Pediatrics Flashcards
Nieman Pick enzyme deficiency?
sphingomyelinase
Nieman Pick H&P
cherry red macula
protruding abdomen
Lymphadenopathy
hepatosplenomegaly
Tay Sachs enzyme deficiency
hexoaminidase
Tay Sachs H&P
hyperacusis
mental retardation
seizures
cherry red macula
Gaucher’s disease enzyme deficiency
glucocerebrosidase
Gaucher’s H&P
hepatosplenomegaly
anemia
leukopenia
thrombocytopenia
Krabbes Disease enzyme deficiency
galactocebrosidase
Krabbes Disease H&P
hyperacusis, irritability, seizures
What is transient neonatal pustular melanosis and TX?
superficial pustules overlying hyperpigmented macules, spont resolves.
TX: tell parents to leave it alone
What is neonatal acne and what is the treatment
hormone stimulation of sebaceous glands occuring in 20% of infants 2-3wks of life
TX: resolves spontaneously, severe cases can be treated with benzoyl peroxide or topical retinoids
What is milia?
accumulation of sweat beneath sweat glands blocked by keratin, occurs in 1st week of life. Associated with increased temperature.
TX: possibly cool child
Mongolian Spot
bluish discoloration of butt and base of spine, benign
TX: document to prevent confusion with child abuse
Cutis marmorata
spider webbing/paleish marbling of neonates skin
tx: none, self resolving
Erythema toxicum
2-3mm yellow pustules with red base (look like white heads)
Arise first 24-72 hours, filled with eosinophils
tx: none, resolves by 3 weeks
Macular stains (stork bites)
permanent vascular malformations usually near nape of neck, upper eyelids, and middle of forehead
tx: none, but persist forever
Childhood immunization schedule
birth/1month = HEP B
2 months = Hep B, RV, DTAP, Hib, PCV, IPV
4 months = RV, DTAP, Hib, PCV, IPV
6 months = RV, DTAP, Hib, PCV, IPV, FLU
12 months = DTAP, HiB, PCV, IPV, FLU, MMR, Varicella
How many Hep B doses?
3 doses
How many MCV doses
2 doses
How many Hep A doses
2 doses
How many MMR doses?
2 doses
How many IPV doses
4 doses
How many PCV doses
4 doses
How many HiB doses
4 doses
How many DTAP doses
5 doses, and 4 years a TDap Booster
How many Rota Doses
3 doses
signs of diabetic ketoacidosis
dehydraton (thrist, polydipsia, dry skin, dry mouth) polyuria nausea/vomiting abdominal pain altered mental status
Labs in diabetic ketoacidosis
increased glucose
elevated anion gap metabolic acidosis
ketones in urine and serum
hyperkalemia, hyponatremia
management of acute coronary syndrome
1st) get a submaximal (or maximal of patient can tolerate it) stress test
2) if stress is negative; patient discharged on aspirin, beta blocker, statin, ACEi
3) if stress test is positive; get coronary angiogram
4) if angiogram shows 1 or 2 vessel disease –> percutaneous coronary intervention is done
5) if angiogram shows 3+vessel disease –> cardiac bypass surgery
diagnostic modality for AAA in a symptomatic obese patient
CT with contrast
labs on autoimmune hepatitis:
anti-nuclear antibodies (anti-ANA), anti-smooth muscle antibodies (anti-ASMA), hypergammaglobulinemia
associated with other immune disorders
treatment for autoimmune hepatitis
low dose corticosteroids (prednisone)
dacryostenosis H&P/treatment
blocked tear duct, usually unilateral and painless
excessive tearing and mucoid material secretion
treated with nasolacrimal massage until 1 year old
nasal duct probing done after age 1 year
criteria for metabolic syndrome
BMI >30 (OBESITY) + 2 of the following:
fasting triglyceride level >150
HDL 130/85
fasting glucose >100
Atherosclerosis H&P:
asymptomatic for most of disease progression
later sequelae include; Angina, claudication, progressive hypertension, retinal changes, extra heart sounds, MI, stroke, renal damage
For stable angina
refer patient for cardiac stress test; if equivocal, refer patient for nuclear stress testing or exercise stress test with echocardiography; if these are positive possible angiography or PET scan
athersclerosis treatment
primarily minimize risk factors (tobacco use, HTN, hyperglycemia, hypercholesterolemia)
Diet low in fats and cholesterol and high in antioxidants is helpful in preventing disease.
When do you screen for hyperlipidemia?
Men >35 yrs
women > 45 yrs
hyperlipidemia H&P:
usually asymptomatic severe disease presents with: 1) xanthomas (lipids on tendons) 2) xanthalesmas (lipid deposits on medial eye) 3) cholesterol emboli in retina
hyperlipidemia TX:
1st line: tobacco cessation, exercise, dietary restrictions,
2nd line: cholesterol lowering agents (statins, niacin, fibric acids and bile acid sequestrants)
Goal LDL in humans
<100 in pts with CAD/PVD/AAA/carotid disease
Angina Pectoris H&P:
temporary myocardial ischemia causes substernal chest pain that may radiate to left shoulder, arm, jaw, or back, will usually occur during exertion
Angina Pectoris treatment
Primary: sublingual nitroglycerin and cessation of intense activity
Secondary: stress testing; followed by nuclear studies (nuclear stress test), and angiography if stress test is negative/positive
1st degree heart block
asymptomatic
PR interval > 0.2sec
No treatment
2nd Degree heart block MOBITZ I
caused by intranodal or his bundle conduction defect or drug side effects
asymptomatic
progressive PR lengthening until a QRS is skipped
tx: adjust medication doses; if there is symptomatic brady cardia you put in a pacemaker
2rd degree heart block MOBITZ II
Infranodal conduction problem (His, Purkinje)
usually asymptomatic
randomly skipped QRS without changes in PR interval
TX: ventricular pacemaker; because it can progress to 3rd degree heart block
3rd degree heart block
absence of conduction between atria and ventricles
syncope, dizziness, hypotension
no relationship between P waves and QRS
TX: ventricular pacemaker, avoid meds that affect AV conduction
What is PVST
tachy cardia (HR>100bpm) arising in atria or AV junction; usually in young patients with healthy hearts
H&P for PVST
sudden tachycardia, possible chest pain, shortness of breath, palpitations, syncope
P waves hidden in T waves, 150-250 BPM, normal QRS
TX for PVST
carotid massage, valsalva maneuver, IV adenosine
If hemodynamically unstable cardioversion or calcium channel blocker
sometimes catheter ablation of accessory conduction pathways is used for long term control
what is MAT (MFAT?)
tachycardia due to several ectopic foci in the atria that discharge automatic impulses, usually asymptomatic
What do you see on ECG for MFAT?
variable morphology of P waves (at least 3), and HR >100
What is the treatment of MFAT?
calcium channel blockers or beta blockers acutely
catheter ablation or surgery to eliminate abnormal pacemakers
define bradycardia
HR < 60 BPM
frequently asymptomatic, possible weakness, syncope
TX: pacemaker if severe, stop precipitating medications
risk factors for Atrial fibrillation
pulmonary disease, CAD, HTN, anemia, valvular disease, pericarditis, hyperthyroidism, rheumatic heart disease, sepsis, alcohol use
Afib H&P:
SOB, chest pain, palpitations, irregularly irregular pulse
ECG findings in Afib
no discernible P waves, irregular QRS rate
TX for Afib
anticoagulaton and rate control!
rate control with CA-channel blockers, beta blockers, digoxin.
Cardioversion if caught within 48hrs; or if after 48 hours but echo shows no thrombus.
If more than 48 hours or thrombus present; anticoagulate for 3-4 weeks and then cardiovert afterwards.
AV nodal ablation can be considered for recurrent cases.
Aflutter risk factors
CAD, CHF, COPD, valvular disease, pericarditis
Aflutter H&P:
asymptomatic, or palpitations, syncope
Aflutter ECG changes:
regular tachycardia >150BPM with a sawtooth pattern of P waves
Aflutter TX:
rate control with Ca-channel blockers, beta blockers
electrical/chemical cardioversion
PVC causes
common, frequently benign; can also be caused by: hypoxia abnormal serum electrolyte levels hyperthyroidism caffeine use
PVC H&P:
usually asymptomatic; possible palpitations, syncope
ECG in PVC
early and wide QRS without preceding P wave, followed by brief pause in conduction
PVC treatment
none in healthy patients, beta blockers in patients with CAD to prevent sudden death
Vtach H&P
series of 3+ PVCs with heart rate of 160-240 BPM,
possibly asymptomatic if brief, but can cause palpitations, syncope, hypotension
Vtach ECG
series of regular, wide QRS complexes independent of P waves.
Vtach treatment
electrical cardioversion followed by antiarrhythmic medications, may need internal defibrillator
Vfib H&P
lack of ordered ventricular contraction leads to loss of cardiac output; presents with syncope, hypotension, pulselessness
Vfib ECG findings
totallly erratic tracing, no P waves or QRS
Ffib treatment
CPR, immediate electric or chemical cardoversion
what causes systolic heart dysfunction
decreased contractility, increased preload, increased afterload, HR abnormalities, high output conditions
what causes diastolic heart dysfunction
decreased ventricular compliance with decreased ventricular filling, increased diastolic pressure decreased CO
CHF risk factors
CAD, HTN, valvular disease, cardiomyopathy, COPD, drug toxicity, alcohol use
systolic heart failure treatment
loop diuretics, ACE-I or ARBs, beta blockers, spironolactone, add digoxin to increase contractility for symptomatic relief
diastolic heart failure treatment
calcium channel blocker, ARB or ACE-I, beta blockers, aldosterone antagonists
aortic stenosis symptoms
chest pain, DOE, SYNCOPE
aortic stenosis murmur
crescendo-decrescendo systolic murmur radiating from right upper sternal border to carotids
aortic stenosis treatment
valve replacement
mitral regurgitation symptoms
palpitations, DOE, orthopnea, paroxysmal nocturnal dyspnea
mitral regurg murmur
harsh blowing holosystolic murmur radiating fro apex to axilla, widely split S2, midsystolic click