-salt losing in males = accelerated bone age, premature pubarche
- adolescents, adult females = hirsutism, acne, infertility, menstrual irregularity

  • some kids remain asymptomatic
338
Q

Investigations ambiguous genitalia?

A
  • karyotype = establish chromosomal sex
  • FISH to evaluate SRY gene
  • r/o CAH = 17-hydroxyprogesterone (or less common types DHEA, androstenedione, testosterone)
  • ACTH stimulation test, cortisol level (suspect adrenal involvement)
  • 5-alpha reductase levels in undervirilized genetic male
  • serum electrolytes if concern for salt-wasting CAH

imaging: US abdo and pelvis for gonads, uterus, vagina, enlarged adrenals (CAH)

339
Q

Most common acquired vs. inherited bleeding disorders?

A
  • acquired = ITP

- inherited = hemophilia A (factor VIII deficiency)

340
Q

Underlying medical condition ddx for child abuse?

A
  • nutritional deficiency (vit K, C)
  • infection (meningococcemia)
  • inflammation (HSP, ITP)
  • malignancy and thrombocytopenia
  • genetic (coagulopathies, osteogenesis imperfecta)
341
Q

Timeline for sexual assault kit?

A

within 72h of assault

MCC Part 1 (22 decks)

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