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415 Cards in this Set

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What are the 5 portions of the APGAR score?
Appearance
Pulse (HR)
Grimace
Activity
Respirations
Dx:
A lump on the face after a foreceps delivery
Subcutaneous Fat Necrosis
Dx:
rash that starts after the first day of life as a flat reddish area with a central papule that becomes a pustule and persists for a couple of weeks

Test?
Erythema Toxicum

test:
Wright stain of vesicular fluid will show Eosinophils
Dx;

Strawberry rash of newborn with difficulty breathing
Epiglottic Hemangioma
Dx:
sharply demarcated bruise-like appearance on sacral area of newborn
Mongolian spots

(do not confuse w/ Abuse, which is not sharply demarcated)
Dx:
orange/yellow warty lesion on the scalp in newborn that displaces hair growth

Tx?

what can it lead to?
Nevus sebaceous

Tx:
leave alone until Adolescence
(should go away)

leads to:
15% chance to become malignant
Dx:
scalp swelling that crosses suture lines and goes away in a few days
Caput
Dx:
scalp swelling that does not cross suture lines and goes away in a couple of months

cause?
Cephalohematoma


cause: Subperiosteal bleed
When do anterior fontanels normally open and close?

Posterior?
Anterior:
opens: birth
closes: 9 - 18 months

Posterior:
opens: sometimes at birth
closes: 4 - 5 months
what is possible diagnosis if:

1. both fontanels are enlarged?

2. One fontanel only is enlarged?
1. Hydrocephalus


2. Hypothyroidism
Newborn eye:

White reflex
Retinoblastoma

(another source: MCC is Congenital Cataracts)
Newborn eye:

lens opacity
Cataracts

(possible Galactosemia)
Newborn eye:

Aniridia and hemihypertrophy (one side of body larger then other)
Wilms Tumor
Dx:
blue baby pinks up on crying

confirmatory test?
Choanal Atresia


test: Catheter doesn’t pass thru nose
MC abdominal mass in newborn
Polycyctic Kidney disease
when should an umbilical hernia close?
by 5 months
which has a sac--Omphalocele or Gastroschisis?
Omphalocele

(“O” is like a closed sac)
what should be avoided in a patient with Epispadias or Hypospadias?
do NOT circumsize
how long can undescended testis remain undescended before you need to bring them down?
one year
MC type of Ambiguous genitalia
Congenital Adrenal Hyperplasia

(21-hydroxylase deficiency is the MC of the CAH)
Dx:
newborn has a mass on the right anterior, superior chest and crepitus on exam

Tx?
Clavicle fracture

(MC newborn fracture)

Tx: None (will heal on its own)
MC birthing brachial plexus injury?

what nerve roots?

another name for this injury?
Erb-Duchenne

roots: C5-C6
(C4: if ipsilateral diaphragmatic paralysis also)

another name:
“waiter’s tip” – wrist flexed
birthing injury where baby has hand extended and fingers flexed

what nerve roots?

another name for this injury?
Klumpke

roots: C8-T1

another name:
“claw hand”
What are the (3) 'diseases' tested for during a newborn screening?
1. PKU

2. Galactosemia

3. Hypothyroidism
Dx:
baby born with Mental retardation, Eczema, musty odor, fair hair, fair skin and blue eyes
PKU
Dx:
baby born with Jaundice, Hypoglycemia, and Cataracts

genetic inheritance?

what is contraindicated in this baby?
Galactosemia

(Autosomal Recessive)

Contraindication: Breastfeeding
Dx:
Newborn with Jaundice, mottled, constipation, Large tongue, Umbilical hernia

what is seen on blood test?
Hypothyroidism

blood test:
high TSH, low T4
Dx:
pre-term baby with ground glass appearance on CXR

Tx? (2 in order)
Respiratory Distress Syndrome

Tx:
1. Surfactant
2. Ventilation
Dx:
baby delivered by C-section has a Rapid respiratory descent but after 72 hours of Tachypnea, then gets better; CXR shows possible fluid in the fissure
Transient Tachypnea of newborn

(when delivered by C-section that do not get a vaginal squeeze and then don’t expel amniotic fluid)

patient does not need respiratory support
(a main way to differentiate)
What are the 3 differences b/t physiologic and pathologic jaundice?
Physiologic:
1. Appears >24 hours of age
2. peaks at or below 12.9 by 3 days
3. resolves by 1 week

Pathologic:
1. Within first day of life
2. Level >13
3. Lasts > 1 week
MC type of pathologic Jaundice?

Tx?
Hemolysis

(Rh or ABO incompatibility)


Tx: Phototherapy
Dx:
newborn with Jaundice within 8 hours of birth, a Direct bilirubin increase; Acholic (or clay-colored) stools
Biliary Atresia
Aside from certain meds, what are the (6)* main contraindications of offering newborn breast milk?
“A Healthy Toddler Gladly Sucks Vitamins”:

AIDS;
Herpes if breast lesions;
TB (active, untreated);
Galactosemia;
Syphilis;
Varicella
“A Healthy Toddler Gladly Sucks Vitamins”
How many calories are in baby formula?

When can Whole milk be started?

When should Iron supplementation begin?
Formula: 20 cal/oz

whole milk: > 1 year-old

Iron: > 6 months old
What test can be done in newborn to test for Turner's syndrome?
Buccal smear

(to test for Barr Bodies)
definition:

child is short but expect a growth spurt at a later age
Constitutional Delay
MCC for childhood obesity?
take in too many calories and don't burn enough off

(don't be tricked and look for a pathologic cause)
when do most reflexes disappear?

which (2) don't at this time?
what time do they disappear?
reflexes disappear: 4 - 6 months

except:
Babinski: until 18 months;
Parachute: never
Rule for Cube stacking in child
(give 3 milestones)
“3 + 4 = 7” are the milestone cubes

…start with 3 and (and multiply by 5 to get first date), then from first date in months is +3 (for 4), then +3, +3 (for 7):

3 cubes at 15 months
4 cubes at 18 months
7 cubes at 24 months
MCC for Enuresis

First Tx?
Primary Nocturnal
(usually a male child that is just a deep sleeper)

Tx:
1. Give it Time
2. Alarms
3. Meds (Imipramine; DDAVP)
In the severely immunocompromised, what vaccination do you not give?
MMR
what 3 vaccinations are contraindicated with an allergy to neomycin?
1. IPV

2. MMR

3. Varicella
which vaccination do you give:

child > 2 yo who has not been vaccinated if they have immunodeficiency of any kind, are asplenic or lack splenic function (sickle cell)
Pneumococcus spp.
which vaccination is contraindicated in patient who has streptomycin allergy?
IPV

(also CI if they have neomycin allergy)
what 2 additional vaccinations are required specifically if patient is asplenic?
1. Meningococcal


2. Pneumococcus spp.
MCC of Meningitis in:

Neonate
Group B Strep


(from mother)
MCC of Meningitis in:

Patient > 2 months old
Pneumococcus
MCC of Meningitis in:

College or Military
(sexually active)
N. Meningococcus
What is the first step in a suspected meningitis of any age?
IV Ceftriaxone
MCC of Meningitis in:

Elderly, HIV+, steroid user
(immunocompromised)
Listeria
When should Ampicillin be added to the treatment of meningitis?
(2)

When should Vancomycin be added?
Ampicillin:
1. Listeria (as bug)
2. Neonates

Vancomycin:
Pneumococcus
Dx:
Lymphocytic meningitis, rash on wrists and ankles moving centrally

Tx?
Rocky Mountain Spotted Fever

Tx:
Doxycycline
(and Ceftriaxone for meningitis prophylaxis)
Differential for Lymphocytic meningitis and next test for each
(3)
Test: IgG and IgM Serology
1. Rocky Mt. Spotted Fever
2. Lyme disease

Test: India Ink
(or cryptococcal antigen)
3. Cryptococcus
Treatment for meningitis caused by Cryptococcus
Amphotericin

(then Fluconazole to take home)
What are the causes of CSF with very high protein and very low glucose?
(2)
1. TB


2. fungal
What should be given prophylactically for a sneaker puncture of a pin that enters the foot?
For what bug?
TMP-SMX

for: Pseudomonas
Bug causing septic arthritis from a dog or cat bite?
Pasturella
Pediatric patient without immunization history has coughing spasms, inspiratory whoop, facial petechiae and leucocytosis with lymphocytosis.

(2) possible confirmatory tests

Tx?
Pertussis

tests:
1. Positive cultured Nasopharyngeal washings
2. Rapid Fluorescent antibody stain

Tx:
Erythromycin
What can Fifth's disease lead to?

what can it cause in unborn child if mother has it?
leads to:
Aplastic Anemia

in unborn child:
Hydrops
Dx:
three-day rash and lymphadenopathy
Rubella
what 2 test should be run if suspecting acetaminophen toxicity?
1. Acetaminophen level in blood

2. LFTs
A child swallowed a bottle with no lable and has Sx of:
Hypernea/Tachypnea, Fever, N/V, Dehydration, Seizures, agitation, tinnitus

Fastest diagnostic test?
Aspirin toxicity

fastest test:
Urine Ferric Chloride Test
what acid/base problems does Aspirin toxicity cause in a child?
Respiratory Alkalosis w/ Metabolic Acidosis

(high pH, low pCO2 and bicarb)

Tx: Charcoal
A child swallowed a bottle with no lable and has Sx of:
Tachycardia, HTN to Hypotension, confusion, drowsiness, dilated pupils, Seizures, Widened QRS; Ventricular arrhythmia

Tx? (2)
Tricyclic Antidepressant toxicity

Antidote/Tx:

(1) Gastric Lavage/Charcoal

(2) Sodium Bicarbonate
(to alkalinize blood for arrhythmias)
What is the first test if you suspect a TCA toxicity?
ECG

(will show Widened QRS, Ventricular arrhythmia)
A child presents with swallowing kerosene.
What is the primary objective?

What are the first (2) tests?

What are (2) Tx?
objective:
Prevent Aspiration resulting in Chemical Pneumonitis

tests:
1. CXR
2. Blood Gas

Tx:
1. Prevent aspiration
2. avoid gastric lavage
a child swallows something from an unlabled bottle:

diarrhea, excessive urination, miosis, bradycardia

Test?
Tx?
Organophosphate (insecticide) toxicity

test: Plasma Cholinesterase activity

Tx:
Atropine w/ Pralidoxime chloride
a child swallows an unlabled bottle of pills:

drowsey, delirius, hallucinate, N/V, DRY state, dilated pupils

Test?
Tx?
Antihistamines

Test: Drug screen

Tx: Physostigmine (if severe)
a child swallows an unlabled bottle of pills:
signs of hemorrhagic gastroenteritis (bloody diarrhea), N/V, diarrhea, acute liver failure, shock, coma

Test?
Tx?
Iron pill overdose

test: Serum Iron level

Tx: Deferoxamine
MCC of conjunctivitis in newborn (less then 24 hours-old)
Chemical

(from Silver Nitrate drops)
MCC of infectious conjunctivitis in newborn
Chlamydia
Medical term:

Conjunctivitis within the first month of life
Ophthalmia Neonatorum
Difference in presentation b/t viral vs. bacterial conjunctivitis
Viral:
Crusting of eyelid;
Clear discharge

Bacterial:
Mucopurulent discharge
At what age is strabismus still normal?

What can it lead to if it is prolonged?
Normal: up to 4 months old

leads to: Amblydopia
Dx:
2 yo child has a "lazy eye"

Dx tests? (2)

Tx?
Amblydopia

tests:
1. Hirschberg test
(shine light directly into eye and look to see if it falls at an angle)
2. Cover test
(cover good eye and lazy eye will move straight ahead)

Tx: Patch good eye
(causes pt to use lazy eye; must be done early in life)
Child presents with a painful buldging eye; eyeball does not move when testing muscles

Dx test?

Tx? (2 possible)
Orbital Cellulitis

Dx test: CT Scan

Tx:
1. Cefuroxime with Clindamycin, or
2. Ampicillin/Sulbactam
Child presents with swelling of eyelids and surrounding tissue; eye moves when checking ROM
Preorbital cellulitis
Tx for a large Retinoblastic tumor of the eye
Enucleation
Definition:

Otitis Media w/ Osteomyelitis of the bony canal or mastoid

Bug?

what Dz is it associated with?
Malignant Otitis Media

bug: Pseudomonas

Assoc Dz: Diabetes
What is the specific bug that produces a positive on throat swab culture?

Tx? (3 possible)

(2) possible complications?
Group A Strep

(A not B)

Tx:
1. Penicillin
2. Erythromycin
3. Clindamycin

complications:
1. Rheumatic Fever
2. Glomerulnephritis
Dx:
High fever, tonsil bulges, uvula deviates to non-involved side, "hot potato voice"

Bug?

Tx? (2 together)
Peritonsillar Abscess

bug: Group A Strep

Tx:
1. Drain abscess
2. Penicillin
(Erythromycin if allergy)
Dx:
High fever, sore throat and ulcers scattered on the soft palate, tonsils and pharynx

cause?

Tx?
Herpangina

cause: Coxsackievirus

Tx: None...will go away
Dx:
High fever, sore throat and ulcers scattered on the soft palate, tonsils and pharynx, plus similar lesions on palms and soles
Hand-Foot-and-Mouth disease

(Coxsackievirus)
Dx:
child with sudden onset respiratory distress, wheezing; hyperinflated lung on one side and tracheal deviation to the opposite side
Foreign body aspiration
Dx:
4 year-old that presents with a Barking cough with Inspiratory stridor

Dx Test?

Tx? (2 together)

Tx if advanced or an emergency?
Croup (Parainfluenza virus)

Test:
P/A neck film: STEEPLE SIGN

Tx:
(1) Humidified air
(2) Steroids beneficial

Emergency: Racemic Epinepherine
Dx:
Dysphagia, Drooling, muffled voice, leaning forward to maximize air entry

Bug?

Dx test?

Tx? (2)
Epiglottitis
(EMERGENCY)

Cause: H. flu type b (HIB)

Dx Test:
Lateral Neck: THUMBPRINT SIGN

Tx:
(1) Secure Airway
CHERRY RED EPIGLOTTIS
(seen w/ intubation)
(2) antibiotics
Dx:
patient less then 2yo had a cold, then Wheezing, rales, tachypnea, Accessory muscles are used during respiration

MCC?

Dx test? (2)

Prevention (for those predisposed)?
Bronchiolitis

MCC: RSV

Dx test:
(1) Rapid assay from Nasal secretions
(2) CXR (Hyperinflation)

Prevention:
Palivizumab – Monoclonal antibodies
MCC of death in 1 - 12 month-old children
SIDS
What are the (3) main Right-to-Left shunt developmental Heart disorders?
1. Tetralogy of Fallot;

2. Transposition of the great vessels;

3. Tricuspid Atresia
What are the (3) main Left-to-Right shunt developmental Heart disorders?
1. VSD

2. ASD

3. PDA
What are the (3) main "Mixed" developmental Heart disorders?
1. Truncus Arteriosus

2. TAPVR

3. Hypoplastic LH
Dx:

Continuous murmur w/ wide pulse pressure heard in the upper left sternal border

Tx?
PDA

Tx: Indomethicin
Dx:

Apical Click followed by a late-systolic murmur
Mitral Prolapse
Dx:

Holosystolic murmur best heard at the Apex
Mitral Regurgitation
Dx:

Mid-diastolic murmur followed by opening Snap
Mitral Stenosis
Dx:

Systolic ejection murmur best heard at the left upper sternal border and a mid-diastolic murmur at the lower left sternal border; Fixed S2
ASD
Dx:

Loud harsh pansystolic murmur heard in the Lower left sternal border

What can it lead to?
VSD

(MC heart defect)

Leads to: Eisenmenger’s syndrome
(VSD shunt reverses right to left when the PVR exceeds the SVR)
Dx:

VSD shunt reverses right to left when the PVR exceeds the SVR
Eisenmenger’s syndrome
Dx:

CHF early in life including a feeding difficulty, sweat while feeding and tachypnea; loud harsh pansystolic murmur heard in the Lower left sternal boarder

What is it Associated with?
Endocardial cushion defect

(Subclass of VSD and ASD; also known as “Common AV canal”)

Assoc w: Down's Syndrome
Dx:

Weak/delayed/absent lower extremity pulses, HTN

What is seen on CXR?

What Dz is it assoc with?
Coarctation of the Aorta

CXR: Rib notching

Dz: Turner's Syndrome
(4) heart defects of the Tetralogy of Fallot

MC presenting Sx?
"PROVe"
1. Pulmonary stenosis
2. RV hypertrophy
3. Overriding Aorta
4. VSD

MC presenting Sx:
Cyanosis
Dx:

Cyanosis; Single second heart sound; Murmur Heard in Upper left sternal border

What is seen on CXR?
Tetralogy of Fallot

CXR: Boot-shaped heart
Dx:

MCC of cyanosis in the first 24 hours of life; loud single S2

What is seen on CXR?

Tx (aside from airway) until surgery?
Transposition of Great Vessels

CXR: "Egg on a string"

(ECG will show right axis deviation and RVH)

Tx:
Prostaglandin
(to keep PDA open until surgery)
Dx:

CHF w/i weeks of birth; Systolic Ejection murmur in the Left sternal border; Single S2; Wide Pulse Pressure; bounding arterial pulses


What Dz is it assoc with?
Truncus Arteriosus

(Single arterial vessel arising from the base of the heart that goes to the coronary, systemic AND pulmonary circulation)

Assoc with:
DiGeorge Syndrome
(22q11 microdeletion)
Dx:

single S2 pansystolic murmur with a CXR showing a Heart that looks like Snowman or Figure 8
Total Anomalous Pulmonary Venous Return
(TAPVR)
Dx:

Loud, palpable S2 without murmur; Common cause of heart failure in first week of life.

What can Patient develop?
Hypoplastic Left Heart

(Underdevelopment of left cardiac chambers, atresia or stenosis of aortic and/or mitral orifices, and hypoplasia of the aorta)

Patient develops Metabolic Acidosis
What are the (5) parts of the Major criteria for Rheumatic Fever?

What is Ailment is it assoc with?
Major (JONES) Criteria:
(2 of these = Rheumatic fever)

J = Joints
♥ = carditis (O looks like a heart)
N = Nodules (subcutaneous)
E = Erythema marginatum
S = Sydenham’s chorea

Assoc with: Strep throat (GAS)
MCC of HTN in infants or children?
Renal disease
MCC of Diarrhea in children
Rotavirus
Dx:

watery to bloody Diarrhea, N/V, fever, possible neurologic Sx, caused by ingesting poultry or raw eggs

Tx?
Salmonella

Tx:
Hydration only
(no Abx - they prolong Dz)
Tx for diarrhea from Shigella
TMP-SMX
Tx of diarrhea from Campylobacter
Erythromycin
Tx for diarrhea from C. Difficile
(2 together)
1. Metronidazole

2. Vancomycin
First test if suspecting Hirshprung's Dz

Diagnostic test?
First: Barium Enema

Dx test: Colon Biposy
Dx:

chronic problem in infant consisting of cough, vomiting and apnea

Dx test?
GERD

Dx test in infant:
pH probe
Dx:

Bilious vomiting and Double-bubble sign on abdominal X-ray

What is it assoc with?
Duodenal Atresia


assoc with: Down's Syndrome
Dx:
Nonbilious, Projectile vomiting; RUQ pain in infant

First Dx test?
Pyloric Stenosis


first test: Ultrasound
what acid/base problem is seen with pyloric stenosis?
Hypochloremic, Hypokalemic Metabolic Alkalosis
MCC of GI blood from neonate?

Dx Test?
Anal Fissure

Dx test: Apt test
(to confirm Dx: tells if fetal Hb versus mother Hb)
If a neonate has a positive Apt test, what is the cause of the GI blood?
Swallowing maternal blood
Dx:

Premature infant with Low Apgar and when fed the baby has bloody stools and abdominal distention

What is seen on Abdominal x-ray?
Necrotizing Enterocolitis


Abd x-ray: Pneumatosis intestinalis
(Air in bowel wall)
Dx:

a child between the ages of 6 – 18 months presents with crampy abdominal pain, Currant jelly-like stool, Sausage-shaped abdominal mass

Dx test?
Intussusception


Dx test: Barium Enema
(will see "Coil spring sign"; also treats problem)
Dx:

Painless rectal bleed in 2 yo that can cause intussusception

Dx test?
Meckel’s diverticulum

(disease of 2’s: 2% of population, 2:1 males, 2 years of age, 2 types of tissue, 2 feet from ileocecal valve, 2 cm in size)

Dx Test: Technetium scan
(Lights-up gastric tissue)
Dx:

Hematuria, Edema, Hypertension, Azotemia and Oligouria

What infection does it follow?

Dx Test? (2 plus results)
Acute Glomerulonephritis

follows: Group A Strep infection

Dx Test:
1. Urine: Red cell Casts
2. Blood: C3 decreased
Dx:

Renal failure in child with hearing loss and possible cataracts

Genetics?
Alport's Syndrome

(Mutation coding type IV collagen; Problem in basement membrane)

genetics:
X-linked Dominant
Dx:

Periorbital Edema / Ascites, High Proteinuria, Hypoalbuminemia, Hyperlipidemia
Nephrotic Syndrome

(MCC in children: Minimal Change Dz)
What is the MCC of Nephrotic syndrome in Pediatrics?

Tx?

Main possible Complication?
Minimal Change Disease (MCD)

Tx: Steroids

Complication:
Spontaneous Peritonitis
Dx:

infant with Toes AND heel deviated inward; Cannot place in neutral, dorsiflex or plantarflex
Tx?
Talipes equinovarus
("Club Foot")

Tx: Serial Casting
(only intoeing needing treatment)
MCC of Limping and hip problem in age 0 - 3 yo

Dx test? (2 together)

Tx?
Congenital Hip Dysplasia

Dx test:
1. Ordalani or Barlows procedure
(Hear a click on PE)
2. Ultrasound

Tx: Harness or Casting
MCC of limping in Age: 4 – 8 years (Begins as Painless limping leading to limping and pain)

Dx test?
Legg-Perthes
(Avascular necrosis of femoral head)

Dx test: Hip x-ray
MCC of hip problems in a > 11 year-old obese adolescent, that may complain of Knee pain

Dx Test? (2 together)
Slipped Capital Femoral Epiphysis

(Will externally rotate leg in an antalgic position)

Dx Test:
(1) Hip x-ray
(2) Thyroid, LH and FSH
(for obesity; think deficient gonads)
Dx:

Toddler with sudden traction to arm causing it to hang limp

Tx?
Radial head subluxation
("nursemaid injury")


Tx: gentle Supination pops it back into place
MC adolescent bone tumor. It occurs in a Metaphyseal area on the Distal Femur, Proximal Tibia or Proximal Humerus

what is seen on x-ray?

Who has an increased risk?
Osteosarcoma

x-ray: Codman triangle
(bony sclerosis)

Inc risk:
patients with Bilateral Retinoblastoma
Dx:
child who has painful bones that wake him from sleep but are relieved by aspirin
Osteoid Osteoma
Dx:

Bone tumor causing "onion-skinning" of mid-shaft of femur

what chromosomal problem?
Ewing's Sarcoma


chromosome: t(11;22)
Dx:
A child presents with recurrent episodes of non-pitting edema on the skin (including swelling of eyelids and lips), GI tract and respiratory system and a family history of the same problem; C4 complement is low

What is the deficiency?

Genetics?

Tx?
Hereditary Angioedema


Deficiency:
C1 Esterase Inhibitor

Genetics: Autosomal Dominant

Tx: Epinepherine and airway
(like for anaphylaxis)
Dx:

a male infant between the ages of 6 – 12 months presents with recurrent lung and sinus infections, esp. pneumococcus and H.influenzae; All Immunoglobulins are Low and B-cells are low or absent

Tx?
Bruton's Agammaglobinemia

(X-linked recessive)

Tx: replace IgG
Dx:
A newborn child presents with hypocalcemia, truncus arteriosus, Fishmouth, tetany and Micrognathia

What is deficient?
DiGeorge syndrome

Deficiency: T-cells
(from hypoplasia of 3rd and 4th pharyngeal pouches; thymus)
Dx:
Chorioretinitis, gingivitis, Granulomas in GI tract, Lupus syndromes; Recurrent Infections of Pneumonia, Abscesses, Lymphadenitis, Osteomyelitis, Bacterimia / Fungemia, and superficial skin infections (cellulitis and impetigo)

Dx Test?

Tx?
Cure?
Chronic Granulomatous Disease

(Deficiency in a step of NADPH oxidase w/i cells; X-linked recessive)

Dx Test:
Nitroblue Tetrazolium dye reduction (NBT)

Tx: Long-term TMP-SMX (or Dicloxacillin)

Cure: Bone Marrow Transplant
What are the symptoms of Wiskott-Aldrich syndrome?
(5)*
MR. TExT:

Ig(M) deficient;
Recurrent respiratory infections;
Thrombocytopenia;
Eczema;
(x-linked recessive);
T and B-cell deficiency
Patient develops anaphylaxis after IgG exposure. What is the first possibility?
IgA Deficiency

(MC primary immunodeficiency)
Dx:

recurrent respiratory, GU and GI infections; IgG subclass 2 and IgA are low; eveything else is normal
IgA Deficiency
What is the deficiency in most cases of Severe Combined Immunodeficiency (SCID)?

The incidence of what cancer is greater in this patient?
Adenosine Deaminase deficiency

cancer: Lymphoma
Dx:
a patient has a specific component of the complement system that is low and continues to get Neisseria infections.
Complement deficiency in any complement from C5 - C9
What type of juvenile RA occurs in a few large joints (like knee, hip shoulder)?
Pauciarticular JRA
What is positive and what sex is predominant in JRA pauciarticular arthritis type 1 versus type 2?
Type 1:
ANA+
Females

Type 2:
HLA B27+
Males
What does a baby have a risk of contracting when born to a mother with SLE?
Congenital Heart block
Immunizations:
1. less then 1 month
2. at 2 months
3. at 4 months
4. at 6 months
1. less then 1 month: (B)
Hep-B

2. at 2 months: (DHIP)
DTP, HiB, IPV, Pneumo

3. at 4 months: (BDHIP)
Hep-B, DTP, HiB, IPV, Pneumo

4. at 6 months: (DHP)
DTP, HiB, Pneumo
Immunizations:
1. at 15 months
2. at 18 months
3. at 4 - 6 years
1. at 15 months: (HeMP)
HiB, MMR, Pneumo

2. at 18 months: (BDIAV)
Hep-B, DTP, IPV, Hep-A, Varicella

3. at 4 - 6 years: (DIM)
DTP, IPV, MMR
Another name for Kawasaki disease?
Mucocutaneous LN syndrome
Dx:
Child presents with Fever (for at least 5 days) + 4 of 5 of following: Bilateral, non-purulent Conjunctivitis, Mucous membrane changes, hot red Tongue or red, cracked lips, Peripheral extremity changes, Edema or peeling of the skin on fingers, Rash (leather-like) and cervical lymph nodes

Dx test?

Tx? (2 together)
Kawasaki Disease

(Mucocutaneous LN syndrome)

Dx test: Echocardiogram
(rule-out possible Coronary Vasculitis or Anneurysm)

Tx:
1. Aspirin (not high dose)
2. IV-IgG therapy
Dx:
Child had a viral or Group A Strep infection, then later gets a rash from the Waist downward, Elbows downward and Face, in addition to Sx of Vomiting, Abdominal pain, Ileus, and possible Upper and Lower GI bleeding

Immuno problem?

Tx? (2 together)
Henoch-Schoenlein purpura

Immuno problem:
IgA-mediated Vasculitis

Tx:
(1) Supportive (recovery in 4 – 6 weeks)
(2) Corticosteroids for GI Sx
MCC of iron deficiency in newborn?

in child 9 - 24 months?
newborn: Low Birth Weight

9 - 24 months: Diet
What can drinking cows milk before 1 year-old cause in an baby?
Iron Deficiency Anemia from gastric bleeding
Dx;
Young child with pallor, scleral icterus and mild splenomegaly. CBC shows reticulocytosis, anemia with an Increased Mean Corpuscular Hemoglobin (MCH) concentration and indirect hyperbilirubinemia

Genetics?

What can Dx lead to?

Next Dx Test?

Tx? (2)
Hereditary Spherocytosis

genetics: Autosomal Dominant

leads to: Aplastic Anemia
(especially after parvovirus B-19)

next Dx Test: OSMOTIC FRAGILITY TEST

Tx:
1. Folic acid supplementation
2. Splenectomy (in patient > 6yo)
What is the first test if you suspect you have a Sickle cell patient?

How is Sickle cell Diagnosis confirmed?

Ongoing Tx? (3)
First test: CBC w/ peripheral smear

Dx test: Hemoglobin Electrophoresis

Tx:
1. Prophylactic Penicillin
2. Folate supplementation
3. Hydration
Dx:
a patient w/ sickle cell begins to have pain in various areas of the body.

Tx? (3 together)
Sickle cell crisis

Tx:
1. Oxygen
2. IV fluids
3. Analgesics (Narcotics)
Dx:
A 2 month-old develops Dactylitis (hand and foot swelling), vaso-occlusion causing Splenic and bone infarcts, multiple Infections
Sickle cell anemia
Dx:
A child has Sx of Petechiae (that do not blanch), but patient appears well and possible bleeding of mucous membranes; very low platelet count; Bone marrow is normal (or an increase in Megakaryocytes)

Tx? (3 together)
Idiopathic Thrombocytopenia
(ITP)

Tx:
(1) IV-IG
(2) Steroids (must do bone marrow first)
(3) Anti-D globulin
What (2) coagulopathies present with:

Normal PT,
Normal PTT,
High Bleeding time

What else in blood work differentiates the two?
ITP and TTP

Both have low platelets, but TTP also has LOW RBC
What (2) coagulopathies present with:

Normal PT,
High PTT,
Normal Bleeding time

What medication also presents this way?
What else in blood work differentiates it?
Coags:
Hemophilia A and Hemophilia B
(X-linked recessive)

Med: Heparin
Difference: Thrombocytopenia
What coagulopathy presents with:

High PT,
High PTT,
High Bleeding Time
DIC

(will also see low platelet count)
What coagulopathy presents with:

Normal PT,
High PTT,
High Bleeding time
vonWillebrand's Disease

(Autosomal dominant)
What coagulopathy presents with:

High PT,
Normal PTT,
Normal Bleeding Time
Warfarin toxicity
Dx:
a boy presents with a high PTT and Hemarthrosis

Tx?
Hemophilia A

Tx: replace Factor VIII
Dx:
A 3 – 5 yo child w/ an acute onset of Anorexia, pallor, fever, bone pain in 1/4 of patients, Pancytopenia.

Dx Test?

Tx? (3)

MC places for Relapse? (2)
Acute Lymphocytic Leukemia
(MC childhood cancer)

Dx test: Bone Marrow bx

Tx:
1. Chemotherapy,
2. Radiation,
3. Transplant

Relapse:
1. TESTES
2. CNS
What Non-Hodgekin's Lymphoma presents as an abdominal mass and is associated with EBV?

what is seen on LN bx?

Chromosomal anomaly and gene?
Burkitt Lymphoma

LN Bx: "Starry sky" pattern

Chromosomes: t(8;14)
Gene: c-myc
Dx:
Persistant cervical lymphadenopathy, night sweats, otherwise normal adolescent

Dx test?
Hodgkin Lymphoma

Dx test: LN biopsy
(Reed-Sternberg cells)
How is a Neuroblastoma and Wilms Tumor distinguished by CT scan?
Neuroblastoma:
1. on Adrenal gland
2. Calcifications seen

Wilm's Tumor:
1. in Kidney
2. no calcifications
Dx:
child with morning Headache, vomiting (causing HA to go away); Ataxia, nystagmus, head-tilt, intention tremor

Specific site?
Cerebellar Astrocytoma
(MC childhood brain tumor)

site: Infratentorial (posterior fossa)
Dx:
Child with short stature, Bitemporal hemianopsia and endocrine abnormalities

Dx test?

What can occur post-op?
Craniopharyngioma(Supratentorial)

Dx test:
X-ray of skull: Large Cella Tursica

Post-op:
Diabetes Insipidus
Dx:
A 3 - 5yo child presents with obstructive hydrocephalus, ataxia and CSF metastasis

Specific site?
Medulloblastoma

site: Infratentorial
Dx:
Child stares into space in middle of sentence, then 20 seconds later he resumes his sentence

Dx test results?

Tx?
Absence seizure

Dx test: EEG showing 3-Hz Spike and Wave pattern

Tx: Ethosuximide
MC type of seizure in pediatrics

Tx?
Febrile Seizure

Tx:
Acetaminophen (for fever)
(check for underlying cause of seizure)
Dx test for Neonatal seizures

Tx?
test: Continuous Bedside EEG

Tx: Phenobarbital
Dx:
A child of 2 – 7 months
presents with Recurrent mixed Flexor-Extensor spasms lasting only a few seconds, but repeat more then 100 times in a row; Looks like multiple Startle or Moro response

Dx Test & Result?

Tx? (2 together)
Infantile spasms
("West syndrome")

Dx Test:
EEG: HYPSARRTHYMIA

Tx:
1. ACTH
2. prednisone
Difference in presentation with Niemann-Pick Dz and Tay-Sachs Dz?
Both have Cherry-red spot on Macula

NP: Hepatosplenomegaly

TS: No hepatosplenomegaly
Dx:

child who constantly clears his throat
Tourette's syndrome
Dx:
MR, spasticity, aggressive behavior, self-mutilation, Gout arthritis
Lesch-Nyhan
What are the (2) disorders where infants have Hypotonia or Flaccidity?
1. Werding-Hoffmann Dz


2. Infant Botulism
Dx:
Infant with spinal muscular atrophy, Fasciculations, frog-leg posture

Dx Test/results? (3)

Tx?
Werdnig-Hoffman Dz
("Floppy-Baby" syndrome)

Dx Test:
1. EMG: Fibrillations
2. Muscle bx: denervation
3. Nerve bx: slowed conduction

Tx: Supportive
Dx:
A child presents following a viral illness with Ascending weakness, Paralysis, Loss DTR, may progress to affect Diaphragm (breathing)

Tx? (3 possible)
Guillain-Barré

Tx:
(1) Supportive
(intubation may be needed)

(2) Plasmapheresis
(3) IVIG
Dx:
A boy 3 - 7 yo with Muscle and Hip girdle weakness and pseudohypertrophy of the calves

Genetics?

Dx Test?
Confirmatory test?
Duchenne muscular dystrophy
(MC inherited neuromuscular disease)

genetics: X-linked recessive
(only boys get it)

Dx test:
Blood shows greatly elevated Creatine Kinase
Confirmatory test:
Muscle Bx
Name the sign:

a patient w/ DMD is walking their hands up their legs to sit/stand properly
Gower’s sign
Dx:
Café-au-lait spots, axillary/inguinal freckling, Learning disorders, Renovascular HTN, Scoliosis, Lisch nodules (seen on slit lamp)

Genetics?

what chromosome has the problem?
Neurofibromatosis
(von Recklinghausen)

genetics:
Autosomal Dominant

chromosome: 17

(Type 2 - Bilateral Acoustic neuromas is chrom: 22)
Dx:
A child presents with pale hypopigmented areas, facial sebaceous adenomas, areas of abnormal skin thickening, MR and Seizures are common

Genetics?

Dx Test/results?
Tuberous Sclerosis

genetics: Autosomal Dominant

(Ash leaf spot – pale hypopigmented area)
(Shagreen patch – areas of abnormal skin thickening)

Dx Test:
CT scan of Head:
periventricular calcified tubers
Dx:
Facial nevus (port wine stain) with trigeminal distribution, MR, seizures difficult to control, visual impairment

Dx Test/results?
Sturge-Weber Dz

Dx Test:
CT Scan of Head:
intracranial calcifications
First sign of Puberty in:

1. Females

2. Males
Females: Breast buds


Males: Testicular enlargement
What is the cause of Hypoglycemia in a newborn from a mother with DM?
Fetal Hyperinsulinemia
What is the highest risk for a child to be born with developmental displasia of the hip?

What is the confirmatory test for DDH?
Female Breech birth


test: Ultrasound of hip
Why does a mother with type O blood cause hemolysis in newborn if the child has type A or B?
Type O people have Antibodies to blood types A and B
When should a child have their electrolytes checked if you suspect dehydration?
Moderate or Severe dehydration

(not Mild dehydration)
Tx for Mild, Moderate and Severe Dehydration in children?
Mild: Oral rehydration

Moderate/Severe: IV rehydration
What is considered Moderate dehydration percentage-wise?

Severe?
moderate: 5-10% dehydrated

severe: > 10% dehydrated
Dx:
Baby is tired when feeding, diaphoretic and breathing strangely
CHF in newborn

(tired when feeding is #1 sign)
what are the (2) criteria for a Dx of failure to Thrive in a newborn?
1. failure to regain birth weight by 3 weeks of age

2. continuous weight loss after 10 days of life
when a child has a decrease in respirations, what does it suggest?

increase in respirations?
Dec: CNS depression


Inc: Infection
What are the (5) Tanner stages for Pubic hair?
1. None
2. Scant, Fine hair
3. Curly, extending laterally
4. Adult-like hair, NOT on medial thigh
5. Adult hair on medial thigh
At what age are solids introduced in an infant?

what is the average weight gain for a term infant per day?

when should they be 2x the original weight?
3x?
Solid foods: 4 - 6 months

Ave weight gain: 20-30g/day

2x: 4 - 5 months
3x: 12 months
What is the only vaccination that can cause a fever 7 days after it is given?
MMR
when should a child's car seat start facing front?
> 1 yo + > 10 kg
What are (4) developmental milestones at 6 months old?
"Six Babbling Strangers Switch Sitting at 6 months":

1. Babbles
2. Stranger recognition
3. Switches hands (transfers objects)
4. Sitting unsupported
What are (4) developmental milestones at 9 months old?
"Pinches Ma-ma's furniture Bye-Bye to cruise"

1. Pincher grasp
2. says: Mama or Dada
3. waves Bye-Bye
4. Cruises around furniture
What is the MC malignant tumor in infancy?
Neuroblastoma
A child with sickle cell presents to your office with a fever.

Next step?
Immediately admit to hospital
A child with sickle cell presents with a stroke.

Tx?
Exchange Transfusion
What is the first sign of Nephrotic syndrome in a child?
Pitting Edema of the extremities
Dx:
A child is involved in a MVA and presents with a HR that goes from 110 to 56 and BP that is 156/96, with irregular respirations.
Increased ICP
Dx:
A child presents with a rash that began on his neck and then spread to his trunk. On exam, you notice both new and old vesicles in the same area
Varicella (Chicken pox)
What is the difference b/t HUS and TTP?
(2)
No Fever and Neurological signs in HUS

HUS = "RAT"
(Renal failure, Anemia, Thrombocytopenia)

TTP = "FAT RN"
(Fever, Anemia, Thrombocytopenia, Renal problems, Neuro problems)
What GI complaint can HSP lead to causing sevre morbidity?
Intussusception
What is the Tx for a sickle cell patient who presents with anemia and an enlarged spleen?
Blood Transfusion
What (5)* medications can cause Thrombocytopenia?
PT CCS:

1. Phenytoin
2. TMP-SMX
3. Chloramphenicol
4. Carbamazepine
5. Sulfonamides
PT CCS
In assessing dehydration, what are the (7)* main findings to evaluate?

How many findings equal <5% dehydration?
More then 10% dehydration?
having Dehydration is "BUM NEWS":

1. BP and HR
2. Urine output
3. Mucous membranes and Tears
4. Neurological status
5. Eye and Fontanels
6. Weight
7. Skin turgor/color and Capillary refill

<5% = <3 findings
>10% = >6 findings
How many mL/kg water loss is considered Mild, Moderate and Severe?
Mild = < 50mL/kg

Moderate = 50 - 99mL/kg

Severe = > 100mL/kg
in the steps of fluid management in children, when is the only time normal (0.9%) saline is given?

why is it given?

what is given the other times?
0.9% = Initial 20cc/kg Bolus

(continue boluses until patient urinates, except for DKA...for them monitor vitals and neuro status)

Given to Restore Intravascular Volume

0.45% (1/2 normal) saline is used to correct dehydration and for maintenance fluids in a child
When is the earliest time to initiate testing for Type 2 DM in children?
How frequent do you test?
What is the best test?
Age of Initiation: 10-yo or at onset of Puberty
(whichever is first)

Frequency: every 2 years

Test: Fasting Plasma Glucose
Pediatrics are usually started on 3 insulin injections per day. What is the dosing pattern?
1. 2/3 of total dose in morning
(1/3 rapid or short-acting, 2/3 intermediate-acting)

2. 1/6 of total at dinner
(as rapid or short-acting)

3. 1/6 of total before bed
(as intermediate-acting)
A child has a seizure following the immunization.
What is the cause?
What is done on the next immunization?
Pertussis

-Do NOT ever give that immunization again!
When a child is bit by a snake, how do you determine the venom dose?
Based on the childs symptoms
(amount of envenomization)
Dx:
A 6-month-old infant presents to the emergency department with the new onset of weak cry, decreased activity, and poor feeding. The mother also states that the infant has been constipated for the past 2 days. On physical examination, the infant has a very weak cry, poor muscle tone, and absent deep tendon reflexes.

Tx?
Botulism

Tx:
Supportive care (Airway PRN)
Dx:
A 16-year-old boy presents with a temperature of 38.4 C (101 F) and low back, wrist, and knee pain. He had a sore throat 1 month earlier. His arthritis is diffuse. Pea-sized swellings are noted over the skin on his knees. He has a serpiginous erythematous area on his anterior trunk. His blood and throat cultures are negative, and his CBC is unremarkable. His antistreptolysin-O (ASO) titer is high.

Tx? (2 together)
Rheumatic Fever

Tx:
1. Penicillin
2. Aspirin
On physical examination, the infant is afebrile with stable vital signs. She can lift her head to 90 degrees, her eyes follow past the midline, she laughs, regards her own hand and has slight awareness of her mother.

How old is she?
4 months
Dx:
A 5-year-old girl presents with a 3-day history of fever, dyspnea, and intermittent joint pain. She has a history of sore throat about 1 month ago. On physical examination, her temperature is 39.6 C (103.2 F), blood pressure is 94/60 mm Hg, pulse is 114/min, and respirations are 22/min. Her knees and elbow joints are swollen and tender to palpation. There is a grade III/VI diastolic murmur best heard at the apex. Multiple fine, pink macules are noted on her trunk. These macules are blanching in the middle.

Tx?
Rheumatic Fever


Tx:
Penicillin
A 4-week-old infant presents with tachycardia, tachypnea, and poor weight gain. His arterial blood gas shows a pH of 7.34, a PaCO2 of 41 mm Hg, and a PaO2 of 74 mm Hg. A chest radiograph shows cardiomegaly. Echocardiography reveals a structurally normal heart, left ventricular dilatation, a left ventricular ejection fraction of 20%, and mild mitral and tricuspid regurgitation.

What IV med is the best first step in management?
Furosemide

(patient has CHF)
Dx:
A term male infant is found to be cyanotic shortly after birth and requires endotracheal intubation. On physical examination, his blood pressure is 68/34 mm Hg (equal in all four extremities), pulse is 180/min, and respirations are 32/min. His precordium is dynamic, has a grade III systolic murmur, and a single S2. Chest radiography shows a normal heart size and increased pulmonary vascular markings. An arterial blood gas on an FiO2 of 100% shows pH 7.34; PaCO2, 47 mm Hg; PaO2, 46 mm Hg
Total Anomalous Pulmonary Venous Return

(characterized by the pulmonary veins forming a confluence behind the left atrium, and draining into the right atrium. Complete mixing takes place in the right atrium, with a right-to-left shunt through the foramen ovale to the left side of the heart)
Dx:
A 1-month-old baby boy has bloody diarrhea. No infectious agent is identified, but the baby is found to be profoundly thrombocytopenic. The baby is also noted to have a skin rash, and a dermatologist diagnoses eczema. By three months of age, the baby begins to develop recurrent respiratory infections.

If this child survives until adolescence, he is at particularly high risk of developing what?
Wiskott-Aldrich syndrome


can lead to: Lymphoma
Dx:
A 9-month-old girl has had one serious infection after another since about 3 months of age, including thrush, pneumonias, and diarrhea. The baby is small for age. An older brother died at age 2 of pneumonia. Immunologic evaluation demonstrates lymphopenia and very low gamma globulin levels. Both T and B cell numbers are very low. Radiologic studies demonstrate "frayed" long bones, abnormally thick growth arrest lines, and dysplasia of the costochondral junctions
Severe Combined Immunodeficiency (SCID)

(an Adenosine deaminase deficiency)
Dx:
A 17-year-old girl presents to the office with a 5-day history of a malodorous vaginal discharge. She is sexually active and uses condoms for sexual intercourse. On examination, a thin, white discharge is seen. A "fishy" odor is produced when KOH is added to the discharge. The vaginal fluid has a pH of 5.
What is the most likely finding on a microscopic examination of the vaginal fluid?
Bacterial Vaginosis

micro: Clue Cells
A 7-yo patient presents with Lyme disease.

Tx?
oral Amoxicillin

(doxycycline is not given to kids under age 8)
In a patient diagnosed with vesicoureteral reflux, what is the first Dx step?

What is the Dx/confirmatory Test (2nd step)?
first: Renal Ultrasound


Dx/confirm Test:
Voiding Cystourethrogram
Dx:
a 12-yo girl presents with a fever and skin rash of 2 days duration. She complained of a sore throat and mild neck pain several days ago. The PE shows lymphadenopathy of the posterior occipital, retroauricular and cervical LN, plus an erythematous macules on the soft palate and a rash on the face, chest and proximal extremities.
Rubella
Tx for Candida-induced oral thrush?
Nystatin therapy
A child presents with "cat-scratch Dz."
what is the bug?
what is the Dx/confirmatory test?
Bartonella henselae

test: Serum antibody titers
what is the bone complication of NF-1?
Thinning of the Long Bone Cortex
what is the difference in RDW levels b/t thalassemia and Iron-deficiency anemia?
RDW:

normal in Thalassemia

High in Iron-deficiency anemia
A patient presents with painful ulcers on his penis. You think it is HSV. What is the Dx test?
Tzanck preparation
what is the Dx test for RSV?
Nasopharyngeal aspirate
Dx:
a 9-yo boy presents with increasing clumsiness, change in speech, wide-based unsteady gait, nystagmus and no DTRs.

what heart problem can it lead to in the future?
Friedrich Ataxia


leads to:
Hypertrophic Cardiomyopathy
Dx:
a 20-mo girl presents with loss of developmental milestones, repetative movements and acquired microcephalopathy
Rett syndrome
What is the confirmatory test for DMD?
Muscle biopsy
What type of exercise induces "exercise-induced" asthma?
High intensity, continuous, prolonged exercise

(ex: a 5-K run)
what deformities of the head are associated with congenital toxoplasmosis?
(2)
1. Microcephaly

2. Hydrocephalus
What vitamin is most likely to cause a pseudotumor cerebri?
Vitamin A
Dx:
a child with strep pharyngitis is given penicillin. The next day he develops a fine, papular rash over his body, which is accentuated in his axilla and groin.
Scarlet Fever

(a rash from the penicillin would be urticarial in nature)
What are the (11)* criteria (of which 4 or more need to be present) to diagnose SLE?
SOAP BRAIN MD:
1. Serositis (pleuritis/pericarditis)
2. Oral ulcers
3. Arthritis
4. Photosensitivity
5. Blood (any can be low)
6. Renal problems
7. ANA+
8. Immunological (ds-DNA+)
9. Nero Sx (psych, seizures)
10. Malar Rash
11. Discoid Rash
SOAP BRAIN MD
what is seen in the serum of someone with a pneumonia caused by Mycoplasma?
Cold Agglutinins
A 13-yo girl presents with lethargy, fever, severe HA and a stiff neck. On exam she has a unilateral fixed, dilated pupil and papilledema.

What is the initial step in management?
Intubation and Hyperventilation
what is antibiotic used for the initial therapy of acute sinusitis?
Amoxicillin-clavulanic acid
In a child, if the monospot test comes back negative and you think the Dx is Mono, what is the next step?
EBV titer

(heterophil test has a poor response in children)
How does a child with Breast milk Jaundice present?
Prolonged unconjugated hyperbilirubinemia in first weeks/months of life
A 4-yo child presents with a platelet count of 30K and you diagnose her with ITP.

what is the most appropriate Tx at this time?
No specific therapy

(when platelets go below 20K start on oral prednisone; a splenectomy is done for those with no response to prenisone or those with recurrent ITP)
Dx:
A 12-yo athelete presents with chest pain and dyspnea on exertion that resolves with rest.

First step in Tx?
Asthma (exercise-induced)

(<5% of kids have heart problems, aside from congenital anomalies)

Tx:
Trial of Albuterol inhaler
A 2-mo is admitted with constipation and an abdominal mass, fever, feeding poorly and vomiting for the past 2 days. A barium enema is ordered and shows Hirschprung's Dz.

what is the most important next step?
IV fluid hydration and IV antibiotics

(reestablish IV volume and prevent sepsis)
What (2) electrolyte disorders accompany Rhabdomyolysis?
1. Hyperphosphatemia

(leading to...)

2. Hypocalcemia
What are the minor criteria of Rheumatic fever?
(5)
FAILS:

1. Fever
2. Arthralgia
3. Increased CRP or ESR
4. Long PR interval
5. Strep culture + or ASO+
what type of acid/base disorder do you see with a 2 day history of diarrhea?
Hyperchloremic Normal Anion gap Acidosis

(loss of bicarb in the stool stimulates renal tubular reabsorption of chloride ions)
What (3) malignancies are associated with EBV?
1. Burkitt Lymphoma

2. Hodgkin Dz

3. Nasopharyngeal CA
What is the difference in AST/ALT initially between Hepatitis B and Hepatitis C?
Hep B: AST/ALT in Thousands


Hep C: minimal or no elevation of AST/ALT
What electrolyte abnormality is commonly seen in a newborn macrosomic infant from a mother with diabetes?
Hypocalcemia

(and hypomagnesemia)
A 3000g female is born at 38 weeks to a mother that is positive for GBS.

What is the next step in managing the baby?
CBC and blood culture

(in addition to 48 hours of observation in the hospital)
An infant is crawling around on the floor at home and suddenly become cyanotic.

what is the first step?
Back blows followed by chest thrusts

(never perform a blind finger sweep in a child, b/c it can push the aspirated object further)
why does a CF patient present with Metabolic Alkalosis?
Potassium is lost in urine
MC presentation of Strep throat?
(4)*
FEAT:

1. Fever
2. Exudate on Tonsils
3. Anterior Cervical lymphadenopathy
4. Throat pain
A full-term newborn is born vaginally after a pregnancy complicated by gestational diabetes that was poorly controlled. He is born with scleral icterus. Three hours after birth the baby is plethoric, irritable and cyanotic. Six hours after birth he has a seizure.

What hematological abnormality is most likely seen on his CBC?
Polycythemia

(a known complication in the infant of a diabetic mother)
Dx:
A previously healthy 3-mo term boy presents to ED with tachypnea, poor feeding and pallor for 24 hours. He is tachycardic with a HR of 240 with narrow QRS complexes. Vagal maneuvers are attempted and unsuccessful.

What drug should be given?
Supraventricular Tachycardia (SVT)

drug: Adenosine

(blocks AV conduction and SA-node pacemaker activity; DOC for SVT; if unavailable, can Cardiovert)
What is the most important risk factor for a PE in children?
Central Venous catheter

(followed by immobility)
What hormone is responsible for growth plate fusion?
Estradiol
Dx:
A patient presents with mild MR, prominent lips and a long philtrum, a systolic ejection murmur and hypercalcemia.
Williams syndrome
What are the erruption times of permanent teeth?
(8)*
"Mama Is In Pain, Papa Can Make Medicine":
1st Molar: 6 years
1st Incisor: 7 years
2nd Incisor: 8 years
1st Premolar: 9 years
2nd Premolar: 10 years
Canine: 11 years
2nd Molar: 12 years
3rd Molar: 18-25 years
"Mama Is In Pain, Papa Can Make Medicine"
What is the MCC of retinopathy in a premie?
Too much Oxygen saturation

(should be <95%)
What occurs if you correct the following problems too fast?

1. DI
2. DKA
3. SIADH
1. DI -> Seizures

2. DKA -> Cerebral edema -> Herniation

3. SIADH -> CPM
What additional (3) things are needed in formula for premature infants?
1. Extra Calories (22kcal/oz)

2. Extra Calcium

3. Extra Phosphorus
What is a normal systolic BP in the following ages of children?

1. Premie
2. up to 1mo
3. 1mo - 1yo
4. > 1yo
1. Premie = meanBP > gestational age at birth

2. up to 1mo = >60

3. 1mo - 1yo = >70

4. > 1yo = 70 + (2 x age)
Diarrhea that is caused by "chitlins" at a picnic
Vibrio
Dx:
Patient presents with absent LN, tonsils, lymphopenia, no thymus, recurrent infections of oral candida, diarrhea, pulmonary infections and viral infections
SCID
(Severe Combined Immunodeficiency Dz)
MC heart defect in congenital Rubella
PDA
Dx:
An infant presents with noisy breathing and on laryngoscopy the epiglottis is seen "rolling from side-to-side"

Tx?
Laryngomalacia


Tx: hold infant upright for 30 minutes after feeding

(most disappear by 2 yo)
What type of Pneumonia is seen in Hyper-IgM syndrome?
PCP
what are the (4)* MC congenital problems in infant of a diabetic mother?
"A Diabetic Causes Trouble":

1. Ancephaly / NT defects
2. Duodenal Atresia / small left colon
3. Caudal Regression syndrome
4. Transposition of the Great Vessels
"A Diabetic Causes Trouble"
Dx:
a child presents with a slowly developing back pain and neuro degeneration with a "step-off" palpated at lumbosacral area
Spondylolisthesis
Dx:
an infant presents with Cyanosis, Left Axis Deviation and VSD

what does EKG show?
Tricuspid Atresia


EKG: LVH (and hypoplastic RV)
Dx:
a cyanotic newborn with a CXR showing cardiomegaly, increased vascular markings and right aortic arch.

what does the EKG show?
Truncus Arteriosus


EKG: Biventricular Hypertrophy
what are the (4)* main features of Beckwith-Wiedmann syndrome?
HOMO:

1. Hypoglycemia & Hyperinsulinemia

2. Omphalocele

3. Macroglossia & Macrosomia

4. Organomegaly
HOMO
If a child presents with asthma and other allergic disorders (allergic rhinitis or eczema), what is the best long term medication?
Mast Cell Stabilizers

(inhaled Cromolyn)
Dx;
A 12-mo with foul-smelling, non-bloody stool 7-8 times/day, irritable, pre-tibial edema, erythematous vesicles on extensor surfaces of elbows and knees and microcytic anemia
Celiac Disease

(begins at age 12 - 15 mo)
MCC of Meningitis in following ages:

1. Newborn
2. 1-mo to 2-yo
3. 2-yo to 18-yo
4. > 18-yo
1. Newborn = GBS

2. 1-mo to 2-yo = S. Pneumonia

3. 2-yo to 18-yo = N. Meningitis

4. > 18-yo = S. Pneumonia
Dx:
a few days after birth a mother realizes that her child cannot move his hand on the right side and also has right lid ptosis
Klumpke Paralysis

(birth injury to CN-7,8 and T1)
What reflex is affected by a child born with E-D palsy?
Moro relex is absent
What is the test of choice in evaluating a child for Hyperlipidemia?
Screening Total Cholesterol level

(not Fasting Lipid Profile)
What are the nodular swellings of the irises in NF-1 patients?
Hammartomas
When does Physiologic Jaundice begin?

Breast milk Jaundice?
Physiologic Jaundice: starts 2nd or 3rd day of life
(resolves in several weeks)

Breast milk Jaundice: starts after 1 week-old
If an infant presents with a fever and no other Sx, what is the best first test?
Catheterized UA with culture
what is the first step in Tx for a patient with Allergic Rhinitis?
Antihistamines

(do be fooled with "environmental change")
what one sign is most suggestive of Primary TB?
Hilar lymphadenopathy
Dx:
a 15-yo boy presents with pain in the right knee, Hx of easy bruising and chronic fatigue. PE shows mild HSM. X-ray of femur shows "Erlenmeyer Flask deformity." Labs show anemia and thrombocytopenia. Bone marrow shows "wrinkled paper" appearance.

Deficient enzyme?
Gaucher's Dz Type 1

(seen in adolescent ashkenazi jewish population)

enzyme: Acid Beta-Glucosidase
A 2-yo boy undergoes end-to-end repair of an aortic coarctation. Seven years later he has HTN symptoms again.

Tx?
Angioplasty via balloon

(TOC for re-coarctation)
Dx:

Marfan features + Thromboembolic events

enzyme deficiency?
Homocystinuria


enzyme: Cystathionine Synthase
Dx:

Onset of HA with focal neurological sx soon after acute otitis media?
Brain Abscess
Dx:
A <1-mo child with bilious vomiting, abdominal distention and passage of bloody stools
Midgut Volvuvus
MCC of Jaundice in premature infant?
TPN-induced jaundice
Dx:
a child presents with a wide-based gait, decreased vibratory and position sense in lower extremities, absent ankle jerk bilaterally, atrophy of cervical spinal cord and T-wave inversions
Friedreich Ataxia

(Trinucleotide repeats; Autosomal Recessive)
Dx:

severe dehydration in neonate with HypoN, HyperK and HypoG plus Metabolic Acidosis
Congenital Adrenal Hyperplasia

(21-hydroxylase deficiency)
A premature infant on a vent has signs of pneumonia.

MC bug?
Oxacillin-Resistant Staph Aureus

(MRSA would also cause skin pustules of periumbilical and diaper areas)
What is the difference in presentation of ABO incompatability and Rh incompatability?
ABO:
Fetal anemia and Jaundice

Rh:
Hydrops
(anemia, edema, ascites, cariomegaly and hepatomegaly)
What is the difference in Tx wth Ewings Sarcoma and Osteosarcoma?
Ewings:
Surgery, Chemo, Radiation

Osteosarcoma:
Surgery & Chemo only!
Aside from blood lead level, what else is increased in the blood with lead poisoning?
Erythrocyte Protoporphyrin

(usually seen w/ > 55mg/dL of lead)
What is the best way to test for acid-fast bacilli of TB?
Early-morning Gastric Aspiration
Dx:
A 6-yo female child with constant leaking of urine
Low implantation of Ureter
(into vagina)
What is the age of precocious puberty in boy and girl?

What is the best Initial test for Evaluation of Precocious puberty?
Boy: < 9-yo

Girl: < 8-yo

test:
Radiograph of Hand and Wrist to determine Bone Age
Tx:

Hereditary Angioedema
Danazol (and Epi if needed)
Dx:

Superoxide deficiency in Macrophages
Chronic Granulomatous Dz
Dx test:

Chronic Granulomatous Dz
Nitroblue Tetrazolium
Dx:

Defect in neutrophil chemotaxis
Chediak-Higashi syndrome
Dx:

MR with rocker-bottom feet and clenched fist
Edward's syndrome

(Trisomy 18)
Dx:

MR with midline defects, cleft lip/palate
Patau syndrome

(Trisomy 13)
Dx:

Tall, infertile, gynecomastia, microtestes
Klinefelter's syndrome

(XXY)
Age:

Social smile
1 - 2 months
Age:

Rolls onto Back
4 months
Age:

Rolls onto Stomach
5 months
Age:

Walks alone
15 months
Age:

Copies Circle
3 years
Age:

Copies Cross or Square
4 years
Tanner stage:

Penile "growth spurt"
Tanner stage 3
Dx:

Vaccination that cannot be taken if allergic to eggs
Influenza A
Dx:

Place of foreign body aspiration in a child that was standing
Right Bronchi in Superior Segment of Lower Lobe
Dx:

Place of foreign body aspiration in a child that was supine or is an epileptic
Right Bronchi in Posterior Segment of Lower Lobe
Dx:

possible Kidney problem in Turner's Dz
Horseshoe kidney
Dx test:

Duodenal Atresia
AXR

(Double bubble)
Dx test:

Rotavirus
Immunoassay
Dx test:

steathorrhea plus protozoal cysts in stool
Duodenal Aspirate
Bacterial Diarrhea:

Ulcerative colitis-like Sx plus Arthritis
Campylobacter

(MCC of infectious diarrhea)
Bacterial Diarrhea:

Child with acute onset of RLQ pain (like appendicitis) plus diarrhea
Yersinia
Bacterial Diarrhea:

Daycare outbreak plus child with diarrhea and new onset seizure
Shigella
Dx test:

Tuberous Sclerosis
CT scan of head

(Calcified periventricular tubers)
Tx:

West syndrome (Infantile spasms)
(2)
ACTH and Prednisone
Dx:

Anaphylaxis after blood trasnfusion
IgA deficiency
Dx:

very low T- and B-cells (lymphopenia) with bacterial and fungal infections
SCID
Prophylaxis needed for SCID
PCP
Tx:

SCID
Bone Marrow Transplant

(or stem cell transplant with IgG)
Dx:

baby less then 2-yo presents with tachypnea and wheezing
Bronchiolitis (RSV)
Dx test:

Bronchiolitis (RSV)
ELISA nasal washings
Bug:

Assoc with Guillain-Barre
Campylobacter Jejuni
Dx:

Corneal clouding, cataracts, early renal failure
Fabry's Dz
Dx:

Optic atrophy, spasticity, Globoid bodies in brain
Krabbe's Dz
Dx:

Inspiratory stridor, barking cough
Croup (Parainfluenza)
Tx:

Croup
Steroids and humidified air
Dx:

Hearing loss, cataracts, microscopic hematuria
Alport's syndrome
Dx:

salmon-colored rash with daily fever spikes, knee pain, leukocytosis, thrombocytosis, Inc ESR
Still's Dz

(Systemic juvenile RA)
Dx:

Surfactant deficiency in preterm infant
Respiratory Distress Syndrome
(RDS)
Dx:

Heart d/o assoc with DiGeorge syndrome
Transposition of the Great Vessels
Dx:

short, Inc calcium, developmental delay, overy friendly, supravalvular aortic stenosis
Williams syndrome
Dx:

Heart problem with Kartagener's syndrome
Dextrocardia
Next step:

positive PPD
Chest X-Ray

(before medicine)
First test:

Nasal polyps found in child
Pilocarpine Sweat Test

(polyps in child = CF)
Dx:

Infant gets repeated pneumonias at beginning of life due to exhaustion of maternal IgG in system
Transient Hypogammaglobinemia
Dx:

child with increased AA in urine, photosensitivity, ataxia and neuro problems
Hartnup Dz
Dx:

6-yo boy presents with dark pubic hair, enlarged penis/testis, growth spurt and acne
Hypothalamic tumor

(without growth spurt may be 21-hydroxylase deficiency)
Dx:

Hereditary eye problem with progressive night blindness, field constriction and loss of acuity
Retinitis Pigmentosa
First step:

Emergency airway in ER on patient < 12-yo
Needle Cricothyroidotomy
Dx:

"sea-blue" histiocytes
Niemann-Pick
Dx:

Acanthocytes (spiny RBC)
Abetalipoproteinemia
Child Brain Tumor:

Originates from cerebellar vermis and grows to the fourth ventricle
Medulloblastoma
Child Brain Tumor:

Cyst and mural nodule assoc with vonHipple-Lindau
Hemangioblastoma
Child Brain Tumor:

Cyst with a mural nodule in the cerebellum
PiloCYSTIC Astrocytoma

(similar to Hemangioblastoma)
Dx:

Delayed passage of meconium, chronic constipation, FTT, air in bowel
Hirschprung's Dz
Dx:

High fever for 3 days, then macular rash
Roseola
Virus assoc with Roseola
HSV-6
another name for Measles
Rubeola

(not Roseola)
First test:

Infant with seizures
Serum chemistries
Dx:

BRBPR in infant 1 week to 3 months old
Food Allergy-induced Colitis
Dx:

slight fever, sore throat, suboccipital and posterior auricular LN enlargement, rash starts on face and moves down body
Rubella
MC genetic defect in CF
CFTR-508 Deletion on chromosome 7
Dx:

Sickle cell patient with acute severe anemia without reticulocytes, but with normal platelets and WBC
Aplastic Anemia
First step:

child is cyanotic from congenital diaphragmatic hernia
Orogastric Tube placement

(with continuous suction to prevent bowel from decompressing lung)
Dx:

2-week-old infant presents with jaundice. He is exclusively breastfed, his stool has a lighter color, has hepatomegaly and direct bilirubin of 4
Biliary Atresia

(light stool, hepatomegaly and inc direct bilirubin)
An increase in what lab in a 2-week-old infant would lead to breastfed jaundice conclusion?
Increased Indirect Bilirubin

(as high as 10 - 30mg/dL)
Next step:

after confirming Dx of Septic Arthritis
Emergent Surgical Drainage
Dx:

Jaundice within 24 hours of life and an increased direct and indirect bilirubin, anemia, pallor, HSM and reticulocytosis
Erythroblastosis Fetalis
Dx:

Jaundice appearing after the 5 days of life, but within the first week
Neonatal Sepsis

(causing Jaundice)
Dx:

Child of 13-yo with femoral head off the metaphysis, with new bone formation
Slipped Capital Femoral Epiphysis
First step:

Slipped Capital Femoral Epiphysis
Emergency Surgery
Tx:

Kawasaki Dz
IV immunoglobulins and Aspirin
Dx:

IgG, IgM and IgA are all very low, very low B-cells, bacterial infections in the first 5 years of life
X-linked Agammaglobinemia
Dx:

low IgG, IgM and IgA levels, normal B-cells and T-cells; Sx begin at 15 - 35 years old.
Common Variable Immunodeficiency
(CVID)
Dx:

infant with Meningitis from Gonorrhea (meningococcemia) begins to get large purpuric lesions on flank
Waterhouse-Friderichsen syndrome

(acute adrenal failure/hemorrhage from meningococcemia)
COD:

Meningococcemia
Adrenal Failure/Hemorrhage

(from advancing to Waterhouse-Friderichsen syndrome)
Tx:

Homocystinuria
Vitamin B6
Dx:

Beta-Galactosidase deficiency
KraBBe's Dz
Dx:

Alpha-Galactosidase deficiency
fAbry's Dz

(fAAAAAbry's)
Dx:

Hyperinsulinemia, omphalocele, macroglossia, organomegaly
Beckwith-Wiedemann syndrome

(HOMO)
Dx:

short child with hypoglycemia, lactic acidosis, hyperuricemia and hyperlipidemia. Enlarged liver and kidneys and possible seizures
Von-Gierke's syndrome

(G6P deficiency)
Dx:

G-6-P deficiency
Von-Gierke's syndrome
Dx:

Floppy baby with macroglossia and heart failure
Pompe's syndrome

(Maltase deficiency)
Dx:

Maltase deficiency
Pompe's syndrome
Age:

walks alone, speaks 2 words, throws objects
12 months old
Age:

walks up and down stairs without help and has 2 - 3 word phrases
24 months old
First step:

Pyloric Stenosis
Rehydrate and correct Electrolytes
When does stranger anxiety start and peak?
starts: 6 - 8 months

peaks: 12 - 15 months
Dx:

IVP shows focal renal parenchymal scarring and blunting of calices
Chronic Pyelonephritis
Dx:

Bilateral ptosis, difficulty getting up from chair and muscle weakness
Myasthenia Gravis
Dx:

palpable purpura, glomerulonephritis, decreased complement, arthralgias, HSM, peripheral neuropathy and positive HCV
Mixed Essential Cryoglobinemia

(hCv = Cryoglobinemia)
MC Sx of mixed Sickle cell trait
painless Hematuria
When is the best time to give VZ-Ig to child who was playing with others that had chickenpox
within 3 - 5 days of exposure

(otherwise tell parents he will get Sx)
Dx:

Non-immunized mother brings 2-week-old in b/c of spasms involving whole body and erythematous, tender umbilical cord and poor suckling
Tetanus
Dx:

newborn lung problem due to right-to-left shunt from PDA or foramen ovale
Persistent Pulmonary HTN of Newborn
Deficient enzyme:

FTT, bilateral cataracts, jaundice and hypoglycemia at birth
Galactose-1-phosphate
Tx:

prevents painful episodes of Sickle cell crisis
Hydroxyurea
MC child brain tumor in cerebrum
Benign Astrocytoma
Next step:

Foreign Body aspiration removal
RIGID bronchoscopy
Define:

Meconium Ileus
Failure to pass meconium within the first 24 hours of birth
MCC of high fever and chills or sepsis in Sickle cell patient
Strep Pneumococcus

(encapsulated organism that cannot be handled well due to asplenia)
Dx:

Sickle cell patient with pain in the hip including restriction of abduction and internal rotation
Avascular necrosis

(common in sickle cell adolescent)
Dx:

premature neonate with "increased gastric residues"
Necrotizing Entercolitis
Dx:

child born normal and asymptomatic until teens, when he began to have delayed muscle relaxation, progressive muscle weakness, thinning cheeks and atrophy of thenar and hypothenar eminences
Myotonic Muscular Dystrophy