Respiratory Manifestations in Pediatric Patients With Interleukin 6 Receptor Deficiency.

IF 2.7 3区医学 Q1 PEDIATRICS

Pediatric Pulmonology Pub Date : 2025-06-01 DOI:10.1002/ppul.71174

Noga Arwas, Eyal Kristal, Inbal Golan-Tripto, Aviv Goldbart, Guy Hazan, Amit Nahum, Shani Arotzker, Arnon Broides, Dvir Gatt

引用次数: 0

Abstract

Interleukin-6 receptor (IL-6R) deficiency is a rare immunodeficiency marked by impaired IL-6 signaling, affecting the inflammatory response and respiratory health. In this report, pediatric patients with IL-6R deficiency presented with a spectrum of respiratory symptoms, from mild to severe infections, but did not commonly develop early bronchiectasis. Due to the absence of typical inflammatory markers in lung infections, routine imaging is advised to monitor for potential complications, even in the absence of overt clinical signs.

查看原文本刊更多论文

白细胞介素6受体缺乏症患儿的呼吸表现

白细胞介素-6受体（IL-6R）缺乏是一种罕见的免疫缺陷，其特征是IL-6信号传导受损，影响炎症反应和呼吸系统健康。在本报告中，IL-6R缺乏的儿科患者表现为一系列呼吸道症状，从轻度到重度感染，但通常不会出现早期支气管扩张。由于肺部感染缺乏典型的炎症标志物，建议常规影像学监测潜在的并发症，即使没有明显的临床体征。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

求助全文

约1分钟内获得全文求助全文

来源期刊

Pediatric Pulmonology 医学-呼吸系统

CiteScore

6.00

自引率

12.90%

发文量

468

审稿时长

3-8 weeks

期刊介绍： Pediatric Pulmonology (PPUL) is the foremost global journal studying the respiratory system in disease and in health as it develops from intrauterine life though adolescence to adulthood. Combining explicit and informative analysis of clinical as well as basic scientific research, PPUL provides a look at the many facets of respiratory system disorders in infants and children, ranging from pathological anatomy, developmental issues, and pathophysiology to infectious disease, asthma, cystic fibrosis, and airborne toxins. Focused attention is given to the reporting of diagnostic and therapeutic methods for neonates, preschool children, and adolescents, the enduring effects of childhood respiratory diseases, and newly described infectious diseases. PPUL concentrates on subject matters of crucial interest to specialists preparing for the Pediatric Subspecialty Examinations in the United States and other countries. With its attentive coverage and extensive clinical data, this journal is a principle source for pediatricians in practice and in training and a must have for all pediatric pulmonologists.