amelogenesis imperfecta

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Introduct ion: Genomic conditions affects up dental enamel. Teeth affected may be discolored/sensitive or prone to disintegration. It may show autosomal dominant, autosomal recessive, sex linked and sporadic inheritance pattern. Diagnosis is based on the family history, pedigree plotting and meticulous clinical observations. Amelogenesis Imperfecta Hereditar y: Hereditary Enamel Dysplasia. Hereditary Brown Enamel. Hereditary Brown Opalescent Teeth.

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Page 1: Amelogenesis imperfecta

Introduction: Genomic conditions affects up dental enamel. Teeth affected may be discolored/sensitive or prone to

disintegration.It may show autosomal dominant, autosomal recessive, sex linked and sporadic inheritance pattern. Diagnosis is based on the family history, pedigree plotting and

meticulous clinical observations.

Amelogenesis Imperfecta

Hereditary: Hereditary Enamel Dysplasia. Hereditary Brown Enamel. Hereditary Brown Opalescent Teeth.

Page 2: Amelogenesis imperfecta

Types Of Enamel Defects Genetic: Hypoplastic (Type 1) Hypomaturation (Type 2) Hypocalcified (Type3) Hypomatured/ Hypoplactric (Type

4) Differential Interpretation

Non-Genetic: Dental Imperfecta Dental Fluorosis Chronological Enamel Formation Caries

Attrition Abrasion

Erosion Localized Nonhereditary Enamel Hypoplasia Localized Nonhereditary Enamel Hypocalcification

Fluorosis

Page 3: Amelogenesis imperfecta

Amelogenesis Imperfecta is a group of conditions caused by defects in the genes encoding enamel matrix proteins.

Amelogenesis Imperfecta

Type 1: Hypoplastic Amelogenesis Imperfecta:

The main defect is in formation of the matrix (protein). Enamel is not formed to full thickness because ameloblasts fail to lay

down sufficient matrix. Enamel is randomly pitted, grooved or very thin, but hard and

translucent. Affected teeth appear small with open contacts due to very thin or

nonexistent enamel causing thermal sensitivity. Teeth are not susceptible to caries unless the enamel is scanty and

easily damaged. Commonly in men.

Page 4: Amelogenesis imperfecta

Amelogenesis ImperfectaType 2: Hypomaturation Amelogenesis Imperfecta

Occurs during matrix apposition. Enamel is softer and chips from the underlying dentin. Enamel has a mottled brown-yellow-white color. Contact points present as enamel is of normal thickness. Radiographically enamel approaches the radio density of dentin.

Page 5: Amelogenesis imperfecta

Amelogenesis Imperfecta

Type 3: Hypocalcified Amelogenesis Imperfecta

Occurs during the calcification stage. Most common type. Enamel is of normal thickness but soft, friable, and easily lost by attrition. Enamel appears dull, lustrous, honey colored and stains easily.

Page 6: Amelogenesis imperfecta

Clinical Features– HypoplasticGeneralized pitted – it appears as thin enamel on teeth that do not contact each other mesiodistally. Pinpoint to pinhead pits are randomly distributed over the surfaceLocalised pitted – horizontal rows of depressions or one large hypoplastic area with hypocalcification adjacent to and below the hypoplastic area is found

Diffuse smooth – in this enamel is thin, hard and glossy with smooth surface

Diffuse rough – enamel is hard with rough granular surface that may be chipped from underlying dentine, rather than abrade away as soon with smooth type

Genetic

Page 7: Amelogenesis imperfecta

Type 1: Hypoplastic AmelogenesisImperfecta

Page 8: Amelogenesis imperfecta

Type 2: Hypomaturation AmelogenesisImperfecta

Page 9: Amelogenesis imperfecta

Type 3: Hypocalcified Amelogenesis Imperfecta

Page 10: Amelogenesis imperfecta

Type 4: Hypomatured/ Hypoplactric Amelogenesis Imperfecta

Page 11: Amelogenesis imperfecta

Clinical features – Hypocalcified

In this, enamel matrix is formed normally but without significant mineralization

The enamel is so soft that it can be removed by prophylactic instrument

Genetic

Page 12: Amelogenesis imperfecta

Clinical Features – Hypomaturation Type

In this there is defect in the maturation of enamel crystal structure

The enamel can be pierced by an Explorer point under firm pressure and can be lost by chipping away from the underlying, normal appearing dentine

Genetic

Page 13: Amelogenesis imperfecta

Differential InterpretationSquarish type of Crown being devoid of the normal mesial and distal contoursThe normal enamel cap is missing and in its place there is thin and opaque layer of enamelAbrasion is advanced then there is obliteration of pulp chamber

Enamel is less radiolucent than normal

Genetic

Page 14: Amelogenesis imperfecta

Non-Genetic

Fluorosis is a cosmetic condition that affects the teeth

It’s caused by overexposure to fluoride during the first eight years of life. This is the time when most permanent teeth are being formed.

Stains ranging from yellow to dark brown

Surface irregularities

Pits that are highly noticeable

Dental fluorosis 

Page 15: Amelogenesis imperfecta