Peds Flashcards
additional risk factors for slipped capital femoral epiphysis
normal age for SCFE
endocrinopathies like hypothyroid, GH deficiency, renal failure and radiation history
-these pts usually have bilateral disease and present at earlier age
normally 10-16
TB meningitis
WBC count
glucose
protein
wbc = 5-1000
glucose <10
protein >250
what test should be performed at the time of dx of Kawasaki disease
echocardiogram
the best diagnostic approach in sx children with rapidly increasing head circumference is what
CT scan
what bleeding disorder presents with predominatly easy mucosal bleeding, ecchymoses or petechiae
VWd
Foodborne botulism differes from infantile how
Prodrome of N/V, abdominal pain and diarrhea
absence seizures
muscle tone?
comorbidities?
treatmetn
preserved muscle tone
ADHD
anxiety
ethosuximide
McCune albright syndomre characterized by what
Precocious puberty, Pigmentation (cafe au lait spots) polyostotic fibrous dysplaiaand multiple bone defects
associated with endocrine disorders
deficiency of niacin vitamin ____ causes what
B3
pellagra = diarrhea, dermatitis, and dementia, can lead to death if sever
can be seen in people with bowel disease that interferes vitamin absorption
classic lab findings in sJIA
leukocytosis, thrombocytosis, and elevated inflammatory markers and anemia from chronic inlfmmation
chest x ray: diffuse reticulogranular appearance, air bronchograms, low lung volume
sx
RSD
tachypnea
grunting
nasal falring
retractions
complications of prematurity
RSD PDA bronchopulmonary dysplasia IVH NEC retinopathy of prematurity
second most common tumor in children and sx
medulloblastoma
infratentorial = comit, ha, ataxia
Dx?
Bilateral bulbar palsies (eg ptosis, sluggish pupillary response to light, poor suck and gag reflexes) then descending flaccid paralysis
Tx
Botulinism
Tx: botulinism immune globulin
biphasic stridor that improves with neck extension
can also have dsphagia, vomit, difficulty feeding
50% of pts also have what
vascular ring
aoritc arch developed wrong and wraps around trachea and esophagus
50% of pts have VSD or TOF
if untreated splenic sequestration can progress to what
lab findings
hypovolemic shock bc lots of total blood volume trapped in the spleen
normocytic anemia, elevated reticulocyte count, thrombocytopenia
newborns are born with a larger RV compared to LV bc the RV pumps systemic blood via the PDA which appears as what deviation and what waves
physiologic right axis deviation and R waves in V1-V3
girl with turner syndrome and bones
decreased bone mineral density bc of no estrogen
increased risk of osteoperosis
another name for osgood schlatters disease
path
pe
tx
traction apophysitis
periods of rapid growth in which quads tendon puts traction on apophysis of the tibial tubercle where patellar tendon inserts
PE: edema and tenderness over tibial tubercle
reproduce pain by extending knee against resistance
rest and nsaids
____ should be suspected in any pt with continued ear drainage for several weeks despite appropriate antibiotic therapy
description
can lead to what ominous things
cholesteatomas
retraction pocket in the tympanic membrane, which can fill with granulation tissue and skin debris
hearing loss and life threat brain abscesses or meningitis
when do you transfuse bili baby
at 20-25 total bili
if body is pink in newborn but extremities or cyanotic is this normal?
yes
when are parents not allowed to make medical decsiion for child
when there is a life saving treatment available for the child
intrinsic pathway factors and what pt or ptt
aptt
XII, XI, IX, VIII, X
extrinsic pt
7 and 10
abduction and internal rotation of hip limited in what disease
LCP
ABIR limited in LCP
x linked agammaglobulinemia
cx manifestations
dx
tx
cx: recurrent sinopulmonary and GI infections after age 6
absence of lymphoid tissue on exam (tonsils LNs)
dx: decreased Igs and B cells, normal T cells, no response to vaccines
tx: IG replacement therapy, prophylactic antibiotics if severe
____ is used to diagnose urinary refulx, what pt would need this
voiding cystourethrogram
pts with recurrent urinary tract infections
abnormal sexual behavior in preadolescents
repeated object insertion into vagina or anus
sex play
force threats or bribes in sex play
age inappropriate sexual knowledge
cyanosis, and dark chocolate colored blood
standard pulse ox readings are low
normal arterial partial pressure of oxygen**
> 5% difference btwn oxygen saturation on pulse ox and ABG
methemoglobenimea
methemoglobinemia is most commonly acquired after what
physiology of it or biochem of it not sure which subject it is but who cares
excessive exposure to an oxidizing agent like
dapsone, nitrites, and local/topical anesthetics
Fe2+ iron gets oxidized to ferric Fe3+ which has decreased affinity for oxygen but the remaining 3 ferrous sites have an increased oxygen affinity which leads to decreased oygen delivery to peripheral tissues
due to poor oxygen affinity of methemoglobin O2 supplementation does not help
bc arterial blood gas testing analyzes unbound arterial oxygen the PaO2 is normal and overestimates degree of true oxygen saturation
abnormal social development and extreme behavioral rigidity, with onset in early development. higher functioning individuals with normal language and intellectual ability may present later when deficits become more paparent with increased social demands
autism
what age group would you find breath holding spells
6 months to 6 years
strawberry hemangioma is also what name
tx
superficial hemangioma
regress spontaneously
what should be suspected in a cyanotic infant with left axis deviation and small or absent R waves in the precordial leads?
what is needed for survical
tricuspid valve atresia
lack of communication btwn right heart chambers = hypoplastic right ventricle and diminished RV forces on ECG
the lack of blood flow to RV and pulmonary outflow tract = underdevelopment of pulmonary valve and or artery = decreased pulmonary markings on chest x ray
associated ASD and VSD are needed for survival
gaucher disease is due to ___ deficiency
cx features
loss of milestones?
glucocerebrosidase def
HAT
HSM, anemia, thrombocytopenia
no loss of milestones or red macula
neonatal treatment for RSD
maternal antenatal glucocorticoids
continuous ppv
intubation if severe
what disease gives a maculopapular polymorphous rash after administration of ampicillin or amoxicillin
EBV
chest xray for transient tachypnea of newborn
path
resolves when
bilateral perihilar linear streaking
think liquid bc alveolar fluid clearance inadequate
resolves by day 2 of life
if pt with sinus infection gets periorbital edema, vision abnormalities or AMS then what management step
CT
cholesteatoma causes
sx
what you see
complications
genetic (younger pt)
acquired usually occur secondary to chronic middle ear disesease
cont ear drainage for sev weeks despite approp abx therapy
granulation tissue and skin debris
lead to hearing loss, CN palsies, vertigo, life threat brain infections
*new onset hearing loss or chronic ear drain with abx therapy
when a radiolucent foreign body is suspeected and not visualized on x-ray, ____ can be considered as the next diagnostic procedure
CT
which kind of cerebral palsy is most common in preterm infants
cx features
resistance to what
spastic diplegia
hypertonia, and hyperreflexia mainly LEs with both feet pointing down and inward (equijnovarus deformity)
resistance to passive muscle movement
optic pathway glioma in what dissease in children
NF type 1
colon in meconium ileus
narrow, underdeveloped colon (microcolon)
hurler syndrome is due to what deficiency?
age presentation?
cx features?
lysosomal hydrolase deficiency
age 6 months to 2 years
coarse facial features, inguinal or umbilical hernias, corneal clouding, and HSM
diagnostic features of acute bacterial rhinosinusitis
persistent sx 10 days of more without improvement
or
severe sx with 102 fever, purulent nasal discharge or face pain 3 days or more
or
worsening sx 5 days after initially improving viral URI
child with conjunctival injection, tarsal inflammation, and pale follicles are concerning for what
types
spreads in what conditions
what kind of conjunctivitis
concomitant what
last thing real bad
trachoma
chlamydia trachomatis serotypes A B and C which is leading cause of blindness worldwide
spreads in crowded conditions
follicaular conjunctiivits
concomitant nasophayngeal infection
scarring of eyelids
celiac disease extraintestinal sx
fatigue
IDA from poor absorption 2ndary to duodenal villous atrophy
dermatitis herpetiformis located on knees, elbows, forearms, and buttocks
legg clalve perthes disease is what and affects what pop
5-7 yrs in the LCP
idiotpathic AVN of hip
erythema tocicum neonatorum
healthy baby and asx papules and pustules
common in full term neonates
spares palms and soles in first 2 weeks of life
goes away on own
treatment for severe pneumonia in CF pts
empritic antistaph antibiotics like Cefepime (covers MSSA and psuedomonas)
MRSA coverage with IV vancomycin
treatment of sJIA
glucocorticoids, NSAIDs, biologic agents
NF1 inheritance what gene, what protein what cx features
AD
NF1
neurofibromin
cafe au lait macules, freckling and lisch nodules
low grade optic pathway glioma in 15% of pts under 6
ependymoma
what cells, location
sx
glial cell tumor that arise in ventricles and spinal cord 4th ventricle in posterior fossa common in children
tumor expansion leads to obstruction of CSF and increased ICP
asthma pts with respiratory failure despite escalation of medical treatment require
endotracheal intubation and mechanical ventilation
risk factors for cerebral palsy
prematurity IUGR Intrauterine infection antepartum hemorrhage placental path multiple gestation maternal alcohol consumption and tobacco
disesae with low IgM IgA IgG in the normal B lymphocyte count
common variable immunodeficiency
what increases HCOM murmur
what inheritance and pop
valsalva bc decreases preload and abrupt standing and nitroglycerin
AD in blacks
how can OM with effusion be distinguished from AOM
om with effusion has lack of acute inflammaotry signs
ewings sarcoma
common sites
metastasis
age
appearance
metaphysis and diaphysis of the femur, followed by the tibia and humerus
lungs and LNs
white males, 0-20
lamellated appearance, o onion skin periosteal rxn, lytic cental lesion
moth eaten appearance
midgut volvulus classically presents in what pop and how
neonate under 1 month with bilious vomiting
can cause bloody stools, bowel perforation, abdominal distension and peritonitis
signs of ischemia or systemic decompensation are indication for emergency laparotomy
universal screening for dyslipidemia in children at what age
9-11 and 17-21
pre and post puberty
the thymus is normally visible on chest x rays in children under what age
3
sail sign bc looks triangular, scalloped and dense
dx pertussis
tx
pertussis culture or PCR
lymphocyte predominant leukocytosis
macrolides
bartonella henselae cx manifestations
tx
type of bacteria
papule at scratch/bite site
regional adenopathy
+/- fever of unknown origin for 14 days or more
azithromycin
gram neg bacillus
affected lns are enlarged, tender and have overlying erythema
cyanosis and respiratory distress during feeding that improves when infant cries
bilateral choanal atresia
dermal melanocytosis aka what occurs in what pop where are they located, what do they look like nad tx
mongolian spot
lower back and buttocks
native americans, africans, asians, hispanices
fades spontaneously
look like brusies but bruises are more varied in color and fade quickly
infected infants of toxo should receive what
pyrimethamine, sulfadiazine, and folate for a year
hepatitis B pt can have what renal disease
membranous nephropathy
pts with galactoinase deficiency present with what
cataracts only
evaluation of primary amenorrhea
pelvic US: uterus present—> serum FSH
- increased = karyotype
- decrease = cranial MRI
US: uterus absent—>karyotype serum testosterone too
- if 46 xx and normal test = abnormal mullerian devel
- if 46, xy and normal test level = AIS
Chronic granulomatous disease results from what
susceptible to what organisms
test
inability of phagocytes to produce hydrogen peroxide in their lysosomes
abscesses due to fungi or catalase positive bacteria like s aureus, serratia, burkholderia, aspergillus
dihydrohodamine 123 test
nitroblue terazolium test
idiopathic precocious puberty
gender
LH and GnRH
girls
basal levels of LH elevated and wil increase with GnRH stimulation
dx of Kawasaki:
pt should have fever for 5 consecutive days as well as 4 of 5 of the following
1) conjunctivitis, spares limbus
2) oral mucosal changes with erythema, fissured lips, strawberry tongue
3) rash
4) extremity changes, erythema, edema, desquamation of the hands and feet
5) cervical LAD: >1.5 cm, usually unilateral
major criteria acute RF
JONES
joins (migratory arthritis) O (carditis) nodules (subcut) erythema marginatum sydenham chorea
iron poisoning dx
iron is radiopaque and can be visualized on abdominal xray
aspirin similar but doesn’t show up on xray
truncus arteriosus is strongly associated with what
Digeorge syndrome
____ is characterized by severe pain, pallor, poikilothermia, paresthesias and late findings of pulselessness and paralysis
comparment syndrome
chronic oligoarthritis, daily fever and rash in child
what kind of disease
almost all pts with it present with what
systemic onset juvenile idiopathic arthritis sJIA
auto-inflammatory
2 weeks of fever occuring once a day, arthritis of 1 or more joints and a pink macular rash that worsens with fever
joint pain worse in morngin and better as day goes on
clinical manifestations of late onset CAH aka non classic
premature adrenarche/pubarche, severe chystic acne resistant to treatment, accelerated linear growth, advanced bone age
normal electrlyts
nephrotic syndrome in a pt with hepatitis B infection what kind
membranous nephropathy
posterior fossa tumor typically presents with what
cerebrellar dysfunction (ataxia, dysmetria)
how does neonatal polycythemia effect hematocrit which effects other stuff
hematorcrit rises with plycythemia which increases viscosity of the blood and impairs blood flow to various organs
celiac disease is associated with what other diseases and should be screened for in these ptws
how to screen
DM1 and thyroiditis
screen with anti-tissueTG antibody IgA followed by endoscopic duodenal biopsy for conifrmation
gold standard for testing for duchennes muscular dystrophy and what is finding
genetic testing
-will find muscle biopsy with absent dystrophin
deletion of dystrop[hin gene on Xp21
x linked recessive
infant or newborn with failure to thrive, bilateral cataracts, jaundice and hypoglycemia has what
pts at increased risk for what infection
tx
galactosemia from galactose-1-phosphate uridyl transferase deficiency (GPUT)
increased blood galactose levels
increased risk for E coli
elimante galactose from diet
angiokeratomas, perpiheral neuropathy, and asx corneal dystrophy
renal and heart failure possible to and risk thromboembolic events
fabrys diseae
FABRY ball CAP (corneal dys, angioker, periph neuorp)
KC (kidney and cardiac failure)
otitis externa
-micro infection
clinical manfest
tx
pseudomonas
staph aurues
-swimmers ear
pain with auricle manipulation, pruritus, discharge
tx: topical antibiotic (fluoroquinolone
topical glucocorticoid
testicles that have not descended by age what are unlikely to descend spontaneously and require surgery at the optimal age of what (orchiopexy)
6 months
1 year
beckwith wiedemann syndrome
pathogenesis (what chrom)
PE
complications
survellance
deregulation of imprinted gene expression in chrom 11
PE: fetal macrosomia, rapid growth unitl late childhood
- omphalocele or umbiical hernia
- macroglossia
- hemihyperplasia
complications: wilms tumor or hepatoblastoma
surveillance: AFP and abdominal/renal US
monitor hypoglycemia, which is transient
treatment for HSP
supportive with hydration and NSAIDs for most pts
hospitalization and systemic glucocorticoids in pts with severe symptoms
orbital cellulitis
location, bugs, clinical manifestations
posterior to orbital septum
staph oaureus, strep pneumo and other streptococci
possible abscess within the orbit or brain, blindness, or sinus venous thrombosis
ophthalmoplegia, pain with extraocular movements, proptosis, virison impairment
inherited forms of QT prolongation and inheritance
sx
tx
standard
mild sx
sx with history of syncope
jervell lange nielsen AR
romano ward syndome AD
syncope, life threatening ventricular arrhytmias like torsades and sudden death
avoid exercise, certain meds, normal calcium, K+, and Mg+
beta blockers like propranolol shorten QT
if sx with history of syncope then need b blocker and pacemaker placement
treat diamond blackfan syndrome
corticosteroids, and transfucsion if needed
biliary atresia presents when and with what
early infancy with obstructive jaundice and acholic stools and is fatal without intervention
guillain barre syndrome pathology
sx
final stage
accompanying sx
tx
demyelination of the peripheral motor nerves
ascending weakness accompanied by feet tingling and neuropathic pain
final stage is flaccid paralysis with absent DTRs and nerve conduction velocities
accompanying autonomic sx can occur
pooled IG or plasmapheresis
carotid pulse with a dual upstroke is what
what other murmur
HCM
and also systolic ejection murmur along LSB with strong apical impulse bc LVOT
erythema multiforme is seen most commonly in what infection
herpes simples
all infants, regardless of maternal screen results should receive what for eyes and infections
topical prophylaxis within an hour of birth
prevents gonococcal disease
erythromycin ointment
maternal estrogen effects in mnewborns
breast hypertrophy in boys and girls
swollen labia
physiologic leukorrhea
uterine withdrawal bleed
medulloblastoma occurs where and causes what
posterior fossa in children
infratentorial in cerebrellar vermis and presents with vomiting, HA, ataxia
0758 and 590
reveiew
juvenile idiopathic arthritis cx features
poly vs oligo
which joints involved
symmetric arthritis for at least 6 weeks
polyarticular is 5 or more joints
oligoarticular is less than 5
knees, wrists, ankles, cervical spine and joints in hands and feet
what is a side effect of hydroxyurea (major)
myelosuppression, predisposes pt to infection
c trachomatis neonatal conjunctivits occurs when and what type of discharge
5-14 days after birth, watery discharge
granulosa cell tumor of the ovary cx featuers in child
precocious puberty
increased estrogen
pelvic US shows ovarian mass, thickened endometrium
edwards syndrome clinical features
head
limbs
organs
micrognathia
prominent occiput
low set ears
clenched hands with overlapping fingers
renal defects
heart defects (VSD)
limited hip abduction
rocker bottom feet
inspiratory stridor that worsens while supine
what helps
laryngomalacia
prone helps
marfanoid habitus has which direction displacement of lens? how about homocystinuria?
what protein?
inheritance?
upward (homocystinuria is AR that has displacement down)
fibrillin 1
AD
is prophylaxis recommended for all close contacts for pertussis
yes, use macrolides even if vaccinated
11B hydroxylase def
same as 21 but no salt wasting, have HTN and salt retention
from increased 11 deoxycorticosterone
normal features of LNs
feels
mobile or not mobile
size
soft
mobile
under 2 cm
cx presentation of measles
prodrome of cough coryza, conjunctivitis fever, koplik spots
maculopapular rash that starts head and goes down
wet diapers in neonates
how ever many days old is how many wet diapers should be up to first week.
after first week should have 6 or more
due to its location, supracondylar humerus fractures may be complicated by ___ injury or ____
neurovascular injury or compartment syndrome
prolonged pt and aptt in newborn from what
vitamin k deficiency
21 hydroxylase deficiency hormonal abnormalities
sx
decreased aldosterone and cortisol
increased testosterone
increased 17 0h progest
sx: ambig genitals in girls, salt wasting with hypotension and decreased na and increased K
caustic injury first and scond steps
remove contaminated clothing and then do upper GI endoscopy within 24 hours
type of strabismus that usually arises in children age <2 and is associated with eye deviation when trying to focus on objects
accommodative esotropia
causes of edema in turner syndrome pt
congenital lymphedema from abnormal development of the lymphatic netowrk
accumulation of pretein rich interstitial fluid in hands, feet and neck (webbed neck)
severe obstruction of lymphatic vessels can lead to cystic hygroma of neck and fetal hydrops
The most common congenital heart malformation is what
Murmur?
VSD
Holosystolic murmur at left lower sternal border
adolescent with a solitary breast mass most likely has what
what kind of tenderness is common
management
FAD
premenstrual
observation in adolescents
-check one cycle later then if decreased reassure
US for a persistent mass or older pt
using ___ in pregnancy causes long smooth philtrum, small palpebral fissures and thin upper lip and also ____
alcohol
CNS abnormalties like mental retardation
infants of diabetic mothers second trimester problems
fetal pancreas sufficient so now increased storage causes organomegaly, incrased GF (macrosomia) and increased oxygen consumption (polycythemia)
what is a GnRH stimulation test used for
evaluation of precocious puberty
pts with known or suspected measles should be what
when to give vaccine
isolated and placed in airbrne precautions
2 doses (age 1 and 4) give btwn age 6 and 11 momths if suspected travel
truncus arteriosus strongly associated with what syndrome and sx
digeorge
neonatal cyanosis, heart failure and systolic ejection murmur with a loud ejection click at LSB
anatomical injury mech with shaken baby syndrome
sx
dx
repetitive acceleration-deceleration forces cause shearing of the dural veins and coup-contrecoup injury with brain impact on the skull
subdural bleed can manifest as seizures, increased head circumference, bulging anterior fontanelles and AMS
also causes vitreoretinal traction and RETINAL HEMORRHAGES (pathognomonic)
dx: CT without contrast and skeletal survey
inattentive staring spell
occurs during boring activity
variable length over 1 minute
respond to stimulus
no automatisms
intussusception presents how
dx
tx
intermittent periodic pain associated with drawing the legs up toward the abdomen, emesis may follow
sausage shaped mass in RUQ
US to detect, use air enema to treat
what can cause neonatal polycythemia
increased EPO from intrauterine hypoxia: maternal diabetes, hypertension, or smoking: IUGR
pre-eclampsia
delayed cord clamp, t-t transfusion
hypothyroidism or hyper, trisomies
juvenile idiopathic arthritis laboratory findings
elevated inflammatory markers
hyperferritenemia, and hypergammaglobulinemia
thrombocytosis
ANEMIA (chronic inflammation and IDA), inflammatory markers increase hepcidin which prevents absoprtion of iron
milk or soy protein induced proctocolitis
pt pop
sx (triad)
from what
tx
infants
non IgE immune respone to proteins in formula or breast milk that cause rectal and colonic inflammation
eczema, regurgitation or vomit and painless bloody stools
mom elimate all dairy and soy from diet when breast feeding
evaluation of bilious emesis in the neonate
first thing you do then next thing
stop feeds
NG tube decompression
IV fluids
abdominal x-ray
positive nikolsky sign
erythema on face and generalizes within 24-48 hrs
superficial bullous
SSSS
xrays for osteosarcoma show what
mix of radiodense and radiolucent areas
weird GI thing that can happen in HSP
location
dx
tx
intussusception
small bowel or ileo ileal
dx by presence of a target sign on ulstrasound
-ileal ileal cannot usual be fixed with air enema and need surgery
bedwetting is normal before age what
5
treatment for
umbilical hernia
gastroschisis
omphalocele
umbilical hernia: monitor for spontanseous resolution by age 5
other 2 need immediate surgery after birth
polyhydramnios and vomiting with inital feeds with no RD
abdomen not distended and intestinal gas absent on xray
duodenal atresia
if have cryptorchid gonads what should you do in AIS
perform gonadectomy after puberty
can become dysgerminoma, gonadoblastoma after puberty
still want testosterone for normal height so do not removed till after puberty
breastfeeding failure jaundice
timing
pathophys
cx features
first week of life
lactation failure results in decreased billy elimination, increased enterohepatic circulation of billy
suboptimal breastfeeding and
signs of dehydration (brick red urate cystals, decreased urine output and losing weight)
jaundice
in congenital diaphragmatic hernia what is next step hfter airway has been secured
nasal or orogastric tube to provide continuous suction and prevent bowel distension against the lungs
also umbilical arterial line and venous cath should be placed
evaluation of primary amenorrhea steps
1) ___ or ____
if uterus absent then what
pelvic exam or US
uterus absent
karyotype and serum testosterone
- 46 xy = AIS
- 46 xx = Abnormal mullerian development
howell jolly bodies are what and seen in what
nuclear remnants within red blood cells that are typically removed by the spleen
single round blue inclusions on wright stain
presence of them indicates physical absense of spleen or functional hyposplenism due to splenic autoinfarction or splenic congestion
birth weight and loss in neonates
may lose up 7% of birth weight in first 5 days and it should be regained by age 10-14 days
differential for lytic bone lesion in a child
if have hypercalcemia narrows to what
infectious osteomyelitis
endocrine: hyperparathroid oseitis fibrosa cystica
neopalstic: ewing, langerhans cell histocytosis, metastasis
idiopathic
narrow to lytic bone neoplasm or hyperpatathyroid state (pituitary adenoma most common but occurs in pts over 50)
what kind of seizures have automatisms
length of these seizures?
absence seizures
last under 20 seconds
cx features of biliary atresia
well appearing at first followed by development of the following over 1-8 weeks
jaundice, acholic stool or dark urine, hepatomegaly, increased hyperbili, mild elevation transaminases
tx for AOM
initial is amoxicialin
2nd line is amox clav
evaluation of bilious emesis in the neonate
get abdominal x ray and see no evidence of free air and the bowel gas pattern is not suggestive of duodenal atresia or distal obstruction then
upper GI series (barium swallow)
fastest way of diagnosin midgut volvulus with malroation
ligmanet of treitz on right = malrotation
corkscrew pattern = volvulus
uric acid crystals in diapers with pink stains or brick dust in neonatal diapers abnormal?
no this is normal
most appropriate step in a 1 month old with VSD
echo to assess the size
if small spont close by age 2 usually with no long term sequelae
large defects have softer murmur bc of leess turbulence
-eisenmenger syndrome
pt with turner syndrome are at risk for decreased what due to what
bone mineral density bc lack estrogen
increased risk of osteoporosis
atresia of the jejunum or ileium is thought to occur due to what
sign
vascular accident in utero
vasoconstritive meds like coaine and tobacco
sign is triple bubble sign and gasless colon on xray
most common bufs for AOM
strep pneumo
H influeznae
moraxella catarrhalis
hemoglobin SC
type of sickle cell where you inherit C and S
similar complications like pain crises as SS but less often and less severity
splenic sequestration/infarction happens in adolescence rather than childhood
is lymphedema in turners pitting or non pitting
non pitting
treatment for radial head subluxation (nursemaid’s elbow)
hyperpronation of forearm or supination of forearm and flexion of elbow
classic presentation of slipped capital femoral epiphysis
metaphysis and proximal femur slip relative to epiphysis at epiphyseal plate
obese adolescent male with complaints of pain
kallman syndrome is what inheritance and cx features
x linked recessive
normal genotype
absence of GnRH = short, and delayed or absent puberty
amenorrhea, absent breast development
anosmia hyposmia
most children are ready to begin toilet training at age what
2 or older
laboratory findings in hemophilia A and B
PTT
platelet
bleeding time
PT
PTT prolonged
normal platelet, bleeding time and PT
vesicoureteral reflux
grade I II III IV V
I: into nondilated ureter
II: into pelvis and calyces without dilation
III mild to mod dilation of ureter, renal pelvis and calyces with min blunting of fornices
IV moderate ureteral tortuosity and dilation of pelvis and calyces
V: gross dilation of ureter, pelvis and calyces, loss of papillary impressions, ureteral tortuosity
management of primary nocturnal enuresis
which is most effective long term intervention of these
lifestyle change enuresis alarm (most effective long term intervention) UA to r/o secondary causes
Desmopressin therapy if above does not work
TCAs only if desmopressin does not work bc of AEs
painful vesicular rash with punched out erosions and hemorrhagic crusting
eczema herpeticum
superimposed primary herpes simplex virus infection
open area that is exposed to HSV type 1 can develop painful vesicles with erythemaouts base that evolve to punched out erosions with hemorrhagic crusting
pt has abnormal uterine bleeding defined as what during adolescence. what causes it
menstrual bleeding under 21 days or over 45 days apart
immaturity of HPO axis fails to produce correct ratios of gonadotropin releasing hormone and therefore LH and FSH to induce ovulation
first few years post menarche, majority of menstrual cycles are anovulatory and present as painless, irreg, heavy bleeding
no progesterone in anovlulatory cycles
normal sex beahvior in preadolescents: toddlers
EMU
explore own or other genitals
masturbate
undressing self or others
purple or red eardrum +/- bulging
hemotympanum
chest radiograph sowing diffuse reticulogranular pattern and air bronchograms is what
RDS
optic pathway glioma found in what pts
NF1
can cause decreased visual acuity, alterations in color vision, optic nerve atophy and proptosis
transient synovitis typically presents in what pop and cx features
tx
follows what
on exam hip is what
kids 2-8 with restricted ROM of hip joint, resolves with conservative therapy
follows viral infection or mild trauma
hip flexed, slightly abducted and externally rotated
FABER Tran Sexuals
complications of prader-willi syndrome
feeding pattern: infancy and later
sleep apnea
DM
gastric distension/rupture
death by choking
poor suck and feeding problems in infancy followed by a life of compulsive binge eating
acute lymphoblastic leukemia
other features with it
peripheral smear
degree of what
diagnostic
peroxidase positive granules
positive for what
30-50% pts present with infections
1/2 have splenomegaly of LAD
lymphoblasts on perioph smear
vary degree of anemia, neutropenia, thrombocytopenia
presence of more than 25% lymphoblasts in BM diagnostic
no peroxidase posigve granules
PAS positive
TdT positive
fungal infection of scalp that causes pruritc patchy, fine, white scales that may resemble SD
does not involve what and not age
tinea capitits
no eyebrows or nasolabial folds
not common first year of life
fanconi anemia
blood stuff
age
clinical sx
progressive pancytopenia and macrocytosis
age 8 is average age
cafe au lait spots, microcephaly, microphthalmia, short stature, horseshoe kidneys and absent thumbs
what is the most common causes of chronic renal insufficiency/failure in children and occurs in what population
anatomy
posterior urethral valves causes obsturction
distended bladder, ureters and hydronephrosis
only in boys
supratentorial tumor presents with what
seizures, weakness, and sensory changes
minor criteria in acute RF
P FACE
Prolonged PR
Fever
Arthralgias
CRP up
ESR up
what kind of blood penia should raise concern for malignancy
thrombocytopenia
comorbidities with cerebral palsy
intellectual disability
epilepsy
strabismus
scoliosis
congenital toxoplasmosis
what risk factors
undercooked meat
unwashed fruits/veggies
cat feces
cx: macrocephaly, intracranial calcifications, jaundice, HSM, blueberry muffin spots
congenital diaphragmatic hernia is what
__ from esophag compresion
PE
tx
herniation of abdominal viscera into chest making pulmonary hypoplaia and PHTN
85% of cases on left
polyhydramnios can occur from esophageal compression
causes concave abdomen and barrel shaped chest on PE
emergecny intubation and cautious ventilation
help preventing SIDS
smoke avoidance during and after pregnancy supine sleep firm sleep surface room sharing but not in same bed pacifier use when sleeping
PE with slipped capital femoral epiphysis
loss of abducrtion and internal rotation of the hip as well as external rotation of the thigh while the hip is being flexed
PE: webbed neck, cleft lip, shielded chest, triphalangela thumbs, pale mucous membranes and conjunctivae
what else on labs
systolic ejction murmur
diamond blackfan anemia
macrocytic anemia, and low retic count
-no hypersegmented neutrophils
osteonecrosis is a common complication of what disease
SCD
groin, buttock, or thigh pain that initially occurs with weight bearing but progresses to occuring at rest
pain and limited ORM with INTERNAL roation and abduction of hip
no erthemia or warmth of jionts, leukocytosis or elevated inflammatory markers
xrays of hip may appear normal early and MRI should be performed to confirm diagnosis
what is the most common congenital heart defect in pts with down syndrome?
from what embryo stuff
what happens with blood flow
sx with feeding
complete atrioventricular septal defect
endocardial cushions don’t merge so both VSD and ASD
HF occurs from blood mixing and valve regurge = volume overload and excessive pulmonary blood flow
diaphoresis/dyspnea with feeds and crackles around age 6 weeks
prophylaxis for measles
vitamin A
what other workup with TE fistula
VACTERL
vertebral, anal atresia, cardiac, TE fistula, renal, limb
heinz bodies are what and seen in what disease
hemoglobin precipitation is seen in G6PD deficiency
hemoglobin becomes oxidized and forms insoluble precipitants called heinz bodies
light microscopy findings with minimal change disesae
tx
IF staining?
electron microscopy?
normal
tx: corticosteroids
no abnormality
diffuse effacement of foot processes on EM
tuberous sclerosis associated with what
cortical tubers, hamartomas, subependymal giant cell astrocytomas, subep nodules
cardiac rhabdomyomas, renal angioleiomyomas, seizures, metbal retartdation
facial angiofibroas
what is a complication of IVH in 1/3 of cases
those who survive may have what
communicating hydrocephalus
cerebral palsy if survive
when does type 1 DM present
4-6 or at early puberty
evaluation of bilious emesis in the neonate
get abdominal x ray and see double bubble sign then what is next
it is duodenal atresia
intellectual disability, fair complexion, eczema and ___ body odor is what
musty
PKU
who is at risk for meconium aspiration syndrome
neonates born after due date
precocious puberty is defined as development of 2ndary sex characteristics before age what
8 in girls
9 in boys
accelerated bone growth is also comon
central
early HPO activation
peripheral: low fsh and lh
- from adrenal or gonadal relaease of excess sex hormones
point tenderness at the inferior pole of the patella
patellar tendonitis
medically emancipated minor
emergency care, sti, substance abuse, prenancy, contrapception
drooling,tripod position (sitting up and leaning forwrd), sniffing position, stridor, high fever, dysphagia what infection
managment
h influenza type B
endotracheal intubation
antibiotics
treatment for chlamydial neonatal conjunctivitis
PO macrolide
congenital diaphragmatic hernia presents how and imaging
cyanosis and RD immediately after birth
polyhydramnios can occur crom esophageal compression
abdominal viscera into thorax shows schaphoid appearing abdomen
xray: displaced cardiac silhoutte, bowel in thorax and gasless abdomen
why is human milk better than formula
it absorbs better and improves gastric emptying
- 70% whey and 30% casein
- whey digested easier
actually has less vitamin D so need to supplement with it if breast feeding
emancipated minor
homeless, parent, married, military, financially independent, high school grad
treatment of slipped capital femoral epiphysis
surgical pinning of the femoral head
physiologic jaundice should be gone when
after 1-2 weeks
question 9 on central vs peripheral precocious puberty
question 9
evaluation of bilious emesis in the neonate
get abdominal x ray and see NG tube in misplaced duodenum then what next
what procedure
upper GI series
if see ligament of treitz on right side of abdomen then
maltoration
need surgery with Ladd procedure
in pediatric pts the most common etiology of osteomyelitis is what
hematogenous seeding by s aureus
enzyme def in niemann pick
sphingomyelinase
definition of neonatal polycythemia
hematocrit over 65% in term infants
what can cause subcutaneous emphysema with leaking from chest wall into subcut tissues
what else can occur this way
managment
severe coughing paroxysms
pneumothorax can occur this way too
get chest xray
what should be obtained in all neonates with suspected sepsis and tx how
CSF culture
antibiotic therapy
what is the best treatment for breastfeeding failure jaundice in otherwise healthy full term newborns
increase freq and duration of feeds to stimulate milk production, maintain hydration
breast feed every 2-3 hours for 10-20 mintues or more per feed in first month of life
cx features adhd sx occur where
tx
sx must occur in at least 2 settings
tx: stimulants: methylphenidate, amphetamines
nonstimulants: atomoxetine, a-2 agonist
behavioral therapy
treatment for neonatal polycythemia
IV fluids
glucose
partial exchange transfusion
refeeding syndrome happens in what pts
anorexic
what does a white reflex in the eye mean
metast where
aka leukocoria
considered retinoblastoma until proven otherwise, child needs prompt referral to an ophthalmologist
liver and brain metastases
dry flaky peeling skin of the hands and feet of newborn problem?
not a problem, this is normal
pertussis tx for close contact
under 1 month
1 month or older
under 1 = azithromycin 5 days (erythromycin can cause pyloric stenosis and clarithromycin not proven safe)
1 month or older
azithromycin 5 days or clarithromycin 7 days or erythromycin 14 days
most common cause of hypothyroidism in infants
thyroid dysgenesis (hypoplasia, aplasia)
monocular visual acuity assessment by snellen letters or numbers can begin when
at age 3
iron poisoning sx
abdominal pain, NVD and hematemesis within 30 min to 6 hrs of ingestion
vasodilator too, so hypotensive shock and anion gap metabolic acidosis from poor perfusion
gastric scarring and pyloric stensosis can occur
pts with complement def are at increased risk for what infections
disseminated bacterial with encapsulated bacteria, giardia wouldn’t effect these pts worse
when does pyloric stenosis most commonly present
at age 3-6 weeks with nonbilious projectile vomiting
esophageal coins in asx pts
what if sx or time of ingestion unknown
observe for up to 24 hrs
flexible endoscopy
tx for hemophilia A and B
factor VIII for hemophilia A
factor IX for hemophilia B
desmopressin for mild hemophilia A
how does neonatal herpes simplex virus infection of eye present
2nd or 3rd week of life with vesicular or ulcerative eye disease
cessation of breastfeeding should be reserved for infants with what
suspected galactosemia
jaundice, lethargy, vomiting and hepatomegaly
xray features with vit d def rickets
osteopenia
metaphyseal cupping and fraying
epiphyseal widening
treatment for SCID
same tx in what other disease
stem cell transplantation
wiskott Aldrich syndrome too
orchiopexy reduces risk of what but not what
testicular torsion and decreases risk of testicular cancer
not help subfertility
blood smear with occasional RBCs with single, round, blue inclusions on wright stain are what and from what
howell-jolly bodies from splenectomy
or hyposplenism
usually removed by spleen
acute unilateral LAD in children is most commonly caused by what
s aureus and s pyogenes
respiratory isolation during first ___ days of antibiotic therapy for pertussis
5
dx of homocystinuria based on elevated what and what is treatement
elevated homocysteine and methionine
tx: B6, folate, B12, antiplatelets or anticoags
newborn with fTT, bilater cataracts, jaundice, and hypoglycemia has what and what enzype
galsctosemia and G 1PUT def
impetigo
bacteria involved, cx features and tx
non bullous
- s. aureus and group A strep
- painful non pruritic pustules, honey crusted lesion
bullous
- s aureus
- rapidly enalrged flaccid bullae with yelow fluid, ruptured lesions in periphery
tx: limited skin involvement = mupirocin topical
extensive skin involvement = orals like cephalexin, dicloxacillin, clindamycin
risk factor of intussusception
recent viral illness or rotavirus vaccine
pathological lead point
-meckel divert, HSP, celiac, intestinal tumor, polyp
features of turner syndrome that aren’t as common
bicuspid aortic valve
coarctation of aorta
broad chest with widely spaced nipples
narrow high arched palate
pt with sickle cell that has decreased reticulocytes and no splenomegaly what is it from
presents with what
tx
aplastic crisis
2ndary to parvovirus B19
pallor, weakness, fatigue (nonspecific for anemia) but funcitonal systolic murmur due to hyperdynamic blood flow
tx: blod transfusion
when should the quadrivalent meningococcal vaccine be given and booster
when give it really early and what age
11-12
booster at 16
age 2 if asplenic, HIV postiive or complement deficiency
accumulation of fluid in the inner ear that leads to hearing loss vertigo, and tinnitus
meniere’s diseae
ADHD dx requires that several sx be present before age what
12
hemoglobin precipitation is seen in what
G6PD deficiency, hemoglobin oxidized and forms insoluble precipitants called heinz bodies
autosomal recesive polycystic kidney disease manifestations
large flank masses in infancy, leads to oligohydramnios
pulmonmary hypoplasia and potter faces
inspiratory stridor infection
treatment?
parinfluenza virus
corticosteroids and racemic epinephrine for pts with stridor at rest
____ children of all ethnicities are also at high risk for precocious develpoment
obese
risk of desmopressin in notcturnal enureisis
relapse in up to 70% and hypnatremia
central vs peripheral precocious puberty
look at what first
bone age
if advanced then basal LH
if low then GnRH test
if low then gonadotrophin indep prec
x linked agammaglobulinemia is also called what
casued by defect in what
susceptible to what organisms
bruton agammaglob
Tyrosine Kinase that prevents development of mature B cells
encapsulated organisms liek H flu, strep pneumo, GIARDIA bc no IgA
how do ulipristal, levonorgestrel and OCPs, stop pregnancy in emergency situation
delay ovulation
child with deep cat bite what should management be and why
antibiotic prophylaxis and irrigation
give amox/clav for pasteurella multocida and aneorobic bacteria
evaluation of bilious emesis in the neonate
get abdominal x ray and see dilated loops of bowel
what is next steps
contrast enema
if see microcolon–> meconium ileus
if see rectosigmoid transition zone–>hirschsprungs
nec x ray shows what
what population
abdominal x ray with air in the bowel wall and portal veins
premies with bloody stool
myotonic muscular dystrophy type 1 inheritance
AD trinucleotide repeat
delayed muscle relaxation
phases of pertussis
catarrhal (1-2 weeks) mild cough rhinitis
paroxysmal (2-6 weeks) gough with inspiratory whoop, posttussive emesis
convalescent (weeks to months) symptoms reslove gradually
Acute OM age groups most common
6-18 months and 5 yrs old
causes of meningitis in children under 3
GBS (*****most common)
e coli
listeria
HSV
serous liquid filled blisters on the typanic membrane
bullous myringitis
the most common cause of syncope in children
vasovagal syncope
organisms in septic arthritis in kids birth to 3 months
and older than 3 months
tx
staph, GBS, gram neg bacilli
nafcillin or vancoymicn plus gentamicin or cefotaxime
older than 3 months
-staph, group A strep, strep pneumoniae
nafcillin, clinda, cefazolin, vanco
need arthrocentesis as dx and tx
medulloblastoma occurs where
cause what sx
posteior fossa in cerebella vermis which is important for balance and coordinaton
truncal and gain instability
can cause obstructive hydrocephalus
auscultation in DS pt with complete AV septal defect
loud S2 due to pulmonary HTN
systolic ejection murmur from increased flow across the pulmonary valve from L to R shunt across the ASD
holosystolic murmur of VSD may be ther too
stages of lyme disease
earl localized
early disseminated
late
EL: erythema migrans
fatigue, ha
mayalgias, arthralgias
ED: multiple erythema migrans bells palsy meningitis carditis migratory arthralgias
lateL arthritis, encephalitis, peripheral neuropathy
pyridoxine (vitamin ____) deficiency causes what
B6
irritability, depression, dermatitis, stomatitis
causes eleveated homocysteine concentration which is increase risk factor for VTE and atherosceleoris
DIDS you see that pyrate
extraintestinal sx in celiac disease
IDA
dermatitis herpetiformis
-pruritic popular or vesicular rash located on knees, elbos, forearms and buttocks
rickets (vit D def)
clinical manifestations
craniotabes (soft skull( delayed fontanel closure enlalargred -skull, costochondral joints, wrist widening genu varum
hemophilic arthropathy in hemophilia a and b
tx
late complication of both types and is caused by iron/hemosiderin deposition leading to synovitis and fibrosis withing the joint
early prophylactic tx with factor concentrates
complications of pediatric constipation
anal fissures
hemorrhoids
enuresis/UTIs
-fecal retention can cause rectal distension and can compress the bladder and prevent complete voiding (urinary stasis)
definition of tourettes disorder
tx
both multiple motor and 1 or more vocal tics that persist for one year after initial onset
onset under age 18
tx: antipsychotics (1st gen approved second get being prescribed more often now), alpha-2 adrenergic receptor (clonidine and guanfacine) agonists, behavioral therapy
iron studies in microcytic anemia
check uworld
brutons agammaglobulinemia presents when and how
3-6 months after birth bc maternal ABs gone
sinopulmonary infection
GI (salmonella, campylobacter)
withdrawal to ____ presents with irritability, a high pitched cry, poor sleeping, tremors, seizures, sweating, sneezing, tachypnea, poor feeds, vomiting, diarrhea
opiates
patients with sickle cell disease are at risk for ____ disease due to increased ____ secondary to chronic anemia and what
cardiac disease, CO, infarction of myocardial microvasculature
renal involvement in HSP
can occur 4-6 weeks after illness
most pts have hematuria and some proteinuria
severe can lead to HTN and acute renal fialure
evaluation of primary amenorrhea steps
1) ____ or ____
uterus present then
pelvic exam or US
if uterus there then check FSH
if increased then get karyotype
if decreased then cranial MRI
SS disease aplastic crisis
reticulocytes and key features
decreased retics
transient arrest of erythropoiesis
secondary to infection of parvovirus B19
pruritic circular patch with central clearing and raised scaly border is what
what pathogen
tinea corporis
trichophyton rubrum
seborrheic dermatitis
clinical features: age, location, description
tx:
what species
peaks first year of life and adulthood
erythemaotous plaques and or yellow greasy scales
on scalp, face, posterior ears, nasolabial folds, umbilicus and diaper area
treatment: first line–> emollients, nonmedicated shampoos
2nd: topical antifungals or low potenency glucocort
malassezia species
wiskott aldrich syndrome
inheritance
increased risk of what infections
gene regualtes what
sx
tx
TIE
thrombocytopenia, Infections (recurrent) and eczema
x linked
infections of bacteria, viral and fungal
hematopoietic cells and regulates cytoskeleton remodeling, dysfunction of cell migration
cytoskeleton of platelets regulated by this too so can lead to petechiae or purpura, severe bleed, IC hem, hematemeis or hematochezia
tx: stem cell transplant
neuroblastoma
what cells arise
Nc cells which become sympathetic chains and adrenal medulla
calcifications and hemorrhages are seen on plain xray and CT
HVA and VMA usually elevated but no pheo sx
dep space neck infeciton most common in children under 4. high fever, muffled voice, and limited rotation of neck
retropharyngeal abscesses
deficiency of riboflavin (vitamin ___)
B2
cheilosis, glossitis, seborrheic dermatitis, pharyhngitis, and edema or erythema of the mouth
central lytic bone defect with surroudning sclerosis termed brodies abscess
osteomyelitis
classic triad of brain abscess
fever, severe headaches and focal neurologic changes
pasteurella mulocida caused by what
normal oral flora in dogs and cats that cuases cellullitis within 1-2 days of dog or cat bite
intraventricular hemorrhage is a common complication in neonates born at ___ weeks gestation or ____ grams
30 weeks or 1500 grams (3.3 lbs)
APGAR score under what may require further evaluation and resuscitation
7
treatment of obsessive compulsive disorder
cognitive behavioral therapy and/or SSRI (fluoxetine)
clomipramine or antipsychotic augmentation for treatment nonresponse
deep brain stim for tx of severe or refractory cases
most common spot for spondylolisthesis
L5 over S1
xray in persistent pulmonary HTN
clear lungs with decreased pulmonary vascularity
is it the toxin or the spore that is ingested in infantile botulinism?
how is it ingested
Spore
Environment, highest incidence in cali, penn, and utah. Leads to production and releaser of the toxin
Raw honey too
immune thromboycytopenia most common in what pts age
2 to 5 (b rush Is The Prohphet)
langerhans cell histiocytosis causes what
tx
solitary lytic long bone lesions
eosinophilic granuloma is least severe form and is in children and young adults as solitary bone lesion
usually resolve on own
evaluation of bilious emesis in the neonate
get abdominal x ray and see
free air, hematemesis, unstable vital signs then whats next
surgery bc this means it is pneumoperitoneum, which is intestinal perforation and need immediate emergency surgery
what should always be high on the differential for neonates (28 days or less) with decreased activity or poor feeding
temperature in these pts?
other signs
sepsis
may have either fever or hypothermia
mild jaundice, irritable, lethargic, hypotonic, abnormal WBC count high or low, left shift
diagnosing biliary atresia
tx
US, absent or abnormal GB
failure of liver to excrete tracer into the small bowel on scintigraphy is suggestive
cholangiogram is diagnostic gold standard
tx: liver transplant
why is bilirubin higher in newborns
hematocrit is 50-60% in newborns and has shorter life span so higher turnover
lower level of uridine diphosphogluconurate glucoronosyltransferase (UGT)
enterohepatic recycling is increased as the sterile newborn gut cannot break down bilirubin to urobilinogen for fecal excretion
tx of ITP
self limited in children and spont recovery in 6 momths so observe
only treat in kids if have cutaneous symptoms, IVIG or glucocorticoids
pt with AIS, what is treament of mass
cryptorchid gonads have 1-5% risk of devolping dysgerminoma or gonadoblastoma AFTER puberty
benefits from undergoing gonad stimulated puberty (attainment of adult height) outweigh low risk of malignancy
so wit to remove until after puberty
complications of cryptorchidism
alsmost all of these pts have associated what
inguinal hernia
testicular torsiojn]subfertility
testicular cancer
processus vaginalis, repair to prevent inguinal hernia
gonococcal conjunctivits
age presentation neonate
tx
prevention
age 2-5 days
one dose IM ceftriaxone or cefotaxime
erythromycin ointment
homocystinuria inheritance, what deficiency, sx besides marfinoid
AR, systathione synthase
intellectual disability, thrombosis, downward lens dislocaiton, megaloblastic anemia, fair complexion
adenitits due to m tuberculosis is strikingly what
nontender
Erythema marginatum
Erythematous ring like rash that comes and goes associated with acute RF
neonatal polycythemia
definition
causes
cx presentation
treatment
hematocrit over 65% in term infants
cause:
- increased EPO from intrauterine hypoxia: maternal DM, hypertension, smoking, IUGR
- erythrocyte transfusion: delayed cord clamping, t-t transfusion
- trisomy, hypo or hyperthyroid
sx:
- mostly asx
- respiratory distress, cyanosis, apnea, hypoglycemia, hyperbili
tx: IV fluids, glucose, partial exchange transfusion
epiglottitis cx features
management
tripod position (sit up and lean forward), sniffing position, stridor
drooling
manage with intubation and abx
tracheotomy if attempts at intubation fail
when give rotavirus vaccine
2-8 months
treatment for enterobius vermicularis (what worm)
pinworm
albendazole or pyrantel pamoate
Rash clasically begins with hearld patch, erythematous annular lesion on trunk which may increase in size and develop scaling around the edge. Within a week clusters of smaller oval red lesions appear on trunk. Scaly macules and papules distributed obliquely along the lines of tension in pattern on back
Can cause pruritis
What may precede this
Tx
Pityriasis rosea
Preceding viral prodrome
Self limited and resolves on its own, can use antihistamines and topical corticosteroids to help itching
avascular necrosis of the femoral head is seen in what children age
legg calve perthes disease which is idiotpathic avascular necrosis of femoral head, presents with limp or hip pain and is common in kids age 4-12
sickle cell diseae can cause too
nearly all pts with Cystic fibrosis dvlop what
sinopulmonary disease
neurofibromatosis type 1 tumor types and what can they causae so management
optic pathway glioma
holocranial HA, vision change, vomiting, and HTN
get MRI of brain and orbits
muscle biopsy with reduced dystrophin is what disease
becker MD
hyper IgM syndrome
immuno level, inheritance
sx
low IgA and IgG with elevated IgM
x linked
CD40 lignad defect
recurrent sinopulmonary infections with encapsulated bacteria
increased viral infections and opportunistic infections like PCP
what should you do if have urinary incontinence in child over 5
UA to screen for infection, DM, and DI
may consider acceleration of continence from alarm or desmopressin therapy
any neoate with delayed passage of meconium for 48 hours should be considered to have what disesae
CF
hirschsprungs
acute cervical adenitis in children
unilateral
bilateral
uni: staph, strep pyog, anaerobic bacteria, bartonella, mycobact avium
bilat: adenovirus, EBV/CMV
infants of diabetic mothers first trimester problems
congenital heart disesae
NT defects
small left colon
spont abortion
transient proteinuria in children is most common cause of what and how to evaluate
cause
most common cause of isolated proteinuria in children and should be reevaluated with a repeat urine dipstick test on 2 separate occasions to rule out persistent proteinuria
can be from fever, exercise, seizures, stress or dehydration
osteoid osteoma commonly affects what bone
clinical features
f/u
tx
proximal femur
increasing pain that worsens at night without relation to physical activity
relieved by NSAIDs****
follow with serial exam and x rays every 4-5 months
most go away on own
tay sachs or niemann pick has hyperreflexia
tay sachs
taylor allison is hyper what the hexosaminidase A is she doing?
tracheoesophageal fistula can cause what
permits air entry into the GI tract and stomach and intestines and they can become quite distended with each breath
gastric fluid can also reflux into the distal esophagus through the fistula and into the trachea and lungs causing aspiration pneumonia —> crackles and x-ray inflitrates
common variable immunodeficiency
manifestations
dx
management
manifestations: recurrent respiratory and GI infections
autoimmune disease
chronic lung disease and GI disorders
dx: decreased IgG and IgA/IgM, no response to vaccines
IG replacement therapy
during the first week of life the normal number of wet diapers a day should be what
equal the amt of the days old the infant is
4 day old infant should have 4 or more wet diapers
cat like cry is seen in what syndrome
characterisitc ____ present
other features
cri du chat 5p deletion
protruding metopic suture present
hypotonia, short, hypertelorism, wide and flat nasal bridge and dumb
most life treatening finding in marfan syndrome is what
aortic root dilation and aortic regurgitation
monitor for aneurysms and aortic arch dissection
common variable immunodeficiency abnormalty
increased risk what infections
other cell lines
growth and other disease
abnormal B cell differentiation results in def of multple IG classes
incrased risk encapsulated organisms and Giardia lambilia infection
sometime T cell immunity affected, so pts at increased risk for enteroviral encephalitis
FTT and chronic rep problems (asthma common)
the majority of infants with congenital toxo are asx at birth but experience ___ in adulthood
chorioretinitis
osteogensiss imperfecta is caused by mutations in what
type 1 collagen
____is ubiquitous where and neonatal __ can result when at risk infants are exposed to it during unhygienic delivery or cord care practices
clostridium tetani, in soil
tetanus
type of arthritis with lyme
monoarticular or asymmetric oligoarthritis most commonly involving the knee
pyloric stenosis is most commonly seen in what pop and what kind of vomit and findings
how dx
boys age 1-2 months
non bilious projectile vomit
visible peristaltic waves and palpable abdominal mass
abdominal US
biliary cyst is what
sx
pts by age
dx
can turn to what so tx
congenital dilation of the biliary tree
PAIN, JAUNDICE, PALPABLE mass (PAP got that bily cyst)
pts usually under 10
- infants have jaundice and acholic stools
- older kids can have pancreatitis
dx: US or ERCP if obstruction suspected
can turn to cholangiocarcinoma and need surgical resection
normal sex behavior in school age kids
AIM to know the most
asking questions about sex and repro
Increased intrest in sex words and play
masturbatory movements
in sickle cell pts the predominant hemoglobin type is HbS but HbF concentration is elevated at baseline to what? compared to healthy individuals
-so if give hydroxyurea the % of HbF should be above what
5-15% baseline
hydroxyurea should raise it above 15%
cx features of HSP
palpable purpura, hematuria, abdominal pain, arthralgias, and occasionally scrotal swelling
pt with a scrotal mass that is cystic and tranilluminate with light
what is this, embryology, and management
hydrocele
fluid in processus or tunica vaginalis (peritoneal projection that accompanies the testis during descent into scrotum. when it fails to obliterate, periotneal fluid may accumulate within making a communicating hydrocele. if fluid collects then obliteration then noncommunicating hydrocele)
differs from other testicular mases bc of transillumination
most resolve spontaneously by age of 12 months, if notthen may need surgery
lead tox in houses built before what year
1978
only test if sx
tetralogy of fallot consists of what
murmur?
VSD, RV outflow tract obstruction (pulmonic stenosis) overriding aorta and RV hypertrophy
DROP
harsh systolic ejeection murmur L upper sternal border
infant with hypothermia, lethargy, and low white blood cell count with left shift is highly concerning for what
also full fontanelles and apnea is concerning for what
neonatal sepsis
meninigitis
17a hydroxylase def hormonal abnormalities and sx
hormonal
decreased cort and test
increased mineral corticoids
all pts phenotypically femal, fluid and salt retention and HTN
____ is used in combination with desmopressin to incrase bladder capacity in children with daytime incontinence
oxybutynin
primary nocturnal enuresis definition
urinary incontinence that occurs at least twice a week age 5 or older
things that increase risk of otitis externa
conditions that disrupt skin barrier (eczema, psoriasis)
retain foreign material and water in canal (headphones, hearing aids, diving caps)
what is the most dangerous SM manifestation in myotonic muscular dystrophy
cardiac involvement?
other manifestations?
dysphagia can lead to aspiration pneumonia
cardiac involvement includes conduction problems and arrhytmias
cataracts, testicular atrophy/infertility, frontal baldness, and insulin resistance, foot drop, facial weakness
congenital lymphedema in what pts and is what exactly
and can lead to what
turner, lead to cystic hygroma of neck and fetal hydrops
dysgenesis of the lymphatic network
protein rich interstitial fluid in the hands, feet, and neck
diffuse intracranial calcifications in baby
dx
get how
tx
congenital toxo
undercooked meat
pyrimethamine, sulfadizine, folate
aspergillus fumigatus is common in _____ and ____ with CF and treatment is what
older children and adults
itraconazole and voriconazole
evaluation of neonate with suspected sepsis
CBC, culture, lumbar puncture, UA, and urine culture
empiric antibiotics
ampicillin and gentamicin after cultures
ITP happens how and cx features
PS?
after viral infection get autoantiboides that bind to platelets and cause purpura and petechiae, sometimes muscosal bleeds
thromboyctopenia and megakaryocytes on PS
how to confirm lyme disesae
organism
test
tx
spirochete
ELISA and Western blot testing
doxy or amoxicillin with no neuro sx
triggers of generalized seizures
sc features
fever, hypoglycemia, sleep deprivation
+/- aura
LOC, convulsions, postictal state
what is the threshold for phototherapy in a full term healthy 4 day old infant
total billy of 20 or more
transfusion if 25 or above and neuro dysfunction from the hyperbilly
what is the most effective nonpharmacological treatment for tourettes disorder
habit reversal training
the most common pathogen isolated from sputum cultures in infants and young children with CF is what
when is the other one most common
s aureus
pseudomonas is more common after age of 20
h flu meningitis tx with what abx
reduces risk of what
dexamethasone
sensorineural hearing loss
what is the leading cause of death in infants under 1
injury with abusive head trauma
what reduces symptoms in TOF and how
squatting may reduce symptoms by increasing SVR and afterload which decreases the right to left shunt across VSD but increases flow across the right ventricular outflow tract obstruction, increasing intensity of systolic murmur
preseptal cellulitis
mild infection of eyelid anterior to the orbital septub
eyelid edema, redness, tenderness, maybe fever and leuko
most common brain tumors in children is what
sx
pilocytic astrocytoma
supratentorial location: seizures, weak, sensory change
posterior fossa location: cerebellar dysfunction
ICP too
ependymomas found where and cause what
ventricles in posterior fossa
obstruction of CSF and increased ICP
hemophilia A and B inheritance
x linked recessive
antidote to methemoglobinemia
methylene blue
accepts electrons for NADPH and is reduced to leucomethylene blue which in turn reduces methemoglobin back to hemoglobin
splenic sequestration crisis
acute anemia, splenic engorgement
cx: tachy, pallor, LUQ pain, splenomegaly
can progress to hypovolemi shock as lots of blood trapped in spleen
lab findings: normocytic anemia, compensatory elevated reticulocyte count, and THROMBOCYTOpenia
tx: fluid, transfusions, and eventual splenectomy
cutis aplasia (absence of epidermis over the skull) and microphthalmia are both classically seen in what?
__ defects too
patau syndrome
midline defects like holoprosencephaly and omphalocele
does cat scratch disease need antibiotic prophylaxis ?
not in immunocompetent people
in ICP yes
comorbidities with duchenne musc dyst and becker and myotonic
DMD: scoliosis and cardiomyop
becker: cardiomyop
myotonic: MD: CAT Bald
- cataracts, arrhymias, testicular atrophy/infertility, baldiing
deep brain stimulation for tx of severe or refractory cases of ___ target what area in brain
OCD, nucleus accumbens
recurrent UTIs in infants and children is often from what
dx
what to evaluate long term renal scarring
follow renal function
monitor pts closely for what complications
vesicoureteral reflux
dx: voiding cystourehtrogram (VCUG), then renal US to see hydronephrosis
reanl scintigraphy with dimercaptosuccinic acid to evaluate renal scarring long term
renal function followed by seriel cr
pts monitored closely for complications of chornic renal insufficiency, like HTN and anemia
what would you see on a muscle biopsy in someone with doucheanes muscular dystrophy
what about beckers
absent dystrophin
becker would show reduced dystrophin
clinical manifestations of refeeding syndrome
arrhythmias
CHF
seizures
wernicke encephalopathy
phosphorus K,MG uptaken
phosphorus is primary deficient electrolyte for nrg
cardiac arrhythmias from mg and potassium
most common cause of subacute unilateral LAD in young children is from what
mycobacterium avium
risk factors for brain abscess
OM, mastoiditis
frontal or ethmoid sinusitis
dental infection
bacteremia from other sites of infection, cyanotic heart disease (TOF)
Spinal muscular atrophy (werdnig-hofffman disease) cx by what
Symmetric proximal m weakness and hyporeflexia
Does not affect the pupils
Greater weakness in lower extremeties
all sexually active women under age what should undergo testing for chlamydia and gonorrhoeae
25
septic arthritis of the hip in children
tx
septic arthritis has fever of 101 or more
cannot bear weight
WBC count over 12,000
ESR over 40
CRP over 2
if have 3 or more of above then immediate arthrocentesisis
what tumor can aphasia and hemiparesis occur in
pts with brain tumors in cerebral hemispheres
most common in children is low grade astrocytomas
disease with low IgG, IgM, and IgA and low or absent B lymphocytes
bruton agammaglobulinemia
low MAG
galactocerebrosidase deficiency
retard, blind, deaf, paralsis, neuorpathy, seizures
krabbe disease
id be krabbe if i was deaf, dumb, blind, and paralyzed
treatment for cat scratch disease
another name
sx
bartonella henselae
azithromycin
localized popular or nodular skin lesion that may go unnoticed
fever of unknown origin and or regional LAD
phsyical exam in older children with x linked agammaglob shows what
underdeveloped lymphoid tissue (tonsils and LNs)
laryngomalacia is caused by what and sx
what makes worse
colapse of supraglottic structures during inspiration
choronic inspiratory stridor that begins in neonatal period and is worse in supine position
hypertoiprhied peyer patch can lead to what
intussusception
happens in AGE
treatment for croup (laryngotracheitis) and why it works
parinfluenza virus
causes tracheal inflammation leading to subglottic edema and narrowing
mild cases use single dose corticosteroids (dexamethasone)
if mod or severe use corticosteroids and nebulized epinephrine (constricts mucosal arterioles in upper airway and alters cap hydrostatic pressure)
Breast milk jaundice
timing
pathophysi
cx features
starts days 3-5 and peaks at 2 weeks
high levels of b glucoronidase in breast milk deconjugates intestinal bilirubin and increases its circulation
adequate breast feed and normal exam
pts with tourettes syndrome have high rates of comorbid conditions like what
ADHD and OCD
precocious puberty uworld
look up
risk factor for development of respiratory distress syndome
what decreases risk
prematurity
maternal diabetes mellitus, increased insulin antagonize cortisol and block the maturation of sphingomyelin
IUGR, mathernal HTN decrease risk
what is the most common pathogenic organism in young children with CF? especially in the concurrence of ___ infection
staphylococcus aureus
influenza
chediak-higashi syndrome inheritance and characteristics
AR
oculocutaneous albinism and recurerent cutaneous infections with staph aureus and srtrep pyogenes
growing pains happens in what age group?
2-12
Age group for osteod osteoma?
10-20
