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Pediatric Boards > Med Study 5 > Flashcards

Med Study 5 Flashcards

1
Q

What is a cholesteatoma?

A

= a lesion consisting of built of squamous epithelium and keratin trapped within the middle ear

needs to be surgically removed

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2
Q

How does a cholesteatoma present?

A

painless otorrhea

often in a kid with ear tubes or history of AOM

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3
Q

Why does a cholesteatoma need to be surgically removed?

A

Because if it is left untreated, it can cause hearing loss, vertigo, CN palsies, or even predispose to brain abscess

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4
Q

Blank is an abnormal growth of squamous epithelium in the middle ear that often presents with otorrhea

A

cholesteatoma

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5
Q

If you don’t take a cholesteatoma out it can

A

grow and destroy the ossicles

thus obviously causing hearing loss

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6
Q

According to med study, by which age can most children walk up and down stairs with 1 foot on each step?

A

4 years

not til 4 years old wow ok

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7
Q

By this age, most children can jump off the ground with 2 feet up

A

30 mo

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8
Q

By this age, most children can skip

A

6

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9
Q

The only type of vascular anomaly where the “misplaced” vessel courses between the trachea and the esophagus is…

A

a pulmonary sling

it’s where the LPA comes off the RPA, not what it’s supposed to do, and ends up coursing between the trachea and the esophagus

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10
Q

If a barium swallow shows anterior indentation of the esophagus in an infant, the diagnosis is

A

a pulmonary sling

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11
Q

What is DRESS?

A

= stands for drug reaction with eosinophilia and systemic symptoms

can be caused by viruses (HHV 6 and 7, EMV/CMV) or drugs (sulfonamides, antiseizure meds)

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12
Q

Do you get eosinophilia in mycoplasma pneumoniae infection?

A

nope

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13
Q

Parvovirus B19 causes a febrile exanthematous illness in young children known as

A

erythema infectiosum

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14
Q

When adolescents or adults get Parvovirus B19 infection, they are likely to get this symptom

A

arthritis of the fingers

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15
Q

Treat DRESS syndrome with

A

steroids

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16
Q

A 14 yo girl presents with a 2 day history of a rash and swollen face. She also has a fever. Labs show eosinophilia. She started a new seizure med 6 weeks ago. You should suspect this diagnosis

A

DRESS syndrome

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17
Q

Reactivation of blank virus may play a role in the development of DRESS syndrome

A

Human herpesvirus 6/7, CMV/EBV

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18
Q

Reactivation of this virus is frequently seen in DRESS syndrome

A

Human herpesvirus 6

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19
Q

What is papular acrodermatitis of childhood?

A

Also called Gianotti-Crosti syndrome

it’s a self limited, uncommon reaction that can happen as a response to some viruses or vaccines

pts get a symmetric, papular eruption

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20
Q

Symmetric papules on the arms/face/butt on a 2 yo with a URI =

A

probably papular acrodermatitis

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21
Q

Papular acrodermatitis usually spares the

A

trunk

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22
Q

How do patients with Truncus Arteriosus present?

A

Because pulmonary blood flow is typically increased, they actually don’t always present with cyanosis right away, or only present with mild cyanosis. But then in the first few weeks, due to PVR falling and tons of pulmonary blood flow, they will quickly present with signs of HR (tachypnea, FTT, diaphoresis) and mild cyanosis. The heart will be hyperdynamic and the infant will have strong, bounding peripheral pulses.

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23
Q

Exam in infant presenting at a few weeks of life found to have Truncus Artertiosus

A

The heart will be hyperdynamic and the infant will have strong, bounding peripheral pulses. Mild cyanosis, but may look more like a HF picture. Widened pulse pressure. Single S2 (only one valve to create the S2 sound!!) w/ejection click typically.

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24
Q

Heart defects typically seen in DiGeorge syndrome:

A

TOF
interrupted aortic arch
VSD
Truncus Arteriosus

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25
Q

Absent thymus think

A

DiGeorge Syndrome

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26
Q

depression + liver failure + abnormal eye exam think

A

Wilson Disease

due to copper accumulation

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27
Q

psych symptoms, tremor, and elevated serum aminotransferases think

A

Wilson disease

copper accumulation

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28
Q

Aplasia cutis congenita think

A

Trisomy 13

(tho can also be an isolated defect)

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29
Q

In a child with NF1, what is the average age that they would present with an optic glioma?

A

age 4

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30
Q

A 4 yo presents with precocious puberty and 20 cafe au lait spots, all of which have smooth borders. Most likely diagnosis is

A

NF1

(In McCune-Albright syndrome, the cafe au lait spots would be fewer and would have irregular borders)

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31
Q

In children with NF1 who develop an optic glioma, how do they usually present?

A

vision loss
or precocious puberty
in a child under age 6 typically

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32
Q

Which vaccines are administered subQ?

A

MMR and varicella

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33
Q

If a child has a mild, acute illness with OR without a fever (like AOM or gastroenteritis), should you defer vaccines that day?

A

Nope, still give them. do NOT defer.

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34
Q

What is Moyamoya disease?

A

= a chronic disease where cerebral vessels stenose over time to the point where an individual often has an ischemic stroke

can be associated with SCD, Trisomy 21, NF1

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35
Q

Moyamoya means

A

“puff of smoke”–refers to all the extensive small collateral vessels seen on imaging as a result of narrowing of the major cerebral vessels over time

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36
Q

How do kids with Moyamoya disease present clinically?

A

with ischemic stroke
or new seizures
or TIA
or chronic headaches actually

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37
Q

On CT scan, blood is

A

bright

so a bleed will look hypERdense

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38
Q

HypOdensity on CT scan think

A

ischemic infarct

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39
Q

How to calculate midparental height for a girl:

A

(Mom’s height + (dad’s height - 5 inches) / 2

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40
Q

How to calculate midparental height for a boy:

A

((mom’s height + 5 inches) + dad’s height)/2

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41
Q

Tx for Chlamydia

A

doxy 100 mg BID x 7 days

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42
Q

How to treat Gonorrhea

A

Ceftriaxone 500 mg single dose IM

(use 1 g if pt is > 150 kg or 300 lbs)

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43
Q

What is metatarsus adductus?

A

= in-toeing present at birth that is caused by intrauterine crowding

resolves on its own within the 1st year of life

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44
Q

Metatarsus adductus occurs from

A

intrauterine crowding

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45
Q

In this condition in babies, the forefoot is adducted, and may be flexible or rigid

A

metatarsus adductus

needs referral to ortho if rigid

otherwise should resolve on its own

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46
Q

What is the most common cyanotic heart defect that presents with cyanosis in the immediate few hours after birth?

A

TGA

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47
Q

What is epidermolysis bullosa simplex?

A

= a type of epidermolysis bullosa that is genetic (AD), and is caused by defects in keratin. It presents at birth or soon after with bullae following only minor trauma.

Luckily, pts usually mostly grow out of it when they get older.

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48
Q

What is epidermolysis bullosa? What are the different types?

A

= a group of genetic diseases where there is a mutation in skin anchoring proteins, resulting in skin blistering and fragility

presents in the newborn

the types are: Epidermolysis bullosa simplex (most common and least severe), and then junctional epidermolysis bullosa and dystrophic epidermolysis bullosa (both very severe)

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49
Q

wood lamp is used to search for

A

fungi

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50
Q

If you suspect that an infant has epidermolysis bullosa, you should get this test to determine the diagnosis

A

need a skin biopsy

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51
Q

If a mom has mastitis can she still breastfeed from the affected breast?

A

yes

unless horrible abscess with visible pus

otherwise, apparently fine to continue

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52
Q

What is alopecia areata?

A

= an autoimmune process where the immune system attacks the hair follicles

presents with sudden onset round or oval patches of hair loss on the scalp

associated with other autoimmune diseases

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53
Q

The most common cause of dysphagia and food impaction in children is

A

EoE

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54
Q

“Snowman sign”

A

= a rounded, figure 8 like cardiac contour with a large supracardiac shadow in the mediastinum

seen in TAPVR and PAPVR (partial anomalous pulmonary venous return), where apparently there is also a left sided SVC (in addition to the right sided SVC), creating the snowman sign

FYI–can be hidden in a neonate by the thymic shadow

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55
Q

What is L-TGA?

A

it’s where the morphological RV/pulm artery and LV/aorta are switched

so like morphologically, RA is attached to LV

but blood circulation pathway is normal

babies and kids can be asymptomatic it sounds like

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56
Q

Straight left heart border on CXR think

A

L-TGA

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57
Q

Classic CXR finding in patients with supracardiac anomolous pulmonary venous return

A

snowman sign

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58
Q

“discordant atrioventricular and ventriculoarterial relationships” =

A

L-TGA

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59
Q

When the RV is where the LV should be and the LV is where the RV should be but everything else is ok, that’s

A

L-TGA

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60
Q

Downward displacement of an abnormal tricuspid valve into the right ventricle is found in

A

Ebstein anomoly

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61
Q

Massive cardiomegaly– a “wall to wall heart” on CXR in a neonate with cyanosis think

A

Epstein anomaly

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62
Q

Anterior deviation of the infundibular septum describes the anomaly seen in which types of congenital heart disease?

A

TOF

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63
Q

CXR findings in Truncus Arteriosus

A

cardiomegaly and increased pulmonary vascular markings with pulmonary edema

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64
Q

Narrow mediastinum with egg shaped heart on CXR, or “egg on a string” appearance =

A

D-TGA

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65
Q

Common cyanotic congenital heart disease with LVH and left axis deviation:

A

Tricuspid atresia

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66
Q

Supracardiac TAPVR will present with blank on CXR

A

snowman sign

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67
Q

What is the ONE cyanotic congenital heart disease that gets WORSE when you start prostaglandins?

A

The infradiaphragmatic form of TAPVR when there is severe obstruction to pulmonary venous return

the neonate will have severe pulmonary edema and cyanosis

(Can probs think of as increases R to L shunt?)

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68
Q

How do neonates with TAPVR present?

A

It will depend on how obstructed the pulmonary venous return is. If not too obstructed, they might actually do ok and get elective surgery. IF severely obstructed, super bad lung white out and cyanosis.

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69
Q

Auscultation findings in neonate presenting with HLHS

A

no murmur
single, loud S2

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70
Q

A random plasma glucose > blank, along with the classic symptoms of diabetes meets criteria for a diagnosis of DM

A

> 200

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71
Q

The first screening test to use to look for a complement deficiency is

A

the CH50 assay

(this measures total complement activity of the classic pathway, and a normal result would show that all factors, C1-C9, are present)

(If CH50 is low or zero, then do tests looking at each specific complement level)

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72
Q

Name two deficiencies that are associated with fulminant meningococcal disease

A

Terminal complement deficiency (C5-C9 deficiency)

Properdin deficiency

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73
Q

Early complement deficiencies refer to a deficiency in

A

C1,2,3 or 4

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74
Q

Symptoms of an early complement deficiency

A

pyogenic infections from encapsulated bacteria (aka neisseria meningitidis, strep pneumo, and h flu) PLUS tendency to have autoimmune diseases, such as lupus

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75
Q

Why are early complement deficiencies associated with also having autoimmune disease?

A

Because without complement functioning properly, immune complexes end up being deposited in different organs and joints

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76
Q

Early complement deficiencies are associated with autoimmune diseases, especially

A

Lupus

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77
Q

Lupus is especially associated with this complement deficiency

A

C2 complement deficiency

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78
Q

What is properdin?

A

= a positive complement regulator

so if you are deficient, you are immune compromised

Properdin deficiency is a rare, hereditary, primary immunodeficiency due to a complement cascade protein anomaly characterized by significantly increased susceptibility to Neisseria species infections.

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79
Q

MOA of montelukast

A

leukotriene receptor antagonist

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80
Q

The iliotibial (IT) band goes from where to where?

A

lateral hip to the lateral lower femoral condyle and lateral aspect of the proximal tibia, just below the knee

81
Q

What is iliotibial band syndrome (also called iliotibial band friction syndrome)?

A

= when the IT band gets inflamed as it passes over the lateral distal femoral condyle

presents with lateral knee pain DURING activity, commonly seen in athletes like long distance runners

82
Q

According to med study, if a sickle cell pt has osteomyelitis, the organism causing it is most likely to be

A

salmonella

83
Q

Most common cause of osteomyelitis in sickle cell patients

A

salmonella

84
Q

Psoriasis is typically present on blank surfaces

A

extensor

like elbows and knees

85
Q

A 5 yo with a known metabolic disorder is started on 500 mg/day of pyridoxine. What metabolic disorder do they have, and how can you assess response to treatment?

A

Homocystinuria

assess response to tx by monitoring homocysteine levels

86
Q

Finding alloisoleucine is diagnostic for this metabolic disease

A

Maple Syrup Urine Disease

87
Q

In Maple Syrup Urine Disease, levels of blank, blank and blank are elevated

A

leucine
isoleucine
valine

88
Q

Blank is a metabolic disorder where methionine metabolism is disrupted and not able to happen normally

A

Homocystinuria

89
Q

In homocystinuria, these two levels are elevated

A

homocystine level, duh
and methionine level
since the whole problem with this disease is that you can’t break down methionine normally

90
Q

How to test a child for homocystinuria if you suspect this clinically:

A

homocysteine level
and amino acids in plasma (to look at methionine level)

91
Q

Tx for homocystinuria

A

LARGE doses of pyridoxine (B6)

because, pyridoxine causes a decrease in the total plasma level of homocysteine

+ also will need to eat low methionine diet (methionine gets broken down into homocysteine)

92
Q

Methionine (a dietary amino acid) normally gets broken down to homocysteine in the body, which is then converted to cystathionine and then cysteine. In the metabolic disease homocystinuria, which step in this process is not able to happen?

A

Can’t convert homocysteine to cystathionine, because the enzyme Cystathionine B-synthase is missing.

so pts get elevated levels of homocysteine and methionine

93
Q

Marfan like habitus + downward lens dislocation + blood clots think

A

Homocystinuria

94
Q

Homocystinuria pattern of inheritance

A

AR

95
Q

Elevated methionine and homocystinuria levels think

A

Homocystinuria

96
Q

Tx for Homocystinuria

A

Pyridoxine (vitamin B6)

bcuz this helps convert homocysteine to cysteine (basically it enhances the activity of the deficient enzyme)

97
Q

If a patient with homocystinuria does not respond to pyridoxine supplementation, what should you do?

A

give folic acid and B12

bcuz the pt won’t respond to pyridoxine if they are folate deplete

98
Q

A child has impetigo caused by strep. They then get post strep (post-infectious) glomerulonephritis. Which antibody will tell you if indeed they recently had a SKIN strep infection?

A

Antibodies to deoxyribonuclease B

(anti-deoxyribonuclease B = anti DNase B for short)

99
Q

This antibody will be positive after a recent strep infection of the SKIN

A

Anti-deoxyribonuclease B

(this is the most sensitive and specific)

100
Q

Syndactly =

A

webbed fingers or toes

101
Q

Meds you an use to treat an acute dystonic reaction

A

benztropine or benadryl

102
Q

Patients with Type 1 diabetes have this HLA type

A

HLA-DQ2-DQ8

103
Q

Do patients presenting with celiac have an elevated CRP?

A

Nope not always

104
Q

The best initial screening test for Celiac is

A

Serum tissue transglutaminase (tTG)-IgA antibodies WITH total IgA

105
Q

Pattern of distribution of Dermatitis herpetiformis (celiac rash):

A

symmetrically, on the extensor surfaces of the arms, legs and butt

vesicular, itchy

106
Q

How do you test for celiac in a child under age 2?

A

tTG IGA and total IgA

AND DEAMINATED gliadin IgG

107
Q

Is serum gliadin IgG a good test to screen for Celiac?

A

nope

bcuz it’s not sensitive or specific

however, deaminated gliadin peptide IgG is useful when testing children < 2 yrs of age

108
Q

CO MOA of poisoning

A

binds super tight, way more tightly than oxygen does, to heme and impairs oxygen delivery

109
Q

Cyanide MOA of poisoning

A

cyanide interferes with oxygen utilization by mitochondria

110
Q

In cyanide poisoning, what will a venous blood gas show?

A

the venous blood gas will be bright red

like it will look arterial

and the O2 content will be high (even of the venous blood), because the O2 is not getting extracted at the capillary level

not good

111
Q

2 ways someone might get cyanide poisoning

A

smoke inhalation, or a nitroprusside drip (bcuz nitroprusside is metabolized to cyanide)

112
Q

How to treat cyanide poisoning

A

patients will be VERY sick with severe lactic acidosis

treat with O2, hydroxocobalamin (a precursor of vitamin B12), or sodium thiosulfate. can also add amyl nitrite. okk.

113
Q

What happens to total body water, intracellular water, and extracellular water as a kid goes from newborn to 10 years old?

A

Total body water decreases, from like 80% as a preemie to 70% as a term baby to 60% of body weight as a 10 year old.

Extracellular water also decreases, like majorly. Babies are born with a lot of extracellular water. Then that decreases.

INTRACELLULAR WATER however INCREASES with ages. Think more cells and cells grow and a bigger PERCENTAGE of total body water is intracellular in a 10 yo compared to an infant.

114
Q

Is more of the body’s fluid intracellular or extracellular?

A

Intracellular (2/3)

extracellular is only 1/3 (in an adult. it’s higher in a baby).

115
Q

What is Scheuermann kyphosis?

A

= when 3 verebrae in a row have endplate abnormalities with anterior wedging in the thoracic spine

cause is unknown

presents in teenage boys with back stiffness and kyphosis that the patient can’t correct

116
Q

Does alcohol increase your risk of kidney stones?

A

Nope!

117
Q

Do coffee and tea increase your risk of kidney stones?

A

Nope!

118
Q

The most important steps for preventing kidney stones are:

A
  1. generous fluid intake
  2. Decreased salt intake
  3. treat with a thiazide diuretic (like hydrochlorothiazide) when needed to decrease the amount of calcium in the urine
119
Q

Most kidney stones are made of calcium oxalate. Should people with kidney stones eat less calcium?

A

Nooo!!! They should actually eat MORE calcium! Eating more calcium will make their GI tract absorb less oxalate, so will actually decrease the incidence of kidney stones.

120
Q

The problem with eating too much supplemental vitamin C is …..

A

it can cause kidney stones

because vitamin C increases the amount of oxalate in the urine, predisposing you to the formation of calcium oxalate stones

121
Q

RsR’ means

A

volume overloaded RV

seen in a kid with an ASD

122
Q

Acute angle closure glaucoma is a rare but dangerous side effect of this antiepileptic drug

A

Topiramate

123
Q

How does acute-angle closure glaucoma present?

A

Eye pain, swelling and redness

124
Q

Side effects of Topiramate

A

fatigue
psychomotor slowing
acute close angle glaucoma yikes
nephrolithiasis (kidney stones)

125
Q

What is X-linked adrenoleukodystrophy?

A

= disease that presents in boys around age 7, with ADHD like symptoms/ seizures/ loss of gait and vision and then death over the course of a few years, due to the accumulation of very long chain fatty acids in the white matter of the CNS.

126
Q

What x-linked disease is caused by the body’s inability to break down very-long-chain fatty acids in peroxisomes, leading to toxic accumulation in the adrenal glands and the brain?

A

adrenoleukodystrophy

127
Q

Imaging findings in a boy presenting with seizure, found to have new diagnosis of adrenaleukodystrophy

A

symmetric periventricular demyelination in the posterior regions of the cerebral white matter

128
Q

Tx for dilated cardiomyopathy in childhood:

A

diuretics
ACE inhibitors

129
Q

Should a 7 yo get Tdap or Dtap?

A

Tdap

!

130
Q

Hib is not recommended for healthy children > blank years of age

A

5

specifically, 60 months

131
Q

If a child who has never gotten any vaccines who is now 5 years old or older and has asplenia or HIV, should they get a Hib vaccine?

A

yes, one dose

132
Q

A child has a phyisican-reported history of chicken pox, do they need the varicella vaccine?

A

nope!!

133
Q

A 7 yo has never received any vaccines. They are previously healthy. What vaccines should you give them?

A

MMR, followed by a 2nd dose at least 1 mo later.

IPV (polio) series.

Hep A and Hep B.

134
Q

Sotos syndrome

A

aka “cerebral gigantism”

patients are big and tall (>97th percentile) until age 5, when growth normalizes

often have low IQ and behavioral issues and are clumpsy

these children apparently speak in a monotone voice

it’s a spont mutation genetic condition. does NOT have any endocrine abnormalities.

135
Q

blank is a genetic syndrome where there is rapid growth in early childhood, but no evidence of an endocrine disorder

A

Sotos Syndrome

136
Q

Growth hormone levels in Sotos syndrome

A

normal

137
Q

If a fetus is exposed to this drug in utero (specifically between 6 and 9 weeks gestation), they get nasal hypoplasia and stippled epiphyses

A

Warfarin

138
Q

What are stippled epiphyses?

A

A congenital abnormality of the epiphyses marked by multiple ossification centers that severely deform the long bone and give it a stippled appearance and a thickened shaft.

139
Q

Fetal Warfarin Syndrome

A

nasal hypoplasia and stippled epiphyses

140
Q

What are ash leaf spots and what should they make you think of?

A

= hypopigemted macules

seen in Tuberous Sclerosis

141
Q

If a patient seizes and you find 3 or more ash leaf (hypopigmented) spots on their skin, they probably have this diagnosis

A

Tuberous Sclerosis

142
Q

Are scorpion bites visible?

A

no, they leave no mark!

yikes

can be tender, but won’t be visible

143
Q

Necrosis, ulceration and eschar formation at the site of the bite happens with this type of spider

A

brown recluse spider

144
Q

What is Smith-Lemli-Opitz syndrome?

A

= an AR disorder of cholesterol metabolism

Due to deficiency of 7-dehydrocholesterol reductase, causing an increase in plasma 7-dehydrocholesterol and a decrease in plasma cholesterol.

Babies have dysmorphic facial features, abnormal thumbs, overlapping fingers, syndactly, and ambiguous genitalia. This all happens because cholesterol is very important to embryogenesis, so without cholesterol you do not get normal embryogenesis.

145
Q

2/3 toe syndactly think

A

Smith-Lemli-Opitz Syndrome

146
Q

A newborn has microcephaly with narrow bifrontal diameter, cleft palate, hypospadias, and 2/3 syndactly. What syndrome does this baby have, and what lab abnormalities will you see?

A

Smith-Lemli-Opitz Syndrome

low total cholesterol
elevated 7-dehydrocholesterol

147
Q

Glycogen storage disease type 1 is due to a deficiency of one of these two enzymes

A

glucose-6-phosphatase
glucose-6-phosphatase translocase

the problem is you can’t break down glycogen

148
Q

If you do a study where you divide groups by outcome and then compare the risk factors that exist between the two groups, that is a blank type of study

A

case-control study

149
Q

In a blank study, the subjects are divided into groups based on the presence or absence of risk factors, and then are followed (either in the past or prospective follow in real time) to see if certain outcomes (like a certain disease) develops.

A

cohort study

150
Q

In a study, if patients are grouped by outcome, that is a blank type of study

A

case-control

151
Q

“Meth mouth” refers to

A

dry mouth leading to tooth decay in meth users

152
Q

How to distinguish epiglottitis from bacterial tracheitis:

A

epiglottitis: drooling and difficulty swallowing from severe sore throat. would not have a prominent cough.

bacterial tracheitis: does have a prominent cough. is like viral croup presentation but then the child rapidly deteriorates.

both will have fever, stridor and resp distress

153
Q

Epligottitis prominent symptom

A

drooling

154
Q

Bacterial tracheitis prominent symptom

A

croup like cough

155
Q

What is cutaneous larva migrans?

A

in Brazil it’s called “geographic bug” “bishu geographico” because it looks like a map, the red serpiginous lines that you get on your feet

it’s caused by dog and cat hookworms

often get when walking barefoot, like on the beach

treat with albendazole

156
Q

Serpiginous map like red itchy rash that you get on your feet after walking barefoot at the beach =

A

Cutaneous larva migrans

aka dog and cat hookworm (ancylostoma braziliense, A. caninum)

157
Q

Treat hookworm infections with

A

albendazole or pyrantel pamoate

158
Q

How to treat cutaneous larva migrans (a type of hookworm infection)

A

albendazole or ivermectin

hookworm
think footworm
to help remember the rash on the feet

159
Q

A 20 mo girl presents with regression of both motor and language milestones, stereotypic hand movements, and deceleration of head growth, all consistent with blank syndrome

A

Rett syndrome :(

160
Q

Why are patients with Rett syndrome at higher risk for sudden cardiac death than the general population?

A

Becuase in Rett syndrome, there is abnormal regulation of the autonomic nervous system, and this leads to QTc prolongation and cardiac electrical instability.

161
Q

Which drugs are known to decrease the efficacy of OCPs?

A

Antiseizure meds– like:

carbamazapine
lamotrigine
oxcarbazepine
phenytoin
phenobarbital
topiramate

AND Rifampin and rifabutin (abx).

162
Q

Which antibiotics are the only antibiotics known to decrease the effectiveness of OCPs?

A

Rifampin and rifabutin

163
Q

Steeple sign on xray in croup is due to narrowing of what?

A

subglottic narrowing

164
Q

Loss of normal shoulders of the subglottic airway refers to…..

A

subglottic narrowing seen in croup

aka steeple sign

165
Q

When should babies with cryptorchidism (aka undescended testes) be referred for surgical consultation?

A

6 mo actually

166
Q

When should babies with cryptorchidism (aka undescended testes) be referred for surgical consultation?

A

6 mo actually

167
Q

Undescended testes have a risk of

A

cancer

and like a MINIMALLY increased risk of infertility

but the major issue is the increased risk of cancer

168
Q

Are retractile testes fine?

A

yes
will almost always end up in the scrotum so no intervention is necessary

169
Q

PCP (Phencyclidine) toxidrome

A

= a hallucinogen

causes hyperreflexia, clonus, muscle rigidity, nystagmus, and drooling. Also seizures.

Causes temp and BP instability. Pts can become acutely psychotic and have frightening hallucinations.

170
Q

Seeing smells and hearing colors think

A

LSD
artsy
think Lucy in the Sky with Diamonds

171
Q

MDMA is known as

A

Molly or Ecstasy

172
Q

How to distinguish PCP toxidrome from MDMA

A

PCP has nystagmus
(MDMA doesn’t)

173
Q

Perioral eczematous changes think

A

inhalants

174
Q

Patients with this inherited disease have recurrent skin infections, pneumonias, and episodes of lymphadenitis caused by catalase positive organisms

A

CGD (chronic granulomatous disease)

ex of a catalase positive organism: staph aureus

175
Q

Hereditary defect where you can’t form reactive oxygen species =

A

CGD

176
Q

Patients who get pneumonias and skin infections caused by catalase positive organisms think

A

CGD

177
Q

What disease is due to a failure of the patient’s phagocytic leukocytes to kill pathogens using the oxidative burst?

A

CGD

NADPH oxidase is defective in these patients

they can form granulomas around pathogens but then can’t do anything else

178
Q

Why are CGD patients prone to infections with catalase positive organisms? Name some catalase positive organisms.

A

Staph aureus
Nocardia
Aspergillus
Burkholderia cepacia
Serratia
Salmonella

179
Q

The test of choice to determine if a patient has CGD is

A

the Dihydrorhodamine oxidation test

“DHR” oxidation test

180
Q

A young child has recurrent otitis media, draining ears, eczema, and FTT. The diagnosis is likely….

A

Wiskott Aldrich Syndrome

181
Q

Why do CGD patients get infections over and over again with catalase POSITIVE organisms, but do fine with catalase negative organisms?

A

It is believed that their phagocytes can use the hydrogen peroxide/oxidative burst materials supplied by catalase neg bacteria. Can’t do this with catalase positive bacteria, since catalse I think must like destroy hydrogen peroxide.

182
Q

Name 3 vaccines that can be administered subQ:

A

MMR
varicella
IPV

183
Q

How to treat a patient with idiopathic hypercalciuria:

A

The patient should be treated, to avoid the risk of developing nephrocalcinosis (calcium building up in the kidneys which can eventually cause kidney failure). Tx means:
-push fluids
-limit sodium intake
-watch for signs of kidney stones

ok, cool…

184
Q

What is rosacea?

A

= a chronic disorder where you get facial redness, often with a papulopustular component

temp changes, exercise, sun, alcohol, spicy foods, hot beverages, all trigger the redness

it’s etiology is unclear. most likely to occur in fair skinned individuals.

185
Q

How to treat papulopustular rosacea

A

topical metronidazole

186
Q

Bones with “ground glass” appearance on xray think

A

Scurvy

187
Q

Bone pain, widening of the wrist and knees, and bowed legs think

A

Rickets

188
Q

Lucent metaphyseal band think

A

Syphillis

189
Q

What is Treacher Collins syndrome?

A

Also called mandibulofacial dysostosis (meaning defective bone). You get midface hypopasia, choanal stenosis or atresia, cleft palate, hypoplastic lower eyelids and ABSENT lower lid lashes. Also get outer and middle ear malformations, so conductive hearing loss is common.

190
Q

Choanal stensis, midface hypoplasia, downward slanting eyes, and absent lower eyelid lashes think

A

Treacher Collins Syndrome

191
Q

Shagreen patches

A

= fleshy, irregular plaques often found on the lower back in patients with Tuberous sclerosis

192
Q

Options for prophylaxis for neisseria meningitis for close contact:

A

CTX once
Rifampin BID x 2 days
or
Cipro once (can be used in pts > 1 month of age)

193
Q

Kallman syndrome is frequently associated with what other defects?

A

midface defects like cleft lip and palate

also congenital heart disease and renal agenesis

194
Q

What is acrodermatitis enteropathica?

A

= an AR disorder where you can’t absorb zinc from the GI tract

195
Q

Genetic disease where zinc is not adequately absorbed

A

Acrodermatitis enteropathica

196
Q

How does acrodermatitis enteropathica present?

A

In this rare AR disorder, the patient’s GI tract is very bad at absorbing zinc.

However, breastmilk contains the missing zinc-binding factor necessary for absorption, so infants who are breast feeding will do just fine.

When they switch to formula, a few weeks later they will present with severe zinc deficiency: irritability, new FTT, diarrhea, and rash.

197
Q

How to treat Acrodermatitis enteropathica

A

give life long zinc supplementation

198
Q

Best treatment for comedones in acne

A

topical retinoids

199
Q

Which type of med is particularly effective for comedonal acne?

A

retinoids

Pediatric Boards (9 decks)

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