Treatment-responsive pandysautonomia in an adolescent with ganglionic α3-AChR antibodies.
Autoimmune autonomic ganglionopathy (AAG) is a rare disorder that presents with pandysautonomia typically in middle age and elderly patients. AAG is typically associated with serum autoantibodies that bind to the alpha-3 subunit of the ganglionic acetylcholine receptor (α3-AChR Ab). We report a 13 y...
| Main Authors: | , , , , , |
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| Format: | Journal article |
| Language: | English |
| Published: |
2012
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| _version_ | 1826268458145284096 |
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| author | Dale, R Lang, B Brilot, F Polfrit, Y Smith, G Wong, M |
| author_facet | Dale, R Lang, B Brilot, F Polfrit, Y Smith, G Wong, M |
| author_sort | Dale, R |
| collection | OXFORD |
| description | Autoimmune autonomic ganglionopathy (AAG) is a rare disorder that presents with pandysautonomia typically in middle age and elderly patients. AAG is typically associated with serum autoantibodies that bind to the alpha-3 subunit of the ganglionic acetylcholine receptor (α3-AChR Ab). We report a 13 year old girl who presented with gut pseudo-obstruction, bladder dysfunction and dilated pupils unresponsive to pilocarpine. She had positive α3-AChR Ab plus other autoantibodies suggesting an autoimmune diathesis. Our patient was initially resistant to steroid therapy but responded to the addition of azathioprine resulting in a near complete clinical remission. We conclude that pandysautonomia associated with α3-AChR Ab can occur in children and has multi-organ involvement. |
| first_indexed | 2024-03-06T21:09:57Z |
| format | Journal article |
| id | oxford-uuid:3dcd93d9-d57e-45e8-9d4a-a84b7aee2064 |
| institution | University of Oxford |
| language | English |
| last_indexed | 2024-03-06T21:09:57Z |
| publishDate | 2012 |
| record_format | dspace |
| spelling | oxford-uuid:3dcd93d9-d57e-45e8-9d4a-a84b7aee20642022-03-26T14:21:39ZTreatment-responsive pandysautonomia in an adolescent with ganglionic α3-AChR antibodies.Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:3dcd93d9-d57e-45e8-9d4a-a84b7aee2064EnglishSymplectic Elements at Oxford2012Dale, RLang, BBrilot, FPolfrit, YSmith, GWong, MAutoimmune autonomic ganglionopathy (AAG) is a rare disorder that presents with pandysautonomia typically in middle age and elderly patients. AAG is typically associated with serum autoantibodies that bind to the alpha-3 subunit of the ganglionic acetylcholine receptor (α3-AChR Ab). We report a 13 year old girl who presented with gut pseudo-obstruction, bladder dysfunction and dilated pupils unresponsive to pilocarpine. She had positive α3-AChR Ab plus other autoantibodies suggesting an autoimmune diathesis. Our patient was initially resistant to steroid therapy but responded to the addition of azathioprine resulting in a near complete clinical remission. We conclude that pandysautonomia associated with α3-AChR Ab can occur in children and has multi-organ involvement. |
| spellingShingle | Dale, R Lang, B Brilot, F Polfrit, Y Smith, G Wong, M Treatment-responsive pandysautonomia in an adolescent with ganglionic α3-AChR antibodies. |
| title | Treatment-responsive pandysautonomia in an adolescent with ganglionic α3-AChR antibodies. |
| title_full | Treatment-responsive pandysautonomia in an adolescent with ganglionic α3-AChR antibodies. |
| title_fullStr | Treatment-responsive pandysautonomia in an adolescent with ganglionic α3-AChR antibodies. |
| title_full_unstemmed | Treatment-responsive pandysautonomia in an adolescent with ganglionic α3-AChR antibodies. |
| title_short | Treatment-responsive pandysautonomia in an adolescent with ganglionic α3-AChR antibodies. |
| title_sort | treatment responsive pandysautonomia in an adolescent with ganglionic α3 achr antibodies |
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