Sale!
Dattani, Neuroendocrine Disorders in Children, Chapter 19 cover

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

This chapter is part of Neuroendocrine Disorders in Children

Product Type: PDF (Sub-Section of Book)

Series: Clinics in Developmental Medicine

Edition: 1st

Publication date: September 2016

Page count: 34

Buy now from Mac Keith Press

£3.91 £1.96 (inc 20% VAT)

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.

Pierre-Marc G Bouloux (Authors) Mehul Dattani (Editor)

Mehul Dattani  leads paediatric and adolescent endocrinology at Great Ormond Street Hospital for Children, London. His research interests are in the area of growth, pituitary development and disorders of puberty. Professor Dattani has received a number of national and international awards for his research work. He is currently Chair of the Programme Organizing Committee of the European Society for Paediatric Endocrinology.

Peter Hindmarsh (Editor)

Peter Hindmarsh is Professor of Paediatric Endocrinology at University College London and Divisional Clinical Director at the University College London Hospitals. His research interests include mathematical modelling of feedback systems including growth hormone, cortisol and insulin. He served as Chair of the working party on diabetes whose work led to major improvements in paediatric diabetes care in the UK.

Lucinda Carr (Editor)

Lucinda Carr  is Consultant Paediatric Neurologist at Great Ormond Street Hospital for Children, London, working across the departments of neurology and neurodisability. She served as the clinical lead in neurology at the hospital from 2008 to 2013. Dr Carr's main research interest is in movement disorders and cerebral palsy and she was a member of the team that developed the NICE guidelines on the management of spasticity in children.

Iain C A F Robinson (Editor)

Iain Robinson was head of the Division of Molecular Neuroendocrinology at the Medical Research Council National Institute for Medical Research until retirement in 2009. A major focus of interest was the study of hypothalmo-pituitary defects that cause growth retardation in children. His is group pioneered microsampling techniques in experimental animals and neonates, as well as developing novel transgenic and imaging techniques.

  • Full book contents
  • FOREWORD xiv
  • PREFACE xvi
  • SECTION 1: THE GH-IGF1 AXIS AND THE BRAIN
  • 1. NEUROENDOCRINE REGULATION OF GROWTH HORMONE
  • SECRETION 1
  • Peter C Hindmarsh and Iain CAF Robinson
  • 2. DISORDERS OF THE HYPOTHALAMO-PITUITARY-SOMATOTROPH
  • AXIS LEADING TO GROWTH FAILURE 13
  • Kyriaki S Alatzoglou and Mehul T Dattani
  • 3. THE EFFECT OF GROWTH HORMONE ON THE BRAIN 30
  • Fred Nyberg, Alfhild Grönbladh, Erika Brolin, Anna Jonsson,
  • Jenny Johansson and Mathias Hallberg
  • 4. IMPACT OF DISORDERS OF THE GROWTH HORMONE–IGF-1
  • AXIS ON NEUROLOGICAL FUNCTION 42
  • Emma A Webb and Mehul T Dattani
  • 5. NEUROENDOCRINE DISORDERS OF SALT AND WATER BALANCE 55
  • Joseph A Majzoub
  • SECTION 2: THE THYROID AXIS AND THE BRAIN
  • 6. THE HYPOTHALAMIC-PITUITARY-THYROID AXIS: ANATOMY
  • AND PHYSIOLOGY 74
  • Mary-Jane Brassill, Apostolos I Gogakos, John G Logan,
  • Julian A Waung and Graham R Williams
  • 7. DISORDERS OF THE HYPOTHALAMO-PITUITARY-THYROID AXIS 87
  • Tim Cheetham and Ramesh Srinivasan
  • 8. IMPACT OF THYROID DISORDERS ON NEUROLOGICAL FUNCTION 104
  • Joanne F Rovet
  • SECTION 3: REGULATION OF APPETITE AND WEIGHT
  • 9. THE ROLE OF THE HYPOTHALAMUS IN NORMAL WEIGHT
  • REGULATION 117
  • Simon M Luckman
  • 10. GENETIC CHILDHOOD OBESITY SYNDROMES 131
  • I Sadaf Farooqi
  • 11. TREATMENT OF OBESITY 142
  • Terry Segal and Bhavni Shah
  • SECTION 4: EFFECTS OF THE TREATMENT OF CANCER ON THE NEUROENDOCRINE SYSTEM
  • 12. LATE EFFECTS OF CANCER AND ITS TREATMENTS ON THE NEUROENDOCRINE SYSTEM 167
  • Wassim Chemaitilly and Charles A Sklar
  • 13. CRANIOPHARYNGIOMA 182
  • Hoong-Wei Gan, Juan Pedro Martinez-Barbera,
  • Helen A Spoudeas and Mehul T Dattani
  • SECTION 5: ADRENAL DISORDERS AND THE BRAIN
  • 14. THE HYPOTHALAMO-PITUITARY-ADRENAL AXIS AND
  • ITS REGULATION 206
  • Elizabeth Baranowski, Tulay Guran and Nils Krone
  • 15. THE EFFECT OF STRESS ON THE HYPOTHALAMIC-PITUITARY-
  • ADRENAL AXIS: AN UPDATE 218
  • Evangelia Charmandari and George P Chrousos
  • 16. ADRENOLEUKODYSTROPHY: NEUROLOGICAL ASPECTS 234
  • Patrick Aubourg
  • 17. CUSHING DISEASE: DIAGNOSIS AND MANAGEMENT 242
  • Helen L Storr and Martin O Savage
  • SECTION 6: NEUROENDOCRINOLOGY AND THE REPRODUCTIVE AXIS
  • 18. THE HYPOTHALAMIC-PITUITARY-GONADAL AXIS 255
  • Pierre-Marc G Bouloux
  • Neuroendocrine Disorders

'I found the remarks on genetic childhood obesity syndromes, Kallmann syndrome, and on effects of stress on the hypothalamic–pituitary–adrenal axis particularly informative. This volume can be recommended as a valuable source of up-to-date information about physiology, pathophysiology, molecular biology, and novel genetic aspects.' Eugen Boltshauser, Neuropediatrics, November 2016

'The Clinics books are intended to be an in-depth, up-to-date study of a particular topic. This book fills the bill. Each chapter provides a review of a specific topic then presents the most current research with a view to clinical application. The forward by Mitchell E. Geffner says it best “a one-stop shop for brain-hypothalamic-pituitary hormonal physiology and pathophysiology”. In each section the interplay between genetics and neuroendrocrine systems are an essential component. The effects of endrocrine disorders on the brain are also discussed in detail... I learned what we should be looking for in children we suspect may have neuroendocrine disorders but I also gained an understanding of how this system drives us and our patients to homeostasis, or not and the far reaching consequences of errors in the system.' AACPDM eNewsletter, Winter 2016