Combination growth hormone and gonadotropin releasing hormone analog therapy in 11beta-hydroxylase deficiency.
Diagnosis of 11beta-hydroxylase deficiency was made in a boy at the age of 2 1/2 years on the basis of peripheral precocious puberty, growth acceleration (height standard deviation score +4.4) with advanced skeletal maturation (bone age 8.4 years) and elevated deoxycortisol levels. Glucocorticoid supplementation led to normalization of blood pressure but was associated with progression to central precocious puberty and increase in bone age resulting in decrease in predicted adult height to 133.7 cm (target height 163 cm). The child was started on GnRH analog (triptorelin 3.75 mg every 28 days), which led to improvement in predicted adult height by 3.1 cm over 15 months. Addition of growth hormone (0.1 IU/kg/day) resulted in improvement in predicted adult height (151 cm) and height deficit (12 cm) over the next 3.6 years. Final height (151 cm) exceeded predicted height at the initiation of GnRH analog treatment by 17.3 cm. This report suggests that combination GH and GnRH analog treatment may be useful in improving height outcome in children with 11beta-hydroxylase deficiency and compromised final height.
Bajpai
Anurag
A
Division of Pediatric Endocrinology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi.
Kabra
Madhulika
M
Menon
P S N
PS
D000312:Adrenal Hyperplasia, Congenital / Q000188:drug therapy*; D001827:Body Height; D001846:Bone Development; D002675:Child, Preschool; D004359:Drug Therapy, Combination; D005500:Follow-Up Studies; D007987:Gonadotropin-Releasing Hormone / Q000031:analogs & derivatives* / Q000627:therapeutic use*; D013006:Growth Hormone / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D011629:Puberty, Precocious / Q000188:drug therapy; D017329:Triptorelin Pamoate / Q000627:therapeutic use
D017329:Triptorelin Pamoate; D007987:Gonadotropin-Releasing Hormone; D013006:Growth Hormone