Pediatric Examination and Board Review (45 page)

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14.
Use a lock and key

15.
Indicate symptoms verbally to a physician

16.
Budget for monthly expenses

17.
Eat with utensils

18.
Do his own laundry

19.
Make change for a dollar

20.
Tell time

21.
Have an intimate sexual relationship

22.
Fill out a job application

23.
Participate in a simple conversation

24.
Use public transportation independently

25.
Recognize traffic and exit signs

26.
Schedule daily activities independently

27.
Use a pay telephone

28.
Choose appropriate clothes to wear

29.
Follow a national news event

30.
Act appropriately toward strangers

31.
Sustain a friendship with another person

32.
Anticipate hazards appropriately

33.
Follow a one-stage command

34.
Address two people by name

35.
Based on the information in question 7, by adulthood, where would Adam most likely live?

(A) in an unsupervised apartment
(B) in a supervised apartment
(C) in a group home
(D) in an institution
(E) in a nursing home

36.
Based on the information in question 7, by adulthood, where would Adam most likely work?

(A) in a skilled, competitive employment
(B) in an unskilled, competitive employment
(C) in a supervised, full-time employment
(D) in a supervised, part-time employment
(E) Adam will be incapable of any productive employment

ANSWERS

 

1.
(C)
Children with preference for one hand early in childhood are often indicating significant motor control abnormalities. This child would not be doing a pincer (a 9- to 11-month developmental skill) with his right hand.

2.
(C)
Children with a brachial plexus palsy do not have a symmetric Moro reflex. Children with diplegic CP have indicators of lower extremity spasticity. Children with Sturge-Weber syndrome have facial vascular abnormalities (facial nevus flammeus). Strong hand preference with motor delay on the uninvolved side indicates hemiplegic CP.

3.
(D)
More than 50% of congenital hemiplegia is of prenatal onset. MRI will often reveal a CNS dysgenesis or a porencephalic cyst. EEG might yield evidence of a partial paroxysmal abnormality. A Wood’s lamp examination will help rule out tuberous sclerosis complex. Plasma amino acids might indicate an increased methionine level, which can be a feature of homocystinuria. In homocystinuria there are increased risks of vascular events, including stroke, dislocated lens, osteoporosis, and mental retardation. Urine for CMV would not be helpful at 1 year because it would not diagnose or exclude congenital CMV.

4.
(B)
Adam has congenital hemiplegic CP. This may be associated with an intrauterine vascular event, especially the death of a co-twin. The purpose of neuroimaging is to assess if his CNS structures might be asymmetric.

5.
(A)
The outcome for children with hemiplegic CP in sequential studies involving large cohorts is 100% for ambulation. There is a range of cognitive outcomes based on the extent of the hemispheric lesion. There is enough hemispheric plasticity that language emerges consistent with cognitive skills. Seizures, if they occur, are readily controllable. If there are visual field problems, they are unilateral. Residential placement is neither in Adam’s best interest nor available.

6.
(E)
Given Adam’s lesion, he will benefit from Early Intervention and quality early childhood educational experiences. Hyperbaric oxygen has not improved motor or functional outcomes in children in randomized clinical trials. Complications of hyperbaric oxygen include perforated ear drums, middle ear effusions, and parental expense. Adam will walk regardless of the intensity of PT. Hyperbaric oxygen is not medically indicated.

7.
(B)
Adam’s scores are more than 3 standard deviations below the mean, which indicates moderate mental retardation.
Table 24-1
indicates the developmental levels of mental retardation, the support required, and the outcomes at key ages.

TABLE 24-1.
Cognitive Adaptive Intellectual Disability

 

DEGREE OF COGNITIVE IMPAIRMENT
PREVALENCE AND CHARACTERISTIC FEATURES
COMMUNICATION AND ACTIVITIES OF DAILY LIVING (ADL)
Mild: IQ 55-69 with concurrent adaptive disability and need for intermittent/limited supports.
20-30/1000. Detected most often in kindergarten and early elementary school years. Major risk factors include poverty and low maternal educational achievement.
Independent in communication and all ADL. Capable of reading and writing to fourth- or fifth-grade level. Some resources required to maximize employment options and independent living.
Moderate: IQ 40-54 with concurrent adaptive disability and need for limited/extensive supports.
5/1000. Detected most often in preschool years as language delay. Major known etiologies include chromosome disorders and genetic syndromes.
Independent in all ADL. Communicative of basic needs; able to learn functional-survival academic skills. Will have a spectrum of housing and employment options requiring some supervision.
Severe: IQ 25-39 with concurrent adaptive disability and need for extensive/pervasive supports.
3/1000. Identified before age 3 years. High rates of genetic, biomedical, and neurologic etiologies.
Able to walk. Limited communication. Difficulty with independence in all ADL although can master many basics. Range of behavior difficulties includes terrible 2s, autistic spectrum, hyperactivity. Requires much family support, respite, and creative caretaking. Benefits from day treatment and recreational programs.
Profound: IQ <25 with concurrent adaptive disability and need for pervasive supports and specialized health services.
1-2/1000. Identified prior to age 2 years. Highest rates of genetic, biomedical, and neurologic etiologies.
Most without CP walk. Some can be toilet trained. Need supervision or assistance for most ADL. May be medically frail (eg, epilepsy, aspiration). May require both nursing and humanistic interventions.

 

Abbreviations: ADL, activities of daily living; CP, cerebral palsy.

 

8.
(E)
100% can dress and use the toilet independently

9.
(A)
Fewer than 10% can enter into a marriage contract

10.
(E)
100% can drink from a cup independently

11.
(C)
50% can cook a meal unsupervised

12.
(A)
Fewer than 10% can raise children

13.
(A)
Fewer than 10% can find their own way in unfamiliar surroundings

14.
(C)
50% can use a lock and key

15.
(C)
50% can indicate symptoms verbally to a physician

16.
(A)
Fewer than 10% can budget for monthly expenses

17.
(E)
100% can eat with utensils

18.
(C)
50% can do their own laundry

19.
(A)
Fewer than 10% can make change for a dollar

20.
(B)
25% can tell time

21.
(B)
25% can have an intimate sexual relationship

22.
(A)
Fewer than 10% can fill out a job application

23.
(E)
100% can participate in a simple conversation

24.
(D)
90% can use public transportation independently

25.
(C)
50% can recognize traffic and exit signs

26.
(B)
25% can schedule daily activities independently

27.
(B)
25% can use a pay telephone

28.
(D)
90% can choose appropriate clothes to wear

29.
(C)
50% can follow a national news event

30.
(D)
90% can act appropriately toward strangers

31.
(E)
100% can sustain a friendship with another person

32.
(C)
50% can anticipate hazards appropriately

33.
(E)
100% can follow a one-stage command

34.
(E)
100% can address two people by name

Adam’s developmental diagnosis over time became one of moderate mental retardation. He has a less than 10% chance of attaining functional literacy but will be independent in self-care, such as eating, dressing, bathing, and continency. Adam will be challenged by adult responsibilities, including marriage, child rearing, budgeting, and driving (
Table 24-1
).

35.
(B)
Adam does not require a nursing home. Current social policy has not resulted in any new admissions to institutions. Adam’s most probable living arrangement will be in a supervised apartment.

36.
(D)
Adam’s most likely employment will be supervised and part time.

S
UGGESTED
R
EADING

 

Accardo PJ, Capute AJ. Mental retardation. In: Capute AJ, Accardo PJ, eds.
Developmental Disabilities in Infancy and Childhood.
Vol 2. 2nd ed. Baltimore, MD: Paul H. Brookes; 1996.

Batshaw ML, Shapiro B. Mental retardation. In: Batshaw ML, ed.
Children with Disabilities.
6th ed. Baltimore, MD: Paul H. Brookes; 2007.

Coulter DL. Intellectual Disability: Diagnostic Evaluation. In: Augustyn M, Zuckerman B, Caronna EB, eds.
Developmental and Behavioral Pediatrics: A Handbook for Primary Care
, Philadelphia, PA: Lippincott, Williams & Wilkins; 2011:254-257.

Palmer FB, Hoon AH. Cerebral Palsy. In: Augustyn M, Zuckerman B, Caronna EB, eds.
Developmental and Behavioral Pediatrics: A Handbook for Primary Care
, Philadelphia, PA: Lippincott, Williams & Wilkins; 2011:164-171.

CASE 25: A CHILD WITH SPINA BIFIDA

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