scholarly journals Dentinogenesis imperfecta type II

Author(s):  
Heloisa Aparecida Orsini Vieira ◽  
Aldevina Campos de Freitas ◽  
Regina Maura Fernandes ◽  
Daniele Lucca Longo ◽  
Raquel Assed Bezerra da Silva ◽  
...  

Dentinogenesis imperfecta (DI) is a hereditary developmental disorder of dentin formation that can occur associated with osteogenesis imperfecta (type I), isolated (type II), or in a specific isolated resident group of Brandywine, in southern Maryland (type III). This work aims at reporting a clinical case of DI type II in childhood with a 34-year follow up. The child at issue was taken to the dental health service at a very young age, which favored an appropriate treatment, avoiding complications, and portending a favorable long-term prognosis, besides safeguarding the patient physical and mental well-being. The clinical aspects of this condition are teeth with short crowns and gray-brown coloration, and an altered consistency of affected dental elements. Radiographically, the teeth present bulbous crowns, cervical constriction, thin roots, and early obliteration of the root canal and pulp chambers due to excessive dentin production. Rehabilitation treatment included the use of stainless-steel crowns for reconstructing deciduous molars and composite resin restorations on the anterior deciduous teeth. As for permanent dentition, it consisted of aesthetic-functional rehabilitation using metal crowns on the first molars and ceramic crowns and facets on the anterior teeth. Endodontic, prosthetic and restorative treatment was performed on other posterior teeth. Preventive measures were instituted. DI may cause serious changes in dentin structure, affecting function and aesthetics in both dentitions. The sooner it is administered, the more promising the multidisciplinary dental treatment will be in promoting health and minimizing damage to affected individuals.

2014 ◽  
Vol 40 (5) ◽  
pp. 593-600 ◽  
Author(s):  
Sompop Bencharit ◽  
Michael B. Border ◽  
C. Russell Mack ◽  
Warren C. Byrd ◽  
John T. Wright

Dentinogenesis imperfecta (DI) is a genetic disorder affecting the structural integrity of the dentin that can result in weakened dentin. The affected teeth, especially posterior teeth, often need to be extracted due to severe wear or fracture. This frequently yields a loss of posterior occlusion and occlusal vertical dimension. Besides wear and fracture, anterior teeth often have an unesthetic appearance because of discoloration. Current treatments of choice, including composite bonding restorations and, more recently, all-ceramic restorations, are typically suggested to preserve the remaining teeth and tooth structure. However, there are a limited number of studies on dental implants in patients with DI. The effectiveness of dentin bonding and dental implants in patients with DI is not known. This clinical report describes a 32-year-old Asian woman with DI who underwent full-mouth rehabilitation. The posterior occlusion, mostly in the molar areas, was restored with dental implants and ceramometal restorations. The anterior teeth and premolars were restored with bonded lithium disilicate glass-ceramic pressed veneers and crowns made with computer-aided design/computer-aided manufacturing. This case demonstrates that restoring functional occlusion and esthetics for a patient with DI can be completed successfully using contemporary implant therapy and adhesive dentistry.


2016 ◽  
Vol 96 (3) ◽  
pp. 308-314 ◽  
Author(s):  
M.S. Segarra ◽  
Y. Shimada ◽  
A. Sadr ◽  
Y. Sumi ◽  
J. Tagami

The aim of this study was to nondestructively analyze enamel crack behavior on different areas of teeth using 3D swept source-optical coherence tomography (SS-OCT). Ten freshly extracted human teeth of each type on each arch ( n = 80 teeth) were inspected for enamel crack patterns on functional, contact and nonfunctional, or noncontact areas using 3D SS-OCT. The predominant crack pattern for each location on each specimen was noted and analyzed. The OCT observations were validated by direct observations of sectioned specimens under confocal laser scanning microscopy (CLSM). Cracks appeared as bright lines with SS-OCT, with 3 crack patterns identified: Type I – superficial horizontal cracks; Type II – vertically (occluso-gingival) oriented cracks; and Type III – hybrid or complicated cracks, a combination of a Type I and Type III cracks, which may or may not be confluent with each other. Type II cracks were predominant on noncontacting surfaces of incisors and canines and nonfunctional cusps of posterior teeth. Type I and III cracks were predominant on the contacting surfaces of incisors, cusps of canines, and functional cusps of posterior teeth. Cracks originating from the dental-enamel junction and enamel tufts, crack deflections, and the initiation of new cracks within the enamel (internal cracks) were observed as bright areas. CLSM observations corroborated the SS-OCT findings. We found that crack pattern, tooth type, and the location of the crack on the tooth exhibited a strong correlation. We show that the use of 3D SS-OCT permits for the nondestructive 3D imaging and analysis of enamel crack behavior in whole human teeth in vitro. 3D SS-OCT possesses potential for use in clinical studies for the analysis of enamel crack behavior.


2019 ◽  
Vol 43 (3) ◽  
pp. 147-154 ◽  
Author(s):  
Arturo Garrocho-Rangel ◽  
Irma Dávila-Zapata ◽  
Ricardo Martínez-Rider ◽  
Socorro Ruiz-Rodríguez ◽  
Amaury Pozos-Guillén

Dentinogenesis Imperfecta type II (DI2), also known as hereditary opalescent dentin, is one of the most common genetic disorders affecting the structure of dentin, not related with osteogenesis imperfecta, which involves both primary and permanent dentitions. The purpose of this article is to perform a scoping review of the published peer-reviewed literature (1986–2017) on DI2 management in children and to outline the most relevant clinical findings extracted from this review. Forty four articles were included in the present scoping review. According to the extracted data, the following are the most important tasks to be performed in clinical pediatric dentistry: to re-establish the oral mastication, esthetics, and speech, and the development of vertical growth of alveolar bone and facial muscles; to reduce the tendency to develop caries, periapical lesions and pain; to preserve vitality, form, and size of the dentition; to avoid interfering with the eruption process of permanent teeth; to decrease the risk of tooth fractures and occlusion disturbances; to return the facial profile to a more normal appearance; and to prevent or treat possible temporomandibular joint problems. Therefore, Pediatric Dentists should bear in mind that early diagnosis and treatment, together a long-term follow-up of DI2 in children, continue to be the best approaches for achieving enhanced patient psychological well-being and, in consequence, their quality of life.


Author(s):  
Murad Alrashdi ◽  
Jason Schoener ◽  
Claudia Isabel Contreras ◽  
Shuo Chen

Background: Dentinogenesis imperfecta (DGI) is a complex anomaly, not only by its structure but by treatment approach. The treatment protocol depends on the severity, behavior, and the age of the patient. Case Description: This paper presents two siblings’ cases of DGI type II (DGI-II) with different treatment based on the patient’s clinical severity, behavior, and age (mixed versus primary dentition). The first case involves a patient in the primary dentition with severe attrition leading to a reduction in the vertical dimension of occlusion (VDO) treated by the fabrication of complete overlay dentures. The second case involves a patient in the early mixed dentition treated with restorations and extractions. Conclusion: Full mouth rehabilitation in the two patients dramatically improves function, aesthetics, and proved to be a significant psychological boost to the patient’s well-being. Practical Implications: Early diagnosis and a multidisciplinary approach for patients with DGI to preserve the remaining teeth and rehabilitation for their function and aesthetics are essential for obtaining a favorable prognosis.


2005 ◽  
Vol 153 (3) ◽  
pp. 353-358 ◽  
Author(s):  
K Raile ◽  
H Stobbe ◽  
R B Tröbs ◽  
W Kiess ◽  
R Pfäffle

Blepharophimosis/ptosis/epicanthus inversus syndrome (BPES), an autosomal dominant syndrome in which eyelid malformation is associated with (type I BPES) or without premature ovarian failure (type II BPES). Mutations of a putative winged helix/forkhead transcription factor FOXL2 account for both types of BPES. We report on a 16-year-old adolescent girl with blepharophimosis and ptosis. Subsequently she developed oligomenorrhea, secondary amenorrhea for 6 months, and an extremely large cyst of one ovary. The cyst contained 8 l of cyst fluid and histopathology displayed a large corpus luteum cyst. Following laparotomy, gonadotropin levels were elevated (LH 17.2 U/l, FSH 29.4 U/l) and estradiol levels decreased (67 pmol/l). Because of clinical aspects of BPES and abnormal ovarian function we suspected a mutation of her FOXL2 gene and found a new in-frame mutation (904_939dup36) on one allele, leading to a 12 alanine expansion within the polyalanine domain. We conclude that the FOXL2 mutation 904_939dup36 may account not only for blepharophimosis and ptosis but also for ovarian dysfunction and growth of the large corpus luteum cyst. In contrast to known FOXL2 mutations with polyalanine expansions and association with BPES type II, clinical aspects of our girl may indicate some degree of ovarian dysfunction that might finally lead to BPES type I with premature ovarian failure.


2018 ◽  
Author(s):  
Ian Towle ◽  
Joel D. Irish

AbstractWe report the frequencies of linear enamel hypoplasia (LEH) and, specifically, pitting enamel hypoplasia (PEH) defects in the teeth of Paranthropus robustus, for comparison with four other South African hominin species and three extant nonhuman primate species. Unlike LEH, the lesser known PEH is characterized by multiple circular depression defects across a tooth crown and is often difficult to interpret in terms of developmental timing and etiology. Teeth in all samples were examined macroscopically with type, position and number of defects recorded. Frequencies of teeth with LEH vary among hominin species, but the differences in PEH are considerable. That is, P. robustus has much higher rates of pitting defects, with 47% of deciduous teeth and 14% of permanent teeth affected, relative to 6.7% and 4.3%, respectively, for all other hominin teeth combined; none of the extant primate samples evidence comparable rates. The defects on P. robustus molars are unlike those in other species, with entire crowns often covered in small circular depressions. The PEH is most consistent with modern human examples of amelogenesis imperfecta. Additionally, the defects are: 1) not found on anterior teeth, 2) uniform in shape and size, and 3) similar in appearance/severity on all molars. A possible reason for this form of PEH is as a side effect of selection on another phenotype that shares the same coding gene(s), i.e., a genetic origin. Recent research on the ENAM gene provides one such possibility. Paranthropus likely underwent rapid evolution in the ENAM loci, with changes in this gene contributing to larger posterior teeth and thicker enamel. This same gene is associated with amelogenesis imperfecta; therefore, pleiotropy effects, relating to high selection on this gene during Paranthropus evolution, could have yielded this unique condition.


2013 ◽  
Vol 2013 ◽  
pp. 1-10 ◽  
Author(s):  
P. Nambiar ◽  
J. John ◽  
Samah M. Al-Amery ◽  
K. Purmal ◽  
W. L. Chai ◽  
...  

Orangutans are believed to have close biological affinities to humans. Teeth being the hardest tissue provide useful information on primate evolution. Furthermore, knowledge of the pulp chamber and root canal morphology is important for dental treatment. A female Bornean orangutan and a Sumatran male orangutan skull were available for this study. Both of their dentitions, comprising 50 teeth, were scanned employing the cone-beam computed tomography for both metrical and nonmetrical analyses. Measurements included tooth and crown length, root length, enamel covered crown height, root canal length (posterior teeth), length of pulpal space (anterior teeth), and root canal width. Nonmetrical parameters included number of canals per root, number of foramina in each root, and root canal morphology according to Vertucci’s classification. It was found that the enamel covered crown height was the longest in the upper central incisors although the canine was the longest amongst the anterior teeth. Both the upper premolars were three-rooted while the lower second premolar of the Sumatran orangutan was two-rooted, with two foramina. The mandibular lateral incisors of the Bornean orangutan were longer than the central incisors, a feature similar to humans. In addition, secondary dentine deposition was noticed, a feature consistent with aged humans.


2015 ◽  
Vol 41 (03) ◽  
pp. 197-203
Author(s):  
Tzong-Fu Kuo ◽  
Shi-Yuan Sheu ◽  
Ching-Chuan Jiang ◽  
Hao-Hueng Chang ◽  
Shuo-Tsung Chen ◽  
...  

To develop a cell-based approach to tooth regeneration, we isolated dental stem cells from tooth buds of mini pigs and culture-expanded them for about three weeks. Flow cytometry revealed that the majority of the proliferating cells were positive for stem cell surface markers, including CD105, CD29, CD44, CD71. These dental stem cells were then seeded in biodegradable agarose gel for autograft in various areas of the jaw bone. Mini pigs with tooth buds removed without transplantation of dental stem cells served as control. After one year, well differentiated tooth crown and root sheath were formed in mini pigs in the experimental group. The tooth formed by dental stem cells transplanted in alveolar bone was similar in size to original tooth crown (approximately 2 cm × 2 cm × 2 cm). The teeth formed by dental stem cells transplanted under the roots of the anterior teeth and in the cortical bone area were smaller. In the control group, the space where the tooth bud was extracted without cell transplantation was seen to be closed by forward movement of the posterior teeth. Histological analysis showed the structures of enamel, dentin and pulp in the regenerated tooth crown. Immunohistochemical analysis of amelogenin, type I collagen and bone sialoprotein confirmed enamel and dentin formation. Enamel and dentin structures on the regenerated tooth were also identified using scanning electron microscopy. These findings demonstrate a successful model of tooth regeneration from dental stem cells in mini pigs. In addition, local environment and available space also significantly affect the extent of tissue regeneration. Our study demonstrates the feasibility of using dental stem cells for tooth regeneration in clinical applications.


2014 ◽  
Vol 2014 ◽  
pp. 1-5
Author(s):  
Ricardo Coelho Okida ◽  
Daniela Micheline dos Santos ◽  
Aljomar José Vechiato Filho ◽  
Agda Marobo Andreotti ◽  
Rodrigo Antonio de Medeiros ◽  
...  

The gastroesophageal reflux disease (GERD) is one of the main causes of dental erosion. The aim of this case presented is to describe the prosthetic rehabilitation of a patient with GERD after 4 years of followup. A 33-year-old male patient complained about tooth sensitivity. The lingual surface of the maxillary anterior teeth and the cusps of the upper and lower posterior teeth presented wear. It was suspected that the feeling of heartburn reported by the patient associated with the intake of sports supplements (isotonics) was causing gastroesophageal changes. The patient was referred to a gastroenterologist and was diagnosed with GERD. Dental treatment was performed with metal-free crowns and porcelain veneers after medical treatment of the disease. With the change in eating habits, the treatment of GERD and lithium disilicate ceramics provided excellent cosmetic results after 4 years and the patient reported satisfaction with the treatment.


2017 ◽  
Vol 15 (2) ◽  
Author(s):  
Patricia Trejos ◽  
Vanessa Hernando ◽  
Claudia De León

Summary: Introduction: Dentinogenesis imperfecta (DI) is an inherited dental diaseasein which dental crowns are affected. DI originates by defects on the histo-differenciation stage ofodontogenesis, constituting a localized mesodermal dysplasia characterized by an expressedalteration of dentin proteins. There are three types of dentinogenesis imperfecta: Type I(associated with osteogenesis imperfecta OI), type II (no associated with OI), and type III(Brandywine’s). Objectives: To determine the type of dentinogenesis imprefecta, the family historyand clinical characteristics of a patient within extensive coronal destruction, to establish aprecise diagnosis and a treatment to recover masticatory function. Case Report: A 3-year-old malechild was referred to the clinic with dental hypersensitivity and pain during mastication. Hepresented generalized and extensive crown destructions and discoloration of erupted teeth,abscesses, attrition and loss of vertical dimension. Radiographs showed obliterated pulp chambers,marked cervical constriction and short roots. After clinical and radiographic analysis, familyhistory and consultation with a Geneticist, a relation with OI was not established. In contrast, adiagnosis of dentinogenesis imperfecta type II was confirmed. DE type II is an autosomyc dominanttrait, without gender predilection. Restorative treatment involved stainless steel crowns, requiredextractions, space maintainers and removable partial dentures placement. Conclusion: Early diagnosisand treatment of DI is recommended because the severe destruction of primary dentition that mayaffect child chewing and nutrition. Consultation wit a Geneticist in DI cases is reccomended todiscard any association with OI or other genetic syndromes. Key words: Dentinogenesis imperfecta.Odontogenesis imperfecta. Opalescent dentin. Hereditary opalescent dentin.


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