© Springer India 2016 109
A. Bhansali et al., Clinical Rounds in Endocrinology,
DOI 10.1007/978-81-322-2815-8_4
4
Childhood Cushing’s Syndrome
4.1 Case Vignette
A 16-year-old girl presented with weight gain, secondary amenorrhea, and
increased facial hair for the past 2 years; however, her appetite was normal and
there was no change in her lifestyle. She also complained of weakness and
appearance of striae over the abdomen for the last 1 year. She had a history of
poor gain in height during the last 4 years. Patient also had diffi culty in climbing
the stairs. However, there was no history of easy bruisibility, back pain, or patho-
logical fracture. She had menarche at the age of 12 years and later oligomenor-
rhea, followed by secondary amenorrhea for the last 2 years. There was no history
of any drug intake, or use of alternative medications, or steroid preparations,
either inhalational or ointments. On examination, her height was 157 cm (<25 th
percentile, height age 13 years, target height 161 cm), weight 67 Kg (>75 th per-
centile, weight age >20 years), BMI 24.1 Kg/m^2 , and blood pressure
112/74 mmHg. She had fl orid features of Cushing’s syndrome including moon
facies, facial plethora, acne, dorso-cervical fat pad, and wide violaceous striae
over lower abdomen and thighs. She also had cuticular atrophy, knuckle hyper-
pigmentation, and proximal muscle weakness. She had generalized obesity with
waist circumference of 90 cm. However, she did not have bruise and pulp atro-
phy. Patient had hirsutism with Ferriman–Gallwey score of 15/36 and did not
have features of virilization. There was no cafe-au-lait macule, lentigines, bony
deformity, spine tenderness, or cutaneous fungal infection. Biochemical evalua-
tion revealed serum potassium 4.2 mEq/L, liver, and renal function tests were
normal. Fasting blood glucose was 92 mg/dl and 2h plasma glucose after glucose
load was 112 mg/dl with HbA1c of 5.0 %. Lipid profi le showed serum choles-
terol 159 mg/dl, LDL-C 99.6 mg/dl, triglyceride 198 mg/dl, and HDL-C 32.2 mg/
dl. Cortisol dynamics showed 0800h cortisol 567 nmol/L; awake 2300h cortisol
520 nmol/L; 0800h ACTH 16.8 pg/ml; awake 2300h ACTH 63.7 pg/ml; late-
night salivary cortisol 12.5 nmol/L and 28.7 nmol/L on two consecutive days,
respectively; and 24h urinary free cortisol on three consecutive days was 448 μg,