Neuroendocrine Disorders in Children – Chapter 19: The Inherited Basis of Hypogonadotropic Hypogonadism (ebook)


Chapter 19 – The Inherited Basis of Hypogonadotropic Hypogonadism

Congenital isolated hypogonadotropic hypogonadism (CIHH) is a well-known cause of absent pubertal development in both sexes and results directly from inadequate secretion of the gonadotrophins, luteinizing hormone (LH) and follicle-stimulating hormone (FSH), most commonly secondary to subnormal secretion of hypothalamic gonadotrophin-releasing hormone (GnRH), with consequent impairment of normal testicular or ovarian function. With a prevalence estimation, based on a civilian and military hospital series, of 1/4000 to 1/10 000 in males, CIHH is reported to be between two and five times more common in boys than in girls. CIHH patients usually come to clinical attention during adolescence or adulthood because of incomplete or absent pubertal development; owing to progress in molecular genetics and clinical practice, the diagnosis can be made earlier in cases where the diagnosis is specifically sought.

About the book

Impairments in the interaction between the central nervous system and the endocrine system can lead to a number of disorders in children.  These include type 1 diabetes, growth disorders, adrenal thyroid and pituitary problems, Addison’s disease and Cushing syndrome, among others.

Neuroendocrine Disorders in Children provides a comprehensive examination of paediatric and adolescent disorders focusing on the basic science and its clinical relevance. Complex issues are discussed in an easy-to-follow manner and the latest developments in the area are reviewed.

Read the full book review by Ieuan Hughes published in DMCN.

Readership: paediatric endocrinologists, paediatric and adult neurologists and trainee paediatricians.