keyword
https://read.qxmd.com/read/38253347/neuronal-ceroid-lipofuscinosis-type-11-diagnosed-patient-with-bi-allelic-variants-in-gnr-gene-case-report-and-review-of-literature
#21
İlknur Sürücü Kara, Engin Köse, Büşranur Çavdarlı, Fatma Tuba Eminoğlu
OBJECTIVES: Neuronal ceroid lipofuscinosis type 11 (NCL11) is a rare disease that presents with progressive cognitive decline, epilepsy, visual impairment, retinal atrophy, cerebellar ataxia and cerebellar atrophy. We present herein a case of NCL11 in a patient diagnosed with neuromotor developmental delay, epilepsy, bronchiolitis obliterans and hypothyroidism. CASE PRESENTATION: A 4-year-old male patient was admitted to our clinic with global developmental delay and a medical history that included recurrent hospitalizations for pneumonia at the age of 17 days, and in months 4, 5 and 7...
January 23, 2024: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://read.qxmd.com/read/38141895/short-term-outcomes-after-third-time-lung-transplantation-a-single-institution-experience
#22
JOURNAL ARTICLE
Vikram F Gupta, Samantha E Halpern, Arya Pontula, Madison K Krischak, John M Reynolds, Jacob A Klapper, Matthew G Hartwig, John C Haney
BACKGROUND: Reoperative lung transplantation (LTx) survival has improved over time such that a growing number of patients may present for third-time LTx (L3Tx). To understand the safety of L3Tx, we evaluated perioperative outcomes and 3-year survival after L3Tx at a high-volume US LTx center. METHODS: This retrospective study included all patients who underwent bilateral L3Tx at our institution. Using an optimal matching technique, a primary LTx (L1Tx) cohort was matched 1:2 and a second-time LTx (L2Tx) cohort 1:1...
December 21, 2023: Journal of Heart and Lung Transplantation
https://read.qxmd.com/read/38117354/risk-factors-and-prediction-models-for-bronchiolitis-obliterans-after-severe-adenoviral-pneumonia
#23
JOURNAL ARTICLE
Shunhang Wen, Ming Xu, Weigang Jin, Luyao Zeng, Zupan Lin, Gang Yu, Fangfang Lv, Lili Zhu, Changfu Xu, Yangming Zheng, Lin Dong, Li Lin, Hailin Zhang
UNLABELLED: Severe adenoviral pneumonia (SAP) can cause post-infectious bronchiolitis obliterans (PIBO) in children. We aimed to investigate the relevant risk factors for PIBO and develop a predictive nomogram for PIBO in children with SAP. This prospective study analysed the clinical data of hospitalised children with SAP and categorised them into the PIBO and non-PIBO groups. Least absolute shrinkage and selection operator (LASSO) regressions were applied to variables that exhibited significant intergroup differences...
March 2024: European Journal of Pediatrics
https://read.qxmd.com/read/38087715/a-personalized-organ-based-approach-to-the-treatment-of-chronic-steroid-refractory-graft-versus-host-disease
#24
REVIEW
Hanaa Fatoum, Robert Zeiser, Shahrukh K Hashmi
Chronic graft-versus-host-disease (cGvHD) remains the leading cause of morbidity among transplant recipients. The efficacy of second-line treatments varies widely based on many factors, including wide differences in the organ overall response-rate response and in the current era where multiple agents are approved, and optimal sequencing of drugs based on organ ORR is unknown. We aimed to evaluate outcomes based on ORRs to the most common agents for the treatment of steroid-refractory/steroid-dependent cGvHD by conducting a systematic literature review...
January 2024: Blood Reviews
https://read.qxmd.com/read/38084407/single-cell-resolution-of-human-airway-epithelial-cells-exposed-to-bronchiolitis-obliterans-associated-chemicals
#25
JOURNAL ARTICLE
Chin-Yi Chu, So-Young Kim, Gloria S Pryhuber, Thomas J Mariani, Matthew D McGraw
RATIONALE: Bronchiolitis obliterans (BO) is a fibrotic lung disease characterized by progressive luminal narrowing and obliteration of the small airways. In the non-transplant population, inhalation exposure to certain chemicals is associated with BO, however, the mechanisms contributory to disease induction remain poorly understood. This study's objective was to use single cell RNA sequencing for the identification of transcriptomic signatures common to primary human airway epithelial cells after chemical exposure to BO-associated chemicals - diacetyl or nitrogen mustard - to help explain BO induction...
December 12, 2023: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://read.qxmd.com/read/38082917/global-and-regional-lung-function-assessment-using-portable-electrical-impedance-tomography-eit-system-clinical-study
#26
JOURNAL ARTICLE
Fedi Zouari, Pak To Cheung, Adrien Touboul, Wang C Kwok, Venice Sin, Eddie C Wong, Iris Y Zhou, Terence C C Tam, Russell W Chan
Recent development of affordable, portable and self-administrable electrical impedance tomography (EIT) system demonstrated the feasibility of using standalone EIT and subject's anthropometrics to predict the gold standard spirometry indicators for lung-function assessment. Compared to spirometry, the system showed the advantage of providing spatial mapping of the spirometry indicators. Nevertheless, the previous study was limited to healthy subjects. Here, we recruited (N=88): 47 lung disease patients and 41 healthy controls to perform simultaneous EIT and spirometry measurements to validate the capabilities of the system...
July 2023: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://read.qxmd.com/read/38082371/castleman-disease-of-plasma-cell-type-accompanied-with-bronchiolitis-obliterans-a-case-report-and-review-of-the-literature
#27
REVIEW
Qingyuan Zhu, Shuiyou Wang
BACKGROUND: Castleman disease, also known as giant lymph node hyperplasia or angiofollicular lymph node hyperplasia, is a highly heterogeneous clinicopathological entity that belongs to the family lymphoproliferative disorders. Castleman disease accompanied by bronchiolitis obliterans is uncommon and often poses a great diagnostic challenge, which is easily confused with respiratory diseases and impeding the correct diagnosis and treatment. The main aim in presenting such rare case studies is to raise awareness and expand the diagnostic horizon of clinicians for appropriate management...
December 12, 2023: Journal of Medical Case Reports
https://read.qxmd.com/read/38062225/sleep-and-quality-of-life-characteristics-in-a-pediatric-population-with-chronic-obstructive-respiratory-diseases
#28
JOURNAL ARTICLE
Magali Santos Lumertz, Frederico Orlando Friedrich, Letícia Bortolini Loch, Leonardo Araújo Pinto
PURPOSE: To describe sleep and quality of life of pediatric patients with chronic obstructive respiratory diseases and to ascertain whether or not sleep quality correlates with quality of life in this population. METHODS: Participants aged 5 to 18 years with cystic fibrosis (CF), severe asthma, or postinfectious bronchiolitis obliterans (PIBO) receiving regular follow-up at a pediatric respiratory medicine center were recruited. Two questionnaires were used: the Brazilian version of the Sleep Disturbance Scale for Children (SDSC) and the Pediatric Quality of Life Inventory (Peds-QL)...
December 7, 2023: Sleep & Breathing
https://read.qxmd.com/read/38044496/impulse-oscillometry-versus-spirometry-to-detect-bronchiolitis-obliterans-syndrome-in-bilateral-lung-transplant-recipients-a-prospective-diagnostic-study
#29
JOURNAL ARTICLE
Thomas D Crowhurst, Jessica A Butler, Lauren A Bussell, Sonya D Johnston, David Yeung, Greg Hodge, Greg I Snell, Aeneas Yeo, Mark Holmes, Chien-Li Holmes-Liew
BACKGROUND: Chronic lung allograft dysfunction (CLAD), and especially bronchiolitis obliterans syndrome (BOS), remain dominant causes of morbidity and mortality after lung transplantation. Interest is growing in the forced oscillation technique, of which impulse oscillometry (IOS) is a form, as a tool to improve our understanding of these disorders. However, data remain limited and no longitudinal studies have been published, meaning there is no information regarding any capacity IOS may have for the early detection of CLAD...
December 4, 2023: Transplantation
https://read.qxmd.com/read/38043865/chest-imaging-classification-in-mycoplasma-pneumoniae-pneumonia-is-associated-with-its-clinical-features-and-outcomes
#30
JOURNAL ARTICLE
Xia Huang, Haiyan Gu, Ruxi Wu, Lei Chen, Tian Lv, Xinyi Jiang, Huili Li, Bin Guo, Jie Liu, Dan Li, Deyu Zhao, Feng Liu
BACKGROUND: The imaging findings of Mycoplasma pneumoniae pneumonia (MPP) vary; however, few studies have focused on the relationship of imaging classification with clinical manifestations and outcomes. OBJECTIVE: To prospectively investigate whether chest imaging classification in Mycoplasma pneumoniae pneumonia (MPP) is associated with its clinical features and outcomes. METHODS: A total of 1,401 hospitalized children with MPP were enrolled from January 2019 to December 2021...
December 1, 2023: Respiratory Medicine
https://read.qxmd.com/read/38018577/evaluation-of-common-protein-biomarkers-involved-in-the-pathogenesis-of-respiratory-diseases-with-proteomic-methods-a-systematic-review
#31
REVIEW
Hadi Rezaeeyan, Masoud Arabfard, Hamid R Rasouli, Alireza Shahriary, B Fatemeh Nobakht M Gh
AIM: Respiratory disease (RD) is one of the most common diseases characterized by lung dysfunction. Many diagnostic mechanisms have been used to identify the pathogenic agents of responsible for RD. Among these, proteomics emerges as a valuable diagnostic method for pinpointing the specific proteins involved in RD pathogenesis. Therefore, in this study, for the first time, we examined the protein markers involved in the pathogenesis of chronic obstructive pulmonary disease (COPD), idiopathic pulmonary fibrosis (IPF), asthma, bronchiolitis obliterans (BO), and chemical warfare victims exposed to mustard gas, using the proteomics method as a systematic study...
November 2023: Immunity, Inflammation and Disease
https://read.qxmd.com/read/38012449/clinical-risk-factors-and-prognostic-model-for-patients-with-bronchiolitis-obliterans-syndrome-after-hematopoietic-stem-cell-transplantation
#32
JOURNAL ARTICLE
Qiu-Sha Huang, Tian-Xiao Han, Qi Chen, Jin Wu, Peng Zhao, Ye-Jun Wu, Yun He, Xiao-Lu Zhu, Hai-Xia Fu, Feng-Rong Wang, Yuan-Yuan Zhang, Xiao-Dong Mo, Wei Han, Chen-Hua Yan, Jing-Zhi Wang, Huan Chen, Yu-Hong Chen, Ting-Ting Han, Meng Lv, Yao Chen, Yu Wang, Lan-Ping Xu, Kai-Yan Liu, Xiao-Jun Huang, Xiao-Hui Zhang
Bronchiolitis obliterans syndrome (BOS) is a common and potentially devastating noninfectious pulmonary complication after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Currently, predictive tools for BOS are not available. We aimed to identify the clinical risk factors and establish a prognostic model for BOS in patients who undergo allo-HSCT. We retrospectively identified a cohort comprising 195 BOS patients from 6100 consecutive patients who were allografted between 2008 and 2022. The entire cohort was divided into a derivation cohort and a validation cohort based on the time of transplantation...
February 2024: Bone Marrow Transplantation
https://read.qxmd.com/read/37976074/defilipp-z-kim-ht-yang-z-et%C3%A2-al-clinical-response-to-belumosudil-in-bronchiolitis-obliterans-syndrome-a-combined-analysis-from-2-prospective-trials-blood-adv-2022-6-24-6263-6270
#33
JOURNAL ARTICLE
https://read.qxmd.com/read/37963296/postinfectious-bronchiolitis-obliterans-in-children-case-series-at-a-pediatric-hospital-in-peru
#34
JOURNAL ARTICLE
Noé Atamari-Anahui, Héctor Nuñez-Paucar, Luz K Paredes-Rodríguez, Meylin Escalante-Oviedo, Johana L Córdova-Meza, Kerly M Cruz-Vallejos, Carlos Valera-Moreno, Alex Untiveros-Tello
BACKGROUND: Postinfectious bronchiolitis obliterans is a rare lung disease; there are limited reports in South America. CASE REPORT: We report 10 patients with this disease diagnosed at the Instituto Nacional de Salud del Niño-Breña (Lima-Peru). The median age at diagnosis was 19 months and all patients had a history of severe acute respiratory infection. The most frequent symptoms were cough, respiratory distress, wheezing, and hypoxemia. The mosaic attenuation pattern was the most frequent on the tomography...
2023: Boletín Médico del Hospital Infantil de México
https://read.qxmd.com/read/37953562/effects-of-respiratory-flora-regulation-on-microbial-environment-and-il-10-stat3-signaling-pathway-in-the-bronchiolitis-obliterans-after-lung-transplantation
#35
JOURNAL ARTICLE
Xin Wang, Tianhua Liu, Yizhuo Chen, Ziqing Xu, Jianghua Zhan, Ming Dong
Our purpose of this study was to explore the application effect of respiratory flora regulation in bronchiolitis obliterans after lung transplantation, and its regulatory effect on the microbial environment of the lesion and the IL-10/STAT3 signaling pathway. 25 clean-grade C57BL/6 male mice and 5 BALB/c male mice were selected for orthotopic tracheal transplantation and postoperative respiratory flora regulation in a hospital animal room from Jan 2019 to Dec 2021. Next, the changes in the microbial environment and the IL-10/STAT3 signaling pathway before and after respiratory flora regulation were compared, so as to evaluate the regulatory effect of this method...
October 31, 2023: Cellular and Molecular Biology
https://read.qxmd.com/read/37936997/bronchiolitis-obliterans-with-recurrent-pneumothorax-after-allogeneic-bone-marrow-transplantation
#36
Masayuki Akatsuka, Naoya Yama
Bronchiolitis obliterans syndrome (BOS) is a non-infectious pulmonary complication that can occur in patients who have undergone allogeneic bone marrow transplantation (BMT). BOS is characterized by the irreversible narrowing and obstruction of bronchi, resulting in severe respiratory distress and poor outcomes. This case report focuses on the complex management of a patient with a multifaceted medical history. A 20-year-old man was initially diagnosed with precursor B lymphoblastic lymphoma and subsequently underwent allogeneic BMT...
October 2023: Curēus
https://read.qxmd.com/read/37932341/transcriptome-analysis-reveals-the-impact-of-nets-activation-on-airway-epithelial-cell-emt-and-inflammation-in-bronchiolitis-obliterans
#37
JOURNAL ARTICLE
Zhongji Wu, Xiaowen Chen, Shangzhi Wu, Zhenwei Liu, Hongwei Li, Kailin Mai, Yinghui Peng, Haidi Zhang, Xiaodie Zhang, Zhaocong Zheng, Zian Fu, Dehui Chen
Bronchiolitis obliterans (BO) is a chronic airway disease that was often indicated by the pathological presentation of narrowed and irreversible airways. However, the molecular mechanisms of BO pathogenesis remain unknown. Although neutrophil extracellular traps (NETs) can contribute to inflammatory disorders, their involvement in BO is unclear. This study aims to identify potential signaling pathways in BO by exploring the correlations between NETs and BO. GSE52761 and GSE137169 datasets were downloaded from gene expression omnibus (GEO) database...
November 6, 2023: Scientific Reports
https://read.qxmd.com/read/37891020/sirolimus-long-term-tolerability-and-impact-on-kidney-function-in-lung-transplantation-a-single-center-experience
#38
JOURNAL ARTICLE
Ashley A Feist, Mark Mariski, Linda Awdishu, Michelle Bremer, Gordon Yung, Chris Jung, Eugene Golts, Kamyar Afshar
BACKGROUND: After lung transplant, 2 common complications are calcineurin inhibitor (CNI) induced nephrotoxicity and bronchiolitis obliterans syndrome. The objective of this study was to investigate the long-term effects of sirolimus conversion after lung transplantation. METHODS: This was a retrospective cohort study of patients who had undergone lung transplantation at a single center from June 2003 to December 2016. We compared patients converted to a sirolimus-based regimen to those maintained on our standard tacrolimus-based regimen...
October 25, 2023: Transplantation Proceedings
https://read.qxmd.com/read/37887076/chronic-lung-allograft-dysfunction-is-associated-with-significant-disability-after-lung-transplantation-a-burden-of-disease-analysis-in-1025-cases
#39
JOURNAL ARTICLE
Roland Diel, Susanne Simon, Jens Gottlieb
BACKGROUND: Chronic lung allograft dysfunction (CLAD) is the leading cause of death after the first postoperative years of lung transplantation (LTx). OBJECTIVE: To assess the number of disability-adjusted life years (DALYs) per patient with severe CLAD. METHODS: The clinical and demographic data of patients who received their lung transplantation between 2010 and 2020 in the Hanover Medical School (Germany) were evaluated. RESULTS: A total of 1025 lung transplant patients were followed for a median of 51 months (4...
October 12, 2023: Advances in Respiratory Medicine
https://read.qxmd.com/read/37870882/prognostic-value-of-chest-ct-findings-at-bos-diagnosis-in-lung-transplant-recipients
#40
JOURNAL ARTICLE
Anke Van Herck, Hanne Beeckmans, Pieterjan Kerckhof, Annelore Sacreas, Saskia Bos, Janne Kaes, Arno Vanstapel, Bart M Vanaudenaerde, Jan Van Slambrouck, Michaela Orlitová, Xin Jin, Laurens J Ceulemans, Dirk E Van Raemdonck, Arne P Neyrinck, Laurent Godinas, Lieven J Dupont, Geert M Verleden, Adriana Dubbeldam, Walter De Wever, Robin Vos
BACKGROUND: Bronchiolitis obliterans syndrome (BOS) after lung transplantation is characterized by fibrotic small airway remodeling, recognizable on high-resolution computed tomography (HRCT). We studied the prognostic value of key HRCT features at BOS diagnosis after lung transplantation. METHODS: The presence and severity of bronchiectasis, mucous plugging, peribronchial thickening, parenchymal anomalies, and air trapping, summarized in a total severity score, were assessed using a simplified Brody II scoring system on HRCT at BOS diagnosis, in a cohort of 106 bilateral lung transplant recipients transplanted between January 2004 and January 2016...
November 1, 2023: Transplantation
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