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Pediatrics > UWorld Tidbits > Flashcards

UWorld Tidbits Flashcards

1
Q

6 yo, not previously varicella vaccinated, boy: visited cousin w/ chicken pox 1 week ago

Next best step?

A

Tell parents to look out for rash w/in next 2 weeks

  • varicella vaccine only beneficial if administered w/in 3-5 days post-exposure
  • VZIG (prophylactic immunoglobulin) only helps w/in 72 (max 96 hrs) (3-4 days)
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2
Q

9 yo w/ sudden onset writing and jerky movements

  • mild sore and fever last week
  • pericardial friction rub and subcutaneous nodules over the hands

(a) Diagnosis
(b) Etiology
(c) Tx

A

(a) Acute rheumatic fever five major criteria
1. polyarthritis
2. carditis
3. chorea
4. subcutaneous nodules
5. erythema marginatum
then often have: fever and arthralgia

(b) Strep A pharyngitis
(c) Tx = PenG

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3
Q

Scrotal swelling, hematuria, abdominal pain

-what to watch out for next

A

HSP: palpable purpura + scrotal swelling + hematuria + abdominal pain

-Children w/ HSP are susceptible to intussusception

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4
Q

12 yo M w/ back pain for several months, bed wetting, and decreased sensation to perianal area
-palpable ‘step-off’ at the lumbosacral area

A

Spondylolisthesis = forward slip of a vertebrae (most commonly L5 on S1) that causes chronic back pain and when severe neurologic deficits and palpable step off

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5
Q

16 yo Sick cell pt presents w/ DOE, fatigue, generalized weakness

A

Folic acid deficiency

-such high turnover => need more folate

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6
Q

Symptoms of niacin deficiency

A

Niacin (vit B3) deficiency => pellagra
-diarrhea, dermatitis, dementia, glossitis

-dermatitis in sun exposed areas (resembles sunburn) is bilateral and symmetric

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7
Q

Newborn that sounds like a cat when he cries

  • moon facies
  • microcephaly
  • high arched palate
A

Cri-du-Chat syndrome = deletion of 5p

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8
Q

What vitamin shot do babies receive at birth and why?

A

Vitamin K

B/c human’s only get vit K from food (not a lot of vit K in breast milk) and gut flora (not yet developed in children)

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9
Q

Workup for leukocoria

A

Leukocoria (white pupillary reflex) is retinoblastoma until proven otherwise!!!!

=> refer to opthalmologist

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10
Q

Presentation of IgA deficiency

(a) Risk of transfusion in ppl w/ IgA deficiency

A

IgA deficiency is most often asymptomatic, but may present w/ recurrent sinopulmonary (strep and hemophilus) and GI (giardia) infxns as a consequence of IgA’s role in mucosal barrier protection

(a) Pts may form antibodies to IgA- predisposing them to anaphylactic transfusion rxns

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11
Q

How does midgut volvulus present?

A

Midgut volvulus associated w/ malrotation of the gut, can be complicated by perforation and peritonitis

-usually presents in child

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12
Q

Describe anemia of prematurity

A

Most common anemia in premature and low birth weight infants

  • diminished RBC production, shortened RBC lifespan, blood loss
  • see normocytic normochromic anemia, low retic count
  • normal WBC, platelets, and bilirubin
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13
Q

Differentiate traction apophysitis and patellar tendonitis

A

Traction apophysitis- more common in peds population (since can be due to growth patterns)
-pain reproduced by knee extending against resistance

Paterllar tendonitis- common as you get older b/c it’s from overuse
-pts have point tenderness at inferior pole of the patella

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14
Q

7 day old neonate w/ 2 days of jaundice and poor feeding

Next step

A

Blood cultures and LP = work up for meningitis

-jaundice in first 3-7 days can indicate bacterial sepsis or UTI in neonates

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15
Q

14 yo AA boy w/ limp and r. hip pain

-HCT 22%

A

Osteonecrosis = avascular necrosis

-present in up to 50% of pts homozygous for sickle cell gene

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16
Q

Risk factor for hip subluxation

A

LGA

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17
Q

13 yo w/ progressive muscle weakness for 2 mo

  • temporal wasting, thin cheeks, emaciated extremities, thenar and hypothenar atrophy, upper lip in shape of inverted V
  • delayed relaxation

(a) Diagnosis
(b) Delayed relaxation?
(c) Mode of inheritance

A

(a) Myotonic muscular dystrophy
(b) Myotonia = delayed relaxation
- classic example = can’t release hand after handshake
(c) Autosomal dominant

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18
Q

Describe cyclic vomiting

(a) Associated finding

A

Cyclic vomiting = recurrent episodes of self-limited nausea and vomiting w/ no other factors indicating something else

(a) Often associated w/ history of migraines

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19
Q

Gait disturbance, pes cavus, ataxia, absent ankle jerks

A

Friederich’s ataxia

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20
Q

Most common type of strabismus

(a) First step of tx

A

50% of cross eye cases are esodeviation = medial deviation of the eye

(a) Cover the normal eye to force the affected eye to correct itself => allows proper visual maturation

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21
Q

9 mo old AA boy w/ swelling of hands and feet for 2 days

Mechanism?

A

Hand-food syndrome = dactylitis = earliest manifestation of vaso-occlusion in SC

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22
Q

Why are sickle cell pts often on prophylactic abx?

A

Due to splenic infarction they’re essentially asplenic by 2-3 yoa => very susceptible to infection by capsulated organisms (strep pneumo and H. influenza) => prophylactic abx

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23
Q

Tongue fibrilliations w/ hypotonia and normal social/language skills

A

Infantile SMA (spinal muscular atrophy) = Werdnig-Hoffman’s

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24
Q

1 yo boy w/ bruising and blood in stool

  • multiple otitis medias + 2 pneumonias
  • dry, scaly patches on cheeks and lower extremities
A

Wiskott-Aldrich Syndrome: X linked hereditary d/o
Presents in boys during early childhood w/ a tendency to bleed due to thrombocytopenia; recurrent bacterial viral and fungal infections due to immune deficiency, and eczema

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25
Q

9 yo overdosed on gmas meds, presents w/ seizures, hypotension, prolonged QRS complexes

(a) Tx

A

Hypotension, seizures, and prolonged QRSs indicative of tricyclic antidepressant intoxication

(a) Sodium bicarbonate to reverse acidosis

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26
Q

1 week old w/ noisy breathing but otherwise normal respiratory exam

Advice for parents?

A

Most common cause of chronic inspiratory noise in infants = laryngomalacia = congenital flaccid larynx

  • bronco finding = epiglottis rolling from side to side
  • advice for parents: it’ll get better w/ age, never feed child while lying down and keep child upright for 30 mins after feeding
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27
Q

Cystic calcified parasellar lesion

A

Calcified lesion above the sella = Craniopharyngioma = cancer from pituitary gland tissue

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28
Q

2 day old infant who is irritable, feeding poorly, sneezing, w/ high-pitched cry

A

NAS = neonatal abstinence syndrome

-newborn in withdrawal from mother’s opiates

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29
Q

Splenic sequestration crisis

(a) Physiology
(b) Retic count
(c) Presentation

A

Splenic sequestration crisis

(a) Due to vaso-occlusion and pooling of RBC in the spleen
(b) High retic count- bone marrow compensating
(c) Presents w/ rapid enlarging spleen, possibly in hypotensive shock

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30
Q

Conjugated hyperbilirubinemia, light colored stool, hepatomegaly

A

Biliary atresia

-light colored stool + hepatomegaly indicates impaired hepatic excretion of bile

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31
Q

Severe pain

  • poikilothermia
  • paresthesias

(a) What is it?
(b) What symptoms come next?

A

Poikilothermia = inability to maintain temp independent of ambient temperature

(a) Compartment syndrome
(b) Late findings: pulselessless and paralysis

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32
Q

Use of topical steroids for conjunctivitis

A

Topical steroids can be used in allergic or viral (typically adenovirus) conjunctivitis

-no use for bacterial conjunctivitis

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33
Q

Treatment for ITP

A

Depends on symptoms

If only have skin manifestations (just purpura, no bleeding)- watch and wait: will usually self resolve within 6 mo

If bleeding: give IVIG or glucocorticoids

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34
Q

Differentiate lab findings of Common variable immunodeficiency and Hyper IgM Syndrome

A

Common variable immunodeficiency: all Ig lines are low, normal T and B cell numbers

Hyper IgM: IgM high, IgG and IgA are low, T and B cells diminished

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35
Q

Infant w/ left calf atrophy, left calcaneum and talus in equinus and varus positions

(a) Treatment

A

No idea what any of this means but common foot deformity in newborns = Clubfoot

(a) Stretching, manipulation, and serial casting
- only resort to surgical if those fail (which usually they don’t)
- tx should be started immediately

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36
Q

Advantage of human breast milk over formula in addition to antibodies

A

Human milk’s major source of protein is whey, more easily digested than casein (cow’s milk) => helps to improve gastric emptying

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37
Q

Compare and contrast ITP and Wiskott-Aldrich syndrome

A

Both can present w/ thrombocytopenia- easy bruising and petechiae

But then in addition Wiskott-Aldrich has associated skin findings (eczema) and recurrent infxns (due to immune deficiency)

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38
Q

Bluish-white spots on buccal mucosa

A

Bluish-white spots on buccal mucosa = Koplik spots of Rubeola (Measles)

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39
Q

Most common fracture in the pediatric population

(a) Complication
(b) Physical exam finding

A

Most common fracture of the pediatric population = supracondylar fracture
ex: fall on outstretched arm

(a) Entrapement of the brachial artery =>
(b) Loss of the radial pulse

After fracture is reduced, need to reassess and see if radial pulse is intact

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40
Q

Locate the lesion:

12 yo unable to walk

  • couldn’t move legs in the morning
  • thigh pain in the afternoon
  • absent DTRs
  • episode of febrile diarrhea 10 days ago
A

Peripheral nerves

Guillain Barre Syndrome: symmetrical ascending paralysis, decreased DTRs- following viral infection

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41
Q

Hypsarrhythmia on EEG

A

Pathognomonic finding for Tuberous sclerosis

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42
Q

Positive osmotic fragility test

A

Hereditary spherocytosis

-jaundice at birth

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43
Q

Give the components of the Apgar scoring

A

A for appearance
0- cyanotic
1- body pink, acrocyanosis
2- body and extremities pink

P for pulse
0- no HR activity
1- HR under 100
2- HR over 100

G for grimace
0- no response to stimulation
1- grimace
2- cough or cry

Activity/tone
0-limp
1- some flexion of extremities
2- active flexion of extremities

Respirations
0- completely absence
1- slow and irregular
2- good respiratory effort

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44
Q

Differentiate the lymphadenopathy seen in EBV vs. Staph aureus infection

A

EBV lymphadenopathy- subacute, bilateral, and w/ systemic symptoms

Staph aureus = most common bacteria cause of lymphoadenopathy- acute unilateral

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45
Q

Differentiate atonic seizures from absence seizures

A

Atonic seizures- sudden loss of postural muscle tone lasting 1-2 seconds

Absence seizure = generalized seizure of sudden and brief lapses of consciousness w/o loss of postural control

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46
Q

Which congenital abnormalities are the following heart defects associated with?

(a) ASD
(b) VSD
(c) Supravalvular aortic stenosis
(d) Conotruncal abnormality
(e) Congenital heart block

A

Congenital abnormalities and associated heart defects

(a) ASD- trisomy 21 (Down’s)
- along w/ endocardial cushion defect
(b) VSD- Trisomy 18 (Edward’s)
(c) Supravalvular aortic stenosis- William’s (microdeletion chr7)
(d) Conotruncal abnormality- DiGeorge syndrome (microdeletion chr22)
(e) Congenital heart block- Neonatal lupus

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47
Q

Iron deficiency anemia

(a) RDW
(b) MCV
(c) TIBC
(d) retic count

A

Iron deficiency anemia

a) RDW high ( > normal 17
(b) MCV- low erythropoiesis
(c) TIBC high
(d) Retic count low

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48
Q

Location of medulloblastoma

A

Cerebellar vermis

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49
Q

2 yo w/ recurrent sinusitis and CXR revealing heart on the right side

A

Kartagener’s syndrome = auto recessive d/o due to dysmotile cilia

Triad

  1. situs inversus
  2. recurrent sinusitis
  3. bronchiectasis
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50
Q

What to do when the next DTaP is up:

first DTaP caused 2 minute seizure

A

Administer diptheria and tetanus toxoids, avoid pertussis component
-adverse rxns to DTaP are usually due to the pertussis component

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51
Q

Indication for mechanical ventilation in an asthmatic

A

Severe exacerbation- refractory severe wheezing

  • no response to nebulizers, IV steroids, or supplemental O2
  • no airway entry bilaterally = ‘silent chest’
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52
Q

Sickle cell pt w/ fatigue and pallor, what is the most likely type of anemia?

A

Hemolytic anemia

-SCD pts are chronically hemolyzing the sickled RBCs

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53
Q

3 yo M w/ gout

A

Defect in purine metabolism, Lesch Nyan syndrome = X linked recessive
-deficiency in HPRT enzyme

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54
Q

Traction apophysitis

A

Traction apophysitis = Osgood-Schlatters

  • adolescent males
  • pain reproduced by knee extending against resistance
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55
Q

Features of Lesch-Nyan syndrome

A

Lesch-Nyan = X-linked recessive HPRT (purine metabolism) deficiency => build up of purine/uric acid

-presents at 6 mo w/ hypotonia and vomiting, then worsens w/ neurological findings (MR), self-mutilation, and gouty arthritis/tophi

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56
Q

Drug of choice for

(a) Infantile spasms
(b) GTCs
(c) Absence seizures
(d) Status epilepticus

A

Drug of choice for

(a) Infantile spasms = intramuscular ACTH
(b) GTCs: carbamazepine, phenobarbitol
(c) Absence seizures: ethosuximide
(d) Status epilepticus = phenytoin

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57
Q

What is osteogenesis imperfecta?

A

Autosomal dominant d/o caused by defect in type I collagen

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58
Q

Cyclic fevers, chills, headache every 2-3 days

A

Cyclic/paroxysmal flu-like symtpoms/fever = Malaria!!

Febrile paroxysms parallel the waves of RBC invasion when parasites are released by the liver into the bloodstream

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59
Q

Pt w/ malar rash and arthralgia
-(+) ANA and (+) RPR

(a) Next step

A

(+) ANA + malar rash and arthralgia = SLE
-lupus is associated w/ false positive results for RPR and VDRL tests (both syphilis tests)

(a) Next step = do anti-Smith antibody test to confirm SLE

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60
Q

Newborn w/ HSM, jaundice, anemia, rhinorrhea, and cutaneous lesions on palms and soles

A

Congenital syphilis

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61
Q

Name 2 things that can increased risk for RDS

A
  1. prematurity

2. maternal diabetes- delays maturation of surfactant production

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62
Q

Rule of 2s for Meckel’s diverticulum

A
  • M:F 2:1
  • occurs in 2% of the population
  • about 2% have lifetime complications
  • happens 2 ft from the ileocecal valve
  • 2 in long
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63
Q

List the 4 main features of Wiskott-Aldrich Syndrome

A

Wiskott-Aldrich Syndrome = X linked hereditary d/o

  1. immune deficiency => presents w/ recurrent bacterial viral and fungal infxns
  2. tendency to bleed due to thrombocytopenia (due to impaired platelet production) => bruising, blood in stool
  3. eczema
  4. increased risk of autoimmune d/o and malignancy
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64
Q

What is a vascular ring?

A

Congenital defect in which the aortic arch and/or its branches completely encircles the trachea and esophagus => present w/ breathing and/or swallowing difficulties

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65
Q

Pt w/ pharyngitis, moderate fever, dysphagia, neck stiffness, muffled voice, posterior pharyngeal bulge

A

Retropharygneal abscess

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66
Q

2 day old cyanotic M

  • holosystolic murmur at LLSB, single S2
  • CXR: decreased pulmonary vascular markings and normal-sized heart
  • EKG: left axis deviation
A

Tricuspid atresia

Left axis deviation indicating LVH b/c LV is dealing w/ both systemic and pulmonary returns

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67
Q

Differentiate the presentation of the following etiologies of back pain in children

(a) ankylosing spondylitis
(b) spondylolisthesis

A

Back pain in children- always is concerning!

(a) ankylosing spondylitis = type of arthritis affecting the spine and surrounding tissues => gradual onset
(b) spondylolisthesis- lumbosacral backpain w/ neurologic symptoms: tingling and numbness, can present more acutely

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68
Q

Differentiate CSF white cell count values for viral vs. bacterial meningitis

A

Viral meningitis- lymphocyte predominance

Bacterial- neutrophil predominance

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69
Q

18 yo w/ SCD presents w/ sudden onset of dyspnea, weakness, fatigue, and pallor.

  • denies fever or CP
  • clear lungs, normal CXR
A

Aplastic crisis = transient onset of erythropoeisis, most commonly associated w/ infection (most commonly parvovirus B19)

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70
Q

Buzzword: infected umbilical stump

A

Infected umbilical stump- buzzword for tetany

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71
Q

10 yo boy w/ abdominal pain and bloody diarrhea

  • jaundice and pale
  • abdominal tenderness and 2+ pedal edema

(a) Etiology

A

HUS = hemolytic uremic syndrome

  • bloody diarrhea
  • red cell hemolysis => jaundice
  • thrombocytopenia

(a) E. coli

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72
Q

3 P’s of McCune Albright syndrome

A
  1. precocious puberty- accounts for 5% of precocious puberty in females
  2. Pigmentation: cafe au lait spots or coast of Maine hyperpigmentation
  3. polyostotic fibrous dysplasia
    - bone abnormality => common fractures
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73
Q

Symptoms caused by mutation in

(a) Fibrilin-1 gene
(b) Defect in collagen structure
(c) Hexosaminidase A
(d) glucocerebrosidase
(e) sphingomyelinase
(f) frataxin

A

(a) Mutation in fibrillin-1 gene =Marfan’s
(b) Defect in collagen structure = Ehler-Danlos syndrome
(c) Deficiency in hexosaminidase = Tay-Sachs
(d) Mutated glucocerebrosidase = Gaucher’s disease
(e) Mutated sphingomyelinase = Niemann-Pick’s disease
(f) Frataxin = Friedreich’s ataxia

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74
Q

Symptoms of cyanocobalmin deficiency

A

Cyanocobalamin (vit B12) causes macrocytic anemia and peripheral anemia

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75
Q

Pneumatosis intestinalis

A

= gas in the bowel wall, ominous finding for ischemia

Abdominal Xray demonstrating pneumatosis intestinalis = NEC (necrotizing enterocolitis) in neonates, especially if preterm

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76
Q

CNS tumor associated w/ premature puberty in boys

A

Pinealoma- causes interruption of hypothalamic inhibiting pathways => endocrine syndrome

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77
Q

Features of Beckwith-Wiedemann syndrome

(a) Complications

A

Beckwith-Widemann = overgrowth d/o present at birth

Macrosomia (LGA), macroglossia, visceromegaly (liver and kidneys), omphalocele, hypoglycemia, and hyperinsulinemia

(a) Increased risk of childhood cancer- Wilm’s (nephroblastoma), pancreatoblastoma, and hepatoblastoma

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78
Q

What is dactylitis?

A

Painful swelling of hands and feet

-complication of SCD

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79
Q

Differentiate number of heart sounds in children vs. adults

A

In children and young adults S3 is a normal finding on auscultation

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80
Q

Hypersegmented neutrophils are seen in what diseases?

A

Hypersegmented neutrophils are seen in folate and vitamin B12 deficiency

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81
Q

7 yo w/ sudden growth acceleration, severe cystic acne, coarse pubic and axillary hair

A

CAH = congenital adrenal hyperplasia
-signs of severe androgen excess

-important to differentiate precious puberty causes: early HPA axis activation (less dramatic) and CAH

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82
Q

Impaired adaptation to darkness, photophobia, dry scaly skin, dry conjunctiva

A

Vitamin A deficiency

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83
Q

Most common cause of acute gastroenteritis in children

A

Rotavirus

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84
Q

After what age do you surgically repair umbilical hernias

A

Surgically repair umbilical hernias if not gone by 3-4

  • most will disappear spontaneously by 1 yoa
  • also remove if enlarges after 1-2 yoa, causes symptoms, becomes strangulated, or exceeds 2 cm diameter
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85
Q

Tx for ITP

A

Immune thrombocytopenia tx depends on symptoms of bleeding and platelet count (if get CBC)

Pts w/ platelets over 30k usually are asymptomatic (not bleeding, just petechiae) => observe and wait

Pts w/ platelets under 30k or actively bleeding/symptomatic- tx w/ corticosteroids

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86
Q

3 mo old constipated w/ poor feeding

-then ptosis, dilated pupils, diminished DTRs and decreased muscle tone

A

Infantile botulism

  • presents initially w/ constipation and poor feeding
  • then later comes cranial nerve abnormalities, diminished DTRs, hypotonia
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87
Q

Features of congenital HIV

A

Congenital HIV => persistent oral thrush, lymphadenopathy, hepatosplenomaegaly
-later: intractible diarrhea, failure to thrive

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88
Q

Elevated 17-alpha-hydroxyprogesterone in serum

A

Indicates CAH = congenital adrenal hyperplasia

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89
Q

Tip off for orbital vs. preseptal cellulitis

A

Tip offs for orbital cellulitis: decreased eye movements, decreased visual acuity, proptosis, double vision

Preseptal cellulitis: eyelid, swelling, discoloration, tenderness, erythema

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90
Q

Hypotonia, macroglossia, umbilical hernia, macrosomia

A

Congenital hypothyroidism

  • large head and tongue
  • umbilical hernia (not omphalocele as seen in Beckwith-Wiedemann syndrome)
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91
Q

Which type of prematurity is concerning in a female and why?

Differentiate thelarche, adrenarche, pubarche

A

Adrenarche = onset of androgen-dependent body changes such as axillary hair, body odor, and acne

Thelarche = onset of breast development

Pubarche = onset of groin hair growth

Premature adrenarche and thelarche are unconcerning, premature pubarche is concerning b/c 50% of cases may be due to CNS disorder

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92
Q

Cardiac abnormality seen in Marfan syndrome

A

Aortic root dilation

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93
Q

Most common causes of viral meningitis

A

Non-polio enteroviruses: echoviruses and coxsackieviruses

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94
Q

Until what age is bedwetting medically normal?

A

Anytime before age 5 bedwetting doesn’t indicate any medical workup

=> reassure parents

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95
Q

Why do SGA infants have an increased risk of polycythemia

A

Polycythemia (high Hgb) b/c hypoxia stimulates EPO secretion => increased RBC production

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96
Q

Most common cause of neonatal sepsis

A

GBS

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97
Q

Common bony complication of sickle cell

A

Osteonecrosis (avascular necrosis of the bone) due to vaso-occlusion of the bone

  • significant joint pain
  • most frequently humerus and femur
98
Q

Most common congenital anomaly of the GI tract

(a) Presentation

A

Meckel’s diverticulum

(a) Often asymptomatic, then sometimes presents in children w/ painless rectal bleeding

99
Q

CXR findings of

(a) Truncus arteriosus
(b) Ebstein’s anomaly
(c) Tricuspid atresia

A

CXR findings

(a) Truncus arteriosus- cardiomegaly
(b) Ebstein’s anomaly- hugeeeee heart w/ enlarged RA
(c) Tricuspid atresia- normal sized heart

100
Q

Organism:

Meningitis + petechiae

A

Meningococcus

-75% of pts w/ meningococcal meningitis present w/ petechial rash

101
Q

A history of intussusception contraindicates which vaccine?

A

Rotavirus- b/c rotavirus slight increases the risk of intussuception => if you have a history you can’t take the vaccine

102
Q

2 yo w/ drug cough, fever, runny nose w/ wheezing and tachycardia
+ for respiratory syncytial virus

Slight risk of developing what?

A

Bronchiolitis = 1st episode of wheezing episode associated w/ URI (RSV)

-increased risk after RSV infection of asthma

103
Q

FSH/LH levels in

(a) Central precocious puberty
(b) Peripheral precocious puberty

-Give an example of each

A

FSH/LH levels

(a) Central precocious puberty = early activation of the HPA axis
- have high FSH and LH

(b) Peripheral precocious puberty = CAH
- have low FSH and LH
- high testosterone gets peripherally converted into estrogen, estrogen has negative feedback to decrease FSH/LH secretion

104
Q

Presentation of Diamond-Blackfan anemia

A

Pallor w/in first few months of life
-often congenital abnormalities (ex: webbed neck, cleft lip, shielded chest): triphalangeal thumbs

Diamond-Blackfan anemia = congenital red cell aplasia

105
Q

Findings of compartment syndrome

(a) early
(b) late

A

Compartment syndrome: extreme pain, poikilothermia (inability to maintain internal temperature independent of ambient temp), paresthesias

(b) Late findings: pulselessness and paralysis

106
Q

Prophylaxis of the following in SCD pts

(a) Aplastic crisis
(b) Pain crisis

A

Prophylaxis in SCD

(a) Aplastic crisis- folic acid supplementation
(b) Pain crisis- hydroxyurea to stimulate HbF production

107
Q

What does axis deviation indicate on EKG?

A

Axis deviation indicates ventricular hypertrophy

Ex: Left-axis deviation indicates LVH (such as seen in tricupsid atresia)

108
Q

2 yo boy in acute respiratory distress

  • afebrile, Sa02 84%
  • CXR: hyperinflation of right lung, mediastinal left shift, severely hypoinflated left lung

Tx

A

Foreign body aspiration

Direct laryngoscopy (for visualization) and rigid bronchoscopy (foreign object retrieval)

109
Q

1 yo presents w/ nuchal rigidity and positive Brudzinsky

  • petechiae and purpura
  • dies

(a) Cause
(b) Mechanism

A

Meningitis + petechiae = meningococcal meningitis

(a) In fulminant meningococcemia look out for Waterhouse-Friederichsen syndrome
(b) Adrenel gland hemorrhage causing sudden vasomotor collapse and skin rash

110
Q

Marfan’s syndrome + thromboembolic events (ex: stroke)

A

Homocystinuria- cystathione synthase deficiency

111
Q

Differentiate the growth chart of hypothyroidism and constitutional growth delay

A

Hypothyroidism- normal childhood growth w/ abrupt falling off the growth curve when the condition develops

Constitutional growth delay- around 3 years of age child remains a normal growth velocity and follows the growth curve at the 5th-10th percentile (so never on normal growth curve) Puberty and adolescent growth spurt are delayed but do eventually occur and child reaches adult height

112
Q

Differentiate chlamydial and gonococcal conjuncitivities

(a) Age
(b) Presentation
(c) Treatment

A

Conjunctivitis

Chlamydial

(a) 5-14 dol
(b) Presents w/ mucopurulent (m in chlamydia) discharge
(c) Tx- ORAL erythromycin (not drops) to reduce risk of chlamydial pneumonia

Gonococcal

(a) 2-5 dol
(b) Purulent discharge
(c) Tx for Ceftriaxone + azithromycin

113
Q

Communicating vs. noncommunicating hydrocephalus signs on CT

A

CT findings:

Communicating hydrocephalus = blocked absorption of CSF thru arachnoid villi => see dilation of the entire ventricular system and enlargement of the subarachnoid space

Noncommunicating/obstructive hydrocephalus = blockage that prevents flow to the subarachnoid space

  • would see only a specific part of the ventricular system dilated
  • wouldn’t have dilated subarachnoid space
114
Q

First step in management when see male infant w/ scaphoid abdomen

A

Scaphoid abdomen = buzzword for diaphragmatic hernia

1st step = place orogastric tube to

  1. determine position of the stomach
  2. provide continuous suction to prevent bowel distention and lung compression
115
Q

Most common cause of hemorrhage

(a) subdural
(b) subarachnoid

A

Types of hemorrhage

(a) Trauma = most common cause of subdural hemorrhage in kids
(b) AVM (arteriovenous malformation) = most common cause of subarachnoid hemorrhage in kids

116
Q

Chronic middle ear infections w/ drainage after multiple abx courses

A

Cholesteatoma = not a tumor, but destructive and expansive growth of epithelium in the middle ear that presents w/ either drainage and/or hearing loss

-often comes after chronic ear infections despite appropriate abx tx

117
Q

Classic triad of CRS (congenital rubella syndrome)

A
  1. sensorineural deafness
  2. cardiac malformations
    - ex: PDA (machine-like murmur), VSD
  3. cataracts
    - white eye reflex
118
Q

14 day old w/ poor suckling and fatigue

-rigidity, spasms, opithotonus

A

Opisthotonus = severe hyperextension and spasticity: bridge/arch position

Neonatal tetany!!

Mortality very high due to apnea (in first week of life) and septicemia (in second week of life) due to umbilical stump infection

119
Q

How does prematurity affect vaccination schedule?

A

Doesn’t- vaccines given by chronological, not gestational age
Prematurity doesn’t markedly change responses to vaccines

120
Q

Cause of edema in Turner’s pts

A

Lymphedema due to abnormal developmental of the lymphatic system

121
Q

Differentiate diarrhea from shigella vs. rotavirus

A

Shigella => blood diarrhea, food born pathogen

Rotavirus- not blood diarrhea

122
Q

Cell of origin for

(a) Wilm’s tumor
(b) Neuroblastoma
(c) Lymphocytic leukemia

A

Cell of origin

(a) Wilm’s tumor = metanephros
(b) Neuroblastoma = neural crest cells
(c) Lymphocytic leukemia = lymphoid stem cell

123
Q

Most common cause of CP

A

Cerebral anoxia at birth

124
Q

Management of breastfeeding jaundice

A

Increase feedings to 15-20 minutes every 2 hours

-basically want to increased the amount of breast milk baby is getting to quicken transit time and decrease dehydration

125
Q

CGG trinucleotide repeats

(a) Disorder
(b) Physical features
(c) Mental features

A

CGG trinucleotide repeats causing mutation in FMR1 gene

(a) Fragile X syndrome
(b) Features: large low set ears, large testicles (macroorchidism), prominent jaw
(c) MR, poor attention span, autism common

126
Q

Most common cause of nephrotic syndrome in kids

(a) Kidney biopsy results

A

Minimal change disease accounts for 80% of nephrotic syndrome in pts under 16

(a) In MCD the kidney biopsy is normal, don’t see anything unless do electron microscopy (foot process effacement)

127
Q

Classic presentation of Friederich’s ataxia

A

Mid-teens (ex: 15) w/ ataxia and dysarthria (neurologic symptoms) due to degeneration of the spinal tracts
-skeletal abnormalities such as hammer toe/scoliosis

128
Q

Age of child:
200 words,
says “me do it” when helped onto table

A

200 words, correct use of prepositions, combining words into short sentences = 24 mo

129
Q

Age of onset and clinical presentation of severe Celiac disease

A

Typically around 12-15 mo when wheat/barley (gluten containing foods) are introduced into the diet

Presents w/ non-bloody diarrhea, FTT, erythamtous vesicles (dermatitis herpetiforms)

130
Q

Post-strep GN

(a) Presentation
(b) Abnormalities detected on labs

A

Post-strep GN

(a) Presents w/ hypertension and edema
(b) Lab abnormalities
- high ASO titers
- low C3 and C50 w/ high C4: indicating activation of alternate complement pathway
- urinalysis: hematuria, proteinuria

131
Q

Tx for

(a) Clavicular fracture
(b) Klumpke’s paralysis

A

Tx for

(a) Clavicular fracture = reassurance, self-resolves
(b) Klumpke’s paralysis = nerve grafting

132
Q

Kartagener’s sydnrome

(a) Mode of inheritance
(b) Physiology
(c) Triad of findings

A

Kartagener’s syndrome

(a) Autosomal recessive
(b) Dysmotile cilia
(c) Triad
1. situs inversus
2. bronchiectasis
3. recurrent sinusitis

133
Q

Differentiate presentation of stranger anxiety vs. separation anxiety of a kid crying at school

A

Age

-stranger anxiety comes earlier

134
Q

Nephrotic vs. nephritic syndrome UA

A

Nephrotic- proteinuria

Nephritic syndrome- hematuria

135
Q

Healthy 3 yo boy for help w/ potty training. boy refuses to use the toilet.
Next best step

A

2-4 yo = range for potty training, and girls before boys

  • he may just not have the ability to feel the need to urinate/defecate => need to wait until certain milestones are reached
  • for reluctant children: best step is to stop training attempts for several months (not to start positive reward system)
136
Q

Which fluids should be given as a bolus to a 9 mo in hypernatremic dehydration?

A

Bolus ALAWYS isotonic (NS or LR) = 0.9% saline

  • wouldn’t give .45% saline (despite hypernatremia) b/c dont want to quickly push water into cells too quickly
  • hypernatremia always has to be corrected very slowly/gradually
137
Q

Describe the features of syndrome caused by TORCH infections

A

TORCH infection => syndrome characterized by

  • microcephaly
  • hepatosplenomegaly
  • deafness
  • chorioretinitis
  • thrombocytopenia
138
Q

Which cell types are

a) CD 19 (+
(b) CD 3 (+)

A

B cells are CD 19 (+)

T cells are Cd3 (+)

139
Q

Distinguish presentation of osteomyelitis and avascular necrosis in SCD

A

Osteomyelitis- presents acutely and w/ fever

Avascular necrosis (osteonecrosis) presents gradually and pts are afebrile
-most commonly presents as gradual hip pain
140
Q

Symptoms/presentation of iron poisoning

A

Iron corrodes the GI mucosa => abdominal pain, bloody vom/dio, metabolic acidosis

141
Q

In what conditions will female babies be noticed at birth, but male babies go unnoticed until 2-4 weeks of age

A

CAH = congenital adrenal hyperplasia

  • females have ambiguous genitalia at birth => noticed earlier
  • males noticed at 2-4 weeks when they present w/ salt wasting features: hyponatremia, hypokalemia, severe dehydration
142
Q

Opalescent teeth + blue sclera

A

Opalescent teeth (variety of colors) + blue sclera = Osteogenesis imperfecta

143
Q

Torticollis vs. Nucchal rigidity

(a) Signs/symptoms
(b) Workup

A

Torticollis

(a) resists passive flexion and rotation to one side, keeps head rotated to the side
- most common cause- URI, minor trama, cervical lymphadenitis
(b) Xray of the neck- rule out cervical spine fracture or dislocation

Nucchal rigidity

(a) Inability to bend neck forward due to rigidity of neck muscles
- not necessarily pain when moving forward but actually loss in FROM (full range of motion)
(b) LP for meningitis workup

144
Q

Vaccination schedule

(a) H. influenza B
(b) Pneumococcus
(c) IPV
(d) MMR
(e) HepB
(f) Varicella

A

Vaccination schedule

(a) HIB- 4 doses by 18 mo
(b) PCV (pneumococcal virus)- 4 doses by 15 mo
(c) IPV- 3 doses by 18 mo + booster at 4-6 yoa
(d) MMR- first at 12-15, booster at 4-6
(e) Hep B- 3 doses before 18 mo (first at birth)
(f) Varicella = same as MMR: first at 12-15, booster at 4-6

145
Q

Think of what kind of jaundice at the following times

(a) First 24 hrs of life
(b) 2-3 dol
(c) 5-7 dol
(d) few weeks of life

A

Jaundice

(a) First 24 hrs of life = erythroblastosis fetalis
(b) 2-3 days of life, gone by 5th day = physiologic jaundice
(c) 5-7 dol- suggests neonatal sepsis
(d) first few weeks of life- suggests breast milk jaundice

146
Q

Describe the pathophysiology/signs of nephrotic syndrome

A

Increase in glomerular membrane permeability causes protein excretion in urine (proteinuria). Loss of protein in urine => low protein in blood (hypoalbuminemia). Low protein in blood allows water to be drawn into soft tissues (edema)

3 features of nephrotic syndrome

  • large (4+) proteinuria
  • hypoalbuminemia
  • edema
147
Q

Distinguish the type of tachycardia that comes from hyperthyroidism vs. Wolf Parkinson White syndrome

A

Tachycardia

(a) Hyperthyroidism => sinus tachy (SA node)
(b) WPW = accessory pathway btwn atria and ventricle that causes early ventricular contraction = supraventricular tachy

148
Q

What is erythroblastosis fetalis?

A

Erythroblastosis fetalis = hemolytic disease of the newborn

-maternal IgG cross the placenta and attack fetal RBC

149
Q

Aplastic crisis

(a) Most common etiology
(b) Lab findings
(c) Tx

A

Aplastic crisis = transient arrest of erythropoiesis, most commonly associated w/ infection

(a) Parvovirus B19
(b) Lab findings: sudden drop in Hgb, absence of reticulotyes (b/c so aacute)
(c) Tx: blood transfusion

150
Q

16 yo w/ h/o UC w/ diarrhea, rash, and beefy red tongue- most likely to be deficiency in which vitamin

A

Niacin (vitamin B3) => pellagra = diarrhea, dermatitis, glossitis, dementia
-mental status changes: poor concentration, irritability

151
Q

WAGR syndrome

A

Wilms’ tumor
Aniridia = absence of the iris
Genitourinary anomaly
Retardation

152
Q

Symptoms of thiamine deficiency

A

Thiamine (Vitamin B1) deficiency = beriberi or Wernicke-Korsakoff
-neurologic + psychiatric symptoms

153
Q

Symptoms of riboflavin deficiency

A

Riboflavin (vit B2) deficiency = cheilosis (fissures/cracks on lips), glossitis, seborrheic dermatitis, pharyngitis, edema

154
Q

Quick distinguishing feature btwn undetected hearing impairment and autism

A

Hearing impairment doesn’t come w/ the repetitive behaviors seen in autism

-although both can have language/communication difficulties and decreased social skills

155
Q

6 mo old w/ FTT and regurgitation after feedings

  • during vomiting child tilts head left and arches back
  • afebrile, abdomen non-tender to palpation

(a) Dx
(b) Management

A

(a) GERD = regurgitation after feeding + FTT
- position (left tilt and arched back) used to protect airway and react to the pain of the acid reflux

(b) Gold standard diagnostic tool of GERD = 24 hr esophageal pH monitoring

156
Q

Indication for maternal Zidovudine tx

A

HIV- prevent transmission to fetus

157
Q

Tx besides ethosuxamide for absence seizures

A

Valproate/valproic acid

158
Q

2 yo w/ intractable diarrhea, FTT

  • oral thrush
  • lymphadenopathy
  • HSN
A

Congenital HIV

159
Q

2 week old w/ blood-stained odorless vaginal discharge

Next step in management?

A

Reassurance- F less than 3 mo can develop vaginal spotting/bleeding due to maternal estrogens that crossed the placenta

160
Q

Risk factors for IVH in a newborn

A

Intraventricular (ex: germinal matrix) hemorrhage most commonly seen in premature and LBW infants

161
Q

Workup for pubertal gynecomastia in adolescent boy

A

Reassurance- it is a benign finding present in about 50% of adolescent boys

  • usually asymmetric, frequently tender
  • prepubertal male testes normally 2 cm in length and 3 mL in volume
162
Q

From what cells of origin do Wilm’s tumors come from?

A

Wilm’s tumor (unilateral flank mass) comes from the metanephros

163
Q

Acute onset of HA + hemiparesis and dilated left pupil after an episode of acute otitis media or sinusitis

(a) Fundoscopic exam?
(b) Febrile?

A

Brain abscess
-acute onset of headache and focal neurologic findings (hemiparesis and dilated left pupil)

(a) Fundoscopic exam normal- papilledema is a later sign
(b) Nope

164
Q

Presentation of pheochromocytoma

A

Pheochromocytoma = hormone-secreting tumor of the adrenals

Presents w/ flank pain, elevated BP/HR, palpitations, anxiety, weight loss

165
Q

Meniere’s disease

A

Inner ear d/o (accumulation of fluid in inner ear) w/ vertigo

166
Q

14 yo F w/ Turner’s presenting w/ leg muscle fatigability

  • HTN
  • mild, continuous murmur all over the chest

Most probably finding on CXR?

A

Rib notching = specific for coarctation of the aorta (7% Turner’s pts), due to dilation of the collateral chest wall vessels

  • Mild continuous murmur all over the chest due to flow of collaterals
  • HTN in arms/upperbody due to coarctation
167
Q

Aplastic crisis vs. hemolytic crisis

A

Retic count

Hemolysis would have high retic count
Aplastic crisis is sudden and retics don’t have time to compensate => high Hgb w/ zero retics

168
Q

Technetium-99m pertechnetate scan

A

Diagnostic test for Meckel’s diverticulum

169
Q

How to kids w/ CAH present?

A

Congenital adrenal hyperplasia = deficiency in an enzyme needed for steroid synthesis (most common is 21-hydroxylase)

  • hirsutism or virilism (low voice or other male secondary sex characteristics)
  • decreased mineralocorticoid production => hyponatremia and hyperkalemia
  • ambiguous genitalia, salt wasting, and dehydration
170
Q

11 mo old w/ rachitic rosary, large anterior fontanelle, thickening of lower end of long bones (wrists)

A

Vitamin D deficiency Rickets

171
Q

Bilious emesis 4 hrs after birth

  • no BM
  • afebrile
  • distended abdomen
  • decreased bowel sounds
A

Intestinal atresia

172
Q

What is Wolff-Parkinson White syndrome?

(a) 3 characteristic EKG findings

A

Accessory pathway btwn the atrium and ventricle which leads to ventricular preexcitation (part of the ventrile depolarizes early) since the accessory pathway conducts faster than thru the AV node

(a) EKG findings: slurred delta wave, shortened PR interval, widened QRS
Early ventricular depolarization = the delta wave which both broadens the ventricular complex (QRS) and shortens the PR interval

173
Q

Most common causes of the following in children

(a) Communicating hydrocephalus
(b) Obstructive hydrocephalus

A

(a) Communicating hydrocephalus- most commonly caused by subarachnoid hemorrhage, which can be due to intraventricular hemorrhage
(b) Obstructive hydrocephalus- Chiari malformation, Dandy Walker anomaly

174
Q

Lab value to distinguish iron deficiency anemia and thalassemia

A

RDW

  • high RDW in iron deficiency anemia (and low retic count)
  • normal RDW in thalassemia (w/ high retic count)
175
Q

Differentiate presentation of rubella virus and herpes virus 6

A

Rubella- presents w/ lymphadenopathy and rash that starts on face

Herpes virus 6- no lymphadenopathy, rash starts on the trunk, pt is no longer febrile when the rash appears

176
Q

Most common bacterial cause of the following in SCD pts

(a) Osteomyelitis
(b) Sepsis

A

Sickle cell pts

(a) Osteomyelitis caused by salmonella
(b) Sepsis caused by capsulated organisms: strep pneumo, nontypable H. influenza

177
Q

7 yo w/ multiple prior fractures

  • hypotonia
  • blue sclera
A

Osteogenesis Imperfecta = congenital bone d/o

  • brittle bones that are prone to fracture
  • blue sclerae, hearing loss, seizures
  • opalescent teeth
178
Q

Next step for pt who swallowed a battery and has it stuck in his/her

(a) Esophagus
(b) Stomach

A

(a) When swallowing a foreign object- need to prevent esophageal ulceration of mucosal damage => if in the esophagus immediately remove endoscopically
(b) If distal to the esophagus, you can watch and wait- 90% of cases will pass into stool w/o intervention

179
Q

Workup: previously healthy 15 mo old after LOC of crying when frsutrated or upset

  • no cyanosis or incontinence
  • no post-LOC state
  • normal physical exam
A

Reassurance to parents

-breath holding spells: apnea and LOC associated w/ anger/frustration

180
Q

Minimal change disease

(a) most common cause in children
(b) Tx

A

Minimal change disease- most common cause of nephrotic syndrome (proteinuria) in children

(a) Idiopathic injury to glomerular basement membrane
(b) Steroids

181
Q

Age at which you come when called

A

12 mo

182
Q

What is an appropriate screening test for 4 yo M

A

Vision test

183
Q

Describe how the following may present at birth

(a) Erb-Duchenne palsy
(b) Klumpke paralysis

A

Presentation at birth

(a) Erb-Duchenne: abscent Moro yet intact grasp reflex on affected side
(b) Klumpke: unilateral hand paralysis w/ ipsilateral Horners (ptosis and miosis)

184
Q

Why is it important to treat VUR?

A

Vesicoureteral reflux can cause renal scaring => hypertension

185
Q

Expected finding in 10 yo SCD pt’s peripheral smar

A

Howell-Jolly bodies indicating functional asplenia

-HJB are remnants of RBCs that would normally be removed by a functional spleen

186
Q

Resolution of post-strep GN timeline

Order: proteinuria, hematuria, kidney function, complement levels

A

Kidney function resolves in 1-2 weeks as inflammation decreases as infection is cleared

  • then complement levels normalize in 8-12 weeks
  • hematuria can last for 6 months
  • proteinuria can really persist for a few years
187
Q

Mechanism of joint injury seen in hemophilia

A

Hemosiderin (iron) deposition and fibrosis (cytokine release)

188
Q

6 mo old w/ sudden jerky mov’ts of neck, arms, and legs in clustered episodes

  • small 1-2 cm oval irregular hypopigmented macules
  • cortical tubers in cerebral cortex on head CT
A

Tuberous sclerosis = genetic d/o

  • often presents w/ infantile spasms
  • ash leaf macules
  • unique EEG findings: hypsarrhythmia
  • cortical tubers = classic finding on EEG
189
Q

Macrosomia, macroglossia, visceromegaly, omphalocele, hypoglycemia, hyperinsulinemia

A

Beckwith-Wiedemann syndrome- exactly cause unknown

190
Q

Which types of infections are pts w/ sickle cell disease at an increased risk for?

A

Pts w/ SCD become functionally hyposplenic due to splenic autoinfarction => susceptible to infxn w/ encapsulated organisms such as S. pneumoniae, H. influenzae, N. menigitides

191
Q

What other findings would you expect in a kid w/ blood in the subarachnoid space?

A

Most common cause of subarachnoid hemorrhage in children = AVM (arteriovenous malformation) w/ rupture into the subarachnoid space

AVM’s local effects = seizures, migraine-like HA

=> expect a history of seizures or migraine-like HA in kid w/ SAH

192
Q

Differentiate the jaundice seen in galactosemia vs. breastfeeding jaundice

A

Galactosemia = conjungated hyperbili

Breast feeding jaundice = unconjungated hyperbili

193
Q

HA worse in morning, vom, visual disturbance, unbalanced gait, trunk dystaxia, horizontal nystagmus

A

Medulloblastoma, posterior vermis syndrome

194
Q

2 mechanisms by which breastfeeding jaundice causes hyperbilirubinemia

A

Usually seen in exclusively breastfed infants who aren’t getting enough milk

  • inadequate enteral intake prolongs intestinal transit time => increased enterohepatic circulation => increased bile reabsorption
  • also relative dehydration

=> jaundice

195
Q

Pyloric stenosis and hypokalemia

Next step in management?

A

1st- give hydration and K+ replacement b/c must ensure surgical stabilization

2nd- then once electrolytes are stable, do the surgery

196
Q

Cherry red macula

(a) Disease
(b) Cause

A

Cherry red macula- buzzword for Niemann-Pick disease

(b) Caused by sphingolipidosis due to deficiency in sphingomyelinase

197
Q

2 yo M who hasn’t started walking yet

  • prolonged labor
  • 3 and 5 APGAR scores at 1 and 5 minutes
  • learning disabilities, hyperactive DTRs
A

Cerebral palsy

  • nonprogressive
  • most likely cause is cerebral anoxia
198
Q

What mom/baby blood types is the worst set up for ABO incompatibility

A

ABO incompatibility (causing jaundice) most commonly seen in mom w/ BT O and baby w/ A, B, or AB

-presents w/in first 24 hrs

199
Q

Tx for hemophilia

(a) Option for mild hemophilia

A

Tx for hemophilia = factor VIII replacement (contains vWF)

(a) Mild hemophilia can be treated w/ desmopressin (DDAVP) which causes factor VIII release from endothelial cells

200
Q

Use of oral tetracycline for conjuncitivitis

A

Depends on age!!!

Tetracycline is contraindicated in kids under 8 yoa b/c of bone and teeth effects (enamel hypoplasia)

201
Q

Bileous vomiting + abdominal distention

Xray: Triple bubble w/ gasless lower abdomen

A

Jejunal atresia

-double bubble = duodenal atresia

202
Q

Signs of heroin/methadone exposure in neonate

A

IUGR, macrocephaly, SIDS, neonatal abstinence syndrome

-opiates not associated w/ dysmorphic features or congenital anomalies

203
Q

Presentation of acute chest syndrome

A

Acute chest syndrome- not uncommon in SCD pts

-chest pain, fever, new infiltrate on CXR

204
Q

16 yo M w/ LROM of left knee

  • history of multiple episodes of l. knee and r. ankle swelling
  • early bruising since childhood
  • same thing in his uncle
A

Hemophilia

Bleeding into joint = hemarthrosis
-recurrent hemarthoses may lead to joint injury called hemophilic arthropathy = iron deposition and fibrosis of the joint

205
Q

Marfans

(a) 3 body systems affected
(b) Gene mutated

A

Marfans = due to mutation in fibrillin 1 gene

Effects 3 systems: MSK, eyes, heart

206
Q

3 absolute contraindications to breast feeding

A

Absolute contraindications for breast feeding

  • maternal HIV
  • maternal HSV active on breast
  • maternal active tuberculosis

Not absolute contraindications: alcohol, tobacco use, hep C, mastitis
-substance abuse: encouraged to stop using drugs but not to stop breast feeding

207
Q

15 yo F presents w/ amenorrhea and tanner 2 breast development w/ scant pubic hair
-decreased femoral pulses

A

Karyotype for Turner’s (XO)

-decreased fem pulses indicate aortic coarctation (seen in about 10% of Turner’s pts)

208
Q

Name some factors that ironically decrease risk of RDS

A
  • maternal hypertension
  • chronic intrauterine stress from prolonged rupture of membranes
  • IUGR = intrauterine growth retardation

Proposed mechanism: intrauterine stress stimulates early fetal lung maturity

209
Q

2 GI findings associated w/ Down’s syndrome

A
  1. Duodenal Atresia

2. Hirschsprung = neural crest cells don’t migrate => part of colon has no enteric nervous system

210
Q

Pneumonic for DiGeorge syndrome- what should be monitored during surgery?

A

DiGeorge = microdeletion of chr22 = Catch 22

“ca” for calcium levels
-monitor during surgery due to hypoparathyroidism

“t” for thymic aplasia

“h” for heart/cardiac abnormalities

211
Q

15 yo w/ gait and speech difficulty x6 mo
-scoliosis, feet deformity, dysarthria, dysmetria, nystagmus, absent DRTs on LEs

(a) Diagnosis
(b) Most common complication
(c) Most common cause of death

A

(a) Friederich’s ataxia = autosomal recessive, most common type of spinocerebellar ataxia
- presents w/ neurologic symptoms and skeletal abnormalities

(b) 90% of pts deelop cardiomegaly
(c) Most common cause of death = cardiomegaly

212
Q

1 mo old w/ w/ harsh, loud holosystolic murmur over LLSB

  • thrill over precordial region
  • no cyanosis or rales
  • CXR: normal sized heart w/ increase in pulmonary vascularity
  • normal EKG

Next step?

A

Reassurance- indicative of VSD (most common type of congenital heart disease)
-most small (aka loud harsh murmur) close spontaneously w/o treatment as long as there is no sign of pulmonary vascular disease

213
Q

Lethal complication of menigococcal meningitis

A

Waterhouse-Friederichsen syndrome- about 100% mortality = sudden vasomotor collapse and skin rash (petechiae) due to adrenal hemorrhage

214
Q

Ashkanazi Jewish population

Differentiate disease of deficiency in:

(a) acid beta-glucosidase
(b) hexosaminidase

A

Ashkanazi Jewish populaiton

(a) Deficiency in acid beta-glucosidase = Gaucher disease of lysosomal storage, appears in adolescents (ex: 15) w/ bone pain, easy bruising, anemia
(b) Deficiency in hexosaminidase = Tay-Sachs lysosomal stroage disease, present early in life w/ neurologic finding and cherry-red retinal finding

215
Q

12 yo boy presents w/ 2 weeks of edema
-proteinuria 3+

(a) Dx
(b) Tx

A

Edema and proteinuria

(a) Minimal change disease = most common cause of nephrotic syndrome in children

216
Q

Drug used to

(a) Keep PDA open
(b) Close a PDA

A

Drug to keep

(a) PDA open = prostaglandin E1 = vasodilator to prevent PDA closure in infants to increased or maintain pulmonary blood flow and improve oxygenation until definitive surgery can be performed
ex: pulmonary atresia, tricuspid atresia, tetralogy of Fallot

(b) Close a PDA: indomethacin = potent prostaglandin inhibitor

217
Q

Which animal is the main reservoir for rabies?

A

Bats

218
Q

Most common presentation of Tuberous Sclerosis

A

Tuberous sclerosis = multisystem genetic disease

  • often presents w/ infantile spasms
  • then also developemental delay, ID, skin findings (ex: ash leaf macules), lung and kidney disease
219
Q

6 yo w/ abdominal pain, hematuria, macpap rash, knee TPA

(a) Diagnosis
(b) Biopsy finding

A

(a) HSP = Henoch Schloein Purpura
- purpura
- abdominal pain
- AKI
- arthralgia

(b) Mesangial deposition of IgA
- HSP is a IgA mediated vasculitis

220
Q

Distinguish the following immunodeficiencies

(a) DiGeorge syndrome
(b) X linked agammaglobulinemia
(c) Wiskott-Aldrich syndrome
(d) Hyper IgM

A

Immunodeficiencies

(a) DiGeorge: thymic aplasia and T cell deficiency => susceptible to fungal infxns
(b) X linked agammaglobulinemia- no B cells => lacks all immunoglobulins
- lack of humoral response => no anaphylaxis ‘
(c) Wiskott-Aldrich = X linked combined B and C cell disorder
(d) Hyper IgM = elevated IgM and depressed IgG and IgA

221
Q

Height- compared to birth

(a) First year
(b) when does it double by?
(c) when does it triple by?

A

Height

(a) Height increases 50% in the first year
(b) Height doubles by 4 yao
(c) Height triples by 13 yoa

222
Q

Delayed separation of umbilical cord, what to expect next?

A

Delayed separation of the umbilical cord is associated w/ a congenital leukocyte adhesion defect

  • recurrent bacterial and endodontal infections
  • normal lymphocyte and gamma globulin level
223
Q

Dicloxacillin

A

= Nafcillin = Oxacillin

-great coverage for strep and staph

224
Q

Duchenne’s muscular dystrophy

(a) Mode of inheritance
(b) characteristic features
(c) diagnostic study

A

DMD

(a) X-linked recessive
- as opposed to autosomal dom of 2nd most common: myotonic muscular dystrophy
(b) Calf pseudohypertrophy and positive Gower’s sign
(c) Diagnostic study = muscle biopsy

225
Q

First line abx regimen for CF pt w/ pneumonia

A

Most likely organism = pseudomonas

IV regimen = Cetazipime (3rd gen that covers pseudomonas) + Gentamicin (aminoglycoside- covers pseudomonas)

-necessary to have double coverage of pseudomonas due to high resistance of it to most abx

226
Q

4 day old premie w/ increased gastric residues

A

Increased gastric residues in premie- think NEC (necrotizing enterocolitis)

227
Q

Painless melena in 2 yo

A

Painless bloody stool in a 2 yr old = Meckel’s diverticulum

rule of 2s

228
Q

Neonate w/ blood diarrhea, eosinophils in stool, FHX of atopy

A

Milk protein intolerance

229
Q

30 minute old ex-28 weeker w/ central cyanosis

  • tachypnea, grunting, retractions, nasal flaring
  • CXR: fine reticular granularity of lung parenchyma

(a) Diagnosis
(b) Tx

A

(a) Hyaline membrane disease
- occurs in 60-80% of infants born less than 28 weeks gestational age

(b) Dx = mechanical ventilation and surfactant administration

230
Q

Differentiate hyaline membrane disease and persistent pulmonary HTN of the newborn

A

Both present very similarly- cyanotic newborn w/ tachypnea, grunting, retractions

  • HMD more in premies, PPHN in full term
  • Different CXR findings: normal in PPHN vs. reticular granulations in HMD
231
Q

9 yo w/ unsteady gait and lower limb weakness

  • wide based gait
  • decreased vibratory and position sense in lower extremities
  • absent ankle jerks
  • high plantar arches

(a) Diagnosis
(b) Mode of inheritance

A

(a) Freiderich’s ataxia

(b) Autosomal recessive

232
Q

Friedreich’s ataxia

(a) Physiology of disease
(b) Features
(b) Prognosis

A

Friedreich’s ataxia

(a) Aut recessive mutation in frataxin => progressive degernation of spinal cord tissue that connects cerebellum to motor neurons
(b) Features
- weakness
- loss of coordination
- pes cavus = high arch foot abnormality
- heart defects such as hypertrophic cardiomyopathy (most common cause of death in these pts)
- scolisosis
- vision/hearing impairment
- diabetes

(c) Prognosis is poor: most are wheelchair bound by 25 and live until 30-35 yoa

233
Q

Which of the following are contraindications to breast feeding?

(a) Maternal HIV
(b) maternal breast cancer
(c) maternal rubella
(d) hemolytic disease of the newborn
(e) acquired immune deficiency syndrome

A

Contraindiations: maternal HIV and aids (acquired immune deficiency syndrom), maternal breast cancer
-transmission of HIV by breastfeeding is very well documented

Not contraindications:

  • hemolytic disease of the newborn
  • maternal rubella
234
Q

Describe in what way the following impair respiratory fxn in a newborn

(a) Fetal lung fluid
(b) Poor alveolar compliance
(c) Meconium
- key feature
(d) Paralysis of the diaphragm
- finding on CXR

A

Respiratory fxn impairment in newborn

(a) Fetal lung fluid => TTN (transient tachpnea of the newborn)
(b) Poor alveolar compliance => RDS in premies
(c) Meconium aspiration- often in SGA and post-mature infants, coarse streaking and patchy opacities on CXR, greenish amniotic fluid

(d) Paralysis of the diaphragm
- elevated hemidiaphragm on CXR

235
Q

Infantile colic

(a) Age of presentation
(b) Most common time for crying
(c) Timeline of resolution

A

Infantile colic

(a) Usually presents w/in the first 1-3 weeks of life
(b) Often the same time everyday, early evening
(c) Usually spontaneously resolves by 4 mo

236
Q

Kid who starts developing recurrent sinopulmonary infxns after 6 mo of age

A

Think B-cell deficiency b/c starts around 6 mo when mother’s antibodies are gone
-lack of IgA predisposes to GIardia

237
Q

Describe features of congenital chlamydial infection

A
  • purulent conjunctivitis
  • pneumonia
  • few days to several weeks after birth
238
Q

What is the most likely cause of congenital hypothyroidism:

(a) in the US
(b) Worldwide

A

Congenital hypothyroidism

(a) In the US: 85% of cases due to thyroid dysgenesis: asplasia, hypoplasia, or ectopic gland
(b) Worldwide- iodine deficiency endemic goiter

239
Q

Differentiate acquired aplastic anemia and Fanconi’s anemia

(a) Causes
(b) Age of presentation
(c) Diagnostic feature

A

Acquired aplastic anemia

(a) Meds, chemo, radiation exposure, usually idiopathic
(b) Can present younger
(c) Diagnostic feature = hypocellular bone marrow biopsy w/ decrease in all 3 line’s stem cells

Fanconi’s anemia

(a) Autosomal recessive
(b) Blood counts start to decreased by 4-12 yoa
(c) Diagnostic feature = congenital abnormalities at birth
- cafe au lait spots

240
Q

Complication of Fanconi’s anemia

A

Due to autosomal recessive mutation in DNA repair enzyme => very high risk for AML

241
Q

Todd’s paralysis

A

Postictal paralysis (ex: hemiparesis)

ex: kid suddenly looses consciousness and is paralyzed on his right side, spontaneously resolves in 24 hrs
- rapidly improves w/ restoration of motor fxn w/in 24 hrs

Pediatrics (10 decks)

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