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Pediatrics > unit 6 > Flashcards

unit 6 Flashcards

1
Q

What is the most common form of MD?

A

-DMD

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2
Q

What gender does DMD most commonly affect and why?

A

-Males; It is a recessive X linked gene

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3
Q

Can Females be carriers of DMD?

A

-Yes, can be recessive in one “x” and be masked by the other “x”

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4
Q

What is the halmark sign of DMD?

A

-Insidious progressive weakness

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5
Q

Who long do those with DMD usually live?

A

-Until late teens or early adult

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6
Q

What causes the progressive weakness with DMD?

A

-The absence of Dystrophin which leads to a break down of muscle fibers

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7
Q

What age is DMD normally Diagnosed?

A

-3 to 7

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8
Q

How do children with DMD present in early infancy?

A

-Normal; gross motor milestones met

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9
Q

What motor milestones are not met by those with DMD?

A

-Advanced (running, Jumping, etc); why it is diagnosed after infancy

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10
Q

Where does weakness due to DMD present first?

A

-Neck flexors and abdominals; then the pelvic girdle

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11
Q

In the Extremities what muscles are usually weakest early on?

A

-Extensors

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12
Q

What is a hallmark of DMD that commonly presents in the calves of patients?

A

-Psuedohypertrophy

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13
Q

What is psuedohypertrophy?

A

-an increase of muscle size without an increase of strength; due to fibrosis and CT accumulation in muscle tissue

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14
Q

What percent of those with DMD have cognitive issues?

A

-30%

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15
Q

What is Gowers Sign?

A

-When a child has to “walk” their hands up their LEs to stand Erect after bending over; indicative of proximal weakness (especially the pelvic girdle)

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16
Q

How will breathing impairments be first commonly seen in those with DMD?

A

-They will not be able to manage their breath while speaking

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17
Q

What is the Vignos Scale?

A

-A way to classify those with DMD by Function; 1 being most functional and 10 being the least

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18
Q

What is a 1 on the Vignos Scale?

A

-walks and climbs stairs without assistance

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19
Q

What is a 2 on the Vignos Scale?

A

-Walks and climbs stairs with use of railing

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20
Q

What is a 3 on the Vignos Scale?

A

-Walks and climbs stairs slowly with railing

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21
Q

What is a 4 on the Vignos Scale?

A

-Walks but cannot climb stairs

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22
Q

What is a 5 on the Vignos Scale?

A

-Walks but cannot climb stair or get out of chair

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23
Q

What is a 6 on the Vignos Scale?

A

-Walks only with assitance or braces

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24
Q

What is a 7 on the vignos scale?

A

-In wheel chair, sits erect and can roll chair and perform bed and wheelchair ADLs

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25
Q

What is an 8 on the VIgnos Scale?

A

-In wheel chair, sits erect- cannot perform ADLs without assistance

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26
Q

What is a 9 on the VIgnos scale?

A

-In wheelchair, can only sit erect with support only, can perform minimal ADLs

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27
Q

What is a 10 on the Vignos Scale?

A

-in bed; can do no ADLs without Assistance

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28
Q

What is the PRIMARY impairment of DMD?

A

-Insidious Muscle Weakness

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29
Q

What are the secondary impairments of DMD? (7)

A

-Contractures, malalignment, osteopenia, respiratory and cardiac dysfunction, easy fatigue, obesity, impaired GI motility

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30
Q

Why is positioning important in those with DMD?

A

-becuase of respiratory dysfunction

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31
Q

By what age are those with DMD usually wheelchair users?

A

-8 to 12

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32
Q

What treatment can prolong a child with DMD’s ability to ambulate?

A

-Corticosteroid (standard medical treatment)

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33
Q

What besides walking can corticosteroids also prolong for those with DMD?

A

-Standing, pulmonary funcition, and isometric strength

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34
Q

What are the predictors for loss of ambulat ability? (3)

A
  • 50% reduction in LE strength
  • MMT <3 for hip flexors and <4 of DFs
  • Inability to climb stairs
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35
Q

A ten meter walk test most than 9 seconds predicts with a person with DMD will lose the ability to ambulate in how many years?

A

-2

36
Q

A ten meter walk test most than 12 seconds predicts with a person with DMD will lose the ability to ambulate in how many years?

A

-1 year

37
Q

Prolonging what can decrease the risk for scoliosis and osteopenia in those with DMD?

A

-Standing and walking

38
Q

What do interventions normally focus on with DMD?

A

-Compensation to perform participations (progressive disease– will not recover)

39
Q

What should an HEP of those with DMD include?

A

-ROM and stretching to prevent contractures

40
Q

How should strengthening exercises be perform with those with DMD?

A

-mildly

41
Q

When should you teach AD management and use for those with DMD?

A

-before the sesation of walking

42
Q

What type of contraction is contraindicated for those with DMD?

A

-Eccentric

43
Q

What interventions are normally recommended for those with DMD?

A

-ADL management and stretching

44
Q

What are Late Effects?

A

-effects from cancer treatment that a survivor may endure years later

45
Q

Cancer Treatment of the CNS can cause what impairments?

A

-Cognitive, hearing and visual impairments

46
Q

Treatment of the CNS, Head, Neck, or Gonads can cause endorcrine abnormalities that can cause what?

A

-short stature, hypothyroidism, and delayed secondary sexual development

47
Q

Cancer treatment of the MSK system can cause what?

A

-scoliosis or spinal shortening

48
Q

What is the most common pediatric cancer?

A

-Acute Lymphoblastic Luekemia (80%)

49
Q

What ages is ALL most common in?

A

-2 years to 5 years

50
Q

What is the survival rate of ALL?

A

-90%

51
Q

What type of luekemia presents between the ages of 0 and 2?

A

-Acute Myelocytic Luekemia

52
Q

What is the Survival Rate of AML?

A

-63%

53
Q

What is the second most common pediatric cancer?

A

-Brain and CNS tumors

54
Q

What are the Signs and Symptoms of Luekemias? (6)

A

-Enlarged Lymph nodes, Enlarged liver or spleen, fever, easy bleeding or bruising, night sweats and weight loss

55
Q

What are the signs and symptoms of Lymphomas? (7)

A

-Painless elargment of lymph nodes, night sweats, persistent fatigue, fever and chills, unexplained weight loss, anorexia, pruritis (itching)

56
Q

What are the signs and symptoms of Sarcomas? (4)

A

-Intermittent pain that is worse at night, swelling, decreased ROM, Altered gait pattern

57
Q

What are the signs and symptoms of Brain and CNS cancers? (9)

A

-Headache, Vomitting, vision, speech and hearing changes, worsening balance, unsteay gait, unusual sleepiness, and weakness

58
Q

What are the late effects of radiation therapy?

A

-Fibrosis and tissue injury

59
Q

Luekemia may present with what physical impairment?

A

-decreased ankle dorsiflexion, hand grip strength

60
Q

The luekemia treatment Vincristine may cause what?

A

-poor balance and peripheral neuropathy

61
Q

What causes SMA?

A

-Degeneration of anterior horn cells (alpha motor neuron)

62
Q

How will reflexes present with SMA?

A

-hypo or areflexic

63
Q

What type of disease is SMA?

A

-Autosomal recessive– also progressive

64
Q

What is the main impairment you will see with SMA?

A

-severe and progressive weakness of skeletal muscle

65
Q

What muscles are more affected by SMA?

A

-Proximal (trunk), but will involve extremities as it progresses

66
Q

Where will fasciculations present with SMA?

A

-the tongue

67
Q

Where will tremors present with SMA?

A

-hands

68
Q

What other impairments will children with SMA have?

A

-impaired feeding and breathing

69
Q

What are two secondary impairments due to SMA?

A

-Contractures and scoliosis

70
Q

What is another name for Type I SMA?

A

-Werdig Hoffman Syndrome

71
Q

What is the most severe and most common type of SMA?

A

-Type I

72
Q

When is the onset of type I SMA?

A

-within the first few weeks or months of life

73
Q

Children with Type I SMA will not acheive the ability to do what?

A

-sit, no antigravity movements,

74
Q

Onset of Type II SMA occurs when?

A

-during the first year of life

75
Q

Children with Type II SMA will be able to do what/ will not be able to do what?

A
  • Will be able to sit independently
  • Stand with assistance
  • Ambulate with external bracing
76
Q

Those with type II SMA are normally what kind of ambulators?

A

-household; or for exercise— not much function

77
Q

What is another name for type III SMA?

A

-Kugelburg Walender Syndrome

78
Q

What is the Mildest form of SMA?

A

-Type III

79
Q

Those with Type III SMA will be able to do what at some point?

A

-Ambulate without assistance

80
Q

What are the most common impairments with type III SMA?

A

-proximal LE weakness and fatigue

81
Q

When does Type III SMA present?

A

-within the 1st decade of life

82
Q

Presentation of Type III SMA is similar to what?

A

-DMD

83
Q

Type IIIA SMA is diagnosed when?

A

-Before 2 years

84
Q

Type IIIB SMA is diagnosed when?

A

-After 2 years

85
Q

50% of those with type IIIA SMA will retain the ability to walk until when?

A

-after 12 years

86
Q

50% of those with type IIIB SMA will retain the ability to walk past when?

A

-44 years

Pediatrics (14 decks)

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