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QOD Development

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Title: QOD Development


1
QOD Development
2
  • During the health supervision visit for an
    18-month-old boy, his parents express concern
    that he is vocalizing but not saying any real
    words. He is holding a small piece of string that
    he moves back and forth repeatedly. When you call
    his name, he does not respond. You point to the
    light in the room and say "look," but he
    continues to look at the string with a sideways
    glance. You try to get him to look at you, but he
    avoids eye contact.

3
Of the following, the MOST likely diagnosis for
this boy is
  1. Asperger disorder
  2. autistic disorder
  3. expressive/receptive language disorder
  4. obsessive-compulsive disorder
  5. Rett syndrome

4
Question 1 Answer B
  • The child described in the vignette shows
    clinical features of an autistic disorder, a
    heterogeneous neurodevelopmental disorder (see
    Table 1 at http//pediatrics.aappublications.org/c
    gi/content/full/120/5/1183 for complete
    diagnostic criteria). Affected individuals have
    impairments in three specific areas reciprocal
    social interactions, verbal and nonverbal
    communication, and range of activities or
    interests. The clinical presentation is specific
    to the child, with differing degrees of
    impairment in each of the three core symptom
    areas.The hallmark of autism is abnormal social
    interactions. Children lack the ability to share
    interests with others (joint attention skills)
    using verbal or nonverbal communication. They
    commonly show weakness in eye contact. Their
    interaction may range from aloofness and an
    unawareness of other people to having varied or
    odd interaction with others. Language development
    commonly is delayed, and children may have
    immediate or delayed echolalia, unusual
    intonation, and repetitive speech. Children who
    have autism may engage in repetitive play and
    show little imaginative play. They may focus on
    sensory aspects of objects or develop obsessions
    about unusual objects (stop signs, elevators).
    They often have difficulty handling transitions
    and may engage in repetitive hand or body
    movements. Many affected children have cognitive
    impairment. Children who have subthreshold
    clinical features (some but not all of the
    features) may receive the diagnosis of pervasive
    development disorder-not otherwise specified
    within the autism spectrum.Red flags of
    development that warrant further evaluation for
    possible autism include
  • No babbling by 9 months
  • No gesturing by 12 months
  • No single words by 16 months
  • No functional nonecholalic 2-word phrases by 24
    months
  • Any loss of language or social skills at any age
  • Neither a diagnosis of expressive language
    disorder nor obsessive-compulsive disorder would
    account for the impaired social engagement
    exhibited by the boy in the vignette. Individuals
    who have Asperger syndrome (Asperger disorder in
    DSM-IV TR) have impairments in social interaction
    and restricted interests and activities, but they
    have relatively preserved cognitive and language
    functioning, in contrast to the delays in
    developing language reported for the child in the
    vignette. Rett syndrome almost exclusively
    affects females and presents with a slowing of
    motor development between 6 and 18 months of age.
    Between ages 1 and 4 years, the child exhibits a
    decline in social interactions, cognitive
    abilities, purposeful hand movements, and
    speech.American Board of Pediatrics Content
    Specification
  • Know the clinical features of autism spectrum
    disorder

5
  • During the health supervision visit for a
    4-year-old girl, her father reports that she has
    developed a stutter over the past 9 months. He
    explains that she is a little frustrated by the
    difficulty in expressing herself but otherwise
    seems happy and well-adjusted. In talking with
    the father, you also note that he has a mild
    stutter. He speaks to the child slowly and
    deliberately and encourages her to take her time
    when speaking to you.

6
Of the following, the risk factor that MOST
strongly suggests the need for speech therapy for
this girl is the
  1. age of onset
  2. child's reaction to stuttering
  3. child's sex
  4. father's stutter
  5. time since onset

7
Question 2 Answer D
  • The onset of stuttering typically occurs during
    the period of intense speech and language
    development as the child progresses from two-word
    utterances to the use of complex sentences,
    generally between the ages of 2 and 5 years but
    sometimes as early as 18 months. About 5 of all
    children go through a period of stuttering that
    lasts 6 months or more. The sex ratio for
    stuttering appears to be equal at the onset of
    the disorder, but studies indicate that three to
    four times as many boys continue to stutter.
    Between 75 and 80 of children who start to
    stutter stop within 12 to 24 months without
    speech therapy. Children who begin stuttering
    before the age of 3½ years and girls are more
    likely to outgrow stuttering. Strong evidence
    shows that almost all children who stutter have a
    family member who stutters.Studies have shown
    that individuals who stutter have a large degree
    of within-word dysfluencies compared with their
    typical peers. Examples are 1) repetition of
    individual sounds or syllables such as
    "W-w-w-what is he doing?", 2) prolongation of
    words such as "Wwwwwwhat is he doing?, and 3)
    blocks or pauses such as ". . . What is he
    doing?" Such dysfluencies may be associated with
    eyelid closing and physical tension around the
    lips. The standard criteria for diagnosing a
    child who has stuttering or is at risk to develop
    stuttering is an average of at least three
    within-word dysfluencies in 100 words of
    conversation.Although the girl described in the
    vignette does not have a strong negative reaction
    to her speech impediment, her father stutters,
    placing her at risk for problematic stuttering
    and indicating the need for speech therapy.
    Criteria for referring children who stutter for
    speech therapy are presented in (Item C107).
    Referral should be made for children who have
    severe stuttering problems, those who have mild
    stuttering problems that do not improve markedly
    within 6 to 8 weeks, or those whose parents are
    very concerned. Referral also is indicated for
    children who have associated behaviors (eg, head,
    body, or limb movement audible breathing prior
    to the disfluencies and observable muscle
    tension in the orofacial region). In time, such
    children may develop anxiety about stuttering
    that could contribute to the chronicity of the
    disorder.American Board of Pediatrics Content
    SpecificationKnow the indications for referral
    of a child to a speech pathologist for evaluation
    of stuttering

8
Item C107
9
  • The parents of a newborn in whom a congenital
    severe-profound hearing loss has been diagnosed
    are seeking guidance about how to promote their
    infant's language development. You recommend
    enrollment in an early intervention program and
    obtaining hearing aids when the infant is young.
    His parents have never been exposed to an
    individual who has a hearing loss. They ask your
    opinion on the best approach for him to learn
    language.

10
Of the following, the MOST important factor in
language development of an infant or young child
who has hearing loss is the use of
  1. a high amount of verbal/nonverbal (gestures)
    communication between parents and child
  2. an oral-aural method emphasizing the teaching of
    speech and the use of a child's residual hearing
  3. hand-cued speech (using combined speech and hand
    cues)
  4. manually coded English between parents and child
  5. the bilingual (ASL and English)-bicultural
    (hearing and deaf culture) approach

11
Question Answer A
  • Multiple factors contribute to a family's
    decisions regarding their child who has confirmed
    hearing loss. Language-based early intervention
    results in superior expressive and receptive
    communication abilities compared with those of
    children identified at later stages of
    development. Family involvement, including verbal
    and nonverbal (gestures) communication, has a
    more significant positive effect on language
    development than any specific type of
    intervention.Hand-cued speech
    (www.cuedspeech.org) consists of eight different
    handshapes (represent consonants) and four
    different hand locations around the speaker's
    face (represent vowels). Manually coded English
    employs a visual representation of the spoken
    English language. Signs and finger spelling are
    used to represent spoken English.The goal of
    the oral-aural method of communication is to
    develop spoken language and gain inclusion in the
    mainstream both in school and society. People who
    employ this auditory-oral approach use their
    aided residual hearing as well as reading of
    speech, facial expressions, and naturally
    occurring gestures. Such an approach requires
    consistent use of hearing aids/FM technology and
    provision of auditory training and speech therapy
    as well as lip-reading.The bilingual-bicultural
    (Bi-Bi) philosophy of the National Association of
    the Deaf (www.nad.org) promotes communication in
    two languages (visual and a form of spoken) for
    children who are deaf so they can be a part of
    both deaf and hearing communities. The Bi-Bi
    approach supports early language learning through
    American Sign Language (ASL) and a form of spoken
    English taught as a second language later in
    elementary school.Of note, children who have
    hearing loss of less than 90 dB usually benefit
    from conventional amplification systems. For
    children who have severe-to-profound
    sensorineural hearing loss affecting both ears,
    cochlear implantation may be considered. This
    procedure leads to the ability to perceive sound
    signals. Although results are highly variable,
    the procedure is most successful when undertaken
    early in life.American Board of Pediatrics
    Content Specification
  • Know the major approaches to education of the
    deaf child

12
  • A well-nourished, healthy child comes to your
    office for his 2-year health supervision visit.
    You find that he has failed an autism-specific
    screening tool (the Modified Checklist for Autism
    in Toddlers) completed by his parents while in
    the waiting room. Results of a previous audiology
    evaluation are normal. His head circumference and
    growth parameters are normal. Findings on a
    general physical examination are unremarkable,
    and the neurologic examination produces nonfocal
    findings. Muscle mass, strength, and tone are
    within normal limits. His deep tendon reflexes
    are physiologic and symmetric.

13
Of the following, the MOST appropriate next step
is
  1. early intervention referral
  2. genetics evaluation
  3. head MRI
  4. metabolic evaluation
  5. sleep-deprived EEG

14
Question 4 Answer A
  • The boy described in the vignette has failed The
    Modified Checklist for Autism in Toddlers
    (M-CHAT). The M-CHAT is an autism-specific
    screening tool that has a sensitivity of 0.85 and
    a specificity of 0.93. The sensitivity measures
    the percentage of individuals correctly
    identified as having autism (85). The
    specificity measures the percentage of
    individuals who are correctly identified as not
    having autism (93). The results described for
    the boy in the vignette suggest that he has
    autism, and the clinician should not wait to
    confirm the diagnosis before referring the child
    for early intervention services. Such services
    are beneficial in addressing the child's
    deficits. If the diagnosis is confirmed, the
    interventions may be altered to a more specific
    intervention for autism spectrum disorder
    (ASD).ASDs are biologically based
    neurodevelopmental disorders. Although genetic
    evaluation may be indicated, especially when
    there is a family history of autism, referral for
    early intervention is more likely to improve the
    child's prognosis than genetic evaluation. Some
    of the conditions that are associated with autism
    include fragile X syndrome, tuberous sclerosis,
    fetal alcohol syndrome, Angelman syndrome, and
    Rett syndrome. There are only a few reports of
    mitochondrial or metabolic abnormalities being
    associated with ASD, and most affected children
    have normal immune function. No relationship has
    been demonstrated between autism and the measles,
    mumps, rubella vaccine or thimerosal.There is
    no evidence that sleep-deprived
    electroencephalography and magnetic resonance
    imaging for children who have ASDs is needed in
    the absence of specific clinical findings (eg,
    seizures, hypopigmented macules). Because
    metabolic abnormalities rarely are associated
    with autism, metabolic evaluation also is not
    warranted.American Board of Pediatrics Content
    Specification
  • Know the medical causes of autism spectrum
    disorder

15
  • A baby girl for whom you provide care has been
    referred on her newborn hearing screen. Physical
    examination results are within normal limits.
    When she is referred on a follow-up hearing
    screen at 2 weeks of age, you recommend auditory
    brainstem response testing, which subsequently
    reveals absent hearing in both ears. A careful
    family history is negative for any individuals
    who have deafness. The parents ask you what could
    have caused deafness in their baby.

16
Of the following, the likelihood that this infant
has a genetic cause for deafness is CLOSEST to
  1. lt1
  2. 5
  3. 25
  4. 50
  5. 75

17
Question 5 Answer D
  • The incidence of prelingual, moderate-to-profound
    sensorineural hearing loss (40 dB) is 1 in 500
    in developed countries, making it the most common
    birth defect in these regions. Approximately 1 in
    1,000 newborns is deaf (hearing loss in the
    severe-to-profound range of 71 to 90 dB).Fifty
    percent of all prelingual, moderate-to-profound
    hearing loss is genetic, 25 is nongenetic (eg,
    having bacterial and viral causes), and 25 is
    idiopathic (Item C183). Of the genetic forms of
    deafness, 30 are syndromic, and 70 are
    nonsyndromic. Syndromic causes of deafness
    include conditions such as Usher, Waardenburg,
    and Treacher-Collins syndromes. Nonsyndromic
    causes can be autosomal recessive (75 to 85),
    autosomal dominant (15 to 24), and X-linked
    (1 to 2).A critical aspect of the evaluation
    of any child who has prelingual hearing loss is
    defining the type of loss as clearly as possible.
    Careful audiologic evaluation should be
    undertaken in concert with examination by an
    otolaryngologist. Affected individuals should
    undergo computed tomography scan of the temporal
    bones to look for malformations of the inner ear.
    The information gleaned from these studies helps
    to guide the evaluation because different gene
    mutations are associated with different clinical
    manifestations. It is important to obtain a
    careful, three-generation pedigree, with
    attention to any individuals who have hearing
    loss, vision loss, dysmorphic features, early
    death, birth defects, and intellectual
    disabilities. Referral to a geneticist is
    important to determine if there is a recognizable
    pattern of features or any genetic testing that
    could be helpful.The gene loci for nonsyndromic
    deafness are designated "DFN" (for DeaFNess). The
    loci are categorized as DFNA (autosomal
    dominant), DFNB (autosomal recessive), and DFN
    (X-linked). Some genes can be involved in both
    dominant and recessive forms of deafness,
    depending on the specific mutation(s), and some
    mutations cause both sensorineural and conductive
    hearing loss. Although there are exceptions, most
    autosomal dominant loci cause postlingual hearing
    impairment, and most autosomal recessive loci
    cause severe-to-profound prelingual deafness.
    X-linked (DFN) loci are associated with both pre-
    and postlingual hearing loss.Because the many
    molecular genetic testing options can be somewhat
    confusing, consultation with a clinical
    geneticist is strongly recommended. Numerous
    laboratories offer panels of common mutations. In
    many populations worldwide, approximately 50 of
    individuals who have prelingual, autosomal
    recessive nonsyndromic hearing loss have GJB2
    mutations the remainder may have a mutation in
    any number of genes that may have been described
    as causing deafness in only one or two
    families.American Board of Pediatrics Content
    SpecificationKnow the common causes of
    congenital deafness

18
Item C183
(Item C183)                                      
                                             
(Reprinted with permission from
(Item C183)                                      
                                             
19
  • A 9-year-old boy comes to your office for a
    health supervision visit. He has difficulty
    seeing large print and recently began learning
    Braille in school. As an infant, he had severe
    retinopathy of prematurity, and he has had
    long-standing difficulty seeing, even with
    corrective lenses. His parents have been told
    that their son is considered legally blind. They
    ask what this designation means.

20
Of the following, the BEST response is that legal
blindness means
  1. ability to detect only the direction of a light
    source
  2. corrected vision of 20/70 or worse in the best
    eye
  3. corrected vision of 20/200 or worse in the best
    eye
  4. no residual visual function in either eye
  5. uncorrected vision of 20/100 or worse in the best
    eye

21
Answer C
  • The child described in the vignette is legally
    blind, defined as a corrected vision of 20/200 or
    worse in the best eye. Approximately 75 of
    legally blind individuals have some residual
    visual ability. Students may be classified as
    educationally visually impaired if their
    corrected vision is 20/70 or worse in the best
    eye.Children who have visual impairment need to
    learn in specialized environments to motivate
    them to move and improve their abilities to
    interpret sensory information accurately. Young
    infants should be encouraged to be mobile and
    orient themselves to the environment using touch,
    smell, and auditory input. Beginning in
    preschool, the goal is to teach skills that
    promote independence in the performance of daily
    activities. Depending on the degree of any
    associated impairments, the child may attend a
    regular school program with input from a teacher
    of the visually handicapped. The educational
    program should emphasize sensory experiences and
    auditory programs. The school-age child should
    have an Individualized Educational Plan. Again,
    depending on any associated impairments, the
    child may participate in regular community-based
    classroom activities, focusing on reading,
    writing, travel needs, and eventually vocational
    training and independent living. Braille is used
    for nonvisual communication and audio recordings
    supplement reading.American Board of Pediatrics
    Content Specification(s) Know the major
    approaches to the education of visually impaired
    children

22
  • A 2-year-old boy is mildly delayed in reaching
    his gross motor milestones, but his language
    development is appropriate for his age. He can
    speak in three- to four-word sentences. His
    parents are concerned that he will have academic
    difficulties due to his motor deficiencies. His
    neurologic examination yields nonfocal
    results.

23
Of the following, the MOST appropriate response
is that
  1. gross motor development is an accurate predictor
    for school success
  2. his delays are associated with learning
    disabilities
  3. his delays will result in poor handwriting skills
  4. his mildly delayed gross motor skills suggest a
    diagnosis of cerebral palsy
  5. language development is an accurate predictor of
    intellectual function

24
Answer E
  • A child's language development in infancy and
    early childhood correlates closely with cognitive
    development and, thus, is a better predictor of
    cognitive function than gross motor development.
    The acquisition and use of language is critical
    to a child's development in the areas of
    cognitive and social development. Phonemic
    awareness is the ability to attend to phonemes
    (speech sounds) that are used in syllables and
    words and is a critical language skill in the
    development of reading.The delay in achieving
    gross motor milestones described for the boy in
    the vignette has the weakest correlation with
    general intellectual functioning and, therefore,
    is not a strong predictor of school success. He
    has mildly delayed gross motor skills and a
    nonfocal neurologic examination. As a result, he
    does not meet the criteria for a diagnosis of
    cerebral palsy. Handwriting skills are affected
    by weakness in fine (not gross) motor
    development.American Board of Pediatrics
    Content Specification(s) Understand that
    language development in infancy and early
    childhood is a better predictor of cognitive
    function than motor development

25
  • Results of a cognitive test given to a 7-year-old
    girl indicate functioning in the moderate
    intellectual disabilities range (intelligence
    quotient between 40 and 50). Her parents ask what
    the future holds for their child academically and
    vocationally.

26
Of the following, the MOST likely expectation is
that the child
  1. can achieve a third- to sixth-grade reading level
    by adolescence
  2. can be employed in a competitive unskilled or
    semiskilled job
  3. can work in a sheltered workshop that provides
    close supervision
  4. will only be able to learn to read simple signs
    such as stop and exit
  5. will require help with activities of daily living
    from caregivers when an adult

27
Answer C
  • The American Association on Intellectual and
    Developmental Disabilities (AAIDD) defines
    intellectual disability (ID) as a disability
    "characterized by significant limitations both in
    intellectual function and in adaptive behavior as
    expressed in conceptual, social, and practical
    adaptive skill. This disability originates before
    the age of 18."Children who have mild ID (IQ,
    50-55 to 70) and no comorbid disorders are
    self-sufficient in activities of daily living
    (ADL) and communication. They can be expected to
    learn at one half to two thirds normal velocity.
    They can reach a third- to sixth-grade reading
    level by late adolescence and can be expected to
    be employed in competitive unskilled,
    semiskilled, or in some cases, skilled jobs. The
    prevalence rate of mild ID is 20 to 30 per 1,000,
    and affected children often are identified in
    kindergarten and early elementary
    school.Individuals who have moderate ID (IQ,
    35-40 to 50-55), such as the girl described in
    the vignette, are able to do ADL and express
    basic needs. They learn at one third to one half
    velocity. They can be expected to achieve a
    first- to third-grade reading level. They may be
    able to function in a supportive employment
    setting, but more often they work in sheltered
    workshops that provide constant supervision. In
    adulthood, they often live in group homes. The
    prevalence rate of moderate ID is 5 per 1,000,
    and the condition most often is identified in
    preschool.

28
  • Children who have severe ID (IQ, 20-25 to 35-40)
    have limited language skills and need support
    with ADL. They might be able to learn to read
    simple signs such as stop and exit. They will
    continue to need help with ADL from caregivers as
    adults. The prevalence rate of severe ID is 3 per
    1,000, and affected children are identified
    before age 3 years. These individuals often have
    identified genetic, medical, and neurologic
    causes.Children who have profound ID (IQ,
    lt20-25) require assistance for ADL. They have the
    highest rates of identified genetic, medical, and
    neurologic causes. The prevalence rate of
    profound ID is 1 to 2 per 1,000, and affected
    children are identified prior to age 2
    years.American Board of Pediatrics Content
    Specification(s) Distinguish between mild and
    moderate intellectual disabilities with regard to
    the potential for educational and
    independence/vocational achievement

29
During a health supervision visit, a mother
reports that her child is only understood about
75 of the time when she speaks to other
adults.Of the following, this finding is MOST
expected for a typically developing child who is
  1. 18 months old
  2. 24 months old
  3. 30 months old
  4. 36 months old
  5. 48 months old

30
Answer D
  • A helpful rule regarding the development of
    articulation is the Rule of Fourths. The average
    2-year-old child should be understood by
    strangers half the time (2/4), the average
    3-year-old child should be understood ¾ of the
    time, and the average 4-year-old should be
    understood all the time (4/4). The girl in the
    vignette is described as being understood by a
    stranger 75 of the time, which is typical for a
    child of 3 years.Children may have difficulty
    with the pronunciation of certain sounds until
    they are 7 years of age, but 100 of speech
    should be understood by age 4 years. In normal
    speech development, the first eight consonant
    sounds that develop are m, b, y, n, w, d, p, and
    h. The last eight consonants are sh, th (as in
    thirty), s, z, th (as in the), zh sound typically
    spelled as s (as in pleasure), l, and r, which
    develop by age 7.American Board of Pediatrics
    Content Specification(s) Know the progression of
    speech intelligibility and that deviation from
    the progression is abnormal (eg, 0 of
    intelligibility to strangers is 2/4 at 2 years, ¾
    at 3 years, 4/4 at 4 years)

31
  • A 7-year-old boy has mild intellectual impairment
    and an autism spectrum disorder. He has a
    pleasant disposition and no aggressive behaviors,
    but he has trouble paying attention in the
    classroom and difficulty staying in his seat.
    Both teacher report and parent rating forms
    document highly significant hyperactivity and
    difficulty paying attention. His parents are
    concerned that his activity level is affecting
    his ability to learn. They ask if any medication
    can help him attain school success.

32
Of the following, the MOST appropriate response
is to
  1. begin an atypical antipsychotic
  2. begin a trial of a serotonin reuptake inhibitor
  3. begin a trial of stimulant medication
  4. explain that medication would be ineffective due
    to his intellectual disability
  5. reassure the family that he is doing his best and
    have them return in 6 months

33
Answer C
  • The child described in the vignette has an autism
    spectrum disorder, symptoms of hyperactivity, and
    a short attention span. According to his teacher
    and parents, these symptoms are affecting his
    ability to learn at school. Therefore, a trial of
    a stimulant medication is indicated to target the
    symptoms. Stimulants can be effective, even in
    individuals who have intellectual disabilities.
    In 2005, the Research Units on Pediatric
    Psychopharmacology Autism Network studied
    methylphenidate in the treatment of children who
    had autism spectrum disorders. The results
    indicated drug efficacy, but with reduced
    effectiveness and a less satisfactory adverse
    effect profile. The most reported adverse effect
    was decreased appetite.The boy is having
    significant difficulty, and simply reassuring the
    family will not help his functioning at school.
    An atypical antipsychotic is not indicated in
    this situation because the child is not
    exhibiting aggressive or self-injurious
    behaviors, irritability, or explosive outbursts.
    Serotonin reuptake inhibitors are indicated for
    the treatment of obsessive-compulsive behaviors,
    anxiety, or symptoms of low mood, symptoms not
    exhibited by the boy in the vignette.American
    Board of Pediatrics Content Specification(s)
    Understand that children with intellectual
    disabilities and/or autism spectrum disorder who
    have symptoms of hyperactivity and short
    attention span may respond to medication

34
  • A 13-year-old boy who has an autism spectrum
    disorder is displaying severely aggressive
    behavior to his parents and teachers. When
    frustrated, he has tantrums, and when upset, he
    hits himself and throws objects. This behavior
    has continued even after intensive behavioral and
    educational interventions. His parents wish to
    begin medication to help handle him safely at
    home.

35
Of the following, the MOST appropriate medication
with which to begin a trial is
  1. atypical antipsychotic
  2. lithium
  3. melatonin
  4. serotonin reuptake inhibitor
  5. stimulant medication

36
Answer A
  • The significant behaviors exhibited by the boy
    described in the vignette include explosive
    outbursts, aggression, and self-injurious
    behaviors. Behavioral and educational approaches
    are the starting point for intervention. Because
    the child's behavior places him at risk for
    harming himself or others, pharmacologic therapy
    should be initiated. Medications that target
    irritability, aggression, explosive outbursts,
    and self-injurious behaviors include atypical
    antipsychotics and anticonvulsant mood
    stabilizers.An atypical antipsychotic is an
    appropriate choice for this boy. The most common
    adverse effects noted with these medications are
    weight gain, increase in appetite, and fatigue.
    Anticonvulsant mood stabilizers also may be used
    to treat target behaviors as well as bipolar
    symptoms and repetitive behaviors. Alpha-2
    agonists such as long-acting guanfacine may be
    used to target attention-deficit/hyperactivity
    disorder (ADHD) symptoms, aggression, and sleep
    dysfunction.Stimulant medications and selective
    norepinephrine reuptake inhibitors are used for
    ADHD symptoms of hyperactivity, impulsivity,
    inattention, and distractibility. They are not
    used to target primarily aggressive behaviors or
    self-injurious behaviors. Lithium is used for
    bipolar disorder and has a low therapeutic-to-toxi
    c ratio. Selective serotonin reuptake inhibitors
    are used to treat repetitive behaviors, anxiety,
    and depressive symptoms. Many selective serotonin
    reuptake inhibitors are used off-label. Melatonin
    is a naturally occurring hormone that is not
    regulated by the United States Food and Drug
    Administration, but it is available over the
    counter in the United States. Several studies
    have indicated its usefulness in promoting sleep
    but not for aggression or self-injurious
    behaviors.American Board of Pediatrics Content
    Specification(s) Know the role of medications in
    the autism spectrum disorders

37
  • You are seeing a 3-year-old boy for a health
    supervision visit. He has a vocabulary of 50
    words that he says clearly, and he is just
    beginning to combine words. He follows simple
    two-step commands. Past medical history reveals
    that he was born at term and had no perinatal
    problems. He has no history of recurrent ear
    infections and achieved gross motor milestones at
    appropriate ages. He resides in a bilingual
    household. According to the family history, his
    father also experienced delayed speech
    development. His older brother received speech
    therapy in elementary school, and his older
    sister had difficulty with reading comprehension
    but does well in math.

38
Of the following, the MOST likely cause of this
boy's language delay is
  1. a bilingual household
  2. being third born
  3. genetic predisposition
  4. hearing loss
  5. his sex

39
Answer C
  • The most likely cause for the delay in language
    development described for the boy in the vignette
    is genetic, based on the strong family history of
    language delays and reading difficulty. Children
    who have specific language impairment or a
    reading disorder often have family histories that
    suggest a genetic basis for the problems. The
    rate of reading disorder in parents of affected
    children ranges from 25 to 60. A child who has
    a family history of both protracted language
    problems and learning issues has greater risk for
    language-based learning difficulties.A language
    delay of greater than 25 (calculated as a
    language level of below 27 months in a
    36-month-old child) should be evaluated and not
    disregarded because the child is a boy, second or
    third born in a family, or part of a household in
    which more than one language is spoken. Language
    development in boys averages only a 1- to 2-month
    lag behind girls. Current research suggests that
    monolingual and bilingual children meet major
    language developmental milestones at similar
    times. A child raised in a bilingual home may use
    the two languages during a conversation, which
    has been found to be a sign of mastery of both
    languages. The child's total vocabulary size and
    length of utterance (number of words in a
    sentence) should be normal by age 2 to 3 years.

40
  • Other causes of delayed language development
    include genetic and chromosomal disorders as well
    as neurologic disorders such as cerebral palsy.
    An autism spectrum disorder should be suspected
    when language is both deviant and delayed. Global
    developmental delay may be suspected if delays in
    motor or cognitive development also are apparent.
    Universal screening of newborns detects most
    infants who have moderate, severe, or profound
    sensorineural hearing loss, although such
    screening may miss individuals who have
    mild-to-moderate, progressive, or acquired
    hearing loss. Hearing loss is a particular
    concern if the child has difficulty saying or
    hearing the sounds s, th, and f (high-frequency
    sounds).American Board of Pediatrics Content
    Specification(s) Know the causes of delayed
    language development
  • Understand the importance of family history in
    the diagnosis of language disorders

41
  • An 8-year-old boy has an above-average
    intelligence quotient, but he is struggling in
    school and consistently brings home failing
    grades. He is generally well behaved, but he gets
    angry with the poor grades. He enjoys being with
    his friends and is active in after-school
    activities.

42
Of the following, the MOST appropriate
intervention is to
  1. have the parents set up a behavioral chart to
    encourage him to improve his grades
  2. reassure the parents that he is smart and
    schedule a follow-up appointment in 6 months
  3. refer him for psychoeducational evaluation
  4. refer him to a psychiatrist
  5. tell the parents to punish him if he continues to
    fail

43
Answer C
  • The boy described in the vignette has
    above-average intelligence but is having academic
    difficulties, warranting psychoeducational
    evaluation for a learning disability. The
    reported prevalence of dyslexia (specific reading
    disability) ranges from 5 to 17 in the general
    population. The rate of dyscalculia (specific
    learning disability in mathematics) is 4 to 6.
    Individuals may have learning difficulties that
    occur independent of intelligence. Frequently,
    individuals who have high intelligence are not
    identified as having a learning issue or are
    considered to lack motivation. Such children who
    have above-average intelligence may have uneven
    profiles when evaluating their cognitive, social,
    and emotional development.The boy in the
    vignette should not be expected to improve
    spontaneously. The academic difficulty is not
    within his control and would not be remediated
    via discipline or use of a behavior chart. He is
    not showing evidence of a primary mood disorder
    for which a referral to a psychiatrist would be
    indicated. Additional causes of school failure
    are attention-deficit/hyperactivity disorder,
    sensory deficits (eg, diminished visual or
    auditory acuity), disordered sleep, or epilepsy.
    An older child should be evaluated for use of
    illicit drugs or alcohol.American Board of
    Pediatrics Content Specification(s) Recognize
    that children with above average intelligence may
    have academic failure and learning disabilities

44
  • A mother is concerned that her 6-year-old boy has
    been struggling to learn his alphabet and numbers
    in kindergarten. He is in good health, and
    results of his vision and hearing screens are
    normal. His preschool teacher commented last year
    that he was a bit shy but enjoyed being with the
    other children.

45
Of the following, the MOST appropriate next step
is to
  1. evaluate the boy for attention-deficit/hyperactivi
    ty disorder
  2. reassure the parents and schedule a 6-month
    follow-up appointment
  3. refer the boy for behavioral counseling
  4. refer the boy for psychoeducational evaluation
  5. refer the boy for vision therapy

46
Answer D
  • Approximately 5 to 17.5 of individuals in the
    United States have learning disabilities
    (depending on the definition used). Dyslexia
    (reading disability) is seen in approximately 80
    of individuals who have learning disabilities.
    Children in preschool may present with delays in
    speech and language and may have difficulty
    following directions and classroom routine. The
    boy described in the vignette may have a learning
    disability, suggested by the difficulty he is
    having in learning the alphabet or numbers in
    kindergarten. Other indicators of learning
    disabilities are difficulty paying attention,
    learning new skills, and connecting letters to
    sounds. There also may be poor coordination,
    including difficulty pasting, coloring, and
    writing. Therefore, reassurance of the parents is
    not appropriate. The boy requires a formal
    psychoeducational evaluation that includes
    cognitive and academic assessment. The evaluation
    should be performed in a timely manner so that
    his learning difficulty does not lead to
    frustration and poor self-image. If the
    evaluation identifies issues with attention or
    self-esteem, behavioral counseling and further
    assessment for attention-deficit/hyperactivity
    disorder should be pursued.Currently, no
    scientific evidence supports the position that
    subtle eye or visual problems cause learning
    disabilities or that vision therapy improves
    academic achievement. Other than
    convergence-insufficiency treatment, therapy to
    improve visual function is not supported and is
    poorly validated. Convergence insufficiency
    occurs when an individual is focusing at a near
    object and the eyes do not turn in properly.
    Treating this disorder with eye-focusing exercise
    can make reading more comfortable, but it does
    not improve decoding or comprehension of
    reading.American Board of Pediatrics Content
    Specification(s) Identify the indicators of
    learning difficulties (eg, preschool delay in
    speech and language, failure to learn letters and
    numbers by the end of kindergarten, failure to
    learn to read simple words by the end of first
    grade)

47
  • A 10-year-old boy has been receiving specialized
    educational services in school due to a learning
    disability. His parents encourage after-school or
    sports activities to promote positive
    self-esteem. They realize that academics are a
    challenge for him, and they express concerns
    about his future as an adult.

48
Of the following, the factor that has been shown
to have the MOST positive effect on prognosis for
such a child is
  1. early intervention therapy
  2. father's level of education
  3. having two or more siblings
  4. high intelligence quotient
  5. strong family support

49
Answer E
  • The presence of strong family support has been
    shown to have a positive effect on the prognoses
    of children who have learning disabilities, such
    as the boy described in the vignette. This effect
    is greater than that associated with the parent's
    education, number of siblings in the family,
    intelligence quotient score, or early educational
    intervention. Parent involvement and
    collaboration with physicians and schools is
    critical to ensure appropriate services.Support
    in the home via interactive learning activities
    (use of readers, audiobooks) is especially
    important for children who have learning
    disabilities because they often are overwhelmed,
    disorganized, and frustrated in learning
    situations. Specific academic support such as
    one-on-one tutoring in the home frequently is
    sought to help focus on homework. Family support
    can augment specialized educational intervention.
    Educational strategies such as remediation work
    targeting the underlying cognitive function that
    is impaired and phonologic programs (those that
    improve word decoding) have shown efficacy up to
    the sixth grade. If the area of weakness is
    unlikely to be corrected, circumvention
    strategies are useful in improving the outcome.
    Such strategies include using a keyboard or
    verbal exams for a child who has motor
    dysgraphia, using audiobooks for a child who has
    dyslexia, and using a calculator for the child
    who has dyscalculia (math disorder).American
    Board of Pediatrics Content Specification(s)
    Recognize factors that affect prognosis in a
    learning-disabled child
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