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Case Report

A Case of Tracheal Stenosis as an Isolated Form of Immunoproliferative Hyper-IgG4 Disease in a 17-Year-Old Girl

1
Department of Pulmonary Diseases, Multiprofile Hospital for Active Treatment of Pulmonary Diseases “St. Sofia”, Medical University–Sofia, 1431 Sofia, Bulgaria
2
Department of Pediatric Thoracic Surgery, Pediatric Surgery Clinic, University Multiprofile Hospital for Active Treatment and Emergency Medicine “N. I. Pirogov”, 1606 Sofia, Bulgaria
3
Department of Pathology, University Hospital ‘’Tsaritsa Uoanna–ISUL’’, 1527 Sofia, Bulgaria
4
Department of Neurosurgery, University Hospital “St. Ivan Rilski”, Medical University–Sofia, 1431 Sofia, Bulgaria
*
Author to whom correspondence should be addressed.
Academic Editor: Joaquim M. B. Pinheiro
Children 2021, 8(7), 589; https://doi.org/10.3390/children8070589
Received: 29 May 2021 / Revised: 29 June 2021 / Accepted: 9 July 2021 / Published: 12 July 2021
(This article belongs to the Special Issue Childhood Innate Immunity and Infectious Diseases)
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. View Full-Text
Keywords: tracheal stenosis; children; IgG4 immunoproliferative disease tracheal stenosis; children; IgG4 immunoproliferative disease
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MDPI and ACS Style

Gabrovska, N.; Velizarova, S.; Spasova, A.; Kostadinov, D.; Yanev, N.; Shivachev, H.; Rangelov, E.; Pahnev, Y.; Antonova, Z.; Kartulev, N.; Terziev, I.; Gabrovski, K. A Case of Tracheal Stenosis as an Isolated Form of Immunoproliferative Hyper-IgG4 Disease in a 17-Year-Old Girl. Children 2021, 8, 589. https://doi.org/10.3390/children8070589

AMA Style

Gabrovska N, Velizarova S, Spasova A, Kostadinov D, Yanev N, Shivachev H, Rangelov E, Pahnev Y, Antonova Z, Kartulev N, Terziev I, Gabrovski K. A Case of Tracheal Stenosis as an Isolated Form of Immunoproliferative Hyper-IgG4 Disease in a 17-Year-Old Girl. Children. 2021; 8(7):589. https://doi.org/10.3390/children8070589

Chicago/Turabian Style

Gabrovska, Natalia, Svetlana Velizarova, Albena Spasova, Dimitar Kostadinov, Nikolay Yanev, Hristo Shivachev, Edmond Rangelov, Yanko Pahnev, Zdravka Antonova, Nikola Kartulev, Ivan Terziev, and Kaloyan Gabrovski. 2021. "A Case of Tracheal Stenosis as an Isolated Form of Immunoproliferative Hyper-IgG4 Disease in a 17-Year-Old Girl" Children 8, no. 7: 589. https://doi.org/10.3390/children8070589

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