Livedoid vasculopathy is a rare, chronic thrombo-occlusive disease characterized by recurrent livedoid skin changes, atrophic white plaques, and ulceration. It most commonly affects the dermal vessels of the distal lower extremities, ankles, and feet, bilaterally. While it is commonly associated with hypercoagulable states, systemic autoimmune diseases, and malignancies, we report a case of a previously healthy 31-year-old female with a history of moderate COVID-19 infection during pregnancy, two years before the onset of livedoid skin changes. The patient presented to a hematologist with a two-month history of erythematous to violaceous macules and patches on the dorsum of the feet, ankles, and lower extremities, along with mild ankle swelling. Multiple punch biopsies of the affected skin demonstrated thrombi, fibrinoid vascular changes, fibrosis, and hyalinization of the vessel walls - findings consistent with vasculopathy. Initial treatment included subcutaneous low-molecule weight heparin (dalteparin). This case highlights the importance of early recognition, the performance of a skin biopsy with subsequent histopathologic diagnosis, and appropriate management of this rare, but potentially debilitating condition.

Background: Acute bronchiolitis is one of the most common lower respiratory tract infections in early childhood and is frequently associated with recurrent wheezing and later development of asthma. Identifying biomarkers related to airway epithelial injury and disease severity may improve risk stratification. Materials and Methods: A total of 155 children aged 1-36 months who presented with their first episode of wheezing were enrolled. Clinical data and bronchiolitis symptom scores were recorded at admission. Serum levels of CC16, surfactant protein-D (SP-D), YKL-40, and isoprostane were measured. Patients were followed for one year to assess recurrence of wheezing. Results: According to symptom scores, 81 patients had mild and 74 had moderate bronchiolitis; no severe cases were observed. The distribution of bronchiolitis severity differed significantly between recurrent and non-recurrent wheezing groups. Serum YKL-40 levels were significantly correlated with disease severity (p < 0.05), and the effect size analysis indicated a moderate effect. SP-D levels showed a non-significant trend with severity (p = 0.17). No significant associations were observed for CC16 or isoprostane. Conclusions: Serum YKL-40 may be a potential biomarker reflecting disease severity in children with acute bronchiolitis. Further longitudinal studies are needed to evaluate the prognostic value of epithelial injury markers for recurrent wheezing and asthma development.

Non-cystic fibrosis bronchiectasis (NCFB) is a heterogeneous chronic airway disease characterized by irreversible bronchial dilatation, impaired mucociliary clearance, and recurrent infection. Historically, research and clinical practice have focused mainly on bacteria, particularly Pseudomonas aeruginosa, as major drivers of exacerbations and disease progression, whereas the contribution of fungi has received far less attention. Over the last decade, evidence from mycobiome studies, large registries, and prospective cohorts has increasingly identified Aspergillus spp. as clinically relevant contributors in a substantial subset of patients with bronchiectasis. Data from the European Bronchiectasis Registry (EMBARC) indicate that approximately one quarter of patients exhibit Aspergillus-related immunological signals, including allergic bronchopulmonary aspergillosis (ABPA), Aspergillus sensitization, and elevated Aspergillus-specific IgG, and that these phenotypes are associated with more severe disease and worse clinical outcomes. Mechanistic studies further suggest that Aspergillus-related disease in bronchiectasis is underpinned by distinct molecular and immunological programs involving epithelial dysfunction, impaired mucociliary clearance, innate fungal sensing, inflammasome-related signaling, and divergent type-2 versus non-type-2 inflammatory responses. In parallel, mycobiome and multi-biome studies indicate that Aspergillus should be interpreted within a broader airway interactome shaped by cross-kingdom relationships with bacterial pathogens and by host immune tone. In this review, we synthesize current evidence on the epidemiology, molecular pathobiology, inflammatory endotypes, biomarker profiles, clinical-radiologic spectrum, and therapeutic implications of Aspergillus in bronchiectasis. Current evidence suggests that Aspergillus-related findings in bronchiectasis should be interpreted within a structured clinical, radiological, microbiological, and immunological framework rather than considered solely as isolated culture results. However, most data remain observational or extrapolated from related airway diseases, and bronchiectasis-specific interventional evidence is limited. A cautious biomarker-informed approach may help standardize phenotyping, identify patients requiring closer follow-up, and define priorities for future prospective trials.

We report a man in his 90s who was referred to our pleural clinic with a left-sided moderate large pleural effusion. Despite the absence of systemic inflammatory features and normal serum inflammatory markers, pleural fluid microbiology was diagnostic of empyema. Initially, the patient was treated with a short course of antibiotics, as the clinical impression of the multidisciplinary team was that the presentation did not align with typical systemic inflammatory features of empyema. However, recurrence of the effusion after drainage led to further bacterial growth from repeat samples, ultimately clarifying the diagnosis. This illustrates how an attenuated systemic inflammatory response, consistent with immunosenescence, can complicate the diagnosis and management of pleural infection in elderly patients.

Children with early-onset structural lung damage or lung and airway malformations - congenital or acquired - are highly susceptible to recurrent respiratory tract infections, leading to frequent hospitalisations, impaired lung function and reduced quality of life. Although azithromycin maintenance therapy is commonly used in this population, robust clinical evidence supporting its efficacy is lacking.

Pulmonary hydatid cysts (PHCs), caused by Echinococcus granulosus, remain endemic in livestock-raising regions worldwide. Surgical removal of cysts is the mainstay of treatment, with the aim of eliminating the parasite while preserving the lung parenchyma and minimizing postoperative complications and recurrences. This article synthesizes the latest evidence on the optimal surgical management of PHCs, emphasizing practical recommendations for operating surgeons based on current best practices and the highest level of available evidence.

Primary ciliary dyskinesia (PCD) is a rare disorder characterized by dysfunction of motile cilia and chronic progressive respiratory disease, mianly inherited in an autosomal recessive manner. Biallelic variants in dynein axonemal heavy chain 11 (DNAH11) have been reported in association with PCD and are typically associated with normal ciliary ultrastructure. In addition, exon-level copy number variants (CNVs) in DNAH11, particularly duplications, remain poorly characterized and are prone to being overlooked by conventional sequencing workflows. We report a 9-year-old Chinese girl with recurrent lower respiratory tract infections, chronic pansinusitis, and bronchiectasis. Pulmonary function testing showed isolated small-airway dysfunction with preserved FEV1, with nasal nitric oxide (nNO) severely reduced (5.4 nL/min). Trio whole-exome sequencing integrated with read-depth CNV analysis detected novel compound heterozygous DNAH11 variants: a paternal inherited missense variant (c.6556A>C, p.Thr2186Pro) and a previously unreported maternally derived intragenic duplication encompassing exons 30-54. The missense variant was confirmed by Sanger sequencing, and the exon-level dosage gain was confirmed by qPCR. This case expands the spectrum of DNAH11 variants and highlights the importance of incorporating CNV detection into exome-based diagnostic process for children with a highly suggestive PCD phenotype.

AUTS2-related intellectual developmental disorder type 26 is a rare neurodevelopmental condition characterised by variable clinical manifestations, including developmental delay, dysmorphic features, and behavioural abnormalities, which may contribute to delayed diagnosis. We report the case of a two and a half years old boy born to consanguineous parents who presented with global developmental delay, poor weight gain, feeding difficulties, recurrent choking episodes, and multiple dysmorphic features. Physical examination demonstrated arched eyebrows, broad nasal bridge, upturned philtrum, strabismus, mild lower limb hypotonia, unilateral cryptorchidism, and hypospadias. Delayed motor and speech milestones with autistic features were also observed. The patient additionally had recurrent upper respiratory tract infections and adenotonsillar hypertrophy requiring adenotonsillectomy. Given the multisystem involvement and developmental concerns, a genetic disorder was suspected. Whole-exome sequencing identified a heterozygous pathogenic variant in the AUTS2 gene, confirming the diagnosis of intellectual developmental disorder, autosomal dominant 26 (MRD26). To our knowledge, this represents the first reported case of MRD26 from the Arabian Peninsula and the Gulf Cooperation Council region. This case highlights the importance of maintaining a high index of suspicion in children presenting with developmental delay and dysmorphic features. It also contributes to the AUTS2 syndrome phenotypic database by adding a patient from a previously under-represented ethnic and geographical background, by documenting an autosomal dominant variant identified within a consanguineous pedigree, and by drawing attention to a constellation of co-occurring hypospadias, unilateral cryptorchidism, and adenotonsillar hypertrophy requiring surgery, which appears infrequently reported in the existing AUTS2 literature. Early diagnosis may facilitate timely multidisciplinary management, appropriate developmental support, and family counselling.

Introduction:Ataxia-telangiectasia (AT) is an autosomal recessive neurodegenerative disease characterized by progressive cerebellar ataxia, oculocutaneous telangiectasia, humoral and cellular immunodeficiency, sensitivity to ionizing radiation, and a tendency to malignancy. The aim of this study was to evaluate the demographic characteristics, immunodeficiency status, laboratory findings, and prognosis of children with AT based on a single-center experience.Patients and Methods:Nineteen pediatric patients diagnosed with AT between 2003 and 2024 were retrospectively analyzed at a single tertiary center.Results:The study included 11 male and 8 female patients, with a mean follow-up duration of 72.6 ± 41.3 months. The mean ages at symptom onset and diagnosis were 30.47 ± 23.7 months and 82.32 ± 26.5 months, respectively. The most common presenting symptoms were gait instability, ocular telangiectasia, and recurrent respiratory tract infections. Immunological evaluation revealed IgA deficiency in 57.9% of the patients and IgG deficiency in 15.8%. A hyper-IgM AT phenotype was identified in two patients (10.5%). During follow-up, bronchiectasis and hepatosteatosis developed in 26.3% of the patients. One patient (5.2%) developed type 2 diabetes mellitus, and malignancy occurred in three patients (15.7%). Overall, 6 patients (31.5%) died during the follow-up period.Conclusion:Ataxia-telangiectasia is a rare multisystem disorder associated with significant morbidity and mortality. Early diagnosis, comprehensive multidisciplinary follow-up, regular malignancy surveillance, and preventive strategies for recurrent sinopulmonary infections are essential for improving clinical outcomes and prognosis in affected patients.

Post-infectious cough (PIC) refers to a type of persistent cough triggered by respiratory tract infections that remains unresolved after the initial infection is controlled. It significantly impacts daily life, work, and overall quality of life. This study aims to evaluate the clinical efficacy and safety of the Shufeng Huashi (SFHS) formula, a Traditional Chinese Medicine (TCM) compound, in patients with PIC.