Laron syndrome (LS), or primary growth hormone (GH) insensitivity, was first described in 1966. Since then, many patients worldwide have been diagnosed with LS, which involves defects in the GH receptor that cause combined congenital deficiency of GH and IGF-I activities. In this comprehensive book the authors draw upon 50 years of multidisciplinary clinical and investigative follow-up of the large Israeli cohort of LS patients. The genetic basis of the syndrome is fully considered, and all aspects of the pathophysiology of IGF-I deficiency are described. Data derived from the recently generated mouse model of LS are reviewed and compared with the human LS experience. Valuable advice is provided on treatment, and treatment effects, such as metabolic effects, adipose tissue alterations, and impact on aging, are fully explored. Together, this book condenses, consolidates, compares, and contrasts data derived from the human and mouse LS experiences and provides a unique resource for clinical and basic scientists to evaluate and compare IGF-I and GH actions.
Author(s): Zvi Laron (auth.), Zvi Laron, J. Kopchick (eds.)
Edition: 1
Publisher: Springer-Verlag Berlin Heidelberg
Year: 2011
Language: English
Pages: 531
Tags: Pediatrics; Endocrinology; Internal Medicine; Human Genetics; Molecular Medicine; Human Physiology
Front Matter....Pages i-xiv
Front Matter....Pages 1-1
History of the Israeli Cohort of Laron Syndrome Patients (1958–2009)....Pages 3-7
Early Investigations: Characterizations of the Circulating Growth Hormone....Pages 9-19
Clinical Evidence of Growth Hormone Resistance in Patients with Laron Syndrome....Pages 21-25
Diagnosis of Laron Syndrome....Pages 27-28
Genetic Aspects....Pages 29-52
Clinical Evaluation....Pages 53-57
Perinatal Development in Laron Syndrome....Pages 59-62
Linear Growth Pattern of Untreated Laron Syndrome Patients....Pages 63-89
Head Shape, Size, and Growth of Untreated Patients with Laron Syndrome....Pages 91-100
Sexual Development in Patients with Laron Syndrome....Pages 101-118
Sex Hormone Binding Proteins and Sex Hormones in Untreated and IGF-I Treated Patients with Laron Syndrome....Pages 119-123
The Adipose Tissue in Patients with Laron Syndrome....Pages 125-134
Investigations to Determine the Cause of Obesity in Patients with Laron Syndrome....Pages 135-142
Nonalcoholic Fatty Liver Disease (Nafld) in Patients with Laron Syndrome....Pages 143-147
Serum Lipids in Patients with Laron Syndrome....Pages 149-155
Adiponectin and Leptin in Laron Syndrome....Pages 157-160
Muscle Force and Endurance in Untreated Adult and IGF-I Treated Children with Laron Syndrome....Pages 161-164
Bone Mineral Density in Untreated and IGF-I or Alendronate-Treated Patients with Laron Syndrome....Pages 165-169
Muscle–Bone Relationship in Patients with Laron Syndrome....Pages 171-174
Imaging Findings in Laron Syndrome....Pages 175-199
Front Matter....Pages 1-1
Ocular Findings in Laron Syndrome....Pages 201-208
Hearing in Patients with Laron Syndrome....Pages 209-212
The Teeth in Patients with Laron Syndrome....Pages 213-217
Hair, Skin, and Nails in Patients with Laron Syndrome....Pages 219-226
Hand, Foot, and Organ Size and Growth in Untreated and IGF-I Treated Patients with Laron Syndrome....Pages 227-234
Lifelong Serum Growth Hormone Levels in Patients with Laron Syndrome....Pages 235-239
IGF-I Binding Proteins in Laron Syndrome....Pages 241-245
Serum Prolactin in Untreated and IGF-I Treated Patients with Laron Syndrome....Pages 247-254
Thyroid Hormones in Untreated and IGF-I Treated Patients with Laron Syndrome....Pages 255-258
Insulin Secretion and Carbohydrate Metabolism in Patients with Laron Syndrome: From Hypoglycemia to Diabetes Mellitus....Pages 259-272
Liver Enzymes in Patients with Laron Syndrome....Pages 273-281
The Hematopoietic System in Patients with Laron Syndrome....Pages 283-291
Cardiovascular Aspects in Laron Syndrome Patients....Pages 293-305
Kidney Functions in Untreated and IGF-I Treated Patients with Laron Syndrome....Pages 307-315
Sleep and Sleep Disorders in Patients with Laron Syndrome....Pages 317-320
Neurological Aspects in Laron Syndrome....Pages 321-322
Orthopedic Problems in Laron Syndrome....Pages 323-324
Psychological Aspects in Patients with Laron Syndrome....Pages 325-333
Adjustment and Rehabilitation Problems of Children, Adolescents, and Adults with Laron Syndrome....Pages 335-337
Laron Syndrome Patients with Congenital IGF-I Deficiency Seem Protected from Malignant Diseases....Pages 339-340
Front Matter....Pages 1-1
Lifespan and Mortality of Patients with Laron Syndrome....Pages 341-342
IGF-I Treatment of Patients with Laron Syndrome....Pages 343-380
IGF-I Stimulation of Head Growth in Patients with Laron Syndrome....Pages 381-387
Effects of Long-Term Administration of IGF-I on the Adipose Tissue and Carbohydrate Metabolism in Children with Laron Syndrome....Pages 389-408
Comparison of the Growth Promoting Response of IGF-I in Children with Laron Syndrome with that of hGH in Children with Isolated GH Deficiency....Pages 409-411
IGF-I Treatment of Adult Patients with Laron Syndrome....Pages 413-418
Adverse Effects Encountered During IGF-I Treatment of Patients with Laron Syndrome....Pages 419-424
Summary of the Clinical History: The Laron Syndrome Clock....Pages 425-426
Front Matter....Pages 427-427
The Laron Mouse....Pages 429-432
Role of GH/IGF-I Deficiency in Aging....Pages 433-440
Body Composition, Adipose Tissue, and Energy Balance....Pages 441-449
Metabolism and Metabolic Regulation....Pages 451-463
Skeletal Muscle....Pages 465-471
Cardiac Function in GHR–/– Mice....Pages 473-479
Bone....Pages 481-487
GHR Knockout and the CNS....Pages 489-493
Cancer....Pages 495-505
Reproduction....Pages 507-512
Conclusions and Future Studies....Pages 513-520
Back Matter....Pages 521-531
