Med Study 4 Flashcards
(327 cards)
1
Q
Why do button batteries need to be emergently removed from the esophagus?
A
because they can cause immediate liquefactive necrosis
2
Q
If a child who swallows a coin is asymptomatic and it is now in the esophagus, what should you do?
A
You can observe for up to 24 hours
once it’s in the stomach, it should pass without a problem
but if it stays in the esophagus for > 24 hours, would need to go get it with a fiberoptic scope
3
Q
If a child swallows a coin and now it’s in the stomach, what should you do?
A
that’s fine
let it pass through
not a problem
4
Q
Infants who are fed goat’s milk are most likely to be deficient in which two vitamins?
A
folate and B6
the folate deficiency can lead to severe anemia
5
Q
Blank deficiency is common in children who are fed regular goat’s milk
A
folate deficiency
can lead to severeee anemia
6
Q
Type 1 glycogen storage disease, which presents in infants with rapid fasting hypoglycemia, is also called
A
von Gierke disease
7
Q
Atropine and blank are used to treat organophosphate poisoning
A
Pralidoxime
put a lid on the poisoning
(praLIDoxime prevents something horrible from happening– which is when the organophosphate/acetylcholinesterase complex permanently binds….. so pradlidoxime stops that process)
8
Q
What does the enzyme 5-alpha reductase do?
A
It converts testosterone to dihydrotestosterone
9
Q
A neonate who is XY is born with ambiguous/almost female looking genitalia, and is found to have a NORMAL testosterone level but an ELEVATED testosterone:dihydrotestosterone ratio (meaning, this neonate has a LOW dihydrotestosterone level). What is the diagnosis?
A
5-alpha reductase deficiency
10
Q
“Guevedoces” refers to what condition
A
5-alpha reductase deficiency
Guevedoces literally means “penis at 12”
refers to this AR condition where babies are born looking female, but then scrotum and penis develop at puberty
due to not being able to convert testosterone to dihydrotestosterone
11
Q
Androgen insensitivity phenotype
A
totally depends on the degree of insensitivity
these pts are XY and can be born looking totally female if complete insensitivity, or more male if only partial insensitivity
12
Q
What are testosterone levels like in androgen insensitivity syndrome?
A
normal or high
and the testosterone : dihydrotestosterone level is normal
13
Q
Elevated testosterone : dihydrotestosterone think
A
alpha-5-reductase deficiency
14
Q
Broad, stalk-like projection of bone on x-ray think
A
osteochondroma
= benign bone tumor
15
Q
This benign bone tumor is usually painless, and often goes unrecognized until bathing or dressing. Happens in metaphyseal regions of bones (for ex, distal femur). Exam shows boney irregular mass that is non tender. X-ray shows stalk like projection arising from the surface of the bone.
A
= osteochondroma
(also called exostosis)
16
Q
Should osteochondromas always be removed?
A
nope, only if they are causing symptoms
17
Q
Pain, palpable bone mass, and “sunburst” appearance on x-ray =
A
osteosarcoma
18
Q
Sunburst appearance of bone formation on x-ray + codman triangle =
A
osteosarcoma
19
Q
Periosteal elevavation (aka onion skinning) on x-ray =
A
Ewing sarcoma
Ewwwwwww onion
20
Q
Do osteosarcomas and Ewing sarcomas present similarly?
A
yup
21
Q
Blank is a benign bone tumor that causes SEVERE nighttime pain
A
osteoid osteoma
22
Q
Which benign bone tumor presents with pain and on x-ray will look like a well circumscribed lucency surrounded by sclerotic bone?
A
osteoid osteoma
23
Q
Nail pitting, dactylitis, and anterior uveitis are associated with this autoimmune disease
A
juvenile psoriatic arthritis
24
Q
What is juvenile psoriatic arthritis?
A
It’s a subtype of JIA.
In fact, it’s actually called “psoriatic juvenile idiopathic arthritis.”
You get small joint involvement, sausage digits (dactylitis), nail involvement, anterior uveitis, and of course psoriasis (but sometimes the skin findings don’t occur until years after the onset of arthritis).
25
Sausage digit think
juvenile psoriatic arthritis
26
8 yo has swollen finger, messed up nail (pitting and onycholysis--aka where nail separates from nail bed), and x-ray of the finger shows "soft tissue swelling and periosteal new bone formation of the left index finger." Her ANA is super positive. The diagnosis is
psoriatic juvenile arthritis
27
The rooting reflex should be gone by
4 months
(so if you see it still present in a 6 month old, that is ABNORMAL)
28
What is the parachute reflex? When does it appear?
Appears at age 6-9 months, and then stays for life, never disappears.
It's to protect you during a fall. If you hold the older baby/child vertical and face towards the ground, they will extend their arms as if to catch a fall. Adults still do this.
29
When does the moro reflex (aka startle reflex) go away?
by 3-6 months
30
What is the asymmetric tonic neck reflex, and when is it present?
= a reflex that shows up by 35 weeks gestation or 2 weeks of age, so it's a primitive reflex. It's where the baby's head is turned to one side, and they in response extend their arm to that side, and flex the other arm. So it's like fencing.
Helps with the development of hand-eye coordination.
31
The tonic neck reflex is often called the
fencing reflex
32
When should the tonic neck reflex disappear by?
by 6 months of age
33
The tonic neck relfex, aka the fencing reflex, is most prominent at this age
1 mo of age
34
Is a rooting reflex at 6 months of age normal?
Nope, that should really be gone by age 4 mo
35
Is an infant who still has a moro reflex and a tonic neck response at age 2 mo normal?
yup totally
36
If a child has pertussis, what should you recommend for their other family members in their household?
chemoprophylaxis
prophylax them all with azithro
37
Lab finding in pertussis
leukocytosis with lymphocytosis
38
The CRAFFT screening is screening for what?
drug and alcohol use in teens
39
SIGECAPS screens for......
depression
40
SAD PERSONS screens for
suicide risk
41
Nail pitting and arthritis are associated with
psoriasis
42
What is dyshidrotic ecezema?
Affects palms, soles and fingers. You get VESICLES that are super itchy and super painful.
Sweating (hyperhidrosis) makes it worse.
43
This type of eczema presents with painful/itching blisters (vesicles and bullae) on the hands and feet, and is made worse by blank
dishidrotic eczema
associated with hyperhidrosis, which makes it way worse
44
What is the corpus callosum?
= the band of white matter in the brain that connects the two hemispheres
45
What is a bronchogenic cyst?
= abnormal budding of the tracheal diverticulum in early in utero development
not connected to the tracheobronchial tree
often filled with mucous
prone to infection, and can rupture and cause pneumothorax
46
Blank is a NON-IgE mediated sensitivity to milk protein
milk protein allergy
seen in infants
47
What is FPIES?
= a non-IgE mediated sensitivity to food proteins
presents in infants with delayed vomiting, diarrhea and abdominal distension, like 2-6 hours after eating the protein that triggers this reaction.
kids will outgrow by age 3-4
48
What is failure modes and effect analysis (FMEA)?
= the process of proactively identifying potential sources of failure in a process BEFORE they fail, identifying possible causes of these failures, and planning specific actions to prevent these from happening
(so its different from a root cause analysis, because FMEA occurs BEFORE the problem)
49
PPV=
TP/(TP + FP)
50
What are the congenital heart defects with left axis deviation?
AV canal (partial or complete), and tricuspid atresia
51
In this type of congenital heart disease, the tricuspid opening does not exist -- and so the only way for blood to get out of the RA is through the PFO.
Tricuspid atresia
52
How does tricupsid atresia present?
With major cyanosis as the ductus begins to close
53
Neonate has cyanosis with sats of 70%, and ECG findings of left superior axis deviation and LVH. The diagnosis is
tricuspid atresia
54
In this type of congenital heart disease, there is often not much blood flow to the lungs since the RV doesn't exist and the PA is usually narrowed, and so you can see DECREASED pulmonary vasculature on xray.
Tricuspid atresia
55
Anatomy in total anomalous pulmonary venous return
= failure of the pulmonary veins to connect with the LA
instead, they drain into the RA, and so the blood from the right heart goes to the lungs and comes straight back to the right heart. not good
56
Congenital heart disease where there is a R to L shunt presents how?
with cyanosis in the neonate
57
Five "T"s of cyanotic congenital heart disease
Tetralogy of Fallot
TGA
Trucus arteriosus
Tricuspid atresia
TAPVR
58
Which vaccine contains the largest amount of egg protein?
The yellow fever vaccine
59
According to med study, can a patient with egg allergy still get the flu vaccine?
yes!!
no need to even do skin testing with the vaccine
apparently most kids even with severe egg allergy just plain do fine with the flu vaccine
ok..
60
Does "abdominal strapping" help for treating umbilical hernias?
NOPE
61
When to recommend surgical repair of an umbilical hernia:
At 5-6 years of age if hasn't resolved on it's own
OR
if it enlarges after 1 year of age
62
The most common infection to cause erythema nodosum is
strep
63
Pink to violaceous tender nodules on the shins think
erythema nodosum
64
What is erythema nodosum?
= a hypersensitivity reaction associated with a bunch of infections, most commonly strep, or autoimmune disease
presents with pink/purple tender nodules on skin
treat with nsaids and remove the trigger
65
After getting IVIG, avoid live vaccines for at least
3 months (at least. sometimes way more, depending on the dose of IVIG)
(and also need to wait 2 weeks after getting live vaccines to then get IVIG)
66
If a parent has HCM, what test should you get in the child?
genetic testing
(usually, a genetic mutation can be identified in the parent, and so this is the most definitive way to know if the child has the diagnosis)
67
What is a fixed drug eruption?
is a distinctive type of cutaneous drug reaction that characteristically recurs in the same locations upon re-exposure to the offending drug.
68
In what locations do fixed drug eruptions typically appear?
On the hands, trunk, genital and perioral areas
69
Well circumscribed dusky, erythematous lesion that is slightly erythematous that occurs at the SAME spot every time a patient is exposed to a certain drug =
a fixed drug eruption
70
The most common trigger for a fixed drug eruption is
Bactrim
71
Name 3 diseases where you get a rash on the palms and soles
syphilis
rocky mountain spotted fever
coxsackie A virus (HFM disease)
72
The most common cause of toilet training refusal is
"a strong-willed child"
LOL
73
The cardiac defect that is most common in Noonan syndrome patients is
pulmonic stenosis
74
Systolic ejection click on the LUSB + a harsh systolic crescendo-decrescendo murmur =
pulmonic stenosis
75
4/6 crescendo-decrescendo systolic murmur with palpable thrill at the 2nd left intercostal space =
PS (pulmonic stenosis)
76
Webbing of the neck in a boy think
Noonan syndrome
(apparently in the past, Noonan syndrome was referred to as "male Turner syndrome" due to some similar features)
77
Prominent webbing of the neck and bilateral ptosis in a boy who is short think
Noonan syndrome
78
What is postpericariotomy syndrome?
= the development of pericarditis and/or pericardial effusion several days to several weeks after having cardiac surgery
It's an autoimmune or inflammatory response, not fully understood
Patients will present with chest pain, cough, SOB, can have fever.
79
Muffled heart sounds think
pericardial effusion
80
Acoustic shadowing in the gallbladder on ultrasound is indicative of
gallstones
81
Male neonate with deficient abdominal musculature, cryptorchidism, and urinary tract anomalies =
prune belly syndrome
82
What does VACTERL stand for?
Vertebral defects
Anal atresia
Cardiac defects
Tracheoesophageal fistula
Renal anomolies
Limb abnormalities
83
The cause of colic is.......
unknown
84
Colic usually resolves by this age
should be gone by age 4 mo
85
When is it normal to see genu valgum (knock knees)?
age 2-4
(before age 2, there is natural bowing of the knees. after age 4, legs should be pretty straight)
86
Is genu valgum in a 3 year old child normal?
Yup
(genu valgum is normal from age 2-4)
87
Is genu valgum normal in a 15 mo child?
NO
they legs should actually be bowed at this age
88
Is persistence of genu valgum after age 4 normal?
NO
89
Genu valgum is normal in
pre school
90
Septic thrombophlebitis of the jugular vein is called blank. It is caused by blank.
Lemierre syndrome
starts as pharyngitis and then spreads to internal jugular vein, becomes clotted and infected
often get associated LUNG EMBOLI
most common in adolescents
91
Lemiere syndrome is associated with what complication?
septic emboli to the lungs
yikes
92
Lupus rash gets worse with
sun exposure
93
Sodium bicarb is given to alkalinize the urine in blank overdoses
aspirin
94
Sodium bicarb is given to address QRS prolongation in blank overdoses
TCA
95
Fomepizole is given to treat ingestions of
toxic alcohols like methanol and ethylene glycol
(fomepizole is the same thing as 4-methylpyrazole)
96
If a patient presents 24-36 hours after a tylenol overdose, should you still give NAC?
actually yes, since it may still have some benefit
97
Tx for Trichomonas vaginalis
Metronidazole
98
Pathophys of why hearing loss can happen after bacterial meningitis
due to damage to the acoustic nerve (8th cranial nerve)
so the result is sensorineural hearing loss
99
Type of hearing loss that can happen after bacterial meningitis and how to evaluate for it
sensorineural hearing loss
ABR (auditory brainstem response)
100
How does an ABR (auditory brainstem response) test work?
A sound occurs, and then brainstem activity is monitored (rather than monitoring patient response to determine if a sound was heard)
101
What does the ABR (auditory brainstem response) test measure?
Measures how cranial nerve 8 responds to sound
(clicks or tones are played through soft earphones into the ears. 3 electrodes are placed on the head and measure the CN 8 response).
102
All infants should have a hearing screening by this age
1 month
(ideally can do before they are discharged from the nursery)
103
What is the otoacoustic emissions (OAE) test and how does it work?
= one of two tests used to assess infant hearing
it measures sound waves produced in the inner ear (a tiny probe is placed inside the ear canal, and measure the echo that occurs when clicks or tones are played)
104
Otoacoustic emissions testing provides information on the function of
the outer hair cells of the cochlea
105
What is Peutz-Jeghers syndrome?
= and AD disorder where you get mucocutaneous lesions and intestinal hamartomas
106
Peutz-Jeghers Syndrome is also called
hereditary intestinal polyposis syndrome
presents with dark mucocutaneous lesions of the lips and gums, and with intestinal hamartomas (that can develop into cancer)
106
Peutz-Jeghers Syndrome is also called
hereditary intestinal polyposis syndrome
presents with dark mucocutaneous lesions of the lips and gums, and with intestinal hamartomas (that can develop into cancer)
also predisposed to non GI cancers as well
107
How does Puetz-Jeghers syndrome usually present?
intermittent colicky abdominal pain
108
Who should get the PPSV23 vaccine?
children who are at least 2 years old and who are at increased risk for invasive pneumococcal disease
109
PCV13 is routinely recommended for all children at what ages? How many total doses should they get?
4 total doses
at ages 2, 4, 6 months and then last dose at 12-15 mo
110
For children getting the PPSV23 vaccine because they are high risk, what should you make sure of first?
That they have received all 4 doses of the PCV13 vaccine first
and that it has been at least 8 weeks since the last dose of PCV13
111
PPSV23 can't be given to anyone under the age of
2
112
How many doses of PPSV23 should sickle cell (and all asplenic) patients get?
2
5 years apart
(however of note, cochlear implant pts only need one dose)
113
Mississippi and Ohio River valleys think
Histoplasmosis
a fungal infection that can present as pneumonia
114
Blank is a fungal infection that presents as pneumonia and occurs in the central, southeastern and mid-Atlantic states. It is also associated with wooded sites (for ex, camping). Sputum culture will show broad based budding yeast (kind of looks like a fat figure 8).
Blastomycosis
115
Blank is a fungal infection that can present with pneumonia and occurs in the deserts of the southwestern US.
Coccidiomycosis
116
Elevated urinary levels of homovanillic acid (HVA) and vanillylmandelic acid (VMA) are suggestive of
the presence of a catecholamine-secreting tumor (like neuroblastoma or a pheochromocytoma)
117
soft palate petechiae think
GAS pharyngitis
but could also be mono
118
What is Herpangina and what is it caused by?
caused by coxsackievirus
= sudden onset fever and vesicular lesions on the posterior pharyngeal wall, uvula, and tonsils
lesions rupture and form ulcers
119
How to distinguish between herpangina (caused by coxsackie virus), and herpetic gingivostomatitis (caused by HSV):
In Herpangina, the tongue and gingival surfaces are NOT affected by the rash
so the tongue and gums are spared
120
Vesicles that have ulcerated that are present only on the posterior pharyngeal wall and NOT the tongue or gums =
Herpangina
121
How do infants and toddlers with Herpangina present?
usually in the summer with high fever, excessive drooling, feeding difficulties and posterior pharynx lesions
122
Ulcerations of the buccal mucosa and tongue with gingival hyperemia = the findings seen in...
herpetic gingivostomatitis
123
HSV occurs in the front part of the mouth and lips, whereas Herpangina occurs in the.....
posterior oropharynx
back of the mouth
124
Cherry-red spot on fundoscopic exam =
Tay-Sachs disease
125
Pathophys of Tay-Sachs disease
= AR disease where there is a defect in ganglioside metabolism, and so lipids accumulate in neural structures. This leads to loss of vision, muscle tone, and general stamina. Babies develop normally until about 3-6 mo and then have a neurological decline.
They get a cherry red spot on the retina.
126
Cherry-red spot on the retina =
Tay-Sachs disease
127
Tay-Sachs disease eye finding
cherry red spot on the retina
wait. apparently you can see it other disease too ahhhh
128
Early sign of Tay-Sachs disease
increased startle response
129
Tay-Sachs disease is most common in which populations?
Eastern European Jewish
French Canadian
Cajun
130
8 mo with developmental regression, startle myoclonus, muscle weakness, and cherry red spot on eye exam =
Tay-Sachs disease
131
How to manage a child under age 5 who has had a direct exposure to TB
PPD skin test and CXR to evaluate them
and then START TREATMENT for latent TB, EVEN if the PPD is negative
then repeat the PPD 8-12 weeks later (and if negative, can stop prophylaxis at this point, whereas if positive would need to complete the treatment course for latent TB).
132
The interferon gamma release assay for TB testing can only be used on children over age
2
so 24 mo and up, you can use this
133
After getting the MMR vaccine, how long do you need to wait until you place a PPD test?
4 WEEKS
Because, the MMR vaccine can temporarily suppress PPD activity
and so, would not be accurate until at least 4 weeks after vaccine is given
134
Which meningococcal vaccine can be given as a 4 dose series at 2,4,6, and 12 mo of age to high risk infants (aka infants without a spleen or with a complement deficiency)?
Menveo
135
For an asplenic infant, which meningococcal vaccine should you give and when
Menveo
4 doses
at 2,4,6 and 12 mo
136
What is juvenile plantar dermatosis?
= a chronic skin condition of the feet (soles) that kids who have allergies or eczema sometimes get. During flares, they get painful skin thickening of the feet, fissures, and a glazed, shiny like appearance of the feet. SPARES the intertriginous areas. Treat like eczema.
(thought to happen on the feet do to sweating in shoes, basically).
137
"Sweat sock dermatitis" or "Sneaker dermatitis" refers to
Juvenile plantar dermatosis
138
What is Intertrigo?
= an irritant dermatitis of the skin folds
caused by moisture and friction in those areas
can get secondarily infected with candida
think baby with red irritated area under neck fat folds area
139
Nummular eczema looks like..
coin like
ROUND
lesions
140
Which type of eczema is characterized by vesicles on the sides of the fingers?
dyshidrotic eczema
VERY itchy, and also painful
141
This condition frequently occurs with dyshidrotic eczema
hyperhidrosis
142
xer means
dry
so xerophthalmia = dry eyes
xerosis = dry skin
143
In babies with eczema, this area is typically spared
diaper area
since it's protected from scratching
144
Most common areas for eczema to occur in pts ages 0-2
cheeks
extensor surfaces of extremities
after these ages it switches to the flexor surfaces
tricky
145
emollient means
hydrator
so like a cream or ointment that is hydrating (these work better than lotions)
146
grouped vesicles on an erythematous base
= HSV
147
What is Lichen Striatus?
= small, hypopigmented or skin colored papules in a "Blaschkoid" distribution (aka follows embryonic ectodermal lines of migration) -- usually occurs in a line on an arm or a leg
asymptomatic or may itch mildly
will go away on its own, no need to treat
148
Blank is an acquired, asymptomatic, and self-limited linear inflammatory skin disorder that predominantly affects children [1,2]. The eruption is typically unilateral, most often involving the extremities, and follows the lines of Blaschko in a continuous or interrupted pattern.
Lichen striatus
149
V1 distribution of port wine stain
= concern for Sturge Weber
V1 = (ophthalmic branch, central face/and nose area)
150
E tox spares which areas?
palms and soles
151
What is lymphogranuloma venerum?
= an STD that mostly occurs in tropical parts of the world, caused by the L1, L2 and L3 serotypes of Chlamydia trachomatis. Initial stage = several nontender lesions on genitals. Secondary stage appears a few weeks later, with tender inguinal lymphadenopathy.
152
Options for prophylaxis against neisseria meningitis after close contact
Rifampin x 2 days
or single dose ceftriaxone
153
Clinda has good gram pos and aneorobe coverage. It does not have blank coverage
gram neg
154
If a patient is allergic to azithro, treat mycoplasma pneumoniae with
doxy
155
Fluid management for burn pts:
3 mL/kg for each %BSA in the first 24 hours, with 50% of this being given over the first 8 hours
156
What is Behcet disease?
= a vasculitis that presents with painful oral and genital ulcers, as well as inflammatory eye disease
157
benign iris hamartomas seen in NF1 are called
Lisch nodules
158
Always refer kids with NF1 to ophtho so they can be watched for
optic gliomas
159
What are neurofibromas?
= benign, peripheral nerve sheath tumors that occur in NF1
160
Pathophys of NF1
AD (tho often sporadic) mutation in the NF1 gene, which encodes for the protein neurofibromin, which is a tumor suppressor gene in nerves sheaths
161
Bony manifestations of NF1
-long bone thinning and bowing (usually the tibia), which predisposes to fracture
-sphenoid wing dysplasia
-dysplastic scoliosis
162
What is the double disc diffusion test ("the D-test")?
= a test to determine if there is macrolide-inducible clinda resistance in a staph aureus species
done on MRSA strains in the lab that show initial clinda sensitivity. However if the D-test is positive, it means DONOT DONT DONT DONOT DONT use clinda, as the organism is ACTUALLY resistant/will be resistant very soon
163
This test determines whether a specific MRSA strain will be sensitive to clinda
D-test
164
Most infants acomplish a pincer grasp by what age?
12 mo
165
When should you be able to do a pincer grasp?
age 1
12 mo
166
What is PANDAS?
= pediatric autoimmune neuropsychiatric disorder associated with streptococcal infections
specifically, pts with OCD and/or tic disorders can have worsening of their symptoms during strep infections
167
The worsening of these specific neuropsych conditions can happen during an acute strep infection
OCD and tic disorders
168
What is Fanconi anemia?
= an AR disorder involving poor DNA repair mechanisms
Presents with pancytopenia in kids typically ages 8-9
also associated with short stature, skeletal abnormalities (like thumb issues), cafe au lait spots, GI atresias at birth, and renal anomalies
At HIGH risk of developing myelodysplastic syndrome and AML
169
Hypersegmented polymorphonuclear lymphocytes on peripheral blood smear are suggestive of
B12 or folate deficiency
170
Trisomy 18 is also called
Edwards syndrome
E for Edwards, E for Eighteen
171
Trisomy 13 is also called
Patau syndrome
172
Neonate with flexed fingers where the index finger is overlapping the 3rd finger, and the pinkie finger is overlapping the 4th finger think
Trisomy 18
Edwards syndrome
173
This type of trisomy has the most severe craniofacial and midline defects
Trisomy 13 (Pataeu syndrome)
includes things like holoprosencephaly, midline cleft defects, cutus aplasia (punched out lesions of the scalp)
174
What is transient myeloproliferative disorder?
= a unique condition that infants with Trisomy 21 get. It's also called "transient leukemia" because it involves the presence of blasts (usually megakaryoblasts) in the peripheral blood but not the marrow. Patients get vesiculopustular skin lesions, but otherwise are asymptomatic, and then it goes away on its own.
But these pts are at increased risk for actual leukemia in the future.
175
Clenched overlapping fingers think
Trisomy 18
176
Trisomy where polydactyly is most common
13
177
Aplasia cutis think
Trisomy 13
178
A baby is born with polydactly and aplasia cutis congenita. The diagnosis is
Trisomy 13
179
A baby is born with holoprosencephaly and microphthalmia. The diagnosis is
Trisomy 13
180
When should a left sided varicocele that is present in an adolescent boy but only when standing be repaired?
when it's causing the testicle on that side to atrophy or not grow normally
so if on exam that testicle is smaller then the non varicocele testicle then that is a problem, and it needs surgery
181
Patients with cyclic neutropenia are at particular risk for sepsis caused by
Clostridium septicum
182
Purulent conjunctivitis (pink eye) is caused by infection with
H flu
183
Clostridium septicum bacteremia think
cyclic neutropenia
184
Multiple areas of osteochondritis and periostitis of the long bones is seen in what disease
congenital syphilis
185
A baby has "widening of the growth plates associated with metaphyseal fraying and cupping of the distal radius and ulna." The diagnosis is
Rickets from vitamin D deficiency
186
What is Rickets?
= inadequate mineralization of the growth plates and generalized softening of the bones due to not enough vit D
187
Widening of costochondral junctions is seen in
Rickets (Vitamin D deficiency) -- this is called "rachitic rosary" when its at the anterior ribs
188
Darker skin makes you more likely to be blank deficient
vitamin D
189
Labs associated with Rickets
low vit D
high PTH
high alk phos
190
Vitamin D deficiency is a level less than
30
191
a submandibular space infection is also called
Ludwig angina
192
Treat Rocky Mountain Spotted Fever with
doxy
193
Where does Rocky Mountain spotted fever occur?
Mid Atlantic states / South East
(despite its name)
for ex, North Carolina
194
Patients who present with Rocky Mountain spotted fever will have these lab findings:
hyponatremia and thrombocytopenia
195
Treat all ricketsial infections (aka rocky mountain spotted fever) with blank, REGARDLESS of the child's age
doxy
EVEN IF younger than 8, STILL treat with doxy, it's THAT important and can die otherwise
196
The rash in this disease starts at the wrists and ankles and then spreads to the palms and soles and all over the body and becomes hemorrhagic quickly
Rocky Mountain Spotted Fever
197
How do you get Rocky Mountain Spotted fever?
from ticks carrying the bacteria Rickettsia rickettsii
198
What is the most common cause of congenital hypothyroidism?
Thyroid dysgenesis
199
What is Bartter syndrome?
= a genetic kidney problem where you don't hang onto salt, and so you get hyperaldosteronism and increased renin secretion
you do NOT get hypertension
you do also get hypercalciuria tho. babies can present with this with severe dehydration.
200
Blank syndrome presents in the neonate with severe dehydration after prenatal polyhydramnios. The infant will have calcium in the urine.
Bartter syndrome
201
At this age, children will parallel play
2 years
(will see more parallel play at this age, not so much cooperative play yet)
202
Most children cannot dress themselves completely, including doing buttons, until this age
until they are 3
203
Children at this age should have 50 words and be able to use 2 word sentences
age 2
204
A child of this age can identify 6 body parts
24 mo
205
What is von Hippel-Lindau syndrome?
= an AD tumor predisposition syndrome where you get both benign and malignant tumors of the CNS, kidneys, pancreas, adrenal and reproductive glands
206
Classic presentation for von Hippel-Lindau syndrome
a child presenting with a cerebellar hemangioblastoma (benign blood vessel tumor) or retinal angioma (which can cause blindness if not treated) by 10 years of age
207
A 10 yo presents with a hemangioblastoma in the cerebellum and a hemangioblastoma in the retina. The diagnosis is
Von Hippel-Lindau syndrome
208
Outbreak of kids at summer camp with fever, conjunctivitis and pharyngitis think
adenovirus
209
Using the Holliday-Segar formula, calculate the daily kcal required under basal conditions for a 23 kg child:
100 kcal/kg for the 1st 10 kg
50 kcal/kg for the 2nd 10 kg
20 kcal/kg for the rest
so, 100x10 + 50x10 + 20x3 = 1560 kcal/day that this 23 kg child needs
210
When should breastfed preterm infants start iron supplementation?
at 1 month of age
through 12 mo of age
211
What is juvenile nephronophthisis type 1?
= a rare AR disease where there is salt wasting WITHOUT nephrotic or nephritic syndrome.
ESRD usually occurs by age 13.
212
What is nephronophthisis?
= AR disease where you get "tubulointersistial sclerosis" that leads to ESRD
213
How do kids with juvenile nephronophthisis type 1 present?
with polyuria, polydipsia, anemia, salt wasting, FTT, and progression to ESRD, + associated ocular problems
214
gram-positive diplococci think
strep pneumo
215
How does strep pneumo pneumonia classically present?
with sudden onset high fever, chills, chest pain with dyspnea, and sometimes blood tinged sputum
children will look clinically ill
treat with high dose amox if outpatient, and ceftriaxone if inpatient
216
All types of osteogenesis imperfecta are caused by an abnormal structure of....
Type 1 collagen
217
Blank disease is a common cause of pediatric heel pain and refers to inflammation of the calcaneal growth plate where the Achilles tendon inserts
Severe disease
218
Sever disease usually occurs in the context of....
running and jumping activities (For ex soccer or basketball) during a child's growth spurt
219
Ludwig's angina is most often a severe complication of
a dental infection
220
What is Ludwig's angina?
= an aggressive, rapidly spreading, polymicrobial cellulitis and infection of hte submandibular space
patients will be ILL, will have a hard time opening their mouth, have pain and fever. often is a bad complicatoin of a dental infection
221
What is spondylolysis?
= a vertebral injury where there is a stress fracture of the pars interarticularis. Usually involves L5 in athelets (especially in things like gymnastics, ballet) where there is a lot of hyperextending the back.
Pain will be reproduced with back extension. Patients will present with lower back pain, but likely will NOT have any history of trauma
222
Spondylolysis of L5 in a gymnast or ballet dancer can be complicated by...
spondylolisthesis (where the L5 vertebra slips onto the S1 vertebra)
223
stress fracture of the pars interarticularis is called
spondylolysis
224
scottie dog appearance on spinal x ray think
spondylolysis
225
How to treat spondylolysis and spondylolisthesis
initially conservatively
rest
heat
ice
NSAIDs
only refer to for surgical intervention if refractory to conservative management
226
Treatment for KD
IVIG and aspirin
227
KD is most common in children under age
5
228
TEN involves >blank percent of the body surface area
>30%
229
IM ceftriaxone is used to treat blank infections
gonococcal
230
This cause of bacterial diarrhea can also cause vulvovaginitis with bloody or serosanguinous vaginal discharge
Shigella
231
Shigella diarrhea can cause blank in infants and children
seizures!
232
Infant with high fever and new onset seizure undergoes LP. During LP, baby has large bloody stool. The diagnosis is....
Shigella
233
A 6 yo girl presents with 2 days of blood tinged yellowish vaginal discharge, abdominal pain and watery diarrhea. The diagnosis is
Shigella
234
Should you treat shigella diarrhea?
Only if severe
with azithro or ceftriaxone
235
What is "premature thelarche?"
= a normal variant of breast budding in infants and toddlers
NOT a diagnosis made in kids or adolescents
236
hypertelorism
= an abnormally large distance between the eyes
237
How to remember that Hunter's disease has NO corneal clouding (unlike Hurler disaese)
you have to be able to see well to hunt
so there is no corneal clouding in Hunter's disease
238
Coarse facial appearance, developmental delays by age 2, and joint stiffness think
Hunter syndrome
239
Why do patients with Hunter syndrome develop myocardial hypertrophy as they grow older?
due to accumulation of glycosaminoglycans in the heart
also accumulate in the valves and lead to valve issues
remember Hunter syndrome pts will get myocardial thickening
240
If a child is going to be around immunocompromised individuals, they should not get these two vaccines
LIVE influenza vaccine
LIVE polio vaccine
but MMR is fine!!!!
241
Symptoms of serotonin syndrome
tachycardia
hypertension
hyperthermia
confusion/hallucinations/agitation
sweating
diarrhea
LE rigiidity, hyperreflexia and myoclonus
242
Myoclonus
= sudden, brief involuntary twitching or jerking of a muscle or group of muscles.
243
The most specific exam finding in serotonin syndrome is
myoclonus
244
What is neuroleptic malignant syndrome?
= bad thing that can happen from antipsychotic drugs
presents similarly to serotonin syndrome, with severe muscle rigidity, hyperthermia, confusion, and autonomic instability
245
How to distinguish between NMS and serotonin syndrome
NMS is characterised by 'lead-pipe' rigidity, whilst serotonin syndrome is characterised by hyperreflexia and clonus.
but really go off of drug hx as well
246
Precocious puberty is defined as the onset of secondary sex characteristics in girls before age blank, and boys before age blank
girls before age 8
or boys before age 9
247
What is koilonychia?
= a spooning deformity of the finger and toe nails that can be seen in IDA (iron deficiency anemia)
248
Hypochromia indicates
a decrease in the concentration of intracellular hemoglobin
249
It's abnormal if a child continues to suck their thumb past this age
4
250
The most common fatty acid beta oxidation disorder is
medium-chain acyl-CoA dehydrogenase deficiency
251
Medium chain acyl CoA dehydrogenase deficiency patients can end up with low carnitine because ....
They can't break down medium chain acylcarnitines, and so some of the extra that builds up binds to free carnitine and gets renally excreted
so give them carnitine
in addition to glucose adn frequent feeds
(this is the msot common fatty acid oxidation disorder)
252
The most common inflammatory myopathy of childhood and adolescence is
dermatomyositis
253
What are chillblains?
Chilblains (CHILL-blayns) are the painful inflammation of small blood vessels in your skin that occur in response to repeated exposure to cold
254
How do patients with juvenile dermatomyositis present?
with rash (heliotrope rash around eyes and Gottron papules on hands) and symmetric, proximal muscle weakness.
255
Gottron papules
= red plaques over the extensor surfaces, most commonly on the hands in patients with dermatomyositis
256
Physical exam in avascular necrosis of the femoral head
the patient will typically stand with the leg slightly externally rotated; internal rotation will cause pain
257
Risk factors for avascular necrosis of the femoral head
chronic systemic steroids
Lupus
chronic renal failure
sickle cell disease
or can be idiopathic (Legg-Calve-Perthes disease)
258
Xray of the hip shows flattening and fragmentation of the left femoral head and epiphysis. The diagnosis is
avascular necrosis of the femoral head
259
"ratty" fuzzy/fragemneted appearance of the femoral head think
avascular necrosis
260
3 things that are contraindications for renal transplanation
active infection
uncontrolled malignancy
ABO incompatibility
261
Does a child have to be on dialysis before getting a renal transplant?
NOPE! In fact, preemptive transplant is preferred
262
2 yo with FTT, history of IUGR, oligodactly, and HIRSUTISM has what syndrome?
Cornelia de Lange syndrome
genetic syndrome caused by sporadic AD mutation
263
Key features of Cornelia de Lange syndrome
hirsutism
growth restriction
characteristic facial features with synophrys (fusion of eyebrows above the nose)
self injurious behaviors
hand/feet/limb shortening or abnormalities
264
This genetic syndrome has hirsuitism as one of its key presenting features
Cornelia de Lange syndrome
265
At what age are 90% of children able to sit without support?
7 months
266
Vitamin C is also called
ascorbic acid
267
Blank are melanocytic hamartomas of the iris, often associated with neurofibromatosis type 1
Lisch nodules
268
Severe vitamin C deficiency leads to impaired blank synthesis, which leads to connective tissue abnormalities
collagen
the lack of normal collagen results in fragile capillaries and hemorrhages
bones also become brittle and fracture easily
269
Why do patients with Scurvy get arthralgias and joint swelling?
due to hemarthrosis
270
Generalized rash involving perifollicular hemorrhages and follicular hyperkeratosis think
Scurvy
271
Gingival swelling and bleeding think
Scurvy
272
White lines of Frankel
= dense white bands at the metaphysis seen in vitamin C deficiency
273
upward lens dislocation is seen in what disease?
Marfan Syndrome
274
What is pyoderma gangrenosum?
= a chronic, painful inflammatory disorder of the skin often associated with IBD
275
Mirror movements (involuntary movements mirroring contralateral voluntary hand movement) are seen in what syndrome?
Kallmann syndrome
276
Port-wine stains are also called
nevus flammeus
277
What is a nevus sebaceous?
it's a hamartoma present at birth, usually on the face or head. It's a yellow or pink, hairless plaque. It gets thicker during puberty. It contains an overabundance of sebaceous glands.
surgery recommended in adolescence due to small risk of it transforming into a basal cell carcinoma
278
A child has a single, well-circumscribed, hairless, yellowish-to-tan plaque on his face, that has been present since he was born. What is the diagnosis?
Nevus sebaceous
279
A 2 yo comes into the ED with a kerosene ingestion. How to manage this patient:
Kerosene is a hydrocarbon. You need to do supportive care (oxygen/bronchodilators as needed) since aspiration pneumonitis is the real concern here. Need to observe and then get a chest x ray in 6 hours. IF seems totally fine and chest x ray is normal, can send home from ED with return precautions.
280
Do steroids help in hydrocarbon ingestions?
nope. no benefit, don't give them
281
Name some hydrocarbons
turpentine
gasoline
kerosene
furniture polish
lighter fluids
282
Give blank to patients taking isonaized to prevent peripheral neuropathy
Pyridoxine (B6)
283
Side effect of isoniazid
peripheral neuropathy, due to pyridoxine (B6) depletion
284
RIPE stands for
Rifampin
Isoniazid
Pyrazinamide
Ethambutol
285
Blank causes orange discoloration of all secretions and urine
Rifampin
286
Tennis elbow is blank epicondylitis
Lateral
reproduce the pain with resisted wrist extension
(extension of the wrist happens during backhand motion)
287
Pain and point tenderness upon palpation around the lateral epicondyl is
tennis elbow
288
Blank is a noninflammatory condition associated with an increase in vaginal pH (>4.5)
BV
(this increase in pH allows for overgrowth of multiple anaerobes like gardnerella vaginalis and decrease in the number of normal lactobacilli)
289
2 types of vaginitis with elevated pH
BV
Trichomoniasis
290
Intraerythrocytic and exoerythrocytic organisms on peripheral Giemsa-stained blood smear =
Babesiosis
291
Ixodes scapularis, the deer tick that transmits lyme disease, also transmits this disease
Babesiosis
292
Where in the US is Babesiosis most common?
NE and northern midwest
293
Do immunocompetent patients usually get really sick from Babesiosis?
No, the problem comes when the patient is immunocompromised, OR has concurrent lyme disease
294
Is Babesia a parasite or bacteria? How is it transmitted?
parasite
tick borne illness
295
A "Maltese Cross" (formed by a tetrad of organisms on blood smear) is pathognomonic for
Babesiosis
however often don't see this
296
How to treat Babesiosis
NOT doxy
Treat with atovaquone + azithro
Or if really severe, clinda + quinine
297
Babesiosis symptoms
febrile, hemolytic anemia especially in patients without a spleen or those who are otherwise immunocompromised
298
This disease can sometimes be misdiagnosed as malaria
Babesiosis
299
Common symptoms of babesiosis in addition to flu like symptoms
hemoglobinuria
300
How to diagnose Babesiosis
See the parasite Babesia microti on Giemsa or Wright stained blood smear
or there is also a PCR test
301
All types of hereditary angioedema are associated with a low level of
C4
302
How does hereditary angioedema present?
with recurrent episodes of cutaneous (often facial) and GI edema (abdominal pain, vomiting) -- withOUT hives
303
Blank is an AD disorder caused by a defect in C1 inhibitor enzyme
Hereditary angioedema
There are diff types -- sometimes C1-INH level is low and sometimes its normal--but in all types, the C1-INH (aka C1 inhibitor enzyme) is NOT functioning well
304
What is the job of C1 inhibitor enzyme?
It inactivates C1, and so is a major control point in the activation of the complement cascade. It helps prevent it from getting activated inappropriately. It also keeps the bradykinin-forming kallikrein-kinin system from getting activated inappropriately.
305
What role does bradykinin play normally in inflammation?
It mediates inflammation by causing vasodilation and by increasing vascular permeability
306
Hereditary angioedema patients, when they get swelling, do not have these symptoms
hives or itching
aka urticaria or pruritus
(however, they can get a serpiginous rash called erythema marginatum on the trunk and limbs)
307
Blank is the key mediator for attacks of angioedema in mpatients with hereditary angioedema
Bradykinin
308
How to diagnose Hereditary angioedema
Check C4 levels. If C4 levels are low, that is suggestive of HAE.
Can then do C1-INH levels and functional assay to determine which type of HAE.
309
How to treat an acute attack in hereditary angioedema
Purified C1 inhibitor enzyme
or FFP if that's not available
310
A neonate presents with refusal to move the right leg. X-ray shows "transverse bands of increased density across the femoral metaphyses" and "patchy areas of destruction in the diaphysis" and "solid, periosteal new bone formation." The diagnosis is....
pseudoparalysis from painful osteochondritis caused by congenital syphilis
311
For children ages 1-13, normal BP is defined as
both SBP and DMP < 90th percentile
312
Prehypertension is now called
"Elevated BP"
stupid naming, but ok sure
313
Age 13 and up, normal BP is
<120/80
314
Define "elevated BP," stage 1 htn, and stage 2 htn for kids age 13 and up:
Normal would be <120/80
Elevated BP is 120-129/<80
Stage 1 HTN is 130/80-139/89
Stage 2 is 140/90 or higher
315
Definition for "elevated BP" in a child ages 1-13:
SBP and DBP > 90th percentile but < 95th percentile, or 120/80 up to the 95th percentile, whichever is lower
316
Define stage 2 hypertension in children ages 1-13:
SBP and/or DBP > 95th percentile + 12 mmHg, or > 140/90
317
blue granules of various sizes dispersed throughout the cytoplasm of red blood cells =
basophilic stippling of the red blood cells, see in lead or other heavy metal poisoning
however, can also be seen in other causes of ineffective erythropoiesis, like thalassemia i guess.... ok that's confusing
318
Is anemia secondary to lead toxicity normocytic or microcytic?
can be either
319
Burton line
= a blue line along the gums seen in lead poisoning
the teeth will also get a slightly bluish blackish edge
320
Why can lead poisoning cause anemia?
lead inhibits several enzymes involved in the production of hemoglobin
you get elevated levels of protoporphyrin, due to failure of the normal heme pathyway to occur
321
Free erythrocyte protoporphyrin is elevated in
both lead toxicity and iron deficiency anemia
322
Common finding on peripheral blood smear in lead poisoning
basophilic stippling (blusih granules of various seizes scattered in the cytoplasm of RBCs)
323
Lab finding in patient of lead poisoning and anemia
elevated levels of blood erythrocyte protoporphyrin
324
Shallow painful ulcers in the posterior pharynx, posterior cervical llymphadenopathy, cough and rhinitis think
Herpangina
caused by coxsackievirus which is an enterovirus
325
When should a healthy, full term, exclusively breastfed infant begin iron supplementation?
iron stores in a neonate run out at about 4 mo
so start giving iron at 4 mo
and stop when baby is regularly consuming iron rich foods
(for preterm breastfed infant, start giving iron at 1 mo, and continue until age 12 mo)
326
Do babies taking regular formula fortified with iron (aka like standard formula) need extra iron supplementation?
nope
so just the breastfed babies need iron
ok
