Pediatric Examination and Board Review (58 page)

SELECT THE ONE BEST ANSWER

1.
Of the following, which is the least likely to help with his diagnosis?

(A) parental heights

(B) growth velocity

(C) actual height

(D) bone age

(E) weight for height

2.
What is the most likely diagnosis if his growth rate is 5 cm/year, height age is 8 years, and bone age is 10 years?

(A) constitutional delay of growth and puberty

(B) undernutrition

(C) hypothyroidism

(D) growth hormone deficiency

(E) intrinsic short stature

3.
What is the most likely diagnosis if his growth rate is 5 cm/year, height age is 8 years, and bone age is 8 years?

(A) intrinsic short stature

(B) growth hormone deficiency

(C) Cushing syndrome

(D) constitutional delay of growth and puberty

(E) hypothyroidism

4.
What is the most likely diagnosis if his growth rate is 3 cm/year, height age is 8 years, and bone age is 8 years?

(A) exogenous obesity

(B) constitutional delay of growth and puberty

(C) growth hormone deficiency

(D) intrinsic short stature

(E) hypogonadism

5.
What is the most likely diagnosis if he has abnormal body proportions and a narrow interpedicular distance in the lower lumbosacral area?

(A) Noonan syndrome

(B) Kallman syndrome

(C) hypochondroplasia

(D) Klinefelter syndrome

(E) Prader-Willi syndrome

6.
Which of the following is one of the most common causes of short stature in children?

(A) hypothyroidism

(B) growth hormone deficiency

(C) poor nutrition

(D) chronic illness

(E) familial intrinsic short stature

7.
Which of the following tests would you recommend to be included in an initial screen for a 10-year-old boy with an attenuated growth rate?

(A) complete blood count (CBC), karyotype

(B) sedimentation rate, thyroid function tests

(C) CBC, growth hormone level

(D) sedimentation rate, gonadotropin levels

(E) thyroid function tests, growth hormone level

8.
Your initial screening tests are normal and you advise that the child return for follow-up so you can assess his growth velocity. When would you advise follow-up?

(A) 1-2 months

(B) 2-3 months

(C) 4-6 months

(D) 7-9 months

(E) 1 year

9.
What is the normal growth velocity of a child between the ages of 3 and 10 years?

(A) 3-4.5 cm/year

(B) 4-5.5 cm/year

(C) 5-6.5 cm/year

(D) 6-7.5 cm/year

(E) 7-8.5 cm/year

10.
Which of the following is the least accurate way to measure a child?

(A) on a scale equipped with a flexible height arm

(B) standing against a wall

(C) with a wall-mounted stadiometer

(D) with a portable stadiometer

(E) lying supine on a flat surface

11.
What is this patient’s genetic height potential if his father is 5'10" and his mother 5'5"?

(A) 5'5"

(B) 5'7"

(C) 5'9"

(D) 5'10"

(E) 6'0"

12.
You would be most concerned about a potential growth problem in a child who crossed from the 50th to the 25th percentile for height at what age?

(A) 8 months of age

(B) 2 years of age

(C) 7 years of age

(D) 13 years of age

(E) 15 years of age

13.
Which of the following would be least helpful in differentiating between intrinsic short stature and constitutional delay of growth and puberty?

(A) growth rate from 0 to 3 years

(B) growth rate from 3 to 10 years

(C) growth rate from 11 to 14 years

(D) final stature

(E) bone age

14.
Which of the following is not a classic feature of growth hormone deficiency?

(A) hypoglycemia during infancy

(B) delayed bone maturation

(C) central distribution of body fat

(D) low serum insulin-like growth factor (IGF)-1

(E) decreased arm span

15.
Your patient eventually gets diagnosed with growth hormone deficiency and is started on growth hormone replacement therapy. He starts complaining of severe headaches. The pretreatment head magnetic resonance imaging (MRI) was normal. What would you be most concerned about?

(A) migraine headaches

(B) pseudotumor cerebri

(C) brain tumor

(D) tension headaches

(E) vision change

16.
Which of the following conditions presents with an attenuated growth pattern?

(A) constitutional delay of puberty

(B) intrinsic short familial stature

(C) hypothyroidism

(D) A and C

(E) B and C

17.
If this child were a 10-year-old girl, what test would you consider checking to help in your evaluation?

(A) karyotype

(B) estradiol

(C) gonadotropin levels

(D) chest radiograph

(E) pelvic ultrasound

18.
Which of the following is not a feature of Turner syndrome?

(A) renal anomalies

(B) webbed neck

(C) lymphedema

(D) cubitus valgus

(E) right-sided cardiac anomalies

19.
Your patient’s weight is greater than the 97th percentile and he has striae on the abdomen. What would be at the top of your differential diagnosis?

(A) genetic obesity syndrome

(B) exogenous obesity

(C) hypothyroidism

(D) growth hormone deficiency

(E) Cushing syndrome

20.
Which of the following is not associated with tall stature as a child?

(A) Klinefelter syndrome

(B) exogenous obesity

(C) hyperthyroidism

(D) excess exogenous corticosteroids

(E) sexual precocity

ANSWERS

1.
(C)
Actual height. The actual height of a patient is least likely to help with the diagnosis because the only information it provides is that the patient is short for age. Statistically, 3% of the population will be below the normal growth curve. Data on parental heights offer information regarding the genetic height potential of the child. The growth velocity allows you to determine if the child is growing at a normal rate for his age. A predictable pattern of bone maturation occurs during bone growth, and a bone age radiograph is useful to determine the bone maturation and the growth potential of a child. Current weight-for-height is also important and helps to rule out a nutritional component to poor growth. Other important parts of the history include family growth and pubertal history, birth length and weight, and overall health history to rule out chronic disease as a cause of short stature.

2.
(E)
Intrinsic short stature. Children with intrinsic short stature typically grow at a normal rate but below the normal growth chart. Their bone age is usually equivalent to their chronological age, which are both greater than the height age (height age is the age at which the child’s height falls at the 50th percentile). They go through puberty at a normal time and end up on the shorter side of normal as an adult.

3.
(D)
Constitutional delay of growth and puberty. Children with constitutional delay of growth and puberty have normal growth rates during childhood, delayed puberty with a delayed pubertal growth spurt, and attainment of normal adult height. Their bone age is usually equivalent to their height age, and both are less than their chronological age. Children with intrinsic short stature also have a normal growth rate, but their bone age would approximate their chronological age. Children with growth hormone deficiency, Cushing syndrome, and hypothyroidism would have an attenuated growth pattern.

4.
(C)
Growth hormone deficiency. A growth rate of only 3 cm/year would be considered an attenuated growth rate, which is seen in endocrine causes of growth failure, such as growth hormone deficiency, hypothyroidism, and Cushing syndrome. Severe chronic disease and malnutrition can also cause an attenuated growth pattern. Children with exogenous obesity tend to be tall with a normal growth rate.
Table 37-1
shows the classification of short stature as it relates to growth rate and bone age.

TABLE 37-1.
Classification of Short Stature

5.
(C)
Hypochondroplasia. Hypochondroplasia is often described as a milder form of achondroplasia. However, although they are both transmitted via autosomal dominant transmission, they have not been reported to occur in the same family. Hypochondroplasia is a result of a mutation in the fibroblast growth factor receptor 3 gene. Patients present with short stature and dysmorphic features that are milder than in achondroplasia. They do not have the typical facial features of achondroplasia. These patients have radiologic evidence of a narrow interpedicular distance in the lower lumbosacral area. Children with Noonan syndrome often present with short stature, webbed neck, malformed ears, and right-sided cardiac abnormalities, such as pulmonic stenosis. They have a normal karyotype. Children with Kallman syndrome have hypogonadotropic hypogonadism and anosmia. Boys with Klinefelter syndrome (47,XXY) tend to be tall and often present in adolescence with small testicles. They tend to have school issues. Children with Prader-Willi syndrome present with hyperphagia, obesity, and short stature.