Dentinogenesis imperfecta
Encyclopedia
Dentinogenesis imperfecta (hereditary Opalescent Dentin) is a genetic disorder
of tooth development
. This condition causes teeth to be discolored (most often a blue-gray or yellow-brown color) and translucent. Teeth are also weaker than normal, making them prone to rapid wear, breakage, and loss. These problems can affect both primary (baby) teeth and permanent teeth. This condition is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. Dentinogenesis imperfecta affects an estimated 1 in 6,000 to 8,000 people.
Type II : Occurs in people without other inherited disorders (i.e. Osteogenesis imperfecta
).It is an autosomal dominant trait. A few families with type II have progressive hearing loss in addition to dental abnormalities.
Mutations in the DSPP gene
have been identified in people with type II and type III dentinogenesis imperfecta. Type I occurs as part of osteogenesis imperfecta.
The teeth may be gray to yellowish brown. They exhibit translucent or opalescent hue. Enamel is usually lost early due to loss of scalloping at the DEJ
. However, the teeth are not more susceptible to dental caries
than normal ones.
Type III shows thin dentin and extremely enormous pulp chamber.These teeth are usually known as Shell Teeth.
.
Due to the weakened condition of the teeth, many common cosmetic procedures such as braces and bridges are inappropriate for patients with Dentinogenesis imperfecta and are likely to cause even more damage than the situation they were intended to correct.
This article incorporates public domain text from The U.S. National Library of Medicine
Genetic disorder
A genetic disorder is an illness caused by abnormalities in genes or chromosomes, especially a condition that is present from before birth. Most genetic disorders are quite rare and affect one person in every several thousands or millions....
of tooth development
Tooth development
Tooth development or odontogenesis is the complex process by which teeth form from embryonic cells, grow, and erupt into the mouth. Although many diverse species have teeth, non-human tooth development is largely the same as in humans...
. This condition causes teeth to be discolored (most often a blue-gray or yellow-brown color) and translucent. Teeth are also weaker than normal, making them prone to rapid wear, breakage, and loss. These problems can affect both primary (baby) teeth and permanent teeth. This condition is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. Dentinogenesis imperfecta affects an estimated 1 in 6,000 to 8,000 people.
Types
Types of dentinogenesis imperfecta with similar dental formalities usually an autosomal dominant trait with variable expressivity but can be recessive if the associated osteogenesis imperfecta is of recessive type. This type is no lType II : Occurs in people without other inherited disorders (i.e. Osteogenesis imperfecta
Osteogenesis imperfecta
Osteogenesis imperfecta is a genetic bone disorder. People with OI are born with defective connective tissue, or without the ability to make it, usually because of a deficiency of Type-I collagen...
).It is an autosomal dominant trait. A few families with type II have progressive hearing loss in addition to dental abnormalities.
Mutations in the DSPP gene
Gene
A gene is a molecular unit of heredity of a living organism. It is a name given to some stretches of DNA and RNA that code for a type of protein or for an RNA chain that has a function in the organism. Living beings depend on genes, as they specify all proteins and functional RNA chains...
have been identified in people with type II and type III dentinogenesis imperfecta. Type I occurs as part of osteogenesis imperfecta.
Clinical features
Clinical appearance is variable. However, the teeth usually involved and more severely affected are deciduous teeth in type 1; whereas in type 2 both the dentitions are equally affected.The teeth may be gray to yellowish brown. They exhibit translucent or opalescent hue. Enamel is usually lost early due to loss of scalloping at the DEJ
Dental-enamel junction
The dentino-enamel junction, abbreviated DEJ, is the boundary between the enamel and the underlying dentin that form the solid architecture of a tooth.It is also known as the amelo-dentinal junction, or ADJ....
. However, the teeth are not more susceptible to dental caries
Dental caries
Dental caries, also known as tooth decay or a cavity, is an irreversible infection usually bacterial in origin that causes demineralization of the hard tissues and destruction of the organic matter of the tooth, usually by production of acid by hydrolysis of the food debris accumulated on the...
than normal ones.
Radiographic features
Type I and II show total obliteration of the pulp chamber.Type III shows thin dentin and extremely enormous pulp chamber.These teeth are usually known as Shell Teeth.
Histology
Dentinal tubules are irregular and are bigger in diameter. Areas of uncalcified matrix are seen. Sometimes odontoblasts are seen in dentinDentin
Dentine is a calcified tissue of the body, and along with enamel, cementum, and pulp is one of the four major components of teeth. Usually, it is covered by enamel on the crown and cementum on the root and surrounds the entire pulp...
.
Treatment
One treatment option is bonding, putting lighter enamel on the weakened enamel of the teeth and with lots of treatments of this bonding, the teeth appear whiter to the eye, but the teeth on the inside and under that cover are still the same.Due to the weakened condition of the teeth, many common cosmetic procedures such as braces and bridges are inappropriate for patients with Dentinogenesis imperfecta and are likely to cause even more damage than the situation they were intended to correct.
This article incorporates public domain text from The U.S. National Library of Medicine