Lyngstadaas syndrome

Lyngstadaas Syndrome, also known as severe dental aberrations in familial steroid dehydrogenase deficiency [1], is a rare autosomal recessive liver disease involving an enzyme (steroid dehydrogenase) deficiency and dental anomalies.[2][3] The disease is named after the Norwegian professor Ståle Petter Lyngstadaas.

Lyngstadaas syndrome
Other names	Steroid dehydrogenase deficiency-dental anomalies syndrome
This condition is inherited in an autosomal recessive manner

Cause

Lyngstadaas syndrome is an autosomal recessive liver disease [4]

Epidemiology

Office of Rare Diseases 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, 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

References

1. "Steroid dehydrogenase deficiency dental anomalies | Disease | Living With | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Retrieved 2016-03-01.
2. Steroid dehydrogenase deficiency - dental anomalies Symptoms, Diagnosis, Treatments and Causes - RightDiagnosis.com
3. RESERVED, INSERM US14 -- ALL RIGHTS. "Orphanet: Lyngstadaas syndrome". www.orpha.net. Retrieved 2016-03-01.
4. RESERVED, INSERM US14-- ALL RIGHTS. "Orphanet: Steroid dehydrogenase deficiency dental anomalies syndrome". www.orpha.net. Retrieved 26 September 2019.

External links

Classification	D ICD-10: K76.8 MeSH: C537490
External resources	Orphanet: 3196