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Pediatric Boards > Med Study 4 > Flashcards

Med Study 4 Flashcards

1
Q

Why do button batteries need to be emergently removed from the esophagus?

A

because they can cause immediate liquefactive necrosis

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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

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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

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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

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5
Q

Blank deficiency is common in children who are fed regular goat’s milk

A

folate deficiency

can lead to severeee anemia

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6
Q

Type 1 glycogen storage disease, which presents in infants with rapid fasting hypoglycemia, is also called

A

von Gierke disease

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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)

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8
Q

What does the enzyme 5-alpha reductase do?

A

It converts testosterone to dihydrotestosterone

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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

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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

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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

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12
Q

What are testosterone levels like in androgen insensitivity syndrome?

A

normal or high

and the testosterone : dihydrotestosterone level is normal

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13
Q

Elevated testosterone : dihydrotestosterone think

A

alpha-5-reductase deficiency

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14
Q

Broad, stalk-like projection of bone on x-ray think

A

osteochondroma

= benign bone tumor

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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)

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16
Q

Should osteochondromas always be removed?

A

nope, only if they are causing symptoms

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17
Q

Pain, palpable bone mass, and “sunburst” appearance on x-ray =

A

osteosarcoma

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18
Q

Sunburst appearance of bone formation on x-ray + codman triangle =

A

osteosarcoma

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19
Q

Periosteal elevavation (aka onion skinning) on x-ray =

A

Ewing sarcoma
Ewwwwwww onion

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20
Q

Do osteosarcomas and Ewing sarcomas present similarly?

A

yup

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21
Q

Blank is a benign bone tumor that causes SEVERE nighttime pain

A

osteoid osteoma

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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

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23
Q

Nail pitting, dactylitis, and anterior uveitis are associated with this autoimmune disease

A

juvenile psoriatic arthritis

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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).

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25
Q

Sausage digit think

A

juvenile psoriatic arthritis

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26
Q

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

A

psoriatic juvenile arthritis

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27
Q

The rooting reflex should be gone by

A

4 months

(so if you see it still present in a 6 month old, that is ABNORMAL)

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28
Q

What is the parachute reflex? When does it appear?

A

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.

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29
Q

When does the moro reflex (aka startle reflex) go away?

A

by 3-6 months

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30
Q

What is the asymmetric tonic neck reflex, and when is it present?

A

= 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.

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31
Q

The tonic neck reflex is often called the

A

fencing reflex

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32
Q

When should the tonic neck reflex disappear by?

A

by 6 months of age

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33
Q

The tonic neck relfex, aka the fencing reflex, is most prominent at this age

A

1 mo of age

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34
Q

Is a rooting reflex at 6 months of age normal?

A

Nope, that should really be gone by age 4 mo

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35
Q

Is an infant who still has a moro reflex and a tonic neck response at age 2 mo normal?

A

yup totally

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36
Q

If a child has pertussis, what should you recommend for their other family members in their household?

A

chemoprophylaxis

prophylax them all with azithro

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37
Q

Lab finding in pertussis

A

leukocytosis with lymphocytosis

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38
Q

The CRAFFT screening is screening for what?

A

drug and alcohol use in teens

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39
Q

SIGECAPS screens for……

A

depression

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40
Q

SAD PERSONS screens for

A

suicide risk

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41
Q

Nail pitting and arthritis are associated with

A

psoriasis

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42
Q

What is dyshidrotic ecezema?

A

Affects palms, soles and fingers. You get VESICLES that are super itchy and super painful.

Sweating (hyperhidrosis) makes it worse.

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43
Q

This type of eczema presents with painful/itching blisters (vesicles and bullae) on the hands and feet, and is made worse by blank

A

dishidrotic eczema

associated with hyperhidrosis, which makes it way worse

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44
Q

What is the corpus callosum?

A

= the band of white matter in the brain that connects the two hemispheres

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45
Q

What is a bronchogenic cyst?

A

= 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

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46
Q

Blank is a NON-IgE mediated sensitivity to milk protein

A

milk protein allergy

seen in infants

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47
Q

What is FPIES?

A

= 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

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48
Q

What is failure modes and effect analysis (FMEA)?

A

= 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)

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49
Q

PPV=

A

TP/(TP + FP)

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50
Q

What are the congenital heart defects with left axis deviation?

A

AV canal (partial or complete), and tricuspid atresia

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51
Q

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.

A

Tricuspid atresia

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52
Q

How does tricupsid atresia present?

A

With major cyanosis as the ductus begins to close

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53
Q

Neonate has cyanosis with sats of 70%, and ECG findings of left superior axis deviation and LVH. The diagnosis is

A

tricuspid atresia

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54
Q

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.

A

Tricuspid atresia

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55
Q

Anatomy in total anomalous pulmonary venous return

A

= 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

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56
Q

Congenital heart disease where there is a R to L shunt presents how?

A

with cyanosis in the neonate

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57
Q

Five “T”s of cyanotic congenital heart disease

A

Tetralogy of Fallot
TGA
Trucus arteriosus
Tricuspid atresia
TAPVR

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58
Q

Which vaccine contains the largest amount of egg protein?

A

The yellow fever vaccine

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59
Q

According to med study, can a patient with egg allergy still get the flu vaccine?

A

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..

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60
Q

Does “abdominal strapping” help for treating umbilical hernias?

A

NOPE

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61
Q

When to recommend surgical repair of an umbilical hernia:

A

At 5-6 years of age if hasn’t resolved on it’s own

OR

if it enlarges after 1 year of age

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62
Q

The most common infection to cause erythema nodosum is

A

strep

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63
Q

Pink to violaceous tender nodules on the shins think

A

erythema nodosum

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64
Q

What is erythema nodosum?

A

= 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

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65
Q

After getting IVIG, avoid live vaccines for at least

A

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)

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66
Q

If a parent has HCM, what test should you get in the child?

A

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)

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67
Q

What is a fixed drug eruption?

A

is a distinctive type of cutaneous drug reaction that characteristically recurs in the same locations upon re-exposure to the offending drug.

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68
Q

In what locations do fixed drug eruptions typically appear?

A

On the hands, trunk, genital and perioral areas

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69
Q

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

a fixed drug eruption

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70
Q

The most common trigger for a fixed drug eruption is

A

Bactrim

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71
Q

Name 3 diseases where you get a rash on the palms and soles

A

syphilis
rocky mountain spotted fever
coxsackie A virus (HFM disease)

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72
Q

The most common cause of toilet training refusal is

A

“a strong-willed child”

LOL

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73
Q

The cardiac defect that is most common in Noonan syndrome patients is

A

pulmonic stenosis

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74
Q

Systolic ejection click on the LUSB + a harsh systolic crescendo-decrescendo murmur =

A

pulmonic stenosis

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75
Q

4/6 crescendo-decrescendo systolic murmur with palpable thrill at the 2nd left intercostal space =

A

PS (pulmonic stenosis)

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76
Q

Webbing of the neck in a boy think

A

Noonan syndrome

(apparently in the past, Noonan syndrome was referred to as “male Turner syndrome” due to some similar features)

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77
Q

Prominent webbing of the neck and bilateral ptosis in a boy who is short think

A

Noonan syndrome

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78
Q

What is postpericariotomy syndrome?

A

= 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.

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79
Q

Muffled heart sounds think

A

pericardial effusion

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80
Q

Acoustic shadowing in the gallbladder on ultrasound is indicative of

A

gallstones

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81
Q

Male neonate with deficient abdominal musculature, cryptorchidism, and urinary tract anomalies =

A

prune belly syndrome

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82
Q

What does VACTERL stand for?

A

Vertebral defects
Anal atresia
Cardiac defects
Tracheoesophageal fistula
Renal anomolies
Limb abnormalities

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83
Q

The cause of colic is…….

A

unknown

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84
Q

Colic usually resolves by this age

A

should be gone by age 4 mo

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85
Q

When is it normal to see genu valgum (knock knees)?

A

age 2-4

(before age 2, there is natural bowing of the knees. after age 4, legs should be pretty straight)

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86
Q

Is genu valgum in a 3 year old child normal?

A

Yup

(genu valgum is normal from age 2-4)

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87
Q

Is genu valgum normal in a 15 mo child?

A

NO

they legs should actually be bowed at this age

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88
Q

Is persistence of genu valgum after age 4 normal?

A

NO

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89
Q

Genu valgum is normal in

A

pre school

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90
Q

Septic thrombophlebitis of the jugular vein is called blank. It is caused by blank.

A

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

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91
Q

Lemiere syndrome is associated with what complication?

A

septic emboli to the lungs

yikes

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92
Q

Lupus rash gets worse with

A

sun exposure

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93
Q

Sodium bicarb is given to alkalinize the urine in blank overdoses

A

aspirin

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94
Q

Sodium bicarb is given to address QRS prolongation in blank overdoses

A

TCA

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95
Q

Fomepizole is given to treat ingestions of

A

toxic alcohols like methanol and ethylene glycol

(fomepizole is the same thing as 4-methylpyrazole)

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96
Q

If a patient presents 24-36 hours after a tylenol overdose, should you still give NAC?

A

actually yes, since it may still have some benefit

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97
Q

Tx for Trichomonas vaginalis

A

Metronidazole

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98
Q

Pathophys of why hearing loss can happen after bacterial meningitis

A

due to damage to the acoustic nerve (8th cranial nerve)

so the result is sensorineural hearing loss

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99
Q

Type of hearing loss that can happen after bacterial meningitis and how to evaluate for it

A

sensorineural hearing loss

ABR (auditory brainstem response)

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100
Q

How does an ABR (auditory brainstem response) test work?

A

A sound occurs, and then brainstem activity is monitored (rather than monitoring patient response to determine if a sound was heard)

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101
Q

What does the ABR (auditory brainstem response) test measure?

A

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).

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102
Q

All infants should have a hearing screening by this age

A

1 month

(ideally can do before they are discharged from the nursery)

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103
Q

What is the otoacoustic emissions (OAE) test and how does it work?

A

= 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)

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104
Q

Otoacoustic emissions testing provides information on the function of

A

the outer hair cells of the cochlea

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105
Q

What is Peutz-Jeghers syndrome?

A

= and AD disorder where you get mucocutaneous lesions and intestinal hamartomas

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106
Q

Peutz-Jeghers Syndrome is also called

A

hereditary intestinal polyposis syndrome

presents with dark mucocutaneous lesions of the lips and gums, and with intestinal hamartomas (that can develop into cancer)

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106
Q

Peutz-Jeghers Syndrome is also called

A

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

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107
Q

How does Puetz-Jeghers syndrome usually present?

A

intermittent colicky abdominal pain

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108
Q

Who should get the PPSV23 vaccine?

A

children who are at least 2 years old and who are at increased risk for invasive pneumococcal disease

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109
Q

PCV13 is routinely recommended for all children at what ages? How many total doses should they get?

A

4 total doses

at ages 2, 4, 6 months and then last dose at 12-15 mo

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110
Q

For children getting the PPSV23 vaccine because they are high risk, what should you make sure of first?

A

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

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111
Q

PPSV23 can’t be given to anyone under the age of

A

2

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112
Q

How many doses of PPSV23 should sickle cell (and all asplenic) patients get?

A

2

5 years apart

(however of note, cochlear implant pts only need one dose)

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113
Q

Mississippi and Ohio River valleys think

A

Histoplasmosis

a fungal infection that can present as pneumonia

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114
Q

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).

A

Blastomycosis

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115
Q

Blank is a fungal infection that can present with pneumonia and occurs in the deserts of the southwestern US.

A

Coccidiomycosis

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116
Q

Elevated urinary levels of homovanillic acid (HVA) and vanillylmandelic acid (VMA) are suggestive of

A

the presence of a catecholamine-secreting tumor (like neuroblastoma or a pheochromocytoma)

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117
Q

soft palate petechiae think

A

GAS pharyngitis

but could also be mono

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118
Q

What is Herpangina and what is it caused by?

A

caused by coxsackievirus

= sudden onset fever and vesicular lesions on the posterior pharyngeal wall, uvula, and tonsils

lesions rupture and form ulcers

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119
Q

How to distinguish between herpangina (caused by coxsackie virus), and herpetic gingivostomatitis (caused by HSV):

A

In Herpangina, the tongue and gingival surfaces are NOT affected by the rash

so the tongue and gums are spared

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120
Q

Vesicles that have ulcerated that are present only on the posterior pharyngeal wall and NOT the tongue or gums =

A

Herpangina

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121
Q

How do infants and toddlers with Herpangina present?

A

usually in the summer with high fever, excessive drooling, feeding difficulties and posterior pharynx lesions

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122
Q

Ulcerations of the buccal mucosa and tongue with gingival hyperemia = the findings seen in…

A

herpetic gingivostomatitis

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123
Q

HSV occurs in the front part of the mouth and lips, whereas Herpangina occurs in the…..

A

posterior oropharynx
back of the mouth

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124
Q

Cherry-red spot on fundoscopic exam =

A

Tay-Sachs disease

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125
Q

Pathophys of Tay-Sachs disease

A

= 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.

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126
Q

Cherry-red spot on the retina =

A

Tay-Sachs disease

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127
Q

Tay-Sachs disease eye finding

A

cherry red spot on the retina

wait. apparently you can see it other disease too ahhhh

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128
Q

Early sign of Tay-Sachs disease

A

increased startle response

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129
Q

Tay-Sachs disease is most common in which populations?

A

Eastern European Jewish
French Canadian
Cajun

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130
Q

8 mo with developmental regression, startle myoclonus, muscle weakness, and cherry red spot on eye exam =

A

Tay-Sachs disease

131
Q

How to manage a child under age 5 who has had a direct exposure to TB

A

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
Q

The interferon gamma release assay for TB testing can only be used on children over age

A

2

so 24 mo and up, you can use this

133
Q

After getting the MMR vaccine, how long do you need to wait until you place a PPD test?

A

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
Q

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)?

A

Menveo

135
Q

For an asplenic infant, which meningococcal vaccine should you give and when

A

Menveo

4 doses

at 2,4,6 and 12 mo

136
Q

What is juvenile plantar dermatosis?

A

= 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
Q

“Sweat sock dermatitis” or “Sneaker dermatitis” refers to

A

Juvenile plantar dermatosis

138
Q

What is Intertrigo?

A

= 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
Q

Nummular eczema looks like..

A

coin like
ROUND
lesions

140
Q

Which type of eczema is characterized by vesicles on the sides of the fingers?

A

dyshidrotic eczema

VERY itchy, and also painful

141
Q

This condition frequently occurs with dyshidrotic eczema

A

hyperhidrosis

142
Q

xer means

A

dry

so xerophthalmia = dry eyes
xerosis = dry skin

143
Q

In babies with eczema, this area is typically spared

A

diaper area
since it’s protected from scratching

144
Q

Most common areas for eczema to occur in pts ages 0-2

A

cheeks
extensor surfaces of extremities

after these ages it switches to the flexor surfaces
tricky

145
Q

emollient means

A

hydrator

so like a cream or ointment that is hydrating (these work better than lotions)

146
Q

grouped vesicles on an erythematous base

A

= HSV

147
Q

What is Lichen Striatus?

A

= 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
Q

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.

A

Lichen striatus

149
Q

V1 distribution of port wine stain

A

= concern for Sturge Weber

V1 = (ophthalmic branch, central face/and nose area)

150
Q

E tox spares which areas?

A

palms and soles

151
Q

What is lymphogranuloma venerum?

A

= 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
Q

Options for prophylaxis against neisseria meningitis after close contact

A

Rifampin x 2 days
or single dose ceftriaxone

153
Q

Clinda has good gram pos and aneorobe coverage. It does not have blank coverage

A

gram neg

154
Q

If a patient is allergic to azithro, treat mycoplasma pneumoniae with

A

doxy

155
Q

Fluid management for burn pts:

A

3 mL/kg for each %BSA in the first 24 hours, with 50% of this being given over the first 8 hours

156
Q

What is Behcet disease?

A

= a vasculitis that presents with painful oral and genital ulcers, as well as inflammatory eye disease

157
Q

benign iris hamartomas seen in NF1 are called

A

Lisch nodules

158
Q

Always refer kids with NF1 to ophtho so they can be watched for

A

optic gliomas

159
Q

What are neurofibromas?

A

= benign, peripheral nerve sheath tumors that occur in NF1

160
Q

Pathophys of NF1

A

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
Q

Bony manifestations of NF1

A

-long bone thinning and bowing (usually the tibia), which predisposes to fracture
-sphenoid wing dysplasia
-dysplastic scoliosis

162
Q

What is the double disc diffusion test (“the D-test”)?

A

= 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
Q

This test determines whether a specific MRSA strain will be sensitive to clinda

A

D-test

164
Q

Most infants acomplish a pincer grasp by what age?

A

12 mo

165
Q

When should you be able to do a pincer grasp?

A

age 1
12 mo

166
Q

What is PANDAS?

A

= 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
Q

The worsening of these specific neuropsych conditions can happen during an acute strep infection

A

OCD and tic disorders

168
Q

What is Fanconi anemia?

A

= 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
Q

Hypersegmented polymorphonuclear lymphocytes on peripheral blood smear are suggestive of

A

B12 or folate deficiency

170
Q

Trisomy 18 is also called

A

Edwards syndrome

E for Edwards, E for Eighteen

171
Q

Trisomy 13 is also called

A

Patau syndrome

172
Q

Neonate with flexed fingers where the index finger is overlapping the 3rd finger, and the pinkie finger is overlapping the 4th finger think

A

Trisomy 18
Edwards syndrome

173
Q

This type of trisomy has the most severe craniofacial and midline defects

A

Trisomy 13 (Pataeu syndrome)

includes things like holoprosencephaly, midline cleft defects, cutus aplasia (punched out lesions of the scalp)

174
Q

What is transient myeloproliferative disorder?

A

= 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
Q

Clenched overlapping fingers think

A

Trisomy 18

176
Q

Trisomy where polydactyly is most common

A

13

177
Q

Aplasia cutis think

A

Trisomy 13

178
Q

A baby is born with polydactly and aplasia cutis congenita. The diagnosis is

A

Trisomy 13

179
Q

A baby is born with holoprosencephaly and microphthalmia. The diagnosis is

A

Trisomy 13

180
Q

When should a left sided varicocele that is present in an adolescent boy but only when standing be repaired?

A

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
Q

Patients with cyclic neutropenia are at particular risk for sepsis caused by

A

Clostridium septicum

182
Q

Purulent conjunctivitis (pink eye) is caused by infection with

A

H flu

183
Q

Clostridium septicum bacteremia think

A

cyclic neutropenia

184
Q

Multiple areas of osteochondritis and periostitis of the long bones is seen in what disease

A

congenital syphilis

185
Q

A baby has “widening of the growth plates associated with metaphyseal fraying and cupping of the distal radius and ulna.” The diagnosis is

A

Rickets from vitamin D deficiency

186
Q

What is Rickets?

A

= inadequate mineralization of the growth plates and generalized softening of the bones due to not enough vit D

187
Q

Widening of costochondral junctions is seen in

A

Rickets (Vitamin D deficiency) – this is called “rachitic rosary” when its at the anterior ribs

188
Q

Darker skin makes you more likely to be blank deficient

A

vitamin D

189
Q

Labs associated with Rickets

A

low vit D
high PTH
high alk phos

190
Q

Vitamin D deficiency is a level less than

A

30

191
Q

a submandibular space infection is also called

A

Ludwig angina

192
Q

Treat Rocky Mountain Spotted Fever with

A

doxy

193
Q

Where does Rocky Mountain spotted fever occur?

A

Mid Atlantic states / South East
(despite its name)

for ex, North Carolina

194
Q

Patients who present with Rocky Mountain spotted fever will have these lab findings:

A

hyponatremia and thrombocytopenia

195
Q

Treat all ricketsial infections (aka rocky mountain spotted fever) with blank, REGARDLESS of the child’s age

A

doxy

EVEN IF younger than 8, STILL treat with doxy, it’s THAT important and can die otherwise

196
Q

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

A

Rocky Mountain Spotted Fever

197
Q

How do you get Rocky Mountain Spotted fever?

A

from ticks carrying the bacteria Rickettsia rickettsii

198
Q

What is the most common cause of congenital hypothyroidism?

A

Thyroid dysgenesis

199
Q

What is Bartter syndrome?

A

= 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
Q

Blank syndrome presents in the neonate with severe dehydration after prenatal polyhydramnios. The infant will have calcium in the urine.

A

Bartter syndrome

201
Q

At this age, children will parallel play

A

2 years

(will see more parallel play at this age, not so much cooperative play yet)

202
Q

Most children cannot dress themselves completely, including doing buttons, until this age

A

until they are 3

203
Q

Children at this age should have 50 words and be able to use 2 word sentences

A

age 2

204
Q

A child of this age can identify 6 body parts

A

24 mo

205
Q

What is von Hippel-Lindau syndrome?

A

= an AD tumor predisposition syndrome where you get both benign and malignant tumors of the CNS, kidneys, pancreas, adrenal and reproductive glands

206
Q

Classic presentation for von Hippel-Lindau syndrome

A

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
Q

A 10 yo presents with a hemangioblastoma in the cerebellum and a hemangioblastoma in the retina. The diagnosis is

A

Von Hippel-Lindau syndrome

208
Q

Outbreak of kids at summer camp with fever, conjunctivitis and pharyngitis think

A

adenovirus

209
Q

Using the Holliday-Segar formula, calculate the daily kcal required under basal conditions for a 23 kg child:

A

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
Q

When should breastfed preterm infants start iron supplementation?

A

at 1 month of age

through 12 mo of age

211
Q

What is juvenile nephronophthisis type 1?

A

= a rare AR disease where there is salt wasting WITHOUT nephrotic or nephritic syndrome.

ESRD usually occurs by age 13.

212
Q

What is nephronophthisis?

A

= AR disease where you get “tubulointersistial sclerosis” that leads to ESRD

213
Q

How do kids with juvenile nephronophthisis type 1 present?

A

with polyuria, polydipsia, anemia, salt wasting, FTT, and progression to ESRD, + associated ocular problems

214
Q

gram-positive diplococci think

A

strep pneumo

215
Q

How does strep pneumo pneumonia classically present?

A

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
Q

All types of osteogenesis imperfecta are caused by an abnormal structure of….

A

Type 1 collagen

217
Q

Blank disease is a common cause of pediatric heel pain and refers to inflammation of the calcaneal growth plate where the Achilles tendon inserts

A

Severe disease

218
Q

Sever disease usually occurs in the context of….

A

running and jumping activities (For ex soccer or basketball) during a child’s growth spurt

219
Q

Ludwig’s angina is most often a severe complication of

A

a dental infection

220
Q

What is Ludwig’s angina?

A

= 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
Q

What is spondylolysis?

A

= 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
Q

Spondylolysis of L5 in a gymnast or ballet dancer can be complicated by…

A

spondylolisthesis (where the L5 vertebra slips onto the S1 vertebra)

223
Q

stress fracture of the pars interarticularis is called

A

spondylolysis

224
Q

scottie dog appearance on spinal x ray think

A

spondylolysis

225
Q

How to treat spondylolysis and spondylolisthesis

A

initially conservatively
rest
heat
ice
NSAIDs
only refer to for surgical intervention if refractory to conservative management

226
Q

Treatment for KD

A

IVIG and aspirin

227
Q

KD is most common in children under age

A

5

228
Q

TEN involves >blank percent of the body surface area

A

> 30%

229
Q

IM ceftriaxone is used to treat blank infections

A

gonococcal

230
Q

This cause of bacterial diarrhea can also cause vulvovaginitis with bloody or serosanguinous vaginal discharge

A

Shigella

231
Q

Shigella diarrhea can cause blank in infants and children

A

seizures!

232
Q

Infant with high fever and new onset seizure undergoes LP. During LP, baby has large bloody stool. The diagnosis is….

A

Shigella

233
Q

A 6 yo girl presents with 2 days of blood tinged yellowish vaginal discharge, abdominal pain and watery diarrhea. The diagnosis is

A

Shigella

234
Q

Should you treat shigella diarrhea?

A

Only if severe

with azithro or ceftriaxone

235
Q

What is “premature thelarche?”

A

= a normal variant of breast budding in infants and toddlers

NOT a diagnosis made in kids or adolescents

236
Q

hypertelorism

A

= an abnormally large distance between the eyes

237
Q

How to remember that Hunter’s disease has NO corneal clouding (unlike Hurler disaese)

A

you have to be able to see well to hunt
so there is no corneal clouding in Hunter’s disease

238
Q

Coarse facial appearance, developmental delays by age 2, and joint stiffness think

A

Hunter syndrome

239
Q

Why do patients with Hunter syndrome develop myocardial hypertrophy as they grow older?

A

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
Q

If a child is going to be around immunocompromised individuals, they should not get these two vaccines

A

LIVE influenza vaccine
LIVE polio vaccine

but MMR is fine!!!!

241
Q

Symptoms of serotonin syndrome

A

tachycardia
hypertension
hyperthermia
confusion/hallucinations/agitation
sweating
diarrhea
LE rigiidity, hyperreflexia and myoclonus

242
Q

Myoclonus

A

= sudden, brief involuntary twitching or jerking of a muscle or group of muscles.

243
Q

The most specific exam finding in serotonin syndrome is

A

myoclonus

244
Q

What is neuroleptic malignant syndrome?

A

= bad thing that can happen from antipsychotic drugs

presents similarly to serotonin syndrome, with severe muscle rigidity, hyperthermia, confusion, and autonomic instability

245
Q

How to distinguish between NMS and serotonin syndrome

A

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
Q

Precocious puberty is defined as the onset of secondary sex characteristics in girls before age blank, and boys before age blank

A

girls before age 8
or boys before age 9

247
Q

What is koilonychia?

A

= a spooning deformity of the finger and toe nails that can be seen in IDA (iron deficiency anemia)

248
Q

Hypochromia indicates

A

a decrease in the concentration of intracellular hemoglobin

249
Q

It’s abnormal if a child continues to suck their thumb past this age

A

4

250
Q

The most common fatty acid beta oxidation disorder is

A

medium-chain acyl-CoA dehydrogenase deficiency

251
Q

Medium chain acyl CoA dehydrogenase deficiency patients can end up with low carnitine because ….

A

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
Q

The most common inflammatory myopathy of childhood and adolescence is

A

dermatomyositis

253
Q

What are chillblains?

A

Chilblains (CHILL-blayns) are the painful inflammation of small blood vessels in your skin that occur in response to repeated exposure to cold

254
Q

How do patients with juvenile dermatomyositis present?

A

with rash (heliotrope rash around eyes and Gottron papules on hands) and symmetric, proximal muscle weakness.

255
Q

Gottron papules

A

= red plaques over the extensor surfaces, most commonly on the hands in patients with dermatomyositis

256
Q

Physical exam in avascular necrosis of the femoral head

A

the patient will typically stand with the leg slightly externally rotated; internal rotation will cause pain

257
Q

Risk factors for avascular necrosis of the femoral head

A

chronic systemic steroids
Lupus
chronic renal failure
sickle cell disease
or can be idiopathic (Legg-Calve-Perthes disease)

258
Q

Xray of the hip shows flattening and fragmentation of the left femoral head and epiphysis. The diagnosis is

A

avascular necrosis of the femoral head

259
Q

“ratty” fuzzy/fragemneted appearance of the femoral head think

A

avascular necrosis

260
Q

3 things that are contraindications for renal transplanation

A

active infection
uncontrolled malignancy
ABO incompatibility

261
Q

Does a child have to be on dialysis before getting a renal transplant?

A

NOPE! In fact, preemptive transplant is preferred

262
Q

2 yo with FTT, history of IUGR, oligodactly, and HIRSUTISM has what syndrome?

A

Cornelia de Lange syndrome

genetic syndrome caused by sporadic AD mutation

263
Q

Key features of Cornelia de Lange syndrome

A

hirsutism
growth restriction
characteristic facial features with synophrys (fusion of eyebrows above the nose)
self injurious behaviors
hand/feet/limb shortening or abnormalities

264
Q

This genetic syndrome has hirsuitism as one of its key presenting features

A

Cornelia de Lange syndrome

265
Q

At what age are 90% of children able to sit without support?

A

7 months

266
Q

Vitamin C is also called

A

ascorbic acid

267
Q

Blank are melanocytic hamartomas of the iris, often associated with neurofibromatosis type 1

A

Lisch nodules

268
Q

Severe vitamin C deficiency leads to impaired blank synthesis, which leads to connective tissue abnormalities

A

collagen

the lack of normal collagen results in fragile capillaries and hemorrhages

bones also become brittle and fracture easily

269
Q

Why do patients with Scurvy get arthralgias and joint swelling?

A

due to hemarthrosis

270
Q

Generalized rash involving perifollicular hemorrhages and follicular hyperkeratosis think

A

Scurvy

271
Q

Gingival swelling and bleeding think

A

Scurvy

272
Q

White lines of Frankel

A

= dense white bands at the metaphysis seen in vitamin C deficiency

273
Q

upward lens dislocation is seen in what disease?

A

Marfan Syndrome

274
Q

What is pyoderma gangrenosum?

A

= a chronic, painful inflammatory disorder of the skin often associated with IBD

275
Q

Mirror movements (involuntary movements mirroring contralateral voluntary hand movement) are seen in what syndrome?

A

Kallmann syndrome

276
Q

Port-wine stains are also called

A

nevus flammeus

277
Q

What is a nevus sebaceous?

A

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
Q

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?

A

Nevus sebaceous

279
Q

A 2 yo comes into the ED with a kerosene ingestion. How to manage this patient:

A

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
Q

Do steroids help in hydrocarbon ingestions?

A

nope. no benefit, don’t give them

281
Q

Name some hydrocarbons

A

turpentine
gasoline
kerosene
furniture polish
lighter fluids

282
Q

Give blank to patients taking isonaized to prevent peripheral neuropathy

A

Pyridoxine (B6)

283
Q

Side effect of isoniazid

A

peripheral neuropathy, due to pyridoxine (B6) depletion

284
Q

RIPE stands for

A

Rifampin
Isoniazid
Pyrazinamide
Ethambutol

285
Q

Blank causes orange discoloration of all secretions and urine

A

Rifampin

286
Q

Tennis elbow is blank epicondylitis

A

Lateral

reproduce the pain with resisted wrist extension

(extension of the wrist happens during backhand motion)

287
Q

Pain and point tenderness upon palpation around the lateral epicondyl is

A

tennis elbow

288
Q

Blank is a noninflammatory condition associated with an increase in vaginal pH (>4.5)

A

BV

(this increase in pH allows for overgrowth of multiple anaerobes like gardnerella vaginalis and decrease in the number of normal lactobacilli)

289
Q

2 types of vaginitis with elevated pH

A

BV
Trichomoniasis

290
Q

Intraerythrocytic and exoerythrocytic organisms on peripheral Giemsa-stained blood smear =

A

Babesiosis

291
Q

Ixodes scapularis, the deer tick that transmits lyme disease, also transmits this disease

A

Babesiosis

292
Q

Where in the US is Babesiosis most common?

A

NE and northern midwest

293
Q

Do immunocompetent patients usually get really sick from Babesiosis?

A

No, the problem comes when the patient is immunocompromised, OR has concurrent lyme disease

294
Q

Is Babesia a parasite or bacteria? How is it transmitted?

A

parasite

tick borne illness

295
Q

A “Maltese Cross” (formed by a tetrad of organisms on blood smear) is pathognomonic for

A

Babesiosis

however often don’t see this

296
Q

How to treat Babesiosis

A

NOT doxy

Treat with atovaquone + azithro

Or if really severe, clinda + quinine

297
Q

Babesiosis symptoms

A

febrile, hemolytic anemia especially in patients without a spleen or those who are otherwise immunocompromised

298
Q

This disease can sometimes be misdiagnosed as malaria

A

Babesiosis

299
Q

Common symptoms of babesiosis in addition to flu like symptoms

A

hemoglobinuria

300
Q

How to diagnose Babesiosis

A

See the parasite Babesia microti on Giemsa or Wright stained blood smear

or there is also a PCR test

301
Q

All types of hereditary angioedema are associated with a low level of

A

C4

302
Q

How does hereditary angioedema present?

A

with recurrent episodes of cutaneous (often facial) and GI edema (abdominal pain, vomiting) – withOUT hives

303
Q

Blank is an AD disorder caused by a defect in C1 inhibitor enzyme

A

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
Q

What is the job of C1 inhibitor enzyme?

A

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
Q

What role does bradykinin play normally in inflammation?

A

It mediates inflammation by causing vasodilation and by increasing vascular permeability

306
Q

Hereditary angioedema patients, when they get swelling, do not have these symptoms

A

hives or itching
aka urticaria or pruritus

(however, they can get a serpiginous rash called erythema marginatum on the trunk and limbs)

307
Q

Blank is the key mediator for attacks of angioedema in mpatients with hereditary angioedema

A

Bradykinin

308
Q

How to diagnose Hereditary angioedema

A

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
Q

How to treat an acute attack in hereditary angioedema

A

Purified C1 inhibitor enzyme

or FFP if that’s not available

310
Q

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….

A

pseudoparalysis from painful osteochondritis caused by congenital syphilis

311
Q

For children ages 1-13, normal BP is defined as

A

both SBP and DMP < 90th percentile

312
Q

Prehypertension is now called

A

“Elevated BP”

stupid naming, but ok sure

313
Q

Age 13 and up, normal BP is

A

<120/80

314
Q

Define “elevated BP,” stage 1 htn, and stage 2 htn for kids age 13 and up:

A

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
Q

Definition for “elevated BP” in a child ages 1-13:

A

SBP and DBP > 90th percentile but < 95th percentile, or 120/80 up to the 95th percentile, whichever is lower

316
Q

Define stage 2 hypertension in children ages 1-13:

A

SBP and/or DBP > 95th percentile + 12 mmHg, or > 140/90

317
Q

blue granules of various sizes dispersed throughout the cytoplasm of red blood cells =

A

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
Q

Is anemia secondary to lead toxicity normocytic or microcytic?

A

can be either

319
Q

Burton line

A

= a blue line along the gums seen in lead poisoning

the teeth will also get a slightly bluish blackish edge

320
Q

Why can lead poisoning cause anemia?

A

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
Q

Free erythrocyte protoporphyrin is elevated in

A

both lead toxicity and iron deficiency anemia

322
Q

Common finding on peripheral blood smear in lead poisoning

A

basophilic stippling (blusih granules of various seizes scattered in the cytoplasm of RBCs)

323
Q

Lab finding in patient of lead poisoning and anemia

A

elevated levels of blood erythrocyte protoporphyrin

324
Q

Shallow painful ulcers in the posterior pharynx, posterior cervical llymphadenopathy, cough and rhinitis think

A

Herpangina

caused by coxsackievirus which is an enterovirus

325
Q

When should a healthy, full term, exclusively breastfed infant begin iron supplementation?

A

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
Q

Do babies taking regular formula fortified with iron (aka like standard formula) need extra iron supplementation?

A

nope

so just the breastfed babies need iron
ok

Pediatric Boards (9 decks)

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