Search Results (97)

Background: Bergamo was the most severely affected Italian province at the onset of the 2020 COVID-19 pandemic. The liver transplant (LT) patient population should be among the more sensitized to the concept of health prevention. Long-term data on both perception and outcomes of SARS-CoV-2 vaccination in LT recipients since the COVID-19 vaccine became available in Italy are still lacking. Methods: From May to October 2023, a survey on actively followed LT recipients at our institution was carried on by the local patient’ advocacy (Associazione Amici del Trapianto di Fegato) to define the rate of vaccinated subjects, SARS-CoV-2 infections and self-reported COVID-19-related outcomes. Results: Out of the consecutive 753 adult LT recipients invited to the survey, 356 responded (47.3%) [71% male, 63 years old (20–85), LT performed a mean of 9 years (1–26) before vaccination] and were included in the analysis. All patients received the first vaccine dose between December 2020 and January 2022 (81.7% Cominarty^®, 17.7% Spikevax^®, 0.3% Vaxzevria^® and 0.3% Jcovden^®). In the following years, adherence to the vaccination policy decreased progressively over time: the second, third, fourth, and fifth vaccine doses were administered to 99%, 94%, 72%, and 22% of the LT population by October, 2023. In total, 43 (12%) and 93 (26%) patients reported a COVID-19 episode before and after [13 (7–21) months] the first vaccination, respectively; none of the LT recipients reported a second COVID-19 infection after the following vaccination cycles. Forty-six (13%) reported short-term post-vaccination mild adverse events but none developed either acute or chronic rejection episodes or hospitalization for COVID-19-related symptoms. A total of 64% of LT recipients resulted positive for anti-nucleocapsid serological test in 2023. Conclusions: COVID-19 vaccines are safe and effective in LT recipients, underlining once again the importance of vaccination in this special population at higher risk of complications from communicable infectious diseases. Full article

Background/Objectives: Adult-onset Still’s disease (AOSD) is an autoinflammatory disorder that can be challenging to diagnose and manage. The aim of this study was to analyze retrospective data to provide insights into the clinical presentation, disease activity, and treatment patterns and outcomes of AOSD during routine clinical care prior to the release of new AOSD guidelines. Methods: This retrospective database analysis evaluated adult patients (≥18 years) with a diagnosis of AOSD who engaged in a clinical visit between 1 January 2010 and 31 December 2020. The evaluated outcomes included demographic characteristics, symptoms, disease activity, and treatment. Results: Our study included 120 patients (67 [55.8%] of whom were female) diagnosed with AOSD according to the Yamaguchi criteria at ten German rheumatology centers. The median (quartile [Q] 1, Q3) age was 51 (36, 62) years, and the median (Q1, Q3) time from diagnosis was 9 (4, 11) years. Approximately half (66 [55.0%]) had a polycyclic disease course. The most frequent symptoms at initial diagnosis were arthralgia (105 [87.5%]) and fever (86 [71.7%]), and these symptoms continued for a substantial proportion of patients at the current visit (35 [29.2%] and 22 [18.3%], respectively). High neutrophil and ferritin levels were also common. The mean Still Activity Score, a measure of disease activity, improved from 4.66 at initial diagnosis to 1.97 at the most recent visit. The treatments most frequently used at some point in the disease course were glucocorticoids (118 [98.3%]), interleukin (IL)-1 inhibitors (89 [74.2%]), and methotrexate (85 [70.8%]). The most common current treatments were IL-1 inhibitors (55 [45.8%]), followed by methotrexate (29 [24.2%}) and glucocorticoids (28 [23.3%]). Conclusions: Our cohort of patients with AOSD seen at German rheumatology clinics showed strong improvements in symptoms and disease activity from initial diagnosis, but a high symptom burden remained for some patients. Future studies may be able to build on our data to document the impact of new guidelines on treatment patterns. Full article

Background: The setup of experimental protocols able to preserve the anatomical integrity also in terms of organ microarchitecture is mandatory to ensure result translatability. Also, the maintenance of structural integrity perfectly aligns with the refinement implementation aiming to reduce procedure severity, a key issue in animal studies deemed compulsory from both ethical and legal standpoints. Here we report a detailed description of all peri-operative and post-operative care and clinical evaluation in a surgical rat model to test the efficacy of a catheter functionalized by a peptide coating with antimicrobial and antibiofilm properties, whose efficacy was previously tested in vitro. Methods: We used male and female adult Fischer 344 rats (tot n = 44, n = 22 each sex), which were divided into four experimental groups. Each animal underwent refined surgery for the implantation of a functionalized or standard catheter, depending on the group, and was observed for 7 and 14 days. The surgical refinement strategy was based on the placement of the catheter into the bladder lumen rather than in the urethra. Still in the refinement perspective, ultrasound examination of the bladder was conducted to confirm the in situ position of the medical device at an intermediate time point, 4 or 10 days post-surgery depending on the group, while, at the same time, but also at days 0, 7, or 14 post-surgery, an ultrasound-guided cystocentesis was performed to collect sterile urine. The imaging approach was used in place of metabolic cages to minimize distress to the animals and to ensure reliable and unbiased scientific outcomes. Hematological and biochemical parameters were monitored along the preclinical trial; namely, blood sampling was performed at the beginning (day 0) and at the end of the trial (day 7 or 14 post-surgery depending on the group). Results: Clinical scores and biochemical analyses of all animals did not reveal signs of distress or disease. At the endpoints, histological analyses of urinary bladder displayed a well-preserved anatomical structure of the organ without significant signs of inflammatory infiltration into the urothelium. Conclusions: Our model represents a refined strategy to achieve the scientific goals required by the preclinical setting related to catheter-associated urinary tract infections. In particular, it ensures the preservation of bladder morphology and urothelium microarchitecture, maintaining an adequate level of animal health and welfare while monitoring the onset of urinary tract infections through the sterile collection of urine in long-lasting experiments. Full article

Objective: To investigate the prevalence and clinical spectrum of atypical or non-classical complications in adult-onset Still’s disease (AOSD) beyond macrophage activation syndrome (MAS) and to identify factors linked to their occurrence. Methods: Multicenter cross-sectional study of AODS cases included in the Spanish registry on Still’s disease. Results: This study included 107 patients (67% women), of whom 64 (59.8%) developed non-classical complications. These include macrophage activation syndrome in 9.5%, atypical skin manifestations in 38.8%, cardiac involvement in 22.7% (comprising pericarditis, myocarditis, pulmonary arterial hypertension, and noninfectious endocarditis), pleuritis in 28.9%, transient pulmonary infiltrates in 4%, significant headache in 14.1%, lower abdominal pain with evidence of peritonitis in 8.4%, and secondary amyloidosis in 0.9%. In the multivariate logistic regression analysis, lymphadenopathy (OR 2.85, 95% CI 1.03–7.91, p = 0.044) and the systemic score system (SSC) index (OR 1.86, 95% CI 1.29–2.69, p = 0.001) were independently associated with the development of non-classical clinical manifestations. In contrast, typical exanthema was associated with a reduced risk of these complications (OR 0.32, 95% CI 0.11–0.95, p = 0.041). Conclusions: In addition to the typical clinical manifestations and MAS, a significant proportion of patients with AOSD develop uncommon complications, some of which can be potentially life-threatening. These should be considered in the evaluation and follow-up of patients. Early recognition and prompt management are crucial to significantly reduce morbidity and mortality. Full article

The COVID-19 pandemic disrupted routine healthcare services, including testing and treatment for sexually transmitted infections (STIs). At the same time, it fostered optimism about the potential of direct-to-consumer (DTC) self-testing solutions, fueled by remarkable progress in self-sampling practices, rapid testing technologies, and the adaptation of regulatory frameworks. Direct-to-consumer (DTC) self-testing and self-sampling for STIs became then still a more critical alternative, offering privacy, accessibility, and the potential to address testing gaps, especially for underserved, at-risk groups. This review critically analyzed the literature published since the onset of the COVID-19 pandemic. DTC testing has evolved significantly since the pandemic, with notable advances in technology, availability, and disease and geographical coverage. It has increased STI testing uptake among underserved or hesitant populations, including men who have sex with men and young adults. However, issues around cost, data reporting, and follow-up care persist, and many at-risk groups still lack access. While these solutions have improved testing uptake and public health outcomes, anticipated widespread adoption and advances have been slower than expected. Limited market options, weak research, and regulatory challenges have hindered growth. Research has often focused on potential user acceptance rather than real-world usage. Future directions should leverage trendy approaches in medical ethnography and transformational marketing to emphasize user- and data-driven innovation, affordability, and regulatory support based on private provider accountability. Full article

Background/Objectives: The objective of this study was to evaluate the diagnostic accuracy of the lactate dehydrogenase-to-albumin ratio (LAR) in adult-onset Still’s disease (AOSD) and compare it with other inflammatory indices, using patients with fever of unknown origin (FUO) as a control group due to their overlapping clinical features with AOSD. The study also compared LAR’s diagnostic performance with other inflammatory indices like the serum immune-inflammatory index (SII), ferritin/erythrocyte sedimentation rate (FER), CRP/albumin ratio (CAR), platelet/lymphocyte ratio (PLR), and neutrophil/lymphocyte ratio (NLR), as well as its combinations with FER, PLR, and ferritin (LAR + FER, LAR + PLR, LAR + ferritin). Methods: A retrospective evaluation was conducted on 70 patients with fever of unknown cause and 78 patients with AOSD, admitted between January 2000 and December 2023 in a tertiary care hospital. Demographic, clinical, and laboratory characteristics were compared between the groups. ROC analysis provided cutoff values, sensitivity, and specificity for each inflammatory index. Results: ROC analysis showed significant p-values (p < 0.05) for indices other than LAR (p = 0.090) LAR + PLR (p = 0.806), and PLR (p = 0.634) in diagnosing AOSD. The highest specificity was found in LAR + ferritin (92.90%), and the highest sensitivity in CAR (100.0%). NLR, SII, FER, and LAR + FER were the indices with both sensitivity and specificity above 50%. LAR had a sensitivity of 76.90% and a specificity of 48.60%. The cutoff values were 3978.0 µg/L for ferritin and 70.98 for LAR. Significant statistical differences between AOSD and non-AOSD groups were observed for all indices except CAR (p = 0.133). Conclusions: LAR can differentiate AOSD patients from FUO, but its specificity is lower than most other indices. The diagnostic utility of these indices in clinical practice remains controversial. Full article

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) is a rare mitochondrial disorder primarily presenting in pediatric patients, with onset after 40 years being exceptionally rare (1–6%). Here, we report a complex diagnostic journey of a 47-year-old male presenting with new-onset seizures, hemiparesis, and neurocognitive deficits. Initial work-up, including MRI, CSF analysis, and extensive antibody screening, yielded inconclusive results, prompting differential considerations such as autoimmune encephalitis and neoplastic conditions. Finally muscle biopsy findings, coupled with genetic confirmation of the m.3243A>G mutation in the MT-TL1 gene, ultimately established the diagnosis of MELAS. This case depicts the atypical presentation of adult-onset MELAS without initial lactic acidemia, diabetes, or hearing impairment. The prolonged diagnostic process underscores the challenges of identifying rare diseases under today’s financial and administrative constraints. Still ee emphasize the importance of comprehensive diagnostics in rare cases to advance generall understanding and improve future patient outcomes, also amidst resource limitations. Full article

Reversible cerebral vasoconstriction syndrome (RCVS) is a rare but significant cause of intracranial arteriopathy and stroke in young adults. The syndrome encompasses a spectrum of disorders radiologically characterized by reversible narrowing and dilation of intracranial arteries, often triggered by vasoactive drugs or the postpartum period. The hallmark clinical feature of RCVS is thunderclap headache with or without other neurological signs. Though endothelial dysfunction and sympathetic hyperactivation are hypothesized to be key mechanisms, the exact pathogenesis of RCVS is still unclear. RCVS’s diagnosis could be challenging, since vasospasm proceeds centripetally, initially involving distal small pial and cortical arteries, and angiographic studies, especially brain magnetic resonance angiography (MRA) and computed tomography angiography (CTA), may miss it in the early phase of the disease, while early signs such as vascular hyperintensities may be visible on T2/FLAIR sequences before vasospasm onset. Catheter angiography is the gold standard and it could be used to assess vasospasm reversibility post-intra-arterial vasodilator administration. Treatment is mainly symptomatic, and nimodipine is the most commonly administered therapy, given orally or intra-arterially in severe cases. Since many aspects of RCVS remain partially known, further research is needed to better understand the complex pathophysiology of this unique clinical condition and to optimize specific management strategies. Full article

Acute diarrhea represents a major public health issue, and the management of adult patients admitted to the emergency department (ED) for this problem is still challenging. In a retrospective analysis on more than 20,000 patients visiting a tertiary ED for acute diarrhea and then being discharged home, we found that age > 65 years, onset of symptoms > 24 h since ED admission, refusal of hospitalization, and a history of chronic renal and liver diseases were independently associated with ED readmission for abdominal symptoms within 7 days. In the younger group, the presence of comorbidities significantly impacted on ED readmission, while fever and alteration of serum creatinine were the main determinants in the older group. Antibiotics were prescribed in about 25% of patients, although diarrhea etiology (viral or bacterial) was usually not available. According to international guidelines, fluoroquinolones were the most prescribed class, showing an inverse correlation to ED readmission. However, β-lactams and probiotics were also commonly prescribed; the latter were independently correlated to ED readmission in the elderly group. A comprehensive, guideline-based approach, including a detailed clinical history and laboratory and comorbidity assessment, should be encouraged to support physicians in the management of different age subgroups of adults admitted to the ED for acute diarrhea. Full article

Introduction: Adult-onset Still’s disease (AOSD) is a rare chronic autoinflammatory condition characterized by a spiking fever, arthritis, a rash, hepatosplenomegaly, lymphadenopathy, leucocytosis, and hyperferritinemia. It is sometimes accompanied by life-threatening complications like macrophage activation syndrome/hemophagocytic lymphohistiocytosis (MAS/HLH). Treatment options for AOSD include glucocorticoids (GCs), immunosuppressive drugs, biological medications, and Janus kinase (JAK) inhibitors. The features that differentiate MAS/HLH from AOSD are: in MAS/HLH, a different type of fever, which is persistent, a sharp decrease in the number of leukocytes and thrombocytes, a further increase in the level of transaminases and ferritin, significant hepatosplenomegaly, lymphadenopathy, symptoms of the central nervous system (CNS), disseminated intravascular coagulation (DIC) and hemophagocytosis in the bone marrow. This study aimed to evaluate the course of AOSD, which results in MAS/HLD. Patients and methods: Nine AOSD patients, four of whom developed MAS/HLH, were treated at the Rheumatology Clinic in the Central Clinical Hospital of the Ministry of Interior Affairs from 1 January 2015 to 15 March 2020 and at the Rheumatology Clinic in the National Institute of Geriatric, Rheumatology and Rehabilitation from 1 September 2021 to 1 March 2024. Medical history, clinical data, demographic data, laboratory data, imaging data, Hscore, and treatment data were collected. Results: All the patients with MAS and an Hscore above 150 recovered. Discussion: MAS/HLH requires rapid diagnosis as well as treatment with methylprednisolone pulses, cyclosporine A, and etoposide. When comparing patients who developed MAS/HLH with those who did not, possible risk factors were identified: the presence of pregnancy (two cases) and an aggressive course of AOSD. The Hscore is a useful tool for identifying patients with MAS/HLH. Full article

Despite coverage of two doses of mumps-containing vaccines, mumps epidemics persist among children and young adults in China. This study aimed to analyze the epidemiological characteristics of mumps in Jiangsu Province, with a particular focus on breakthrough cases among high-incidence groups. Mumps cases reported in 2023 were systematically collected from the Infectious Disease Surveillance and Reporting System. A comprehensive descriptive epidemiological analysis was performed to elucidate the characteristics of the reported cases. A joinpoint regression (JPR) model was utilized to identify the temporal trends across various periods. Subsequently, immunization information for cases under 15 years of age was obtained through the Jiangsu Province Vaccination Integrated Service Management Information System to identify breakthrough cases and conduct exploratory analyses. A total of 4142 mumps cases were reported in Jiangsu Province in 2023, yielding an annual incidence rate of 4.86/100,000. A total of 81.75% of the cases were students and childcare children, and the gender ratio was 1.5:1 (male/female). The JPR model analysis of weekly reported cases identified five distinct trend segments (1st: 1–8, weekly percent change (WPC) = 26.67 *; 2nd: 9–28, WPC = 3.11 *; 3rd: 29–34, WPC = −5.31; 4th: 35–37, WPC = 15.48; 5th: 38–52, WPC = −4.06 *), and the gender subgroups demonstrated similar trends to the overall pattern. Notably, 89.14% (3692/4142) of the total cases were among individuals under 15 years, with 96.02% (3545/3692) having been vaccinated against mumps. The number of single-dose breakthrough cases (SdBCs) was approximately fourfold (2847/698) that of two-dose breakthrough cases (TdBCs). The main population composition of TdBCs was children aged 0–5 years old, and the classification was dominated by childcare children and scattered children. The median time interval between initial immunization and onset were shorter in TdBCs than in the SdBCs group, and the median time interval between the last immunization and onset was interestingly similarly shorter. However, these situations were interestingly reversed in 105 laboratory-confirmed breakthrough cases. Therefore, the current vaccination strategies have demonstrated tangible effectiveness in preventing and controlling mumps. However, the high incidence of breakthrough cases among high-risk pediatric populations indicates that mumps immunization strategies still deserve more attention and research for better herd protection. Full article

Atopic dermatitis and psoriasis are prevalent inflammatory skin conditions that significantly impact the quality of life of patients, with diverse treatment options available. Despite advances in understanding their underlying mechanisms, recent research highlights the significance of interleukins IL-18 and IL-37, in Th1, Th2, and Th17 inflammatory responses, closely associated with the pathogenesis of psoriasis and atopic dermatitis. Hence, IL-18 and IL-37 could potentially become therapeutic targets. This narrative review synthesizes knowledge on these interleukins, their roles in atopic dermatitis and psoriasis, and emerging treatment strategies. Findings of a literature search up to 30 May 2024, underscore a research gap in IL-37-targeted therapies. Conversely, IL-18-focused treatments have demonstrated promise in adult-onset Still’s Disease, warranting further exploration for their potential efficacy in psoriasis and atopic dermatitis. Full article

We use this study to analyze the trends in in-hospital length of stay, total hospital charges, and mortality among adult patients with a primary diagnosis of adult-onset still’s disease (AOSD). We used the 2016–2019 National Inpatient Sample (NIS) database to conduct a retrospective study on adult AOSD patients (≥18 years old). We analyzed data on baseline patient and hospital characteristics and determined trends in in-hospital mortality, length of stay (LOS), and total hospital charges (TOTCHG). Univariate and multivariate linear and logistic regression analyses were performed to identify factors that independently affected these outcomes. Among the 1615 AOSD hospitalizations, the mean LOS was 7.34 days and the mean TOTCHG was 68,415.31 USD. Macrophage activating syndrome (MAS), disseminated intravascular coagulation (DIC), and a large hospital size were shown to statistically increase the LOS and TOTCHG, while a Native American background was shown to statistically decrease both. The mean in-hospital mortality was 0.929%, with age being the only independent predictor. Our findings reveal an increase in the economic burden of AOSD hospitalizations despite declining admissions and mortality rates. Complications, like MAS and DIC, were found to significantly contribute to this burden despite treatment advancements. Our study indicates the importance of investigating new strategies to prevent these complications. Full article

Schnitzler syndrome is a rare disorder characterized by a chronic urticarial rash associated with immunoglobulin M (IgM) monoclonal gammopathy. Schnitzler syndrome shares strong clinicopathologic similarities with monogenic IL-1-mediated autoinflammatory disorders and is now considered an acquired adult-onset autoinflammatory disease. The spectacular effect of interleukin-1 inhibitors demonstrates the key role of this cytokine in the pathogenesis of the disease. However, the physiopathology of Schnitzler syndrome remains elusive, and the main question regarding the relationship between autoinflammatory features and monoclonal gammopathy is still unanswered. The purpose of this narrative review is to describe what is currently known about the pathogenesis of this peculiar disease, as well as to address its diagnosis and management. Full article

Hereditary transthyretin amyloidosis (hATTR) is a multisystemic, rare, inherited, progressive and adult-onset disease, affecting the sensory-motor nerves, heart, autonomic function, and other organs. There are over 130 mutations known in the TTR gene. The His90Asn mutation has been previously reported in several reports, but its pathogenetic role is still debated. We report two sporadic cases of adult women with a heterozygous His90Asn mutation in TTR gene and neurological involvement extensively investigated. A typical Congo red-positive pathologic deposition of amyloid fibrils in the salivary glands was documented in one subject. Patients were successfully treated with patisiran with a good clinical outcome. These data support a pathogenetic role of His90Asn mutation in hATTR, and suggest early treatment in symptomatic carriers of His90Asn mutation. Full article