Pediatrics2 Flashcards
The most common congenital heart defect
VSD
Inv for congenital CHF
Echo ECG CXR Pre- and post-ductal O2 saturation 4limb BP Hyperoxia test
Amount of deoxyhemoglobulin causing cyanosis
30 d/dL
O2 sat <75%
Snowman heart
Total anomalous pulmonary venous return
Egg-shaped heart
Transposition of great arteries
Boot-shaped hearts
TOF
Types of STD
Ostium premium (common in DS) Ostium secundum (most common) Sinus venosus
Natural Hx of ASD
80-100% spontaneous closure rate if diameter < 8 mm
If remains patent: CHF and pulmonary HTN
Clinical presentation of ASD
Asymptomatic in childhood
If large: HF
2-3/6 pulmonary outflow murmur
Widely split and fixed S2
Inv in ASD
ECG: RAD, mild RVH, RBBB
CXR: increased pulmonary vasculature, cardiac enlargement
Echo: test of choice
Mx of ASD
Elective surgical/catheter closure between 2-5 yr of age
Clinical presentation of VSD
Small:
Asymptomatic
Early systolic/holosystolic murmur at LLSB, thrill
ECG: normal
CXR: normal
Echo: confirms Dx
Mod-large VSD:
CHF by 2 mo, late 2° pHTN
Holocystolic murmur at LLSB, mid-diastolic rumble at apex
ECG: LVH, LAH, LVH
CXR: increased pulmonary vasculature, cardiomegaly, CHF
Echo: diagnostic
Mx of VSD
Small:
Closes spontaneously
Mod-large:
Mx CHF
Surgical closure by 1yr
Functional and anatomical closure of PDA
F: within 15 h of birth
A: within first days
Natural Hx of PDA
Spontaneous closure common in premature, less common interm
Clinical presentation of PDA
Asymptomatic
Or
Bounding pulses, wide pulse pressure, hyperactive precordium.
Continuous machinery murmur at left infraclavicular area
Inv in PDA
ECG:left arterial enlargement, LVH, RVH
CXR: nl or mildly enlarged heart, increased pulmonary vasculature, prominent pulmonary artery
Mx of PDA
Indomethacin for premature
Catheter/surgical closure if:
Persistent beyond 3 mo
Respiratory compromise
FTT
Sx of obstructive cardiac lesions
Decreased U/O Palloe Cool extremities Poor pulses Shock Sudden colapse
Coarctation of aorta associations
Turner
Bicuspid aortic valve
Coarctation Sx
Blood pressure discrepancy between upper and lower extremities (esp if > 20)
Deminished/delayed femoral pulses, relative to brachial
Systolic murmur with late peak at: apex, left axilla, left back
If severe: shock in neonate with PDA closure
Inv of coarctation
ECG: LVH
Echo/MRI for Dx
Mx of coarctation
PG to keep PDA patent
Surgical correction in neonate
Balloon arterioplasty in older children
Aortic stenosis Sx
Asymptomatic
Or
CHF
Exertional chest pain, syncope, sudden death
SEM at RUSB
Aortic ejection click at apex (if valvular)
Echo for Dx
The most common type of aortic stenosis
Valvular
Mx of aortic stenosis
Valvular: balloon valvuloplasty
Sub/supra-valvular: surgery
Exercise restriction
Pulmonary stenosis, most common type
Valvular
Pulmonary stenosis Sx
If critical:
Dependent on PDA
Hypoxia, cyanosis
Wide split S2 on expiration
SEM at LUSB
Pulmonary ejection click if valvular
Associations of PS
TF
Congenital rubella
Noonan
Inv for PS
ECG: RVH
CXR: post-stenotic dilation of the main pulmonary artery
Echo
Mx of PS
Surgery if critically ill or symptomatic older infants/children
Wide split S2 in
ASD:
Fixed, wide
PS:
Wide in expiration
Characteristic features of non-cyanotic heart defects
ASD:
Wild split and fixed S2
VSD:
Early systolic/holosystolic murmur at LLSB
Mid-diastolic rumble at apex
PDA:
Bounding pulses, wide pulse pressure, precordial hyperactivity
Continuous machinery murmur at left infraclavicular area
Coarctation:
BP discrepancy between upper and lower extremities (>20), diminished/delayed femoral pulses
Systolic murmur at apex, axilla, back
AS:
Syncope, exertional chest pain
SEM at RUSB, click at aoex
PS:
Wide split S2
SEM at LUSB
ejection click
Hyperoxic test
Performed in cyanosis (O2 sat <75%)
Differentiates between cardiac and other causes of cyanosis
Obtain Rt radial ABG in room air(preductal)
Repeat after child inspires 100% O2
If PaO2 improves to >150, cyanosis less likely cardiac in origin
Pre-, post-ductal pulse oximetry
If > 5% difference= suggests R to L shunt
TOF Sx
Peak Dx :2-4 mo
Initially no cyanosis (L to R shunt)
Later, R to L shunt and cyanosis
Hypoxic tet spells:
During exertional states: => increase in R to L shunting => paroxysms of rapid and deep breathing, irritability, crying, increasing cyanosis, decreased intensity of murmur
May lead to: decreased LOC, seizures, death
Single loud S2
SEM at LSB
Inv for TOF
ECG: RAD, RVH
CXR: boot-shaoed heart, decreased pulmonary vasculature, Rt aortic arch
Echo
Mx of tet spells
O2
Knee-chest position
Fluid bolus
Morphine
Propranolol
Tx of TOF
Surgical repair at 4-6 mo
Earlier if marked cyanosis or tet spell
Transposition of great arteries Sx
PDA closure causes rapid deterioration: severe hypoxemia, acidosis, death
If VSD present: no prominent cyanosis. But CHF within first weeks of life
Inv for TGA
ECG: RAD, RVH,
CXR: egg-shaped heart, narrow mediastinum
Echo
Mx of TFA
PGE1 infusion
Then balloon atrial septoplasty
Arterial switch surgery performed in 1st 2 wks
Mx of total anomalus pulmonary venous return
Surgery, urgent if severe cyanosis
Epstein anomaly associations
Maternal Li an BDZ in T1
Ebstein anomaly
Septal and posterior leaflets if tricuspid valve are malformed and displaced into the RV
RV dysfunction
Functional PS
TR
WOW
congenital heart defect with potential for coronary ischemia
Truncus arteriosus
Tx: surgery within first 6 wk
Most common cause of death from CHD in first month of life
Hypoplastic left heart syndrome
Mx of hypoplastic left heart syndrome
Intubate,
Correct metabolic acidosis
IV PGE1
Surgical palliation/heart transplant
Heart anomalies depending on PTA
Hypoplastic left heart syndrome
TGA
Coarctation of aorta
Uncommon CHF Sx in children
Orthopnea
PND
Dependent edema
Key features of CHF
Tachycardia
Tachypnea
Cardiomegaly
Hepatomegaly
Inv for CHF
CXR
ECG
Echo
CBC, lytes, BUN, Cr, LFT
Mx of CHF
Setting up
O2
Sodium and water restriction
Increased caloric intake
Diuretics
Afterload reduction (ACEI)
BB
Dig rarely
Correction of underlying
Tachycardia in infants
> 150
Pediatric ECG findings that may be normal
HR >100
Shorter PR interval, QT interval, QRS duration
Inferior and lateral small Q waves
RV larger than LV in neonates
RAD
Large precordial R waves
Upright T waves
Inverted T waves in the anterior precordial leads
Benign PVC features
Single
Uniform
Disappear with exercise
No associated structural lesion
Causes of PAC
Normal variant Or Lyte disturbances Hyperthyroidism Cardiac surgery Dig toxicity
SVT in children
HR > 220 in infants
HR > 180 in children
P absent or abnormal
No beat-to-beat variability
Narrow QRS
PR indeterminable
How to differentiate sinus tachycardua from SVT
Slow the sinus rate by vagal massage, BB to identify P wave
Congenital heart block
Mother SLE,
Anti-RO, anti-La
Dx in utero (hydrops)
Tx: pace maker
Innocent murmur characteristics
Asymptomatic
SEM
< 3/6
Soft, blowing, vibratory
Physiologic S2
No extra sounds
Varies with change in position
Pathological murmur
Sx/signs of cardiac disease
All:
Diastolic
Pansystolic
Continuous (except venous hum)
3/6 or more
Fixed split or single S2
May have other sounds/clicks
Unchanged with position change
Innocent heart murmur locations
LLSB:
Still (flow across pulmonic valve leaflets)
LUSB:
Pulmonary ejection
Peripheral pulmonic stenosis
Supraclavicular:
Supraclavicular arterial bruit (carotid)
Infraclavicular:
Venous hum
Definition of global developmental delay
Performance significantly below average in two or more domains of development (gross motor, fine motor, speech/language, cognitive, social/personal, activities of daily living) in a child 5 yr of age
Inv for global developmental delay (GDD)
Neurodevelopmental assessment
Neuroimaging
Vision and hearing test
EEG
Sleep study
OT, PT, SLP assessments
Psychosocial evaluation
Lead level, CBC, ferritin, TSH
Glucose, Lytes, lactate, ammonia, liver function, pyruvate, alb, TG, aa, urine organic acids, acylcarnithines, CPK
Genetic: microarray, fragile X testing, testing for inborn errors of metabolism
Mx of global developmental delay
Depends on specific area of delay
Speech and language therapy for language delay
OT/PT for motor delay
Early intervention services
Intellectual disability definition
State of functioning that begins in childhood
Limitation in intelligence and adaptive skills
IQ< 70
Often preceded by Dx of GDD
Areas of adaptive functioning deficit in itellectual disability
At least 2 of: Communication Self-care Home-living Social skills Self-direction Academic skills Work Leisure Health Safety
Classification of intellectual diabilities
Mild 50-70
Moderate 35-49
Severe 20-34
Profound <20
Inv for intellectual disability
Standardized psychology assessment (IQ, measure of adaptive functioning)
Vision
Hearing
Neurologic
Genetic/metabolic testing
Mx of intellectual disability
Main objective: enhance adaptive functioning level
Community-based Tx,
Early intervention
Individual/family therapy
Behavior management services
Therapy services: OT, SLP
Medications
Education: life skills, vocational training, communication skills, family education
Psychosocial support, respite care
Language delay definition
At least 1 SD below mean of age on standardized testing
Types:
Expressive
Receptive
Both
M>F
RFs for SNHL
Genetics syndromes/family history
Congenital (TORCH)
Craniofacial abnormalities
<1500 g birthweight
Hyperbilirubinemia/kernicterus
Asphyxia/low APGAR
Bacterial meningitis, viral encephalitis
RFs for language delay
FHx Male Prematurity LBW Hearing loss
Inv for language delay
Language specific screens: The Early Language Milestone
CAT/CLAMS, MCHAT…
Developmental evaluation
Refer to audiologist if needed
CBC, lead level
Genetic, metabolic W/U
Mx of language delay
Multidisciplinary
Early intervention services
Special education services
SLP, OHNS, dental professionals, general support services
Prevention:
Parents can read aloud to their child
Engage in dialogue reading
Avoid baby talk
Narrates daily activities
Best prognosis among etiologies of speech delay
Developmental speech delay
Persistence of language delay beyond this age is likely to persist into adulthood
5 yr
The effect of bilingualism on language delay
No effect
Specific learning disorder
Specific and persistent failure to acquire academic skills despite conventional instructions, adequate intelligence, and sociocultural opportunity
Types:
Dyslexia
Dyscalculia
Writing
Psychiatrist comorbidities associated with specific learning disorders
Anxiety Dysthymia Conduct disorder Major depressive disorder Oppositional defiance disorder ADHD
Possible etiologies of specific learning disorder
Turner Kleinfelter Prematurity LBW Birth trauma/hypoxia CNS damage Hypoxia Environmental toxins FAS Psychosocial deprivation Malnutrition
POOR VISUAL ACUITY IS NOT A CAUSE
RFs for specific learning disorders
Positive family history
Prematurity
Developmental/mental health conditions
Neurologic disorders
CNS infection/irradiation/traumatic injury
Inv for specific learning disorder
Psychoeducational assessment
Scores on achievement tests on reading, writing or math, > 2 SD below that expected for age, education, IQ
Evaluate: attention, memory, expressive language, coordination skills
Mx of Specific learning disorder
Quality instruction for specific learning disability
Modification of the curriculum
Providing accommodations
Grade retention in certain students
Specialized education placements
Most common preventable cause of intellectual disability
Fetal alcohol spectrum disorder
3 facial features of fetal alcohol syndrome
Flattened philtrum
Thin upper lip
Short palpebral fissures
Criteria for Dx of FAS
Growth deficiency:
LBW, decelerating weight over time
Characteristic facial features
CNS dysfunction: 3 or more of: Motor skills Neuroanatomy/neurophysiology Cognition Language Academic achievement Memory Attention Executive function Affect regulation Adaptive behavior Social skills Social communication Microcephaly
Criteria for Dx of ARBD (alcohol related brain-damage)
Congenital anomalies including: Cardiac Skeletal Renal Ocular Auditory
Criteria for diagnosis of ARND (alcohol-related Neurodevelopmental disorder)
CNS dysfunction similar to FAS
Behavioral and cognitive abnormalities
Mx of FASD
Early diagnosis is essential
Patients and their families should be linked to community resources and services to improve outcome
DM Dx in children (1 or 2)
- Symptoms + random BS> 11.1
- Two of:
Fasting glucose > 7
2 h OGTT > 11.1
Random glucose > 11.1 - One of the following on 2 separate occasions:
Fasting glucose >7
2h OGTT > 11.1
Random > 11.1
Random glucose is not appropriate for confirmation
Mx of DM1
Diet: Consistent levels of carbs Avoiding food with high glycemic index Exercise Yearly influenza vaccine Blood glucose monitoring
Screen for complications:
Ophthalmology assessment
Microalbuminuria
BP
Screen for concurrent AI disorders, mental health issues, hyperlipidemia
Extensive activity involving legs in DM1 pts
May cause prolonged hypoglycemia
Tx of DKA
ABC 100% O2 Admit Monitor Correct fluid ECG ( for abn T) Ins (1st SC, then IV) Correct lytes Identify/treat participating event Avoid complications Low threshold to investigate and treat cerebral edema (CT, MRI, mannitol) Frequent BG, lyte, fluid monitoring)
Tx of hypoglycemia in DM1
Dextrose
Glucagon
Glucose
RFs for DM2
Obesity FHx Female PCOS Hyperglycemia exposure in utero Certain ethnic groups
Breastfeeding effect on risk of Childhood DM2
Reduces the risk
Inv for DM
FPG
If very obese with multiple RFs:
OGTT
Mx of DM2
Ins if severe metabolic decompensation: DKA A1C > 9% Unclear Dx (DM1/DM2) Random BG > 14
Can wean off ins later
Initially LSM for 3-6 mo: Diet Wt loss Physical activity (at least 60 min/d) Screen time < 2h/d \+/- metformin
HbA1c target: < 7%
If target not achieved with LSM:
Metformin (1st line. Could start at Dx)
Glimepiride
Insulin
Screening:
Same as DM1
Also: PCOS, NAFLD annually
DM2 monitoring
A1c q 3 mo
Finger-stick blood glucose if: On meds with risk of hypoglycemia If changing med regimen If have not met Tx goals If intercurrent illness
A1c target in different age ranges
<6 yr : <8%
6-12 yr: <7.5%
> 12 yr: <7%
Definition of short stature
Ht < 3rd percentile
Poor growth evidences
Growth deceleration:
Ht crosses major percentile lines
Growth velocity < 25th percentile
RFs for GH deficiency
Previous head trauma
Hx of intracranial bleed or infection
Head surgery/RT
FHx
Breech delivery
Inv for short stature
Mid-parental Ht
AP Xray of left hand and wrist
GH testing
Other inv guided by Hx and PEx
Requirements for GH therapy for short stature
GH shown to be deficient by 2 different stimulation tests:
Arginin
Ins
Glucagon
Growth velocity < 3rd percentile or height «_space;3rd percentile
Bone Xray shows unfused epiphyses/delayed bone age
DDx of proportional (upper body/lower body) short stature with normal growth velocity
Constitutional growth delay
Familial
Constitutional growth delay
Delayed puberty
May have family Hx of delayed puberty
May need short-term: androgen/estrogen
Delayed bone age
Mid-parental height is often nl
Familial short stature
Normal bone age
Tx not indicated
FHx of short stature
DDx of proportionate short stature with slow growth velocity
Endocrine: GH deficiency Hypothyroidism/Hyperthyroidism Hypercortisolism Hypopituitarism Adrenal insufficiency
Chronic disease: Cyanotic CHD Celiac IBD CF Chronic infections CRF
Psychosocial neglect:
Decreased Ht and Wt (and HC if severe)
Tall stature
Ht > 2 SD above the mean for a given age, sex, race
Tall stature DDx
Constitutional/familial
Endocrine: Hyperthyroidism Gigantism Precocious puberty Beckwith-Wiedemann
Genetic: Homocystinuria Kleinfelter Marfan Sotos
Questions to ask when short stature:
IUGR?
Proportional?
Velocity?
Bone age?
The most common cause of congenital hypothyroidism
Thyroid gland dysgenesis
Causes of transient hypothyroidism
Maternal:
Antibody mediated
Iodine deficiency
Prenatal exposure to anti-thyroid medications
Neonatal:
Neonatal iodine deficiency or excess
Congenital liver hemangiomas
Certain gene mutations
Screening for congenital hypothyroidism
Screen all neonates: TSH or free T4
Repeat screening at 2 wk if: Preterm LBW Infant in NICU Specimen collection < 24 h of life Multiple births
If abn results:
Confirm with serum levels from venupuncture