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TRI 6 - Peds > Exam 2 > Flashcards

Exam 2 Flashcards

1
Q

What is the main type of MD that only affects’ Boys

A

Duchenne

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2
Q

Why Does Duchenne MD only affect Boys

A

It is a recessive gene tied to the X chromosome, boys only get 1 x so it is always expressed even though it is recessive

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3
Q

What is the pathophysiology of MD

A

Absence of dystrophin leads to breakdown of muscle fibers

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4
Q

When is MD usually diagnosed in boys

A

3-7 years

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5
Q

Describe the pattern of weakness in MD

A

Neck flexors
Abdominals
Pelvic girdle
Proximal to distal UE musculature

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6
Q

Describe pseudohypertrophy as seen in MD

A

Larger muscles due to increased fat and connective tissue content
Not actually stronger

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7
Q

What percent of MD patients experience intellectual or behavioral problems

A

30%

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8
Q

What is the primary impairment of MD

A

Insidious weakness

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9
Q

What are secondary impairments of MD

A

Contractures
Postural malalignment
Osteopenia
decreased respiratory and cardiac capacity, GI motility issues

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10
Q

How old are MD patients when they first need a wheelchair

A

8-12

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11
Q

What 5 factors predict loss of ambulatory ability

A

50% reduction in leg strength
MMT <3 for hip extensors and <4 for ankle DF
Inability to climb stairs
More than 9 sec 10 meter time and inability to rise from the floor, predicts loss of walking within 2 years
10 meter time more than 12 seconds predicts ambulation loss within 1 year

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12
Q

What is the primary goal for MD treatment

A

Function and participation, prolonged standing and ambulation

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13
Q

What are some things to avoid with MD

A

Immobilization
aggressive strengthening

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14
Q

What is the rough survival rate of children with cancer and why

A

83%
increased efficacy if interventions

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15
Q

Survivor of cancer are ag great risk or what additional medical problems

A

Cognitive deficits
Functional impairments
Cardiovascular and pulmonary disease

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16
Q

Treatment of pediatric cancer in the CNS alone can cause what late effects

A

Cognitive, hearing and visual deficits

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17
Q

Treatment of pediatric cancer in the CNS, head, neck and gonads can cause what late effects

A

Endocrine abnormalities such as short stature, hypothyroidism, delayed secondary sexual development

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18
Q

Treatment of pediatric cancer in the MSK system alone can cause what late effects

A

Scoliosis and spinal shortening

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19
Q

What risk comes with receiving chemo or radiation as a child

A

10x greater chance of developing second malignancy

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20
Q

Describe acute lymphoblastic leukemia (ALL)

A

80% of all pediatric cases
Commonly occurs in 2-5 years of age
Chemo for 2-3 years
over 90% survival rate

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21
Q

Describe acute myelocytic leukemia (AML)

A

Most frequent in 0-2 years old
Survival rate of 63%

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22
Q

What is the second most common type of cancer in pediatrics and third in adolecents

A

Brain and CNS tumors

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23
Q

Name some brain and CNS tumors

A

Astrocytomas
Medullablastmas
Ependyomas

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24
Q

Describe posterior fossa sydrome

A

Ataxia
cranial nerve involvement
Decreased cervical AROM
Cerebellar mutism
Headaches
Drowsiness
Irritability

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25
Q

Name some bone and soft tissue tumors

A

Osteoma
Ewing Sarcoma

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26
Q

What are some treatment strategies for bone and soft tissue tumors

A

Chemo
Surgery to remove

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27
Q

What are some SS of pediatric leukemias

A

Enlarged lymph nodes
Enlarged liver or spleen
Fever
Easy bleeding or bruising
Night sweats
Weight loss

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28
Q

What are some SS of pediatric lymphomas

A

Painless enlargement of lymph nodes
Night sweats
Persistent fatigue
Fever
Chills
Unexplained Weight loss
Anorexia
Pruritus

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29
Q

What are some SS of pediatric sarcomas

A

Interment pain that worsens at night
Swelling
Decreased ROM and altered gait

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30
Q

What are some SS of brain and CNS tumors

A

Headache
Vomiting
Vision, speech, hearing changes
Worsening balance
Poor gait
Unusual weakness or sleepiness

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31
Q

What are some acute side effects of chemotherapy

A

Anemia, fatigue, tiredness, reduced endurance, headaches, dizziness, damage to bone marrow, immune suppression, increased bruising, loss of appetite

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32
Q

What are some late effects of chemotherapy

A

Pulmonary, cardiac, endocrine and reproductive dysfunction
Osteoporosis and neurocognitive / neurosensory loss

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33
Q

What are some acute side effects of radiation therapy

A

N/V, Diarrhea, hair loss, mucositis, fatigue, skin changes

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34
Q

What are some late effects of radiation therapy

A

Fibrosis and tissue injury

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35
Q

During the conditioning phase before a bone marrow transplant what are patients most at risk for

A

Infection
Bruising
Fatigue

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36
Q

Describe the engraftment period of bone marrow transplant

A

In hospital for 28-35 days after treatment
Waiting to see if immune system begins to recover
Looking for absolute neutrophil count to increase by more than 500 cell/microliter 2 days in a row

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37
Q

What are some physical limitations caused by lymphoma or leukemia

A

Decreased ankle dorsiflexion strength and ROM and handgrip strength
Poor balance and postural control
Peripheral neuropathy

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38
Q

What are some physical limitations caused by osteosarcomas or Ewing’s sarcoma

A

Increased effort with locomotion
Decreased sensation
Neuropathic and nociceptive pain from tumor

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39
Q

What are some physical limitations caused by central and peripheral nervous system tumors

A

Poor motor control
Abnormal muscle tone
Decreased Strength and ROM

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40
Q

What are some symptoms of anemia

A

Fatigue
Reduced endurance
Headaches
Dizziness

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41
Q

What are some treatment considerations for Leukemia

A

Bone pain should subside
May have peripheral neuropathy
Encourage aerobics
Rick of osteonecrosis present

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42
Q

What are some treatment considerations for Lower extremity sarcomas

A

Gait training with an AD
Avoid high impact and high torsion activities

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43
Q

What are some treatment considerations for brain tumors

A

Risk of posterior fossa syndrome

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44
Q

Describe spinal muscular atrophy

A

Degeneration of anterior horn cells with subsequent progressive weakness

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45
Q

Describe the clinical presentation of SMA

A

Symmetrical weakness of skeletal muscles
Progressive wasting
Hypotonia
Normal intellect and sensation

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46
Q

Describe SMA onset type 1

A

Infantile onset
Onset at 0-4 months
Rapidly progressive
Severe weakness
Mortality dependent on disease aggression and medial support

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47
Q

Describe SMA onset type 2

A

childhood onset
Onset at 6-12 months
Initial progression that becomes slowly progresive over years
Moderate to severe weakness

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48
Q

Describe SMA onset type 3

A

Juvenile onset
Onset 1-10 years
Slowly progressive
Mild impairment

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49
Q

Describe SMA function type 1

A

Non-sitter
Infantile onset
Significant issues with breathing and swallowing
Do not attain ability to sit without assistance
Muscle fasciculations of tongue are common

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50
Q

Describe SMA function type 2

A

Sitter
Onet around 18 months
Delayed milestones
Variable course - stable over long period of time
Fatigue is significant
May stand with support but will not ambulate without support or bracing

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51
Q

Describe SMA function type 3A

A

Walker
Diagnosed before 2 years old
50% retained the ability to walk past the age of 12
Tongue fasciculations in about 50%
Proximal LE weakness and fatigue are most common impairments

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52
Q

Describe SMA function type 3B

A

Walker
Diagnosed after 2 years old
50% retained the ability to walk pas 44th birthday
Tongue fasciculations in about 50%
Proximal LE weakness and fatigue are most common impairments

53
Q

Describe intervention strategies of SMA type 1

A

ROM and strengthening, positioning to maintain flexibility, wedge for positioning, respiratory care, developmental activities like supported sitting with focus on head control, adaptive equipment / technology

54
Q

Describe intervention strategies of SMA type 2

A

ROM and stretching, sitting posture, developmental activities aquatics, encouraging standing by 12-18 months, early fitting for KAFOs for standing, supported walking, mobility, respiratory care and fatigue management, managing scoliosis with corset until ages 10-12

55
Q

Describe intervention strategies of SMA type 3

A

Exercise and AROM, strengthening and endurance, fatigue and overhead lifting, adaptive equipment, transfers and mobility training

56
Q

What types of treatments are emerging in the evidence as potentially valuable for SMA

A

Gene replacement therapy
Spiranza

57
Q

What is the most common cause of pediatric SCI

A

MVA
Sports for adolescents

58
Q

What are some preventative measures that can be taken to avoid SCI

A

Seatbelts, car and booster seats
Rear facing car seats until 2 years of age
Water safety
Physicals before sports participation

59
Q

Describe anterior cord syndrome

A

Motor paralysis
Decreased pain and temperature sensation
Poor prognosis

60
Q

Describe the presentations of hemorrhages in the central part of the cervical spinal cord

A

Flaccid UE
Spastic LE
ambulatory, bowel and bladder intact

61
Q

Describe posterior cord lesions

A

Loss of proprioception
Preserved motor function
Ambulation unlikely

62
Q

Describe a brown sequard lesion

A

Ipsilateral paralysis and loss of proprioception
Contralateral loss of pain and temperature
Ambulation and bowel and bladder control likely

63
Q

Describe cauda equina syndrome

A

Injury to L5 nerve roots
Areflexia of LEs and bladder

64
Q

What are some treatment options for kids with SCI

A

Steroids
Hypothermia - may have neuroprotective effect
Stem cell transplants
Surgical stabilization
Cervical traction
Halo traction
Orthotics

65
Q

Describe autonomic dysreflexia

A

Noxious stimulus
Elevate, remove compression stockings and abdominal binder or manage bowels

66
Q

What are some OMs for SCI patients

A

FIM, WeeFIM
Spinal Cord Independence Measure
PEDI
Pediatric Neuromuscular Recovery Scale

67
Q

Describe mobility device needs for various levels of injury

A

C5 and up - power chair
C6 - manual chair at home, power in community
C7-T1 Manual

68
Q

Define spina bifida

A

Failure of neural tube to close primarily in the thoracolumbar region within the 1st 30 days post-conception

69
Q

What are some causes of spina bifida

A

Genetic predisposition
Too little folic acid in mother
Maternal use of valproic acid

70
Q

Describe spina bifida occulta

A

Asymptomatic
Found on x-ray
Tuft of hair or hyperpigmentation
May have tethered cord
May have urinary tract problems

71
Q

Describe meningocele

A

Protruding sac
Only contains meninges and CSF
No motor or sensory problems

72
Q

Describe Lipomeningocele

A

Fatty mass in lumbosacral region
Bowel and bladder dysfunction
Lower leg / foot disorders due to tethered cord syndrome

73
Q

What are some surgical managements of spina bifida

A

Correction post Nataly
Shunt for hydrocephalus
Orthopedic repairs

74
Q

What are some common impairments associated with spina bifida

A

Decreased strength and function below level of lesion
Decreased sensation below lesion
Hip sub and dislocations
Talipes equinovarus
hydrocephalus
Tethered cored syndrome
Bowel and bladder issues

75
Q

What is the prevalence of hydrocephalus in spina bifida patients and what are the SS

A

90%
sunsetting eyes, bulging anterior fontanelle

76
Q

What are some SS of shunt malfunction

A

Redness along shunt site
Headaches
Vomiting
Sunsetting eyes

77
Q

What is a Arnold-chiari malformations

A

Cerebellum and brainstem displaced distally through foramen magnum

78
Q

Describe tethered cord syndrome

A

Spinal cord adheres to the site of occlusion
Scoliosis
Gait issues
Bowel and bladder dysfunction
Decreased strength or sensation

79
Q

How do you treat tethered cord syndrome

A

Dx with myelogram
Rx with surgical release

80
Q

What group of people are at high risk of having a latex alergy

A

Spina bifida kids

81
Q

What are some causes of TBI

A

Trauma
Anoxia
Non-traumatic event

82
Q

Describe some injuries with shaken baby syndrome

A

Retinal swelling
Subdural hematoma
Retinal hemorrhaging

83
Q

What are some secondary injuries associated with TBI

A

Hypoxia
Seizures
Hemorrhage
Brain Swelling
Extracranial causes

84
Q

Describe dry drowning

A

Aspirating water
Laryngospasm cuts off wind pipe
Neurologic damage 33% of the time

85
Q

Describe some behavioral and cognitive impairments seen in TBI

A

Emotional issues
Attention deficits
Distractibility
Impulsivity
Aggression
Decreased memory and learning difficulties
Language issues

86
Q

Describe RLCF 3 treatment

A

Side Lying positioning
Contracture prevention
Prevent pressure ulcers
PROM
Avoid supine due to reflexes
Elevate head above heart to prevent increased intracranial pressure
Tilt table - 45 - 60 mins

87
Q

Describe RLCF 2 treatment

A

Constant supervision
Constant daily routine
Repetition of tasks including orientation x3
Child can become frustrated, bores so trips to park or playground are helpful

88
Q

Describe RLCF 1 treatment

A

Strengthening
Endurance
Locomotor training
CIMT
Gait and transfer
Stair climbing
Cardio fitness

89
Q

Define a concussion

A

Complex physiologic process affecting the brain, induced by traumatic biomechanical forces
May or may not involve loss of consciousness
Functional rather than structural brain injury

90
Q

What are the conditions to diagnose a concussion

A

Period of loss or decreased level of consciousness
Loss of memory either before or after the event
Any alteration in MS at the time of injury

91
Q

Describe the stages of rehabilitation for concussion

A

No activity - complete rest
Light aerobic - <70% intensity no resistance
Sport specific exercise - Sports related drills, no head contact
Noncontact training drills - more complex drills, resistance training may begin
Full contact practice - normal activities after medical clearance
Return to play - normal play

92
Q

How long is typical recovery after a concussion

A

2-4 weeks

93
Q

Define intellectual disability

A

Significant limitations in both intellectual functioning and in adaptive behavior
< 70-75 IQ

94
Q

Describe down syndrome

A

Genetic disorder resulting from trisomy 21

95
Q

Describe how down syndrome is diagnosed

A

Amniocentesis at 13-14 weeks gestation
Chorionic villus sampling at 9-12 weeks
Alpha fetoprotein screening
Physical characteristics, extra skin on the back of the neck

96
Q

What are some characteristics of down syndrome patients

A

Intellectual disability
Stereotypical facial features
Hypotonia
Ligamentous laxity
Joint hypermobility
Gross motor delay
Decreased strength

97
Q

What are some comorbidities associated with down syndrome

A

GI tract anomalies
Thyroid issues
Visual and hearing impairments
Alzheimer’s and dementia
Diabetes and obesity

98
Q

When do down syndrome kids walk by

A

74% by 30 months
92% by 36

99
Q

What are some common exam findings of down syndrome kids

A

Poor head control
Head lag with pull into sitting
Scapular winging
Genu recurvatum
Excessive flexibility
Slipping through sensation when held
Open mouth posture with tongue thrust

100
Q

What findings at the ankle are to be expected with down syndrome kids

A

Pes planus
Calcaneal valgus

101
Q

What are some intervention strategies for hypotonia

A

Stimulate co-contraction
Strengthen antigravity muscles
Prevent splits during transfers

102
Q

What are some appropriate activities for down syndrome kids

A

bike riding
Sports
Aerobics
Swimming
Special Olympics

103
Q

Describe DCD

A

Developmental coordination disorder
Lifetime condition
5-6% of kids
Boys more than girls
increased incidence with kids of low birth weight

104
Q

What are some characteristics of DCD

A

Clumsy
Poor proprioception and motor planning
Difficulty adapting to environmental contexts

105
Q

How is DCD diagnosed

A

DSM-5
difficulty with age appropriate activities even after extensive coaching
Absence of other explanatory diagnosis

106
Q

What are some intervention strategies for DCD

A

Part task training
Process oriented approach
Perceptual motor training
Promote repetition

107
Q

Define CP

A

Group of non-progressive motor disorders affecting movement and posture

108
Q

What other types of disorders accompany CP

A

sensation, perception, cognition, communication, behavior, epilepsy and MSK problems

109
Q

What group of kids have the highest incidence of developing CP

A

born before 28 weeks
Low birth weight, 2.2-2.3 lbs

110
Q

What are some genetic causes of CP

A

Premature birth
Placenta abruption
Preeclampsia

111
Q

Describe Periventricular leukomalacia

A

Developed in 80% of CP kids
Caused by hypoxic ischemic encephalopathy

112
Q

Describe outcomes of PVL and IVH grades

A

1-2 likely to walk by age 2 and minimal risk of neurological impairment
3-4 only 10% walk and high risk of neurological impairment

113
Q

What is a common risk factor of CP

A

Hypoxia or ischemia

114
Q

What is the average age of diagnosis of CP

A

19 months

115
Q

What are the 5 major atributes of gait

A

Sability in stance
Sufficient foot clearance in swing
Appropriate prepositioning of the foot for initial contact
Adequate step length
Energy conservation

116
Q

Describe gait at 1 year

A

Wide BOS
High guard arm position
Absent reciprocal arm swing
Flat food initial contact
Exaggerated hip rotation

117
Q

Describe gait at 1.5 year

A

Heel strike
High guard arm position
Reciprocal arm swing in most
171 steps per minute on average
Exaggerated hip rotation

118
Q

Describe gait at 2 years

A

100% heel strike
Narrow BOS
Decreased hip rotation
Increased step length

119
Q

Describe gait at 2.5 years

A

Reciprocal arm swing in nearly all
Mature control of hip abduction and adduction
Average cadence now 156

120
Q

Describe gait at 3 years

A

Adult like BOS
All joint rotations smooth
Reciprocal arm swing 100%

121
Q

Describe gait at 4 years

A

Reciprocal arm swing
All joint rotations look adult like
Adult like BOS

122
Q

Describe gait at 5 years

A

Reciprocal arm swing
All joint rotations look adult like
Adult like base of support
Increased stride length
Increased velocity 1.08m/sec
Cadence now 154 steps/ min

123
Q

Describe gait at 6 years

A

Cadence now 146 steps/min

124
Q

Describe gait at 7 years

A

Increasing stride length and gait velocity (1.14m/sec)
Cadence now 143 steps/min

125
Q

What can cause Trendelenburg gait

A

weak hip abductors and decreased pelvic stability

126
Q

What can cause crouch in gait

A

poor hip and knee extension, increased pronation

127
Q

What can cause foot drop in gait

A

weak ankle DFs

128
Q

What can cause toe walking in gait

A

increased tone onto ankle PFs, tissue tightness on ankle PFs

TRI 6 - Peds (7 decks)

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