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1
Q

What is MELAS

A
  • mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes)
  • childhood-onset myopathy, seizures, recurrent vomiting, migrainous headaches, sensorineural hearing loss, stroke-like episodes associated with periods of regression
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2
Q

What is Menkes disease

A
  • copper transport disorder
  • infants appear developmentally normal until approximately 2 to 3 months of age followed by progressive developmental regression, low tone, feeding difficulties, failure to thrive, and seizures.
  • “kinky,” and hypopigmented hair.
  • low copper and ceruloplasmin levels.
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3
Q

When to give measles immunoglobulin?

A

-Infants younger than 6 months, pregnant women, individuals with immunocompromising conditions, or individuals who received a previous dose less than 28 days prior

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4
Q
  • high risk pts for PCV13 and PPSV23 vac schedule
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

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5
Q

diagnosis of acute bacterial sinusitis

A

Persistence of symptoms beyond 10 days without improvement
Worsening of symptoms or new onset of symptoms after initial improvement
Severe symptoms at onset lasting for 3 consecutive days

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6
Q

meds for bite for allergic to penicillin

A

clindamycin. + 3rd gen cephalosporin

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7
Q 
what group of metabolic disorders have:
normal or high ammonia
high anion gap
metabolic acidosis 
neutropenia
A

1) Organic acidemia

ex.
propionic Acidemia (elevated propionic acid and methylcitrate on urine)
and Methylmalonic Acidemia(elevated methylmalonic acid)

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8
Q

TTP
Cause:
Labs:
Tx:

A

antibodies againstADAMTS13
increased LDH, bili and BUN, decreased plts, schiztocytes
tx plasmapheresis and steroids

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9
Q

apical systolic murmur with a mid systolic click

A

mitral regurg

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10
Q

cyanotic heart disease with left axis deviation

A

tricuspid atresia

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11
Q

acyanotic heart disease with left axis deviation

A

AV canal defect

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12
Q

hyper IgM is characterized by

A

repeated episodes of pneumonia and sinusitis. at particular risk for PCP pneumonia

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13
Q

Symptoms of Klebsiella granulomatis vs Treponema pallidum vs Haemophilus ducreyi vs Chlamydia trachomatis

A

Haemophilus ducreyi (chancroid)- painful shallow ulceration with painful inguinal lymphadenopathy

Treponema pallidum- painless ulcer no lymphadenopathy

Klebsiella granulomatis (lymphogranuloma inguinal)- painless ulcer without regional lymphadenopathy (usually hx of travel to india, south africa etc)

Chlamydia trachomatis- painless ulcer with painful lymph nodes 4-6 weeks later (
(hx of travel to tropical areas)

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14
Q 
what group of metabolic disorders have:
High ammonia
and 
Normal anion gap
and Respiratory alkalosis
A

Urea cycle disorder

get plasma amino acids to confirm

MC type is ornithine transcarbamylase deficiency. shows elevated urine orotic acid and low or absent citrulline

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15
Q

what group of metabolic disorders have:
normal ammonia
and
normal anion gap

A

aminoacidopathies or galactosemia

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16
Q

critical labs to draw while pt is hypoglycemic

A

glucose, insulin, c-peptide, ketones, growth hormone and cortisol

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17
Q

11B-hydroxylase deficiency vs 21-hydroxylase deficiency

A

11B-hydroxylase deficiency- hypertension and increased androgens (think of the two 1s as two up arrows)

21-hydroxylase deficiency has increased adrogens and salt wasting

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18
Q

HPV when can it begin and what age do you have to do the 3 dose series

A
  • Can being at 9 years if abused

- At 1`5 or above needs 3 doses

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19
Q

When to test for reinfection after gonorrhea tx

A

in 3 months

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20
Q

teen STD screening

A
  • screen annually for GC in females
  • Screen males if hx of STI or areas of high prevalence
  • HIV once for everyone, HIV yearly if high risk
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21
Q

schistosome parasite

A

swimmers itch

from snail larvae in fresh water

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22
Q

when does babinski reflex stop

A

can be up going until 1 year

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23
Q

Hib prophylaxis

A

single case of Hib as long as they immunized no prophylaxis

double hib, 20 mg/kg/day for 4 days

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24
Q

Anti DNase B vs antistreptolysin O

A

Anti DNase-impetigo

Anti streptolysin- strep throat

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25
Q

CGD
1-test
2-presentation

A

Neutrophil function test/DHR

Presents with recurrent bacterial and fungal infections and granulomas

(Defect in phagocyte NADPH oxidase)

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26
Q

Blount disease

A

Disruption of normal cartilage at medial aspect of proximal tibia. Progressively worse bowing as child walks

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27
Q

MEN2B

Presentation

A

Tall, thin, mucosal neuromas, hyper mobility, intermittent constipation and diarrhea

100% will develop medullary thyroid carcinoma

Also associated is pheichromocytona

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28
Q

Modified DUKE criteria

A

Major:

  1. POS bcx
  2. evidence of endocardial involvement

Minor

  1. Predisposing factor
  2. temp >48
  3. Vascular phenomenon
  4. Immunologic phenomena
  5. Microbiological evidence

Need 2 major+1 minor
Or 1 major + 3 minor

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29
Q

What to do with dyslipidemia in pts

A
  • first confirm with another flp
  • if LDL 130-249
    • life styles changes, pharmacotherapy if >10 years, look for other causes
  • if LDL >249
    • refer to a lipid specialist
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30
Q

TST test positive

  1. who is positive if >5mm
  2. who is positive if >10 mm
A
  1. Close contact with TB, clinical evidence of TB and immunosuppressive conditions or tx
2. 
<4 yrs
- born or travel to high risk areas
- exposure to high risk adults
- other medical conditions (DM, chronic renal failure, malnutrition)

***Prior BcG does not alter interpretation of results (but still better to get IGRA if >2 yearsº

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31
Q

When is peak growth velocity in males and females

A

stage 4 for males, stage 3 for females

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32
Q

what drugs decrease the efficacy of oral contraceptives?

A

Rifampin
Anticonvulsants (except levetiracetam)
Most retrovirals

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33
Q

What do OCP due to Thyroxine binding globulin

A

OCPs increase Thyroxine binding globulin therefore need to look at FREE t3 and T4 not total

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34
Q

electrolyte changes in refeeding syndrome

A

low phosphate, low potassium, low magnesium

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35
Q

criteria for hospitalization for anorexia

A
  • weight <75% average body weight
  • hypotension or orthostatic BP
  • HR <45 or dysrhythmia
  • Edema and/or heart failure
  • electrolyte imbalances
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36
Q

Hydrocele description

1) when to repair?

A

soft, painless asymptomatic mass anterior to the testis

1) repair if not resolved by 1 year of age

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37
Q

Spermatocele description

A

non-painful cyst located at the head of the epididymis, distinct from the testis

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38
Q

Acute painful scrotum differential diagnosis

A 
torsion of the testis
torsion of the appendix testis
epididymis 
orchitis
Incarcerated hernia
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39
Q

description of neoplasms of the testicle

A

firm, painless, irregular mass WITHIN the testes or INDISTINGUISHABLE from the testes

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40
Q

Sunburst pattern in bone

A

Osteosarcoma

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41
Q

Ludwig’s angina

A

Polymicromibal (oral flora) cellulitis of the submandibular and sublingual spaces

Presents with fever, severe dysphasia, trismus and stuff neck

Usually a complication of the mandibular molar roots

Tx with ampicillin/Sulbactam

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42
Q

Vit K deficiency, factors affected and labs

A

2,7,9 and 10.

PT and PTT prolonged

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43
Q

lemierre disease presentation

A

Fevers, resp distress, trismus, dysphasia, decreased range of motion of the neck, peritonsillar swelling, and tenderness, swelling erythema overlying the jugular vein and angle of the jaw

Watch for septic emboli to the lungs

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44
Q

primary amenorrhea definition

A
  • no secondary sexual characteristic by 13
  • no period by 15
  • or no period by 3 years after onset of breast development
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45
Q

Functional hypothalamic amenorrhea

A

like in girls who are excessively exercising

will have decreased GcRh secretion low or normal LH

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46
Q

Amenorrhea with no breast development, differential based on FSH:
High FSH
Normal FSH
Low FSH

A

High FSH- ovarian failure
Normal FSH- intact HPA axis (get US)
Low FSH- hypothalamic or pituitary causes

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47
Q

Androgen Insensitivity vs swyer syndrome

A

both: no period, no pubic har

Androgen Insensitivity- normal male testosterone levels

swyer syndrome- little to no testosterone

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48
Q

BV vs Trichomoniasis

A

both have pH >4.5

Trichomoniasis: strawberry cervix, flagellated pear shaped motile organisms, need to tx sexual partners

BV: clue cells, fishy odor, don’t need to tx sexual partners,

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49
Q

MDMA toxicity

A
  • Waterloading is seen because of use during rave parties, sweating, hyperthermia, seizures, SIADH
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50
Q

X-linked agammaglobulinemia (Bruton agammaglobulinemia)

cause:
testing:
treatment:

A

Cause: mutation in bruton tyrosine kinase, which arrests B cells in pre-bcell stage

testing: Flow cytometry with no showed any CD19 because there are no mature B cells
tx: IVIG

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51
Q

CVID

cause:
testing:
treatment:

A

Cause: Mature Bcells are unable to differentiate into plasma cells

testing: flow cytometry-CD19 are present but have low antibody levels (but cannot call it CVID until 5 years of age because of transient immunoglobulinemia of infancy)

Tx: IVIG

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52
Q

Specific Antibody Deficiency

Cause:
Testing:
treatment:

A

Cause: make antibody but it doesn’t function well

testing: Flow cytometry CD19 present, normal antibody levels, poor vaccination response
tx: IVIG

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53
Q

X-linked Hyper-IgM syndrome

cause:
testing:
treatment:

A

cause: inability to class switch from IgM to IgG or IgA because don’t have CD40 and T cells cannot communicate properly with B cells and macrophages. They are susceptible to sinopulmonary infections and PCP
testing: Flow cytometry shows no CD40L, high IgM and low IgG and IgA
tx: IVIG and bactrim PCP prophylaxis and eventually BMT

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54
Q

X-linked Lymphoproliferative Syndrome

presentation:

A

overwhelming, near-fatal infection with EBV (fulminant hepatitis and BM failure) progresses to lymphoma

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55
Q

SCID

presentation:
testing:
treatment:

A

Presentation: presents in the 1st few months of life: FTT, chronic diarrhea, chronic lung infections, no thymus shadow. Presents with every type of infection because have defect in Bcell fn and Tcell fn

testing: Depends on the type of SCID, examples: molecular analysis or low adenosine deaminase in RBCs
tx: BMT

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56
Q

Bloom Syndrome

presentation:

A

small stature, telangiectasia, CNS abnormalities and immunodeficiency

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57
Q

Nijmegen Breakage Syndrome

cause:
presentation:

A

cause: can’t make nibrin which helps to repair damage to dsDNA
presentation: “bird-like” facies, microcephaly, near normal IQ, immunodeficiency

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58
Q

Job syndrome (hyper IgE syndrome)

presentation:

A

cause: neutrophils fail to adhere to the endothelium and enter the tissues
presentation: recurrent abscesses esp with staph aureus, eczema, scoliosis, delayed eruption or primary teeth, pneumatoceles

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59
Q

Chediak Higashi Syndrome

cause:
presentation:
testing:

A

cause: impaired lysosome degranulation
presentation: recurrent cutaneous and sinopulmonary infections, partial oculocutaneous albinism, ID, progressive neuropathy
testing: peripheral smear shows giant granules

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60
Q

Tumor lysis affect on calcium and why

A

Hypocalcemia, because lysis of cells releases phosphorus which then bonds to the serum calcium (the calcium phosphate can then damage the kidneys)

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61
Q

Type I allergic reactions

A

-immediate, IgE mediated

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62
Q

Type II allergic reactions

A
  • cytotoxic, IgM or IgG mediated

ex: goodpasture syndrome, myasthenia gravis, autoimmune hemolytic anemia

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63
Q

Type III allergic reactions

A
  • immune complex

ex: Vasculitis, serum sickness

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64
Q

Type IV allergic reactions

A
  • Delayed, T-cell mediated

occurs 24-72 hours after

ex: poison IVY, TB test

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65
Q

serum sickness

time frame:
sx:

A

time frame: 1-3 weeks after administration of drug (venom stings can also cause this)

sx: fever, rashes (serpiginous rash of hands and feet and urticaria, purple urticaria), joint pain, lymphadenopathy, n/v

66
Q

Urticarial Vasculitis

sx:
dx:

A

sx: hives last >24 hours in a fixed location, may see residual ecchymosis, hyperpigmentation or purpura when gone.

Hives don’t itch, they are tender and burn

dx: skin biopsy, decreased C3/C4 and antibodies to C1q

67
Q

Type 1 Drug reaction

timing:
example:

A

IgE mediated causes urticaria, anaphylaxis

timing: within minutes to hours
example: penicillin

68
Q

Type II drug reaction

timing:
example:

A

cytotoxic reaction (mediated by IgG or IgM)

timing: Days
example: drug-induced hemolytic anemia and thrombocytopenia secondary to penicillin, methyldopa or cephalosporins

69
Q

Type III drug reaction

timing:
example:

A

immune-complex mediated

timing: 1-3 weeks after drug initiation
example: Get fever, rash, urticaria, lymphadenopathy

serum sickness

70
Q

Type IV drug reaction

timing:
example:

A

Delayed type (mediated by drug specific T lymphocytes)

timing: 48-72 hours
example: contact dermatitis (neomycin, local anesthetics, topical antihistamines)

71
Q

DRESS Syndrome

sx/timeline:
Drugs:
Tx:

A

eosinophilia and systemic symptoms 2 to 8 weeks after starting a drug

drugs: anticonvulsants, sulfonamides, allopurinol
tx: stop the drug and steroids

72
Q

Tx for radiocontrast reaction

A

pre med with prednisone and diphenhydramine

73
Q

screening test for systemic macrocytosis

A

tryptase level

74
Q

Right atrial enlargement vs left atrial enlargement on EKG

A

right-tall

left-wide

75
Q

adenosine mechanism of action

A

miscellaneous anti-arrhythmic, works on AV node and

76
Q

amiodarone mechanism of action

A

class III anti-arrhythmic. Blocks potassium rectifier currents that are responsible for repolarization of the heart

77
Q

Causes of fixed splitting

A

ASD, right bbb, or severe pulmonary stenosis

78
Q

Myasthenia graves pathophysiology

A

Antibodies against post synaptic ach receptors which decrease the amount of receptors

79
Q

Juvenile myoclonic epilepsy vs benign rolandic epilepsy

A

JME: combo of myoclonic jerks, generalized tonic clinic seizures or absence seizures that usually occur early in the morning or while waking. EEG shows generalized poly spike and wave discharges ar 4 to 6 hz. Lifelong. Required drugs.

BRE (also called benign focal epilepsy of childhood): presents btw 5to10years old. Focal seizures during sleep (unilateral facial twitching, pro facial paresthesias and speech arrest). Have preserved consciousness. Centrotemporal benign focal discharges on EEG. Usually out grow, usually no meds needed

80
Q

Zero order kinetics

A

zerO order: Oversaturated

81
Q

Loop diuretics

examples:
MOA:
Electrolyte disturbances:

A

examples: furosemide and bumetanide
MOA: inhibit Na+,K+, Cl- co-transporter
Electrolyte disturbances: hypokalemia, hypochloremia, hyponatremia, hypocalcemia and metabolic alkalosis

82
Q

thiazide diuretics

MOA:
Electrolyte disturbances:

A

Examples:

MOA: block Na and Cl resorption (therefore more NA reaches the distal tubes where it is exchanged for potassium)

Electrolyte disturbances: decreased Na and increase Ca

83
Q

Dopamine
mechanism of action:
effects:

A

mechanism of action: stimulates B1, increases release of Norepi and decreases degradation of norepi
effects: increased myocardial activity, at high doses is an alpha agonist and causes vasoconstriction

84
Q

Dobutamine

MOA:

A

B1 and mild vasodilation

85
Q

Epi

MOA:

A

stimulations alpha and beta (more alpha at high doses)

86
Q

Milrinone

MOA:

A

phosphodiesterase inhibitor and has positive ionotropic and vasodilator effects

87
Q

Digoxin

MOA:
toxicity:

A

mechanism of action: inhibiting NA+/K+-ATPase, intracellular NA rises which causes activation of the NA/Ca exchanger, and the subsequent rise in Ca increased inotropic action
toxicity: N/v, diarrhea, color vision changes, confusion and arrhythmias (AV block, SVT or VT)

88
Q

ASD murmur

A

no murmur from flow across ASD because the right and left atria have very similar pressures, but you hear a murmur from the increased flow across the right ventricular outflow tract.

crescendo decrescendo murmur loudest at the left upper sternal border

89
Q

organophosphate poisoning

MOA:
SEs:
tx:

A

MOA: inactivates acetylcholinesterase, leading to increased Ach in parasympathetic nervous system
SEs: stimulation of both nicotinic and muscarinic receptors. At low doses, the muscarinic symptoms predominate, including the “SLUDGE” symptoms of salivation, lacrimation, urination, diarrhea, gastrointestinal cramping, and emesis.

TX: atropine

90
Q

Extrinsic pathway clotting factors

A

1, 2,7,10

91
Q

Hypocalcemia ekg findings

A

Prolonged QTc

92
Q

hyperchloremic metabolic acidosis is a form of…

A

normal anion gap metabolic acidosis

93
Q

normal osmolarity gap

A

<10 (if bigger than that in AG metabolic acidosis think toxic alcohols)

94
Q

Urine anion gap calculation

what does it mean?

A

UAG= NA+K-Cl

If>10—> low NH4+ excretion think RTA (distal, type 1 is trouble excreting acid)

If<0–> high NH4+ excretion think Diarrhea

95
Q

winter formula

use for acidosis or alkalosis?

A

PCO2=1.5 x bicarb+8 +/-2

use for acidosis

96
Q

expected COs for metabolic alkalosis

A

PCO2=0.7 x(bicarb-24)+40 +/-2

97
Q

CHARGE

A 
Coloboma
Heart
Atresias of the Choanae
Retarded growth
Genitourinary malformations
Ear malformations
98
Q

VACTERL

A 
Vertebral defects
Anal atresia
Cardiac defects
Tracheo-Esophageal fistula, 
Renal anomalies
Limb abnormalities.
99
Q

HUS

what is it?:
sxs:
tx:

A

what is it?: thrombotic microangiopathy following shiga toxin producing E coli diarrhea
sxs:
microangiopathic hemolytic anemia
thrombocytopenia
AKI
GI involvement (intussusception, rectal prolapse, pancreatitis, bowel perforation)
tx: supportive care

100
Q

hematuria differential:

A

low complement:
Postinfectious glomerulonephritis
Membranoproliferative
Lupus nephritis

normal complement:
IgA nepropathy
HSP 
Vasculitis (MPA, GPA)
Anti-GBM disease (like thin basement membrane disease, aport syndrome, etc)
101
Q

proteinuria differential:

A
  • Transient
  • Orthostatic/postural (first thing in the morning negative for protein)
  • Persistent (actual renal disease, DM etc)
102
Q

nephrotic syndrome differential

A
  1. MCD (more likely in younger children)
  2. Focal segmental glomerulosclerosis (more likely in older children)
  3. Membranous nephropathy (rare in children)
103
Q

Denys-drash Syndrome

A
  • steroid resistant infantile nephrotic syndrome
  • XY gonadal dysgenesis and ambiguous genitalia
  • Wilms tumor
104
Q

Nail-patella syndrome

A
  • hypoplasitic patella, dysplasia of elbows, iliac horns
  • dystrophic nails
  • microhematuria and proteinuria, 10% develop ESRD
105
Q

AD polycystic kidney disease vs multi cystic dysplastic kidney

A

for multi-cystic dysplastic kidney the cyst will be present at birth and go away with time (AD will only have one or two cysts at birth and will get worse with time)

106
Q

Prophylaxis for cluster headaches

A

Verapamil

107
Q

AD polycystic kidney disease vs multi cystic dysplastic kidney

A

for multi-cystic dysplastic kidney the cyst will be present at birth

108
Q

Fanconi Anemia

A

hypergimented intertriginous areas, cafe-au-lait spots, absent of hypoplastic radii bone marrow is hypocellular with fatty infiltrate.

109
Q

Valgus and varus normal ages

A

Varus birth to 2 years

Valgus 2 to 4 years

110
Q

Bone findings in rickets

A

Widening of the growth plate, enlargement of the wrists and ankles, craniotabes

111
Q

Bartonella henselae

Sxs:

A

Sxs: tender regional lymphadenopathy and tender erythematous papules, malaise, low fever, nausea, headache. Can get micro abscess in liver or spleen.

112
Q

Minimum length between varicella doses

A

3 months

113
Q

symptoms of Bardet-biedl syndrome

A

Obesity, retinitis pigmentosa, hypogonadism, intellectual disability, polydactyly, medullary cystic dysplasia

114
Q

Acute interstitial nephritis

symptom:

causes:

A

symptoms: AKI, fever, rash, eosinophilia
causes: Penicillins, cephalosporins, NSAIDS, thiazides

115
Q

Fanconi Syndrome

symptoms:

A

Proximal RTA, hypophosphatemia, hypokalemia, aminoaciduria

116
Q

AKI approach

A

FeNa: (SCr × UNa ) / (SNa × UCr)

Prerenal: FeNa <1% , Ur osm >400

Renal: FeNa >1%, Ur Osm 300-350

117
Q

Tularemia

sxs:

A

sxs: regional lymphadenopathy with or without a painful ulcer at the site of inoculation, fever, hepatosplenomegaly, anorexia. Association with rabbits, beavers and muskrats
tx: gentamicin or streptomycin for 10 days

118
Q

vertical nystagmus is associated with

A

demyelinating diseases, tumors and increased ICP

119
Q

Autoimmune hepatitis antibodies

A

ANA and antiSMA

Labs: hypergammaglobulinemia elevated AST and ALT

120
Q

Dietary recommendations for calcium oxalate stones

A

Decreased vit C intake because increased vit C can increase the amount of oxalate in the urine

121
Q

Congenital varicella sxs

A

Hypoplastic extremities, microphthalmia, cataracts, chorioretinitis

122
Q

Congenital syphilis findings

A

Frontal bossing, saddle nose, interstitial keratitis (will show corneal clouding), hutchingson teeth anterior bowing of the shins

123
Q

Shwachman-Diamond Syndrome sxs

A

exocrine pancreatic insufficiency, cyclic neutropenia +/- thrombocytopenia, bifid thumbs

124
Q

Medium-chain acyl-CoA dehydrogenase deficiency disorder (MCADD)

what is it:
presentation:
management:

A

what is it: disorder of fatty acid oxidation

presentation: vomiting, dehydration, lethargy, and seizures, usually presents during an infection
management: high carb diet (because prolonged fasting leads to hypoglycemia and then they body turns to ketogenesis) and restricting medium-chain triglycerides

125
Q

Yellow fever

symptoms:

A

travel to africa and south america

fever, headache and myalgias remission and then worsening symptoms including jaundice, parotitis, and multiple organ dysfn

126
Q

PHACES syndrome

A 
Posterior fossa abnormalities 
hemangiomas
arterial anomalies
cardiac lesions
eye or endocrine abnormalities
supraumbilical raphe or sternal cleft

should get an ECHO and MRI

127
Q

physiologic anemia of infancy

cause:
hgb level:
age:

A

cause: increased oxygenation after birth that results in decreased erythropoiesis
hgb level: 9 to 11
age: 6 to 9 weeks

128
Q

Hemolytic transfusion reaction symptoms

A

fever/chills, headache, nausea, chest pain, oozing from transfusion site, dark urine, hypotension

tx: hydration to protect kidney’s and may need furosemide to keep good urine output

129
Q

HLH

presentation:

A

febrile illness with multiorgan involvement, pancytopenia, suggestion of liver involvement, and very high levels of ferritin

130
Q

Loss of which urinary proteins predisposes patients with nephrotic syndrome to thromboses?

A

Antithrombin III and protein S

131
Q

lab triad with NEC

A

Hyponatremia, hyperglycemia, thrombocytopenia

132
Q

hand, foot and mouth vs herpangina

A

hand, foot and mouth (coxsackievirus A16 or enterovirus 71): ulcerations are on the tongue and buccal mucosa). Do supportive care and resolves in 7 to 10 days

Herpangina: coxsackievirus A or B or echoviruses. It presents with oral lesions without any skin involvement

133
Q

Second impact syndrome

A

Uncommon yet serious complication of repeated closed head injury resulting from a second episode of closed head injury while the patient is still symptomatic from an initial concussion event. See rapid mental deterioration, mental status changes, and uncal herniation, and has a mortality rate of 70% to 80%

134
Q

causes of non-ketotic hypoglycemia

A

hyperinsulinism and fatty acid oxidation defects such as medium-chain acyl-CoA dehydrogenase deficiency (MCADD). In these conditions, patients cannot generate energy via oxidative phosphorylation in the tricarboxylic acid cycle, and cannot produce ketones to provide fuel for the brain and other organs

135
Q

stickler syndrome

A

also called: hereditary arthro-ophthalmopathy

Stickler syndrome is a connective tissue disorder that is associated with midfacial hypoplasia, cleft palate, Pierre Robin sequence, hearing loss, and abnormalities of the eye.

136
Q

TENET

what is it for:
labs:

A

Intrinsic pathway
see prolonged ptt

Twelve 
Eleven
Nine 
Eight 
Ten
137
Q

prolonged PT but not PTT

A

deficiency of factor 7

138
Q

prolonged PTT but not PT

A

deficiency of factors 11, 9 and 8 (12 and lupus anticoagulant but they aren’t clinically significant)

139
Q

prolonged both PT and PTT

A

factor ten deficiency

140
Q

when can you give just one dose of hib

A

15 to 60 months

141
Q

PHACE

A 
posterior fossa abnormalities
hemangioma
Arterial malformations
cardiac malformation
eye and endocrine abnormalities
142
Q

Bernard-Soulier syndrome vs Glanzman

A

Both have macrocytic plts

Bernard-Soulier syndrome- deficiency of glycoprotein 1b in the platelet membrane. platelets do not aggregate correctly. Mild thrombocytopenia

Glanzman- defect in the platelet glycoprotein IIb/IIIa fibrinogen complex. The platelet count is NORMAL. but platelets do not aggregate

143
Q

osler-weber-rendu syndrome

A

hereditary hemorrhagic telangiectasia

spots on lips, vascular malformations of the lungs, CNS, and GI

144
Q

holt oram syndrome also called

A

heart hand syndrome

145
Q

when is 1 PCV13 needed

A

unvaccinated 24 months to 59 months

146
Q

AFP prenatal screening high and low

A 
high:
Renal stuff
Abdominal wall defects
Incorrect dating, multiples
Neural tube defects

low:
trisomy 18 or 21

147
Q

when you get hypoketotic hypoglycemia think of

A

fatty acid oxidation disorder. Types of fatty acid oxidation disorders include deficiencies in medium chain acyl-coA dehydrogenase (MCAD), long chain acyl-coA dehydrogenase (LCAD), and very long chain acyl-coA dehydrogenase (VLCAD).

Diagnosis is made by obtaining a plasma carnitine and acylcarnitine profile.

148
Q

chronic lymphocytic thyroiditis

A

Hashimoto thyroiditis
high levels of antithyroid peroxidase antibodies and antithyroglobulin antibodies.
Most patients with chronic lymphocytic thyroiditis are euthyroid and only present with a firm and nontender goiter found on a routine examination without other symptoms

149
Q

Hurler syndrome

A

deficient alpha-L-iduronidase activity in leukocytes
developmental delay and recurrent ear, sinus, and pulmonary infections d ue to thick secretions. Eventually, the patient develops coarse facial features, corneal clouding, umbilical or inguinal hernias, growth failure, cardiomyopathy, joint contractures, and neurodegeneration

150
Q

tx for psoriatic arthritis

A

methotrexate

151
Q

MC presentation for Francisella tularensis

A

Ulceroglandular syndrome (maculopapular lesion at the site of the tick bite which ulcerates with painful inflammed regoinal lymph nodes

152
Q

linear igA bullous dermatosis

A

blistering disorder that resolves spontaneously prior to puberty. bilsters form annular lesions that resemble a “string of pearls” which surround a central crust

153
Q

lichen striatus

A

grouped 2 to 5 mm pink yo hypo-pigmented, flat-topped papules in a curvilenear distribution. self limited. no tx required topical corticosterois may help if there is any itching

154
Q

autoimmune hepatitis types

A

i: female adolescent with sudden onset jaundice, negative viral studies, no meds and no stones. ANA and anti smooth muscle antibodies are positive.
ii: young children, fever , fatigue, RU’Q pain, transaminases, bili, ggt and alp are elevated. antiliver kidney microsomal antibody is positive

155
Q

metatarsus adductus vs talipes equinovarus

A

metatarsus adductus: adducted forefoot with a normal hindfoot

talipes equinovarus: foot that rests in rigid equinovarus position and cannot be passively corrected to a neutral position

156
Q

tx for hypoparathyroidism

A

calcitriol and calcium

157
Q

HIDS (hyper immunoglobulin D syndrome)

A

mutation in MVK gene leading to excessive production of interluekin 1

periodic fever syndrome with fevers for 3 to 7 days occurring every 1 to 2 months, abd, vomiting, nondestructive arthritis, rash, can get ulcers and splenomegaly during fevers

dutch and french descent

158
Q

PFAPA

A

fever 5 to 7 days
joint pain, abd pain, rash, headache, vomiting or diarrhea
every 4 weeks

159
Q

FMF

A

familial mediterranean fever

AR, MERV on chromosome 16
can benefit from IL1 inhibition, tx with colchicine

fever from hours to 5 days
pleuritis, pericarditis and scrotal swelling, rash, arthritis, mylagias

concern is amyloidosis if not treated

160
Q

TRAPS

A

TNF receptor 1 associated periodic syndrome

fever 5 days to 2 weeks
conjunctivitis and periorbital edema, abd pain, migratory mylagias

tx NSAIDS, prednisone, etanercept (TNF inhibitor) and anakinra

161
Q

early onset neonatal hypocalcemia

A 
<72 hours 
prematurity
maternal DM
maternal hyperparathyroidism 
IUGR
sepsis
hypomag
RDS
162
Q

late onset neonatal hypocalcemia

A 
>72 hours
vit D deficiency with hypomag
hypoparathyroidism
high phosphorus load
pseudohypoparathyroidism (PTH resistance)

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