An apparently hereditary disorder of dentin formation, marked by a normal appearance of coronal dentin associated with pulpal obliteration, faulty root formation, and a tendency for peripheral lesions without obvious cause. (From Dorland, 27th ed)
An autosomal dominant disorder of tooth development characterized by opalescent dentin resulting in discoloration of the teeth. The dentin develops poorly with low mineral content while the pulp canal is obliterated.
The hard portion of the tooth surrounding the pulp, covered by enamel on the crown and cementum on the root, which is harder and denser than bone but softer than enamel, and is thus readily abraded when left unprotected. (From Jablonski, Dictionary of Dentistry, 1992)
"Space maintenance in dentistry refers to the use of an appliance (such as a band or a crown) to maintain the space created by a missing primary tooth, preventing the drifting of adjacent teeth and allowing the correct eruption path for the permanent successor."
Horizontal and, to a lesser degree, axial movement of a tooth in response to normal forces, as in occlusion. It refers also to the movability of a tooth resulting from loss of all or a portion of its attachment and supportive apparatus, as seen in periodontitis, occlusal trauma, and periodontosis. (From Jablonski, Dictionary of Dentistry, 1992, p507 & Boucher's Clinical Dental Terminology, 4th ed, p313)
'Dental pulp calcification' is a pathological condition characterized by the deposition of hard tissue within the pulp chamber and root canal(s), which can result in the obliteration of pulpal space, potentially leading to various clinical symptoms such as pain or dental sensitivity.
Glycoproteins which contain sialic acid as one of their carbohydrates. They are often found on or in the cell or tissue membranes and participate in a variety of biological activities.
Macromolecular organic compounds that contain carbon, hydrogen, oxygen, nitrogen, and usually, sulfur. These macromolecules (proteins) form an intricate meshwork in which cells are embedded to construct tissues. Variations in the relative types of macromolecules and their organization determine the type of extracellular matrix, each adapted to the functional requirements of the tissue. The two main classes of macromolecules that form the extracellular matrix are: glycosaminoglycans, usually linked to proteins (proteoglycans), and fibrous proteins (e.g., COLLAGEN; ELASTIN; FIBRONECTINS; and LAMININ).
The property of dentin that permits passage of light, heat, cold, and chemical substances. It does not include penetration by microorganisms.
Substances which reduce or eliminate dentinal sensitivity or the pain associated with a source of stimulus (such as touch, heat, or cold) at the orifice of exposed dentinal tubules causing the movement of tubular fluid that in turn stimulates tooth nerve receptors.
Dentin sensitivity is a short, sharp pain originating from exposed dentin in response to stimuli, typically thermal, evaporative, tactile, osmotic, or chemical changes in the oral environment.
A disease of bone marked by thinning of the cortex by fibrous tissue containing bony spicules, producing pain, disability, and gradually increasing deformity. Only one bone may be involved (FIBROUS DYSPLASIA, MONOSTOTIC) or several (FIBROUS DYSPLASIA, POLYOSTOTIC).
Cements that act through infiltration and polymerization within the dentinal matrix and are used for dental restoration. They can be adhesive resins themselves, adhesion-promoting monomers, or polymerization initiators that act in concert with other agents to form a dentin-bonding system.
A group of hereditary disorders involving tissues and structures derived from the embryonic ectoderm. They are characterized by the presence of abnormalities at birth and involvement of both the epidermis and skin appendages. They are generally nonprogressive and diffuse. Various forms exist, including anhidrotic and hidrotic dysplasias, FOCAL DERMAL HYPOPLASIA, and aplasia cutis congenita.
Dentin formed by normal pulp after completion of root end formation.
A chronic lung disease developed after OXYGEN INHALATION THERAPY or mechanical ventilation (VENTILATION, MECHANICAL) usually occurring in certain premature infants (INFANT, PREMATURE) or newborn infants with respiratory distress syndrome (RESPIRATORY DISTRESS SYNDROME, NEWBORN). Histologically, it is characterized by the unusual abnormalities of the bronchioles, such as METAPLASIA, decrease in alveolar number, and formation of CYSTS.

Mutation of the signal peptide region of the bicistronic gene DSPP affects translocation to the endoplasmic reticulum and results in defective dentine biomineralization. (1/12)

Dentine dysplasia type II is an autosomal dominant disorder in which mineralization of the dentine of the primary teeth is abnormal. On the basis of the phenotypic overlap between, and shared chromosomal location with, dentinogenesis imperfecta type II, a second disorder of dentine mineralization, it has been proposed that the two conditions are allelic. As recent studies have shown that dentinogenesis imperfecta type II results from mutation of the bicistronic dentine sialophosphoprotein gene (DSPP ), we have tested this hypothesis by sequencing DSPP in a family with a history of dentine dysplasia type II. Our results have shown that a missense change, which causes the substitution of a tyrosine for an aspartic acid in the hydrophobic signal peptide domain of the protein, underlies the phenotype in this family. Biochemical analysis has further demonstrated that this mutation causes a failure of translocation of the encoded proteins into the endoplasmic reticulum, and is therefore likely to lead to a loss of function of both dentine sialoprotein and dentine phosphoprotein.  (+info)

Phenotypic variation in dentinogenesis imperfecta/dentin dysplasia linked to 4q21. (2/12)

Dentinogenesis imperfecta (DGI) and dentin dysplasia (DD) are allelic disorders that primarily affect the formation of tooth dentin. Both conditions are autosomal-dominant and can be caused by mutations in the dentin sialophosphoprotein gene (DSPP, 4q21.3). We recruited 23 members of a four-generation kindred, including ten persons with dentin defects, and tested the hypothesis that these defects are linked to DSPP. The primary dentition showed amber discoloration, pulp obliteration, and severe attrition. The secondary dentition showed either pulp obliteration with bulbous crowns and gray discoloration or thistle-tube pulp configurations, normal crowns, and mild gray discoloration. Haplotype analyses showed no recombination between three 4q21-q24 markers and the disease locus. Mutational analyses identified no coding or intron junction sequence variations associated with affection status in DMP1, MEPE, or the DSP portion of DSPP. The defects in the permanent dentition were typically mild and consistent with a diagnosis of DD-II, but some dental features associated with DGI-II were also present. We conclude that DD-II and DGI-II are milder and more severe forms, respectively, of the same disease.  (+info)

Hereditary dentine disorders: dentinogenesis imperfecta and dentine dysplasia. (3/12)

 (+info)

A dentin sialophosphoprotein mutation that partially disrupts a splice acceptor site causes type II dentin dysplasia. (4/12)

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Overlapping DSPP mutations cause dentin dysplasia and dentinogenesis imperfecta. (5/12)

Dentinogenesis imperfecta (DGI) and dentin dysplasia (DD) are allelic disorders due to mutations in DSPP. Typically, the phenotype breeds true within a family. Recently, two reports showed that 3 different net -1 bp frameshift mutations early in DSPP's repeat domain caused DD, whereas 6 more 3' frameshift mutations were associated with DGI. Here we identify a DD kindred with a novel -1 bp frameshift (c.3141delC) that falls within the portion of the DSPP repeat domain previously associated solely with the DGI phenotype. This new frameshift mutation shows that overlapping DSPP mutations can give rise to either DGI or DD phenotypes. Furthermore, the consistent kindred presentation of the DD or DGI phenotype appears to be dependent on an as-yet-undescribed genetic modifier closely linked to DSPP.  (+info)

Dentin dysplasia type I with pyogenic granuloma in a 12-year-old girl. (6/12)

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Familial hypophosphatemic rickets. (7/12)

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Variation of dentin dysplasia type I: report of atypical findings in the permanent dentition. (8/12)

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Dentin dysplasia is a rare genetic disorder that affects the development and formation of dentin, which is the hard tissue beneath the tooth's enamel. There are two types of dentin dysplasia: type I and type II.

Type I dentin dysplasia is also known as "radicular dentin dysplasia" and primarily affects the roots of the teeth. The roots may be short, thin, or even absent, which can make the teeth appear darkened or discolored. Despite the abnormal root structure, the teeth are often resistant to decay.

Type II dentin dysplasia is also known as "coronal dentin dysplasia" and primarily affects the crowns of the teeth. The teeth may appear normal in size and shape, but they can be prone to fractures and abscesses due to the thinness or absence of dentin beneath the tooth's enamel.

Both types of dentin dysplasia are inherited in an autosomal dominant manner, which means that a child has a 50% chance of inheriting the disorder if one parent is affected. Treatment for dentin dysplasia typically involves restorative dental procedures to address any tooth decay or fractures, and regular dental checkups to monitor the health of the teeth and gums.

Dentinogenesis Imperfecta (DI) is a genetic disorder that affects the development and formation of dentin, which is the hard tissue beneath the enamel of teeth. This condition results in teeth that are discolored, translucent, and sensitive to temperature changes. The teeth may also wear down easily and be prone to fractures.

There are two main types of Dentinogenesis Imperfecta: type I and type II. Type I is associated with a genetic disorder called osteogenesis imperfecta (OI), which affects the development of bones as well as teeth. Type II, on the other hand, is not associated with OI and only affects the teeth.

The inheritance pattern for Dentinogenesis Imperfecta is autosomal dominant, meaning that a child has a 50% chance of inheriting the condition if one parent has it. However, some cases may be due to new mutations in the gene and not inherited from a parent. Treatment for DI typically involves dental restorations such as crowns or veneers to improve the appearance and function of the teeth.

Dentin is the hard, calcified tissue that lies beneath the enamel and cementum of a tooth. It forms the majority of the tooth's structure and is composed primarily of mineral salts (hydroxyapatite), collagenous proteins, and water. Dentin has a tubular structure, with microscopic channels called dentinal tubules that radiate outward from the pulp chamber (the center of the tooth containing nerves and blood vessels) to the exterior of the tooth. These tubules contain fluid and nerve endings that are responsible for the tooth's sensitivity to various stimuli such as temperature changes, pressure, or decay. Dentin plays a crucial role in protecting the dental pulp while also providing support and structure to the overlying enamel and cementum.

In dental terminology, "space maintenance" refers to the use of a device or appliance to maintain the proper space between teeth following the loss of a primary (baby) tooth. This is especially important when the lost tooth is a molar, as it plays a crucial role in maintaining the alignment and spacing of the remaining teeth and the eruption path for the developing permanent tooth.

Space maintainers can be fixed or removable and are typically made from materials such as stainless steel, plastic, or acrylic. They help prevent dental issues like crowding, misalignment, and impaction of adjacent and/or succeeding teeth, which may lead to more complex orthodontic treatments in the future. It is essential that space maintainers are custom-made and properly fitted by a dentist or an orthodontist to ensure their effectiveness and avoid potential damage to surrounding tissues.

Tooth mobility, also known as loose teeth, refers to the degree of movement or displacement of a tooth in its socket when lateral forces are applied. It is often described in terms of grades:

* Grade 1: Tooth can be moved slightly (up to 1 mm) with finger pressure.
* Grade 2: Tooth can be moved up to 2 mm with finger pressure.
* Grade 3: Tooth can be moved more than 2 mm or can be removed from its socket with manual pressure.

Increased tooth mobility can be a sign of periodontal disease, trauma, or other dental conditions and should be evaluated by a dentist. Treatment may include deep cleaning, splinting, or surgery to restore stability to the affected teeth.

Dental pulp calcification, also known as pulp stones or denticles, refers to the formation of hard tissue within the pulp chamber of a tooth. The pulp chamber is the central part of a tooth that contains its nerves, blood vessels, and connective tissues.

Pulp calcification occurs when the soft tissue of the pulp gradually transforms into a harder, calcified substance. This can happen as a result of aging, injury, or inflammation in the pulp chamber. Over time, these calcifications can build up and make the pulp chamber smaller, which can potentially lead to problems with the tooth's nerve and blood supply.

While dental pulp calcification is not usually harmful on its own, it can cause issues if it becomes severe enough to compress the tooth's nerve or restrict blood flow. In some cases, calcifications may also make root canal treatment more difficult, as there may be less space to work within the pulp chamber.

Sialglycoproteins are a type of glycoprotein that have sialic acid as the terminal sugar in their oligosaccharide chains. These complex molecules are abundant on the surface of many cell types and play important roles in various biological processes, including cell recognition, cell-cell interactions, and protection against proteolytic degradation.

The presence of sialic acid on the outermost part of these glycoproteins makes them negatively charged, which can affect their interaction with other molecules such as lectins, antibodies, and enzymes. Sialglycoproteins are also involved in the regulation of various physiological functions, including blood coagulation, inflammation, and immune response.

Abnormalities in sialglycoprotein expression or structure have been implicated in several diseases, such as cancer, autoimmune disorders, and neurodegenerative conditions. Therefore, understanding the biology of sialoglycoproteins is important for developing new diagnostic and therapeutic strategies for these diseases.

Extracellular matrix (ECM) proteins are a group of structural and functional molecules that provide support, organization, and regulation to the cells in tissues and organs. The ECM is composed of a complex network of proteins, glycoproteins, and carbohydrates that are secreted by the cells and deposited outside of them.

ECM proteins can be classified into several categories based on their structure and function, including:

1. Collagens: These are the most abundant ECM proteins and provide strength and stability to tissues. They form fibrils that can withstand high tensile forces.
2. Proteoglycans: These are complex molecules made up of a core protein and one or more glycosaminoglycan (GAG) chains. The GAG chains attract water, making proteoglycans important for maintaining tissue hydration and resilience.
3. Elastin: This is an elastic protein that allows tissues to stretch and recoil, such as in the lungs and blood vessels.
4. Fibronectins: These are large glycoproteins that bind to cells and ECM components, providing adhesion, migration, and signaling functions.
5. Laminins: These are large proteins found in basement membranes, which provide structural support for epithelial and endothelial cells.
6. Tenascins: These are large glycoproteins that modulate cell adhesion and migration, and regulate ECM assembly and remodeling.

Together, these ECM proteins create a microenvironment that influences cell behavior, differentiation, and function. Dysregulation of ECM proteins has been implicated in various diseases, including fibrosis, cancer, and degenerative disorders.

Dentin permeability refers to the ability of various substances to penetrate or diffuse through the dentin, which is the hard, calcified tissue that lies beneath the enamel and forms the bulk of a tooth. Dentin is composed of microscopic tubules that run from the pulp chamber (which contains the dental pulp) to the exterior of the tooth. These tubules contain fluid and are lined with odontoblastic processes, which are extensions of the cells that form dentin.

When the dentin is exposed due to tooth decay, wear, or other factors, various substances can penetrate through these tubules and cause sensitivity, discomfort, or pain. The permeability of dentin can be influenced by several factors, including the diameter and number of tubules, the thickness and composition of the dentinal tissue, and the presence of dental sealants or other protective coatings.

In general, a higher dentin permeability is associated with increased susceptibility to tooth decay, sensitivity, and other dental problems. Therefore, understanding the factors that influence dentin permeability and developing strategies to reduce it is an important area of research in dental medicine.

Dentin desensitizing agents are chemical substances or materials applied to the teeth to reduce sensitivity in the dental tissues, specifically in the dentin. Dentin is a calcified tissue that lies beneath the tooth's enamel and cementum. It has numerous microscopic tubules that, when exposed due to various factors like gum recession, tooth wear, or dental procedures, can lead to hypersensitivity.

Dentin desensitizing agents work by occluding these dentinal tubules, thus preventing the stimuli (like cold, heat, or touch) from reaching the nerve endings inside the pulp chamber. These agents may contain various active ingredients like fluorides, strontium salts, calcium sodium phosphosilicate, potassium nitrate, arginine, and oxalates. They can be found in different forms, such as toothpaste, gels, varnishes, or bonding agents, and are often used in dental treatments and at-home oral care to alleviate dentinal hypersensitivity.

Dentin sensitivity is a common dental condition characterized by the short, sharp pain or discomfort in response to external stimuli, such as cold air, hot or cold foods and drinks, sweet or sour substances, and physical touch. This pain is typically caused by the exposure of dentin, the hard tissue beneath the tooth's enamel, due to receding gums, tooth decay, or other factors that wear down or damage the protective enamel layer.

When the dentin is exposed, the microscopic tubules within it become sensitive to temperature and pressure changes, allowing external stimuli to reach the nerve endings inside the tooth. This results in the characteristic pain or discomfort associated with dentin sensitivity. Dentin sensitivity can be managed through various treatments, including desensitizing toothpaste, fluoride applications, and dental restorations, depending on the underlying cause of the condition.

Fibrous Dysplasia of Bone is a rare, benign bone disorder that is characterized by the replacement of normal bone tissue with fibrous (scar-like) and immature bone tissue. This results in weakened bones that are prone to fractures, deformities, and pain. The condition can affect any bone in the body but most commonly involves the long bones of the legs, arms, and skull. It can occur as an isolated finding or as part of a genetic disorder called McCune-Albright syndrome. The exact cause of fibrous dysplasia is not fully understood, but it is believed to result from a genetic mutation that occurs during early bone development. There is no cure for fibrous dysplasia, and treatment typically focuses on managing symptoms and preventing complications.

Dentin-bonding agents are substances used in dentistry to create a strong and durable bond between the dental restoration material (such as composite resin, glass ionomer cement, or crowns) and the dentin surface of a tooth. Dentin is the hard tissue that lies beneath the enamel and consists of microscopic tubules filled with fluid.

The primary function of dentin-bonding agents is to improve the adhesion of restorative materials to the tooth structure, enhancing the retention and durability of dental fillings, crowns, veneers, and other types of restorations. These agents typically contain one or more types of bonding resins, such as hydroxyethyl methacrylate (HEMA), 4-methacryloxyethyl trimellitate anhydride (4-META), and/or phosphoric acid ester monomers.

The application process for dentin-bonding agents usually involves several steps, including:

1. Etching the dentin surface with a mild acid to remove the smear layer and expose the collagen network within the dentin tubules.
2. Applying a primer that penetrates into the etched dentin and promotes the infiltration of bonding resins into the dentinal tubules.
3. Applying an adhesive, which is typically a mixture of hydrophilic and hydrophobic monomers, to form a stable bond between the tooth structure and the restoration material.
4. Light-curing the adhesive to polymerize the resin and create a strong mechanical bond with the dentin surface.

Dentin-bonding agents have significantly improved the clinical success of various dental restorations by enhancing their retention, reducing microleakage, and minimizing postoperative sensitivity. However, they may still be susceptible to degradation over time due to factors such as moisture contamination, enzymatic degradation, or hydrolysis, which can lead to the failure of dental restorations. Therefore, continuous advancements in dentin-bonding technology are essential for improving the long-term success and durability of dental restorations.

Ectodermal dysplasia (ED) is a group of genetic disorders that affect the development and formation of ectodermal tissues, which include the skin, hair, nails, teeth, and sweat glands. The condition is usually present at birth or appears in early infancy.

The symptoms of ED can vary widely depending on the specific type and severity of the disorder. Common features may include:

* Sparse or absent hair
* Thin, wrinkled, or rough skin
* Abnormal or missing teeth
* Nail abnormalities
* Absent or reduced sweat glands, leading to heat intolerance and problems regulating body temperature
* Ear abnormalities, which can result in hearing loss
* Eye abnormalities

ED is caused by mutations in genes that are involved in the development of ectodermal tissues. Most cases of ED are inherited in an autosomal dominant or autosomal recessive pattern, meaning that a child can inherit the disorder even if only one parent (dominant) or both parents (recessive) carry the mutated gene.

There is no cure for ED, but treatment is focused on managing the symptoms and improving quality of life. This may include measures to maintain body temperature, such as cooling vests or frequent cool baths; dental treatments to replace missing teeth; hearing aids for hearing loss; and skin care regimens to prevent dryness and irritation.

Secondary dentin is a type of dentin that is formed after the initial development of the tooth. It is produced in response to stimuli such as tooth wear or injury and continues to form throughout an individual's life. Unlike primary dentin, which is laid down during tooth development and has a more uniform structure, secondary dentin is often deposited in a less organized manner and can vary in thickness. The formation of secondary dentin can help to protect the pulp tissue within the tooth from further damage or infection.

Bronchopulmonary dysplasia (BPD) is a chronic lung disease that primarily affects premature infants. It is defined as the need for supplemental oxygen at 28 days of life or beyond, due to abnormal development and injury to the lungs.

The condition was first described in the 1960s, following the introduction of mechanical ventilation and high concentrations of oxygen therapy for premature infants with respiratory distress syndrome (RDS). These treatments, while lifesaving, can also cause damage to the delicate lung tissue, leading to BPD.

The pathogenesis of BPD is complex and involves an interplay between genetic factors, prenatal exposures, and postnatal injury from mechanical ventilation and oxygen toxicity. Inflammation, oxidative stress, and impaired lung development contribute to the development of BPD.

Infants with BPD typically have abnormalities in their airways, alveoli (air sacs), and blood vessels in the lungs. These changes can lead to symptoms such as difficulty breathing, wheezing, coughing, and poor growth. Treatment may include oxygen therapy, bronchodilators, corticosteroids, diuretics, and other medications to support lung function and minimize complications.

The prognosis for infants with BPD varies depending on the severity of the disease and associated medical conditions. While some infants recover completely, others may have long-term respiratory problems that require ongoing management.

DentinDentin dysplasia • Dentine bonding agents • Dentine hypersensitivity • Dentinogenesis • Dentinogenesis imperfecta • ... Radioactive dentin abrasion • Ragas Dental College • Raman Bedi • Randy Starr • Ranula • Receding gums • Reduced enamel ... Segmental odontomaxillary dysplasia • Sheila Faith • Shovel-shaped incisors • Sialogram • Signal • Simon Hullihen • Sinodonty ... Cemento-osseous dysplasia • Cementoblast • Cementoblastoma • Cementoenamel junction • Cementogenesis • Cementum • Central giant ...
ATN1 Dentin dysplasia, type II; 125420; DSPP Dentinogenesis imperfecta, Shields type II; 125490; DSPP Dentinogenesis imperfecta ... MAPK10 Epiphyseal dysplasia, multiple 1; 132400; COMP Epiphyseal dysplasia, multiple, 2; 600204; COL9A2 Epiphyseal dysplasia, ... FXN Frontometaphyseal dysplasia; 305620; FLNA Frontonasal dysplasia 2; 613451; ALX4 Frontonasal dysplasia 3; 613456; ALX1 ... PAX3 Craniofrontonasal dysplasia; 304110; EFNB1 Cranio-lenticulo-sutural dysplasia; 607812; SEC23A Craniometaphyseal dysplasia ...
Dentinal dysplasia is another disorder of dentin. Congenital erythropoietic porphyria (Gunther disease) is a rare congenital ... Secondary dentin is darker and more opaque than primary dentin. This gives the dentin an overall darker appearance. At the same ... The underlying dentin layer is darker than enamel, yellow-brown in color, and less transparent. Dentin forms the bulk of the ... The pulp is pink/red due to its vascularity, but is rarely visible through the overlying enamel and dentin unless the thickness ...
de La Dure-Molla M, Philippe Fournier B, Berdal A (April 2015). "Isolated dentinogenesis imperfecta and dentin dysplasia: ... Secondary dentin is a layer of dentin formed after the tooth's root has fully formed. Tertiary dentin develops as a result of a ... Thus, interglobular dentin is slightly less mineralized than globular dentin. Interglobular dentin is especially evident in ... Below it lies the circumpulpal dentin, more mineralized dentin which makes up most of the dentin layer and is secreted after ...
... dentin dysplasia MeSH C07.650.800.270 - dentinogenesis imperfecta MeSH C07.650.800.370 - fused teeth MeSH C07.650.800.600 - ... dentin dysplasia MeSH C07.793.700.270 - dentinogenesis imperfecta MeSH C07.793.700.370 - fused teeth MeSH C07.793.700.600 - ... dentin, secondary MeSH C07.793.237.820 - pulpitis MeSH C07.793.237.910 - tooth, nonvital MeSH C07.793.494.293 - dental ...
... dentin dysplasia MeSH C16.131.850.800.270 - dentinogenesis imperfecta MeSH C16.131.850.800.370 - fused teeth MeSH C16.131. ... ectodermal dysplasia MeSH C16.131.260.800.240.350 - focal dermal hypoplasia MeSH C16.131.260.800.300 - fragile X syndrome MeSH ... ectodermal dysplasia MeSH C16.320.180.800.240.350 - focal dermal hypoplasia MeSH C16.320.180.800.300 - fragile X syndrome MeSH ... ectodermal dysplasia MeSH C16.131.077.350.398 - Ellis-van Creveld syndrome MeSH C16.131.077.350.424 - focal dermal hypoplasia ...
Dental fluorosis Dental tissue neoplasm Dentatorubral-pallidoluysian atrophy Dentin dysplasia sclerotic bones Dentin dysplasia ... coronal Dentin dysplasia, radicular Dentinogenesis imperfecta Dentophobia Dependent personality disorder Depersonalization ... chronic spasmodic Dysplasia epiphysealis hemimelica Dysplasia Dysplastic cortical hyperostosis Dysplastic nevus syndrome ... Dysprothrombinemia Dysraphism cleft lip palate limb reduction defects Dyssegmental dysplasia glaucoma Dyssegmental dysplasia ...
Mesectodermal derivatives affected can include the dermis, hypodermis, dentin, head muscles and conjunctival cells, ... Ectrodactyly-ectodermal dysplasia-cleft syndrome, or EEC, and also referred to as EEC syndrome and split hand-split foot- ... ectodermal dysplasia-cleft syndrome: 520 is a rare form of ectodermal dysplasia, an autosomal dominant disorder inherited as a ... Ectodermal dysplasia is characterized by absent sweat glands resulting in dry (hypohydrotic), often scale-like skin, sparse and ...
Enamel dysplasia - insufficient hardness or amount of enamel due to not properly forming. The enamel is soft, flakes off, ... The lesions erode the dentin within a single tooth (or several simultaneously). It spreads rapidly once it reaches the pulp of ... stains easily, has a rough or pitted surface, and exposes underlying dentin once chipped away. In dogs, as in humans, daily ...
... such as in dentin dysplasia. Secondary dentin is formed after root formation is finished and occurs at a much slower rate. It ... mantle dentin, primary dentin, secondary dentin, and tertiary dentin. Odontoblasts differentiate from cells of the dental ... Root dentin is considered different from dentin found in the crown of the tooth (known as coronal dentin) because of the ... Tertiary dentin can be divided into reactionary or reparative dentin. Reactionary dentin is formed by odontoblasts when the ...
Hao J, Narayanan K, Muni T, Ramachandran A, George A (May 2007). "Dentin matrix protein 4, a novel secretory calcium-binding ... "Mutations in FAM20C also identified in non-lethal osteosclerotic bone dysplasia". Clinical Genetics. 75 (3): 271-6. doi:10.1111 ... Hao J, Narayanan K, Muni T, Ramachandran A, George A (May 2007). "Dentin matrix protein 4, a novel secretory calcium-binding ... while their dentin and enamel defects are largely independent from the hypophosphatemia and appear to be a local effects of ...
The different stages of dentin formation result in different types of dentin: mantle dentin, primary dentin, secondary dentin, ... It is rare, most often occurring in a condition called hypohidrotic ectodermal dysplasia. Hypodontia is a lack of some tooth ... tubular microscopic appearance of dentin is a result of the formation of dentin around these extensions. After dentin formation ... Outside the dentin are the newly formed ameloblasts in response to the formation of dentin, which are cells that continue the ...
For example, due to trauma or wear, tertiary dentin can be deposited which can then alter some measurements; thus, caution ... ectodermal dysplasia and tricho-dento-osseous syndrome. Taurodontism may be related to: A retrograde character A primitive ...
Periapical cemental dysplasia is usually found in the lower anterior teeth and does not cause shifting of teeth. Odontomas have ... Although symptoms may not be noticeable, a dull pain and dentin hypersensitivity can occur as growth increases. Visibility of ... Cementomas reveal a more uniform radiolucent band surrounding the lesion than the bands found in cement dysplasia. ... Four types of cementomas are described: Benign cementoblastoma Periapical cemental dysplasia Multiple apical cementomas ...
... (DD) is a rare genetic developmental disorder affecting dentine production of the teeth, commonly exhibiting ... "Dentin Dysplasia Type I - NORD (National Organization for Rare Disorders)". NORD (National Organization for Rare Disorders). ... "Dentin Dysplasia Type II - NORD (National Organization for Rare Disorders)". NORD (National Organization for Rare Disorders). ... Both primary and permanent dentitions can be affected by either type I or type II dentin dysplasia. However, deciduous teeth ...
Dean JA, Hartsfield JK, Wright JT, Hart TC (1998). "Dentin dysplasia, type II linkage to chromosome 4q". J. Craniofac. Genet. ... The preproprotein is secreted by odontoblasts and cleaved into dentin sialoprotein and dentin phosphoprotein. Dentin ... 2006). "Phenotypic variation in dentinogenesis imperfecta/dentin dysplasia linked to 4q21". J. Dent. Res. 85 (4): 329-33. doi: ... 1997). "Dentin phosphoprotein and dentin sialoprotein are cleavage products expressed from a single transcript coded by a gene ...
Dentin dysplasia is a disorder in which the roots and pulp of teeth may be affected. Regional odontodysplasia is a disorder ... Secondary dentin is a layer of dentin produced after root formation and continues to form with age. Tertiary dentin is created ... Dentin, which is less mineralized and less brittle, compensates for enamel and is necessary as a support. Unlike dentin and ... Dentin has microscopic channels, called dentinal tubules, which radiate outward through the dentin from the pulp cavity to the ...
Several genetic diseases such as dentin dysplasia and dentinogenesis imperfecta are also accompanied by pulpal calcifications ... partly fused with dentin 3) Embedded: entirely surrounded by dentin Pulp stones are discrete calcifications found in the pulp ... They are classified: A) On the basis of structure 1) True pulp stones: formed of dentin by odontoblasts 2) False pulp stones: ... Another theory suggests that individuals with Marfan syndrome have connective tissue dysplasia or vascular defects which in the ...
de La Dure-Molla M, Philippe Fournier B, Berdal A (April 2015). "Isolated dentinogenesis imperfecta and dentin dysplasia: ... "radicular dentin dysplasia" in the new classification. Shields' Dentinogenesis Imperfecta type I - this sub-type is not ... "Phenotypic variation in dentinogenesis imperfecta/dentin dysplasia linked to 4q21". Journal of Dental Research. 85 (4): 329-333 ... This sub-type is used in place of Shields' dentine dysplasia type I, in which only the roots of the teeth are affected. Both ...
Dentin dysplasia (DD) is a rare genetic developmental disorder affecting dentine production of the teeth, commonly exhibiting ... "Dentin Dysplasia Type I - NORD (National Organization for Rare Disorders)". NORD (National Organization for Rare Disorders). ... "Dentin Dysplasia Type II - NORD (National Organization for Rare Disorders)". NORD (National Organization for Rare Disorders). ... Both primary and permanent dentitions can be affected by either type I or type II dentin dysplasia. However, deciduous teeth ...
Here is more information on how both types of this hereditary anomaly affect dentin and oral health: ... is rare genetic disorder that affects the dentin and pulp. ... HOW COMMON IS DENTIN DYSPLASIA? Dentin Dysplasia is a rare ... What Is Dentin Dysplasia?. June 19, 2018. By Shakara Robinson Our teeth are composed of 4 dental tissues-enamel, dentin, ... Dentin dysplasia (DD) is rare genetic disorder that affects the dentin and pulp. ...
The signs and symptoms of dentin dysplasia type II are very similar to those of dentinogenesis imperfecta. However, dentin ... Mutations in this gene are also responsible for dentin dysplasia type II. Dentinogenesis imperfecta type I occurs as part of ... Some researchers believe that dentinogenesis imperfecta type II and type III, along with a condition called dentin dysplasia ... Phenotypic variation in dentinogenesis imperfecta/dentin dysplasia linked to 4q21. J Dent Res. 2006 Apr;85(4):329-33. doi: ...
Amelogenesis imperfecta, dentinogenesis imperfecta and dentin dysplasia revisited: problems in classification. J Oral Pathol ... the reconstruction of the intraoral region is very important for the facial aesthetics in the case of genetic dysplasia, trauma ...
Amelogenesis imperfecta, dentinogenesis imperfecta and dentin dysplasia Revisited: problems in classification. J Oral Pathol ...
Short roots = Dentin Dysplasia type I. Obliterated Pulps = DI type 2 or DD type I ... leave affected dentin; change caries environment **(we also had. something similar, but want to leave affected dentin) ... b. Enamel and dentin without pulp exposure. c. Enamel and dentin with pulp exposure. d. Root fracture ... reparative dentin), and interface with dentin that resembles hydroxyapatite ...
dentin dysplasia * blue-grey or brown teeth because the dentin didnt form correctly. the enamel (though healthy) chips off ... dentin secreting cells that retreat to the south (away from the basement membrane) ... growth by layers, layers of dentin, cementum, and enamel to be exact ... Odontoblastic processes that are contained and protected in mineralized cylinders of dentin ...
Dentin is less radiopaque than enamel and has a radiopacity similar to that of bone. The pulp tissue is not mineralized and ... and dentinal dysplasia (DD). These are hereditary diseases with a propensity for intrinsic tooth discoloration. ... These include a surface enamel layer covering only the crown; an inner layer of dentin in both the crown and root; and the core ... Tetracyclines diffuse through dentin to the enamel interface, chelating calcium ions and incorporating into hydroxyapatite as a ...
Palavras-chave : Dentin dysplasia; Dentin; Pediatric Devtistry; Radiography; Panoramic; Radiography; Dental. · resumo em ... Dentinal dysplasia type I (DD1) is a rare inherited dentin abnormalitie caractherized by the presence of normal enamel and ... Dentinal Dysplasia Type I: different aspects of the same condition. Odontol. Clín.-Cient. (Online) [online]. 2012, vol.11, n.2 ... atypical dentin. Clinically, the teeth have normal morphological caractheristics and radiographically the teeth display ...
dentin dysplasia DOID:701 * Hypoplasia of the tooth germ HP:0006353 * periapical periodontitis ...
Dentin Dysplasia, Coronal Whats New Last Posted: Jan 01, 2011 * Dentin dysplasia, coronal From NCATS Genetic and Rare Diseases ...
Dentin Dysplasia. *Gingival Enlargement. *Hairy Tongue. *Oronasal Fistulae. *Periodontal Disease. Teaching Methodologies:. This ...
Dentin Dysplasia, Coronal. *Coronal Dentin Dysplasias. *Dentin Dysplasias, Coronal. *Dysplasia, Coronal Dentin ...
1991) and O Carroll and Duncan (1994) reviewed dentin dysplasia and proposed 4 subtypes of dentin dysplasia type I, which they ... Dentin dysplasia type I. MedGen UID: 97996. •Concept ID: C0399379. •. Congenital Abnormality. ... In dentin dysplasia type I, both primary and secondary dentitions are affected. The color and general morphology of the teeth ... Sponastrime dysplasia is an autosomal recessive spondyloepimetaphyseal dysplasia (SEMD) named for characteristic clinical and ...
Clinical features and causative genes of dentin dysplasia type Ⅰ with a 13-year follow-up Chinese Journal of Stomatology, ...
Dentin Dysplasia Type I *Tooth Agenesis *Dentin Dysplasia Type II *Amelogenesis Imperfecta ...
Dentin is less radiopaque than enamel and has a radiopacity similar to that of bone. The pulp tissue is not mineralized and ... and dentinal dysplasia (DD). These are hereditary diseases with a propensity for intrinsic tooth discoloration. ... These include a surface enamel layer covering only the crown; an inner layer of dentin in both the crown and root; and the core ... Tetracyclines diffuse through dentin to the enamel interface, chelating calcium ions and incorporating into hydroxyapatite as a ...
See also Dentin Dysplasia Explained: Symptoms, Causes, and Treatment. /* custom css */ .tdi_92{ vertical-align: baseline; }.tdi ...
This category includes amelogenesis imperfecta, dentinogenesis imperfecta, dentin dysplasia, hypophosphatasia, and ... Inherited dental syndromes affect the dental hard tissues specifically the enamel, dentin, and/or cementum in a generalized ...
This is due to the similarity to symptoms caused by Dentin Dysplasia II. The primary difference between these conditions is ... Under this layer is a softer, porous material known as dentin. It tends to have a somewhat yellow tone and is the transitional ... that dental dysplasia typically only impacts baby teeth, while dentinogenesis imperfect can impact either or both. ...
Dentin Dysplasia Type I-Diagnosis and Treatment: Case Report. Advances in Dentistry & Oral Health, v. 6, p. 1-4, 2017. ... BOHN, A.C.C.E. ; VALE, G.C. ; PADOVANI, G. C. ; RODRIGUES, L. K. A. ; PRADO JÚNIOR, R. R. . Sucrose Induced Dentin ...
Swollen joint, anemic, loose teeth and dentin dysplasia are because of deficiency of: A. Vitamin C. B. Vitamin D. C. Vitamin B1 ... True about dentin conditioner is: A. It removes the smear layer.. B. It exposes collagen.. C. It opens dentinal tubules.. D. It ... C. Used to machine the dentin. D. Made up of triangular/square blank. # In class III case to prevent the growth of mandible, ... D. To prevent the fracture of dentin. # Bevelling of internal line angle is done to: A. prevent the fracture of amalgam. B. ...
Amelogenesis imperfecta, dentinogenesis imperfecta and dentin dysplasia revisited: problems in classification. J Oral Pathol. ... Furthermore, enamel appeared to have higher radio-density compared to the dentin. Moreover, the dentin appeared normal and ... high radio-density and distinction from the dentin, while (v) and (vi) are the clinical photographs of the patient IV-5 showing ...
... hypomineralized and missing teeth and a case report on dentin dysplasia. Finally, an update on the alternative techniques in ... Other topics were related to tooth dysplasias and more specifically the treatment modalities of hypoplastic, ...
... radicular dentin dysplasia with consequent obliterated pulp chambers, apical translucent cysts, recurrent infections, and early ... Cortical dysplasia, complex, with other brain malformations 9. Complex cortical dysplasia with other brain malformations-9 is a ... Cole-Carpenter syndrome-2 (CLCRP2) is a skeletal dysplasia associated with low bone mass or an osteogenesis imperfecta-like ...
So far ectodermal dysplasia seems to be the most common disorder which arguments need for using dental implants among growing ... Can the Density of Mineralized Dental Tissues (Dentin and Enamel) Be Measured and Compared with 3D Cone Beam Computed ... Key aspects of dental diagnostics and treatment specifics in ectodermal dysplasia patients: comprehensive literature review ... Dental implants survival rates among ectodermal dysplasia patients: aggregation and synthesis of literature data ...
... pathogenesis of radicular dentin dysplasia is still obscure. Type I dentin dysplasia is characterized by normal or slightly ... Dentin dysplasia type II is characterized by yellow, brown, grey, translucent primary teeth with complete pulpal obliteration. ... Also dentine dysplasia type I has subtypes Ia, Ib, Ic and Id. Dentine dysplasia Ia is characterized by complete obliteration of ... A third type of dentine dysplasia or focal odontoblastic dysplasia has radiographic aspects of the other two types of disease. ...
A) Regional odontodysplasia (B) Dentin dysplasia (C) Amelogenesis imperfecta. (D) Dentinogenesis imperfecta. Ans: A. ... B) Fibrous dysplasia (C) Cements-ossifying fibroma (D) Cemento-osseous Dysplasia. Ans: D. 91. Generalized osteopenia in initial ... C) Blue Sclera, Brittle Bones, Opalescent Dentin (D) Blue Sclera, Arachnodactyly, Brittle Bones. Ans: C. 32. All are true for ... A positive correlation between oral candidiasis & epithelial dysplasia is due to capacity of yeast cells (A) To induce ...
Maxillofacial Trauma in Children Amelogenesis Imperfecta Osteogenesis Imperfecta With Blue Sclerae Dentin Dysplasia, Coronal ...
Maxillofacial Trauma in Children Amelogenesis Imperfecta Osteogenesis Imperfecta With Blue Sclerae Dentin Dysplasia, Coronal ...
  • Differential diagnosis is very important to have a definitive diagnosis as some radiographic or histologic features of dentine dysplasia may bear a resemblance to different disorders: Dentinogenesis imperfecta Odontodysplasia Calcinosis Osteogenesis imperfecta Ehlers-Danlos syndromes Goldblatt syndrome Schimke immuno-osseous dysplasia Brachio-skeleto-genital syndrome. (wikipedia.org)
  • In the primary teeth, coronal dentin dysplasia may appear similar to Dentinogenesis Imperfecta type II (DG-II) but if abnormalities features appear to be more pronounced in the permanent teeth, then consider changing the diagnosis to DGI-II instead of DD-2. (wikipedia.org)
  • Some researchers believe that dentinogenesis imperfecta type II and type III, along with a condition called dentin dysplasia type II, are actually forms of a single disorder. (medlineplus.gov)
  • The signs and symptoms of dentin dysplasia type II are very similar to those of dentinogenesis imperfecta. (medlineplus.gov)
  • Phenotypic variation in dentinogenesis imperfecta/dentin dysplasia linked to 4q21. (medlineplus.gov)
  • Dong J, Gu T, Jeffords L, MacDougall M. Dentin phosphoprotein compound mutation in dentin sialophosphoprotein causes dentinogenesis imperfecta type III. (medlineplus.gov)
  • Amelogenesis imperfecta, dentinogenesis imperfecta and dentin dysplasia revisited: problems in classification. (thieme-connect.com)
  • The primary difference between these conditions is that dental dysplasia typically only impacts baby teeth, while dentinogenesis imperfect can impact either or both. (galinisaesthetics.com)
  • Developmental conditions include abnormal morphology of the crown (eg, dens invaginatus, dens evaginatus) and abnormal structure of the dentine (eg, dentine dysplasia, dentinogenesis imperfecta, osteogenesis imperfecta , familial hypophosphatemia). (medscape.com)
  • In addition, genetic diseases such as dentinogenesis imperfecta, dentine dysplasia or some forms of amelogenesis imperfect may also cause tooth discoloration or staining. (justia.com)
  • Type I has been known as radicular dentine dysplasia because the teeth have undeveloped root(s) with abnormal pulp tissue. (wikipedia.org)
  • Dental X-rays are the key to diagnosing dentine dysplasia, especially on permanent teeth. (wikipedia.org)
  • Aim of this study was to analyse clinical case of dentine dysplasia in 8,5- year-old boy. (edu.ua)
  • Thus, history, clinical and radiographic findings revealed this case as dentine dysplasia type I subtype 1c inherited from mother. (edu.ua)
  • Oral rehabilitation of patients with dentine dysplasia type I require effective prevention and a complex approach. (edu.ua)
  • Despite the fact that molecular genetics studies allow to discriminate some dentine disorders, pathogenesis of radicular dentin dysplasia is still obscure. (edu.ua)
  • Branchioskeletogenital syndrome is a rare multiple congenital anomalies/dysmorphic syndrome characterized by moderate intellectual disability distinctive craniofacial features (including brachycephaly facial asymmetry marked hypertelorism blepharochalasis proptosis a broad nose with concave nasal ridge and bulbous nasal tip midface hypoplasia bifid uvula or partial cleft palate and prognathism) progressive dental anomalies (dentigerous cysts radicular dentin dysplasia and early tooth loss) vertebral fusions (particularly of C2-C3) and hypospadias. (globalgenes.org)
  • It is characterized by the presence of normal enamel but atypical dentin with abnormal pulpal morphology. (wikipedia.org)
  • Dentin is the hard, mineralized tissue beneath the enamel and cementum that surrounds and protects the pulp (soft tissue with blood vessels and nerves). (deltadentalnjblog.com)
  • Tooth enamel can fall out prematurely when the dentin layer under the enamel crown is too weak to support it. (deltadentalnjblog.com)
  • Dentin is less radiopaque than enamel and has a radiopacity similar to that of bone. (medscape.com)
  • Dentinal dysplasia type I (DD1) is a rare inherited dentin abnormalitie caractherized by the presence of normal enamel and atypical dentin. (bvsalud.org)
  • Inherited dental syndromes affect the dental hard tissues specifically the enamel, dentin, and/or cementum in a generalized manner, involving both primary and permanent teeth. (bvsalud.org)
  • Can the Density of Mineralized Dental Tissues (Dentin and Enamel) Be Measured and Compared with 3D Cone Beam Computed Tomography in Cases of Ectodermal Dysplasia? (researchgate.net)
  • Dental caries erode the protective layers of the tooth (ie, enamel, dentin) and allow bacteria to invade the pulp, producing a pulpitis. (medscape.com)
  • A tooth is comprised of outer hard enamel and an inner dentin layer, which are highly mineralized tissues. (justia.com)
  • In addition to the high prevalence of dental enamel anomalies, in many enamel alterations, its presence is absent or in small amount, and therefore there is a greater possibility of dental caries, since the dentin is unprotected 3 , which hinders or overlaps diagnosis of the initial lesion. (bvsalud.org)
  • Amelogenesis imperfecta refers to a group of development anomalies of the teeth (also referred as hereditary dysplasia) that affects the genome of the individual and is related to at least one of the stages of enamel formation, being a hereditary characteristic that affects both the deciduous as the permanent dentition. (bvsalud.org)
  • On light microscopic examination of the permanent teeth, the coronal dentin is normal, but further apically becomes irregular, fills the pulp chamber, and has a 'sand-dune' morphology. (nih.gov)
  • Consensus statements of several international congresses and conferences include option of using dental implants as a potentially effective treatment alternative for ectodermal dysplasia patients. (researchgate.net)
  • So far ectodermal dysplasia seems to be the most common disorder which arguments need for using dental implants among growing patients Object. (researchgate.net)
  • Dentin dysplasia (DD) is a rare genetic developmental disorder affecting dentine production of the teeth, commonly exhibiting an autosomal dominant inheritance that causes malformation of the root. (wikipedia.org)
  • However, deciduous teeth affected by type II dentin dysplasia have a characteristic blue-amber discolouration, whilst the other dentition appears normal. (wikipedia.org)
  • Dentin Dysplasia is a rare hereditary disturbance, seen in 1 in 100,000 people, characterized by irregular development of dentin in a person's teeth. (deltadentalnjblog.com)
  • With Type 1 DD, care and treatment recommended by your endodontist makes it possible to maintain the appearance and function of teeth affected by dental dysplasia. (deltadentalnjblog.com)
  • However, dentin dysplasia type II affects the primary teeth much more than the permanent teeth. (medlineplus.gov)
  • Teeth with defective dentin are discolored, weak, and more likely to decay and break. (medlineplus.gov)
  • Type I dentin dysplasia is characterized by normal or slightly coloured crowns with no roots or only rudimentary roots, reduced pulp space in permanent teeth and incomplete or total obliteration of the pulp chambers, and periapical radiolucent arradiolucencies are possible as well. (edu.ua)
  • These proteins are involved in the formation of dentin, which is a bone-like substance that makes up the protective middle layer of each tooth. (medlineplus.gov)
  • It may also show hypersensitivity of dentin and tooth pain in some cases. (cancerworld.info)
  • Bleaching agents cross the DEJ and interact in the subjacent dentin with the chromophores, pigments, and ions that are cause tooth staining. (justia.com)
  • Sponastrime dysplasia is an autosomal recessive spondyloepimetaphyseal dysplasia (SEMD) named for characteristic clinical and radiographic findings, including spine (spondylar) abnormalities, midface hypoplasia with a depressed nasal bridge, and striation of the metaphyses. (nih.gov)
  • DI-2, DI-3 and DD-2 share the same genetic mutation of dentin sialophosphoprotein, that is located on chromosome 4. (wikipedia.org)
  • Dentin dysplasia (DD) is rare genetic disorder that affects the dentin and pulp. (deltadentalnjblog.com)
  • Therefore, the reconstruction of the intraoral region is very important for the facial aesthetics in the case of genetic dysplasia, trauma, or even in cases for rejuvenating purposes only. (thieme-connect.com)
  • Cole-Carpenter syndrome-2 (CLCRP2) is a skeletal dysplasia associated with low bone mass or an osteogenesis imperfecta-like syndrome. (beds.ac.uk)
  • Both primary and permanent dentitions can be affected by either type I or type II dentin dysplasia. (wikipedia.org)
  • Mutations in this gene are also responsible for dentin dysplasia type II. (medlineplus.gov)
  • In dentin dysplasia type I, both primary and secondary dentitions are affected. (nih.gov)
  • 1980). Subclassification of Dentin Dysplasia Type I O Carroll et al. (nih.gov)
  • 1991) and O Carroll and Duncan (1994) reviewed dentin dysplasia and proposed 4 subtypes of dentin dysplasia type I, which they designated as DD1a-d. (nih.gov)
  • LIMA, M. D. M. . Dentin Dysplasia Type I-Diagnosis and Treatment: Case Report. (ufpi.br)
  • DSPP gene mutations alter the proteins made from the gene, leading to the production of abnormally soft dentin. (medlineplus.gov)
  • Although runt-related transcription factor 2 (RUNX2) has been considered a determinant of cleidocranial dysplasia (CCD), some CCD patients were free of RUNX2 mutations. (biomedcentral.com)
  • Defectos en la AMELOGÉNESIS normal causados por cualquier factor local, sistémico, ambiental o genético. (bvsalud.org)
  • Differential diagnosis is very important to have a definitive diagnosis as some radiographic or histologic features of dentine dysplasia may bear a resemblance to different disorders: Dentinogenesis imperfecta Odontodysplasia Calcinosis Osteogenesis imperfecta Ehlers-Danlos syndromes Goldblatt syndrome Schimke immuno-osseous dysplasia Brachio-skeleto-genital syndrome. (wikipedia.org)
  • In the primary teeth, coronal dentin dysplasia may appear similar to Dentinogenesis Imperfecta type II (DG-II) but if abnormalities features appear to be more pronounced in the permanent teeth, then consider changing the diagnosis to DGI-II instead of DD-2. (wikipedia.org)
  • Dentinogenesis imperfecta (DGI) and dentin dysplasia (DD) are allelic disorders due to mutations in DSPP. (nih.gov)
  • Hereditary dental developmental anomalies include amelogenesis imperfecta (AI), dentinogenesis imperfecta (DI), and dentin dysplasia (DD). (blueprintgenetics.com)
  • Some researchers believe that dentinogenesis imperfecta type II and type III, along with a condition called dentin dysplasia type II, are actually forms of a single disorder. (medlineplus.gov)
  • The signs and symptoms of dentin dysplasia type II are very similar to those of dentinogenesis imperfecta. (medlineplus.gov)
  • Phenotypic variation in dentinogenesis imperfecta/dentin dysplasia linked to 4q21. (medlineplus.gov)
  • Dong J, Gu T, Jeffords L, MacDougall M. Dentin phosphoprotein compound mutation in dentin sialophosphoprotein causes dentinogenesis imperfecta type III. (medlineplus.gov)
  • A type of dental dysplasia occurring in dentinogenesis imperfecta in which the pulp chambers are enlarged and there is a reduced amount of coronal dentin. (nih.gov)
  • Dentinogenesis imperfecta affects a different part of the tooth, the dentin. (healthline.com)
  • 5 Some forms of ectodermal dysplasia and of amelogenesis imperfecta are inherited as autosomal recessive traits. (ada.org)
  • People who have weak enamel due to genetics or childhood illnesses like Amelogenesis imperfect and Enamel dysplasia are easily susceptible to dental decay. (sanjivanidentalcare.co.in)
  • Dentin dysplasia (DD) is a rare genetic developmental disorder affecting dentine production of the teeth, commonly exhibiting an autosomal dominant inheritance that causes malformation of the root. (wikipedia.org)
  • Type I has been known as radicular dentine dysplasia because the teeth have undeveloped root(s) with abnormal pulp tissue. (wikipedia.org)
  • Dental X-rays are the key to diagnosing dentine dysplasia, especially on permanent teeth. (wikipedia.org)
  • A rare ectodermal dysplasia syndrome characterized by the association of sparse, fine, dry, slow growing hair with variable dental abnormalities including oligodontia, peg-shaped incisors, and shell teeth. (nih.gov)
  • DI is another group of disorders of tooth development characterized by severe hypomineralization of dentin and altered dentin structure. (blueprintgenetics.com)
  • These proteins are involved in the formation of dentin, which is a bone-like substance that makes up the protective middle layer of each tooth. (medlineplus.gov)
  • Vps4b heterozygous mice do not develop tooth defects that replicate human dentin dysplasia I. (bvsalud.org)
  • Dentin has tiny tubes that are directly connected with the nerve of the tooth. (sanjivanidentalcare.co.in)
  • This large field may be shared in two main categories, skeletal dysplasia and metabolic bone diseases. (europa.eu)
  • The diagnosis odontodysplasia requires clinical and radiological exams, in which unusually large pulp chambers and large pulp room chambers with thin enamel and dentin are visible. (nih.gov)
  • DSPP gene mutations alter the proteins made from the gene, leading to the production of abnormally soft dentin. (medlineplus.gov)
  • Treatment of HDPCs with 1α,25(OH) 2 D 3 at a concentration of 10 nM or 100 nM significantly upregulated the expression of dentin sialophosphoprotein (DSPP) and dentin matrix protein1 (DMP1), the odontogenesis-related genes. (molcells.org)
  • DI-2, DI-3 and DD-2 share the same genetic mutation of dentin sialophosphoprotein, that is located on chromosome 4. (wikipedia.org)
  • Hidrotic ectodermal dysplasia 2, or Clouston syndrome (referred to as HED2 throughout this GeneReview) is characterized by a triad of major clinical features including partial-to-complete alopecia, nail dystrophy, and palmoplantar hyperkeratosis. (beds.ac.uk)
  • Establish four disease specific modules on Fibrous Dysplasia/McCune Albright syndrome (FD/MAS), Osteogenesis Imperfecta (OI), Rare Hypophosphatemia and Achondroplasia (ACHO), that capture genetic, clinical and patient related outcome measures in a scalable manner to other RBMCs. (europa.eu)
  • 2 Disorders with X-linked inheritance include X-linked hypohydrotic ectodermal dysplasia, fragile X syndrome, and factor VIII deficiency (hemophilia). (ada.org)
  • It is characterized by the presence of normal enamel but atypical dentin with abnormal pulpal morphology. (wikipedia.org)
  • Dentinal dysplasia type I (DD1) is a rare inherited dentin abnormalitie caractherized by the presence of normal enamel and atypical dentin. (bvsalud.org)
  • Dysplasias include developmental abnormalities in size, organization, and location of various elements of the neural structures. (nih.gov)
  • Results: Compared to controls, AD Brtl+/- mice featured thinner dentin, ectopic pulp calcification and alveolar bone defects, while Gly610Cys+/- mice had relatively normal morphology at young ages. (nih.gov)
  • Background Osteogenesis imperfecta (OI) is a heritable bone dysplasia caused by defects in collagen processing and synthesis. (nih.gov)
  • Both Crtap-/- and Ppib-/- mice featured parallel phenotypes including increased acellular cementum, and reduced cellular cementum, dentin width and root length. (nih.gov)
  • Odontomas consist of an irregular opaque mass of different dental tissues such as enamel, dentin and cementum surrounded by a radiolucent line or band representing the fibrous capsule. (musculoskeletalkey.com)
  • Cheng ES, Tsuji M, Suzuki S, Moriyama K. An overview of the intraoral features and craniofacial morphology of growing and adult Japanese cleidocranial dysplasia subjects. (tmdu-mort.com)
  • Our focus includes over 150 severe rare bone diseases that affect cartilage, bones and dentin and more than 40 severe rare diseases affecting phosphate and calcium metabolism. (europa.eu)
  • 31. Dentin Dysplasia in Notum Knockout Mice. (nih.gov)
  • Models of neural dysplasia in rats include treatment with methylazoxymethanol acetate followed by induction of epileptiform seizures by pilocarpine. (nih.gov)
  • Dentin is less radiopaque than enamel and has a radiopacity similar to that of bone. (medscape.com)
  • Role of cortical dysplasia in epileptogenesis following prolonged febrile seizure. (nih.gov)
  • Persistent hyperplastic main vitreous and recessive oculo-dento-osseous dysplasia. (thequantumdrift.com)
  • shows, at low magnification, the appearance of uncommonly recognized incidental rodent hippocampal dysplasia. (nih.gov)