Child with Rapid Growth and Precocious Sexual Maturation

Phyllis W. Speiser

A 6-year-old boy was admitted to the medical center with a 4-year history of rapid somatic growth and a 6-month history of pubic hair growth. The patient was the full-term product of a normal vaginal delivery following an uncomplicated first gestation in a 34-year-old healthy female. Birth weight was 8 lb 9 oz (3.9 kg) and length 21.5 in. (54.6 cm). There were no neonatal problems. The mother ceased breastfeeding the infant at 10 days of life and changed to formula because he did not seem to gain weight. Thereafter, weight gain was normal. Between 9 and 18 months of age, the patient's linear growth was just above the 95th percentile, but by 21 years of age, his height was average for a 4| -year-old child. His tall stature was disregarded by his family and pediatrician, who considered this normal since his parents were tall [father 74 in. (1.90 m) and mother 66 in. (1.68 m)]. When the patient was 3 years old, his mother observed that his penis was larger than that of age-matched peers, and by the age of 4 some acne had developed. Pubic hair developed at 5| years of age, at which time he was referred to a pediatric endo-crinologist for evaluation.

Physical examination revealed a tall, well proportioned, muscular boy with mild facial acne. His height at 54.6 in. (1.39 m) was average for a boy of 10 years 3 months; his weight of 68 lb (31 kg) was average for 9 years 10 months. Blood pressure was normal (100/64 mm Hg). Dentition was advanced for age. The thyroid was not palpable. Examination of the chest and abdomen was unremarkable. The penis measured 7 cm semierect; fine, dark pubic hair was observed at the base of the phallus (Tanner stage II). Testes were each 3 mL in volume, without palpable masses. There were no pigmented cutaneous lesions. Neurological examination was normal.

Laboratory results were as follows:

Value,

Reference

Reference

Conventional

Interval,

Value, SI

Interval,

Analyte

Units

Conventional Units Units

SI Units

Sodium

138 mmol/L

135-145

Same

Same

Potassium

4.0 mmol/L

3.5-4.7

Same

Same

Chloride

103 mmol/L

99-108

Same

Same

Bicarbonate

25 mmol/L

24-32

Same

Same

Luteinizing hormone

1.7 mU/mL

< 1 (prepuberty)

1.7 U/L

<1

(LH), basal

Follicle-stimulating

<1 mU/mL

< 1 (prepuberty)

<1 U/L

<1

hormone (FSH), basal

Testosterone

172 ng/dL

2-12

5.9 nmol/L

0.07-0.46 nmol/

17-Hydroxyprogesterone,

11,690 ng/dL

<100

354 nmol/L

<3 nmol/L

basal

17-Hydroxyprogesterone,

22,000 ng/dL

< 200

666.6 nmol/L

<7.5 nmol/L

60 minutes after ACTH

stimulation

Cortisol, basal

7 g/dL

5-20

193 nmol/L

138-552

Cortisol, after ACTH

10 g/dL

2-3 x basal

276 nmol/L

2-3 x basal

stimulation

Computed tomography of the head was normal. Bone age based on x-ray examination of the wrist was read as compatible with a maturation of 12 years 9 months. Ultrasonographic examination of the testes showed no masses. The history of chronic accelerated growth velocity accompanied by signs of sexual maturation, the presence of relatively small testes for the degree of masculinization, and markedly elevated concentrations of serum 17-hydroxyprogesterone, the principal substrate for 21-hydroxylase, led to the diagnosis of congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

The patient was started on treatment with hydrocortisone. Reevaluation following 3 months of medical therapy indicated that linear growth was still accelerated, the testes had enlarged slightly, and the testosterone concentration had further increased. Repeat measurement of serum gonadotropins during sleep showed an LH of 19.9 mU/mL (19.9 U/L) and an FSH of 3.5 mU/mL (3.5 U/L), indicating pituitary stimulation of the testes. A gonadotropin-releasing hormone analog was added to the medical regimen to suppress central puberty.

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