In this work two cytokines were used in combination with inactivated bacteria (bacterin) to test the bovine conjuctival immune response to the pathogen Moraxella bovis, the causative agent of ...Infectious bovine keratoconjunctivitis (IBK). Treatments using the bacterin vaccine combined with interleukin-2 and interferon-α as adjuvants (Group A), the bacterin vaccine only (Group B), and controls without treatment (Group C), were applied by ocular spraying to evaluate the local immune response in the corneal structure of cattle experimentally infected with M. bovis. Six weeks after infection, 14 out of a total of 34 animals presented different corneal lesions; 9 corresponding to the control group C, 4 to the group B and only one to the group A. According to the clinical manifestations, a numeric score was calculated. Control animals presented the highest score value (12 points), followed by group B (7.5 points) and group A (1 point). These results suggest that the addition of cytokines to M. bovis treatments can reduce not only eye injuries caused by IBK but also the number of diseased animals.
Infectious bursal disease (IBD) is a viral disease of young chickens that produce severe lesions in the bursa of Fabricius and other organs inducing immunosuppression and mortality in birds. This ...study indicates that oral administration of IFN-α and IL-2 during 16 days produced a significant reduction in animals’ morbidity and mortality to IBD virus (IBDV) infection accompanied with a decrease in symptoms and bursal tissue damage. The treatment also increased body weight, not only in birds challenged with IBDV, but also in uninfected controls. Infected birds treated with cytokines presented the same bursal index and organs’ weight that controls; since untreated animals showed a significant decrease in these parameters. Finally, cytokine administration represents a new alternative to IBDV vaccination.
Background. Streptococci are not an infrequent cause of periprosthetic joint infection (PJI). Management by debridement, antibiotics, and implant retention (DAIR) is thought to produce a good ...prognosis, but little is known about the real likelihood of success. Methods. A retrospective, observational, multicenter, international study was performed during 2003–2012. Eligible patients had a streptococcal PJI that was managed with DAIR. The primary endpoint was failure, defined as death related to infection, relapse/persistence of infection, or the need for salvage therapy. Results. Overall, 462 cases were included (median age 72 years, 50% men). The most frequent species was Streptococcus agalactiae (34%), and 52% of all cases were hematogenous. Antibiotic treatment was primarily using β-lactams, and 37% of patients received rifampin. Outcomes were evaluable in 444 patients: failure occurred in 187 (42.1%; 95% confidence interval, 37.5%–46.7%) after a median of 62 days from debridement; patients without failure were followed up for a median of 802 days. Independent predictors (hazard ratios) of failure were rheumatoid arthritis (2.36), late post-surgical infection (2.20), and bacteremia (1.69). Independent predictors of success were exchange of removable components (0.60), early use of rifampin (0.98 per day of treatment within the first 30 days), and long treatments (≥21 days) with β-lactams, either as monotherapy (0.48) or in combination with rifampin (0.34). Conclusions. This is the largest series to our knowledge of streptococcal PJI managed by DAIR, showing a worse prognosis than previously reported. The beneficial effects of exchanging the removable components and of β-lactams are confirmed and maybe also a potential benefit from adding rifampin.
Posterior cortical atrophy in clinical practice Maure‐Blesa, Lucía; Agüero, Pablo; Sainz, María José ...
Alzheimer's & dementia,
December 2023, 2023-12-00, Letnik:
19, Številka:
S18
Journal Article
Recenzirano
Odprti dostop
Background
A consensus classification framework for posterior cortical atrophy (PCA) was proposed in 2017 to define the core clinico‐pathological syndrome. Main features include a variety of ...visuospatial deficits associated with posterior cortex dysfunction, together with predominant posterior cortical atrophy. Additional neurological symptoms may be present in what is termed PCA‐plus. Several proteinopathies could be the underlying neuropathology, though in vivo biomarkers only allow for identification of Alzheimer’s disease (AD).
Method
We described the clinical phenotype and biomarkers in a series of patients diagnosed at our memory clinic of PCA (n = 13). Retrospective review of age at onset, family history, cognitive symptoms, neurological deficits, patterns of brain atrophy in MRI, AD biomarkers in CSF, and available genetic analyses.
Result
There were eight women and five men; five had a family history of dementia (Goldman score 3.5) while eight were apparently sporadic cases. Age at onset of symptoms ranged from 30 to 75 years (median 66) and first neurological consultation was 1 to 2 years later, though both psychiatrists and ophthalmologists had already evaluated 46% of the cases. Diagnosis of PCA was established between 5 months and 5 years (mean 2 years) of first assessment. Brain atrophy was parieto‐occipital in 11 cases and temporo‐parietal in two (bilateral in seven, left predominant in four and right in two). Three cases had a PCA‐plus syndrome, one with parkinsonism an another two with CBD features. Five cases (including one with CBD) had AD CSF biomarkers studied, all with an A+T+N+ pattern. APOE was available in eight cases, five carried ε3/ε3 and three ε3/ε4 alleles. The patient with younger onset carried a PSEN1 pathogenic mutation (p.Pro436Gln). Three cases had C9orf72 expansion and plasma progranulin levels analyzed with negative results. Most cases were dependent for activities of daily living after five years of evolution.
Conclusion
This series further contributes to the characterization of the clinical heterogeneity of PCA. Anxiety and depression are common in early stages of the disease. Biomarkers are usually consistent with AD.
The purpose of this study is to characterize the risk of cancer in a large cohort of patients with primary Sjögren syndrome (SjS).
We had analyzed the development of cancer in 1300 consecutive ...patients fulfilling the 2002 SjS classification criteria. The baseline clinical and immunological characteristics and systemic activity (ESSDAI scores) were assessed at diagnosis as predictors of cancer using Cox proportional hazards regression analysis adjusted for age at diagnosis and gender. The sex-and age-specific standardized incidence ratios (SIR) of cancer were estimated from 2012 Spanish mortality data.
After a mean follow-up of 91 months, 127 (9.8%) patients developed 133 cancers. The most frequent type of cancer was B-cell lymphoma (including 27 MALT and 19 non-MALT B-cell lymphomas). Systemic activity at diagnosis of primary SjS correlated with the risk of hematological neoplasia and cryoglobulins with a high risk of either B-cell or non-B-cell lymphoma subtypes. Patients with cytopenias had a high risk of non-MALT B-cell and non-B-cell cancer, while those with low C3 levels had a high risk of MALT lymphomas and those with monoclonal gammopathy and low C4 levels had a high risk of non-MALT lymphomas. The estimated SIR for solid cancer was 1.13 and 11.02 for hematological cancer. SIRs for specific cancers were 36.17 for multiple myeloma and immunoproliferative diseases, 19.41 for Hodgkin lymphoma, 6.04 for other non-Hodgkin lymphomas, 5.17 for thyroid cancer, 4.81 for cancers of the lip and oral cavity, and 2.53 for stomach cancer.
One third of cancers developed by patients with primary SjS are B-cell lymphomas. The prognostic factors identified at SjS diagnosis differed according to the subtype of B-cell lymphoma developed. Primary SjS is also associated with the development of some non-hematological cancers (thyroid, oral cavity, and stomach).
The Amazon Basin is an unquestionable biodiversity hotspot, containing the highest freshwater biodiversity on earth and facing off a recent increase in anthropogenic threats. The current knowledge on ...the spatial distribution of the freshwater fish species is greatly deficient in this basin, preventing a comprehensive understanding of this hyper-diverse ecosystem as a whole. Filling this gap was the priority of a transnational collaborative project, i.e. the AmazonFish project - https://www.amazon-fish.com/. Relying on the outputs of this project, we provide the most complete fish species distribution records covering the whole Amazon drainage. The database, including 2,406 validated freshwater native fish species, 232,936 georeferenced records, results from an extensive survey of species distribution including 590 different sources (e.g. published articles, grey literature, online biodiversity databases and scientific collections from museums and universities worldwide) and field expeditions conducted during the project. This database, delivered at both georeferenced localities (21,500 localities) and sub-drainages grains (144 units), represents a highly valuable source of information for further studies on freshwater fish biodiversity, biogeography and conservation.
To analyse the clinical features and outcomes of patients presenting with life-threatening systemic disease in a large cohort of Spanish patients with primary Sjögren's syndrome (SS).
The GEAS-SS ...multicentre registry was formed in 2005 with the aim of collecting a large series of Spanish patients with primary SS, and included more than 20 Spanish reference centres with substantial experience in the management of SS patients. By January 2018, the database included 1580 consecutive patients fulfilling the 2002 classification criteria for primary SS. Severe, life-threatening systemic disease was defined as an activity level scored as "high" in at least one ESSDAI domain.
Among 1580 patients, 208 (13%) were classified as presenting a severe, potentially life-threatening systemic disease: 193 presented one ESSDAI domain classified as high, 14 presented two high scored domains and only one presented three high activity domains. The ESSDAI domains involved consisted of lymphadenopathy in 78 (37%) cases, CNS in 28 (13%), PNS in 25 (12%), pulmonary in 25 (12%), renal in 21 (10%), cutaneous in 19 (9%), articular in 18 (9%), haematological in 7 (3%) and muscular in 4 (2%). Patients with severe systemic disease were more frequently men (p=0.001) and had a higher frequency of anaemia (p<0.001), lymphopenia (p<0.001), rheumatoid factor (p=0.021), low C3 levels (p=0.015), low C4 levels (p<0.001) and cryoglobulins (p<0.001). From a therapeutic point of view, systemic patients received more frequently glucocorticoids (p<0.001), immunosuppressants (p<0.001), intravenous immunoglobulins (p=0.008) and rituximab (p<0.001). We found an overall mortality rate of 20% in severe systemic patients, a rate that reached to 33% in patients presenting two or more high systemic involvements; these patients had a higher frequency of low C4 levels (p=0.012) and cryoglobulins (p=0.001) in comparison with those with a single severe organ involved.
13% of patients with primary SS develop a potentially life-threatening systemic disease (mainly lymphoma, but also severe internal organ involvements including nervous system, the lungs and the kidneys). This subset of patients requires intensive therapeutic management with a mortality rate of nearly 20% of cases.
Introduction
Transthyretin amyloidosis (ATTR amyloidosis) is primarily associated with a cardiac or neurologic phenotype, but a mixed phenotype is increasingly described.
Methods
This study describes ...the mixed phenotype cohort in the Transthyretin Amyloidosis Outcomes Survey (THAOS). THAOS is an ongoing, longitudinal, observational survey of patients with ATTR amyloidosis, including both hereditary (ATTRv) and wild-type disease, and asymptomatic carriers of pathogenic transthyretin variants. Baseline characteristics of patients with a mixed phenotype (at enrollment or reclassified during follow-up) are described (data cutoff: January 4, 2022).
Results
Approximately one-third of symptomatic patients (
n
= 1185/3542; 33.5%) were classified at enrollment or follow-up as mixed phenotype (median age, 66.5 years). Of those, 344 (29.0%) were reclassified to mixed phenotype within a median 1–2 years of follow-up. Most patients with mixed phenotype had ATTRv amyloidosis (75.7%). The most frequent genotypes were V30M (38.9%) and wild type (24.3%).
Conclusions
These THAOS data represent the largest analysis of a real-world mixed phenotype ATTR amyloidosis population to date and suggest that a mixed phenotype may be more prevalent than previously thought. Patients may also migrate from a primarily neurologic or cardiologic presentation to a mixed phenotype over time. These data reinforce the need for multidisciplinary evaluation at initial assessment and follow-up of all patients with ATTR amyloidosis.
Trial Registration
ClinicalTrials.gov: NCT00628745.
Pregnant women and their babies are among the populations most vulnerable to untoward health outcomes. Yet current standards for evaluating health interventions cannot be met during pregnancy because ...of lack of adequate evidence. The situation is even more concerning in low-income and middle-income countries, where the need for effective interventions is the greatest. Meeting the Sustainable Development Goals for health will require strengthened attention to maternal and child health. In this paper we examine ongoing initiatives aimed at improving the assessment of maternal interventions. We review current methodologies to monitor outcomes of maternal interventions and identify where harmonisation is needed. Based on this analysis we identify settings where different minimal data sets should be considered taking into consideration the clinical realities. Stronger coordination mechanisms and a roadmap to support harmonised monitoring of maternal interventions across programmes and partners, working on improving pregnancy and early childhood health events, will greatly enhance ability to generate evidence-based policies.