Multiple pituitary hormone deficiency: Management of puberty for optimal auxological results

The overview in this paper focuses on ways of achieving optimal auxological results in puberty, principally in idiopathic and congenital multiple pitu itary hormone deficiency (MPHD), suggested by the co-authors. We agreed tha t diagnosing gonadotrophin insufficiency/deficiency is difficult in young c hildren and should be repeated in late prepuberty, but a firm diagnosis of MPHD helps avoid endocrine re-testing at the end of growth. The hypothalami c-pituitary axis must be reassessed periodically in evolving endocrinopathi es, though current practice varies widely. Optimum age to induce puberty is 11-12 years in girls and 13-14 boys, and sex steroids are the preferred ag ents. Short-course testosterone to increase micropenis size is advantageous , but inducing early testicular maturation is not known to improve later fe rtility. There is also little evidence for increasing the dose of GH during puberty, though therapy should continue to final height, and possibly unti l peak bone mass is achieved. Delaying puberty is an option in septo-optic dysplasia, and minimising the dose of hydrocortisone is crucial in treating ACTH/ cortisol insufficiency. Many unresolved questions remain in this dif ficult area.