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Osteofibrous dysplasia is treated with marginal resection with or without bone grafting, depending on the size of the lesion and the extent of bony involvement. However, due to the high rate of recurrence in skeletally immature individuals, this procedure is usually postponed until skeletal maturity.
Four types of cementomas are described :
- Benign cementoblastoma
- Periapical cemental dysplasia
- Multiple apical cementomas
- Gigantiform cementoma
Cementoma is an odontogenic tumor of cementum. It usually occurs after root development is finished, found predominantly in African American women older than 40. It is found most commonly in the mandible in the region of the lower molar teeth. It causes distortion of surrounding areas but is a usually painless growth, at least initially. Considerable thickening of the cementum can often be observed. A periapical form is also recognized.
Osteofibrous dysplasia (also known as ossifying fibroma) is a rare, benign non-neoplastic condition with no known cause. It is considered a fibrovascular defect. Campanacci described this condition in two leg bones, the tibia and fibula, and coined the term. This condition should be differentiated from Nonossifying fibroma and fibrous dysplasia of bone.
Dysplasia (from Ancient Greek δυσ- "dys-", "bad" or "difficult" and πλάσις "plasis", "formation") is a term used in pathology to refer to an abnormality of development or an epithelial anomaly of growth and differentiation (epithelial dysplasia).
The terms hip dysplasia, fibrous dysplasia, and renal dysplasia refer to an abnormal development, at macroscopic or microscopical level.
Myelodysplastic syndromes, or dysplasia of blood-forming cells, show increased numbers of immature cells in the bone marrow, and a decrease in mature, functional cells in the blood.
Examples of dysplasia include epithelial dysplasia of the cervix (cervical intraepithelial neoplasia – a disorder commonly detected by an abnormal pap smear) consisting of an increased population of immature (basal-like) cells which are restricted to the mucosal surface, and have not invaded through the basement membrane to the deeper soft tissues. Analogous conditions include vaginal intraepithelial neoplasia and vulvar intraepithelial neoplasia. Metanephric dysplastic hematoma of the sacral region is a dysplastic overgrowth observed in infants.
Individuals affected by certain ED syndromes cannot perspire. Their sweat glands may function abnormally or may not have developed at all because of inactive proteins in the sweat glands. Without normal sweat production, the body cannot regulate temperature properly. Therefore, overheating is a common problem, especially during hot weather. Access to cool environments is important.
Several studies have examined salivary flow rate in individuals and found parotid and submandibular salivary flow ranging from 5 to 15 times lower than average. This is consistent with the salivary glands being of ectodermal origin, although some findings have suggested that there is also mesodermal input.
Polyostotic fibrous dysplasia is a form of fibrous dysplasia affecting more than one bone.
McCune-Albright syndrome includes polyostotic fibrous dysplasia as part of its presentation.
One treatment that has been used is bisphosphonates.
There is no treatment necessary for any type of COD. Diagnosis is important so that the treating doctor does not confuse it for another periapical disease such as rarefying osteitis or condensing osteitis. Incorrect diagnosis could lead to unnecessary root canal treatments. It can be diagnosed by radiographic appearance. Confirming the tooth is vital, as is noting the demographic (African American females).
Cemento-osseous dysplasia (COD) is a benign condition of the jaws that may arise from the fibroblasts of the periodontal ligaments. It is most common in African-American females. The three types are periapical cemental dysplasia (common in those of African descent), focal cemento-osseous dysplasia (Caucasians), and florid cemento-osseous dysplasia (African descent). Periapical occurs most commonly in the mandibular anterior teeth while focal appears predominantly in the mandibular posterior teeth and florid in both maxilla and mandible in multiple quadrants.
Hydrops-ectopic calcification-moth-eaten skeletal dysplasia is a defect in cholesterol biosynthesis. It is also known as Greenberg dysplasia. Greenberg characterized the condition in 1988.
It has been associated with the lamin B receptor.
The disorder is progressive, with the ultimate severity of symptoms often depending on age of onset. In severe cases amputation has been performed when conservative measures such as physical therapy and regional anesthetics have been ineffective.
Pacman dysplasia (alternatively known as epiphyseal stippling with osteoclastic hyperplasia) is a lethal autosomal recessive skeletal dysplasia. The dysplasia is present during fetal development.
In utero exposure to cocaine and other street drugs can lead to septo-optic dysplasia.
Can be caused by many things. A way to remember the causes is "PIG ON TAP"
Local factors-
- Occlusal Trauma
- Trauma
- Non-functional tooth
- Unopposed tooth (and impacted teeth, embedded teeth, teeth without antagonists)
Systemic factors-
- Idiopathic
- Pituitary Gigantism
- Paget's Disease
- Acromegaly
- Periapical granuloma
- Arthritis
- Calcinosis
- Rheumatic fever
It may be one of the complications of Paget's disease of bone in the form of generalized hypercementosis.
It may also be a compensatory mechanism in response to attrition to increase occlusal tooth height.
Pure hair-nail type ectodermal dysplasia is a genetic mutation in the "hair matrix and cuticle keratin KRTHB5 gene" that causes ectodermal dysplasia of hair and nail type. Manifestations of this disorder include onychodystrophy and severe hypotrichosis. It represents as an autosomal dominant trait.
Rare familial recurrence has been reported, suggesting at least one genetic form (HESX1). In addition to HESX1, mutations in OTX2, SOX2 and PAX6 have been implicated in de Morsier syndrome, but in most cases SOD is a sporadic birth defect of unknown cause and does not recur with subsequent pregnancies.
Monostotic fibrous dysplasia (or monostotic osteitis fibrosa) is a form of fibrous dysplasia where only one bone is involved. It comprises a majority of the cases of fibrous dysplasia.
A rare bone disorder characterized by benign bone growths which can cause very painful swellings and bone deformities and makes bone prone to fractures.
Intraepithelial neoplasia (IEN) is the development of a benign neoplasia or high-grade dysplasia in an epithelium. The exact dividing line between dysplasia and neoplasia has been very difficult to draw throughout the era of medical science. It varies between persons. In the localizations shown below, the term "intraepithelial neoplasia" is used to describe more accurately what was historically referred to as epithelial dysplasia. IEN is not cancer, but it is associated with higher risk for developing cancer in future. It is thus sometimes a precancerous condition.
Ghosal hematodiaphyseal dysplasia is a metabolic disorder.
It is associated with diaphyseal dysplasia and refractory anemia.
It is associated with a deficiency of Thromboxane-A synthase, which produces Thromboxane A2.
It was characterized in 1988.
Spondyloepimetaphyseal dysplasia is a genetic condition affecting the bones.
Types include:
- Spondyloepimetaphyseal dysplasia, Strudwick type
- Spondyloepiphyseal dysplasia congenita
- Spondyloepimetaphyseal dysplasia, Pakistani type
Acromicric dysplasia is an extremely rare inherited disorder characterized by abnormally short hands and feet, growth retardation and delayed bone maturation leading to short stature. Most cases have occurred randomly for no apparent reason (sporadically). However, autosomal dominant inheritance has not been ruled out.
According to the disease database, Acromicric dysplasia is synonymous with Geleophysic dysplasia
(or Geleophysic Dwarfism) and Focal mucopolysaccharidosis.
Hypohidrotic ectodermal dysplasia (also known as "anhidrotic ectodermal dysplasia", and "Christ-Siemens-Touraine syndrome") is one of about 150 types of ectodermal dysplasia in humans. Before birth, these disorders result in the abnormal development of structures including the skin, hair, nails, teeth, and sweat glands.
Spondyloepimetaphyseal dysplasia, Pakistani type is a form of spondyloepimetaphyseal dysplasia involving "PAPSS2" (also known as "ATPSK2"). The condition is rare.