Pediatric Board Study #1 10/2020 Flashcards

1
Q

What is an “apophysis”?

A

A protuberance

TIP: It “POP”s out

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2
Q

What is the presentation of Sever’s disease? What type of activities cause it?

A

Sever’s disease is calcaneal apophysitis

Athletes with cleats or poorly cushioned heels

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3
Q

What are the 3 places the calcaneus experiences stress?

A

1) Plantar fascia pulls
2) Achilles tendon pulls
3) Ground impact

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4
Q

What physical exam finding can demonstrate a patient tends to pronate their foot?

A
Pronate foot (pinky toe up in the air)
Supinate foot (inverting foot like in an ankle roll)

Patient jump in air: foot will pronate when bending knees and knees will become valgus (knock kneed)

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5
Q

Relationship between pronating foot and sever disease?

A

Pronating feet -> more stress on plantar fascia -> risk for Sever disease

Hence: medial arch support to keep from pronating

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6
Q

First test to evaluate esophageal dysphagia?

A

Upper GI series

CLINICAL TIP: In adults, EGD is first choice

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7
Q

Charactersistics of Beckwith Weidemann Syndrome?

A

Overgrowth syndrome for neonate

Overgrowth of body: hemihypertrophy/macroglossia
Overgrowth of organs: hepatosplenomegaly
Overgrowth of pancreas: prolonged neonatal hypoglycemia
Overgrowth of cancer cells: Wilms tumor/Neuroblastoma/Hepatoblastoma
Overgrowth of heart: Cardiomegaly
Overgrowth of kidney: Nephromegaly

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8
Q

Differential diagnosis of patient with hemihypertrophy?

A
Beckwith weidemann syndrome
Klippel Trenaunay Weber Syndrome 
Proteus Syndrome (PTEN hamartoma tumor syndrome)
NF Type I 
Isolated Hemihyperplasia
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9
Q

Autonomy is to adult patients as BLANK is to pediatric patients

A

“Parental Authority”

That is the autonomy that parents have to make a decision on behalf of their children

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10
Q

How diagnose acute rheumatic fever?

A

Evidence of strep infection AND 2 major criteria

Evidence of strep infection AND 1 Major/2 Minor criteria

TIP: “Major Jones” sounds like a person in the military

J: joints
O: heart - carditis
N: subcutaneous nodules 
E: erythema marginatum 
S: Syndeham Chorea
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11
Q

Treatment of Acute Rheumatic Fever (ARF)?

A

TIP:

Think of ARF as an inflammatory sequalae of a an infection

Treat infection: Penicillin
Treat inflammation: ASA

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12
Q

What is the age appropriate way of thinking for preschoolers?

A

Preschoolers are 3-5 yo

  • Magical thinking, that is, unable to separate fantasy from reality
  • See themselves as the “center of the world”
  • Do not appropriately see the link between cause and effect
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13
Q

Where can I find out guidelines to care for survivors of cancer?

A

www.survivorshipguidelines.org.

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14
Q

Why is rectal temperature measurement contraindicated for neutropenic patients?

A

Concern for risk of bacteremia from rectal trauma

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15
Q

At what elevated temperature is there a posed risk to a patient?

A

Difference between fever and hyperthermia is that the former is physiologic while the second is pathophysiologic

107 F
42 C

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16
Q

At what age is rectal temp no longer the way to go?

A

> 3 years old given the trauma

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17
Q

Preferred means of measuring temperature in a child < 3 yo?

A

Rectal thermometer

Infrared of the ear or the temporal artery is NOT accurate in < 6 months

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18
Q

Definition of “Severe” Malaria?

A

Any one of the following:

  • Severe anemia
  • Organ damage: renal failure, ARDS, cerebral malaria,
  • Shock
  • Metabolic acidosis or hypoglycemia
  • Parasitemia > 5%
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19
Q

Treatment for malaria?

A

Quinidine + Tetracycline (OR Clinda)

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20
Q

What physical exam finding distinguishes Crouzon vs Apert Syndrome?

A

Apert has mitten hands

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21
Q

What is the chromosomal abnormality of Klinefelter Syndrome?

A

XXY

Picmonic

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22
Q

What are the features of Klinefelter Syndrome?

A
  • Tall
  • Gynecomastia
  • Small testicles (primary hypogonadism)
  • Poor social skills
  • delayed puberty
  • Poor social skills, Learning disability, language delay
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23
Q

What is a normal arm span to height difference?

A

Arm span - height < 2.5 inches

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24
Q

How is venous return affected during the following maneuvers:

  • Squat:
  • Valsalva:
  • Standing:
A
  • Squat: increase venous return (opposite of standing, or think of squatting like exaggeration of walking which causes venous return)
  • Valsalva: decrease venous return (MOA: increased intrathoracic pressure takes up more space in the thoracic cavity leaving less room for venous return)
  • Standing: decrease venous return
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25
Q

How should murmur of HCM vs AS differ when valsalva? When squatting?

A

HCM: louder with valsalva, lower with squat

Explanation:

  • In normal circumstances, increase flow through a cardiac lesions causes a louder murmur. Increased venous return is an example.
  • HCM is the opposite. Murmur is caused by the interaction between Septum and Mitral valve. Since increased venous return “separates” the two from each other, murmur decreases
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26
Q

How does hang grip maneuver affect murmurs? (General framework)

A
  • Handgrip increases afterload
  • Forward flowing murmurs (e.g. AS, HCM) decrease with hand grip since it’s a counter force
  • Backward flowing murmurs (e.g. mitral regurgitation) increase with hand grip
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27
Q

What part of the body do actinomycetes infections present?

A

Cervicofacial

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28
Q

Actinomycetes picmonic

A
  • Golden tree: branching chains
    gram positive bacilli
  • Head and neck with tree coming from top: cervicofacial presentation like neck massage
  • dentures hanging on the tree: oral flora
  • Abe Lincoln with blue light saber cutting down teeth: penicillin
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29
Q

Why does humidity increase the risk of heat exhaustion and heat stroke?

A

Humidity means more water in the air. Keeps sweat on skin from evaporating. Less cooling.

COOL Phys: the process of evaporating liquid sweat provides cooling. It requires energy, energy taken as heat from the body, leading to cooling.

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30
Q

How to distinguish between heat stroke and exhaustion?

A

Stroke: CNS sx and 104 (40 c)

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31
Q

Relationship between Noonan Syndrome vs Turner syndrome?

A

Phenotypically similar to each other but Noonan has normal karyotype.

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32
Q

Cardiac defect of Noonan Syndrome

A

Pulmonary stenosis

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33
Q

What are the skin changes associated with long term topical steroid use?

A

Hypopigmentation, atrophy and TELANGIECTASIA

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34
Q

What type of topical formulations are best tolerated?

A

Ointments > creams > lotions > solutions > foams

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35
Q

What type of potency level should be used for eczema?

A

Low to medium potency

Low for face and intertriginous areas

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36
Q

Presentation of Babesia?

A

Picmonic:

  • ixodes scapularis: ixodia head on tick
  • Maltese cross in RBC: throwing Maltese crosses
  • Northeast and Midwest: Jew
  • Hepatomegaly: liver shield hit by Maltese crosses
  • hemolytic anemia and thrombocytopenia: broken plates and RBC from stars
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37
Q

What type of parenting style fosters resilience?

A

Authoritative parenting style

TIP: balance the rules a parent sets with encouragement and love. Does NOT mean there is no room for personal choice, but has to be age appropriate.

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38
Q

What is resilience?

A

The ability to first withstand hardship, then adapt to it, followed by recovery from it.

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39
Q

What is the definition of hematocrit?

A

Volume of RBC/the Volume of Blood usually expressed as a percentage

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40
Q

How does the hematocrit differ between a full term neonate and a preterm neonate? Why?

A

In the third trimester, iron stores from the mother are transferred to the fetus. In addition, the liver becomes more sensitive to hypoxia, producing more EPO. Preterm infants miss this last stage of increased RBC production, hence lower Hematocrit

TIP: In the fetus, the liver, NOT the kidney, acts as the producer of EPO in response to hypoxia

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41
Q

Most common cause of congenital hypothyroidism?

A

Thyroid dysgenesis which is the abnormal development of the thyroid, which is either of 3 things:

  • incomplete development of thyroid tissue
  • lack of thyroid tissue
  • ectopic thyroid tissue that is dysfunctional
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42
Q

Mother has Hashimoto Thyroiditis - can fetus be affected?

A

No

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43
Q

How does cold panniculitis present? Treatment?

A
  • Red indurated plaques, somewhat similiar appearing to cellulitis
  • No treatment; reduce exposure to cold; resolvs in 3 weeks
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44
Q

What is panniculitis?

A

Inflammation of the subcutaneous fat (e.g. erythema nodosum)

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45
Q

What level of proteinuria in UPC is diagnostic of nephrotic range proteinuria in children?

A

> 2mg/g

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46
Q

What labs can aid in differentiating glomerulonephritis from nephrotic syndrome?

A
  • Bun/Cr
  • Complement levels

GN is associated with HTN and azotemia

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47
Q

Most important lab to obtain in ALL patients who present with AMS?

A

POC Glucose

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48
Q

Gold standard for diagnosing narcolepsy?

A

Polysomnography with multiple sleep latency tests

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49
Q

What are the 4 core clinical features of Narcolepsy?

A

1) Cardinal feature: Daytime sleepiness
- TIP: VERY COMMON SYMPTOM IN GENERAL
2) Hynagogic hallucination:
3) Sleep paralysis
4) Cataplexy

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50
Q

What is entailed in a Multiple Sleep Latency Test? What are features suggestive of Narcolepsy?

A

Patient instructed to sleep every 2 hours for 20 minutes

In Narcolepsy, sleep latency is short and REM is reached in 2 out of 4 sleep periods

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51
Q

What is Lemierre’s syndrome? Associated bacteria?

A
  • Septic thrombophlebitis of the internal jugular vein

- Fusobacterium Necrophorum

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52
Q

What are the associated illnesses in patients with Lemieere’s syndrome?

A
  • Classically, pharyngitis

- However, any head and neck infections, including otic and dental

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53
Q

10 year old previously fully a vaccinated. Underwent HSCT and is now in transmission and OFF immunosuppressive therapy - what to do in regards to Hib vaccination?

A

ALL children that received HSCT require 3 doses of Hib vaccine 6 months AFTER HSCT/completion of immunosuppressive therapy - REGARDLESS of prior vaccine status

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54
Q

What are the vaccines scheduled from 0 - 6 months of age?

A
  • “TWO 6-MONTH-old Pediatric POLIticians DIscussed Rejecting HIs HEP B at 4 MONTHS”
  • 2/6 months have the same 6 shots
  • 4 mos has same shots EXCEPT Hep B
  • P: PCV
  • Poli: POLIO
  • DI: DTAP
  • R: RV
  • HI: Hib
  • HEP: Hep B

TIP: First 6 months, 6 shots each time.

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55
Q

Classic presentation of MCL sprain?

A
  • Pain in medial aspect of knee
  • Localized swelling in the medial aspect of knee
  • Pain with valgus stress
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56
Q

Clues that MCL sprain is accompanied with other severe injuries?

A

Joint effusion around entire knee

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57
Q

What type of hyperbilirubinemia would Gilbert and Crigler Najjar present with?

A

Unconjugated hyperbilirubinemia

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58
Q

What is the pediatric definition of cholestasis from a lab stand point?

A

Conjugated bilirubin > 1.0 - SIMPLE!

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59
Q

Most common cause of neonatal cholestasis?

A

Biliary atresia, a progressive obliterative disease of intra/extra hepatic biliary ducts that leads to liver diseas

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60
Q

What are the features of Stickler Syndrome?

A

PICMONIC

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61
Q

What is Pierre Robin Sequence?

A

Presence of:

  • Micrognathia
  • Cleft palate
  • Glossoptosis (retracted and downward displaced tongue, leads to obstruction)
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62
Q

Children with obesity should be screened for HLD and HTN. Which patients with obesity should be screened for diabetes?

A
  • Obesity with addn risk factors:
    • Family hx of Type II DM
    • High risk ethnicity: Native American, Asians, Pacific Islanders, Hispanics, African americans (TIP: NOT whites/indians/middle east)
  • PE signs of insulin resistance

Styne DM, Arslanian SA, Connor EL, et al. Pediatric obesity-assessment, treatment, and prevention: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2017;

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63
Q

What are the tests of choice to screen pediatric patients for diabetes?

A
  • Fasting glucose
  • A1c
  • Oral glucose tolerance test
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64
Q

Can pediatric patients with substance use disorder be enrolled into rehab without their consent?

A

YES!

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65
Q

Can chronic use of marijuana be considered a substance use disorder?

A

YES! Like any other substance, even as culturally acceptable as marijuana, as long as it causes dysfunction in a patient’s life, it can be considered a disorder that requires treatment

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66
Q

Otalgia may be referred pain from what other part of the body?

A

Dental pain is the most common cause of referred otalgia

COOL PHYS: Referred pain comes from misinterpretation of the sensory cortex 2/2 overlapping innervation

The auriculotemporal branch of the mandibular division of the trigeminal nerve supplies sensory innervation to parts of the auricle, external auditory canal, and tympanic membrane, as well as the cranial cavity, scalp, orbit, face, nose, nasal cavity, nasal sinuses, mouth, teeth, temporomandibular joints, and parotid glands.

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67
Q

In patients with splenectomy, what characteristics place them at higher risk of sepsis 2/2 encapsulated organisms?

A
  • Splenectomy 2/2 a primary blood disorder
  • Splenectomy at a young age
  • Recent splenectomy
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68
Q

What type of organisms are asplenic patients at risk for?

A

Encapsulated bacteria:

  • SHiNSKiS
    • Salmonella
    • Hemophilus Inf
    • Neisseria Meningitiditis
    • Staph aureus
    • Klebsiella
    • Strep Pneumoniae

EXTRA: Pseudamonas, E. Coli, Group B Strep

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69
Q

What age group, if contracted influenza, should be treated with antivirals?

A

< 5 yo (high risk for complications)

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70
Q

Behaviors concerning for suicidal ideation?

A
  • Distributing possessions

- Posting about suicidal thoughts via social media

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71
Q

Pathogen responsible for RMSF?

A

Rickettsia rickettsia

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72
Q

What is the defective enzyme of galactosemia?

A

Galactose 1 phosphate uridyltransferase

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73
Q

Associated lab abnormalities in galactosemia?

A
  • Metabolic acidosis
  • Hypoglycemia
  • Coagulation disorder
  • Hyperbilirubinemia
  • Hepatic injury
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74
Q

What are the phases of Lyme disease?

A
  • Early localized: erythema migraña
  • Early disseminated:
    • Carditis, heart block
    • CNS, meningitis, facial palsy
    • Arthritis
    • Diffuse rash
  • Late disseminated: large joint arthritis effusion which tends to be out of proportion with pain and inflammatory markers
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75
Q

Relationship between JIA and Ankylosing Spondylitis?

A

Ankylosing spondylitis is a subtype of JIA called enthesitis related arthropay (ERA)

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76
Q

Describe the arthritis of Ankylosing Spondyloarthritis

A

Starts with oligoarthritis of hip, knee and ankle

Then later axial skeleton

Imagine the arthritis creeping up the body to reach the back

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77
Q

Describe the schober test?

A

he Schober test is used to measure forward flexion of the lumbar spine—with the patient standing straight, a mark is made over the spinous process of the 5th lumbar vertebra; a second mark is then made 10 cm above it. After asking the patient to bend maximally forward, the distance between the 2 points is normally > 15 cm. Measurements ≤ 15 cm are consistent with limited forward flexion.

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78
Q

What organizations need to be contacted if non-adolescent pediatric patient is diagnosed with STI?

A

CPS and Health Department

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79
Q

What are ways to BF twins?

A
  • Sequential

- At the same time

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80
Q

What technique can be used to measure patient’s fine motor skills in years when asking them to draw a person?

A

3 + 1/4( # points) = age

1 point for every body part:

  • nose
  • mouth
  • pair of eyes
  • pair of arms
  • pair of legs
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81
Q

What is the one obligatory lab work for all college athletes per National Collegiate Athletic Association?

A

Sudden death attributed to exertional rhabdomyolysis (a.k.a. “exertional sickling”) has been reported among athletes with sickle cell trait

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82
Q

Patient develops herpes zoster. Under what conditions can he return to work?

A

Anytime as long as the lesions are covered

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83
Q

What is the difference between the location of the bleed in epidural hematoma vs subdural hematoma?

A
  • Epidural hematoma: “Above” the dura. Between the skull and the dura mater
  • Subdural hematoma: “Below” the dura. Between the dura and the arachnoid mater
  • Review:
    • Skull -> Dura -> Arachnoid mater -> pia mater
    • Veins draining blood from brain parenchyma cross all the layers until reaching the dural sinuses
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84
Q

What is the CT findings of epidural hematoma vs subdural hematoma?

A
  • Subdural: Crescent shaped.
    • Exp: location of bleed between dura and arachnoid mater, hence, it can cross suture lines, but not midline
  • Epidural hematoma: Lenticular shaped
    • Exp: location is between periosteum and dura; since dura is attached to the sutures, blood can’t cross sutures
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85
Q

What is the most common cause of SDH in children?

A

Child Abuse

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86
Q

Most common cause of acute hoarsness?

A

Viral infection

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87
Q

Most common cause of chronic hoarseness?

A

Voice abuse

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88
Q

What is the best test to evaluate if a newborn had been exposed to illicit drugs during pregnancy?

A

Drug Screen of the meconium as UDS only evaluates drug exposure in the past 5 days.

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89
Q

What test, if present only once, is diagnostic of Type II DM?

A

Random glucose > 200 + Symptoms of hyperglycemia

TIP:
- A1c, Fasting Glucose > 126, or OGTT > 200 - all x 2 are diagnostic of diabetes

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90
Q

What autoimmune diseases are recommended to be screened at the type of diagnosis of Type I DM?

A
  • Celiac disease

- Hypothyroid

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91
Q

What is the recommended caloric intake for infants with congenital heart disease?

A

140kcal/kg/day

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92
Q

Framework Question:

How much formula an infant needs each feed?

A
  • 0-6 months: 110 kcal/kg/day
  • 7-12 months: 100 kcal/kg/day
  • Formula is 20kcal/oz
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93
Q

Framework Question:

A parent asks you: How much formula an infant needs each feed

A

0-3 months: at least 5 oz/kg/day

3 - 12 months: anywhere between 26 to 32 oz/day
- TIP: AKA if feeding 8x/day, anywhere between 3 oz/feed to 4 oz/feed

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94
Q

Pediatric patient just diagnosed with Juvenile Myasthenia Gravis, what is the next step in work up?

A

CT Chest to evaluate for thymoma

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95
Q

What is the treatment for Myasthenic Crisis?

A
  • Plasma exchange OR IVIG, both aiming to reduce the burden of autoantibodies
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96
Q

What common meds can worsen Myasthenia Gravis?

A
  • Antibiotics: Fluoroquinolone, Macrolides and Aminoglycosides
  • Magnesium
  • STEROIDS! - cause a transient worsening of symptoms in the first 2 weeks
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97
Q

What is areas are evaluated in a FAST exam?

A
  • Hepatorenal recess
  • Splenorenal recess
  • Suprapubic recess
  • Subxyphoid view to evaluate for pericardial effusion
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98
Q

Presentation for ichthyosis vulgaris?

A
  • Hyperkeratotic scales on the extensor surfaces
  • Hyperlinear palms
  • Sparing the flexural surfaces
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99
Q

Treatment for ichthyosis vulgaris?

A
  • Emollient containing alpha hydroxy acid (e.g. ammonia lactate)
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100
Q

What is the preferred treatment for C. Diff in children? Adults?

A

Children:

  • Non severe: Metro vs oral vanc
  • Sever: Vanc

Adult:

  • Non severe: Vanc
  • Severe: Vanc +/- metro

TLDR: Adult use vanc, vanc, vanc; Children use metro or vanc, but def vanc in severe disease

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101
Q

Are there any adverse effects of lead exposure at low levels, that is < 5 µg/dL?

A

YES!

  • Children with lead levels < 5 are more likely to be diagnosed with ADHD and lower IQ’s
  • Pregnant mothers with lead levels < 5 are more likely to have small birthweight children
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102
Q

Mother of a newborn is found to have elevated lead levels. How should she be counseled in regards to breastfeeding?

A

No breastfeeding since lead can be transmitted through breast milk.

PICMONIC: breast with lead pencils

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103
Q

What is the recommendation in regards to the use of renal ultrasound and VCUG in infants with UTI?

A
  • ALL children < 24 mos of age require RUS for their first febrile UTI because there are high risks for anatomic abnormalities.
  • VCUG is pursued later if RUS demonstrates any abnormalities to evaluate for VUR
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104
Q

What amount of bacteria in urine specimen is diagnostic of UTI in infants? (depending on the method of obtaining urine culture)

A

TIP: The move invasive the procedure, the lower amount of bacteria required to diagnose UTI since it is more sterile and less likely to be contaminated

  • Suprapubic aspiration: ANY bacteria
  • Urinary catheterization: >50k CFU
  • Clean catch specimen: > 100k CFU
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105
Q

What is the BEST test and timing of test to diagnose chronic HCV in a newborn, born to a mother with untreated HCV?

A

HCV Ab at 18 months

  • Exp: Key is chronic infection. If HCV RNA is measured soon after birth and is positive, it may clear eventually, so positive test does NOT mean chronic Hep C. At 18 months, maternal HCV Ab will have cleared from baby’s system.
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106
Q

What is the perinatal transmission rate of Hep C from mother with untreated Hep C?

A

5% only!

TIP: Hep C as an adult will likely be chronic, less likely transmit to baby. Opposite to Hep B, which is less likely to be chronic, high likelihood of perinatal transmission.

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107
Q

In “adolescent idiopathic scoliosis”, what is the degree of curvature that all patients will need bracing or surgical intervention?

A

25%

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108
Q

Framework question:

What are the 2 most important factor in determining if an adolescent will need referral to Orthopedics for evaluation of scoliosis for treatment?

A
  • Skeletal maturity (Risser Score)
  • Degree of curvature (“Cobb angle”)
  • Exp: Skeletal maturity matters because patients that have yet to reach full skeletal maturity have more room to grow. It is during the growth phase that scoliosis worsens the most. If skeletal maturity is near, further changes are NOT expected. Degree of curvature predicts symptoms and morbidity.
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109
Q

What is the typical organism to cause septic joint/osteomyelitis in toddlers?

A

Kingella Kingae

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110
Q

Toddler with septic joint has aspiration performed, but no growth of fluid. What next step can help diagnose the bacterial cause?

A

PCR assay can help diagnose Kingella (and other organisms) that are difficult to culture

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111
Q

What are the characteristics of Treacher Collins Syndrome? (PICMONIC)

A
  • Small Ears, Large nose, Small mouth: Rat
  • Eye abnormalities/Vision abn (vision loss, amblyopia, refractive errors, strabismus): Binoculars
  • Pierre Robin Sequence: bird watching
  • Treacher: “Preacher”
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112
Q

Parent asks you: What will be some of the intellectual disabilities that my son, who has Treacher Collins, will have?

A

NONE!

Peds Hospitalists who had Treacher Collins Syndrome!

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113
Q

What is the pubertal sequence of events for boys vs girls?

A
  • Girls: Breast development → Pubic hair → Peak height velocity → Menarche.
  • Boys: Testicular enlargement → Pubic hair → Increased penile length → Peak height velocity.
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114
Q

When does puberty start for girls vs boys?

A

Puberty in girls begins between 8 and 13 years of age and in boys between 9 and 14 years of age.

TIP: 8 looks like breasts; 9 looks like testicle/penis

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115
Q

What is the diagnosis of delayed puberty for girls vs boys?

A

Puberty in girls begins between 8 and 13 years of age and in boys between 9 and 14 years of age.

So, for girls it is no puberty (Breast buds) > 13, boys is no puberty (no testicular enlargement) > 14

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116
Q

When does vaginal leukorhhea occur in regards to puberty?

A

Anytime within the year prior to menarche

TIP: think of the white discharge as the herald for the red discharge (menses)

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117
Q

Explain the physiology of Eisenmenger syndrome in patients with VSD?

A

Unrestricted VSD, that is, a VSD in which the pressure between the right and left ventricle is normalized, leads to increased blood flow from the left side of the heart to the right. This increased volume to the right side leads to pulmonary HTN and resistance. Eventually, right side pressure becomes greater than left such that shunt goes from right to left - blue blood goes to red blood.

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118
Q

What scenario does a patient presenting after a human bite require Tetanus IG?

A

Patient who has < 3 tetanus vaccinations OR unknown who comes in with a human bite needs TIG.

THIS IS THE ONLY SCENARIO IN HUMAN BITE THAT REQUIRES TIG

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119
Q

When does a patient presenting with a human bite require a tetanus vaccination?

A
  • < 3 tetanus vaccinations or unknown
  • > 3 tetanus vaccinations, BUT > 5 yo since you are nearing the time of booster which means immunity is waning (booster in general is needed between 4-7 years of age)
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120
Q

Differential diagnosis for a patient who develops a fever and hypotension in the middle of a transfusion?

A
  • Sepsis
  • Febrile hemolytic transfusion reaction
  • Febrile non-hemolytic transfusion reaction
  • Allergic reaction given hypotension

TIP: blood cultures, antibiotics and labs to evaluate for hemolysis

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121
Q

What is the toxidrome of clonidine toxicity?

A

Clonidine is a central alpha 2 agonist, working centrally to decrease the transmission of NE (think of ADHD) as well as decrease the sympathetic outflow peripherally.

Hence, central AND peripheral effects

  • Central: sedation
  • Peripheral: bradycardia, hypotension (as a result of decreased sympathetic outflow)
  • Respiratory: apenea, respiratory depression
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122
Q

What is the treatment of choice for children with OCD?

A
  • If mild, CBT

- If CBT is not sufficient, addition of SSRI is recommended

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123
Q

What are the FDA approved medications to treat pediatric OCD?

A
  • Fluoxetine
  • Sertraline
  • Fluvoxamine
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124
Q

What are some characteristic differences between OCD in children and adults?

A

The obsessions (or intrusive thoughts) for children are more harmful or violent than adults

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125
Q

What is the presentation of osteoid osteoma?

A

Localized pain at tibia or fibula ( 50% of the cases)

Not associated with activity

Responds well to NSAIDS

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126
Q

What is the main treatment for ostenopenia/osteoporosis due to anorexia nervosa?

A

Adequate caloric intake is the main stay

Secondary treatments include Vit D/Calcium, weight bearing exercises

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127
Q

How does CKD (and ESRD) in children affect their linear growth?

A

Decreased linear growth compared to normal children

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128
Q

How to treat impaired linear in children with CKD (and ESRD)?

A

Growth hormone replacement (before puberty)

INTERESTING (and sad): Short adult height in patients with end-stage renal disease is also associated with lower self esteem, decreased education, marital status, and decreased income.

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129
Q

What is the first line treatment for Guillain Barre: IVIG or Plamapharesis?

A

IVIG (AND Plasmapheresis). Both have similar efficacy. IVIG is more tolerable. And plasmapheresis is reserved for cases of severe respiratory failure

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130
Q

What are the classic characteristics of Miller Fisher Variant of Guillain Barre?

A

Triad of Ophthalmoplegia, Areflexia and Ataxia.

TIP: the ataxia and ophthalmoplegia are the key differences between GBS and Miller Fisher Variant

TIP: any CN OR brainstem involvement, think of Miller Fisher

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131
Q

Pediatric patient presents after a house fire sustaining severe burns.

1) How do you calculate the fluid deficit?
2) Over what time period should this fluid deficit be replaced?

A
  • Parkland formula: 4ml/kg x weight (in kg) x body surface area affected (%)

TIP: think of a park on fire.

  • First 24 hours
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132
Q

What is the way to calculate the surface area % affected in a burn victim?

A

“Rules of 9” Girl diagram

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133
Q

What is the total fluid requirement of a child who presents following severe burns?

A

Fluid deficits from burns + fluid maintenance for the day = fluid deficit for the first day

Parkland formula + Holliday Segar

4ml/kg x weight (kg) x surface area burned (%) + 100 x weight (kg) = total fluid needs of the first day

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134
Q

What is the relationship between postpartum depression and developmental delay?

A

Post partum depression is a risk factor for developmental delay

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135
Q

What are the milestones of a 2 mos old

A

PICMONIC or Mnemonic

  • Chest up in prone position
  • Holds hands together at midline; holds rattle
  • Coos
  • Social smiles

Picmonic: Imagine baby swan; prone; rattle between 2 hands; chin propped up and chest off the ground; smiling big to camera;

“2 is coo”

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136
Q

What is first line treatment for functional constipation in kids?

A

Polyethylene Glycol

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137
Q

What are signs that a child presenting with functional constipation may need disimpaction?

A

Fecal incontinence

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138
Q

What is the characteristic presentation of HSP?

A

Palpable Purpura
Abdominal Pain
Arthritis
Hematuria

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139
Q

What are the most common organs involved in HSP?

A

Skin (edema/palpable purpura) > Arthritis > GI > Renal

TIP: Though renal is the least common organ affected in the classic presentation. It is the one that warrants follow up

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140
Q

What are the possible renal manifestations of HSP?

Which require Nephrology co-management?

A
  • Renal involvement is seen in 30% to 50% of children and presents with varying severity including NO inolvement, microscopic hematuria, macroscopic hematuria, proteinuria, nephrotic syndrome, nephritic syndrome, and rapidly progressive glomerulonephritis

No involvement: monthly UA
Hematuria or non-nephrotic range proteinuria: weekly UA
The rest: Nephrology

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141
Q

What is the presentation of nasal foreign body?

A

Unilateral rhinitis, possible green-yellow discharge

Foul breath odor

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142
Q

What is the presentation of allergic rhinitis?

A

Characteristic physical examination findings include edematous, pale, or bluish nasal turbinates; allergic shiners; Dennie lines (folds under the eyes because of edema); and a transverse nasal crease.

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143
Q

What is the best screening test for Lesch-Nyhan Syndrome?

A

The best screening test for Lesch-Nyhan syndrome is a urinary urate-to-creatinine ratio, which will be greater than 2.0 in a child who is younger than 10 years of age

WATCHOUT: Hyperuricosuria and hyperuricemia (serum uric acid concentration >8 mg/dL [476 µmol/L]), while often present, are not sensitive or specific enough to confirm the diagnosis.

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144
Q

What is the biochemical defect in Lesch Nyhan? Inheritance?

A
  • Hypoxanthine-guanine phosphoribosyltransferase enzyme activity is reduced leading to excess uric acid that deposits
  • X Linked - hence, ONLY boys are affected; girls who are carriers are NOT affected
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145
Q

What is the treatment of uncomplicated sexually transmitted N. Gonorrhea?

A

The recommended treatment regimen for uncomplicated N gonorrhoeae infections of the cervix, urethra, and rectum is ceftriaxone 250 mg intramuscularly in a single dose (if unavailable, cefixime 400 mg orally as a single dose) PLUS azithromycin 1 g orally in a single dose or doxycycline 100 mg orally twice daily for 7 days

Reason is because of resistance

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146
Q

What is the difference between Indomethacin and Prostaglandin in regards to congenital heart disease?

A
  • They are the opposite - one is an NSAID the other is a prostaglandin
  • Prostaglandin OPENS the PDA is needed in ductal dependent pulmonary or systemic flow
  • Indomethacin closes the PDA
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147
Q

5 day old presents with cyanosis and tachypnea, but is comfortable. Start Prostaglandin. Why?

A
  • Patient has CHD that is ductal dependent, likely to pulmonary flow. At birth PDA was open so pulmonary flow was adequate, but as PDA closes, cyanosis occurs in the case of ductal dependent pulmonary flow
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148
Q

When does PDA close for term infants?

A
  • Functionally, the first 24 hours of birth

- Complete closure doesn’t occur until 3 weeks

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149
Q

What age group is at risk of severe rotavirus infection, that is, severe dehydration?

A
  • 6 mos to 2 years, hence the recommendation of RV vaccination from 2-6 mos of age.
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150
Q

Parent asks you: when should they start brushing their child’s teeth?

A

Brushing with fluoridated toothpaste should begin with first tooth eruption

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151
Q

Parent asks you: How much toothpaste does their child need?

A

< 3 yo: grain of rice size

> 3 yo: pea size

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152
Q

What is the AAP recommendation in regards to applying fluoride varnish?

A

The AAP recommends application of fluoride varnish every 3 to 6 months to both primary and permanent teeth starting at tooth eruption

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153
Q

What is the definition of acute liver failure?

A

Coagulopathy (INR > 1.5) and encephalopathy that occurs acutely (< 26 weeks)

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154
Q

In children, how is the definition of acute liver failure different from adults?

A

If INR > 4, diagnosis can be made without the presence of encephalopathy

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155
Q

What are the causes of ALF in children (across all age groups)?

A
  • 50%: unknown
  • Infectious: EBV, Adenovirus, Parvovirus, HSV are most common.
    • TIP: Hep A, B and C are uncommon causes in children
  • Toxins: Acetaminophen, phenytoin, valproic acid, isoniazid
  • Metabolic: Wilsons, galactosemia, tyrosinemia, urea cycle defects, and mitochondrial disorders
  • Autoimmune:
  • Ischemic: Budd Chiari Syndrome
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156
Q

Parents of a severely visually impaired child as you what type of training their child may need to make sure they can navigate school safely. What available training is there?

A

Orientation and mobility training is essential to ensure that children with visual impairment are able to navigate their environment safely and effectively

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157
Q

Definition of blindness in regards to visual acuity?

A

20/400 or worse when best corrected for visual acuityP

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158
Q

Parent asks you: what is a good resource to learn about the special learning needs of my visually impaired child. You respond:

A

These are outlined in the American Foundation for the Blind Expanded Core Curriculum

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159
Q

Differential diagnosis of neonate with decreased activity or level of consciousness

A

HYPOGLYCEMIA, early onset sepsis, seizure, metabolic disorder

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160
Q

What type of infections, if recurrent, help you distinguish between a primary immunodeficiency of the innate immune system vs the adaptive immune system?

A
  • Recurrent bacterial infections suggest innate immune system
  • Recurrent or atypical viral infections suggest adaptive immune system
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161
Q

Chronic Granulomatous Disease Picmonic

A
  • X linked: X ninja stars
  • Oxidative burst defect: bubbles of grandmas stuck with X marks the spot
  • Skin and lung infections: girl in bikini flying around with a jet pack, with staff popping bubbles
  • Staph aureus abscesses
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162
Q

What is the gold standard for testing for CGD?

A

Flow cytometry to access oxidative burst, achieved through testing of the Dihydroxy-rhodamine 123 reduction

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163
Q

What is the difference between hamartoma vs teratoma?

A

Hamartoma is a tumor made up of the cells normally found in the anatomic site where it is found. It is also always benign.

Teratoma is a tumor made up of cells that differentiate toward somatic cells (typical of adult or embryonic development) NOT normally found in the anatomic site where is is found. Can be malignant

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164
Q

What is the presentation of nevus sebacus?

A

Tan, hairless plaque with a velvety texture that was present at birth.

COOL PHYS: A nevus sebaceus is a hamartoma, composed of sebaceous and apocrine glands and epidermal elements, that is present in 0.3% of newborns. It will grow in puberty because androgens will have affect on the sebaceous glands as is the case in other parts of the body

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165
Q

What is the diagnosis of HHS?

A

TIP: Hyperglycemia and the absence of DKA labs

BG > 600 + OSM>320 + absence of DKA (pH >7.3, Bicarb > 15, little to no ketones)

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166
Q

What is the difference in treatment of HHS in regards to insulin therapy?

A

Insulin rate is lower: 0.05 u/kg/hour (instead of 0.1 u/kg/hour)

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167
Q

What scenario do most mumps outbreaks occur?

A

VACCINATED close quarters!

TIP: 2 dose mumps vaccination only confers 88% immunity

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168
Q

What is the approach to evaluating a child with concern for acute cerebellar ataxia?

A
  • Ruling out other dangerous causes of acute ataxia
    • Normal MRI
    • Normal Lumbar puncture
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169
Q

Difference between “acute cerebellitis” vs “acute cerebellar ataxia”?

A

“Acute cerebellitis” is more severe and associated with encephelopathy

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170
Q

What age group does acute cerebellar ataxia usually affect?

A

2-5 years old

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171
Q

What is the difference between non-typhoidal salmonella vs typhoidal salmonella?

A

Typhoidal salmonella: resource limited countries, fever and constitutional sx predominate some abdominal pain due to bacteremia

TYPHOID FEVER occurs in resource limited places

Nontyphoidal salmonella: US, gastroenteritis predominant presentation

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172
Q

What is scanning speech? What neurologically is affected in a patient who presents with scanning speech?

A
  • slow speech that emphasizes al the syllables, almost like slurred speech
  • Ataxia, hence cerebellar
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173
Q

Long QT syndrome and sensorineural hearing loss - what is the syndrome?

A

Jervell Lange Nielson Syndrome

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174
Q

What does the presence of reducing substances in the urine indicate?

A

Carbohydrate metabolism disorder

Explanation: Sugars or carbohydrates have the ability to reduce other substances e.g. cupric ions to cuprous ions. The presence of the reducing substances in the urine implies carbohydrates in the urine. The presence of carbohydrates in the urine implies excess carbohydrates. This implies carbohydrate metabolism disorder

TIP: so fatty chain oxidation disorders do NOT have reducing substances in the urine

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175
Q

Describe the rash and fever relationship/characteristic of roseola infantum

A

Rash comes AFTER the fever (when defervescence occurs)

  • Starts in the trunk and spreads to the face, neck and extremities; lesions are distinct, discrete and can be papular
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176
Q

Age of Roseola infection

A

6-15 months (TIP: same as febrile seizures)

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177
Q

What are the adverse effects of taking recombinant growth hormone for athletic performance?

A

Development of diabetes
Hypertension
Cardiomegaly

TIP: become the opposite of an athlete, someone with heart disease, HTN and diabetes

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178
Q

What is the transmission rate of syphilis in utero if mother is untreated?

A

Nearly 100%

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179
Q

What are the bone manifestations of congenital syphilis?

A

Osteochondritis and periostitis

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180
Q

Characteristic findings of Trisomy 13?

A
  • Overlapping fingers
  • Dysplastic nails
  • Rocker bottom feet
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181
Q

Prognosis of Trisomy 13?

A

Most die within 1 year
Median survival is 2 weeks
Cause of death usually due to renal anomalies or heart defects

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182
Q

Does adverse effect of a medication need to be reported to the FDA or the manufacturer of a drug?

A

No, even death

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183
Q

Empiric antibiotics for retropharyngeal abscess?

A

Penicillin + Beta Lactamase Inhibitor

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184
Q

What is the classic presentation of retropharyngeal abscess?

A

Triad: Torticollis, Fever and Odynophagia

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185
Q

What is the associated cardiac defect with Williams Syndrome?

A

Supravalvular aortic stenosis

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186
Q

What is the characteristic disposition of those with William Syndrome?

A

Strong expressive language skills, socially engaged, polite and very endearing

“Elfin appearance”

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187
Q

What are the most common cardiac anomalies associated with fetal alcohol syndrome?

A

VSD

TIP: Remember that alcohol ablation is used to treat HOCM. So, alcohol during fetal period is a natural “ablater” of the septum

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188
Q

Infant is born to Hep B Positive mother. What are the next steps and what are the recommendations for Hep B vaccination in this population?

A
  • At birth: HBIG and HBV vaccine
  • Then HBV at 1, 2, 6 months

TIP: DO NOT COUNT HBV at birth as one of his vaccines.

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189
Q

Preterm infant is born and weights 1.7 kg. HBV vaccine?

A

No, because seroconversion rate is low in infants < 2kg. So, no HBV at birth. But at 1 month of age, regardless of weight, seroconversion rate is similar to term infants

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190
Q

How is treatment of Ramsey Hunt Syndrome different from other herpes zoster infections ?

A

Acyclovir AND STEROIDS

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191
Q

What is the presentation of Ramsey Hunt Syndrome?

A

TIP: presentation is the sequelae of cranial nerve 7 and 8 involvement

  • Vesicular rash around and in the ear canal (oropharynx, soft palate and 2/3 of tongue)
  • Facial nerve palsy
  • Tinnitus, vertigo, hearing loss, ataxia
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192
Q

PICMONIC Ramsey Hunt Syndrome

A

Ramsey Hunt - King tut hunter shooting an arrow

  • Shooting arrow into a giant ear
  • Missing and hitting the bell located on the ear lobe
  • Standing on a cliff (CN 7)
  • Handcuffed to the ground (CN 8)
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193
Q

What is the disorder in which acute infection worsens psychiatric symptoms such as OCD?

A

PANDAS (Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal infections)

COOL PHYS: Autoimmune anti streptococcal antibodies cross the BBB and cross react with tissue and affect caudate, putamen and pre-frontal cortex

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194
Q

Treatment of C1 esterase inhibitor deficiency associated with Angioedema?

A

Danazol increases the level of C1 esterase inhibitor

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195
Q

How does daycare attendance affect with antibiotic resistance?

A

Increases it in Strep Pneumoniae

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196
Q

What is the most common gene defect known to cause hereditary pancreatitis?

A

PRSS1 gene

TIP: Think press the pancreas

Cool phys: Gene mutation leads to defective trypsinogen that prematurely converts to trypsin while still in the pancreas and causes inflammation

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197
Q

What is the duration of immunosuppressive therapy in renal transplant patients?

A

As long as the graft is there, immunosuppresive therapy will be required

TIP: LIFELONG

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198
Q

What accounts for the difference between living and deceased donor renal transplant survival?

A

The deceased donor has longer cold ischemia time. So reperfusion injury is more common

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199
Q

Initial imaging test in cases of choledocolithiasis?

A

Abdominal U/S

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200
Q

What visual acuity, if present, warrants further evaluation prior to clearance for sports?

A

Corrected visual acuity worse than 20/40

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201
Q

Do Down Syndrome patients require radiographs of spine prior to participating in sports?

A

Although AAI has been reported in up to 27% of individuals with DS, only 1% to 2% experience symptoms. Best evidence indicates that identification of AAI on cervical spine radiographs in asymptomatic DS patients does not predict future injury in those who participate in unrestricted sports activity. AAP does not recommend screening cervical spine radiographs in these athletes if they are asymptomatic and have normal neurologic examination findings.

Interesting: Special Olympics requires radiographs

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202
Q

Pediatric patient has moderate pain post-op. Morphine vs tramadol?

A

Tramadol and Codeine are NOT recommended given variable metabolism and concern for undertreatment of pain.

Morphine is preferred

In 2012, the World Health Organization published the Guidelines on the Pharmacologic Treatment of Persisting Pain in Children with Medical Illnesses

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203
Q

What is the leading cause of morbidity and mortality in drowning?

A

Cerebral hypoxia

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204
Q

What is the best way to diagnose West Nile Virus meningoencephalitis?

A

Serology (IgM/IgG) of BOTH the CSF and blood

TIP: Though called WNV, it is common all over the US

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205
Q

Where is West Nile Virus common?

A

Most US states, with the highest numbers seen in California, North Dakota, South Dakota, Oklahoma, Colorado, and Nebraska

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206
Q

How does the presence of viral symptoms in the setting of pharyngitis affect the likelihood that a patient has strep pharyngitis?

A

Viral symptoms such as conjunctivitis, cough, coryza make the likelihood of Group A Strep pharyngitis less likely.

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207
Q

In a patient with hypercarbic respiratory failure from opioid overdose, he is also found to be hypoxic. Explain how?

A

Simply, how is hypercarbia accompanied by hypoxia in hypopnea?

  • Decreased air intake leads to decrease O2 in alveoli
  • Decreased ventilation leads to alveolar collapse, which leads to intrapulmonary shunting (pulm blood flow through collapsed alveoli)
  • As CO2 increases in the alveoli, O2 decreases, as consistent with the ideal gas law that PV= nRT. P is total pressure exerted by the sum of the partial pressure of each molecule. In order for P remains the same, if partial pressure of CO2 increases in hypercapnia, then partial pressure of O2 must decrease.
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208
Q

How common is it for a patient with ADHD to have co-morbid illnesses? What are some co-morbid illnesses?

A

75%! It is the rule, not the exception

  • Anxiety, depression, speech/language disorder, autism spectrum disorder, learning disability, oppositional defiant disorder, and tic disorders
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209
Q

Which ADHD meds are associated with high blood pressure?

A

Stimulant meds such as amphetamine/methylphenidate and Atomoxetine

TIP: NE reuptake inhibitor, hence more NE, vasoconstrictor

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210
Q

What children is it recommended to obtain a chromosomal microarray?

A

All children with developmental delay, multiple congenital anomalies or intellectual disability of unknown etiology

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211
Q

Wolf Hirshhorn Syndrome PICMONIC? Chromosomal abnormality?

A

4 p deletion: Sail boat
- picmonic for “P”: pearl
- sailing toward an island with pearls and clams
Facies: wolve with a “greek helmet”, small ears with large holes (like african tribes)
- Greek helmet nose
- Ear pits
Antibody deficiencies (69%) - Zangief tied to the mast by kidneys and ureters
cardiac defects (50%) - Wolf with spear pierces piercing heart/brain
urinary tract anomalies (30%) -
central nervous system malformations (80%) -

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212
Q

What infants require screening for ROP?

A
  • Born 30 weeks or less
  • Born less than 1500 gm

COOL PHYS: abnormal growth of the retinal blood vessels sometimes INTO the vitreous and causing retinal detachment. Hence laser for photocoagulation to stop vessel growth

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213
Q

What is the difference between the timing of the pain from Mittelschmerz vs Primary dysmenorrhea?

A

Mittelschmerz occurs from ovulation, hence 1-2 weeks BEFORE menses and should ONLY last 1 day the most

Primary dysmenorrhea occurs right before menses and into menses

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214
Q

What is an abnormal congenital heart screen with pulse ox for a newborn?

A
  • O2 sat < 90% x 1 - DUH

- 90-95% OR greater than 3% difference between right foot and right hand x 3

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215
Q

How common is renal involvement in SLE?

A

2/3

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216
Q

What is the difference in the presentation of “jerk nystagmus” and “pendular nystagmus”?

A
  • Jerk nystagmus involves a slow phase followed by fast phase.
  • Pendular nystagmus is a slow sinusoidal oscillation
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217
Q

What is the presentation of Spasmus Nutans?

A

Spasmus nutans is a childhood condition characterized by a clinical triad of dysconjugate pendular nystagmus, torticollis, and head bobbing (this is the “spasms”)

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218
Q

Key difference between congenital nystagmus vs spasmus nutans?

A

Congenital nystagmus present at birth, but usually diagnosed at 3 mos of age

Spasmus nutans diagnosed between 6 mos to 1 year of age

They are both on the differential for infants with nystagmus

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219
Q

What are clues from urine electrolytes and urinalysis with microscopy that implies ATN?

A
  • SG < 1.020 - tubular dysfunction
  • epithelial casts - tubular dysfunction
  • FeNA> 2% - tubular dysfunction
  • Na > 20
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220
Q

First line options of GER in infants (pharmacological)?

A
  • Thicken feeds and avoid overfeeding

- If persistent, can trial hydrolyzed formula or have mother eliminate dairy from her diet (if breastfeeding)

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221
Q

Patient presents after having over extended a finger during football practice. How to assess the functionality of the flexor digitorum profundus and flexor digitorum superficialis?

A

TIP: flexion of the fingers occurs through these 2 tendons

Test flexor digitorum profundus: hold the middle phalanx of the suspected injured finger to prevent PIP from flexing; ask patient to flex so to test flexion of the DIP

Test flexor digitorum superficialis: Stabalize the 2 fingers flanking the suspected injured finger, ask patient to flex the injured finger to test flexion of the PIP and MCP

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222
Q

What type of tendon injury of the hand requires an urgent referral to hand specialist?

A

Tendon tear as noted by digitorum profundus and flexor digitorum superficialis on exam

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223
Q

What are the characteristics of a Still’s Murmur?

A

A Still murmur is a classic innocent murmur typically described as a low-pitched, musical, 2/6 systolic murmur heard best at the lower left sternal border that changes with position and is not heard during the Valsalva maneuver.

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224
Q

What are scenarios in which minors are emancipated?

A
  • Married
  • Joined the military and are active
  • Judge granted
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225
Q

Difference between consent and assent?

A

Assent is an interactive and ongoing process between a child and a clinician wherein developmentally relevant information is disclosed about a particular intervention. During the assent process, the child is engaged and his or her input is sought. The goal of assent is to protect children’s rights by allowing them to voice their preferences when it is appropriate to do so.

TIP: Assent is like consent, but developmentally appropriate for the age.

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226
Q

Diagnosis of Kawasaki’s Disease?

A

“5,4,3,2,1” Rule

5 days of fever
4/5 symptoms:
- B/l conjunctivitis, non-purulent
- Oral findings: red tongue, chapped lips
- Lymphadenopathy > 1.5cm
- Hands/Feet: Desquamating rash, edema
- Diffuse rash described as maculopapular or erythoderma
3 supplementary labs: Leukocytosis, Anemia, Thrombocytosis, AST/ALT, hypoalbuminemia, leukouria
2 inflammatory markers: CRP/ESR
1 ECHO finding

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227
Q

Treatment of Kawasaki’s?

A

High dose ASA (100mg/kg)

IVIG 2gm/kg

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228
Q

Skin infection from Pseudomonas - What is it called? Presentation?

A

Ecythema Grangenosum

Painless, necrotic, ulcerative cutaneous lesion with a gray-black eschar with surrounding redness.

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229
Q

By what age are feeds in the middle of the night unneccessary for infants?

A

6 months

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230
Q

Describe the extinction technique and how that may reduce the amount of night time awakenings an infant may have

A
  • Put the baby to bed when awake, but drowsy
  • Nightly, wait progressively longer times prior to checking up on infant (extend by 10 minutes/night)
  • When checking on child, limit to 1-2 minutes and soothe by patting, NOT carrying
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231
Q

What house hold items have methanol?

A

Winshield wiper fluid
Household solvents (TIP: solvents are substances that dissolve other things)
Perfumes
Cooking fuels

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232
Q

How does fomepizole treat methanol toxicity?

A

It competitively inhibits alcohol dehydrogenase

Alcohol dehydrogenase metabolizes methanol into formaldehyde, which later metabolizes into FORMIC acid. This is the toxic metabolite

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233
Q

Antibiotic of choice for neutropenic fever?

A

Cefepime

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234
Q

When do urea cycle disorders present?

A

Rapid decompensation first 24-72 hours of birth

Neonatal period. Hence, Urea cycle defect should be on the diff/diag in a neonate who decompensates

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235
Q

What labs are indicative of a urea cycle disorder?

A
  • Hyperammonemia
  • Progressive respiratory alkalosis due to the above
  • NAGMA
  • Normal glucose

COOL PHYS: Urea cycle is the cycle by which proteins are broken down. Nitrogen is broken down to urea that is excreted in the urine. Build up of nitrogen leads to accumulation in the form of ammonia, hence hyperammonemia implies a protein metabolism defect, hence urea cycle disorder

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236
Q

What is the inheritance pattern of OTC?

A

X linked

TIP: this is the ONLY urea cycle disorder that is NOT recessive. ALL are recessive.

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237
Q

What labs can be ordered to help distinguish between the urea cycle disorders?

A

Urine Orotic acid and plasma amino acids

Urine orotic acid, if high, indicates OTC
Plasma amino acids demonstrate which AA are not being metabolized appropriately

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238
Q

Newborn with congenital toxoplasmosis is asymptomatic. Treat?

A

Yes, 3 months of Pyrimethamine, Sulfadiazine and Folinic acid

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239
Q

Can healthy children present with severe Toxoplasmosis infection?

A

Yes, no need to be immunocompromised

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240
Q

Patient is diagosed with Toxoplasmosis but is asymptomatic. No need for treatment. When will patient need prophylaxis?

A

Whenever patient becomes immunocompromised. For example, new diagnosis of leukemia.

Reason: Acute toxoplasmosis is due to reactivation of previous infection instead of new primary infection

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241
Q

What ethnicity places a patient at risk for human trafficking?

A

American Indian

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242
Q

What populations do AAP recommend the quadravalent meningococcal conjugate vaccine?

A

American Academy of Pediatrics (AAP) approved updated recommendations for the use of quadrivalent (serogroups A, C, W-135, and Y) meningococcal conjugate vaccines in adolescents and in people at persistently high risk of meningococcal disease (e.g. asplenia, complement deficiency)

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243
Q

What is the recommended vaccination schedule for normal patients in regards to the vaccination against meningococcus?

A

11-12, then booster at age 16 (right before college!)

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244
Q

How does idiopathic pulmonary hemorrhage present?

A

Repeat episodes of pulmonary infiltrates associated wtih anemia and hemoptysis

PHYS: milk allergy possibly

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245
Q

Framework Question: How to think or characterize Spinal Muscular Atrophy to better make sense of the presentation?

A

Spinal Muscular Atrophy

“Muscular atrophy” occurs because of “spinal” cause, that is, neurodegeneration of the anterior horn cells.

Hence, presentation is ALL lower motor neuron: areflexia, hypotonia, flaccid paralysis, fasciculations

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246
Q

Difference between SMA 1, 2, 3

A

SMA 1: diagnosed < 6 mos, never sits
SMA 2: diagnosed between 6-18 months, sits, but never walks
SMA 3: Diagnosed > 18 mos, walks, but falls

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247
Q

Describe how the presence of the XX chromosome vs the XY chromosome leads to female and male genitalia respectively.

A

In the process of normal sex differentiation, the gonads and internal and external structures begin as bipotential. The sex determining region (SRY) and other genes on the Y chromosome are responsible for gonadal differentiation into a testis.

TIP: srY is located on the Y chromosome

In the presence of SRY gene, testicular Leydig cells secrete testosterone and Sertoli cells secrete anti-Müllerian hormone (AMH). The testosterone stabilizes the Wolffian ducts, which become the epididymis, vas deferens, seminal vesicles, and ejaculatory ducts. Anti-Müllerian hormone causes the Müllerian structures to regress. The Müllerian structures are the uterus, fallopian tubes, cervix, and upper vagina. Testosterone is converted to the more potent androgen, DHT, by 5α-reductase. Dihydrotestosterone is responsible for virilizing the external genitalia, causing growth of the phallus, fusion of the labioscrotal folds, and migration of the urethra to its normal position in the penis.

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248
Q

What hormone is responsible for virilization of genitalia?

A

Testosterone is converted to the more potent androgen, DHT, by 5α-reductase. Dihydrotestosterone is responsible for virilizing the external genitalia, causing growth of the phallus, fusion of the labioscrotal folds, and migration of the urethra to its normal position in the penis

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249
Q

What physical exam findings on a newborn is highly indicative of XY chromosomes?

A

Palpable gonads

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250
Q

During what part of resuscitation (PALS) should access be obtained?

A

First 2 minutes of CPR - AKA the first round of CPR. If no IV, then move to IO.

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251
Q

CPR being performed: what is the fastest way of obtaining access - PIV, IO or Central line?

A

IO

” In a large meta-analysis, the IO route achieved more frequent successful access and improved time to achieving access as compared to peripheral and central venous techniques in patients requiring emergent vascular access in the emergency department.”

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252
Q

What physical exam finding is consistent with hepatomegaly in neonate? Children?

A

> 3.5 cm liver edge below the right costal margin

> 2cm in children

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253
Q

Patient with hip pain. What type of radiographs to order?

A

Anteroposterior view of the pelvis and a “frog leg” lateral view of the hip.

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254
Q

Radiograph demonstrates Legg Calve Perthes disease. Next step?

A

Crutches and non-weight bearing, referral to Ortho. The key is to maintain the femoral head in the acetabulum. In order to do so, non-weight bearing to keep from persistent strain

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255
Q

Explain to a patient what an advanced directive is.

A

Advance directives, also known as “living wills”, outline patient’s wishes for medical treatment in event of lost decision-making capacity.

This does include end of life care, but is not limited to that.

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256
Q

At what age is DEET safe for a child?

A

2 months! Super early!

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257
Q

What percent DEET is sufficient?

A

30% is sufficient. More than that yields no added benefit.

TIP: protection from BOTH ticks and mosquitoes! Didn’t know that!

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258
Q

What is the definition of “elevated blood pressure” vs “Stage 1 HTN” vs “Stage 2 HTN” in pediatrics?

A

The updated definition and stages of hypertension are:

  • normal BP (< 90th percentile)
  • elevated BP (≥ 90th percentile to < 95th percentile);
  • stage 1 hypertension (≥ 95th percentile to <95th percentile + 12 mm Hg)
  • stage 2 hypertension (≥ 95th percentile + 12 mm Hg).
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259
Q

What is “dual protection” in regards to contraception?

A

Condoms + another method of contraception to prevent BOTH pregnancy and STI’s

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260
Q

What amount of cow’s milk intake places toddlers at risk for IDA?

A

> 32 ounces of milk

COOL PHYS:

  • Low BA of iron
  • The induction of an exudative enteropathy with associated mucosal injury and persistent enteric blood loss not associated with gross bleeding
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261
Q

Explain expected RDW and TIBC in IDA

A

The RDW is a measure of the variability of red blood cell volume. As the production of hemoglobin becomes more unreliable when iron levels fall, the variability of red blood cell sizes increases, as represented by an increased RDW. The TIBC is a measure of the blood’s capacity to bind iron with transferrin. As the iron concentration falls, the capacity to bind iron increases, and thus the TIBC increases.

RDW - red cell distribution width

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262
Q

What is the treatment of choice for JIA with low disease activity?

A

NSAID!

TIP: few rheumatologic diseases can be treated with NSAIDS alone

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263
Q

What is the diagnosis of JIA?

A

“4 + 5 + 6 ~ 16” Rule

< 16 years old - “Juvenile”
4 Joints = oligo
5 joints = poly
> 6 weeks of persistent sx

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264
Q

What is the reccommeded treatment of JIA with moderate to severe disease?

A

MTX

TIP: Just like RA! Used to be called JRA.

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265
Q

What is the difference between phimosis and paraphimosis?

A

Phimosis is the inability to retract the foreskin
Paraphimosis is the opposite, that is, after retraction, you can’t reduce the foreskin
- Paraphimosis is an EMERGENCY due to ischemia

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266
Q

By what age will most phimosis resolve for most boys?

A

90% by age 3

99% by adolescence

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267
Q

How do disorders of sex development present in infancy? (DSD) AKA What presentations should suggest an underlying DSD?

A

DSD presents in 2 ways:

1) Ambiguous genitalia - neither fully male in appearance nor fully female in appearance
2) Discordant genotype/phenotype sex:
- Ex: XY prenatally, but undervirilized genitalia at birth (micropenis)
- Ex: XX prenatally, but virilized genitalia (clitoromegaly)

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268
Q

Framework question:

What is considered disorders of sex development (DSD)?

A

A group of congenital conditions in which the development of chromosome, gonad or anatomic sex (external genitalia) is atypical. And any combination of the three.

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269
Q

Framework question:

What are broad ways to categorize DSD?

A

Categorize by the chromosomes:

  • 46 XX DSD - females with virilization
  • 46 XY DSD - males with undervirilization
  • DSD with sex chromosome abnormalities - e.g. Klinefelter in which XXY w/ undervirilization
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270
Q

What type of temperament is a risk factor for anxiety disorder in children?

A

Behavioral inhibition

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271
Q

What does MELAS stand for?

A

Mitochondrial Encephalomyopathy Lactic Acidosis with Stroke-like symptoms

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272
Q

Framework Question:

What organs tend to be involved in mitochondrial disorders?

A

Organs highly dependent on aerobic metabolism:

  • Heart
  • Muscles
  • Nervous system
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273
Q

What is the inheritance pattern of mitochondrial disorders?

A

“Maternal inheritance” which means the disorder is transmitted from the mother. This is the case since ALL mitochondrial DNA comes from mothers.

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274
Q

Framework Question:

Why is the presentation of MELAS extremely variable?

A

A woman can transmit a variable amount of mutated mtDNA to each of her offspring, thus yielding significant clinical variability among her children, the “bottleneck effect.” In addition, tissue distribution can vary among organ systems, a concept known as “heteroplasmy.” Because of heteroplasmy, a mtDNA pathogenic variant may not be detectable in leukocytes, thus warranting molecular genetic testing of mtDNA in other tissues, such as cultured skin fibroblasts or skeletal muscle for diagnosis.

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275
Q

Menke Syndrome PICMONIC

A
  • Copper transport defect: Copper monkey riding a copper roller coaster ride
  • Kinky, twisted hair - Corkscrew looking hair
  • Neurologic/Connective tissue/Bone manifestations are the most commonly affected organs
  • Neurologic manifestations include seizures
  • Connective tissue defects include saggy skin and abnormal blood vessels
  • Bone manifestations include: osteopenia, long bone fractures
  • X linked
  • Death by 3 years of age

Copper monkey with kinky hair in a transporter, bowling with a clear ball with brain inside, aiming at skeleton bones (instead of bowling pins) and X marks the spot for all the bones

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276
Q

Child exposed to patient with proven measles. Next step in management?

A

Depends if they are fully vaccinated. If < 2 vaccines, then administer MMR vaccine within 72 hours

TIP: HIGHLY contagious, that is, 90% of exposed patients will contract measles

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277
Q

When is MMR vaccination recommended?

A

The first dose of MMR is recommended at 12 to 15 months of age, and the second dose is recommended between 4 to 6 years of age.

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278
Q

Newborn is born with no prenatal care and gestational age is unknown. What are physical exam findings consistent with late pre term (34-36 weeks)?

A
  • Breast development will show a stippled areola with 1- to 2-mm breast bud
  • Movements of the arms may be characterized as jerky but not irregular
  • They may exhibit periodic breathing. However, they should not have periods of apnea (cessation of breathing for 20 seconds)
  • Their ear cartilage should be soft with instant recoil
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279
Q

What are some associated risk factors for the development of persistent asthma?

A
  • Obesity
  • Vitamin D deficiency
  • Allergies
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280
Q

Most common type of presentation for Bacillus Anthracis?

A

Cutaneous anthrax

TIP: Pulmonary anthrax has a mortality rate as high as 90%. But this only occurs if bacillus anthracis is aerosolized, something that occurs in bioterrorism

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281
Q

Neonate with severe pulmonic stenosis. Next step?

A
  • Neonates with severe pulmonary stenosis should urgently undergo balloon valvuloplasty, and therefore be transferred as soon as possible to a center that performs such procedures regularly. With severe pulmonary stenosis, the right ventricle can ultimately fail because of the increased work needed for pulmonary blood flow in the context of a high pressure gradient.
  • If O2 sat is abnormal, prostaglandin infusion to keep PDA open
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282
Q

Vitamin deficiency associated with biliary atresia?

A

ADEK given abnormal biliary flow

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283
Q

Vitamin deficiency associated with biliary atresia?

A

ADEK given abnormal biliary flow

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284
Q

Framework question:

What is the difference between minimal, moderate, deep sedation and general anesthesia?

A

The level of sedation is characterized by patient’s ability to respond, the effect of the medication on the respiratory/circulatory system/airway.

E.g.

  • Moderate sedation: purposeful response to verbal commands with or without gentle stimulation, maintenance of cardiovascular function, spontaneous ventilation, and no required intervention for airway maintenance
  • Deep sedation: INTUBATED; interventions may be required to maintain the airway and ventilation, cardiovascular function is usually maintained, and purposeful response will occur after repeated stimulation.
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285
Q

When is insulin recommended for youth’s with Type II DM?

A

The American Diabetes Association recommends that insulin should be initiated at diagnosis for youth with type 2 diabetes if the blood glucose is greater than or equal to 250 mg/dL (13.9 mmol/L) or hemoglobin A1c is greater than or equal to 8.5%

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286
Q

Framework Question:

What is the progression of the understanding of rules/right/wrong for toddlers/preschoolers to early school age children to to school age children?

A
  • 2-4 year olds do not have a concept of rules and ownership
  • early school age: understand wrong though they have difficulty fighting the impulse to steal
  • school age children: around 8 develop a conscience and aware of rules/expectations
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287
Q

At what size does an ovarian cyst require gynecological consultation?

A

> 6cm

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288
Q

What is the cause of a “simple ovarian cyst”?

A

Simple cysts result from mature ovarian follicles that did not ovulate and failed to involute.

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289
Q

What is the difference between a “Simple Ovarian Cyst” and a “Luteal Cyst”?

A
  • Simple cysts come from mature follicles that fail to ovulate and fail to involute thereafter. Luteal cysts come from follicles that ovulate, but fail to involute.
  • Can only be distinguished by US. Luteal cysts has “echoes”
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290
Q

What signs should suggest the presence of shock in a child (not including hypotension)?

A

Shock should be suspected with tachycardia and poor perfusion even without hypotension, as it is often a late finding.

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291
Q

What is the recommended fluid resuscitation for adults in septic shock? Children?

A
  • Adults: 30ml/kg within the first 3 hours

- Children: 20ml/kg boluses, repeat up to 60ml/kg

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292
Q

After what period of time does a newborn’s creatinine, as obtained by lab, reflect his/her normal creatinine and not that of his/her mother?

A

2 weeks

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293
Q

Framework Question:

What age do children reach adult levels of GFR?

A

2 years old

TIP: 2 year old > term infant > preterm infant GFR (full renal embriogenesis occurs at 35 weeks, so a 32 weeker hasn’t even fully developed his kidneys). Hence, GFR is lower at younger gestation. And lower at birth.

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294
Q

Why are children/adolescents at higher risk of hypercalcemia due to immobility compared to adults?

A

Children and adolescents are at higher risk than adults for hypercalcemia during immobilization because of the high bone turnover state normally seen in these age groups.

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295
Q

When is it usually recommended that solid organ transplant patients receive their vaccinations - pre or post transplant?

A

Although pediatric SOT candidates may have a decreased immune response to vaccines compared with healthy children, they are more likely to develop vaccine-induced immunity than SOT recipients receiving post-transplant immunosuppressive agents.

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296
Q

Patient is 7 years old and will be receiving a renal transplant soon. He has NOT received any PCV13 or PPSV23. What vaccinations should he receive?

A

In children aged 6 through 18 years at high risk of pneumococcal disease who have not received any doses of PCV13 or PPSV23, administration of 1 dose of PCV13 followed by 1 dose of PPSV23 at least 8 weeks later and a second dose of PPSV23 5 years after the first is recommended.

TIP: PCV 13 ALWAYS comes before PPSV23

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297
Q

What is the prevalence of reflux in preterm infants?

A

UNIVERSAL

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298
Q

What is the recommended agent to thicken formula?

A

Oatmeal since rice cereal carries the risk of Arsenic poisoning

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299
Q

What is the next step of treatment for preterm infant with persistent GER despite non-pharm techniques?

A

Extensively hydrolyzed formula or thicken feeds with oatmeal

PHYS: reduced transit time

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300
Q

What is the treatment of choice for Klinefelter patients with for their hypogonad state?

A

Testosterone replacement

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301
Q

When is mefloquine contraindicated?

A

In individuals with active or recent history of a major psychiatric disorder, including depression, anxiety, psychosis, or schizophrenia, because use of the drug can lead to psychoses and other psychiatric symptoms.

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302
Q

When is primaquine contraindicated?

A

It should not be used in individuals with glucose-6-phosphate dehydrogenase deficiency because it can cause hemolytic anemia.

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303
Q

When is Atovaquone-proguanil contraindicated?

A

Breast feeding mothers and renal dysfunction

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304
Q

What are alternative prophylaxis for Plasmodium Falciparum if chloroquine is not available or resistance is a concern?

A

Atovaquone-proguanil
Doxycycline
Mefloquine

TIP: Like chloroQUINE, they have similar endings except Atovaquone

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305
Q

What is the recurrence of congenital diaphragmatic hernia in patients with CDH s/p repair?

A

Up to 50%

Hence, recommendation of yearly CXR until 16

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306
Q

How many doses of DTaP should a child receive before age 7?

A

5: 2,4,6 mos, 15-18 mos, then 4-6 yo

Then Tdap at 11-12 yo

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307
Q

Pediatric patient is getting catch up vaccines for TDaP. What is the interval between his first TDaP and second one?

A

4 weeks

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308
Q

What are the clinical criteria to help distinguish a viral sinusitis vs a bacterial sinusitis?

A

Any of the 3:

1) > 10 days of symptoms
2) Worsening of symptoms after an initial improvement of symptoms
3) 3 days of severe symptoms at the onset of illness

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309
Q

What is Posterior Urethral Valve?

A

Posterior urethral valve is a condition caused by obstructing membranous folds in the posterior urethra

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310
Q

Framework Question:

How does obstructive uropathy present depending on the level of obstruction?

A

PUV: Bilateral hydroureteronephrosis
UVJ: Unilateral hydroureteronephrosis
UPJ: Unilateral hydronephrosis with normal ureter dilation

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311
Q

What is the definition of status epilepticus?

A

> 5 minutes of seizure activity

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312
Q

What is first line treatment for status epilepticus?

A

Ativan: 0.1mg/kg IV (max: 4mg)
Midazolam: 0.2 mg/kg IM (max: 10mg)
Diazepam: 10mg rectal or 0.1mg/kg IV (max: 10mg)

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313
Q

Framework question:

What nutritional deficiencies are affected depending on the part of bowel removed?

A

Proximal small bowel: iron, Vit D and Folate

Distal small bowel (ileum): B12, bile salt absorption

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314
Q

What is the recommended post-exposure prophylaxis for rabies?

A
  • Rabies immunoglobulin and rabies vaccine on day 0

- Rabies vaccine on day 3, 7, 14

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315
Q

Interesting presentation of rabies

A

Ascending paralysis

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316
Q

What animals, if bitten by, are considered rabid?

A

Foxes, bats, skunks, racoons and wood chucks

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317
Q

Most common cause of hyperthyroidism in adolescents?

A

Graves disease

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318
Q

What antibodies are associated with Graves vs Hashimoto’s?

A

Graves: Thyrotropin Ab (that is, Ab to the TSH receptor)

Hashimoto’s: Anti TPO Ab

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319
Q

What is the relationship between HCG and hyperthyroidism?

A

HCG is similar to TSH, hence, can bind to the TSH receptor and cause hyperthyroidism E.g. molar pregnancy.

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320
Q

Can inhaled corticosteroids lead to long term effects on linear growth?

A

Inhaled corticosteroids have no adverse effect on long-term growth in children. However, poorly controlled asthma can affect growth, as can many other factors.

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321
Q

How to diagnosed head lice?

A

Gold standard: Head lice infestation is diagnosed by finding live lice on the scalp or eggs attached to hairs that likely are viable (darker in color and located ≤ 1 cm from the scalp)

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322
Q

What are findings on PE of successfully treated head lice?

A
No lice 
White eggs (empty eggs) > 1 cm away from scalp

COOL: Eggs are attached to hairs (within 4 mm of the scalp). Viable eggs are pigmented to match the color of surrounding hair. In contrast, empty eggs (ie, those that have hatched) appear white. Eggs are incubated by the host’s body heat and hatch in 8 to 9 days (range, 7-12 days). Based on this information and the knowledge that scalp hair grows approximately 2.5 mm/wk, eggs located more than 1 cm from the scalp likely have hatched.

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323
Q

What is first line therapy for head lice?

A

1% Permethrin on day 1 then day 10

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324
Q

Adolescent shares that he is smoking E-cigarettes because it is safer than cigarettes. Your response?

A

Electronic-cigarette use is associated with an increased risk of cigarette smoking. Meta-analysis has confirmed that e-cigarette use is associated with an increased risk of cigarette smoking.

The use of tobacco and nicotine-containing products is also associated with an increased risk of using drugs like marijuana

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325
Q

What is a “Burner” or “Stinger”?

A

These are neuropraxic injuries to the upper trunk of the brachial plexus due to traction or compression commonly occurring in football players

Unilateral numbness or weakness may also develop

326
Q

What are concerning signs that a “Burner” or “Stinger” has involved injury to the cervical spinal cord?

A

Significant pain with active neck motion or midline tenderness over the cervical spine should prompt further evaluation, and bilateral upper extremity or any lower extremity involvement must be considered a cervical spine injury until proven otherwise

327
Q

What is the recommended treatment for human bite? Penicillin allergic?

A

Augmentin

Clinda + TMP-SMX
Cinda + 3rd generation cephalosporin

328
Q

Most common cause of an adrenal mass in the newborn/fetus?

A

Neuroblastoma or adrenal hemorrhage

329
Q

Screening test for patient concerning for neuroblastoma?

A

Because neuroblastoma is a tumor of the sympathetic nervous system, the catecholamine metabolites VMA and HVA are detectable in the urine of approximately 90% of patients with neuroblastoma

330
Q

Child presents for abnormal movements and syncope. At what age do most breath holding spells occur?

A

< 18 mos, 90% of breath holding spells occur

  • On average, they can occur between 6 mos and 4 years of age
331
Q

What are clinical clues from the history that are characteristic of breath holding spell vs seizures?

A

Breath holding spells are characterized by strong emotional event prior to episode and color change (pallid or cyanotic)

332
Q

What is the associated underlying disease to breath holding spells?

A

IDA

333
Q

What is aneuploidy?

A

Aneuploidy is the presence of an abnormal number of chromosomes in a cell, for example a human cell having 45 or 47 chromosomes instead of the usual 46

334
Q

What is the main purpose of maternal noninvasice cell-free fetal DNA testing?

A

First of all, this is fetal cells detected in maternal serum.

Main purpose is SCREENING for aneuploidies (abnormal NUMBER of chromosomes). (Even though it has HIGH sens/specificity)

Means 2 things:

1) NOT diagnostic, so any abnormal findings need confirmation - amniocentesis or chorionic villus sampling
2) Does NOT eval for microdeletions, microduplications, translocations or gene mutations

335
Q

What are the 4 markers measured in a maternal quadruple screen?

A
  • HCG
  • AFP
  • Inhibin A
  • Estriol
336
Q

Detection rates for T21, 18 and 13 are 99%, 97% and 91% by cfDNA. Why does it need to be confirmed with invasive testing and not considered diagnostic?

A

The chromosomal makeup of the placenta is typically identical to that of the fetus; however, sometimes a mutation is confined to the placenta and the fetus has a normal karyotype. Placental-fetal discordance can occur in 1% to 2% of cases and is known as confined placental mosaicism.

Other reasons for placental-fetal discordance in cfDNA include a vanishing twin, chromosomal anomalies in the mother, or chromosomal anomalies resulting from a maternal malignancy.

Therefore, an abnormal cfDNA result always warrants confirmation with a diagnostic test such as a fetal karyotype or prenatal microarray, especially before considering termination of a pregnancy.

337
Q

Treatment for oral thrush?

A

Topical antifungals including nystatin, clotrimazole, or miconazole

338
Q

What is the direction of fluid shifts (ECF and ICF) in hyponatremic states?

A

Sodium, as evident from the osmolarity formula, has the maximal osmolar contribution and is important to maintain the ECF volume. Low serum sodium (hyponatremia) mostly equates to hypo-osmolality and indicates relative excess of water as compared to sodium in the ECF. To balance the osmoles, water moves from ECF to ICF in hyponatremic conditions

339
Q

What restrictions are there in regards to the flu vaccine for children with severe egg allergies?

A

Children who have experienced an allergic reaction of any severity to eggs should still receive the influenza vaccine

340
Q

What is the dosing for children with suspected opioid overdose?

A

< 5 yo (or < 20kg): 0.1 mg/kg
>5 yo (or >20kg): 2mg

TIP: dosing is typically HIGHER than that of adults because children don’t have opioid tolerance. The higher the tolerance, the easier to precipitate withdrawal or reversal with naloxone.

If unresponsive to first dose, repeat with SAME dose

TIP:

  • A patient with HIGH tolerance to opioids needs a lot of opioids to maintain. In this case, a small decrease in the amount of opioids he/she is consuming can easily lead to withdrawal. Hence, small doses of naloxone can trigger withdrawal
  • On the other hand, an opioid naive patient who has overdosed has no tolerance. Hence, small amounts are need to cause toxicity. Hence, to reverse opioid toxicity, one needs to completely remove all the opioids attached to the mu receptor, hence higher doses of naloxone.
341
Q

Patient with tension pneumothorax, what’s the next step?

A

A needle is inserted perpendicular to the skin above the second intercostal space in the midclavicular line. After a gush of air is heard, the needle is retracted and the angiocatheter can be left in place and secured. This procedure relieves the high pressure in the intrapleural space, but it does not resolve the pneumothorax, so a chest tube will be required.

TIP: 3-4 inch long catheter

342
Q

What is dyskinetic CP?

A

TIP: Dyskinetic CP is CP with extrapyramidal manifestations or ABN MOVEMENT manifestations. Think of ADE of antipsychotics i.e. bradykinesia, dystonia

Extrapyramidal or dyskinetic type, typically affecting the arms more than the legs with various abnormal movements (instead of tone) such as bradykinesia, choreoathetosis, hemiballismus, or dystonia

343
Q

Most common causes for dyskinetic CP?

A

Although there are a variety of causes for dyskinetic CP, the 2 most common are neonatal and include hypoxic-ischemic encephalopathy and kernicterus

344
Q

Explain the how SMA syndrome leads to vomiting?

A

It occurs after loss of adipose tissue anterior to the duodenum. Normally, adipose tissue separates the SMA from the third portion of the duodenum; however, when the loss of this tissue occurs, the SMA compresses the duodenum, creating a partial or complete small bowel obstruction.

345
Q

What is your differential diagnosis for cyclic vomiting?

A

Cyclic vomiting (episodic vomiting with periods of wellness in between) can be associated with malrotation and intermittent midgut volvulus, abdominal migraine, cyclic vomiting syndrome, drug use (marijuana), SMA syndrome, and eating disorders.

346
Q

How to diagnose SMA syndrome?

A

Upper GI series

347
Q

What is the double bubble sign indicative of in a radiograph?

A

Duodenal atresia - dilated stomach and duodenum because they are 2 discontinuous loops; the disconnected duodenum fills up from its own contents

348
Q

Bilious vomiting indicates what location of obstruction?

A

Small intestine

349
Q

Following a wound, whether or not a child receives tetanus post-exposure prophylaxis depends on 2 factors only. What are they?

A

Appropriate postexposure tetanus prophylaxis depends on 2 factors: if the child has received at least 3 doses of tetanus toxoid (typically from a primary series of vaccinations) and if the wound is considered to be clean or dirty

If dirty: need DTaP/Td/Tdap IF > 5years since last one
If clean: need DTaP/Td/Tdap IF > 10 years since last one

350
Q

What determines what tetanus post-exposure prophylaxis a patient gets following a wound (DTaP/Td/Tdap)

A

Children younger than 7 years who require a tetanus vaccine under this management algorithm should receive the DTaP vaccine, children 7 to 10 years old should receive Td, and children 11 years and older should receive Tdap.

TIP:

  • DTaP for < 7 years
  • Td for 7 to 10 years
  • Tdap for > 11 years

Pattern: DTaP -> Td -> Tdap

351
Q

What is an alternative to treat AOM if patient is penicillin allergic?

A

Cefdinir

352
Q

What is the cross reactivity of cephalosporins to penicillin?

A

Only 2% of individuals with a penicillin allergy are expected to react to cephalosporins, and less than 1% will react to carbapenems

353
Q

What is the difference between Marfans and Homocystinuria in regards to the direction of ectopic lens dislocation?

A

Downward dislocation of the eye lens is typical in Homocystinuria rather than the upward dislocation seen in Marfan syndrome.

354
Q

Infant presents with shortness of breath and EKG demonstrates large Q waves in the II, III and aVF. What disease does the patient need to be evaluated for ?

A

Electrocardiogram showing deep Q waves in the inferior leads (II, III, AVF) should be evaluated for an anomalous left coronary artery from the pulmonary artery (ALCAPA)

355
Q

What is occurring anatomically in ALCAPA (Anomalous left coronary artery from pulmonary artery)?

A

When the left coronary artery arises from the pulmonary artery and not the aorta, it leads to an ALCAPA. he natural history of pulmonary vascular resistance is that it begins to drop in the first few months after birth. As the pulmonary vascular resistance begins to drop, and consequently, the pulmonary artery pressure begins to drop, the perfusion pressure of this coronary artery also drops, resulting in myocardial ischemia and necrosis.

356
Q

What is the difference in the presentation of Transient Neonatal Pustular Melanosis and Erythema Toxicum?

A

BOTH have pustules

ET: red base, appears 24-48hours after birth
TNPM: pustules without erythematous base, resolves within 24-48 hours; base is hyperpigmented

357
Q

There are very few reasons NOT to breast feed, including infections. Which infections is it contraindicated to breastfeed?

A

Specific maternal infections (untreated active tuberculosis, HIV in developed countries, human T-cell lymphotropic virus type I or II, untreated brucellosis, and ebola virus)

358
Q

What is the youngest age that a patient may be able to provide assent?

A

While the minimum age of assent varies, children as young as 7 years have developed limited logical thought processes and have the capacity for reasoned decision-making.

359
Q

How will antibiotics affect the CSF yield of culture, cell count, PCR?

A

It will not affect the CSF cell counts or identification of the organism by molecular methods and is unlikely to obscure the diagnosis.

Will affect the culture

360
Q

What is the cause of Hirschsprung?

A

Hirschsprung disease is caused by failed migration of ganglion cells to the rectosigmoid region of the colon

361
Q

What test to perform first if Hirschsprung is suspected?

A

Barium enema will show a narrow region (the affected region) and the proximal dilated bowel loops

362
Q

What is the general rule for when a patient post concussion is medically cleared to return to their full exercise activity?

A

Medical clearance for return to contact sports requires:

  • full symptom resolution at rest
  • full symptom resolution when in academic setting
  • full symptom resolution with physical activity
363
Q

Children with Horner Syndrome need to be evaluated for what underlying disease?

A

Neuroblastoma: Young children with isolated Horner syndrome should undergo careful examination for cervical and abdominal masses, measurement of urinary HVA and VMA levels, and radiologic imaging of the head, neck, and chest.

364
Q

What muscle is affected in Horner syndrome to cause ptosis vs CN III palsy?

A

Superior tarsal muscle vs the Levator palpebrae

365
Q

What is the most common cause of acute mastoiditis?

A

Similar to the microbiology of AOM, the most common etiologic agent of acute mastoiditis is Streptococcus pneumoniae, even in the era of the pneumococcal conjugate vaccine (PCV). Other bacterial causes include Staphylococcus aureus (including methicillin-resistant S aureus), group A Streptococcus, nontypeable Haemophilus influenzae, Moraxella catarrhalis, and Streptococcus anginosus

366
Q

What syndromes are associated with pheochromocytoma?

A

Pheochromocytoma is commonly associated with familial syndromes such as von Hippel-Lindau syndrome, multiple endocrine neoplasia type 2, and neurofibromatosis type 1

All children diagnosed with pheochromocytoma should undergo genetic testing because of the association with these familial syndromes

367
Q

What cells are pheochromocytomas derived from?

A

Pheochromocytomas are catecholamine-secreting tumors that develop from the chromaffin cells of the adrenal medulla

TIP: DO NOT mistake pheochromocytomas with neuroblastomas. Former from chromafinn cells and latter from embryonic neural crest cells that are supposed to give rise to the sympathetic chain.

368
Q

Infant with new onset bilateral lower extremity flaccid paralysis - what is the diagnosis until proven otherwise ?

A

New-onset bilateral lower extremity weakness, a neuroblastoma should be suspected. Neuroblastoma is the most common extracranial solid tumor in children. Can arise from the paraspinal ganglia. It can invade through the spinal neural foramina and create an epidural mass causing spinal cord compression

369
Q

What population is at higher risk of TEN/SJS when treated with Oxcarbamazepine?

A

Patients of Asian descent who have the HLA-B*1502 allele are at risk for serious hypersensitivity reactions, such as Stevens-Johnson syndrome or toxic epidermal necrolysis (TEN), when treated with oxcarbazepine. FDA recommends testing.

370
Q

When are children vaccinated for measles?

A

MMR: 12-15 mos and 4-6 years

371
Q

What are the associated electrolyte abnormalities with refeeding?

A

Hypophosphatemia, hypomagnesemia, and hypokalemia can occur due to intracellular electrolyte shifts as refeeding begins

372
Q

What patients are at risk for invasive pneumococcal disease?

A

Risk for invasive pneumococcal pneumonia is increased in children with HIV disease, primary immunodeficiency, immunosuppression related to medical therapy for cancer or inflammatory processes, cochlear implants, or sickle cell disease.

Hence, PCV 13 followed by PPSV 23 in this population

TIP:

  • Cochlear implants are a risk factor for invasive pneumococcal diseases - NOT meningococcal disease
  • Complement deficiencies are a risk factor for invasive meningococcal disease - NOT pneumococcal disease
373
Q

What are some complications of CAP, that if present, may imply a staph aureus component?

A

These may include pleural effusion, empyema, necrotizing pneumonia, lung abscess, atelectasis, pneumatocele, or pneumothorax

374
Q

What is the natural history of an infantile hemangioma?

A

Appear within 1 month of age (BUT not present at birth! That would be congenital hemangioma)

Grows until 6 months the most

Starts to involute by 12 mos

Completely gone by 4 years

375
Q

First line treatment of infantile hemangioma?

A

Propanalol

COOL History: The effectiveness of propranolol was first discovered in 2008 when it was observed that infants with infantile hemangiomas who received propranolol for cardiac abnormalities had improvement of their hemangiomas compared to infants who did not receive propranolol

376
Q

What is the cardiac defect associated with William syndrome?

A

Supravalvular aortic stenosis

75% of individuals!

377
Q

What is the difference between penetrance and expressivity (e.g. variable expression)?

A

Penetrance refers to the probability of a gene or trait being expressed. In some cases, despite the presence of a dominant allele, a phenotype may not be present.

Expressivity on the other hand refers to variation in phenotypic expression when an allele is penetrant

378
Q

What is a contiguous gene deletion syndrome?

A

A contiguous gene deletion syndrome is caused by a microdeletion that encompasses 2 or more genes in tandem position along a chromosome. By virtue of the fact that several genes are involved, contiguous gene syndromes often affect multiple systems of the body.

William Syndrome!

379
Q

Does a privately run child care centers have the right to refuse to care for a child because they have a G tube?

A

Privately run child care centers are considered “public accommodations,” similar to private schools, recreation centers, restaurants, hotels, movie theaters, etc, and must comply with the ADA (Americans with Disabilities Act)

380
Q

What is the treatment of choice for congenital CMV?

A

Oral valganciclovir for 6 months for SYMPTOMATIC CMV

381
Q

What is the most common congenital VIRAL infection?

A

Cytomegalovirus is the most common congenital viral infection in the United States, occurring in 0.5% to 1% of all live births

382
Q

What scenario during pregnancy places a fetus at the highest risk of CMV infection and severe sequelae?

A

Primary infection

Early gestation

383
Q

First line treatment for labial adhesions in young girls?

A

Nearly all children with labial adhesions will have complete self-resolution within 18 months, therefore asymptomatic individuals should receive reassurance.

Symptomatic individuals (those with urinary problems such as urinary tract infections or urinary retention) should first undergo a trial of twice daily topical estrogen cream.

384
Q

What are the developmental milestones of a 3 year old?

A
Gross motor:
 Pedals a tricycle (3 wheels)
 Alternates feet going up stairs (3 points of contact)
 Walks on tiptoe
 Catches a ball
 Dresses with supervision
Fine motor (3 table things)
 - fork, spoon, pitcher 

Language:

  • 3/4 words understandable
  • 3-4 word phrases

Social emotional

  • Knows first and last name and age
  • Engages in make-believe play
  • Understands turn taking
385
Q

When are children screened for ASD?

A

“9, 18, 27” (TIP: technically 24-30 months)

Every 9 months

386
Q

What is the treatment of nasal septal hematomas?

A

Nasal septal hematomas should be drained expeditiously to prevent permanent cosmetic defects

387
Q

After a displaced nasal fracture, when should a patient be seen by ENT for reduction?

A

ON presentation. Sometimes reduced on the spot, but sometimes within 5 days since there is so much swelling initially

388
Q

What are some labs to obtain to support diagnosis of myositis?

A

Laboratory evaluation showing evidence of muscle breakdown, including elevated levels of creatinine kinase, lactate dehydrogenase, and aldolase, is consistent with dermatomyositis

389
Q

If patient has suspected hyperinsulinemia, what should the follow labs demonstrate:

  • Insulin
  • BHOB and Fatty acids
  • GH
  • Cortisol
A

In addition to confirming the plasma glucose level, measurements of insulin, β-hydroxybutyrate (a ketone body), free fatty acids, growth hormone, and cortisol levels are useful tests to perform. Results consistent with hyperinsulinism include a detectable insulin level, low β-hydroxybutyrate and free fatty acid levels, and appropriately high growth hormone and cortisol levels. Insulin, an anabolic hormone, suppresses fatty acid oxidation and ketone body formation. Hypoglycemia is a normal stimulus for growth hormone and cortisol secretion

390
Q

What is the initial treatment of hypoglycemia in an infant that can’t take PO?

A

Immediate treatment of hypoglycemia includes an intravenous bolus of 2 mL/kg of 10% dextrose followed by continuous infusion of dextrose.

391
Q

What is the triad of symptoms for Reiter Sydnrome?

A

AKA Reactive arthritis, that is, reactive to an infection

1) Arthritis
2) Conjunctivitis
3) Urethritis

392
Q

What is the characteristics of the arthritis associated with reactive arthritis?

A

Asymmetric, large joints

393
Q

Treatment for reactive arthrtitis?

A

NSAID

394
Q

When to provide PPV and CPR for neonate?

A

PPV: HR < 100 despite warm, dry, stim
CPR: HR < 60 despite the above

395
Q

What is MR-SOPA? Purpose?

A

If neonate HR < 100 regardless of PPV, MRSOPA is an aid to re-evaluate/adjust

MR-SOPA: M-adjust the mask, R-reposition the head, S-suction the mouth then nose, O-open the mouth and lift jaw forward, P-gradually increase the pressure, A-consider airway alternative (endotracheal tube).

396
Q

Mother of a special needs child would like more clear information in regards to her child’s ability to adapt and function in the real world (e.g. self care, vocation, etc). What test would you suggest?

A

Commonly used standardized measures of adaptive behaviors include the Vineland Adaptive Behavior Scales (VABS) and the Adaptive Behavior Assessment System (ABAS). These rating scales gather information from parents, caregivers, and teachers. The results can be used to assist with diagnosis, identify the child’s strengths and weaknesses, monitor progress, and develop treatment plans.

TIP: level of adaptive functioning determines the severity of impairment and is related to the amount of care and supervision that a person needs in the future.

397
Q

What is the relationship between school completion due and adolescent pregnancies?

A

The majority of adolescent pregnancies end in school drop out

60% drop out
2% finish a college degree by 30 yo

398
Q

Presentation of imperforate hymen?

A

Imperforate hymen will present during adolescence in the form of primary amenorrhea, cyclical abdominal or pelvic pain, a lower abdominal mass, or a blue discoloration of the hymen because of hematocolpos.

Due to vaginal distention, it can also present with back pain, or difficulty with urination and defecation

399
Q

RUS findings of UPJ obstruction in infant?

A

RUS findings of hydronephrosis WITHOUT ureteral dilation favor a UPJ obstruction.

400
Q

RUS of infant with febrile UTI shows unilateral hydronephrosis with ureteral dilation. Differential diagnosis?

A

Ureterovesical junction obstruction, ureterocele, and an ectopic ureter have ureteral dilation along with hydronephrosis favoring a distal obstruction

401
Q

Causes of myocarditis?

A

Viral: Adenovirus, enterovirus, parvovirus, Epstein-Barr virus, influenza, and human herpesvirus 6

TIP: bacteria, parasites, tick can cause it too! Borrelia! Chlamydia! Listeria!

402
Q

What is the most common STI in the US?

A

HPV

Almost all sexually active people acquire human papillomavirus at some point in their lifetime.

403
Q

What is the difference between condyloma lata and condyloma acuminatum?

A

Condyloma lata - Syphilis - you get it “later” because of secondary syphilis

Condyloma acuminatum - HPV

404
Q

What is the treatment for tympanostomy tube otorrhea?

A

Topical ofloxacin is the treatment of choice for tympanostomy tube otorrhea.

TIP: imagine water entering the ear and pseudamonas colonizing the inner ear causing infection

405
Q

What is the definition of chronic AOM?

A

Chronic suppurative otitis media is defined as purulent otorrhea that lasts for longer than six weeks

406
Q

What organisms are responsible for tympanostomy tube otorrhea?

A

Aside from the typical AOM organisms, Pseudamonas and Staph Aureus are causative agents

TIP: Once you have a tympanostomy, you are exposed to the organisms of EOM, hence staph and pseudomonas

407
Q

What is the preferred initial test for patient with possible primary ciliary dyskinesia?

A

Based on the 2018 Clinical Practice Guideline for diagnosis of PCD , the preferred test in a patient five years of age or older with at least two of the four key clinical features and for whom cystic fibrosis (CF) has been ruled out, is measurement of exhaled nasal nitric oxide

408
Q

What are the 4 KEY clinical features of Primary Ciliary Dyskinesia?

A

The four key clinical features of PCD are 1) year round, daily, productive cough; 2) year round, daily non-seasonal rhinitis; 3) neonatal respiratory distress in a term infant; and 4) laterality defects (eg, situs inversus totalis).

Clinical tip: 2/4 features AND ruled out CF -> pursue exhaled nasal nitric oxide test

409
Q

What is a normal sweat chloride test?

A

The sweat chloride value for this patient is <30 mMol/L

410
Q

Treatment for the common wart?

A

Although a variety of treatments for common warts are available, for most children keratolytic therapy using topical salicylic acid is the preferred initial approach.

411
Q

What is the preferred analgesia for circumcision?

A

A subcutaneous ring block or dorsal penile block should be used during circumcision

Acetaminophen and oral sucrose can be used as adjunct therapies but should not be used as monotherapy during a circumcision

412
Q

What does hypoketotic hypoglycemia imply in regards to the possible subtype of IEM?

A

Fatty Acid Oxidation Deficiency (FAOD)

Naturally, in hypoglycemic states, fatty acids are broken down to provide another source of glucose. The lac of ketones, especially in the face of hypoglycemia means FA aren’t being broken down - FAOD

413
Q

What organs are often affected in Fatty Acid Oxidation Disorders?

A

Heart
Skeletal muscles
Liver

Thus, fatty acid oxidation disorders commonly lead to hypoketotic hypoglycemia, hepatomegaly, hepatic dysfunction, myopathy, rhabdomyolysis (elevated creatine kinase), and encephalopathy

Phys: Heart and skeletal muscles use fatty acids as their PRINCIPAL source of energy during exercise. Hepatic fatty acid oxidation is an important source of ketone synthesis

414
Q

What are the FOUR specific labs that can help diagnose suspected inborn error of metabolism?

A

1) Urine organic acids
2) Plasma amino acids
3) Plasma acylcarnitine profile
4) Carnitine Analysis (aka total and free carnitine and carnitine esters)

  • TIP: only one of the labs is from the urine (UOA) urine organic acids
  • Interesting thing is that each lab can help diagnose disease in multiple categories. For example, PAA will help diag not only amino acidopathies, but also organic acidemias
  • 1) Urine organic acids (UOA)- helps diagnose organic acidemia
    - Tip: can help diag amino acidopathies since AA that can’t be broken down may go down other pathways and form organic acids instead
    2) Plasma amino acids (PAA) - helps diagnose aminoacidopathies
    3) Plasma acylcarnitine profile (PAC) - profile of acylcarnitine means which acylcarnitines predominate. For example, acylcarnitine profile with C6-C10 is indicative of MCAD. Diagnose most FAOD and organic acidemias
    - TIP: Carnitine commonly known because it helps transport fatty acid chains across the mitochondrial membrane for break down. Generally, it is used in BOTH fatty acid break down and organic acid break down.
    4) Carnitine Analysis (aka total and free carnitine and carnitine esters).
    - TIP: For example, simple deficiency of free carnitine is suggestive of carnitine deficiency. Helps diagnose FAOD and organic acidemias
415
Q

Very long-chain acyl-coenzyme A dehydrogenase (VLCAD) deficiency (VLCAD) picmonic

A
  • Pig on a long chain leash
  • Pulling ship with flag
  • Magnet pulling the pig
  • Liver dysfunction
  • Heart failure
  • Rhabdomyolysis
416
Q

What is the presentation of VLCAD?

A

Present in 1 of 3 scenarios:

1) severe early-onset cardiac failure that can progress to multiorgan failure
2) hepatic or hypoketotic hypoglycemic form; this type presents in early childhood with hypotonia and hepatomegaly but typically lacks cardiomyopathy
3) Later onset, manifesting with episodic myopathy and intermittent rhabdomyolysis typically provoked by muscle cramps, pain, or exercise

PICMONIC:
- long bouncy chain attached to a pig vascillating back and forth hitting 1) heart and causing blood to gush out 2) liver on a swan 3) hulk’s arm with a heart tattoo

417
Q

What are PE findings and maneuvers that imply medial epicondylar apophysitis or avulsion injury of the medial epicondyle?

A

Physical examination in a patient with medial epicondyle apophysitis or avulsion reveals tenderness and possible swelling over the medial epicondyle. There is often pain with full extension and with resisted wrist flexion and pronation.

418
Q

What factor in premature infants is the risk factor most associated with neurodevelopmental outcomes?

A

For the premature infant, after gestational age, intraventricular hemorrhage (IVH) of any grade is the risk factor most closely associated with neurodevelopmental impairment

419
Q

What are risk factors for IVH in the neonate?

A

Intraventricular hemorrhage is primarily seen among neonates of less than 32 weeks’ gestation or with birthweights of less than 1,500 g

TIP: Just like ROP, size is just as important as age.

420
Q

What is the difference between pre and post axial polydactyly?

A

The extra digit is most common on the ulnar (little finger) side of the hand, less common on the radial (thumb) side, and very rarely within the middle three digits.

Postaxial (little finger)
Preaxial (thumb)
Central (ring, middle, index fingers)

TIP: axial means central, like axial skeleton, which refers to the spine. In the anatomic position, the palms of the hand are facing forward, pinky toward the body and thumb lateral.

PICMONIC: imagine the little pinky holding up a POST with a monster truck (Auto dominant). Post axial

421
Q

What type of polydactyly is more likely to be associated with an underlying syndrome?

A

Children with polydactyly in the preaxial (thumb) and central locations are more likely to have other congenital abnormalities

422
Q

Suggested first line test to evaluate intellectual disability?

A

In the nonsyndromic patient with intellectual disability, chromosomal microarray and DNA analysis for fragile X are the recommended first-line tests for identifying a cause

423
Q

Framework Question:

What is chromosomal microarray?

A

“Array-based” describes the use of preselected segments of DNA, which are adhered to a surface.

Detecting gains or losses involves either comparison of the patient sample with that of a normal control individual or comparison of signal intensity at the various probes to expected intensity levels. For example, if there is loss of a gene, then the probe will have a low intensity. If there is gain in the gene, the probe will have a higher intensity.

Short answer: CMA only quantifies the amount of DNA present- either too much or too less.

424
Q

What is the set back of Chromosomal Microarray?

A

CMA only quantifies the amount of DNA present. Therefore, it does not detect structural chromosomal changes that do not result in deletions or duplications (eg, balanced translocations or balanced inversions) as the total amount of DNA is normal.

425
Q

What are the 2 benign neonatal seizure syndromes?

A

Benign familial neonatal convulsions

Benign neonatal seizures

TIP: Terrible names! They both sound the same

426
Q

What is the most common cause of neonatal seizures?

A

HIE

427
Q

What area of delays is most affected in children with achondroplasia?

A

Gross motor delays, but otherwise normal development (e.g. cognitive, fine motor, social/langauge, etc)

428
Q

What is the presentation of achondroplasia?

PICMONIC

A
  • Autosomal dominant
  • Shortened long bones - mainly femur and humerus
  • Brachydactyly - short fingers and toes
  • Macrocephaly
  • Elves/dwarves riding a monster truck
  • Monster truck has a large a bobble head
429
Q

What is the cause of achondroplasia?

A

Mutation in the fibroblast growth factor receptor 3 gene (FGFR3) that affects bone development

430
Q

Framework question:

What is normal infant weight gain per day in the first year of life?

A

0-3 months: 30 gm/day (TIP: 1 oz every day)
3-6 months: 15 gm/day (TIP: 1 oz every 2 days)
7-12 months: 10 gm/day (TIP: 1 oz every 3 days)

431
Q

What is considered catch up growth in preterm?

A

Refers to rate of weight gain > 50th percentile for age.

That is, a preterm infant reaches the 50th percentile for his/her adjusted gestational weight

432
Q

What is the general rule in calculating a patient’s normal MCV?

A

72 fL + age in years

433
Q

Expected findings in BM biopsy in patient with pancytopenia due to aplastic anemia vs leukemia?

A
  • Aplastic anemia: hypocellular

- Leukemia: hypercellular

434
Q

Risk factors of CoNS infection?

A

Tied to its ability to create a BIOFILM

Risk factors for CoNS infection include prematurity, especially in infants who weigh less than 1,500 g, and foreign devices or material. Indwelling devices at risk for colonization with CoNS include central venous catheters, ventriculoperitoneal shunts, and peritoneal catheters. Other foreign material at risk for colonization includes orthopedic hardware, baclofen pumps, prosthetic joints, pacemakers, and prosthetic valves

435
Q

What are common sources of salicylate poisoning that is not from aspirin?

A

Salicylates are found in over-the-counter products that have a wintergreen odor including topical analgesics (methyl salicylate) and anti-indigestion medications (bismuth subsalicylate).

Oil of wintergreen is a highly concentrated methyl salicylate solution that has a variety of uses ranging from topical analgesics to fragrances. Just a few milliliters of oil of wintergreen can be lethal to a young child

436
Q

What is the treatment of salicylate poisoning?

A

Aggressive intravenous fluid resuscitation is done with the addition of sodium bicarbonate to promote urinary alkalinization to facilitate salicylate excretion. Hemodialysis may be indicated in extremely severe cases of salicylism.

437
Q

First line test to diagnosed congenital adrenal hyperplasia?

A

17-hydroxyprogesterone level is the first-line diagnostic test for congenital adrenal hyperplasia

17-hydroxyprogesterone is an intermediate steroid in the adrenal glucocorticoid pathway, which is a substrate for 21-hydroxylase and builds up in 21-hydroxylase deficiency

438
Q

What is the difference in the presentation of males and female infants with CAH?

A

Males: no PE findings; later adrenal salt-wasting crisis, hence the importance of the newborn screen

COOL PHYS: defect in production of glucocorticoids and adrenal mineralocorticoids

Females: virilization of the genitalia and no palpable testis

COOL PHYS: This defect in adrenal glucocorticoid production results in an elevated adrenocorticotropic hormone (ACTH) level, which stimulates the adrenal gland to make excess androgen

439
Q

What is the cause of CAH?

A

21 hydroxylase deficiency

440
Q

When should an infant undergo surgical correction for undescended testis to prevent malignancy?

A

Surgical treatment of undescended testes is recommended between 6 and 12 months of age

441
Q

At what age should almost ALL undescended testis descend into the scrotum?

A

By 3 months of age, only 1% remain undescended

Those infants with testes that remain undescended require surgery to bring the testicle into the scrotum (orchiopexy) or remove the testicle (orchiectomy) because they will not spontaneously descend from the abdomen into the scrotum after this age

442
Q

Most common diagnosis for lower back pain in adolescents?

A

Mechanical (muscular) lower back pain

443
Q

What is the treatment of choice for adolescents with Mechanical (muscular) lower back pain ?

A

Physical therapy for core stabilization is the next best step in management

444
Q

What is spondylolysis? Relationship with Spondylolisthesis?

A

Spondylolysis is a crack in the pars interarticularis, the posterior aspect of the vertebral ring

When spondylolysis is bilateral (ie, 2 cracks occur in the same vertebral ring), the vertebral body may shift forward leading to spondylolisthesis

445
Q

How to diagnose pancreatic insufficiency?

A

Fecal elastase < 100

Demonstrates a deficiency in the pancreas to secrete proteases

446
Q

Swachman-Diamond Syndrome presentation?

PICMONIC

A
Swachman-Diamond: 
Short stature
Bone involvement
Pancreatic insufficiency (exocrine) 
Pancytopenia (particularly neutropenia) 
Associated increased infections
447
Q

Most common cause of exocrine pancreatic insufficiency?

A

The most common cause of EPI in children is cystic fibrosis, which is caused by mutations in the cystic fibrosis transmembrane regulator protein, many of which can result in pancreatic insufficiency

448
Q

What are the symptoms of EPI - exocrine pancreatic insufficiency?

A

Clinical presentation may include:

  • steatorrhea
  • fat soluble vitamin deficiency
  • weight loss
  • poor growth in children
  • nonspecific abdominal symptoms (such as bloating, diarrhea)
449
Q

What prenatal exposures are associated with aggressive disposition in children?

A

Tobacco
Alcohol
Cocaine

450
Q

What type of parenting style is associated with aggressive behavior in children?

A

An authoritarian style of parenting, characterized by a high level of control and low level of warmth (eg, orders and commands given with expectations for obedience) is the parenting style most associated with aggression.

Better psychosocial and mental health outcomes are seen with an authoritative parenting style, where a high level of control occurs in the setting of a high level of warmth (eg, expectations and rules clearly established with engaging and interactive parents)

TIP: difference between authoritaRIAN vs authoritaTIVE

451
Q

What are risk factors for Guillain Barre Syndrome?

A

Antecedent respiratory or gastrointestinal infection, back surgery, and bone marrow transplantation

452
Q

What may be clues that a concurrent infection is occurring in the setting of Guillain Barre Syndrome?

A

Cerebrospinal fluid white blood cells more than 50 per high-power field should prompt a search for concurrent infection.

453
Q

What is the most common disease associated with chronic urticaria?

A

Thyroid disease

454
Q

What is the definition of chronic urticaria?

A

Presence of hives, with or without angioedema, for a period of 6 weeks or longer.

455
Q

Treatment of chronic urticaria?

A

H1 antihistamines, sometimes in higher than usual doses, serve as first-line treatment in combination with H2 antihistamines and leukotriene modifiers.

456
Q

Most common presentation of G6PD?

A

Asymptomatic, even in severe enzyme deficiency, there is no anemia and no hemolysis.

Symptoms ONLY occur in the setting of acute sickness, infection, ingestion of sulfa drugs and fava beans

457
Q

What are findings on peripheral blood smear to suggest G6PD?

A
Heinz bodies (denatured, precipitated hemoglobin) 
Bite cells
458
Q

G6PD PICMONIC

A
  • Africans
  • Malaria
  • X linked
  • Oxidative burst:
  • Sulfa drugs
  • Fava beans
  • Hemolysis
  • Jaundiced baby
  • Blister cells/Heinz body/Bite cells
459
Q

What non-IV analgesia options are there?

A

Intranasal Fentanyl

Studies have demonstrated that doses of fentanyl delivered via a transmucosal route have similar analgesic action to intravenous opioids. An advantage of transmucosal analgesics is the ability to provide rapid pain relief without the need for intravenous access.

460
Q

Triad for HUS?

A

Hemolytic uremic syndrome is characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury

SUSPECT in ALL children with the combo of bloody diarrhea AND AKI

461
Q

Cause of HUS?

A

The most common cause of diarrhea-positive HUS is Shiga toxin–producing Escherichia coli (STEC). Enterohemorrhagic E coli (EHEC) is the most common organism identified in STEC HUS; EHEC O157:H7 is the most common strain of EHEC associated with HUS in the United States

462
Q

What is the suggested Vit D dose/frequency for exclusively breastfed infants?

A

400 IU of Vit D daily

463
Q

What are natural sources of Vitamin D?

A

Vitamin D is naturally found in liver and other organ meats; egg yolk; oily fish such as salmon, sardines, and mackerel; and cod liver oil.

464
Q

What are the orthopedic or bone manifestations of Vitamin D def?

A

Bony abnormalities including delayed dental eruption, valgus deformity of the leg, kyphosis, widening of the metaphysis of the wrists, and rachitic rosary (an enlarged costochondral junction along the anterolateral aspect of the rib cage)

Tip: Bowing of the legs and spine

Long bones are osteopenic with thin cortices

Widened cranial sutures, frontal bossing

465
Q

What are the most common pathogens to cause epiglottis?

A

Haemophilus influenzae type b, a gram-negative coccobacillus, is the most frequent cause of epiglottitis in unimmunized children, followed in order by group A Streptococcus (GAS), Streptococcus pneumoniae, and Staphylococcus aureus

466
Q

Can pathogens other than bacteria cause epiglottis?

A

Acute epiglottitis can also be caused by viruses (eg, herpes simplex virus), fungi (eg, Candida albicans), and noninfectious sources (eg, physical trauma, chemicals, and heat), especially in adults

467
Q

When can peripheral parenteral nutrition be used instead of TPN?

A

Peripheral parenteral nutrition may be considered for very short-term parenteral nutrition supplementation in patients who are not at significant nutritional risk

468
Q

Erythromycin ointment for the eyes in a newborn is prophylaxis against what organism/disease?

A

N. Gonorrhea

Neonatal conjunctivitis

HISTORICAL DETAIL: Historically, ophthalmia neonatorum was the leading cause of neonatal blindness, caused primarily by Neisseria gonorrhoeae.

469
Q

What physical exam finding is consistent with delayed puberty in boys?

A

Less than 4 mL in volume or 2.5 cm in length prior to age 14 years in boys.

470
Q

What historical detail implies Kallmans as the cause of delayed puberty in a patient?

A

Lack of smell

471
Q

Explain the cause of delayed puberty in Kallman?

A

Delayed puberty caused by Kallmann syndrome is due to a hypogonadotropic hypogonadism associated with anosmia or hyposmia, the lack of or reduced ability to smell.

The association results from disrupted migration of gonadotropin-releasing hormone and olfactory neurons in the developing brain.

Testosterone and DHT are both essential in gonadarche

472
Q

What is the cause of phenylketonuria?

A

Phenylketonuria is a metabolic disorder caused by the enzymatic deficiency of phenylalanine hydroxylase

473
Q

Why will I probably never diagnose PKU, at least, in the US?

A

Universal newborn screening can detect PKU in nearly 100% of cases, thus preventing significant cognitive deficits through early intervention and treatment.

474
Q

PKU PICMONIC

A

PKU - pikachu
epilepsy - static bowl
eczema - rash on mice
varying levels of intellectual disability and behavioral problems that also include autistic features
Parkinson-like features (especially in adults)
musty body odor - mustard
decreased skin and hair pigmentation - albino mouse

Pickachu in a glass bowl causing electricity, white mice running around, putting mustard on their rashes

475
Q

What are concerning signs of possible autism at 18mos of age?

A
  • Lack of joint attention
  • Does not copy others’ actions
  • Has fewer than 6 words
  • Is not learning new words
  • Does not show distress when separated from a familiar caregiver
476
Q

What is the first step in clarifying if a patient (school age) has some form of intellectual disability?

A

Psychoeducational evaluation through the school

477
Q

What is the recommendation for initial fluid resuscitation in children presenting with DKA?

A

The 2014 International Society of Pediatric and Adolescent Diabetes (ISPAD) guidelines recommend an initial normal saline bolus of 10-12 mL/kg over 1-2 hours, followed by initiation of an insulin infusion. Subsequently, the free water deficit should be corrected over 48 to 72 hours.

478
Q

What is the recommended polio vaccination dose/schedule for children?

A

In the United States, a total of 4 doses of inactivated polio vaccine at ages 2, 4, and 6 to 18 months and 4 to 6 years is currently recommended for all infants and children

TIP: Two 6 mos, pediatric politicians…

479
Q

Do travelers to polio-affected countries require vaccinations?

A

Adults and children who intend to travel to and stay for more than 4 weeks in a polio-affected country should receive a single lifetime booster dose of IPV before departure- REGARDLESS if received a complete polio immunization series in childhood and

480
Q

Most common cause of superior vena cava syndrome?

A

Over 90% of cases of superior vena cava syndrome are caused by cancer

In children the most common cause is a mediastinal mass.

481
Q

Explain how SVC can lead to cardiac arrest? (AKA it’s an emergency)

A

There should be concern not just for compression of the superior vena cava, but also of the right atrium. Should the mass completely compress the right atrium, there would be no blood return to the heart, and cardiac arrest would ensue.

482
Q

What is the radiographic sign for acute epiglottis in XR?

A

Thumb sign - that is the epiglottis is enlarged and swollen, appearing like a thumb

483
Q

Dexamethasone in epiglottitis?

A

Intramuscular dexamethasone would not be the recommended first step in management of epigottitis. The role of corticosteroids in the treatment of epiglottitis has not been clearly established, as retrospective studies have not supported their efficacy in improving outcomes, and data from randomized clinical trials are not available.

484
Q

The presence of differential pulse oximetry (right hand O2 sat > right leg O2 sat) is indicative of what 2 processes? Explain

A

The presence of pulmonary hypertension or an aortic obstruction (coarctation of aorta or interrupted aortic arch) with a PDA can result in differential cyanosis, which is characterized by a pulse oximetry reading that is higher in the right hand relative to a lower extremity.

TIP: in BOTH scenarios, the pulmonary system has higher pressure than the systemic pressures

Explain:
- In coarctation, if the PDA is after the obstruction, blood will shunt from pulmonary system to systemic system via the PDA, since post obstruction, the systemic pressure is low. And since blue blood to red blood, hypoxia or lower saturations

  • In pulmonary HTN, blood will shunt from pulmonary system to systemic system via the PDA because the pressure is greater in the pulmonary system in comparison to the systemic circulation, that is, blue good to red blood
485
Q

What is the underlying disease when reverse differential cyanosis (right hand O2 sat < lower extremity O2 sat) is discovered from CHD screening of a newborn?

A

Reverse differential cyanosis is seen in D-transposition of the great arteries when either pulmonary hypertension or aortic arch obstruction is also present

Explanation:

  • In coarctation, if the PDA is after the obstruction, blood will shunt from pulmonary system to systemic system via the PDA since post obstruction, the systemic pressure is low. In the case of TGA, blood from the pulmonary system is red blood, so RED, oxygenated blood, is entering the systemic circulation, hence higher O2 saturations in the leg
  • In pulmonary HTN, blood will shunt from pulmonary system to systemic system via the PDA because the pressure is greater in the pulmonary system in comparison to the systemic circulation. In the case of TGA, the pulmonary system is RED, oxygenated blood, hence oxygenated blood is entering the systemic system via the PDA and the lower extremities have higher O2 sats.
486
Q

At what age should disconjugate gaze be further evaluated by an ophthalmologist?

A

Although it is normal for infants to have a disconjugate gaze in the first 2 to 4 months after birth, eye movements should be conjugate by 6 months.

AKA strabismus - abnormal alignment of eyes

487
Q

What is amblyopia?

A

Amblyopia, or reduced visual acuity because of abnormal visual development early in life, is the most common cause of visual impairment in children

488
Q

What is the recommended dose/schedule for pertussis containing vaccines?

A

In childhood, a total of 6 doses of pertussis-containing vaccine are recommended:

  • DTaP in a 5-dose series, typically at ages 2, 4, 6, and 15 to 18 months as well as 4 to 6 years
  • 1 dose of Tdap at 11 to 12 years.
489
Q

Child gets pertussis, who should this change his/her vaccine schedule?

A

Infection with Bordetella pertussis provides only short-term protection against future pertussis infection. Thus, a documented infection should not alter the standard immunization schedule.

490
Q

What is the ONLY population that receives multiple Tdap vaccinations? (rest of the population receives only 1)

A

The only group for which more than one dose of Tdap is recommended is pregnant women. The vaccine should be administered with each pregnancy between 27 and 36 weeks’ gestation as the best means of protecting young infants from pertussis.

491
Q

What is the greatest risk factor for childhood sexual abuse?

A

The greatest risk for child sexual abuse is living in a single parent family (family structure)

TIP: NOT associated with socioeconomic status or ethnicity

492
Q

Thrombocytopenia Absent Radius Syndrome - what is the natural history of the thrombocytopenia?

A

Normal after the first year of life

The thrombocytopenia in the first year of age can be life-threatening, but severe bleeding can be prevented through the use of prophylactic transfusions of single-donor platelets when the platelet count drops below a predetermined, age-dependent threshold

493
Q

What is the make up of “Corrosive” or “Caustic” agents?

A

These products may cause significant toxicity if ingested. Caustics—also known as corrosives—are concentrated acidic, alkaline, or oxidizing agents.

494
Q

What are common household items that can be caustic and corrosive when ingested?

A

Many are found in common household products including drain cleaners, toilet bowl cleaners, laundry detergents, stain and mildew removers, floor cleaners, oven cleaners, swimming pool cleaners, rust removers, phenol-based disinfectants, swimming pool products, and batteries.

NOT BLEACH

495
Q

What is the most common metabolic cause of renal stones?

A

Hypercalciuria is the most commonly identified metabolic cause of renal stones

496
Q

What are urinary inhibitors of nephrolithiasis?

A

Citrate (most important), magnesium, and pyrophosphate

497
Q

What pediatric patient requires a lipid panel as screening?

A

Patients 2-8 years of age whose parents have a total cholesterol >240

498
Q

What phase of adolescence is the most dangerous one?

A

Middle adolescence (14-17yo): most STI, #1 cause of injury and death are accidents

499
Q

What phase of adolescence is abstract thinking developed?

A

Late adolescence (18-21 yo)

500
Q

Framework Question:

Describe how the adolescents think differently in each stage of adolescence: early, middle and late adolescence?

A
  • Early adolescence (10-13): concrete thinking
  • Middle adolescence (14-17): concrete thinking and some abstract
  • Late adolescence (18-21): abstract

TIP: abstract thinking allows you to plan and to contemplate consequences

501
Q

What are organisms associated with recreational-water associated outbreaks of acute GI?

A

Common pathogens causing recreational water–associated outbreaks of acute gastroenteritis include Cryptosporidium, Shigella, Giardia, norovirus, and Escherichia coli O157:H7

502
Q

What pathogen that leads to gastroenteritis can also lead to self-limited seizures?

A

Shigella

Clinical tip: no need to treat the seizures with AED as they are self-limited

503
Q

Newborn has a pre auricular pit. Renal ultrasound?

A

It is not necessary to perform ultrasonography to screen for renal anomalies in newborns with isolated preauricular skin tags or pits and no other congenital anomalies or risk factors

504
Q

A newborn has a pre auricular pit or skin tag. What other details would warrant renal ultrasound?

A

In newborns with external ear anomalies who have other congenital anomalies or dysmorphic features, a family history of deafness, ear, or renal anomalies, or a maternal history of gestational diabetes, the likelihood of clinically significant structural renal anomalies is higher, and renal ultrasonography is warranted.

TIP: the ear and the kidneys look similar. Think of kidneys with ears

505
Q

How does ingestion of hydrocarbons (like from air freshener) lead to ARDS?

A

Hydrocarbons are volatile substances with low viscosity and surface tension, which favors aspiration, as opposed to swallowing. When aspirated, low surface tension causes the substance to be distributed over a large surface area in the lungs.

Aspiration of hydrocarbons causes direct injury to airway and alveolar epithelium. It also causes surfactant inactivation and decreased production of surfactant because of injury to type II alveolar pneumocytes. The decreased quantity and function of surfactant leads to increased alveolar surface tension, alveolar collapse, hypoxia, and increased pulmonary vascular resistance

506
Q

How to diagnose Tay Sachs Disease?

A

Demonstrate low to absent levels of β-hexosaminidase A enzymatic activity in the white blood cells

507
Q

What is the motor presentation of Tay Sachs Disease?

A

Combination of LMN and UMN (like ALS!)

Spasticity, hyperreflexia, ankle clonus, and diffuse muscular hypotonia

508
Q

Tay Sachs Disease PICMONIC

A
509
Q

What is the AAP recommendation for developmental and ASD screening?

A

The American Academy of Pediatrics recommends developmental screening at 9, 18, and 24 or 30 months, and screening specifically for autism at 18 and 24 months.

510
Q

What other organ system needs to be evaluated in patients with Klippel Fiel Syndrome?

A

Because renal abnormalities can be clinically silent, renal ultrasonography should be performed for all children with Klippel-Feil syndrome

Thoracolumbar radiography should be performed to look for additional vertebral fusions and associated scoliosis

TIP: Children with vertebral anomalies often have associated renal anomalies.

511
Q

How to determine the correct blood pressure cuff size for patient?

A

1) Find the midpoint between the acromion and the olecranon
2) Measure the circumference of the arm and note what length is 40% of the circumference and what length is 80% of the circumference

3) Find appropriate blood pressure cuff:
- bladder width is 40% arm circumference
- bladder length is 80% arm circumference

512
Q

What is the most common symptom for mumps?

A

Development of unilateral or bilateral salivary gland swelling, typically parotitis, occurs in 95% of patients with symptomatic disease

513
Q

Mumps presentation PICMONIC

A
514
Q

What does the term “second victim” refer to?

A

This refers to the providers involved in medical errors. The can be second victims as these adverse events may negatively impact them. Hence, counseling is recommended for all parties involved to insure appropriate coping skills

515
Q

What are the areas that seborrheic dermatitis tends to involve?

A

In adolescents and young adults, seborrheic dermatitis affects the scalp, eyebrows, glabella, alar or retroauricular creases, beard or sideburn areas, or ear canals

TIP: Dandruff is seborrheic dermatitis of the scalp!

516
Q

Framework Question:

What is the cause of seborrheic dermatitis?

A

Seborrheic dermatitis is a chronic and relapsing inflammatory disorder that affects areas in which sebaceous glands are concentrated. It is especially common in adolescents and young adults in whom sebaceous gland activity is greatest. Although the cause is not fully understood, it may be the result of an inflammatory response to the yeasts of the genus Malassezia

Clinical Tip: Hence, treatment is either low potency steroids to address the inflammation or anti fungals to treat the underlying yeast or the combination of both.

517
Q

What are the milestones for 12 mos old infants?

A

GROSS:

  • Stands well
  • Starts to take independent steps

FINE:

  • Pincer grasp (pick up 1 thing)
  • Holds 1 crayon

SOCIAL:
- Points (1 finger) to desired objects

LANGUAGE:
- Follows 1 step command

518
Q

What is the Expert Panel on Integrated Guidelines for Cardiovascular Health and Risk Reduction in Children and Adolescents’s recommendation for lipid screening for pediatric patients at normal risk?

A

The guidelines now state that universal screening should take place between the ages of 9 and 11 years and then again at age 17 to 21 years.

TIP: Additionally, it is normal for lipid levels to decrease during puberty.

519
Q

What is considered premature CAD?

A

“Early” is considered to be age 55 years or younger for a man and age 65 years or younger for a woman.

520
Q

What is the Expert Panel on Integrated Guidelines for Cardiovascular Health and Risk Reduction in Children and Adolescents of the National Heart, Lung, and Blood Institute recommendation for lipid screening in pediatric patients in moderate to high risk?

A

Children with a positive family history or a moderate- or high-risk medical condition (Item C112) should be screened at ages 2 to 8 years and 12 to 16 years with 2 fasting lipid profiles. (See suggested reading 1 for more information.) The average of these 2 results should be used to dictate next steps.

521
Q

What is the most narrow part of a child’s airway?

A

The narrowest part of the upper airway in a child is the subglottic region

522
Q

How does the presentation of wheezing vs inspiratory stridor aid in localizing the airway obstruction in a patient who presents with shortness of breath?

A

Extrathoracic airway obstruction tends to cause inspiratory stridor, because the highly negative intrathoracic pressure required to draw air into the lungs causes the obstructed area to collapse during inspiration.
- TIP: inspiratory stridor = extra-thoracic location

In contrast, intrathoracic airway obstruction tends to cause expiratory wheezing, because the positive intrathoracic pressure required to push air out of the lungs causes the obstructed airway to collapse during expiration.
- TIP: expiratory stridor = intrathoracic obstruction

Caveat: Biphasic obstructive sounds can occur in ANY pathology if moderately severe.

523
Q

Why can ingestion of “prenatal vitamins” pose a risk of iron toxicity?

A

Most prenatal multivitamins contain 60 to 65 mg of elemental iron, which is much higher than the 15 to 18 mg in a typical multivitamin.

524
Q

When to order serum iron level on children with suspected iron toxicity from ingestion?

A

4-6 hours after ingestion as that is when serum peaks for supra therapeutic ingestions of iron.

Serum iron level aids in determining the use of deferoxamine

525
Q

What are the 5 stages of iron toxicity?

A

TIP: MOST are GI-related, one is total system related

Gastrointestinal phase:
- Abdominal pain, nausea, vomiting, diarrhea, hematemesis, melena, lethargy, shock, metabolic acidosis

Latent phase: recovery

Shock phase: shock, coagulopathy, ARDS, renal failure, GI hemorrhage

Hepatic phase: hepatic injury

Bowel obstruction phase: GOO

526
Q

What is a sentinel event?

A

Unexpected event that threatens or results in serious injury or death.

This is in contrast to potential adverse event in which NO harm is done to the patient

527
Q

What is the difference between intercepted and non-intercepted adverse events?

A

BOTH are potential adverse events, in that , BOTH don’t lead to any harm to the patient. (otherwise, called sentinel)

There are 2 types of potential adverse events: intercepted and nonintercepted.

  • An intercepted error is recognized and corrected before it reaches the patient.
  • A nonintercepted error actually reaches the patient but does not result in harm
528
Q

What are symptoms of heat exhaustion?

A
Increased core temperature < 104
Vomiting, nausea
Cramping 
Dizziness, syncope
Tetany
529
Q

How does cow’s milk differ from human milk in content?

A

Because cow milk is intended for calves, protein and minerals (such as sodium, potassium, calcium, chloride, and phosphorus) essential for the higher growth velocity, are significantly higher than human milk.

TIP: think of cow’s milk and protein used by body builders - higher protein. cow’s milk can lead to GI bleed, hence iron is lower. All solutes are higher, which can cause dehydration given high solute load

530
Q

Framework Question:

What is the relationship between gut microbiota and Vitamin K deficiency?

A

Gut bacteria synthesize vitamin K2, an important isoform of vitamin K. Disruption of the gut microbiome (eg, by broad-spectrum antibiotics) can result in vitamin K deficiency

For children on long-term antibiotic therapy or prophylaxis, vitamin K deficiency can be prevented through the administration of vitamin K orally once weekly.

531
Q

What are the 2 sources of Vitamin K?

A

Some vitamin K (phylloquinone [vitamin K1]) is derived through the ingestion of green leafy vegetables, but an important isoform of vitamin K (menaquinones [vitamin K2]) is dependent on synthesis or modification by gut bacteria

532
Q

What are common causes of gross hematuria in children?

A
  • UTI/pyelonephritis
  • Trauma
  • Perineal irritation
  • PSGN (PISGN) - tends to be cola colored
533
Q

What is the underlying pathology in a patient who presents with blood clots with dysuria?

A

Cystitis

534
Q

What is the basic work up for child who presents with asymptomatic gross hematuria?

A
  • Urinalysis with microscopy (to differentiate between RBC and heme pigments)
  • Rrine calcium-creatinine ratio (for hypercalciuria) which may be indicative of nephrolithiasis
  • Serum creatinine and C3 levels (for nephritis)
  • Renal ultrasonography (for congenital anomalies or bladder mass)
  • Hemoglobin electrophoresis (for sickle cell disease) since the most common complication of sickle cell trait is renal papillary necrosis
535
Q

What defect may the presence of a bifid uvula reveal?

A

Submucosal cleft palate

536
Q

What is a flat mid face?

A

Midface hypoplasia describes a situation in which the upper jaw, cheekbones and eye sockets have not grown as much as the rest of the face.

537
Q

The definitive choice of imaging for osteomyelitis?

A

Magnetic resonance imaging

538
Q

What type of sports should be avoided in patients with seizure disorder?

A

Certain sports, such as swimming, diving, archery, and powerlifting, may pose a high risk to an athlete with a poorly controlled seizure disorder (and potentially to teammates and officials)

539
Q

How to determine a child’s potential height given parents’ heights?

A

Girl’s height = [ (father’s height - 5 inches) + mother’s height]/2

Boy’s height = [(mother’s height + 5 inches) + father’s height]/2

TIP: The patient’s potential height will be within 2 SD of this value

TIP:

  • G, B, G
  • B, G, B
540
Q

All patients with infantile spasms should be evaluated for what underlying syndrome?

A

Tuberous Sclerosis

541
Q

What is the appearance of an ashleaf spot?

A

Ash-leaf spots, which are flat, white, oval macule.

TIP: As if ash has fallen around a leaf and you removed the leaf and see a clear spot

542
Q

Treatment for Otitis Externa?

A

Neomycin plus polymyxin B plus hydrocortisone solution or suspension (considered the first-line therapy due to efficacy and low cost)

543
Q

What orthopedic pathologies is a patient with joint hyper mobility syndrome at risk for?

A

Dislocations

544
Q

Relationship between joint hyper mobility syndrome and fibromyalgia?

A

BOTH are characterized by chronic pain

545
Q

Newborn born to a mother with what appears to be an active genital HSV lesion, what is the next step? (treat and work up baby?)

A

Work up baby at 24 hours:
- If mother with preceding HSV infection (that is, this new infection i more likely recurrent): HSV surface culture and HSV blood PCR

  • If mother with NO preceding HSV infection (that is, concern for primary HSV infections): HSV surface/blood and CSF + start empiric acyclovir

Reason: primary HSV infection higher risk of perinatal transmission (as high as 50%) and need to be conservative

546
Q

Framework Question:

Treatment for perianal dermatitis?

A

Because perianal bacterial dermatitis may be caused by Streptococcus pyogenes or Staphylococcus aureus, empiric treatment is with oral cephalexin or another antistaphylococcal antibiotic based on local sensitivity patterns.

Perianal bacterial dermatitis = cellulitis of the anus

547
Q

What are the features of XYY sex chromosomal disorder?

A

Classic features include taller than normal stature, speech and language delay, cystic acne in adolescence, and learning disabilities.

TIP: interesting that the triple sex chromosomal disorders all have tall stature including XXY and XXX and all have learning disabilities , but relatively normal intelligence

548
Q

Neonate with fever. Amino glycoside started. What is the CSF penetration of aminoglycosides?

A

Poor CSF penetration

549
Q

What type of infections are commonly implicated in post infectious arthritis?

A

GU and GI

550
Q

What is the definition of enthesitis?

A

Enthesitis is defined as inflammation at the site of insertion of ligaments, tendons, joint capsule, or fascia to bone.

551
Q

What medications can be used to decrease swelling and increase chances of removal of nasal foreign body?

A

Use of a topical vasoconstricting agent, such as neosynephrine or oxymetazoline, is generally recommended before any attempt is made to remove a nasal foreign body.

These agents decrease localized swelling (which improves the chances of successful foreign body removal) and decrease bleeding.

TIP: if you rearrange the letters, neosynephrine looks like NOSE SYNEPHRINE, which looks like Epinephrine for the nose

552
Q

What re some techniques to remove a nasal foreign body?

A
  • Patient blows her nose
  • Suction catheter
  • Alligator forceps or a hook can be used to grasp and remove foreign objects from the nose.
  • Insertion of a small French foley catheter beyond the object followed by gentle retraction of the catheter with its balloon inflated to dislodge the object.
553
Q

Patient with a peritonsillar abscess - needle aspiration?

A

Yes, think of it as an abscess that needs both drainage and diagnosis, but happens to be in the mouth

554
Q

What is empiric treatment of peritonsillar abscess in children?

A

Clinda and Cefotaxime

555
Q

Presentation of peritonsillar abscess?

A

A peritonsillar abscess in a child may present with a severe sore throat (sometimes unilateral), fever, muffled or “hot potato” voice, and trismus. Patients may have drooling and decreased oral intake because of odynophagia.

556
Q

What illnesses place premature infants at risk for apnea?

A

Viral bronchiolitis

557
Q

What are the GI manifestations of ascaris lumbricoides?

A

TIP: They are asymptomatic in most cases, but when they are symptomatic, they have a wide range of GI manifestations:

  • abdominal pain
  • abdominal distention
  • intestinal obstruction
  • biliary obstruction- colic, cholangitis, pancreatitis
  • intestinal perforation
  • peritonitis
558
Q

What is “oral communication method”?

A

The oral communication method uses the child’s residual hearing, speech, and lipreading to develop spoken language and thus facilitate participation in mainstream environments.

In short, the goal of oral communication method is to help the child develop spoken language with what residual hearing he has left

559
Q

What is the presentation of pseudo tumor cerebri?

A

Following symptoms occur at least 50% of the time for patients:

1) Headache
2) Visual changes, especially visual darkening
3) Pulsatile tinnitus
4) Dizziness
5) Back pain

560
Q

What is normal opening pressure in children?

A

Normal cerebrospinal fluid opening pressure in pediatric patients is between 10 and 28 cm H2O

561
Q

Medications associated with pseudo tumor cerebri?

A

Tetracyclines (eg, doxycycline and minocycline), excess vitamin A ingestion (including medications that are vitamin A derivatives such as isotretinoin), and growth hormone.

562
Q

If a mother has a wild-type RB1 allele and one mutated RB1 allele, what are the chances of the child developing retinoblastoma?

A

25% to 50%; around 45%

Retinoblastoma acts like autosomal dominant in the hereditary type and autosomal recessive in the non-hereditary type.

Explain:
- Hereditary: offspring receive mutated RB1 gene from parent (mutation in the ova or sperm, aka germ line mutation) and one somatic mutation silences. In this case, there is 90% penetrance. Hence, 50% chance of receiving the gene from mom, and 90% chance that the gene penetrates, that is, 0.5 x 0.9 = 0.45

  • Nonhereditary: Child is born with 2 normal RB1 genes, but has 2 somatic mutations in life so that he has 2 abnormal RB1 alleles
563
Q

What are the lab findings of Thyroid-binding globulin deficiency?

A

Thyroid-binding globulin deficiency is associated with:

  • normal TSH
  • normal free T4 levels
  • low total thyroxine
  • low thyroid-binding globulin

Clinical tip: in reality, I will likely NOT diagnose this as most thyroid disease work up dose NOT include total thyroxine. Since, TSH and Free T4 are normal, I would rarely know that a patient has thyroid binding globulin deficiency.

Total T 4 is low because TBG is low

564
Q

What is the only medication to prevent RSV bronchiolitis? What are the qualifications for it?

A
  • Palivizumab
  • Born < 28 weeks + 6 days
  • Hemodynamically significant congenital heart disease
  • Chronic lung disease of prematurity
565
Q

Next step in mgmt in a patient receiving Palvizumab found to be RSV positive?

A

Stop Palivizumab

566
Q

What is the seasonality of RSV infections?

A

There is a distinct seasonality to RSV infections. Although the pattern varies by region, outbreaks usually begin in November or December. The peak usually occurs 2 months into the season and ends by March or April.

567
Q

How to distinguish between tinea corporis and granuloma annulare?

A

Granuloma annulare may be differentiated from tinea corporis by the absence of scale, the firmness of the border, and tendency of lesions to have a violaceous color

Because the pathology of GA occurs in the dermis, lesions have a “deeper” or “firmer” feel than the border of a tinea corporis lesion

568
Q

What are the areas of learning disability?

A

Math - Dyscalculia
Reading - Dyslexia
Writing - Dysgraphia

569
Q

What is the definition of intellectual disability according to IQ scores?

A

2 SD below the mean

Each SD is 15

Hence IQ <70 is considered ID

570
Q

What is the definition of intellectual disability according to IQ scores?

A

2 SD below the mean

Each SD is 15

Hence IQ <70 is considered ID

571
Q

What is standard medical treatment for non-displaced sinus fractures?

A
  • 5 days of Augmentin
  • Sinus precautions: avoidance of nose blowing, swimming, straw use, and playing wind instruments should be put in place.
  • Follow up ENT 1 week
572
Q

When developing a tool, such as a diagnostic test, what is the definition of valid vs reliable? What is the relationship between the two?

A

Reliability is the ability of a tool to give consistent results on repeated trials

Validity is the ability of a tool to measure what was intended. For example, a personality test that scored an outgoing individual as an introvert would be a invalid test.

If an instrument is valid, it should be reliable. But, not all reliable instruments are valid.

573
Q

Cryptosporidium PICMONIC

A
  • Associated with zoonotic (livestock, especially preened calves), recreational water sources (untreated like lakes and treated like pools) and consumption of meals from farms and gardens
  • Eval with direct immunofluorescence Ab or Ag testing x 3 in the stool

Floating coffin, mushrooms growing in it, being grazed by cow, sitting on top is Zangief; floating flower pots as well

574
Q

What is the inheritance pattern of NF1 vs NF 2?

A

Neurofibromatosis type 2, neurofibromatosis type 1, and tuberous sclerosis are neurocutaneous disorders with autosomal dominant inheritance

575
Q

What are the associated tumors of NF2?

A

Bilateral vestibular schwannomas that result in balance dysfunction, tinnitus, and hearing loss typically in the adolescent or early adult years.

Other tumors include meningiomas, ependymomas, astrocytomas, and schwannomas of other cranial and peripheral nerves as seen in this boy’s mother

576
Q

Diagnosis of Entamoeba Histolytica?

A

Serum antibodies to Entamoeba histolytica are detectable in 95% of individuals with invasive amebiasis

577
Q

Treatment of E. Histolytica?

A

Metronidazole and intraluminal agent (Paromamycin, Iodoquinol or Dilaoxanide)

COOL: intraluminal agent since E. Histolytica resides in the large intestine and produced most of its symptoms there.

578
Q

Intrapartum antibiotics for prophylaxis for GBS is considered adequate if given at what time?

A

> 4 hours prior to delivery

TIP: think that you need time for the antibiotics to kicki in

579
Q

In regards to GBS and GBS prophylaxis, which new borns can be discharged at 24 hours?

A
  • Well-Appearing, >37 weeks, GBS negative mothers OR GBS positive BUT received adequate IAP
  • All others discharge at 48 hours at least
580
Q

What is the management of a premature infant born to a mother with inadequately treated GBS prophylaxis?

A

Asymptomatic neonates born before 37 weeks of gestation whose mothers received inadequate group B Streptococcus prophylaxis may be evaluated with a complete blood cell count and blood culture, and should be monitored in the hospital for 48 hours before discharge.

581
Q

What is the relationship between hypospadias and disorders of sex development?

A

Hypospadias can occur as a result in disrupted masculinization, hence a sign of disorder of sexual development

582
Q

In what age group does transient erythroblastopenia usually occur?

A

This condition usually affects children between 18 and 26 months of age

583
Q

What patients with flail chest need intubation?

A

Any patient with respiratory distress

584
Q

Explain why pediatric patients are at higher risk of internal organ damage from chest wall trauma compared to adults.

A

The pediatric chest wall is much more pliable than the adult chest wall, therefore providing less protection from blunt traumatic forces. In children, energy forces applied to the thorax are likely to be transferred through the chest wall to the underlying organs, resulting in internal injuries such as pulmonary contusions without evidence of external injuries (such as rib fractures).

585
Q

What are the expected ultrasound findings of Multicystic Dysplastic Kidney Disease?

A

The classic findings on renal ultrasonography include UNILATERAL multiple noncommunicating cysts of varying sizes with intervening dysplastic renal tissue.

586
Q

What is the difference between ARPKD and ADPKD in regards to timing of presentation?

A

ARPKD presents normally in childhood

ADPKD normally presents in adulthood

587
Q

What is the most common cystic kidney disease in childhood?

A

Multi cystic Dysplastic Kidney Disease (basically non-functioning unilateral kidney)

588
Q

Benefits of early discussions about sexual health between parents and children?

A

Studies have shown that adolescents of parents who communicate early on about decision making and sexual behavior often have a delay in sexual debut, use a contraceptive method when they do become sexually active, and have fewer sexual partners

589
Q

Framework Question:

What are varicoceles?

A

A varicocele is an abnormal collection of tortuous veins in the pampiniform plexus surrounding the spermatic cord in the scrotum

590
Q

What are concerning findings that a varicocele is secondary?

A
  • Secondary varicoceles are do not vary in size from supine to standing
  • Right sided varicoceles (It is important to remember that 85% to 95% of idiopathic varicoceles occur on the left side)
591
Q

Main presentation hypopituitarism in the newborn?

A

Hypoglycemia is the predominant presenting symptom of congenital hypopituitarism.

592
Q

Definition of micropenis in newborn?

A

<2.5 cm phallic length stretched

593
Q

Most common cause of vertigo in children?

A

Otitis media and other middle ear conditions are the most common cause of vertigo in young children.

594
Q

Differential diagnosis of vertigo in children?

A

1) Otological complications of otitis media, including cholesteatoma, can cause vertigo as well.

2 ) Benign paroxysmal vertigo of childhood is the next most common etiology in younger children.

3) A vestibular migraine can also cause vertigo, and children with this condition will have a history of migraine but may not consistently have a headache along with episodes of vertigo.

595
Q

What findings are most closely associated with NF1?

A

> 6 CALMS (cafe au lait macules): 75% of patients end up with NF1 diagnosis

Lisch nodules (or iris hamartomas) present in 95% of patients with NF1 by age 20

596
Q

Neurofibromatosis 1 PICMONIC

A
597
Q

Framework Question:

What are the 3 questions that need to be addressed to determine what post exposure Tetanus prophylaxis a child needs following a wound?

A

1) Has the child received 3 TDaP’s? (2,4,6 most and 15-18 most, 4-6 years)
- If no: automatic needs Tetanus vaccine
2) Clean or dirty?
- < 3 vaccines/clean: Tetanus vaccine
- < 3 vaccines/dirty: ONLY scenario TIG/Tetanus vaccine
- > 3 vaccines/clean: if > 10 years since last vaccine, Tetanus vaccine
- > 3 vaccines/dirty: if > 5 years since last vaccine, Tetanus vaccine
3) How old is the child? (determines the form of tetanus the child gets)
- <7 TDaP
- 7-10 Td
- >11 Tdap

598
Q

Framework Question:

What is the cause of pityriasis alba?

A

Post inflammatory hypo pigmentation that occurs in children with eczema.

599
Q

Treatment of pityriasis alba?

A

Treatment of pityriasis alba is with an emollient or a short course (7 days) of a low-potency topical corticosteroid.

600
Q

What are characteristics that distinguish pityriasis alba from tine corporis?

A

Tinea corporis tends to have scaling, and the borders are distinct between normal and abnormal skin

TIP: fungus eating melanin

601
Q

Framework Question:

What is the relationship between severe eczema in a child and peanut allergy?

A

The expert pannel recommends that children with severe eczema, egg allergy, or both have introduction of age-appropriate peanut-containing food as early as 4 to 6 months to reduce the risk of peanut allergy

BUT! Prior to introducing peanut, the expert panel recommends evaluation with serum peanut-specific IgE (sIgE) measurement

2017 addendum to the 2010 Guidelines for the Diagnosis and Management of Food Allergy in the United States

602
Q

How should an IgE level for peanuts be interpreted?

A

A peanut sIgE of less than 0.35 kU/L has a strong negative predictive value for the diagnosis of peanut allergy. However, the expert panel emphasizes that a peanut sIgE level of 0.35 kU/L or greater lacks adequate positive predictive value for the diagnosis of peanut allergy, and infants should be referred to a specialist for further evaluation during which a supervised oral challenge may be attempted

603
Q

What are tests to evaluate if a patient may have a peanut allergy?

A

Serum IgE level for peanuts: good NPV, poor PPV
Skin prick test: performed at any age. For patients under 2 years of age, skin prick test reactions to peanuts that have wheal diameters of 8 mm or greater are almost 95% predictive of clinical reactivity

TIP: Expert panel does not recommend food allergen panel testing or the addition of sIgE testing for foods other than peanut because of their poor positive predictive value, leading to misinterpretation, overdiagnosis, or unnecessary dietary restriction

604
Q

What is the prognosis of Wilms Tumor in general?

A

Approximately 90% of children with nephroblastoma will be cured of their disease, although the prognosis varies with the age of the child, the stage and histology of the tumor, and the presence or absence of 1p and 16q loss of heterozygosity

605
Q

What does it mean to have “loss of heterozygosity”?

A

When the genomic copies derived from each parent have different bases for these polymorphic regions (SNPs) the region is said to be heterozygous. Most of the chromosomes within somatic cells of individuals are paired, allowing for SNP locations to be potentially heterozygous. However, one parental copy of a region can sometimes be lost, which results in the region having just one copy. The single copy cannot be heterozygous at SNP locations and therefore the region shows loss of heterozygosity (LOH).

606
Q

What is a cholesteatoma?

A

A collection of squamous epithelial cells in the middle ear or mastoid

607
Q

Management of cholesteatoma?

A

All children with a suspected or confirmed cholesteatoma should undergo audiologic evaluation.

Referral to ENT for removal. The recurrence rate after surgical excision is high (up to 50%), so children must have regular follow up

608
Q

What are the populations that should receive Varicella Immunoglobulin as post exposure prophylaxis?

A

Candidates who should receive varicella-zoster immune globulin as postexposure prophylaxis include:

Immunocompromised:

  • Immunocompromised children without a history of varicella disease or varicella vaccination
  • HSCT patients regardless of previous vaccine history

Pregancy:
- Susceptible pregnant women

Newborns:

  • Newborns whose mothers have signs and symptoms of chickenpox within 5 days before delivery to 48 hours after delivery
  • Hospitalized preterm infants (≥ 28 weeks of gestation) whose mothers lack varicella immunity
  • Exposed hospitalized preterm infants (< 28 weeks of gestation or ≤ 1,000 g birth weight), regardless of maternal history of varicella or VZV serostatus
609
Q

For infants born to mothers without adequate antibiotic prophylaxis during labor, what are the risk factors most strongly associated with early onset sepsis?

A
  • Prematurity (<37 weeks of gestation)
  • Post-term gestation (>40 weeks of gestation
  • Intrapartum maternal fever
610
Q

When should a newborn, born at home, need to be seen by a pediatrician?

A

Within 24 hours of birth

611
Q

Framework Question:

Recommendations for meningococcal vaccination in special population?

A

There are 2 types of meningococcal vaccines:

  • 1) Quadrivalent - AWCY
  • 2) Serogroup B

In regards to the quadrivalent (ACWY):
- All patients: 11-12 and booster at 16

Exception: special populations get it sooner

Anatomic or functional asplenia (including sickle cell disease), HIV infection, persistent complement component deficiency, or use of complement inhibitor (such as eculizumab or ravulizumab)

There are 2 types of quadrivalent vaccines available: Menveo and Menactra. They differ in how young they can be given and the total number of of doses

Special populations will need booster every5 years given persistent risk

612
Q

Should children be recommended to ride their bikes with or against traffic flow?

A

With traffic flow

613
Q

What is the AAP stance in regards to tanning beds?

A

Recommends against tanning salons

614
Q

What is the difference between UVA and UVB? What is the relationship to sunscreen SPF?

A
UVA = A for aging 
UVB = B for burns/block 

SunBlock only Blocks B, that is, UVB. SPF is the rating for how well sunblock protects against UVB, NOT UVA

615
Q

What are AAP recommendations in regards to sun exposure to infants?

A

Infants younger than 6 months should avoid direct sunlight, stay in shaded areas, wear a protective hat and clothing, and have sunscreen applied to unprotected sun, including face and hands, when sun exposure is unavoidable

616
Q

What is the difference between time of PDA closure between term and preterm infants?

A

The “normal” course for a PDA in a term neonate is closure within 72 hours of birth.

In preterm neonates, this course is delayed, and the PDA remains open in 10% of neonates born at 30 to 37 weeks’ gestation, 80% born at 25 to 28 weeks’ gestation, and 90% born at 24 weeks’ gestation.

By 7 days after birth, these rates decline to 2%, 65%, and 87%, respectively.

617
Q

Can persistently open PDA lead to clinical signs?

A

Yes, as the pulmonary vascular resistance drops, the amount of blood being shunted away from the body increases and in turn the amount of blood being shunted to the lungs increases.

This leads to pulmonary overcirculation resulting in pulmonary congestion, edema, and worsening respiratory status.

618
Q

Preterm with PDA - to close or not to close?

A

Controversial: Prolonged presence of a PDA has been associated with prolongation of mechanical ventilation, bronchopulmonary dysplasia, increased mortality, pulmonary hemorrhage, necrotizing enterocolitis, impaired renal function, and intraventricular hemorrhage. However, no study has shown that closing a PDA improves long-term outcomes.

619
Q

Framework Question:

What is the normal degree of passive neck rotation and lateral flexion for children (<3)?

A

Children younger than 3 years have a normal passive neck rotation of about 100° and a lateral flexion of about 70° on each side

If NOT, then torticollis

620
Q

Connection between plagiocephaly and torticollis?

A

Congenital muscular torticollis is a major risk factor for positional plagiocephaly; likewise, positional plagiocephaly is a major risk factor for CMT

TIP: Test for torticollis in ALL patients with plagiocephaly.

621
Q

What sinus is usually implicated in orbital cellulitis?

A

Ethmoid sinus

622
Q

Empiric antibiotics for orbital cellulitis?

A

No real regimen preferred

Generally, coverage should include Staph aureus, Streptococci, organisms of sinusitis and possibly anaerobes

Ex:

  • Third generation cephalosporin + Clinda (or Metro)
  • Simply Zosyn
623
Q

Characteristic presentation of orbital cellulitis?

A

The clinical manifestations of orbital cellulitis include proptosis, ophthalmoplegia, and pain with eye movement

624
Q

What is conversion disorder?

A

Functional neurologic symptom disorder that doesn’t correlate with the known pathophysiology of the medical or neurologic condition

625
Q

What is the gold standard of diagnosing PNES?

A

Video electroencephalography (EEG) is the gold standard test for this diagnosis and will have normal findings at the time of the apparent seizure behavior

626
Q

What are some presentations consistent with PNES as opposed to seizures?

A

Forward pelvic thrusting, side-to-side head or body movements, closed eyes resistant to opening, and a lack of postictal confusion are more often seen in PNES

627
Q

How does drowning lead to cardiac arrest in children?

A

The moment the airway is submerged below the surface of the water, reflex laryngospasm is initiated by the contact of liquid with the larynx. As the larynx relaxes after a period of hypoxia and hypercarbia, water is aspirated into the lungs. When the water reaches the alveoli, hypoxia occurs due to ventilation-perfusion mismatch. The most important consequence of drowning is decreased oxygen delivery to the tissues. Decreased myocardial oxygen delivery can result in asphyxial cardiac arrest

628
Q

What are possible complications of bicuspid aortic valves?

A

BAV can develop regurgitation, stenosis, endocarditis, and aortopathy, and therefore need follow-up throughout life.

629
Q

What size VSDs will need surgical closure?

A

Moderate to large muscular and perimembranous VSDs

630
Q

Connection between Rotavirus vaccine and intussusception?

A

Slight increase risk of intussusception

TIP: Reason we give RV so young is that at older ages patients are at higher risk of intussusception. So there is an age limit to receiving RV.

631
Q

Framework Question:

Explain transfusion associated graft versus host disease

A

Small lymphocytes are the same size as red blood cells and will therefore not be removed through leukodepletion. When presented with a foreign HLA type, lymphocytes (from donor or recipients) are activated and undergo massive replication.

Blood products are matched for red blood cell surface antigens (A, B, and O), not HLA types.

The immune system of an immunocompetent recipient will recognize HLA-mismatched donor lymphocytes as foreign and eliminate them. However, the immune system of an immunocompromised recipient may be unable to eliminate the donor lymphocytes. In this situation, the donor lymphocytes may recognize the HLA-mismatched host as foreign, undergo massive replication, and cause transfusion-associated graft-vs-host disease (GVHD).

Irradiation of a blood product will damage the DNA of donor lymphocytes, thereby rendering them replication incompetent and definitively eliminating the risk for transfusion-associated GVHD.

632
Q

What are the most common organs involved in graft versus host disease?

A

Skin/GI/Liver are the MOST common organs involved

633
Q

What is the difference between stereotypy and tics?

A

In contrast to tics, there is no internal compulsion in stereotypy to do the movement or sense of relief afterwards.

634
Q

What are the treatment of tics?

A

The 2 medications approved by the Food and Drug Administration for use in tics are haloperidol and pimozide. The side effects of these medications often outweigh the benefit. Many providers first try an α2-adrenergic agonist, such as clonidine or guanfacine, but this is an off-label use. Another treatment for tics is comprehensive behavioral intervention for tics (C-BIT), a behavioral technique or therapy taught by psychologists or other behavioral health specialists that has been shown to reduce tic severity.

635
Q

Difference between tics and tourettes?

A

1 year of symptoms

636
Q

At what age should transition of care start?

A

Discussions regarding transition of care for adolescents and young adults with chronic medical conditions should be started around age 12 years

637
Q

Framework Question:

What are the 2 triggers to ADH release?

A

Release of AVP is induced by hyperosmolality detected by osmoreceptors in the hypothalamus and by low cardiac output detected by baroreceptors located in the carotid sinus, aortic arch, and left atrium.

638
Q

What is the expected urinary findings for a patient with SIADH?

A

High urine sodium for the degree of hyponatremia

639
Q

What are the 5 subtypes of JIA?

A
  • Oligoarthritic JIA
  • Polyarthritic JIA
  • Systemic JIA
  • ERA - enthesitis related arthropathy
  • Psoriatic arthritis
640
Q

What is the presentation of Systemic JIA?

A

TIP: Systemic JIA = JIA + systemic signs

  • Arthritis in ≥ 1 joints (> 50% of arthritis is destructive)
  • Fever
  • Rash
  • Lymphadenopathy
  • Hepatosplenomegaly
  • Serositis
641
Q

What is the recommendation in regards to polio vaccination in the US?

A

4 doses of inactivated polio vaccine:

  • at ages 2, 4, and 6 to 18 months
  • 4 to 6 years

TIP: “2,4,6 and 4-6”

TIP: imagine 4 polo players on horses and in the iron lung machine

642
Q

Do patients traveling to polio endemic countries require vaccination for polio?

A

YES, in the case of stays > 4 weeks

Adults: 1 dose even if 4 doses already obtained

Children: 1 dose IF last dose > 12 months before trip departure. Children do NOT need to have all 4 doses prior to traveling to a polio endemic country

643
Q

What is the threshold of polycythemia in children that requires intervention?

A

Neonates should be treated with partial exchange transfusion if

  • they are symptomatic with a Hct greater than 60%
  • Hct greater than 70%, whether symptomatic or not.
644
Q

What are risk factors of polycythemia in newborns?

A
  • Delivery in high-elevation areas
  • gestational age of >40 weeks
  • small size for gestational age
  • maternal diabetes
  • hypertension
  • history of cigarette smoking
  • trisomy 13, 18, or 21
  • neonatal Graves disease and hypothyroidism
  • congenital adrenal hyperplasia
  • cyanotic congenital heart disease.
645
Q

Definition of polycythemia in children?

A

Polycythemia is defined by a hematocrit (Hct) greater than 65% on a sample obtained from a vein or central vessel.

TIP: Samples obtained by heel stick often have a falsely elevated Hct.

646
Q

What are characteristics of pediatric melanoma?

A

ABCDE Rule!

A = Amelanotic: Most amelanotic melanomas (70%) will appear pink or red and may mimic a pyogenic granuloma or be skin colored like warts
B = Bleeding, bump: Lesions often are papules or nodules that may ulcerate or bleed, not flat lesions.
C = Color uniformity
D = De novo (that is, do not arise from previously existing melanocytic nevi), any diameter
E = recent evolution

647
Q

What does “Diabulimia” refer to?

A

Intentional insulin omission for weight loss

648
Q

How to diagnose CHARGE syndrome?

A

4 major criteria
OR 3 major/3 minor criteria

TIP: ChArgE

Major criteria:

  • Coloboma
  • Atresia, choanal atresia
  • Ear anomalies
  • Cranial nerve dysfunction
649
Q

What is the inheritance pattern of CHARGE syndrome?

A

Autosomal Dominant

650
Q

What signs are almost always present in septic arthritis for children?

A

Fever and limited range of motion

651
Q

What tool can aid in distinguishing between transient synovitis and septic arthritis?

A

Modified Kocher criteria: (each criteria leads closer to the diagnosis of septic arthritis)

  • Fever > 38.5
  • ESR >40
  • CRP > 25
  • WBC >12
  • Inability to ambulate
652
Q

Patient with myopia would like to join baseball. What is the definition of “one-eyed”, that is, functionally dependent one eye?

A
  • Athletes are considered functionally 1-eyed if they have corrected vision of worse than 20/40 in 1 eye.
653
Q

Children that are considered “one-eyed” should not participate or need to wear protective goggles in which sports considered high risk for eye injury?

A

Baseball, basketball, hockey, lacrosse, and racket sports are considered high risk for eye injury.

654
Q

What vehicle of measuring out liquid medications has the LOWEST risk of medication error?

A

Syringe

655
Q

Framework Question:

What are symptoms of malabsorption?

A

Bloating, cramping and diarrhea

TIP: All related to osmotic load and increased distention in the intestines

656
Q

What ethnicities are at highest risk for primary lactase deficiency?

A

African Americans, Native Americans, and Asian Americans

657
Q

What are the symptoms of Bipolar Disorder?

A

DIGFAST

D - Distractible
I - Impulsivity 
G - Grandiosity 
F - Flight of ideas
A - Increase activity
S - Sleep
T - Talkative
658
Q

Difference between Bipolar 1 and Bipolar 2?

A

TIP:

  • Bipolar 1: just 1 manic episode
  • Bipolar 2: 2 things - 1 hypomanic episode and 1 major depressive episode

Hence, the presence of full mania precludes bipolar 2. Mania and hypomania easily distinguished because 1 needs hospitalization or 4 days worth

659
Q

What is cataracts?

A

A cataract is a lens opacity that may occur at any age

PICMONIC: Cat stuck in a lens

660
Q

Most common presentation of superior vena cava syndrome in children?

A
Facial edema
Prominent superficial chest veins
Cough
Wheezing
Shortness of breath
Stridor 

TIP: Note the prevalence of respiratory symtpoms

661
Q

What are the common organs involved in Alport’s syndrome?

A

Kidneys (always)
Ears (often)
Eyes (occasionally)

662
Q

What is the prognosis in regards to kidney function for children with Alport’s syndrome?

A

Male patients fare poorly, with progression to renal failure by age 20 years (juvenile type) or 40 years or later (nonprogressive or adult type).

663
Q

What is the cause of Alport syndrome?

A

It is an X linked basement membrane disease a collagen Type 4 defect

Genes encoding for these chains have been identified most commonly on chromosome Xq22 (COL4A5, COL4A6)

664
Q

What is the difference between hypoxia and hypoxemia?

A
  • Hypoxia is the decreased delivery or supply of oxygen
    Ex: Sepsis can lead to hypoxia without hypoxemia being present
  • Hypoxemia is a low partial pressure of O2 in the blood
665
Q

What is the relationship between CO2 levels and intracranial pressure?

A

Hypercapnia can lead to vasodilation of the cerebral vasculature, and subsequent elevation in cerebral blood flow, cerebral blood volume, and intracranial pressure.

TIP: think hypercapnia is interpreted by the body as impending hypoxia, so it increases blood flow to the brain

666
Q

What is amblyopia?

A

Amblyopia, or reduced visual acuity due to abnormal visual development early in life, is the most common cause of visual impairment in children. It can be caused by strabismus, refractive errors that differ significantly between the 2 eyes, or by interruption of the visual axis

667
Q

What is the most common cause of blindness in childhood?

A

Amblyopia

668
Q

What what age should gaze be conjugate?

A

6 months, hence normal to be dysconjugate prior, but abnormal if present after. Needs referral to Ophtho

TIP: Straight eyes at Six months

669
Q

What are the 5 causes of amblyopia?

A

5 types of amblyopia classified by cause

  • strabismic amblyopia - due to misalignment of eyes
  • stimulus deprivation amblyopia - due to obstacle in anterior visual pathway e.g. one eye with cataracts or ptosis
    - anisometropic amblyopia - due to difference in refractive (focusing) error of both eyes
    - ametropic amblyopia - significant refractive error of both eyes
    - meridional amblyopia - astigmatism of both eyes
670
Q

What is astigmatism?

A
  • Abnormal curvature of the cornea or lens leading to inability to focus, which in itself can lead to refractive errors

TIP: you can have refractive errors not related to astigmatism

671
Q

What organism is implicated in epidemic cases of keratoconjunctivitis?

A

Adenovirus

Actually called “adenovirus associated epidemic keratoconjunctivitis”

672
Q

What is the presentation of adenovirus keratoconjunctivitis?

A

Symptoms include significant PAIN, PURULENT discharge, PHOTOPHOBIA, and blurry vision.

Sensation of something on the eye.

Preauricular lymphadenopathy and subconjunctival hemorrhage may be noted.

Symptoms are based on the fact that the cornea is involved. Just as in corneal abrasion, pain, sensation of foreign body, tearing and photophobia are present

673
Q

What part of the limbs are affected in amniotic band syndrome?

A

Distal limbs are affected and proximal limbs are normal

674
Q

What is the cause of amniotic band syndrome

A

Either constrictive bands of amniotic tissue of ischemic insult

675
Q

What are the findings consistent with oligohydramnios syndrome or sequence?

A

Oligohydramnios sequence describes pulmonary hypoplasia, growth restriction, compressed facies, and abnormal positioning of the hands and feet in neonates born to mothers with low amniotic fluid levels

676
Q

During what phase of respiration does vocal cord dysfunction have the most effect?

A

Inspiration

Hence, VCD is on the differential diagnosis of inspiratory stridor

677
Q

What are characteristic findings on a volume flow loop consistent with VCD?

A

Flattened inspiratory arm of the flow-volume loop

678
Q

What are characteristics of a dermoid cyst?

A

Firm, noncompressible, slowly growing FLESH COLORED nodules located most often on or near the LATERAL EYEBROW, present since BIRTH (congenital)

679
Q

What is the connection between meningitis and dermoid cysts?

A

Lesions involving the nose or midline scalp may be associated with intracranial extension, and lesions over the spine may be associated with spinal dysraphism

All patients who have midline dermoid cysts or sinuses should undergo imaging

680
Q

What is the difference between thyroid nodules and their association with cancer between children and adults?

A

Thyroid cancer occurs in about 25% of pediatric thyroid nodules, vs about 5% in adults.

681
Q

What si the most common thyroid cancer in children ? Adults?

A

Papillary thyroid carcinoma, the most common thyroid cancer type in both children and adults, is more aggressive in childhood than adulthood but survival is better

682
Q

What is the inheritance pattern of MEN 2A and 2 B?

A

Autosomal dominant

TIP: Men think they are dominant, especially in groups - hence MEN 2 and NOT MEN 1

683
Q

Connective Tissue Disease PICMONIC

A

Mr Fantastic from Fantastic 4

Rubber band ball

Gumby

684
Q

What are the main organs involved in Marfans?

A
  • Cardiovascular:
    • MVP, TVP
    • Aortic root dilation, Proximal pulmonary artery dilation
      TIP: BOTH systemic and pulmonary systems are affected, like mirror images, so not only aortopathy, but pulmonary arteriopathy, not only MVP, but also TVP@
  • Ocular:
    • Len dislocation, glaucoma, cataracts, retinal detachment, myopia
  • Skeletal:
    • Pectus excavatum/carinatum, scoliosis, arachnodactyly
685
Q

Marfans PICMONIC

A
  • Arachnodactyly/bone manifestation - spider made up of bones
  • Heart involvement - ***
  • Connective tissue disease - Mr fantastic riding spider
  • Ocular manifestations - each leg pierces an eyeball
  • Autodominant - monster truck
686
Q

Why is Loeys Dietz on the differential diagnosis of Marfans? Distinguishing features?

A
  • MANY overlapping feature and they both are autosomal dominant
  • Distinguishing features
    • hypertelorism, bifid uvula, craniosynostosis, cleft palate, and generalized arterial tortuosity along with dissections throughout the ENTIRE arterial tree.
687
Q

How to counsel a parent in regards to the use of melatonin in their autistic child to aid in sleep?

A

Initial dosing is 1 to 3 mg given 30 to 60 minutes prior to bedtime.

The dose can be increased by 1 to 3 mg every 1 to 2 weeks up to a maximum dose of 10 mg.

If there is no response after titrating to 6 mg per night, higher doses are unlikely to be helpful.

688
Q

How many doses of Tetanus vaccine is recommended in childhood? Schedule?

A

In childhood, a total of 6 doses of pertussis-containing vaccine are recommended:

  • DTaP in a 5-dose series, typically at ages 2, 4, 6, and 15 to 18 months as well as 4 to 6 years
  • 1 dose of Tdap at 11 to 12 years

TIP:

  • Don’t confuse with IPV
    • IPV: 2,4,6 mos, then 4-6 years
    • DTaP: 2, 4, 6 mos, then 15-18 mos, then 4-6
689
Q

Framework Question:

What is the long term morbidity that accompanies benign bone lesions and determines the regularity of surveillance?

A
  • Risk for pathologic fractures

Ex:

  • Non ossifying fibromas: low risk for pathologic fractures
  • Non ossifying fibromas >50% of cortex or diameter: risk of pathologic fractures
  • Fibrous dysplasia: : risk of pathologic fracture
690
Q

Radiographic findings of non-ossifying fibromas?

A

Small, well-defined radiolucent cortical lesion with a surrounding rim of sclerosis

691
Q

Most commonly used date rape drug?

A

Alcohol remains the most common date rape drug

692
Q

What are ways to diagnose suspected Giardia?

A
  • Direct microscopic examination of a diarrheal stool (ova and parasite examination) has a sensitivity of 75% to 95%.
  • Specific enzyme immunoassay has a sensitivity of 95% and specificity of 98% to 100%
693
Q

What are the historical details or presentation consistent with a malabsorptive diarrhea?

A
  • steatorrhea, gas, and/or bloating

TIP: Malabsorption is quite the large umbrella terms.It can encompass carbohydrate malabsorption, which would be categorized under osmotic diarrhea. It could encompass fat malabsorption from pancreatic insufficiency. It could encompass bile acid malabsorption which would be categorized under the secretory diarrhea

694
Q

What is the preferred psychotherapy for PTSD in children?

A

Trauma focused CBT

695
Q

What is the Langley Model of Improvement framework in regards to developing an idea to improve efficiency?

TIP: think of a personal example in regards to admitting patients

A

The Langley Model of Improvement is a framework for quality improvement that addresses 3 questions:

1) What are we trying to accomplish?
- Increased efficiency in admitting direct admission patients
2) How will we know that a change is an improvement?
- Decrease the total time of the admission process from start to finish to 1 hour maximum
3) What changes can we make that will lead to an improvement using multiple, rapid Plan-Do-Study-Act cycles.
- Reviewing records: 2) Review records
- Interview/Med rec/Examine patient: 4)
- Note: 5)
- Problem list: 3) List problems
- Admission orders: 1) Place admission orders and order set first (except for home meds)

696
Q

Explain the anatomy of the Roux En Y procedure

A

The most common type of bariatric surgery is the Roux-en-Y gastric bypass, during which the surgeon creates a small gastric pouch (about 1 ounce in size, like a newborn!).

The small intestine is then divided into 2 portions.

The Roux limb, which connects the small gastric pouch to the jejunum, is then attached to the bottom portion of the duodenum, creating the Y connection

TIP: ultimately there are gastric contents entering the JEJUNUM at 2 entrances: Gastric to jejunum; distal duodenum to jejunum (at the Y portion)

697
Q

What are the symptoms of zinc deficiency?

A

Symptoms of mild zinc deficiency include taste and smell impairment, night blindness, and depressed immunity.

Severe zinc deficiency is associated with alopecia, bullous pustular dermatitis, diarrhea, and frequent infections due to a depressed immune system

TIP: Severe zinc deficiency presents with mucocutaneous manifestations

698
Q

What are the micronutrient deficiencies associated with bariatric surgery?

A
  • Zinc
  • Copper
  • Iron
  • Vit D
  • Calcium
699
Q

Difference between Fanconi Anemia and TAR in regards to limb abnormalities?

A

TAR - affects the radius bilaterally

Fanconi - affects the THUMB, hands, wrists

700
Q

Does ingesting household bleach lead to corrosive injury of the esophagus?

A

Although household bleach (sodium hypochlorite) is a substance with an alkaline pH (pH ~12), retrospective studies of children ingesting liquid household bleach have found that accidental ingestion is typically associated with a benign clinical course and does not require hospitalization or pharmacologic interventions

701
Q

Explain how obtaining a gas from a central line for a patient being treated for cardiogenic shock can help estimate how cardiac output is improving?

A

Basic underlying concept: Oxygen consumption is directly related to cardiac output. In low cardiac output states, for example, tissue will extract more O2, to compensate physiologically. Hence, O2 sat from the arterial to the venous side will demonstrate greater difference given more extraction. Hence the SaO2 – SmvO2 is an indirect measure of cardiac output.

High difference: low cardiac output
Low difference: sepsis (hyperdynamic state)
20-30%: normal cardiac output (since normal SmvO2 = 70, hence difference is between 20-30%)

702
Q

When attempting to determine patient’s cardiac output, where should venous gas be measured?

A

GOLD standard: pulmonary artery from PAC

2nd best: Internal jugular vein

Antecubital fossa/capillary stick/radial artery are NOT representative of the whole body venous return

703
Q

What is the difference between TEN/SJS?

A

The extent of skin/mucous involvement

  • <10%, SJS
  • 10-30%: SJS/TEN overlap
  • > 30%: TEN
704
Q

Most common trigger of SJS/TEN in children?

A

Medications

Clinical tip: If no identifiable meds, evaluate for underlying infection as a trigger: Mycoplasma, CMV, HSV and HAV

705
Q

What is the skin manifestation of SJS/TEN?

A

Macules with purpuric centers that evolve into vesicles and bullae

706
Q

In what period in a child’s life is the “crossing” of height percentiles considered pathologic?

A

After age 3 AND outside of the peripubertal period

707
Q

When evaluating a patient with concerns for short stature, what aspect of the growth chart can suggest an underlying endocrine or genetic disorder as the cause?

A

Decreased height velocity, BUT preserved weight

708
Q

How to calculate the predicted final height of a child?

A

Use the midparental height equation:

For girls: [(Dad’s height -5 inches) + mom’s height]/2

For boys: [(Mom’s height + 5 inches) + Dad’s height]/2

The final height is within 2 inches of the calculated height (that is, 1 SD)

709
Q

When evaluating a patient with concerns for short stature, what aspect of the growth chart can suggest familial short stature?

A

Normal growth velocity along the a percentile consistent with an individual’s genetic growth potential

How:

1) Calculate midparental height
2) Find the percentile that corresponds to parent’s height
3) Compare to the percentile of the child’s current height (normally, will be within 2 percentiles in the setting of familial short stature)

Clinical Tip:

  • Height Velocity is a key point of narrowing the differential
    • Normal height velocity: Familial short stature and constitutional delay
710
Q

How to calculate growth velocity?

A

2 separate points of growth 3 months apart (ideally 6-12 months apart)

711
Q

When evaluating a patient with concerns for short stature, what aspect of the growth chart can suggest constitutional delay of growth?

A

Normal growth velocity along a percentile below expected individual’s genetic growth potential

712
Q

What is a palpebral fissure?

A

The palpebral fissure is the elliptic space between the medial and lateral canthi of the two open eyelids.

713
Q

3 required cardinal features of Fetal Alcohol Syndrome?

A

Growth deficiency
Abnormal Facies
CNS problems

714
Q

What are epicanthal folds?

A

Epicanthal folds are the folds of skin that run from the upper eyelid to the inner corner of the eye, sometimes covering the medial canthus

715
Q

What are possible long term morbidity that a newborn with preauricular pit or tag may be at risk for?

A

5x at risk for permanent hearing loss compared to the general population

716
Q

What are lab abnormalities present in RMSF?

A

Thrombocytopenia, Hyponatremia and elevated liver enzymes occur in over half of the cases of RMSF

717
Q

Outside of the US, where else do infections from Rickettsia Rickettsii occur?

A

Central and South America

718
Q

Where in the US are most RMSF infection occurring?

A

South central US and Midatlantic

North Carolina, Tennessee, Oklahoma, Arkansas, and Missouri is where > 60% of cases occur

719
Q

When should RMSF be suspected?

A

Whenever encountering the illness script involving

FEVER
RASH
HEADACHE

720
Q

How should the foot of a newborn appear that is consistent with talipes equinovarus?

A

High arch
Adducted forefoot
Hindfoot varus
Plantar flexion contracture – unable to dorsiflex on exam

TIP: Varus = supination or inversion

721
Q

How to distinguish between metatarsus adductus from talipes equinovarus?

A

Metatarsus adductus – ankle motion is normal

While TEV patients have fixed, plantar flexed ankles

722
Q

Most common type of colonic polyp in children?

A

Juvenile

TIP: funny that the most common polyp in “children” is the “juvenile” one

723
Q

What are the disorders under the category of “polyposis syndromes”?

A
  • Juvenile Polyposis Syndrome (JPS) (hamartomatous polyps)
  • Peutz Jeghers Syndrome (hamartomatous polyps)
  • PTEN Hamartomatous Tumor Syndrome
  • Lynch Syndrome (adenomatous polyps)
  • FAP (adenomatous polyps)
724
Q

How many colonic polyps are needed to qualify as Juvenile Polyposis Syndrome?

A

5

725
Q

What should the expected finding be when testing plantar reflex (Babinski)?

A

Normal: toes down
Abnormal: toes upward

TIP: Toes point to the brain because there is problem. OR toes go up, upper motor neuron

726
Q

What is the AAP recommendation in regards to screening for lead toxicity?

A

Screen at 12 and 24 months for children

  • Eligible for Medicaid
  • Receiving government assistance
  • Live in high risk environments

Upon arrival for all refugees

727
Q

What newborn blood type is at the LOWEST risk of hemolytic disease of the newborn because of incompatibility

A

O-

They have NO antigens to be immunogenic

728
Q

Newborn presents with jaundice and severe anemia. Labs to order to evaluate for hemolytic disease of the newborn?

A
CBC
Total bili
Direct/Indirect 
Reticulocyte
DAT – shows presence of Ab on RBC
729
Q

Framework Question:

Think of hemolytic disease of the newborn as another kind of transfusion reaction. The mother is the recipient and she received pRBC that were unmatched. The newborn is the unmatched pRBC

A

TIP:

Think of hemolytic disease of the newborn simply as transfusion of mismatched blood products

EX: If pregnant patient experiences maternal-fetal hemorrhage, it is as if mother has received a small transfusion from the fetus. If the ABO or Rh are incompatible, there can be a transfusion reaction

730
Q

What is the difference between hemolytic disease of the newborn in ABO incompatibility and Rh incompatibility?

A

Hemolysis caused by ABO mismatch does not need a prior exposure

731
Q

What population is epistaxis suggestive of child abuse?

A

Preambulatory children

732
Q

What is the diagnostic criteria of SLE?

A

4/11

Skin:
1)	Malar rash – ACLE
2)	Photosensitive rash – SCLE
3)	Discoid lupus – CCLE
4)	Oral ulcers 
Serosal 
5)	Pericarditis/Pleuritis 
Hematologic
6)	Anemia/Thrombocytopenia/Leukopenia
Kidney:
7)	Nephritis 
CNS
8)	Psychosis/Seizures 
Joint
9)	Non-erosive arthritis 
Lab
10)	ANA
11)	Anti DS DNA and Anti Smith
733
Q

What nutritional deficiency is a child at risk for if provided with only a vegetarian or vegan diet?

A

B12 deficiency since that is only found in animal based products

734
Q

What is the relationship between necrotizing fasciitis and varicella infection?

A

Known association between the two diseases as varicella can act as portal entry for any spectrum of invasive group A streptococcal infection, including necrotizing fasciitis

735
Q

What does the APGAR score entail?

A
  • A: Appearance (skin color)
  • P: Pulse
  • G: Grimace (aka reflex irritability)
  • A: Activity (muscle tone)
  • R: Repirations
736
Q

What does the APGAR score tell us?

A

How the newborn is transitioning soon after birth

737
Q

When to initiate a patient on daily inhaled corticosteroids who has asthma (pediatrics)?

A

EASY: ANY severity, as long as patient fits the definition of “persistent” asthma

Out side of inhaled glucocorticoids, ALL other options are considered second-line add-on therapy e.g. leukotriene antagonist

738
Q

What is the indication to start a controller medication for a child with asthma (<12 yo)?

A

GINA Guidelines:

  • Asthma symptoms or need for SABA > 2 times/week (BEST EVIDENCE)

Other soft reasons:

  • risk for severe exacerbations, e.g. admitted to ICU or needed steroids in the past year
  • Symptoms or SABA use 2 times/month or wakes up 1/week due to asthma
739
Q

What is the presentation of lichen sclerosus et atrophicus?

A

Typical lichen sclerosus lesions are shiny, wrinkled, ivory-colored atrophic patches located in a figure-of-8 or hourglass distribution surrounding the vulva, perineum, and anus. Blisters may occur

Interesting: almost exclusively girls younger than 7 years (outside of the classic prevalence in older women)

TIP: Hourglass around the private parts

740
Q

What one syndrome should be considered in ALL girls who present with short stature concerning to be pathologic?

A

Turner Syndrome

Short stature is apparent in childhood and may be the only presenting feature. Turner syndrome should be considered in all girls with short stature.

741
Q

What is specificity and sensitivity of “surveillance” for developmental delays?

A

Surveillance is defined as a physician’s observation and physical exam of a child for developmental delay.

Known to have good specificity, but poor sensitivity.

Hence, good PPV, but poor NPV. Hence can miss developmental delay when it is there.

Hence, AAP recommendations for screening tools.

742
Q

What is the recommendation by the AAP for screening for developmental delays and autism?

A

The American Academy of Pediatrics recommends developmental screening at 9, 18, and 24 or 30 months, and screening specifically for autism at 18 and 24 months.

743
Q

Isolation precaution when caring for patient with CMV?

A

Universal precautions

744
Q

Patient with congenital vertebral anomalies should have what other organ evaluated for pathology?

A

Children with vertebral anomalies should undergo renal ultrasonography to look for underlying renal anomalies

745
Q

What is a caput succedaneum? Where is it located?

A

Caput succedaneum is localized superficial edema of the scalp most likely due to venous congestion from cervical pressure on the presenting part during delivery or the use of vacuum extraction

Between the periosteum and the skin

746
Q

Difference in the presentation and the location of Caput Succadeneum vs Cephalohematoma vs Subgaleal hemorrhage

A

Caput:

  • Between skin and above the periosteum
  • Crosses midline/sutures, discoloration

Cephalohematoma:

  • Between the skull (specific cranial bone) and periosteium
  • Does NOT cross suture lines, NO discoloration

Subgaleal :

  • beneath the epicranial aponeurosis and above the periosteum of the skull, which can extend the length of the occipitofrontalis muscle
  • fluctuant mass that increases in size soon after birth
747
Q

Loperamide in infectious diarrhea?

A

Loperamide (an antimotility agent) is not indicated in the treatment of infectious diarrhea, given the potential risk of ileus or toxic megacolon.

748
Q

By what age, can C Diff test in a child be trustworthy? (AKA colonization rates similar to adults)

A

By age 3 years, colonization rates approach adult colonization rates, which range between 0% and 3%.

749
Q

Myelomeningocele - what is on the differential diagnosis for new neurologic symptoms?

A

Tethered cord - which can present later in life

Shunt falilure

Clinical tip: Meylomeningocele, like cerebral palsy, is a static problem (does not worsen or change over time). Hence, new symptoms indicate a new problem, NOT progression of disease

750
Q

Presentation of tethered cord?

A

TIP: depend on the age group: infants vs toddlers vs adolescents vs adults

Toddler and adolescents:

  • Worsening lower extremity weakness/sensory changes
  • Worsening gait from (above)
  • Scoliosis
  • Bladder function regression
  • Back and leg pain
751
Q

What is the pathophysiology of cholesteatoma?

A

Acquired cholesteatomas, which result from a history of prolonged eustachian tube dysfunction, retraction pockets, and chronic middle ear effusion and inflammation (otitis media).

Congenital - rare

TIP: imagine recurrent inflammation that encourages replication of the epithelial cells as in healing that then leads to benign tumor

752
Q

What is the test to order to evaluate for Von Willebrand Disease?

A

The evaluation for vWD includes tests for vWF antigen and activity, which are commercially available as a test panel

753
Q

What is the most common fracture pattern in children ?

A

A greenstick-type injury is the most common fracture pattern seen in children, making up 50% of the fractures

754
Q

What is the indication for reduction of green stick fractures?

A

Greenstick fractures with angulation of more than 15 degrees require closed reduction, immobilization in a splint, and orthopedic follow-up.

755
Q

Salter Harris fractures imply involvement of what part of the bone?

A

The growth plate, that is, the physis

756
Q

What is the relationship between physis and the metaphysis and epiphysis?

A

The physis is between the metaphysis and epiphysis

757
Q

What is the difference between Salter Harris Classifications?

A

Type I S: Straight across the physis
Type II A: Above the physis
Type III L: Lower (or beLow) the physis
Type IV T: Through the physis (involves the metaphysis, physis and epiphysis)
Type V ER: Erase the growth plate, that is, Crush injury

758
Q

What is the diagnosis of orthostatic proteinuria?

A

Orthostatic proteinuria occurs when the patient is active (random urine sample) and disappears when the patient is supine/asleep for at least 2 hours (first-morning sample).

759
Q

Which type of hormonal contraceptives are contraindicated in patients with mirgraines with aura?

A

COMBINED hormonal contraceptives of any kind

760
Q

What type of IUD can last up to 10 years?

A

Copper IUD, which would be a good option for the patient who’d prefer not to be exposed to hormones.

761
Q

How can an IUD be used to treat dysmenorrhea as a secondary side effect?

A

Hormonal IUD’s have progestin in the form of Levonorgestrel. Progestin only hormonal contraceptives thin the endometrium while thickening the cervical mucosa. Thin mucosa means less bleeding/cramping, thick cervical mucosa means no sperm to penetrate

762
Q

Framework Question:

What is the difference between Estrogen and Progesterone’s effects on the endometrium?

A

Estrogen promotes the proliferation of he endometrium (e.g. higher estrogen exposure is a risk factor for endometrial cancer)

Progresterone is the opposite, in that it is anti-mitogenic, combating the effects of estrogen. It maintains the endometrium. Progresterone is also called the pregnancy hormone, hence it makes sense that it thickens the cervical mucosa to prevent any further sperm from entering

It is the withdrawal of progesterone that leads to menses

763
Q

Framework Question:

Where does progesterone come from? Role with menses?

A

Corpus Luteum and if patient becomes pregnant, the placenta takes over as the progesterone-producing factory

After every ovulation, corpus luteum produces progesterone to make the endometrial lining ready for implantation if it does occur. If implantation doesn’t occur, the corpus luteum will involute, progesterone will decrease and menses from progesterone withdrawal will occur since no hormone is left to maintain the endometrial lining

764
Q

Tip to remembering products that contain progesterone?

A

“gest” = gestation, as in, progestational which is progesterone’s purpose

Ex: Levonorgestrel, Etonogestrel

765
Q

What is the contraceptive of choice to treat patients with catamenial epilepsy?

A

Progestin-only methods are also used to treat catamenial (menstrual-associated) epilepsy and suppress menstruation in patients with physical and mental disabilities.

766
Q

What is the relationship between sedation and subglottic stenosis?

A

Inadequate sedation is a risk factor for the development of subglottic stenosis

767
Q

Differential diagnosis of supraglottic obstruction?

A

Retrognathia and adenotonsillar hypertrophy, laryngomalacia, and epiglottitis

TIP: Anything from the entrance of the mouth to the vocal cords is technically supraglottic, such as OSA

768
Q

Differential diagnosis of subglottic stenosis?

A

Subglottic obstruction can be caused by laryngotracheitis (croup), bacterial tracheitis, complete tracheal ring, laryngeal web, and subglottic stenosis

TIP: Anything affecting the trachea is automatic sub-glottic

769
Q

What is the dose/weight of iron that if consumed is unlikely to cause a child any symptoms of toxicity?

A

20mg/kg

TIP: Good to know since prenatal vitamins and iron pills have very specific dosages and a good history can lead to a good assessment

770
Q

What patient with iron ingestion should receive deferoxamine?

A

Children with severe symptoms, a significant number of radiopaque pills on radiography, or a peak serum iron concentration above 500 μg/dL (90 μmol/L) should receive intravenous deferoxamine

771
Q

How prevalent is EBV?

A

Epstein-Barr virus is a ubiquitous human γ-herpesvirus that infects more than 95% of the global population by adulthood

772
Q

How to diagnose mononucleosis?

A

1) In a patient with symptoms and signs consistent with a diagnosis of acute IM
2) the detection of more than 10% atypical lymphocytes on a peripheral blood smear
3) in conjunction with a positive heterophile antibody test result is diagnostic of acute EBV infection

773
Q

Which paitents is it receommneded that the diagnosis of mononucleosis is done via EBV specific antibodies?

A

Chilldren < 4 yo since they don’t like “heterophiles”
OR Heterophile negative IM in adolescent

Sounds like “pedophile”
Heterophiles are often absent in children < 4 yo

774
Q

The vast majority of EBV infections lead to recovery with no sequelae. What are some severe complications however?

A

Hematologic complications include hemolytic anemia, thrombocytopenia, aplastic anemia, disseminated intravascular coagulation, and hemophagocytic lymphohistiocytosis (HLH).

Neurologic complications include aseptic meningitis, encephalitis, optic neuritis, facial nerve palsy, transverse myelitis, and Guillain-Barré syndrome.

Malignancy: solid tumors (eg, Burkitt lymphoma, nasopharyngeal carcinoma) and lymphoproliferative disorders in immunocompromised patients

TIP: reason why EBV is so often tested in patients with strange presentations of any kind

775
Q

How does the causes of duodenal atresia and jejunal/ileal atresia differ in pathophysiology?

A

Duodenoal: incomplete recanalization of intestine in utero, 25% related to T21 and have other congenital anomalies

Jejunal/Ileal atresia: vascular insult leading to ischemia and infarction such as tobacco use/cocaine use; NOT associated with other congenital defects

776
Q

What is the treatment of X linked hypophosphatemic rickets?

A

Treatment of X-linked hypophosphatemic rickets is with phosphorus and calcitriol (activated vitamin D or 1,25-dihydroxyvitamin D

777
Q

Framework Question:

What is the cause of X linked hypophosphatemic rickets?

A

It is a renal phosphate wasting disease caused by defect in 1 alpha hydroxylase that prevents the production of 1,25OH Vitamin D in the face of hypophosphatemia

In unaffected individuals, low phosphorus levels stimulate 1α-hydroxylase, resulting in increased conversion of 25-hydroxyvitamin D to 1,25-dihydroxyvitamin D, the activated form of vitamin D

Presents as rickets, but “hypophosphatemic rickets”

778
Q

What is the biochemical profile consistent with X linked hypophosphatemic rickets?

A

TIP: profile shows an inappropriately normal or low 1,25 OH Vitamin D in the face of low phosphate

Low Phosphate, Normal 1,25 OH Vit D
Normal Ca, PTH (since Ca is normal)
High Alk Phos

779
Q

What is the distribution of weakness in children with SMA?

A

Spares the face (BUT however, suck and swallow capabilities are typically affected)

Proximal mm then distal

780
Q

What is a defining feature of the presentation of SMA?

A

Fasciculations of the tongue are seen in most children, which is a defining physical examination finding not commonly seen in pediatric neuromuscular disorders

781
Q

What is the number of GAS pharyngitis that may warrant tonsillectomy?

A

7 in 1 year

5 or more in each of the past 2 years

TIP: While tonsillectomy is very effective in reducing the frequency of episodes of severe pharyngitis, most children meeting the Paradise criteria but who did not undergo tonsillectomy in controlled trials experienced a natural reduction in the frequency and severity of throat infections.

782
Q

What are the characteristics of a BRUE?

A

CRAFT BRUE

C – color change
R – respirations 
A – AMS
F – Fully resolved
T – Tone
783
Q

What are risk factors that place an infant at risk for recurrent BRUE?

A

Infant < 2 mos
Born < 32 weeks (with corrected gestational age < 45 weeks)
Previous BRUE

784
Q

What are symptoms of a corneal abrasion?

A
Eye pain
Foreign body sensation 
Conjunctivitis 
Photophobia
Tearing
785
Q

Treatment of corneal abrasion?

A

ANTIBIOTIC eye drops

NOT analgesic eye drops NOT steroid eye drops. BOTH are contraindicated

786
Q

How to confirm corneal abrasion?

A

Flourescein staining

787
Q

What are corticosteroid and anesthetic eye drops CONTRAINDICATED in corneal abrasions?

A

Anesthetics – delay healing, mask worsening symptoms and toxic to corneal epithelium

Steroids – delay healing and increase risk for infection

788
Q

What is the recommended schedule for MMRV vaccination?

A

12-15 mos

4-6 years

789
Q

Why does seborrheic dermatitis affect adolescents?

A

It targets sebaceous glands which are quite active in the adolescent period

790
Q

What parts of the face does seborrheic dermatitis tend to affect?

A
Eyelid
Eyebrows 
Glabella 
Alar region 
Retroauricular creases
791
Q

What parts of the face does seborrheic dermatitis tend to affect?
What is the difference between the presentation of seborrheic dermatitis in infants and adolescents?

A

Cradle cap vs dandruff

TIP: Treat cradle cap with shampoo: selenium sulfide or ketoconazole of pyrithione zinc

792
Q

What is the most common cause of thyroid enlargement in children

A

Hashimoto’s thyroiditis

793
Q

What thyroid diseases are T21 patients at risk for?

A

Congenital hypothyroidism

AND

Acquired thyroid disease: BOTH Hashimoto’s and Graves

794
Q

What is the cause of galactosemia?

A

Deficiency in the GALT enzyme, that is, the galactose 1 phosphate uridylyltransferase enzyme

795
Q

Why is it that I will rarely diagnose galactosemia in the US?

A

100% screened in the newborn screen

796
Q

Milestones of a 1 year old?

A

Gross: First place stance (stand with arms up and legs splayed)

Fine: 
-	Pincer grasp (grasp 1 thing)
-	Points with 1 finger
-	Holds 1 crayon
Social
-	Points with 1 finger to desired object  

Language:

  • Follows 1 step command
  • 1 word
797
Q

What is the risk of HCC in patients with chronic Hep B compared to the general population?

A

100-fold increase!

798
Q

What labs indicate higher risk of development of HCC in patients with Hep B?

A
Hep B E Antigen 
Viral load 
Hepatic dysfunction 
HCV 
HIV
799
Q

What 3 joints need to be evaluated in a child that presents with intoeing? (AKA 3 causes of intoeing?)

A

Metatarsus Adductus
Internal tibial rotation
Femoral anteversion

800
Q

Cause of most cases of intoeing?

A

Intrauterine positioning

801
Q

What specialty should be consulted in the case of a patient swallowing magnets?

A

Surgical

802
Q

What are prenatal US findings of duodenal atresia?

A

Polyhydramnios

803
Q

What syndrome is associated with duodenal atresia?

A

T21

804
Q

Child is on long acting ADHD medications and is working well until the afternoon. What to do?

A

Add a short acting medication to be taken in the afternoon

Yes, she will be on a long acting AND a short acting

805
Q

Inheritance pattern for Hereditary Hemorrhagic Telangiectasia?

A

Autosomal dominant

806
Q

What age does testicular cancer usually present?

A

15-34

That is, high schoolers, college students, med students, residents and new attendings are all at risk of developing testicular cance

807
Q

Framework Question:

Purpose of Lymph nodes

A

Lymph nodes are structured buildings that house lymphocytes. Lymph carries antigens from the vasculature along the lymphatic vessels. When lymph enters lymph nodes, the lymphocytes that recognize the specific antigens presented will react, replicate and be sent off to attack.

808
Q

How to distinguish between reactive lymphadenopathy vs infectious lymphadenitis? What is the difference?

A

Reactive lymphadenopathy is a reactive process to distant infection. Lymphocyte replication in the lymph node leads to enlarged lymph nodes

Infectious lymphadenitis is an infection of the lymph node itself (NOTE: how ballsy, that a pathogen would enter the lymph node, house of all the lymphocytes, and try to infect it)

Infectious lymphadenitis: very tender, overlying erythema, systemic signs

809
Q

What are the 2 and ONLY 2 dangerous spiders in the US?

A

Black widow – Lactrodectus

Brown recluse

810
Q

What are signs of systemic toxicity from lactrodectus bite?

A

Rigidity and severe pain

NOTE: these signs may warrant antivenin

811
Q

Experts recommend that under a certain size, patients bit by latrodectus should receive antivenin. What is that weight?

A

< 40kg