General information it is a rare disturbance of dentine formation, characterised by normal but a typical dentine formation, with abnormal pulp morphology it represents an autosomal dominant trait some systemic diseases are also manifested as dentin dysplasia features. Molecular genetics studies allow to discriminate only two pathologies. To date, the pathogenic genes of dgi type i, which is considered a clinical manifesta tion of syndrome osteogenesis imperfecta, include col1a1 and col1a2. This unusual case of generalized short roots presents a case demonstrating both classic and atypical features of dentinal dysplasia type i ddi in the mixed and permanent dentitions.
Dentin dysplasia type inovel findings in deciduous and. Typical radiographic findings of dentin dysplasia type 1b with dental fluorosis typical radiographic findings of dentin dysplasia type 1b with dental fluorosis. Due to the rare occurrence with the prevalence rate of 1. Introduction dentin dysplasia is a rare hereditary disturbance of dentin formation characterized by defective dentin development with clinically normal appearing crowns, severe hypermobility of teeth and spontaneous dental abscesses or cysts. Dentin dysplasia type i is a genetic defect of dentin formation, which is inherited as an autosomal dominant trait. Radiographic analysis shows obliteration of all pulp chambers, short, blunted, and malformed roots, and periapical radiolucencies. Apr 06, 2020 representing the largest portion of the tooth, the dentin is a hard material found beneath the enamel, surrounding the pulp center of the tooth.
What are the risk factors for dentin dysplasia type i. Both primary and permanent dentitions can be affected by either type i or type ii dentin dysplasia. Dec 22, 2015 dentin dysplasia type i ddi is a rare autosomal dominant hereditary disorder which seriously affects the root development of teeth, causing spontaneous tooth loss in teenagers. It occurs more frequently than dentin dysplasia type i, which is estimated to affect 1 in 100,000 people in the general population. The same gene is implicated in type iii dentinogenesis imperfecta and in type ii dentin dysplasia ddii. The mutation in collagen type 1 col1 a1, col1 a2 causes di 1. It affects both the primary and permanent dentition teeth development no racial, ethnic, or gender predominance is observed. Dentin dysplasia type i nord national organization for. In dd1a, there is complete obliteration of pulp chambers and no root development, with many periapical radiolucent areas. Pdf type1 dentine dysplasia is a rare hereditary condition, associated with an abnormality in dentine formation. Radiographic analysis shows obliteration of all pulp chambers, short, blunted, and malformed. The topic thistle tube teeth dentin dysplasia type i you are seeking is a synonym, or alternative name, or is closely related to the medical condition dentin dysplasia type i.
Dentin dysplasia is a rare disturbance of dentin formation characterized by normal enamel but atypical dentin formation with abnormal pulpal. Dentin dysplasia is a defect of dentin development that is inherited as an autosomal dominant trait and classified into two types 1,2. Case report dentin dysplasia type i aarti singh, 1 sangesh gupta,2 monal bhaurao yuwanati,3 shubhangi mhaske3 1department of oral medicine and radiology, maulana azad institute of dental sciences. Isolated dentinogenesis imperfecta and dentin dysplasia. Pdf dentin dysplasia type i swapnil mhaske academia. However, deciduous teeth affected by type ii dentin dysplasia have a characteristic blueamber discolouration, whilst the other dentition appears normal. Dentinogenesis imperfecta shields type ii dgiii is an autosomal dominant hereditary disease caused by mutations in the dspp gene dentin sialophosphoprotein coding for dentin sialoprotein and dentin phosphoprotein. Dentin dysplasia type 1 clinical management dilemmas. Nov 26, 20 dentin dysplasia dd type i is an inherited autosomal dominant genetic defect affecting the dentin formation. Aug 22, 2007 dentin dysplasia is a defect of dentin development that is inherited as an autosomal dominant trait and classified into two types 1,2. Dentin dysplasia is a rare inherited autosomal dominant disorder characterized by rootless teeth. Dentin dysplasia type i is an inherited disorder characterized by atypical development of the dentin of a persons teeth. In this case, the changes observed are not characteristic of any dd i subtype described 6,9,10. Epidemiology prevalence of ddi is reported to be 1 100,000.
The pulp is a soft tissue that is well supplied with blood vessels and nerves. In 1972, witkop classified it into type i and type ii which affect both dentitions. Epidemiology prevalence of ddi is reported to be 1100,000. Dentin dysplasia dd is a rare genetic developmental disorder dentine production of the teeth, commonly exhibiting an autosomal dominant inheritance that causes malformation of the root. Which of the following isare associated with dentin dysplasia type i. Dd type i radicular dentin dysplasia characterized by. Pdf on jan 1, 2016, sonika achalli and others published dentin dysplasia type i. The paper highlights anatomical and radiological aspects of dental abnormalities and emphasizes the significance of the education of both general practitioners and paediatricians as regards referring patients with diagnosed dentin dysplasia for a multispecialty therapy. Dentin dysplasia is a rare autosomal dominant disorder affecting dentin and resulting in early loss of teeth. A rare case of hereditary disturbance of dentine, dentin dysplasia type i is presented, which is characterized by short or total absence of roots, obliterated pulp. A rare case find, read and cite all the research you need on researchgate. In this anomaly, the teeth generally appear unremarkable with normal crowns. Dentin dysplasia type inovel findings in deciduous and permanent. International journal of dental sciences and research, vol.
A radiolucent area in a radiograph occurs as a result of 1. Pdf dentin dysplasia type i is a rare hereditary disturbance of dentin formation characterised clinically by nearly normal appearing crowns and. Hereditary dentin disorders include dentinogenesis imperfecta dgi and dentin. Dentin dysplasia type i should be differentiated from dentin dysplasia type ii, dentinogenesis imperfecta and odontodysplasia. Jul 08, 2014 educational video made by unmc college of dentistry class of 2016 dental students. Therapy includes extraction of all teeth, ectomisation of cystic alteration, revision of paranasal sinus. Dentin dysplasia type i ddi is a rare autosomal dominant hereditary disorder which seriously affects. Click on the link to view a sample search on this topic. Sep 01, 2012 dentin dysplasia type i ddi is a rare form of dentin dysplasia dd, see this term characterized by sharp conical short roots or rootless teeth. Pdf type1 dentine dysplasia diagnostic and clinical. Dentin dysplasia type i ddi is a rare form of dentin dysplasia dd, see this term characterized by sharp conical short roots or rootless teeth. Age and sex distribution dentin dysplasia type i is an inherited disorder that is present at birth. In our patient, the calcified pulp chambers, rootless teeth, periapical radiolucent areas and the nature of the periapical lesion are characteristic findings for the diagnosis of dd type 1, sub type 1a. Dentin dysplasia type ii, also known as coronal dentin dysplasia, is a rare.
Research article open access dentin dysplasia type inovel findings in deciduous and permanent teeth xin ye1, kunyang li2, ling liu1, fangfang yu1, fu xiong3, yun fan1, xiangmin xu3, chunran zuo2 and dong chen1 abstract background. Deceptively, teeth have the clinical appearance of normality, however, radiographically, a different picture is seen. Dentin dysplasia type i is characterized by the presence of primary and permanent teeth with normal appearance of the crown but no or only rudimentary root development, incomplete or total obliteration of the pulp. Educational video made by unmc college of dentistry class of 2016 dental students. Pubmed is a searchable database of medical literature and lists journal articles that discuss dentin dysplasia, coronal. Type1 dentine dysplasia is a rare hereditary condition, associated with an abnormality in dentine formation. We hereby report a case of dentin dysplasia type 1b with typical radiographic findings. At present, the study of ddi focuses on familial and phenotypic analyses and reports regarding the ultrastructural study of ddi are few. This paper describes a rare case of genetically determined dentin dysplasia type i in 26yearold male patient. A rare case of hereditary disturbance of dentine, dentin dysplasia type i is presented, which is characterized by short or total absence of roots, obliterated pulp chambers, and periapical radiolucencies.
Dentin dysplasia type i ddi is a rare autosomal dominant hereditary disorder which seriously affects the root development of teeth, causing spontaneous tooth loss in teenagers. Dentin dysplasia is a rare abnormality of dentin that involves both primary and permanent. A third type of dentin dysplasia or focal odontoblastic dysplasia, with radiographic aspects of both types of dysplasia, has also been described 10. Dentin dysplasia type i ddi is a rare autosomal dominant hereditary disorder which seriously affects the root development of teeth, causing. Dd1 is characterized clinically by nearly normal appearing crowns and hypermobility of the teeth 11,12. Here, the teeth are characterized by normal appearing crowns, with absence or severe restriction of root formation, obliterated pulp chambers and periapical radiolucencies without an obvious cause. Dentin dysplasia type i is a rare hereditary disturbance of dentin formation characterised clinically by nearly normal appearing crowns and hypermobility of teeth that affects one in every 100 000. Dentin dysplasia type i is a rare hereditary disturbance of dentin formation characterised clinically by nearly normal appearing crowns and hypermobility of teeth that affects one in every 100 000 individuals and manifests in both primary and permanent dentitions. It is characterized by presence of normal enamel but atypical dentin with abnormal pulpal morphology.
Dentin dysplasia dd type i is an inherited autosomal dominant genetic defect affecting the dentin formation. Representing the largest portion of the tooth, the dentin is a hard material found beneath the enamel, surrounding the pulp center of the tooth. A case report on dentin dysplasia type i, a congenital disease autosomal dominant gene defect affecting deciduous and permanent teeth, is depicted including representations of clinical and histological features, xray and ctfindings. Dentin dysplasia is a rare, genetic disorder of the teeth that is characterized by. Dentin dysplasia is a rare developmental disorder affecting the dentin formation. It appears to be normal clinically, but, radiographi cally, it is characterized by obliteration of all pulp chambers and short, blunted, and malformed roots. Dentin dysplasia type i a case report sciencedirect. To date, the pathogenic genes of dgi type i, which is considered a clinical manifestation of syndrome osteogenesis imperfecta, include col1a1 and col1a2. Dentin dysplasia dd is a rare hereditary disturbance is inherited as an autosomal dominant trait. Case report dentin dysplasia type i aarti singh,1 sangesh gupta,2 monal bhaurao yuwanati,3 shubhangi mhaske3 1department of oral medicine and radiology, maulana azad institute of dental sciences, new delhi, india 2department of oral medicine, diagnosis and radiology. Dentin dysplasia type ii nord national organization for rare.
Jun 20, 2018 dentin dysplasia dd is a rare hereditary disturbance is inherited as an autosomal dominant trait. Management of dentin dysplasia and facial disharmony. This condition is a type of dentin dysplasia that causes teeth to be discolored most often a bluegray or yellowbrown color and translucent giving teeth an opalescent sheen. Although dentin dysplasia type i is reported to have an incidence of 1 in 100,000, dentin dysplasia type ii is considered to be an extremely rare genetic disorder, the prevalence of which is not clearly reported in the literature so far. Eastman and othersb also described several patients with a focal dentinal dysplasia type 3 in which only isolated teeth were affected. Histological analysis shows whorls of tubular dentin and atypical osteodentin. Dentin dysplasia is a rare defect of dentin development with an autosomal dominant pattern of inheritance 1,2 that affects one in everyindividuals 3,4 and manifests in both primary 2, and permanent 2,5, dentitions. Dentin dysplasia type ii ddii is a rare mild form of dentin dysplasia dd, see this term characterized by normal tooth roots but abnormal primary dentition. It affects both primary and permanent dentitions in approximately 1 in every 100,000 patients.
Typical radiographic findings of dentin dysplasia type 1b. Dentin dysplasia, coronal genetic and rare diseases. Mutations of the dspp gene, which encodes the dentin sialophosphoprotein, a major non. Mar 25, 2018 to date, the pathogenic genes of dgi type i, which is considered a clinical manifestation of syndrome osteogenesis imperfecta, include col1a1 and col1a2. It was ballschmiede in 1920 who first reported such a condition as rootless teeth and in 1939 rushton termed this condition as dd. Hereditary dentin defects are divided into two main categories, namely dentinogenesis imperfecta and dentin dysplasia dd 1, 2. A case report with a 6year followup dentinal dysplasia type i. Dentin dysplasia, type 1 genetic and rare diseases.
Dentin dysplasia is a defect of dentin development that is inherited as an autosomal dominant trait and classified into two types 1, 2. Bmc oral health dentin dysplasia type inovel findings in deciduous and permanent teeth xin ye 0 kunyang li 2 ling liu 0 fangfang yu 0 fu xiong 1 yun fan 0 xiangmin xu 1 chunran zuo 2 dong chen 0 0 department of periodontics, school of stomatology, zhengzhou university, zhengzhou, henan, china 1 department of medical genetics, southern medical university. Bmc oral health dentin dysplasia type inovel findings in deciduous and permanent teeth xin ye 0 kunyang li 2 ling liu 0 fangfang yu 0 fu xiong 1 yun fan 0 xiangmin xu 1 chunran zuo 2 dong chen 0 0 department of periodontics, school of stomatology, zhengzhou university, zhengzhou, henan, china 1 department of medical genetics, southern medical university, guangzhou, china 2. Sep 01, 2012 dentin dysplasia type ii ddii is a rare mild form of dentin dysplasia dd, see this term characterized by normal tooth roots but abnormal primary dentition. Dentin dysplasia type i is characterized by the presence of primary and permanent teeth with normal appearance of the crown but no or only rudimentary root development, incomplete or total obliteration of the. Witkop2 later described type i as radicular dentin dysplasia and type ii as coronal dentin dysplasia to indicate the parts of the teeth that are primarily involved. Dentin makes up most of the tooth and is the bonelike material under the enamel. Dentin dysplasia type ii nord national organization for. In this article, we report two cases of type i dentin dysplasia dd. Dentin dysplasia type ia dental disease with genetic. Which of the following isare associated with dentin. Dentin dysplasia dd, a rare anomaly is an autosomal dominant hereditary disturbance in dentin formation affecting either the primary or both the dentitions in approximately one patient in every 100,000. Dd is a hereditary disorder in the formation of dentin that comprises a group of autosomal dominant genetic 3, 4 conditions that are characterized by abnormal dentin structure affecting both the decidu. Dentin dysplasia type ii affects males and females in equal numbers.