Hunter' syndrome. Ultrastructural features in young children.
Ultrastructural abnormalities in two stepbrothers with Hunter's syndrome, ages 1 and 4 years, were found in cortical neurons, neurons of the myenteric plexus, and skin. Inclusions containing little or no electron-dense material were noted in most tissues, and lamellar figures were restricted to cortical neurons and neurons of the myenteric plexus. These changes correlate with those described in tissues obtained at post mortem.
D002540:Cerebral Cortex / Q000648:ultrastructure; D002675:Child, Preschool; D004817:Epidermis / Q000648:ultrastructure; D006801:Humans; D002479:Inclusion Bodies / Q000648:ultrastructure; D007223:Infant; D008214:Lymphocytes / Q000648:ultrastructure; D008297:Male; D008854:Microscopy, Electron; D016532:Mucopolysaccharidosis II / Q000473:pathology*; D009186:Myelin Sheath / Q000648:ultrastructure; D009197:Myenteric Plexus / Q000648:ultrastructure; D009474:Neurons / Q000648:ultrastructure*; D012867:Skin / Q000648:ultrastructure; D014617:Vacuoles / Q000648:ultrastructure