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Peds Exam (5900) #3 > Muscular Diseases > Flashcards

Muscular Diseases Flashcards

1
Q

Most serious and most common of the MDs in childhood?

A

Duchenne Muscular Dystrophy (DMD)

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2
Q

Duchenne Muscular Dystrophy (DMD) genetic type?

A

X-linked inheritance pattern

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3
Q

Duchenne Muscular Dystrophy (DMD) is also known as?

A

pseudohypertrophic muscular dystrophy

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4
Q

Onset of DMD?

A

between ages 3 and 5 years

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5
Q

S/S of DMD?

A
  • Progressive muscle weakness, wasting, and contractures
  • Calf muscles hypertrophy in most patients
  • Loss of independent ambulation by age 9 to 12 years
  • Progressive generalized weakness in adolescence
  • Death from respiratory or cardiac failure
  • GOWER’S SIGN
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6
Q

Loss of independent ambulation with DMD occurs at what age?

A

age 9 to 12 years

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7
Q

Death from DMD is due to?

A

respiratory or cardiac failure

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8
Q

Serum CPK and AST levels high are high during what period of DMD?

A

first 2 years of life, before onset of weakness

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9
Q

What happens to Serum CPK and AST levels as muscle deterioration occurs due to DMD?

A

they diminish

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10
Q

Which serum marker levels are high during the first 2 years of life, before onset of weakness due to DMD?

A

CPK and AST

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11
Q

Major complications of DMD include?

A
  • Contractures & disuse atrophy
  • Obesity
  • Infection
  • Respiratory and cardiopulmonary problems-
    • may need Respirator, trach
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12
Q

DMD: Clinical Manifestations

A
  • Waddling gait, frequent falls, Gower sign
  • Lordosis
  • Profound muscular atrophy in later stages
  • Mild to moderate mental impairment; emotional disturbance common
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13
Q

patient that has to use their hands and arms to “walk” up their own body from a squatting position due to lack of hip and thigh muscle strength.

A

Gower’s sign

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14
Q

Gower’s sign

A

patient that has to use their hands and arms to “walk” up their own body from a squatting position due to lack of hip and thigh muscle strength.

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15
Q

Therapeutic Management of DMD

A
  • No tx or cure
  • maintain function as long as possible
  • maintain good quality of life
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16
Q

protein that is critical to the health and survival of the nerve cells in the spinal cord responsible for muscle contraction

A

Survival Motor Neuron Protein

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17
Q

Muscular disorder cause by a defective auto recessive SMN protein?

A

Spinal Muscular Atrophy (SMA)

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18
Q

S/S of SMA?

A
  • Breathing/feeding problems
  • Low muscle tone (Hypotonia or floppy baby)
  • Poor head control
  • Little spontaneous movement
    • Tongue fasiculations-twitching
  • Progressive weakness moving distal to proximal
  • Frequent and increasingly severe respiratory infections
  • Nasal speech quality
  • Worsening posture
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19
Q

Forms of the SMA?

A

Type I: Occurs in infancy and is most severe
Type II: Also occurs in infancy, although less severe symptoms
Type III: Least severe, symptoms may not occur until age two

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20
Q

Prognosis of the SMA?

A
  • Type I: 2-3 years
  • Type II: Longer, but death occurs during childhood
  • Type III- May survive to early adulthood
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21
Q

Diagnostic Tests for SMA?

A

EMG, MRI of spine, Muscle biopsy, DNA test confirms diagnosis

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22
Q

Complications of SMA?

A
  • Aspiration
  • Contractures
  • Respiratory infections
  • Scoliosis
  • Pain from immobility and secondary impairments
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23
Q

Purpose of PT in tx of SMA?

A

to prevent contractures in joints and scoliosis

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24
Q

Purpose of OT in tx of SMA?

A

to prevent contractures in upper extremities and for adapting environment for feeding, ADLS, access to writing and art activities

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25
Q

Purpose of Speech Therapy in tx of SMA?

A

for respiratory and feeding issues

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26
Q

Secondary Impairments R/T Immobility and Disease Progression of SMA?

A
  • Scoliosis
  • Hip misalignment
  • Pain
  • Joint contractures
  • Calcium deficiency and osteopenia
  • Constipation
  • Respiratory issues
  • Feeding and nutrition issues (may need G-tube)
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27
Q

Condition characterized by chronic inflammation of the synovial membrane leading to joint effusion and eventual erosion, destruction and fibrosis of the articular cartilage?

A

Juvenile Idiopathic Arthritis (JIA)

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28
Q

Juvenile Idiopathic Arthritis (JIA)

A

Condition is characterized by chronic inflammation of the synovial membrane leading to joint effusion and eventual erosion, destruction and fibrosis of the articular cartilage

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29
Q

Cause of JIA is unknown but at least two events are considered necessary:

A
  • Immunogenetic susceptibility

- An external, environmental trigger

30
Q

Clinical Manifestations of JIA?

A
  • Joint swelling, stiffness especially after inactivity and in am,
  • loss of ROM
  • joints may be warm to touch & painful to touch
  • Symptoms increase with stressors
  • Growth retardation
  • Uveitis in 8-20%
31
Q

Onse of JIA?

A

before age 16 years

32
Q

JIA peaks at what age?

A

1-3 & 8-10 years

33
Q

Pauciarticular onset of JIA?

A

involves 4 or fewer joints

34
Q

Polyarticular onset of JIA?

A

involves 5 or more joints

35
Q

S/S of systemic onset of JIA?

A

high fever, rash, hepatosplenomegaly, pericarditis, pleuritis, lymphadenopath & joint involvement

36
Q

Meds to tx JIA?

A

NSAIDs, SAARDs, corticosteroids, cytotoxic agents

37
Q

The most common spinal deformity?

A

Scoliosis

38
Q

3 planes of deformity in Scoliosis?

A
  • Lateral curvature,
  • spinal rotation
  • thoracic hypokyphosis
39
Q

average age of onset of Scoliosis?

A

10 years

40
Q

average age start of puberty in boys?

A

11-12

41
Q

average age start of puberty in girls?

A

10-11

42
Q

Therapeutic Management of Scolosis Curves < 10°?

A

normal, no tx required

43
Q

Therapeutic Management of Scolosis Curves < 20° and not progessing?

A

no Tx

44
Q

Therapeutic Management of Scolosis Curves < 20°?

A
  • treated with braces or surgery depending on the magnitude, location, type of curve, age and skeletal maturity of child, and any underlying or contributing disease processes
45
Q

How many hours a day is the brace worn in a patient with scoliosis?

A

16-23 hrs/day

46
Q

condition where the socket is too shallow and the ball (thigh bone) may slip out of the socket, either part of the way or completely?

A

Developmental Dysplasia of the Hip (DDH)

47
Q

Developmental Dysplasia of the Hip (DDH)

A

condition where the socket is too shallow and the ball (thigh bone) may slip out of the socket, either part of the way or completely

48
Q

Developmental DDH is more common in males or females, and what ethnicity?

A
  • females

- caucasions

49
Q

Risk factors of developing DDH?

A
  • Low levels of amniotic fluid in the womb
  • Being the first born
  • Being female
  • Breech position during pregnancy, in which the baby’s bottom is down
  • Family history of the disorder
  • Large birth weight
50
Q

The Barlow and Ortolani tests are done to diagnosis which condition?

A

Developmental Dysplasia of the Hip (DDH)

51
Q

This procedure dislocates the hip to test or DDH?

A

Barlow Test

52
Q

This procedure reduces the hip to test or DDH?

A

Ortolani Test

53
Q

A child affected with DDH and with bilateral dislocations will have what type of gait?

A

waddling gait

54
Q

What is the Trendelenburg sign?

A

stand on affected side, unaffected hip goes down (normally goes up)

55
Q

incomplete dislocation with some residual contact between femoral head and acetabulum?

A

Subluxable

56
Q

Subluxable

A

The incomplete dislocation with some residual contact between femoral head and acetabulum?

57
Q

What is the Pavlik Harness used for?

A

used to treat DDH

58
Q 
Therapeutic Management
of DDH (6-18 months)?
A

Traction (e.g Pavlik Harness)

59
Q 
Therapeutic Management
of DDH (after 18 months)?
A

surgical repair and placement in spica cast

60
Q

avascular necrosis of the femoral head (usually unilateral)

A

Legg-Calve-Perthes Disease

61
Q

Legg-Calve-Perthes Disease

A
  • avascular necrosis of the femoral head (usually unilateral)
  • Self-limiting, self-healing disease
62
Q

Legg-Calve-Perthes Disease Usually occurs in children ages?

A

3-12

63
Q

Legg-Calve-Perthes more common in?

A

males ages 4-8

64
Q

Clinical Manifestations of Legg-Calve-Perthes Disease?

A
  • Mild or intermittent pain in the anterior thigh and a “painless” limp
  • Decreased abduction and internal rotation
65
Q

Spontaneous displacement of the proximal femoral epiphysis in a posterior and inferior direction

A

Slipped Femoral Capital Epiphysis

66
Q

Slipped Femoral Capital Epiphysis

A

Spontaneous displacement of the proximal femoral epiphysis in a posterior and inferior direction

67
Q

When does Slipped Femoral Capital Epiphysis occur?

A

shortly before or during accelerated growth periods of puberty

68
Q

Causes of Slipped Femoral Capital Epiphysis?

A
  • Usually idiopathic, multifactorial

- hypothyroid, pituitary disorders

69
Q

S/S of Acute SFCE?

A

severe hip pain with inability to bear weight?

70
Q

S/S of Chronic SFCE?

A
  • able to bear weight and walk with mild limp

- unable to internally rotate leg during an exam

71
Q

Tx of SFCE?

A
  • make patient non-weight bearing followed by surgical repair

Peds Exam (5900) #3 (10 decks)

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