Review questions 2-Table 1 Flashcards

1
Q
  1. What congenital orthopedic/skeletal problem is characterized by blue sclera?
A

Osteogenesis imperfect

(also see broken bones pre and post natal – if you see it in US, it is almost pathopneumonic

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2
Q
  1. What meds are used to treat OI?
A

bisphosphonate

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3
Q
  1. Most common form of short-limb dwarfism?
A

Achondroplasia

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4
Q
  1. A patient is diagnosed with achondroplasia. What long-term orthopedic problem are they at increased risk for?
A

Scoliosis

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5
Q
  1. A mother brings her 6 y/o son in with complaints that he has been limping. He denies any trauma, and says it doesn’t hurt. On PE you note poor internal rotation and abduction. What is your suspicion?
A

Legg-Calf-Perthes disease

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6
Q
  1. What exams would you order and what would you expect to find?
A

XRAY: Necrotic bone (avascular necrosis of hip)
LABS: CBC, ESR
TX: protect joint, limit movement.

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7
Q
  1. Toxic synovitis is usually an autoimmune response following:
A

An infection, esp URI

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8
Q
  1. Clinical features distinguishing L-C-P dz and septic arthritis of the hip include:
A
    • fever in septic arthritis

- + pain in septic arthritis (LCP = PAINLESS)

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9
Q
  1. What is “nursemaid’s elbow” and how is it treated.
A

Subluxation of the radial head

TX: Flex the FA, supinate the elbow

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10
Q
  1. This type of fx is described as “oblique fx of the distal tibia without fibular involvement” and usually manifests in children ages 1-3 without hx of significant trauma, and with only minimal pain/swelling.
A

Toddler’s Fx

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11
Q
  1. A common fracture of the forearm, usually about 1/3 way up the radius, is a ____
A

Buckle fracture (aka Taurus Fx)

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12
Q
  1. Describe the various Salter-Harris classifications.

Which is worst?

A

Class V = worst (crushed growth plate, likely to arrest growth)

(III and IV bad too, b/c go into joint space)

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13
Q
  1. Describe distinguishing factors of a limp caused by SCFE
A

Slipped Capital Femoral Epiphysis

  • Adolescents 10-16
  • especially very active or obese males
  • Vague syptoms: + limp, +pain knee/med thigh/ hip
  • limited internal rotation
  • SURGICAL EMERGENCY
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14
Q
  1. Congenital deformity of the foot, characterized by inward deviation with angulation at the base of the 5th metatarsal.
A

Metatarsus varus

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15
Q
  1. ID Causes of #14. Differentiate from club foot
A

Intrauterine positioning

MV can be passively moved to midline, club foot is a rigid skeletal abnormality.

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16
Q
  1. An infant born with metatarsus varus is at increased risk of what OTHER ortho finding?
A

Hip dysplasia (2-10%)

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17
Q
  1. 14/F presents with lump on knee. She is an active soccer player and says it hurts every now and then, but usually doesn’t bother her. She appears very thin but well –nourished. The lump is visible and makes her nervous. You note a 3-4cm hard, palpable mass on the medial side of the knee. You suspect?
A

Ostechondroma (most common bone neoplasm)

TX: typically benign, you can leave it there, or surgically remove if problematic

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18
Q
  1. What is the most common malignant bone tumor in kids? Where are they typically found?
A
  • Osteosarcomas

- Typical in long bones

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19
Q
  1. What is the first treatment done for osteosarcoma?
A
- Chemotherapy
THEN Excision (excision = curative)
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20
Q
  1. 6 y/o Female presents with pain in L arm, especially if you touch it. Pain worsens day by day. She also presents with fever. What are your differentials?
A
  • Ewing’s sarcoma vs. Osteomyelitis
  • Do Labs + XRay to ID. Fever + lytic lesion in bone = osteomyelitis
  • Fever, Inc WBC also osteomyelitis
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21
Q
  1. An infant is diagnosed with hip dysplasia. You want to apply an orthopedic splint called ____
A
  • Pavlik Harness
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22
Q
  1. A 4 year old presents in status epilepticus. What is your first-line treatment?
A
  • Diazepam (Valium)
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23
Q
  1. EEG’s _____ (can/not) be used to diagnose epilepsy
A

They are NOT used

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24
Q
  1. What is the most common type (manifestation) of childhood epilepsy?
A
  • partial seizures (usually due to genetics)
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25
Q
  1. A form of epilepsy characterized by very brief spasms, severe EEG, and poor prognosis is called:
A
  • Infantile spasms
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26
Q
  1. Describe facts associated with febrile seizures. When would you work them up?
A
  • Typically ages 6m – 6y
  • Usually do not progress to epilepsy
  • If resolves quickly and returns to normal, no workup need. If difficult to arouse or vomiting after seizure, work up neuro
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27
Q
  1. Patient presents for 2m check up. Home birth with educated midwife. NB Screen and vaccines done by midwife. Mom’s concern – baby not eating in last week or so, separated sutures and bulging fontanelle. What do you think of?
A
  • Increased ICP – mass? Hydrocephalus?
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28
Q
  1. What symptoms would you expect to see in a child with increased ICP?
A
  • HA (may be worse in AM, worsens with cough, vagal, or bending over)
  • Strabismus, diplopia, wandering eye
  • Vomiting WITHOUT nausea
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29
Q
  1. 13/F overweight, presents with complaint of HA. Mother states she complains a lot, but recently also complains of tinnitus and vision being off. What is your concern?
A

*most commonly= idiopathic cause

30
Q
  1. Most common causes of childhood stroke?
A
  • cyanotic heart disease
  • sickle cell disease
  • meningitis
  • hypercoagulable state
  • Sxs: similar to adult: u/l flaccidity, facial droop
31
Q
  1. Neurofibromatosis, Tubosclerosis, and Sturge-Webber are all associated with what finding?
A

Seizures

32
Q
  1. 5 y/o presents with concerned grandparents due to him “not like other kids”, has to climb to stand. What do you suspect? What is this finding called?
A
  • Suspect Muscular dystrophe

- Gower sign

33
Q
  1. What pathopneumonic finding would further heighten suspicion of MD?
A
  • calves appear full (hypertrophic)
34
Q
  1. Match diseases to their pathologies:
    a) Muscular dystrophy
    b) Guillan-Barre
    c) Myesthenia gravis
    d) Spinal Muscular Atrophy (SMA)
A

a) Muscle fiber
b) nerve
c) Neuromuscular junction
d) Anterior horn

35
Q
  1. 15y/o mom brings him in in a panic because hisfeet felt numb, by lunch he felt weird standing up. He can talk but he feels like he’s getting weaker in his upper legs. Hx significant for URI this week. You suspect? How do you treat?
A
  • Suspect Guillan-Barre
  • Tx: supportive care, ADMIT
  • May need ventilator support
36
Q
  1. A 12 y/o presents with purpuric rash 1 week following a URI. She also has a blood-shot eye and red colored urine. You suspect?
A
  • Henloch Schenlein pupura (autoimmune against blood vessels)
37
Q
  1. Describe tx for meningomyeloceles.
A
  • Surgery

- * be sure to assess for paralysis at birth (active & symmetric movement? Incontinence? )

38
Q
  1. Compare and contrast Henoch-Shonlein vs Idiopathic Thrombocytic Purpura
A

BOTH: Autoimmune, Purpuric rashes, Hx URI (or infection).
HSP = autoimmune a/g blood vessels
ITP = autoimmune a/g platelets

39
Q
  1. 18m/f brought in for pallor. Family think something’s wrong because she’s getting pale and they’re dark Hispanic. Nml diet, no PMH. Normal vitals… You only note pallor. No hepatosplenomegaly, lymphadenopathy, no bruising. What do you consider?
A
  • consistent with anemia

- Order: CBC, Iron studies

40
Q
  1. What lab would you order in #40 to ID whether this is an acute or chronic anemia and how the body is compensating?
A
  • Order reticulocyte count. High reticulocyte count = enough time AND able to make new cells.
  • Low retic = acute onset OR aplastic/hypoplastic (use hx!)
41
Q
  1. 3 y/o female in ER presents with jaundice, fatigue x 3 days, Low hgb, high retic. She also has organomegaly. You want to consider what?
A
  • Hemolytic/destructive anemias (organs large from processing broken cells/ bilirubin excess):
  • sickle -G6PD
  • spherocytosis - thalassemias
42
Q

43.T/F heart murmurs are commonly associated with anemia.

A

TRUE

43
Q
  1. Diamond Black-fan is a _____ anemia that can be treated by ____ and cured by ___
A
  1. Congenital hypo/aplastic anemia
  2. tx by steroids
  3. cured by bone marrow transplant
44
Q
  1. Diamond black-fan has __ (more/less) association with other anomalies than it’s cousin Fanconi’s anemia
A

Less: 30% dbf, 50% Fanconi

45
Q
  1. How is Fanconi’s managed?
A
  • steroids or IVIG, BM trasfusion

- 20% self-limited auto-immune and often grow out of it.

46
Q
  1. Differentiate Fanconis vs DBF:
A

DBF: onset @ birth-1yr, short stature, autosomal recessive,

Fanconis” onset 2-10 yrs, higher rate congenital problems, autosomal recessive

47
Q
  1. 4 y/o male with pallor, fatigue x 2 months. You order a CBC and find low RBC, WBC, AND platelets. What do you think?
A
  • 2+ depressed cell lines think leukemia

- age 4, boy, most common leukemia in kids = ALL

48
Q
  1. What lab finding would be consistent with your differential in #48?
A
  • high lymphoblast numbers (overgrow the BM so the BM can’t work)
49
Q
  1. Most common cancer in teens?
A

Lymphomas (3rd most common cancer of all: #1- ALL, #2-brain tumors #3-lymphoma)

50
Q
  1. What are the two types of lymphoma? Which is worse?
A
  • Hodgkin vs non-Hodgkin lymphoma

- non-Hodge = worse (also less common)

51
Q
  1. What clinical findings would you find in a pt with Hodgekin lymphoma? What would you use to confirm?
A
  • Findings: PAINLESS lymphadenopathy without illness, splenomegaly, nml CBC
  • Do CXR to confirm: look for mediastinal widening/mass, enlarged thymus
52
Q
  1. What characteristic finding will differentiate between Hodgekin and non-Hodgekin lymphoma?
A
  • Reed Steinberg cells in Hodgekin lymphoma

- hodgekin = cervical lymph most common

53
Q
  1. A patient presents with a triad of symptoms including papilledema, HA worse in AM, and vomiting. You recognize this as alarming for ___
A

Increased ICP

54
Q
  1. What is the most common brain tumor in kids?
A

Astrocytoma

55
Q
  1. A brain tumor where allows for the best prognosis?
A

SUPRAtentorial

56
Q
  1. Definitive tx for a brain tumor includes:
A

Surgical removal (successful in 50-60% of all CA)

57
Q
  1. A 3y/o presents with poor feeding, abd distention, and refuses to walk. When you pick him up, he cries loudly. He also appears pale and miserable. On exam you note a large, palpable abdominal mass and HTN. What do you consider?
A

-Diff dx: HTN=renal?, mass = neuro/nephroblastoma

58
Q
  1. Differentiate between neuroblastoma and nephroblastoma. Which is called Willm’s tumor?
A
  • Neuroblastoma can cross midline

- Nephrobllastoma (Willms) does not cross midline

59
Q
  1. The most common sign of retinoblastoma is:
A
  • absence of red reflex (white reflex)

- Strabismus

60
Q
  1. The soft tissue tumor aka most common sarcoma of childhood is called:
A

-Rhabdomyosarcoma

61
Q
  1. Differentiate presentation of rhabdomyosarcoma by age.
A
  • Young – head, neck, GU

- Older kids: extremities, trunk

62
Q
  1. A patient presents with jaundice. It is determined that they do not posess the enzyme that metabolizes and conjugates bilirubin. This diagnosis is called ___
A

Guilbert’s (specifically elevation in unconjugated bilirubin d/t deficiency of conjugating enzyme.

63
Q
  1. ID types of congjugating enzyme deficiencies.
A
  • No enzyme AT ALL = Kraigler Najar. (requires tx or fatal; tx = phenobarbiotol)…)
  • Diminished activity (Guilberr’s) (usually benign, but keep a close eye out. Often don’t require tx)
64
Q
  1. Protein C deficiency, antitrombin 3 deficiency, and protein S deficiency all result in what?
A
  • Hypercoagulation state (clotting problems)
65
Q
  1. A pt presents with septic shock. You also note hematuria, bleeding gums, etc. What labs should you order?
    What is the most likely diagnosis?
    What is the treatment?
A
  • CBC, PTT, PT, fibrinogen, INR
  • DIC highly associated with sepsis

Tx: FFP (contains a lot of clotting factors)

66
Q
  1. How can you treat severe thrombocytopenia?
A
  • transfusion of platelets
67
Q
  1. A 14 y/o presents with heavy periods and occasional nose bleeds. She denies any spontaneous bruising though. What problem might you check for?
A
  • VonWillebrands
68
Q
  1. Define whether each problem is due to a problem with production, consumption, or abnormal function:
    a) ITP
    b) Leukemia
    c) Sickle cell (in crisis)
A

a) Destruction (auto immune)
b) production
c) function

69
Q
  1. ID and differentiate between most common childhood leukemias.
A

ALL (acute lymphoblastic leukemia (most common = 75%
AML (15-20%)
CML (

70
Q

What are some key features of ALL?

A

Peak age is 4, boys>girls, Lymphoblasts in smear,

71
Q

What are some key features of AML?

A
  • more agressive

- bone pain, night sweats, fatigue

72
Q

What are some key features of CML?

A

more agressive

  • bone pain, night sweats, fatigue- may be asymptomatic
  • Philadelphia chromosome