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https://swslhd.intersearch.com.au/swslhdjspui/handle/1/12774| Title: | Idiopathic Laryngeal granulomatosis in a teenage girl responsive to Sirolimus |
| Authors: | Samtani, P. Li, M. Soma, M. Teng, A. Jacobson, I. Banks, C. Solinas, A. Williams, P. Gray, P. |
| SWSLHD Author: | Samtami, Prerna |
| Affiliates: | Department of Paediatrics, Children's Hospital at Westmead, Australia Department of Paediatrics, Fairfield Hospital, Australia Discipline of Paediatrics and Child Health, Faculty of Medicine & Health, University of New South Wales Sydney, Australia Department of Allergy and Immunology, Sydney Children's Hospital Randwick Australia, Australia Paediatric Department, Faculty of Medicine, Western Sydney University, Australia Department of Sleep Medicine, Sydney Children's Hospital, Randwick, Australia Department of Otolaryngology Head and Neck Surgery, Sydney Children's Hospital, Randwick, Australia School of Women's Health, Paediatric and Child Health, University of NSW, Australia Sydney Children's Hospital Network, Department of Infectious Diseases, Australia School of Public Health, Faculty of Medicine, The University of Sydney, Australia School of Women and Children's Health, UNSW, Australia Department of Anatomical Pathology, Prince Of Wales Hospital, Australia |
| Department: | Fairfield Hospital, Department of Paediatrics |
| Issue Date: | 2024 |
| Journal: | Otolaryngology Case Reports |
| Publisher: | Elsevier Inc. |
| Abstract: | Background: Laryngeal granulomatosis may occur in isolation or in conjunction with systemic diseases including ANCA-associated vasculitis and Sarcoidosis. There are a number of case reports of Idiopathic Laryngeal Granulomatosis occurring in teenagers, with little evidence as to what might constitute effective treatment modalities. Case: A previously well 13 year old girl presented with non-infectious necrotising & suppurative granulomatous cervical lymphadenopathy and went on to develop upper airway obstruction, with reduced exercise tolerance and obstructive sleep apnoea requiring CPAP. Laryngoscopy revealed swelling of the supraglottis and epiglottis, with biopsy showing granulomatous inflammation. No underlying diagnosis could be identified. She was relatively non-responsive to systemic or intraepiglottic steroids, debulking procedures, topical laser therapy or immunosuppression with mycophenolate (MMF) or Adalimumab. Sirolimus therapy led to a marked reduction in her supraglottic oedema and resolution of her symptoms including sleep apnoea. Conclusion: Sirolimus was extremely effective in treating protracted refractory Idiopathic Laryngeal granulomatosis, with reduced airway swelling and symptom resolution. � 2024 |
| URI: | https://swslhd.intersearch.com.au/swslhdjspui/handle/1/12774 |
| ISSN: | 24685488 (ISSN) |
| Digital object identifier: | 10.1016/j.xocr.2024.100599 |
| Appears in Collections: | Fairfield Hospital |
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