Misc Flashcards
0
Q
2 very specific auto antibodies in lupus
A
dsDNA
Smith
1
Q
SSA/SSB associated with what 2 things
A
Sjogrens
Neonatal lupus
2
Q
RNP autoantibody associated with
A
MCTD
3
Q
Young boy with lupus: think these 2
A
Klinefelters
Complement deficiency
4
Q
Poisoning: plastics company, dysrrhythmia, sever metabolic acidosis
A
Cyanide
Give antidote kit or hydroxocobalamin
5
Q
paraquat poisoning is often due to suicide attempt and causes ____
A
ARDS
6
Q
pneumonia at birth, HSM, jaundice, rashes, hemolytic anemia
what is this?
A
congenital syphilis
7
Q
c section baby
tachypnea
xray shows retained fluid in the fissures
A
transient tachypnea of the newborn
8
Q
context of chlamydia pneumonia
A
mild pna develops in an exposed infant at several weeks of life
9
Q
what to do for strabismus in a 3 month old
A
refer immediately to ophtho
10
Q
most common cause of pediatric orbital cellulitis
A
local spread from places like the paranasal sinus
11
Q
orbital vs. periorbital/preseptal cellulitis management
A
orbital- admit for IV abx
periorbital- outpatient PO abx
12
Q
delayed umbilical cord separation, think _____
A
leukocyte adhesion deficiency
- leukocytosis
- risk for overwhelming bacterial infection
- antibody production is normal
- dx with flow cytometry
13
Q
antidote for western diamondback rattlesnake
A
CroFab
14
Q
antidote for arsenic
A
dimercaptosuccinic acid
15
Q
antidote for clonidine overdose/poisoning
A
naloxone
16
Q
which bites definitely need abx ppx
A
cat, monkey, human
17
Q
long face, large ears, prominent jaw, macroorchidism, hypotonia, repetitive speech, gaze avoidance, hand flapping
A
fragile X
18
Q
hypotonia, epicanthal folds, simian crease, cardiac lesions (VSD or AV canal), MR, propensity for leukemia
A
trisomy 21 (Down syndrome)
19
Q
SGA, micrognathia, low set ears, cardiac defects, small palpebral fissures, microcephaly, cleft lip/palate, rocker bottom feet
A
trisomy 18 (Edwards syndrome)
20
Q
microcephaly, cutis aplasia of the scalp, cardiac defects, holoprosencephaly, cleft lip/palate, coloboma
A
trisomy 13 (patau syndrome)
21
Q
short stature, blue irides, cardiac abnormalities involving the pulmonary vessels, hypercalcemia in infancy, friendly attitude
A
williams syndrome
22
Q
small sharply edged lesions that occur most commonly on head and neck of infants
-yellow orange, slightly elevated, hairless
A
sebaceous nevi
23
Q
AA baby with 1-2mm pustules that rupture –> hyperpigmented lesion encircled by a collarette of scale
A
pustular melanosis
24
deficiency: cheilosis, glossitis, ocular problems, seborrheic dermatitis
riboflavin deficiency
25
seizure, peripheral neuritis, dermatitis, microcytic anemia are seen in -_____ deficiency
vitamin B6 (pyridoxine)
26
megaloblastic anemia, glossitis, pharyngeal ulcers, impaired immunity suggest ______ deficiency
folate
27
biotin deficiency causes ______
dermatitis/seborrhea
28
bushy eyebrows, hirsutism, limb defects, VSD, MR
cornelia de lange syndrome
29
treatment of lead poisoning if severe
DMSA and calcium EDTA
30
aspirin/salicylate poisoning treatment
acetazolamide, IV Na bicarb +/- dialysis
31
organophosphate poisoning tx
atropine then pralidoxime
32
iron poisoning tx
deferoxamine
| -causes red/vin rose urine
33
dimercaprol (BAL) for _____ ingestions
heavy metal
34
frothy malodorous vaginal discharge assoc with ____
| how to tx
trichomonasl
| metronidazole or tinidazole
35
tx for cyclospora diarrhea
bactrim then cipro
| it's a nonbloody diarrhea
36
what is diphyllobothrium latum, how does it present, how to tx
tapeworm
can present with B12 deficiency
tx with praziquantel
37
Li during pregnancy causes _____
ebstein anomaly
38
ACEI during pregnancy causes ______
renal dysgenesis, oligohydramnios, skull ossifications defects
39
isotretinoin during pregnancy causes _____
hydrocephalus, CNS defects, microtia/anotia, small or missing thymus, conotruncal heart defect, micrognathia, fetal death
40
congenital rubella syndrome
deafness, cataracts, MR, and heart defects
41
returns from Central America with pruritic lesion on foot; raised, red, serpiginous, few associated bullae
what is it and how to tx
cutaneous larva migrans
| tx with ivermectin (antihelminth)
42
treatment for cradle cap (seborrheic dermatitis)
topical steroids or selenium sulfide-containing product
43
meds that can cause a photoallergic eruption include ____ and _____
griseofulvin
| tetracycline
44
what med do you use to close a PDA
indomethacin
45
babies born to moms with lupus are at risk for this heart condition
heart block
46
kids on phenytoin should get this vitamin supplement
folate
47
in the developing world, give this vitamin to kids with measles
vitamin A
48
breastfed infant should get this vitamin
vitamin D
49
sickle cell patients should get this vitamin
folate
50
newborns get this vitamin
vitamin K
51
immunodeficiency: multiple abscesses
| what is it and how to dx
chronci granulomatous disease
| NBT/DHR
52
patient with weird infections and severe eczema
| what is it, and what lab would you want
wisott aldrich syndrome
low platelets
-also eosinophilia and elevated IgE
53
kid with recurrent sinusitis, otitis, osteomyelitis
no lymph nodes and tonsillar tissue
what might this be and how do you diagnose
B cell defect- send serum Ig levels
54
```
frequent infections
loud systolic murmur
posteriorly rotated ears that are small and low set, down slanting and widely spaced eyes, small jaw, upturned nose
hypocalcemic seizures
what is this and how to dx real quick
```
digeorge syndrome
| do intradermal skin test with Candida albicans- no response in kids with T cell deficiencies
55
protruding tongue, brushfield spots, neck webbing, MR, brachycephaly, upslanting palpebral fissures, epicanthal folds, flat face, small ears, cardiac issues, palmar creases, clinodactyly of 5th digit
trisomy 21 (Down syndrome)
56
which things increase risk of neuro damage in jaundiced infants
which things decrease the risk
increase: neonatal sepsis
decrease: metabolic alkalosis, increased attachment of bili to binding sites caused by drugs like sulfisoxazole, hyperalbumin, maternal ingestion of phenobarb during pregnancy
57
how to tx choanal atresia
surgery and placement of nasal tubes
58
when to give varicella zoster immunoglobulins to newborn babies
when mom has it within 5 days prior to delivery
| when mom is diagnosed with it within 2 days after delivery
59
contraindications to breastfeeding
active TB
HIV
if taking antineoplastic agents, Li, cyclosporine, cocaine/heroin, amphetamines, ergotamines, bromocriptine, tetracycline
60
asphyxia at birth puts babies at risk for _____
persistent pulmonary HTN of the newborn
61
mom who has one congenital CMV kid... what about getting pregnancy again?
mother has antibodies to CMV that are passed to the fetus and it will most likely be fine
62
if neonate passes a bloody meconium in the setting of marginal placental separation, what test do you run?
apt-downey test
- can tell difference between fetal vs. maternal hemoglobin
- if it's the mom's blood from the delivery, then that's fine
63
premie presents with distended abdomen, feeding intolerance, bloody stool
what is it and what to do
NEC
get a KUB -->
-if positive but no perf, then stop feeds, start IVF, order serial abdominal films, and start systemic abx
-if positive and perfed, then ex lap immediately
64
if two babies are premie and one is normal weight and one is SGA, what are the outcomes?
SGA baby has a higher risk of congenital malformations
65
how to tx galactosemia
stop breastfeeding and start soy based formulas
66
what is Erb-Duchenne palsy
C5 and C6
arm cannot be abducted, externally rotated at the shoulder, and forearm cant be supinated
waiter's tip
67
what is Klumpke's palsy
C7, C8, T1
| palsy of the hand, Horner syndrome
68
subgaleal hemorrhage feels like a cephalohematoma that crosses the midline
when rapidly expanding and baby is tachy, what to do?
send baby to NICU for obs +/- fluid resuscitation
69
tremulousness and jitteriness that can progress to seizure activity 2/2 sludging of blood in the cerebral microcirculation
-what is it and how to tx?
```
hyperviscosity syndrome (2/2 polycythemia)
tx with partial exchange transfusion with NS or LR
```
70
severe purulent conjunctivitis within 5 days of birth
| what is it and how to tx
GC conjunctivitis
topical and systemic abx
give ppx with erythromycin upon birth if mom has it
71
conjunctivitis within 5-14 days of birth
| what is it and how to tx?
chlamydial conjunctivitis
tx wit systemic abx
*however, asymptomatic infants born to chlamydia positive mothers are not routinely treated with oral abx at birth as ppx (in contrast to GC moms)
72
increased incidence of ______ in neonates receiving erythromycin
hypertrophic pyloric stenosis
73
how to reduce HIV infection from being vertically transmitted
anti-retrovirals for mom
a course of zidovudine to the child
-no need for special monitoring or routine radiographs
74
```
low weight
short stature
edema over dorsum of hands and feet
loose skin folds at nape of neck
shield chest
coarctation of aorta
horseshoe kidney
```
Turner syndrome
75
baby being born to a mother with chronic Hep B (HBsAg positive)
what to do?
give hep B Ig and hep B vaccine to the baby
76
```
which of the following when given to the mom is most likely to cause harm in a newborn infant at delivery:
propranolol
pcn
Al hydroxide
phenytoin
heparin
```
propranolol
77
if neonatal jaundice is due to sepsis, you should see _____
increase in both direct and indirect bili
78
goat milk does not have enough ____ and ___
folate and iron
79
complications of cleft palate/lip
recurrent otitis media and hearing loss, speech defects
80
when to repair cleft palate and lip
lip- 2-3 months
| palate- 6 months-5years
81
transient tachypnea of the newborn often occurs in babies who ______
are born via scheduled c section... 2/2 retained fluid in the lungs
82
baby with HSM, anemia, persistent, rhinitis, maculopapular peeling rash on face, palms, and soles
congenital syphillis
83
in twin to twin transfusions, what happens to the donor twin and what happens to the recipient twin?
donor twin- oligohydramnios, anemia, hypovolemia
recipient- polyhydramnios, plethora, larger, hyperviscosity, respiratory distress, hyperbili, hypocalcemia, renal vein thrombosis, CHF, convulsions
84
a _________ natal too requires further investigation
midline or markedly pointed
85
duodenal atresia, TE fistula, and trisomy 18 can cause ______hydramnios
renal agenesis can cause ______hydramnios
poly
| oligo
86
longitudinal striations in the metaphyses are characteristic of congenital ______
osteochondritis or periostitis usually indicates congenital ______
rubella
| syphilis
87
newborn with bilateral cataracts, micophthalmia, IUGR, hemorrhagic skin lesions scattered throughout body (blueberry muffin), harsh systolic murmur heard at left sternal border radiating to the lung fields
congenital rubella
88
newborn with hydrocephalus, chorioretinitis, intracranial calcifications, anemia
congenital toxo
89
newborn with microcephaly, intracranial calcifications, HSM, marked hyperbili and thrombocytopenia
can also have sensorineural hearing loss
congenital CMV
90
widely spaced eyes, low set ears, broad nose, receding chin, limb abnormalities
what is this and what does it put you at risk for
oligohydramnios (potter sequence) due to bilateral renal agenesis
pulmonary hypoplasia
91
tx for biotinidase deficiency is lifelong administration of ______
biotin (a vitamin)
92
maple syrup urine disease requires limiting intake of _____
leucine, isoleucine, and valine (AAs)
93
aniridia, GU anomalies, and hemihypertrophy most often associated with ____
Wilms tumor
94
lateral displacement of medial canthi, broad nasal bridge, medial hyperplasia of the eyebrows, partial albinism (white forelock or heterochromia), deafness
waardenburg syndrome- AD inherited
95
sturge weber with port wine stain puts you at risk for _____
seizures, mental deficiency, hemiparesis or hemianopsia
96
hypopigmented oval or irregularly shaped skin macules (ash leaf spots), cerebral growths, myoclonic seizures, adenoma sebaceum
-AD inheritance
tuberous sclerosis
97
shaken babies tend to have _____ hemorrhages in the brain
subdural
98
P anca associated with what
Churg Strauss
99
C anca associated with
Wegeners granulomatosis
100
Most specific antibody for lupus
Anti smith
101
Antibody that measures lupus activity
Anti dsDNA
102
NBD and DHR tests are for what
Chronic granulomatous disease
103
Jones criteria for rheumatic fever
| which one is most frequent?
```
joints- polyarthritis ** most frequently seen
heart- carditis
nodules
erythema marginatum
sydenham chorea
```
104
how to tx tet spells if severe
most of the time, they are self limited and squatting helps
| can give morphine and propranolol or sodium bicarb if super acidotic
105
Down syndrome is assoc with these two cardiac things
VSD
| endocardial cushion defects
106
hydrops of the gallbladder is assoc with ______
KD
107
MCC myocarditis in kids
adenovirus
| coxsackievirus
108
Osler nodes- small tender nodules in the tips of fingers and toes
janeway lesions- nontender hemorrhagic lesions on the hands and feet
splinter hemorrhages- dark lines under nails
uncommon findings of infective endocarditis
109
MCC organisms causing infective endocarditis in kids
strep viridans and staph aureus
110
what to give if cardiac massage doesn't work for SVT
IV adenosine
111
what is quadruple rhythm (S3 and S4 present) associated with
ebstein anomaly
- RA hypertrophy
- RV conduction defects
112
this is the one cyanotic congenital heart disease that causes LVH and left axis deviation
tricuspid atresia
| most of the other cyanotic congenital heart diseases cause RVH and right axis deviation
113
- 3 day old with single S2, progressive deepening cyanosis since birth, no respiratory distress
- CXR: no cardiomegaly, normal pulmonary vasculature
- EKG: right axis deviation
TGA
114
thrombocytopenia, bilateral absence of radius, abnormally shaped thumbs
what is it, what heart stuff is associated
thrombocytopenia absent radius (TAR) syndrome
| TOF and ASD
115
short stature, normal weight, shield chest (guy), cryptorchidism, low set and malformed ears, ptosis, pectus excavatum
what is it and what cardiac issue?
```
noonan syndrome (male version of turner)
pulmonic stenosis
```
116
inherited long QT associated with ________ in _______
| not associated with ______ in ______
deafness in jervell lange nielsen- AR
| romano-ward syndrome- AD
117
in hemiparalysis of the diaphragm, CXR might be normal so you order ______
chest ultrasound
118
numerical cutoffs for sweat chloride test
< 40 is normal
| > 60 is diagnostic
119
how to tx gasoline, kerosene, and furniture polish aspiration
hydrocarbons
| symptomatic tx sometimes with intubation and MV
120
adult in the house has active TB
| what to do with the child who has pulmonary signs and what to do with the baby?
baby- 3 months of isoniazid then PPD
| child- 6 months of INH, rifampin, and pyrazinamide
121
what appears to be recurrent lobar pneumonia in kids, hemoptysis, rapid clearing of radiographic findings
what is it and how to dx
idiopathic pulmonary hemosiderosis
| bronchoalveolar lavage reveals hemosiderin laden macrophages
122
bacterial tracheitis vs viral croup
| how to tx each
croup does not have high fever and usually only has inspiratory stridor
tracheitis tends to have high fever and both inspiratory and expiratory stridor
tracheitis- intubation, IV abx
croup- inhaled epi and oral steroids
123
-thumb sign on lateral neck xray
-drooling, dysphagia, muffled voice, sitting forward with neck hyperextended
what is it and what to do
epiglottitis- used to be HIB but now GABHS, moraxella, or strep pneumoniae
-intubation in the OR with skilled personnel
124
kid eats dirt and gets difficulty breathing and eosinophilia
| what test to send
ELISA for toxocara
| cannot dx with stool ova or parasites b/c it migrates out of the intestines and does not return
125
which sinuses drain into middle meatus
| which sinuses drain into superior meatus
middle meatus- maxillary, frontal, anterior ethmoid
| superior meatus- sphenoid or posterior ethmoid
126
developmental timeline of sinuses
maxillary and ethmoid in infancy
frontal 6-10 years
sphenoid 3-5 years
127
situs inversus
chronic sinusitis and otitis media
airway disease
primary ciliary dyskinesia, kartagener syndrome
128
h/o pharyngitis, abrupt onset of fever, refusal of food, drooling, muffled/noisy breathing
what is it and how to tx?
retropharyngeal abscess
| -I&D if fluctuant
129
how to tx functional constipation
dietary changes, counseling on proper toileting behavior
| can try stool softeners
130
curly Q on barium enema indicates _____
malrotation and volvulus
131
echogenic bowel on prenatal ultrasound
xrays show dilated loops of bowel
enema shows mircocolon
meconium ileus indicating CF
132
two molecular markers seen in Crohn's vs. UC
Crohn's- ASCA
| UC- p-ANCA
133
intractable chronic constipation w/o fecal soiling
| what is it and how to dx
hirschsprung
| dx with Ba enema and rectal manometry
134
how to dx and tx intussusception
air contrast enema
135
currant jelly stools and sausage shaped mass in RUQ associated with what
intussusception
136
sandifer syndrome
neck arching caused by gastroesophageal reflux
137
tx for giardia
single dose tinidazole
3 days of nitazoxanide BID
5-7 days of metronidazole TID
138
painless GI bleeding in a 2 year old
most likely meckel's diverticulum
139
how to dx lactose intolerance
hydrogen breath test
| acidic stool pH in the presence of reducing substances
140
doughy skin is seen in what kind of dehydration
hypernatremic
141
hyperglycemia can be seen in _____ dehydration
hypernatremic
142
what to do with congenital indirect inguinal hernias in babies
consider getting surgery before discharge
143
activated charcoal is not useful for the following:
```
alcohols
acids
ferrous sulfate
strong bases
cynaide
lithium
K
```
144
activated charcoal is useful for the following
phenobarbital
TCAs
sustained release theophylline
145
what is sorbitol-MacConkey agar used to culture
Enterohemorrhagic E Coli (produces shiga-like toxins) found in poorly cooked beef
146
exclusive cow's milk intake can lead to _____ deficiency
iron
147
night blindness, dry skin, conjunctiva, and sclera
vit A deficiency
148
exclusively breast fed infants should get ______ supplementation
vitamin D
149
how to dx GERD
esophageal pH probe
150
what to do for labial adhesions in pre-pubescent girl
observe or can use estrogen cream
151
lax wrinkled abdominal wall
dilated urinary tract
intra-abdominal testicular tissue
prune belly syndrome
152
to to manage central nephrogenic DI
maintenance of adequate fluid intake and diet
| use of saluretic meds
153
anorexia, polydipsia and polyuria, vomting, unexplained fevers, glucosuria but normal blood sugar, abnormally high urine pH in the face of mild or moderate serum hyperchloremic metabolic acidosis, mild albuminuria in the presence of normal serum protein and albumin
fanconi syndrome (global proximal tubular dysfunction)
154
RTA1 (distal) and RTA4 both have positive ______
urine anion gap
155
how to tx acute lobar nephronia
prolonged course of antibiotics
156
isolated facial nerve palsy can be the sole manifestation of severe HTN in children
yep
157
rheumatogenic strains of GAS are only associated with ______
| nephritogenic strains of GAS are associated with _____ and _____
pharyngitis
| pharyngeal or skin infections
158
hereditary hearing loss, hematuria esp after URIs, ESRD common by 2nd-3rd decade of life, ocular abnormalities
alport's syndrome
159
Bartter syndrome
| lab values and how to tx
hypokalemia, hypercalciuria, alkalosis, hyperaldosteronism, hyperreninemia
normal BP
tx by preventing dehydration, nutritional support, normalize K
160
where is the gene for malignant hyperthermia
| how to dx and tx
chromosome 19, ryanodine receptor
dx with caffeine contracture test
tx with dantrolene
161
what to do with the urine in aspirin overdose
acidification of the urine helps
162
demyelinaton in the CNS after an infection in kids < 10 years old
what is it and how is it tx
acute disseminated encephalomyelitis (ADEM)
- it's an autoimmune demyelinating disease that resembles MS in history and physical and imaging (white plaques scattered) but occurs in young children, has systemic findings like fever and emesis
- tx with high dose steroids
163
tuft of hair over the spine indicating possible occult spinal dysraphism --> what history and physical exam findings might you see
what to do next
frequent UTIs
lower extremity hypotonia and muscle atrophy
-get imaging with CT, MRI, or US
164
this metabolic disease can cause poor growth and acute infantile hemiplegia
homocystinuria 2/2 thromboembolic events
165
what's the ocular icepack test
test for myasthenia gravis
| put an ice pack on the affected eye --> that eye should elevate within 5 minutes
166
what is acute infantile hemiplegia
essentially a stroke in kids
167
cherry red spot
sensitivity to noise/startles easily
what is it and what can you find on labs
tay sachs disease- AR inherited (GM2 gangliosidosis)
| absence of beta-hexosaminidase A activity in WBCs
168
deficiency of both beta-hexosaminidase A and B
Sandhoff disease
169
normal child at birth then HSM, LAD, psychomotor retardation in the first 6 months, followed by regression after that
niemann-pick disease (type A) spingomyelinase
170
infant with increased tone, strabismus, organomegaly, FTT, stridor, several years of psychomotor regression before death
infantile gaucher disease
171
early infancy with irritability, seizures, hypertonia, optic atrophy, severe delay, death in first 3 years
krabbe disease- galactocerebroside beta-galactosidase
172
childhood with angiokeratomas in the bathing trunk area --> severe pain episodes
frequent low grade fevers
sometimes cataracts
fabry disease- alpha galactosidase reduced activity
173
distal muscle weakness in childhood, elevated CK, what is it and what else should you look for and how to dx
myotonic muscular dystrophy (unlike most muscle problems, it causes distal weakness)
- ptosis, baldness, hypogonadism, facial immobility with distal muscle wasting, cataracts
- dx with molecular blood test
174
what do you see on contrast head CT in TB meningitis
exudate in the basal cisterns that shows enhancement
175
subacute sclerosing panencephalitis (SSPE)
| when does it occur and what do you see?
after measles
| insidious behavior changes, deterioration in schoolwork, dementia
176
triplet repeat expansion on chromosome 9 makes you think of ____
Friedreich's ataxia
177
abnormal neuro exam at birth --> over next few weeks, progressive CNS degeenration, enlarged liver and spleen, macroglossia, coarse facial features, cherry red spot, gingival hyperplasia, hernias, joint stiffness, dorsal dyphosis, edema of extremities
complete lack of acid beta-galactosidase activity
| GM1 generalized gangliosidosis type 1
178
normal child at birth, rapid decline in motor and cognitive functions age 6-18 months of age
affected girls lose use of hands, communication, socialization
Rett syndrome- MECP2 gene on the X chromosome
179
onset 1-2 years
progressive ataxia, weakness, peripheral neuropathy
gray macular lesions
metachromatic leukodystrophy- deficient activity of galactosyl-3-sulfateceramide sulfatase
180
cherry red spot
GM1 generalized gangliosidosis type 1
sandhoff disease
niemann pick disease type A
tay sachs disease (GM2 gangliosidosis)
181
GBS tx
IVIG, steroids, plasmapheresis
182
anterior horn disorder that presents either in utero or by first 6 months of life with hypotonia, weakness, delayed milestones
werdnig-hoffmann disease/SMA type 1
| SMN gene problem
183
calcifications in CMV vs. toxo
CMV- periventricular
| toxo- scattered throughout the cortex
184
bullous eruptions that become hyperpigmented lesions, seizures
incontinentia pigmenti
185
milkmaid grip, emotional lability, hypotonic, darting tongue
sydenham chorea
186
chromosome of NF1
17
187
lisch nodules (hamartomas of the eyes) found in _____
NF1
188
PHACE syndrome
```
posterior fossa malformations
hemangiomas
arterial anomalies
coarctation of the aorta
eye abnormalities
```
189
shagreen patch (roughened, raised lesion over the sacrum)
tuberous sclerosis
190
white pinpoint lesions on a bright red buccal mucosa in the area opposite lower molars
koplik spots of measles
191
- mild sore throat and malaise --> adherent membrane covering the tonsils and extending to cover the uvula, palate, posterior oropharynx, hypopharynx, and glottic area
- LAD and soft tissue swelling --> bull neck and maybe airway compromise
diphtheria (corynebacterium diphtheria)
192
infant born to HIV + mom
| what do you do while you await final determination of the kid's status
bactrim for PCP ppx starting at 6 weeks
193
mitochondrial hepatopathy due to interaction of influenza or varicella infection and aspirin use
- high mortality
- elevated liver enzymes and ammonia (bili is normal)
- seizures, coma, hyperventilation, decorticate posturing
- death from cerebral edema and subsequent herniation
reye syndrome
194
seizure followed by diarrhea with mucus and blood
Ekiri syndrome- rare and fatal toxic encephalopathy
what is this, how to dx, how to tx
shigella
dx with stool culture
supportive care, 3rd gen cephalosporin abx
195
how to tx alopecia areata
exclamation hair
| tx with UV light therapy, topical/intradermal steroids
196
roseola virus
HHV 6
197
hand foot and mouth disease
coxsackie A16 virus
198
echo 11 virus causes _________
viral meningitis
199
7 days of fever, chills, severe muscle pain, pharyngitis, HA, scleral injection, photophobia, cervical adenopathy --> seemed to get better --> then fever, nausea, emesis, HA, mild nuchal rigidity
-CSF shows monocyte predominant increased WBC and elevated protein
leptospirosis
most cases are mild or subclinical
two types: anicteric and icteric (weil syndrome)
200
anicteric leptospirosis course
-septicemic phase --> immune phase in which meningitic sxs return and can last up to 1 month
201
icteric leptospirosis course
-septicemic phase --> severe liver and kidney dysfunction
202
how to tx leptospirosis
PCN and tetracycline in kids > 10 years are good if started in the first week of sxs
203
antibiotic for pertussis
erythromycin
204
pertussis immunity: infection vs immunization
immunity 2/2 infection is lifelong
| immunity 2/2 immunization wanes... thus the Tdap boosters that adults get
205
diffuse adenopathy, tonsillar enlargement, enlargedspleen, small hemorrhages on soft palate, periorbital swelling, atypical lymphocytes
EBV- infectious mononucleosis
206
infant who appears normal who has a sudden onset of high fever and marked elevation and left shift of the WBC count
pneumococcal bacteremia
207
prolonged low grade insidious nasopharyngitis that sometimes occurs in infants infected with GABHS
streptococcosis
208
_____ is an infection of the nasolacrimal sac
dacryocycstitis
209
_______ is a firm, nontender nodule that results from a chronic granulomatous inflammation of the meibomian gland
chalazion
210
what's another word for measles?
rubeola
211
viral exanthem- high fever abruptly abates as a rash appears
roseola
212
mumps orchitis occurs mostly in ______ males
| mumps can also cause meningitis at the same time as parotitis or 10 days after parotitis
post-pubertal
213
after rabies exposure, do these things
wound cleansing
rabies Ig
five shot vaccine series
214
treatment for RMSF
< 8 years old
| 8 and over- doxycycline
215
thrombocytopenia, eczema, increased susceptibility to infection
wiskott-aldrich (X linked recessive)
216
how to tx lyme
< 8 amoxicillin
| 8 and over- doxycycline
217
what is enterobius vermicularis
pinworm- anal itching
218
what is DRESS
drug rash, eosinophilia, and systemic sxs
| -assoc with abx and AEDs
219
contraindications to LP in kids suspected to have meningitis
increased ICP in a kid with closed fontanelles
severe cardiorespiratory distress
skin infection at puncture site
severe thrombocytopenia or other coagulation disorder
220
barking cough
croup- parainfluenza
221
CATCH mnemonic is for ______
DiGeorge
cardiac, abnormal faces, thymic hypoplasia, cleft palate, hypocalcemia
T cell issues lead to issues with fungi, protozoa, acid fast bacteria, certain viruses
222
deficiency in all 3 classes of Igs
| what is it and how does it present
X linked agammaglobulinemia (Bruton, XLA)
-after 3 months of age with recurrent and often simultaneous bouts of otitis media, pnuemonia, diarrhea, and sinusitis but w/o fungal and viral infections
223
```
mild T cell dysfunction
low IgM
high IgA and IgE
eczema
recurrent middle ear infections
lymphopenia
thrombocytopenia
```
wiskott-aldrich (combined T and B cell disease)
224
disorder of phagocytic chemotaxis associated with hypergammaglobulin E, eczema-like rash, recurrent severe staph infections
Joe Buckley syndrome
225
ampicillin rash is seen in _____
EBV infectious mononucleosis
226
name the organism:
foot wound through a shoe
foot wound through bare foot
pseudomonas
| staph
227
thayer martin media is used to culture _____
gonorrhea
228
pneumonia 2/2 chlamydia shows _____ in peripheral blood
CXR shows ________
afebrile often
eosinophils
| hyperinflation with interstitial infiltrates
229
very serious pneumonia causing ptx in kids < 1 year
staph pneumonia
230
pleural effusions and empyemas often seen in _____ pneumonia in kids who are a bit older
strep pneumo
231
how to manage ITP
if mild sxs- observe
if significant bleeding- IVIG and steroids (??steroids before ruling out leukemia with bone marrow biopsy)
if Rh (+) pt with working spleen, give anti-D immunoglobulin
if sxs > 1 year, consider splenectomy
232
when iron deficiency anemia occurs, FEP _______ in the blood
accumulate
233
hemoglobin A2 is increased in _______
beta thal trait --> family counseling only as tx
234
who might you give daily folate and PCN
sickle cell anemia pts
235
how to tx lead poisoning
dimercaptosuccinic acid
236
what is the kasabach merritt phenomenon and how to tx
large vascular anomalies in new borns --> plt and RBC sequestration within the vascular tumor causes thrombocytopenia, coagulopathy, and MAHA
-tx with steroids, alpha interferon, vincristine
237
inheritance of G6PD deficiency
| what's tricky about testing
X linked recessive
-testing for the enzyme right after a hemolytic episode might give you a false negative b/c only the young RBCs with good amounts of enzyme survive the episode
238
how to tx cyclic neutropenia
rhG-CSF
239
when does sickle cell disease manifest
4-6 months of life when fetal hemoglobin is replaced with sickled hemoglobin
240
newborn with anemia that's not due to ABO or Rh incompatibility
fetomaternal transfusion
| do kleihauer-betke stain to look for fetal hemoglobin in mom's blood
241
EBV usually causes significant anemia and thrombocytopenia (T/F)
F
242
chromosome of wilms tumor
11
243
hereditary spherocytosis can cause neonatal hyperbili
T
244
in DIC, there is consumption the following (5 things)
fibrinogen
factors II, V, VIII
platelets
--> prolonged PT, PTT, TT; decreased factor VIII and plts; increased fibrin split products
245
PT tests for the following factors
I, II, V, VII, X
246
vitamin K dependent factors and what is prolonged
II, VII, IX, X
PT and aPTT
normal TT and plts
247
what does aminocaproic acid (amicar) do?
interferes with fibrinolysis and stabilizes a clot once it's already formed by inhibiting plasmin
248
if you suspect Hodgkin lymphoma, what do you do next?
CXR which may show mediastinal mass
249
some target cells indicate _____
| a lot of target cells indicate _____
thalassemia
| hemoglobin C disease
250
______ presents in the second 6 months of life with severe anemia requiring transfusion, heart failure, HSM, weakness
later facial deformities (maxillary hyperplasia and malocclusion)
alpha thalassemia major
251
8 year old with hgb 8 and repeated admissions for hand pain and swelling
what is it and what might you see on smear?
SS disease
| howell jolly bodies
252
usually a pretty mild anemia but you see tons of target cells
hemoglobin C disease
253
accumulation of _____ and its metabolites cause issues with liver, kidney, and CNS
notably acute liver failure in infancy or progressive cirrhosis and then liver failure... dietary management does not prevent liver disease
tyrosinemia type 1
254
3-4 months of age: FTT, hypoglycemia, hepatomegaly, acidosis
glucose 6 phosphate deficiency
255
infancy: cholestasis, bleeding into CNS/GI tract/umbilical stump, elevated transaminases --> chronic hepatitis and portal HTN
alpha 1 antitrypsin deficiency- chromosome 14
256
anemia, leukopenia, thrombocytopenia
liver and spleen involvement
erlenmeyer-flask appearance of long bones
what is it, what's the cause, how to dx
gaucher disease
- beta glucocerebrosidase deficiency --> abnormal accumulation of glucocerebroside in the reticuloendothelial system
- dx with absence of glucocerebrosidase activity in leukocytes, skin fibroblasts, liver cells
257
- sphingomyelinase deficiency
- hexosaminidase A deficiency
- sulfatase A deficiency
- serum trihexosidase deficiency
type A Niemann-Pick
Sandhoff
juvenile metachromatic leukodystrophy
Fabry
258
babies born to hyperthyroid moms can develop ______
heart failure
259
distinctive white forelock, heterochromia irides, unilateral or bilateral congenital deafness, lateral displacement of the inner canthi
waardenburg syndrome- AD
260
short stature, microcephaly, centrally placed hair whorl, small ears, redundant skin on nape of neck, upslanting palpebral fissures, epicanthal folds, flat nasal bridge, brushfield spots, protruding tonue, short and broad hands, simian creases, widely spaced first and second toe, hypotonia, duodenal atresia, VSD/TOF/endocardial cushion defect
Down syndrome
261
small testes that are firm and fibrotic
reduced upper to lower body segment ratio
high LH after age 12 years
Klinefelter syndrome
262
short stature, sexual infantilism, streak gonads, broad chest, low hairline, webbed neck, congenital lymphedema of hands and feet, coarctation of aorta
Turner syndrome
263
most likely cause of striae and central obesity in child
exogenous steroid administration and then bilateral adrenal hyperplasia
264
this IEM puts neonates at risk for E Coli sepsis
galactosemia
| cataracts, ascites, elevated LFTs, low serum glucose levels
265
tx for ornithine transcarbamylase deficiency
protein restriction and supplementation
266
tx maple syrup urine disease
diet free from branched chain amino acids
267
high doses of vitamin B6 for this IEM
homocystinuria
268
IEM with mousy smell
PKU
269
elevated urine succinylacetone indicates _____
tyrosinemia
| liver and renal dysfunction, growth failure, rickets, neuro sxs
270
elevated methionine and homocystine are assoc with ________
| also cabbage smell, delays in walking, MR, muscle weakness
methioninemia
| could also be asymptomatic
271
achondroplasia... are the limbs and trunk proportionate?
no
head is too big
limbs are too short
272
in _____ deficiency, the U:L ratio is normal often w/o other signs or sxs
GH
273
thyroid hormone deficiency causes stunting of growth with ______ proportions
persistence of immature body proportions
274
short trunk with short stature, marked slowing of growth, severe scoliosis, pectus carinatum, short neck
morquio syndrome (mucopolysaccharidosis IV)
275
hypothyroidism in a newborn can present as ________
prolonged jaundice and distended abdomen
276
umbilical hernia, kyphoscoliosis, deafness, cloudy cornea, claw hand deformity, progressive delay, HSM with coarse facial features, short stature, macrocephaly, hirsutism, decreased joint mobility
hurler syndrome (mucopolysaccharidosis type 1)
277
retinoblastoma chromsome
13
278
short stature with delayed boneage, MR, increased bone density esp in the skull, brachydactyly of 4th and 5th digits, obesity with round facies and short neck, subcapsular cataracts, cutaneous and subcu calcifications, perivascualr calcifications of the basal ganglia
pseudohypoparathyroidism (low Ca, high phosphorus but high PTH)
279
black diapers when exposed to air
homogentisic acid oxidase deficiency = alcaptonuria
280
ketoacid decarboxylase deficiency is what
maple syrup urine disease
281
wilson disease can cause fanconi syndrome
yep
282
what electrolyte issues do you see in nutritional rickets (vit D deficiency)
normal serum Ca
hypocalciuria
phosphaturia
283
what Ca and PO4 do you see?
- vitamin D resistant rickets
- pseudohypoparathyroidism (brachydactyly of 4th and 5th digits)
- osteogenesis imperfecta
- hypoparathyroidism (tingling and numbness --> seizures)
- medullary thyroid carcinoma
```
low PO4, normal Ca
high PO4, low Ca
normal PO4, normal Ca
high PO4, low Ca
normal PO4, normal Ca
```
284
severe hypotonia and poor feeding in a 6 day old
prader willi
285
obesity, mental retardation, hypogonadism, polydactyly, retinitis pigmentosa with night blindness
laurence-moon-biedl syndrome
286
rare cause of childhood obesity assoc with hypothalamic tumor
frohlich syndrome
287
tx of pseudohypoparathyroidism
large doses of vitamin D and reduction of phosphate load
288
return to play guidelines for concussions
grade 1: no LOC, confusion < 15 minutes
grade 2: no LOC, confusion > 15 minutes
grade 3: LOC
grade 1- can return to game
grade 2 or two grade 1s in one game- stay out for a week
grade 3- hospital eval
289
STD: inguinal adenopathy occurs at same time as ulcer
chancroid- chocolate agar
290
STD: LGV is caused by _________
| inguinal adenopathy occurs after the ulcer has healed
chlamydia trachomatis
