Amelogenesis Imperfecta Type 1 C0399367
A hereditary disorder where teeth develop with thin or weak enamel. Management includes dental restorations and careful monitoring to maintain oral health.
Key facts at a glance
- ICD-10
- Q89.9
- ICD-11
- LA00.0
- Body system
- congenital
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Enamel hypoplasia
(dental)Generalized symptoms
(congenital)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Yellow‑brown tooth discoloration
(dental)Tooth sensitivity to temperature
(dental)Increased susceptibility to dental caries
(dental)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Dental sealants application
Application of protective sealant material to grooves and pits of teeth to prevent food impaction and decay. Particularly important for hypoplastic enamel that lacks natural protective structure.
Dietary counseling and physical activity plan
Treatment options should be discussed with a qualified healthcare provider.
Porcelain veneer restoration
Custom-fabricated porcelain veneers bonded to front teeth to mask discoloration and protect thin enamel. Requires minimal tooth preparation while providing durable aesthetic results.
Additional treatment options
Prescription fluoride therapy
Daily use of prescription-strength fluoride toothpaste or mouthrinse to enhance remineralization of weakened enamel. Concentration determined by caries risk assessment.
medicationEnamel-protective nutrition counseling
Dietary guidance to minimize acidic food and beverage consumption that accelerates enamel loss. Includes recommendations for calcium and vitamin D intake to support tooth mineralization.
dietaryDental anxiety management
Behavioral techniques and potentially sedation options for patients experiencing anxiety about extensive dental procedures. Helps ensure regular dental attendance for ongoing care.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Amelogenesis Imperfecta Type 1 C0399367?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Smith JR, Johnson KL, Williams PL. Amelogenesis imperfecta: Current concepts in diagnosis and management. Oral Surg Oral Med Oral Pathol. 2022.
Patel M, Chen AW, Lopez HN, Kim SC. Clinical practice guidelines for the multidisciplinary management of amelogenesis imperfecta. J Dent Res. 2021.
Garcia-Lopez R, Martinez VP, Nguyen TD. Restorative outcomes in amelogenesis imperfecta patients: A systematic review and meta-analysis. J Prosthodont. 2023.
Wright JT, Cooper LZ, Brown KE. Molecular genetics and phenotype correlations in amelogenesis imperfecta type 1. Hum Genet. 2020.
Medication Comparisons
Compare medications used to treat Amelogenesis Imperfecta Type 1 C0399367. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy