Immunoglobulin G4-related disease (IgG4-RD) is a lymphoproliferative disease which is described almost exclusively in adults. There are only a few pediatric patients who have been observed with this disorder. Here, we describe a rare case of IgG4-RD in a 17-year-old girl with a single manifestation-tracheal stenosis without previous intubation or other inciting event. She had mixed dyspnea and noisy and weakened breathing. Immunoproliferative hyper-IgG4 disease was diagnosed, based on elevated serum IgG4 and histological findings. Until now we have chosen to treat the girl only with corticosteroids with a good response so far. The general condition as well as the respiratory function are regularly monitored. The tracheal involvement of IgG4-RD is uncommon. Nonetheless, it is a manifestation that should be included in the differential diagnosis of tracheal stenosis.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8307327 | PMC |
| http://dx.doi.org/10.3390/children8070589 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Macrophage STING signaling promotes fibrosis in benign airway stenosis via an IL6-STAT3 pathway.
Nat Commun
January 2025
Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Naval Medical University, Shanghai, China.
Acute and chronic inflammation are important pathologies of benign airway stenosis (BAS) fibrosis, which is a frequent complication of critically ill patients. cGAS-STING signalling has an important role in inflammation and fibrosis, yet the function of STING in BAS remains unclear. Here we demonstrate using scRNA sequencing that cGAS‒STING signalling is involved in BAS, which is accompanied by increased dsDNA, expression and activation of STING.
View Article and Find Full Text PDFTherapeutic Bronchoscopic Interventions for Non Foreign-Body Removal Indications in Children.
Pediatr Pulmonol
December 2024
All India Institute of Medical Sciences, New Delhi, India.
Background: The indications for pediatric airway endoscopy are expanding and a variety of therapeutic interventions are feasible for central airway obstruction (CAO) and other central airway pathologies, apart from foreign body removal.
Methods: In this retrospective chart review from four centers, we describe the indications, procedures, outcomes, and complications of therapeutic bronchoscopic interventions in children for non foreign-body removal indications.
Results: A total of 72 children (mean age:140 [60.
Unmasking Subglottic Stenosis in an Asthmatic Emergency Needing Front-of-Neck Access: A Case Report.
Cureus
November 2024
Pain Management, Singapore General Hospital, Singapore, SGP.
Subglottic stenosis poses a rare but life-threatening risk for difficult tracheal intubation. Here, we report a unique case of undiagnosed subglottic stenosis discovered during emergency intubation of an 80-year-old woman with type 2 respiratory failure from infective exacerbation of asthma. A small calibre size 5.
View Article and Find Full Text PDFTwenty years of anastomotic stenosis combined with tracheocolonic fistula after colon replacement esophagectomy: a case report.
Front Oncol
December 2024
Lanshan District People's Hospital, Department of Thoracic Surgery, Linyi, Shandong, China.
Esophageal stricture is the most common and disabling complication of esophageal injury caused by ingestion of corrosive substances. In our case, the patient developed esophageal stenosis due to ingestion of strong alkaline substances and underwent colon replacement surgery after repeated failed dilation treatments. After surgery, anastomotic stenosis and tracheocolonic fistula occurred successively, and the entire diagnosis and treatment cycle of this disease lasted for more than 20 years.
View Article and Find Full Text PDF[Application of spiral tracheoplasty in thyroid cancer with tracheal invasion].
Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi
December 2024
Department of Otorhinolaryngology Head and Neck Surgery, the Second Xiangya Hospital, Central South University, Changsha410011, China.
To explore the application of spiral tracheoplasty in the repair of large tracheal defects after the resection of trachea invaded by thyroid cancer. A retrospective analysis was performed on the clinical data of 11 patients, including 4 males and 7 females, aged from 36 to 67 years old, with large tracheal defects after tracheal resection due to thyroid papillary carcinoma invading the trachea in the Department of Otorhinolaryngology Head and Neck Surgery, Second Xiangya Hospital, Central South University from January 2019 to January 2022. The range of tracheal defects, time of tracheal reconstruction, postoperative complications and airway were recorded, and the patients were followed up for more than 24 months.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!