scholarly journals The Antimicrobial Activity of Peripheral Blood Neutrophils Is Altered in Patients with Primary Ciliary Dyskinesia

2021 ◽  
Vol 22 (12) ◽  
pp. 6172
Author(s):  
Maaike Cockx ◽  
Marfa Blanter ◽  
Mieke Gouwy ◽  
Pieter Ruytinx ◽  
Sara Abouelasrar Salama ◽  
...  
Keyword(s):  
Peripheral Blood ◽  
Primary Ciliary Dyskinesia ◽  
Bacterial Infections ◽  
Respiratory Infections ◽  
Neutrophil Extracellular Trap ◽  
Oxygen Species ◽  
Recurrent Respiratory Infections ◽  
Blood Neutrophils ◽  
Low Levels ◽  
Ciliary Dyskinesia

The airways of patients with primary ciliary dyskinesia (PCD) contain persistently elevated neutrophil numbers and CXCL8 levels. Despite their abundance, neutrophils fail to clear the airways from bacterial infections. We investigated whether neutrophil functions are altered in patients with PCD. Neutrophils from patients and healthy controls (HC) were isolated from peripheral blood and exposed to various bacterial stimuli or cytokines. Neutrophils from patients with PCD were less responsive to low levels of fMLF in three different chemotaxis assays (p < 0.05), but expression of the fMLF receptors was unaltered. PCD neutrophils showed normal phagocytic function and expression of adhesion molecules. However, PCD neutrophils produced less reactive oxygen species upon stimulation with bacterial products or cytokines compared to HC neutrophils (p < 0.05). Finally, the capacity to release DNA, as observed during neutrophil extracellular trap formation, seemed to be reduced in patients with PCD compared to HC (p = 0.066). These results suggest that peripheral blood neutrophils from patients with PCD, in contrast to those of patients with cystic fibrosis or COPD, do not show features of over-activation, neither on baseline nor after stimulation. If these findings extend to lung-resident neutrophils, the reduced neutrophil activity could possibly contribute to the recurrent respiratory infections in patients with PCD.

scholarly journals A Case Report of Kartagener Syndrome

2020 ◽  
Vol 24 (3) ◽  
pp. 284-293
Author(s):  
Mahnaz Moradi ◽  
◽  
Leili Yekefallah ◽  
Mohammadali Zohal ◽  
Peyman Namdar ◽  
...  
Keyword(s):  
Primary Ciliary Dyskinesia ◽  
Rare Case ◽  
Pulmonary Infection ◽  
Respiratory Infections ◽  
Genetic Disorders ◽  
Vasomotor Rhinitis ◽  
Kartagener Syndrome ◽  
Recurrent Respiratory Infections ◽  
Ciliary Dyskinesia ◽  
Timely Treatment

Primary Ciliary Dyskinesia (PCD) and Kartagener Syndrome (KS) are rare genetic disorders. PCD occurs in patients with recurrent sino-pulmonary infection, dextrocardia, chronic vasomotor rhinitis, and bronchiectasis. This study reports a rare case of KS for having further awareness of this disease. According to this study, this disease should be considered in patients with recurrent respiratory infections, because early diagnosis and timely treatment of these patients can lead to reduced irreversible complications and increased life expectancy.

scholarly journals Chemotaxis of Blood Neutrophils from Patients with Primary Ciliary Dyskinesia

2003 ◽  
Vol 18 (1) ◽  
pp. 36 ◽  
Author(s):  
Young Yull Koh ◽  
Yong Han Sun ◽  
Yang-Gi Min ◽  
Je G. Chi ◽  
Chang Keun Kim
Keyword(s):  
Primary Ciliary Dyskinesia ◽  
Blood Neutrophils ◽  
Ciliary Dyskinesia

scholarly journals Receptors on Peripheral blood Neutrophils and Exudate Neutrophils in Acute Bacterial Infections

Ensho ◽  
1981 ◽  
Vol 1 (5) ◽  
pp. 680-680
Author(s):  
Shigeyo Sakurada ◽  
Toru Hara ◽  
Toru Kimura ◽  
Yoki Hayashi ◽  
Saburo Kakuta ◽  
...  
Keyword(s):  
Peripheral Blood ◽  
Bacterial Infections ◽  
Blood Neutrophils ◽  
Acute Bacterial Infections

scholarly journals COVID-19 vaccinations: perceptions and behaviours in people with primary ciliary dyskinesia

2021 ◽  
Author(s):  
Eva Sophie Lunde Pedersen ◽  
Maria Christina Mallet ◽  
Yin Ting Lam ◽  
Sara Bellu ◽  
Isabelle Cizeau ◽  
...  
Keyword(s):  
Side Effects ◽  
Support Groups ◽  
Primary Ciliary Dyskinesia ◽  
Respiratory Infections ◽  
Public Information ◽  
Social Contact ◽  
Vaccine Uptake ◽  
Vaccination Uptake ◽  
Contact Behaviour ◽  
Ciliary Dyskinesia

Primary ciliary dyskinesia (PCD) is a rare genetic disease that causes recurrent respiratory infections. People with PCD may be at high risk of severe COVID-19 and vaccination against SARS-CoV-2 is therefore important. We studied vaccination willingness, speed of vaccination uptake, side effects, and changes in social contact behavior after vaccination in people with PCD. We used data from COVID-PCD, an international participatory cohort study. A questionnaire was e-mailed to participants in May 2021 that asked about COVID-19 vaccinations. 423 participants from 31 countries replied (median age: 30 years; 261 (62%) female). Vaccination uptake and willingness was high with 273 of 287 adults (96%) being vaccinated or willing to be in June 2021; only 4% were hesitant. The most common reasons for hesitancy were fear of side effects (reported by 88%). Mild side effects were common but no participant reported severe side effects. Half of participants changed their social contact behaviour after vaccination by seeing friends and family more often. The high vaccination willingness in the study population might reflect the extraordinary effort taken by PCD support groups to inform people about COVID-19 vaccination. Clear and specific public information and involvement of representatives is important for high vaccine uptake.

scholarly journals NFIL3 Facilitates Neutrophil Autophagy, Neutrophil Extracellular Trap Formation and Inflammation During Gout via REDD1-Dependent mTOR Inactivation

2021 ◽  
Vol 8 ◽  
Author(s):  
Honghu Tang ◽  
Chunyu Tan ◽  
Xue Cao ◽  
Yi Liu ◽  
Hua Zhao ◽  
...  
Keyword(s):  
Peripheral Blood ◽  
Expression Patterns ◽  
Gouty Arthritis ◽  
Mtor Pathway ◽  
Neutrophil Extracellular Trap ◽  
Inflammatory Factors ◽  
Loss Of Function ◽  
Blood Neutrophils

Autophagy pathways play an important role in immunity and inflammation via pathogen clearance mechanisms mediated by immune cells, such as macrophages and neutrophils. In particular, autophagic activity is essential for the release of neutrophil extracellular traps (NETs), a distinct form of active neutrophil death. The current study set out to elucidate the mechanism of the NFIL3/REDD1/mTOR axis in neutrophil autophagy and NET formation during gout inflammation. Firstly, NFIL3 expression patterns were determined in the peripheral blood neutrophils of gout patients and monosodium urate (MSU)-treated neutrophils. Interactions between NFIL3 and REDD1 were identified. In addition, gain- or loss-of-function approaches were used to manipulate NFIL3 and REDD1 in both MSU-induced neutrophils and mice. The mechanism of NFIL3 in inflammation during gout was evaluated both in vivo and in vitro via measurement of cell autophagy, NET formation, MPO activity as well as levels of inflammatory factors. NFIL3 was highly-expressed in both peripheral blood neutrophils from gout patients and MSU-treated neutrophils. NFIL3 promoted the transcription of REDD1 by binding to its promoter. REDD1 augmented neutrophil autophagy and NET formation by inhibiting the mTOR pathway. In vivo experimental results further confirmed that silencing of NFIL3 reduced the inflammatory injury of acute gouty arthritis mice by inhibiting the neutrophil autophagy and NET formation, which was associated with down-regulation of REDD1 and activation of the mTOR pathway. Taken together, NFIL3 can aggravate the inflammatory reaction of gout by stimulating neutrophil autophagy and NET formation via REDD1/mTOR, highlighting NFIL3 as a potential therapeutic target for gout.

scholarly journals Frequency and Types of Ciliary Ultrastructural Defects in Patients With Suspected Primary Ciliary Dyskinesia Symptoms Analyzed by Transmission Electron Microscopy

2021 ◽  
Author(s):  
Mitra Rezaei ◽  
Amirali Soheili ◽  
Atefeh Fakharian ◽  
Hamid Jamaati ◽  
Jahangir Ghorbani ◽  
...  
Keyword(s):  
Electron Microscopy ◽  
Transmission Electron Microscopy ◽  
Primary Ciliary Dyskinesia ◽  
Respiratory Infections ◽  
Descriptive Analysis ◽  
Final Diagnosis ◽  
International Consensus ◽  
Tem Analysis ◽  
Transmission Electron ◽  
Ciliary Dyskinesia

Abstract Background: Primary ciliary dyskinesia (PCD) is a rare autosomal recessive condition of often chronic respiratory infections in early life. A useful tool for early diagnosis of such ciliary abnormalities is transmission electron microscopy (TEM). This study aimed to use TEM to examine these defects and speculate on a diagnosis.Methods: From 2017 to 2019, all referral patients with suspected PCD symptoms were included in this study. Nasal samples were taken after exclusion of further potential differential diagnosis and prepared for TEM. The final diagnosis was based on the International Consensus Guideline for reporting transmission electron microscopy results in the diagnosis of PCD. A descriptive analysis of demographic and ciliary ultrastructural data was performed by SPSS ver 21.Results: Study population consisted of 37 women and 30 men (mean age=20.34±10.7 years). The clinical presentations were as follows: bronchiectasis: 26 patients (38.8%); sinusitis: 23(34.3%); recurrent respiratory infection: 21 patients (31.3%); auditory symptoms: 5 patients (7.5%); situs inversus: 3 patients (4.4%); productive cough: 2 patients (3%); infertility: 2 patients (3%); polyposis: 1 patient (1.5%). According to TEM analysis, 12 (17%) of patients were PCD, 11 (15.7%) were indicating PCD cases, 26 (37.1%) of them had no criteria of PCD and 18 (25.7%) of cases had normal ciliary ultrastructure. Compound cilia and extra-tubule were reported in 29 (41.4%) and 31(44.3%) of patients, respectively. The outer dynein arm defect was seen in 11(16.4%) cases and the inner dynein arm (IDA) defect was seen in 20 (29.8%) cases. Two patients (3%) had microtubular disorganization.Conclusion: Bronchiectasis and sinusitis were the most common complications. The compound cilia and extra-tubule were the most prevalent TEM finding among all participants. However, the most prevalent hallmark diagnostic defects among PCD patients were ODA and IDA defects among PCD patients. Other diagnostic PCD tests should also be performed in patients in the indicating PCD group, those without PCD criteria, and normal patients with a highly suggestive history. Cell-culture, as well, should confirm IDA defects. This study highlights the fundamental need to consider ciliary defect among probable diagnoses and use TEM as a practical diagnostic tool.

scholarly journals Chemiluminescence of Peripheral Blood Neutrophils in Mares with Endometritis

2012 ◽  
Vol 56 (1) ◽  
pp. 51-56
Author(s):  
Wiesław Krumrych ◽  
Janusz Danek
Keyword(s):  
Reactive Oxygen Species ◽  
Peripheral Blood ◽  
Oxygen Metabolism ◽  
Oxygen Species ◽  
Significant Relation ◽  
Cytological Examination ◽  
Blood Samples ◽  
Severe Inflammation ◽  
Activated Neutrophils ◽  
Blood Neutrophils

Abstract The aim of the study was to evaluate the oxygen metabolism of neutrophils in peripheral blood of mares in relation to intensity of endometrium inflammations. The study involved 36 half-breed mares, aged 4-22 years, showing fertility disturbances. In 26 mares neutrophils were found in uteral smears, which indicated endometritis (15 - moderate inflammation and 11 - severe inflammation). In the rest mares, cytological examination excluded inflammation. Blood samples were evaluated in terms of neutrophils chemiluminescence without stimulation (CL-WS) and with stimulation by opsonised zymosan (CL-OZ). The study demonstrated (only in case of CL-WS) an increase in chemiluminescence of cells in mares with a severe inflammation of the endometrium. The increased chemiluminescence activity was accompanied by a decrease in activation index (OZ/WS) of neutrophils, suggesting some imbalance between production and elimination of reactive oxygen species (ROS). The correlation analysis demonstrated a statistically significant relation between the intensity of the uterus inflammation, which was verified by cytological examination and CL-WS of peripheral blood neutrophils, as well as their activation index. The obtained results suggest that activated neutrophils are an important source of ROS which can play a role in the pathogenesis of endometritis in mares.

scholarly journals Impaired Growth during Childhood in Patients with Primary Ciliary Dyskinesia

2013 ◽  
Vol 2013 ◽  
pp. 1-5 ◽  
Author(s):  
Tamara Svobodová ◽  
Jana Djakow ◽  
Daniela Zemková ◽  
Adam Cipra ◽  
Petr Pohunek ◽  
...  
Keyword(s):  
Body Length ◽  
Primary Ciliary Dyskinesia ◽  
Respiratory Infections ◽  
Growth Period ◽  
Growth Delay ◽  
Childhood Growth ◽  
Population Standards ◽  
Children And Young Adults ◽  
Nutritional Deficits ◽  
Ciliary Dyskinesia

Primary ciliary dyskinesia (PCD) leads to recurrent/chronic respiratory infections, resulting in chronic inflammation and potentially in chronic pulmonary disease with bronchiectasis. We analyzed longitudinal data on body length/height and body mass index (BMI) for 29 children and young adults with PCD aging 1.5–24 years (median, 14.5) who had been diagnosed at the age of 0.5–17 years (median, 8). Of these, 10 carried pathogenic mutations in eitherDNAH5orDNAI1. In children with PCD, body length/height progressively decreased from+0.40±0.24SDS (the 1st birthday),+0.16±0.23SDS (3 years old), and-0.13±0.21SDS (5 years old) to-0.54±0.19SDS (7 years old;P=0.01versus0),-0.67±0.21SDS (9 years old;P=0.005versus0),-0.52±0.24SDS (11 years old;P=0.04versus0), and-0.53±0.23SDS (13 years old;P=0.03versus0). These results reflect low growth rates during the childhood growth period. Thereafter, heights stabilized up to the age of 17 years. The growth deterioration was not dependent on sex or disease severity but was more pronounced inDNAH5orDNAI1mutation carriers. BMI did not differ from population standards, which suggests that nutritional deficits are not the cause of growth delay. We conclude that PCD leads to chronic deprivation with significant growth deterioration during childhood.

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