Board Vitals #4 Flashcards

1
Q

MOA of Trazadone
Uses
Side effects

A

seratonin modulator; antagonist of postsynaptic sterotonin receptors and blocks reuptake
Used for MDD, dyspepsi adn insomnia
SE: sedation, dizzy, dry mouth, arrythmia, nausea
*oral absorption 20% higher when taken with food

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2
Q

Infant with elevated ammonia, ketosis, elevated anion gap metabolic acidosis in setting of tachypnea, poor feeding, FTT
What group of inborn error of metabolism is this?
What is treatment

A
Organic acidemias (Propionic acidemia or MMA)
Tx Biotin
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3
Q

Present with fever, fatigue, malaise, nausea and decreased appetite with headache. Some have Rose spots or blanching, faint erythematous maculopapular rash.
BRADYcardia +Fever + dehydration and transanimitis

A

Typhoid fever
from salmonella typhi
Bradycardia + fever + dehydration is classic for thypoid fever

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4
Q

Disease that causes hypersensitivity to stimuli, hypertonicity–> opisthotonus, low grade fevers, pyschomotor devo arrest and demyelinating polyneuropathy with arreflexia.
See accumulation of material in white matter with sparing of U fibers

A

Krabbe disease
Auto recessive lysosomal storage
Deficiency galactoxylceramidase
Lots of galactocerebroside in macrophages of brain–> death by one in infantile disease

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5
Q

Monoarticular arthritis in child under 6. More common in females, usually resolves
Dx
Most common morbidity
Tx

A
Pauciarticular JIA
onset for kids <10, more common in 2nd year of life
often resolves by 6 months
Morbidity is UVEITIS
Tx NSAIDS
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6
Q

Benign systolic ejection murmur, vibratory and best heard over precordium. Decreases in intensity with expiration and positional change which decreases venous return (standing)

A

Stills

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7
Q

Low pitched cont murmur loudest when patient has head extended and absent when supine

A

venous hum

from blood draining from jugular veins to intrathoracic veins

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8
Q

preterm infant with increased oxygen requirement, bounding pulses, infraclavicular and interscapular systolic murmur. Hepatomegaly on ex
Dx and tx

A

patent PDA

Indomethacin or ibprofen

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9
Q

What are the four defects in TOF?

A

pulmonary stenosis, overriding aorta, RV hypertrophy, VSD

See right axis deviation on EKG

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10
Q

Present early in life with severe metabolic acidodsis and heart failure with L–> R shunting. See tall P waves adn left axis deviation with LVH

A

Tricuspid atresia

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11
Q

What do you hear on auscultation and see on CXR of patient with Ebstein anomaly?

A

Auscultate holosystolic murmur at left lower sternal border
See ‘wall to wall heart’
From tricuspid valve with apical displacement of leaflets==> atrializationof RV

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12
Q

What presents with ‘egg on a string’

A

Transposition of great vessels

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13
Q

What kids get 0.15mL of epi

What about 0.3ml?

A
  1. 15mL for 15-30kg

0. 3ml for >30kg

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14
Q

Patient presents with oral bleeding, frequent epistaxsis and easy bruising
You suspect vWB, what do you see for the following labs
Risocetin cofactor
PT
aPTT
platelets

A

low risocetin cofactor
normal PT
slightly long aPTT
normal plts

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15
Q

Pt present with axillary LN swelling but no hx of weight loss or fevers. Has a vesicle and rash on her hand mom says she has been itching. They have a new kitten

A

Cat scratch
Bartonella henselae
tx Azithromycin, bactrim or rifampin

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16
Q

Anterior displacement of vertebral body due to defect in posterior arch of vertebrae. see radicular back pain and parethesias

A

Spondylolisthesis

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17
Q

Contraindicated in babies <1 month due to risk of hyperbilirubinemia

A

Ceftriaxone

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18
Q

Contraindicated up to 120 days of life due to risk of pyloric stenosis

A

macrolides

azithro, clarithro, erythro

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19
Q

Infants born to mom’s with autoimmune thyroiditis should have this lab drawn

A
risk for hypothyroid
Get TPO (thyroid peroxidase antiB levels)
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20
Q

9 mo old exposed to measles 24 hours ago, tx?

What if exposed 4 days ago?

A

Give MMR if <12 months and w/in 72 hrs of exposure

From 72 hrs to 6 days can give IVIF or IGIM

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21
Q

What is the treatment recommendation for moderate persistent asthma?

A

ICS plus LABA

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22
Q

asthma symptoms occur >2/wk but not daily, 3-4 night wakenings and minor interference with activities

A

Mild persistant asthma

start ICS

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23
Q

asthmasymptoms occur daily, >1 night wakening/wk and some limitiation on daily activity

A

Moderate persistant asthma

start ICS + LABA

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24
Q

Patient with hepatomegaly, FTT and fanconi syndrome (proximal tubule dysfunction = renal wasting of phosphate, glucose, aa, protein, bicarb).
Labs with elevated serum succinylacetone and hypophosphatemia

A

Tryosinemia type 1

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25
Q

Patient with optic nerve hypoplasia, midline brain defects and pituitary abnormalities

A

Septo-optic dysplasia
mutations in HEXS1 and SOX2
GH deficiency leads to hypoglycemia and short stature

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26
Q

Patient with recent illness presents with hematuria with tubular casts. Low C3

A

PSGN

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27
Q

How do you manage an iron overdose?
What level is toxic?
What is tx?

A

Get serum iron level; xray doesn’t rule out ingestion.
toxic over 60mg/kg (less thatn 20mg/kg usually asymptomatic)
Tx =support and deferoxamine treatment

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28
Q

Deficiency of this vitamin causes vesiculobullous dermatitis, alopecia, diarrhea, opthalmic disorders and growth retardation/delayed sexual growth

A

Zinc

See acrodermatitis enteropathica

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29
Q

Biphasic stridor is hallmark in what type of airway obstruction

A

Extrathoracic airway obstruction that occurs above the thoracic inlet.
SUBGLOTTIS STENOSIS associated with intubation

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30
Q

Disruption of normal cartilage growth at medial aspect of proximal tibial physis. Kids present with angular varus deformity of proximal tibia and progressive genu varum

A

Blount disease

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31
Q

What is our first line screen in patient you suspect has celiacs?
What do you do to confirm the diagnosis?

A

Screen with IgA-tTGA as first line screen

Confirm with endoscopy and small bowel biopsy

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32
Q

Patient with 7 cafe au lait macules on body and sessile masses on skin of back with nontender freckles on bridge of nose.
What tumor is this patient most at risk of developing?
What is disease and inheritance?

A
Optic glioma
**Risk of peripheral nerve sheath tumors**
NF-1
Auto Dominant
(risk of juvenile CML)
33
Q

Sudden onset of low back pain with muscle weakness and paresthesias in toes and feet. Patients will have paralysis, urinary incont and loss of bowel control.
Best diagnostic modality

A

Transverse myelitis
MRI of spine to r/o other pathology and will see inflammation
*can see pleocytosis in CSF

34
Q

Newborn is 3 days old with midline mass in lower abdomen, poor UOP. Most likely cause

A

Posterior urethral valves

blocks urine flow out of bladder; see FTT, weak urine stream, palpable kidney and abdominal mass

35
Q

What is associated with Posterior Urethral valves?

A

undescended testicles, renal dysplasia, VUR, bladder cysts

36
Q

What is the reason we don’t give infants cows milk the first year of life?
What symptoms would you see?

A

High levels of phosphate i cows milk–> this leads to hyperphosphatemia then hypocalcemia
See poor musclular contractility, irritability, tetany

37
Q

Newborn presnets 4 hours after delivery tachypneic with clear lungs and central cyanosis with difficulty breathing. You hear 3/6 cont murmur and pre-ductal sats are 70s. Most likely group of disorders and treatment?

A

Cyanotic heart lesion
Tx immediately with prostaglandins to keep PDA open
Most likely transposition as this presents earliest in extrauterine life

38
Q

What are the criteria for diagnosing T1DM

A
  1. HbA1C >6.5
  2. fasting >126 on 2 occasions
  3. Symptoms hyperglycemia >200
  4. Plasma glucose >200 obtained 2 hours after standard glucose load
39
Q

What are the key labs we see in patient with SIADH?

A
Euvolemic hyponatremia
hypoNa with serum Na <135
serum osmolality <280
high urine sodium >40
urine osmolality >100
40
Q

What is the inheritance of hypophosphatemic rickets?

A

X linked domimant

41
Q

Infant with 3-5 days of high fever, irritable and lymphadenopathy.
Fever gone then rash with blanching maculopapular lesions around neck and trunk–> to face and arms

A
Roseola infatum (6th dx)
from HHV-6
42
Q

Patients with Wiskott Aldrich syndrome which is _____linked present with which symptoms and why

A

X linked
eczema, thrombocytopenia, bacterial/viral/fungal infections
WASp gene issue–> causes problems with cytoskeletal remodeling.

43
Q

What are Ig levels like for Wiskcott aldrich?
IgM and IgG
Ig E and IgA

A

M and G are HIGH

A and E are LOW

44
Q

Patient comes in after passing out with exercise and rest. PE without murmur in multiple body positions with normal BP. EKG shows epsilon waves

A

Rt Ventricular dysplasia (ARVD)
fibrous/fatty infiltration of myocardium.
Dx on cardiac MRI
Affects 1 in 5000 people

45
Q

What is empiric outpatient therapy for UTI in children?

A

2nd gen (cefuroxime or cefprozil) or 3rd gen cephalosporin (cefdinir, cefixime, cefpodoxime, deftibuten)

46
Q

What are the 10 live or live attenuated vaccines

A

oral polio, varicella, MMR, rota (oral and injectables), shingles, smallpox, thyphoid, BCG, yellow fever

47
Q

Reflex elicited when placing infant supine and turning head to the side; ipsi extension of arm and leg and flexion of contralateral arm

A

Asymmetric tonic neck reflex
‘fencing’
established by 1 month, gone around 3-4 months

48
Q

When does Moro reflex disappear?

A

3 months

49
Q

See primary cortisol deficiency (hyperpigmentation, fasting hypoglycemia) as well as hypoaldosteronism (hypoNa and hyperK) when there is deficiency of this enzyme

A

21 alpha hydroxylase

50
Q

Female with ambiguous genitalia at birth, hypokalemia, hypertension

A

11 b hydroxylase defiency

51
Q

MCC of macrocephaly

definitoin of macrocephaly

A

Familial megalencephaly

HC 2 standard deviations above for given age/sex or >97%

52
Q

First line treatment for patients with mild to moderate dehdration

A

3-4 hour rapid oral rehyrdation

53
Q

Sedative good for rapid intubation, good safety profilee and brief sedation period (5-15mins) low rate of adverse events.
Reduces ICP ad maintains hemodynamic stability

A

Etomidate

Good for sedating pts with uncertain CV status or trauma patient

54
Q

Sedation med contraindicated if soy or egg allergy

A

Propofol

55
Q

Unilateral anterolateral angulation or bowing of tibian can progress to thinning of cortex and fracture.
This is called ____
Its a dx criteria for this disorder

A
Congenital pseudoarthrosis (false joint)
NF1
56
Q

Treatmetn for methanol and ethylene glycol overdose

A

Fomepizole

57
Q

Damage to this or nerve root causes Hoerner syndrome

A

T1

58
Q

You suspect a patient is having a seizure due to meningitis was the first thing you need to do for this patient

A

Benzodiazepines! Stop seizure then IV antibiotics

59
Q

In an infant born to mom with hepatitis see what test should be done on the infant and at what point

A

HCV RNA by RNA

Can do IgG antibody assay but not available til 18 months

60
Q

A rash that is symmetrically distributed erythematous patches or Dominus plaques that have a dusky center and give them a target like appearance they may be mildly burning or itchy and often located on the palms and soles of feet. What are these and what other rash do you see them with?

A

Erythema multiforme, this can be seen after a HSV infection

61
Q

A mass found at the distal part of a bone, usually asymptomatic and incidentally discovered. Most commonly found in patients between five and 15 years of age

A

Osteochondroma, just observe

62
Q

A benign bone forming tumor that will cause local distraction of the bonus rolls of a time, most often they located in the vertebrae

A

Osteoblastoma, they will present with all aching pain for months

63
Q

What is the abnormal innovation in Marcus gun which is jaw winking phenomenon

A

Abnormal innervation of the trigeminal and ocular motor nerves. This is due to the trigeminal nerve which controls mastication as well as ocular motor nerve which controls the elevator palpebre mscl which is involved in winking. You will see a phenomenon in which you wink as you chew

64
Q

Damage to this nerve causes symptoms like loss of bitter and sour tastes as well as impaired swallowing

A

Glossopharyngeal nerve

65
Q

What are symptoms of autoimmune hemolytic anemia and what labs do you expect to find?

A

Occurs after an illness, you can find icterus and splenomegaly
Labs including reticulocytosis, hyperbilirubinemia, and increased LDH

66
Q

Newborn with fetal distress and hypoxemia at birth has a unilateral flank mass at day of life 4

A

RVT

Risk factors include shock, dehydration, maternal diabetes

67
Q

And what situation can you do involuntary drug testing on teens?

A

Szs, emergency, attempted suicide

68
Q

A patient was recently started on a medication and 2 to 3 weeks later developed the following symptoms: facial edema and dusky erythematous papules and later development of vesicualr and bolus lesions. There is no involvement of the mucosal membranes. They have a fever and are very ill. What is the syndrome and what labs are typically found

A

DRESS Drug rash with eosinophilia and systemic symptoms
Scene with seizure medication’s. Labs include Eosinophilia and atypical lymphocytosis and elevated liver enzymes, elevated Cr
Tx stop Med and corticosteroids for weeks

69
Q

Pulseless child with nonshockable rhythm.

Steps for CPR?

A

PEA

start CPR, epi and then compressions for min two mins before checking

70
Q

What are shockable rhythms in CPR?
For defibrillation?
For synchronized cardio version?

A

Defibrillator for pulseless Vtach or V fib first 2J/kg then 4J/kg
cardiovert for hemodynamically unstable tachyarrythmia with pulse

71
Q

Positive non-glucose reducing substances suggest

A

Positive non-glucose reducing substances suggest galactosemia or fructose intolerance

72
Q

The absence of urinary reducing substances and ketones in an infant with enlarged liver suggest defect in?

A

Fatty acid metabolism

73
Q

What substance is contraindicated in patients with GSD type one?

A

Galactose. They have glucose-six-phosphatase deficiency

74
Q

Cyclic febrile neutropenia is associated with which infectious disease?

A

Clostridium

75
Q

What seizure disorder is associated with central temporal sharp waves?

A

Benign rolandic epilepsy of childhood. You typically see nighttime episodes of facial twitching and difficulty talking, me generalize into full body shaking

76
Q

3 Hz generalized spike and wave discharges associated which was type of seizure?

A

Absence: staring

77
Q

Hypsarrhythmia is associated with with seizure disorder

A

Infantile spasms

78
Q

Patient with recurrent nosebleeds and skin telengiectasias specifically on the lips and in the mouth.
Inheritance pattern and other complications?

A

Hereditary Hemorrhagic telangiectasia
AD
Increase risk of GI and Pulm bleed or hemorrhage and can see hepatic bleeds