Search Results (2,810)

Background: Postnatal corticosteroids (CS) improve respiratory outcomes in preterm infants, but effects on growth and neurodevelopment remain incompletely understood. Methods: This third instalment of a narrative review series builds on physiologic principles to examine systemic CS consequences. Main Findings: We explore the interplay between growth restriction, hypoxia, and neurodevelopmental vulnerability, discussing brain imaging, metabolic disruptions, and HPA axis suppression. Conclusion: This review advocates for a holistic, physiology-informed approach to optimize outcomes by integrating nutritional vulnerability with cardiorespiratory status. Full article

Background: Corticosteroid injections provide short-term relief for chronic subacromial bursitis but are associated with high recurrence rates. This study investigates the efficacy of a mobile health-supported home-based resistance exercise program compared with exercise education in patients with chronic recurrent subacromial bursitis after ultrasound-guided corticosteroid injections. Methods: Participants with chronic subacromial bursitis were assigned via computer-generated block randomization to either an intervention group receiving ultrasound-guided corticosteroid injections followed by a 12-week home-based exercise program (50 min strengthening and resistance/session, 5 days per week) supported via instant messaging applications, or a control group receiving the same injection followed by printed educational materials covering the same exercise protocol. Shoulder Pain and Disability Index (SPADI) scores, Visual Analog Scale (VAS) pain scores and active pain-free range of motion (ROM) were evaluated by a blinded assessor at weeks 4 and 12. Between-group comparisons were analyzed using two-way ANOVA after confirming normality and homoscedasticity. Results: Fifty-three patients (mean age: 55.6 ± 10.5 years; 47.2% female) were randomized to the intervention (n = 27) or control (n = 26) groups. Significant interaction effects were identified for SPADI (p = 0.040) and ROM (abduction: p = 0.036/ flexion: p = 0.032). Post hoc analysis revealed that the intervention group exhibited a significantly greater reduction in SPADI scores (p = 0.007, d = 0.72) and greater increase in abduction ROM (p = 0.004, d = 0.84) at 12 weeks; both gains surpassed the MCID. Conclusions: A mobile health-supported home-based resistance exercise program can significantly extend the benefits of corticosteroid injections in patients with chronic subacromial bursitis. Trial Registration: NCT06220643, registered 14 December 2023. Full article

Background and Objectives: Osteonecrosis of the jaw (ONJ) occurring after infection with SARS-CoV-2 has emerged as an increasingly reported complication in the post-COVID-19 era. Post-COVID-19 osteonecrosis of the jaw (PC-ONJ) has been described in association with both COVID-19-associated mucormycosis (CAM) and non-fungal phenotypes. This narrative review aims to synthesize and critically analyze the available evidence regarding terminology and classification, epidemiology and risk factors, pathophysiological mechanisms, clinical and imaging characteristics, diagnostic challenges, and management strategies relevant to oral and maxillofacial surgery practice. Materials and Methods: An extensive literature search was conducted in the PubMed/MEDLINE, Scopus, Web of Science, ScienceDirect, and Google Scholar databases. The search targeted peer-reviewed publications published between 2020 and 2025, reflecting the post-pandemic emergence of this clinical spectrum. Original studies, systematic and narrative reviews, multicenter case series, consensus guidelines, and well-documented case reports were considered. Results: Available data, largely derived from case reports and small series, demonstrate a predominance of maxillary involvement and frequent association with diabetes mellitus and systemic corticosteroid therapy. Proposed mechanisms include COVID-19-associated endothelial dysfunction, microvascular thrombosis, immune dysregulation, metabolic imbalance, and treatment-related effects. Clinically, patients may present with persistent orofacial pain, tooth mobility, exposed or probeable bone, and frequent sinonasal extension, with symptoms sometimes preceding bone exposure. Diagnostic challenges arise from the overlap with medication-related osteonecrosis of the jaw (MRONJ), osteoradionecrosis (ORN), and chronic osteomyelitis. Imaging is essential for assessing disease extent but remains insufficient for etiologic differentiation, making histopathological examination and targeted microbiological investigations necessary, particularly to exclude invasive fungal infection. Conclusions: Management must be etiology-driven. CAM requires urgent antifungal therapy combined with surgical debridement, whereas non-fungal forms are generally managed with conservative surgery and appropriate antimicrobial stewardship. Standardized diagnostic criteria and prospective multicenter studies are needed to reduce nosological ambiguity and optimize clinical decision-making in this emerging post-viral condition. Full article

Background: Duchenne muscular dystrophy (DMD) is a rare, disabling, and life-threatening X-linked recessive disorder caused by mutations in the dystrophin gene. The current standard of care is treatment with corticosteroids, which aim to decrease inflammation-induced muscle damage and delay disease progression. Here, we aim to describe clinical, genetic, and diagnostic characteristics and evaluate current management practices of DMD patients in the Kingdom of Saudi Arabia (KSA). Methods: This was an ambispective (prospective and retrospective) observational multicenter study evaluating characteristics of patients aged 1–14 years with genetically confirmed DMD in the KSA. The variables of interest were demographics, genetic mutations, clinical characteristics, and initial management. The relationship between the age at diagnosis, initial management plan (standard of care), and age at initiation of treatment on disease outcomes was also evaluated. Results: A total of 226 patients (181 in the retrospective part and 45 in the prospective part) were enrolled. The most common type of genetic mutation was large deletions (134 patients, 59.3%). The median age of first symptom was 2.7 years (IQR: 2.0–4.6 years) and the median age at diagnosis was 7.0 years (IQR: 4.8–8.5 years). Among these patients, the most common initial symptoms were difficulty in walking (87.7%) and waddling gait (41%). The initial management plan for DMD patients involved medication (75.6%) and physical therapy (71.0%). The most frequently prescribed initial medications were vitamin D (82%) and corticosteroids (62.3%). In total, 6/226 patients (2.6%) received ataluren; they all had identified nonsense mutations. The median age of corticosteroid initiation was 7.1 years (IQR: 5.7–8.7). The median age at loss of ambulation (LoA) was 9.8 years (IQR: 8.0–11.4 years) in the non-treated patients; it was 10.1 years (IQR: 9.3–11.2 years) in the steroid-only group and 10.8 years (10.8, 10.8) in the combined ataluren and steroid treatment group. Discussion: Age of diagnosis and age of treatment initiation is relatively late in the KSA. However, early diagnosis and early treatment onset is associated with better clinical outcomes, mainly a delay in LoA. Therefore, there is an urgent need for raising awareness and enhancing early screening in the KSA. Full article

Background: Diffuse alveolar haemorrhage (DAH) is a rare but potentially life-threatening pulmonary complication of systemic autoimmune diseases. Although interstitial lung disease (ILD) is a hallmark of antisynthetase syndrome (ASyS), DAH has been only exceptionally reported in this setting. Methods: We present two patients with ASyS who developed DAH confirmed by bronchoalveolar lavage. Results: The first case involved a 52-year-old woman initially diagnosed with rheumatoid arthritis, later reclassified as rheumatoid arthritis–ASyS overlap, who developed DAH in the context of progressive ILD. The second case concerned a 37-year-old man with newly diagnosed ASyS who presented with recurrent DAH early in the disease course. Both patients required high-dose corticosteroids, followed by escalation of immunosuppressive therapy with rituximab or mycophenolate mofetil, resulting in clinical and radiological improvement. These cases illustrate the diagnostic challenges posed by DAH in ASyS, particularly due to overlapping features with infection and ILD exacerbation. They also highlight the importance of early bronchoscopy and timely intensification of immunosuppression. Conclusions: Increased awareness of DAH as a rare manifestation of ASyS may facilitate earlier recognition and improve outcomes in patients presenting with acute respiratory deterioration, anaemia, or haemoptysis. Full article

Background: Brain radiation necrosis is a side effect of radiotherapy that can occur months, or even years, after the end of treatment. From an anatomical–pathological perspective, it is characterized by avascular damage, demyelination, and necrosis. Methods: We present a case of a patient with breast cancer cT2N1M0 and multiple brain metastases occurring at 2 years after diagnosis, who was treated with whole-brain radiotherapy (WBRT) and Stereotactic Radiotherapy (SRT) for tumor progression. Dynamic imaging revealed right parietal post-therapeutic changes in aggravation, requiring differential diagnosis between tumor progression (TP) and brain radionecrosis (BRN). Results: Brain radionecrosis and tumor progression are difficult to differentiate due to their similar radiological and clinical characteristics. MRI perfusion plays an important role in differentiating the two entities. Conclusions: Differentiating radiation necrosis from a recurrent tumor is crucial for appropriate treatment. Medical management includes corticosteroids as first-line treatment, after which bevacizumab is administered as secondary therapy. Full article

Lumbar spinal stenosis (LSS) is caused by multiple degenerative changes including the hypertrophy of the ligamentum flavum (LFH). Inflammation and fibrosis contribute to LFH and glucocorticoid drugs (GCDs) are generally used to manage LSS symptoms. However, a thorough understanding of the molecular mechanisms exerted by GCD in ligamentum flavum (LF) cells remains incomplete. Primary human LF cells were isolated from surgical specimens and stimulated with pro-inflammatory agents (IL-1α, IL-1β, LPS) or the profibrotic cytokine TGFβ1, in the presence or absence of dexamethasone. Gene and protein expression levels of inflammatory, fibrotic, and ossification-related markers were analysed using RT-qPCR and Western blotting. Dexamethasone significantly suppressed the expression of key pro-inflammatory, fibrotic, and ossification markers (IL-6, COX2, COL3A1, MMPs, TNFRSF11b) in both acute and prolonged models of LF inflammation. However, under TGFβ1 stimulation, dexamethasone attenuated inflammatory gene expression but failed to reduce the expression of major fibrosis-associated genes, such as COL3A1, bFGF, and POSTN. Dexamethasone effectively suppresses inflammation-mediated fibrosis in LF-derived cells, indicating its potential to both prevent and reverse LFH progression in the context of LSS. However, its limited efficacy against TGFβ1-driven fibrotic pathways highlights the need for combination therapies targeting both inflammation and fibrosis for more comprehensive management of LFH. These findings support further exploration of corticosteroids as therapeutic agents for hypertrophic ligament disorders. Full article

Background and Clinical Significance: Mucopolysaccharidosis type VI is a rare lysosomal storage disorder due to arylsulfatase B enzyme deficiency, leading to progressive multisystem disease and complex airway. Acute respiratory infections can precipitate airway embarrassment. A structured treatment guideline is currently lacking. We present a 7-year-old MPS VI male with respiratory distress, highlighting the challenges in management. Case Presentation: Case review focusing on clinical presentation, imaging findings, and multidisciplinary decision-making during acute deterioration. A child diagnosed with MPS VI at the age of 3.5 years old, due to low arylsulfatase B enzyme activity and homozygous for c.275C>A p.(Thr92Lys) variant in the ARSB gene. At 7 years of age, he showed the signs of dyspnoea, increased respiratory effort with bilateral crepitations, and noisy breathing. Initial management included facemask oxygen, nebulised adrenaline, corticosteroids, and bronchodilators. Computer tomography scan of the neck and chest showed a complex upper airway, multiple tracheal narrowing, tortuosity, and an extra loop of truncus brachiocephalicus from the arch of the aorta. Potential interventions carried substantial risks due to abnormal airway and multisystem disease. Following extensive multidisciplinary discussion after careful consideration of the significant risks associated with invasive airway interventions, a shared decision was reached with the family to adopt a comfort-focused palliative care approach. Despite the best supportive care, the child unfortunately passed away after 3 months. The family was involved in every decision process and was fully supported. Conclusions: MPS VI is associated with complex airways and multisystem disease. Multidisciplinary decision-making with family is critical to safe and appropriate care. The rarity of the disease, lack of guidelines, complex airways, and multiple comorbidities make management challenging. Full article

Dry eye disease (DED) is a complex, multifactorial, progressive disease that has consequences both for individuals and society. Symptoms reported by patients include discomfort in the eye and periodic blurred vision, while in the broader perspective, the disease is associated with economic burdens and challenges for healthcare systems. Globally, dry eye disease remains a growing problem observed in many countries. It is estimated that symptoms of dry eye syndrome occur in approximately 10 to 20 per cent of people over the age of 40. This prevalence is on the rise, which is associated with both the aging population and increased incidence among younger adults. In this group, factors such as contact lens wear and prolonged use of digital devices are considered to be contributing factors. Further epidemiological studies, conducted in different regions of the world, covering diverse populations and a wide range of age groups, with a particular focus on younger cohorts, may contribute to a more accurate understanding of the prevalence of dry eye disease. There are more and more methods of diagnosing DED. In addition to well-known procedures like the Schirmer test or tear break-up time, there are also methods that focus on the evaluation of the tear film or imaging of the ocular surface. Moreover, usage of artificial intelligence is also playing a significant role in it. However, the key issue in individual cases is introducing the most effective treatment based on combining available substances, including corticosteroids, antibiotics and supplements, which leads to a reduction in inflammation and improvement in visual comfort. Full article

Facial vascularized composite allotransplantation (fVCA) is one of the most complex forms of vascularized composite allotransplantation and requires lifelong immunosuppression to ensure graft survival. Despite significant advances in surgical techniques and postoperative care over the past two decades, the true incidence of cutaneous malignancies in fVCA recipients remains poorly characterized due to the limited number of procedures, heterogeneous immunosuppressive protocols, and relatively short follow-up. This narrative review summarizes current evidence on oncologic risk in facial VCA, focusing on the effects of different immunosuppressive regimens and the challenges posed by the high immunogenicity of skin and mucosa. Available data indicate that malignancies, including cutaneous and other neoplasms, occur in approximately 10% of recipients, based on heterogeneous case-series data with immunosuppressive therapies largely extrapolated from solid organ transplantation. Calcineurin inhibitors, corticosteroids, and azathioprine are associated with increased oncologic risk, whereas mycophenolate mofetil and mTOR inhibitors may confer a more favorable profile. Overall, fVCA, unlike solid organ transplantation, is a life-enhancing procedure, highlighting the need for tailored immunosuppressive strategies, rigorous dermatologic surveillance, and further research supported by dedicated registries to better define long-term malignancy risk. Full article

Background: Clinical remission has become a realistic treatment goal in severe asthma, but current evidence mostly reports global remission rates without accounting for baseline disease burden. No simple tool exists to quantify baseline severity and estimate an individual patient’s probability of achieving remission under biologic therapy. Methods: This prospective observational study included 93 adults with severe asthma initiating tezepelumab across 14 specialised severe asthma units in Spain. Four baseline domains—poor symptom control (ACT < 20), ≥1 severe exacerbation in the previous 12 months, maintenance oral corticosteroid (OCS) use, and FEV₁ < 80% predicted—were used to construct an empirically weighted composite score (Base4Score) based on the inverse probability of correcting each abnormal domain at 12 months. Strict clinical remission at 12 months was defined as ACT ≥ 20, no severe exacerbations, no maintenance OCS, and FEV₁ ≥ 80%. Logistic regression was used to assess the association between the score and non-remission, adjusting for age, sex, smoking status, T2 phenotype, and biologic-naive status. Results: Of 93 treated patients, 81 had complete baseline data for Base4Score derivation and 77 had complete 12-month data for strict clinical remission analysis. Strict clinical remission was achieved in 16/77 patients (20.8%). Remission rates decreased across increasing baseline score strata: 40.0% for scores < 5, 17.6% for scores 5 to <9, and 12.5% for scores ≥ 9 (linear p-trend = 0.022). Each 1-point increase in the continuous Base4Score was associated with higher adjusted odds of non-remission (OR 1.22; 95% CI 1.00–1.49; p = 0.047), and patients with scores ≥ 9 had approximately sevenfold higher adjusted odds of non-remission than those with scores < 5 (OR 6.77; 95% CI 1.40–32.84; p = 0.018). Conclusions: The Base4Score is a simple, empirically derived baseline severity index that predicts 12-month strict clinical remission in severe asthma treated with tezepelumab. If externally validated, it could help personalise expectations, optimise timing of biologic initiation and guide treat-to-target strategies in severe asthma. Full article

Lichen planus (LP) is a chronic immune-mediated inflammatory disease of unknown etiology affecting the skin and mucous membranes and is frequently associated with comorbid conditions, although data from Swedish populations remain limited. This retrospective population-based case–control study included all registered citizens in Region Jönköping, Sweden, between 2013 and 2022, to examine comorbidities, estimate prevalence and incidence, assess diagnostic validity of ICD-10 coding (L43), and evaluate treatment patterns. Incidence and prevalence were calculated, demographic and treatment characteristics were described, and diagnostic validity was assessed through independent medical record review of 70 randomly selected cases to determine positive predictive value (PPV). Associations between LP and predefined comorbidities were analyzed using binomial logistic regression adjusted for age and sex. Among 361,812 individuals, prevalence was 235.5 and incidence 19.6 per 100,000 inhabitants. The PPV of the LP diagnosis was 78.6%, yielding an adjusted prevalence of 184.9 per 100,000 inhabitants. Over one third of prevalent patients received topical therapy, primarily corticosteroids. LP was significantly associated with thyroid, malignant, metabolic, and autoimmune conditions. LP is relatively uncommon, ICD-10 coding shows acceptable validity, and its association with clinically relevant comorbidities highlights the need for comprehensive patient assessment. Full article

Cardiac sarcoidosis (CS) is a critical and frequently underdiagnosed phenotype of sarcoidosis, characterized by non-caseating granulomatous infiltration of the myocardium. This review synthesizes current knowledge regarding the pathogenesis, diagnosis, and management of CS. The disease manifests with a heterogeneous clinical spectrum ranging from asymptomatic conduction abnormalities to life-threatening ventricular arrhythmias and heart failure. Diagnosis remains challenging due to the patchy distribution of granulomas, which limits the sensitivity of endomyocardial biopsy. Consequently, a multimodal diagnostic approach is essential, integrating advanced imaging modalities such as cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE) and ¹⁸F-fluorodeoxyglucose positron emission tomography (FDG-PET). These tools not only facilitate detection but also enable the differentiation of active inflammation from chronic fibrosis. Histopathological assessment, supported by specific immunophenotyping and electron microscopy, remains the gold standard for confirming diagnosis and excluding mimics like giant cell myocarditis or infectious granulomatous diseases. Management requires a multidisciplinary strategy combining immunosuppressive therapy, primarily corticosteroids and steroid-sparing agents, with guideline-directed cardiac care, including implantable cardioverter-defibrillators for arrhythmia risk stratification. Emerging biomarkers and artificial intelligence-driven imaging analysis promise to further refine risk stratification and therapeutic monitoring, advancing precision medicine in this complex disorder. Full article

Background/Objectives: Severe infusion-related reactions to rituximab are rare; we aim to extend our knowledge about them in children, focusing on rituximab-induced serum sickness (RISS) and anaphylaxis. Methods: We conducted a monocentric retrospective study on children and adolescents who received rituximab. Patients were defined as having RISS if they had fever and at least rash and/or arthralgia, 1 to 30 days following infusion, and without another diagnosis to explain symptoms. Anaphylaxis was defined according to the diagnostic criteria proposed by the World Allergy Organization. Results: 1534 rituximab infusions in 391 patients were analyzed. Seven patients developed RISS; all received rituximab for an autoimmune disease, including four for immune thrombocytopenia (ITP). Six patients had fever, rash, and arthralgia. C-reactive protein or sedimentation rate was increased in all patients, and complement was decreased in 83%. Evolution was favorable within a few days with corticosteroids and/or intravenous immunoglobulins. Rituximab was reinfused in one patient, which resulted in an immediate anaphylactoid reaction. Lower doses of rituximab were less likely to induce RISS. RISS was associated with a greater chance of achieving ITP remission. Seven patients developed anaphylaxis; five successfully received further infusions using desensitization protocols. Conclusions: RISS in children is a severe complication of rituximab infusion. Our study suggests that it may be more frequent in individuals treated for autoimmune conditions, especially ITP. The classical triad of fever, rash, and arthralgia appeared to be frequently present, and biological inflammation and/or low complement can further support the diagnosis. In contrast to anaphylaxis, where rituximab may be safely rechallenged upon desensitization protocol, treatment alternatives should be pursued in patients experiencing RISS, given the higher risk of severe RISS recurrence. Full article

Background/Objectives: The aim of this study is to evaluate whether preoperative intra-articular corticosteroid injections (CSIs) are associated with an increased risk of reoperation following matrix-associated or autologous chondrocyte implantation (MACI/ACI). Secondary aims included comparing reoperation-free survival, patient-reported outcomes (PROMs), and patient acceptable symptom state (PASS) achievement. Methods: A retrospective cohort study was conducted on adults undergoing primary MACI/ACI between 2011 and 2023 at a single academic institution. Patients with documented CSI status and ≥2 years of follow-up were included. Exclusion criteria were prior MACI/ACI, osteochondral allograft transplantation, multi-ligament reconstruction, or inadequate follow-up. Propensity score matching (2:1, no steroid/steroid) based on age, sex, BMI, laterality, procedure type, and prior surgery yielded 138 matched patients (92 no steroid, 48 steroid). The primary outcome was ipsilateral reoperation, analyzed as a binary outcome, with Kaplan–Meier reoperation-free survival and restricted mean survival time (RMST). PROMs and PASS achievement were also assessed. Statistical significance was set at p < 0.05. Results: Baseline characteristics and follow-up (6.55 ± 3.74 vs. 6.73 ± 3.99 years; p = 0.80) were similar. Graft failure rates were identical (4.3% each; p = 1.00). Reoperation occurred in 21.7% of patients without CSI and 23.9% with CSI (p = 0.83). CSI was not associated with reoperation (adjusted OR 2.28; 95% CI 0.54–9.95; p = 0.26). No significant difference in reoperation-free survival or PROMs was observed. Conclusions: Preoperative intra-articular corticosteroid injections were not associated with increased reoperation risk, inferior reoperation-free survival, or worse functional outcomes following MACI/ACI. Full article