Dentin dysplasia
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Dentin dysplasia is a rare genetic disorder that affects dentin formation, resulting in abnormal dentin and pulpal morphology. There are two types: type 1 affects the roots and causes them to be short and conical, while type 2 affects the crowns and causes bulbous or rounded crowns. Symptoms include premature loss of teeth due to extreme mobility from short roots or wearing away of rounded crowns. Radiographs show obliterated pulp chambers in primary teeth and crescent-shaped remnants in permanent teeth for type 1, while type 2 shows "thistle tube"-like enlarged pulp chambers in permanent tooth crowns. There is no treatment for the disease other than dental monitoring and extraction of mobile teeth.
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14. esmino dentinogenesis imperfecta
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27.dentine dysplasia
Dentine dysplasia is a rare genetic disorder characterized by abnormal dentin formation and pulp morphology. There are two main types - Shield Type I, which affects the roots and causes extremely small, translucent teeth with excessive mobility. Shield Type II affects both the roots and crowns, resulting in differences in tooth color and normal root size. Radiographs reveal short, conical roots with obliterated pulp chambers and canals for Shield Type I, and a "thistle tube" appearance with multiple pulp stones for Shield Type II. Proper oral hygiene and endodontic treatments are recommended for management.
Dentin Dysplasia
Dentin dysplasia (DD) is a rare hereditary disturbance is inherited as an autosomal dominant trait.
unknown etiology that affects approximately 1 :100,000.
In 1972, Witkop classified it into type I and type II which affect both dentitions.DD Type I
Radicular dentin dysplasia
Characterized by:-
1.Both dentitions are affected.
2.Normal appearing crowns
3.No or only rudimentary root development (rootless teeth)
4.Incomplete or total obliteration of the pulp chamber.
5.Teeth may exhibit extreme mobility and exfoliate prematurely.DD type II
coronal dentin dysplasia
Characterized by:-
1.partial pulpal obliteration.
2.Thistle-tube-or flame-shaped coronal pulp chambers
3. Thread-like root canals
4. Usually the absence of periapical radiolucencies.
5. In this type of anomaly, teeth roots are of normal shape and contour.The enamel and the immediately subjacent dentin appear normal.
Deeper layers of dentin show an atypical tubular pattern with an amorphous, atubular area, and irregular organization.
Normal dentinal tubule formation appears to have been blocked so that new dentine forms around obstacles and takes on the characteristic appearances described as “lava flowing around boulders”The radiograph revealed features of dentine dysplasia type I with normal appearance of crown but no root development Autosomal Dominant Disorder:
Manifested in heterozygous states
At least one parent of index case is usually affected
Both males and females are affected.
Clinical feature can be modified by variation in penetrance and expressivity. Some individual inherit the mutant gene but are phenotpically normal. This is reffered to as “incomplete penetrance”.
In many condition the age of onset is delayed.
Inheritance Pattern:
Typical pattern is a heterozygous affected parent with a homozygous unaffected parent.
Every child has one chance in two of having the disease
Both sexes are affected equally..Autosomal Recessive Disorder
Largest category of Mendelian disorder
Usually does not affect the parent of the affected individual, but sibling may show the disease.
Complete penetrance is common.
Onset is frequently early in life.
Usually affect enzymatic proteins.
Pattern Of Inheritance:
Typical pattern is two heterozygous unaffected (carrier) parent.
The triat does not usually affect the parent, but siblings may show the disease
Siblings have one chance in four of being affected
Both sexes affected equally.
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(1) The document discusses amelogenesis imperfecta (AI), which refers to hereditary defects in enamel formation. It describes several classifications of AI based on clinical, radiographic, and histological findings. (2) The main types are hypoplastic (reduced enamel), hypocalcified (soft enamel), and hypomaturation (improperly formed enamel). Clinical features, inheritance patterns, and prevalence are provided for each type. (3) The case report describes a 26-year-old female patient diagnosed with hypoplastic AI based on yellow-brown tooth discoloration and decreased vertical dimension. She was treated with periodontal therapy and full-mouth fixed bridges.
Dentin dysplasia
Dentin Dysplasia (DD) :-
• is a rare disturbance of dentin formation characterized by normal enamel but atypical dentin formation with abnormal pulpal morphology.
• It was Ballschmiede in 1920 who first reported such a condition as “rootless teeth”.
• In 1939 Rushton termed this condition as “Dentin dysplasia”.
• It is an autosomal dominant • hereditary disease.
unknown etiology that affects approximately 1 :100,000.
TYPES
• Shields and associates separated it in:
1. Type I (dentin dysplasia)
2. Type II (anomalous dysplasia of dentin)
• Witkop refereed as,
1. Radicular (Type I)
2. Coronal (Type II)
Developmental disturbances
This document provides an overview of developmental disturbances of the jaws and teeth. It begins with introductions and terminology. It then discusses various developmental disturbances that can affect the jaws, such as agnathia, micrognathia, macrognathia, and clefts. Tooth disturbances covered include anomalies in size, number, morphology, structure, and position. Specific conditions discussed include microdontia, hypodontia, taurodontism, amelogenesis imperfecta, and dens invaginatus. Finally, the document examines some developmental cysts of the jaws, such as the nasopalatine duct cyst, median palatal cyst, and Stafne cyst. The conclusion thanks the reader.
A rare condition affecting the primary and permanent by dr ibrahim
Dentin dysplasia is a rare genetic condition that affects tooth development. There are two types:
Type I affects both dentitions and causes teeth to have normal crowns but abnormal, stunted roots. The pulp chambers and root canals are often completely obliterated. Teeth frequently exfoliate prematurely.
Type II primarily affects the primary dentition, causing teeth to appear translucent. However, the permanent dentition appears normal. It is characterized by an enlarged pulp chamber and abnormal pulp canal morphology.
The document describes a case study of a 7-year-old girl diagnosed with Type I dentin dysplasia based on clinical and radiographic features including rootless teeth and obliterated
Dentinogenesis imperfecta
Dentinogenesis imperfecta is a disorder that causes teeth to be discolored and translucent. The teeth are also weaker, making them prone to breakage and loss. It affects approximately 1 in 6,000 to 8,000 people. There are three main types - Type I occurs with osteogenesis imperfecta, Type II is autosomal dominant and may cause hearing loss, and Type III shows extremely thin dentin and enlarged pulp chambers. Treatment focuses on bonding to strengthen and whiten teeth, as other cosmetic procedures could further damage the weakened teeth.
DENTINOGENESIS IMPERFECTA TYPE I: A CASE REPORT
dentinogenesis imperfecta
case report
classification
radiographic findings
histologic findings
treatment
developmental anomalies of teeth
This document provides definitions and an overview of oral pathology. It defines key terms like pathology, oral pathology, disease, signs, symptoms, etiology, and pathogenesis. It then discusses variations that can affect teeth, including variations in number (increase or decrease), size (macrodontia or microdontia), shape (gemination, fusion, etc.), and structure (enamel hypoplasia, hypocalcification, etc.). For each variation, it provides examples and classifications. It discusses specific conditions like amelogenesis imperfecta and dental fluorosis in more depth. Overall, the document concisely covers a wide range of topics relating to anomalies and defects that can affect dental development and structure.
Clinical consideration in tooth development, eruption and shedding
This document discusses various clinical considerations related to tooth development, eruption, and shedding. It begins with an introduction on tooth composition and classifications of developmental defects. It then examines specific defects that can occur during different stages of tooth development, such as anodontia, supernumerary teeth, gemination and fusion during the initiation stage. Enamel hypoplasia, dens invaginatus, taurodontism and amelogenesis imperfecta are among the defects discussed during the apposition stage. Syndromes associated with certain defects and systemic diseases that can cause tooth abnormalities are also reviewed.
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Dentin dysplasia
- 2. Dentin dysplasia- Introduction • Disturbance of dentin formation. • Rare Genetic developmental disorder affecting dentin production of the teeth. • Normal enamel but atypical dentin formation with abnormal pulpal morphology
- 3. First description : “Ballschmiede” -reported spontaneous exfoliation of multiple teeth in four children of one family This phenomenon is called “Rootless teeth”
- 4. ETIOLOGY: Unknown etiology-that affect approximately 1:100000 Type1&2- hereditary disease, transmitted as autosomal dominant . Age: Affects the children because of early loss of teeth. Gender: Both males and females are affected.
- 5. Short and conical roots Pulp chambers are obliterated by multiple nodules of poorly organised dentin. Affected teeth may exhibit increased mobility and exfoliate prematurely .
- 6. Types: There are two types of dentin dysplasia Type 1- Radicular Type 2- Coronal
- 7. Dentin dysplasia Dentin dysplasia –Type 1
- 9. Sign and Symptoms: Radicular Dentin dysplasia Undeveloped roots with abnormal tissues Morphology and colour - normal Slightly amper or bluish brown shine in primary teeth with no or immature root development.
- 10. Coronal dentin dysplasia: Tooth Discoloration Abmormlaities of the pulp chambers. Abnormally rounded (bulbous crown) Wearing away Premature loss of teeth
- 11. Clinical features: Radicular Both dentition are affected. Exhibit extreme mobility &exoliated prematurely as result of short roots. Coronal Both dentition are affected . Deciduous teeth have same yellow,brown,appearance as seen in dentinogenesis imperfect However ,clinical appearance of permanent dentition is normal.
- 12. Clinical features: Clinical features can be modified by variation in penetration and expressivity. Some individual inherit the mutant gene but are phenotypically normal. This is referred to as incomplete penetrance.
- 13. Radiographic features: Radicular In both dentition,root are short,blunt,conical . In decidious teeth,pulp chambers &root canals are completely obliterated. In permanent teeth,crescent shaped pulpal remanent still seen in pulp chamber. Coronal In deciduous teeth,pulp chamber obliterated in type 1& dentinogenesis obliterated. In permanent, exhibit abnormal large pulp chamber in Coronal portion of tooth = “thistle tube”resemble pulpstone.
- 14. Histologic features Radicular Type 1-normal Apical to this may be area of tubular dentin but most of that which obliterates pulp is calcified tubular dentin ,osteodentin &fused denticles. Coronal Deciduous teeth exhibit amorphous and atubular dentin in Radicular portion While Coronal portion is normal. Permanent teeth also shows Relatively normal Coronal dentin ,but the pulp has multiple pulpstones or denticles.
- 15. Treatment and prognosis: There is no treatment for the disease. Dental X-rays are the key to diagnose dentin dysplasia especially on permanent teeth. Its prognosis depends on the occurrence of Periapical lesion due to increased mobility.