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Pediatrics and Research BOARD REVIEW

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Title: Pediatrics and Research BOARD REVIEW


1
Pediatrics and Research BOARD REVIEW
2
Question
  • On examination, a 3-month-old girl still has a
    Moro reflex, asymmetric tonic neck reflex, and
    plantar grasp reflex. She does not have any
    protective extension. You advise her parents that
  • (a) further diagnostic evaluation is indicated.
  • (b) she requires a physical therapy evaluation.
  • (c) she needs a neurology evaluation.
  • (d) these reflexes are normal reflexes.

3
Answer
  • (d) These are normal reflexes in a 3-month-old
    child. The Moro and asymmetric tonic neck
    reflexes (ATNR) usually are integrated by
    approximately 6 months. The plantar grasp reflex
    is integrated by 12 to 14 months after walking
    has begun. Protective extension in sitting is
    seen anteriorly at 5 to 7 months, lateral at 6 to
    8 months, and posterior at 7 to 8 months.

4
Question
  • A 2-month-old infant presents to you for
    evaluation of delayed development. He was the
    product of a normal term pregnancy, labor, and
    delivery. Birth weight was 3500 grams. He has had
    difficulty feeding since birth. Family history is
    negative for developmental problems. On physical
    examination, he is awake, but not alert. Weight
    is 3600 grams. Respiration is unlabored. He has
    poor head control and decreased tone throughout.
    Deep tendon reflexes are absent. What is the most
    likely diagnosis?
  • (a) Kugelberg Welander syndrome
  • (b) Duchenne muscular dystrophy
  • (c) Infantile botulism
  • (d) Tetraplegic cerebral palsy

5
Answer
  • (d) This patient illustrates the diagnostic
    dilemma of the floppy infant. Causes of this
    problem include central nervous system lesions
    (both brain and spinal cord), myopathies,
    neuropathies, and neuromuscular junction
    problems. This infant has had abnormalities since
    birth, which argues against infantile botulism.
    Kugelberg Welander syndrome (also known as spinal
    muscular atrophy type 3) has onset during
    childhood, as does Duchenne muscular dystrophy.
    Tetraplegic cerebral palsy often presents in
    infancy with floppiness and hyporeflexia, which
    later change to spasticity and hyperreflexia.

6
Question
  • Professionalism is the basis of medicines
    contract with society. Which item is a
    fundamental principle of medical professionalism?
  • (a) Social justice
  • (b) Physician paternalism
  • (c) Patient disclosure
  • (d) Free enterprise

7
Answer
  • (a) According to the Charter on Medical
    Professionalism, there are 3 fundamental
    principles of medical professionalism. They are
    (1) the primacy of patient welfare, (2) patient
    autonomy, and (3) social justice.

8
Question
  • Your co-resident presents an article in journal
    club on a new medication and its impact on
    outcomes following traumatic brain injury. On
    which point would you NOT need assurance before
    you decide to use this medication in your
    clinical practice?
  • (a) That the research study results are
    clinically significant
  • (b) That bias was eliminated from the study
  • (c) That the research study results are
    statistically significant
  • (d) That research investigators used valid
    outcome measures

9
Answer
  • (b) When critically evaluating the medical
    literature, it is important to consider if the
    results of the study are both clinically and
    statistically significant. It is also important
    to consider whether the outcome assessment tools
    have been validated for both accuracy and
    reliability. While biases that may impact the
    outcome of the study also must be considered, it
    is often impossible to completely eliminate bias
    from the study.

10
Question
  • Which statement accurately characterizes a
    meta-analysis?
  • (a) It summarizes the results of randomized
    controlled trials.
  • (b) It summarizes the findings of an expert
    panel.
  • (c) It groups research on a particular topic area
    into 3 tiers.
  • (d) It summarizes findings of a single research
    protocol that is carried out a multiple centers.

11
Answer
  • (a) A meta-analysis summarizes the results of
    randomized controlled trials on a particular
    topic or research question. A consensus statement
    summarizes the findings of an expert panel. In
    developing a consensus statement and reviewing
    the literature, research studies are typically
    divided into 3 tiers based on the type of
    research performed. A multi-center study
    implements a particular research protocol at
    multiple centers at different institutions.

12
Question
  • Investigators must address ethical considerations
    when designing and implementing research studies.
    One such consideration requires investigators to
    design protocols that will provide generalizable
    knowledge and ensure that the benefits of the
    research are proportionate to the risks assumed
    by the subjects. This ethical consideration is
    referred to as
  • (a) respect.
  • (b) beneficence.
  • (c) justice.
  • (d) autonomy.

13
Answer
  • (b) Beneficence requires investigators to design
    protocols that will provide generalizable
    knowledge and ensure that the benefits of the
    research are proportionate to the risks assumed
    by the subjects.

14
Question
  • In instances where a researcher has financial
    investments in a company and is researching the
    effectiveness of one of the companys products,
    the researcher is obligated to
  • (a) terminate the investigation if the
    investigational agent is found to be not
    effective.
  • (b) disclose this involvement in writing to
    subjects that are being enrolled in the study.
  • (c) end financial involvement in the company
    before the results of the research are revealed
    to the public.
  • (d) disclose this involvement to the
    investigator's medical center, to funding
    organizations, and to journals publishing the
    results.

15
Answer
  • (d) Conflicts of interest in biomedical research
    are becoming more apparent as private companies
    increasingly develop relationships with academic
    research scientists. Avoidance of real or
    perceived conflicts of interest in clinical
    research is necessary if the medical community is
    to ensure objectivity and maintain individual and
    institutional integrity. Financial investments
    should only transpire outside of the time that
    the investigator is involved in any research
    activity and the results of the research are
    known to the public. If conflicts of interest
    exist, the investigator is obligated to disclose
    this involvement in writing to the investigator's
    medical center, organizations funding the
    research, and anytime that the research is
    presented or published.

16
Question
  • 149. A 6-month-old infant presents to you with
    hypotonia. You perform an electrodiagnostic study
    which shows normal motor conduction velocity,
    normal sensory conduction velocity and amplitude,
    normal motor units, and occasional fibrillations
    and positive waves. The most likely cause of
    these findings is
  • (a) congenital myotonic dystrophy.
  • (b) spinal muscular atrophy.
  • (c) metachromatic leukodystrophy.
  • (d) infantile botulism.

17
Answer
  • 149. (a) Hypotonia in infants can be caused by
    many abnormalities, including cerebral lesions,
    spinal cord pathology, polyneuropathies, and
    myopathies. These electrodiagnostic findings are
    most consistent with congenital myotonic
    dystrophy.

18
Question
  • In children with spastic cerebral palsy, which
    intervention strengthens weak muscles?
  • (a) Ankle-foot orthotics
  • (b) Tendon transfer surgery
  • (c) Intrathecal baclofen
  • (d) Functional training program

19
Answer
  • (d) Children with cerebral palsy often have
    weakness as part of their disorder. Treatments
    such as bracing, tendon lengthening or transfers,
    and medications such as botulinum toxin or
    intrathecal baclofen add to this weakness.
    Strengthening programs or functional training
    programs can help to strengthen weak muscles.

20
Question
  • One of your 4-year-old patients exhibits the
    following characteristics distress over minor
    changes in environment, echolalia, lack of
    awareness of the existence of feelings in others,
    nonparticipation in simple games. The most likely
    diagnosis is
  • (a) autism.
  • (b) cerebral palsy.
  • (c) hearing impairment.
  • (d) mental retardation.

21
Answer
  • (a) Autism is characterized by echolalia,
    inability to play reciprocally, and abnormal
    relationships with people. While children with
    mental retardation, cerebral palsy, and hearing
    impairment may have some of these features, they
    do not have all of them in the absence of autism.

22
Question
  • A 17-year-old boy from India presents with a
    longstanding history of areflexia and asymmetric
    muscular atrophy after a febrile illness as a
    child. The likeliest site of neurologic pathology
    is
  •  
  • (a) myelin sheath of peripheral nerves.
  • (b) axons of peripheral nerves.
  • (c) anterior horn cells.
  • (d) muscle membrane.

23
Answer
  • (c) Poliomyelitis involves the anterior horn
    cells. During an acute infection, the virus is
    transported to the anterior horn cells followed
    by inflammation and loss of spinal and bulbar
    motor neurons.

24
Question
  • Which finding is normal in newborn infants?
  • (a) Extensor tone predominates
  • (b) Hands are kept fisted
  • (c) Spine is straight when held in sitting
    position
  • (d) Unable to turn head to side in prone position

25
Answer
  • (b) In normal newborn infants flexor tone
    predominates and hands are kept fisted. In prone
    position a normal newborn is able to turn the
    head to either side. The newborn has a rounded
    spine when placed in supported sitting.

26
Question
  • A 10-year-old child with L4-5 myelodysplasia and
    shunted hydrocephalus develops spasticity in her
    legs. The most likely cause of this spasticity is
  • (a) shunt malfunction.
  • (b) symptomatic Chiari malformation.
  • (c) growth.
  • (d) tethered cord.

27
Answer
  • (d) Tethered cord is the most common cause of new
    onset spasticity in patients with myelodysplasia.
    Linear growth does not cause new spasticity.
    Symptoms of Chiari malformation include cranial
    nerve disorders and respiratory problems. Shunt
    malfunction may be associated with headaches,
    vomiting, eye muscle abnormalities, and sometimes
    abdominal symptoms.

28
Question
  • Which positive effect of ankle-foot orthotics has
    been proven beneficial in the treatment of
    children with cerebral palsy?
  • (a) Improved gait efficiency as measured by gait
    analysis
  • (b) Prevention of contractures
  • (c) Improved knee extensor strength
  • (d) Decreased plantar flexor posture

29
Answer
  • (a) There are no large, randomized, controlled
    studies that show the long-term effects of any
    type of Ankle-Foot Orthosis (AFO) on function or
    contracture formation. Small studies have shown
    that both rigid and hinged AFOs improve gait
    efficiency by preventing plantar flexion.

30
Question
  • A 5-year-old boy is brought to your office with
    pain in his left groin for the last 2 months. He
    has an antalgic gait and favors his left leg. He
    has been unable to play with his siblings because
    of pain and reports increased severity of the
    pain at night. There is no history of trauma.
    Passive range of motion of the left hip is
    extremely painful. Which of the following is most
    likely?
  • (a) The child is not telling the truth about the
    trauma for fear of being punished by his parents.
  • (b) A simple radiograph will demonstrate this
    problem to be a benign syndrome that only
    requires
  • monitoring.
  • (c) This child is at risk for permanent
    disability and treatment requires the use of an
    abduction
  • brace.
  • (d) You are very concerned about physical abuse
    by the parents, since the child is very quiet
    when the parents are in the examination room.

31
Answer
  • (c) This child has Legg-Calvé-Perthes disease.
    This is avascular necrosis of the femoral head
    (also known as idiopathic osteonecrosis of the
    femoral head) and typically affects children
    between the ages of 4 and 8 years. Boys are
    affected 4 times more than girls are, and it is
    unilateral in 90 of children. It is uncommon in
    African American children. The bone dies and
    loses its structural integrity, leading to
    collapse of the femoral head with deformity and
    arthritis.

32
Question
  • What condition casues the typical myopathic
    gait seen in a young boy with Duchenne muscular
    dystrophy with accentuated lumbar lordosis and
    toe walking?
  • (a) Hip and knee extensor weakness
  • (b) Hip flexion and ankle plantar flexion
    contractures
  • (c) Hip extensor weakness and plantar flexion
    contracture
  • (d) Hip flexion contracture and knee extensor
    weakness

33
Answer
  • (a) The typical myopathic gait seen in early
    Duchenne muscular dystrophy is caused by weakness
    of the gluteus maximus and quadriceps muscles. In
    order to maintain upright posture the child
    assumes the hyperlordotic stance. Contractures of
    the gastrocsoleus and iliopsoas muscles occur
    later in the disorder.

34
(No Transcript)
35
Question
  • The most useful clinical criterion to distinguish
    Becker muscular dystrophy from Duchenne
  • muscular dystrophy is ?
  • (a) creatine kinase values at the time of
    diagnosis.
  • (b) walking ability during the teen-age years.
  • (c) Gowers sign and calf enlargement.
  • (d) age at onset of diagnosis.

36
Answer
  • (b) The most useful clinical criterion to
    distinguish Becker muscular dystrophy (BMD) from
    Duchenne muscular dystrophy (DMD) is the
    continued ability of the patient to walk into
    late teen-age years. Persons with BMD will
    typically remain ambulatory beyond 16 years.
    Outlier DMD cases generally stop ambulating
    between 13 and 16 years of age. Creatine kinase
    values cannot be used to differentiate DMD from
    BMD. Calf enlargement and the presence of Gowers
    sign are a nonspecific findings. Studies have
    shown significant overlap in the observed age at
    onset between DMD and BMD.

37
Question
  • The leading cause of childhood disability is
  • (a) traumatic brain injury.
  • (b) spinal muscular atrophy.
  • (c) spina bifida.
  • (d) cerebral palsy.

38
Answer
  • (d) Cerebral palsy is the leading cause of
    childhood disability. The reported incidence is
    approximately 2-3 per 1,000 live births. The
    incidence of spina bifida is .5 per 1,000, of
    spinal muscular atrophy 1 in 25,000. The annual
    incidence for traumatic brain injury in children
    is 1-2 per 1,000. However, the great majority of
    cases are minor and result in no long-term
    disability. Approximately 15 of brain-injured
    children have moderate and severe injuries
    resulting in permanent impairment.

39
Question
  • The earliest weakness seen in skeletal muscle in
    Duchenne muscular dystrophy is located in
  • (a) knee extensors.
  • (b) hip flexors.
  • (c) neck flexors.
  • (d) ankle plantar flexors.

40
Answer
  • c. neck flexors

41
Question
  • The most common complication after amputation in
    the immature child is
  • (a) phantom limb pain.
  • (b) diffuse edema.
  • (c) terminal overgrowth.
  • (d) painful neuroma.

42
Answer
  • (c) Terminal overgrowth at the transected end of
    a long bone is the most common complication after
    amputation in the skeletally immature child. It
    occurs most frequently in the humerus, fibula,
    tibia, and femur, in that order. The oppositional
    growth may be so vigorous that the bone pierces
    the skin. The treatment of choice is surgical
    revision.
  •  

43
Question
  • The earliest marker of abnormal central nervous
    system maturation is ?
  • (a) diffuse fasciculations.
  • (b) gross motor delay.
  • (c) delay of postural responses.
  • (d) persistence of primitive reflexes.
  •  

44
Answer
  • (d) In neonates and young infants, motor
    behavior is influenced by primitive reflexes
    because of the immature central nervous system.
    These reflexes gradually become suppressed.
    Concurrently, more sophisticated postural
    responses emerge. Obligatory persistent primitive
    reflexes are the earliest markers of abnormal
    neurologic maturation.

45
Question
  • A parent of an 18-month-old child reports that
    the child babbled as an infant but became much
    quieter after about 8 months of age. She has no
    true words, though she will wave bye-bye. She
    follows no verbal commands but will follow
    occasional pantomime commands. Her gross and fine
    motor skills have been normal. The most likely
    diagnosis is ?
  • (a) autism.
  • (b) mental retardation.
  • (c) hearing impairment.
  • (d) oral motor apraxia.

46
Answer
  • (c) A history of delay in communication
    development raises several diagnostic
    possibilities, including true language
    dysfunction or a motor dysfunction or significant
    hearing loss. Infants with hearing loss start to
    fall behind after 6-8 months of age, when
    learning of auditory-dependent vocalization
    begins. Oral motor dysfunction is often
    associated with cerebral palsy, most often
    spastic quadriparesis. Difficulty with drinking
    from a cup and difficulty with the introduction
    of solid food are early symptoms of oral motor
    dysfunction. Autism is a spectrum disorder with
    qualitative abnormalities in communication and in
    social and behavioral realms.

47
Question
  • The most severe form of mental retardation in
    cerebral palsy occurs in association with
  • (a) spastic diplegia.
  • (b) ataxia.
  • (c) spastic quadriplegia.
  • (d) athetosis.

48
Answer
  • (c) Mental retardation is the most common serious
    associated disability in cerebral palsy. The
    overall incidence of mental retardation is
    approximately 30-50. Severe mental retardation
    is present in about one-half of the retarded
    group. Approximately one-third of cases have mild
    cognitive deficits. The greatest retardation is
    seen in rigid, atonic, and severe spastic
    quadriplegic cerebral palsy.

49
Question
  • A 7-year-old boy is referred for evaluation and
    management of gradually progressive pain located
    in the right medial thigh and knee, a limp, and
    limited painful hip motion. There was a similar
    episode 3 months before which resolved
    spontaneously. Of the following conditions, the
    most likely diagnosis is
  • (a) congenital hip dysplasia.
  • (b) slipped capital femoral epiphysis.
  • (c) Legg-Calvé-Perthes disease.
  • (d) epiphyseal fracture.

50
Answer
  • (c) Legg-Calvé-Perthes disease, avascular
    necrosis of the femoral head, occurs most
    commonly in boys between the ages of 4 and 8
    years. It is characterized by medial thigh,
    groin, and knee pain, is associated with a limp,
    and is often preceded by a transient episode of
    hip synovitis. Congenital hip displasia is
    usually apparent much earlier slipped capital
    femoral epiphysis occurs during periods of rapid
    growth, typically in adolescent boys, and may be
    preceded by trauma.

51
Question
  • In evaluating a hypotonic infant with
    electromyography you find low-amplitude,
    short-duration motor units with early
    recruitment. Based on these findings, the LEAST
    likely diagnosis would be
  • (a) central core disease.
  • (b) nemaline myopathy.
  • (c) type II glycogenosis (acid maltase
    deficiency).
  • (d) infantile spinal muscular atrophy.

52
Answer
  • (d) The motor unit changes noted are typically
    seen in myopathies. Spinal muscular atrophy is an
    anterior horn cell disease.
  •  

53
Question
  • In traumatic brain injury in children, outcome is
    primarily related to
  • (a) severity of original injury.
  • (b) location of injury.
  • (c) age at time of injury.
  • (d) associated injuries.

54
Answer
  • (a) Although there is considerable variability
    from case to case, outcome is primarily related
    to the severity of the injury.

55
Question
  • Which reflex is typically NOT seen in a normal
    4-month-old infant?
  • (a) Extremities extend on the face side as the
    head is turned to the side.
  • (b) Fingers flex when the palm is touched.
  • (c) Extremities extend to the direction of
    displacement when center of gravity is displaced.
  • (d) Shoulder abduction, and shoulder, elbow, and
    finger extension occur when the neck is
    suddenly extended.

56
Answer
  • (c) These options all describe reflexes. (a)
    asymmetric tonic neck reflex, (b) palmar grasp,
    and (d) Moroare seen until a baby is about 6
    months old. Protective extension or parachute
    reaction (c) does not appear until after 6
    months.

57
Question
  • Which statement is true regarding spinal cord
    injury without obvious radiologic abnormality in
    children?
  • (a) It most commonly occurs in lumbar rather than
    cervical injuries.
  • (b) There is a lower incidence in younger
    children.
  • (c) It is associated with larger head size and
    relatively weak neck muscles.
  • (d) Neurologic impairmen, if it occurs, is
    usually apparent within 2 to 4 hours post-injury.
  •  
  •  

58
Answer
  • (c) Spinal cord injury without obvious radiologic
    abnormality (SCIWORA) usually occurs in young
    children, is thought to be due to the relatively
    large head size and weak neck muscles, and motor
    abnormalities may not be apparent for up to
    several days. SCIWORA most commonly occurs in the
    cervical region.
  •  

59
Question
  • Which is the following represents the best
    description a cohort study?
  • A group examined at one point in time
  • 2 groups selected based on the presence or
    absence of an outcome.
  • A group followed over time
  • A group that is divided into an intervention
    versus a placebo.

60
  • C A group followed over time

61
Question
  • True or false A case control study, the
    investigator makes all of the measurements on a
    single occasion or within a short period of time.
  • ?

62
Answer
  • False
  • In a case control study, the investigator works
    backwards. They began by choosing one sample from
    a population of patients with the outcome (but
    cases) and another from a population without
    outcome (the control) then they compare the
    distribution levels of the predictor variables in
    the 2 samples to see which ones are associated
    with and might cause the outcome.
  • It is the cross sectional study where the
    investigator makes all of the measurements on a
    single occasion or within a short period of time.

63
Question
  • Acquired subluxation or dislocation of the hips
    in spastic cerebral palsy is usually due to
    muscular imbalance and pull of the
  • (a) hip flexors and tensor fascia lata.
  • (b) hip flexors and hip adductors.
  • (c) rectus femoris and hip abductors.
  • (d) tensor fascia lata and hip extensors.

64
Answer
  • (b) Strong hip flexor and adductor muscles can
    overpower weak extensors and abductors. Acquired
    hip dislocation can be prevented in some cases by
    release of spastic hip flexors and adductors.
  •  

65
Question
  • Your 15-year-old patient with Duchenne muscular
    dystrophy complains of new onset morning
    headaches. What is the most likely cause?
  • (a) Neck extensor tightness
  • (b) Hypercarbia
  • (c) Migraines
  • (d) Vision changes

66
Answer
  • (b) Migraines do not typically occur only in the
    morning. Neck extensor tightness usually occurs
    before the loss of ambulation in boys with
    Duchenne muscular dystrophy, which usually occurs
    before the age of 15 years. Vision changes
    usually do not cause morning headaches.
    Hypercarbia results from hypoventilation during
    sleep and is an early sign of impending
    respiratory failure.

67
Question
  • Which insult is the most likely cause of spastic
    diplegic cerebral palsy?
  • (a) Intrauterine stroke
  • (b) Hyperbilirubinemia in the neonatal period
  • (c) Postnatal intraventricular hemorrhage
  • (d) Perinatal asphyxia

68
Answer
  • (c) Spastic diplegic cerebral palsy occurs most
    commonly in premature infants who have had an
    intraventricular hemorrhage during the neonatal
    period. Intrauterine stroke causes hemiplegia.
    Neonatal hyperbilirubinemia most commonly causes
    athetosis. Birth asphyxia is more commonly
    associated with spastic quadriplegic cerebral
    palsy.

69
Question
  • A 6-month-old child presents in your office for
    rehabilitation assessment. She was born at full
    term. There was mild transient respiratory
    distress at birth. The patient was noted to be
    diffusely hypotonic at birth except for normal
    cranial nerves. There were no feeding issues once
    the respiratory distress resolved within 24
    hours. The baby has remained relatively hypotonic
    since birth. However, she has become very
    socially alert and aware and attempts to use her
    arms to reach for toys and pick up lightweight
    objects. She doesnt roll. She cannot sit except
    very briefly when propped and bearing weight
    through both arms with elbows extended. On
    examination, head circumference is normal, length
    is normal, as is weight. There is a pronounced
    head lag. Arms, while in the supine position,
    maintain a jug-handle posture. Reflexes are
    present but dimished There is no spasticity. The
    cranial nerves are normal except for fine
    fasciculations of the tongue.The most likely
    diagnosis is
  • (a) myotonic muscular dystrophy.
  • (b) cerebral palsy.
  • (c) infantile botulism.
  • (d) spinal muscular atrophy.

70
Answer
  • (d) Spinal muscular atrophy (SMA)is a term used
    to describe a group of inherited disorders
    characterized by weakness and muscle wasting due
    to degeneration of anterior horn cells of the
    spinal cord and brainstem motor nuclei. Three
    subtypes of autosomal recessive predominantly
    proximal SMA have been linked to chromosome 5q.
    The majority of cases of SMA type I present
    within the first 2 months of life with
    generalized hypotonia and symmetric weakness.
    Children typically sit only with support. Tongue
    fasciculations have been reported in 56-61 of
    patients. Proximal muscles are weaker than
    distal.

71
Question
  • What is the most common cause of traumatic brain
    injury in a child under the age of 1 year?
  • (a) Motor vehicle crash
  • (b) Near drowning in bath
  • (c) Inflicted injuries
  • (d) Fall from changing table

72
Answer
  • (d) For infants, more than two-thirds of all
    traumatic brain injuries result from falls only
    8 of these result in moderate or severe
    injuries. For preschool children, falls account
    for 51 of TBI and motor vehicle crashes for 22.
    For children 8 to 9 years of age, etiology of TBI
    is evenly divided between falls, sports, and
    recreational activities, and motor vehicle
    crashes.

73
Question
  • Secondary injury in pediatric brain trauma is
    caused by
  • (a) hypotension, hypoxia, and hydrocephalus.
  • (b) growing skull fractures.
  • (c) coup and contrecoup cerebral contusions.
  • (d) diffuse axonal injuries and punctate
    hemorrhages

74
Answer
  • (a) Any disorder that interferes with cerebral
    perfusion or oxygenation can cause further damage
    following traumatic brain injury. This includes
    hypotension, hypoxia, increased intracranial
    pressure because of cerebral edema, acute
    hydrocephalus, or space-occupying lesions.
    Midline shift or herniation may lead to
    infarction because of pressure or traction on
    cerebral vessels. Therefore, efforts are made to
    control intracranial pressure through fluid and
    electrolyte management, hyperventilation, and
    maintenance of normal blood pressure and
    oxygenation. Growing skull fractures result from
    the arachnoid protruding through a dural tear,
    producing a cyst that can contribute to a
    widening skull deficit, which usually requires
    operative repair. This is a complication of
    traumatic brain injury but not a secondary
    injury. Coup and contrecoup cerebral contusions
    and diffuse axonal injuries are examples of
    primary injury.

75
Question
  • To prevent contractures, which position is the
    correct placement for children with major burns?
  • (a) Shoulder in external rotation
  • (b) Wrist in extension
  • (c) Hip in flexion
  • (d) Metacarpophalangeal joints in hyperextension

76
Answer
  • (b) Children with major burn injuries should be
    placed in positions that tend to prevent
    contractures. These include neck extension (no
    pillows) shoulders at 90 abduction and neutral
    rotation with elbows, wrists, hips, and knees
    extended feet at neutral dorsiflexion,
    metacarpophalangeal joints at 70 to 90 flexion
    and finger interphalangeal joints in full
    extension.

77
Question
  • Bracing for curves developing after age 3, but
    before puberty onset (juvenile idiopathic
    scoliosis) should commence when the curve reaches
    approximately how many degrees?
  • (a) 15
  • (b) 25
  • (c) 40
  • (d) 60

78
Answer
  • (b) Unlike infantile idiopathic scoliosis, the
    juvenile type almost never spontaneously
    resolves, and owing to the many years of growth
    during which progression can take place,
    extremely severe curves can develop. Because of
    the very poor prognosis of this scoliosis, and
    the great desire to avoid fusion at a young age,
    bracing becomes an extremely important method of
    management. Therefore, the standard of care is to
    begin bracing when the curve reaches
    approximately 25. It is not necessary to brace
    curves less than 20, and curves as high as 60
    can still respond to a brace. This is a much
    higher value than for successful bracing of
    adolescent idiopathic scoliosis where the upper
    limit is 40 to 45.

79
Question
  • In children with spastic cerebral palsy, which
    intervention strengthens weak muscles?
  • (a) Ankle-foot orthotics
  • (b) Tendon transfer surgery
  • (c) Intrathecal baclofen
  • (d) Functional training program

80
Answer
  • (d) Children with cerebral palsy often have
    weakness as part of their disorder. Treatments
    such as bracing, tendon lengthening or transfers,
    and medications such as botulinum toxin or
    intrathecal baclofen add to this weakness.
    Strengthening programs or functional training
    programs can help to strengthen weak muscles.

81
Question
  • One of your 4-year-old patients exhibits the
    following characteristics distress over minor
    changes in environment, echolalia, lack of
    awareness of the existence of feelings in others,
    nonparticipation in simple games. The most likely
    diagnosis is
  • (a) autism.
  • (b) cerebral palsy.
  • (c) hearing impairment.
  • (d) mental retardation.

82
Answer
  • (a) Autism is characterized by echolalia,
    inability to play reciprocally, and abnormal
    relationships with people. While children with
    mental retardation, cerebral palsy, and hearing
    impairment may have some of these features, they
    do not have all of them in the absence of autism.
  •  

83
Question
  • During discussion with the parents of a
    2-year-old leukemic patient, you inform them that
    significant brain irradiation almost uniformly
    produces
  • (a) attention deficits.
  • (b) focal motor weakness.
  • (c) ataxia.
  • (d) anosmia.

84
Answer
  • (a) An adverse sequela of intensive pediatric
    anticancer therapy is learning difficulty.
    Impaired learning can exert a deleterious
    long-term impact. Whole brain irradiation for
    leukemic prophylaxis results in enlarged cerebral
    sulci and ventriculomegaly on cranial
    imaging.Clinical
  • symptomatology roughly correlates with scan
    findings. Virtually all patients in whom a
    substantial portion of the brain is radiated
    complain of memory loss and attentional deficits.

85
Question
  •  
  • A 17-year-old boy from India presents with a
    longstanding history of areflexia and asymmetric
    muscular atrophy after a febrile illness as a
    child. The likeliest site of neurologic pathology
    is
  •  
  • (a) myelin sheath of peripheral nerves.
  • (b) axons of peripheral nerves.
  • (c) anterior horn cells.
  • (d) muscle membrane.
  •  

86
Answer
  • (c) Poliomyelitis involves the anterior horn
    cells. During an acute infection, the virus is
    transported to the anterior horn cells followed
    by inflammation and loss of spinal and bulbar
    motor neurons.

87
Question
  • Which finding is normal in newborn infants?
  • (a) Extensor tone predominates
  • (b) Hands are kept fisted
  • (c) Spine is straight when held in sitting
    position
  • (d) Unable to turn head to side in prone position

88
Answer
  • (b) In normal newborn infants flexor tone
    predominates and hands are kept fisted. In prone
    position a normal newborn is able to turn the
    head to either side. The newborn has a rounded
    spine when placed in supported sitting.

89
Question
  • Which measure is the first sign of respiratory
    muscle dysfunction in boys with Duchenne muscular
    dystrophy?
  • (a) Vital capacity
  • (b) Oxygen saturation
  • (c) Maximal expiratory force
  • (d) Negative inspiratory force

90
Answer
  • (c) Recent studies by McDonald and by Bach showed
    that reduction of maximal expiratory force (MEF)
    to 4060 of normal in the 7- to 14-year-old age
    group was the first sign of respiratory muscle
    dysfunction in boys with Duchenne muscular
    dystrophy (DMD). The earlier and more severe
    decreases of MEF that are greater than the
    decreases in maximal inspiratory force,
    correspond to the clinically observed weakness of
    abdominal muscles, which like coughing are
    important in forced expiration. Vital capacity
    was not found to decrease until an average of
    1516 years. Low oxygen saturation is a late
    manifestation in DMD, developing after
    hypercapnia.
  •  

91
Question
  • A 10-year-old child with L4-5 myelodysplasia and
    shunted hydrocephalus develops spasticity in her
    legs. The most likely cause of this spasticity is
  • (a) shunt malfunction.
  • (b) symptomatic Chiari malformation.
  • (c) growth.
  • (d) tethered cord.

92
Answer
  • (d) Tethered cord is the most common cause of
    new onset spasticity in patients with
    myelodysplasia. Linear growth does not cause new
    spasticity. Symptoms of Chiari malformation
    include cranial nerve disorders and respiratory
    problems. Shunt malfunction may be associated
    with headaches, vomiting, eye muscle
    abnormalities, and sometimes abdominal symptoms.

93
Question
  • Juvenile rheumatoid arthritis (JRA) differs from
    adult onset rheumatoid arthritis in JRA
  • (a) joint destruction occurs earlier.
  • (b) large joint involvement is less frequent.
  • (c) the cervical spine is involved less
    frequently.
  • (d) systemic features are more common.

94
Answer
  • (d) Children with juvenile rheumatoid arthritis
    are more likely to have systemic features, have
    large joints involved, and have cervical spine
    involvement. Adults with rheumatoid arthritis
    have joint destruction earlier.

95
Question
  • Children with which physical disorder tend to
    have higher verbal skills compared to overall
    cognitive ability?
  • (a) Muscular dystrophy
  • (b) Myelodysplasia
  • (c) Cerebral palsy
  • (d) Autism

96
Answer
  • (b) Children with myelodysplasia have deceptively
    good verbal facility that creates the impression
    of higher intellectual functioning than is found
    on formal testing (cocktail party syndrome).
    Children with cerebral palsy, autism, and
    muscular dystrophy do not typically demonstrate
    this finding.

97
Question
  • A 5-year-old boy is brought to your office with
    pain in his left groin for the last 2 months. He
    has an antalgic gait and favors his left leg. He
    has been unable to play with his siblings because
    of pain and reports increased severity of the
    pain at night. There is no history of trauma.
    Passive range of motion of the left hip is
    extremely painful. Which of the following is most
    likely?
  • (a) The child is not telling the truth about the
    trauma for fear of being punished by his parents.
  • (b) A simple radiograph will demonstrate this
    problem to be a benign syndrome that only
    requires monitoring.
  • (c) This child is at risk for permanent
    disability and treatment requires the use of an
    abduction brace.
  • (d) You are very concerned about physical abuse
    by the parents, since the child is very quiet
    when the parents are in the examination room.
  •  

98
Answer
  • This child has Legg-Calvé-Perthes disease. This
    is avascular necrosis of the femoral head (also
    known as idiopathic osteonecrosis of the femoral
    head) and typically affects children between the
    ages of 4 and 8 years. Boys are affected 4 times
    more than girls are, and it is unilateral in 90
    of children. It is uncommon in African American
    children. The bone dies and loses its structural
    integrity, leading to collapse of the femoral
    head with deformity and arthritis. An abduction
    brace is worn all day in an attempt to position
    the femoral head in the acetabulum. This will
    hopefully lead to a more spherical head of the
    femur. The femoral head can revascularize and
    remodel, but this occurs over several months.
    Osteotomy is usually reserved for older children.
  •  

99
Question
  • What condition casues the typical myopathic
    gait seen in a young boy with Duchenne muscular
    dystrophy with accentuated lumbar lordosis and
    toe walking?
  • (a) Hip and knee extensor weakness
  • (b) Hip flexion and ankle plantar flexion
    contractures
  • (c) Hip extensor weakness and plantar flexion
    contracture
  • (d) Hip flexion contracture and knee extensor
    weakness

100
Answer
  • (a) The typical myopathic gait seen in early
    Duchenne muscular dystrophy is caused by weakness
    of the gluteus maximus and quadriceps muscles. In
    order to maintain upright posture the child
    assumes the hyperlordotic stance. Contractures of
    the gastrocsoleus and iliopsoas muscles occur
    later in the disorder.

101
Question
  • The most common spinal problem seen with
    achondroplasia during childhood is
  • (a) kyphosis.
  • (b) scoliosis.
  • (c) spinal stenosis.
  • (d) low back pain.

102
Answer
  • (a) While scoliosis may occur in children with
    achondroplasia, it is less common than kyphosis,
    which begins in infancy. Spinal stenosis occurs
    frequently in individuals with achondroplasia,
    with 38 years being the average age of symptom
    onset. Low back pain is extremely frequent in
    adults with achondroplasia, but rare in children.
    Progressive kyphosis that occurs in infants and
    young children with achondroplasia is treated
    with a spinal orthosis.

103
Question
  • What is the pathophysiology of Duchenne muscular
    dystrophy?
  • (a) merosin deficiency
  • (b) abnormally low levels of dysferlin
  • (c) absence of dystrophin
  • (d) mutations of alpha-sarcoglycan

104
Answer
  • (c) The absence of dystrophin is the basis of the
    pathophysiology of Duchenne muscular dystrophy
    (DMD). Most genes in the affected area of the X
    chromosome encode for components of the
    dystrophin-glycoprotein complex (DGC), an
    assembly of transmembrane and membrane-associated
    proteins that form a structural linkage between
    the F-actin cytoskeleton and the extracellular
    matrix in muscle. The proteins that comprise the
    DGC are organized into 3 subcomponents, the
    cytoskeletal proteins, the sarcoglycans and the
    sarcospan. Many of the different types of
    muscular dystrophies arise from primary mutations
    in genes encoding components of this complex.
    However, the other choices noted above are not
    involved in DMD or Becker MD. Deficiencies in
    those proteins are associated with forms of limb
    girdle muscular dystrophy.

105
Question
  • Your 6-month-old patient had burns to his head
    and both arms in a house fire. What approximate
    percent of his total body surface area (TBSA) was
    burned?
  • (a) 37
  • (b) 18
  • (c) 27
  • (d) 49

106
Answer
  • (a) An infants head is approximately 19 and
    each arm constitutes 9 of the total body surface
    area (TBSA). In adults and older children the
    head is approximately 9 of the TBSA.

107
Question
  • Which joints are most commonly involved in
    juvenile rheumatoid arthritis?
  • (a) Shoulder, hip, fingers
  • (b) Atlantoaxial, costomanubrum, hip
  • (c) Sternomanubrum, shoulder, sacroiliac
  • (d) Elbow, hip, temporomandibular

108
Answer
  • (d) The elbow is involved 90 of the time in
    juvenile rheumatoid arthritis (JRA), the
  • temporomandibular and hip 50 each. The
    shoulder is involved about 8 of the time in
    early JRA and about 33 later.

109
Question
  • You are asked to evaluate an 8-month-old child
    with developmental delay. On exam you find low
    tone, but brisk deep tendon reflexes at the knees
    and biceps, full passive range of motion, and
    poor head and trunk control. This childs
    diagnosis is likely
  • (a) myotonic dystrophy.
  • (b) cerebral palsy.
  • (c) spinal muscular atrophy.
  • (d) Hunters syndrome.

110
Answer
  • (b) This patient presents with hypotonia,
    weakness, and hyperreflexia, a combination most
    commonly seen in central nervous system lesions
    such as cerebral palsy. A child with a
    neuromuscular disorder would not have
    hyperreflexia with the hypotonia and weakness. In
    severe cerebral palsy it is common to see early
    hypotonia with brisk reflexes that changes to
    hypertonia as the child gets older.

111
Question
  • Which endocrine abnormality is most likely to
    occur 5 years after severe traumatic brain injury
    in a 2-year-old girl?
  • (a) Diabetes insipidus
  • (b) Precocious puberty
  • (c) Hypothyroidism
  • (d) Hyperparathyroidism

112
Answer
  • (b) Precocious puberty occurs in up to 50 of
    girls who sustain a severe traumatic brain injury
    (TBI) in early childhood. Diabetes insipidus is
    an early complication of TBI. While other
    endocrine abnormalities may occur, they are less
    common.

113
Question
  • What percentage of American children with
    myelomeningocele requires a shunt to manage
    hydrocephalus?
  • (a) 1020
  • (b) 2550
  • (c) 6070
  • (d) 8090

114
Answer
  • (d) Seventy-five percent of lesions in spina
    bifida cystica (myelomeningocele) affect the
    lumbosacral spine. Ninety percent of children
    with spina bifida have hydrocephalus that
    requires a shunt for management.

115
Question
  • A 16-year-old with Duchenne muscular dystrophy
    presents to your office with a 3-month history of
    worsening shortness of breath and pressure-like
    chest pain. His pulmonary function testing has
    not changed significantly. The most likely cause
    for his complaints is
  • (a) decreased cardiac output.
  • (b) bacterial pneumonia.
  • (c) pulmonary embolus.
  • (d) aspiration pneumonia.

116
Answer
  • (a) Given that his pulmonary function has not
    changed, the most likely cause for his shortness
    of breath is cardiac decompensation due to
    cardiomyopathy. Respiratory failure due to
    neuromuscular weakness would typically correspond
    to worsening of pulmonary function testing.
    Bacterial and aspiration pneumonia would both
    present with much more acute symptoms. Boys and
    men with Duchenne muscular dystrophy rarely get
    deep venous thromboses or pulmonary emboli. If
    left untreated, the patient will likely develop
    congestive heart failure from cardiomyopathy. The
    decreased cardiac output usually responds to
    treatment with digoxin and ACE-inhibitors.

117
Question
  • A normal 6-month-old infant may demonstrate which
    reflex?
  • (a) Rooting
  • (b) Automatic walking
  • (c) Plantar grasp
  • (d) Posterior protective extension

118
Answer
  • (c) Rooting and automatic walking reflexes are
    present at birth, and are integrated by 4 months
    of age. Posterior protective extension does not
    appear until 7 to 8 months of age. Plantar grasp
    is present at birth and not integrated until
    after independent walking occurs at approximately
    12 months of age.
  •  
  • Reflex Age of Emergence Age of Suppression (or
  • Integration)
  • Moro Birth 46 months
  • Rooting Birth 4 months
  • Asymmetric tonic neck reflex (ATNR)
  • 13 months 67 months
  • Plantar grasp Birth 1214 monthsie, when walking
    well
  • Automatic walking Birth 34 months
  • Posterior protective extension 78 months

119
Question
  • Which statement is TRUE about swallowing in
    infants?
  • (a) Sucking and swallowing are well-coordinated
    for oral intake by 34 weeks of gestation.
  • (b) The infants larynx is low, about the level
    of the sixth cervical vertebra.
  • (c) The infants tongue fills less of the oral
    cavity than the adults tongue.
  • (d) Oral breathing occurs at birth and may
    interfere with sucking.

120
Answer
  • (a) Sucking and swallowing are well-coordinated
    at 34 weeks gestation. Because the infants
    tongue is more anterior than the adults, the
    tongue fills more of the oral cavity than in the
    adult. The larynx in the infant is high, about at
    the C23 level. Newborn infants are obligate nose
    breathers and oral breathing is not observed
    until 3 to 4 months of age.
  •  

121
Question
  • You are performing a consult on an 8-year-old
    child who has sustained a traumatic brain injury.
    The child has hyperthermia, hypertension,
    tachycardia, and rigidity. The best management
    for this child would be
  • (a) Propranolol (Inderal).
  • (b) Baclofen (Lioresal).
  • (c) Nonsteroidal anti-inflammatory drugs
    (NSAIDs).
  • (d) Amantadine (Symmetrel).

122
Answer
  • (a) Fever in a child with a severe traumatic
    brain injury should be investigated and
    infections treated with appropriate antibiotics.
    In the absence of infection, the fever in central
    autonomic dysfunction is poorly responsive to
    nonsteroidal anti-inflammatory drugs. Baclofen
    may help to control the spasticity, but
    propranolol is more effective in controlling the
    hypertension, tachycardia, and hyperthermia.

123
Question
  • The Education for All Handicapped Children Act
    (EHA, PL 94-142), passed in 1976, and the
    Individuals with Disabilities Education Act
    (IDEA, PL 105-17), passed in 1997, guarantee that
    children with disabilities have
  •  
  • (a) education in special schools.
  • (b) medical care at school.
  • (c) education in the least restrictive
    environment.
  • (d) education in regular classrooms.
  •  

124
Answer
  • c) The Education for All Handicapped Children Act
    and the Individuals with Disabilities Education
    Act guarantee children with disabilities
    education in the least restrictive environment.
    They also guarantee necessary health care be
    provided in the school environment (eg,
    intermittent
  • catheterization) but do not require medical
    care be provided.
  •  

125
Question
  • Randomized controlled trials examining
    intrathecal baclofen (ITB) use in children with
    cerebral palsy show that children who receive ITB
    have
  • (a) improved upper extremity function.
  • (b) reduced spasticity in lower extremities.
  • (c) improved walking and transfers.
  • (d) improved knee range of motion.

126
Answer
  • (b) A comprehensive review of published English
    language studies on intrathecal baclofen (ITB)
    showed evidence of statistically significant
    improvement in upper and lower extremity tone
    with ITB use in children with cerebral palsy
    (CP). Other reported improvements with ITB in
    children with CP are either anecdotal or not
    substantiated by randomized controlled trials.
  •  

127
Question
  • The most common congenital limb deficiency is a
  • (a) transverse tibial and fibular limb deficiency
    (below-knee limb deletion).
  • (b) transverse transmetacarpal limb deficiency
    (partial hand deletion).
  • (c) longitudinal fibular deficiency (fibular
    hemimelia).
  • (d) transverse radial limb deficiency
    (below-elbow limb deletion).

128
Answer
  • (d) The left short transradial congenital limb
    deficiency (below-elbow limb deletion) is the
    most common congenital limb deficiency. It is
    thought to be caused by a clot which occludes the
    artery, resulting in resorption of the distal
    limb, often leaving nubbins of fingers at the end
    of the stump.

129
Question
  • 46. You are covering for your partner who is a
    pediatric physiatrist. Today, you are admitting a
    2-year old girl who sustained a burn. You suspect
    that this girl is a victim of a non-accidental
    injury (ie,child abuse) because you see
  • (a) non-symmetrical burns.
  • (b) splash marks in a scald-type injury.
  • (c) injuries in various stages of healing.
  • (d) non-uniform burn depth.

130
Answer
  • (c) Non-accidental injuries account for
    approximately 10 to 28 of pediatric burns. Of
    all nonaccidental injuries experienced by
    children, 10 are due to burns. Among abused
    children 30 to 70 suffer a repeat injury. Signs
    of abuse include injury that does not correlate
    with the type and location of injury observed on
    examination uniform burn depth sharp lines of
    demarcation between burned and non-burned areas
    symmetrical wounds (eg, in a stocking or glove
    pattern) an absence of splash marks in scald
    injuries the presence of other injuries in
    various stages of healing.

131
Question
  • A 6-month-old infant presents to you with
    hypotonia. You perform an electrodiagnostic study
    which shows normal motor conduction velocity,
    normal sensory conduction velocity and amplitude,
    normal motor units, and occasional fibrillations
    and positive waves. The most likely cause of
    these findings is
  • (a) congenital myotonic dystrophy.
  • (b) spinal muscular atrophy.
  • (c) metachromatic leukodystrophy.
  • (d) infantile botulism.

132
Answer
  • (a) Hypotonia in infants can be caused by many
    abnormalities, including cerebral lesions, spinal
    cord pathology, polyneuropathies, and myopathies.
    These electrodiagnostic findings are most
    consistent with congenital myotonic dystrophy.

133
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