Exam II Flashcards
what is Duchenne Muscular Dystrophy (DMD)? what causes it?
progressive genetic disorder characterized by muscle degeneration and weakness due to the alterations of dystrophin (causing breakdown of muscle fibers)
what muscles get weak first in patients with DMD?
extensors are weaker than flexors early on in the disease
what is Gower’s sign? what condition is this sign present with?
(1) when the patient has to use their hands to push up on their knees to obtain an erect position
(2) DMD (due to weak extensors)
what is a hallmark sign of DMD?
pseudohypertrophy of the calves
what is the main reason majority of kids with DMD die?
decreased pulmonary function leading to respiratory failure
what is a contraindication for treatment of DMD?
eccentric exercises and aggressive stretching due to the muscle damage it causes
what is the most common type of Leukemia? what percentage of leukemia patients have this type?
(1) acute lymphoblastic leukemia (ALL)
(2) 80%
what are the two types of leukemia?
(1) acute lymphoblastic leukemia (ALL)
2) acute myelocytic leukemia (AML
which type of leukemia has the better survival rate?
ALL (>90% survival rate)
what are the first and second most common types of pediatric cancers?
(1) leukemia
(2) brain / CNS tumor
when would a platelet count be a contraindication for exercise?
<20,000 aerobic exercise is contraindicated due to increased risk for internal bleeding
what is Spinal Muscle Atrophy (SMA)? what causes it?
(1) genetic disorder leading to progressive weakness of skeletal muscles
(2) degeneration of the Alpha motor neurons in the anterior horn
what are the main impairments seen with SMA?
(1) weakness
(2) hypotonia
what is the onset age for the 3 types of SMA?
Type I: onset 2 weeks - 4 months old
Type II: onset within first year of life
Type III (A): diagnosed before 2 y/o
Type III (B): diagnosed after 2 y/o
what is the prognosis for SMA types 3A and 3B?
3A: 50% retain the ability to walk past age 12
3B: 50% retain the ability to walk past age 44
what is a patient with SMA at a higher risk for once they lose the ability to walk?
scoliosis
what are 3 conditions that occur secondary to a SCI in children?
(1) neuromuscular scoliosis
(2) hip subluxations
(3) longer lifespan post-injury
REVIEW SCI
REVIEW SCI
what is spina bifida? what causes it?
birth defect caused by failure of the neural tube to close (typically in the thoracolumbar region) which can lead to neurological issues
what are 3 possible causes of spina bifida?
(1) genetic predisposition
(2) too little maternal folic acid
(3) maternal use of valproic acid (used to treat seizures and mental disorders)
how are spina bifida meningocele and lipomeningocele characterized?
(1) meningocele: protruding sac containing meninges and CSF; no motor or sensory issues
(2) lipomeningocele: fatty mass in lumbosacral region; bowel and bladder dysfunction, lower leg and foot disorders
what condition occurs in 90% of patients with spina bifida? why does this happen?
(1) hydrocephalus
(2) occurs when the open spinal cord is closed following surgery; the closure causes excess fluid to be put back into the spinal column causing increased pressure
how is hydrocephalus corrected?
a shunt is surgically put in to drain CSF
what are Arnold-Chiari malformations? how are they fixed?
(1) in patients with spina bifida, the cerebellum and brainstem are displaced distally through the forearmen magnum
(2) cranial laminectomy or posterior fossa decompression
what causes a tethered cord in patients with spina bifida?
spinal cord adheres to site of occlusion
what are children with spina bifida at a much higher risk to be allergic to?
latex
what position do kids with spina bifida at the thoracic level like to be in?
frog position (hip abduction and ER, knee flexion, plantar flexion)
what position do kids with spina bifida at the high lumbar level like to be in?
hip flexion and adduction (causing weak quads)
physiologically what happens with shaken baby syndrome?
retinal hemorrhaging and subural hematomas occur due to increased brain swelling
are children more or less prone to acceleration and deceleration (whiplash) injuries? why?
more prone; children have higher water content in the brain
what causes secondary brain damage in patients with a TBI?
(1) hypoxia or ischemia
(2) seizures
(3) intracranial swelling
what is post-traumatic amnesia?
patient doesn’t remember the events surrounding the injury
what is retrograde amnesia?
not remembering things they learned prior to the injury
how are the pediatric Rancho levels of consciousness classified?
Level I: higher level response
Level II: agitated and confused
Level III-V: early or no response
what is down syndrome? although their are 3 types, which one is the most common?
(1) genetic disorder leading to intellectual disability
(2) Trisomy 21 is the most common cause
what type of instability should be screened in patients with down syndrome?
C1-C2 instability
what are some characteristics of patients with down syndrome?
(1) hypotonia
(2) ligamentous laxity
(3) joint hypermobility
(4) gross motor delay
(5) decreased strength
can pathological hypotonia be improved?
no; motor performance can be improved though. a physio ball can be used to facilitate co-contraction and improve motor performance
by what age do patients with down syndrome start exhibiting characteristics of Alzheimer’s?
35 y/o
due to the fact patients with down syndrome present with pes planus and over pronate, which orthotic would be ideal for them? why?
SMO; provides medial / lateral stability
what is developmental coordination disorder (DCD)? what causes it?
(1) a condition where the child is very clumsy, has poor motor planning, and proprioception (normal cognition)
(2) unknown cause
what are kids with DCD at increased risk for?
falls and obesity (less physically active)
what is cerebral palsy (CP)? what causes it?
(1) a group of disorders that is non-progressive with a motor disorders that can be accompanied by other complications
(2) genetic links; low birth weight is a risk factor, >80% of infants with PVL develop CP, IVH increases risk for CP
