Foreword Janet H. Silverstein / v
Foreword Robert M. Blizzard / v
Foreword Joseph I. Wolfsdorf / vii
Foreword Maria I. New / vii
Preface / ix
Acknowledgments / xi
Contributors / xxi
VOLUME 1
SECTION I: OBESITY
1. Obesity in Children / 1
Ramin Alemzadeh, Russell Rising, and Fima Lifshitz
Prevalence / 1
Morbidity / 2
Economic Impact of Obesity / 6
Assessment / 7
Who Is at Risk? / 10
Genetics / 11
Food Intake Regulation / 14
Hormonal Alterations / 18
Energy Balance / 20
Physical Activity and Fitness / 22
Nutritional Considerations / 23
Final Considerations / 25
References / 25
2. Treatment of Pediatric Obesity / 37
Diego Botero and Fima Lifshitz
Introduction / 37
Prevention / 38
Lifestyle-Based Treatments / 40
Therapeutic Goals / 47
Intensive Therapies / 48
Conclusion and Final Considerations / 51
References / 52

SECTION II: DIABETES
3. Diabetes in the Child and Adolescent: Diagnosis
and Classification / 57
Arlan L. Rosenbloom
Introduction / 57
Diagnosis / 57
Etiologic Classification of Diabetes / 57
Classification of Diabetes in Childhood / 59
References / 61
4. Type 1 Diabetes in the Child
and Adolescent / 63
Michael J. Haller, Janet H. Silverstein, and
Arlan L. Rosenbloom
Type 1 Diabetes Mellitus / 63
Epidemiology / 63
Etiology and Prevention / 64
Clinical Picture / 67
Complications / 68
Conclusion / 76
References / 77

5. Diabetes Autoimmunity / 83
William E. Winter
Autoimmunity to the Pancreatic Islets / 83
Etiopathogenesis / 84
Natural History of Type 1 Diabetes / 84
Genetics of Type 1 Diabetes / 85
Environmental Triggers in the Pathogenesis of Type 1
Diabetes / 87
Islet Autoantibodies / 87
Other Immune Abnormalities in Type 1 Diabetes / 89
Prediction, Immunotherapy, and Prevention of Type 1
Diabetes / 89
Pancreas, Islet, and Stem Cell Transplantation / 90
Clinical Approach to the Diagnosis of Diabetes / 91
References / 93
6. Management of the Child with
Type 1 Diabetes / 101
Oscar Escobar, Allan L. Drash, and Dorothy J. Becker
Introduction / 101
Components of Management of Diabetes in Children / 102
Management of Acute Complications / 113
Therapeutic Objectives and Monitoring
Requirements / 114
The Therapeutic Team / 117
Clinical Assessment and Therapeutic
Decision Making / 118
Consultations and Referrals / 119
Future Directions / 120
References / 121
7. Practical Aspects of Diabetes Care / 125
Shannon P. Lyles, Janet H. Silverstein, and Arlan L. Rosenbloom
Introduction / 125
Blood Glucose Monitoring / 125
Insulin Injections / 128
Continuous Subcutaneous Insulin Infusion / 130
Sick Day Management / 139
Hypoglycemia / 140
Exercise / 144
Travel / 145
New Gadgets / 146
Diabetes Education / 149
School Care / 150
Diabetes Camp / 152
Support Groups / 153
Conclusion / 153
References / 153
8. Hyperglycemic Comas in Children / 155
Arlan L. Rosenbloom
Introduction / 155
Frequency / 155
Pathophysiology / 156
Morbidity and Mortality / 156
Hyperglycemic Hyperosmolar State / 156
Rhabdomyolysis / 156
Mucormycosis / 157
Pancreatitis / 157
Peripheral Venous Thrombosis / 157
Intracerebral Complications Other than Idiopathic
Cerebral Edema / 157
xiii

Cerebral Edema / 158
Management of Diabetic Ketoacidosis / 161
Conclusions / 165
References / 165
9. Type 2 Diabetes in the Child and Adolescent / 169
Jennifer Miller, Janet H. Silverstein, and Arlan L. Rosenbloom
Introduction / 169
Epidemiology / 169
Etiology / 170
Clinical Picture / 174
Prevention and Treatment / 176
References / 183
10. Maturity-Onset Diabetes of Youth and Other Genetic
Conditions Associated with Diabetes / 189
William E. Winter
Introduction / 189
Beta Cell Function: A Review of Normal Physiology / 189
Genetic Defects in Beta Cell Function / 192

Genetic Defects in Insulin Action: Insulin Receptor and Post-
Receptor Events / 197

Diseases of the Exocrine Pancreas / 198
Other Genetic Syndromes Associated with DM / 199
Clinical Commentary / 200
Conclusion / 202
References / 202

SECTION III: INSULIN RESISTANCE AND ITS COMPLICATIONS
11. Insulin Resistance Syndrome in Childhood
and Beyond / 211
Berrin Ergun-Longmire and Noel K. Maclaren
Introduction / 211
Epidemiology / 211
Pathophysiology of IR / 212
Pathogenesis and Genetics of IR / 222
Clinical Features / 225
Diagnosis / 230
Screening / 231
Treatment / 232
Summary / 235
References / 235
12. Low Birth Weight and Endocrine Dysfunction in
Postnatal Life / 251
Maria Vero ́nica Mericq G.
Introduction / 251
Genetic Factors of Fetal Growth / 251
In Utero Programming / 252
Postnatal Programming of Endocrine Dysfunction / 254

Mechanisms of Development of Insulin Resistance in Small-for-
Gestational-Age Subjects / 255

Gonadal and Adrenal Axis / 256
Somatotropic Axis / 256
Conclusions / 257
References / 257
13. Hypertension in Children: Endocrine
Considerations / 261
Julie R. Ingelfinger
Introduction / 261
Normal Blood Pressure and Its Definition / 261
Etiology of Hypertension / 261
Complications of Hypertension: Patterns
and Diagnosis / 261
Steroidogenic Enzyme Defects / 265
Primary Aldosteronism: Aldosterone-Producing Adenoma and
Bilateral Adrenal Hyperplasia / 268

Glucocorticoid-Responsive Aldosteronism–Dexamethasone-
Suppressible Hyperaldosteronism

(OMIM # 103900) / 268
Apparent Mineralocorticoid Excess (OMIM # 218030) / 268
Mutations in Renal Transporters Causing Low-Renin
Hypertension / 269
Cushing Syndrome and Hypertension / 270
Hypertension in Pheochromocytoma and Neural Crest
Tumors / 270
Hypertension in Thyroid Disease / 271
Hyperparathyroidism and Hypertension / 271
Prevention of Hypertension and Endocrine Systems / 271
Primary Hypertension: How Often Endocrine? / 273
Treatment / 274
References / 274
14. Lipid Disorders in Children and Adolescents / 279
Hofit Cohen and Raanan Shamir
Introduction / 279
Lipids and Lipoproteins / 279
Rationale for Cardiovascular Disease Preventive Measures
During Childhood / 281
Monogenic Disorders Resulting in High
LDL-C Levels / 283
Inherited Hypertriglyceridemia / 284
Initial Evaluation of Children with Hyperlipidemia / 285
Management of Children with Hypercholesterolemia / 285
Management of Children with Hypertriglyceridemia / 287
Conclusions / 288
References / 288

SECTION IV: HYPOGLYCEMIA
15. Hypoglycemia in Children / 291
Joseph I. Wolfsdorf and David A. Weinstein
Introduction / 291
Definition of Hypoglycemia / 291
An Overview of Fuel Metabolism / 292
Regulation of Insulin Secretion / 294
Clinical Manifestations of Hypoglycemia / 295
Causes of Hypoglycemia in Infants and Children / 296
Determining the Cause of Hypoglycemia / 311
Treatment / 313
Hypoglycemia and Diabetes Mellitus / 314
References / 317
16. Hypoglycemia in the Newborn / 329
Hussien M. Farrag and Richard M. Cowett
Introduction / 329
Neonatal Euglycemia and Hypoglycemia / 329
Glucose Metabolism / 333
Clinical Assessment / 338
Preterm AGA Neonates / 340
Small for Gestational Age Infants / 340
Congenital Heart Disease / Congestive
Heart Failure / 342
Perinatal Stress/Hypoxia / 343
Cold Injury and Sepsis / 343
Hyperinsulinism: The Infant of the Diabetic Mother / 344
Rh Incompatibility and Hypoglycemia / 346
Exchange Transfusion and Umbilical Catheter / 346
Persistent Hyperinsulinemic Hypoglycemia / 346
Hypoglycemia Following Maternal Ethanol
Consumption and Miscellaneous Causes / 348
Beckwith–Wiedemann Syndrome / 348
Defective Gluconeogenesis/Glycogenolysis / 348
Evaluation / 349
Treatment / 350
References / 353

xiv Contents

17. Emergencies of Inborn Metabolic Disease / 359
Jose E. Abdenur
Introduction / 359
Urea Cycle Defects / 359
Organic Acidemias / 364
Maple Syrup Urine Disease / 371
Fatty Acid Oxidation Defects / 372
Newborn Screening with Tandem Mass Spectrometry / 378
Primary Lactic Acidemias / 387
References / 393

SECTION V: SPECIAL CONSIDERATIONS AND RESOURCES
18. Private Practice of Pediatric Endocrinology / 403
Richard Mauseth
Introduction / 403
Private and Academic Practices / 403
Personal Experience / 404
Type of Practice / 405
Building a New Practice / 407
Billing / 410
Electronic Medical Records / 413
Outreach Clinics / 413

Participating in Research / 414
Final Considerations / 415
References / 415
19. Clinical Research in Children / 417
Phillip D. K. Lee
Introduction / 417
Definitions / 418
Pediatric Considerations / 419
Protocol Design / 420
Special Considerations / 423
Clinical Research and Evidence-Based Medicine / 427
Conclusions / 428
References / 428
20. Reference Charts and Tables Frequently Used by
Endocrinologists / 431
Adriana A. Carrillo and Fima Lifshitz
Standards of Growth / 433
Miscellaneous Measurements and Standards / 448
Growth Charts for Children with Genetic Conditions / 471
Index of Volume 1 / 501

 

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