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500 Cards in this Set

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Eye findings pathognomonic for child abuse.
Retinal Hemorrhages
Kid with fractures in various stages of healing. Diagnosis?
Child Abuse
Likely cause of stocking/glove burns.
Child Abuse
Child's story of injury does not match caretakers story.
Suspect Child Abuse
Child abuse can create what kind of fracture of the humerus or femur in a child less than 3?
spiral fracture
Shaking or jerking a child's limbs (abuse) can cause what kind of fracture?
Bucket Fracture
Rib fracture in what location is a clue to child abuse?
Posterior
When do you need further evaluation for child abuse in a child with genital trama.
Always
Under what age can you rule-in child abuse when you find an STD in a child?
Pre-pubertal
Classic inherited bone disorder often confused as child abuse.
Osteogenesis Imperfecta
Cigarette-like burns on a child. Rule this diagnosis out before calling it child abuse.
bullous impetigo
What is "coining"?
alternative medicine in some cultures where skin is rubbed raw in certain areas by a coin
Congenital skin condition common in Asian, East African, and Native American races which is commonly confused with child-abuse.
Mongolian "Blue" Spot aka Congenital Dermal Melanocytosis
4 STDs to test for when suspecting sexual abuse.
Gonorrhea, Chlamydia, Syphilis, HIV
CT head to look for what when you suspect child-abuse?
subdural hematoma
Can you be sued for reporting suspicious activity to child-protective-services?
no
Child with poor hygiene, poor social skills, behavioral abnormalities, or malnutrition. What social factor needs to be in the differential?
Neglect (Child Abuse)
Lithium is associated with what particular heart defect?
Ebstein's Anomaly (tricuspid too inferior)
What drug's teratogenicity profile resembles Fetal Alcohol Syndrome?
Phenytoin
Thalidomide, a classic teratogen, was found to cause defects where?
limbs
What infection causes congenital cardiac, cerebral, ophthalmic and auditory defects?
Rubella
What common disease of the American lifestyle is associated with an increase risk of congenital heart defects?
DM
What maternal metabolic disorder has a teratogenicity profile similar to Fetal Alcohol Syndrome?
Maternal PKU
Which shunt direction presents with cyanosis?
right-to-left
Noncyanotic heart diseases (3 D's)
VSD, ASD, PDA
Cyanotic heart diseases (5 T's)
Truncus arteriosis, Transposition of the great Vessls, Tricuspid Atresia, Tetralogy of Fallot, Total Anomalous pulmonary venous return
Most common congenital heart defect.
VSD
Why wait at least until after 12 months of age to fix a VSD in an asymptomatic child?
most close spontaneously
Murmur of a VSD.
harsh holosystolic best at LLSB
Cat like Cry in newborn. Diagnosis?
Cri Du Chat
Down Syndrome babies have a higher occurance of these 2 heart defects.
VSD and ASD
Common ECG changes even with small VSDs.
LVH
Diagnostic test for work-up of a congenital murmur.
Echocardiogram
Infant with VSD now has signs of Pulmonary Hypertension. Treatment?
surgery
Infant with VSD now has signs of CHF. Treatment?
Medical: diruetics, inotropes, and ACEI
Infant with VSD and his CHF is now not responding to medical treatment. Next step in management?
Surgery to close defect
Infant with recurrent respiratory infections found to have harsh holosystolic murmur. Cause of infections?
VSD
What is eisenmenger's syndrome?
Left to Right shunt leads to Pulmonary Hypertension leads to Right to Left shunt.
Blue when feeding, red when crying. Likely Diagnosis?
Choanal Atresia
How to confirm the diagnosis of choanal atresia?
fails to pass NG tube
Murmur of ASD.
systolic ejection murmur best at ULSB w/ fixed split S2
Fetal Alcohol Syndrome babies have a higher occurance of these 2 heart defects.
VSD and ASD
Holt-Oram syndrome means absent radii, first degree heart block, and this heart defect.
ASD
Common ECG changes with ASD (2).
RVH and PR prolongation
At what level of Pulmonary Hypertension is surgery indicated to close ASD in an infant?
More than 2:1 Pulm to Systemic blood flow
ASD with CHF in an infant. Treatment.
Surgery to close defect
Murmur of a PDA.
Machine-like, continuous best at ULSB
Pulse Pressure in PDA pt.
wide
This congenital/in-utero infection is associated with PDA.
Rubella
Males or Females more likely to have a PDA?
Females
Common ECG change with PDA.
LVH
Medication that closes PDA.
Indomethacin
Kid with PDA after 6 months despite Indomethacin. Treatment?
Surgical closing
Cyanotic neonate with PDA. Treatment for PDA?
Prostoglandin E1 analog to keep it open
Top 4 differential diagnoses if neonate goes into shok.
sepsis, IEM, PDA closes on ductal dependent heart defect, CAH
Blue Neonate, Blue Infant, Blue Kid. What 3 respective heart defects?
TGV (neonate), Tet of Fallot (infant), Eisenmengers e.g. VSD (kid)
Genetic defect associated with Coarctation of the Aorta
Turner's syndrome
Valve malformation associated with Coarctation of the Aorta.
Bicuspid Aortic Valve
Asymptomatic hypertension in childhood. Echo may show what?
Coarctation of the Aorta
Coarctation of the Aorta shows what 2 signs on CXR?
3 sign (pre&postdilation) and rib notching (collateral circulation)
Immediate treatment for Coarcation of Aorta that presents with severe cyanosis in infancy.
PGE1
BP is decreased in leg compared to right arm in an infant. Diagnosis?
Coarctation of the Aorta
Differential Cyanosis (relatively higher O2 saturation in R arm) is pathognomonic for what?
Coarctation of the Aorta
Murmur with Coarcation of the Aorta
short systolic murmur best in L Axilla
Radiofemoral delay in an infant. Diagnosis?
Coarctation of the Aorta
Most common CYANOTIC congenital defect in newborns?
Transposition of the Great Vessels (TGV)
Rare genetic syndrome associated with TGV.
DiGeorge Syndrome
Most common CYANOTIC congenital defect in infants (4 to 18 months)?
Tetralogy of Fallot; (Eisenmengers from VSD is in differential)
Immediate treatment for Transposition of the Great Vessels (TGV)? (before surgery)
PGE1
Reverse Differential Cyanosis (deeper cyanosis Upper extremities compared to LEs) is pathognomonic for what?
Transposition of the Great Vessels (TGV)
DiGeorge CATCH22 mnemonic.
Cardiac abnormalities, Abnormal facies, Thymic aplasia, Cleft palate, Hypocalcemia (tetany), 22q11 deletion
"Egg Sillhouette" on CXR of cyanotic neonate. Diagnosis?
Transposition of the Great Vessels (TGV)
PROVo menomonic in Tetralogy of Fallot.
Pulmonary stenosis, RVH, Overriding aorta, VSD
Maternal PKU is a risk factor for this congenital heart defect.
Tetralogy of Fallot
Toddler squatting for hypoxemia relief. Diagnosis?
Tetralogy of Fallot (Tet spell)
Murmur in Tetralogy of Fallot.
systolic ejection murmur best at ULSB w/ single S2
Common ECG changes with Tetralogy of Fallot.
R axis dev. And RVH
CXR shows boot shaped heart. What congenital heart defect?
Tetralogy of Fallot
CXR shows increased vascular markings in these 2 congenital heart defects.
ASD and VSD
Severe pulmonary stenosis and early presentation of Tetralogy of Fallot. What immediate treatment (before surgery)?
PGE1
Treatment for Tet Spells.
O2, morphine, propranolol
1 month developmental milestone: social.
makes eye contact
2 month developmental milestone: gross motor
lifts head/chest when prone
2 month developmental milestone: social
social smile (more than reflex smile)
Four month developmental milestones: F sound mnemonic (2).
Four: rolls Front to back (gross motor); lauGHs (social)
Six month developmental milestones: "S" mnemonic.
Sits, Switches objects hand-to-hand, Stranger anxiety
When does a baby roll from back to front?
5-6 months (just remember 6 months)
How long to keep baby on back to sleep.
Until can roll from back to front (usually 6 months)
Non-specific "mama" or "dada" sounds. How old?
9 months (remember it takes 9 mos to be a "mama")
Immature pincer grasp (three fingers). How old?
9 months
Mature pincer grasp (tips of thumb and pointer finger). How old?
12 months
Gross motor developmental milestone at 12 months.
Walks alone
Language developement at age two years old.
2 words together at 2 (2 word phrases); 1/2 understood by strangers
Language developement at 2, 3, and 4 years old.
2/4ths (1/2), 3/4ths, then 4/4ths (all) of language understood by strangers
Language development at 3 years old.
3 words together at 3 (3 word sentances); 3/4ths understood by strangers.
Parallel play: how old?
2 years old
Group play: how old?
3 years old
4 years old fine motor skills: draws what?
square or cross (4 sides at 4 years old)
Copies a circle at how old?
3 years old
Stacks 2-4 blocks at how old?
2 years old
3 year old developmental milestones: "3" mnemonic for motor, language, and social.
rides a TRIcycle, uses 3 words together, group play (3 or more people)
Child took off his clothes and is found naked. How old is he at this milestone?
2 years old
Skipping is developmental milestone for what age?
5 years old
Head circumference rule of 3s, 9s, and 5s.
birth=35cm, 3mo=40cm, 9mo=45cm, 3yr=50cm, 9yr=55cm
Weight change as a function of Birth Weight (BW).
0-2wks=0.9xBW
2wks=BW
5mo=2xBW
12mo=3xBW
24mo=4xBW
Cooing milestone at what age?
2 months
Babblying milestone at what age?
6 months
How many words at one year old?
one: usually mama or dada (specific)
Writes name and counts to 10. how old?
5 years
How to calculate development in premature kids.
adjust for gestational age: use due-date as adjusted birth date
Define Failure to Thrive (2).
on growth chart: persistently less than 5th percentile or crossing 2 major percentile lines
in Failure to Thrive (FTT), list the usual order of falling off the curve: head, weight, height.
1st weight, 2nd height, 3rd head circumference
Average age of onset of puberty in girls.
10.5 years
Average age of onset of puberty in boys.
11.5 years
Female usual order of progression of puberty.
thelarche
pubarche
growth spurt
menarche
Male usual order of progression of puberty.
gonadarche
pubarche
adrenarche
growth spurt
What is thelarche?
breast development
What is gonadarche (male)?
testicular enlargement
age definition of delayed puberty (female) vs. primary amenorrhea
13 years w/ no developement=delayed puberty
14 years w/ no devo.=1*amenorrhea
16 year old has not had menarche, but has begun to develope pubic hair and breasts. Dx Amenorrhea?
Yes, she has 1*amenorrhea
definition of delayed puberty in males.
no testicular enlargement by age 14.
Most common cause of delayed puberty.
consitutional growth delay (considered a normal variant despite the diagnosis of delayed puberty)
Definition of consitutional growth delay.
low on growth curve, but consistent growth (will eventually acheive target height potential)
precocious puberty age: girls vs. boys
under 8 in girls
under 9 in boys
Memorize these 4 signs of autism (age 2 mo, 12 mo, 16 mo, and 24 mo).
no eye contact by 2 mo
no babbling by 12 mo
no single words by 16 mo
no 2-word phrases by 24 mo
Simian crease; think what?
down syndrome
Why do down-syndrome kids often have ASD or VSD?
endocardial cushion defects
What cancer is down syndrome associated with?
ALL
What neurologic disease is down syndrome associated with?
early alzheimer's
More down syndrome babies have mothers with advanced maternal age. True or False.
False (women under 30 have way more babies, therefore, this group has more down syndrome babies total)
Mother with Down Syndrome baby due to Robertsonian translocation. What advice to give?
higher risk of recurrence
2 most common pathologies (non-asian race) associated with "epicanthal folds."
Fetal Alcohol Syndrome and Down Syndrome
Hirschsprung's disease found; consider what genetic disorder?
Down Syndrome
2 GI pathologies associated with Down Syndrome
Hirschsprung's disease and duodenal atresia
3 As and 3 Hs associated with Down Syndrome.
ALL
Alzheimer's
ASD (and VSD)
Hearing (and vision problems)
Hirschsprung's
Hypothyroidism
Long philtrum and thin upper lip. Suspected Diagnosis?
Fetal Alcohol Syndrome
Percentage of people with Fetal Alcohol Syndrome with mental retardation? percent with learning disabilities?
25% MR; nearly 100% have learning disabilities
What percent of people with down syndrome are only "Mildly" retarded?
15%
Usually death occurs in Edwards' syndrome (tri 18) and Patau's syndrome (tri 13) in what time period?
within 1 year of birth.
Edward's syndrome is trisomy of what chromosome?
18 (remember Edward is 6 letters and it is trisomy, so 6x3=18)
Patau's syndrome is trisomy of what chromosome?
13 (Patau has 5 letters and remember Edward's is chr. 18, so 18-5=13)
Patau's Syndrome (tri 13); remember P is for what?
Palate is cleft
Edwards' Syndrome; remember E is for what? R is for what?
Eighteenth Chromosome; Rocker Bottom Feet
Trisomy 13 often has what abnormality of the extremity?
polydactyly: remember 13 fingers
Remember these 3 things for Trisomy 18.
low-set ears, microagnathia, rocker-bottom feet
Remember these 3 things for Trisomy 13.
microphthalmia, microcephaly, polydactyly
Genetics of Klinefelter's
XXY
Tall stature, long extremities, and gynecomastia. What chromosomal abnormality is on the differential?
Klinefelter's
Test which confirms klinefelter's.
karyotype
XXY versus fragile X: size of testicles.
XXY: small
Fragile X: large
Asymptomatic Klinefelter's usually presents in what way?
infertility
Treatment for Klinefelter's.
testosterone
Female with no barr body on karyotype. Diagnosis.
Turner's
why do turner's syndrome patients often present with swelling?
dysgenesis of the lymphatics causes lymphedema in hands and feet
Short stature, widely spaced nipples. Suspected diagnosis?
Turner's
Turner's associated with what heart abnormalities (2).
Coarctation of Aorta and bicuspid aortic valve
This chromosomal abnormality is observed with increased frequency in prisons.
XYY
What chromosomal abnormality: tall, acne, antisocial behavior.
XYY
What important preparation for PKU testing?
baby must eat a protein meal first
Long face, large jaw, large ears, and autism. Diagnosis?
Fragile X
Undiagnosed PKU presents what way? (2 besides mental retardation)
mousy urine odor
eczema/fair skin
retardation
Increase what in diet of PKU baby?
Tyrosine
CFTR is on what chromosome?
7
What race gets CF more often?
Caucasians
Why does Fragile X syndrome get worse in each successive generation?
Genetic anticipation from triplet repeat expansion
FTT and recurring sinopulmonary diseases. 1st on the differential?
CF
infants presenting with CF usually present this way.
Meconium ileus
Man presenting for infertility found to have congenital absence of the vas deferens. Diagnosis?
Likely Mild CF
Fat soluble vitamins.
ADEK
positive (suspected false) CF test on newborn screening. What follow-up test?
sweat chloride test
Treatment for CF related FTT: diet and supplements.
high calorie/high protein diet; supplement with pancreatic enzymes and vitamins ADEK
medical treatment for CF related pulmonary dxs (ABCD).
antibiotics, bronchodilators, corticosteroids, DNase
2 transplants to consider in CF patient
lung and pancreas
Niemann Pick disease: deficiency of what? Build up of what?
sphingomyelinase deficiency means build up of sphignomyelin cholesterol
Tay-Sachs disease: absence of what?
Hexosaminidase: remember tay-saX with heXose.
Cherry-red spot on macula. Diagnosis?
Tay-Sachs Disease
Tay Sachs is more common in what group?
Jewish decent
Prognosis for Tay-Sachs
death occurs by age 3
Tay Sachs presents with weakness and mental regression at what age?
3-6 months
Fabry's disease (X-linked lysosomal storage disease) causes ceramide trihexoside accumulation in these three organs increasing risk for:
kidney: renal failure
brain: stroke
heart: MI
Optic atrophy, spasticity, and early death because of galactocerebroside accumulation in the brain. What disease? (auto recessive lysosomal storage disease)
Krabbe's
Pt. with hepatosplenomegaly, anemia, and thrombocytopenia found "crinkled paper" cytoplasm in bone marrow cells. Caused by glucocerebroside accumulation in brain, liver, spleen, and bone marrow. What lysosomal storage disease?
Gaucher's disease
Accumulation of sulfatide in brain, kidney, liver, and peripheral nerves: what lysosomal storage disease?
metachromatic leukodystrophy
Deficiency in iduronidase that leads to corneal clouding and mental retardation. What lysosomal storage disease?
Hurler's syndrome
For difference between Hurler's Syndrome and Hunter's Syndrome (lysosomal storage diseases), remember this mnemonic
Hunters need to see to aim for the X. (Hunters=no corneal clouding, X-linked instead of Auto recessive)
Most common cause of bowel obstruction in first 2 years of life.
intussusception
Intermittent abdominal pain/cramp with vomiting in otherwise healthy kid; pain-free between episodes. Diagnosis?
intussusception
current jelly stool. Diagnosis?
intussusception (late)
Sausage shaped RUQ abdominal mass and abd. pain. Diagnosis?
intussusception
Untreated intussusception may progress to shock because?
blood flow is compromised which leads to necrosis
Meckel's diverticulum is a risk factor for this common cause of bowel obstruction.
Intussusception
Intussusception: Diagnosis? Treatment?
Air-Contrast Enema
When to consider surgery for intussusception? (3 reasons)
unstable
peritoneal signs (perforation)
barium enema unsuccessful
Classic metabolic derangement in pyloric stenosis.
Hypochloremic, Hypokalemic, Metabolic Alkalosis
Baby INITIALLY FEEDS WELL, then begins to vomit more and more often. Suspected diagnosis?
Pyloric Stenosis
Baby regurgitates and chokes on FIRST feed and not able to feed despite nursing coaching. Suspected diagnosis?
Esophageal atresia
Baby feeds fairly well, but often vomits after feeds. Suspected diagnosis?
Normal infantile GERD: "spitting-up"
Typical onset of symptoms in pyloric stenosis (age of infant).
3 weeks
Diagnostic of choice for pyloric stenosis.
Abd. U/S
"String sign" with barium X-ray is in Step 2 question: your differential based on age.
infant: pyloric stenosis (upper GI)
adult: Chron's (lower GI)
elderly: Cancer (lower GI)
Olive-shaped epigastric mass with no abdominal pain. Classic diagnosis?
Pyloric Stenosis
Infant with "projectile emesis." Diagnosis?
Pyloric Stenosis
Treatment for pyloric stenosis.
surgery (pyloromyotomy)
Immediate management of pyloric stenosis.
correct dehydration, hypokalemia, and alkalosis
Treatment for normal infantile GERD ("spitting-up").
thicken formula and sit baby upright while feeding
Meckel's rule of 2's (6 rules).
Most common in children under 2
2 times as common in males
2 types of tissue (pancreatic and gastric)
2 inches long
found within 2 feet of ileocecal valve
Occurs in 2 percent of the population
What duct can create a Meckel's Diverticulum?
Omphalomesenteric (vitelline) duct
Sudden, painless rectal bleeding in 1 year old. Diagnosis?
Meckel's Diverticulum
Usual presentation of Meckel's Diverticulum.
usually incidental finding (asymptomatic)
Diagnostic test of choice for Meckel's Diverticulum.
scintigraphy scan (technetium-99m pertechnetate)
Treatment for Meckel's Diverticulum.
excision
Hirschsprung's Disease pathophysiology.
lack of ganglion cells in distal colon (uncoordinated peristalsis and decreased motility)
Presentation of severe Hirschsprung's (symptoms and age).
complete constipation, bilious vomiting, and FTT presenting at birth
Presentation of mild Hirschsprung's (symptoms and age).
chronic constipation presenting in childhood
Imaging study of choice for Hirschsprung's (not difinitive diagnosis).
Barium enema XR
Difinitive diagnositic test for Hirschsprung's.
full thickness rectal biopsy
What surgery for Hirschsprung's?
Coloectomy and Colostomy, followed by pull-through later
Bilious emesis at birth in Step 2 question. Your differential (based on stool habits).
Hirschsprung's: complete constipation
Volvulus: blood or mucus in stool (do AXR for possible NEC)
Malrotation (e.g. Cecum in RUQ) and Ladd's bands (congenital) or Adhesions (postsurgery). Complications? (2)
prone to bowel obstruction and ischemia
Unstable newborn with bilious emesis. Work up?
emergent laparotomy
Stable newborn with bilious emesis. Work up?
AXR (r/o NEC) and EGD (r/o Malrotation); consider barium XR if constipated (Hirschprung's).
Management of bowel obstruction due to congenital malrotation.
NG decompression and IVF
Management of bowel ischemia due to volvulus.
emergent surgery
Biggest risk factor for NEC.
prematurity (but can occur in full-term as well)
Bloody stools in newborn. Diagnostic test?
serial AXRs (for NEC)
Pneumatosis intestinalis. Diagnosis?
NEC
When to consider surgery for NEC.
if worsening or unstable
Management of NEC (if stable).
nutrition: NPO, so TPN
sx: orogastric tube decompression
ppx: antibiotics
What does pneumatosis intestinalis look like.
air in bowel wall (double white line)
The feared complication of GI surgery in a child.
short bowel syndrome
What to remember about Lab findings in NEC.
they are non-specific, so you must maintain index of suspicion based on clinical picture
Chronic thrush in child. Suspect what?
immunodeficiency
50% of immunodeficiencies are this type of cell deficiency.
B-Cell
age of presentation of B-Cell immunodeficiencies
after six months (breast milk immunity wains)
age of presentation of T-Cell immunodeficiencies
1-3 months
Asplenia or splenic dysfunction (e.g. sickle cell) patients have what type of immunodeficiency.
complement deficiency
Recurrent mucous membrane infection and poor wound healing. What type of immunodeficiency?
phagocyte deficiencies (CGD, LAD, CHS, Job sx, MPD)
X-linked agammaglobulinemia (XLA) (Bruton's Agammaglobulinemia) is what type of cell deficiency and what gender?
Bruton's is B-Cell deficiency in Boys
Usual age of onset of Common Variable Immunodeficiency (CVID).
20s
Child presents at 1 year with 6 month history of recurrent pseudomonas, strep pneumo, and H. Flu infections. What specific immunodeficiency?
X-linked agammaglobulinemia (Bruton's) (or XLA)
Screening test for male with recurrent encapsulated infections which began at 6 months of age.
Quantitative immunoglobulin levels
Confirmation test for child with low Ig quantity (no 5th peak) and suspected X-linked agammaglobulinemia.
B-Cell and T-Cell subsets by flow cytometry (low B-Cells, usually high T-Cells)
Treatment for X-linked Agammaglobulinemia.
IVIG and prophylactic antibiotics
Treatment for CVID.
IVIG
Most common immunodeficiency (1/600).
IgA deficiency
Patient has anaphylactic transfusion reaction. What immunodeficiency is on the differential? Why?
IgA deficiency; reaction is due to formation of anti-IgA antibodies
Recurrent H. Flu and Giardia infections. What immunodeficiency? What diagnostic test?
IgA deficiency; do quantitative IgA levels
Why not IVIG for IgA deficiency (a B-Cell type deficiency).
anti-IgA antibodies will develop
Treatment for IgA deficiency.
treat infections as they arise
15-25% of patients with CVID get this cancer.
lymphoma
Screening test for CVID when patient in 20s gets recurrent pyogenic upper and lower respiratory infections.
Quantitative immunoglobulin levels
Confirmation test for child with low Ig quantity (no 5th peak) and suspected CVID.
B-Cell and T-Cell subsets by flow cytometry (low B-Cells)
Step 2 question shows Hypogammaglobulinemia. Your deferential based on age.
child over 6 months: XLA
adult in 20s: CVID
what percentage of patients with CVID get an auto-immune disease (e.g. ITP or hemolytic anemia)?
25%
What infection to r/o when considering diagnosis of immunodeficiency.
always r/o HIV
Congenitally absent tonsils. What immunodeficiency?
XLA
What type of immunodeficiency is DiGeorge syndrome?
T-Cell deficiency
Test to screen for DiGeorge syndrome
absolute lymphocyte count (CBC w/ diff)
Treatment for DiGeorge syndrome.
BM transplant and IVIG
Baby with cleft palate, low set ears, and wide epicanthal fold. What feature in the question would clue you in to DiGeorge rather than a trisomy? (2)
More mild (older presentation); or can present with Tetany (hypocalcemia) in first days of life
what percent of SCIDs is x-linked?
50%
Frequent bacterial infections, thrush, and opportunistic infections in baby under 6 months. What immunodeficiency?
Severe Combined Immunodeficiency (SCID)
Oculocutaneous telangiectasias and progressive cerebellar ataxia; developmental delay. Found to have a DNA repair defect. What immunodeficiency?
ataxia-telangiectasia
Why try to avoid imaging with patients with Ataxia-Telangiectasia?
radiation induced cancers
Treatment for Ataxia-Telangiectasia?
no specific treatment; may need IVIG. Maintain high index of suspicion for associated cancer
Immediate management for baby with suspected SCID.
isolation
Treatment for SCID.
BM transplant and IVIG
Prophylaxis needed for SCID.
Jiroveci (e.g. TMP-SMX) and Fungal ppx
What percent of SCID is due to adenosine deaminase deficiency?
20%
No HLA expression and inherited auto-recessive. What type of (combined) immunodeficiency?
Bare-Lymphocyte syndrome.
Male infant or toddler with thrombocytopenia (bleeding) and h/o recurrent otitis media, Eczema, and Asthma. What immunodeficiency?
Wiskott-Aldrich Syndrome
Prognosis for Wiskott-Aldrich Syndrome.
Rarely survive to adulthood
Treatment for Wiskott-Aldrich Syndrome.
BMT; IVIG and antibiotics as needed
Why wear a helmet in Wiskott-Aldrich Syndrome?
prone to hemorrhage (thrombocytopenia etc.)
Chronic skin and bone infections. What immunodeficiency?
Chronic Granulomatous Disease (CGD)
This neutrophil oxygenation assay is used for diagnosing CGD.
TMP-SMX
Itraconazole
IFN-gamma
Three step prophylaxis for CGD.
TMP-SMX
Early omphalitits or delayed separation of the umbilical cord. What immunodeficiency?
Leukocyte Adhesion Deficiency (LAD)
Catalase positive skin infections (especially Staph and Aspergillis). What immunodeficiency?
Chronic Granulomatous Disease (CGD)
Common problem in GI and GU tracts in patients with CGD.
obstruction from granulomas
Treatment of colitis or granuloma obstruction in patients with CGD.
prednisone
Rare immunodeficiency syndrome that includes oculocutaneous albinism, neuropathy, and neutropenia.
Chediak-Higashi Syndrome
Treatment for Chediak-Higashi Syndrome
BMT
Wide nose, prominent forehead, doughy skin. What immunodeficiency?
Job syndrome (Hyper IgE)
Staph abscesses and Candida infections. Eczema is common. No thrombocytopenia. What immunodeficiency?
Job syndrome (Hyper IgE)
Appropriate vaccinations for terminal complement deficiencies.
Meningogoccal Vaccine
Hereditary Angioedema (episodes lasting hours provoked by stress; can be life-threatening) is what type of immunodeficieny?
Complement deficiency (C1 esterase deficiency)
Test for Complement Deficiency.
CH50
Use this before surgery in patients with complement deficiency C1 esterase.
purified C1 esterase and FFP
CRASH and Burn mnemonic for Kawasaki Disease criteria.
High fever for AT LEAST 5 days and:
Conjunctivits
Rash (truncal)
Adenopathy
Strawberry tongue/mouth
Hands and Feet red/swollen
Age for Kawasaki Disease.
under 5 years old
Classic race for Kawasaki Disease.
Asian
Why Aspirin for acute Kawasaki Disease?
sx treatment (inflammation and fever)
Two part treatment for Kawasaki Disease.
IVIG and ASA
Why IVIG for Kawasaki Disease?
prevent coronary aneurisms
Why Aspirin for 6 weeks after Kawasaki Disease?
reduces risks (possible coronary aneurism and MI)
What regular follow-up is necessary after Kawasaki Disease?
Echocardiograms
Treatment for Juvenile Idiopathic Arthritis (JIA) (3).
1. NSAIDS
2. Corticosteroids
3. Immunosuppression (Methotrexate or Etanercept)
Usual RF and ANA results in Juvenile Arthritis.
RF negative, ANA positive
Morning Stiffness for over six weeks in patient under 16 years old. Meets criteria for this diagnosis?
Juvenile Idiopathic Arthritis (JIA) - formerly JRA
Child with recurrent high fever, hepatosplenomegaly, rash, and Morning Stiffness in joints. Diagnosis?
Still's Disease (Systemic Onset Arthritis)
3 most common Acute Otitis Media (AOM) pathogens.
Strep pneumo, H. Flu, Moraxella cat.
When to give antibiotics in AOM? (3 age ranges)
under 6 months: always
6-24 months: if certain of diagnosis or if severe (high fever)
2-12 years old: if severe (high fever)
Usual AOM antibiotic regimine.
Amoxicillin 80-90 mg/kg/day for 10 days
Add Clavulonate to Amoxicillin for AOM if (2 criteria)?
if failed Amoxicillin (H. Flu or Moraxella suspected) or if severe sx (high fever)
Treatment option to consider with chronic/recurrent AOM.
tympanostomy tubes
Most common cause of bronchiolitis.
RSV (often used interchangeably)
Age of RSV.
under 2
Season of RSV.
fall and winter months; longer in Florida
What 3 indications for RSV prophylaxis with RespiGam or Synagis (during RSV season).
premature, chronic lung disease, or congenital heart disease
How to diagnose RSV bronchiolitis.
clinically: fever and wheezing; NG aspirate not needed
When to hospitalize kid with RSV.
if under 3 months
if Desaturation or unstable
if RFs: premature, cardio, or pulmonary disease
Outpatient treatment for RSV bronchiolitis.
hydration and nebulizers
Inpatient treatment for RSV bronchiolitis (other than hydration and nebulizers)
O2 if needed, albuterol if it works
2 year old with stridor: most probable diagnosis (90%)?
croup (laryngotracheobronchitis)
What is the "steeple sign" in Croup?
XR shows subglottic narrowing of the trachea
Triple therapy if Croup requires hospital admission.
O2, IM corticosteroids, nebulized epinephrine
Age of Croup patient.
under 3 years
Age of Epiglottitis patient.
3-7 years
Age of Tracheitis patient.
under 2 years
Peritonsilar vs. Retropharyngeal abscess: classic associated symptoms.
Peritonsilar: can't open mouth (trismus)
Retropharyngeal: supine with neck extended
Most common cause of Croup.
parainfluenza
Most common cause of pertionsilar and retropharyngeal abscesses.
Group A Strep (pyogenes)
Child with muffled voice and drooling. Retropharyngeal abscess vs. Epiglottitis: what is their posture?
Abscess: supine with neck extended
Epiglottitis: seated (tripod) with neck extended
What does the "thumb print" on XR classically indicate?
Epiglottitis
Why has the etiology of Epiglottitis changed over the last 30 years?
H. Flu decreasing incidence since vaccination
Most common cause of Tracheitis.
Staph Aureus
Age of Peritonsillar Abscess patient.
usually over 10 years old
Age of Retropharyngeal Abscess patient.
6 months to 6 years
What is different on exam with peritonsillar abscess?
affected tonsil is deviated medially (no imaging necessary for diagnosis)
Diagnosis of Retropharyngeal Abscess (since there is no change visible on exam like Peritonsillar).
CT
Treatment of Retropharyngeal Abscess.
incision and drainage; antibiotics
Treatment of Peritonsillar Abscess
incision and drainage (+/- tonsillectomy); antibiotics
Immediate management if epiglottitis suspected.
intubate in OR
IV antibiotic for epiglottitis.
Ceftriaxone or Cefuroxime
Why should you not use a tongue depressor aggressively if evaluating for epiglottitis?
it may provoke airway spasm and collapse
Headach, high fever, nuchal rigidity. Diagnosis?
meningitis
Top 3 causes of meningitis in kids under 6 years old.
e. coli, s. pneumo, n. meningitis
How to remember Kernig and Burdzinski's signs for meningeal irritation.
K=Knee extension pain, B=bowed neck pain
Neonate with lethargy, poor tone, bulging fontanelle, low temperature, and vomitting. Diagnosis?
meningitis or encephalitis
What bacteria causes meningitis associated with petechial rash?
Neisseria Meningitidis
Most common causes of aseptic (viral) meningitis.
enterovirus (e.g. echovirus or coxsackie virus)
If testing LP for bacterial meningitis, what 2 things do you do first?
CT to check for ICP elevation, and order empiric antibiotics
Neonate with suspected meningitis: what empiric antibiotics?
Ampicillin and Gentamicin (or Amp and Cefotaxime)
Child with suspected meningitis: what empiric antibiotics?
Ceftriaxone and Vancomycin
HSV encephalitis panel ran as an add-on to LP for suspected meningitis. What must you do as well?
Start empiric Acyclovir
What bacteria causes Whooping Cough?
Bordetella Pertussis
What is the vaccine to prevent Whooping Cough?
DTaP
Why do we vaccinate to prevent whooping cough if immunity usually wains by adolescence?
Pertussis is lifethreatening in infants; milder infection in older children.
Three stages of whooping cough.
1) catarrhal (URI sx): 1-2 weeks
2) paroxysmal (cough): 2-3 months
3) convalescent (getting better)
Whooping Cough patients report emesis even without nausea. Mechanism?
post-tussive gag reflex
Which of the 3 stages of whooping cough is the most contagious stage?
catarrhal
When to hospitalize kid with Pertussis.
if less than 6 months
Treatment for Pertussis.
Erythromycin x 14 days to patient and close contacts.
When can a kid go back to school after Pertussis (2 options).
After 3 weeks of sx or after 5 days of antibiotics
What is another name for Rubeola?
Measles
What causes Erythema Infectiosum (Fifth Disease)?
Parvovirus B19
Aplastic crisis in sickle cell or severe iron deficiency patients. Cause?
Parvovirus B19
Erythema Infectiosum (Fifth Disease) rash and progression.
maculopapular, pruritic; Starts on arms then spreads to trunk and trunk and legs
slapped cheek rash. Diagnosis?
Erythema Infectiosum (Fifth Disease)
Mom's rash not so bad, but congenital infection is associated with fetal hydrops and death. Viral Exanthum?
Erythema Infectiosum (Fifth Disease): Parvovirus B19
Erythema Infectiosum (Fifth Disease) prodrome characteristics.
usually none; sometimes low grade fever
What virus causes measles (Rubeola)?
Paramyxovirus
Measles (Rubeola) prodrome charcteristics.
The 3 c's: Cough, Coryza, and Conjunctivitis
What are Koplik's spots?
small irregular red spots with central blue/gray specks (associated with measles)
Measles (Rubeola) rash and progression.
maculopapular; head to feet
Rare complication of Measles used on House Season 1 Episode 2.
Subacute Sclerosing Panencephalitis
What virus causes Mumps?
Mumps virus
Bilateral Parotitis in non-vaccinated patient. Diagnosis?
Mumps
Males past puberty who develop Mumps have 30% risk of this complication.
Orchitis
The 3 day measles or German Measles.
Rubella
What virus causes Rubella?
Rubella virus
Clinical presentation of Rubella
low-grade fever; the "not-so-ill" measles
Rubella rash and progression.
macupapular, tender; face to trunk to extremities
What virus causes Roseola Infantum (Sixth Disease)?
HHV-6
Prodrome of Roseola Infantum (Sixth Disease)
3 days of high fever (usually over 40); no other sx
Rash of Roseola Infantum (Sixth Disease) and progression.
maculopapular rash appears as fever breaks; trunk to face and extremities
Most common cause of febrile seizures.
Roseola Infantum (Sixth Disease): due to rapid fever onset
What virus causes Chicken Pox?
Varicella Zoster Virus (VZV)
Prodrome of Varicella.
MILD fever, no appetite, malaise (for 24 hours before rash)
Teardrop on rose petal. Diagnosis?
Varicella
Congenital Rubella Syndrome (triad)
neuronal deafness, PDA or ASD, cataracts
Varicella rash description and progression.
vesicular lesions at different stages of healing; generalized at onset
Why vaccinate women who have never had chicken pox?
Prevent Congenital Varicella Syndrome
VZV vaccine effects on Zoster.
halves the incidence of zoster rash and lowers incidence of post-herpetic neuralgia by 2/3s!
What is the first symptom of Zoster?
dermatomal neuralgia
What test to order in young patient with Herpes Zoster?
HIV (Zoster is more likely when patient is immunocompromised)
Remember this complication can occur with any of the viral exanthums.
Encephalitis
What causes Hand-Foot-Mouth disease?
Coxsackie A virus
Rash description and location in Hand-Foot-Mouth disease.
maculopapular vesicular; located on hands, feet, and mouth AND BUTTOCKS
APGAR mnemonic.
Appearance
Pulse
Grimace
Activity
Respiratory
What is the intermediate range of APGAR scores that requires at least stimulation and observation.
4 to 7
Which type of hyperbilirubinemia in neonate is always pathologic (2)?
conjugated (direct) or any jaundice in first 24 hours
How to remember if Conjugated and Unconjugated is direct or indirect?
consanants (c=d) and vowels (u=i)
Pathophysiology of Kernicteris.
bilirubin deposition in the basal ganglia, pons, and cerebellum.
Neonatal jaundice now with high pitched cry, lethargy, poor feeding, and seizures. Likely Diagnosis?
Kernicteris
First 2 tests in work-up for neonatal jaundice with direct hyperbilirubinemia.
Observe stool (pale?) and test Urine: UA and Cx
Work-up for neonatal jaundice with direct hyperbilirubinemia that is normally done on all newborns anyway.
newborn metabolic screening
2 most common causes of direct hyperbilirubinemia on newborn screen.
galactosemia and hypothyroidism
imaging study to order if source for direct hyperbilirubinemia in neonate.
U/S or HIDA scan
7 tests to evaluate liver function (for neonatal hepatitis) in neonate with direct hyperbilirubinemia (LFT panel plus 3 more)
Alk Phos, AST, ALT, albumin, INR, blood glucose, and ammonia levels
in neonate with direct hyperbilirubinemia, what test will confirm elevated Alk Phos indicates obstruction as responsible for cholestasis?
gamma glutamyltransferase (GGT)
Fairly common inherited cause of direct hyperbilirubinemia in a neonate that is also associated with lung disease and diarrhea.
Alpha 1 antitrypsin deficiency
Rotor syndrome, Dubin-Johnson, Gilberts, and Crigler-Najjar syndrome: which hereditary cause of hyperbilirubinemia is NOT benign?
Crigler-Najjar (direct hyperbilirubinemia which predisposes to kernicteris)
Jaundice neonate who is febrile. Immediate work-up?
full sepsis work-up: blood cxs and LP
What mnemonic to remember for infections that may cause neonatal jaundice.
TORCH
Normally, when does jaundice present (physiologic)?
after 72 hours
Unconjugated bilirubin over what level is considered pathologic in a neonate?
15 mg/dL
If jaundice lasts longer than this, needs monitoring or further work-up.
1 week (2 weeks if preterm)
Treatment if suspecting breast-milk juandice.
increase frequency of feeds (hydration)
What non-rare mutation more common in caucasions can also cause neonatal hyperbilirubinemia?
CF (do a sweat test)
What to test in mom (as well as in baby) when neonate has pathologic jaundice.
blood type and coombs test
Tracheoesophageal fistula presentation.
aspiration on first feed as well as copious salivation
Treatment for Tracheoesophageal fistula.
Surgery
Air in GI tract on AXR; how to confirm tracheoesophageal fistula?
bronchoscopy
Congenital diaphragmatic hernias usually (90%) on this side.
Left
Presentation of congenital diaphragmatic hernia.
respiratory distress
U/S in utero suggests congenital diaphragmatic hernia. What confirmatory test?
post-natal CXR
Gastroschisis vs. Omphalocele: anatomic location of hernia
Gastroschisis: NEXT TO umbilicus (no sac)
Omphalocele: AT the umbilicus (into amniotic sac)
Gastroschisis vs. Omphalocele: which one requires emergent surgery?
Gastroschisis (no sac, so peritoneum exposed)
C-Section can prevent Omphalocele sac rupture. What to do if it does rupture?
emergent surgery
Double Bubble on AXR. Diagnosis?
Dudodenal Atresia
Triple Bubble on AXR. Diagnosis?
Jejunal Atresia
Hyperbilirubinemia in Neonate above 20. Treatment?
exchange transfusion
Hyperbilirubinemia in Preterm Neonate (above 10). Treatment?
phototherapy
Ground Glass appearnace of CXR. Diagnosis?
Respiratory Distress Syndrome
What causes Respiratory Distress Syndrome
surfactant deficiency
Respiratory Rate over 60/min with hypoxemia and increased respiratory effort. Diagnosis?
Respiratory Distress Syndrome
CXR findings in Transient Tachypnea of the Newborn?
prominent perihilar streaking
Sign of probable Meconium Aspiration
green amniotic fluid
Newborn with patchy infiltrates on CXR, neutropenia. Suspected diagnosis?
pneumonia
Mom's at risk for baby with RDS: what prophylaxis to mom if under thirty weeks?
corticosteroids
If over 30 weeks gestation, how to tell if baby needs mom to get corticosteroid prophylaxis for RDS?
L/S ratio less than 2:1 indicates need
Most common presenting sign of Cerebral Palsy.
Delayed motor development
Most common movement disorder in children.
Cerebral Palsy (CP)
Intrauterine infection, perinatal asphyxia, and prenatal trauma all are risk factors for nperinatal neurologic insult which can result in this.
Cerebral Palsy (CP)
Common neuro co-morbidity with CP.
Seizure Disorder
What percent of CP patients have normal cognition?
10%
Toe walking and scissor stepping is called what?
spastic gait
Imaging of choice in older children to diagnose CP.
MRI
Treatment for spasticity of CP.
baclofen etc.
Typical age in Febrile seizures.
6 months to 5 years
Work-up for Febrile seizures under 12 months.
sepsis work up (find infection)
Work-up for Febrile seizures over 18 months
none needed if not concerned about CNS infection
Why avoid Aspirin in children?
Reye's syndrome
Work-up for Febrile seizures if longer than 15 minuts.
EEG and MRI
When to treat a Febrile seizure (diazepam or phenobarb).
if complex (more than 15 minutes, focal, or more than 1 in 24 hours)
What is the increase risk of Epilepsy disorder after a Febrile seizure?
no increased risk
Most common childhood malignancy.
ALL
Top 3 childhood malignancies
1) ALL
2) CNS
3) Lymphomas
AML is more common than ALL in this cohort of children.
Black
Most patients with neuroblastoma are under this age.
under 2 years old
Nontender abdominal mass in child under 2. Diagnosis.
Nueroblastoma
Nontender abdominal mass in 2-5 year old.
Wilm's Tumor
WAGR Syndrome. W is for Wilm's Tumor. What is A for?
Aniridia (no iris)
Beckwith-Wiedemann syndrome has hemihypertrophy (one side of body is bigger than the other). What is the associated cancer?
Wilm's Tumor
Why get a CXR when suspecting Leukemia?
rule out mediastinal mass
Peripheral smear characteristic in Leukemia.
more than 90% lymphoblasts
What does immunophenotyping of a BM aspirate do in work-up for leukemia?
finds the exact type of cell that has undergone monoclonal expansion
Chemo for Leukemia often has this initial complication.
tumor lysis syndrome
what is tumor lysis syndrome (3 things) (caused by chemo)
hyperkalemia, hyperphosphatemia, hyperuricemia
How to treat tumor lysis syndrome
allopurinal, urine alkalinization, diruetics and fluids, reduced phosphate intake
What is chloroma (greenish soft-tissue tumor on skin or spinal cord) associated with?
AML
Treatment for neuroblastoma.
resection then chemo or radiation
Why chemo/radiation after resection of neuroblastoma.
most patients have metastases at presentation
How to assess kidney damage in Wilm's tumor.
regular way: BUN, Crt, UA
Why do a CT chest and abdomen in patient with Wilm's tumor?
check for metastases
Treatment of Wilm's tumor
resection (plus nephrectomy) then chemo and radiation
Ewing sarcoma and Osteosarcoma: systemic symptoms?
Ewins: yes (fever, fatigue)
Osteosarcoma: rare
Ewing sarcoma and Osteosarcoma: which one more commonly has metastases?
Osteosarcoma: metastes to lung in 20% (profoundly worse prognosis)
2 childhood bone tumors which are important to differentiate.
Ewing's and Osteosarcoma
Child bone tumor presentation.
local pain and swelling
Ewing sarcoma and Osteosarcoma: location?
Ewing's: midshaft of longbones
Osteosarcoma: metaphysis (near knee or shoulder)
Onion skin periosteal reaction on plain xray. What bone tumor?
Ewing's Sarcoma
Sunburst lytic bone lesion. What bone tumor?
Osteosarcoma
Treatment differences between Ewing Sarcoma and Osteosarcoma
Ewing's responds to radiation; both need resection and chemo
Most common cohort to get bone cancer in childhood.
Male adolescents
Most common primary childhood bone tumor.
Osteosarcoma
Water heater anticipatory guidance.
keep temperature of water under 48.8 C or 120 F
When can a child face forward in car seat?
current guidelines say at 1 year and over 20 lb (some say 2 years)
When to introduce solid foods (age)?
6 months or more (introduce no more than one per week)
When to introduce cow milk (age)?
12 months or more
Syrup of Ipecac recommendations.
not recommended anymore for poisoning; just call poison control
Leukoria (lack of red reflex). Top 3 ddx.
retinoblastoma, congenital cataracts, retinopathy of prematurity
Strabismus can be considered normal until what age?
3 months
Treatment of Strabismus.
cover the normal eye
Why treat Strabismus?
to avoid Amblyopia (permanently suppressed vision in a mis-aligned eye)
Tested false contraindications to vaccinations (3)
mild illness, current antiobiotics, premature
Vaccine schedule in a premature infant.
give as normal and NOT according to adjusted age for gestation
Egg allergy is a contraindication to these two vaccinations.
MMR and Influenza
3 live vaccines
MMR, Polio, Varicella
Avoid live vaccines in what 2 patient types?
Immunocompromised or pregnant
Seizure after DTaP. Which one is the culprit, and should be avoided in follow up vaccinations?
Pertussis; give Dyptheria and Tetanus by themselves later
Routine screening ages (2) for Lead poisoning (for high risk areas.)
12 and 24 months
Homes built before this year considered at high risk for lead-exposure
1950
Neuropathy of lead poisoning.
Wrist or foot drop
Lead level 45-70: treatment?
chelation with inpatient EDTA or outpatient oral succimer (DMSA)
Lead level over 70: treatement?
chelation with inpatient EDTA and intramuscular dimercaprol (BAL)
Lead level under 45: treatment?
enviornmental changes; retest in 3 months
CBC and peripheral smear results for Lead Poisoning.
microcytic anemia with basophilic stippling