Oral Manifestations of Inherited Disorders focuses on inherited systemic disorders presenting oral manifestations that have been reported as an integral part of the disorder. This book discusses some inherited conditions affecting only calcified dental tissues.
Organized into seven chapters, this book begins with an overview of the distinctive pattern of signs and symptoms that enable the clinician to make a diagnosis. This text then examines the hereditary defects in enamel. Other chapters consider the characteristics of acroosteolysis. This book discusses as well the oral structures in patients with Apert syndrome. The final chapter deals with the condition of beard-headed dwarfism, which is characterized by low birth weight, dwarfism, microcephaly, mental retardation, and beak-like appearance of the mid-face.
This book is a valuable resource for those professionals interested in the malformation syndromes and in the oral manifestations of inherited disorders. Clinicians, geneticists, dentists, and physicians will also find this book useful.

Contenu

Preface
1 Genetic Counseling and Oral Examination
1.1 Genetic Counseling
1.2 Physical Examination of the Oral Cavity
1.3 Radiologic Interpretation of Some Oral and Paraoral Structures
2 Some Inherited Conditions Affecting Only Calcified Dental Tissue
2.1 Amelogenesis Imperfecta
2.2 Coronal Dentin Dysplasia
2.3 Hereditary Opalescent Dentin
2.4 Radicular Dentin Dysplasia
3 Systemic Inherited Disorders with Dental Involvement
3.1 Acroosteolysis
3.2 Amelocerebrohypohidrosis Syndrome
3.3 Cryptodontic Brachmetacarpalia
3.4 Hypodontia and Nail Dysgenesis
3.5 Hypophosphatasia (Phosphoethanolaminuria)
3.6 Hypophosphatemic Vitamin D-Resistant Rickets
3.7 Hypoplastic-Hypocalcified Enamel, Onycholysis, and Functional Hypohidrosis
3.8 Incontinentia Pigmenti
3.9 Oculodentoosseous Dysplasia
3.10 Otodental Syndrome
3.11 Pseudohypoparathyroidism and Pseudopseudohypoparathyroidism
3.12 Rothmund-Thomson Syndrome
4 Systemic Inherited Disorders with Oral or Perioral Soft Tissue Involvement
4.1 Acrodermatitis Enteropathica
4.2 Bloom Syndrome
4.3 Chédiak-Higashi Syndrome
4.4 Congenital Indifference to Pain
4.5 Cutis Laxa
4.6 Cyclic Neutropenia
4.7 Double Lip, Blepharochalasis, and Nontoxic Thyroid Enlargement
4.8 Dyskeratosis Congenita with Pigmentation, Dystrophic Nails, Aplastic Anemia, and Leukoplakia Oris
4.9 Fabry Syndrome
4.10 Familial Dysautonomia
4.11 Gingival Fibromatosis with Ear, Bone, and Nail Defects and Hepatosplenomegaly
4.12 Gingival Fibromatosis with Hypertrichosis, Epilepsy, and Mental Retardation
4.13 Gingival Fibromatòsis with Multiple Hyaline Fibromas
4.14 Hereditary Benign Intraepithelial Dyskeratosis
4.15 Hereditary Hemorrhagic Telangiectasia
4.16 Hermansky-Pudlak Syndrome
4.17 Hyperkeratosis Palmoplantaris and Attached Gingival Hyperkeratosis
4.18 Lesch-Nyhan Syndrome
4.19 Moebius Syndrome
4.20 Multiple Hamartoma and Neoplasia Syndrome
4.21 Multiple Mucosal Neuromas, Medullary Carcinoma of the Thyroid, Pheochromocytoma, and Marfanoid Body Build with Muscle Wasting
4.22 Neurofibromatosis
4.23 Pachydermoperiostosis
4.24 Pachyonychia Congenita, Jadassohn-Lewandowski Type
4.25 Peutz-Jeghers Syndrome
4.26 Pseudoxanthoma Elasticum
4.27 Xerodermie Idiocy
4.28 White Sponge Nevus
5 Systemic Inherited Disorders with Involvement of Jaw Bones
5.1 Apert Syndrome
5.2 Carpenter Syndrome
5.3 Cerebrohepatorenal Syndrome
5.4 Cherubism
5.5 Chondrodysplasia Punctata
5.6 Craniofacial Dysostosis
5.7 Craniometaphyseal Dysplasia and Craniodiaphyseal Dysplasia
5.8 Infantile Cortical Hyperotosis
5.9 Mandibulofacial Dysostosis
5.10 Marfan Syndrome
5.11 Melnick-Needles Syndrome
5.12 Mucopolysaccharidosis VII
5.13 Pfeiffer Syndrome
5.14 Smith-Lemli-Optiz Syndrome
5.15 Waardenburg Syndrome
6 Systemic Disorders Associated with Facial Clefting
6.1 Cleft Lip-Palate and Congenital Lip Pits
6.2 Cleft Lip-Palate and Tetraphocomelia
6.3 Cleft Lip-Palate, Popliteal Pterygium, Digital and Genital Anomalies Syndrome
6.4 Cleft Palate, Flattened Facies, and Multiple Congenital Dislocations
6.5 Diastrophic Dwarfism
6.6 Ectrodactyly-Ectodermal Dysplasia-Clefting (EEC) Syndrome
6.7 Hereditary Progressive Arthroophthalmopathy
6.8 Meckel Syndrome
6.9 Multiple Pterygium Syndrome
6.10 Otopalatodigital Syndrome
7 Systemic Inherited Disorders with Involvement of Multiple Oral Structures
7.1 Bird-Headed Dwarfism
7.2 Cleidocranial Dysplasia
7.3 Cockayne Syndrome
7.4 Frontometaphyseal Dysplasia
7.5 Gardner Syndrome
7.6 Hypertelorism-Hypospadias Syndrome
7.7 Mucopolysaccharidosis IV
7.8 Multiple Nevoid Basal Cell Carcinoma Syndrome
7.9 Osteogenesis Imperfecta
7.10 Pyknodysostosis
7.11 Rieger Syndrome
7.12 Saethre-Chotzen Syndrome
7.13 Trichodentoosseous Syndrome
7.14 Chondroectodermal Dysplasia
7.15 Congenital Hypertrophy of the Gingiva, Altered Eruption of Teeth, and Corneal Dystrophy
7.16 Endocrine, Candidosis Syndrome
7.17 Epidermolysis Bullosa
7.18 Hyalinosis Cutis et Mucosa
7.19 Hypohidrotic (Anhidrotic) Ectodermal Dysplasia
7.20 Mucolipidosis II
7.21 Mucopolysaccharidosis III
7.22 Mucopolysaccharidosis VI
7.23 Craniocarpotarsal Dysplasia
7.24 Cryptophthalmos Syndrome
7.25 Generalized Gm1 Gangliosidosis
7.26 Gingival Fibromatosis, Hypopigmentation, Microphthalmia, Oligophrenia, and Athetosis
7.27 Mucopolysaccharidosis II
7.28 Myotonic Dystrophy
7.29 Trichorhinophalangeal Syndrome
7.30 Tuberous Sclerosis
7.31 Coffin-Lowry Syndrome
7.32 Dubowitz Syndrome
7.33 Ehlers-Danlos Syndrome
7.34 Fetal Face Syndrome
7.35 Focal Dermal Hypoplasia Syndrome
7.36 Hyperkeratosis Palmoplantaris and Periodontoclasia in Childhood
7.37 Macroglossia-Omphalocele-Visceromegaly Syndrome
7.38 Mucopolysaccharidosis I-H
7.39 Orofacialdigital Syndrome I
7.40 Orofacialdigital Syndrome II
7.41 Jaw-Winking and Winking-Jaw Syndromes
Glossary of Dental Terms
Photo Credits
Index