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Sjogren-Larsson Syndrome

An autosomal recessive neurocutaneous disorder characterized by severe ichthyosis MENTAL RETARDATION; SPASTIC PARAPLEGIA; and congenital ICHTHYOSIS. It is caused by mutation of gene encoding microsomal fatty ALDEHYDE DEHYDROGENASE leading to defect in fatty alcohol metabolism.
Also Known As:
Congenital Icthyosis Mental Retardation Spasticity Syndrome; FALDH Deficiency; Fatty Alcohol:NAD+ Oxidoreductase Deficiency; Fatty Aldehyde Dehydrogenase Deficiency; Fatty Aldehyde Dehydrogenase Deficiency Disease; Ichthyosis Oligophrenia Syndrome; Ichthyosis, Spastic Neurologic Disorder, and Oligophrenia; Sjögren-Larsson Syndrome; Sjogren Larsson Syndrome
Networked: 142 relevant articles (2 outcomes, 7 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Ichthyosis (Xeroderma)
2. Leukoencephalopathies
3. Pruritus (Itching)
4. Neoplasms (Cancer)
5. Intellectual Disability (Idiocy)

Experts

1. Rizzo, William B: 20 articles (03/2022 - 08/2004)
2. Willemsen, Michèl A A P: 8 articles (11/2020 - 04/2004)
3. Wanders, Ronald J A: 8 articles (07/2012 - 05/2004)
4. Rotteveel, J J: 6 articles (12/2001 - 02/2000)
5. Willemsen, M A: 6 articles (12/2001 - 02/2000)
6. Theelen, Thomas: 5 articles (05/2022 - 05/2010)
7. Keller, Markus A: 5 articles (01/2021 - 06/2010)
8. Carney, Gael: 5 articles (11/2011 - 08/2004)
9. Mayatepek, E: 5 articles (12/2001 - 02/2000)
10. Cruysberg, Johannes R M: 4 articles (05/2022 - 05/2010)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Sjogren-Larsson Syndrome:
1. AcitretinFDA Link
2. Fatty AlcoholsIBA
3. Fatty Acids (Saturated Fatty Acids)IBA
4. Protons (Proton)IBA
5. Amino AcidsFDA Link
6. zileuton (Zyflo)FDA Link
7. Triglycerides (Triacylglycerol)IBA
8. Phospholipids (Phosphatides)FDA LinkGeneric
9. Genetic Markers (Genetic Marker)IBA
10. Nonesterified Fatty Acids (NEFA)IBA

Therapies and Procedures

1. Therapeutics
2. Anesthesia
3. Catheters
4. General Anesthesia