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Master the boards: Pediatrics > Orthopedics/Rheumatology > Flashcards

Orthopedics/Rheumatology Flashcards

(88 cards)

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1
Q

What age does Congenital Hip Dysplasia Typically present?

A

Infants (Birth to 1year)

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2
Q

How does Congenital Hip Dysplasia Commonly present?

A

Usually found on newborn screening exam

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3
Q

How is Congenital Hip Dysplasia Diagnosed?

A

Ortolani and Barlow Maneuver: Click or Clunk in the hip

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4
Q

How do you treat Congenital Hip Dysplasia?

A

Pavlik Harness

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5
Q

What age group does Legg-Calve-Perthes Disease present in?

A

Ages 2-8

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6
Q

What is Legg-Calve-Perthes Disease?

A

Avascular necrosis of the femoral head

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7
Q

How does Legg-Calve-Perthes Disease commonly present?

A

Painful limp

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8
Q

How do you diagnose Legg-Calve-Perthes Disease?

A

X-ray: showing joint effusion and widening

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9
Q

How do you treat Legg-Calve-Perthes DIsease?

A

Rest and NSAID followed by surgery on both hips

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10
Q

Why do you perform surgery on both hips in Legg-calve-Perthes disease?

A

If one hip nocroses eventually so will the other

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11
Q

What age does Slipped Capital Femoral Epiphysis commonly present?

A

Adolescence: espeically in obese patients

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12
Q

How does Slipped Capital Femoral Epiphysis typically present?

A

Painful limp with an externally rotated leg

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13
Q

How is slipped capital femoral epiphysis diagnosed?

A

X-ray: showing widening of the joint space

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14
Q

How is Slipped Capital Femoral Epiphysis treated?

A

Internal fixation with pinning

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15
Q

What causes Rickets?

A

Lack of Vitamin D, Calcium or Phosphate

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16
Q

What is Rickets?

A

Softening and weakening of the bones increasing susceptibility to fracture due to lack of Vitamin D, Ca, or PO4

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17
Q

What age group is at the highest risk of Rickets?

A

6-24 months because bone are rapidly growing

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18
Q

What are the 3 major etiologies of Rickets?

A

Vitamin-D Deficient

Vitamin D-Dependent

X-linked Hypophosphatemic

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19
Q

What is Vitamin-D Deficient Rickets?

A

Lack of Vitamin D in the child’s diet

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20
Q

What is Vitamin D-Dependent Rickets?

A

Inability to convert 25-OH to 1,25(OH)2: therefore the infant is dependent on Vit D supplementation

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21
Q

What is X-Linked Hypophosphatemic Rickets?

A

Innate kidney defect results in the inability to retain phosphate: without phosphate bone mineralization cannot occur and bones are weakened

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22
Q

How does Rickets commonly present?

A

Ulnar/radial bowing and a waddling gait to to tibial/femoral bowing

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23
Q

How do you diagnose Rickets?

A

CXR of Costchondral Joints: rachitic rosary-like appearance with cupping and fraying of the epiphyses

Bowlegs

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24
Q

What is the characteristic sign of Rickets?

A

Bowlegs

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25
How do you treat Rickets?
replace PO4, Ca and Vit D
26
What form of Vit D do you use in Rickets?
Ergocalciferol: 1,25(OH)2, calcitriol
27
How often do you monitor Vit D levels in Rickets?
Annually
28
What causes Duchenne Muscular Dystrophy (DMD)?
XLR frameshift or deletion of the dystrophin gene that leads to complete loss of dystrophin
29
What causes Becker Muscular Dystrophy (BMD)?
XLR non-frameshift mutation that results in partial function of dystrophin
30
What are DMD and BMD?
XLR traits leading to progressive muscle weakness
31
What are common symptoms of DMD and BMD?
Progressive muscle weakness in the proximal muscles and lower extremity
32
What does the Gower sign signify?
Weakness of the proximal lower extremity muscles
33
What is the best initial test for DMD and BMD?
Elevated Creatine Kinase
34
What is the most accurate test for DMD and BMD?
Molecular genetic testing
35
What is the mainstay of treatment for DMD and BMD in children >4?
Glucocorticoids
36
What is Casimersen used for in DMD?
Mutation amenable to exon 45 skipping
37
What is Eteplirsen used for in DMD?
mutation amenable to exon 51 skipping
38
What are Golodirsen and Vitolarsen used for in DMD?
mutation amenable to exon 53 skipping
39
How does Lead Poisoning commonly present?
Recent loss of appetite, intermittent abdominal pain vomiting, decreased hours of sleep at night withdrawal from school activities Learning disbilities and behavioral problems
40
What is the best initial test for lead poisoning?
Capillary Blood finger-stick for lead level
41
What is the most accurate test for lead poisoning?
Serum venous blood level
42
What value of serum lead level is intervention indicated?
>10mcg/dL
43
What is the best initial step in managing lead poisoning?
remove the child from the exposure
44
What are potential treatment options for lead poisoning?
Chelation therapy: Dimercaprol or Succimer
45
How does Osteogenesis Imperfecta (OI) commonly present?
young child with repeated fractures due to fragile bones, blue sclerae and early deafness
46
What is the most accurate test for OI?
Skin biopsy analyzed for Collagen synthesis by culturing dermal fibroblasts
47
How do you manage OI?
There is no cure: try to manage fractures, increase bone mass and correct deformities
48
How does Ewing Sarcoma appear on X-ray?
Onionskin pattern due to lytic lesions causing laminar periosteal elevation
49
What is the most accurate diagnostic test for ewing sarcoma?
Analysis for t11;22 via bone biopsy
50
How is Ewing Sarcoma Managed?
Multidrug chemotherapy with local disease control via surgery and radiation
51
How does Osteogenic Sarcoma present on X-ray?
Sclerotic destruction causing "sunburst" appearance
52
What is the most accurate diagnostic test for Osteogenic Sarcoma?
CT scan of the leg
53
How is Osteogenic Sarcoma managed?
Chemotherapy and ablative surgery
54
How does Osteoid Sarcoma present on x-ray?
Round central Lucency with a sclerotic margin
55
What is the most accurate diagnostic test for Osteoid Sarcoma?
CT or MRI of the affected leg
56
How is Osteoid sarcoma managed?
NSAIDS for pain it will spontaneously resolve
57
What is the most common type of arhtritis in children?
Juvenile Idiopathic Arthritis (JIA): umbrella term for 6 conditions
58
What do all 6 subtypes of JIA have in common?
Associated with synovitis of peripheral joints, soft tissue swelling, joint effusion and elevated inflammatory markers (ESR, CRP)
59
How does Systemic-onset JIA present?
Inflammation in 1 or more joints High-spiking fever >2weeks Salmon-pink macular rash that comes and goes possible: hepatosplenomegaly, lyphadenopathy, serositis, hepatitis aneima, lymphadenopathy
60
What is Oligarticular JIA?
Arthritis in up to 4 joints: typically larger joints uveitis is common: greatest risk with (+) ANA
61
How often do you screen eyes in Oligoarticular JIA?
ANA (+): Q3 months ANA(-): Age 7 then Q6 months
62
What major joint is spared in Oligoarticular JIA?
Hip joint
63
What is Polyarticular JIA?
Inflammation in 5 or more joints: symmetric commonly in hands, neck and jaw Can be (+) or (-) or RF: (+) associated with more severe course
64
What is Juvenile Psoriatic Arthritis?
Arthritis in combination with Psoriasis Commonly has: Dactylitis, pitting of the fingers, onycholysis: hx in 1st degree relative
65
What is Enthesistis-Related JIA?
Tenderness where the bone meets a tendon, ligament or other CT Commonly: SI joint tenderness, Axial joint involvement and fam Hx of IBD Often HLA-B27(+)
66
What is the most important feature of JIA/JRA?
Presence of high, spiking fever Often >104F in a younger person, no clear etiology and a rash
67
What are the features of STILLS disease?
Salmon-Colored Rash Temperature elevation Ill-appearing patient Lymphadenopathy Leukocytosis splenomegaly
68
What is the diagnostic testing workup for JIA?
No clear test Anemia, Hypoalbuminemia and Leukocytosis are common Normal ANA Markedly elevated Ferritin
69
How is JIA treated?
Start with Aspirin or NSAIDS -if no response use steroids: if still no response use TNF drugs
70
What is Ehlers-Danlos Syndrome?
Group of CT disorders that are AD
71
What are classic features of Ehlers-Danlos?
Extremely elastic, smooth skin that is fragile and bruises easily Large and small joint hypermobility MVP or aortic root dilation
72
How do you diagnose Ehlers-Danlos?
Clinical diagnosis confirmed gentically
73
What should all patients with Ehlers-Danlos receive?
Screening Echocardiogram as the next step in management
74
How do you treat Ehlers-Danlos?
Physical and Occupational Therapy
75
What is Marfan Syndrome?
AD mutation of the FBN1 gene on Chr15 that encodes fibrillin: a major component of bones, CT and blood vessels
76
How does Marfan Syndrome commonly present?
Tall, thin patient with long extremities, arachnodactylyl, pectus excavatum and hypermobile joints
77
What is the most accurate test for Marfan syndrome?
Genetic testing
78
What are 2 common complications associated with Marfan Syndrome?
Aortic Root Dissection Ectopia Lentis
79
How often do you perform an echo in marfan syndrome?
TTE at time of diagnosis then 6 months later
80
Ho often do you screen for Ectopia Lentis in Marfan syndrome?
Annual Ophthalmologic evaluation
81
How do you treat Marfan Syndrome?
Supportive Care
82
What is Kawasaki Disease?
necrotizing febrile vasculitis of medium-sized vessels that primarily affects large coronary blood vessels
83
What age group does Kawasaki Disease occur in ?
children
84
How does Kawasaki Disease typically present?
>5 days of fever and all 5 of the following -Rash -Mucositis -Edema or Erythema of hands and feet -Cervical Lymphadenopathy -Limbus sparring bilateral Conjunctivitis
85
What lab results are commonly Seen in Kawaski Disease?
Elevated WBC, Platelets, Transamnases and acute phase reactants anemia Pyuria
86
How do you treat kawasaki Disease?
IVIG and Aspirin as soon as the diagnosis is made
87
What do you want to prevent by treating Kawasaki Disease?
Coronary artery aneurysm: IVIG reduces the risk
88
Why are steroids not used in kawaski disease?
They increase the risk of aneurysm formation

Master the boards: Pediatrics (13 decks)

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