Pediatric Examination and Board Review (53 page)

SELECT THE ONE BEST ANSWER

1.
Jason’s differential diagnosis includes

(A) CP

(B) Tourette disorder

(C) blindness from ROP

(D) autistic spectrum disorder

(E) a developmental adjustment because of his mom’s return to work

2.
What key developmental disorder is least likely to be part of Jason’s developmental disability?

(A) translocation chromosomal disorder

(B) velocardiofacial syndrome

(C) tuberous sclerosis complex

(D) fragile X syndrome

(E) untreated phenylketonuria (PKU)

3.
At school, a picture exchange system is started. Jason is able to express his needs and begins to take turns. Educational practices that may not be helpful for children with autism include which of the following?

(A) structured experiences that promote communication and social skills

(B) intensive patterning

(C) psychotherapy

(D) large-group activities without any demanding tasks

(E) B, C, and D

4.
Jason’s mother wonders if special dietary interventions might be helpful. Which of the following statements is/are true?

(A) there is indisputable evidence that in countries where vegetarianism is the dominant practice, there is no autism

(B) children with celiac disease have high rates of autism

(C) children with untreated PKU never get autism

(D) children with autistic behavior and recurrent diarrhea should be evaluated for malabsorption

(E) A and B

5.
Jason’s mother is concerned about the effect of immunizations on her son. Which of the following statements is/are true?

(A) children with congenital rubella have high rates of neuro-disability including autism

(B) measles encephalitis does not cause any adverse developmental sequelae

(C) children who experience mild measles do not get subacute sclerosing panencephalitis (SSPE)

(D) mumps does not cause deafness

(E) C and D

6.
Jason’s mother wonders if he might benefit from secretin, a GI hormone that she learned about in a TV special. Which of the following is (are) true?

(A) secretin has been approved for use in treating autism

(B) several randomized scientific trials have shown that secretin is not superior to placebo

(C) secretin is only available as a compassionate use medication

(D) the cost of secretin is covered by Medicaid and private insurance

(E) A and D

7.
Jason has difficulty shopping or going to a relative’s home. He will scream, bite, and kick. Useful support systems for parents include which of the following?

(A) making no demands on Jason

(B) implementing a behavioral management program

(C) instituting a major tranquilizer

(D) telling the parents he will outgrow it

(E) A and D

8.
Jason began to participate in Special Olympics. Potential benefits include

(A) regular physical activity

(B) dental screening through Healthy Smiles

(C) being able to skip gym in high school

(D) mentoring

(E) A, B, and D

9.
Key management areas for long-term success for people with autism include which of the following?

(A) increasing positive behaviors

(B) decreasing negative behaviors

(C) teaching social skills

(D) promoting communication and functional skills

(E) all of the above

ANSWERS

1.
(D)
Jason’s difficulty in communication and social skills may indicate an autistic spectrum disorder. Manifestations of CP include motor delay with abnormal neurologic signs of spasticity, motor control, and posture. Manifestations of Tourette disorder include tics and attentional difficulties. Neither the mother’s behavior in returning to work nor immunizations cause autism. Children with severe visual disabilities can have an autistic spectrum disorder. Jason did not have a severe visual impairment.

2.
(E)
Major known etiologies for autistic spectrum disorder include fragile X, untreated PKU, sequelae of congenital rubella, chromosomal disorders, genetic malformation disorders, and tuberous sclerosis. If Jason, born in the United States, underwent newborn screening for PKU, it is unlikely that he has that disease. There is a range of developmental and communicative disorders in children with velocardiofacial syndrome (22q-deletion) including autistic spectrum disorders.

3.
(E)
Key principles of management include special education, family supports, interventions that enhance communication and adaptive skills, and behavioral interventions. Educational practices that are not helpful for children with autistic spectrum disorders include patterning, psychotherapy, and large-group activities without any demands. A comprehensive IEP as well as recreation and after-school supports are the best ways to optimize outcomes.

4.
(D)
There is autism in India. The overwhelming majority of children with celiac disease do not have autism. More than 50% of children with untreated PKU develop autism. If there are symptoms of growth delay, bloating, retching, diarrhea, or other GI disturbances, then appropriate tests for celiac disease are indicated.

5.
(A)
Avoidance of vaccines does not eliminate autism and has other untoward consequences (ie, risk of vaccine-preventable disease). Measles can cause both encephalitis and SSPE. Mumps can cause deafness and a spectrum of disabilities.

6.
(B)
Secretin in several randomized clinical trials has been shown not to benefit children with autism. Secretin has not been approved for use in treating autism. Neither Medicaid nor private insurance cover secretin.

7.
(B)
Children with autism can benefit from behavioral management. There are several explicit strategies to enhance management outside the home. If these behavioral difficulties are more widespread, then consideration of judicious psychopharmacology and behavioral management is required. For the patient’s family to access certain events, in-home respite services are required.

8.
(E)
Children in special education can receive both adaptive physical education and after-school Special Olympics training. Individuals in Special Olympics receive health, dental, vision, and hearing screening as well as mentoring and regular physical activity. This does not excuse them from participation in gym or adapted physical education in high school.

9.
(E)
Behavior management, communication enhancement, including Picture Exchange Communication System (PECS), and social skill training can help children who have autistic spectrum disorders learn. Key management areas for long-term success for people with autism include increasing positive behaviors, decreasing negative behaviors, and teaching social skills. After-school recreation including swimming, bowling, and horseback riding can be helpful. Hobbies, such as animal husbandry, horticulture, and music are also potential resources. Promoting communication and functional skills across home, education, and community settings is important in ongoing management.

S
UGGESTED
R
EADING

Meyer GA, Batshaw ML. Fragile X. In: Wolraich ML, ed.
Disorders of Development and Learning.
3rd ed. Hamilton, Ontario, Canada: BC Decker; 2003:321-332.

Parish JM. Promoting adaptive behavior while addressing challenging behavior. In: Batshaw ML, ed.
Children with Disabilities.
6th ed. Baltimore, MD: Paul H. Brookes; 2007.

Ruble LA, Brown S. Pervasive developmental disorders: Autism. In: Wolraich ML, ed.
Disorders of Development and Learning.
3rd ed. Hamilton, Ontario, Canada: BC Decker; 2003:249-266.

CASE 32: A 7-YEAR-OLD BOY WITH ADHD FAILS FIRST GRADE

Arnold is a 7-year-old boy who presents with his mother after being told by his school that he needs to repeat first grade. The teacher said he seemed bright but had not learned to read and that he was out of his seat all the time. The school psychologist saw him and reported that his IQ was 112 but that he might have “organic” problems. His mother says she trusts you, knows you are interested in school problems, and is willing to pay you to spend extra time with Arnold. Further discussion reveals that the mother is angry that school problems were not anticipated when you did your 5-year school entry checkup. You note that your nurse had done a Denver Development Screening Test, 2nd ed. (DDST-2), which Arnold passed.

There were no complications of pregnancy, labor, or delivery. You have been following Arnold since infancy with no significant illnesses. In infancy, he had several bouts of otitis media, which responded to medication. His hearing tests have been normal.

SELECT THE ONE BEST ANSWER