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Senoir 21-2 > LAST PEDS! > Flashcards

LAST PEDS! Flashcards

1
Q

Define Epilepsy

A

recurrent, UNPROVOKED seizures

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2
Q

SZR that arises from one region of the brain cortex

A

Focal/ partial SZR

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3
Q

SZR that arises form both hemispheres of the brain

A

Generalized

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4
Q

4 categories of SZR

A

Focal

Generalized

Unknown onset

Unclassified

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5
Q

A SZR that begins diffusely think

A

Generalized

Tonic clonic, Absence, Febrile

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6
Q

Difference between simple and complex focal SZRs

A

Simple: no altered consciousness
Complex: altered consciousness

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7
Q

MC cause of SZRs in kids

A

Idiopathic is 75% of all cases

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8
Q

Tx for Generlizedd Motor SZRs

A

oxcarbazepine or levetiracetem

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9
Q

How to DDX tics vs SZR

A

Tics are partially under voluntary control

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10
Q

Focal SZRs are preceded by..

A

Aura prodrome

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11
Q

What are automatisms

A

unconscious actions seen in focal complex SZRs

Automatic movements commonly of mouth or extremities

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12
Q

Age of onset for Absence SZRs

A

Around 5-8 yrs old

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13
Q

A 5 yr old haas a brief period of eye flutter and upward eye rolling

Think what SZR D/o? And what is the treatment?

A

Absence SZR

Treat with ethosuximide

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14
Q

Do absence SZRs present with post ictal phases

A

NO!

->immediate resumption of activity

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15
Q

MC cause of SZR from age 6months to 5 yrs

A

Febrile SZR

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16
Q

What is the DDX for simple febrile SZR compared to Complex/Atypical Febrile SZR

A

Simple: Tonic/Clonic lasts less than 15 min and only occurs x1 in 24 hours

Complex/Atypical: Focal S/s lasts longer than 15 min, recurres several times in 24 hours and/or child has preexisting nuero d/o

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17
Q

What should be ruled out in SZR evaluation

A

need to rule out meningitis, encephalitis, brain abscess

If focal neurologic signs or papilledema → CT before LP

MRI is imaging modality of choice for detecting brain lesions in subacute setting (in the ED -> CT)

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18
Q

Febrile SZR MGMT

A

USUALLY NO INTERVENTION NEEDED

High risk pt: Rectal Diazepam to abort SZR >5min

Daily AntiSZR medication NOT needed unless at risk for epilepsy

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19
Q

Do Antipyretic’s prevent Febrile SZRs

A

Antipyretics during febrile illnesses does NOT prevent febrile seizures

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20
Q

SZR for 30 min or more…

A

Status Epilepticus

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21
Q

Status Epilepticus MGMT

A

ABCs

ECG + O2/Pulse Ox

IV access -> Benzo

LABS:
Glucose, BMP, Therapeutic Rx level, Toxicology, CBC+ platelets and Differential

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22
Q

Proper disposition for Status Epilepticus MGMT

A

PICU for monitoring

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23
Q

Head aches in the morning

Think

A

Tumor

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24
Q

Headache Red Flags

A

“Worst headache of my life” → subarachnoid hemorrhage

Morning headache → tumor

Pain awakens child at night

Chronic, progressive headaches (most ominous headache pattern)

Abnormal/focal findings on neuro exam

Changes w/ body position

Recurrent vomiting (especially in morning)

No previous family history

=GET IMAGING!

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25
Q

A younger pt iwth a bilateral, bifrontal headache

Think

A

Migraine

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26
Q

Migraine Headache

A

Common recurrent headache in peds

Focal, bilateral and bifrontal

Seeking dark quiet rooms

N./V, pallor, photophobia, phonophobia

+/- Aura

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27
Q

If a kid presents with headache but no FMHx of headaches

Think

A

A worse secondary cause

Because 90% have a 1st or 2nd degree relative with recurrent headaches

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28
Q

DDX for 2nd Headaches

A 
Head trauma
Viral illness
Sinusitis
Medication overuse headaches
Increased ICP—mass, vascular malformation, pseudotumor cerebri
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29
Q

Headache that is worse when lying down or early in the AM

Think

A

Secondary cause to headache

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30
Q

After a CT w/u for headache what is the next step

A

If CT negative → obtain a lumbar puncture → measure opening pressure & evaluate for RBCs, WBCs, protein, glucose, or xanthochromia

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31
Q

What is the best way to find posterior fossa lesions

A

Brain MRI with/without gadolinium contrast (study of choice)

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32
Q

1st line Tx for Migraines

A

Acetaminophen or NSAIDs (ibuprofen or naproxen)
Adjunct therapies: hydration & antiemetics

2nd line: Triptan agents

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33
Q

What are the contra/I for using triptans for migraines

A

Focal neurologic deficits with migraines

Basilar migraine signs (syncope) → stroke risk

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34
Q

What drug can you not give to peds with migraine with aura

A

NO OCPs!

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35
Q

When to start migraine prophylaxis

A

If more than 1 disabling headache per week

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36
Q

What is pseudo tumor cerebri

A

AKA idiopathic intracranial hypertension
Elevated ICP with normal brain imaging

S/s : Diplopia, abducens palsy, transient visual obscurations, papilledema

MC Cause: idiopathic

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37
Q

Tx for Idiopathic Intracranial hypertension

A

Untreated? permanent visual field loss!

Treatment: acetazolamide (or other diuretics), topiramate, or corticosteroids

(Also wt loss and remove any triggering medications)

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38
Q

Retrograde vs Anterograde amnesia

A

Retrograde amnesia: inability to recall events leading up to trauma

Anterograde amnesia: inability to form new memories after trauma

Variable duration: length of amnesia typically correlates w/ severity of trauma

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39
Q

Highest timeline risk for concurrent concussions

A

Increased in the 1st 10 days after concussion occurs

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40
Q

Prognosis of SZR with concussions

A

At time of injury: due to cortical stimulation → excellent prognosis

Within 7 days: typically due to localized edema or contusion → good prognosis

After 7 days: attributed to glial scarring → leads to epilepsy

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41
Q

Role of CT in concussion MGMT

A

CT not used to diagnose concussion, but rather to rule-out serious traumatic brain injury (i.e., hemorrhage)

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42
Q

What is second impact syndrome

A

Acute, sometimes fatal brain swelling occurring when a second concussion is sustained before complete recovery from a previous concussion

May cause rapid increase in ICP which is impossible to control

3 deaths every 2 years

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43
Q

Return to play guidelines for concussion

A
  1. No activity
  2. Light aerobic
  3. Sports specific excercise
  4. Non contact sports play
  5. Full contact practice
  6. Game play

Each stage should be a minimum of 24 hours without s/s to move to the next stage

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44
Q

Most at risk for Post concussive syndrome

A

Risk factors: younger age, level of play, ongoing clinical symptom, chronic neurobehavioral impairments, catastrophic outcomes (i.e., subdural hematoma)

S/s: Hallucinations, Dizziness, HA, Unclear thinking, sleep d/o

Can last weeks and effect school performance

Refer to NEURO!

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45
Q

Stage IV sleep….

A

REM (rapid eye movement)
Active, awake-like EEG pattern & muscle atonia
Active sleep: REM sleep in neonates

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46
Q

Who sleeps longer, bottle fed or breastfeed babies

A

Bottle-fed babies generally sleep for longer periods (2-5 hr bouts) than breastfed babies (1-3 hr).Sleep periods are separated by 1-2 hr awake.

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47
Q

Should peds less than 1 years old

Share the bed

A

American Academy of Pediatrics issued a revised recommendation in 2016 advocating against bed-sharing in the 1st yr of life, instead encouraging proximate but separate sleeping surfaces for mother and infant for at least the 1st 6 mo and preferably 1st yr of life.

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48
Q

Best sleeping position for babies

A

On back, firm mattress, do not use pillows or comforters

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49
Q

NML sleep patterns for infants 2-12 months old

A

great individual variability

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50
Q

At what age does the capacity to self soothe start?

A

capacity to self-soothe begins to develop in the 1st 12 wk of life and is a reflection of both neurodevelopmental maturation and learning.

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51
Q

NML nap time for toddlers

A

Naps decrease from 2 to 1 nap at average age of 18 mo.

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52
Q

When do nighttime fears start to develop

A

1-2 years old

Have a good night routine to abate this

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53
Q

Should preschoolers cosleep

A

Persistent cosleeping tends to be highly associated with sleep problems in this age-group.

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54
Q

Sleep hygiene for 6-12 years old

A

School and behavior problems may be related to sleep problems.

Avoid media and electronics at bed time

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55
Q

What is sleep onset associated insomina

A

child awakens under conditions different from those experienced as they fell asleep and cannot return to sleep independently

Leads to sleep d/o

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56
Q

What is limit-setting subtype of insomina

A

Bedtime resistance or refusal due to a caregiver’s unwillingness or inability to enforce bedtime rules & expectations

Nighttime fears → also common cause of bedtime refusal

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57
Q

When do night terrors occur

When do nightmares occurs

A

Terrors: 1st 1/3 of sleep

Nightmares: last 1/3 of sleep

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58
Q

Circadian rhythm disorders in adolescence

A

Exaggerated delayed sleep phase
Leads to inability to arouse in the mornings & failure to meet sleep requirements

Attempt to recoup lost sleep on weekends

Leads to decreased cognition and emotional regulation

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59
Q

What is the most common cause of flaccid paralysis in peds

A

Guillian-Barre

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60
Q

Peds presents with Areflexia, flaccidity, & symmetrical ascending weakness

Think

A

Guillian Barre syndrome

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61
Q

Tx for Guillian Barre

A

Early stages → admit to hospital for observation & respiratory support if needed

Treatment: IVIG; plasma exchange & immunosuppressive drugs if IVIG fails or rapidly progressive disease

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62
Q

A pt presents with constipation and poor suck reflex that progresses to hypotonia and weakness with a decreased gag reflex

Think

A

Botulism 2/2 Canned foods or Honey

Tx: IVIG and RR support

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63
Q

X linked muscular dystrophy MC in boys that presents with Toe walking, wide broad lordonic stance

Think

A

Dúchennes

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64
Q

What is Gower sign

A

Clumsiness and easy fatigability, then weakness of muscles

Seen in dúchennes

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65
Q

Prognosis for Duchenne Muscual dystrophy

A

Arm weakness by age 6

Wheelchair bound by age 12

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66
Q

What enzyme is elevated in muscular dystrophies

A

CK

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67
Q

Cafe au lait spots are assoc with

A

Neurofibromas type 1

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68
Q

Dx criteria for Neurofibromatosis type 1

A

Need 2 or more of following:

-Café au lait spots (≥6)
- >5 mm (prepubescent child)
-Axillary or inguinal region freckling
-2+ Lisch nodules (iris hamartoma)
-2+ neurofibromas
-Optic gliomas
-Relative (1st degree) with NF by criteria
-Osseous lesions
(sphenoid dysplasia or long bone abnormalities)

Need cranial imaging to r/o neoplasms

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69
Q

Complications of Neurofibromatosis type 1

A

Learning disabilities, scoliosis, seizures, cerebrovascular abnormalities

Other tumors possible: optic nerve gliomas, brain & spinal cord astrocytomas, malignant peripheral nerve tumors, sarcomas

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70
Q

What is the finding that really tells you that its Neurofibromatosis type II

A

NF2 gene codes for merlin

Cataracts common

NO axillary freckling, Occasionally Café-au-lait

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71
Q

Mutation of chromosome 9
-TSC1 gene makes hamartin
Mutation of chromosome 21
-TSC2 gene makes tuberin

Think

A

Tuberous Sclerosis

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72
Q

Hamartomas =

A

Tuberous sclerosis

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73
Q

An pt presents with facial angiofibromas (adenoma sebaceum), intellectual delay, epilepsy

Think

A

Tuberous Sclerosis

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74
Q

3 types of skin manifestations of Tuberous Sclerosis

A

Adenoma sebaceum
(facial angiofibromas): small, red nodules over nose & cheeks confused w/ acne

Ash leaf spots: hypomelanotic areas of skin

Shagreen patches: elevated, rough plaques of lumbosacral/gluteal areas

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75
Q

What is the most common cause of adult death 2/2 tuberous sclerosis

A

Renal manifestations:

->Renal angiomyolipomas: can have malignant transformation
most common cause of death in adults)

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76
Q

Sturgeon Weber syndrome

A

Sporadic (not inherited)

Abnormal leptomeningeal blood vessels (angiomas) overlying cerebral cortex associated with:
->Ipsilateral facial port-wine stain (nevus flammeus) involving ophthalmic division of trigeminal nerve (forehead & upper eyelid)

+Glaucoma

+SZRs (MC presentation)

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77
Q

What is the tx for unilateral disease w/ difficult to control seizures

A

hemispherectomy

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78
Q

What is the foramen ovale

A

Opening between right & left atrium allowing for a right to left shunt
Allows oxygenated blood to bypass fetal lungs

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79
Q

What is the ductus arteriosus

A

Opening between the pulmonary artery & aorta allowing for a right to left shunt

Prostaglandin E occasionally used to maintain patency when a cyanotic lesion also present

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80
Q

What is the ductus venosus

A

Opening between the umbilical vein & inferior vena cava allowing oxygenated blood from the placenta to bypass the liver & flow to the heart

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81
Q

A baby that sweats during feeding

Think

A

Congenital Heart Defects

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82
Q

Fixed s2 split=

A

Atrial Septal Defect

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83
Q

Is an S4 sound ever NML

A

S4 = Poor diastolic function
Never normal!
Decreased ventricular compliance

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84
Q

Clicks in the heart =

A

valvular abnormality or dilated great vessel

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85
Q

What is the most common symptomatic arrhythmia

A

SVT is most common symptomatic arrhythmia

Responds to vagal maneuvers or adenosine

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86
Q

1st test to order in the W/u for murmurs or HDz

A

CXR r/o pulm involvement

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87
Q

What findings in chest pain lead to a cardio referal

A

Hx of chest pain w/ syncope, exertion, palpitations or acute onset w/ fever → cardiac etiology

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88
Q

DDX for kids with complete hr block

A

Congenital Complete Heart Block
->Associated w/ maternal collagen vascular disease (i.e., systemic lupus erythematous or Sjogren syndrome) or congenital heart disease

Acquired complete heart block:
_>most often occurs secondary to cardiac surgery

Other causes: infection, inflammation, or drugs

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89
Q

MC congenital heart defect

A

VSD

<3 mm = asymptomatic
3-5 mm = moderate symptoms
>5 mm = CHF & FTT

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90
Q

Tx options for VSD

A

~One third close spontaneously

Initial treatment
(moderate-large VSDs):
diuretics (± digoxin)
& afterload reduction

Poor growth, failure to thrive (FTT) or pulmonary HTN despite treatment → closure required (usually surgical)

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91
Q

When do you do Tx correction for ASD

A

If significant shunt still present around age 3 → closure recommended:
Many closed via catheter closure devices
Otherwise, surgical closure

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92
Q

A pt that presents with “Bounding” pulses w/ widened pulse pressure
Continuous machine-like murmur at LUSB

Think

A

PDA

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93
Q

Tx for PDA

A

Indomethacin IV
->Most effective in premature infants
May cause transient renal insufficiency

Cardiac catheter closure:
Coil embolization or PDA closure device

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94
Q

Click that is at the LUSB that radiates to the back

Think

A

Pulm Stenosis

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95
Q

Click that happens at the RUSB and radiates to the neck think

A

AS

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96
Q

Tx for stenotic valves

A

Balloon valvuloplasty (more effective in pulmonary than aortic stenosis)

Surgery:
If ballon valvuloplasty fails OR subvalvular pulmonic stenosis

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97
Q

Pt presents with Femoral pulses weaker & more delayed than R radial pulse
BP in legs < BP in arms

Think

A

Coart

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98
Q

Grade I-II systolic murmur at LUSB w/ radiation to L upper back, next to scapula

Think

A

Coart

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99
Q

Rib notching in older peds think

A

Coart

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100
Q

Components of tetralogy

A

Overriding aorta (into the RV)
Pulmonary stenosis
VSD
RVH

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101
Q

Tx for Tetralogy

A

PGE is indicated if infants are cyanotic at birth

Surgical repair
(Tet spells are an indication to proceed)

SBE Prophylaxis 
(until 6 mos s/p surgery or indefinitely due to residual VSD)
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102
Q

Boot shaped heart

Think

A

Tetralogy

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103
Q

Egg on a string on CXR =

A

Transpo

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104
Q

Any cyanotic baby should get..

A

PGE 1 to maintain a PDA

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105
Q

Define Ebsteins anomaly

A

Tricuspid valve leaflet(s) malformed & partly attached to RV endocardium & fibrous tricuspid valve annulus

Leads to an enlarged RA and Abn Tricuspid valve / regurg

RA enlargement → ↑ RA pressures causing R → L shunt through foramen ovale (leading to PFO) → cyanosis (deoxygenated blood by-passing lungs)

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106
Q

Globular cardiomegaly, decreased pulmonary vascular markings, box-shaped heart

Think

A

Ebsteins anomaly

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107
Q

Rhematic HDz effects what valves

A

Mitral, aortic, or pulmonic insufficiency or stenosis

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108
Q

Lab test for rheumatic fever

A

antistreptolysin O titer (streptococcal antibody test)

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109
Q

Major Cx for Rhematic HDz

A 
Polyarthritis 
Carditits 
Chorea (syndham chorea) 
Erythema Marginatum 
Sub Q nodules
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110
Q

MGMT for Rheumatic HDz

A

Consult Cardio

-> PCN G/ Amoxicillin
(Allergic; Erythro)

  • > ASA/ NSAIDs
  • > Steroids (severe)

Long term: PCN prophylaxis q 28 days
(If VHDz then for life)

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111
Q

MC Cause of Pericarditis

A

VIRAL VIRAL VIRAL

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112
Q

MC Causes of infectious endocarditis

A

Principal cause in children w/ congenital defect w/out prior surgery: streptococcus Viridans

Important causes in children s/p cardiac surgery & children w/ prosthetic cardiac & endovascular materials:
Staphylococcus aureus & coagulase-negative staphylococci

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113
Q

Risk fxs for infective endocarditis

A

Prosethic valves

Dental procedures

REcent surgeries

Or Neonate central lines

114
Q

Fever, malaise, & weight loss
+ Tachycardia & new/changed heart murmur

Think

A

Infective endocarditis

115
Q

Roths spots
Splinter hemorrhages
Oslers nodes
And Janeway lesions

Think

A

Infective endocarditis

116
Q

Tx for endocarditis

A

Get blood Cx

Infective carditis by viridans streptococci → monotherapy penicillin G for 4 wks

If medical treatment unsuccessful → surgery

117
Q

Prophylaxis Rx for prevention of Endocarditis

A

Recommended regimen: Oral amoxicillin 50mg/kg (maximum dose: 2g) 30-60 mins before procedure

Alternative regimen: β-lactam allergy → clindamycin or azithromycin

118
Q

Primary vs Secondary HTN

A

Primary (Essential) HTN:
Most common cause of HTN in adolescents
More likely in obese children

Secondary HTN:
Younger age
More severely elevated BP
Renal disease (most common cause in children)

119
Q

Mc cause of 2nd HTN in peds

A

Renal disease (most common cause in children)

120
Q

MGMT for Ped HTN

A

2 BP averaged together

Pre-HTN (90-95)—repeat BP in 6 months and education

Stage 1 HTN (95-99)—repeat BP in 1-2 weeks (average BP over all 3 visits) + education

Stage 2 HTN (>99)—evaluate and/or + refer for treatment within 1 week + education

121
Q

When to evaluate for 2ndary causes of HTN

A

Especially if BMI <85th percentile

BP >140/100

122
Q

1st line tx for peds HTN

A

Most common 1st line:
Calcium channel blockers
Angiotensin-converting enzyme inhibitors (ACEI)

Other 1st line:
Angiotensin receptor blockers (ARB)
β-blockers
Diuretics

123
Q

When do we screen for Hgb/Hct

A

At 12 months

124
Q

DDX MCV <80

A

Iron deficiency, Thalassemias, Lead

125
Q

Anemia DDx for MCV 80-100

A

Associated w/systemic illness that impairs adequate marrow synthesis of RBCs

126
Q

Anemia DDX with MCV above 100

A

Vitamin B12 & folic acid deficiencies

Ex: Hypothyroid, Trisomy 21, Newborns

127
Q

What dictates transfusion threshold for anemia

A

PRBC transfusion dictated by cardiovascular & functional impairment more than hemoglobin level

128
Q

Most common S/s for Anemias

A

Hemolysis:
Pallor, jaundice (most common signs) & splenomegaly

Coagulopathy or platelet abnormalities:
Petechiae, purpura, & deeper bleeding (i.e., generalized hemorrhage)

129
Q

A peds pt with dementia, ataxia, or neuropathy

What type of anemia?

A

Vit b12 def.

130
Q

DDX for elevated RDW

A

Suspect increased RBC destruction

  • > G6PD deficiency
  • > Sickle cell
  • > Spherocytosis
131
Q

Anemia with a NML RDW

Think

A

RDW normal? Suspect decreased RBC production

  • > Transient erythroblastopenia of ->childhood
  • > Anemia of chronic disease
  • > Bone marrow failure (pancytopenia)
132
Q

High Retic # + bleeding

Look at

A

GI/GU cause

133
Q

High Retic # + hemolysis

Think

A

Immune vs non immune cause of anemia

134
Q

DDX for Microcytic anemia

A

FLATS

(Fe) Iron 
Lead 
Anemia of Chronic Dz 
Thalassemia 
Sideroblastic Anemia
135
Q

MC nutritional deisorder in the world

A

IRON def.

2/2:

Cow’s milk:  9-24mo
 (inadequate iron intake)
Blood loss
Menses
Intestinal
Epistaxis
Undernutrition (Worldwide)
136
Q

CBC microcytic anemia
High RDW
Reduced RBC

Think

A

Iron Def,.

137
Q

How long do we treat for Iron Def ANEMIA

A

3 months ! To replenish iron stores

138
Q

DDX for Macrocytic anemia

A 
Common DDX (“FLAHB”)
Folate deficiency/Fanconi
Liver disease
Alcohol/Aplastic anemia
Hypothyroidism
B12 deficiency
139
Q

MC Cause Aplastic anemia

A

Often idiopathic

Dx with Bone Bx

Tx with Stem cells

140
Q

↑ Bilirubin
↑ Free (aka serum) hemoglobin
↓ Haptoglobin
Schistocytes

Think

A

Hemolysis

141
Q

1 alpha gene mutation =

A

1 gene mutation: “Silent” carrier thalassemia
Completely asymptomatic → normal CBC
Only detectable through genetic studies

142
Q

2 alpha chain defects=

A

2 gene mutations: α-Thalassemia trait

Mild microcytic anemia
Detectable through mildly decreased Hgb & Hct

143
Q

3 gene thalassemia defects

A

3 gene mutations: “Hgb H disease”

Microcytic anemia & mild hemolysis
Not transfusion-dependent

144
Q

4 gene thalassemia defects

A

4 gene mutations:
Bart Hgb/Hydrops fetalis (γ4)

Severe anemia, intrauterine anasarca from congestive heart failure, death in utero or at birth

145
Q

1 beta mutation =

A

1 gene mutation:
Thalassemia minor (heterozygous)
Mild microcytic anemia

146
Q

2 beta gene mutations =

A

2 gene mutations:
Thalassemia intermedia
(compound heterozygous)

Moderate hemolysis, splenomegaly, moderately severe anemia
(not transfusion dependent)

High Hgb A2 (α2δ2) & Hgb F (α2γ2)

147
Q

2 beta gene deletion=

A

2 gene deletion: Homozygous

Severe hemolysis, ineffective erythropoiesis, transfusion dependent, hepatosplenomegaly

Frequent anemic crises requiring regular transfusions by 2 mos

High Hgb F (α2γ2)
→ due to lack of nml Hgb A (α2β2)

Iron overload (hemochromatosis) common

148
Q

Fever in a kid with sickle cell =

A

Medical emergency!

149
Q

Onset of spleen disfunction in peds with Sickle Cell

A

As early as 6mo abnormal immune function due to splenic dysfunction

By 5yr most with functional asplenia

150
Q

When would be the most likely agent in a sickle cell pt with osteomyelitis

A

Salmonella or Staph aureus

151
Q

If a pt presents in an Aplastic crisis

Think

A

Parvovirus B19

152
Q

What is often the first manifestation of plenipotentiary failure in kids

A

Dactylitis-> often first manifestation of pain infants/children

Symmetic or unilateral swelling of hands and/or feet

153
Q

A pt presents with Symmetic or unilateral swelling of hands and/or feet

A

Often the first S/s of Sickle Cell in peds

154
Q

Stroke in a pediatric pt

Think

A

Sickle Cell Sequestration

155
Q

What is the most common vascular event of sickle cell

A

Pain crisis
-Last 2-7 days
-Pain usually localizes to arm/leg long bones
(Femur → possible femoral head avascular necrosis)

Treatment: fluids, analgesics
(narcotics & NSAIDs), O2

156
Q

What is Acute Chest Syndrome in Sickle Cell Pts

A

Vasoocclusive crisis w/in lungs

On CXR= new infiltrate

Associated w/ infection & infarction

1st chest pain → w/in few hrs, cough, increasing RR & HR, hypoxia, & progressive respiratory distress

PE: decreased breath sounds & dullness to chest percussion

Treatment: O2, fluids, analgesics, antibiotics, bronchodilators, incentive spirometry, & RBC transfusion

157
Q

Sickle cell pts with a new infiltrate on CXR

Think

A

Acute Chest Syndrome

S/s 1st chest pain → w/in few hrs, cough, increasing RR & HR, hypoxia, & progressive respiratory distress

PE: decreased breath sounds & dullness to chest percussion

158
Q

Tx for Acute Chest Syndrome

A 
O2
 fluids
analgesics
antibiotics
bronchodilator
 incentive spirometry
& RBC transfusion
159
Q

MGMT for sickle Cell

A

Hydroxyurea (start at 9mo old)

-Increases production of Hgb F (α2γ2)
(Which normally decreases at 9mo)
-Decreases number & severity of vasoocculusive events

Or

Hematopoietic stem cell transplantation
-If HLA matched sibling donor used → curative

160
Q

Sickle Cell Preventative MGMT approach

A

Daily prophylactic oral penicillin begun at diagnosis

Vaccinations (against pneumococcus, H. influenzae type b, hepatitis B virus, & influenza virus)

Folate supplementation

Heme/Onc referral

161
Q

Heinz bodies=

A

G6PD deficiency

162
Q

What are the oxidizing agents that G6PD pts should avoid

A 
Fava beans 
sulfa drugs
ASA
primaquine
nitrofurantoin

Exposure to mothballs (naphthalene)

Also Serious infection can precipitate hemolysis

163
Q

Define Thrombocytopenia

A

Thrombocytopenia: platelet counts <150,000/mm3

Platelet counts >80,000 /mm3
→ no bleeding risk unless surgery or major trauma

Platelet counts <20,000/mm3
→ risk for spontaneous bleeding

164
Q

Difference between DVT and Arterial Clots in pts with hemostatic Dz

A

Deep venous thrombi
-Warm, swollen (distended), tender, PURPLISH discolored extremities or organs

Arterial clots

  • Acute, painful, PALE, & poorly perfused extremities
  • Internal organ involvement: signs & symptoms of infarction
165
Q

MC childhood bleeding D/o

A

ITP

Young children → 1-4 wks after viral infection w/ abrupt onset of petechiae, purpura, & epistaxis

166
Q

ITP tx for pts with #>30,000

A

monitor; unlikely to treat
~80% resolve w/in 6 mos

Treatment does not affect long-term outcome but does increase platelets in short-term

167
Q

ITP tx for pts with #<10,000

A

-IVIG (intravenous immunoglobulin)
1 g/kg/d x1-2 ds
-Prednisone 2-4 mg/kg/d x2 wks

Splenectomy only for life-threatening bleeding

Chronic ITP: lasts ≥6 months

  • Rule out secondary causes (SLE, HIV)
  • Splenectomy for definitive treatment (70-80% remission)
168
Q

Fx VIII deficiency

A

Hemophilia A

169
Q

Fx IX deficiency

A

Hemophilia B

170
Q

A crawling peds pt presents with Bleeding with minor trauma, spontaneous bleeding, hemarthrosis (bleeding into joints/muscle)

Think

A

hemophilia A or B

171
Q

W/u for Hemophilia

A

PTT prolonged

PT and Bleeding Time usually normal

PTT corrects to normal when sample mixed with normal serum

172
Q

Does Desmopressin help Hemophilia A or B ?

A

Mild or moderate hemophilia A only (no effect on factor IX)

Increases factor VIII & vWF production

173
Q

What is the most common CONGENITAL bleeding d/o

A

VWF dz

174
Q

Function of vWF

A

Bridge between platelets & subendothelial collagen

Binds & protects factor VIII from rapid clearance from circulation

175
Q

Type 1, 2 , and 3 vWF Dz

A

Type 1: Decreased production of vWF
Autosomal dominant & most common

Type 2: Normal production but defective vWF (dysproteinemia)

Type 3: No production
Rare

176
Q

Lab for vWF Dz

A

vWF quantity is measured and then function is measured with ristocetin*

177
Q

Tx for vWF Dz

A

Desmopressin (DDAVP) effective for type 1 & 2

vWF concentrate available for type 3

178
Q

Define Henoch Schonlein Purpura (HSP)

A

Vasculitis of unknown etiology
Most common childhood systemic vasculitis

Ages 3-15 yrs (most before age 6)
More frequent in winter

50% of cases follow URI’s
More frequent during winter months

Characterized by inflammation of SMALL blood vessels w/:

  • Leukocytic infiltration of tissue
  • Hemorrhage
  • Ischemia
  • IgA immune complex deposition →
179
Q

Child presents with palpable purpura, arthralgia, arthritis, and abdominal pain

NO FEVER

Think

A

HSP

180
Q

Dx criteria for HSP

A

Palpable purpura
Bowel Angina
Dx Bx -> IgA in the vessel wall
And being <20

181
Q

What is the post complication of HSP

A

Rash resolves in a year

Increased risk of GI involvement → temporary abnormal peristalsis → intussusception risk!!

May be followed by complete obstruction or infarction w/ bowel perforation

Evaluate for intussusception if recent HSP history w/ acute abdominal pain, obstipation, or diarrhea

182
Q

Tx for HSP presentation

A 
Supportive
->Follow renal function
NSAIDS for arthritis
Steroids for GI  disease & nephritis → controversial
If severe, hospitalize
183
Q

Vasculitis of unknown etiology
Small to medium-sized arterial inflammation w/ aneurysm formation

Think

A

Kawasakis

184
Q

Greatest risk complication of Kawasaki’s

A

Hematoma around the heart

/ Coronary artery aneurysm

185
Q

Child with a fever for 5 days!!

Think

A

Kawasakis

186
Q

High fever x 5 days + strawberry tongue/ mucosal changes and cervical lymphadenopathy

Think

A

Kawasakis

187
Q

Kawaksi treatment should be followed until…

A

until ESR normalizes

188
Q

Pediatric presents with morbilliform rash, conjunctivitis and red chapped lips

Think

A

Kawasakis

189
Q

Cx to Dx Kawaskis

A 
Fever x 5 days 
Bilateral conjunctivitis 
Mucus involvement 
Desquamation 
Polymorphous rash on the trunk 
Cervical Lymphadenopathy
190
Q

Tx approach to Kawasakis

A

echocardiogram at diagnosis, 2-3 wks in & again at 6-8 wks

Treatment
IVIG (mainstay of therapy)

Goal: coronary aneurysm prevention

+Aspirin
-> High dose first then reduce dose
Risk of Reye syndrome low & acceptable because other NSAIDs are not as effective

191
Q

Define Tumor Lysis Syndrome

A

Seen in Cancer emergencies (2/2 chemo)

Rapid cellular lysis leads to potassium, phosphate, & uric acid released into circulation

Manifests as arrhythmias, cardiac arrest, gout, uric acid nephropathy

Common in: leukemia & lymphoma treatment

192
Q

Usual infections seen in cancer pts

A

Central catheter or port infections

Atypical pneumonia
(Pneumocystis jiroveci)

Aspergillosis

Human herpes virus infections
(severe)

Cryptococcal meningitis

193
Q

Fever in a cancer pt =

A

Emergency!!! ADMIT

Treat early & aggressively w/ broad spectrum antibiotics

194
Q

Immature blast cells

Think

A

Leukemia

195
Q

W/u for myeloproliferative d/o with pts with trisomy 21

A

Transient myeloproliferative disorder (unique to patients with Trisomy 21)

CBC: Elevated WBC w/ peripheral blasts, anemia, thrombocytopenia

Bone marrow aspirate: very minor marrow infiltration (key to differentiating from leukemia)

196
Q

Suggestive agent of Lymphoma

A

Unknown etiology, but evidence suggests Epstein-Barr virus (EBV) may play a causal role

197
Q

Bimodal distribution of Hodgkin’s

A

Peaks 15-35yo and again age >50

198
Q

Non Hodgkin lymphoma

A

Males > females

Almost always diffuse, highly malignant, & little differentiation

199
Q

2 types of B cell Non Hodgkin’s

A 
B cell 
(Burkitt lymphoma/small noncleaved cell)
Two forms:  
-sporadic form (North America)
-endemic form (Africa; strong EBV association)
200
Q

A child presents with Painless, nontender, firm,rubbery cervical & supraclavicular adenopathy

Think

A

Hodgkin’s

201
Q

Reed Sternberg Cells =

A

Hodgkin’s

202
Q

MC type of Brian tumor

A 
Astrocytomas:
Most common
Usually found in posterior fossa
Often low grade
Usually good prognosis
203
Q

Should you do LP prior to CT/ MRI?

A

Never perform an LP prior to neuroimaging if tumor is a concern—herniation risk

204
Q

What is the Most common childhood solid neoplasm outside CNS

A

Neuroblastoma

At age 22 months

Derived from neural crest cells

205
Q

What is paraneoplastic syndrome in pts with neuroblastoma

A

Profuse sweating
Secretory diarrhea
Opsoclonus/myoclonus (dancing eyes & dancing feet)

206
Q

90% of pts with neuroblastomas will have what lab finding

A

90% will have positive urine catecholamines

(vanillylmandelic acid; homovanillic acid): eval with urine catecholamines test

207
Q

MC primary malignant renal tumor

A

Nephroblastoma= Wilms Tumor

208
Q

What is WAGR

A

WAGR (Wilms tumor, Aniridia, Genitourinary malformation, Range of developmental delays) syndrome → germline deletion at chromosome 11p

209
Q

What is Beckwith Wiedemann Syndrome

A

Macroglossia,
Umbilical hernia
And omphalocele

With Nephroblastoma increased risk

210
Q

Abdominal mass + painless hematuria

Think

A

Nephroblastoma

211
Q

Most common soft tissue sarcoma

A

Rhabdomyosarcoma

212
Q

Most common primary malignant bone tumor in chlidren

A

Osteosarcoma

213
Q

Most common bone sarcoma in children less than 10

A

Ewing sarcoma

214
Q

What pts are at an increased risk for soft tissue sarcomas

A

Pts with Li-fraumeni and Neurofibromatosis

215
Q

Pts with Heriditary retinoblastoma are at an increased risk of what cancer

A

increased osteosarcoma risk

216
Q

Small round blue cell tumors on microscopy

Think

A

Rhabdomyosarcoma

MC;

GU (24%)
Head & neck (25%)
Extremities (19%)
Orbit (9%)

ALSO Ewing Sarcoma

217
Q

A peds with pain and mass at the epiphysis with osteoid substance in the joint

Think

A

Osteosarcoma

218
Q

Starburst pattern on XR

Think

A

Osteosarcoma

219
Q

Onio skin/ Moth eaten appearance on XR

Think

A

Ewing Sarcoma

220
Q

How can you DDx Ewing on Anaylsis

A

Immunohistochemical & cytogenic analysis differentiates rhabdo & Ewing
Ewing: t(11;22)

221
Q

Tx option for Rhabdomyosarcoma

A

Surgical resection w/ POST OP chemo & radiation depending on stage & site

222
Q

Tx for osteosarcoma

A

Neoadjuvant chemotherapy followed by surgery & adjuvant chemotherapy

223
Q

Tx for Ewing sarcoma

A

Ewing radiation sensitive

224
Q

4 distinct phases of DM type I

A
  1. Preclinical B-cell autoimmunity with progressive defect of insulin secretion
  2. Onset clinical diabetes
  3. Transient remission honeymoon period
  4. Established diabetes during which there may occur acute and/or chronic complications
225
Q

Tx for T1DM vs T2DM

A

T1: life long insulin

T2: metformin and life style

226
Q

How do we eval the honeymoon period for DM

A

Measure residual function with c-peptide

227
Q

Glucose > 300, pH <7.3,
bicarbonate <15

Think

A

DKA

228
Q

What intervention should you NOT do in pts with DKA

A

DO NOT DO NOT DO NOT Bolus them!!

229
Q

Rapid decreases in serum glucose levels >100 mg/dL/hr

Leads to

A

Cerebral edema

230
Q

T1 DM Goals in peds

A

A1C
:Age <6: HgbA1c = 7.5-8.5%
Age 6-13: HgbA1c = <8%
Age 13-18: HgbA1c = <7.5%

Blood Glucose:
Children <5 yrs: 80-200 mg/dL
School-age children (5-15): 80-150 mg/dL
Adolescents (age 16+): 70-120 mg/dL

231
Q

Definitve Dx for Hashimotos

A

TPO abs

232
Q

An infant that presents with poor feeding, vomiting, lethargy, and convulsions with no response to glucose WITHOUT A FEVER

Think

A

Inborn error of metabolism

PKU

233
Q

Define PKU

A

Defect in hydroxylation of phenylalanine to form tyrosine
Primarily affects the brain

Autosomal Recessive

Appears completely NML that then develops into profound developmental delays

234
Q

How to check for PKU

A

Positive Newborn Screen ->check quantitative plasma amino acid analysis (positive: phenylalanine >360µM or 6mg/dL)

235
Q

MGMT for PKU

A

Low Phenylalanine diet to maintain plasma phenylalanine levels between 120-360 mMol/L throughout life (frequent labs)

236
Q

Define Galactosemia

A

Autosomal Recessive
Galactose-1-phosphate uridyltransferase deficiency

When a neonate is fed milk ->
-Liver failure 
(hyperbilirubinemia, disorders of coagulation, hypoglycemia)
-Renal tubular dysfunction
 (acidosis, glycosuria, aminoaciduria)
-Cataracts

Need rapid neonatal screening test turnaround time ->affected infants may die in 1st week of life

237
Q

MGMT for galatosemia

A

Tx: eliminate dietary galactose!

Learning disorders may persist despite dietary compliance

Premature ovarian failure usually develop despite treatment

238
Q

What infections are pts with galactosemia at risk for

A

Increased risk for severe neonatal Escherichia coli

239
Q

S/s of Addisons

A 
Hyperpigmentation (tan color to skin)
Salt craving
Postural hypotension
Fasting hypoglycemia
Anorexia
Weakness
Shock can occur during severe illness
240
Q

Lab findings for Addisons

A

Cortisol
-Subnormal at baseline and also low with ACTH-stimulation

Other labs: BMP—hyponatremia, hyperkalemia, elevated plasma renin (indicate mineralocorticoid deficiency)

241
Q

MGMT for Addisons

A

Hydrocortisone (10-15 mg/m2/24 hours)

  • Baseline daily dosing
  • Stress dosing = three times daily dosing or IM hydrocortisone
  • Dose titrated to allow a normal growth rate

Mineralocorticoid deficiency treated with fludrocortisone
-Monitor with plasma renin, Na+, K+ levels

242
Q

A male pt that presents with normal genitalia
Salt loser: present within 2 weeks in adrenal crisis
Non salt loser: present at 6mo with androgen side effects on exam but NO testicular enlargement

Think

A

CAH

Refer to Endo

243
Q

Cushing Syndrome

A 
Progressive central or generalized obesity
Marked failure of longitudinal growth
Hirsutism
Weakness
Nuchal fat pad
Acne
Striae
Hypertension
Hyperpigmentation (if ACTH elevated)
244
Q

Neonatal Surgical Rep Flags

A

Maternal Polyhydramnios
-Various causes—surgical concern if inability of fetus to swallow/digest amniotic fluid causing “back-up” of fluid

Delayed meconium passage

Abdominal distention
-Obstruction?

Perinatal infant vomiting

  • Bilious or non-bilious?
  • Can indicate location of pathology
245
Q

NML development of the tracheal and esophageal tissue

A

Tracheal and esophageal tissue develop in close proximity at 4-6 weeks gestation

A fistula represents dysgenesis (abnormal development) of this tissue

246
Q

Peds with a single umbilical artery are at an increased risk of…

A

Tracheo-esophageal fistula

247
Q

What does VACTERL mean

A

VACTERL association:

  • Vertebral anomalies (70%)
  • Anal atresia (imperforate anus) (50%)
  • Cardiac anomalies (30%)
  • TEF (transesophageal fistula) (70%)
  • Renal anomalies (50%)
  • Limb anomalies (polydactyly, forearm defects, absent thumbs, syndactyly) (70%)
248
Q

Dx for Fistulas of the esophagus

A

Gastrografin swallow

Methylene blue challenge

249
Q

What is the number 1 cause of GI obstruction before age 3 months old

A

Pyloric Stenosis

250
Q

2-6 week-old

Post-prandial, progressive nonbilious* “PROJECTILE” vomiting

Initially ravenously hungry, then lethargic as malnutrition/dehydration progresses

Think

A

Pyloric stenosis

251
Q

Olive shaped mass in the abdomen

Think

A

Pyloric stenosis

252
Q

Pyloric stenosis on a Lab

A

Hypochloremic, hypokalemic metabolic alkalosis

253
Q

String Sign on upper barium study

A

Pyloric stenosis

254
Q

Prognosis of umbilical hernias

A

Typically 1-5 cm in size

> 2 cm = less likely to close on their own

255
Q

SRGRY criteria for Umbilical hernias

A

Hernia persists past 5 years of age
Painful
Becomes strangulated
Increasing in size after age of 1-2 yrs

256
Q

Pt presents with BILIOUS vomiting

Think

A

Intestinal Malrotation & Volvulus

257
Q

Corkscrew effect on upper GI studies thing

A

Intestinal Malrotation & Volvulus

258
Q

MC congenital d/o assoc with Intestinal atresia

A 
Trisomy 21 (30%)
Malrotation (25%)
Annular pancreas (20%)
Meconium ileus w/ cystic fibrosis
Check for CF if infant has atresia
259
Q

DOuble bubble sign on CXR

Think

A

intestinal atresia

260
Q

Define Gastrochisis

A

Definition “split or open stomach”
-Misnomer as actually abdominal wall that is split

Linear abdominal wall defect

  • Lateral to the median plane of the anterior abdominal wall
  • More often on right side

Does not involve umbilicus

261
Q

MGMT for Gastrochisis

A

Surgical correction requires → return of normal bowel function often slow

Prolonged parenteral nutrition (TPN) often required

262
Q

DDx of ompahlocele and Gastrochisis

A

Omph: bowel remains in the umbilical cord & covered by peritoneum & amniotic membranes

263
Q

Rule of 2s of meckels

A 
The rule of 2’s:
2% of the population
2 ectopic mucosae
-Gastric/Pancreatic
Presents by 2 years 
Within 2 feet of the cecum
2 inches long
264
Q

MASSIVE painless GI bleeding

Think

A

Meckles Diverticulum

265
Q

Any evidence on imoperforate anus

Needs what follow up image

A

All imperforate anus require MRI of the lumbosacral spinal cord

high incidence of tethered spinal cord

266
Q

When should you do a DRE in pediatrics

A

HIRSHPRUNGS

267
Q

MGMT for necrotizing enterocoliitis

A

Stop enteral feedings → start Total Parenteral Nutrition (TPN), GI decompression w/ NG, Fluid & electrolyte replacement
Broad spectrum abx
Surgery: laparotomy w/ excision of affected bowel

268
Q

Intussusception is associated with what viral infection

A

Associated w/ rotavirus infection & old rotavirus immunization*

269
Q

Pt presents with “Currant jelly” stools: mixture of mucus, sloughed mucosa, & blood

Think

A

Intussusception

270
Q

Donut sign on US

Think

A

Intussusception

271
Q

MGMT for intussusception

A

Pneumatic or contrast (i.e., barium) enema under fluoroscopy

Diagnostic & therapeutic

Pneumatic preferred: NO barium peritonitis if bowel perforates & allows for subsequent radiologic studies

272
Q

Most common surgical emergency in children

A

Appendicitis

273
Q

ALVARADOS Score

A

Score 1 point for each:

  • Migration of pain to RLQ
  • Anorexia
  • Nausea/vomiting
  • Rebound pain
  • Fever (at least 37.3° C)
  • WBC shift >75% neutrophils

Score 2 points for each:

  • RLQ tenderness
  • Leukocytosis >10,000/µL

Score < 4 Unlikely
Score > 7 Likely

274
Q

Primary cause of arrest in peds

A

Respiratory arrest, not cardiac, is the primary cause of cardiopulmonary arrest in children (contrast to adults)

275
Q

define dissociative shock

A

O2 not appropriately bound or released from hemoglobin

Causes:
Carbon monoxide poisoning
Methemoglobinemia

Clinical presentation:
Tachycardia
Tachypnea
Alterations in mental status
Cardiovascular collapse
276
Q

Cervical spine radiographs are not sufficient to rule out a spinal cord injury → due to immature vertebral column:

Allow stretching of the cord or nerve roots w/ no radiologic abnormality (Spinal Cord Injury Without Radiologic Abnormality [SCIWORA])

If suspect SCIWORA=

A

Get MRI

277
Q

MGMT of BRUE

A

Tests for RSV & pertussis (for respiratory infections)
Barium swallow or pH probe study (for GERD)
High risk patients: Admit to hospital for12-24 hr cardiorespiratory monitoring

Full workup

278
Q

Antidote for iron poisoning

A

Deferoxamine

279
Q

Treatment for Lead poisoning

A

Edetate calcium disodium (EDTA)

Dimercaprol (BAL = British Anti-Lewisite)

Prepared in peanut oil → do not use in patients w/ peanut allergy

Succimer (DMSA = dimercaptosuccinic acid ) → few toxic effect

280
Q

S/s of lead poisoning

A 
Insidious onset:  weakness, lethargy, ataxia, growth delays, school problems
Alopecia
Gum lines
Seizures
Coma (if severe)
Hypochromic microcytic anemia
Basophilic stippling
281
Q

S/s of methanol poisoning

A

optic papillitis & retinal edema

282
Q

MGMT for Methanol/ Ethylene Poisoning

A

Treatment:
10% ethanol & D5W

Fomepizole (Antizol)

  • Alcohol dehydrogenase inhibitor
  • 8,000 times more affinity than ethanol*

Supplementation

  • Thiamine & B6 (for ethylene glycol)
  • Folic acid (for methanol)

Senoir 21-2 (77 decks)

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