PEDIATRICS

Question 1

Genetics in Noonan’s syndrome

Answer 1

Common AD disorder due to mutation to genes involved in RAS-MAP-K pathway
Can affect both males and females (big difference between Turners)

Question 2

Thickened helices

Answer 2

Noonans syndrome

Question 3

Severe h/a and dizziness in pt with Noonans syndrome – > screen for

Answer 3

Arnold Chiari malformation

Question 4

Cardiac malformations that contrast Noonans vs Turners

Answer 4

Noonans: right sided heart lesions, HOCM
Turners: left sided

Question 5

Diseases associated with Turners

Answer 5

Lymphedema
DM-1
Hashimotos
Autoimmune disorders

Question 6

Difference between Sotos syndrome and Beckwith-Wiedemann

Answer 6

BW features hypoglycemia during newborn period, macroglossia, umbilical hernia and/or omphalocele.

Question 7

Tumors assoc with Beckwith Wiedemann

Answer 7

10% will go on to develop small round blue cell tumors, most commonly Wilm’s.

Question 8

Croup pathogens

Answer 8

RSV, influenza, adenovirus, and parainfluenza virus ** most common.

Question 9

Moderate/severe croup tx

Answer 9

oral or IM corticosteroids, INHALED racemic epinephrine.

Question 10

Pathogens for Epiglottitis

Answer 10

USED to be H. influenza type B.

Now, strep, non-typable H influenza and viral agents.

Question 11

Subglottic narrowing

Answer 11

croup

Question 12

Supraglottic narrowing

Answer 12

epiglottitis

Question 13

Epispadias is associated with/

Answer 13

Bladder extrophy

Question 14

Where is the testicle usually found in cryptorchidism?

Answer 14

Inguinal canal.

Question 15

Hydrocele is a remnant of what embryological structure

Answer 15

Tunica vaginalis.

Question 16

How do you treat hydocele

Answer 16

It will usually resolve within six months.

Question 17

Abnormality associated with hypospadias

Answer 17

Chordee, a ventral curvature of the peniis. The growth differences between the attenuated ventral urethra and its associated tissues, and the normal growing dorsal tissue, is thought to cause this. It is often apparent during an abreaction.

It is also highly a/w cryptorchidism and inguinal hernias.

Question 18

How many DTaPs do you get

Answer 18

5. Four doses are adequate if the last dose was given on or after the 4th birthday. Should be completed through age 6.

Question 19

If DTaP was given before age of 4 and the fifth vaccine was not given, what vaccine should you administer?

Answer 19

Trap starting at 7 years of age.

Question 20

Vaccine given to children under 7 y.o. who have a contraindication to pertussis vaccine

Answer 20

DT

Question 21

Tx of pertussis

Answer 21

Macrolide like azithromycin.

Question 22

Tx usually given in a pt allergic to penicillin with strep pharyngitis

Answer 22

Azithromycin or other macrolide.

Question 23

Atypical pneuma usually antibiotic class of choice

Answer 23

Azithromycin/macrolide.

Question 24

Iron studies in a thalassemia patient

Answer 24

Normal to high ferritin

Normal to low TIBC

Question 25

Labs in sickle cell

Answer 25

Low Hgb | Elevated reticulocyte count, as it is a hemolytic anemia.

Question 26

Sideroblastic anemia : iron labs

Answer 26

Microcytic, incrased serum ferritin | TIBC saturation is normal or elevated.

Question 27

An alternative to inhaled glucocorticoids as long-term asthma control agent

Answer 27

Montelukast, a leukotriene receptor antagonist

Question 28

Kienbock's disease

Answer 28

AVN and collapse of the lunate so frequently encountered in individuals who overuse the wrist through repetitive compression loading.

Question 29

Downey cells

Answer 29

MONO!

Question 30

Metamyelocytes differentiate into?

Answer 30

Eosinophils Basophils Neutrophils

Question 31

RMSF rash

Answer 31

Blanching erythematous rash with macule and is located on palms of hands and soles of feet. Moves centrally and becomes more petechial in appearance.

Question 32

Other sx of RMSF

Answer 32

``` Headache Confusion Conjunctival erythma Pedal edema Fever ```

Question 33

Micro of rickettsia rickettsii

Answer 33

Gram negative intracellular obligate coccobacillus.

Question 34

Rickettsia rickettsii injures blood vessels how?

Answer 34

Cause direct vascular injury. Release of prostaglandins --> increased vascular permeability. This causes edema, hypovolemia, hypoalbuminemia, and hypotension. Occasionally, thrombi form due to destruction of endothelial lining by bacteria, resulting in vascular necrosis and thrombosis.

Question 35

If you get RMSF and can't take tetracyclines, how do you treat it

Answer 35

Chloamphenicol.

Question 36

How do tetracyclines work

Answer 36

Inhibit protein synthesis by binding to the 30S unit of ribosomal bacteria.

Question 37

MOA of quinine

Answer 37

Inhibits parasite growth by changing the internal pH of the organism.

Question 38

SE of anti malarials

Answer 38

Prolongation of QT intervals | Methemoglobinemia.

Question 39

Tx of choice in non bullous impetigo

Answer 39

Mupirocin. Its effective at eradicating staph aureus and GABHS skin infections.

Question 40

How do we eradicate nasal colonization of MRSA in adult patients and health care workers

Answer 40

Mupirocin

Question 41

Neurologic impairment: TTP or HUS?

Answer 41

TTP

Question 42

Tx for TTP or HUS

Answer 42

Plasma exchange.

Question 43

Low APGAR scores + persistent respiratory distress + CXR with bilateral patchy opacities

Answer 43

Meconium aspiration syndrome

Question 44

Meconium aspiration syndrome is most often seen in what cases

Answer 44

Advanced gestational age.

Question 45

Patients with meconium aspiration syndrome are increased risk of developing what?

Answer 45

Persistent pulmonary hypertension. CAuses chronic stress to pulm vasculature, leading to thickening of the vessel walls. This leads to acidosis and hypoxia.

Question 46

MeCP2 gene mutation

Answer 46

Rett syndrome.

Question 47

HFE mutation

Answer 47

Hemochromatosis.

Question 48

ECG in PDA

Answer 48

High voltage in elads V1 - V6 (LVH) RAD and inverted T waves in leads V1-V3 consistent with RVH Tall, peaked P waves in lead II consistent with right atrial enlargement.

Question 49

Type II arnold chiari presents with herniation of?

Answer 49

cerebellar vermis

Question 50

Type I arnold chiari presents with herniation of

Answer 50

Cerebellar tonsils.

Question 51

Tx of shigella

Answer 51

Azitrhomycin or ciprofloxacin.

Question 52

Fanconi anemia

Answer 52

Inheritedd syndrome of BM failure presenting with short stature, hypo pigmented spots on skin, thus abnormalities, microcephaly, and hypogonadism. Mild to moderate thrombocytopenia with mild MACROcytic anemia.

Question 53

Chills, diaphoresis, anorexia, dry cough, a dull frontal headache and myalgia BEFORE the onset of a high fever

Answer 53

Typhoid fever. Diarrhea may accompany in children.

Question 54

2 congenital dz assoc with mitral regurg

Answer 54

Marfan syndrome | HOCM

Question 55

Marfan syndrome and syphilis are assoc with what heart valve defect?

Answer 55

Aortic regurg

Question 56

Heart valve abnormality in Turners

Answer 56

Besides coarctation, also aortic stenosis d/t bicuspid aorta.

Question 57

Why would coarctation o the aorta present with cariogenic shock in the first few weeks of life?

Answer 57

ductus arterioles closed. Tx if IV PGE1 immediately after the airway and breathing are secured.

Question 58

Tx of diaper rash

Answer 58

Topical antifungals such as miconazole.

Question 59

Diff between candidal diaper rash and irritant contact diaper rash

Answer 59

In irritant contact, this form doesn't affect skin folds.

Question 60

Bilious emesis on day 1, x-ray shows "double bubble." Normal gas pattern beyond. Next step?

Answer 60

Do enema, and then upper GI series if negative. Its likely malrotation/volvulus.

Question 61

Mom has polyhydramnios in preg. Baby now has biliary emesis on day 1. NO GAS beyond double bubble on X-ray. Dx?

Answer 61

Duodenal atresia (a/w Down syndrome) OR annular pancreas

Question 62

What is the cause of intestinal atresia

Answer 62

Vascular compromise in utero --aka mom smoked crack or something.

Question 63

Multiple air fluid levels in a baby along with double bubble

Answer 63

Intestinal atresia. Resect these segments.

Question 64

What causes annular pancreas

Answer 64

Failure of ventral bud to rotate with the duodenum, causing envelopment.

Question 65

"Donut sign" on ultrasound

Answer 65

Pyloric stenosis.

Question 66

CMP in pyloric stenosis

Answer 66

Low chloride | Hypokalemic metabolic alkalosis

Question 67

Before doing a myomectomy, how should you treat pyloric stenosis

Answer 67

Look at CMP and make sure baby is hydrated.

Question 68

Meckels diverticulum is a remnant of what

Answer 68

Vitelline duct

Question 69

"Melena" in a kid ..look for what

Answer 69

H/o of epistaxis, iron ingestion, or beets. Kids don't get melena.

Question 70

Mom has HIV but doesn't take antiretrovirals. What ppx should you give the newborn?

Answer 70

AZT within 12 hours of delivery for 6 weeks.

Question 71

How can we boost a proteae inhibitor

Answer 71

Add ritonavir. Its another protease inhibitor that enhances the protease inhibitor it is combined with.

Question 72

"-Vudine"

Answer 72

NRTI

Question 73

"-Avir"

Answer 73

Protease inhibitor

Question 74

You just had sex with someone who has HIV and you don't want it. Ppx?

Answer 74

2 NRTI + either NNRTI, protease inhibitor, or entry inhibitor.

Question 75

What mutations are involved in resistance to HIV

Answer 75

CCR5 | CXCR4

Question 76

How do you screen for HIV in pt very early on (aka when they present with flu syndrome)

Answer 76

Viral load RNA

Question 77

Pt has PCP pnuemo and G6PD. How do you prophylax?

Answer 77

ATOVAQUONE

Question 78

CD4

Answer 78

Coccidiomycosis with fluconazole. Especially if they live in endemic regions like Arizona or California

Question 79

Alternative in prophylaxis for MAC if macrocodes cannot be used

Answer 79

Rifabutin. However, get CXR first to rule out active TB.

Question 80

Measles rash spread

Answer 80

Starts on face and spreads down. Thats how it clears too.

Question 81

Subacute sclerosing panencephalitis is a late complication of what childhood disease

Answer 81

Measlers

Question 82

Measles is caused by what virus

Answer 82

Paramyxovirus

Question 83

Rash of rubella

Answer 83

Starts on face and spreads down

Question 84

Prodrome of rubella

Answer 84

Generalized, tender lymphadenopathy. Fever.

Question 85

Roseola is due to what virus

Answer 85

HHV-6

Question 86

Roseola: which comes first, fever or rash?

Answer 86

Fever first, THEN a rash.

Question 87

Rash of roseola

Answer 87

Starts on trunk and spreads outward.

Question 88

Grade III Concussion

Answer 88

Loss of consciousness with amnesia. 1 month off.

Question 89

Grade II concussion

Answer 89

LOC without amnesia. 1 week off.

Question 90

Grade I concussion

Answer 90

No LOC and no amnesia...go back in 20 min

Question 91

Punctate hemorrhages on CT

Answer 91

Cerebral contusion

Question 92

4 ft and 40 lbs -- carseat?

Answer 92

Booster seat.

Question 93

Maintenance fluid in kid 0-10 kg

Answer 93

100 cc/kg

Question 94

Maintenance fluid in kid 10-20 kg

Answer 94

50 cc/kg

Question 95

Maintenance fluid in kid >20 kg

Answer 95

20 cc/kg

Question 96

%BSA in CHILDS head

Answer 96

18

Question 97

%BSA in CHILDS thorax (anterior)

Answer 97

18

Question 98

%BSA in Child's arm

Answer 98

9

Question 99

% BSA in childs legs together

Answer 99

27

Question 100

Clean wound, unknown Td status

Answer 100

Give them the Td vaccine

Question 101

Dirty wound, unknown Td status

Answer 101

IVIg + vaccine

Question 102

Dirty wound and booster > 5 years gets what tx

Answer 102

Vaccine

Question 103

Any wound and booster

Answer 103

Observe

Question 104

Egg is fiund in which 3 vaccines

Answer 104

MMR Yellow fever FLU

Question 105

Live atten vaccine

Answer 105

MMR Varicella | FLU**** newer vaccine is not live

Question 106

Nml vaccine response

Answer 106

Temp

Question 107

What if mom has hep b how do you treat baby

Answer 107

Give ivig and hep b vaccine now

Question 108

If moms hep b is unknown how do you treat baby

Answer 108

hep b vaccine now

Question 109

First 3 doses of dtap given when

Answer 109

1st year of life

Question 110

what is tracheitis

Answer 110

crpoup that doesn't get better. treat with abx

Question 111

what happens if you treat retinoblastoma with radiaton

Answer 111

you just provided the second hit and now they'll get more tumors

Question 112

cause of retinopathy off prematurity

Answer 112

high O2 administered

Question 113

4 dz of prematurity

Answer 113

IVH retinopathy off prematurity NEC Bronchopulm dysplasia

Question 114

Tx of malrotation

Answer 114

EMERGENT SURGERY

Question 115

Tx of gastroschisis/omphalocele

Answer 115

Silo