Hormone Resistance and Other Endocrine Paradoxes

1. Introduction: The Journey of the Endocrine Signal: A Paradigm of Murphy’s Law.- I. Chemical Control of Morphogenesis.- II. Hormone Biosynthesis.- III. Hormone Storage and Release.- IV. Hormone Transport.- V. Hormone Binding and Recognition.- VI. Transduction of the Endocrine Signal.- VII. Extinction of the Endocrine Signal.- VIII. Drug-Induced Changes of the Endocrine Signal.- IX. Summary.- 2. Insulin Gene Mutations and Abnormal Products of the Human Insulin Gene.- I. Insulin Biosynthesis.- II. Abnormal Insulin Gene Products.- III. Structure-Function Relationships and Biological Activity.- IV. Clinical Relevance.- V. Summary and Perspectives.- 3. Euthyroid Hyperthyroxinemia.- I. Binding Abnormalities.- II. Hormone Resistance Syndromes.- III. Nonthyroidal Illness.- IV. Drugs.- V. Exogenous T4 Administration.- VI. High Altitude.- VII. Summary.- 4. Pseudohypoparathyroidism: Target Organ Resistance to Parathyroid Hormone and Other Metabolie Defects.- I. A Selective Historical Review.- II. Classification.- III. Pathogenesis.- IV. Function of Other Endocrine Systems in PsHP.- V. Genetics of PsHP.- VI. Treatment of PsHP.- 5. Syndromes of Vitamin D Resistance.- I. Vitamin D Metabolism.- II. Vitamin D Physiology.- III. Hormone Action.- IV. Vitamin D and Elemental Deficiency.- V. Phosphate-Wasting Syndromes.- VI. Vitamin D—Dependent Rickets.- VII. Summary.- 6. Androgen Insensitivity Syndromes: Paradox of Phenotypic Feminization with Male Genotype and Normal Testicular Androgen Secretion.- I. Historical Considerations.- II. Normal Androgen Physiology.- III. Classification of Male Pseudohermaphroditism.- IV. Molecular Mechanisms of Androgen Action in Target Cells.- V. Clinical Syndrome of 5?-Reductase Deficiency.- VI. Androgen Receptor and Postreceptor Defects.- VII.Concluding Remarks.- 7. States of Aldosterone Deficiency or Pseudodeficiency.- I. Physiologic Effects of Mineralocorticoid Hormones.- II. Syndromes of Aldosterone Deficiency and Pseudodeficiency.- 8. Bartter’s Syndrome.- I. Case Summaries.- II. Historical Perspective.- III. Etiology and Pathophysiology.- IV. Differential Diagnosis.- V. Clinical Evaluation.- VI. Treatment.- VII. Analysis of Cases.