Key Laboratory of Major Disease in Children, Ministry of Education, Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Laboratory of Tumor Immunology, Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Acta Otolaryngol. 2020 Sep;140(9):779-784. doi: 10.1080/00016489.2020.1767804. Epub 2020 Jun 3.
Aggressive juvenile-onset recurrent respiratory papillomatosis (JORRP) threatens patient lives if not receives immediate surgical intervene. Even with surgical intervene, complete remission of this disease is not approachable. Therefore, understanding the factors relevant to disease severity and prognosis will do help to the treatment strategy and health management of this disease. This study aimed to explore the clinic, laboratory and socioeconomic characteristics of patients and evaluate the risk factors for aggressive JORRP. The information of clinical and socioeconomic status of the patients was reviewed and its association with disease severity was analyzed. Papilloma from JORRP patients undergone surgeries was used to determine HPV subtypes by real-time PCR. The profiles of mRNA expression in the papilloma were assessed using microarray. Age at diagnosis and socioeconomic status were shown to associate with the severity of JORRP. There was no differential severity considering different HPV subtype. The mRNA expression of nucleotide binding oligomerization domain receptor protein 3 (NLRP3) and Gasdermin B (GSDMB) was reduced in papillomas. A younger age at diagnosis and low socioeconomic status were associated with the severity of JORRP. mRNA expression of NLRP3 and GSDMB in the papillomas of JORRP patients was significantly reduced. JORRP: Juvenile-onset recurrent respiratory papillomatosis; RRP: Recurrent respiratory papillomatosis; OSAS: Obstructive sleep apnea syndrome; NLRP3: Nucleotide binding oligomerization domain receptor protein 3; GSDMB: Gasdermin B.
侵袭性早发性复发性呼吸道乳头瘤病（JORRP）如果不及时接受手术干预，会威胁到患者的生命。即使进行了手术干预，这种疾病也无法完全缓解。因此，了解与疾病严重程度和预后相关的因素将有助于制定这种疾病的治疗策略和健康管理方案。本研究旨在探讨患者的临床、实验室和社会经济学特征，并评估侵袭性 JORRP 的危险因素。回顾了患者的临床和社会经济学状况信息，并分析了其与疾病严重程度的关系。通过实时 PCR 确定接受手术的 JORRP 患者的乳头瘤中的 HPV 亚型。使用微阵列评估乳头瘤中 mRNA 表达谱。诊断时的年龄和社会经济状况与 JORRP 的严重程度相关。不同 HPV 亚型的严重程度没有差异。核苷酸结合寡聚化结构域受体蛋白 3（NLRP3）和 Gasdermin B（GSDMB）的 mRNA 表达在乳头瘤中减少。诊断时年龄较小和社会经济地位较低与 JORRP 的严重程度相关。JORRP 患者的乳头瘤中 NLRP3 和 GSDMB 的 mRNA 表达明显降低。JORRP：早发性复发性呼吸道乳头瘤病；RRP：复发性呼吸道乳头瘤病；OSAS：阻塞性睡眠呼吸暂停综合征；NLRP3：核苷酸结合寡聚化结构域受体蛋白 3；GSDMB：Gasdermin B。
