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

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

The role of increased red cell distribution width as a negative prognostic marker in patients with COPD

Introduction: Chronic obstructive pulmonary disease (COPD) remains a major burden with no clinically applicable biomarkers. Aim: To investigate the association of Red cell Distribution Width (RDW) values on admission with previous hospitalizations, need of non-invasive mechanical ventilation (NIMV) and long term oxygen therapy (LTOT) in patients with COPD. Methods: Patients with AECOPD admitted to our department during 2018 were included in the study. Results: One hundred sixty patients were enrolled (M/F 95/65, median age 71.00 years, mean FEV1 +/- SD = 46.6 +/- 28.9). Median RDW was significantly higher for patients in need of NIMV (14.8, 95% CI: 14.2 to 15.6) than patients not in need of NIMV (13.5, 95% CI: 13.2 to 13.8) (p < 0.001). Median RDW was significantly higher for patients in need of LTOT (14.2, 95% CI: 13.7 to 14.6) compared to patients not receiving LTOT (13.2, 95% CI: 12.5 to 13.6) (p = 0.001). Patients with hospitalization during the last 12 months had increased RDW values compared to patients with no hospitalizations [median RDW 14.3, (95% CI: 13.5 to 14.9) versus median RDW 13.5, (95% CI: 13.1 to 13.9)](p = 0.001). Conclusion: Patients with COPD in need of LTOT, NIMV or patients with previous hospitalizations presented with increased RDW values. Increased RDW values could serve as a negative prognostic marker in patients with COPD

Low penetrance of antibiotics in the epithelial lining fluid. The role of inhaled antibiotics in patients with bronchiectasis

Plasma drug concentrations, spectrum of antibacterial activity and minimum inhibitory concentration (MIC) had been widely considered as markers of the efficacy of antibiotics. Nonetheless, in several cases, antibiotics characterized by all these features were ineffective for the treatment of respiratory tract infections. A typical paradigm represented the case of patients with bronchiectasis who do not always benefit from antibiotics and thus experiencing increased sputum production, worse quality of life, more rapid forced expiratory volume in the first second (FEV1) decline, more frequent exacerbations and increased mortality rates, especially those with Pseudomonas aeruginosa (P. aeruginosa) chronic infection. Subsequently, penetrance of antibiotics in the epithelial lining fluid has gradually emerged as another key factor for the outcome of antibiotic treatment. Given that a plethora of antibiotics presented with poor or intermediate penetrance in the epithelial lining fluid, inhaled antibiotics targeting directly the site of infection emerged as a new option for patients with respiratory disorders including patients with bronchiectasis. This review article intends to summarize the current state of knowledge for the penetrance of antibiotics in the epithelial lining fluid and present results from clinical trials of inhaled antibiotics in patients with bronchiectasis of etiology other than cystic fibrosis

Metabolic Disorders in Chronic Lung Diseases

Chronic lung diseases represent complex diseases with gradually increasing incidence, characterized by significant medical and financial burden for both patients and relatives. Their increasing incidence and complexity render a comprehensive, multidisciplinary, and personalized approach critically important. This approach includes the assessment of comorbid conditions including metabolic dysfunctions. Several lines of evidence show that metabolic comorbidities, including diabetes mellitus, dyslipidemia, osteoporosis, vitamin D deficiency, and thyroid dysfunction have a significant impact on symptoms, quality of life, management, economic burden, and disease mortality. Most recently, novel pathogenetic pathways and potential therapeutic targets have been identified through large-scale studies of metabolites, called metabolomics. This review article aims to summarize the current state of knowledge on the prevalence of metabolic comorbidities in chronic lung diseases, highlight their impact on disease clinical course, delineate mechanistic links, and report future perspectives on the role of metabolites as disease modifiers and therapeutic targets

Pulmonary embolism and abdominal pain in a young patient: A case report of a rare clinical entity

A 20-year-old man with reduced mobility, from a car accident, presented to the Emergency Department of our hospital due to progressive dyspnea and heart palpitations the lasted three days. A bedside cardiac ultrasound and a Computed Tomography Pulmonary Angiogram (CTPA) were immediately performed, revealing strain of the right ventricle and pulmonary embolism (PE). The patient subsequently complained about abdominal pain and a Computed Tomography of the Abdominal Aorta (CTAO) revealed arterial embolism in the renal and splenic circulations, along with the right common femoral artery. Phlebography of inferior limbs exhibited deep vein thrombosis in the left popliteal vein. A percutaneous suction thrombectomy had been performed successfully. The patient underwent a transesophageal echocardiography with agitated saline test that revealed a patent foramen ovale (PFO), a diagnosis which explained the paradoxical embolism in both arterial and venous circulations. Paradoxical embolism is quite uncommon and should not be ignored in cases with indications of arterial embolism after PE