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Zellweger Syndrome (Zellweger's Syndrome)

An autosomal recessive disorder due to defects in PEROXISOME biogenesis which involves more than 13 genes encoding peroxin proteins of the peroxisomal membrane and matrix. Zellweger syndrome is typically seen in the neonatal period with features such as dysmorphic skull; MUSCLE HYPOTONIA; SENSORINEURAL HEARING LOSS; visual compromise; SEIZURES; progressive degeneration of the KIDNEYS and the LIVER. Zellweger-like syndrome refers to phenotypes resembling the neonatal Zellweger syndrome but seen in children or adults with apparently intact peroxisome biogenesis.
Also Known As:
Zellweger's Syndrome; Cerebro-Hepato-Renal Syndrome; PBD, ZSS; Peroxisome Biogenesis Disorders, Zellweger Syndrome Spectrum; Zellweger Disease; Zellweger Spectrum; Zellweger Syndrome Spectrum; Cerebro Hepato Renal Syndrome; Spectrum, Zellweger; Zellweger Like Syndrome; Cerebrohepatorenal Syndrome; Zellweger-Like Syndrome
Networked: 511 relevant articles (7 outcomes, 33 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Liver Diseases (Liver Disease)
2. Refsum Disease (Refsum's Disease)
3. Peroxisomal Disorders (Peroxisomal Disorder)
4. Adrenoleukodystrophy (Adrenoleukodystrophy, X-Linked)
5. Cerebrotendinous Xanthomatosis

Experts

1. Wanders, Ronald J A: 18 articles (01/2021 - 01/2002)
2. Waterham, Hans R: 15 articles (01/2021 - 01/2002)
3. Ferdinandusse, Sacha: 9 articles (01/2021 - 07/2004)
4. Shimozawa, Nobuyuki: 9 articles (01/2021 - 10/2003)
5. Heubi, James E: 8 articles (01/2020 - 01/2003)
6. Poll-The, Bwee Tien: 8 articles (01/2019 - 02/2012)
7. Klouwer, Femke C C: 7 articles (01/2021 - 08/2016)
8. Setchell, Kenneth D R: 7 articles (01/2020 - 01/2003)
9. Bove, Kevin E: 6 articles (11/2018 - 01/2003)
10. Braverman, Nancy: 5 articles (01/2022 - 11/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Zellweger Syndrome:
1. Fatty Acids (Saturated Fatty Acids)IBA
2. AcidsIBA
3. Bile Acids and Salts (Bile Acids)IBA
4. EnzymesIBA
5. Cholic AcidIBA
6. PlasmalogensIBA
7. LipidsIBA
8. pipecolic acidIBA
9. Ether (Diethyl Ether)IBA
10. Phytanic AcidIBA

Therapies and Procedures

1. Therapeutics
2. Nursing Care
3. Lasers (Laser)
4. Liver Transplantation
5. Intravenous Administration