UW

Question 1

Prolonged PTT
Normal Plt, BT, PT

Decreased F8 v. F9

Answer 1

Hemophilia A and B

Question 2

Hemarthrosis, intramuscular hematoas, GI/GU bleeding

X-R

Answer 2

Hemphilia A and B

Question 3

Presents at 3-4m with hypoglemia induced seizures, lactic acidosis, hyperuricemia, hyperlipidemia. Thin extremities, doll like face, short stature, protuberant abdomen (HM).

Answer 3

Glucose-6-phosphatase deficiency (type 1 - von gierke)

Question 4

HM, glucocerebrocide accum in macrophages in liver, spleen, marrow = bone pain, cytopenia.

Answer 4

Glucocerebrosidase enzyme deficiency - Gaucher

Question 5

When do you NOT CT a kiddo with head trauma

Answer 5

GCS=15
Non-severe mechanism
No vomiting, HA, LoC, signs of fracture

Question 6

Management of a kiddo with head trauma and
GCS=15 w/any:
Vomiting, HA, brief/questionable LoC, high-risk mechanism, severe mechanism

Answer 6

Observe for 4-6hrs

OR

Head CT WITHOUT contrast

Question 7

Sore throat, dysphagia, odynophagia, pharyngeal/tonsillar erythema

+ cough, rhinorrhea, conjuctivitis, oral ulcers

Answer 7

Viral pharyngitis

Question 8

Sore throat, dysphagia, odynophagia, pharyngealtonsillar erythema

+ edema, palatal petechiae

Next step?

Answer 8

Rapid antigen test for strep pharyngitis

MCC: GAS

Question 9

Rapid antigen strep pharyngitis test is negative, next step?

Rapid antigen strep pharyngitis test is pisitive, next step?

Answer 9

If NEGATIVE - throat culture (if negative again, then viral dx)

If POSITIVE - tx with oral amoxicillin or penicillin

Question 10

Hemophilia A and B are what type of bleeding disorder (clotting defect, platelet aggregation defect, thrombocytopenia)

Answer 10

Clotting defect

Question 11

Increased PTT with hemarthrosis and deep tissue hematomas. Dx?

Answer 11

Hemophilia a or B

Question 12

vWD and bernard soulier syndrome - what type of bleeding disorder? (clotting defect, platelet aggregation defect, thrombocytopenia)

Answer 12

Platelet aggregation

Easy or prolonged bleeding, ecchymosis, and petechiaex

Question 13

Easy or prolonged mucosal bleeding, ecchymosis, petechiae, and decreased platelet count. Bleeding disorder type?

Answer 13

Thrombocytopenia

ITP or leukemia

Question 14

HBsAg
HBeAg
IgM Anti-HBcAg

Which is marker of viral replication

Which is first evidnce of infection

Which is antibodiy to hep B core antigen?

Answer 14

Which is marker of viral replication - HBeAg

Which is first evidnce of infection - HBsAg

Which is antibodiy to hep B core antigen?
IgM Anti-HBcAg

Question 15

What is the clinical feature of iron poisoning present at 2d?

What about during 2-8wk?

Answer 15

2d = hepatic necrosis

2-8wk = pyloric stenosis

Question 16

MCC of otitis externa

Tx

Answer 16

Pseudomonas *fluoroquinolones

Question 17

Vitamin deficiency - diarrhea, dermatitis, dementia, possible death

Associated with UC/nutritional absorption deficiencies and third world countries

Answer 17

Pellagra - niacin (B3) deficiency

Question 18

Vitamin deficiency - cheilosis, glossitis, seborrheic dermatitis, pharyngitis, erythema/edema of mouth

Answer 18

Riboflavin (B2)

Question 19

Vitamin deficiency - irritability, depression, dermatitis, stomatitis, elevated homocysteine concentraiton (atherosclerosis and venous thromboemb RF)

Answer 19

Pyridoxine (B6)

Question 20

Genu varum

Answer 20

Rickets - vit D def

Question 21

Three signs of pancreatic insufficiency in CF

Answer 21

Vitamin D deficiency

Vitamin K defiiency - easy bruising

Poor growth

Question 22

Cat bites (pasteurella) and anaerobic bacteria - ppx tx after bite

Answer 22

Amox/clav

Question 23

Preceeding triggers for intussusception

Answer 23

Gastroenteritis, meckel’s, polyps, hematomas (HSP - dt bowel wall edema and localized hemorrhage)

Question 24

MCC acute bacterial sinusitis

Tx

Answer 24

Strep pneumo or nontypable Haemoph. Infl

Amox-clav

Question 25

MCC of otitis externa

Answer 25

Pseudomonas

Question 26

Asian child with sore throat and joint swelling and new onset heart murmur - name the dz, the heart defect, and the abx ppx.

Answer 26

Pharyngitis dt GAS --> RF MS Penicillin

Question 27

Tx for enterobius vermicularis pinworm

Answer 27

Albendazole for the whole house

Question 28

Ectopic/upward facing lens and joint hypermobooloty, pectus excavatum

Answer 28

Marfans - fibrillin1

Question 29

Lens downward, pectus deformity, tall stature, arachnodactyly, **fair complexion, *thromboembolic events/CVAccident, intellectual disability**

Answer 29

Homocystinuria (AR - def cystathionine synthase - met. Methionine) Marfanoid body habitus with the triad of CVA, fair complexion, intellectual disability

Question 30

3-4m with hypoglycemia (seizures), lactic acitosis (glycogen b/u in liver), doll like face, hyperuricemia/lipidemia, HM

Answer 30

G6PD (type 1 glycogen storage dz - von Gierke)

Question 31

HM, bone pain, cytopenia due to ___ b/u in macrophages of liver, spleen, marrow

Answer 31

Gaucher - glucocerebrosidase enzyme deficiency

Question 32

Hypoketotic hypoglycemia during fasting states

Answer 32

MCAD

Question 33

When do you give seizure abortive therapy

Answer 33

If >5min (dt increased risk of airway compromise)

Question 34

HA, jaundice, SM, increased MCHC, incrased osmotic fragility test on ACIDIFIED GLYCEROL LYSIS TEST And ABNORMAL EOSIN-5-MALEIMIDE BINDING TEST

Answer 34

Hereditary spherocytosis

Question 35

# Define IgA-mediated leukocytoclastic vasculitis and tx Nml to inc creatinie Hematuria +/- RBC casts +/- protienuria Hx: infection

Answer 35

HSP Supportive or if severe, systemic GC Tetrad: palpable purpura, GI pain, hematuria, arthralgia

Question 36

Posttussive emesis in a child w/o vacinnations after 4y - think?

Answer 36

Pertussis Tx with macrolides - azythro or clarithro

Question 37

Common complication in neonates born <30wk or <3.3lb Presents with lethargy, hypotonia, high pitched cry, rapidly increasing head circumference, buldging fontanelles)

Answer 37

Intraventricular hemorrhage

Question 38

2-6m with HM, protuberant abdomen, hporeflexia, cherry red macula

Answer 38

Neimann pick disease (sphingomyelinase)

Question 39

2-6m with hyperreflexia, cherry red macula, loss of motor milestones

Answer 39

Tay sachs (beta hexosaminidase A def(

Question 40

Pertussis ppx for household: <1m >1m

Answer 40

<1m = azithromycin x5d >1m = azithromycin x 5d; clarithromycin x 7d; erythromycin x 14d

Question 41

Tx for radial head sublixation

Answer 41

Hyperpronate the forearm OR Supinate forearm and flex elbow

Question 42

3-10y male with hip pain. Follows viral infection. Pain, deceased ROM, limping. Flexed, abd, ER. Dx

Answer 42

Transient synovitis

Question 43

High pitched cry in newborn. Mom drug user. What drug

Answer 43

Heroin

Question 44

G.C testing in what aged

Answer 44

all sexually active females < or equal to 24y

Question 45

DKA following a viral illness -what happens to potassium?

Answer 45

Decreased total body K due to net renal loss. Serum K may be elevated dyue to acidemia and decreased insulin activity, causing K reditribution into Extracellular space

Question 46

``` DKA causes... K RAAS Lipolysis Volume status Gluconeogenesis ```

Answer 46

``` Loss of K Increased RAAS Increased lipolysis Hypovolemia/osmotic diuresis Incrased gluconeogenesis dt increased ration of circulating glucagon:insulin ```

Question 47

Turners are at increased risk for what due to ovarian dysgenesis?

Answer 47

Estrogen deficiency --> decrersed inhibition of osteoclast-mediaeted bone resorption --> decreased bone density --> OSTEOPOROSIS

Question 48

Difference between laryngomalacia and vascualr rings in presentation

Answer 48

Laryngomalacia is INSPIRATORY stridor that is worse in supine and better prone. Vascular rings are BIPHASIC stridor that is better with neck extension

Question 49

Precocious ouberty, cafe au lait spots, multiple bone defects (responsible fo r5% of female precocious puberty)

Answer 49

Mccune-albright

Question 50

``` LAD HSM anemia/pallor petechiae/thrombocytopenia bone pain marrox bx: >25% blasts ```

Answer 50

ALL

Question 51

1. Low pitched muscial, pure, or squeaky tone at LLSB - Name - B9 or pathologic? 2. High pitched at LUSB - Name - B9 or pathologic?

Answer 51

1. Stills, b9 | 2. Pulmonary flow murmur, b9

Question 52

Subcutaneous emphysema secondary to severe coughing... next step in management.

Answer 52

CXR to r/o pneumothorax

Question 53

Tx for tourette disorder (>1y)

Answer 53

Antipsychotic - risperidone

Question 54

Pericardial effusion can lead to ____ after cardiac surgery?

Answer 54

Cardiac tamponade, a result of postpericardiotom syndrome