Dentinogenesis imperfecta: an early treatment strategy.

Department of Pediatric Dentistry, the Hebrew University-Hadassah Faculty of Dental Medicine, Jerusalem, Israel.
Pediatric dentistry (Impact Factor: 0.56). 01/2001; 23(3):232-7.
Source: PubMed

ABSTRACT Dentinogenesis imperfecta (DI) type 2 is a disease inherited in a simple autosomal dominant mode. As soon as the teeth erupt the parents may notice the problem and look for a pediatric dentist's advice and treatment. Early diagnosis and treatment of DI is recommended, as it may prevent or intercept deterioration of the teeth and occlusion and improve esthetics. The purpose of this article is to present the objectives, treatment options, and problems encountered in the treatment of DI in the early primary dentition. A two-stage treatment of a toddler under general anesthesia is described and discussed. This paper recommends for severe cases of DI two treatment stages performed under general anesthesia. Stage 1 is early (around age 18-20 months) and is directed to covering the incisors with composite restorations and the first primary molars with preformed crowns. Stage 2 (around age 28-30 months) seeks to protect the second primary molars with preformed crowns and cover the canines with composite restorations.

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    ABSTRACT: INTRODUCCIÓN Alguna mutación o alteración accidental del geno-ma humano, da como resultado ciertas condiciones o síndromes hereditarios, que pueden ser identificados durante la práctica odontológica; dentro de las enfer-medades que involucran la estructura dental se en-cuentran aquellas que afectan al esmalte y las que afectan a la dentina, con patrones hereditarios autosó-mico-dominante, recesivo o ligado al cromosoma X. El propósito de este estudio es describir el patrón hereditario de dentinogénesis imperfecta en el pacien-te, así como los signos y síntomas que se presentan para llegar a un diagnóstico correcto y a la elaboración de un plan de tratamiento adecuado. ABSTRACT Introduction: Dentinogenesis imperfect (DI) is an inherited condition originated in the histodifferentiation stage during odon-togenesis, constituting a localized mesodermal dysplasia form, characterized by an expressed alteration of dentin proteins. There are three types of dentinogenesis imperfect: Type I (asso-ciated with osteogenesis imperfect OI), type II (not associated with OI), and type III (Brandywine's). Objectives: To determine the type of dentinogenesis, family history trend and clinical char-acteristics of the patient, to establish early diagnosis and heredi-tary pattern. Case report: A 3-year-old boy patient was referred to the clinic with dental hypersensitivity and pain during mastica-tion. He presented a structure anomaly and discoloration of erupted teeth, abscesses, attrition and loss of vertical dimen-sion. Radiographs showed obliterated pulp chambers, marked cervical constriction and short roots. After clinical and radio-graphic analysis, family history and consultation with Genetics, there was no relation with OI. A diagnosis of dentinogenesis im-perfect type II was made, transmitted as an autosomal dominant trait, without gender predilection. Restorative treatment involved stainless steel crowns, extractions, space maintainer and re-movable partial denture placement. Conclusion: Early diagno-sis and treatment of DI is recommended because of the severe alterations of primary dentition. In this kind of patients it is very important to carry out consultations with Genetics to discard any association with other disorders.
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    ABSTRACT: Dentinogenesis imperfecta is an autosomal dominant disease characterized by severe hypomineralization of dentin and altered dentin structure. Dentin extra cellular matrix is composed of 90% of collagen type I and 10% of non-collagenous proteins among which dentin sialoprotein (DSP), dentin glycoprotein (DGP) and dentin phosphoprotein (DPP) are crucial in dentinogenesis. These proteins are encoded by a single gene: dentin sialophosphoprotein (DSPP) and undergo several post-translational modifications such as glycosylation and phosphorylation to contribute and to control mineralization. Human mutations of this DSPP gene are responsible for three isolated dentinal diseases classified by Shield in 1973: type II and III dentinogenesis imperfecta and type II dentin dysplasia. Shield classification was based on clinical phenotypes observed in patient. Genetics results show now that these three diseases are a severity variation of the same pathology. So this review aims to revise and to propose a new classification of the isolated forms of DI to simplify diagnosis for practitioners.European Journal of Human Genetics advance online publication, 13 August 2014; doi:10.1038/ejhg.2014.159.
    European journal of human genetics: EJHG 08/2014; 23(4). DOI:10.1038/ejhg.2014.159 · 3.56 Impact Factor
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    ABSTRACT: Dentinogenesis imperfecta (DI) is an inherited disorder affecting dentin. Defective dentin formation results in discolored teeth that are prone to attrition and fracture. Mutation in dentin sialophosphoprotein (DSPP) has been found to cause the dentin disorders DI - I and II (shields II and III). Early diagnosis and treatment of DI is recommended as it may prevent or intercept deterioration of the teeth and occlusion and improve esthetics. Here, we report a case with characteristic clinical, radiological and histological features of DI-I. The etiology and classification followed in literature is confusing since dentinoenamel junction (DEJ) in DI seems to be structurally and functionally normal and DI is clearly a disorder distinct from osteogenesis imperfecta (OI), but we still relate etiology of DI to DEJ and follow Shields classification. Therefore, we have briefly reviewed etiology and nomenclature system of DI.
    Journal of Oral and Maxillofacial Pathology 09/2014; 18(Suppl 1):S131-4. DOI:10.4103/0973-029X.141363


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