Lyngstadaas syndrome, also known as severe dental aberrations in familial steroid dehydrogenase deficiency, is a rare autosomal recessive liver disease involving an enzyme (steroid
dehydrogenase
A dehydrogenase is an enzyme belonging to the group of oxidoreductases that oxidizes a substrate by reducing an electron acceptor, usually NAD+/NADP+ or a flavin coenzyme such as FAD or FMN. Like all catalysts, they catalyze reverse as well as ...
) deficiency and dental anomalies. The disease is named after the Norwegian professor
Ståle Petter Lyngstadaas
Ståle Petter Lyngstadaas is a Norwegian professor and researcher with a focus on biomaterials and bone regeneration. Since 2013, he is the Vice-Dean of Research at Faculty of Dentistry, Oslo University
The University of Oslo ( no, Universitet ...
.
Cause
Lyngstadaas syndrome is an autosomal recessive liver disease.
Diagnosis
Management
Epidemiology
Office of Rare Diseases
The Office of Rare Diseases Research is a division of the National Center for Advancing Translational Sciences (NCATS) that oversees the Rare Diseases Clinical Research Network
The Rare Diseases Clinical Research Network (RDCRN) is an initiative ...
listed Lyngstadaas syndrome as a "rare disease". This means that Lyngstadaas syndrome, or a subtype of Lyngstadaas syndrome, affects less than 200,000 people in the US population.
Orphanet
Orphanet is a knowledge base dedicated to rare diseases as well as corresponding diagnosis, orphan drugs, clinical trials and expert networks
The website is managed by a network of academic establishments from 40 countries, led by Inserm.
It c ...
, a consortium of European partners, currently defines a condition rare when if affects 1 person per 2,000. They list Lyngstadaas syndrome as a "rare disease".
See also
*
Rare disease
A rare disease is any disease that affects a small percentage of the population. In some parts of the world, an orphan disease is a rare disease whose rarity means there is a lack of a market large enough to gain support and resources for discov ...
References
External links
{{Medical resources
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, ICD10 = K76.8
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, MeshID = C537490
, Orphanet = 3196
Rare syndromes
Syndromes affecting teeth
Rare genetic syndromes