Peds E1- Emphasized only

What is Eisenmenger’s syndrome?

complication of longstanding/uncorrected congenital heart anomalies that produce L→R shunting

What is the tx for Eisenmenger’s syndrome?

supportive, heart/lung transplant, endocarditis prophylaxis

What are sx of PPHN?

tachypnea, resp. distress, cyanosis, poor cardiac funciton/perfusion, hypotension, shock, loud single S2

What is the GOLD standard for dx Pulm HTN?

cardiac cath

What is the tx for Pulm HTN?

no cure; can use medications to improve sx and life expectancy

*Warfarin, Digoxin, Vasodilators

What heart defects are associated w/ Downs Syndrome?

AV canal/septal defects, ASD, VSD

What heart defects are associated w/ Marfan Syndrome?

aortic root dilation/dissection, MVP

What heart defect is associated w/ maternal Lithium ingestion?

Ebstein’s Anomaly of the Tricuspid Valve

What is Marfan Syndrome caused by?

mutation in FBN1 gene → effects fxn of fibrillin-1 in connective tissues

Which heart murmurs are innocent?

Still’s, Pulm ejection, Pulm flow, Venous hum, Carotid bruit

What is a key characteristic of Still’s murmur?

systolic, vibratory or musical nature; usually 3-6 yo

What are some characteristics of a Pulm Ejection murmur?

low grade, blowing, dec w/ inspiration, sitting up, standing up; 8-14 yo

What is a Venous Hum caused by?

turbulence in the SVC

What are the characteristics of a Venous Hum murmur?

loudest @ infra-clavicular area (especially w/ sitting/standing), disappears while lying supine, intensity changes w/ rotation of head, disappears w/ jugular compression; 3-6 yo

What are the 8 S’s of innocent murmurs?

Soft, Systolic, Short, Sounds (S1/2), Symptomless, Special tests normal, Standing/sitting variation, Sternal depression

What acute tx can be used to stop a tachyarrhythmia?

Vagal (ice to face, valsalva, gag reflex), IV adenosine or verapamil, DC cardioversion

What causes vasovagal syncope?

venous pooling → dec venous return → hypotension/bradycardia

What are the 2 factors that help infective endocarditis develop?

bacteremia & structural abnormality of heart/great vessels w/ significant flow turbulence or pressure gradient (VSD, TOF, IHSS (HOCM))

What is the major Duke criteria for dx infective endocarditis?

1-blood cultures (+)

2-evidence of endocardial involvement

What is the leading cause of acquired heart disease in children?

Kawasaki disease

What is one of the MC vasculitis in childhood?

Kawasaki disease

What age is most likely to get Kawasaki disease?

6 mo - 5 yo

*more frequent in those of Asian ancestry

T/F: Kawasaki is a contagious disease

False

What is the etiology of Kawasaki?

unknown, thought to be an immune rxn

Why do you need to act fast if Kawasaki disease is suspected?

affects the heart muscles and valves early

What are the sx of Kawasaki disease?

persistent high fever (> 5 days) + 2 or more of the following:

• strawberry tongue, cherry red lips

• changes in hands and feet

• diffuse rash (worse in diaper area)

• bloodshot eyes w/o d/c

• swollen glands

*may not all be present at the same time

Pneumonic for remembering Kawasaki sx

FEARS ME

Fever x 5 days, Eyes -conjunctivitis, Arthralgia, Rash, Skin desquamation (hands/feet), Mucosal involvement (strawberry tongue, dry lips), Elevated ESR/Plts

What are the cardiac manifestations of Kawasaki disease?

coronary artery aneurysms, HF, depressed contractility, MI, arrhythmias, peripheral arterial occlusion

How are coronary artery aneurysms dx?

Echo & angiography

When are coronary artery aneurysms of concern?

*> 50% resolve w/in 2 years

giant aneurysms >8mm are more likely to cause MI

What are signs that the Kawasaki infxn is affecting the heart?

tachycardia out of proportion to fever, gallop heart sounds, muffled heart sounds if effusion, risk of coronary artery aneurysms (highest risk in infants)

What lab values are suggestive of Kawasaki?

elevated WBC, inc plts, inc acute-phase reactants (CRP, ESR, fibrinogen), anemia, pyuria

What is the Kobayashi score used for?

predict pts w/ KD at high risk of failing to respond to primary IVIG tx

What is the tx for Kawasaki?

IVIG

• most effective in 1st 7-10 days onset

• 5x reduction in CA vs untx

• high risk of resistance → add glucocorticoids

Aspirin

• 30-50g/kg daily divided into 4 doses

• continue until plt, ESR, CRP are normal

What are the most common causes of Myocarditis in children?

Coxsackie B, echovirus

How do you prevent RHD?

proper tx of strep pharyngitis w/ abx

What infxns inc risk of CHD?

TORCHES CLAP

toxoplasmosis, other (zika), rubella, CMV, Herpes & Hepatitis, Entero virus, Syphilis, Chicken pox, Lyme disease, AIDs, Parvovirus B19

What CHDs are acyanotic?

CoA, AS, PDA, VSD, ASD, A-V canal defect, PS

What does Lithium use during pregnancy cause?

Epstein anomaly

What heart defect does Fetal Alcohol Syndrome cause?

ASD or VSD

What would a CXR show in pts w/ a VSD?

cardiomegaly, LAE, inc pulm vasculature

What CHD is common in Down’s syndrome pts?

ASD

What murmur is present w/ ASD?

soft systolic ejection @ ULSB; wide (fixed) split S2

What murmur is associated with a PDA?

pansystolic, machinery-like w/ bounding peripheral pulses & wide PP

What is the tx for a PDA?

Indomethacin or Ibuprofen OR surgery

What is the triad of sx associated w/ AS?

SAD: syncope, angina, dyspnea

How does CoA present in infants?

CHF or shock

How does CoA present in older children?

HTN

What are the MC clinically significant defects associated w/ CoA?

PDA, VSD, AS

What PE findings are associated with CoA?

SEM @ ULSB; bounding radial pulses, diminished femoral pulses, BP elevated in UE & lower in LE, cardiogenic shock

What is the tx for CoA?

surgery/cath = definitive

severe: prostaglandins to temp. reverse PDA closure

What would you see on a CXR of CoA?

figure 3

What are cyanotic CHD?

ToF, TGA, HLH, tricuspid atresia, truncus arteriosus, total anomalous pulm venous connection

What are the 4 components of ToF?

PROV: PS, RVH, Overriding aorta, VSD

What are sx of ToF?

“tet spells” in infants (hypoxic episodes, thromboembolism, squatting, polycythemia

What would you see on a CXR of ToF?

normal size heart; boot-shaped heart; dec pulm vasculature

What do see on a CXR of TGA?

cardiomegaly “egg-on-string”; inc pulm vasculature; narrow mediastinum

What would you see on a CXR of TAPVC?

cardiomegaly, “snowman” appearance, inc pulm vasculature

What is a Temper tantrum?

Child cries and/or harms him/herself to manipulate the parents/guardians

When do temper tantrums usually resolve?

By age 5

How should you handle tantrums?

redirect child to something else, avoid provoking situation, ignoring the tantrum

What are breath holding spells?

Child holds their breath and suddenly loses consciousness

Are cyanotic or pallid breath holding spells more common and is related to a temper tantrum?

Cyanotic

What is ADHD?

Chronic pattern of attention difficulties, impulsivity, and/or hyperactivity that interferes with daily life

What is the diagnostic criteria for ADHD?

6+ sx x min 6 months (inattention & hyperactivity), inconsistent w/ developmental level, have negative impacts on social/academic activities, present in 2+ settings

What is the gold standard questionnaire for diagnosis of ADHD?

*> 90% sensitivity & specificity

Conner's scale

What questionnaire is designed for Teacher/Parents and commonly used to dx ADHD?

*high sensitivity & specificity if both used

Vanderbilt ADHD Diagnostic rating scale

Which questionnaire is good for monitoring the effectiveness of medications in children with ADHD?

Conner's scale

What stimulant is commonly used for ADHD?

Methylphenidate (Ritalin)

Which ADHD medications are stimulants?

Ritalin, Concerta, Adderall, Vyvanse

What is a common side effect of Ritalin?

reduced growth, wt loss, poor weight gain

Which stimulant is less addictive than Adderall and Concerta?

Vyvanse

Which ADHD medications have less appetite suppression?

Non-stimulants: Stattera and Intuniv

What is Oppositional defiant disorder?

Pattern of negative, hostile, and defiant behavior that is excessive compared with other children of the same age for at least 6 months

What is the ODD-DSM criteria?

A: pattern of negativistic, hostile, and defiant behavior x 6 months

• At least 4 sx: loses temper, argues w/ adults, actively defies requests/rules, deliberately annoys ppl, blames mistakes on others, easily annoyed, angry/resentful, spiteful/vindictive

B: disturbance causes impairment in social, academic, occupation

C: do NOT occur during the course of Psychotic/Mood disorder

D: criteria for Conduct disorder (18+ Antisocial personality disorder) are not met

What is Conduct disorder?

Repetitive/persistent pattern of behavior in which the basic rights of others or major age-appropriate societal norms and rules are violated

What is the DSM criteria for Conduct disorder?

A: 3 criteria w/in 12 months, at least 1 w/in the past 6 months

Categories:

• Aggression to people & animals

• Destruction of property

• Deceitfulness or theft

• Serious violations of rules

B: behavior impairs social, academic, occupational fxning

C: if 18+, criteria for Antisocial Personality Disorder are not met

ODD or CD:

Defiance of rules and argumentative verbal interactions

ODD

ODD or CD:

Symptoms emerge during pre-school years and persist for at least 6 months

ODD

ODD or CD:

Deliberate aggression, destruction, deceit, and serious rule violations, such as staying out all night or chronic school truancy

CD

ODD or CD:

Clear pattern of behavior established over a 6 month period

CD

What are the signs of Depression that you need to watch for?

SIGECAPS

Sleep changes, Interest loss, Guilt, Energy low, Concentration low, Appetite change (inc/dec), Psychomotor fxn (agitation/hopeless), Suicide

Which parasomnia:

Child awakens screaming from stage N3 sleep with dilated pupils, sweating, & tachycardia (usually 18 mo - 6 yo)

Night terrors

Does the child remember a night terror?

No

What conditions may precipitate night terrors?

Stress, Illness, Sleep deprivation

Night terrors or Nightmares:

Stage 4 sleep, No recall, First 1/3 of night, Not consolable, confused/agitated

Night terrors

Night terrors or Nightmares:

REM sleep, May recall, Consolable, Second 1/2 of night, upset but awake

Nightmares

What factors can exacerbate restless leg syndrome?

Caffeine, Alcohol, Nicotine, Iron deficiency, Pregnancy

Normal VS for a newborn

Temp: 97.7-99.5 F

RR: 35-60 bpm

HR: 120-160 bpm (lower in sleep)

Pulse Ox: 98%

BP: not obtained usually

How does weight change immediately following birth?

lose up to 10% of birthweight and gain it back by week 3 of life

What is Caput Succedaneum?

superficial edema/ecchymosis that crosses the suture line; reconfigure in ~1wk

What may cause a fontanelle to bulge?

inc ICP

What may cause a fontanelle to be sunken?

dehydration

What does an absent red light reflex indicate?

retinoblastoma, cataract, opacified cornea

What is CHARGE syndrome?

Coloboma, Heart anomalies, Atresia of choanae, Retardation of growth, GU abnormalities, Ear anomalies

What are key signs of Turner’s syndrome?

wideset nipples, excessive nuchal skin, lymphedema

What might weak LE pulses indicate?

coarctation of aorta/L ventricular tract obstruction

What does Scaphoid abdomen indicate?

diaphragmatic hernia

What are RF for developmental hip dysplasia?

breech position in utero, FMH, Females

What is the Barlow maneuver?

*used for hip dysplasia

infant lying supine w/ knees flexed → grab thigh along great trochanter w/ thumb on inner thigh → ADDUCT and apply downward pressure to promote dislocation