Clinical improvement in Job syndrome following administration of co-trimoxazole, omalizumab and inhaled tobramycin

Established treatment regimens for the autosomal dominant hyperimmunoglobulin E syndrome, denominated Job syndrome, are lacking. Thus, Job syndrome still exerts a dramatic impact on patients’ quality of life. Our aim was to present safety and effectiveness of a regimen including co-trimoxazole, omalizumab and inhaled tobramycin in Job syndrome. A 26-year-old woman diagnosed with Job syndrome since infancy through sequencing revealing G342D mutation in STAT3 gene was initiated in the above mentioned treatment regimen; she was followed for 6 months, and to date, none recurrent pulmonary or skin infection was noticed. Furthermore, a considerable improvement in skin lesions was observed. A combination of anti-IgE and longitudinal use of inhaled antibiotics seems well-founded in Job syndrome

Evaluation of admission chest X-ray findings in patients with respiratory infection during the COVID-19 pandemic

AIMS: To evaluate the prevalence of X-ray findings in hospitalized patients requiring hospitalization with suspected Coronavirus disease 2019 (COVID-19) infection and potential differences in the laboratory values and clinical outcomes related to the presence of abnormal chest X-ray (CXR) findings. METHODS: RESULTS: CONCLUSION: CXR is a routine examination in all patients with symptoms of lower respiratory tract disease and its findings relate to in-hospital mortality and Pa

Post-COVID-19 interstitial lung disease: Insights from a machine learning radiographic model

IntroductionPost-acute sequelae of COVID-19 seem to be an emerging global crisis. Machine learning radiographic models have great potential for meticulous evaluation of post-COVID-19 interstitial lung disease (ILD).MethodsIn this multicenter, retrospective study, we included consecutive patients that had been evaluated 3 months following severe acute respiratory syndrome coronavirus 2 infection between 01/02/2021 and 12/5/2022. High-resolution computed tomography was evaluated through Imbio Lung Texture Analysis 2.1.ResultsTwo hundred thirty-two (n = 232) patients were analyzed. FVC% predicted was ≥80, between 60 and 79 and <60 in 74.2% (n = 172), 21.1% (n = 49), and 4.7% (n = 11) of the cohort, respectively. DLCO% predicted was ≥80, between 60 and 79 and <60 in 69.4% (n = 161), 15.5% (n = 36), and 15.1% (n = 35), respectively. Extent of ground glass opacities was ≥30% in 4.3% of patients (n = 10), between 5 and 29% in 48.7% of patients (n = 113) and <5% in 47.0% of patients (n = 109). The extent of reticulation was ≥30%, 5–29% and <5% in 1.3% (n = 3), 24.1% (n = 56), and 74.6% (n = 173) of the cohort, respectively. Patients (n = 13, 5.6%) with fibrotic lung disease and persistent functional impairment at the 6-month follow-up received antifibrotics and presented with an absolute change of +10.3 (p = 0.01) and +14.6 (p = 0.01) in FVC% predicted at 3 and 6 months after the initiation of antifibrotic.ConclusionPost-COVID-19-ILD represents an emerging entity. A substantial minority of patients presents with fibrotic lung disease and might experience benefit from antifibrotic initiation at the time point that fibrotic-like changes are “immature.” Machine learning radiographic models could be of major significance for accurate radiographic evaluation and subsequently for the guidance of therapeutic approaches

Association of chronic obstructive pylmonary disease pathogenesis with polymorphisms and haplotypes of the endothelin-1 gene

Chronic Obstructive Pulmonary Disease (COPD) is an entity with many phenotypes, different pathophysiological characteristics, that exhibits in all cases a diminished FEV to FVC ratio. Nevertheless, recent studies show that there is not a strict relationship between airway inflammation and FEV1 decline in patients with COPD, contrasting a 40 year literature [Roy K, et al 2009]. The aim of all recent studies dealing with genetics in COPD is the distinction of different phenotypes and the elucidation of the genetic profile of the disease. COPD is a multi-gene disorder, and knowing that it is composited by many phenotypes, one can say that, in the near future, “holistic COPD phenotype” will be unraveled in many distinguished phenotypes, leading to a personalized and patient-targeted diagnostic and therapeutic approach. The current study contributed in: a. Designing primers and probes for the +134InsA/DelA polymorphism that could clearly distinguish both 3A and 4A alleles. b. Exhibiting that both +134InsA/DelA & G198T polymorphisms are implicated in COPD progression and severity (as defined by FEV1 values). At the same time, we managed to highlight the genetic profile of the susceptible to smoke smoker, associating haplotypes and polymorphisms of Endothelin-1 (ET-1) gene (+134InsA/DelA & G198T) with COPD. c. Showing the implication of the +134InsA/DelA polymorphism with static lung hyperinflation and increased airway resistance. d. Revealing the association of +134InsA/DelA & G198T polymorphisms with exercise tolerance. According to our knowledge, the current study is the first in the literature showing association of ET-1 gene with lung function deterioration.Η ΧΑΠ είναι νόσος με πολλούς φαινοτύπους, με παθοφυσιολογία που διαφέρει σε κάθε έναν από αυτούς, με κοινό χαρακτηριστικό την πτώση του λόγου FEV1/FVC. Παρόλα ταύτα πολύ πρόσφατες μελέτες δείχνουν πως δεν υπάρχει σαφής συσχέτιση μεταξύ της προσεκτικά μετρούμενης φλεγμονής στους αεραγωγούς και της πτώσης της FEV1 σε ομάδες ασθενών με ΧΑΠ, κάτι που δυνητικά μπορεί να ανατρέψει βιβλιογραφία 40 ετών [Roy K, et al 2009]. Στόχος όλων των γενετικών μελετών στη ΧΑΠ είναι ο διαχωρισμός των φαινοτύπων και η δημιουργία του γενετικού προφίλ της νόσου. Γνωρίζοντας πως η ΧΑΠ είναι πολυγονιδιακή νόσος και σε συνδυασμό με τη μεγάλη ποικιλότητα των φαινοτύπων της, οι γενετικές αλλοιώσεις φαίνεται πως οδηγούν σε διαφορετικό φαινότυπο, που τώρα ολιστικά ορίζεται ως νόσος «ΧΑΠ», αλλά σίγουρα στο εγγύς μέλλον θα διαχωριστεί σε επιμέρους ομάδες στα πλαίσια μιας πιο αποτελεσματικής και εξατομικευμένης θεραπευτικής προσέγγισης. Η παρούσα μελέτη μεταξύ άλλων, συνέβαλε στα εξής: α. Σχεδιασμός και αξιολόγηση εκκινητών και ιχνηθέτων για τον +134InsA/DelA πολυμορφισμό, με υψηλή διακριτική ικανότητα έναντι των αλληλίων 3Α και 4Α. β. Ανέδειξε την εμπλοκή των πολυμορφισμών+134InsA/DelA και G198T στην εμφάνιση αλλά και στη βαρύτητα της ΧΑΠ (όπως αυτή αξιολογείται με την FEV1), ενώ σκιαγραφήθηκε και το γενετικό προφίλ του ευαίσθητου στον καπνό του τσιγάρου καπνιστή, με λεπτομερή συσχέτιση των απλοτύπων των πολυμορφισμών της ΕΤ-1 που εμπλέκονται στη ΧΑΠ. γ. Ανέδειξε πιθανή εμπλοκή του +134InsA/DelA πολυμορφισμού στην στατική υπερδιάταση και στις αυξημένες αντιστάσεις στη ροή του αέρα στους πνεύμονες δ. Σκιαγράφησε τη σχέση των +134InsA/DelA και G198T με την ανοχή στην άσκηση και συνέκρινε τα αποτελέσματα αυτά με όσα ήδη υπάρχουν στη βιβλιογραφία. Η παρούσα μελέτη είναι η πρώτη που συσχετίζει πολυμορφισμούς με πολλαπλές αξιολογήσεις της αναπνευστικής λειτουργίας, πέραν της FEV1, κάτι που σκιαγραφεί με λεπτομέρεια το φαινότυπο της ΧΑΠ

Pulmonary adverse events following immune checkpoint inhibitors

Purpose of review: Immune checkpoint inhibitors (ICIs) have rapidly become a mainstay of cancer treatment. However, immune modulation resulting from checkpoint inhibition can cause inflammation in any organ system, with pneumonitis being one of the most severe immune-related adverse events (irAEs). Here, we review the most recent literature on pulmonary adverse events following ICIs. Recent findings: Several systematic reviews and meta-analyses of data from trials of antiprogrammed death-1 (PD-1; nivolumab, pembrolizumab), anti-PD-ligand-1 (PD-L1; atezolizumab, avelumab, durvalumab) and anticytotoxic T lymphocyte antigen-4 (CTLA-4; ipilimumab or tremelimumab) in patients with advanced cancer have explored the relative risk and incidence of lung toxicity among different tumor types and therapeutic regimens. They have showed that the incidence of all-grade (1-4) and high-grade (3-4) pneumonitis is significantly higher in nonsmall cell lung cancer (NSCLC) compared with other tumor types. In addition, they have demonstrated that immunotherapy, especially monoimmunotherapy, has a significantly lower risk of irAEs compared to immune-chemotherapy. Treatment for lung cancer, preexisting interstitial lung disease, smoking history and male sex appear to increase the risk for ICI-related pneumonitis. Summary: Lung toxicity is an uncommon but potentially severe and even fatal complication of ICIs. Timely recognition is critically important but challenging, particularly in patients with lung cancer wherein drug toxicity can mimic disease progression or recurrence

High-flow versus conventional nasal cannula oxygen supplementation therapy and risk of hypoxia in gastrointestinal endoscopies: Α systematic review and meta-analysis.

BACKGROUND AND AIMS Gastrointestinal endoscopy (GIE) represents a mainstay diagnostic and therapeutic procedure in modern clinical practice. Hypoxemia and respiratory failure during endoscopy constitute major complications and concerns for endoscopists. Emerging evidence supports the utilization of high-flow nasal cannula (HFNC) over conventional nasal cannula (CNC) for oxygen supplementation and avoidance of hypoxemia. Aim of our study was to compare the risk of hypoxemia in patients undergoing GIE with HFNC versus CNC oxygen supplementation recruited by randomized controlled trials (RCTs). METHODS We conducted an electronic literature search in established medical databases i.e. PubMed, EMBASE and Cochrane to identify RCTs investigating the abovementioned association. The enrolled studies were evaluated for risk of bias and inserted into a random effects model for meta-analysis; sub-group analyses and publication bias were also assessed. RESULTS Out of 271 initially retrieved articles, five RCTs were eligible for meta-analysis with totally 2656 recruited patients (1299 HFNC and 1357 CNC). A statistically significant reduced relative risk (RR) of hypoxemia among HFNC patients was revealed (RR=0.18, CI95%: 0.05-0.61), whilst with high heterogeneity (I2:79.94%, p<0.01). Patients undergoing upper GIE with HFNC displayed a significantly lower risk of hypoxemia (96%, p<0.001, I2:15.59%), even after exclusion of endoscopic retrograde cholangiopancreatography (ERCP) cases (RR:0.03, CI95%: 0.01-0.21), albeit with higher heterogeneity (I2:41.82%). Contrariwise, colonoscopy with HFNC did not yield a statistically significant RR reduction (p=0.14). CONCLUSION Patients undergoing upper GIE with HFNC oxygen experience significantly less hypoxemia burden than CNC counterparts. Further research is warranted to establish this emerging association to target optimal safety during endoscopy

High-flow versus conventional nasal cannula oxygen supplementation therapy and risk of hypoxia in gastrointestinal endoscopies: a systematic review and meta-analysis

Background Gastrointestinal endoscopy (GIE) represents a mainstay diagnostic and therapeutic procedure in clinical practice. Hypoxemia constitutes a major complication for endoscopists. Emerging evidence supports the utilization of high-flow nasal cannula (HFNC) over conventional nasal cannula (CNC) for avoidance of hypoxemia. Our aim was to compare the hypoxemia risk in patients undergoing GIE with HFNC versus CNC oxygen supplementation recruited by randomized controlled trials (RCTs). Methods We searched in medical databases PubMed, EMBASE and Cochrane to identify RCTs investigating the abovementioned association. Enrolled studies were evaluated for risk of bias and inserted into a random effects model for meta-analysis; subgroup analyses and publication bias were also assessed. Results Out of 271 articles, five RCTs were eligible (patients n=2656, HFNC 1299 and CNC 1357). A statistically significant reduced relative risk (RR) of hypoxemia among HFNC patients was revealed (RR=0.18, CI95%: 0.05-0.61), whilst with high heterogeneity (I2:79.94%, p&lt;0.01). Patients undergoing upper GIE with HFNC displayed a significantly lower hypoxemia risk (96%, p&lt;0.001, I2:15.59%), even after exclusion of endoscopic retrograde cholangiopancreatography cases (RR:0.03, CI95%:0.01-0.21), albeit with higher heterogeneity (I2:41.82%). Conclusion Patients undergoing upper GIE with HFNC experience significantly less hypoxemia burden than CNC counterparts. Further research is warranted to target optimal safety during endoscopy