Acronym AI1H Synonyms Amelogenesis imperfecta, type 1H Keywords Disclaimer Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. [uniprot.org]

As with this patient, who presented with tricho-dento osseous syndrome, patients may present with associated expression of a syndrome (partial or full) and secondary changes in the periodontium. [aegisdentalnetwork.com]

Only two articles presented data with a longer follow-up [40]. [bmcoralhealth.biomedcentral.com]

Permanent crowns are often delayed by dentists until early adulthood, when all the teeth are present and stable. Orthodontic treatment People with amelogenesis often need orthodontic treatment, such as braces or appliances. [healthline.com]

Research on orthodontic treatment for patient with AI relies on case reports that present orthodontic treatment progress of AI patient. [ncbi.nlm.nih.gov]

• Dentist

  Your dentist will take X-rays both inside and outside your mouth, but usually the diagnosis can be made by visual examination. A radiographic exam can help your dentist see contrast between the enamel and dentin of your teeth. [healthline.com]

  The dentist may use a simple hand instrument to distinguish the different types of AI. By one to two years of age, the diagnosis can be made. [rarediseases.org]

  In this respect, establishing a good trust relationship between child and dentist is critical. Genetic and biological knowledge of AI physiopathology is also helpful in treatment plan decision. [bmcoralhealth.biomedcentral.com]

• Pathologist

  J Oral Pathol & medicine : official publi of the Int Ass of Oral Pathologists and the Am Acad Oral Pathol. 1992;21:390–4. 7. El-Sayed W, Shore RC, Parry DA, Inglehearn CF, Mighell AJ. [bmcoralhealth.biomedcentral.com]

• Eruptions

  However, rehabilitation in the mixed dentition is complex, since teeth have different eruption sequence, and definitive treatment cannot be rendered until complete eruption of the permanent dentition. [ncbi.nlm.nih.gov]

  Among 15 cases of delayed eruption, nine cases of pulp stones have been reported. [aegisdentalnetwork.com]

  In mixed dentition, rehabilitation must be done as soon as teeth erupt. Treatment main goals should be the preservation of tooth integrity and vitality [25]. [bmcoralhealth.biomedcentral.com]

  Diagnosis Diagnosis of AI is usually made by visual examination, family history and X-ray examination at the time teeth erupt. The dentist may use a simple hand instrument to distinguish the different types of AI. [rarediseases.org]

• Fracture

  In certain types of AI such as hypocalcified type, enamel fracture frequently results in defective margins of the restoration. [ncbi.nlm.nih.gov]

• Fear

  The relationship of AI status to fear of negative evaluation, lower mastery and self-esteem was age-dependent. Additionally, subjects with AI tended to show an increase in mastery and self-esteem scores with age [4]. [ncbi.nlm.nih.gov]

ORTHODONTIC TREATMENT Patients with AI often need orthodontic treatment due to dental and/or skeletal problems. [ncbi.nlm.nih.gov]

At the moment, there is no standard treatment for amelogenesis imperfecta. Treatment depends on the type and severity of the condition. [healthline.com]

In other cases, primary restoration could be done before orthodontic treatment and reassessed at the end of the treatment. [bmcoralhealth.biomedcentral.com]

This treatment adequately addressed the functional and esthetic aspects of the patient’s problems. [aegisdentalnetwork.com]

It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. Our staff consists of biologists and biochemists that are not trained to give medical advice. [uniprot.org]

With such evidence, clinicians may then select more favorable approaches to treat individual AI patient and to optimize their patient’s oral health and long-term prognosis. REFERENCES 1. Witkop CJ. [ncbi.nlm.nih.gov]

Along with poor oral hygiene, factors such as patient noncompliance affect the prognosis of prosthetic treatment.4 Esthetic crowns are sometimes used to compensate for soft enamel after thorough oral prophylaxis. [aegisdentalnetwork.com]

The molecular etiologies and associated phenotypes of amelogenesis imperfecta. Am J Med Genet A. 2006;140(23):2547-2555. 8. Borovjagin AV, Dong J, Passineau MJ, et al. Adenovirus gene transfer to amelogenesis imperfecta ameloblast-like cells. [aegisdentalnetwork.com]

[1] or traumatic etiologies as they will only affect defined teeth and rarely both dentitions. [bmcoralhealth.biomedcentral.com]

They stated that the etiology of open bite malocclusion in the different types of AI is heterogeneous. [ncbi.nlm.nih.gov]

An epidemiological, genetic and clinical study in a Swedish child population. Swed Dent J Suppl. 1986b;31:1–38. [PubMed] [Google Scholar] 9. Seow WK. Dental development in Amelogenesis imperfecta: a controlled study. Pediatr Dent. 1995;17(1):26–30. [ncbi.nlm.nih.gov]

Philippe Fournier & Ariane Berdal Université Pierre et Marie Curie-Paris, F-75006, Paris, France Benjamin Philippe Fournier, Ariane Berdal & Muriel de La Dure– Molla Centre de Recherche des Cordeliers, INSERM UMRS 1138, Laboratory of Molecular Oral Pathophysiology [bmcoralhealth.biomedcentral.com]

A mutation in any one of these genes can prevent it from making the correct protein, leading to enamel that is thin or soft. [healthline.com]

In hypomineralized forms, glass ionomer cements on occlusal surfaces were efficient in preventing pain and allowing temporizing until teeth eruption was achieved. [bmcoralhealth.biomedcentral.com]

Desensitizing toothpaste can prevent painful sensitivity to heat and cold. Good oral hygiene is important. Genetic counseling is recommending for families of children with AI. Investigational Therapies References TEXTBOOKS Hu JC-C, Simmer JP. [rarediseases.org]

Care should be taken to prevent soft enamel from chipping and to decrease the chances of gouging exposed dentin. • Subgingival curettage can be performed in deep pockets, taking care that soft cementum not be removed excessively. • Complete restoration [aegisdentalnetwork.com]

Early screening for these abnormalities should be done so that interceptive treatment can be rendered to prevent further damages to the developing affected dentition. [ncbi.nlm.nih.gov]