Clinical_Rounds_in_Endocrinology_Volume_II_-_Pediatric_Endocrinology

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fl udrocortisone (100 μg/day) was continued. The annual follow-up of the patient
with clinical and biochemical parameters is depicted in the table given below.

Parameters

2

years

3

years

4

years

5

years

6

years

7.5

years a

8

years

12

years

13

years

Height (cm) 81 93 100 105 115 125 128 153 154

Weight (Kg) 12 14.5 17 17 24 28 30 50 54

Tanner stage A − P 1 B 1 A − P 2 B 1 A − P 2 B 1 – A − P 3 B 1 A − P 3 B 3 A − P 3 B 3 A + P 5 B 4 A + P 5 B 4

Bone age

(year)

• 7 years 8 years – – 10 – – 14

17(OH)P

(ng/ml)

26.8 250 0.5 0.1 – 0.78 – 174 90

T (nmol/L) 2.7 – 0.36 0.3 0.06 0.09 – 16.8 5.7

Hydrocortisone

(mg/day)

7.5 10 6 6 7 10 10 17.5 0.25 b

Fludrocortisone

(μg/day)

100 100 100 100 100 100 100 150 150

T testosterone
a LH 0.3 mIU/ml, E 2 42 pg/ml
b Hydrocortisone was substituted with dexamethasone 0.25 mg/day

At the age of 3 years, she had appearance of pubic hair along with growth
spurt with a steep rise in 17(OH)P which was suggestive of gonadotropin-
independent precocious puberty (GIPP) that required increase in the dose of
hydrocortisone. Between the age of 3–5 years, her growth velocity was appro-
priate for her age, and there was no progression of Tanner staging, but she had
cushingoid facies. Serum 17(OH)P was suppressed and testosterone was in
prepubertal range; therefore, the dose of hydrocortisone was reduced at this
point of care. Between the age of 5–7.5 years, she had spurt in growth velocity
followed by initiation of thelarche. Serum 17(OH)P continued to remain sup-
pressed, while serum LH increased to pubertal range 0.3 mIU/ml, which was
suggestive of gonadotropin-dependent precocious puberty (GDPP). However,
in the next 6 months, patient did not have progression of pubertal events. From
the age of 8 year onward, she had progression of pubertal events with serum
LH of 1.5 mIU/ml, and she was initiated with leuprolide 3.75 mg at monthly
interval. With this therapy, her growth velocity was approximately 6 cm/year,
and there was no progression of breast development till the age of 12 years. At
the age of 12 years, leuprolide was discontinued, and after 3 months, she had
menarche and her Tanner staging was A +, P 5, B 4. Six months later, she presented
with worsening of hyperpigmentation, secondary amenorrhea, and deepening
of voice. Serum 17(OH)P was 174 ng/ml, testosterone 16.8 nmol/L, LH
0.07 mIU/ml, FSH <0.5 mIU/ml, estradiol 73.1 pg/ml, and ACTH 384 pg/ml.
Ultrasonography of pelvis showed uterine size 4 × 3 cm, endometrial thickness
5 mm, and ovarian volume 3.5 ml each. Hydrocortisone dose was increased to
17.5 mg/day and fludrocortisone to 150 μg/day. After 3 months of this therapy,
17(OH)P was 90 ng/ml and testosterone 5.7 nmol/L. She was switched to

10 Congenital Adrenal Hyperplasia