Uworld + NBME Flashcards

1
Q

Type 1,2,4 RTA:

Describe urine pH

A

1: distal, above 5.5
2: proximal, below 5.5
4: below 5.5

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2
Q

Which of the RTA’s is associated with high potassium?

A

Type 4

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3
Q

Causes of Type 1,2,4 RTA

A

1: poor H+ secretion
2: poor bicarb resorption
3: aldo resistance

(1= H+1, 2= BI)

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4
Q

What syndrome is most commonly associated with Type 2/ proximal RTA

A

Fanconis Anemia

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5
Q

What disorders may be associated with RTA 1?

A

Autoimmune: Sjogrens, Rheumatoid

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6
Q

Most common presentation RTA

A
  • FTT

- low bicarb/ normal anion gap acidosis

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7
Q

Sun screen guidelines:

A

SPF 15-30 or higher
apply 30 mins before exposure
reapply every 2 hours

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8
Q

Diagnosis associated with:

  • recurrent sinopulmonary infection
  • absent lymph tissue
  • low immunoglobulins
A

Brutons Agammaglobulinemia

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9
Q

Pnuemocystis pneumonia in a baby is suggestive of

A

HIV infection

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10
Q

Diagnosis associated with:

  • profound lymphopenia
  • recurrent infection
  • failure to thrive
A

ADA deficiency (SCID)

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11
Q

Recurrent severe infection with staph, serratia, aspergillus is assc with with condition?

A

Chronic granulomatous disease

impaired phagocytosis

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12
Q

Niemann Pick vs Tay Sachs enzyme deficiencies :

+ inheritance pattern, age of onset

A
  • Niemann Pick: sphingomyelinase
  • Tay Sachs: hexosaminadase

both are AR, onset at 2-6 months

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13
Q

Symptoms assc with both tay sachs and neimann pick

A
  • hypotonia
  • failure to thrive
  • cherry red macula
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14
Q

How are neimann pick and tay sachs distginuished?

A
  • tay sachs: no HSM, hyperreflexia

- neimann pick: HSM, areflexia

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15
Q

On imaging, where are trachea and esophagus located?

A

-front: trachea
-behind: esophagus
(on lateral view)

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16
Q

C1 inhibitor deficiency is assc with what condition?

A

Hereditary angioedema

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17
Q

Gold standard for diagnosis of muscular dystrophy

A

genetic testing, not muscular biopsy.

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18
Q

Do health departments track vaccine refusal?

A

no

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19
Q

Cause of strokes in kiddos

A

Sickle Cell Anemia

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20
Q

Condition most commonly predisposing to orbital cellulitis

A

bacterial sinusitis

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21
Q

DOC for px in cat bites?

Most common bacteria assc?

A
  • amox/clauvulanate

- pasteurella multocida (anaerobe)

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22
Q
Diagnosis assc with:
hematemesis 
hypotensive shock 
anion gab acidosis 
gastric scarring 
radiopaque substances in abdomen
A

iron poisoning

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23
Q

Treatment for iron to

A

deferoxamine

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24
Q

Tinnitus
fever
tachypnea
acidosis

cause

A

aspirin tox

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25
Q

Whole body convulsions + umbilical stump infection:

diagnsosis

A

tetanus

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26
Q

Dihydrorhadamine + nitroblue tetrazolium tests are used to diagnose what condition?

A

CGD

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27
Q

Treatment for cat scratch disease

A

azithro

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28
Q

Contrast TB and CSD adenitis

A

TB adenitis is nontender

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29
Q

Bacteria in dog/ cat mouths causing cellulitis

A

p. multocida

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30
Q

What animals carry salmonella?

What is the presentation of salmonella?

A

reptiles/ turtles

enteritis/mesenteric adenitis

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31
Q

Common causes of acute lymphadenitis

A
  • staph aureus

- strep pyo

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32
Q

Treatment of radial head subluxation

A

hyperpronation of forearm

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33
Q

Describe tennis elbow

A
  • lateral epicondylitis

- caused by excessive extensor use

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34
Q

Describe golfers elbow

A

-medial epicondylitis

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35
Q

What is Panner disease?

A

osteochondritis of the capitellum

loss of pronation/supination

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36
Q

Treatment for tinea corporis

A

(Ringworm)

clotrimazole

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37
Q

Congenital rubella findings

A
  • sensorineural hearing loss
  • cataracts
  • PDA
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38
Q

Congenital toxo findings

A
  • chorioretinitis
  • hydrocephalus
  • intracranial calcifications
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39
Q

Congeital varicella findings

A
  • scarring
  • limb hypoplasia
  • cataracts
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40
Q

Signs of deliberate scald injury

A
  • sparing of the flexor surfaces
  • lack of splash marks
  • sharp line of demarcation
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41
Q

When is significant microcytosis seen in iron deficiency anemia?

A

When Hct drops well below 30

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42
Q

Treatment with what vitamin improves measles

A

Vitamin A

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43
Q

Most common cause of vaginal bleeding in the neonate

A

Maternal withdrawl of estrogen

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44
Q

Neonatal chlamydia effects

A
  • conjunctivitis

- pneumonia

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45
Q

Ddx excess testosterone + low LH in boys

A
  • CAH

- exogeneous use (not in younger kiddos!)

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46
Q

Signs of viral vs strep pharyngitis

A
  • viral: cough, rhinorrhea, ulcers, conjunctivitis

- bacterial: exudates, edema, no viral symptoms

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47
Q

Treatment of strep pharyngitis

A
  • amox

- penicillin

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48
Q

Symptoms of galactokinase deficiency

A

-cataracts only

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49
Q

Symptoms of galactosemia (G1PUTD def)

A
  • FTT
  • hepatomegaly
  • jerky movements
  • cataracts
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50
Q

Most common cardiac defect in trisomy 18

A

VSD

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51
Q

Karyotype assc with Kallmans

A

46 XX

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52
Q

Treatment of neonates with Hep B exposure

A

HepB IVIG + Vaccination

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53
Q

Common manifestation of chronic constipation

A

anal fissures

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54
Q

How does constipation cause bladder infections?

A

urinary retention

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55
Q

Frequent UTIs + parenchymal scarring suggests ____.

A

vesicoureteral reflux

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56
Q

Definition of precocious puberty in girls

A

puberty before age 8

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57
Q

Mass causing precocious puberty in girls

A

granulosa cell tumor

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58
Q

Pathogenesis SCID

A

ADA def –> T cell failure –> B cell dysfxn

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59
Q

Treatment of SCID

A

stem cell transplant

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60
Q

Butthole excoriations=

A

helminth- pinworms-vermicularis

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61
Q

Cause of noninfectious hydrocephalus in low birth weight baby

A

IVH

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62
Q

Treatment of chlamydial conjunctivitis

A

ORAL erythromycin

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63
Q

Most common symptom assc with sickle cell trait

A

painless hematuria

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64
Q

CD19 cells are what type? CD3 cells are what type?

A

CD19- B cells

CD3- T cells

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65
Q

Leukocyte Adhesion Deficiency symptoms

A
  • skin infections without pus, poor wound healing
  • delayed cord separation
  • ^^^neutrophils
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66
Q

Treatment of tourettes

A
  • Antipsychotics

- A receptor agonists

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67
Q

CGD:

  • inheritance pattern
  • assc infections
A
  • XR

- catalase + organisms: staph, serratia, burkholderia, aspergillus

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68
Q

Most common heart defect seen in Downs

A

-Complete atrioventricular septal defect

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69
Q

Life threatening GI condition in neonates with VLBW

A

necrotizing enterocolitis

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70
Q

Abdominal xray finding in NEC

A
  • visible air in bowel wall

- portal venous air (pneumatosis intestinalis)

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71
Q

Afebrile bone pain in SCA cause:

A

-osteonecrosis (avascular necrosis)

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72
Q

Reyes Syndrome histo findings

A

-microvesicular fatty infiltration of the liver

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73
Q

3 bugs patients with SCA are susceptible to

A

s. pneumo
Neisseria
h flu

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74
Q

Jaundice type assc with breast milk jaundice

A

indirect

within 2 weeks of birth

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75
Q

Biliary jaundice type

A

direct

within 8 weeks of birth

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76
Q

Von Gierkes assc enzyme + metabolic findings

A

Glucose 6 Phosphatase

  • low gluc
  • lactic acidosis
  • hyperuricemia
  • hyperlipidemia
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77
Q

Ocular trachoma findings

A
  • neovascularization

- follicular conjunctivitis

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78
Q

Cause of anemia with ^ LDH in SCA

A

extravascular hemolysis

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79
Q

Primary ciliary dyskinesia:

  • mutation in ?
  • low nasal ?
A

dynein

nitrous oxide

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80
Q

Contraindications to breast feeding (maternal)

A
  • street drugs
  • HIV
  • active TB (until 2 weeks antiTB therapy)
  • herpetic lesions
  • varicella infection 5 days before- 2 days after delivery
  • chemo/radiation
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81
Q

Breast feeding reduces maternal risks of which cancers?

A
  • breast

- ovarian

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82
Q

Contraindications to breast feeding (neonatal)

A

galactosemia

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83
Q

In addition to improved immunity, how does breast feeding benefit baby?

A
  • improved GI function

- decreased risk cancer, DM1, NEC

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84
Q

Most common cause of osteomyelitis

A

staph aureus

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85
Q

Cause of osteomyelitis in:
SCA
prosthetic devices

A

SCA: salmonella
Prosthetics: staph epi

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86
Q

Diarrhea, Dermatitis, Dementia
Beefy red tongue

=deficiency of ?

A

niacin

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87
Q

Wernickes cause

A

B1, thiamine deficiency

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88
Q

dermatitis
high homocysteine levels
stomatitis

cause?

A

B6 deficiency, pyridoxine

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89
Q

Absence seizures are assc with what psych condition?

A

ADHD

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90
Q

Cardiac rhabdomyoma + renal angiomyolipomas are assc with?

A

TS

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91
Q

Cataplexy is assc with _____

A

narcolepsy

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92
Q

Tourettes syndrome is assc with what other psych condition?

A

OCD

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93
Q

Optic Gliomas are assc with what syndrome?

A

NF 1

+café au lait; neurofibromas

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94
Q

Most common GI anomaly

A

Meckels diverticulum

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95
Q

Drug assc with rash in mononucleiosis

A

amoxicillin

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96
Q

Thymus location in neonate on CXR

A

Rt sided, triangular

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97
Q

Cause of edema in Turners syndrome

A

dysgenesis of the lymphatic network

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98
Q

Contrast syncope and seizure

A
Syncope= immediate return to consciousness 
Seizure= delayed return to baseline consciousness
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99
Q

Contrast measles and rubella

A

measles= high fever, usually over 104, takes several days to spread
rubella= rash spreads 1 day, lower fever
both spare hands, feet

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100
Q

Location of adenopathy in measles vs rubella

A

measles- cervical adenopathy

rubella- posterior auricular, suboccipital adenopathy

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101
Q

Describe disseminated gonococcal presentation

A
  • rash (vesiculopustular race sparing face)
  • fever
  • polyarthralgias
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102
Q

Describe RMSF rash

A

-wrists ankles —-> centripetal spread

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103
Q

Secondary syphilis rash

A

-full body including palms and soles

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104
Q

Rash assc with varicella

A

-vesicular, appears in successive crops

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105
Q

Symptoms assc with Celiacs

A
  • weight loss
  • IDA
  • dermatitis herpetiformis
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106
Q

Describe appearance of dermatitis herpetiformis in celiacs

A

vesicular rash on extensor surfaces

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107
Q

Risk factors for celiacs

A
  • family hx
  • autoimmune disease (DM1, thyroiditis)
  • IgA deficiency
  • Down Syndrome
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108
Q

Abrupt onset changes in mood, bedwetting, academic difficulties = must rule out

A

abuse

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109
Q

Chronic stridor in an infant is termed

A

laryngomalacia

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110
Q

How is laryngomalacia diagnosed? treated?

A

laryngoscopy, will spontaneously resolve within 18 months

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111
Q

Most common primary immune deficiency?

Causes anaphylactic reaction to ….?

A

Selective IgA deficiency

Blood products

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112
Q

Most common cause of hip pain in children?

PE finding?

A

transient synovitis

abduction, external rotation

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113
Q

Septic arthritis
CRP?
WBC?
ESR

A

CRP greater than 20
White count greater than 12k
ESR greater than 40

+fever

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114
Q

Most common cause of proteinuria in children

A

transient proteinuria –> caused by fever, stress, exercise etc

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115
Q

Followup for isolated event of proteinuria

A

two subsequent tests

if continue to be negative, benign condition, no further workup

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116
Q

Treatment of impetigo

A

Topical mupirocin

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117
Q

Pertussis exposure prophylaxis

A

azithromycin for all contacts

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118
Q

Time of pertussis vaccination

A

-2,4,6 months
-15-18 months
-4-6 years
+ boosters

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119
Q

fever
urticaria
joint pain

cause?

A

serum sickness –> MC cause = B lactams, sulfa drugs

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120
Q

Growing pain features

A
  • bilateral lower extremities
  • primarily at night
  • normal PE
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121
Q

Constitutional growth delay is assc with _____

A

delayed bone age

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122
Q

Hypothyroid assc growth pattern

A
  • abrupt falling off of curve

- delayed bone age

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123
Q

When is VZV vaccination given?

A

1 & 4 years

+post exposure prophylactic vaccination with exposure between 1-4 years. (use VZIG only if immunodeficient)

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124
Q

How often should vision screen be done in kiddos?

A

every well child visit

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125
Q

When is meningococcal vaccination given?

A

11-12 –> booster at 16

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126
Q

When is rotavirus vaccination given?

A

2-8 months

127
Q

In addition to binge eating and temper tantrums, what are some features of prader willis? (4)

A
  • almond eyes
  • small hands and feet
  • hypotonia
  • cryptorchidism
128
Q

Pure red cell aplasia + triphalangeal thumbs

A

Diamond Blackfan

129
Q

Wiskott Aldrich triad

A
  • eczema
  • thrombocytopenia
  • hypogammaglobulinemia
130
Q

Causes of idiopathic aplastic anemia

A
  • chemicals (benzene, phenylbutazone)
  • drugs (chloramphenicol, sulfa)
  • viruses
  • radiation
131
Q

When is transient erythtroblastopenia of childhood typically diagnosed? MCV value?

A

After 1 year of age

usually macrocytic

132
Q

Most common congenital cause of aplastic anemia

A

Fanconis

X linked –> chromosomal breaks

133
Q

Fanconis

  • skin finding
  • blood finding
  • features that are “small”
  • eye and ear anomalies
A
  • hypopigmentation + café au lait
  • aplastic anemia
  • small stature, head, gonads, thumbs
  • strabismus + low set ears
134
Q

Leading cause of B12/cobalamin deficiency

A

pernicious anemia/ IF deficiency

135
Q

Diagnosis of turner

A

Pelvic U/S –> streak ovaries

Karyotype analysis= 45 XO

136
Q

HUS triad

A
  • microangiopathic hemolytic anemia
  • thrombocytopenia
  • acute kidney injury
137
Q

Ferritin increases with ______

A

inflammation, it is an acute phase reactant

138
Q

Lab finding assc with pyloric stenosis

A

hypochloremic metabolic alkalosis

139
Q

Idiopathic avascular necrosis of the hip in young boys

A

Legg Calve Perthes disease

140
Q

Most common population with SCFE

A

obese adolescent boys

141
Q

PE finding in SCFE

A

pts hold hip externally rotated/ fail to internally rotate

142
Q

Pineal gland mass signs

A

-limited upward gaze
-upper eyelid retraction
-pupils respond to accommodation but not light
(dorsal MIDBRAIN SYNDROME!!)

143
Q

What is Colliers sign?

A

upper eyelid retraction caused by pinealoma

144
Q

Ab mediated dancing eyes and feet:
name
cause

A

opsoclonus myoclonus syndrome

neuroblastomas

145
Q

Hep B infection is a risk factor for what type of nephropathy?

A

-membranous

146
Q

How is membranous nephropathy diagnosed?

A

more than 3g/day protein excretion

147
Q

Most common infection assc with focal segmental sclerosing glomerulonephritis

A

HIV

148
Q

Most common cause of nephrotic syndrome in preadolescent children

A

minimal change disease

149
Q

Most common cause of nephritic syndrome

A

post streptococcal glomerulonephritis

150
Q

How does medulloblastoma lead to hydrocephalus

A

4th ventricle obstruction

151
Q

In addition to rockerbottom feet, overlapping digits….

What are other physical characteristics of trisomy 18?

A
  • micrognathia
  • prominent occiput
  • low set ears
  • limited hip motion
152
Q

What two organ systems are affected by trisomy 18?

A
  • renal

- cardiac

153
Q

Trisomy 13 effects

A
  • midline defects (holo, omphalocele, microphtalmia)

- cutis aplasia

154
Q

Typical location Mongolian spots + management

A

buttocks, totally benign

155
Q

Bedwetting is normal up to what age?

A

5; may intervene after age 5 with alarm or DDAVP

156
Q

How is pulmonary function tested in GBS?

A

spirometry

157
Q

Maneuvers that increase the murmur of HCOM

A

Valsalva
Abrupt standing
NG

(all decrease preload)

158
Q

Maneuvers that decrease the murmur of HCOM

A
hand grip (^afterload)
squatting (^pre+after load) 
passive leg raise (^preload)
159
Q

CT finding in retropharyngeal abscess

A

widened prevertebral space

160
Q

Retropharyngeal abscess assc bugs

A

usually polymicrobial including staph aureus, strep pyo, and anaerobes

161
Q

Cyclical vomiting is common in kiddos diagnosed with _______

A

migraines

162
Q

Most common cause of congenital hypothyroid

A

dysgenesis

163
Q

Lymph nodes in what region are always abnormal?

A

supraclavicular

164
Q

Enzyme assc with Lesch Nyhan

A

HGPRT

165
Q

Marfans genetic cause

A

AD mutation of fibrillin 1

166
Q

Lyme disease

  • assc bacteria
  • assc tick
A

borrelia burgdorferi

ixodes scapularis

167
Q

Chemicals for lyme prevention

A
  • DEET

- permethrin

168
Q

Endoscopy is recommended how long after caustic ingestion

A

within 24 hours

169
Q

Contrast Ca/ Po content in breast milk v formula

A

more in formula but better gastric absorption in breast milk

170
Q

Medial deviation of forefoot only=

A

metatarsus adductus –> recovers spontaneously

171
Q

Medial deviaton of forefoot + hindfoot=

A

clubfoot= serial manipulation and casting

172
Q

Ddx of rash in neonate

A
  • ETN
  • HSV
  • Varicella
  • SSS
173
Q

ETN:

  • timing
  • palms+ soles?
A

first two weeks, spares palms and soles

174
Q

Appearance of coxsackie herpangina vs herpetic gingivostomatitis

A

coxsackie: gray, posterior oropharynx
HSV: clusters, posterior oropharynx, erythematous gingiva

175
Q

Treatment of herpangina vs herpetic gingivostomatitis

A

herpangina- supportive

HSV- acyclovir

176
Q

Triple bubble sign cause

A

jejunal atresia – vascular accident

177
Q

Defintive diagnosis of lead poisoning

A

venous lead levels

capillary= false +

178
Q

At what level of lead is chelation therapy begun?

A

45+ DMSA, succimer

70+ EDTA

179
Q

Osteoid osteoma:

  • MC location
  • pain relief
  • malignant?
A
  • proximal femur
  • improved with NSAIDs
  • benign
180
Q

Symptom associated with vaso-occlusive hand foot swelling in SCA

A

fever

181
Q

1 risk assc with RSV

A

apnea

182
Q

Who gets palivizumab for RSV px?

A

preterm less than 29 weeks
BPD
congenital heart disease (hemodynamically significant)

183
Q

Hammer toes + ataxia in males=

A

Freidrichs, AR, GAAepeat

184
Q

MC COD in Freidrichs

A

cardiomyopathy

185
Q

When does milk protein enterocolitis onset?

A

2-8 weeks

spontaneous resolves by 1 year

186
Q

Amenorrhea with evidence of uterus–> next diagnostic step =

A

FSH

187
Q

Treatment for Jervell Lange Neilsen/ Romano Ward

A

propranolol + pacer

188
Q

Contrast JLN and RW

A

JLN: AR, hearing loss
RW: AD no loss hearing

Both= K+ channels

189
Q

BB that is avoided in long QT syndrome

A

Sotalolol, also class III anti-arrhythmic, blocks K+ channels = long QT

190
Q

2 MC common causes viral myocarditis

A

adeno

coxsackie

191
Q

When is renal/ bladder US indicated in children?

A

less than 24 months first febrile UTI

192
Q

Treatment for vaginal foreign body in kiddos

A

irrigation with warm fluid

193
Q

How long should a breath holding spell last

A

less than 1 minute

194
Q

Cause of newborn jaundice in Asian baby

A

decrease hepatic uridine diphosphogluconuate glucuronosyltransferase activity (UGT)

195
Q

Trendelenburg sign cause

A

SGN/gluteus minimus weakness

196
Q

Risks assc with IUGR:

A

hypo- calcemia, glycemia, thermia
polycythemia
asphyxia, meconium aspiration, hypoxia

197
Q

Management of central precocious puberty

A
  • brain imaging

- GnRH analog therapy

198
Q

Contraindications to rotavirus vaccination

A
  • anaphylaxis to ingredient
  • history of inussusception, uncorrected malformation of GI tract
  • SCID
199
Q

Timing of rotavirus vaccination

A

2-6 months

200
Q

Treatment of acute abnormal uterine bleeding

A
  • high dose estrogen, progestin, OCPs

- tranexamic acid

201
Q

Most common cause of abnormal uterine bleeding in adolescents

A

immature HPA axis –> anovulatory cycles

202
Q

How to dx meckels diverticulum

A

technetium 99m pertechnetate scan

203
Q

Rule of 2s for meckels diverticulum?

A

2% prevlance
2:1 male: female ratio
2% symptomatic at age 2
within 2 feet of the ileocecal valve

204
Q

Key symptom of meckels

A

painless hematochezia

205
Q

How is intussusception diagnosed? treated?

A

U/S –> air enema

206
Q

Workup of newborn bilious emesis

A

Abdominal xray to rule out perforation –>
contrast enema to distinguish between hirschsprungs and meconium ileus

(M.I.= obstruction at terminal ileus, hirschsprungs= colon)

207
Q

Erythema chronicum migrans = what?

A

localized lyme disease

208
Q

Treatment of lyme disease meningitis and heart block?

A

ceftriaxone

209
Q

Treatment of localized lyme disease in kiddos under 8 + preggos

A

amoxicillin, cefuroxime

210
Q

Which NF type is assc with megalocephaly?

A

NF1

211
Q

How are pituitary adenomas and craniopharyngiomas distinguished on CT/MRI?

A

craniopharyngioma has calcifications

212
Q

Treatment of hydrocele

A

reassurance… most resolve by 12 months

213
Q

Most common bug in KIDS with CF?

A

staph aureus

214
Q

Treatment of pneumonia in kids with CF?

A

cefepime (MSSA) + vanc (MRSA)

215
Q

MC cause otitis externa

A

pseudomonas

216
Q

Treatment of croup

A

steroids + epi(if mod —> severe)

217
Q

Cause of bilateral chemosis in 5-14 day old?

A

chlamydia

218
Q

MC cause of eye discharge in babies?

A

dacryostenosis (no conjunctival/eyelid inflammation)

219
Q

Treatment of ocular misalignment

A

patch or cycloplegic drops for NORMAL eye

220
Q

Testing needing for patients with emphysema

A

CXR to rule out PTX

221
Q

Two GI complications assc with HSP

A

intussusception

GI hemorrhage

222
Q

ECG and CXR findings assc with tricuspid atresia

A
  • decreased pulmonary markings
  • left axis deviation
  • tall P waves
223
Q

Peripheral smear finding in SCA

A

Howell Jolly Bodies

224
Q

Cause of recurrent sinopulmonary & GI infections AFTER 6 months?

A

X linked agammaglobulinemia

225
Q

Treatment of minimal change disease

A

steroids

226
Q

Angular cheilosis + normocytic anemia cause

A

B2 (riboflavin) def

227
Q

Cheilosis, irritability, glossitis, stomatitis cause

A

B6, pyridoxine def

228
Q

Punctate hemorrhages, corkscrew hair, gingivitis cause

A

scurvy, vit C def

229
Q

MCC causes of bilateral lymphadenopathy

A

EBV,CMV, adeno

230
Q

2 most common causes of acute unilateral lymphadenitis

A

staph aureus

strep pyo

231
Q

Radiographic sign of compartment syndrome

A

triangular large fat pad surrounding bone

232
Q

Acute otitis media:

  • most common bugs
  • accepted treatment
A
  • strep pneumo, h flu, m cat

- 10 days amoxicillin

233
Q

Three foodborne diseases that present with mainly VOMITING

A
  • staph aureus
  • bacillus cereus
  • noroviruses
234
Q

Foodborne illnesses with WATERY, noninflammatory diarrhea (5)

A
  • clostridium
  • ETEC
  • crypto
  • cyclospora
  • tapeworms
235
Q

Most common bacterial cause of rhinosinusitis

A

strep pneumo, h flu
followed by mcat

(same as AOM)

236
Q

21 hydroxylase def causes elevation of what hormone?

A

17-hydroxyprogesterone

237
Q

Several weeks ear drainage + failed abx = suspicion for

A

cholesteatoma

238
Q

First line treatment for epiglottitis

A
endotracheal intubation 
(not steroids + racemic epi!)
239
Q

Virus assc with atopic dermatitis

A

eczema herpeticum

painful vesicles, crusting, fever, lymphadenopathy

240
Q

Wiskott Aldrich:
inheritance
underlying deficit

A

XR

impaired cytoskeleton regulation

241
Q

Café au lait spots + precocious puberty

A

McCune Albright, cAMP kinase defect

242
Q

GI polyposis + mucocutaneous pigmentation, estrogen producing tumor =

A

putz jegher

243
Q

Syndrome assc with McCune Albright

A

Cushings

244
Q

3 P’s of McCune Albright

A
  • precocious puberty
  • pigmentation
  • polyostotic fibrous dysplasia
245
Q

How is measles transmitted

A

airborne!

246
Q

Myotonic Dystrophy genetic cause

A

AD CTG repeat DMPK gene

247
Q

COD in MD

A

respiratory/ cardiac failure

248
Q

Duchenne/Becker genetic causes

A

XR absence vs decrease in dystrophin

249
Q

Neuroblastoma origin cells

A

neural crest cells

250
Q

Radio-opaque ingestion
+ metabolic acidosis
+ hematemesis

cause and treatment

A

iron tox

deferoxamine

251
Q

Humoral deficiency assc with:

  • lw B cells + Igs
  • low Igs
  • low IgA
  • low IgA, IgG
  • Low IgG
A
  • low B Cells + Ig’s: X linked agammaglobulinemia
  • low Igs: CVID
  • low IgA: IgA Def (MC)
  • low IgA, IgG: Hyper IgM
  • Low IgG: IgG subclass def
252
Q

Treatment of stroke in SCA

A

exchange transfusion

253
Q

Prolonged arthropathy in hemophilia cause

A

iron/hemosiderin deposition

254
Q

UMN findings in down syndrome should raise suspicion for

A

atlantoaxial instability

255
Q

Lymphocytes (as opposed to myelocytes) are + for what markers?

A

PAS

TdT

256
Q

Lumbar Puncture + abx–> which comes first in meningitis management?

A

lumbar puncture unless acutely unstable

257
Q

First step in management of CDH

A

CABs! endotracheal intubation

258
Q

Treatment in ITP

A

if asymptomatic –> observe
if symptomatic –> steroids/ IVIG
(symptomatic= bleeding)

259
Q

Most common predisposing factor for bacterial sinusitis

A

viral URI

260
Q

Back pain + neuro dysfunction + palpable step off =

A

spondylolisthesis

261
Q

Most common location for spondylolisthesis

A

L5 over S1

262
Q

Fluid type used for stabilization

A

normal saline, no dextrose until maintenance fluids are began

263
Q

Evanescent pink rash is assc with _____

A

ARF

264
Q

JIA lasts how long?

A

more than 6 weeks joint pain

265
Q

Pain at patellar tendon insertion site

A

Osgood Sclatter/ traction apophysitis

266
Q

Site of patellar tenson insertion

A

tibial tubercle

267
Q

Treatment for Osgood Schlatter

A

NSAIDs

268
Q

Cyanosis that improves with crying

A

choanal atresia

269
Q

Treatment of pyloric stenosis

A

stabilize with fluids/ correct electrolytes –> surgery

270
Q

In addition to prematurity, what four factors increase the risk of respiratory distress syndrome?

A
  • male sex
  • perinatal asphyxia
  • maternal diabetes
  • cesarean section
271
Q

Palpable mass assc with torticollis

A

SCM hypertrophy

272
Q

What should be avoided for stabilization in case of cardiac lesions?

A

Intubation –> not effective for cardiogenic shock

273
Q

Motion assc with absence seziures

A

eyelid fluttering

274
Q

HSP glomerular abnormality assc

A

mesangial deposition of IgA

275
Q

How does knee-chest position alleviate cyanotic spells in TOF?

A

increases systemic vascular resistance –> increased pulmonary blood flow

276
Q

Refeeding syndrome is caused by a surge in _____

A

insulin

277
Q

SCFE symptoms in boys younger than ten may be caused by

A

Legg Calve Perthes Disease

limited internal rotation/ abduction

278
Q

Harsh systolic ejection murmur at LUSB + Single S2 =

A

TOF

279
Q

What are the four types of hypersensitivity reactions?

A

Type I: immediate, IgE
Type II: cytotoxic, IgG/IgM autoantibodies
Type III: antibody, antigen complex
Type IV: delayed, Tcell/ macs

280
Q

Three examples of Type III hypersensitivity:

A

serum sickness
PSGN
lupus nephritis

281
Q

Autoimmune hemolytic anemia + goodpastures syndrome are what types of hypersensitivity reactions?

A

Type II, cytotoxic, IgG/IgM autoantibodies

282
Q

Falling with object in mouth may cause what vascular injury?

A

carotid artery dissection = hemiplegia

283
Q

MC cause urinary tract obstruction leading to Potters?

A

posterior urethral valves

284
Q

1st line therapy for bedwetting

A

alarm –> desmopressin –> tricyclics

285
Q

Ddx of acute stridor

A

croup

foreign body aspiration

286
Q

Ddx of chronic stridor

A

laryngomalacia

vascular ring

287
Q

Cause of anemia of prematurity

A

decreased EPO

288
Q

Risk assc with orchiopexy

A

subfertility

289
Q

Two causes of neonatal botulism

A

ingestion of spores from environmental dust

ingestion of preformed toxins from food

290
Q

Treatment of paraphimosis

A

urgent reduction of prepuce

291
Q

In addition to macrosomia, macroglossia, hemihyperplasia, what defects are seen in BWS?

A

abdominal wall defects, monitor abdominal US + AFP

292
Q

Tumors assc with BWS

A

Wilms

Hepatoblastoma

293
Q

Chromosome assc with BWS

A

11p15

294
Q

Three complications of Mumps

A
  • parotitis
  • orchitis
  • aseptic meningitis
295
Q

Treatment for scabies

A

permethrin

296
Q

mesdiastinal mass + anemia + high ferritin =

A

thymoma

297
Q

midline mass in abdomen w/ calcifications=

A

neuroblastoma

298
Q

linear consolidation in lung lobe at birth

A

congenital emphysema

299
Q

cough + post-tussive emesis - fever=

A

pertussis

300
Q

infantile UTI appropriate abx therapy

A

cefotaxime

301
Q

ingestion:

  • ulcers on lips + tongue
  • agitated + drooling
A

drain cleaner

302
Q

Treatment for HTN emergency

A

nitroprusside

303
Q

air fluid level at tympanic membrane-

A

otitis media with effusion

304
Q

Causes of calcium deficiency with ^^PTH

6

A

1) lack of intake/sun
2) malabsorption
3) liver disease
4) anticonvulsants
5) renal osteodystrophy
6) Vd dep Type 1

305
Q

Causes of PO4 def without ^^ PTH (5)

A

1) Genetic Primary Hypophosphatemia
2) Fanconis
3) RTA II
4) Oncogenic
5) Phosphate malabsorption

306
Q

Vitamin D Dep II is caused by

A

End organ resistance to 1,25 D3

AR

307
Q

Most common non-nutritional tickets cause

A

Genetic primary hypophosphatemia (XD)

Poor 1,25 D formation and PO4 absorption

308
Q

Rachitic rosary and tetany are seen in what rickets type?

A

Nutritional, not familial

309
Q

Vitamin D Dep II cause + inheritance pattern

A

AR, poor 1,25 D formation

310
Q

Cause of ricketts as scheduled with malignancy

A

PEX protein —-> excess PO4 loss in urine and poor 1,25 D formation

311
Q

Two substances found in urine with fanconis

A

AAs

Phosphate

312
Q

What RTA is assc with urinary loss of PO4?

A

RTA II (proximal)

313
Q

Schmid metaphyseal dysplasia

Inheritance pattern + labs

A

AD
Normal labs

(Bowing waddling gait, abnormal imaging)