Growth and Development

Sleep

100

Risk factors for obstructive sleep apnea syndrome include all of the following EXCEPT: A. Retroposition of the mandible B. Trisomy 21 C. Prader-Willi syndrome D. Cleft palate repair E. Chronic rhinitis F. All of the above

What is All of the above? Other causes of airway obstruction include allergies associated with chronic rhinitis/nasal obstruction; craniofacial abnormalities, including hypoplasia/displacement of the maxilla and mandible; gastroesophageal reflux with resulting pharyngeal reactive edema; nasal septal deviation; and velopharyngeal flap cleft palate repair. Reduced upper airway tone may result from neuromuscular diseases, including hypotonic cerebral palsy and muscular dystrophies, or hypothyroidism. Reduced central ventilatory drive may be present in some children with Arnold-Chiari malformation and meningomyelocele. In other situations, the etiology is mixed; individuals with Down syndrome, by virtue of their facial anatomy, hypotonia, macroglossia, and central adiposity, as well as the increased incidence of hypothyroidism, are at particularly high risk for OSA, with some estimates of as great as 70% prevalence.

200

A 10 yr old African-American boy is referred to you by the school psychologist for medical management of suspected ADHD because of inattention, behavior problems, and poor school performance. He goes to bed at 10:30 P.M. and falls asleep immediately. His brother will no longer share a room with him because of loud snoring. It is difficult for his parents to wake him for school in the morning at 7:00 A.M. He reports that he struggles to stay awake and pay attention during the day. He usually naps for 1-2 hr after school, but not in school. On the weekends, he stays up until midnight and sleeps until 9:00 A.M. He denies sudden losses of muscle tone, dreaming during the day, or sleep paralysis. Findings on physical examination are remarkable for a BMI of 30 kg/m2 and 3+ tonsillar hypertrophy. Which of the following is the most likely diagnosis? A. Insufficient sleep B. Delayed sleep phase syndrome C. Obstructive sleep apnea syndrome D. Narcolepsy E. Idiopathic hypersomnia

What is Obstructive sleep apnea syndrome? One of the most important but frequently overlooked sequelae of OSA in children is the effect on mood, behavior, learning, and academic functioning. The neurobehavioral consequences of OSA in children include daytime sleepiness with drowsiness, difficulty in morning waking, and unplanned napping or dozing off during activities, although evidence of frank hypersomnolence tends to be less common in children compared with adults with OSA. Mood changes include increased irritability, mood instability and emotional dysregulation, low frustration tolerance, and depression/anxiety. Behavioral issues include both "internalizing" (i.e., increased somatic complaints and social withdrawal) and "externalizing" behaviors, including aggression, impulsivity, hyperactivity, oppositional behavior, and conduct problems. There is a substantial overlap between the clinical impairments associated with OSA and the diagnostic criteria for ADHD, including inattention, poor concentration, and distractibility. (See Chapter 30.) There also appears to be a selective impact of OSA specifically on "executive functions," which include cognitive flexibility, task initiation, self-monitoring, planning, organization, and self-regulation of affect and arousal; executive function deficits are also a hallmark of ADHD.

300

Night terrors are associated with: A. REM sleep B. Overeating after 7:00 P.M. C. The use of antipsychotic medication D. Inception in preschool years and occasional somnambulism E. Anger within the family

What is Inception in preschool years and occasional somnambulism? Partial arousal parasomnias, which include sleepwalking, sleep terrors, and confusional arousals are more common in preschool- and school-aged children because of the relatively higher percentage of slow-wave sleep (SWS) in younger children.

400

You suspect narcolepsy in an adolescent patient who complains of persistent daytime sleepiness and who is noted by his teacher to "stare off" in class periodically. A polysomnogram is normal. What is the next step in diagnosis? A. Multiple sleep latency test B. EEG C. Iron studies D. HLA testing

What is Multiple sleep latency test? Overnight polysomnography (PSG) followed by a multiple sleep latency test (MSLT) are strongly recommended components of the evaluation of a patient with profound unexplained daytime sleepiness or suspected narcolepsy. The purpose of the overnight PSG is to evaluate for primary sleep disorders, such as OSA, that may cause EDS. The MSLT involves a series of 5 opportunities to nap (20 min long), during which narcoleptics demonstrate a pathologically shortened sleep-onset latency as well as periods of REM sleep occurring immediately after sleep onset.

500

The stage of sleep where night terrors typically appear AND FOR BONUS POINTS!! Duration of sleep cycle and sequence of sleep stages during the sleep cycle

What is Stage 3/4 non-REM sleep? Night terrors most commonly occur in stage 3/4 non-REM sleep with abrupt arousal from this stage. Most occur in the first 1/3 of the night. The child appears to be awake but is unresponsive and cannot be consoled. The child cannot remember the episode. These occur in about 5% of children. Occasionally they can persist into adolescence Sleep cycle 90 minutes: N1-N2-N3-N2-REM

How Old Am I?

100

The ability to manipulate small objects with the pincer grasp is usually noted at age: A. 0-2 mo B. 3-5 mo C. 6-7 mo D. 8-9 mo E. 10-12 mo

What is 8-9 mo? Pincer (thumb-to-forefinger) grasp occurs around the age of 8 months.

200

A child can feed himself with a spoon, build a tower of 5 blocks, and walk up steps with help. He has a vocabulary of over 200 words and combine 2 or 3 words in his speech. He laughs at simple humor and can point to basic body parts when asked. He does not use his first and last name in combination nor can he identify colors. The options are - 9 months - 12 months - 18 months - 24 months - 36 months

What is 24 months? A 24 month old should be able to drink from a straw, feed self with a spoon, help in washing hands, walk up stairs with help and take steps backward. Cognitively, he should be able to take things apart, explore surroundings and point to 5 or 6 body parts. Language/social skills include vocabulary of several hundred words, speaking in 2 -3 word sentences, comfort a distressed friend or parent, engage in pretend play, show awareness of parental response to actions and laugh at simple humor.

300

A young boy is able to correctly name several colors, describe what to do if hungry, thirsty, or sleepy and understands the difference between 'today' and 'not today'. He is able to state his age, tell his full name, and form 3-4 word sentences The majority of children are able to meet these receptive and expressive milestones by this average age. - 24 months - 30 months - 36 months - 42 months - 48 months

What is 36 months? Language skills include use of 3 word phrases and 3/4 of speech is intelligible

400

The probable age of a child who scribbles, walks alone, speaks one real word, and pretends to drink from a cup is: A. 8 mo B. 13 mo C. 16 mo D. 20 mo E. 24 mo

What is 13 mo?

500

A father brings his daughter in for a health supervision visit. She skips (alternating feet) into the room. You give her a piece of paper and a crayon and ask her to draw a person. She proudly draws a person with 6 body parts. You ask her to take off her shoes and sweater in order to obtain an accurate weight and she quickly complies with your request.

What is a 5 year old? She is able to dress and undress herself, draw a person with 6 body parts, and skip (alternating feet)

Intellectual Disability

100

Common identifiable causes of intellectual disability include all of the following EXCEPT: A. Trisomy 21 B. Hypothyroidism C. Fetal alcohol syndrome D. Fragile X syndrome E. Cystic fibrosis

What is Cystic fibrosis? Trisomy 21, hypothyroidism, fetal alcohol syndrome, and fragile X syndrome are all associated with intellectual disability.

200

Medical diagnostic testing in children with intellectual disability is not always indicated. Which of the following clinical scenarios does not clearly warrant the indicated testing? A. Mild intellectual disability—Blood lactate level B. Progressive neurologic disorder—Urine organic acids, plasma amino acids C. Child with intellectual disability and seizures—EEG D. Child with microcephaly and severe intellectual disability—MRI of brain E. Child with cardiac anomaly, midface hypoplasia intellectual disability—DNA microarray

What is Mild intellectual disability—Blood lactate level? The most commonly used medical diagnostic testing for children with intellectual disability include neuroimaging; metabolic, genetic, and chromosomal testing; microarray analysis; and electroencephalography (EEG). These tests should not be used as screening tools for all children with an intellectual disability. In some children there is a reasonable yield for testing, whereas in others the yield of <1% does not support its use.

300

n male patients with intellectual disability and positive family history, the next step in evaluation should be: A. Plasma ammonia assay B. Blood lead level determination C. EEG D. Molecular genetic testing for fragile X syndrome E. Cranial CT

What is Molecular genetic testing for fragile X syndrome? Molecular genetic testing for fragile X syndrome is appropriate for a boy with moderate intellectual disability, unusual physical features, and/or a family history of intellectual disability or for a girl with more subtle cognitive deficits associated with severe shyness and a relevant family history.

400

The most common reason for people with intellectual disability to be placed out of the home is: A. Single-parent family B. Greater degree of mental retardation C. Greater intensity of medical needs D. Lack of an appropriate school, necessitating residential placement E. Behavioral disturbance

What is Behavioral disturbance? Most children with an intellectual disability do not have a behavioral or emotional disorder as an associated impairment, but challenging behaviors (aggression, self-injury, oppositional defiant behavior) and mental illness (mood and anxiety disorders) occur with greater frequency in this population than among children with typical intelligence. These behavioral and emotional disorders are the primary cause for out-of-home placements, reduced employment prospects, and decreased opportunities for social integration.

500

To meet criteria for intellectual disability, a person must display significant deficit in adaptive behavior. Adaptive behavior is broadly defined and may refer to all of the following EXCEPT: A. Skills that are required for people to function in their everyday lives B. Conceptual, social, and practical skills C. Self-stimulatory behavior and/or aggression D. School performance

What is Self-stimulatory behavior and/or aggression? Three criteria must be met to establish the diagnosis of intellectual disability: significantly sub-average intellectual function, significant impairments in adaptive function, and onset before 18 years of age. Significant impairment in adaptive behavior reflects the degree that the cognitive dysfunction impairs daily function. Adaptive behavior refers to the skills that are required for people to function in their everyday lives. Adaptive behavior may be assessed by three different constructs: the classification of DSM-IV-TR, the classification of AAIDD, and the IDEA. The DSM-IV-TR classification of adaptive behavior addresses 10 domains: communication, self-care, home living, social and interpersonal skills, use of community resources, self-direction, functional academics, work, leisure, and health and safety. The AAIDD classification of adaptive behavior addresses 3 broad sets of skills: conceptual, social, and practical. The IDEA requires that the cognitive dysfunction affect school performance.

Normal (or not)

100

A mother brings her 8½ mo old circumcised boy to you for a "sick" visit. You saw the child 2 mo previously for health maintenance, including a DTP immunization, and the child appeared well. The mother's complaint is that the infant is waking up every night and is fussy during the day, especially when she leaves him. The child's history is otherwise normal, and physical examination reveals no problems. The most appropriate approach to management is to: A. Perform urinalysis and obtain a complete blood cell count to rule out urinary tract infection B. Request that the mother feed the infant more C. Reassure the mother that the behavior is normal and will pass in time D. Reassure the mother that the behavior will pass because it is a reaction to the DTP shot

What is C? Reassure the mother that the behavior is normal and will pass in time The advent of object permanence around the age of 8-9 months corresponds with qualitative changes in social and communicative development. Infants look back and forth between an approaching stranger and a parent and may cling or cry anxiously, demonstrating stranger anxiety. Separations often become more difficult. Infants who have been sleeping through the night for months begin to awaken regularly and cry, as though remembering that the parents are in the next room. The pediatrician has an important role in providing anticipatory guidance and reassurance for this normal developmental phase.

200

All of the following characteristics describe a child as being developmentally ready for toilet training EXCEPT: A. Cortical inhibition of reflex bladder contractions B. Awareness of when bladder is full C. Voluntary control of external sphincters D. Motivation by the child to stay dry E. Ability to use toilet paper appropriately

What is Ability to use toilet paper appropriately? At 2-4 yr, the child is developmentally ready to begin toilet training. To achieve conscious bladder control, several conditions must be present: awareness of bladder filling, cortical inhibition (suprapontine modulation) of reflex (unstable) bladder contractions, ability to consciously tighten the external sphincter to prevent incontinence, normal bladder growth, and motivation by the child to stay dry.

300

Which scenario correctly describes pica? A. A Canadian 6 mo old who bites plastic blocks B. A Japanese toddler who chews on books C. A Mexican-American 4 yr old who eats hair D. A pregnant teen in Africa who eats earth E. All of the above

What is A Mexican-American 4 yr old who eats hair? Pica involves the persistent eating of non-nutritive substances (e.g., plaster, charcoal, clay, wool, ashes, paint, earth). The eating behavior is inappropriate to the developmental level (e.g., the normal mouthing and tasting of objects in infants and toddlers) and not part of a culturally sanctioned practice. It usually remits in childhood but can continue into adolescence and adulthood. Geophagia (eating earth) is associated with pregnancy and is not seen as abnormal in some cultures (e.g., rural or preindustrial societies in parts of Africa and India).

400

Crying in the first 2 mo of life is characterized by all of the following EXCEPT: A. Teething B. Peaking at 6 wk C. Peaking at a total of 3 hr/day D. Crying in response to obvious stimuli E. Crying when no stimulus is obvious

What is Teething? Crying in the first 2 months occurs in response to stimuli that may be obvious (a soiled diaper) but are often obscure. Crying normally peaks at about 6 wk of age, when healthy infants may cry up to 3 hr/day, then decreases to 1 hr or less by 3 mo. Teething is not a cause of crying in infants of this age. Tooth eruption occurs between 6-12 months of age, usually starting with the mandibular central incisors. Tooth development reflects skeletal maturation and bone age, although there is wide individual variation.

500

The parents of a 7 year old boy ask for your advice about his nailbiting. He has been a nail biter for a couple of years and parents are very upset about it. He has no medical problems and no significant medical history. The best advice to give the parents is - A. take away privileges whenever he is seen biting his nails - B. apply bitter tasting nail coating to his nails - C. ask his teacher to remind him not to bite his nails - D. ignore the behavior

What is D? Ignore the behavior. Nail biting is a common habit of both children and adults. It can lead to irritation, bleeding and infection of he fingers. The 'treatment' is aimed at positive reinforcement (praise for the periods of time of no nail biting). Parents tend to overreact. They should be advised to, instead, ignore the behavior. Attempts by parents to stop the behavior may actually reinforce it. A specific measure may help, depending on habit. For example, bitter nail coating for thumb sucking or nail biting may work, but it requires constant reapplication.

Behavioral Disorders

100

Which of the following statements about Gilles de la Tourette syndrome is true? A. Coprolalia occurs in the most patients B. It is characterized by motor tics and stuttering C. It is often comorbid with OCD and ADHD D. It usually resolved by adolescence E. It affects girls more often than boys

What is It is often comorbid with OCD and ADHD? Tourette syndrome (TS) is characterized by multiple motor and vocal tics (not necessarily present concurrently). TS occurs in approximately 4-5 persons per 10,000. It is 1.5-3 times more likely in boys than in girls. TS commonly manifests in childhood, beginning with simple motor tics, often before age 7 yr. In many cases, multiple tics and complex vocal sounds, such as barking and grunting, develop over time and peak in severity by age 10-12 yr. Shouting obscene words (coprolalia) is characteristic but occurs in only 10% of affected patients. Children with TS often have behavioral, emotional, and academic problems. In particular, these children have higher rates of obsessive-compulsive disorder (OCD), attention-deficit/hyperactivity disorder (ADHD), and oppositional-defiant disorder. The fact that TS is highly comorbid with these specific psychiatric disorders suggests dysfunction in particular regions of the brain.

200

Which of the following statements regarding behavior is false? A. Iron supplementation may reduce breath-holding spells in some children B. To prevent tantrums, parents should intervene early and provide choices if appropriate C. Lying may be used by preschool children as a way to play with language D. Truancy and run-away behavior are developmentally appropriate in preteens E. Children exposed to aggressive models on television display more aggressive behavior than that noted in children not so exposed

What is Truancy and run-away behavior are developmentally appropriate in preteens? Truancy and running away are never developmentally appropriate. Truancy may represent disorganization within the home, caretaking needs of younger siblings, developing conduct problems, or emotional problems, including depression or anxiety. Whereas younger children may threaten to run away out of frustration or a desire to get back at parents, older children who run away are almost always expressing a serious underlying problem within themselves or their family, including violence, abuse, and neglect. Adolescent runaways are at high risk for substance abuse, unsafe sexual activity, and other risk-taking behaviors.

300

Development of conduct disorder in childhood and adolescence is associated with all of the following factors EXCEPT: A. Ineffective parenting B. Academic failure C. Physical abuse D. Rejection by peers E. Mental retardation

What is Mental retardation? A reliable sequence of events leads to the progression of subsyndromal disruptive behavior to oppositional defiant disorder to conduct disorder. This sequence often occurs in the context of a disadvantaged environment and begins with ineffective parenting strategies, followed by academic failure, parental hostility, and peer rejection, which leads to depressed mood, conflict with authority, and involvement in a deviant peer group. Children with difficult temperamental characteristics are more vulnerable to this sequence of events. Prevention efforts that intervene early in this sequence have the greatest chance of success.

400

Hallucinations in adolescents may be due to any of the following conditions EXCEPT: A. Illicit drug use B. Schizophrenia C. Severe traumatic stress D. Seizure disorder E. Attention-deficit/hyperactivity disorder

What is Attention-deficit/hyperactivity disorder? Among adults, hallucinations are viewed as synonymous with psychosis and as harbingers of serious psychopathology. In children, hallucinations can be part of normal development or can be associated with nonpsychotic psychopathology, psychosocial stressors, drug intoxication, or physical illness. The first clinical task in evaluating children and adolescents who report hallucinations is to sort out those that are associated with severe mental illness from those that derive from other causes. Nonpsychiatric disorders can manifest with hallucinations, including drug intoxications (cannabis, LSD, cocaine, amphetamines, barbiturates), medication side effects (steroids, anticholinergic medications, stimulant medications), and physical illnesses (thyroid, parathyroid, adrenal, and Wilson disease; electrolyte imbalance; infections; migraines; seizures; and brain tumors). In addition, nonpsychotic hallucinations commonly occur in the context of severe traumatic stress, developmental difficulties, social and emotional deprivation, parents whose own psychopathology promotes a breakdown in the child's sense of reality, cultural beliefs in mysticism, and unresolved mourning.

500

A 20 yr old college student is referred from the university health service because of very poor grades during the previous semester. His class participation is good, he seems to understand the concepts, but he is unable to finish his tests. His admission folder indicates that he was diagnosed as having a reading problem in fourth grade and received special help in grades 5-8. He was allowed to take his SATs untimed. Of the following, the most therapeutic recommendation is: A. Intensive phonologic awareness training B. Provision of extra time on tests C. Systematic, explicit instruction in phonics D. Multisensory training E. Referral to ophthalmology

What is Provision of extra time on tests? For those in high school, college, and graduate school, provision of accommodation rather than remediation most often represents the most effective approach to dyslexia. Imaging studies now provide neurobiologic evidence for the need for extra time for dyslexic students; accordingly, college students with a childhood history of dyslexia require extra time in reading and writing assignments as well as examinations. Many adolescent and adult students have been able to improve their reading accuracy but without commensurate gains in reading speed. Other helpful accommodations include the use of laptop computers with spelling checkers, use of recorded books, access to lecture notes, tutorial services, alternatives to multiple-choice tests, and a separate quiet room for taking tests.