Zellweger spectrum disorders
ICD-10 Code
E71.510
Zellweger spectrum disorders
Definition
The most severe variant seen in the peroxisome biogenesis disorders that is characterized by neuronal migration defects in the brain, dysmorphic craniofacial features, profound hypotonia, neonatal seizures, and liver dysfunction.
Also known as cerebrohepatorenal syndrome, Zellweger spectrum disorders, Zellweger syndrome, ZS, ZWS — per MONDO
Also identified as
- DOID 905 per MONDO
- ICD10CM E71.510 per MONDO
- MESH D015211 per MONDO
- NCIT C85239 per MONDO
- Orphanet 772 per MONDO
- Orphanet 912 per MONDO
- SCTID 88469006 per MONDO
- UMLS C0043459 per MONDO
Affected Organs
| Organ | Involvement | Source |
|---|---|---|
| Nervous system | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Nervous system | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |