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Deep Learning Technology: Sebastian Arnold, Betty van Aken, Paul Grundmann, Felix A. Gers and Alexander Löser. Learning Contextualized Document Representations for Healthcare Answer Retrieval. The Web Conference 2020 (WWW'20)
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Unerupted microdonts may require surgical removal to prevent the formation of cysts. Erupted microdonts, peg laterals especially, may cause cosmetic concern. Such teeth may be restored to resemble normal sized teeth, typically with composite build ups or crowns. Orthodontics may be required in severe cases to close gaps between the teeth.
Teeth affected by macrodontia are either contoured, aligned or extracted. Contouring involves shaving the tooth down to change shape and size. However, the result is minimal change as this could be dangerous for the dentin and dental pulp. Aligning involves the use of braces to straighten, align, and make space for larger teeth to grow. When extracted, they are replaced with an implant or bridge. This is done in cases in which the patient suffers from pain that cannot be treated by other methods.
Females are affected more than males, and the condition occurs in permanent (adult) teeth more than deciduous (baby teeth or milk teeth).
Macrodontia (or megadontia or megalodontia) is a type of localized gigantism in which teeth are larger than normal for the particular type(s) of teeth involved. The three types of macrodontia are true generalized macrodontia, relative generalized macrodontia, and macrodontia of a single tooth. True generalized macrodontia is rare. Macrodontia of a single tooth is more common. Some kind of macrodontia in the permanent dentition occurs in 1.1% of the total population. It should not be confused with taurodontism (bull teeth), fusion (double tooth) or the jaws being relatively small, giving the appearance of macrodontia.
While Larsen syndrome can be lethal if untreated, the prognosis is relatively good if individuals are treated with orthopedic surgery, physical therapy, and other procedures used to treat the symptoms linked with Larsen syndrome.
Treatment for Larsen syndrome varies according to the symptoms of the individual. Orthopedic surgery can be performed to correct the serious joint defects associated with Larsen syndrome. Reconstructive surgery can be used to treat the facial abnormalities. Cervical kyphosis can be very dangerous to an individual because it can cause the vertebrae to disturb the spinal cord. Posterior cervical arthrodesis has been performed on patients with cervical kyphosis, and the results have been successful Propranolol has been used to treat some of the cardiac defects associated with Marfan's syndrome, so the drug also has been suggested to treat cardiac defects associated with Larsen syndrome.
This can be done by annual evaluations by multidiciplinary team involving otolaryngologist, clinical geneticist, a pediatrician, the expertise of an educator of the deaf, a neurologist is appropriate.
Presence of inner ear abnormalities lead to Delayed gross development of child because of balance impairment and profound deafness which increases the risk of trauma and accidents.
- Incidence of accidents can be decreased by using visual or vibrotactile alarm systems in homes as well as in schools.
- Anticipatory education of parents, health providers and educational programs about hazards can help.
Unlike other autoinflammatory disorders, patients with CANDLE do not respond to IL-1 inhibition treatment in order to stop the autoinflammatory response altogether. This suggests that the condition also involves IFN dysregulation.
Sensenbrenner syndrome (OMIM #218330) is a rare (less than 20 cases reported by 2010) multisystem disease first described in 1975. It is inherited in an autosomal recessive fashion, and a number of genes appear to be responsible. Three genes responsible have been identified: intraflagellar transport (IFT)122 (WDR10), IFT43 — a subunit of the IFT complex A machinery of primary cilia, and WDR35 (IFT121: TULP4)
It is also known as Sensenbrenner–Dorst–Owens syndrome, Levin Syndrome I and cranioectodermal dysplasia (CED)
These are pleomorphic and include
- dolichocephaly (with or without sagittal suture synostosis)
- microcephaly
- pre- and postnatal growth retardation
- brachydactyly
- narrow thorax
- rhizomelic dwarfism
- epicanthal folds
- hypodontia and/or microdontia
- sparse, slow-growing, hyperpigmented, fine hair
- nail dysplasia
- hypohydrosis
- chronic renal failure
- heart defects
- liver fibrosis
- visual deficits
- photophobia
- hypoplasia of the posterior corpus callosum
- aberrant calcium homeostasis
Electroretinography shows gross abnormalities.
Two fetuses of 19 and 23 weeks gestation have also been reported. They showed acromesomelic shortening, craniofacial characteristics with absence of craniosynostosis, small kidneys with tubular and glomerular microscopic cysts, persistent ductal plate with portal fibrosis in the liver, small adrenals, an enlarged cisterna magna and a posterior fossa cyst.
Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated Temperature (CANDLE) syndrome is an autosomal recessive disorder that presents itself via various autoinflammatory responses throughout the body, multiple types of skin lesions, and recurrent long-term fever symptoms. The current known cause for the disorder is a mutation in the PSMB8 gene or mutations in other closely related genes. The syndrome was first named and classified in March 2010 after four patients were reviewed with similar symptoms. There have been approximately 30 cases ever reported in the scientific literature, as of 2015.