General pediatrics Flashcards

1
Q

Which growth curve is best for breastfed babies?

A

WHO

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2
Q

Which growth curve is best for formulafed babies?

A

CDC

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3
Q

What is major difference in growth of breast and formula fed babies that is reflected in WHO and CDC?

A

Breastfed babies gain weight faster in first 3 months and then velocity slows down a bit. WHO reflects that.

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4
Q

Describe typical growth percentile pattern from 6 to 12 months.

A

They can cross 1 or 2 major percentile lines because weight at birth is intrauterine and weight during this period is more genetic potential.

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5
Q

Define malnutrition in children <3 yrs old.

A

Weight to height <5th percentile

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6
Q

Growth at 2 yrs of age signifies what?

A

Mean parenteral height

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7
Q

Pattern: mucopurulent cervical discharge, intermenstrual bleeding, cervical friability, and lower abdominal pain

A

Gonorrhea

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8
Q

What is the treatment for gonorrhea?

A

Ceftriaxone 250mg IM x1; Azithro 1g x1 or doxy 100mg twice a day for 7 days.

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9
Q

Best testing for gonorrhea

A

NAAT

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10
Q

Can gonorrhea be tested by NAAT in child abuse?

A

No, because hasn’t been verified for recto and oropharyngeal secretions

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11
Q

Pattern: numerous erythematous papules located on the trunk and extremities, including the hands and feet, penis in boys and areola in girls. Found mostly in flexure surfaces and can see burrowing

A

Scabies

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12
Q

What does rash represent in scabies?

A

hypersensitivity 3 weeks after infestation. So can use hydroxyzine and hydrocortisone, might take 2 to 3 weeks to subside.

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13
Q

What is the treatment for scabies?

A

Permethrin from neck to toes for 8 to 14 hours and then repeat in 7 to 14 days because it is not completely ovicidal.

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14
Q

How do you clean clothing and bedding with scabies?

A

Wash and dry in high heat 60C for 10min or store items in bags for >3 days.

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15
Q

How should asymptomatic households of scabies infection be treated?

A

single application of permethrin at the time the index case is first treated.

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16
Q

What is the difference between action of 3% saline and mannitol?

A

Saline expands intravascular volume while mannitol decreases blood viscosity and causing reflex vasoconstriction and because it is filtered by kidney, can lead to osmotic diuresis. So saline is preferred in trauma to keep intravascular space full.

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17
Q

What is the continuum to coma in bad brain?

A

confusion, delirium, lethargy, and stupor

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18
Q

What is the schedule for meningococcal vaccine?

A

1) All preteens at 11 and 12. Booster at 16. Meningoconjugate
2) 2 months for asplenia or complement deficiency

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19
Q

What serotype is most common? Is there a vaccine for it?

A

Serotype B, yes, there is a vaccine for it; typically used for outbreaks.

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20
Q

Modality best for urinary tract stone evaluation.

A

CT (can better localize, catch small ones, and uric acid stones)

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21
Q

Limitations of US for urinary stones.

A

Can’t visualize small ones and those in the renal calyces, ureters, papilla and other renal structures.

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22
Q

Limitations of Xray for urinary stones.

A

Can’t visualize small ones and uric acid stones

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23
Q

What should be measured on first couple of days of starting TPN?

A

1) Glucose
2) Ca, P, Mg
3) Lipids
4) LFT more chronic problem

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24
Q

What minerals are typically too hi or lo in premature infants?

A

Ca is too lo

P is too hi

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25
Q

Difference in pattern of presentation between urea cycle disorder and organic acidemia?

A

Organic academia -metabolic acidosis; elevated LFT, low blood sugar, neutropenia
Urea cycle - respiratory alkalosis; normal LFT (ammonia from inability to process protein)

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26
Q

Pattern: feeding problems in first 2 days of life, neuro, hyperammonemia, normal glucose, respiratory alkalosis

A

Urea cycle

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27
Q

pattern: feeding problems in first 2 days of life, neuro, hyperammonemia, ketosis, neutropenia, deranged liver function, ketosis, low glucose, metabolic acidosis

A

organic acidemia

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28
Q

Indication for removal of battery immediately.

A

1) If in esophagus
2) if it doesn’t move within 24 hrs
3) symptomatic
4) >20mm

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29
Q

Indication for removal of magnets immediately

A

If 1+ magnets

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30
Q

Treatment for coin ingestions

A

If it has gone past GE sphincter, then watch. Observe for it in stool.
Repeat imaging

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31
Q

Indication for immediate removal of sharp or pointed objects

A

1) If object is >4cm and wider than 2cm

2) Or showing no movement on day 3 after ingestion

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32
Q

Warning signs for s/l delay in 6 month

A

Does not respond to sound

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33
Q

Warning signs for s/l delay in a 9 month

A

Lack of babbling

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34
Q

Warning signs for s/l delay in a 18 month

A

No mama and dada

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35
Q

Warning signs for s/l delay in a 24 month

A

No words

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36
Q

Warning signs for s/l delay in a 30 month

A

No 2-word phrases

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37
Q

Warning signs for s/l delay in a 36 month

A

No 3-word phrases

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38
Q

Age at which they follow 3-step commands

A

4yo

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39
Q

Age at which they can point to 4 or 5 colors

A

4yo

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40
Q

Age at which they can talk about time

A

4yo

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41
Q

What is the first screening for 6 or 9 months old who don’t meet milestones for speech and language?

A

hearing test

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42
Q

Beyond 9 months what is first screening for those who don’t meet speech and language milestones

A

detailed speech eval that will include hearing test

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43
Q

Most common functional innate immune system deficiency? What cell type is affected?

A

CGD

Neutrophil

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44
Q

What is the test for chronic granulomatous disease?

A

neutrophil oxidative burst

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45
Q

Pattern: toddler or child with frequent skin, lung, sinus problems with mainly staph aureus and/or aspergillus

A

CGD

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46
Q

How to treatment pt suspected of having CGD

A

start bactrim

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47
Q

What is the cbc like for pts with CGD?

A

Normal - it s a function disorder

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48
Q

What other immune deficiency looks like CGD but is more rare?

A

Leukocyte adhesion deficiency - inability to leukocytes to leave blood stream and traffic to area of infection

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49
Q

What is most common cause of CHF in newborn? Describe mechanism.

A

HLHS - critical dependent cyanotic congenital heart disease
PVR lowers –> cardiac output shifts from L to R –> lungs congested, systemic output decrease in proportion to increase in pulmonary flow

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50
Q

What is most common cause of CHF in later newborn? Describe mechanism.

A

Large PDA
Blood flow from aorta to pulmonary artery –> increases blood return to pulmonary veins, left atrium, left ventricle and cause overload

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51
Q

What is most common cause of CHF at 4 weeks of age? Describe mechanism.

A

Large VSD
PVR lowers –> increase flow across VSD –> pulmonary vascular congestion and tachypnea –> volume load to the right and subsequently to the left ventricle

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52
Q

What is most common cause of CHF in first few months? Describe mechanism.

A

AV canal or endocardial cushion

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53
Q

What is most common cause of CHF later in childhood? Describe mechanism.

A

Rheumatic heart disease - mitral valve regurgitation, valve will thicken and not close properly so when LV contracts, some of it goes back up to atrium and then comes back down –> over load volume in left atrium and ventricle –> dilate LV –> both systolic and diastolic dysfunction

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54
Q

What is most common cause of CHF in older children?

A

Cardiomyopathy

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55
Q

What is most common cause of CHF in any age?

A

Myocarditis

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56
Q

Which viruses are associated with myocarditis

A

Coxsackie, parvovirus, adenovirus

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57
Q

How is lupus different from rheumatic fever?

A

Many more organs are typically involved.

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58
Q

What are the criteria for rheumatic fever?

A

Joints, cardic, nodules, erythematous patches on skin

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59
Q

Group A strept (GAS) is also called what?

A

Strept pyogenes

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60
Q

Soft tissue infection after trauma is typically caused by what bacteria?

A

MSSA, MRSA, GAS

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61
Q

Of MSSA, MRSA, GAS, which antibiotic would cover which?

1) Clindamycin
2) Cephalexin
3) Cefixime
4) Doxycycline
5) Penicillin

A

1) Clindamycin - MSSA, MRSA, GAS
2) Cephalexin
3) Cefixime
4) Doxycycline
5) Penicillin - MSSA

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62
Q

Describe headache from Chiari I

A

Increased with valsalva, coughing, sneezing, laughing, defecating

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63
Q

Molluscum is caused by what virus

A

Poxvirus

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64
Q

What is typically co-morbid with molluscum?

A

Atopic dermatitis

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65
Q

What are enlarging molluscum lesions that become erythematous, two possibilities?

A

1) immune reaction

2) infection with bacteria

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66
Q

When does molluscum resolve?

A

usually self-resolves

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67
Q

How long can molluscum persist?

A

many months to year if recurrent

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68
Q

Treatments for molluscum?

A

Beetle toxin, iretinonin

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69
Q

What are s/sx of imperforate hymen?

A

bluish bulge, Valsalva causes bulge to increae

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70
Q

What age is most susceptible to growth failure in children with CKD?

A

<1 yr

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71
Q

What are some causes of growth failure in CKD pt?

A

Metabolic, lower insensitivity to GH, acidosis

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72
Q

Pattern: firm, nontender, smooth abdominal mass that does not cross midline with abdominal pain, hematuria, hypertension

A

Wilm’s tumor

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73
Q

What are clinical findings of multicystic dysplastic kidney?

A

Typically asymptomatic

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74
Q

What are clinical findings of ADPK in childhood?

A

Typically asymptomatic but can have hypertension

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75
Q

Pattern: noncommunicating abdominal cysts with intervening dysplastic renal tissue

A

Multicystic dysplastic kidney

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76
Q

Pattern: renal macrocysts +/- hematuria, flank or abdominal pain, infection, asymptomatic hypertension

A

ADPK

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77
Q

Most common dx for antenatal hydronephrosis

A

UPJ obstruction

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78
Q

How does UPJ obstruction present in newborn?

A

Typically US

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79
Q

How does UPJ obstruction present in older children?

A

Recurrent abdominal pain

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80
Q

How do you dx UPJ obstruction?

A

US or can do voiding scan

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81
Q

How does VUR typically present?

A

UTI

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82
Q

What is primary cause of VUR?

A

Ureter is too small

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83
Q

What is secondary VUR cause?

A

Abnormally high pressures in the bladder leading to incompetence of the ureterovesical junction and associated reflux

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84
Q

Difference in Dx test for enterovirus and arbovirus?

A

Arbovirus - IgM antibody

Enterovirus - PCR

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85
Q

If CSF is negative for enterovirus, but still highly suspected, what should you test?

A

Throat and rectal swab

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86
Q

Prognosis for VUR

1) Grade 1 and 2
2) Grade 3 and 4
3) Grade 4

A

1) 60-80% self resolution
2) 10-20%
3) need surgery

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87
Q

How do allergic and nonallergic rhinitis differ?

A

5.26

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88
Q

How many calories does a 12kg child require?

A

1100kcal per day

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89
Q

Which cardiac disorder should PDA patency be maintained?

A

Hypoplastic left hear
Pulmonary atresia
Tricuspid atresia

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90
Q

What are red flags that staph coagulase negative might not be a contaminant of normal flora?

A

1) Repeatedly positive blood cultures
2) symptomatic infection (fever, hypotension, lethargy)
3) IV cath for more than 3 days
4) susceptibility show multiple antibiotic resistance

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91
Q

Mild scoliosis degree and Rx

A

10 to 25 degrees - observation

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92
Q

Moderate scoliosis degree and Rx

A

25 to 45 - bracing

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93
Q

Severe scoliosis degree and Rx

A

45 to 50 - spinal fusion

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94
Q

What should you ask in patient you suspect of scoliosis (ie what are the expected complications)

A

H/o of worsening shoulder or back asymmetry, neurologic symptoms including bowel or blader dysfunction, weakness or pain radiating into the extremities and famil history of scoliosis

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95
Q

What test should you order if you suspect scoliosis and when should you order it?

A

X-ray with any degree of suspicion

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96
Q

When does scoliosis did worse?

A

During years of rapid growth

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97
Q

What are causes of scoliosis?

A

Neuromuscular, NF1 and Marfan

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98
Q

What are signs of toxicity from diphenhydramine?

A

Agitation, confusion, hyperactivity, hallucinations, dry mouth and eyes, dry flushed skin, urinary retention, dilated pupils, tachycardia, tremor and seizures

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99
Q

Lithium toxicity

A

AMS, GI prob, dehydration, tremor, weakness, hyperreflexia, slurred speech, visual disturbances, and seizures

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100
Q

Verapamil toxicity

A

bradycardia and hypotension, CVS toxicity and shock

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101
Q

In T21 pt with AV canal defects, what other problems can occur?

A

Valvular problems such as MVR

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102
Q

Which murmur is both diastolic and systolic

A

Mitral valve regurgitation

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103
Q

What are indications for immunotherapy for allergies?

A

Cardiac disease. Other conditions you should be careful of: autoimmune, malignancy, immunodeficiency

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104
Q

What are contraindications for immunotherapy for allergies?

A

Asthma

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105
Q

What drugs are contraindicated during immunotherapy for allergies?

A

ACE-inhibitors can accentuate angioedema; B-blockers can decrase response to epinephrine

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106
Q

What precautions should be taken during immunotherapy?

A

Should observe for 30 minutes after each injection.

Should have epi pen at home in case

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107
Q

Pattern: Child between 4 to 8 years old, h/o limp and mild activity-related pain, decreased internal rotation and abduction fo the affected hip.

A

Legg-Calve-Perthese

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108
Q

What is the etiology of Legg-Calve-Perthes?

A

Inadequate blood supply to the femoral head epiphysis leads to synovitis and early necrosis, resulting in collapse of femoral head, which reossifies in the healing phase and may not fit well in the acetabulum

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109
Q

What is the xray finding for Legg-Calve-Perthes?

A

Collapse of femoral head

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110
Q

Pattern: Rapidly progressive, severe pain, fever, and refusal to bear weight

A

Septic arthritis

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111
Q

Pattern: bilateral hip pain with morning stiffness and improves with activity

A

JIA

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112
Q

Pattern: unilateral hip pain in obese child between 11 and 15 years

A

Slipped capital femoral epiphysis

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113
Q

Duration of a transient synovitis?

A

Typically less than 1 week

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114
Q

What is erysipelas?

A

Skin infection mainly caused by GAS, Group A strep, strep pyogenes

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115
Q

What is major cause of erysipelas?

A

GAS (strep pyogenes)

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116
Q

What is the most convenient treatment of erysipelas?

A

IV ceftriaxone (once daily so can do outpatient)

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117
Q

Pattern: large, raised, tender area of nonfluctuant induration and erythema with clear borders over the left cheek

A

Erysipelas

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118
Q

Strep throat caused by which streptococcus?

A

GAS (strep pyogenes)

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119
Q

In chidren 1 to 3 years of age, the most common manifestation of GAS infection is what?

A

Febrile, protracted illness accompanied by rhinitis called streptococcosis

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120
Q

If negative rapid antigen testing for GAS, then what?

A

should send for bacterial culture

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121
Q

How do you make diagnosis of streptococcal toxic shock syndrome?

A
Isolation of GAS +2/many
1) hypotension
2) erythematous macular rash
3) Severe soft tissue infection
4) Respiratory distress; 
5) Coagulopathy
6) Elevated liver enzymes 
7) Increased bilirubin
7) isolated creatinine levels
.
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122
Q

What antibiotics for GAS infection?

A

penicillin

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123
Q

How do you treat GAS in pt allergic to penicillin?

A

clindamycin, erythromycin, azithro, clarithro

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124
Q

Should clindamycin be used for life-threatening infections? Alternative?

A

No, need vancomycin

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125
Q

Pattern: CNS excitation, HTN, tachy, hyperthermia, diaphoresis, and dilated pupils?

A

cocaine

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126
Q

What is the agent of choice for patient with mild to moderate cocaine toxicity?

A

IV diazepam

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127
Q

How would you describe 46, XY DSD?

A

Male with amibiguous genitalia

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128
Q

How would you describe 46, XX DSD?

A

Female with ambiguous genitalia

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129
Q

What is the most common cuase of 46, XY DSD?

A

partial androgen insensitiviy

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130
Q

What is the inheritance pattern of androgen insensitivity?

A

X-linked

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131
Q

Would a boy or girl with 21-hydroxylase deficiency have ambiguous genitalia

A

Girl

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132
Q

Pattern: child looks female, but has blind vaginal pouch and no Mullerian strutures?

A

Full androgen insensitivity

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133
Q

Pattern: 46, XY DSD, undescended testes, severe kidney disease

A

Denys-Drash

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134
Q

What are the three critieria for distinguishing acute bacterial from viral rhiosinusitis?

A

1) no improvement in 10 days
2) Onset with severe symptoms/sign of high fever, purulent
3) Worsening symptoms or signs characterized by new onset fever, headache or increase in nasal discharge following typical upper respiratory infection

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135
Q

What one process is most critical in development of metabolic syndrome?

A

insulin resistance

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136
Q

What is nonalcoholic steatohepatitis (NASH)?

A

inflammation from fatty liver

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137
Q

Pattern: 1 to 5mm flesh-colored, verrucous papules in the genital that may coalesce into large plaques

A

papillomavirus or condylomata acuminata

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138
Q

What is condylomata acuminate caused by?

A

HPV

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139
Q

How do you treat condylomata acuminate?

A

topical products - imiquimod or podophyllotoxin

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140
Q

How do you treat mild acne?

A

benzoyl peroxide, BPO/antibiotic once daily for inflammatory or mixed; topical retinoid for comedonal

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141
Q

How do you treat moderate acne?

A

Only face: topical RA at bedtime and antimicrobial in the AM; face and chest or back - topical retinois and oral antibiotic

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142
Q

How do you treat severe acne?

A

Topical retinoid for face (increase potency); high-dose oral antibiotic; Add BPO to the face; contraceptive for woman

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143
Q

What changes make up the diving reflex?

A

bradycardia, apnea and laryngospasm

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144
Q

In a drowning or near-drowning case, what should be maintained during therapy?

A

oxygenation, ventilation, maintenance of blood pressure

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145
Q

Malnutrition can affect what apart of immune system? What specifically?

A

atrophy of lymphoid tissue, decreased cell-mediated immunity, decreased immunoglobulin and complement levels, and diminished phagocytosis

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146
Q

What is most important arm of immune system for fighting TB?

A

lymphocytes via helper T cells

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147
Q

PPD is based on activity of what part of immune system?

A

adaptive

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148
Q

Rx for edema and HTN in patietns with GN?

A

furosemide

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149
Q

ACE acts on blunting RAA system via decreased production of ATII, what does it do?

A

1) arteriolar vasoconstriction
2) sympathetic nervous system activation
3) aldosterone secretion leading to sodium and water reabsorption
4) stimulation of vascular and myocardial fibrosis

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150
Q

When is IV hydralazine used?

A

in emergency situations to lower blood pressure

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151
Q

What drug is most commonly used for persistently elevated BP despite edema resolution such as in cases with BP associated with prednisone or other immunosuppressive therapy or chronic kidney disease?

A

ACE inhibitor or calcium channel blockers

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152
Q

Fat malaborption in chronic liver disease is caused via multiple mechanisms. What are they?

A

1) inadequate bile production
2) vascular congestion
3) small bowel bacterial overgrowth resulting in bile deconjugation

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153
Q

Pattern: liver problem, peripheral neuropathy, and hemolysis

A

Vit E deficiency

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154
Q

Pattern: liver problem, osteomalacia and rickets

A

Vit D deficiency

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155
Q

Pattern: liver problem and coagulopathy

A

Vit K deficiency

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156
Q

Pattern: liver problem and night blindness

A

Vit A deficiency

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157
Q

How you manage fat malabsorption from chronic liver disease?

A

give medium chain fatty acid and supplement with ADEK

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158
Q

Describe all defects in VACTERL?

A
Vertebra abnormalities
Anal atreasia
Cardiac defects
TE fistula
Renal anomalies
Limb abnormalties
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159
Q

Describe all defects in CHARGE?

A

Choanal atresia
Heart deficits
Anal
Renal

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160
Q

What is limitation on breastfeeding mother with varicella 4 days before delivery (any perinatal period)?

A

Cannot breastfeed, but can pump

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161
Q

What is limitation on breastfeeding with mother recently diagnosed with untreated brucellosis?

A

Cannot breastfeed or provide milk

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162
Q

What is limitation on breastfeeding with mother in US with HIV, HTLV1, 2?

A

Cannot breastfeed or provide milk

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163
Q

What is limitation on breastfeeding with mother who received live attenuated rubella virus vaccine immediately after delivery?

A

Ok to breastfeed

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164
Q

Palatal purpura or petechiae are seen in context of what disorder?

A

Clotting problem

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165
Q

What is special about the glabella area with regards to trauma?

A

Blood doesn’t dissolve there readily so will accumulate

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166
Q

What three infection would be contraindicated for breastfeeding, but expressed milk can be offered?

A

Varicella, untreated active infectious tuberculosis, or herpes simplex lesions on the breast

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167
Q

What vitamin/mineral deficiencies have also been linked to impaired immune responses?

A

VitD and zinc

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168
Q

What would a small for gestational newborn look like in general and then specifically?

A

General - thin and lanky
Specifically - decreased muscle mass, dry peeling skin, increased plantar creasing, diminsed breast tissue, underdeveloped ear cartilage and clitoral prominence

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169
Q

What are small for gestational age newborns at risk for?

A

hypothermia, hypoglycemia, polycythemia

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170
Q

Pattern: linear growth failure, weight gain, pubertal changes from excess androgen production, easy bruising, facial plethora, myopathy, striae

A

Cushing

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171
Q

What are all the different ways you can evaluate for Cushing?

A

1) 24 hour urine free cortisol
2) overnight dex suppression
3) late night salivary cortisol
4) diurnal variation in cortisol

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172
Q

Which minerals accumulated during the third trimester so that premature newborns are at risk for deficiencies of them?

A
Calcium
Phosphorus
Iron
Copper
Zinc
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173
Q

What is the recommendation for vitD in preterm babies?

A

<1500g - 200 to 300IU

>1500g 400IU

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174
Q

Recommendtion for calcium and phosphorus in premature newborns?

A

150 to 200mg/kg of calcium

60 to 75mg/kg of phosphorus each day

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175
Q

How much iron is recommended for preterm infants?

A

2 to 3mg/kg per day beginning at 1 to 2 months of age

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176
Q

What abnormal lab findings can be seen during normal puberty?

A

1) increase alkaline phosphatase
2) Increased insulin secretion
3) increased LH, FSH (will plateau when sexual maturity rating 3 is achieved)
4) estradiol and testosterone

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177
Q

Tanner 2 and 3 male

A

2 - enlargement of testes and scrotum

3- enlargement of testes and scrotum with penile growth

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178
Q

Tanner 4 and 5 male

A

4 - Continued growth with enlargement of the glans

5 - mature male genitalia with mature pubic hair

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179
Q

Tanner 2 and 3 for girls

A

Breast
2 - breast bud
3 - breast elevation

Pubic hair
2 - presexual hair
3 - sexual hair

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180
Q

Tanner 4 and 5 for girls

A

Breast
4 - Areolar mound
5 - Adult contour

Pubic hair
4 - mid-escutcheon
5 - female escutcheon

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181
Q

Pattern: round to oval patches of complete hair loss

A

alopecia areata

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182
Q

Pattern: round to oval patch of hair thinning located at the occiput in young infants who spend much of their time in the supine position

A

Friction alopecia

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183
Q

Pattern: 1 or more patches of alopecia are present as well as scale and black-dot hairs

A

Tinea capitis

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184
Q

Pattern: thinning of hair in areas where the hair is being tretched as the result of braiding or creating ponytails

A

Traction alopecia

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185
Q

Who is at risk for HIV and should be tested at least once?

A

Pt that live in area where prevalence of HIV is more than 0.1%
Teens who are sexually active

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186
Q

Who is at high risk of HIV and should be tested annually?

A

Teens with multiple sexual partners
Men with men
IV drug user

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187
Q

What are causes of transient antenatal hydronephrosis?

A

UT obstruction or VUR

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188
Q

What kidney feature on US suggest patient is at greatest risk for congenital anomalies of the kidneys and urinary tract?

A

1) severe hydronephrosis (renal-pelvic diameter >15mm)

2) Bilateral hydronephrosis

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189
Q

Protein requirement for 24-30 weeker

A

3.5 to 4g/kg/day

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190
Q

Protein requirement for 30-40 weeker

A

3g/kg/day

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191
Q

Protein requirement for term infant to 12 months

A

2-3g/kg/day

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192
Q

Protein requirement for children 1-12 years

A

1.5 to 2.5g/kg/day

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193
Q

Protein requirement for children >12

A

1-1.5g/kg/day

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194
Q

Pattern: microcephaly, epicanthal folds, micrognathia, broad nasal bridge, hypertelorism, downward-slanting palpebral fissure, ID, congenital heart disease, renal anomalies, hypospadias, cryptocorchdism, ear tags

A

Cri-du-chat deletion of short arm of chromosome 5

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195
Q

Pattern: Bleeding in child with normal PT/PTT/platelet number

A

Platelet function problem
1) Bernard-Soulier syndrome (adhesion)
2) Glanzmann thrombastenia (aggregation)
So transfuse functional platelets

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196
Q

Pattern: mild to moderate mucosal bleeding and patients present later in life after toddler hood

A

VWD

197
Q

What other abnormality is associated with bicuspid aortic valve?

A

Coarctation

198
Q

What is normal differential between arm and leg blood pressure?

A

Let>arm because of amplification of systolic pressure as blood travels down the aorta

199
Q

BP in leg< arm, suspect what?

A

Coarctation

200
Q

What percentage of those with bicuspid aortic valve will develop complications? What are the complications?

A

30% during lifetime; aortic regurgitation, aortic stenosis, dilation of the proximal aorta in adulthood
2% in adolescent

201
Q

What labs are typically elevated in juvenile dermatomyositis?

A

CPK, AST, ALT, aldolase, LDH

202
Q

Palpation of medial joint is useful for assessment of what?

A

Medial meniscal tear

203
Q

Tenderness on patellar facet palpation indicates what?

A

Patellofemoral pain syndrome

204
Q

Apley grind test evaluates what?

A

Meniscal tear

205
Q

What are some comorbid endocrine conditions with SCFE?

A

HypoTH

Panhypopit

206
Q

What is the treatment for SCFE?

A

Stop bearing weight, orthopedics for urgent surgery

207
Q

The higher degree of slippage in SCFE is associated with a higher risk of what?

A

osteoarthritis

208
Q

What is the treatment for toxoplasmosis?

A

Pyrimethamine + sulfadiazine, clindamycin, atovaquone or Bactrim

209
Q

What are indications for treating toxoplasmosis?

A

1) Pregnancy
2) Immunocompromised status
3) Chorioretinitis or organ damage

210
Q

Differentiate eye problems associated with CMV, HSV, Acanthamoeba, and toxoplasmosis

A

1) CMV - anterior chamber of the eye
2) HSV - keratitis presenting as ocular pain, visual blurring and discharge (conjunctivitis, chemosis, dendritic lesions of the cornea, and decreased corneal sensation) or retinal necrosis
3) Acanthamoeba - Keratitis, similar to HSV; present with severe pain, photophobia, tearing and sensation of a foreign body in the eye (dx with scrapings)
4) Toxo - chorioretinitis

211
Q

How does congenital toxoplasmosis present?

A

Most are asymptomatic but can have impairment later in life with learning disability, mental retardation, hearing loss or vision impairment later in life
Symptomatic patients: lymphadenopathy, rash, hepatosplenomegaly, petechie, thrombocytopenia, pneumonitis, meningoencephalitis, chorioretinitis.

212
Q

How does toxo I immunocompromised person present?

A

PNA, myocarditis, pericarditis, hepatitis, and cutaneous involvement, encephalitis

213
Q

What is the insensible water loss in extremely premature infants and what is it in full-term infant?

A

200ml/kg and 20ml/kg

214
Q

Activation of the erythropoietin system occurs at what age?

A

34 to 36 weeks

215
Q

Transfusion of infants with anemia of prematurity can change what vital sign parameters?

A

Decrease resting heart rate and respiratory rates, increase arterial oxygen content; may not change apnea frequency or oxygen saturation

216
Q

HHV-6 outbreak occurs through which mechanism?

A

Everyone is exposed by 2yo. They remain latent and re-activate with immunosuppression.

217
Q

When is HHV-6 likely to appear in transplant patient?

A

2-4 weeks after transplant

218
Q

What kind of immunosuppression is caused by tacrolimus?

A

Lymphopenia, thrombocytopenia, and anemia

219
Q

What kind of immunosuppression is caused by Bactrim?

A

Lymphopenia, thrombocytopenia and anemia

220
Q

What are manifestations of HHV-6 infection post-transplant?

A

Rash, encephalitis, hepatitis, bone-marrow suppression, PNA, fever, graft rejection

221
Q

What are the benefits of circumcision?

A

Lower UTI, HIV and other STD transmission, penile cancer risk

222
Q

Anesthesia for circumcision

A

Penile nerve block and subcutaneous ring block, topical can irritate the skin

223
Q

Contraindication for circumcision

A

Blood dyscrasia or family history of bleeding disorder, congenital penile abnormalities (hypospadias or chordee)

224
Q

What is the Rx for recurrent lower urinary tract infection?

A

Postcoital antibiotic PPx - TMP, TMP-SMX, nitrofurantoin

225
Q

How do you diagnosis urethral stricture?

A

retrograde urethrography or direct visualization (cystourethroscopy)

226
Q

What is elevated and reduced in tumor lysis syndrome?

A

Elevated - potassium, uric acid, LDH and phosphorus (PULP)

Reduced - calcium

227
Q

What is the risk of having elevated uric acid?

A

Obstructive uropathy

228
Q

What is the difference in mechanism between allopurinol and rasburicase in decreasing risk for obstructive uropathy?

A

Allopurinol inhibits xanthine and hypoxanthine from becoming uric acid and rasburicase transforms uric acid to soluble allantoin

229
Q

Describe the four characteristics of X-linked recessive disorder

A
  • 1) Affects mainly males
  • 2) No male to male transmission
  • 3) Affect males usually born to unaffected parents; mother is the carrier, is asymptomatic, and has an affected male relative
  • 4) females may be affected if skewed X inactivation or father affected and mother is carrier
230
Q

Describe the 4 characteristics of X-linked dominant disorders

A
  • 1) Affects either sex, but female more than males
  • 2) Females often milder phenotype and with more variable expressivity
  • 3) Offspring of affected female have 50% chance of being affected
  • 4) affected male has all affected daughters but no affected sons
231
Q

First sign of puberty in female

A

Breast development - thelarche

232
Q

When does thelarche typically occur? Average

A

10.7+/- 1 year

233
Q

What is the span of time between the onset of breast development and the age of menarache?

A

2.3+/-1year but range is 0.5 to 5.75 years

234
Q

When does males begin development of secondary sexual characteristics?

A

9-14 years of age

235
Q

How long does it take to progress through puberty for males from stages 2 to 5

A

2 to 5 years

236
Q

What factor has been isolated for earlier development in girls of breast?

A

Obesity

237
Q

What should you think of it you see a girl with early puberty or rapid pubertal progression?

A

Pathology such as granulosa cell tumor; something that causes increase in estrogen production

238
Q

What lab values are increased during puberty?

A

Alk Phos during linear growth
LH/FSH
All sex hormones
Insulin

239
Q

What is average peak growth velocity of girls?

A

8 to 9 cm/year by sexual maturity

240
Q

What is average peak growth velocity of boys?

A

10cm/year by sexual maturity

241
Q

After menarche, how much more growth is there?

A

7.5cm linear growth potential remaining

242
Q

What is hgb level at birth?

A

17g/dL

243
Q

when does hgb nadir?

A

2 months to 9-11g/dL

244
Q

What is preterm hgb nadir level?

A

can be 9.5 to 9

245
Q

What is normal reticulocyte count at 4 mo?

A

<2%

246
Q

Name all causes of microcytic anemia

A

lead poisoning, iron def, beta ( more common) thal, chronic inflammation, hemoglobinopathy, sideroblastic anemia

247
Q

What is the RDW for iron deficiency

A

> 14.5

248
Q

what is the mentzer index and interprestation

A

MCV/RBC count

<13 then beta thal

249
Q

Most common transfusion reaction and cause

A

fever, from cytokines, so wash with leukodepletion of granulocytes

250
Q

When should babies start taking iron supplementation?

A

At 4 mo if exclusively breastfed

251
Q

What are nondietary causes of iron deficiency

A

blood loss, PNH, pulm hemosiderosis

252
Q

How much iron for deficiency

A

2-4mg of elemental iron which is 20% of iron sulfate, best with acidic fluid

253
Q

Description route of vitB12 absorption

A

bins to haptocorrin and travels to duodenum than released, then binds to intrinsic factor and goes to ileum and enters blood stream and binds to transcobalamin

254
Q

Pernicious anemia causes

A

absence of terminal ileum or deficiency of intrinsic factor

255
Q

Labs for 21-hydroxylase deficiency

A

17-hydroxyprogesterone, glucose and electrolyte

256
Q

How do you treat 21-hydroxylase deficiency

A

hydrocortisone -mineral and glucocorticoid

257
Q

Clinical sign of 46 XY DSD with partial androgen insensitivity

A

bifid scrotum, gonads, small phallus, hypospadias

258
Q

Clinical sign of total androgen insensitivity

A

look female

259
Q

Inheritance of androgen insensitivity

A

X-linked

260
Q

Mutation in WT1 gene, testes undescended, severe kidney disease

A

Denys-Drash

261
Q

Endocrine causes of short stature

A

GH deficiency, hypoTH, Cushing

262
Q

Test for Cushing

A

24-hour urine-free cortisol

263
Q

Tests for GH deficiency

A

IGF-1, IGF-BP3

264
Q

Rx for idiopathic short stature

A

2.25 SD below or 1.2 percentile can get GH

265
Q

Signs of peripheral precoious puberty

A

body odor, pubic hair, signs of virilization, but no breast development

266
Q

Labs for peripheral precocious puberty

A

17-hydroxyprogesterone; total T

267
Q

Labs for central

A

LH, FSH, estradiol, hCG

268
Q

How can hCG contribute to central puberty

A

can bind to LH receptor and lead to testicular activation in the absence of GnRH release

269
Q

Signs and significance of premature thelarche

A

Breasts only; no workup needed; growth not delayed

270
Q

Causes of congenital hypoTH

A

Aplasia of TH

Mother has Graves and antibodies cross to inhibit TSH receptors

271
Q

When should treatment begin for congenital hypoTH

A

before 2 weeks of life with 10ug/kg

272
Q

Symptoms of Addison

A

Ill-defined; fatigue, generalized muscular weakness, loss of appetite, poor weight gain

273
Q

Typical lab results for Addison

A

hyponatremia, hyperkalemia, hypoglycemia, adrenal antibodies

274
Q

What is secondary AI

A

Due to discontinuation of exogenous corticosteroid

275
Q

How is secondary different from primary AI

A

Secondary AI, isolated hyponatremia without potassium

276
Q

What additional testing for another disease should you routinely evaluate in pt with new-onset type 1 DM

A

Q1 to 2 years check thyroid function

277
Q

Signs of hypocalcemia

A
  • Trousseau - blood pressure in place for a long time depletes tissue of calcium and causes spasm of hand/forearm
  • Chvostek - Tap facial nerve at angle of jaw, facial muscles on same side of the face will contract momentarily
278
Q

Signs of hypercalcemia

A

polyuria, polydipsia, headache, nausea, abdominal pain, mental status changes can also occur

279
Q

Why is there urinary concentrating defect in hypercalcemia?

A

Vasopressin action is inhibited

280
Q

What are common conditions associated with congenital scoliosis?

A

CP, SMA, NF, Marfan, thethered spinal cord, Chiari I malformation with syrinx

281
Q

What is genu varum

A

bowing of legs

282
Q

What are causes of genu varus?

A

Rickets, infantile tibia vara (walking an early age and obesity), skeletal dysplasic, achondroplasia

283
Q

What is genu valgum?

A

knocked knees; kids with genu varum will subsequently develop valgum that peaks around 3 and gradually improved by age 8

284
Q

Pain worsen with hyperextension

A

Spondylolysis

285
Q

Dx for spondylolysis

A

oblique films

286
Q

How do you manage back pain?

A

1) If nothing specific on exam, NSAID and PT first.
2) Revaluate in 1 mo and get AP and lat films maybe with oblique view;
3) lab tests to rule out infection, inflammation cancer;
4) if nothing than MRI

287
Q

How do you manage Down patient who wants to participate in sports?

A

1) Asymptomatic children do not need routing radiographic screening.
2) If there is cervical spine compression, then get AP and lat x-ray and urgently refer to peds ortho or neurosurgery

288
Q

Clinical findings of heat stroke

A

AMS, hypotension, tachypnea, sustained tachycardia, vomiting and decreased sweating with dry skin

289
Q

Evaluation and management of heat stroke

A

Check temp

Rapid cooling with ice water bath or subsequent fluid bolus after intiating cooling

290
Q

What is exercise-associated collapse

A

venous pooling in legs –> hypothension; so place supine, elevate legs and offer oral fluids

291
Q

Confusion and loss of consciousness after sports event

A

hyponatremia

292
Q

Return to play after concussion

A

step-wise gradual return once asymptomatic for 24 hours

293
Q

What does a hyphema look like?

A

collection blood in the anterior chamber of the eye from disruption of the blood vessels of the iris

294
Q

How do you treat hyphema?

A

urgent evaluation by ophthalmologist because of risk of additional bleeding and large collection that can stain cornea or cause glaucoma

295
Q

What does globe rupture look like

A

sever pain, lack of normal extraocular motion, disruption of the sclera or cornea, decreased visual acuity
Emergency ophthalmologic evaluation

296
Q

Management of overuse injury

A

Rest for 4 weeks and PT afterwards; for avulsions >0.5 to 1cm, surgery may be required

297
Q

What is little league elbow?

A

widening of the distal humerus medial epicondyle epiphysis

298
Q

How does little league elbow present?

A

tendernss over the medial epicondyle of the elbow with decr ROM; Xray show widening of the epiphysis +/- avulsion of piece of the medial epicondyle epiphysis

299
Q

What is Osgood-Schlatter syndrome

A

Irritation of the tibial tubercle apoplysis

300
Q

Heel pain that worsens when you squeeze it

A

Sever

301
Q

How do you treat sever?

A

padded help cups and allow return to play because this is a chronic condition that can last for 1-2 years

302
Q

What is sever disease

A

calcaneal apophysisitis from traction on the apoplysis by the Achilles tendon and repetitive impact to the heel

303
Q

What kind of protective eyewear for sports?

A

Shatter-resistant polycarbonate lenses

304
Q

What is the treatment for auricular hematoma

A

prompt drainage with application of a compressive dressing

305
Q

What is a burner or stinger?

A

mild brachial plexus injury; pain radiating down the arm, weakness and paresthesias in the arm and shoulder for <24 hours

306
Q

What is the natural history of shoulder dislocation?

A

High rates of rediclocation after initial traumatic dislocation

307
Q

How do you manage a dislocated shoulder?

A

1) Xray first to look for specific lesion
2) Reduce to restore normal position of humeral head
3) Shoulder immobilizing for 2 to 3 weeks
4) PT
5) Elective surgical repair of torn ligaments and labrum

308
Q

What is the optimal age-appropriate replacement for fluid losses?

A

Hydration every 20 minutes during physical activity in pre and adolescents
For exercise lasting 1 hour or less, water is sufficient

309
Q

Name the performance enhancing drug

Adrogen effects

A

Androstenedione, nadrolone

310
Q

Name the performance enhancing drug:

improve energy level and exercise tolerance but side effect is abdominal discomfort

A

Coenzyme Q

311
Q

Name the performance enhancing drug:

Enhance oxygen-carrying capacity but can cause hyperviscosity of the blood and thrombosis

A

EPO

312
Q

Name the performance enhancing drug

Simulants - weight loss and trouble sleeping

A

Phentermine, methylphen

313
Q

Name the performance enhancing drug

Short burst of high-intensity exercise; muscle cramping and diarrhea

A

creatine

314
Q

Name the performance enhancing drug

Stimulat that can casue arrhythmias, seizures and stroke and HTN

A

ephedrine

315
Q

What are the symptoms of congenital CMV?

A

blueberry muffin rash, thrombocytopenia; IUGR, microcephaly, cerebral atrophy, periventricular intracerebral calcifications, hepatosplenomegaly, jaundice, chorioretinitis

316
Q

What cause oral hairy leukoplakia in HIV patient

A

CMV

317
Q

What disease is EBV associated with

A

X-linked lymphoproliferative syndromes, Burkitt lymphoma, nasopharyngeal carcinoma

318
Q

EBV IgM-viral capsid antigen

A

Acute primary or very recent-past infection

319
Q

EB nuclear antigen

A

convalescing or post-EBV

320
Q

EBV IgG

A

not much help

321
Q

At what age can monospot test be sensitive

A

> 50% after 2-4 years

322
Q

What are symptoms of EBV?

A

Fever, pharyngitis, tonsillitiss, lymphadenopathy, abnormal liver function

323
Q

What are complications of EBV infection

A

Airway obstruction, neurologic complications, severe hepatitis, myocarditis or hemolytic anemia (can use corticosteroids)

324
Q

What are the three forms of neonatal HSV

A

45% SEM (skin, eyes, mouth); 30% CNS only; 25% disseminate (liver, lung and CNS involvement)

325
Q

How do you test for HSV?

A

1) culture of mouth, nasopharynx, conjunctivae, and anus
2) HSV culture and PCR of skin lesions and CSF; blood PCR
3) liver enzymes

326
Q

what is the treatment for HSV?

A

14 days of acyclovir for SEM and 21 days for CNS and disseminated; continue oral for 6 months;
IV always in immunocompromised host

327
Q

Describe clinical findings associated with OI

A

bowing, thin cortices, thin beaded ribs, severe osteoporosis, Wormian bones in the skull, codfish vertebrae, multiple fractures, blue or grey sclerae, SGA, and relative macrocephaly

328
Q

Describe OT type 1 findings and complications

A

mild, normal lifespan, near-normal stuature, ligamentous laxity; no bony deformity

329
Q

Describe OT type 2

A

bad, perinatal lethal due to respiratory insufficiency because of narrow horax, multiple rib fracture or falail chest

330
Q

Describe OT type 3

A

Dentinogeneis imperfect, denal care but can have respiratory insufficiency too

331
Q

Describe OT type 4

A

Common variable OI with normal sclerae

332
Q

What are some common long-term complications?

A

short stature <3 ft, hearing loss both conductive and sensorineural because of fractures in the middle ear bones; basilar impression (upward protrusion of the top of the spine into the base of the skull - HA, double vision, poor coordination or limb weakness)

333
Q

What are clinical findings of achondroplasia

A

short stature, short arms and legs, macrocephaly, frontal bossing and midfacial retrusion; normal intelligence and life span

334
Q

What are some complications of achondroplasia?

A

Craniocervial junction compression, hydrocephalus, delayed motor milestones, sleep apnea, kypohosis, bowing of the legs, obesity and lumbar spinal stenosis

335
Q

What evaluation should infants with achondroplasia have?

A

Repeat hearing at 1 yr, CT or MRI of craniocervical junction, o/n polysomnography and thorough neurologic examination for cervical myelopathy - signs include lower limb, hyperreflexia or clonus, central hypopnea on sleep study or reduced formane magnum on CT

336
Q

Are the three causes of in-toeing

A

Infants - metatarsus adductus
Toddlers - tibial torsion
>3yrs - femoral anteversion

337
Q

What is femoral anteversion?

A

Increase in angle of the femoral neck >20 degrees relative to shaft of the femur

338
Q

When does femoral anteversion happen mostly and what is its nature history

A

In girls who sit with W position, increases in severity until 5 years of age, then spontaneously resolve by 12 years of age

339
Q

How do kids with tibial torsion often present?

A

Parents report clumsiness and frequent tripping, can occur unilater or bilateral

340
Q

What evaluation do you do for tibial torsion?

A

Thigh-foot angle to confirm presence of tibial torsion

341
Q

When is orthopedic evaluation necessary for tibial torsion

A

When it causes out-toeing which can be associated with neuromuscular conditions

342
Q

What are etiology of osteomyelitis in patients of various ages

A

Neonate - GBS, G- enteric
<3 years old - kingella kingae
Older - staph aureus
children with hemoglobinopathies - sickle cell disease

343
Q

Where does hematogenous osteomyelitis typically occur?

A

Metaphysis of long bones and may follow minor trauma to the infected area

344
Q

Management for osteomyelitis

A

pending cx - start clindamycin

345
Q

What are etiology of pyogenic arthritis in different age groups

A

Neonates - GAS, coag- staph, enterobactericiae, candida
Young children - Kingella kingae, strep PNA, H. influenza
Adolescents - neiserria gonorrhoeae

346
Q

What is the clinical findings of pyogenic arthritis

A

ill-appearing with fever, pain and swelling, pseudoparalysis; CRP/ESR elevated, widening of joint space with join effusion on radiographic imaging, synovial fluid WBC >50K with a neutrophil predominance

347
Q

Evaluation of pyogenic arthritis in patients

A

CRP/ESR, snovial fluid, x-ray

348
Q

Antimicrobial management of pyogenic arthritis

A
GAS  - PNC
Gram+ resistant, MRSA - vancomycin
Staph, S pneuomoniae - clindamycin
Gram- pathogen and susceptible strains of pneumococcus - 3rd gen cefotxaime
Pasteurella - Augmentin
349
Q

4 differential for bone cyst

A

1) unicameral (epiphysis humerus and femur)
2) aneurysmal (diaphysis or metaphysis - needs orthopedist to remove immediately
3) Malignant tumors - destroy adjacent bone and lead to irregularity
4) fibrous cortical defects - femur well-delineated lucent lesions surrounded by sclerosis; if 50% of bone diameter than need attention

350
Q

Decreased internal rotation and abduction of the affected hip is caused by what

A

Legg-Calve-Perthes disease

351
Q

What is Legg-Calve-Perthes disease

A

avascular necrosis

352
Q

What is the differential diagnosis of avascular necrosis in a patient with a limp?

A

JIA, septic arthritis, SCFE, transient synovitis

353
Q

Hip is held in externally rotate position and have limited internal rotation

A

SCFE

354
Q

What is rx for SCFE

A

no weight bearing and refer to orthopedic specialist for urgen surgery. internal fixation of the femoral head with screws is the most common treatment

355
Q

Differential of back pain in children

A

spondylolysis, discitis

356
Q

Back pain worsen with hyperextension and how do you evaluate

A

spondylolyis, oblique films; rest and avoidance of aggravating activities

357
Q

Limping or refusal to walk, back pain in child <5 years

A

Discitis - MRI. Treat staph aureus

358
Q

MRI for back pain is best to evaluate what

A

infection, herniated disc, or tumor in children

359
Q

CT for back pain is best to evaluate what

A

suspected spondylolylsis if normal plain xray

360
Q

How do you eval and treat back pain?

A

If nothing specific, than NSAID and PT first. Re-evaluate in 1 month and get AP and lat films with oblique view. Lab tests to rule out infection, inflammation, cancer; if nothing than do MRI to look for infection, herniated disc or tumor

361
Q

What is the most common motor neuron condition associated with arthrogryposis

A

SMA, mito cytopathy, primary myopathy

362
Q

Pattern: fixed in-turning of the hindfoot, plantar flexion at the ankle, and cavus appearance of the distal foot

A

clubfoot

363
Q

Rx for clubfoot

A

serial manipulation and casting ASAP

364
Q

Poison: Esophageal perforation, mucosal and skin surface by liquefaction necrosis; odynophagia, dysphagia, drooling, intraoral burns, ulcerations vomiting with hematemesis

A

corrosives (toilet bowl cleaners, laundry detergents, stain and mildew removers, floor cleaners, laundry detergents, stain and mildew removers, floor cleaners, oven cleaners, rush removers

365
Q

Poison: depressed mental status, nausea, vomiting; seizure and coma within a few hours; hypocalcemia

A

ethylene glycol,

366
Q

Poison. chemical conjunctivitis, cough with inhalation, vomiting after ingetions

A

glycophosphate-containing weed killers

367
Q

Poison. salivation, lacrimation, urination, defecation or diarrhea, GI upset and emesis, tachypnea and bradycardia

A

organophosphate

368
Q

Poison. Respiratory distress due to pulmonary aspiration, acute coughing gagging and choking, fever, tachypnea, cyanosis, and abnormal lung sounds, crackles and wheezing

A

hydrocarbon-based (furniture polish, pulmonary aspiration)

369
Q

Pattern: dilatation of the proximal muscular urethra against a closed or narrow distal urethral sphincter

A

spinning top urethral

370
Q

Rx for spinning top

A

Will resolve on own

371
Q

Indications for VZV IgG

A

1) neonates born to mothers who develop chickenpos withing 5 days before and 48 hours after delivery
2) Neonates >28wks if mother lacks immunity
3) Neonates <28wks or <1000g regardless of maternal immunity
4) Pregnant women without evidence of immunity
5) Immunocompromised persons without evidence of immunity

372
Q

Indication for VZV vaccine

A

1) postexposure PPx for healthy people without evidence of immunity 12 mo or older
2) if administered within 72hrs after exposure to varicella

373
Q

Pattern: gradual development of GI symptoms over 3 weeks progressing to development of dysenteric, bloody stools, and can lead to liver abscess

A

Amebiasis; E histolytica

374
Q

Pattern: travelers’ diarrhea, self-limited 1 to 5 day with fever, watery stolls and abdominal cramps

A

ETEC

375
Q

Pattern: shiga toxin-producing strain associated with diarrhea, hemorrhagic colitis and hemolytic-uremic syndrome

A

O157:H7

376
Q

Pattern: abdominal bloating, watery diarrhea, and malabsorption

A

giardia

377
Q

Problems typically seen in infants of diabetic mothers

A

1) Fetal macrosomia
2) Cardiomyopathy
3) Decreased surfactant production –> RDS
4) polycythemia
5) hypocalcemia
6) congential anomalies - TGV, VSD, caudal regression syndrome, neural tube defects; small left colon syndrome

378
Q

Pattern: FAS hemoglobin

A

Sickle cell trait

379
Q

Pattern: FS hemoglobin

A

Sickle cell anemia

380
Q

Pattern: FSC

A

milder phenotype of SC disease

381
Q

Pattern: FSA

A

S beta+ thalassemia (mild or no anemia)

382
Q

HbF

A

alpha and gamma

383
Q

HbA2

A

alpha and delta

384
Q

HbA

A

alpha and beta

385
Q

HbA2 is elevated in what

A

beta+ thalassemia

386
Q

Describe causes of atypical PNA

A

mycoplasma or chlamydia PNA

387
Q

Describe symptoms of atypical PNA

A

school-aged or older, not highly febrile, have a dry cough that develops later in the illness as symptoms of HA, myalgias, and malaise improves

388
Q

Rx for atypical PNA

A

azithromycin

389
Q

Most common cause of PNA in children 3 months to 4 years of age

A

virus, RSV, Influenza, parainflu, adno, human metapneumo, rhino

390
Q

Rx for bleeding from varices

A

octreotide infusion

391
Q

TSH at different time points after birth

1) 24hours of life
2) 2 weeks

A

1) 20-25 mIU/L

2) 10 mIU/L

392
Q

What dose of levothyroxine

A

10-15ug/kg per day

393
Q

What about borderline TSH bwteen 5 and 10?

A

Can treat, but trial off therapy at 3 years of age

394
Q

BCG will give positive TST during what period of time?

A

within 10 years of age

395
Q

Pattern: lilac right or violaceous inflammatory border, skin is indurated, skin thickening, ivory-colored center of sclerosis, dermal and subcutaneous atrophy can develop later in the disease course, resulting in visible veins, flat or concave depression of the subcutaneous tissues, lack of hair growth

A

Localized scleroderma

396
Q

Pattern: Scaly and typically hypopigmented lesions with a cigarette paper-like wrinkled appearance and varying degrees of sclerosis

A

lichen sclerosus

397
Q

What further testing should you do if you have linear scleroderma on the head or scalp

A

should do MRI to look for calcifications, white matter changes, vascular malformations, and signs of CNS vasculitis

398
Q

What are complications of untreated localized scleroderma?

A

growth abnormalities, disfiguring lesions and joint contractures

399
Q

What 2 types of fractures are typically seen in child abuse cases?

A

spiral and metaphyseal chip fractures

400
Q

Pattern: immune-mediated skin problem characterized by erythematous and edematous papules or targetlike lesion

A

Erythema multiforme

401
Q

What is the most common cause of EM in young adults

A

HSV, mycoplasma, GAS

402
Q

What are extrapulmonary manifestations of mycoplastma infection

A

arthritis, polymorphous skin eruptions, hemolytic anemia, carditis and nervous system disease

403
Q

If small bowel involvement is seen in suspected IBD what test first?

A

Small bowel series

404
Q

What is the most common consequence of submucosal cleft palate?

A

conductive hearing loss

405
Q

Onset and duration of SABA and LABA

A

SABA within 15 min for 4-6 hrs

LABA within 30 min for 12 hrs

406
Q

What can happen with regular use of LABA and SABA?

A

dimunition of bronchoprotective effect

407
Q

Positive DAT is consistent with what?

A

AIHA

408
Q

What two agents/condition is associated with AIHA

A

Infection and antibiotic

409
Q

What are 3 forms of AIHA

A

1) Warm-reactive IgG autoantibodies
2) Paroxysmal cold hemoglobinuria
3) IgM-mediated cold agglutinin

410
Q

Which AIHA:

Extravascular

A

Warm and cold

411
Q

Which AIHA:

Intravascular

A

Paroxysmal cold

412
Q

Which AIHA:

Intra and extravascular hemolysis

A

IgM cold

413
Q

Which AIHA:

associated with mycoplasma

A

IgM cold

414
Q

Which AIHA:

leads to intravascular hemolysis at cold temperatures but binds complement at 37C

A

Paroxysmal cold

415
Q

Rx for severely low hemoglobin in AIHA

A

steroids and transfusion

416
Q

Tri21 pts have increased risk of what after surgery

A

1) 3x infection
2) 2x respiratory complications
3) 3x PHTN
4) 3x AV block

417
Q

Cardiac condition with 22q11

A

conotruncal and great artery defect

418
Q

Cardiac condition with Turner syndrome

A

coarctation, aortic stenosis or bicuspid aortic valve

419
Q

Cardiac condition with FAS

A

septal defects

420
Q

Comorbidity with ADPKD

A

cerebral aneurysms

421
Q

ADPKD pt with fmhx of intracranial aneurysms are at increased risk of developing intracranial aneurysm rupture with what other symptoms?

A

> 7mm, poorly controlled hypertension and <50 year old

422
Q

When is routine imaging needed for aneurysm in pt with ADPKD

A

1) h/o rupture
2) + fmhx of intracerebral bleeding or IC aneurysm
3) occupation that place them at risk
4) hemodynamic instability and HTN associated with surgeries

423
Q

Screening recs for high risk ADPKD patients

A

1) Q5 years after initial neg radiographic studies

2) Q2-3 if there was bleed and Q2-5 thereafter if aneurysm is stable

424
Q

Difference in description of retinitis versus chorioretinitis

A

Retinitis - loss or blurring of vision, blind spots, floaters, and flashing lights, floaters and photopsia, retinal detachment, hemorrhage in association with white (or yellow) fluffy retinal lesion close to the retinal vessels
Choriretinitis - white, infiltrative, moundlike lesions on the retina

425
Q

What causes retinitis, what cause chorioretinitis

A

1) retinitis - CMV, Bartonella henselae, hsv

2) Chorio - candida, toxo

426
Q

Sudden bilious vomiting should be concerning for what?

A

partial or complete intestinal obstruction beyond the ampulla of Vater

427
Q

Study of choice for suspected malrotation

A

Upper GI series

428
Q

When should you seek care for child with femoral anteversion?

A

If it persists beyond 11

429
Q

What are most common complications in foreign body aspiration?

A

PNA and atelectasis

430
Q

Schedule for meningococcal

A

11 to 12 yrs with booster at 16

431
Q

Meningococcal vaccine schedule for travelers to endemic regions by age

A

<9 mo - 3 doses (2, 4, and 6)
9 -23 (2 doses)
24mo (single)

432
Q

Who is at increased risk for meningococcal B

A

1) persistent complement component
2) anatomic or functional asplenia
3) outbreak

433
Q

When do you use 23-valent pneumococcal vaccine

A

in high-risk children >2yrs in addition to PCV13

>8 weeks after last PCV13 dose, revaccinate high-risk children once after 5 years

434
Q

When do you need to give a pneumococcal vaccine in kids 6-18 years

A

1) anatomic or functional asplenia, SC
2) HIV or other immune compromise, malignancy, transplant recipients
3) cochlear implant or CSF leak
4) chronic renal failure, nephrotic syndrome

435
Q

Schedule for Hep A vaccine

A

> 1 yr of age; 2 doses at least 6 months apart

436
Q

Indication for hepA vaccine other than scheduled

A

Travelers to endemic areas, close contact with international adoptees, HepA outbreak post-exposure ppx, chronic liver disease

437
Q

Age for DTaP or DT

A

<7yrs

438
Q

Age for Tdap or Td

A

> 7yrs

439
Q

Contraindications for pertussis vaccine

A

anaphylactic reaction, encephalopathy within 7 days

440
Q

Waning immunity rate to pertussis

A

10-15% per year

441
Q

When is Tdap used

A

Routine at age 11-12yrs
Catch-up for adolescent 13-18yrs
Single dose in undervaccinated children
During pregnancy

442
Q

VZV schedule

A

12-15 months and 4-6 years of age

443
Q

Maximum age for rotavirus

A

8 months

444
Q

Contraindications for rotavirus

A

SCID, h/o intussusception

445
Q

Routine schedule for HPV

A

9-14 years, 2 doses, 6 month interval

446
Q

Schedule for HPV immunocompromised

A

3 dose series

447
Q

Reye syndrome with aspirin and which virus

A

influenza

448
Q

Major cause of croup

A

parainfluenza

449
Q

Parainfluenze type 3 can cause what neuro problem

A

aspetic meningitis, encephalitis

450
Q

Who should get palivizumab for RSV

A
1) CLD <32 weeks gestation
<12 months of age and require 21% oxygen for 28 days
CHD
Anatomic pulmonary abnormalities
Neuromuscular disease
Immunocompromised children
451
Q

For ankle sprains, what are the treatment

A

If no pain, stirrup brace. If pain, then crutches to avoid weight bearing

452
Q

How do you diagnose hyperinsulinism?

A

Normal insulin >13uIU/ml in setting of very low glucose

453
Q

What to do immediately for snake bite

A

splint and keep arm at level of heart

454
Q

What is the official recommendation for hearing screening?

A

ABR, OAE in newborns, audiologic assessment by 3 months of age who fail their newborn screening; individualized intervention by 6 months of agefor those with significant hearing impairment

455
Q

PNA at birth is usually cuased by

A

chlamydia

given erthyromycin

456
Q

Which renal disorder has low C3

A

Lupus, postinfectios/poststreptococcal, membranoprliferative GN, shunt nephritis, subacute bacterial endocarditis

457
Q

Factors that predict high school completion of pregnant mother

A

reading material in home, black race, employment of the adolescent’s mother, being raised in a smaller family, and having parents with higher educational levels

458
Q

Treatment for corneal abrasion

A

Do not give topical anesthetics. Just antibiotics

459
Q

Pancreatic elastase in stool is a test for what?

A

Pancreatic insufficiency

460
Q

Pattern: anemia, neutropenia, low platelets, elevated liver enzymes, hepatomegaly, recurrent infection

A

Shwachman-Diamond syndrome

461
Q

What imaging test can help diagnosis Shwachman-Diamond

A

Fatty pancreas

462
Q

Rx for otits externa

A

Topical fluoroquinones

463
Q

Overdose: hypotension, sinus bradycardia, heart block, AMS, coma, delirium and seizures, increased airway resistance, respiratory depression, hypoglycemia and hyperkalemia

A

beta blocker overdose

464
Q

Lachman, describe and what is it for

A

ACL, hold femur and pull tibia forward

465
Q

Valgus stress test; describe and what is it for

A

MCL sprain; heel of the hand over the alteral aspect of the knee and applies a medially directed force with the joint flexed to 30 degrees; pain indicates stretch or partial tear of the MCL, laxity is complete tear

466
Q

Drawer test, describe and what is it for

A

PCL; knee flexed enough to allow the foot to rest flat on the examination table, examiner stabilizes the foot and pushes the tibia posteriorly

467
Q

McMurray, describe and what is it for

A

meniscal tear; pain or there is a palpable click with valgus stress to the knee, external rotation of the tibia and passive motion of the knee from extreme flexion to extension

468
Q

Recurrent hypoglycemia associated with intercurrent illness in the absence of metabolic acidosis or other electrolyte imbalances

A

Carnitine deficiency

469
Q

Apgar scores

A
  • Activity (1) some flexion (2) active
  • Breathing (1) weak cry (2) good cry
  • Color (1) acrocyanotic (2) pink
  • Grimace reflex irritability (1) grimace (2) cry, withdrawal
  • Heart (1) <100 (2) >100
470
Q

PT

A

Extrinsic

Factor X, VII, V, prothrombin, fibrinogen NL 9-13s

471
Q

PTT

A

Intrinsic, HMWK, XII, XI, X, IX, VIII, V, prothrombin, fibrinogen
NL 23.4 - 32.4s

472
Q

normal coag factor levels in newborn

A

At birth, VitK reduced to about 50% (II, VII, IX, X);
XI and XII are also 50%
Factor V, VIII, XIII similar to adults
ATIII, ProtC/S reduced (30-60%)

473
Q

Pattern: prolonged PT and PTT with Nl fibrinogen

A

Vit K deficiency

474
Q

Drugs that antagonize Vit K deficiency

A

warfarin, INH, phenobarb

475
Q

Other causes of VitK deficiency

A

Diet, antibiotics, malabsorption

476
Q

Long term complications of hemophilia

A

Chronic hemarthroses leading to joint destruction

477
Q

Name the 7 thrombophilia

A
ProtS, C, ATIII
Factor V Leiden
Antiphospholipid
Hyperprothrombinemia
Hyperhomocysteinemia
478
Q

Basic thrombophilia work-up

A

PT, PTT, INR
Protein C and S
Factor V Leiden/Activated Protein C, MTHFR mutation/homocsteine level, Prothrombin mutation, DRVVT, ATIII

479
Q

What is neonatal purpura fulminans

A

Sudden, massive microthroombosis, skin necrosis, systemic DIC, Homozygous Protein C or S deficiency

480
Q

Presentation of Protein C and S deficiency heterozygous

A

VTE

481
Q

If heparin fails to anticoag, what should you think of

A

ATIII deficiency

482
Q

Describe Factor V Leiden mutation and how that causes disease

A

Mutation renders FV resistant to cleavage by Protein C

483
Q

Prothrombin gene mutation

A

Promotes enhanced thrombin generation

484
Q

Rx for hyperhomocysteinemia

A

Folate

485
Q

Which prothrombotic disorder gives you prolonged PTT

A

anti-phospholipid

486
Q

What are some acquired thrombotic states

A

nephrotic syndrome, IBD, paroxysmal nocturnal hemoglobinuria, malignancy, medications (L-asparaginase, warfarin, estrogen, heparin); HIT

487
Q

Congenital cataracts

A

Tri,WAGR, CHARGE, NF-2, Sturge-Weber, Alport, Galactosemia, copper metabolism

488
Q

Indication for MT tube insertion

A

1) AOM not response to 3rd line treatment
2) Recurrent AOM: 3 episodes/6 months or 4 episodes/12mos
3) Immunocompromised, eriously ill
4) anatomical abnormalities