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Peds uworld Flashcards

1
Q

Most common cause of viral meningitis

A

non-polio enterovirus’s (echovirus, coxsackievirus)

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2
Q

Which precaustions are needed if you suspect a Measles kid is in your ER?

A

Airborne precautions

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3
Q

Cough
Coryza
Conunctivitis
Maculopapular rash that starts ate the head and spreads to the rest of the body

A

Measles (rubeola)
Everyone needs a N95 mask
Dx - titers and PCR
Tx - vit A in severe cases

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4
Q

At what age do kids get their first MMR?

A

1 year
Ideally get two doses between 1 and 4
If international travel is planned, an additional dose can be given b/w 6 and 11 months

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5
Q

Herpangina is caused by?

A

Coxsackie A virus
typically kids age 3-10
Gray vesicles on the tonsillar pillars and posterior oropharynx
Can occur w/ or w/o the hand and foot rash
Tx - supportive

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6
Q

Herpetic gingivostomatitis is caused by?

A

HSV1
kiddos 6mo - 5yrs
Tx - po acyclovir
Presents on lips, anterior oropharynx

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7
Q

Group A strep

A

Strep pharyngitis

can be complicated by acute rheumatic fever

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8
Q

Tx for enterobius

A

Albendazole

Or pyrantel pamoate

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9
Q

If you suspect meningitis

A

Do a LP and give abx

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10
Q

Most common cause of bacterial meningitis in kids >1 months?

A

strep pneumo

Neisseria meningitidis

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11
Q

mild infection of the eyelid anterior to the orbital septum

A

Preseptal cellulitis

Tx - po abx

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12
Q

Infection posterior to the orbital septum presenting w/ pain w/ EOM, diplopia, ophthalmoplegia

A 
Orbital cellulitis
Emergency 
Complications -> blindness, intracranial infection
Red flags - vision changes
If unclear - get a CT
Need to be admitted
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13
Q

work up for a baby w/ suspected sepsis

A 
CBC
Blood cultures
LP
UA
Urine culture
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14
Q

Low grade fever
Maculopapular rash starting at head and spreading to body
Posterior auricular and suboccipital lymphadenopathy
Arthralgias

A

Rubella (German measles)

Fever is lower grade than seen in measles and arthralgia is unique to rubella

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15
Q

congenital rubella causes

A

sensorineural hearing loss
cataracts
PDA

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16
Q

Inspiratory stridor
Barky cough
Hoarseness

A

Croup (parainfluenza)
kids 3-36 months
Xray - subglottic edema “steeple sign”
Tx - attempts to reduce subglottic edema; corticosteroids (dexamethasone), nebulized racemic epinephrine if severe (stridor at rest)

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17
Q

Kiddo w/ painful swelling in groin

PE - nontender papule on ant thigh, inguinal lymph node w/ overlying erythema

A

Bartonella henslae
Dx - clinical or serology
Tx - usually self limiting, Azithromycin if disseminated dz or immunocompromised host

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18
Q

Healthy kid w/ soft mobile cervical lymph nodes

A

Observe unless

  1. Systemic syx
  2. LN is >2cm, firm, immobile
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19
Q

nonvax kid w/ facial swelling and fever

A

Mumps
Fever and parotitis
Complications - aseptic meningitis, orchitis

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20
Q

Most common cause of sepsis in sickle cell dz pt

A

Strep pneumo (but also H. flu, Neisseria meningitidis)
Asplenia makes pt more susceptible to encapsulated organisms
Should receive vax and penicillin prophylaxis

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21
Q

Fever, pharyngitis, tonsillar exudate
Strawbery tongue
Tender ant cervical LN
Rash worse in axillae and groing

A

Scarlet fever (S. pyogenens, GAS)
“sandpaper” rash is prominent along skin folds and often results in desquamation
Dx - rapid strep Ag test, throat culture
Tx - Penicillin

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22
Q

Best way to prevent congenital rubella?

A

Vax prior to conecption (live attenuated)

Syx - Cataracts/glaucoma, sensorineural hearing loss, congenital heart dz (PDA)

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23
Q

If you suspect Strep pharyngitis

A

Rapid strep ag test +/- throat culture

Tx -amoxicillin

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24
Q

Most likely cause of osteomyelitis in Sicle cell pt

A

Salmonella and staph aureus

Tx - w/ abx that cover GP and GN (cephalosporing + vanc)

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25
Q

What supplements should be considered in exclusively breastfed infants?

A 
Vit D (400 IU in first month of life)
If premie of introduc ed to cows milk before 1 yr of life - also needs Fe
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26
Q

Kiddo swallowed a coin, seen in the esophagus on CXR. Now what?

A

Flexible endoscopy\

IF object was sharp or impacted - rigid endoscopy

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27
Q

Management of a kiddo that injested a coin that is now in his trachea?

A

Rigid bronchoscopy

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28
Q

Kiddo has rash, weight loss, and microcytic anemia

A

Celiac
Get a anti-tissue transglutaminase ab assay (IgA), if + confirm w/ endoscopic duodenal bx
Intestinal malabsorption -> weight loss, Fe deficient anemia
associated with T1DM and dermatitis herpetiformis (vesicles filled w/ clear fluid that crust over)

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29
Q

What is the advantage of breastfeeding

A

Ideal nutrition for infants
Human milk protein (70% whey) absorbs better than caesin
Improves gastric emptying
Passive immunity w/ IgA
Recommend exclusively BF until 6 months and slowly introduce pureed foods

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30
Q

Kid swallowed a button battery. Now what?

A

Immediate endoscopic removal to prevent esophageal ulceration
If battery is distal to the esophagus - let it pass through and confirm excretion through stool exam

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31
Q

2 day old exclusively BF w/ jaundice
High total bili
NL conjugated

A

Physiologic Jaundice
Pathophys - decreased hepatic uridine diphosphoguconurate glucuronosyltransferase activity
Usually benign, elevated indirect bili (increased bili production, decreased clearance, increased enterohepatic recycling)
Tx - phototherapy
Risk of kernicterus

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32
Q

When is exchange transfusion considered for a jaundice baby?

A

Total bili >20

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33
Q

Excessive crying for >3hrs/day, >3days/wk x 3 weeks in an otherwise healthy kid

A

Colic
Review soothing and feeding techniques
Reassure

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34
Q

Boy 3-5 wks old w/ projectile nonbilious vomiting after feedings

A 
Infantile hypertrophic pyloric stenosis
Confirm w/ abd U/S
Can have an olive shaped mass on exam
Can cause hypochloremic metabolic alkalosis
Tx - pyloromyotomy
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35
Q

Initial management of a kiddo presenting w/ constipation?

A

po laxatives

At risk of constipation during transitional events (dietary, toilet training, school)

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36
Q

Best way to fluid resuscitate a kid

A

NS bolus (20mL/kg)

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37
Q

2 y/o male, painless hematochezia

A

Meckels diverticulum
Outpouching of gastric mucosa d/t incomplete obliteration of the fetal vitelline (omphalomesenteric) duct
Dx - technetium-99m pertechnetate scanning

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38
Q

Where is upper and lower GI bleeding divided?

A

At the ligament of Treitz

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39
Q

Abd pain, elevated direct and indirect bili, RUQ mass

Y/S extrahepatic cystic mass

A 
Biliary cyst
Congenital or acquired cyst of the CBD
single or multiple
extrahepatic or intrahepatic
Tx - surgical excision
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40
Q

Premmie with increased gastric residual volume, vomiting, abd distension is highly suspicious for?

A

necrotizing enterocolitis

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41
Q

Kiddo has tx resistant GERD and eczematous patches

A

Eosinophilic esophagitis
Suspect if a GERD kid does not improve after 2 months of tx w/ PPI
EGD - circular rings and thickened linear furrowing of the esophagus
bx - >15 eos/hpf
Tx - dietary modification +/- topical (po) glucocorticoids

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42
Q

Next step for a suspected intussusception?

A

U/S guided air contrast enema

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43
Q

Kiddo has green vomit. Now what?

A

Neonatal bilious emesis = bowel obstruction

Get an immediate Xray to determine need for surgery, if stable do a contrast study to eval the level of obstruction

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44
Q

2-6 m/o w/ areflexia, hepatosplenomegaly, “cherry-red” macula, developmental regression

A

Niemann-Pick dz type A (A most severe, C least)
Sphinomyelinase deficiency
Fatal by 3 y/o
Tx - supportive

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45
Q

Pathophys of Guillain-Barre

A

demyelination of Peripheral motor n.

LMN signs

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46
Q

Grip myotonia (delayed muscle relaxation), facial weakness, foot drop, dysphagia, cardiac conduction anomalies

A

Myotonic muscular dystrophy (type 1, steinert dz)
AD expansion of trinucleatides
Other problems - cataracts, testicular atrophy/infertility, baldness

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47
Q

New onset seizures + a birthmark that is non blanching, erythematous and macular

A

Sturge-Weber syndrome
a neurocutaneous disorder
Port wine stain
Pathophys - mutation in GNAQ gene
Can also have - capillary-venous malformation, IDD, visual field defects, glaucoma
Dx - MRI w/ contrast
Tx - laser therapy, antiepileptic drugs, intraocular pressure reduction

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48
Q

Greatest RF for CP?

A

Although the etiology is multifactorial, the primary RF is prematurity

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49
Q

kiddo has staring spells where he turns his head and “chews” and won’t respond

A

Focal seizure
Originates in 1 cerebral hemisphere +/- LOC
Cannot envoke focal seizures w/ hyperventilation
Absence would just be a staring spell w/o moving

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50
Q

kiddo has a fall, then hours later presents with unilateral hemiparesis

A

Suspect a internal carotid a. dissection or thrombus, especially if there is a oropharyngeal injury
Hemiparesis, facial droop, aphasia
Dx - Ct or MR angio

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51
Q

kiddo w/ limb ataxia, wide based gait, decreased vibratory and position sense, absent ankle DTR. Feet have high plantar arches. MRI of brain and spinal cord show atrophy of the cervical spinal cord and minimal cerebellar atrophy. NCS are NL T wave inversion on EKG.

A

Friedreich ataxia
ar, excessive GAA repeats leading to abn of the frataxin protein
Offer prenatal counseling for future pregnancies

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52
Q

Most common form of inherited IDD

A

Fragile X syndrome
X linked, w/ trinucleotide repeat expansion
Speech, motor delys, prominent jaw, large ears, macroorchidism

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53
Q

DDx for flaccid paralysis

A

Inant botulism - ingestion of botulinum sporse from environment, descending, Tx w/ human botulinum IVIG
Foodborne botulism - ingestion of botulinum toxin, descending, Tx w/ equine botulism antitoxin
Guillain-Barre syndrome - autoimmune peripheral n. demyelination, ascending, Tx w/ pooled human IVIG

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54
Q

Minor trauma followed by breath-holding, pallor, diaphoresis, LOC

A

Pallid

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55
Q

Neonate w/ FTT, b/l cataracts, jaundice, hypoglycemia

A

Galactosemia
Galactose-1-phosphate uridyl transferase deficiency
elevated blood galactose
Tx - eleminate galactose from diet

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56
Q

RF’s for brain abscess

A 
Congenital Heart Dz
Recurrent sinusitis
Otitis media/mastoiditis
Dental infection
Presents with fever, HA (nocturnal or morning), FND, seizure
Dx w/ CT
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57
Q

kiddo has an increasing head circumfrence and bulging fontanelles, fussy x 1 month. now what?

A

Suspicious for hydrocephaus
Get a CT
Might need a VP shunt

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58
Q

Characteristics of absence seizures

A

Impairment of conciousness w/o loss of postural tone
occur w/o warning
<20s
Accompanied by simple automatisms (ie eyelid fluttering, lip smacking)

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59
Q 
Marfanoid body habitus
IDD
Downward lens dislocation
hypercoagulability
Fair complexion
A

Homocystinuria
ar, error in methionine metabolism d/t cystathionine synthase deficiency
Tx - vitamin supplementation (B6, folate, B12) to lower homocysteine levels, antiplatelet or anticoag

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60
Q

How do you eval respiratory fxn in Guillain-Barre?

A

Spriometery
Gold standard in eval for respiratory fxn (FVC)
Peak flow meter only measures the airflow out of the lungs

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61
Q

Neonate with scalp swelling that does NOT cross suture lines

A

Cephalohematoma
Subperiosteal hemorrage
Reabsorb in weeks to months
NO skin discoloration

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62
Q

Neonate w/ scalp swwlling that does cross suture lines

A

Caput succedaneum

Swelling is in the skin layers

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63
Q

Kiddo has sudden onset unilateral hemiparesis

A

Pediatric stroke

Most common cause is sickle cell dz, get a Hgb electrophoresis

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64
Q

Sickle cell pt with high ret count and thrombocytopenia

A

Splenic sequestration, pooling of red blood cells w/in the spleen
Occurs in younger pts with sickle cell dz whose spleens have not yet become fibrotic from recurrent vasooclusion
Anemia + splenomegaly
normocytic anemia, high ret count, thrombocytopenia

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65
Q

Pt is pancyotopenic weeks after a URI

A

Acquired aplastic anemia

dx w/ BM bx

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66
Q

Pt has half Hgb A and half Hgb S on Hgb electrophoresis

A

Sickle cell trait

Have normal lives, but increased risk for painless hematuria

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67
Q

tumor in the posterior fossa causing truncal and gait ataxia

A

Medulloblastoma
second most common posterior fossa in kids and arises from the cerebellar vermis
Can also have signs of increased intracranial pressure due to poximity to the 4th ventricle -> obstruction

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68
Q

How does hydroxyurea protect SCD pts from occlusive crisis

A

Increases fetal Hgb levels

Can cause myelosuppression (neutropenia, anemia, thrombocytosis), dose dependent

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69
Q

Xray with “sunburst” periosteal reaction and Codman triangle

A

Osteosarcoma
Most common primary bone tumor in kids
Involves the metaphyses
Large tender mass on PE

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70
Q

kiddo presents with limitation of upward gaze, bilateral eyelid retraction, and light-near dissociation

A

Parinaud syndrome d/t pineal glad tumor

Can also include HA and vomiting d/t obstructive hydrocephalus

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71
Q

CF kid is presenting with easy bruising. Why?

A

Vit K defeciency

CF are at risk of ADEK vit dfeciencies d/t lack of pancreatic enzymes and malabsorption.

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72
Q

Vit K is a cofactor for?

A

II, VII, Ix, X
1972 (X, IX, VII, II)
Prolonged PT

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73
Q

ar cause of congenital marrow failure, poor growth, morphologic abnormalities, macrocytic anemia

A

Fanconi anemia
Dx made by chromosomal breaks on genetic analysis, genes are believed to be involved in DNA repair
Tx - hematopoietic stem cell transplant
Most are diagnosed by 16, predisoposed to cancer

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74
Q

Family gets bloody diarrhea after cook out, 2 weeks later kiddo develops scleral icterus, abdominal pain

A

Hemolytic uremic syndrome
Initial infection with Shiga toxin producing E. coli (o157:H7)
Microangiopathic hemolytic anemia, thrombocytopenia, AKI

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75
Q

Types and inheritance of hemophilia

A

Hemophilia
A - Factor VIII def
B - Factor IX def
X-linked, only males are affected

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76
Q

Hemophilia kid presents with a big swollen knee

A

Hemophilic arthropathy
Occurs in both types of hemophilia and represents significant morbidity
Caused by deposition of iron/hemosiderin deposition leading to synovitis and fibrosis of the joint
Dx w/ MRI to get a good assessment of the extent of the damage
Tx early with the deficient factor

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77
Q

kiddo w/ brain mass has bitemporal hemianopsia and DI

A

Craniopharyngiomas
Calcified tumor in the suprsellar region
Comes with pituitary hormone deficiencies (DI, growth hormone deficiency)

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78
Q

Acute severe anemia in SCD. Sudden drop in Hgb, very low ret count, no hepatosplenomegaly

A

Aplastic crisis
Parvo is the most common trigger
splenic sequestration would have a higher ret count and a large spleen

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79
Q

Healthy kid has URI and 3 weeks later presents with scattered petechiae, thrombocytopenia, and peripheral smear showing large platelets

A

Immune thrombocytopenia
Observe, resolve spontaneously in 6 months
If kiddo has bleeding - give IVIG, glucocorticoids

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80
Q

Jaundice kid has anisocytosis, spherocytes, and polychromatophilia on smear

A

Hereditary spherocytosis
Triad of: Coombs-negative hemoltic anemia, jaundice, splenomgaly
Dx is confirmed with eosin-5-maleimide binding and acidified glycerol lysis tests

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81
Q

infant has acute onset of b/l hand and foot swelling, tenderness

A

Dactylitis
Earliest manifestation of vaso-occlusion in SCD
Presents 6m-4y

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82
Q

If a kid has a big swollen knee after a minor fall, do what?

A

Get a CBC and coag studies. Suspicious for hemarthrosis, hemophilia

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83
Q

Pts with functional asplenia will have what finding on peripheral smear

A

Howell-Jolly Bodies
Seen in SCD pts with functional asplenia
Small purple dot w/in the RBC

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84
Q

RF for retropharyngeal abscess

A

Pre-existing URI (rhinorrhea, nasal congestion
Polymycrobiol
ages 6m-6y
Decreased after 6y d/t regression of retropharyngeal LN and fewer viral URI

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85
Q

Newborn with hydrocephaly w/ diffuse intracerebral calcifications and vetriculomegaly

A

Congenital toxo

Mom had contact with cat poop or undercooked meat

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86
Q

Coughing fits followed by vomiting

A

Pertusis
Get a PCR from the nasopharynx
Tx - Macrolides (Azithromycin clarithromycin)
Start tx before getting results back

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87
Q

Food poisoning that causes vomiting

A 
Staph aurea
ingestion of preformed toxin
occurs w/in 1-6 hours after exposure
\+/- diarrhea
Usually transmitted via contaminated food handlers
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88
Q

What can cause decreased morbidity from malaria

A

Sickle cell trait protects from severe complications
Syx of febrile paroxysms
Dx with thick and thin blood smears

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89
Q

Most common RF for bacterial sinusitis?

A

Viral URI, mucociliary clearance is inhibited and pt is less able to clear bacteria
bacterial sinusitis - nasal drainage, congestion, cough, appear ill, high fevers (102.2), purulent nasal drainage x 3 days

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90
Q

infant with meningitis becomes hypotensive, develops petechia, and dies. Why?

A

Waterhouse-friderichsen syndrome

Sudden vasomotor collapse and skin rash due to adrenal hemorrhage

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91
Q

2 weeks s/p strep pharyngitis kid presents with a big swollen knee and new onset of fever, leukocytosis, + CRP, + ESR

A

Suspicious for septic joint

Get a artrocentesis, cultures and empiric abx

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92
Q

Kiddo has insidious onset of limp, hip pain and an antalgic gait. Internal rotation and abduction of the hip is limited

A

Legg-Calve-Perthes dz
Idiopathic avascular necrosis of the femur
Boy 3-12
Xray - sclerosis of the femoral head w/ flattening and fragmentation
Tx - non weight bearing, splint, potential surgery

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93
Q

Kiddo has unilateral knee swelling. Aspiration reveals translucent fluid with a WBC coung of 20k, no organisms on gram stain

A

Lyme dz (Borrelia burgdorferi)
Presents as an inflammatory monoarticular or oligoarticular arthritis
Synovial fluid = inflammator w/ negative Gram stain
Look for travel hx to the east coast
spirochete, deer tick (ixodes scapularis)
Dx with ELIDA and estern blot
Tx - doxycycline or amoxicilin

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94
Q

Trendelenberg sign (drooping of contralateral pelvis) is caused by?

A

Weakness of paralysis of the gluteus medius and minimus m., inn by superior gluteus n.

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95
Q

kid w/ fever > 5 days and 4 of the 5: nonexudative conjunctivitis, extremity changes, cervical lymphadenopathy, oral mucosal changes, polymorphous rash

A 
Kawasaki dz
Vasculitis of small and medium arteries
children <5, asian
Elevated inflammatory markers (CRP, ESR), thrombocytosis, sterile pyuria
typically self-resolves in 2 weeks
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96
Q

What is a complication of Kawasaki dz

A

Coronary a. aneurysm and thrombosis
Early tx with IVIG and ASA decrease MandM (start w/in 10days of fever onset)
Get an EKG at diagnosis and repeat every 6-8 wks

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97
Q

10 days after starting tx for strep pharyngisits kiddo has fever, uticarial rash, arthralgi, lymphadenopathy

A

Serum Sickness like rxn
Caused by B-lactams and sulfa drugs (PCN, Bactrim) w/ syx 1-2 after exposure
Resolves spontaneously after removal of abx
type III hypersensitivity

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98
Q

Hip pain in kiddo 2wks after URI and NL xray

A

Transient synovitis
NL lab results, able to bear weight, lack of fever r/o septic arthritis
tx - NSAIDs and rest
typically boys 3-8

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99
Q

knee pain, tenderness over proximal tibia at the site of the patellar tendon

A

Osgood-Schlatter
Traction apophysitis of the tibial tubercle
Xray - ant soft tissue swelling, lifting of the tubercle from the shaft, irregularity or fragmentation of tubercle
worse w/ activity, improves w/ rest

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100
Q

RF for vitamin D deficiency rickets

A

Exclusive BF
Increased skin pigmentation
Lack of sun exposure
Exam - craniotabes, rachitic rosary, genu varum
Xray - cupping and fraying of the metaphyses of the long bones

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101
Q

What is recommended for postexposure prophylaxis after exposure to an animal risky for rabies?

A

Rabies IG
Rabies vaccine
Prognosis - coma, Resp Failure, death w/in weeks

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102
Q

Newborn w/
copious rhinorrhea
ABN long-bone radiographs
Desquamating or bullous rash

A

Syphilis

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103
Q

Newborn w/ periventricular calcificaitons

A

CMV

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104
Q

Kiddo gets successive patches of intensely pruritic vesicles

A

Varicella
Self-limiting
Transmitted in aerosols w/ 2wk incubation
Varicella vax at age 1 and 4

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105
Q

Acute, unilateral cervical lymphadenitis is caused by

A

Staph aureus

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106
Q

Tx for Lyme dz in kids <8y/o

A

po amoxicillin
rash = erythema chronicum migrans
Doxycycline is contra in kids <8y/o and preggos

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107
Q 
Shortly before delivery preggo has Hep panel:
HBsAg +
Anti-HBsAg -
IgM Anti-HBcAg +
HBeAg +
Anti-HBeAg -
A

Mom has active Hep B

Kiddo needs hepB IG followed (passive) by Hep B vax (active)

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108
Q

1 wk after MMR vax kiddo has fever and rash

A

Reassure

Some kiddos get fever and rash 1-3 weeks after vax, doesn’t need tx

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109
Q

Tx for cat bite

A

po Amoxicillin w/ clavulanate

Tx against Pasturella multocida and po anaerobes

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110
Q

1 wk s/p heart surgery kiddo presents with tachycardia, tachypnea, and distant “muffled” heart sounds

A

Pericardial effusion
w/in days or months after surgery
Postpericardiotomy syndrome
LIfe threatening, needs drainage

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111
Q

Cardiovascular abn in Turners

A

Bicuspid aortic valve
Coarctation of aorta
aortic root dilation
45, X

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112
Q

Harsh holosystolic murmur at LLSB

A

VSD
Get an echo
75% close spontaneously by 2 y/o
Large defect -> PHTN, growth failure, CHF, Eisenmenger syndrome

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113
Q

Blue spells, murmur gets louder and cyanosis improves w/ squatting

A

Tetralogy of Fallot
RV outflow tract obstruction and R to L shunting during exercise or agitation
Squatting increases afterload, increasing flow across RVOT

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114
Q

Kawasaki tx

A

ASA + IVIG w/in 10 days of fever onset to prevent coronary a. aneurysms

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115
Q

Which med can cause Reye syndrome

A

ASA

Kawasaki is an exception

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116
Q

Pt has a hx of rheumatic fever. How do you manage?

A

Continuous abx prophylaxis w/ PCN to prevent recurrent GAS and limit progression to rheumatic heart dz
IM benzathine PCN G q4wks

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117
Q 
Low set ears
Micrognathia
cleft palate
Truncus arteriosis
At risk for?
A

DiGeorge baby
Need immediate eval for hypocalcemia
Suspect in craniofacial abn, thymic hypoplasia, congenital HD
22q11 poor development of pharyngeal pouch

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118
Q

baby gets blud during feedings, different BPs in different limbs, crescendo decrescendo at LUSB

A

Tetralogy
LUSB murmur d/t turbulence at stenotic pulmonary a.
Give Oxygen and put knees to chest

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119
Q

Kiddo has grade II murmur that disappears w/ standing

A

Benign murmur

reassure

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120
Q

Febrile kid gets ASA then presents with vomiting, encephalopathy, hepatic dysfunction, abn behavior -> seizures, lethargy

A

Reye syndrome

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121
Q

vomiting, abd distension, Tripple bubble sign on Xray w/ gasless colon

A

Jejunal atresia

RF - prenatal exposure to cocaine/vasoconstricive drugs

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122
Q

ASA during flu or varicella infection causes?

A 
Reye syndrome
Encephalopathy/cerebral edema
Acute fatty liver failure
Elevated LFTs, coagulopathy, hyperammonemia
Bx - microvesicular steatosis
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123
Q

newborn chokes and coughs during first feedings

A

esophageal atresia w/ tracheoesophageal fistula
ng tube insertion ill fail w/ tube in the esophageal pouch
Polyhydramnios, aspiration PNA
Workup for VACTREL

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124
Q

What is VACTREL?

A 
vertebral, anal atresia
cardiac
tracheoesophageal fistula
renal 
limb
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125
Q

Ulcerative Colitis kid has diarrhea, rash, and irritability

A

Niacin deficiency
Pellagra
Death in severe cases

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126
Q

First week of life, kiddo has suboptimal breastfeeding, dehydration. Decreased bili elimination

A 
Breastfeeding failure jaundice
unconj hyperbioi
inadequate stooling
Caused by lactation failure, 
Tx - optimize lactation, BF 15 min easch side q 2-3 hr
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127
Q

Newborn w/ feeding intolerance, abd distension, bloody stool

A

Nec enterocolitis
RF - prematurity, hypoension, congenital heart dz
Xray pneumatosis intestinalis

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128
Q

Small palpebral fissures
smooth philtrum
Thin vermillion border

A

Fetal alcohol syndrome

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129
Q

Kid has head trauma and vomiting. Order a?

A

CT w/o contrast

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130
Q 
Cafe au lait spots
macrocephaly
feeding problems
short stature
learning diabilities
A

Nuerofibromati=osis `

May later develop fibromas, neurofibromas, or different tumors

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131
Q

> 50 words, 2 word phrases

A

2 y/o

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132
Q

Loc, hemiparesis resolving in hours

A

Seizures

Todd paralysis, self-limited focal weakness after a seizure

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133
Q

unilateral HA, photophobia

A

Migraine

Kids - NSAIDs supportive, try tirptans if this isn’t effective

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134
Q

Most common tumor in kids?

A

Pilocytic astrocytoma

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135
Q

IDD, fair complexion, musty odor

A

Phenylketonuria
Deficiency of phenylalanine hydroxylase
Build up of phenylalanine and its neurotoxic metabolites
Tx - low Phe diests
If missed on newborn screen - get quantitative amino acid analysis for elevated Phe

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136
Q

Most common cancers in kids

A

Leukemia
CNS tumors
Neuroblastoma

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137
Q

KIddo has increased urine catecholamines and other metabolites (Homovenillic acid, vaillylmandelic acid)

A

Neuroblastoma
Arises from neural crest cells
median age 2 yrs

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138
Q

Kiddo < 3y w/ fever >102.2 w/o obvious syx

A

Get a cath and urine culture

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139
Q

FHx of RF
Hematuria
Sensorineural deafness

A

Alport’s syndrome

EM - alternating thin and thick capillary loops w/ splitting of the GBM

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140
Q

FTT
Metabolic acidosis
NL anion gap

A

Renal tubular acidosis
defect in either H+ excretion or bicarb reasorptoin in the kidney
FTT - cells don’t grow well in acidic environments

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141
Q

Kids that have recurrent or chronic pyelo

A

severe vesicoureteral reflux
Complications - parenchymal scarring, HTN, renal insufficiency
Dx on voiding cystourethrogram

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142
Q

Kiddo has abd pain, vomiting, erythematous macular rash on her legs and back, hematuria, proteinuria

A

Henoch-Schonlein purpura
IgA mediated small vessel vasculitis (nephritic syndrome + rash)
renal Bx - IgA deposition in the mesangium

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143
Q

Fluid resuscitation in vomiting kid with Na 165

A

NS, intial fluid of choice in severe hypovolemic hypernatremia

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144
Q

How do UTI’s happen?

A

When bacteria ascend into the bladder from the vaginal introitus
Sex is a big RF for UTI d/t introduction of uropathogens into the urethra

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145
Q

Renal dz in Active Hep B infection

A

Membranous nephropathy

Nephrotic syndrome in adolescents/adults, Hep B is a huge RF

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146
Q

Causes of nephrotic syndrome

A

Minimal change dz (kids)
Focal Segmental glomerulosclerosis (FSGS)
Membranous nephropathy
Membranoproliferative glomerulonephritis

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147
Q

Causes of nephritic syndrome

A 
Poststrep GN (peds)
Hemolytic uremic syndrome (peds) 
IgA nephropathy
Membranoproliferative glomerulonephritis (nephrotic, nephritic)
Crescentic glomerulonephritis
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148
Q

Why does constipation increase risk for cystitis?

A

Urinary stasis

Impacted stool -> rectal distension -> compresses the bladder -> incomplete voiding -> stasis

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149
Q

What is normal weight loss after birth?

A

Newborns lose up to 7% of birth weight in first 5 days of life
No tx is required
Birthweight should be regained by age 10-14 days

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150
Q

Pink stains, or brick dust in a kids diapers are?

A

Uric acid crystals

Uric acid excretion is especially high at birth and tapers until adolescence

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151
Q

New born has dry flaky hands and feet after birth

A

totally normal. Skin is adjusting to the dry extrauterine environment

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152
Q

Most common cause of anemia in preterm infants

A

Anemia of prematurity
D/t diminished erythropoietin levels, shortened RBC life span, blood loss
Low Hgb, HCT, low ret count
epo diminishes after delivery because theres more oxygen in the tissues, further exacerbated by HgbF turnover every 40-50 days, frequent phlebotomy

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153
Q

Kiddo has fever, irritability and skin tenderness that progresses to generalized erythema, superficial flaccid blisters, epidermal shedding w/ light pressure

A

Staphylococcal scalded skin syndrome (SSSS)
Exfoliative toxin-producing strains of Staph aureus
Typically in kids and young children

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154
Q

Kiddo has a hoarse cry, increased TSH and low T4

A

Congenital hypothyroidism
Thyroid dysgenesis is the most common cause of congenital hypothyroidism
Associated wth neurodevelopmental injury if not treated early
Syx develop as maternal T4 wanes (lethargy, englarged fontanelle, protruding tongue, umbilical hernia, poor feeding, constipaiton, dry skin, jaundice)
Tx - levothyroxine

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155
Q

3-4 m/o w/ hypoglycemia, lactic acidosis, hepatomegaly, elevated triglycerides

A

Glucose-6-P deficiency (type 1 glycogen storage dz, von Gierke dz)
Often presents w/ seizures
Pts have doll-like face, thin extremities, short stature, protuberant abdome (d/t hepatomegaly)

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156
Q

Low IgG
Low IgA
High IgM
NL WBC

A

Hyper IgM syndrome

Defect in CD40 ligand

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157
Q

Low or absent B cells, low Immunoglobulins

A

Xlinked agammaglobulinemia

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158
Q

NL B cells

Low Immunoglobulins

A

Common Variable immunodeficiency

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159
Q

NL B cells

Low IgA

A

IgA deficiency

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160
Q

NL B cells

low IgG

A

IgG subclass deficiency

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161
Q

Most common complication of babies born to diabetic moms

A

Hypoglycemia

GDM is significant enough

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162
Q

Kiddo flexes hips when neck is flexed and there is a erythematous nonblanching pinpoint ras on the lower limbs and trunks

A

Neisseria meningitidis
Common cause of meningitis in kids > 3m
Meningeal signs + petechial or purpuric rash
Tx 3rd gen cephalosporin (ceftriaxone) and vanco

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163
Q

Dad picked kid up by the arm and now she holds her hand pronated and won’t use it. Hyperpronation reduces the injury

A

Radial head subluxation (nursemaids)
Caused by ligament displacement d/t axial traction on the arm
tx - forearm hyperpronation or supination w/ flexion

164
Q

knee Xray w/ central lytic lesions, ornion skinning, and moth-eaten appearance w/ extension into soft tissue

A

Ewings sarcoma
Presents similar to osteomyolitis
Highly malignant, usually in lower extremity
Metaphysis and diaphysis of the femur
Mets to lung and LN
White males 10-20yo
Tx - surgery, w/ pre op radiation, multi-drug chemo

165
Q

II/VI holosystolic murmur over LLSB, diastolic rumbler over cardiac apex

A

VSD (L to R)
Eisenmenger when shifts to R to L
FTT, fatigability, HF
Diastolic rumble d/t flow across mitral valve

166
Q

RF’s for Respiratory distress syndrome

A 
Prematurity (#1)
Maternal DM
Male
Perinatal asphyxia
C-section w/o labor
RDS is caused by surfactant deficiency
Tx - antenatal corticosteroids and postnatal tx with exogenous surfactant w/ respiratory support
167
Q

Precocious puberty d/t an ovarian mass

A

Granulosa cell tumor
Malignant, estrogen secreting tumor
U/S Ovarian mass, thickened endometrium

168
Q

local rash with a thick golden yellow crust

A 
Impetigo
(GAS or staph)
Bullous is always staph
Tx with topical mupirocin
If skin involvement is extensive give po abx (cephalexin, dicloxacillin, clindamycin)
169
Q

Kid has viral URI that improves and a week later develops new cough and nasal discharge

A

Acute bacterial rhinosinusitis
Tx - po abx (amox-clavulancin acid)
Persistant or worsening syx should raise concern for bacterial sinusitis

170
Q

Really tall waves on lead I on EKG

A

Lshift deviation
Suggestive of tricuspid atresia
Cyanotic congenital heart defect, L axis deviation on EKG, decreased pulmonary markings on CXR (hypoplasia of the RV and pulmonary outflow tract)

171
Q 
Microcephaly
Prominent occiput
IUGR
Micrognathia
Closed fists w/ overlapping digits
A

Edwards syndrome (trisomy 18)
Also rocker bottom feet
95% mortality in the first year of life

172
Q

RF for polycythemia in a newborn

A 
Delayed cord clamping
Maternal HTN
Maternal DM
HCT>65%
Syx - RDS, hypoglycemia, neurologic syx
Tx - hydration is asyx, syx need partial exchange transfusion (blood removed and replaced with NS)
173
Q

episodes of vomiting lasting up to 10 days; >3 episodes in a 6 month period

A

Cyclic vomiting syndrome
Tx- anti-emetics, anti-migaine agents
Typically have a Fhx of migraines

174
Q

Most common causes of PNA in CF kids?

A

Kids/teens - Staph aureus
Adults - Pseudomonas aeruginosa
The change in flora occurs around 20 y/o

175
Q

Kid with Tourette syndrome is at increased risk for?

A

ADHD and/or OCD

176
Q

Lesch-Nyhan syndrome is caused by?

A

Hypoxanthine-guanine phophoribosyl transferase (HPRT)
self-mutulation, neuro, gouty arthritis, tophus formation
kid w/ gout - suspect Lesch-nyhan

177
Q

Copious purulent eye drainage and eyelid swelling in the first week of life

A

Gonococcal conjunctivitis

prevented w/ top erythromycin rigth after birth

178
Q

Watery eye discharge w/in 5-14 days after birth

A

Chlamydial conjunctivitis

179
Q

Kiddo is verbal at home but silent at school

A

Selective mutism
syx for > 1 month
Considered to be an anxiety disorder and should be treated early to prevent long term educational and social impairment

180
Q

kiddo has recurrent skin/mucosal bacterial infections and severe periodontal dz, high WBC with PMN predominance

A

Leukocyte adhesion deficiency
Also see delayed umbilical cord seperation (>21 days)
Pathophys - defective integrins on the leukocyte surface, which normally let PMN’s adhere to the vascular endothelium
Infection will have a lack of purulence

181
Q

How do you define sm all for gestational age and what are they at risk for?

A 
<10th percintile for weight at birth
Hypoxia
Polycythemia
hypoglycemia
hypothermia
hypocalcemia
182
Q

Precocious development of pubic and axillary hair, acne, and body odor in a kid with a normal bone age

A

Premature adrenarche
Caused by early activation of adrenal androgens and is more common in obese kids
estrogen and test are WNL so no premature puberty or virilization
Mild elevation of DHEA presents with adrenarche but does not affect skeletal growth so bone age is NL

183
Q

Tx for septic arthritis

A

Emergency surgical drainage and IV abx to prevent permanent joint destruction
Staph is most common

184
Q

Neurofibromatosis kid has left eye squinting, proptosis, esoropia, and optic disk pallor

A

Optic pathway glioma
associated with NF 1
Unilateral vision loss, proptosis, esotropia, optic disc pallor
Mutation in NF1 which codes for neurofibromin

185
Q

Teen with abn uterine bleeding shortly after undergoing menarche

A

Hypothalamic-pituitary-ovarian axis prematurity

anovulatory cycles w/o sluffing cause increased bleeding

186
Q

Tx for chlamydial conjunctivitis

A

po macrolide (erythro)
top is ineffective
watch fpr pyloric stenosis - side effect of azithro and erythro

187
Q

Tx for gonococcal conjunctivitis

A

IM ceftriaxone

188
Q

Superficial infantile hemangiomas AKA

A

Strawberry hemangiomas
Benign, grow rapidly but regress spontaneously
If complications do arise - BBlockers

189
Q

Pediatric HTN, Higher pulse ox in upper extremities than lower

A

Coarctation of the aorta
Important cause of pediatric HTN
Caused by thickening of the tunica media of the aortic arch

190
Q

Etiology of marfan

A

Mutation in fibrillin 1
AD
Tall, thin extremities, arachnodactylyl, joint hypermobility, upward lens dislocation, aortic root dilation (most dangerous)

191
Q

Third world
cc eye redness, watery discharge
pe pale follicles and inflammatory changes in the tarsal conjunctivae bilaterally. Conjunctivae are mildly thickened
Visual acuity is 20/20

A

Trachoma
Caused by chlamydia trachomatis A,B,C
Leading cause of blindness in the world
Presents with follicular conjunctivitis and inflammation
Repeat or chronic infection causes inversion of the eyelashes and scarring of the cornea
tx - po azithromycin, need surgery to preserve vision

192
Q

when should vaccines be given to premmies?

A

According to chronologic age, not gestational

Weight must be >2kg (4lb6oz) prior to first HBV

193
Q

If a SCD pt is taking hydroxyurea, their HbF level will likely be?

A

> 15%

Hydroxyurea can cause myelosuppression -> neutropenia

194
Q 
Infant w/
Macrocytic anemia
Short
webbed neck
cleft lip
shield chest
triphalangeal thumbs
A

Diamond-Blackfan syndrome
AKA congenital hypoplastic anemia
Macrocytic anemia, low ret count, congenital anomalies
elevated HgbF on electrophoresis
Normal chromosomes
Tx - corticosteroids, transfuse if unresponsive

195
Q

Painful lytic bone lesion w/ overlying swelling and hypercalcemia

A

Langerhans histiocytosis

196
Q

Thrombocytopenia
Eczema
Recurrent infections

A

Wiskott-Aldrich syndrome
X-linked defect in WAS
Impairment in cytoskeleton changes in leukocytes, platelets
Low platelet count and small platelets is the most pronouced finding
Tx - stem cell transplant

197
Q

Hematochezia
Shock
Metabolic acidosis
Radiopaque tablets on Xray

A

Iron poisoning
Occurs in kids whose mom has pre-natal vitamins because kids think it’s candy
Tx - deferoxamine binds ferric iron and allows for urinary excretion

198
Q 
Persistant jaundice
Hemolytic anemia 
Splenomegaly
\+ FHx
Spherocytes on peripheral blood smear
A

Hereditary spherocytosis
Defect in RBC membrane
AD defect in ankyrin gene leads to spectrin deficiency
Dx - RBC fragility on acidified glycerol lysis and eosin-5-malemide binding tests
Negative coombs test

199
Q

Tx for QT syndrome

A

Beta blockers w/ pacemaker

Avoid all meds that block K+

200
Q

Management of asthma that is unresponsive to nebulized steroids (AMS, minimal aeration, hypercarbia, worsening hypoxemia)

A

Intubate

201
Q

Teen notices a breast lump days before her next period. Now what?

A

Reschedule for a few days after her period
Most likely a fibroadenoma
Reexamine after her period to assess if the mass decreases in size/tenderness

202
Q

Kiddo has wight patches w/ scaling and itching

A

Tinea versicolor
Usually trunk and upper extremities
Most noticeable after sun exposure
Dx - KOH w/ yeast cells and hyphae (spaghetti and meatballs)
Tx - TOP selenium sulfide, antifungals
Vitilgo - looks similar but not itchy or scaly

203
Q

Contras to rota vax

A

Hx of intussesception
Hx of uncorrected GI malformatin (ie meckels)
SCID

204
Q

kids can say mama dada by?

A

9 months

205
Q

kids can respond to name and babble by?

A

6 months

206
Q

Say words other than mama dada?

A

1 year

207
Q

seperation anxiety is normal at?

A

9-18 months

208
Q

young girl w/
pubic/axillary hair
advanced bone age
elevated LH

A

Central Precocious puberty
Needs an MRI to evaluate for tumor
If no tumor -GnRH agonist
Note that they’ll be shorter despite bone age because estrogen closes the plate

209
Q

Tx for immature HPO axis presenting with heavy bleeding?

A

high dose OCP’s

Stabilizes the endometrium and stop the acute bleeding

210
Q

Neonate w/ blanchin erythematous papules and pustules

A

Erythema toxicum neonatorum
Benign
Resolves spontaneously w/in 2 wks

211
Q

Frequent infections

Small tonsils

A

X-linked agommaglobulinemia (Bruton)
Abn B cell development d/t defect in tyrosine kinase
Typicaly presents around 6mo when maternal immunity wanes
Tx - IVIG, prophylactic abx

212
Q

What do you tell parents that are concerned for imaginary friends?

A

Totally normal for 3-6 y/o and can be seen throughout school age
May help with social relationships as this is a form of rehearsal and lets them express creativity

213
Q

Kids that get frequent
GI (Giardia, salmonella, campy)
Respiratory (PNA, sinisitis, OM)
also has FTT, autoimmune dz, chronic lung dz, GI disorders

A

Common variable immunodeficiency
B cell differentiation is abn, decreased production of multiple IG classes
Risk of encapsulated bacteria, Giardia, and enterovirus
FTT and chronic pulmonary dz
Tx - IVIG to prevent severe infection
No response to vax, severely low IgG

214
Q

IF CF kids is hospitalized for flu, also prophylax for?

A

Staph aureus

Start IV vanc (MRSA) in addition to cefepime (MSSA, pseudomonas) and oseltamivir

215
Q

Comorbidity of absence seizures?

A

ADHD, anxiety
ADHD tends to be inattentive subtype
absence = 3Hz spike wave changes on EEG
ADHD is a common comorbidity in any child with epilepsy

216
Q

Croup kid being treated with steroids is entering respiratory failure

A

Give nebulized racemic epinephrine

subglottic edema causes barky cough and inspiratory stridor

217
Q

SCD pt presents with nagging groin/thigh/butt pain w/o erythema or elevated inflammatory markers

A

Osteonecrosis

Xray appears normal, MRI more revealing

218
Q

Teen has excessive urination despite no UTI syx and restricting fluid intake. Serum and urinary Sodium is essentially normal but SG low

A

Hyposthenuria
Inability of the kidneys to concentrate urine
Occurs in pts with SCD or trait
Polyuria, low SG, NL serum sodium

219
Q

Kid has been exposed to varicella but asymptomatic. Now what?

A

Give varicella vax if <4y/o
Kids <4 have only had 1 vax and are considered non immune. They will need postexposure prophylaxis.
If kiddo cannot receive vax give varicella IG instead

220
Q

Puberty is considered precocious in kids

A

<8, girls

<9, boys

221
Q

Bedwetting is normal until

A

5 y/o

222
Q

HSP increases the risk for which GI dz?

A

intussusception
d/t intestinal edema and bleeding
IgA mediated vasculitis
Kiddo has palp purpura, abd pain, arthralgi, +/- hematuria

223
Q

Cardiac anomaly in Edwards syndrome

A

VSD

holosystolic murmur at LLSB

224
Q

Most common cause of acute bacterial rhinosinusitis?

A

strep pneumo (30%)
H. flu (30%)
Moraxella catarrhalis (10%)
Tx - amoxicillin-clavulanic acid

225
Q

solitary herald patch followed by clusters of smaller oval lesions in a “christmas tree” pattern

A

Pityriasis rosea

Tx - reassurance, symptomatic relief (antihistamines, top corticosteroids)

226
Q

Which maneuvers decrease the intensity of the murmur in hypertrophic cardiomyopathy?

A

maneuvers that increase preload or afterload
Squatting, leg raise, hand grip
Increase in LV cavity size decreases the obstruction

227
Q

Teen w/ back pain
urinary incontinence
NL straight leg test
step off on PE

A

Spondylolistesis
Forward slip of vertebrae (L5,S1), manifests in preadolescent kids
back pain, neuro dysfunction, step-off are usually less severe

228
Q

If kiddo has pertusis, what do you do for the rest of the family?

A

Give a macrolide abx to all household contacts

229
Q

After repair of cryptorchidism, pt is at greatest risk for?

A

subfertility
Testicles that haven’t descended by 6 months need an orchipexy, ideally before 1 yr
Almost all also have a patent processus vaginalis so this repair also prevents future inguinal hernias
No risk of torsion

230
Q

Kiddo has coughing paroxysms and subQ emphysemsa

A

Get a CXR to r/o PTX

231
Q

Kiddo has decreased hip ROM and foot points outward. Flexion of the hip causes external rotation

A

Slipped capital femoral epiphysis
Needs surgical pinning to lessen risk of avascular necrosis
Obese boys in early adolescence

232
Q

Kiddo has proteinuria on dipstick. now what?

A

Repeat dipstick on two subsequent occasions

This may be transiet proteinuria, but if elevated at 3 visits its considered persistant and needs further workup

233
Q

After using a topical anesthetic, kiddo turns blue

A

Methemoglobinemia
Cyanosis after exposure to an oxidizing agent (dapsone, nitrities, local/topical anesthetic)
Pulse ox will be low, but PaO2 is NL
1 out of 4 of the Hgb becomes oxidized to ferric (Fe3+) vs the normal ferrous (Fe2+). Ferric has weaker O2 affinity, causing the other 3 ferrous to have increased O2 affinity -> makes off loading in the tissues harder
O2 does not improve sats, blood will be dark chocolate colored
If oxygen delivery is impaired enough -> AMS, seizures, death

234
Q

Tx for methemoglobinemia

A

Methylene blue

Acts as a e- receptor for NADPH and reduces methemoglobin back to hemoglobin

235
Q

Daily fever x 2 months
Arthritis of at least 1 joint
Pink macular rash
Leukocytosis, thrombocytosis, elevated ESR, and anemia

A 
Juvenile idiopathic arthritis
Arthritis worse in the morning
Anemia of chronic dz
Dx of exclusion
Tx - NSAIDs, glucocorticoids, biologics
236
Q

PDA is what type of shunt?

A

Extracardiac L to R

237
Q

AFter repair of truncus arteriosus defect kiddo is gittery. Micrgnathia, hypertelorism, low calcium, no thymus. Why gittery?

A

Hypoparathyroidism

DiGeorge syndrome

238
Q

15 y/o boy hasn’t started shaving,

A

Reassure

239
Q

Collection of fluid in the middle ear, but tympanic membrane is mobile

A

Serous otitis media

Can accumulate d/t UTI

240
Q

dark urine 10 days s/p URI

A

PSGN

Antigen-antibody immune complexes

241
Q

7 d/o Bilious vomit, one bloody stool. Poor barium movement through GI on swallow study

A

Failure of normal bowel rotation

242
Q

Kiddo presents with bloody mucous diarrhea after completing a 10 day course of amoxicillin-clavulanate

A

C. diff

243
Q

Trisomy 18 pt is also exposused to alcohol in utero. Which will cause the baby to have a low birth weight?

A

Trisomy 18 (Edwards)

244
Q

Newborn is having coughing during feeds and polyhydramnios. Next you?

A

Insert a NG tube

245
Q

Newborn has a lobulated and compressible lesion overlying the buttocks. Petechiae and bruises. Why?

A

Thrombocytopenia from platelet sequestration

246
Q

Humeral fx, now can’t extend wrist or fingers

A

Radial n.

247
Q

Stidor is caused by pathology at the?

A

Larynx

248
Q

kid has itching and hair loss on the scalp. Alopecia, numerous broken hairs. Occipital LN

A

Trichophyton tonsurans

249
Q

Nonproductive cough worse during day. Does not worsen w/ exercise. Does not respond to INH steroids. Xray - linear consolidation in the R middle lobe. Dx?

A

Bronchiectasis

Middle lobe syndrome?

250
Q

Teen has seizure after deciding to d/c his CCB which controls his HTN. Current BP 250/154. Now what?

A

Give sodium nitroprusside

251
Q

Severe intermittent abdominal cramping, reddish discoloration of stool

A

Intussusception

252
Q

New born w/ jaundice, BF. HCT was 54%, now 5@. High total bili, direct is NL. Dx?

A

Physiologic jaundice

253
Q

Teen girl w/ mass on one side of neck, progressive axillary and inguinal lymphadenopathy, pruritis, weight loss.
CXR - mediastinal mass
Icreased ESR, ferritin. Dx?

A

Hodgkin dz

254
Q

Kiddo has low BP and Urine output.

A

Give IV dopamine

255
Q

Teen female w/ short stature and delayed puberty. Never had a period. Multiple nevi over face and trunk. b/l pedal edema. Tanner 1

A

Gonadal dysgensis (Turners)

256
Q

Kid was unattended now has bomiting and drooling. Two small superfical ulcers on lips and tongue. What did she ingest?

A

Drain cleaner

basic

257
Q

kiddo has a hard time clearing chickenpox. Why?

A

Defect in T cells

258
Q

Toddler with aniridia and mass in the flank. You order a?

A

U/S of the abdomen

259
Q

Kiddo w/ SOB, JVD, swelling of the face and Upper extremities
CXR - mass in the superior mediastinum and large pleural effusion in the right hemithorax
Thoracentesis - 400mL of reddish brown serosanguineous fluid w/ numerous erythrocytes. Why?

A

Malignant pleural effusion

Classic presentation of non-Hodgkin lymphoma

260
Q

URI kid has BP 60/palp and an S3

A

Cardiogenic shock s/t myocarditis

261
Q

16 y/o female comes to the office and hasn’t had vax’s since she was 10. What is she due for?

A

HPV
Meningococcal
TdaP

262
Q

Tx for UTI in a 3 m/o

A

Cefotaxime

263
Q

Premmie infant gets frequent PNA s/t grade 4 intraventicular hemorrage. Barium swallow shows grade 4 GERD. What will help decrease the frequency of his PNA?

A

Fundoplication

264
Q

Retinal hemorrhages and subdural tears are caused by what type of injury?

A

Acceleration-decelaration head injury

265
Q

Excess of which vitamin can cause pseudotumor cerebri?

A

Vitamin A

266
Q

kiddo has non billious projectile vomiting in first days of life

A

Get a abd U/S to r/o pyloric stenosis

267
Q

Toddler w/ abd pain, firm nodular midline mass. 120/80

Xray and U/S - 12 cm midline mass w/ calcifications

A

Neuroblastoma

also look for HTN

268
Q

Sexually active teen should be screened for ?

A

Chlamydia, gonorrhea

269
Q

Teen has intermittent swelling of lips, arms, face, chest that seems random. also gets abd and diarrhea w/ these episodes

A

Get measurement of serum C1 esterase inhibitor concentration

270
Q

electrolyte abnormalaties in pyloric stenosis?

A

Low Cl-, K+
pH high
hypochloremic alkalosis

271
Q

Teen w/ increasing facial hair, 12lb weight gain, striae

A

Cushings

Get a morning fasting cortisol

272
Q

2 greatest RF’s for interventricular hemorrhage in a newborn?

A

Prematurity

Low birth weight

273
Q

1st line therapy for unexplained enuresis

A

Desmopressin

274
Q

Endometriosis vs primary dysmenorrhea

A

PD - pain starts w/in 24 hours of menstruation, no pain w/ sex
Endo - pain days prior, pain with sex

275
Q

Primary amenorrhea is defined as

A

absence of menarche by age 15

276
Q

1st step in management of a pyloric stenosis pt?

A

IV hydration and K replacement

Followed by pyloromyotomy

277
Q

Precocious puberty (secondary characteristics; pubic hair, acne)
Accelerated borne growth/age
Low-NL LH
LH levels do not respond to GnRH stimulation

A

Nonclassic congenital adrenal hyperplasia
21 hydroxylase deficiency
low-NL LH level d/t inhibition of the hypothalamus by high androgen levels from the adrenals or testes
NO salt wasting

278
Q

Turners newborn has peripheral edema

A

congenital lymphedema d/t abd development of the lymphatic network
Accumulation of protein rich interstitial fluid in the hands, feet, and neck (webbed neck). Can cause cystic hygroma if severe enough
Tuerners - webbed neck, corshoe kidney, carpal/pedal edema, nail dysplasia

279
Q

Blue sclerae

Recurrent fx’s

A

Osteogenesis imerfecta
Also associated w/ hearing loss and opalescent teeth (dentinogenesis imperfecta; caused by discoloration of dentin)
AD mutation in COL1A1

280
Q

Osteogenesis imperfecta is a defect in?

A

Type 1 collagen

281
Q

Toddlers behaviors toward sex

A

Normal - exporing own or others genitals, mastubatory movements, undressing self/others
NL for school kids - Interest in sex words/play, asking about sex, masturbatory movements
ABN - Repeated object insertion into V or A, sex play involving g-g, o-g, or a-g contact, use of force/threats/bribes in play, sexual knowledge not appropriate for age (ie oral sex)

282
Q

Newborn

Dehydration, salt wasting (hypotensive), virilization

A

Congenital adrenal hyperplasia

21-hydroxylase deficiency causes 17-hydroxyprogesterone to be elevated

283
Q

Meconium ileus is diagnostic for?

A

Cystic fibrosis

Inspissated (viscous) meconium can cause life-threatening obstruction of the ileum

284
Q

Newborn has b/l medial deviation of the foot, but ROM and DTR’s are NL

A

Metatarsus adductus
Tx - reassurance
Feet are flexible tat over corrected in utero

285
Q

Newborn has rigid medial/upward deviation of the forefoot and hindfoot. Hyper-planta flexion of the foot

A

Clubfoot

Tx - serial manipulation and casting, surgery for refractory cases

286
Q

Inspiratory and expiratory stridor that improves with extension of the neck, but not when prone

A

Vascular ring
Rings of vessels encircle the trachea and or esophagus and present with respiratory syx that improve with neck extension
abn development of aortic arch
can also have dysphagia

287
Q

Stridor that is worse when supine, improves when prone

A

Laryngomalacia

288
Q

Kiddo w/
sudden behavioral problem
Unstable home environment OR
Parents with active drug/alcohol abuse

A

Be suspicious for physical or sexual abuse

289
Q

sharply defined rash extending circumferentially around the anus

A

Strep perianal dermatitis
look for recent contact with strep pharyngitis pt
Tx - po abx (penicillin, amox)

290
Q

Causitive organism in impetigo?

A

Staph
Strep pyogenes
Tx - TOP abx

291
Q

kid walks on toes
b/l calf enlargement
Gower sign

A

Duchenne muscular dystrophy
presents ages 2-5 y/o
X-linked recessive, deletion of dystrophin gene
Dx - genetic testing

292
Q

How do you manage a vaginal foreign body?

A

Toilet paper is most common
Remove w/ calcium alginate swab or irrigation w/ warmed fluid after top anesthetic is applied
If this fails - general anesthesia

293
Q

When should burn injuries concern you?

A

Sparing of the flexor surfaces (“zebra pattern”, doughnut pattern)
Sharp lines of demarcation (no splash marks)
Uniform burn depth

294
Q

What happens to the CBC in splenic sequestration?

A

Normocytic anemia
Rets increase
Thrombocytopenia
Caused by splenic vaso-occlusion -> rapid splenomegaly

295
Q

Kid with limp, Xray looks normal in early dz

A

Legg Calve Perthes

Avascular osteonecrosis of the femoral head

296
Q

Kiddo w/ purulent eye discharge in first week of life

A

Gonococcal conjunctivitis

needs IM cefotaxime/ceftriaxone

297
Q

Kiddo w/ purulent eye discharge in second week of life

A

Chlamydial conjunctivis

needs po erythro

298
Q

How do you tell the difference b/w X-linked agammaglobulinemia (Bruton’s) and Common variable immunodeficiency

A

Brutons - low B cell count and low Ig’s (all types)

Common variable immunodeficiency - NL B cell count, low Ig’s (all types)

299
Q

Clear vesicles over erythematous skin, just over the cheeks

A

HSV
“punched out” lesions w/ hemorrhagic crusting
if kiddo has atopy w/ superimposed HSV = eczema herpeticum

300
Q

Best way to prevent SIDS?

A

Avoidance of second had smoke
Avoid pacifiers in bed
sleep supine w/ firm bedding
don’t share the same bed

301
Q 
Macrosomia
Macroglossia
Umbilical hernia/omphacele
hemihyperplasis (ie R extremity larger than L)
Hypoglycemia
A

Beckwith-Wiedemann syndrome
Overgrowth disorder, prediosposed to neoplasms
11p15, alter insulin-like growth factor 2
Monitor closely for hypoglycemia
Get abd U/S and serum alpha fetoprotein q 3m from birth - 4y/o to asses for Wilms tumor or hepatoblastoma

302
Q

Most common risk factor for orbital cellulitis

A

Bacterial sinusitis

303
Q

Male teen has gynecomastia

A

Reassure
Pubertal gyneo is seen in 2/3 of boys during mid to late puberty
d/t transient increased estrogen from the testicles
unilateral or b/l or painful
Usually resolves in months - 2yrs

304
Q

Which beta blocker should be avoided in kids with long QT syndrome?

A

Sotalol

305
Q

New heart murmur after strep pharyngitis needs?

A

PCN prophylaxis

306
Q

Tx of lead poisoning must be based on?

A

The venous level of lead

307
Q

Systolic murmur best heard when lying down. Improves with standing

A

Innocent murmur, reassure

308
Q

Kid under 2 y/o tx for UTI also needs a?

A

Renal U/S to evaluate for anatomic abnormalities

309
Q

TX for OCD

A

SSRI + CBT

310
Q

Obese kid with limp

A

SCFE

311
Q

When should a drain clearner kid get an EGD?

A

Within 24 hours

312
Q

Anaphylaxis during blood transfusion

A

Selective IgA deficiency

Most common immune deficiency

313
Q

Why does putting knees to chest help in tetrology?

A

Increases systemic vascular resistance
Increases pulmonary blood flow
Help pt overcome RV obstruction

314
Q

Obese girl w/ pubic hair, no other signs of puberty

A

Premature adrenarche

Early activation of adrenal glands, related to obestiy

315
Q

Anterior R sided cervical mass x 1 day

A

Staph aureus, strep pyogenes

Most common causes of acute unilaterlal lymphadenitis

316
Q

Rare, but potentially serious complication of infectious mono?

A

Acute airway obstruction
Suspect in pts with throat tightness of difficulty swallowing
tx - corticosteroids

317
Q

Raised macular lesion w/ hair

A

Congenital melanocytic nevus

Presents in first few months of life

318
Q

Neonates (<2m) are at greatest risk of which complication of bronchiolitis

A

Apnea

319
Q

Why does viral illness increase risk of intussusception?

A

Hypertrophy of Peyer patches in terminal ileum serves as nidus for telescoping

320
Q 
Male 2-5
Diffuse bone pain
pallor petechiae
Rubbery LN
Elevated WBC
CXR with wide mediastinum
A

Acute lymphoblastic anemia
Most common cancer in kids
BM bx confirms dx if >25% lymphoblasts

321
Q

Newborn w/ bilious vomiting, no meconium

A

SBO
Either meconium ileus or Hirschprung’s
Get contrast enema to find the level of the blockage

322
Q

Tx for homocysttinuria

A

Vitamin B6, B12, Folate to lower homocysteine levels

323
Q

Inheritance patter of muscular dystrophies

A

myotonic - AD
Duchenne, Becker Xlinked
Duchene - more severe dz, Gower sign, younger kids
Becker - milder dz, older kids

324
Q

Describe duchenne muscular dystrophy

A

X linked deletion of dystrophin on p21
Onset 2-3
Progressive weakness, Gower sign, calf pseudohypertrophy
Comorbids: scoliosis, cardiomyopathy
Prognosis: wheelchair by adolescence, death 2-30 d/t heart failure

325
Q

Describe becker MD

A 
X linked deletion of dystrophin on p21
onset 5-15 y/o
Milder dz
Comorbid: Cardiomyopathy
Death by age 40-50 from HF
326
Q

Describe myotonic muscular dystrophy

A

AD, expansion of CTE trinucleotide repeat in DMPK gene on 19q13
Onset 12-30
Facial weakness, hand grip, myotonia, dysphagia
Comorbid: arrhythmia, cataract, balding, testicular atrophy/infertility
Death from repirator or heart failure, depends on age of onset

327
Q

Complications of mumps

A

Aseptic meningitis

Orchitis

328
Q

Breast development
Absent uterus and upper vagina
Minimal to absent pubic hair

A

Complete androgen insensitivity
X linked mutation of adrogen receptor
Cryptorchid testes

329
Q

Breast development
Absent or rudimentary uterus and upper vagina
NL ovaries
NL pubic hair

A

Mullerian agenesis
Hypoplastic or absent mullerian ductal system
Female, just don’t menstruate

330
Q

Hypotonia
Hyperpahgia
Obesity

A

Prader-Willi
Loss of paternal 15q11
Increased risk of OSA, DM, gastric rupture

331
Q

Anosmia

Delayed puberty

A

Kallman syndrome
Low FSH/LH, low GNRH
X-linked
hypogonadotropic hypogonadism
Absence of GnRH -> short w/ delayed puberty
Females - no boobs
Boys - eunich appearance, small external genitalia and absent pubic hair, voice deepening, libido

332
Q

Tx for tourette’s

A

Habit reversal training
Alpha-2 agonist (clonidine, guanfacine)
Antipsychotics (risperidone, second gens preferred)

333
Q

Infant w/
asymmetric gluteal/thigh/inguinal creases
Apparent leg length discrepancy

A

Developmental dysplasia of the hip
Get a U/S, after 4m/o bones are radioopaque and Xrays are preferred
RF: breech, female, FHx
Clunk on hip exam maneurvers (Barlow, Ortolani)
Tx - Pavlik harness

334
Q

Back pain w/ step off

New bed wetting

A

Spondylolisthesis

Forward slip of vertebrae in preadolescents

335
Q

Microangiopathic hemolytic anemia
Thrombocytopenia
AKI

A

Hemolytic uremic syndrome (HUS)

Also schistocytes

336
Q

Macroglossia
Umbilical hernia/omphalocele
Hypoglycemia

A

Beckwith-Wiedemann syndrome
Needs to be monitored for Wilms tumor or hepatoblastoma
Get an abd U/S

337
Q

Pubic hair
Breast development
Irregular cafe-au-lait macules
Recurrent long bone fx

A

McCune-Albright syndrome
Premature secretion of sex hormones w/o GnRH
Recurrent fx d/t polyostotic fibrous dysplasia
Mutation in GNAS gene -> overproduction of pituitaty hormones (LH/FSH)
can also have thyrotoxicosis (TSH), acromegaly (GH) and Cushing syndrome (ACTH)

338
Q

Situs inversus
Recurrent sinusitis
Bronchiectasis

A

Kartagener syndrome
Type of primary ciliary dyskinesia
Mutation in dynein
Dx - low nasal NO, Bronchoscopy, Genetic testing

339
Q

Healthy exclusively BF kiddo has painless bloody stool, eczema

A

Milk or soy protein induced colitis

Confirm dx if syx resolve after mom eliminates dairy and soy from her diet

340
Q

Postural neck deformity

A

Congenital torticollis

SCM m. mass, ipsilateral head tilt, and contralateral chin deviation

341
Q

Newborn is cyanotic but pinks up with crying. Cyanosis worse with feeding

A

Choanal atresia
Dx - failure to pass catheter from the nose to oropharynx
CT shows narrowing of pterygoid plate in posterior nasal cavity

342
Q

CHARGE syndrome

A 
Coloboma
Heart Defects
Atresia choanae
Retardation of growth/development
GU anomalies
Ear abn/deafness
343
Q

Dx and management of laryngomalacia

A

Dx - direct laryngoscopy showing collapse of the supraglottic structures during inspiration
Typically spontaneously resolves by 18 months

344
Q

Newborn with painless scrotal swelling that transilluminates on exam

A

Newborn hydrocele
Observe, most resolve by 1 y/o
Hydrocele = fluid collection w/in the tunica vaginalis (sac surrounding the testis)
A hernia containing bowel would not transilluminate

345
Q

Swimmers ear

A

Otitis externa
Pseudomonas
Loss of cerumen d/t swimming increases risk
Tx - fluoroquinolone drops

346
Q

Feeding intolerance
Abdominal distension
Bloody

A

Necrotizing enterocolitis

RF’s prematurity, hypotension, congenital heart disease

347
Q

Most common cause of sepsis in SCD

A

Strep pneumo

This is why SCD kids should receive PCN until at least 5 y/o

348
Q

Life threatening complication of hereditary angioedema

A

Laryngeal edema
Caused by C1 inhibitory defiency/destruction -> elevated levels of edema producing C2b, bradykinin
episodes typically follow infection, dental proceudre, trauma

349
Q

Healthy kid with severely microcytic anemai

RDW, Red blood cell count NL

A

Thalassemia trait
Caused by reduced production of globin chains
Target cells
NL serum iron studies

350
Q

Recurrent skin infections and pulmonary infections w/ Staph, serratia

A

Chronic granulomatous dz
defect in NADPH oxidase
Dx - abn dihyrohodamine testing

351
Q

If parents refuse to consent to a life saving treatment for their child you?

A

Can get a court order to mandate tx

352
Q

Exudative effusion into the chest

A

Chylothorax
Disruption of lymphatic flow w/in the throacic duct
Milky white fluid w/ triglycerides

353
Q

Atopic dermatitis kid should be tx w/ what for rash?

A

Triamcinolone

354
Q

Scrotal pain x hours

Unilateral absence of cremastic reflex

A

Testicular torsion

355
Q

Newborn has a growing dark macule on her leg. No hair

A

Congenital melanocytic nevus

Present at birth and can darken during following months

356
Q

Teen with dysmenorrhea, Tender normal sized uterus and no masses. LMP 7 days ago. What next?

A

NSAIDs

357
Q

CF pt 6 month hx of SOB. Hepatosplenomegaly and edema
Hyponatremia
Low albumin
Urine prot 1+
FVC 30%
CXR hyperinflamation, coarse nodular infiltrates b/l
Why does she have edema?

A

Cor pulmonale

358
Q

1 wk hx of fever, lethargy, joint pain
Today, petechiae
adenopathy in cervical chain, splenomegaly
Anemia, high high high WBC, thrombocytopenia

A

ALL

359
Q

Teen has reddish brown urine when he gets a cold
U/A shows nephritis
NL serum C3

A

IgA nephropathy

360
Q

Anaphylaxis tx w/ epineprhine, methylprednisone. Hemodynamically stable. Waht do you give next?

A

Fluids

361
Q

Kid with adrenal insufficiency s/t neisseria meningitis has b/l patchy infiltrates. Why?

A

Increased vascular permeability

362
Q

s/p tonsilectomy kid has seizures in PACU. He got 5% dextrose in water intraoperative. What is wrong?

A

Hyponatremia

363
Q

Cephaohematoma is caused by

A

blood under to the periostium of the skull bone (ie parietal)

364
Q

Kid swallows a foreign body and there is obstruction in the lung. Where is most likely

A

Partial obstruction of the R main stem bronchus

365
Q

Teen is hallucinating, sweating, and belligerent. Give him?

A

IM lorazepam

Beware of naloxone as that would be a rapid w/d

366
Q

Abdominal cramps and knee pain, intermittent x weeks
Cramps improved with BM
Multiple bloody stool daily
elevated leukocytes in the Knee

A

Ulcerative colitis

367
Q

Head trauma with lens chaped extracerebral density

A

epidural hematoma

368
Q

Head trauma w/ crescent shape

A

Subdural hematoma

369
Q

Kiddo with pulse > 200, QRS 0.05, no P wave. Does not improve w/ ice bags over the eyes

A

Give adenosine

370
Q

New born w/ afebrile vomtiting, jaundice, hepatomegaly
Tot bili 14
Direct bili 8
Urine + for reducing substances

A

Galactosemia (Galactose 1 phosphate uridyltransferase deficiency)

371
Q

4 w/o increasing jaundice x 1 week
exclusively BF
No anemia
Total bili 12, direct 8

A

Decreased biliary excretion

NOT decreased hepatic glucuronosyltransferase activity

372
Q

DKA kid w/ orthostatic hypotension

A

Give insuline and NS

373
Q

Frequent OM, sinusitis, small anterior LN

A

Antibody deficiency (d/t lack of B cells)

374
Q

2/6 crescendo-decrescendo systolic murmur with musical quality at LLSB w/ no radiation

A

Still’s murmur/innocent murmur

375
Q 
3 day hx of fever, cough, sputum
afebrile
PMH - respiratory problems since infancy
c/c on exam w/ hyperexpanded ches
Diffuse rhonchi/weezin
CXR - hyperinflation, atelectasis, chronic interstitial changes
A

Cystic fibrosis

376
Q

Healthy teen acute onset fever, malaise, pain in LLE, can’t walk
Febrile
No skin break, but swelling and point tenderness
Technetium 99m bone scan w/ uptake at metaphysis of tibia

A

Acute osteomyelitis

377
Q

Best way to prevent spreading rotavirus?

A

Good handwashing technique

378
Q

Worsening HA x 6 wks, b/l papilledema, R abducens palsy, gait ataxia

A

Medulloblastoma

379
Q

Hot day, sudden onset of HA, nausea, vomiting. Exam - aox3, diaphoretic febrile, tachy

A

Heat exhaustion

380
Q 
Fever, lymphadenopathy x 3 weeks
Weight loss, steatorrhea, hepatosplenomegaly
Febrile
Low WBC w/ high PMN
High IgG, IgM, NL IgA
A

HIV?

381
Q

Kid on maintnence chemo has patch beneath the scapula w/ 6-8 superimposed small clear fluid filled vesicles

A

Herpes zoster

382
Q

How do you tx a androgen insensitivity kiddo?

A

B/l gonadectomy after completion of puberty (after adult height is attained) to decrease chance of malignancy

383
Q

hematureia in SC trait is caused by?

A

Renal papillary necrosis

Mild episodes, resolve spontaneously

384
Q

Kiddo has febrile sezure after receiving dTap. Should he get that vax again?

A

Yes

Contras to dTap are Immediate anaphylaxis, unstable neuro disorders, encephalopathy w/in 1 week of administration

385
Q

1 yr milestones

A 
Weight should triple
Height increase by 50%
Learning to walk independently, but should be able to stand and walk w/ support
2-finger pincer grasp
mama dada + 1
1 step commands
386
Q

Anorexia kiddo in tx has arrhythmias and cardiopulmonary failure. Why?

A

Refeeding syndrome
Taking in carbs -> increased insulin -> promotes cellular uptake of Phos, K+, and Mag causing intervascular deficiencies of the electrolytes -> arrhythmias

387
Q

Kiddo is short despite having tall parents
Little behind to start puberty
Bone age is less than chronologic age

A

Constitutional growth delay

See again in 6 months

388
Q

Dihydrorhodamine or nitroblue tetrazolium testing

A

Chronic granulomatous dz

Impaired phagocytic killing

389
Q

Hx of PNA, sinus stuff, vit d deficiency
B/l nasal polyps
Multiple bruises

A

Cystic fibrosis

Primary ciliary dyskinesia looks very similar but they have situs inversus and do not have pancreatic insufficiency

390
Q

new onset hearing loss

Chronic ear drainage despite abx

A

Cholesteatomas

Granulation tissue, skin debris may be seen on otoscopy

391
Q

Tx for ring worm

A 
tinea corporis
Top antifungal (clotrimazole, terbinafine)
392
Q

Biolious vomiting
Double bubble on Xray
Associated w/ Down’s

A

Duodenal atresia

393
Q

cytoplasmic aggregates of periodic acid Schiff + material

A

ALL

NO auer rods (AML)

394
Q

Dx for malrotation/volvulous

A

Upper GI series

395
Q

Flat facies and limb deformieies d/t IUGR s/t oligohydramnios

A

Potter sequence

396
Q

New born w/ barrel shaped chest, concave abdomen

A

Congenital diaphragmatic hernia

Needs intubation

397
Q

How do you keep a PDA open?

A

Protaglandin E1

398
Q 
Malnourished pt
Angular cheilitis
Stomatitis
Glossitis
Anemia
Seborrheic dermatitis
A 
Vitamin B2 (riboflaving) deficiency
meat, eggs, dairy, veggies
399
Q

Tx for hair loss d/t tinea capitis?

A

po griseofulvin or terbinafine

400
Q

Fail to pass meconium in first 48 hours

Down’s baby

A

Hirschprung dz

Failure of migration of neural crest cells

401
Q

Neonate w/ irritability, high pitched cry, sneezing, poor feeding

A

Neonatal abstinance syndrome

w/d from opiates

402
Q

Workup for primary amenorrhea, uterus present

A

Get a FSH if there is no boobs
If low - pituitary MRI
If hi - Karyotype

403
Q 
Nurologic syx (ataxia)
skeletal deformities (scoliosis, feet deformities)
Cardiac dz ( hypertrophic cardiomyopathy)
A

Friedreich ataxia
ar
COD - cardio or respiratory

404
Q

Microcytic anema w/ elevated RCDW

A

Iron deficiency

405
Q

Microcyt anemia w/ NL RCDW

A

Thalassemia

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