Growth without growth hormone in combined pituitary hormone deficiency caused by pituitary stalk interruption syndrome

• Main Authors: Sang Soo Lee, A-Leum Han, Moon Bae Ahn, Shin Hee Kim, Won Kyoung Cho, Kyoung Soon Cho, So Hyun Park, Min Ho Jung, Byung-Kyu Suh
• Format: Article
• Language: English
• Published: Korean Society of Pediatric Endocrinology 2017-03-01
• Series: Annals of Pediatric Endocrinology & Metabolism
• Subjects: Growth without growth hormone; Combined pituitary hormone deficiency; Pituitary stalk interruption syndrome
• Online Access: http://e-apem.org/upload/pdf/apem-22-55.pdf

Growth hormone (GH) is an essential element for normal growth. However, reports of normal growth without GH have been made in patients who have undergone brain surgery for craniopharyngioma. Normal growth without GH can be explained by hyperinsulinemia, hyperprolactinemia, elevated leptin levels, and GH variants; however, its exact mechanism has not been elucidated yet. We diagnosed a female patient aged 13 with combined pituitary hormone deficiency (CPHD) caused by pituitary stalk interruption syndrome (PSIS). The patient has experienced recurrent hypoglycemic seizures since birth, but reached the height of 160 cm at the age of 13, showing normal growth. She grew another 8 cm for 3 years after the diagnosis, and she reached her final adult height of 168 cm which was greater than the midparental height, at the age of 16. The patient's blood GH and insulin-like growth factor-I levels were consistently subnormal, although her insulin levels were normal. Her physical examination conducted at the age of 15 showed truncal obesity, dyslipidemia, and osteoporosis, which are metabolic features of GH deficiency (GHD). Herein, we report a case in which a PSIS-induced CPHD patient attained her final height above mid parental height despite a severe GHD.