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A systematic review regarding the prevalence of malignancy in patients with the hyper-IgE syndrome
AbstractThe hyper-immunoglobulin E syndrome (HIES) is a primary immunodeficiency disease originally described as Job syndrome. The fundamental causative variant of the HIES is an autosomal dominant mutation in the signal transducer and activator of transcription 3 (STAT3) gene. It is characterized by recurrent staphylococcal cold skin abscess, sinopulmonary infection, eczema, head and face anomalies, frequent bone fractures, eosinophilia and extremely high serum IgE levels (IgE ≥ 2000 IU/mL). However, multiple other genetic defects are also known as HIES-like disorders. Apart from infectious manifestations,STAT3,DOC...
Source: Clinical and Experimental Medicine - November 4, 2023 Category: Research Source Type: research

Too much of a good thing: a review of primary immune regulatory disorders
Primary immune regulatory disorders (PIRDs) are inborn errors of immunity caused by a loss in the regulatory mechanism of the inflammatory or immune response, leading to impaired immunological tolerance or an exuberant inflammatory response to various stimuli due to loss or gain of function mutations. Whilst PIRDs may feature susceptibility to recurrent, severe, or opportunistic infection in their phenotype, this group of syndromes has broadened the spectrum of disease caused by defects in immunity-related genes to include autoimmunity, autoinflammation, lymphoproliferation, malignancy, and allergy; increasing focus on PIR...
Source: Frontiers in Immunology - October 31, 2023 Category: Allergy & Immunology Source Type: research

The diagnostic role of transbronchial lung cryobiopsy (TBLC) in Granulomatous-Lymphocytic Interstitial Lung Disease (GLILD) associated to Common Variable Immunodeficiency (CVID)
This study demonstrates that transbronchial cryobiopsy is a safe and minimally invasive approach for characterizing GLILD in CVID patients, providing a high diagnostic confidence and low rate of complications. (Source: European Respiratory Journal)
Source: European Respiratory Journal - October 27, 2023 Category: Respiratory Medicine Authors: Menichini, I., Ravaglia, C., Piciucchi, S., Dubini, A., Poletti, V. Tags: Rare ILD/DPLD Source Type: research

Extracellular matrix degradation products in serum of patients with chronic airway disease
Conclusion: ECM degradation products can be measured in the circulation of patients with airway inflammation and may be suited as disease-monitoring biomarkers. Further studies are required to ensure the accuracy of ECM quantification in sputum. (Source: European Respiratory Journal)
Source: European Respiratory Journal - October 27, 2023 Category: Respiratory Medicine Authors: Schnell, A., Graeter, S., Saxenhofer, M., Chan, S., Rees, M., Douglass, J., Stewart, A., Mckenzie, A., Schulze, I., Schaub, A. Tags: Monitoring airway disease Source Type: research

Pediatric non-CF bronchiectasis in the Vinnytsia region in Ukraine - what can we do better?
Conclusions: This is the first-time reported data for pediatric non-CF bronchiectasis from Ukraine. The disease seems to be underdiagnosed in the Vinnytsia region compared to epidemiological data in the European region. The etiology of pediatric bronchiectasis in Vinnytsia differs from published results from other countries probably because of some undiagnosed causes like protracted bacterial bronchitis, aspiration etc. More studies need to evaluate the true incidence of pediatric bronchiectasis in Ukraine. (Source: European Respiratory Journal)
Source: European Respiratory Journal - October 27, 2023 Category: Respiratory Medicine Authors: Mazulov, O., Khromykh, K., Tykholaz, O. Tags: Paediatric respiratory epidemiology Source Type: research

Patients with chronic airway disease: Insights from a sputum proteome analysis
Conclusion: Sputum proteomic analysis offers a powerful tool to explore a broad set of proteins in the airway lumen and improve our understanding of the disease pathophysiology. (Source: European Respiratory Journal)
Source: European Respiratory Journal - October 27, 2023 Category: Respiratory Medicine Authors: Graeter, S., Capriotti, L., Schnell, A., Saxenhofer, M., Chan, S., Rees, M., Douglass, J., Steward, A., Roca, C., Schulze, I., Schaub, A. Tags: Monitoring airway disease Source Type: research

Immunosuppressed COVID-19 patients: experience from a tertiary Chest hospital in Athens, Greece
Conclusion: Patients on anti-CD20 agents showed prolonged viremia, persistent fever, high risk for clinical deterioration and longer hospitalization. The ever-increasing number of these patients dicates a need for tailoring of treatment and constantly updated training. (Source: European Respiratory Journal)
Source: European Respiratory Journal - October 27, 2023 Category: Respiratory Medicine Authors: Vlachantoni, I.-T., Levounets, A., Chatzipetrou, R., Kyriacou, A., Skontzou, E., Vlassi, L., Kontogianni, C., Samitas, K., Sardelis, Z., Markozannes, E., Grigoratou, T., Zervas, E., Kolilekas, L. Tags: Respiratory infections and bronchiectasis Source Type: research

Bioinformatics Integrative Analysis of Circadian Rhythms Effects on Atopic Dermatitis and Dendritic Cells
CONCLUSION: The findings of this study suggested that the circadian rhythm is involved in the progression of AD, and RNTL2, NOCT, and RORC as well as dendritic cells are differentially expressed in AD. These findings could be used to introduce diagnostic and chronotherapeutic modalities for AD.PMID:37873510 | PMC:PMC10590565 | DOI:10.2147/CCID.S424343 (Source: Clinical, Cosmetic and Investigational Dermatology)
Source: Clinical, Cosmetic and Investigational Dermatology - October 24, 2023 Category: Dermatology Authors: Nan Sun Dan Dai Shihang Deng Xun Cai Ping Song Source Type: research

Identification of useful biomolecular markers in kidney renal clear cell carcinoma: an in silico and in vitro analysis-based study
CONCLUSION: Our integrated in silico and in vitro analysis identified important hub genes (TYROBP, PTPRC, LCP2, and ITGB2) involved in the pathogenesis of KIRC as possible diagnostic biomarkers.PMID:37854221 | PMC:PMC10579006 (Source: American Journal of Translational Research)
Source: American Journal of Translational Research - October 19, 2023 Category: Research Authors: Khalida Sheikh Khalida Naz Memon Humera Usman Mostafa A Abdel-Maksoud Sajid Ullah Taghreed N Almanaa Aqsa Chaudhary Muhammad Jamil Owais Bin Qadeer Gill Muhammad Ahmed Yar Ahmed M Hussein Adel M Zakri Source Type: research

Identification of useful biomolecular markers in kidney renal clear cell carcinoma: an in silico and in vitro analysis-based study
CONCLUSION: Our integrated in silico and in vitro analysis identified important hub genes (TYROBP, PTPRC, LCP2, and ITGB2) involved in the pathogenesis of KIRC as possible diagnostic biomarkers.PMID:37854221 | PMC:PMC10579006 (Source: Cell Research)
Source: Cell Research - October 19, 2023 Category: Cytology Authors: Khalida Sheikh Khalida Naz Memon Humera Usman Mostafa A Abdel-Maksoud Sajid Ullah Taghreed N Almanaa Aqsa Chaudhary Muhammad Jamil Owais Bin Qadeer Gill Muhammad Ahmed Yar Ahmed M Hussein Adel M Zakri Source Type: research