| CASE REPORT | |
| Year : 2016 | Volume : 9 | Issue : 4 | Page : 534-536 | |
|
Mental health perspectives of Hunter syndrome: Case reports of two biological siblings Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0975-2870.186068 |
|
| Hunter syndrome is a rare X-linked recessive disorder caused by deficiency of the lysosomal enzyme iduronate-2-sulphatase, leading to progressive accumulation of a substance called glycosaminoglycans in nearly all cell types, tissues, and organs. Hunter syndrome presents with facial dysmorphism, airway diseases, skeletal defects, cardiomyopathies, and neuropsychiatric manifestations. Mental subnormality is a cardinal feature in Hunter syndrome. This is a progressive cognitive decline that is not amenable to enzyme replacement therapy. Due to progressive cognitive decline, training the children to improve the adaptive functioning is a challenge that creates immense stress for the caregivers. Patients with Hunter syndrome should undergo serial assessment of intellectual ability and may be trained accordingly. | |
|
|
|
| [FULL TEXT] [PDF]* | |
 |
|
 Next article
|
|
Previous article
|
|
Table of Contents
|
|
Similar in PUBMED |
| Search Pubmed for |
| Search in Google Scholar for |
| Related articles |
|
Citation Manager
|
|
Access Statistics
|
|
Reader Comments
|
|
Email Alert * |
|
Add to My List * |
| * Requires registration (Free) |
| Article Access Statistics | |
| Viewed | 2674 |
| Printed | 79 |
| Emailed | 0 |
| PDF Downloaded | 160 |
| Comments | [Add] |
