Hypocalcified autosomal recessive amelogenesis imperfecta—A case report


Amelogenesis imperfecta is an unusual hereditory disorder affecting both primary and permanent dentition.

We present a rare case of hypocalcified autosomal recessive amelogenesis imperfecta occuring in primary dentition in a 7-year-old girl with a family history of consanguineous marriage.

Clinical and radiological examination revealed discoloration and hypoplasia of enamel with easy chipping affecting both maxillary and mandibular dentition.

INTRODUCTION : Amelogenesis imperfect (AI) encompasses a heterogeneous group of developmental disorders that demonstrate alterations in the enamel.

Read Also : PERIODONTICS : Periodontal Diseases of Children and Adolescents


AI is caused by mutations in genes that control amelogenesis and follows inheritance patterns of autosomal-dominant, autosomal recessive or X-linked modes of transmission.

The enamel may be hypoplastic, hypomineralised or both, and teeth affected may be discoloured, sensitive or prone to disintegration either post eruption (post-eruptive breakdown) or preeruption (idiopathic resorption).

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Open Journal of Stomatology
Ali Azhar Dawasaz / M. Zakirulla / Meer Allahbaksh




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