Peds Flashcards
ASA in children should be avoided because risk of…
Reye’s syndrome
Tissue that grows primarily in first 2 years
CNS
Tissue that grows primarily in mid-childhood
Lymphoid tissue
Tissue that grows primarily in puberty
Gonads
Premature infants (<37wk) use corrected GA until age…
2yo
Birth weight
Average is 3.25kg (7lb)
20-30d/g in term neonate
Up to 10% weight loss in first 7d of life is normal
Should regain birth weight by 10-14d of age
2x birth weight by 4-5mo
3x birth weight by 1yr
4x birth weight by 2yr
Length/height
Average is 50cm (20in) 25cm in 1st yr 12cm in 2nd yr 8cm in 3rd then 4-7cm/yr until puberty 1/2 adult height at 2yo Measure supine lenght until 2yo then measure standing height
Head circumference
Average is 35cm (14in)
2cm/mo for first 3mo
1cm/mo at 3-6mo
0.5cm/mo at 6-12mo
Moro reflex
Abduction/extension of arms, opening of hands, followed by flexion/adduction of arms
Disappears by 4-6mo
Galant
Pelvis moves in direction of side that back is stroked along paravertebral line
Disappears by 2-3mo
Grasp
Disappears by 3-4mo
ATNR (asymmetric tonic neck reflex)
Fencing posture when you turn infant’s head to one side
Disappears by 4-6mo
Rooting
Disappears by 2-3mo
Parachute reflex
Ipsilateral arm extension to side infant is tilted toward while sitting
Present by 6-8mo
Does not disappear
Gross motor: 1mo
Turns head side to side when supine
Gross motor: 2mo
Briefly raises head when prone
Holds head erect when upright
Gross motor: 4mo
Lifts head and chest when prone
Holds head steady when supported sitting
Rolls prone to supine
Gross motor: 6mo
Tripod sit
Pivots in prone position
Gross motor: 9mo
Sits well without support, crawls, pulls to stand, stands with support
Gross motor: 12mo
Gets into sitting position without help
Stands without support
Walks while holding on
Gross motor: 15mo
Walks without support, crawls up stairs/steps
Gross motor: 18mo
Runs, walks forward pulling toys or carrying objects
Gross motor: 24mo
Climbs ups and down steps with 2 feet per step, runs, kicks ball
Gross motor: 3yr
Rides tricycle
Climbs up 1 foot per step, down 2 feet per step
Stands on one foot briefly
Gross motor: 4yr
Hops on 1 foot
Climbs down 1 foot per step
Gross motor: 5yr
Skips
Rides bicycle
Fine motor: 1mo
Fist with thumb in fist
Fine motor: 2mo
Pulls at clothes
Fine motor: 4mo
Briefly holds object when placed in hand
Reached midline objects
Fine motor: 6mo
Ulnar or raking grasp
Transfers objects from hand to hand
brings objects to mouth
Fine motor: 9mo
Early pincer grasp with straight wrist
Fine motor: 12mo
Neat pincer grasp
Releases ball with throw
Fine motor: 15mo
Picks up and eats finger foods
Scribbles
Stacks 2 blocks
Fine motor: 18mo
Tower of 3 cubes
Scribbling
Eats with spoon
Fine motor: 24mo
Tower of 6 cubes
Undresses
Fine motor: 3yr
Copies circle
Turns pages one at a time
Puts on shoes
Dress/undress fully except buttons
Fine motor: 4yr
Copies cross
Uses scissors
Buttons clothes
Fine motor: 5yr
Copies a triangle and square
Prints name
Ties shoelaces
Gross motor red flag
Not walking at 18mo
Rolling too early at <3mo
Fine motor red flag
Hand preference at <18mo
Speech: 1mo
Cries, startles to loud noises
Speech: 2mo
Variety of sounds (coos)
Speech: 4mo
Turns head towards sounds
Speech: 6mo
Babbles
Speech: 9mo
Mama, dada
Imitates 1 word
Responds to no regardless of tone
Speech: 12mo
2 words Follows 1 step command Uses facial expression Sounds Actions to make needs known
Speech: 15mo
4-5 words
Points to needs/wants
Speech: 18mo
10 words
Follows simple commands
Speech: 24mo
2-3 word phrases
50% intelligible
Understands 2 step commands
Speech: 3yr
Combines 3 or more words in a sentence
Recognizes colours, preopositions, plurals, counts to 10
75% intelligble
Speech: 4yr
Speech 100% intelligble
Uses past tense
Understands 3 part directions
Speech: 5yr
Fluent speech, future tense, alphabet
Retells sequence of story
Babinski sign
Present up to 2yo
Upgoing plantar reflex
Social: 1mo
Calms when comforted
Social: 2mo
Smiles responsiveley
Recognizes and calms down to familiar voice
Follows movement with eyes
Social: 4mo
Laughs responsively
Follows moving toy or person with eyes
Responds to ppl with excitement
Social: 6mo
Stranger anxiety
Beginning of object permanence
Social: 9mo
Plays games
preaches to be picked up
Social: 12mo
Responds to own name
Separation anxiety begins
Social: 15mo
Looks to see how other react (ie. after falling)
Social: 18mo
Shows affection towards other
Points to show interest in something
Social: 24mo
Parallel play
Helps to dress
Social: 3yo
Knows sex and age
Shares some of time
Playes make believe games
Social: 4yo
Cooperative play
Fully toilet trained by day
Tries to comfort someone who is upset
Social: 5yo
Cooperates with adult request most of the time
Separates easily from caregiver
Speech red flag
<10 words at 18mo
Social red flag
Not smiling at 3mo
Not pointing at 15-18mo
Breastfed infants require supplements
Vitamin D 400IU/d
Fluoride after 6mo if not sufficient in water
Iron at 6-12mo if not receiving fortified cereals/meat/meat alternatives
Foods to avoid in early infancy
Honey until past 12mo (botulism)
Added sugar, salt
Excessive milk (no more than 750mL per day after 1y)
Limit juice intake (1/2 cup daily)
Milk
Breastfeeding recommended
9-24mo: homo milk
2-6yr: 2% milk
C/I to breastfeeding
Medications known to cross into breast milk (chemo)
HIV/AIDS
Active untreated TB
Herpes in breast region
>0.5g/kg/d of EtOH or illicit drugs
OCPs are NOT a C/I to breastfeeding (estrogen may decrease lactation but is safe for baby)
Meds that cross into breast milk
Bromocrimptine High dose diazepam Gold Metronidazole Tetracycline Lithium Cyclophosphamide
Wet diaper amount
1 wet diaper per day of age for first wk
1-2 black or dark green stools/d on day 1 and 2
3+ brown/green/yellow stools per day on day 3 and 4
3+ yellow, seedy stools per day on day 5
Infantile colic
Unexplained paroxysms of irritability and crying for >3h/d, >3d/wk for >3wk in an other wise healthy, well-fed baby (rule of 3s)
Peaks at 6-8wk
Usually resolves by 3mo
When to see dentist
6mo after eruption of first tooth and definitely by 1yo
First tooth typically at 5-9mo
Enuresis
Involuntary urinary incontinence by day and/or night in child >5yo
Secondary enuresis
Involuntary loss of urine at night after child had sustained period of bladder control (>6mo)
Pharmaco treatment for enuresis
Don’t do before age 7 because often resolves spontaneously
Consider DDAVP oral tablets (antidiuretic)
Encopresis
Fecal incontinence in child >4yo at least once per mo for 3mo
Failure to thrive
Weight <3rd percentile
Falls across two major percentile curves or <80% of expected weight for height and age
Decreased weight, normal ht, normal HC
Caloric insufficiency
Decreased intake
Hypermetabolic state
Increased losses
Decreased wt, ht and normal HC
Structural dystrophies
Endocrine d/o
Constitutitional growth delay (BA < CA)
Familial short stature (Bone age = chronological age)
Decreased wt, ht, hc
Intrauterine insult
Genetic abnormality
Overweight BMI
> 85th percentile
Obesity BMI
> 95th percentile for age and height
Risk factors for SIDS
Prematurity <37wk Early bed sharing <12wk Alcohol use during pregnancy Soft bedding Low birthweight Bed sharing Aboriginals Male No prenatal care Smoking in household Prone sleeping Poverty
Prevwntion of SIDS
Back for sleeping
Avoid sharing bed, overheating, overdressing
Appropriate infant bedding
Exclusive BF in first month and no smoking
Pacifiers appear to have a protective effect
Do not reinsert if falls out during sleep
Infant monitors do not reduce incidence
Tests to order to R/O pathologic causes of fractures
Ca2+ Mg2+ PO4 ALP PTH Vit D Albumin
Tests to order to R/O pathologic causes of bruising
CBC INR PTT vWF Factors VIII/IX
Sexual assault examination kit
Within 24h if prepubertal
Within 72h if pubertal
Ductus arteriosus
Connection btwn pulmonary artery and aorta
Ductus venosus
Connection btwn umbilical vein and IVC
Prenatal circulation of oxygenated blood
Oxygenated blood from placenta –> umbilical vein –> IVC –> RA –> foramen ovale –> LA –> LV –> aorta –> brain/myocardium/upper extremties
Prenatal circulation of deoxygenated blood
Deoxygenated blood returns via SVC to RA –> 1/3 of blood goes to RV –> pulmonary arteries –> ductus arteriosus –> arota –> systemic circulation –> placenta
Ductus venosus closure
Separation of low resistance placenta –> systemic circulation becomes high resistance system
Foramen ovale closure
Increased pulmonic flow –> increased LA pressures
Ductus arteriosus closure
Increased oxygen concentration in first breath –> decreased prostaglandins –> ductus arterosus closure
Acyanotic heart disease
L to R shunt
Obstructive
Cyanotic heart disease
R to L shunt
L to R shunt
ASD
VSD
PDA
Atrioventricular spetal defect
Obstructive causes of acyanotic CHD
Coarctation of aorta
Aortic stenosis
Pulmonic stenosis
R to L shunt
Tetralogy of Fallot
Ebstein’s anomaly
5Ts of cyanotic CHD
Tetralogy of Fallot Transposition of the great arteries Truncus arteriosus Total anomalous pulmonary venous drainage Tricuspid atresia Hypoplastic left heart syndrome
Boot shaped heart on CXR
Tetralogy of Fallot
Tricuspid atresia
Egg-shaped heart
Transposition of great arteries
Left to right shunts
ASD
VSD
PDA
3 types of Atrial Septal Defects
Ostium primum
Ostium secundum
Sinus venosus (located at entry of SVC into right atrium)
Ostium primum
AKA endocardial cushion defect
Defect in atrial septum at level of tricuspid and mitral valves
Common in DS
Ostium secundum
Most common type, 50-70%
Foramen in septum primum
Foramen ovale
Foramen in septum secundum
Normally closes at birth when pulmonary vascular pressure decreases and the LA > RA
Mgmt of ASDs
80-100% spontaneously close if ASD diameter is <8mm
Elective surgical or catheter closure btwn 2-5yo
ASD heart murmur
Grade 2-3/6 pulmonic outflow murmur, widely split, and fixed S2
ECG for ASD
RAD
Mild RVH
RBBB
Most common congenital heart defect
VSD (30-50%)
Majority are small and close spontaneously
ECG for VSD
LVH
LAH
RVH
VSD heart murmur
The size of the VSD is inversely related to the intensity of the mumur
Holosystolic murmur at LLSB
Treatment of VSD
If small –> most close spontaneously
If mod/large –> tx CHF and surgical closure by 1yo
PDA
Patent vessel btwn descending aorta and the left pulmonary artery
Normal functional closure is at 15h
Normal anatomical closure within first days of life
Treatment for PDA
Premature infants have higher rates of spontaneous closure
Indomethacin (antagonizes prostaglandin E2, only effective in preterm)
Catheter or surgical closure if PDA causes resp compromise, FTT or persists beyond 3mo
PDA heart murmur
Machinery murmur continuous through systolic and diastolic at L infraclavicular area
Coarctation of the aorta
Narrowing of aorta almost always at level of ductus arteriosus
Syndrome often a/w coarctation of aorta
Turner syndrome (15-35%)
Obstructive lesions
Coarctation of the aorta
Aortic stenosis
Pulmonary stenosis
Coarctation of the aorta tx
Give prostaglandins to kep ductus arteriosus patent
Surgical correction in neonates
Balloon arterioplasty may be considered for older children
Test to differentiate btwn cardiac and other causes of cyanosis
Hyperoxic test
Obtain preductal, right radial ABG in room air, then repeat after pt inspires 100% O2
If PaO2 improves to >150mmHg –> less likely cardiac cause
Preductal and post ductal oximetry
> 5% difference suggests R to L shunt
Tetrology of fallot
VSD
Pulmonary stenosis
Aortic root overriding VSD
RVH
Most common cyanotic heart defect dx beyond infancy
Tetrology of fallot
ToF murmur
Single loud S2 due to severe pulmonary stenosis, systolic ejection murmur at LSB
ToF ECG
RAD, RVH
ToF CXR
Boot shaped heart
Decreased pulmonary vasculature
Right aortic arch in 20%
ToF mgmt
Surgical repair at 4-6mo of age
Most common cyanotic CHD in neonates
TGA
Transposition of the Great Arteries
Parallel systemic and pulmonary vasculature
Systemic: Body –> RA –> RV –> aorta –> body
Resp: Lungs –> LA –> LV –> pulmonary artery –> lungs
Survival dependent on mixing through PDA, ASD, VSD
TGA mgmt
Prostaglandin E1 infusion to keep ductus open until surgical mgmt
Total anomalous pulmonary venous return
All pulmonary veins drain into right sided circulation
ASD must be present to mix oxygenated blood to LA
Total anomalous pulmonary venous return mgmt
Surgical repair
Ebstein’s anomaly
Septal and posterior leaflets of tricuspid valve are malformed and displaced into RV
–> RV dysfunction, tricuspid dysfunction
Often A/W ASD and/or patent foramen ovale causing R–>L shunt
A/W maternal benzo and lithium use in 1st trimester
Ebstein’s anomaly mgmt
Newborn: consider closure of tricuspid valve and aortopulmonary shunt, or transplant
Older: Tricuspid valve repair or replacement
Truncus arteriosus
Single great vessel giving rise to aorta, pulmonary and coronary arteries
Requires surgical repair within first 6wk of life
Hypoplastic left heart syndrome
Hypoplastic LV
Narrow mitral/aortic valves
Small ascending aorta
Coarctation of aorta
Most common cause of death from CHD in first mo of life
Hypoplastic left heart syndrome
Hypoplastic left heart tx
Surgical palliation or heart transplant
4 key features of CHF in peds
2 tachys and 2 megalys Tachycardia Tachypnea Hepatomegaly Cardiomegaly
PVCs
Common in teens
Benign if single, uniform, disappear with exercise and no associated structural lesions
Most frequent sustained dysrhythmia in children
SVT
Still’s murmur
Innocent
3-6yo
High-pitched, vibratory, LLSB or apex, SEM
Flow across pulmonic valve leaflets
Global developmental delay
Performance significantly below average in 2 or more domains of development in a child <5yo
Intellectual disability
Historically defined as IQ<70
Limitations in both intelligence and adaptive skills
Bilingual exposure and language delay
Bilingual exposure generally does NOT explain frank delay in language development
Developmental disorder with high incidence of psychiatric comorbidity
Specific learning disorder
Most common preventable cause of intellectual disability
Fetal alcohol spectrum disorder
Fetal alcohol spectrum disorder diseases
FAS
Partial FAS
ARBD (Alcohol related brain damage)
ARND (Alcohol related neurodevelopmental disorder)
Criteria for dx of FAS
- Growth deficiency not due to nutrition
- Characteristic pattern of facial anomalies (short palpebral fissures, flattened philtrum, thin upper lip)
- CNS dysfunction, need >/= 3 (motor skills, neuroanatomy/neurophysiology, cognition, language, academic achievement, memory, attention, executive function, affect regulation, adaptive behaviour, social skills or social communication OR microcephaly in infant/young children)
Criteria for dx of ARBD
Congenital anomalies (malformations and dysplasias of cardiac, skeletal, renal, ocular and auditory systems)
Criteria for dx of ARND
CNS dysfunction
Complex pattern of behavioural or cognitive abnormalities inconsistent with developmental level that can’t be explained by familial background or environment alone
Dx criteria for DM (Types 1 and 2) in children
- Symptoms (polyuria, polydipsia, weight loss) and hyperglycemia (random glucose >/= 11.1)
OR 2. 2 of the following on one occasion: fasting glucose >/= 7, 2h plasma glucose during OGTT >/=11.1, Random glucose >/= 11.1
OR 3. One of the following on 2 separate occasions: fasting glucose >/= 7, 2h plasma glucose during OGTT >/= 11.1, random glucose >/= 11.1
T1DM
Most common form of DM in children
M=F
Bimodal, peaks at 5-7yo and at puberty
Major negative outcome of DKA in children
Cerebral edema
Tx for cerebral edema
Mannitol
Decrease fluids
Elevate head of bed
Intubate
Kussmaul breathing from DKA
Deep laboured breathing for respiratory compensation of metabolic acidosis
DKA management
ABCs
100% O2
Correct fluid losses first (NS + 40mEQ/L KCl)
Insulin 0.05-0.1U/kg/h after fluids running for 1-2h
Add glucose once glucose levels drop to keep in 8-12 range
Can replace fluids with D10NS + 40mEq KCl
DON’T GIVE BICARB
Cushing’s Triad of cerebral edema
HR low
High BP
Irregular respirations (Cheyne-Stokes)
T2DM
F > M
Less common in children but increasing rates due to child obesity
Glycemic taret HbA1c = 7%
Metformin first line
Can start on insulin if A1c > 9% at dx
Screening: Add annual screening for PCOS and NAFLD
Short stature
Height <3rd percentile
Poor growth evidenced by growth deceleration (height crosses major percentile lines, growth velocity <25th percentile)
Short stature ddx
ABCDEFS Alone (neglect) Bone dysplasias (rickets, scoliosis, mucopolysaccharidoses) Chromosomal (turner, down) Delayed growth (constitutional) Endocrine (low GH, Cushing, hypothyroid) Familial GI malabsorption (Celiac, Crohn's)
Investigations for short stature
Calculate mid-parental height Boys: (mother + father’s height in cm + 13)/2
Girls: (mother + father’s height in cm - 13)/2
AP xray of left hand and wrist fot bone age
GH testing
Other tests based on hx/pe
GH therapy for GH deficiency
May help reach adult height if given at an early age AND
1. GH shown to be deficient by 2 diff stimulation tests (arginine, glucagon, insulin)
2. Growth velocity <3rd percentile or height <3rd perventile
Bone age xrays show unfused epiphyses/delayed bone age
Measure proportionality
Calculate Upper/lower segment ratio using pubic symphysis as landmark Normal newborn: 1.7 Normal child: 1.4 Normal adult: 0.9 Normal female: 1
Proportionate short stature with slow growth velocity
Endocrine (height more affected than weight) Chronic disease (weight affected more than height) Psychosocial neglect
Tall stature
Height greater than 2 SD above the mean for a given age, sex and race
Beckwith-Wiedemann Syndrome
Overgrowth syndrome
Growth slows by ~8yo and adults are not unusually tall
May grow asymmetrically (hemihyperplasia)
A/w omphalocele, umbilical hernia, macroglossia, visceromegaly, creases near ears, hypoglycemia, renal abnormalities
Increased risk of cancerous and noncancerous tumours (esp Wilms tumour and hepatoblastoma) - 10%
Normal life expectancy
Tall stature etiology
Constitutional/familial
Endocrine: Beckwith-wiedemann syndrome, hyperthyroidism, hypophyseal gigantims, precocious puberty
Genetic: homocystinuria, klinefelter syndrome, Marfan syndrome, sotos syndrome
Homocystinuria
Disorder of methionine metabolism causing abnormal accumulation of homocysteine
Characterized by myopia, dislocation of lens, bloot clotting, osteoporosis, developmental delay
Sotos syndrome
Genetic disorder
Distinctive facial appearance (long, narrow face, high forehead, flushed cheeks, pointed chin, down-slanting palpebral fissures), overgrowth in childhood, learning delay
Adult height usually normal
A/W ADHD, phobias, OCD, impulsivity
Scoliosis, sz, heart or kidney defects, hearing loss, vision problems
Neonatal grave’s disease/Congenital hyperthyroidism
Typically caused by transplacental transfer of TSH receptor antibody
A/w low birthweight, IUGR, microcephaly, prematureity, tachy, frontal bossing, triangular facies, hepatosplenomeg, goiter
Neonatal grave’s investigations
TSH receptor antibody levels during 3rd trim or in cord blood
Neonatal TSH, T3, free T4
Neonatal grave’s mgmt
Methimazole and beta adrenergic blocker (ie. propranolol)
Should resolve within a few weeks
Congenital hypothyroidism epidemiology
F:M = 2:1
One of the most common preventable cause of intellectual disability
Congenital hypothyroidism clinical manifestations
Usually asymptomatic in neonatal period b/c maternal T4 crosses placenta
Prolonged jaundice, feeding difficulty, lethargy, constipation, umbilical hernia, macroglossia, large fontanelles, puffy face, swollen eyes
Congenital hypothyroidism investigations
Most commonly detected at newborn screen of TSH
Rpt screening at 2wks in high risk infants (preterm, very low birth weight, NICU, specimen collection <24h
Abnormal results confirmed with serum levels from venipuncture
Primary congenital hypothyroidism lab results
Increased TSH, low free T4
Secondary congenital hypothyroidism lab results
Low TSH, low free T4
CH treatment
Thyroxine replacement within 2wk to avoid cognitive imapirment
If tx started after 3-6mo, may result n permanent developmental delay
Congenital adrenal hyperplasia
Autosomal recessive disorder characterized by partial or total defect of various synthetic enzymes required for cortisol and aldo production in adrenal cortex
Most common cause of ambiguous genitalia in genotypically normal females (46XX)
Congenital adrenal hyperplasia
Enzyme responsible for CAH 95% of the time and function
21-hydroxylase mutation
Leads to decrease in cortisol and aldosterone production –> increased shunting of precursors to androgens
Cortisol deficiency leads to elevated ACTH = adrenal hyperplasia
Classic 21-OH deficiency with salt wasting
Inadequate aldosterone resulting in FTT, hyper kalemia, hyponatremia, hypoglycemia, acidosis (majority of classic type_
Classic 21-OH deficiency without salt wasting
Simple virilization with adequate aldosterone
Females: Amenorrhea, precocious puberty, polycystic ovaries, hirsutism
Males: Typically asymptomatic at birth, may have hyperpigmentation, penile enlargement, rapid growth and accelerated skeletal maturation, virilization later in life
Non-classic CAH
Mild androgen excess, sometimes asymptomatic, virilization present later in life
CAH investigation
High serum levels of 17-OH progesterone
Newborn screening
Assess plasma ACTH, serum electrolytes, plasma glucose, plasma aldo, plasma renin, blood gas
U/S to look for enlared adrenal gland and presence of uterus
CAH treatment
Glucocorticoids (ie. hydrocortisone); more in times of stress
Mineralocorticoids (ie. fludrocordisone) as necessary to reduce ACTH levels
HPG axis during puberty
Pulsatile release of GnRH –> increased release of LH and FSH –> maturation of gonads, release of sex steroids –> secondary sexual characteristics
Female puberty
Onset: age 8-13 (7 in African descent), earlier common
- thelarche (breast budding)
- pubarche (axillary hair, body odour, mild acne)
- growth spurt
- menarche (mean age 12.5)
Male puberty
Onset: age 9-14yo, earlier uncommon 1. Testicular enlargement 2. Penile enlargement 3. Pubarche (axillary and facial hair, body odour, mild acne) 4. Growth spurt (occurs later in boys) Gynecomastia common and self limited
Female breast tanner staging
1: papilla elevation
2: breast and papilla elevated as small mound, areola enlarging
3: Enlarging breast and areola, no contour separation
4: areola and papilla form secondary mound
5: mature, nipple projects, no secondary mound
Female genital tanner staging
1: no hair, prepubertal
2: small amount of long, straight or curled, slighlty pigmented along labia majora
3: Darker, coarser, curlier hair distributed sparsely over pubis
4: adult-type hair; no extension to medial thighs
5: mature distribution with spread to medial thighs
Male genital tanner staging
1: no hair, prepubertal
2: small amount of long, straight or curled slightly pigmented hair along base of penis. enlargement of testes and scrotum, reddening of scrotal skin
3: darker, coarser, curlier hair distributed sparsely over pubis. lengthening of penis, further enlargement of testes and scrotum
4: adult type hair, no extension to medial thighs. Increasing penile circumference and length, development of glands, further enlargement of testes and scrotum, darkening of scrotal skin
5: mature distribution with spread to medial thighs. Adult size
Precocious puberty in females
<8yo
Precocious puberty in male
<9yo
Central cause of precocious puberty
GnRH dependent
hypergonadotropic hypergonadism
Hormone levels as in normal puberty
Premature activation of HPG axis
May be normal, CNS disturbance (tumours, hamartomas, post-meningitis, increased ICP, radiotherapy), NF, primary severe hypothyroidism
If proven central cause, get MRI of brain
Peripheral cause of precocious puberty
GnRH independent
Hypogonadotropic hypergonadism
Adrenal d/o (CAH), testicular/oavarian tumour, gonadotropic/hCG secreting tumour (ie. hepatoblastoma, intracranial teratoma, germinoma), exogenous steroid administration, McCune-Albright syndrome, primary severe hypothyroidism
McCune-Albright Syndrome
Genetic disorder
Develop fibrous tissue in bones (polyostic fibrous dysplasia) –> asymmetric growth
Cafe au lait spots w/ irregular borders
Precocious puberty (menstrual bleeding at very young age)
Goiter
Hyperthyroidism
Acromegaly
Tx of central precocious puberty
GnRH agonists (ie. leuprolide)
Tx of peripheral precocious puberty
Meds that decrease production of specific sex steroid or block its effects (ie. ketoconazole, spironolactone, tamoxifen, anastrozole)
Surgery
Delayed puberty in males
Lack of testicular enlargement by 14yo
Delayed puberty in females
Lack of breast development by 13yo or absence of menarche by 16yo or within 5yr of pubertal onset
Central causes of delayed puberty
Constitutional delay in HPG axis activation (most common)
Hypogonadotropic hypogodanism
Peripheral causes of delayed puberty
Hypergonadotropic hypogonadism (ie. primary gonadal failure, gonadal damae, Turner’s syndrome, hormone deficiency, androgen insensitivity syndrome)
Tx of delayed puberty
Hormone replacement
Cyclic estradiol and progesterone for females
Testosterone for males
Mild degree of dehydration
5% in <2yo, 3% in >2yo Normal pulse Normal BP Decreased UO Slightly dry oral mucosa Normal ant fontanelle Normal eyes Normal skin turgor Normal cap refill
Moderate degree of dehydration
10% in <2yo, 6% in >2yo Rapid pulse Low to normal BP Markedly decreased UO Dry oral mucosa Sunken anterior fontanelle Sunken eyes Decreased skin turgor Normal to increased cap refill
Severe degree of dehydration
15% in <2yo, 9% in >2yo Rapid, weak pulse Decreased in shock (very late finding in peds and very dangerous) Anuria Parched oral mucosa Markedly sunken anterior fontanelle Markedly sunken eyes Tenting Increased cap refill
Mild dehydration tx
Rehydrate with ORT at 50cc/kg over 4h
Moderate dehydration tx
Rehydrate with ORT at 100cc/kg over 4h
Severe dehydration tx
IV resuscitation with NS or RL at 20-40cc/kg over 1h
Begin ORT when pt stable
IV fluid for newborn
D10W
IV fluids for first month of life
D5W/0.45 NS + KCL 20mEq/L (only add KCl if voiding well)
IV fluids for children
D5W/NS + KCl 20mEq/L or D5W/0.45NS + KCl 20 mEq/L
Pyloric stenosis - 3Ps
Peristalsis
Pyloric mass
Projectile vomiting (2-4wk after birth)
Fussiness after feeds, spit ups, arching of back, poor weight gain
GERD
GERD investigations
Empiric trial of acid suppression
pH monitoring study
Upper GI study
Endoscopy
Bilious emesis ddx
Malrotation with volvulus
Meconium ileus
Duoenal atresia/stenosis
Hirschsprung’s disease
Upper GI series sign for duodenal atresia
Double bubble sign
Triad of intussusception
- colicky progressive abdo pain
- bilious vomiting
- red currant jelly stool
Gastroesophageal reflux vs GERD
Reflux is vomiting typically soon after feeding, non-bilious, very common, rarely progresses to GERD
GERD = reflux when it causes troublesome symptoms (ie. poor weight gain, heart burn, asthma, URTIs, OMs)
Sigmoid volvulus AXR sign
Coffee bean sign
Diarrhea
Infants = increase in stool frequency to 2x as often per day
Older children = 3+ loose or watery stools/day
Acute: <2 wk
Chronic: >2wk
Most common virus causing gastroenteritis
Rotavirus
Stool analysis (bacterial vs viral)
Leukocytes/erythocytes –> bacterial/parasitic
pH <6 and presnece of reducing substances –> viral
Most common cause of chronic diarrhea in thriving child
Toddler’s diarrhea
Toddler’s diarrhea
Onset at 6-36mo
Ceases spontanesouly btwn 2-4yo
4-6 BM/d
Dx of exclusion in thriving child
Toddler’s diarrhea tx
4Fs Fibre Normal fluid intake 35-40% fat Discourage excess fruit juice
Skin rash a/w celiac disease
Dermatitis herpetiformis
Diagnosing celiac disease
Serum anti-tTG antibody (type of IgA antibody)
IgA deficient pts have false negative anti-tTG
MUST measure IgA concomitantly
Small bowel mucosal biopsy (usually duodenum) is dx with increased intraepithelial lymphocytes (earlist path finding)
Milk allergy
IgE mediated
Skin, upper and lower respiratory symptoms within hours of milk exposure
Cow’s milk protein allergy
Non-IgE mediated, more common
Most common cause of acute abdo after 5yo
Appendicitis
Common site for intussusception
Ileocecal junction
Intussusception tx
Air enema or hydrastatic pressure
Sugery rarely needed
Physiologic anemia
Normal to fall to as low as 100g/L at 8-12wk of age due to shorter fetal RBC lifespan
Usually no tx required
Most common cause of childhood anemia
Iron deficiency anemia
Mentzer index
MCV/RBC
Helps distinguish Fe def anemia vs thalassemia
Ratio < 13 = thalassemia
Ratio > 13 = iron deficiency
Blood smear indicative of fe-def anemia
Hypochromic, microcytic RBCs
Pencil shaped cells
Poikylocytosis
Iron studies in iron-def anemia
Low ferritin Low Fe High TIBC High transferrrin Low TSat
Fe def anemia tx
Oral iron therapy: 4-6mg/kg/d elemental iron divided BID to TID for 3 mo
Repeat Hb after 1mo of treatment
Most common cause of thrombocytopenia in childhood
Immune thrombocytopenic purpura
Immune thrombocytopenic purpura
Binding of antiplatelet antibody to platelet surface, leading to removal and destruction of platelets in spleen and liver
ITP presentation
50% present 1-3wk after viral illness
No lymphadenopathy
No hepatosplenomegaly
Sudden onset petechiae, prupura, epistaxis in otherwise well child
ITP management
Spontaneous recovery in >70% of cases within 3mo
IVIg or prednisone if significant bleeding, plt <10 or at risk of significant bleeding
avoid contact sports
avoid ASA/NSAIDs
Most common type of pediatric malignancy
Leukemia
Leukemia classification
ALL (80%)
AML (15%)
CML (<5%)
Most common presentation of Hodgkin lymphoma
Persistent, painless, firm, cervical or supraclavicular lymphadenopathy
Hodgkin lymphoma treatment
Combo chemotherapy and radiation
Non-Hodgkin lymphoma treatment
Combo chemotherapy
No added benefit of radiation in peds protocols
Most common primary renal neoplasm of childhood
Nephroblastoma (Wilm’s Tumour)
Most common cancer occurring in first year of life
Neuroblastoma
Nephroblastoma prognosis
90% long term survival
Neuroblastoma prognosis
Poor
Often late detection
Fever without a source
Acute febrile illness (typically <10d) with no cause discerned even after careful hx and physical
Fever of unknown origin
Daily or intermittent fevers for at least 2 consecutive wk of uncertain cause after careful hx and P/E and initial lab assessment
More common bacterial causes of AOM
S. pneumoniae
H. influenza
M. catarrhalis
GAS
AOM tx
Watchful waiting if appropriate
Abx if <6mo or moderately/severely ill (Amoxicillin 75-90mg/kg/d dosed BID x10d for 6mo-2y and 5d if >/2 yr)
Most common cause of pediatric hearing loss
OM with effusion
Erythema infectiosum
AKA Fifth disease/slapped cheek
Parvovirus B19
Flu-like illness for 7-10d then rash appears ~10-17d after
Erythematous maculopapular rash, lacy on cheeks, may affect trunk/extremities
Supportive mgmt
Giannoti-Crosti Syndrome
EBV and Hep B
* Asymptomatic symmetric papules
* On face, cheeks, extensor surfaces of extremities, spares trunk
* Viral prodrome
* May have lymphadenopathy, hepatosplenomegaly
* Asymptomatic symmetric papules
* On face, cheeks, extensor surfaces of extremities, spares trunk
* Viral prodrome
* May have lymphadenopathy, hepatosplenomegaly
Supportive mgmt
Pain control
Resolves in 3-12wk
Hand, foot, mouth dz
Coxsackie group A
* Vesicles and pustules on erythematous base
* Extremities
* May have vesicles in posterior oral cavity
Supportive mgmt
Measles
Morbillivirus
* Airborne transmission
* Prodrome of fever, cough, coryza, conjunctivitis
* Maculopapular rash starts on neck, behind ears and along hairline 3d after start of symptoms
* Rash spreads downward, reaching feet in in 2-3d
* Initial rash on buccal mucosa as red lesions with bluish white spots in center (Koplik spots)
Supportive
Unimmunized contacts: measures vaccine within 72h of exposure or IgG within 6d of exposure
Roseola
HHV6
* Exanthem subitum = blanching, erythematous macules start on neck and trunk —> spread to arms (less commonly face and legs)
* Rash typically preceded by 3-4d of high fevers –> end as rash appears
* Usually in children <2yrs
Supportive tx
Rubella
- Droplet transmission
- Prodrome of low grade fever, sore throat, arthritis
- Pink, maculopapular rash appears 1-5d after start of symptoms
- Starts on face and spreads to neck and trunk
Supportive
MMR vaccine for prevention (caution with pregnant women)
Varicella
Varicella zoster virus Mainly airborne transmission Groups of polymorphic lesions (macules, papules, vesicles, crusts) Generalized distribution Significant pruritus Supportive Consider antiviral
Mono classic triad
Febrile
Generalized non-tender lymphadenopathy
Pharyngitis/tonsillitis
Coarctation of the aorta often associated with…
Bicuspid aortic valve (75-85%)
CXR findings of coarctation of the aorta
Upper left mediastinal shadow
Cardiomegaly may be seen in older children
Dilated intercostal collateral arteries may erode 3rd to 8th ribs causing rib notching (years to develop)
Coarctation of the aorta murmur
Grade 2-3/6 ejection systolic murmur best heard in left interscapular area
Ejection click may be aduible when associated with bicuspid aortic valve
Treatment for GAS pharyngitis
Penicillin V
Amoxicillin
or Erythromycin x 10d
Tx prevents rheumatic fever but does NOT alter risk of post-strep GN
PANDAS
Pediatric autoimmune neuropsychiatric disorder associated with group A streptococci
Scarlet fever
Hypersensitivity rxn to exotoxin produced by GAS
Fever, sore throat, strawberry tongue
Sandpaper rash ~24-48h after pharyngitis (blanchable, perioral sparing, non-pruritic, non-painful)
Tx: Penicillin, amoxicillin, or erythromycin x 10d
Rheumatic fever
Due to antibody cross-reactivity following GAS
Tx: Penicillin or erythromycin for acute course x 10d, prednisone if severe carditis
Post-strep GN
Occurs 1-3wk after initial GAS infection
Dx confirmed with elevated serum antibody titres against strep antigens (ASOT, anti-DNAse B), low serum complement (C3)
Tx: fluids, Na+ restriction, loop diuretics for HTN and edema
Tx: Penicillin or erythromycin if evidence of persistent GAS infection
Prognosis: 95% children recover completely within 1-2 wk
Bacterial meningitis CSF findings
WBC < 100x10^5 Neutrophils 100-10000 x 10^5 (may be normal) Glucose <0.4 (CSF:Blood), may be normal Protein > 1 (may be normal) RBC 0-10
Viral meningitis CSF findings
WBC 10-1000 x 10^5 (may be normal) Neutrophils usually <100 x 10^5 Glucose level usually normal Protein 0.4-1 ( can be normal) RBC 0-2
Herpest meningitis CSF findings
WBC 50-1000 (can be normal) Neut <100 Glucose <0.3 Protein 1-5 RBC 10-50
Common culprits of bacterial meningitis in 0-4wks
KLEG Klebsiella (-) L. monocytogenes (+) E. coli (-) GBS (+)
Common culprits of bacterial meningitis in 1-23mo
H. SN-EG GBS E. coli S. pneumoniae N. meningitidis H. influenzae
Common culprits of bacterial meningitis in >2yo
SNL
S. pneumo
N. meningitis
L. monocytogenes
Empiric abx for 0-28d suspected meningitis
Ampicillin + Cefotaxime
Empiric abx for 28-90d suspected meningitis
Cefotax + Vancomycin (+ Amp if immunocompromised)
Empiric abx for >90d
Ceftriaxone +/- vancomycin
HSV meningitis tx
Acyclovir
Pertussis bacteria
Bordtella pertussis
Gram negative pleomorphic rod
Stages of pertussis
Prodromal catarrhal stage: 1-7d, URTI symptoms, no or low-grade fever
Paroxysmal stage: 4-6 wk, whooping cough
Convalescent stage: 1-2wk, decreased frequency of coughing episodes, may last up to 6mo
Pertussis treatment
Macrolide (azithro, erythromycin, clarithromycin) if B. pertussis isolated or symptoms present <21d
Droplet isolation until 5d of tx
Report to Public Health
Macrolide abx for all household contacts
Vaccine
Common bacterial culprit of preseptal cellulitis in children
H. influenza
Common bacterial culprits of preseptal cellulitis in adults
S. aureus
Streptococcus
Treatment for preseptal cellulits
Amox-clav
Treatment for orbital cellulitis
Admit
Blood cultures x 2
Orbital CT
Ceftriaxone + Vancomycin IV x 7d
Cardinal signs of orbital cellulitis
- Ophthalmoplegia/diplopia
- Decreased VA
- Pain with extra ocular eye movement
Common bacterial culprits for UTIs
KEEP (Gram neg) Klebsiella E. Coli Enterobacter Pseudomonas and S. saprophyticus (+) Enterococcus (+)
Empiric abx for UTI in neonate
IV amp and gent
Abx ourse: 7-10d
Empiric abx for infant/older children
Oral abx (based on local E. coli sensitivity
If requiring IV: IV amp and gent
Abx course: 7-10d
UTI imaging for neonates
Kidney/bladder U/S for:
- Children < 2y.o. with first febrile UTI
- Children of any age with recurrent febrile UTIs
- Children of any age with UTI who have fam hxof renal or urologic disease, poor growth, hypertension
- Children who do not respond as expected to appropriate antimicrobial therapy
Voiding cystourethrogram
X-rays to take pictures of urinary system
- Children of any age with ≥ 2 febrile UTIs OR
- Children of any age with first febrile UTI AND abnormality on renal U/S OR
- Fever ≥ 39C and pathogen other than E. coli OR
- Poor growth or hypertension
Small for gestational age
2 SD < mean weight for GA or <10th percentile
Large for gestational age
2 SD > mean weight for GA or >90th percentile
Low birthweight
<2500g
Very low birthweight
<1500g
Extremely low birthweight
<1000g
APGAR
Appearance (colour) Pulse Grimace (irritability) Activity (tone) Respirations
If <7 at 5min, then reassess q5min until <7
APGAR scores of 1
HR <100/min Slow, irregular resps Grimace Some flexion of extremities Body pink, extremities blue
APGAR scores of 2
HR > 100/min Good, crying resps Cough/cry Active motion Completely pink
HR at which to start chest compressions in neonate
HR < 60
Treatment for apnea of prematurity (<34wk)
Methylxanthines (caffeine) to stimulate CNS and diaphragm
Not for term infants
Hemorrhagic disease of the newborn
Vitamin K deficiency
Both PT and PTT are abnormal since factors X, IX, VII, II are affected
Hypoglcyemia in newborn
Glucose <2.6mmol/L
Preferred imaging modality to investigate intraventricular hemorrhage
Head U/S
Neonatal hyperbilirubinemia
Total serum bili >95th percentile (high risk zone) on Butani nomogram in infants >3wks GA
Jaundice is pathological if
- It occurs within 24h of birth
- Conjugated hyperbilirubinemia is present (>35)
- Unconjugated bilirubin rises rapidly or is excessive for pt’s age and weight (>257 in full term)
- Persistent jaundice lasts beyond 1-2 wk of age
Enzyme used to conjugate bilirubin
Glucoronyl transferase (Immature in neonates)
Enzyme in meconium used to hydrolyze conjugated bili back to unconjugated bili
Beta glucuronidase
Causes increased enterohepatic circulation
Physiologic jaundice
Total bili = 257umol/L
Peaks at 3-4d of life, resolves by 10d of life
Breastfeeding jaundice
Lack of milk production –> dehydration –> exaggerated physiologic jaundice
Early in first week of life
Breast milk jaundice
Onset at 7d of life, peaks at 2-3wk, usually resolved by 6wk
Glucoronyl transferase inhibitor in breast milk
Galactosemia
Genetic disorder (GALT gene) causing inability to properly process galactose
A/W jaundice, cataracts, lethargy, repro problems in females
Decreased level of erythrocyte galactose-1-phosphate uridyltransferase
Criggler Najjar Syndrome
- RARE auto recessive conditions that causes severe unconjugated hyperbilirubinemia starting in first few days of life
- Results from decreased bili clearance caused by deficient (type 2) or completely absent (type 1) UDPGT = kernicterus
Gilbert Syndrome
- UGT1A1 gene (auto recessive): Decreased enzyme function interferes with gluronidation àconjugation of bili is slowed
- Intermittent, self-resolving epis of unconjugated hyperbilirubinemia
- Usually STARTS in adolescence
Kernicterus
Unconjugated bili concentrations exceed albumin binding capacity –> bili deposited in brain = tissue necrosis and permanent damage to basal ganglia or brainstem
Incidence increases as bili > 340umol/L
Unconjugated hyperbilirubinemia tx
- Phototherapy
- Exchange transfusion (mostly for hemolytic or G6PD)
- IVIg if severe (DAT+)
Biliary atresia
Atresia of extrahepatic bile ducts which leads to cholestasis and increased conjugated bili after 1st week of life
Tx: Kasai procedure (anastomosis to allow bile to drain directly into intestine); 2/3 require liver transplantation
Necrotizing enterocolitis
Primarily affects terminal ileum and colon
Internal inflammation a/w focal or diffuse ulceration and necrosis
Necrotizing enterocolitis AXR hallmark
Pneumonitis intestinalis (intramural air)
Persistent Pulmonary Hypertension of the Newborn
Persistence of fetal circulation due to persistent elevation of pulmonary vascular resistance (ongoing R–>L shunt through PDA, foramen ovale –> decreased pulmonary blood flow and hypoxemia –> further pulmonary vasoconstriction)
Respiratory distress syndrome
Deficiency of lung surfactant —> poor lung compliance due to high alveolar surface tension —> atelectasis —> decreased SA for gas exchange —> hypoxia + acidosis —> respiratory distress
- Resp distress within first few hours of life, worsens over next 24-72h
- Hypoxia
- Cyanosis
Most common cause of respiratory distress in premature infants
Respiratory distress syndrome
RDS treatment
Resuscitation, oxygen, ventilation
Surfactant (decreases alveolar surface tensions, improves lung compliance and maintains functional residual capacity)
RDS CXR findings
Ground glass
Air bronchograms
Decreased lung volume
Transient tachypnea of the newborn
Delayed clearance of fluid from lungs following birth
Usually in late and late preterm babes born via C/S to diabetic moms
Tachypnea with no hypoxia or cyanosis
TTN Tx
Supportive
O2 if hypoxic
Ventilator support
Recovery expected in 24-72h
TTN CXR
Perihilar infiltrates
No consolidation/air bronchograms
Snowman heart on CXR
Total anomalous pulmonary venous return
Tricuspid atresia murmur
Single S2 with 2-3/6 systolic regurg murmur at LLSB if VSD is present
Hirschprung disease
Absence of myenteric plexus in distal colon
40% of children with this do not pass mec in first 24h
Dx via full-tickness rectal bx
Tx: remove aganglionic bowel and restore continuity of healthy bowel with distal rectum
Most commonly used diuretic for CHF edema in children
Lasix
then thiazide diuretic as second line/in combo
Mongolian spots
Congenital dermal melanocytosis
Hemolytic uremic syndrome
Simultaneous occurrence of triad of:
Non immune MAHA
Thrombocytopenia
Acute renal injury
Most common cause of acute renal failure in children
Hemolytic Uremic Syndrome
Most common cause of HUS in peds
E. coli O157:H7, shiga toxin
Tx of HUS
- Mainly supportive (nutrition. hydration, ventilation if needed, blood transfusion for symptomatic anemia
- Steroids not helpful
- Abx are C/I as death of bacteria leads to increased toxin release and worse clinical course
Nephritic syndrome
PHAROH: Proteinuria (>50mg/kg/d) Hematuria (>5 RBCs/hpf) Azotemia RBC casts Oliguria HTN
Most common in 5-15yo
Most common cause of acute GN in pediatrics
Post-infectious GN
Causes of nephritic syndrome
Post-infectious GN Membranoproliferative IgA nephropathy Idiopathic rapidly progressive GN Anti-GBM disease HSP Granulomatosis with polyangitis Goodpasture's syndrome Polyarteritis nodosa SLE Bacterial endocarditis Abscess or shunt nephritis Cryoglobuminemia
Nephrotic syndrome
PALE Proteinuria (>50mg/kg/d) HypoAlbumoinemia (<20g/L) HyperLipidemia Edema Most common in 2-6yo, M>F
Most common cause of nephrotic syndrome in peds
Primary idiopathic
Nephrotic syndrome etiology, glomerular inflammation absent on renal biopsy
Minimal change disease (85%)
Focal segmental glomerular sclerosis
Nephrotic syndrome etiology, glomerular inflammation present on renal biopsy
Membranoproliferative GN
IGA neprhopathy
Causes of nephrotic syndrome
AI (SLE, DM, rheumatoid arthritis)
Genetic (sickle cell dz, alport)
Infections (hep B/C, post-strep, infective endocarditis, HUS, HIV)
Malignancies
Meds (NSAIDs, antiepileptics)
Vasculitides (HSP, granuloamtosis with polyangiitis)
Congenital
Often first sign of nephrotic syndrome
Edema (periorbital, pretibial)
Urine findings of nephrotic syndrome
3-4+ proteinuria, microscopic hematuria
Microscopy (oval fat bodies, hyaline casts)
First morning urine protein/creatinine ratio (>200mg/mmol)
Tx for nephrotic syndrome
Often corticosteroids
Furosemide + albumin for generalized edema
May need statin therapy
ACEI/ARBs for persistent HTN
HTN in childhood
sBP and/or dBP >/= 95th percentile for sex, age and height on >/= 3 occasions
Treatment of HTN in peds
Gradual BP lowering using thiazide diuretics
If HTN emergency, use hydralazine, labetalol, sodium nitroprusside
Cerebral palsy
Non-progressive central motor impairment syndrome due to insult to or anomaly of immautre CNS
Types of cerebral palsy
Spastic (70-80%) – UMN of pyramidal tract affected
Athetoid/dyskinetic (10-15%) – Basal ganglia
Ataxic (<5%) – cerebellum
Mixed
Simple febrile seizure
ALL of: <15min Generalized tonic-clonic No recurrence in 24h period No neuro impairment or developmental delay before or after seizure
Complex febrile seizure
At least 1 of: Duration >15min Focal onset or focal features Recurrent (>1 in 24h period) Previous neuro impairment or neuro deficit after sz
Neurofibromatosis type I
Autosomal dominant
Dx requires 2 or more of:
>/= 6 cafe au lait spots (>5mm if prepuberal, >1.5cm if postpubertal)
>/=2 neurofibromas of any type or one plexiform NF
>/=2 Lisch nodules (hamartomas of iris)
Optic glioma
Freckling in axillary or inguinal region
Distinctive bony lesion
First degree relative with confimed NF-1
Neurofibromatosis type II
Autosomal dominant
Bilateral vestibular schwannomas or 1st degree relative with NF2 and either unilat vestibular schwannoma OR any 2 of: meningioma, glioma, schwannoma, NF, posterior subcapsular lenticular opacities
Infantile spasms
Brief repeated symmetric contractions of neck, trunk, extremities lasting 10-30s, often a/w developmental delay
Infantile spasm EEG
Hypsarrhythmia (high voltage slow waves, spikes and polyspikes, background disorganization)
West syndrome
Infantile spasms
Psychomotor developmental arrest
Hypsarrhythmia
Infantile spasm tx
ACTH injections
Vigabatrin (GABA inhibitor)
Benzo
Lennox Gastaut
Triad of:
Multiple seizure types
Diffuse cognitive dysfunction
Slow generalized spike and slow wave EEG
Seen with underlying encephalopathy and brain malformations
Lennox-Gastaut tx
Valproic acid
Benzos
Ketogenic diet
Juvenile Myoclonic epilepsy (Janz Syndrome)
Generalized tonic clonic sz
Autosomal dominant with variable penetrance
Juvenile myoclonic epilepsy EEG
3.5-6Hz irregular spike and wave, increased with photic stimulation
Juvenile myoclonic epilepsy mgmt
Lifelong treatment with valproic acid
Childhood absence epilepsy
Multiple absence sz per day lasting <30s without post-ictal state
May resolve spontaneously or become generalized in adolescence
Childhood absence epilepsy EEG
3Hz spike and wave
Childhood absence epilepsy mgmt
Valproic acid or ethosuximide
benign focal epilepsy of childhood with rolandic/centrotemporal spikes
Focal motor sz involving tongue, mouth, face, upper extremity usually in sleep-wake transition states
Remains conscious but aphasic post-ictally
Benign focal epilepsy with rolandic spikes EEG
Repetitive spikes in centrotemporal area with normal background
Benign focal epilepsy tx
If frequent sz: carbamapazemine
If infrequent sz: no tx needed
When to initiate tx for sz
Often if >2 unprovoked afebrile sz within 6-12mo
When to discontinue tx for sz
Until pt free of sz for >2yr then wean over 4-6mo
Chronic asthma management
Rescue ventolin (SABA) 1st line: low dose ICS 2nd line <12yo: moderate dose of daily ICS 2nd line >12yo: leukotriene receptor antagonist OR LABA + low dose ICS or Leukotrine antagonist monotherapy
Age to start PFTs
> 6yo
self-monitor with peak flows to improve self-awareness of status
Bronchiolitis
LRTI usually in children <2yo
Leads to increased incidence of asthma in later life
Most common viral etiology of bronchiolitis
RSV
Time course of bronchiolitis
Peaks at 3-4d, lasts 2-3wk
Cystic fibrosis presenting signs
CF PANCREAS
Chronic cough and wheeze
FTT
Pancreatic insufficiency (ie. steatorrhea)
Alkalosis and hypotonic dehydration
Neonatal intestinal obstruction (ie. mec ileus), Nasal polyps
Clubbing, CXR findings
Rectal prolapse
Electrolyte elevation in sweat, salty skin
Absence or congenital atresia of vas deferens
Sputum with S. aureus or P. aeruginosa
Testing for CF
Sweat chloride test x 2
CFTR gene mutation analysis
Croup
Laryngotracheobronchitis
Subglottic laryngitis
Barking cough, stridor, worse at night
Most commmon in 6mo-3yo
Croup etiology
Parainfluenza (75%)
Influenza A and B
RSV
Adenovirus
Croup CXR
Steeple sign from subglottic narrowing
Not needed for dx
Croup tx
Stridor at rest is emergency No evidence for humidifed O2 Dexamethasone PO 1 dose Racemic epi neb 1-3 doses, q-12h Intubation if unresponsive to tx
Bacterial tracheitis
Subglottic tracheitis Rare Caused by S. aureus, H. influenza, pneumococcus, M. catarrhalis Rapid deterioration IV abx and intubation
Epiglottitis
Supraglottic laryngitis
4Ds: Drooling, dysphagia, dysphonia, distress
Avoid examining throat to prevent further exacerbation
Epiglottitis etiology
H. influenzae
beta-hemolytic strep
Juvenile idiopathic arthritis
Arthritis in >/=1 joint(s) for >/= 6wk, onset age <16yo
Exclusion of other causes of arthritis
Classification defined by features/numbers of joints affected in first 6mo of onset
Still’s disease/systemic arthritis
Onset at any age
1-2x/d fever spikes (>38.5C) >/= 2d/wk with temps returning below baseline
Erythematous salmon-coloured maculopapular rash, lymphadenopathy, hepatosplenomegaly, leuk, thrombocytosis, anemia, serositis
Oligoarticular arthritis
1-4 joints Most common type of JIA Typically large joints (knees, elbows, wrists) ANA + in 60-80% RF neg
Polyarticular arthritis
>/=5 joints RF neg (usually) - symmetrical, large and small joints of hands and feet, TMJ, cervical spine RF pos - severe, rapidly destructive, symmetrical, large and small joints
Enthesitis-related arthritis
Weight-bearing joints, esp hip and intertarsal joints
Risk of developing ank spond
Psoriatic arthritis
Arthritis + psoriasis OR
arthritis and at least 2 of dactylytis, nail pitting or other abnormalities, or fam hx of psoriasis in a 1st degree relative
Asymmetric or symmetric small or large joint involvement
Juvenile idiopathic arthritis tx
1st line: NSAIDs, intra-articular corticosteroids
2nd line: DMARDs (MTX, sulfasalazine), corticosteroids, biologics (IL1, IL6 inhibition for systemic arthritis, TNF antagonist for polyarticular JIA)
Lyme arthritis
Caused by spirochete Borrelia burgdorferi
Do not treat children <8yo with doxycycline (may cause permanent tooth discolouration)
Reactive arthritis
Salmonella, Shigella, Yersinia, Campylobacter, Chlamydia, Streptococcus
Reiter’s syndrome
Conjunctivitis
Urethritis
Arthritis
Occuring after an infection
Transient synovitis of the hip
Benign, self-limited inflammatory joint disorder usually after URTI, pharyngitis, AOM More common on right side Painful limp but full ROM Symptomatic and anti-inflammatory meds Usually resolves in 24-48h
Most common vasculitis of childhood
Henoch-Schonlein Purpura
Henoch-Schonlein Purpura
Vasculitis of small vessels, often following 1-3wk of URTI
Clinical triad:
Palpable purpura (non-thrombocytopenic purpura in lower extremities, scrotal swelling)
Abdo pain (GI bleed, intussusception)
Arthritis (large joints)
HSP tx
Mainly supportive
Anti-inflammatory meds for joint pain, corticosteroids for select pts
Self-limited, resolves within 4wk
Most common cause of acquired heart disease in children in developed countries
Kawasaki disease
Kawasaki disease
Medium-sized vasculitis with predilection for coronary arteries
Likely triggered by infection
Kawasaki dx criteria
HOT CREAM
Fever persisting >/= 5d AND >/=4 of:
Conjunctival injection (bilateral, non exudative)
Rash (polymorphous)
Edema/erythema of hands and feet
Adenopathy (cervical >1.5cm in diameter, usually unilateral)
Mucosal changes (fissured lips, strawberry tongue, injected pharynx)
Kawasaki tx
IVIG and high dose ASA
Once afebrile >48h, low dose ASA until plts normalize
Baseline echo and follow up periodic echo usually at 2 and 6wk
Risk a/w kawasaki
Coronary aneurysm
If receive IVIg within 10d onset, reduces risk
Most common organisms a/w septic arthritis
Staph aureus Streptococcus (neonate --> group B, infant/older child --> group A and strep pneumo) H. influenza type B N. gonorrhea Kingella kingae
Slipped capital femoral epiphysis
Posterior displacement of capital femoral ephiphysis from femoral neck through cartilage growth plate
Causes limp and impaired internal rotation
Common in adolescents
Tx: Internal reduction of femoral head
Legg-Calve Perthes dz
Most commonly affects boys btwn 4-10yo
Avascular necrosis of capital femoral ephiphysis
Refer to ortho
Developmental dysplasia of hip
Most common in females born breech
Femoral head not properly aligned with acetabulum
Most common cause of neonatal and antenatal hydronephrosis
Ureteropelvic junction obstruction
Potter’s syndrome
Bilateral renal agenesis
Cryptorchidism
Most common genital progrem
Presents with empty and hypoplastic or poorly rugated scrotum or hemiscrotum
DiGeorge Syndrome
CATCH-22
Congenital heart abnormalities
Abnormal facies
Thymic aplasia
Hypocalcemia from hypoparathyroidism Failure of 3rd and 4th branchial pouch development
–> poorly developed thymus and parathyroid
HypoPTH –> HypoCa –> Tetany
Low T-cell maturation –> recurrent infections (esp candida)
Gene deletion in chromosome 22q11
Tx: Ca supplement and thymus tissue transplant
Suggestion of impending respiratory failure in asthma attack
PaCO2 > 43
3 most common bacterial infections in cystic fibrosis patients
Staph aureus
H. influenza
Pseudomonas aeruginosa (most common in pts >10)
Bacterial infection in CF a/w multiple antibiotic resistance
Burkholderia cepacia
Most beneficial agent for long-term mucus clearance in cystic fibrosis patient
Aerosolized dornase alfa (DNAse to help break down polymerized DNA dervied from degraded neutrophils in viscous mucus)
Bilirubin induced neurologic dysfunction
Caused by indirect hyperbilirubinemia
Kernicterus, opisthotonus , delayed motor skills, choreoathetosis, sensorineural hearing loss
Tx: Immediate exchange transfusion
Epiphora
Overflow tearing in neonates
Congenital nasolacrimal duct obstruction
Occurs in 5% normal newborns
Blockage commonly at valve of Hasner at distal end of duct
Blockage can be unilateral or bilateral
Spontaneous resolution is estimated to be 90% within first year of life
Test with dye disappearance test – should drain into nose within 5 minutes if no obstruction
If obstructed, dye remains in eye and is seen as bright green tear meniscus or dye escapes over eyelid and drains down cheek
Car seats (stage 1)
Rear-facing seats should be used until children weigh at least 10kg and are at least 1y of age and able to walk
No more than 2.5cm/1in of movement in either direction
Car seat (stage 2)
Forward- facing infant/child car seat
10-18kg
Car seat (stage 3)
Child booster seat
18-36kg
Car seat (stage 4)
Seatbelt
At least 36kg or 145cm or 8yrs ago
Fragile X Syndrome
X-linked disorder (expansion of CGG trinucleotide repeat) Most common inherited cause of intellectual disability Prominent jaw, forehead, nasal bridge Long thin face with large ears Macroorgchidism Hyperextensibility High arched palate May have sz, scoliosis, MV prolapse
Botulism symptoms
Poor ability to suck pooled secretions Poor head control Hypotonia Weak cry Constipation Lethargy Facial weakness Extraocular muscle paralysis Irritability Hyporeflexia Sluggish pupils Resp difficulty
Type of NTD that is not compatible with life
Anencephaly
AFP trend in maternal serum consistent with NTD
Increased at 16-18wks
What to test in amniotic fluid to dx NTD
AFP
Acetylcholinesterase
PECARN >2yo
CT Head if one of: AMS (altered mental status) GCS <15 Signs of basal skull fracture Observe or CT head if: History of LOC History of vomiting Severe headache Severe mechanism (>5 feet fall) If none of the above, no CT head required
PECARN <2yo
CT Head if one of: AMS GCS <15 Palpable skull fracture Observe or CT head if: LOC > 5 seconds Nonfrontal hematoma Not acting normally Severe mechanism (>3 feet fall) If none of the above, no CT head required
CXR for inspiratory body
Deviates away from side of obstruction
Most common locations for foreign body obstruction
Upper esophageal sphincter
Middle of esophagus where it crosses over aortic arch
Lower esophageal sphincter
Tx for developmental dysplasia of the hip
<6mo: Pavlik harness
>6mo: Closed reduction with spica cast
Most common cause of painless hematochezia
Meckel’s diverticulum
Juvenile Idiopathic Arthritis
<16yo, for at least 6 wks
Morning stiffness at least 1h in the morning, decreased ROM, swelling, fevers, rash, post-exercise pain
ANA+ (a/w uveitis –> most examine with slit lamp for inflammatory cells/increased protein in anterior chamber of eye within 1mo of dx)
Anti-cyclic citrullinated peptide antibody (specific)
Abdo pain in DKA can be a/w…
Hypokalemia causing skeletal muscle weakness and ileus
Order ECG –> flattening of T- waves and prolonged QRS
Klinefelter features
Tall
Small testicles
Infertile
Osgood Schlatter
Common Begins at onset of teen growth spurt, resolves in 18-24mo Clinical dx Pain confined to tibial tubercle of knee Worse with activity Conservative mgmt
Most common neuro sequelae after meningitis
Sensorineural hearing loss
Test hearing 4-6wk post tx
Respiratory distress syndrome long-term complication
Bronchopulmonary dysplasia
Respiratory distress syndrome acute complication
Alveolar rupture
Hypothyroidism and bone/height age relative to chronological age
Delayed bone age relative to height age and chronological age
CF and bone/height age relative to chronological age
Bone and height age are equivalent and both lag behind chronological age
Chromosomal abnormalities and maternal substance abuse and bone/height age relative to chronological age
Height age delayed relative to bone age
ITP purpura vs vasculitic purpura (ie. HSP)
ITP purpura is non pruritic and non palpable in dependent areas of body
Most common congenital abnormality causing primary hypogonadism
Klinefelter
Physiologic genu varum
Resolves by 2 yo
Xrays would show normal mineralization of growth plates