Intro; Preface; Contents; 1: Hirschsprung's Disease: A Historical Overview; 1.1 Description of Hirschsprung's Disease; 1.2 Clarification of Pathophysiology; 1.3 Development of the First Successful Surgical Procedure; 1.4 Improvement of the Surgical Procedure; 1.5 Innovation of the Diagnostic Technique; 1.6 Recent Advance of the Research on Genetics and Embryonic Background; 1.7 Further Innovation of the Surgical Procedure; 1.8 Future Tasks; References; 2: Hirschsprung's Disease: Pathogenesis and Overview; 2.1 Historical Aspect; 2.2 Etiology; 2.2.1 Migration of Neural Crest Cells

2.2.1.1 Cranio-caudal Migration2.2.1.2 Sacral Nerve Compensation; 2.2.2 Microenvironment; 2.2.3 Cell Adhesion Molecules; 2.2.4 Neurotrophic Factors; 2.2.5 Immunologic Response; 2.2.6 Epithelial-Derived Signal; 2.2.7 Autophagy; 2.2.8 Genetics; References; 3: Genetic Aspect of Hirschsprung's Disease; 3.1 Hirschsprung's Disease; 3.2 Epidemiology and Classification; 3.3 Explorations in Genetics of HSCR; 3.4 The RET and the Endothelin Signalling Pathway; 3.5 Interaction Between Pathways; 3.6 Other Genes Related to Non-syndromic HSCR; 3.7 Multigenic Inheritance of Isolated HSCR

3.8 The Unanswered Question of Male Sex Bias of HSCR3.9 Syndromic HSCR; 3.10 Genetic Counselling; 3.11 Recent Progress and Future Directions; References; 4: Craniocaudal Migration/Neurocristopathy; 4.1 Craniocaudal Migration Theory; 4.2 Neural Crest Cell Migration; 4.3 Neurocristopathy; 4.3.1 Simple and Complex Neurocristopathy; 4.3.2 Complex Neurocristopathies Associated with HD; References; 5: Sacral Pathway Theory of Hirschsprung's Disease; References; 6: Vascular Abnormality and Ischemic Theory; 6.1 Ischemic Theory; 6.2 Abnormal Arteries in Hirschsprung's Disease

6.3 Abnormal Arteries in Allied Disorders of Hirschsprung's Disease6.4 Experimental Study; 6.5 Discussion; References; 7: Incidence and Sexual Difference; 7.1 Early Investigations on Incidence; 7.2 Incidences Around the World; 7.3 Recent Studies on Incidence; 7.4 Chronological Change of Incidence; 7.5 Difference of Incidence Between Ethnic Groups and Other Factors; 7.6 Sexual Difference; 7.7 Frequency of Types of Hirschsprung's Disease; 7.8 Sexual Difference of Patients with Distinctive Types of Hirschsprung's Disease; 7.9 Familial Incidence of Hirschsprung's Disease

7.10 Frequency of Hirschsprung's Disease Associated with Down SyndromeReferences; 8: Classification; References; 9: Symptom; 9.1 Introduction; 9.2 Symptoms; 9.2.1 Delayed Passage of Meconium; 9.2.2 Abdominal Symptoms; 9.2.3 Total Colonic Aganglionosis (TCA); 9.2.4 Impact of DS on Symptomatic Presentation; 9.3 Major Diseases Mimicking HD in the Neonatal Period; 9.3.1 Food Allergy; 9.3.2 Congenital Hypothyroidism; 9.4 Closing; References; 10: Hirschsprung's Disease Pathology; 10.1 Introduction; 10.2 Pathological Definition; 10.3 Histological Features; 10.4 Hirschsprung's Disease Form