Pretest Questions Flashcards
HSP also known as?
anaphylactoid purpura
treatment for retentive encopresis
clear fecal impaction and use lots of stool softeners
unilateral bowing of leg past 2 years of age =
blount disease aka idiopathic tibia vera. Abnormality in medial aspect of tibia
What is legg-Calve perthes disease
avascular necrosis of the femoral head
painless limp think
legg-calve perthes disease
overweight patient with a limp think
slipped capital femoral epiphysis
What are two types of breath holding spells
cyanotic - kid misbehaves and wants something
pallid - painful experience, kid stops breahting and passes out
bedwetting and fat think
diabetes
bitter almond odor think
poisoning w/ hydrogen cyanide
treatment for hydrogen cyanide poisoning
high level oxygen, and hydroxocobalamin antidote
headache, agitated and confused, works with plastics, gets dysrhythmia
cyanide poisoning
rash that peaks at 2-4 weeks of life and is harmless
neonatal acne from mothers hormones
white keratin plugs on nose, cheeks, chin
milia - benign
treatment for scabies
permethrin cream
how is scabies different in kids and adults
in kids it can occur on palms, soles,face, and head where as it is normally spared in adults
pruritic papular rash that is in many family members
scabies
diffuse infiltrates in newborn bilaterally, rapid onset, low WBC, left shift
group B strep
Most common way that kids get orbital cellulitis
direct extension from nearby site like paranasal sinuses
Common causes of orbital celluliits
MRSA, strep, Haemophilus
orbital vs periorbital cellulitis
orbital has restriction of eye movement,. Periorbital can be managed as outpatient with oral antbioitcs
general direction of motor development in kids?
cephalocaudal, trunk then arms then fingers
How do you test for HIV in neonate and why?
use an antigen test like HIV PCR. Can’t do ELISA bc it will reflect maternal antibodies
Where is eczema located when younger than 2
cheeks, wrists, scalp, arms and legs.Afterwards goes to the extensor surfaces
Unique contraindication to DTaP
encephalopathy - coma, AMS, prolonged seizure within 7 days of administration of last dose
What is the problem with 1% lidane?
It can cause neurotoxicity from transdermal absorption. Especially when under 2 months?
3 weeks of progressive cognitive decline, listlessness, vomiting think
tuberculous meningitis
whitish polyp extending through TM in patient with recurrent OM
cholesteatoma - from recurrent infection or chronically draining ear
Once a child has low blood pressure how what percent dehydration has occurred?
10-15%
umbilical cord intact after 1 month think?
leukocyte adhesion deficiency type 1 - CBC with marked leukocytosis
How do you diagnose leukocyte adhesion deficiency type 1
measure surface CD11b with flow cytometry.
What can form after umbilical cord has separated
umbilical granulomas
how do you treat umbilical granulomas
silver nitrate
Most common cause of septic arthritis is?
S Aureus
Td 10 years ago what do you give now?
Tdap to help reduce pertussis
Mammalian bite how do you care for it?
Lots of irrigation. Unsure whether to do antibiotics. Definitely if monkey, less so if cat or dog
deferoxamine treats?
iron overdose
N acetylcysteine treats
acetaminophen ingestion
Dimercaptosuccinic acid treats
arsenic poisoning
CroFab treats
western diamond back rattlesnake
developmental delay, autism, abnormal temperament test for?
fragile X syndrome
long face, large ears, prominent jaw, macroorchidism, hypotonia, repetitive speech, hand flapping =
fragile X syndrome
What are normal transmitting males
guys with fragile X but they are normal and it doesn’t show up on karyotypes. Pass it on to all daughters and then also to those grandchildren.
Genetics of fragile X syndrome
Dominant X-linked disorder with reduced penetrance
rocker bottom feet think
trisomy 18
erythematous macules with central yellow-white pustule,. What does fluid from lesions show?
This is erythema toxicum benign and found in newborns. Will show eosinophils
rash on neck and sometimes eyelid that gets worse with emotion and activity.
salmon patches or nevus simplex/nevus flammeus. The ones on face go away ones on nuchal and occcipital areas persist.
african america, present at birth, small pustules with hyperpigmented lesions encircled by a collarette of scale when u rupture the pustule
pustular melanosis- pustules first few days and then hyperpigmentation for weeks to months
Scalp area without hair and scaly yellow-orange lesions
sebaceous nevi. They can become malignant in adolescence
What are epstein pearls
epithelial cysts on the palage associated with milia
nonerythematous fine yellowish-white lesions on face of neonate think
milia
erythematous, ulcerative lesions on his fingertips. Painful and itchy - was in the snow all day
chillbain
girl with erythematous, firm, slightly swolen skin at edge of mouth extending to cheek in the summer
popsicle paniniculitis - destruction of fat cells by exposure to cold weather.
small, firm, white cold patches of skin after cold =
frostnip
cold exposure then stinging in tisseu followed by aching and then numb .Area becomes red, blotchy, and painful when you warm it back up
frostbite
scaling of lips with fissures at corners, glossitis, ocular problems, seborrheic dermatitis, poor growth, burning and itching of eyes =
riboflavin deficiency
seizure, peripheral neuritis, dermatitis, hypochromic anemia =
B6 deficiency
thiamine deficiency think besides korsakoff or wernicke
beriberi
NIacine deficiency think
dermatitis, diarrhea, dementia, death
Biotin deficiency think
dermatitis/seborrhea
High pitched cry, small for age, microcephalic
Cri du chat syndrome - abnormal larynx. delete short arm of 5
Deleted segment in angelman and prader willi
15q11-13
painful limp, WBC count and ESR are normal. Ruled out osteo and septic hip
transient synovitis
progressive neuro symptoms in kid in new house being redone think what and treat with what?
Lead poisoning, if symptomatic give dimeraprol and calcium EDTA assuming its severe. One or the other in a more mild form
When is N acetylcysteine ineffective?
after 36 hours of acetaminophen overdose
aspirin overdose what will help?
sodium bicarb helps in excretion
organophosphate poisoning treat with
washing, emesis, atropine followed by pralidoxime (2-PAM)
Megaloblastic anemia not from deficiency think?
infection with tapework diphyllobothrium latum - longest tapeworm. Treat with Praziquantel
Mother on lithium - kid gets
Ebstein anomaly - heart defect where tricuspid valve is in the right ventricle with attachment to wall instead of annulus
mother on ACE i - baby gets
renal sygenesis, oligohydramnios, skull ossification
mother on isoretinoin baby gets
teratogenic effects = hydrocephalus, CNS porb, microtia, smallor missing thymus, conotruncal heart defect, micrognathia
low leafy greens and stuff in mom, baby gets
neural tube defects like spina bifida
mom gets 3 day measles, baby gets
(mom got rubella) deafness, cataracts, mental retardation, heart defects
Guy at the beach gets intensely ithy lesion on feet that progresses up- raised, red, serpiginous.
called creeping eruption or cutaneous larva migrans. from dog and cat hookworms. Treat with ivermectin
what is ivermectin
anti-helminth medication
treatment for seborrheic dermatitis
topical steroids or selenium-sulfide compounds
people out at barbecue and they get photosensitive rash
can happen from lime juice
to cause heart block, SLE mothers must have antibodies to?
SSA/RO or SSB/La
Isoniazid therapy needs what vitamin
pyridoxine or B6 supplements
kids on pheytoin need?
folate supplementation
Kids with measles in developing countries need what vitamin
Vitamin A
Baby who is breast fed needs vitamin
D
Patients with sickle cell disease need supplementation wtih
folate
1 hour old newborn may need what supplement
Vitamin K to prevent hemorrhagic disease
Kid with repeated abscesses and a liver abscess think
chronic granulomatous disease - use NBT to assess
Kid with severe eczema and unusual infections think of?
Wiskott-Aldrich syndrome
Wha tis an easy way to rule out Wiskott aldrich syndrome in kid with severe eczema and unusual infection
check platelets. Wiskott-Aldrich syndrome unlikely if platelets are normal
How can you test for T cell deficiencies,
You could do an intradermal skin test using candida albicans, Won’t get response
Kid with repeat meningitis from neisseria and then strep worry about?
asplenia - will see howell-Jolly bodies
children of diabetic mothers have what GI problem
small left colon
Baby becomes hypoxic when feeding but is ok when crying. What is happening
choanal atresia - need to stick an NG tube down
What is the CHARGE association with choanal atresia?
coloboma, heart disease, atresia of choanae, retarded grwoth, genital hypoplasia, ear abnormalities
How do you manage baby with mother who has VZV close to birth
VZV 5 days before delivery or within 2 days after need to give VZIG and isolation. If after 2 days you don’t need to do anything
True contraindications to breastfeeding
active pulmonary TB, HIV, antineoplastic agents, malaria, typhoid fever, septicemia
What happens to second kid of a mother who had CMV during pregnancy
protected by mother IgG
What trimester is worst to get CMV infection
first
What is the apt-downey test and what is it used for?
Its a test that differentiates fetal from adult hemoglobin in bloody specimen since fetal hgb is alkali resistant. Used when baby has some blood stools bc if mothers blood then don’t need to do anything
Safe medicine for breastfeeding
safe medicine for breastfeeding
Infants born to diabetic mothers have sugars that are initially?
hypoglycemic
What electrolyte abnormalities occur in neonates of diabetic mothers at 2-4 days?
Hypocalcemia and hypomagnesemia bc delayed PTH synthesis
Which is more severe Rh incompatibility or major blood group incompatibility
Rh is more severe
how is direct coombs test different in Rh and ABO incompatibility
in Rh its strongly positive. In ABO it can be weakly positive or not at all
Describe why babies get cold easily and how they respond
Going to colder environment, wet, with large surface area, no fat. To bring up temp start increasing metabolic rate, ventilate faster, they DON”T sweat, and often build up lactic acid
In premature infants what is the cause of apnea
icnompletely developed respiratory center
What defines an apneic episode
absence of respirations for more than 20 seconds
Mass on the right neck at 1 week of age
borken clavicle that is only now apparent with callus formation
Erbe-Duchene paralysis which nerves are affected and how do they present
cervical 5,6. Present with pronated forearm that is extended. With arm that is internally rotated and at side. CAN”T abduct or externally rotate at shoulder
Klumpke paralaysis which nerves are affected and presentation
cervical 7,8, T1. palsy of hand and can produce horner syndrome.
When there is paralysis of upper extremity from brachial plexus also worry about?
phrenic nerve damage. Will see elevated diaphragm on CXR
newborn with enlarging head and fluid wave in the skull worry about
subgaleal or subaponeurotic hemorrhage - need to observe in ICU.
LIke a cephalohematoma that crosses midline
Elevated hematocrit in premature baby can cause?
seizure from hyperviscosity syndrome
Complication of erythromycin in newborn?
increased incidence of hypertrophic pyloric stenosis
What do you give to infants of HIV mothers
Ziduvodine
Two things always given to neonates at birth
Hep B vaccine and Vitamin K supplements
congenital cystic adenomatoid malformation vs diaphragmatic hernia
in diaphragmatic hernia the ng tube will go into chest, whereas normal location in CCAM
Baby born to Hep B ag positive mother what do you do
give vaccine and immunoglobulins
Why are beta blockers dangerous at pregnancy
When throughout pregnancy cause growth retardation
but near birth they prevent baby from increasing heart rate if asphyxiated. Can also get hypoglycemia and apnea
If there is bilious fluid in a neonates vomit you know that the GI anomaly is not?
Esophageal atresia or pyloric stenosis
Cleft lip and palate predisposes you to?
recurrent otitis media and hearing loss and speech deficits
How do you make prenatal diagnosis of neural tube defects?
detection of elevated alpha feto-protein in the amniotic fluid
CXR in newborn showing pulmonary gascular markings with fluid in fissures and hyperexpansion of lungs - has difficulty breathing and grunting. Csection
transient tachypnea of the newborn
How do you evaluate a fifth-finger polydactyly in whites and blacks
In black its common and familial
in whites need to do a cardiac exam.
thumb sided polydactyly is always bad!
What is harlequin syndrome?
transient change in skin color of asymptomatic newborn. dependent side turns red and upper side remains pale
Two wtins have hematorcrit that differ by more than 15 what happened/
twin-to-twin transfusions that happens in monochorionic
What happens to donor in twin-to-twin transfusions? recepient?
donor gets oligohydramnios, anemia, hypovolemia
recipient gets polyhydraminios, larger, hyperviscosity syndrome if hematocrit goes over 65%
What do you do with natal tooth that is loose
have to remove it because worried about aspiration
longitduinal striations in the metaphysis charactersitic of
congenital rubella
osteochondritis or periostitis indicates
congenital syphilis
Triad of toxo congenital infection
hydrocephalus, chorioretinitis, intracranial calcifications
Which is most common TORCH infection
CMV
Cardiac defects make which TORCH infection more likely?
congenital rubella
Stable pneumothorax in a neonate can be treated with?
100% oxygen to cause a “nitrogen washout”
widely spaced eyes, low set ears, broad nose, bilateral renal agenesis and you get?
Potter sequence with pulmonary hypoplasia
What proteins do you care about in maple urine syrup disease
leucine, isoleucine, valine
hemihypertrophy and aniridia think
Wilms tumor
lateral displacement of medial canthi, broad nasal bridge, single eyebrow, partial albinism (white forelock or heterochromia, deafness
Waardenburg syndrome - AD inheritance
flat vascular malformation in distribution of Cn III think
What else do you get?
Sturge-Webber syndrome
malformation involves meninges, get atrophy of cerebral cortex, seizures, mental deficiency, hemiparesis, hemianopsia
tuberous sclerosis - can get them in brain and also have seizures
What brain abnormalitiy do you get with shaken baby syndrome?
subdural hematoma
What is caput succedaneum
soft-tissue swellin go fscalp where the baby was delivered. Can be ecchymotic and cross sutures. Resolves in first few days
preterm infant with enlarging head and lethargy worry about?
intraventricular hemorrhage aka germinal matrix hemorrhage. - in gelatinous subependymal germinal matrix
cephalohematomas vs caput succedaneum
cephalohematomas never cross the suture line.
Treatment for tricyclic antidepressant overdose when you have prolonged QT =
sodium bicarbonate to alkanize the blood.
Jones major criteria for ARF
carditis, polyarthritis, erythema marginatum, chorea, subcutaneous nodules
What one symptoms is enough to make diagnosis of rheumatic fever
Syndenham chorea
What is most common symptom of acute rheumatic fever
polyarthritis
Medical treatment for tet spells
propranolol and morphine to calm kid down and improve blood flow into the lungs
What do you do with child with FH of early heart disease
Test for lipid levels after 2 years of age to see if there are any lipid lowering therapy that you will need to do
swelling in feet and hands for a few days in kid with other family members with similar problem =
Hereditary angioedema - AD
Can get abdominal pain, vomiting, diarrhea if the edema happens in the GI tract. Can also get in larynx and cause problems breathing
What is wrong in hereditary angioedema
deficiency of C1-esterase inhibitor - get excessive vasoactive kinin
Common cardiac defect in turner’s syndrome
coarctation of the aorta
Risk in kid with another child in family with congenital heart disease?
2-6%
right ventricular systolic lift. Fixed S2 think
atrial septal defect
conjunctivitis in someone with rash, arthritis, and other constitutional symptoms think
Kawasaki’s disease - conjunctivitis is more unique to Kawasaki than ARF or other things
Treatment for Kawasaki disease
IVIG and high dose aspirin
aspirin stays until the thrombocytosis comes down
Most common causes of myocarditis in babies
coxsackievirus B and adenovirus
3 groups most at risk of endocarditis
unrepaired cyanotic heart disease
prosthetic material
prior endocarditis
Organisms that cause endocarditis in children
Most are strep viridians and staph aureus
also think about HACEK group, strep pneumo, coag neg staph,
first line treatment for SVT in infant
carotid massage, face in cold water, voluntary straining
Verapamil or adenosine for SVT treatment in infant
Can’t use verapamil bc causes hypotension cardiac arrest
quadruple heart sounds with right atrial enlargement, right conduction defect with systolic murmur and diastolic rumble =
ebstein’s anomaly
girl becomes pale while running and then returns to normal after 30 minutes. No evidence of structural heart disease
WPW
spiking fevers, spindle shaped swelling of fingers and joints. Salmon colored rash that develops with fever
Systemic juvenile idiopathic arthritis
Cyanotic newborn with LVH and LAD. holosystolic murmur
tricuspid atresia - have hypoplastic right ventricle so shows LVH
Transposition of the great arteries
Transposition of the great vessels with an intact ventricular septum presents with early cyanosis, a normal-sized heart (classic “egg on a string”
radiographic pattern in one-third of cases), normal or slightly increased pulmonary vascular markings, and an ECG showing right-axis deviation and
right ventricular hypertrophy. In tetralogy of Fallot, cyanosis is often not seen in the first few days of life. Tricuspid atresia, a cause of early
cyanosis, causes diminished pulmonary arterial blood flow; the pulmonary fields on x-ray demonstrate a diminution of pulmonary vascularity, and
left-axis and LVH are shown by ECG. Total anomalous pulmonary venous return below the diaphragm is associated with obstruction to pulmonary
venous return and a classic radiographic finding of marked, fluffy-appearing venous congestion (“snowman”). In pulmonic atresia with an intact
ventricular septum, cyanosis appears early, the lung markings are normal to diminished, and the heart is large.
Cyanotic patient hours after birth and not responding to oxygen. Give them?
prostaglandin E1
Thrombocytopenia-absent radius has what heart defects
TOF and ASD
Male with turner syndrome like stuff has what disease and what cardiac symptoms
Noonan syndrome think right sided heart stuff like pulmonic stenosis
Ehlers-Danlos syndrome associated with what heart defect
Mitral valve prolapse is seen in patients with Ehlers-Danlos syndrome. Patients with Ehlers-Danlos syndrome have hyperextensibility and easy
bruising, joint hypermobility (leading to joint dislocations and sprains), skin that is velvety to touch, and tissue fragility. Six different kinds are
described with “classic” and “hypermobility” forms predominating.
Adolescent with syncopal episodes and deafness
QT elongation and deafness = Jervell-Lange-Nielsen syndrome
child with difficulty breathing, right arm palsy, but normal CXR. What do you do next
Do a chest ultrasound to look for hemidiaphragm paralysis
1 year old runny nose and sneezing then with worsening cough and wheezing on exam? What is it and what to do
Bronchiolitis - monitor oxygen and fluid status. Most things don’t really help
ingestion of gasoline, kerosene, or furniture polish, what do you do?
symptomatic management. Don’t want NG, charcoal etc
At what age does a PPD become effective
3 months. Before that treat a child empirically if you are worried about exposures.
Repeat pneumonia with hemoptysis. Rapid resolution each time. think about?
Idiopathic pulmonary hemosiderosis
What are typical findings in idiopathic pulmonary hemosiderosis
recurrent infections w hemoptysis. Microcytic, hypochromic anemia, low serum iron levels, occult blood in stool (swallowing pulmonary secretions)
How do you diagnoise idiopathic pulmonary hemosiderosis
bronchalveolar lavage showing hemisoderin laden macrophages.
Some people with pulmonary hemosiderosis have what other hypersensitivity
hypersensitivity to cows milk. called Heiner syndrome.
Staph pneumonia with acutely worsening respiratory symptoms worry about?
Tension pneumothorax. Need large bore needle and chest tube aspiration kit
Biphasic stridor following a few days of URI. Looks toxic and has difficulty breathing. Normal epiglottis
bacterial tracheitis
Kid who eats a lot of dirt with first episode of pneumonia. Found to have a lot of eos
Infection from dog parasite toxocara canis. Need to test with ELISA bc the larvae go from gut to organs but don’t return to gut
Pus in middle meatus suggests what source?
Superior meatus
Middle meatus - maxillary, frontal, anterior ethmoid
Superior meatus - sphenoid or posterior ethmoid
How do sinuses change over time in terms of infectio
From birth maxillary and ethmoid can have infection
sphenoid large enough at 3-5 years
frontal large enough at 6-10 years
Which organism causing pneumonia can also cause a number other upper respiratory infections?
Mycoplasma pneumonia
reversed heart, chronic sinuopulmonary infections
Kartagener syndrome - have primary ciliary dyskinesia
When and what causes congenital cystic adenomatoid malformation
embryonic disruption before 35th day - improper bronchiole development
Fluctuant mass on the posterior wall of pharynx after few days of low grade fever and sore throat
retropharyngeal abscess
2-12 week old who had conjunctivitis earlier now with afebrile pneumonia think
chlamydia trachomatis
Young child with decreased breath sounds on one side where you hear normal inspiration but don’t hear expiration
Think of foreign body causing air trapping
stool filled rectal vault with poor anal sphincter tone
functional constipation
Hirschrung disease would have normal or excess sphincter tone
systemic signs of eosinophilic esophagitis
peripheral eosinophilia or elevated IgE levels
will see food allergies and eczema etc.
When do children start getting intussuception?
After 6 months of age
echogenic bowel on prenatal ultrasound hints at
CF meconium ileus developing
What is sandifer syndrome
Bending or arching of neck caused by reflux - can be confused for infantile spasm
Triad of crohns disease
diarrhea, abdominal pain, weight loss
What do you do with a child who regurgitates food and then swallows some of it agtain
behavioral modification. its rumination and related to an attention seeking behavior
How do you diagnose meckel’s diverticulum
technetium 99m
diagnostic test for lactose intolerance
breath hydrogen or acidic stool pH in the presence of a reducing substance
When do you consider it to be direct obstructive jaundice?
When dbili is more than 20% of the increase
When can ABO and Rh incompatibility lead to direct hyperbilirubinemia
if there is significant hemolysis at birth that causes inspissated bile syndrome
How is hypertonic dehydration different in presentation
skin can be doughy and you get less tachycardia and hypotension bc the high sodium means a lot of the fluid loss is from intracellular compartment rather than the extracellular compartment
Maxillary frontal teeth decay but normal mandibular frontal teeth
bottle feeding esp with things with high concentration of sugar
If you break your teeth how should you transport them?
In saliva or milk
Management after ingestion of drain cleaner
Don’t try to vomit bc re-expose esophagus again. Just do endoscopic exam in 12-24 hours
What are some drugs that charcoal is actually useful for
pnenobarbital and tricyclic antidepressants. sustained release theophylline
How do you diagnose EHEC?
Need special sorbitol-MacConkey agar to isolate. Or you can now look for shiga toxin
What laboratory test can show vitamin C deficiency
plasma ascorbate level <.2mg/dl
CXR with generalized bone atrophy and epiphyseal separation?
Vitamin C deficiency
how do you treat labial adhesions?
can just observe and wait for estrogen to kick in during puberty or put topical estrogen cream. Then need petroleum after separation to ensure it doesn’t stick together again
gray and bloody vaginal discharge since birth with normal exam?
physiologic trauma related to estrogen withdrawal
lax, wrinkled abdominal wall, dilated urinary tract, inta-abdominal testicular tissuse. Oligohydramnios, pulmonary problems, other renal problems. Could have club feet
prune belly syndrome
get lack of abdominal wall muscles, cryptorchidism, and urinary tract abnormalities
Baby who is always thirsty and peeing all the time
congenital nephrogenic diabetes insipidus
Review fanconi syndrome
Fanconi
syndrome can be hereditary or acquired; hereditary forms are usually secondary to a genetic abnormality such as cystinosis, galactosemia,
Wilson disease, and some mitochondrial abnormalities. A number of agents can cause Fanconi syndrome, including gentamicin (or other
aminoglyco-sides), outdated tetracycline, cephalothin, cidofovir, valproic acid, strepto-zocin, 6-mercaptopurine, azathioprine, cisplatin, ifosfamide,
heavy metals (eg, lead, mercury, cadmium, uranium, platinum), paraquat, maleic acid, and toluene (from sniffing glue). The mechanism of action
of these agents is through acute tubular necrosis, alteration of renal blood flow, intratubu-lar obstruction, or allergic reactions within the kidney
itself. Many of these toxic effects are reduced or eliminated with removal of the offending agent.
Asian boy with pain radiating from his abdomen down to his groin
urolithiasis
large bladder, large kidneys, weak urinary stream in a male
posterior urethral valve - an obstructing membrane in the posterior urethra
what is a pyelonephritis that is taking longer to respond to antibiotics and a wedge shaped focal enlargement of one lobe that has minimal contrast enhancement
acute lobar nephronia - need to do prolonged IV and PO antibiotics
On CT has no cortical rim sign just an area of decreased blood flow
Fixing an undescended testis only helps with what potential complication
can help prevent testicular torsion through orchiopexy
3 day old infant with blood found in diaper, new abdominal mass. Baby was large for gestational age when born
renal vein thrombosis - kid could also be dehydrated and have polycythemia making it more likely
how are rheumatic strains and nephritogenic strains of strep different?
Rheumatic disease only comes from group A strep of pharynx
post strep nephritis can come from pharyngeal or skin infection (impetigo)
coombs test in HUS?
negative
Epididymitis is different than testicular torsion how?
Normal cremasteric reflex, pain reduces when you elevated testicle, posterior pain
rash on buttocks and extensor surfaces, with renal failure, and abdominal pain - not on face, soles, trunk, and palms
HSP
HSP is what type of disease
An IgA mediated systemic vasculitis
Unilateral lymphadenopahty is part of what disease?
Kawasaki’s disease
Initial management of varicocele
Education and wait
Management for hydrocele found in first year of life
Just observe
Asymptomatic microscopic hematuria - that becomes gross hematuria after respiratory infections is what? and what else is it associated with
Alport syndrome - also get ocular abnormalities and hearing abnormalities
What is inheritance of Alport syndrome?
X linked dominant - so guys get it worse
Idiopathic hypercalciuria initially presents as?
hematuria, often persistent. dysuria, abdominal pain, don’t always get stones formed early
What do you have to worry about with acute glomerulonephritis?
Get oliguria (looking like dark,cola urine). Need to sometimes restrict fluids to make sure you don’t get volume overload
Where do you normally get edema in nephrotic syndrome
periorbital and scrotal in boys
Berger nephropathy associated with?
HSP
prevention/treatment for malignant hyperthermia is
dantrolene
How to identify patients at risk for malignant hyperthermia?
caffeine contracture test
Headache, seizure, atazia, fatigue 2 weeks after infection with multiple white lesions on MRI think
ADEM - acute disseminated encephalomyelitis
What do you use instead of edrophonium to test for MS in young kids
neostigmine. Avoid bc can cause cardiac arrhythmias in kids
hemisyndrome with eyes looking away from the paralyzed side makes you think of?
infantile hemiplegia
developmental deterioration, macular cherry-red spots, sensitivity to sound is what disease and what mutation
Absence of Beta hexosaminidase A activity in WBCs and is Tay Sach’s disease
Fabry disease - absence of alpha galactosidase
myotonic muscular dystrophy is different than most other muscular dystrophies in that it?
affects distal muscles more than proximal
What is common problem that occurs alongside myotonic muscular dystrophy?
cataracts
Tuberculous meningitis findings on CT scan with contrast
exudate on basal cisterns that enhances
tuberous sclerosis sometimes treated with?
rapamycin
cranial nerve abnormalities, long-tract signs, unsteady gate and behavioral changes
pontine glioma
Patient with tetrology of fallot and an infection suddenly has neuro symptoms why?
He has a brain abscess bc tetralogy of fallot allows for right to left shunts
unilateral pupillary defect, hemiplegia, seizures after a fall worry about
an epidural hematoma causing downward herniation of temporal lobe compressing ipsilateral CN III and brainstem
triplet repeat expansion on chromosome 9
Friederich’s Ataxia
What is more common infratentorial or supratentorial tumors in kids
Until 1 year of life supratentorial more common
1-10 get more infratentorial tumors
>10 supratentorial tumors become more common again
constipation is presenting first symptom of?
Botulism
CNS degeneration, liver/spleen elargement, macroglossia, coares facial features, cherry-red spot on eye at birth
GM1 gangliosidosis type 1 with complete absence of acid Beta galactosidase activity
Normal children at birth with rapid decline in motor and cognitive function, btwn 16-18 mo. In girls lose control of their hands and get social withdrawal
Rett’s syndrome
How long do febrile seizures normally last?
Only 2-5 minutes. Longer and you should be worried
peroneal and intrinsic foot muscle atrophy, then intrinsic hand muscle atrophy, then legs?
Charcot-Marie-Tooth disease
Periventricular calcifications classic for
congenital CMV infection
scattered and soft appearing calcifications in cortex classic for?
congenital toxo infection
achromic skin patches and infantile spasms
tuberous sclerosis
decreased fetal movement, general hypotonia and arefelxia with fasciculations
degeneration of anterior horn cells in SMA type 1
Normally you have selective degeneration bc functional gene
When does duchenne’s muscular dystrophy usually start to present itself?
Kid reaches normal milestones but starts showing the weakness around 2 years. by 7-12 years in a wheel chair
Syndeham chorea triggered by?
An infection with Group A beta hemolytic strep
unilateral facial lesion and blindness suggests
Sturge-Webber syndrome or PHACE syndrome
What does PHACE syndrome involve?
posterior fossa malform, hemangioma, arterial anomalies, coarctation of the aorta, cardiac defects, eye abnormality
large, irregular unilateral cafe-au lait spots and fibrous dysplasia of bones in precocious puberty in girls, goiter
McCune Albright syndrome
coryza, conjunctivitis, widespread maculopapular rash with white pinpoint lesions on mucosa
measles
maculopapular rash for 3 days, enlargement of posterior cervical and occipital lymph nodes
german measles or rubella
sore throat that progresses to adherent membrane covering tonsils, uvula, palate, posterior oropharynx etc.
Diphtheria
What do you do in infant born to HIV mother before confirm HIV status
PCP prophylaxis
aspirin + viral infection =
Reye syndrome an acquired mitochondrial hepatopathy
Common cause of death in Reye’s syndrome
cerebral edema
Seizures and bloody diarrhea
Shigella
Kawasaki disease treatment?
apsirin gama globulin
What cause of tinea capitis fluoresces under wood lamp
microsporum canis
Exposure to animals fever, rash, meningismus
leptospirosis - most are mild
Age range for kawasaki’s disease
under 5 years
Any general infection like otitis media with a new change in AMS in young infant
LP to check for meningitis
Newborn with sibling who developed pertussis
Give newborn erythromycin
CBC findings in EBV mononucleosis
predominance of lymphocytes with at least 10% being atypical
How long does it take to get finding on Xray for osteomyelitis
Can take days to get soft tissue changes and up to 12 days to see actual changes in bone
16 day old infant with meningitis from gram positive rod
Late onset Listeria monocytogenes
well appearing infant with sudden elevated fever and and left shift in WBC and markedly elevated WBC
pneumococcal bacteremia - can also consider H flu and meningococcus
When does rubella really harm the fetus?
at the first 2 months
What is dacrocystitis
infection of nasolacrimal sac
posterior and occipital lymphadenopathy with 3 days of fever
rubella
What is a possible worrisome complication of mumps
CNS meningitis that can occur during mumps or 10 days after
prophylaxis for bat bite?
wound cleaning + rabies immunoglobulin + five injection rabies vaccine
RMSF treatment options
have to use doxy. Amox doesn’t work like it does in lyme
thrombocytopenia + eczema + increased susceptibility to infection
Wiskott-Aldrich syndrome = X linked recessive combined immunodeficiency
2 weeks of diarrhea in kid who is at daycare with people getting diarrhea
Crypto - even though immunocompetent can get it. Can pick it up on microscopy
erythema multiforme + vesicular, ulcerated lesions of mucous membranes
stevens-johnson syndrome
DRESS stands for? and associated with?
Drug rash, eosinophilia, systemic symptoms
associated with anticonvulsants
weakness, facial swelling, muscle pain, fever eos on CBC. GI symptoms earlier. from rural central america
ate improperly cooked T spiralis
How do you treat trichanella spiralis
mebendazole or albendazole
contraindications to LP
severe ICP without an open fontanelle
skin infection at puncture site
uncontrolled bleeding disorder
too unstable
Most common cause of croup
parainfluenza virus
Digeorge syndrome has hypoplasia of what?
Thymus and parathyroid glands bc defective embryologic development of third and fourth pharyngeal pouches
Immunoglobulins in Wiskott-Aldrich syndrome
diminished IgM. Elevated IgA and IgE
Also have thrombocytopenia
hypergammaglobulin E, eczema like rash, recurrent severe staph infections
Job-Buckley syndrome - disorder of phagocytic chemotaxis
How is wound through a tennis shoe vs on bare foot different?
Through tennis shoe can get pseudomonas bc warm wet conditions
Through bare foot get outdoor stuff like staph
Bartonella Henselae causes
catch-scratch disease
young child with few days of high fever that quickly goes away and followed by an evanescent maculopapular rash
Roseola - mostly caused by HHV-6
infant with interstitial infiltrates, eosinophilia. No fever. Mom had?
Chlamydia trachomatis infection
Under 1 year old with URI for a few days that suddenly becomes bad fever with severe respiratory symptoms?
Staph pneumonia
Learn the fungal causes of pneumonia
learn fungal causes of pneumonia
What is the concern with prednisone for treating suspected ITP
Would want to do a bone marrow biopsy first to rule out leukemia.
When are levels of erythrocyte protoporphyrin elevated?
lead poisoning or when iron levels are severely limited
Sickle cell patient with stroke. What is first thing you do
blood transfusion to dilute out sickled cells
sickle cell daily treatment?
folate and penicillin
What is kasabach-Meritt phenomenon
large vascular anomalies cause sequestration at birth getting thrombocytopenia, coagulopathy and MAHA
infantile fibrosarcoma in an infant often mistaken for a
infantile hemangioma - think this when the hemangioma is not resolving
rash, fever, lymphadenopathy wtih associated pneumonia or sepsis every 3 weeks. Treat with?
cyclic neutropenia - treat with recom human granulocyte colony-stimulating factor
When does hgb reach physiologic nadir
6-8 weeks
pale baby who is otherwise normal with good retic coung and no ABO incompatability what happened?
fetomaternal transfusion
Why do you get a reading of atypical lymphocytes on smear in leukemia
Actually blasts but read as atypical lymphocytes
aniridia in an infant, check for?
Wilm’s tumor aka nephroblastoma
Both associated with chromosome 11 mutation
Eosinophilia in a healthy newborn likely caused by?
helminth infestation
What can cause spherocytosis other than hereditary spherocytosis
ABO incompatibility (not Rh), G6PD, hyperthermia,
PT in hemophilia A and B?
Not prolonged
thrombin time test for
conversion of fibrinogen to fibrin
VWF type 1, with some acute bleeding
Give desmopressin
Ewing sarcoma rarely occurs in?
Blacks
Bone pain that occurs in the middle of the night is concerning or not concerning?
very concerning
lymphadenopathy of cervical and inguinal areas with cough and nightly fevers what should you do first?
Get a CXR. suspecting hodgkins. Get CXR before lymph node biopsy
Child starts having jaundice, hepatomegaly, seizures after starting to drink fruit juice.
hereditary fructose intolerance
Will have reducing substance on urine test
Gaucher disease characterized by? What do you see in Xray
Beta glucocerebrosidase deficiency
- get erlenmyer flask appearing long bones
Child born to mother with hyperthyroidism gets
heart failure, thrombocytopenia, microcephaly etc.
All low things because the body is overdoing it
What penile length and testicular volume indicates that some pubertal development has occured
penis longer than 2.5cm, Testicular volume greater than 3 cubic centimeters
unilateral or bilateral deafness, heterochromia irides, white forelock
Waardenburg syndrome
Most common cause of goiter in western child
lymphocytic thyroiditis
10 year old very overweight, short, has osteopenia on xray, hypertension has?
If this was an infant?
congenital adrenal hyperplasia
if infant its adrenal carcinoma
patients with galactosemia at increased risk of?
E. Coli sepsis
elevated blood or urine succinylacetone
tyrosinemia
elevated leucine, isoleucine, valine, depression of alanine
maple syrup urine disease
In achondroplasia what ratios are off
the limbs are short and head is large
prolonged jaundice and constipation
hypothyroidism
macrosomic w/ macroglossia, abdominal wall defects, organomegaly, linear ear creases. Hypoglycemia as newborn
Beckwith- Wiedemann syndrome
have increased incidence of cancer
constitutional short stature have what bone age and height age
bone age and height age that are younger than chronologic age. So although shorter will have time to grow and reach a good height
neuro complication of achondroplasia
hydrocephalus bc boney overgrowth at foramen magnum
first step in evaluating solitary thyroid nodule found on exam
serum thyroid function tests
long term complication of patients with bilateral retinoblastoma
Some other primary tumor - most commonly an osteosarcoma
short, delayed bone age, mental retardation, increased bone density, brachydactyly, obesity, subcapsular cataracts, lots of calcifications
pseudohypoparathyroidism or Albright hereditary osteodystrophy
body doesn’t respond to PTH so look hypoparathyroid but have high PTH
Immobilized following fracture of weight bearing bone can get
hypercalcemia
In 21 hydroxylase deficiency what do you find on labs
elevated 17 hydroxyprogesterone deficiency beyond 3 days of life
urine turns black on exposure to oxygen
alkaptonuria - deficiency of homogenistic acid oxidase
What do you get in the kidneys with wilson syndrome
Fanconi syndrome - renal tubular dysfunction. RTA type 2 with lots of other absorption problems
Menkes syndrome vs wilsons disease
In menkes have low ceruloplasmin and low copper
hypothermia, hypotonia, myoclonic seizures in first months of life
Menkes syndrome
What are the renal findings in vitamin D deficiency
phosphaturia and hypocalciuria bc low calcium and phosphate stimulates PTH which triggers release of phosphate and reabsorprtion of calcium
tetany and convulsions in first 72 hours of life likely
idiopathic hypocalecmia - from maternal problems, resp distress, low birth weight, hypomagnesemia
lab findings in patients with vitamin-D resistant ricketts
genetic abnormality in reabsorption of phosphate in kidneys
get hypophosphatemia with usually normal calcium
blue sclera, easily broken bones what is it and what are lab ca and phos
osteogenisis imperfecta - have normal Ca and Phos
obesity, mental retardation, hypogonadism, polydactyly, retinitis pigmentosa with night blindness
Laurence - Moon- Biedl syndrome also called Bardet-Biedl
Think moon is fat and at night.
what abnormality do you see on lab values of G6PD deficiency
patients are hyperlipidemic. Increased TG levels cause factitiously low electrolyte values
newborn with low sloping shoulders, hand attached at elbow, agenesis of forearm, missing chest wall musculature, birid thumb
Holt-Oram syndrome
Mitochondrial disease mainly involves what organs?
Neuro and muscle
hypopigmented whorls on upper extremities
hypomelanosis of Ito
isoretinoin problems?
teratogenic
liver abnormalities. Messes with your lipids
can cause muscle pain
treatment of trunk acne
benzoyl peroxide with some added retinoic acid if you want
first hint of hypertrophic cardiomyopathy can be?
passes out during practice or at a game
What defines a grade 1 concussion?
no loss of consciousness and gets back to normal under 15 minutes
small papules on genitals that become pustular, erode, ulcerate with concomitant painful inguinal lymphadenopathy
chancroid
painless papule on genital that resolved. now has unilateral draining inguinal lymphadenitis
lymphogranuloma venereum - serotype of C trachomatis
How to differentiate chancroid and lymphogranuloma venereum
chancroid has lymphadenopathy at same time as ulcer or ulcers.
Mostly shoulder injuries including rotator cuff tendinitis think
swimming
osgood schlatter seen in what sports
basketball and volleyball
Most common injury in skiiers
skiers thumb - abductiona nd hyperextension of thumb spraining of ulnar collateral ligament
Diseases that involve triplet repeats
Fragile X, DMD, Huntington chorea, Friedreich ataxia
