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#1 Prep Pearls > Board Vitals #3 > Flashcards

Board Vitals #3 Flashcards

1
Q

Patient with two different colored eyes, patches of hypopigmented skin, confluent eyebrows and broad nasal bridge needs what additional test?
What is this?

A 
Needs hearing tested (50% have congenital sensorineural hearing loss)
Waardenburg syndrome (gray or white forelock)
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2
Q

Most brachial plexus birth injuries occur to which portion?

A

Upper trunk
Most unilateral and from stretching of C5-6 with decreased moevement of affected arm; upper arm adducted and internally rotated

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3
Q

What infants should receive Palivizumab?

A

born <29/0

born <32 with BPD until 12 months old

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4
Q

Infant presents with diffuse maculopapular rash, started on neck and trunk and spread to arms and face. Had fever 3-4 days before rash

A

Sixth disease or roseola infantum from HHV-6

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5
Q

Parvovirus B19 can lead to this serious complication, esp in SS patients

A

Aplastic Crisis

VERY infectious

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6
Q

When are patients with classic Parvo B19 (erythema infectiousum) most contagious

A

Get fevers, nausea, HA and coryza then slapped cheek facial rash; once rash appears no longer infectious

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7
Q

Neurocutaneous disorder with telangiectasia of skin, GI, retina and mucous membranes.
What chromosome and gene are involved?
How do these pts present?

A

Hereditary hemorrhagic telangiectasia or Osler-Weber-Rendu
Auto Dominant
mutationon HHT1 gene on chrom 9
See epistaxsis, GI bleeds, telangiectasia on lips, oral mucosa and fingertips

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8
Q

Predisposes individuals to freckling and malignancies when exposed to UV light. Get basal cell, melanoma and systemic tumors as well as progressive hearing loss, chorea, ataxia

A

Xeroderma pigmentosum

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9
Q

What gross motor milestones are present at 9 months?

A

Throw ball overhead, jump, walk up and down stairs one at a time
Stack 6 blocks, copy a line, point to a picture

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10
Q

What patients require immediate referral to a burn center?

A

3rd degree burns (full thickness; white, dry and painless), >10% body surface, or sensitive areas (face, perineum, hands, feet, mouth)

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11
Q

What is the Parkland formula, when do we use it?

A 
Fluid management for burns >15% of body surface
Add EXTRA (4ml/kg) _ % body burned to maint fluids and gie 1/2 over the first 8 hrs from time of burn.
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12
Q

Most common pediatric arrhythmia

Why does this occur?

A

Paroxysmal SVT

From AV and AV node reentrant tachycardia ~ narrow complex with rapid onset

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13
Q

Tx for SVT

A

vagal, adenosine and AV nodal active agents

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14
Q

An event that results in death, permanent harm or severe temporary harm

A

Sentinel event

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15
Q

A event, preventable or non that caused harm to patient as result of medical error

A

Adverse event

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16
Q

Patient is diagnosed with Developmental hip dysplasia. What is treatment if
<6 months
>6 months

A

<6months treatment is Pavlik harness (splint to prevent hip extension and limits adduction)
>6 months is closed or open reduction with hip cast

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17
Q

What imagin modality do we use if we suspect DDH?

A

Ultrasound if >2 weeks but <6 4 months

Hip XRAY after 4 months

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18
Q

What children need imaging for DDH?

A

All female breech (get US at 6 weeks)
Consider boy breech or in history of first degree relative
IF 2 consecutive exams that are concerning or equivicol
ANY UNEQUIVICOL exam

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19
Q

Deficiency presents with weakness, cerebellar ataxia, partial opthalmoplegia, muslce aches and altered sensation in fingers and toes

A

Vitamin E
only one to cause spinocerebellar degeneration
See if issues with fat absorption

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20
Q

Presents with irritability, excessive startle response and seizures not responsive to AEDs

A

Pyridoxine or B6 deficiency

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21
Q

What is PPX for people in contact with someone who has N.men?

A

1 dose IM CTX or 4 doses PO Rifampin over 2 days OR once dose of Ciprofloxacin orally

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22
Q

Patient with devo delay and macrocephaly with cherry red spot and exaggerated startle reflex. No organomegaly with decreased tone at 9 months
Disease and pathology?

A 
Tay Sachs (no organ involvement)
Sphingolipidses from deficiency of Hexosaminidase A enZ
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23
Q

Axillary freckles, neurofibromas, cafe au lait macules (6 or more and HYPERpigmented) are concerning for?
What screening should be done

A

Neurofibromatosis

Screen for opthalmologic exam for optic pathway glioma

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24
Q

Facial angiofibromas, shagreen patches, ash leaf macules and subependymal nodules are associated with

A

Tuberous sclerosis

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25
Q

What are shagreen patches and when do we see them?

A

Fleshy, light pink, have raised and ‘orange peel’ appearance. On trunk or lumbrosacral area
Seen with Tuberous sclerosis

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26
Q

Patient with isolated hematuria on repeat urinalysis with negative culture. Next step?

A

If history is neg for infection, trauma or FHx, get urine calcium and creatinine
Uca:Ucr ratio >0.2 suggests hypercalciuria

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27
Q

When starting a patient on prostaglandins or PGE1 to keep PDA patent, what else needs to be done?

A

Intubate; PGE’s can cause central apnea

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28
Q

10 month old with croupy cough, SpO2 in high 80s wit fever and inspiratory stridor. Normal breath sounds and clear. Was given decadron earlier in the day without effect

A

Bacterial tracheitis; inflammation of larynx, trachea and bronchi. Seen more in peds
Stridor or barky cough, high fevers and hard to breath

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29
Q

What does CHARGE stand for

A 
Coloboma
Heart defects
Atresia of chonae
Retardation of growth and devo
GU anomalies
Ear anomalies or deafness
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30
Q 
Patient with Rickets from Vit D defiency has
\_\_\_\_\_ 25 OH Vit D
\_\_\_\_ PTH
\_\_\_\_\_Calsium
\_\_\_\_ alk phos
A

Low Vit D
Elevated PTH
Low Ca
Elevated Alk phos

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31
Q

When do we get Tylenol level for accidental or intentional ingestion?
When can we give charcoal

A

4 hours after

Gastric decotamination with charcoal can be done w/i 4 hours post ingestion

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32
Q

When do we give NAC for tyenol ingestion

A

Above tx line on Rumack-Matthew nomogram
ingestion >150mg/kg
pt with delayed presention and serum tylenol >10mg/L

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33
Q

Pt with this disease can present months after birth with albinism, musty odor, stunted growth, devo delay and microcephaly

A

PKU

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34
Q

Accumulaiton of glucocerebrosides in tissue, pt present with splenomegaly, hepatomegaly, thrombocytopenia, osteopenia, bone pain

A

Gauchers; lysosomal dx

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35
Q

When would you order a total CH50 assay?

A

Concern for complement pathway pathology

See recurrent sinopulmonary infections, recurrent neisserial infections and issues with MAC

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36
Q

Lab abnormality seen with pyloric stenosis

A

HYPOchlroemic HYPOkalemic metabolic alkalosis

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37
Q

Most common cause of hyperchloremic metabolic acidosis

A

RTA

If associated with hypoKalemia then its type 1 or 2

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38
Q

When do we see hyperchloremic hyperkalemic metabolic acidosis

A

RTA type 4

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39
Q

6 year with left sided limp. Had a URI 4 days ago. Left hip w/o redness or erythema and antalgic gait with hip abducted and externally rotated. Most likely diagnosis and treatment

A

Toxic synovitis and tx with NSAIDS

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40
Q

What are the indications for starting insulin therapy in diabetic patient

A

Patients with hA1c >8.5% need to start or if hx of DKA

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41
Q

All streptococcus organisms are gram_____ and are in ____ and ____

A

gram positive
are in pairs and chains
Gram staining = BLUE

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42
Q

Which strep organisms are alpha hemolytic

A

viridans and pneumonia

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43
Q

Which strep organisms are beta hemolytic

A

Group A = pyogenes

Group B= agalactiae

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44
Q

You have a patient that is strep positive with sympotms. Known PCN allergy. What do you treat with?

A

Erythromycin
OR
Clindamycin

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45
Q

Patient presents with peritonsillar abscess. What is treatment

A

Drain and Tx with IV antibiotics: Clinda and amp-sulbactam to cover for anerobes–> amox-clavulanate

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46
Q

What is the correct order of puberty in females?

A

Thelarche–> Pubarche–> Growth spurt 1-2 years before–> Menarche

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47
Q

Hyperbili due to Rh incompatibility is from ____

A

Antibody in mom

anti-D IgG

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48
Q

What do we give to tx overdose due to beta blocker with symptomatic bradycardia, resp depression, hypoT and hypoglycemia

A

Glucagon and Atropine

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49
Q

What is the MOA of aminogycosides and which organisms are they effective against?

A

Binds aminoacly site of 16S rRNA within the 30S subunit to block protein synthesis
good for anaerobes and gram negatives

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50
Q

Patients ___ to ___ require 2 doses of flu vaccine the first time they receive spaced ___ weeks apart

A

6mo to 8 years

seperated by 4 weeks

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51
Q

How do you calculate midparental height for male

A

Moms + Dads ht in cm + 13
Divided by 2
*ht should be 5 cm above or below

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52
Q

How do you calculate midparental height for female

A

Moms + Dads ht in cm - 13cm and can be 5 cm above or below this number

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53
Q

MOA of Clonidine

A

alpha-2-adrenergic receptor stiumulator–> reduced sympathetic outflow
-decrease in peripheral vascular resistance, renal vascular resistance, and decreases HR and BP

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54
Q

SE of Clonidine

A

worsen or cause depression, fatigue, dizzy, dry mouth, HA, abdominal pain,hypoT

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55
Q

Which drugs can be given through an ET tube?

A 
LEAN
lidocaine
Epinephrine
Atropine
Naloxone
And surf
56
Q

2 day old with acute onset poor feeding, fTT, vomiting and lethargy.
Labs notable for ketoacidosis, elevated anion gap and moderate hyperammonemia.
Cause?

A

Organic acidemia

57
Q

Patient with ketosis, elevated ammonia, anion gap and thrombocytopenia. Has sweaty feet and a seizure. 1 week old.

A

Organic acidemia

*Isovaleric Acidemia

58
Q

What causes organic acidemias?

Lab abnormalities?

A

Enzyme deficiencies lead to difficulty breaking down organic acids from AA and FA.
See anion gap MA, Ketosis, Hyperammonia (often)
*present by DOL2

59
Q

What is Tx for organic acidemias?

A

Limit protein and give L carnitine to repleate depleted stores, IV glucose and ammonia scavengers

60
Q

How do MMA and proprionic acidemia present?

A

Poor feeding, vomiting, dehydration, tachypnea and hypotonia. Have anion gap MA, ketonuria, hyperammonimia.
Get urine organic acids and see elevated MMA or PA

61
Q

Treatment for MMA?

A

Vit B12

62
Q

4 year old presents with fevers to 104 and oral lesions in the oropharynx with decreased PO intake. No other skin lesions.

A

Herpangina from Coxsackie virus

63
Q

Patient presents with fever and new onset seizure. EEG shows periodic lateralizing epileptiform discharges. If you obtain CT what area of the brain is likely infected?
What is treatment?

A

HSV encephalitis
Temporal lobe involvement
Tx stat IV acyclovir and get LP for PCR testing

64
Q

5 year old with hearing loss and delayed growth. Normal at birth. History of strokes and labs notable for elevated lactate and uric acid. Has frequent headaches and is acutely encephalopathic

A

MELAS
Mitochondrial Encephalopathy Lactic acidosis ad Stroke like episodes
Confirm with gene analysis

65
Q

Patient’s with Hashimoto Thyroiditis present with ___ TSH ____ T4 and elevated ____ antibody

A

HIGH TSH
LOW T4
elevated TPO or thyroglobulin

66
Q

Used when doing case control study to compare exposed and non-exposed group to determine probability of outcome of interest between the two

A

Relative Risk

Divide probability of outcome of interest in: Exposed/Unexposed

67
Q

Used in Case Control studies

Tells you chance of having a disease given an exposure

A

Odds ratio

Divide odds of disease after exposure/odds of disease without exposure

68
Q

Patient with respiratory alkalosis, metabolic acidosis and ear ringing, they have elevated HR and RR.
Dx adn tx

A

ASA or salicylate toxicity
Tx with urine alkalynization
may be able to give activated charcoal if acutely presented

69
Q

Any patient with JIA that is 6 years or less and ANA positive needs to have…

A

optho eval for uveitis. Done w/in 1 month of diagnosis and then every 3 months for 4 years.
Then every 6 months for 3 years then yearly.

70
Q

Features present in Prune belly syndrome (triad)

A

Deficient devo of abdominal muscles, GU anomalies and cyrptorchidism.
Often prostatic and ureteral hypoplasia–> obstructive uropathy in utero leads to megauretery/hydronephrosis

71
Q

What are the HACEK organisms?

A

Haemohilus aphrophilus
Actinobaccilus
Cardiobaterium hominims
Eikenella
Kingella
*Fastidious gram negative bacilli; take up to 5 days to grow
*resist amox, tx with 3rd gen ceph plus gent

72
Q

Marfanoid habitus with poster/downward displaced lens and devo delay
dx
tx

A

Homocystinuria
Autosomal recessive
cystathione-B-synthase deficiency and see elevated methionine levels and homocysteine
Tx: cysteine

73
Q

Patient with several days of explosive diarrhea and persisetant diarrhea with bloating that is painless and no blood or mucous with hyperactive bowel sounds. Most likely infectious cause and treatment

A

Giardia duodenalis
Tx Metronidazole
Dx with stool microscopy or antigen

74
Q

Patient with recurrent staph and strep infections and oculocutaneous albinism.
Cause and genetics

A

Chedaik Higashi
Auto-Recessive
abnormal neutrophil function; fusion of primary and secondary granules–> giant granules in neurtrophils, lymphocytes, plts and melanocytes

75
Q

Patient with direct hyperbili at 7 days of life. Paucity of bile ducts present. What cardiac and skeletal anomalies as well as facial features are seen

A

Pulmonic stenosis
butterfly vertebrae
hypertelorism and triangle facies

76
Q

Presents in teens that are during sports with sudden and intense muscle contracture and seen in kicking, springing, jumping and frequently misdiagnosed.
Pt present with posterior thigh pain and pain at hip

A

Avulsion fracture; seen frequently at anterior superior iliac spine
Heal over 4-6 wks with rest, ice, and non weight bearing

77
Q

Infectious diarrhea that causes fever, body aches then abdominal pain, vomitting and bloody diarrhea.
Complications include HUS, seizures, bactermia, reactive arthritis.
Dx and tx

A

Shigella

Tx supportive

78
Q

Associated with progressive non-atherosclerotic bilatearl stenoses of distal internal carotid arteries and trunks of anterior and middle cerebral arteries. MC in Japanese girls

A

Moyamoya

See ‘puff of smoke’ on angiogram

79
Q

Patient is newborn presents with hypocalcemia and TOF. What is the syndrome?
What cell line is affected?
How do we diagnose?

A

DiGeorge; 22q del from abnormal 3/th brachial pouches
T cell immunity defective bc no thymus (thus no maturation)
Dx with FISH with DNA probe

80
Q

What is cause of Down Syndrome

A

meiotic nondisjunction

81
Q

Patient presents wtih recurrent S. PNA infections as well as H influenza and PSA.
Arrest of B cells in Pre-B cell state.

A

X-link agammaglobulinemia

mutationin Btk gene on Xq22

82
Q

Symptom of deficit of voluntary motor function in pat w/o anatomic of physiologic basis. Present with impaired coordination, paralysis, vision changes, nonepileptic seizures
More common in females

A

Conversion disorder

83
Q

Intentional feigning of physical or psychological symptoms without secondary gains (no financial or legal gain) and mimics a known syndrome

A

Factitious disorder

84
Q

Systolic crescendo-decrescendo murmur, II/VI in healthy patient

A

Vibratory or Stills murmur; benign

85
Q

Low pitched mid-diastolic mrumur at cardiac apex

A

Mitral stenosis

86
Q

In prerenal acute kidney failure renal tubules respond to decreased perfusion by conserving water and sodium so that urine osmolality is > ______ and urine sodium is

A

urine osmolality is >400-500

urine sodium is <10-20mEq/L and FENa is < 1%

87
Q

Most children double their birthweight by _____ and triple their weight by _____

A

double by 4-5 mo
triple by 12 mo
quad by 2 years

88
Q

Teen with recurrent Sinus infections and two episodes of diarrhea from giardia. Normal CBC, LFT.
What test do you want, what do you suspect if its CVID

A

get Ig levels
See low GAM (IgG, A, M)
picture teenager with big (GAMS = thighs) with runny nose running to get her Izs once again!

89
Q

Patient with recurrent infections, eczema, male. What do you see on cbc

A

thrombocytopenia with small platelets

wiskott aldrich

90
Q

Immune system is made up of the INNATE immune system =

A

Innate = neutrophils, eosinophils mono/macrophage, complement, cytokine/chemokines

91
Q

Immune system is made up of Innate and ADAPTIVE which is

A 
Adaptive = Humoral which is B cells (from plasma cells) to make antiBodies
Cellular = T cells for cell mediate immunity against virus/fungus/intracellular organisms
92
Q

What test will diagnose a humoral or cellular immunity defect? (issue with B cells or T cells)

A

Lymphocyte phenotyping by Flow cytometry

93
Q

Patient with recurrent derm and pulm infections, gets lots of abcesses and has bone and joint infections likely has issue with…

A

Neutrophil defect (innate)

94
Q

Patient with recurrent bacterial infections with encapsulated organisms and severe or recurrent resp tract infections has issue with…

A

Complement defect

95
Q

Patient with recurrent pyogenic infections with encapsulated organisms, otitis, sinusisits, PNA, conjuctivitis and decreased levels of immunoglobulins has issue with..

A

Humoral immunity defect

96
Q

Patient with reuccrent infection with opportunistic or less virulent organisms like viruses, has growth retardation, diarrhea, FTT and poor survival past childhood has issue with…

A

Cellular immunity defect

97
Q

Depression screening and risk based assessment for drugs/EtOH begins at age ____

A

11

98
Q

At what age do we screen for dyslipidemia?

A

risk based age 2 if FHX

screen at 9-11 yo and again 18-21

99
Q

Risk based screening for STIs/HIV occurs as early as ____ with mandatory test at ____

A

11

16-18

100
Q

What are complications of pinworms and what is treatment?

A

UTI and vulvovaginitis

Tx albendazole, mebendazole or pyrantel

101
Q

Nephrotic syndrome is often accompanied by what change in CBC

A

thrombophilia

102
Q

What injuries are sustained when children wear a seatbelt with just lap belt?

A

Transverse abdomimal wall contusion, lumbar Chance fracture (in upper lumbar spine) visceral trauma like jejunal rupture or liver lac

103
Q

Patient presents with pityriasis rosea. What is expected course and treatment?

A

Rash resolves on own. No tx needed, could do topical steroid if really itchy

104
Q

How frequently do we screen thyroid function in Trisomy 21?

A

birth, 6 mo 12mo and annually

105
Q

Patient with recurrent thrush and fevers has issue with ___ part of immune system and has issue with what ene?

A

T cell immunity issue; Chronic mucocutaneous candidiasis or CMCC
Defect of AIRE gene or autoimmune regulatory gene

106
Q

Seizure dx that starts with myoclonic jerks and progresses into GTC, worse with sleep deprivation. What is treatment and diagnosis?

A

Juvenile myoclonic epilepsy
Sz in AM, is hereditary and is lifelong condition.
Tx valproic acid`

107
Q

What is the MC testicular mass seen in pre-pubertal boys?

A

Teratoma

108
Q

Infants less than 1000 grams are at risk of ____ and should have this type of screenign done.

A

IVH
head US
70% will devo grade IV IVH

109
Q

Weight of <1000 grams is linked to this complications long term

A

neuro impairment and risk

110
Q

Normal hearing has a threshold of ___ to ___ dB

A

0 to 20 dB; anything abnormal on screening should be referred to audiology

111
Q

Cleft in vertebral column with defect in skin, exposed meninges and spinal cord.

A

Myelomeningocele; spina bifida

MC NT defect

112
Q

Fluid filled gliosis lined cavity within the spinal cord and can lead to progressive scoliosis, spasticity and weakness of extremities

A

Syringomyelia

113
Q

What are the requirements for BPD diagnosis?

A

O2 requirement at 28 days postnatal or 36 weeks postmentral age.
RF: <1250g, maternal smoking, IUGR, mechanical vent, infections

114
Q

What are pathological findinds on lungs of BPD infants?

A

decreased septation, alveolar hypoplasia, increased elastic tissue formation, thickened interstitium.

115
Q

What is Bornholm syndrome? Who gets is and what is infection??

A

fever, paroxymal spasms of chest and abdominal muscles.
Causes are Coxsackie B virus
See in summer time; often adolescents.

116
Q

Induration of > ___mm indicates positive results even if BCG vaccine.
Assuming neg CXR, what is treatment for latent TB?

A

> 15mm
Rifampin daily for 4 months
Isoniazid + Rifampin weekly for 3 months or
Isoniazid daily for 9 months

117
Q

Patient is diagnosed with active TB (CXR was +), what is treatment?

A

RIPE

Rifampin, isoniazid, pyrazinamide and ethambutol for 2 months then Isoniazid/rifampin for additional 4 months

118
Q

Anabolic steroids have ___ feedback on hypothalamus

Leads to ____ LH and FSH secreation

A

NEGATIVE
LOW LH and FSH secretion
= small balls, azoospermia and gynecomastia

119
Q

MOA of clonidine

Uses of Clonidine

A

central alpha adrenergic agonist; decreases peripheral resistance
Used for HTN but also can work for insomnia, ODD, tics and opioid withdrawl.

120
Q

SE of clonidine

A

HypoT, irritability, dysphoria, parasomnias

121
Q

One year old with fatty stools and FTT, ANC of 600 with hypochromic, microcytic anemia and low levels of trypsin, lipase adn colipase. Low Ig’s and recurrent bacterial infections
Dx

A

Schwachman Diamond syndrome

Auto Recessive

122
Q

What do we see in Swachmann Diamond sydnrome

A

exocrine pancreatic insufficiency (fatty stools)
Bone marrow dysfuncion( low ANC, anemia)
Recurrent infection with los Ig

123
Q

Zoonotic disease that causes fever, body aches–> cough and SOB with pulmonary capillary edema.
See thrombocytopenia, leukocytosis, elevated crit and immunoblasts

A

Hantavirus

Confirm with serology

124
Q

Patient with this tumor present with abdominal mass, constipation, proptosis, periorbital ecchymoses.
Can met to bones

A

Neuroblastoma

125
Q

Disorder in newborns, see FTT, hepatomegaly, direct hyperbili, hypoglycemia and delayed healing of umbilicus. Smell like cabbage

A

Tyrosinemia

AutoRecessive from fumarylacetoacetate hydrolase deficiency

126
Q

Causes black urine when exposed to air, can see heart issues, kidney stones, prostate stones

A

Alkaptonuria

Auto Recessive

127
Q

Infant with FTT, lethargy, jaundice and coagulopathy. High risk for salmonella sepsis and cataracts

A

Galactosemia

128
Q

Dog bites needed to be treated with ____ aBx when they occur to what areas?

A

Amoxicillin-clavulanate
To face, hands, genitals
MC organism = pasteurella

129
Q

Naegleria fowler is an amoeba seen with transplant patients, causes meningoencephalitis. What is treatment

A

Miltefosine with medically induced hypothermia

130
Q

What is the natural progression of the atopy march?

A

Eczema–> food allergies–> allergic rhinitis–> asthma

131
Q

What is the gold standard to diagnose asthma

A

Methacholine challenge; has a high negative predictive value
It directly stims airway smooth msl receptors.
Pre/post spirometry is recommended initial screen in kids >5yo

132
Q

What is the relationship between food allergies and eczema?

A

Food allergies do not cause eczema; thus do not routinely test! only test food allergies if IgE mediated reaction has occurred.

133
Q

Gram negative sepsis can lead to shock and release of endotoxins which can cause…

A

DIC

see coag cascade triggered–> thrombi and fibrin deposit in small vessels leads to schistocytes

134
Q

Class of chemo drugs that are cardiotoxic

A

Anthracyclines; doxorubicin

135
Q

Cells occur in ESRD and liver disease as well as pyruvate kinase deficiency

A

echinocytes or burr cells

136
Q

tear drop cells that act as a marker for bone marrow infiltration and fibrosis

A

Dacrocytes

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