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Farber Lipogranulomatosis

A sphingolipidosis subtype that is characterized by the histological appearance of granulomatous deposits in tissues. It results from the accumulation of CERAMIDES in various tissues due to an inherited deficiency of ACID CERAMIDASE.
Also Known As:
Acid Ceramidase Deficiency; Ceramidase Deficiency; Farber Disease; Farber's Disease; N-Laurylsphingosine Deacylase Deficiency; Acid Ceramidase Deficiencies; Ceramidase Deficiencies; Ceramidase Deficiency, Acid; Deficiencies, Ceramidase; Deficiencies, N-Laurylsphingosine Deacylase; Deficiency, Acid Ceramidase; Deficiency, Ceramidase; Deficiency, N-Laurylsphingosine Deacylase; Disease, Farber's; Diseases, Farber's; Farber's Diseases; Farbers Disease; Lipogranulomatosis, Farber; N Laurylsphingosine Deacylase Deficiency; N-Laurylsphingosine Deacylase Deficiencies
Networked: 115 relevant articles (2 outcomes, 9 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Neoplasms (Cancer)
2. Lysosomal Storage Diseases (Lysosomal Storage Disease)
3. Farber Lipogranulomatosis
4. Globoid Cell Leukodystrophy (Krabbe Disease)
5. Gaucher Disease (Gaucher's Disease)

Experts

1. Levade, Thierry: 13 articles (01/2020 - 11/2008)
2. Medin, Jeffrey A: 12 articles (01/2020 - 11/2008)
3. Schuchman, Edward H: 11 articles (06/2017 - 02/2002)
4. Dworski, Shaalee: 7 articles (01/2020 - 06/2013)
5. He, Xingxuan: 6 articles (06/2017 - 02/2002)
6. Ehlert, Karoline: 5 articles (01/2020 - 01/2004)
7. Yu, Fabian P S: 4 articles (10/2019 - 01/2018)
8. Carpentier, Stéphane: 4 articles (06/2013 - 11/2008)
9. Sandhoff, Konrad: 4 articles (01/2012 - 02/2002)
10. Park, Jae-Ho: 4 articles (05/2007 - 02/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Farber Lipogranulomatosis:
1. Acid CeramidaseIBA
2. CeramidesIBA
3. CeramidasesIBA
4. SphingolipidsIBA
5. SphingomyelinsIBA
6. Proteins (Proteins, Gene)FDA Link
7. AcidsIBA
8. Sulfoglycosphingolipids (Sulfatides)IBA
9. Sphingomyelin Phosphodiesterase (Sphingomyelinase)IBA
10. N-lauroylsphingosineIBA

Therapies and Procedures

1. Therapeutics
2. Transplantation
3. Enzyme Replacement Therapy
4. Hematopoietic Stem Cell Transplantation
5. Stem Cell Transplantation