Amelogenesis imperfecta: causes, symptoms, diagnosis and treatment

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Amelogenesis imperfecta: causes, symptoms, diagnosis and treatment
Amelogenesis imperfecta: causes, symptoms, diagnosis and treatment

Video: Amelogenesis imperfecta: causes, symptoms, diagnosis and treatment

Video: Amelogenesis imperfecta: causes, symptoms, diagnosis and treatment
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Amelogenesis imperfecta is a rather rare genetic disease, which is a violation of enamel formation with further destruction of the tooth structure. The formation of defective enamel can disrupt the mineralization of teeth. In the future, discoloration may be observed along with a change in the color of the enamel, which begins to acquire a brown or gray tint. A disease such as imperfect amelogenesis is rarely found in its pure form. As a rule, this disease is accompanied by other dental disorders. For example, there are imperfect amelogenesis and dentinogenesis, the latter is a hereditary malformation of dentin. The combination of the two pathologies is called the Stanton-Capdepon syndrome. Next, we will find out what are the main causes, and, in addition, the symptoms of the development of this disease, and find out how the diagnosis and treatment of the pathology in question is carried out.

amelogenesis imperfectaclassification
amelogenesis imperfectaclassification

Description of pathology

Amelogenesis imperfecta is a concern for many people. This disease suggests the presence of a violation of the formation of enamel. The described disease is observed in men and women. But it is worth noting that among the fair sex, this disease is fixed much more often.

This dental disease is usually inherited. Finally, the factors of its occurrence have not been studied. The key causes of the disease are gene mutations. They may appear in different ways. People may develop a thin layer of enamel or it may be completely absent. The color of the enamel also often changes. It comes in opaque brown or white.

With amelogenesis imperfecta, teeth look like plaster, they lack shine.

In some cases, the enamel has a rough surface. With a similar clinical picture, the thickness of the outer protective shells of the teeth decreases slightly.

Classification of amelogenesis imperfecta

There are 4 main forms of the disease.

amelogenesis imperfecta causes symptoms diagnosis and
amelogenesis imperfecta causes symptoms diagnosis and
  1. Hypoplastic form. It is all to blame for the violation of tissue differentiation and the secretory activity of ameloblasts.
  2. Hypomaturation. The reason for this is a failure at the stages of formation and primary mineralization of the enamel matrix.
  3. Hypocalcification form. The phase of mineralization is broken. There is an abnormal growth of crystallites and a decrease in the mineral component of the enamel.
  4. Hypomaturation withhypoplasia and taurodontism. Even at the stage of tissue differentiation and layering of the enamel matrix, a failure occurs.

Hereditary pathologies as factors in the occurrence of the disease

All hereditary pathologies are conditionally divided into two large categories: gene and chromosomal. In dentistry, gene diseases are often encountered that are transmitted to people from generation to generation, even without certain changes. So, in the event that one of the parents was diagnosed with a pathological process in hard dental tissues, then the probability of the appearance of exactly the same disease in a child is approximately fifty percent. At the same time, anomalies can equally affect both girls and boys, that is, the pathology does not depend on gender.

Dysplasia of tooth enamel, accompanied by damage to the dentin, is a severe hereditary pathology caused by a mutation of the genes that are responsible for the formation of a specific matrix protein. In a child, this disease can affect milk or permanent teeth.

In the presence of imperfect amelogenesis, the connection of depleted enamel with dentin is broken. The layer of hard dental tissue in this disease has a weak connection. Against the background of the destruction of the enamel and dentine junction, caries begins to rapidly spread to the area of hard tissues. As a result of this process, as a rule, replacement dentin is soon formed, and the root canals of the tooth, in turn, are affected by calcification.

Main causes of pathology

Hereditary amelogenesis imperfecta is the most commonly diagnosed. Excepthereditary predisposition to the appearance of dentin pathology, doctors distinguish the following reasons:

  • Use of certain drugs by a woman during pregnancy, especially antibiotics.
  • Failure of the intrauterine metabolic process in the baby.
  • The appearance of severe toxicosis in the expectant mother, which causes a violation of the supply of nutrients to the child.
  • Use of poor-quality water and food in the first years of a child's life.

All of the above factors are not the root causes of amelogenesis. These reasons can only increase the risk of developing pathology if the child has a hereditary predisposition.

amelogenesis imperfecta and dentinogenesis
amelogenesis imperfecta and dentinogenesis

Symptoms of pathology

Amelogenesis imperfecta can have several variants of its manifestation, each of them will have its own symptoms.

  • Sick children's teeth may come out on time, but will be smaller. The distance between them is usually quite large. The enamel will have a smooth structure, and the roots will not be changed.
  • Enamel in this disease may have a more pronounced destructive change, and the teeth themselves, as a rule, are formed in the form of a cone or cylinder. Due to insufficient calcification of the enamel, its surface has a rough structure. In some places, the teeth are completely bare to the very dentin, while their root system can develop directly without any pathology.
  • If there is a regularthe size of the teeth, the enamel may have a corrugated appearance. On the hard surface of the enamel, there are chaotic or vertical grooves. Such dysplasia usually affects all teeth.
  • In the presence of a normal form of teeth, the enamel is very depleted and has a chalky texture against the background of this disease. Even with minor injuries, enamel can easily separate from dentin. Patients are additionally able to develop hyperesthesia in the form of increased sensitivity of dental tissues.

Characteristic signs of the disease

A characteristic manifestation of this disease, regardless of its form, is the color of the enamel, which can vary from gray to brown. Often there are the first and fourth types of enamel dysplasia. This disease accounts for about sixty-six percent of all dental diseases. Such a disease as dentinogenesis imperfecta is extremely rare in its pure form and clinically capable of not manifesting itself in any way. The development of this disease can only be indicated by excessive sensitivity of the dentin.

In patients with this disease, the enamel may also take on a purple hue. This color is due to the filling of the dentin with blood. Due to the failure of mineralization processes, a structural violation of the dentin occurs. In the future, this process can lead to tooth decay. Crowns may have sharp edges that can injure the mucous surface of the oral cavity.

amelogenesis imperfecta clinic
amelogenesis imperfecta clinic

Amelogenesis imperfecta in children

This pathology of teeth in children is usually non-carious in nature and is an extremely dangerous disease. Signs of anomalies in children practically do not differ from those in adults. This disease in children is characterized by clear symptoms that differ from other dental problems. These signs include:

  • The appearance of changes in the color of the enamel.
  • Presence of increased tooth sensitivity.
  • Presence of enamel chips.
  • The presence of cutting, and, in addition, sharp edges.
  • Presence of destruction of tooth crowns.
  • The appearance of spots and irregularities on the surface of the enamel.

Enamel dysplasia of milk teeth is diagnosed most often. This dental disease can affect the entire jaw of a child, and, in addition, some of its parts.

Diagnostics

In the presence of imperfect amelogenesis, the clinic must be contacted for examination.

amelogenesis imperfecta differential diagnosis
amelogenesis imperfecta differential diagnosis

Diagnosis is usually based on history taking. In addition, it is necessary to conduct a clinical examination of the patient and perform additional studies. The genealogical method for diagnosing this disease is the most common and effective. During its implementation, the patient's pedigree is compiled and a genealogical analysis is carried out.

Differential diagnosis of amelogenesis imperfecta also performed.

The dentist evaluates the current state of the teeth of not only the patient himself, but also hisrelatives. In this case, it is extremely important to evaluate the color along with the structure of the enamel, the presence of possible irregularities, etc. As part of the analysis of genetic material, the frequency is noted along with the severity of various hereditary traits. In some situations, the patient is prescribed x-rays, as well as checking the pulp for excitability.

Next, we will find out how this disease is currently being treated.

Treatment of disease

Treatment of non-carious diseases of dental tissue begins with the process of enamel restoration. The affected tooth surface not only disrupts the aesthetics of a smile, but can additionally damage dental integrity.

In the early stages of the development of pathology, the doctor usually resorts to endodontic therapy, which is aimed at saving the tooth. To strengthen the tooth enamel, the doctor prescribes complex remineralizing therapy. Patients are advised to take calcium supplements along with vitamin and mineral complexes.

What else is the treatment for amelogenesis imperfecta?

hereditary amelogenesis imperfecta
hereditary amelogenesis imperfecta

For oral and dental hygiene, it is best to use fluoride toothpaste along with rinse formulations. Patients need to undergo a course of fluoridation, as a result of which tooth enamel can be strengthened, and as a result, resistance to caries will increase.

In the event that the remineralizing procedure does not bring the proper result, and the integrity of the dental tissues remains impaired,resort to prosthetics.

Composite materials are also used. Under an artificial crown, dentinogenesis imperfecta can continue to progress, destroying the dentin. Prosthetics against this background will only be temporary and is usually aimed at eliminating aesthetic inconvenience.

When treating a disease in children, remineralizing therapy should be carried out as early as possible, as soon as the first milk teeth appear. With complex therapy by dentists, a good result is predicted.

Glass ionomer cement

In the event that imperfect dentinogenesis is diagnosed in babies, then glass ionomer cement is used to restore crowns. This material has good adhesion to dentin and enamel, which provides reliable protection against caries. Glass ionomers can perfectly integrate into the child's body and do not cause side effects.

Carrying out the prevention of pathology

The best prevention of dental disease is regular visits to the dentist. People who suffer from a hereditary predisposition to the occurrence of enamel dysplasia should visit the dentist's office at least once every six months. Do not forget about a balanced nutritious diet, and, in addition, about taking vitamin and mineral complexes. A he althy lifestyle along with quitting smoking and alcohol is always beneficial for dental he alth.

treatment of amelogenesis imperfecta
treatment of amelogenesis imperfecta

Parents should be especially careful. Pediatricians and pediatric dentists strongly recommend that adults monitor the he alth of the oral cavity of babies. Thus, it is very important to consult a specialist for any dental problem.

We looked at the causes, symptoms and diagnosis of amelogenesis imperfecta.

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