TS-HDS and FGFR3 antibodies in small fiber neuropathy and Dysautonomia.

INTRODUCTION: The specificity of trisulfated heparin disaccharide/fibroblast growth factor receptor 3 (TS-HDS/FGFR3) antibodies in the diagnosis of autoimmune small fiber neuropathy (SFN) is unclear. METHODS: This was a retrospective study of patients evaluated for SFN and dysautonomia in the Brigham and Women's Faulkner Hospital Autonomic Laboratory in 2019-2020. Associations were assessed between TS-HDS/FGFR3 antibodies and SFN markers, including epidermal nerve fiber density (ENFD), sweat gland nerve fiber density (SGNFD), and autonomic dysfunction assessed by Valsalva maneuver, deep breathing, sudomotor, and tilt testing. RESULTS: Of 322 patients; 28% had elevated anti-TS-HDS, 17% had elevated anti-FGFR3, 96% had autonomic dysfunction, 71% had abnormal ENFD, and 49% had abnormal SGNFD. TS-HDS/FGFR3 antibodies were present in patients with autonomic dysfunction irrespective of whether they had normal or abnormal skin biopsies unless ENFD/SGNFD were combined for anti-FGFR3 seropositivity. DISCUSSION: TS-HDS/FGFR3 antibodies are present in patients with evidence of autonomic dysfunction. Further studies are needed to document the clinical value of these antibodies in assessment of immune mediated dysautonomia.

Efectividad del uso de la bolsa adiposa de bichat para la reconstrucción de defectos en el paladar: reporte de un caso / Effectiveness of the use of the bichats fat pad for the reconstructionof defects in the palate. report a case

La bolsa adiposa de Bichat está constituida por un cuerpo y tres extensiones de tejido adiposo, se localiza dentro del espacio bucal y seextiende siguiendo el borde anterosuperior del músculo masetero. En la actualidad su uso como colgajo pediculado ha demostrado excelentesresultados en el tratamiento de reconstrucción de defectos postumorales de tejido blando en el paladar. Lo anterior se debe a su gran aporte vascular, composición histológica y a la presencia de células madre en tejido adiposo que fomentan una metaplasia del tejido, convirtiéndose en tejido fibroso y superfi cialmente con epitelio en tan sólo cinco semanas. La técnica de abordaje y reposicionamiento del colgajo pediculado en paladar fue modifi cada con la extirpación de la tuberosidad del maxilar para corregir el defecto ocasionado por un adenoma pleomorfo en el paladar. Se destacan las características y cualidades de la bolsa adiposa de Bichat para su uso en reconstrucción de defectos tumorales.

Bichat’s buccal fat pad is constituted by a body and three extensions ofadipose tissue within the buccal space and extending to the anteriorsuperiorborder of the masseter muscle. To this days, the use of thebuccal fat pad as a pedicled graft has shown excellent results onoral post tumoral reconstruction treatment. This is due to its greatvascularity, histological composition and perhaps to the presenceof stem cells that promotes a metaplasia, turning adipose tissue intofi brous and superfi cially epithelized tissue within fi ve week aftersurgery. Surgical approach and repositioning technique of the pedicledgraft was modifi ed, extirpating part of the maxillary tuberosity, topreserve vascularity and cover up a hard-soft tissue defect caused by apleomorphic adenoma on a patient’s palate. Buccal fat pad’s qualitiesand characteristics are taken into consideration to demonstrate theeff ectiveness on its surgical reconstructive uses.