Native Mitral Valve Endocarditis Caused by Neisseria elongata subsp. nitroreducens in a Patient with Marfan Syndrome: First Case in Italy and Review of the Literature

Neisseria elongata (NE) is an aerobic Gram-negative organism that constitutes part of the commensal human normal oropharyngeal flora. Although previously considered not to be pathogenic, it has been recognized as an occasional cause of significant infections in humans. We report here the first case in Italy of infective endocarditis of a native prolapsing mitral valve in a patient with Marfan syndrome, caused by NE subspecies nitroreducens which has been rarely isolated from clinical specimens. The culprit organism has been confirmed by mass spectrometry directly from the positive blood culture, as previously reported. The amplified gene has been deposited in GenBank under accession number KT591873. In spite of the reported aggressive nature of NE, clinical remission was promptly obtained, there being no requirement for surgery

In-vitro NET-osis induced by COVID-19 sera is associated to severe clinical course in not vaccinated patients and immune-dysregulation in breakthrough infection

: Since neutrophil extracellular traps formation (NET-osis) can be assessed indirectly by treating healthy neutrophils with blood-derived fluids from patients and then measuring the NETs response, we designed a pilot study to convey high-dimensional cytometry of peripheral blood immune cells and cytokines, combined with clinical features, to understand if NET-osis assessment could be included in the immune risk profiling to early prediction of clinical patterns, disease severity, and viral clearance at 28 days in COVID-19 patients. Immune cells composition of peripheral blood, cytokines concentration and in-vitro NETosis were detected in peripheral blood of 41 consecutive COVID-19 inpatients, including 21 mild breakthrough infections compared to 20 healthy donors, matched for sex and age. Major immune dysregulation in peripheral blood in not-vaccinated COVID-19 patients compared to healthy subjects included: a significant reduction of percentage of unswitched memory B-cells and transitional B-cells; loss of naïve CD3+CD4+CD45RA+ and CD3+CD8+CD45RA+ cells, increase of IL-1β, IL-17A and IFN-γ. Myeloid compartment was affected as well, due to the increase of classical (CD14++CD16-) and intermediate (CD14++CD16+) monocytes, overexpressing the activation marker CD64, negatively associated to the absolute counts of CD8+ CD45R0+ cells, IFN-γ and IL-6, and expansion of monocytic-like myeloid derived suppressor cells. In not-vaccinated patients who achieved viral clearance by 28 days we found at hospital admission lower absolute counts of effector cells, namely CD8+T cells, CD4+ T-cells and CD4+CD45RO+ T cells. Percentage of in-vitro NET-osis induced by patients' sera and NET-osis density were progressively higher in moderate and severe COVID-19 patients than in mild disease and controls. The percentage of in-vitro induced NET-osis was positively associated to circulating cytokines IL-1β, IFN-γ and IL-6. In breakthrough COVID-19 infections, characterized by mild clinical course, we observed increased percentage of in-vitro NET-osis, higher CD4+ CD45RO+ and CD8+ CD45RO+ T cells healthy or mild-COVID-19 not-vaccinated patients, reduced by 24 h of treatment with ACE inhibitor ramipril. Taken together our data highlight the role of NETs in orchestrating the complex immune response to SARS-COV-2, that should be considered in a multi-target approach for COVID-19 treatment

Bone turnover markers in patients with type 1 Gaucher disease

Bone complications occur frequently in Gaucher disease (GD) and reduce the quality of life of these patients. Skeletal involvement is an important indication for treatment to ameliorate symptoms and reduce the risk of irreversible and debilitating disease. Bone biomarkers have been used to assess disease status and the response to therapy in a number of bone disorders. Here, we examine the literature for evidence of abnormalities in bone turnover markers in patients with type 1 GD to assess whether they might be useful for the assessment of bone involvement in GD. We have found that bone biomarkers in GD show highly variable results which do not currently support their routine use for clinical assessment of bone status, as an indication for therapy initiation, or for monitoring the response to therapy. A greater understanding of bone markers and their relation to the bone manifestations of GD is required

Inside-out: the role of anger experience and expression in the development of postpartum mood disorders

PURPOSE: Among negative emotions, anger has not been studied in as much depth in her connection to postpartum mood disorders. The study aimed to investigate the role of anger as a potential vulnerability factor increasing the risk of Maternity Blues (MB) and Postpartum Depression (PPD). MATERIALS AND METHODS: Pregnant women in their third trimester of pregnancy underwent the following tests: the State Trait Anger Expression Inventory 2 - STAXI-2 (baseline visit), the Blues Questionnaire -BQ (3 and 5 days after delivery), and the Edinburgh Postnatal Depression Scale - Edinburgh Postnatal Depression Scale (EPDS) (3 and 6 months following delivery). RESULTS: One hundred ten subjects were included in this study. The prevalence rate of mothers with MB was about 35%, whereas about 4% of women developed a PPD. Significant positive correlations were found among State anger (SANG), Trait anger (TANG), anger expression out (AXO) and in (AXI) and postpartum depressive disorders, as measured by blues questionnaire (BQ) and EPDS. CONCLUSIONS: Anger experience and expression can be considered as vulnerability factors for postpartum mood disorders onset. Particularly, the expression of angry feelings toward other persons or objects in the environment (AXO) predicts the onset of MB, whereas holding in or suppressing angry feelings (AXI) could be a risk factor for subsequent PPD

Inside–out: the role of anger experience and expression in the development of postpartum mood disorders

PURPOSE: Among negative emotions, anger has not been studied in as much depth in her connection to postpartum mood disorders. The study aimed to investigate the role of anger as a potential vulnerability factor increasing the risk of Maternity Blues (MB) and Postpartum Depression (PPD). MATERIALS AND METHODS: Pregnant women in their third trimester of pregnancy underwent the following tests: the State Trait Anger Expression Inventory 2 - STAXI-2 (baseline visit), the Blues Questionnaire -BQ (3 and 5 days after delivery), and the Edinburgh Postnatal Depression Scale - Edinburgh Postnatal Depression Scale (EPDS) (3 and 6 months following delivery). RESULTS: One hundred ten subjects were included in this study. The prevalence rate of mothers with MB was about 35%, whereas about 4% of women developed a PPD. Significant positive correlations were found among State anger (SANG), Trait anger (TANG), anger expression out (AXO) and in (AXI) and postpartum depressive disorders, as measured by blues questionnaire (BQ) and EPDS. CONCLUSIONS: Anger experience and expression can be considered as vulnerability factors for postpartum mood disorders onset. Particularly, the expression of angry feelings toward other persons or objects in the environment (AXO) predicts the onset of MB, whereas holding in or suppressing angry feelings (AXI) could be a risk factor for subsequent PPD

Three-Way Translocation t(12;15;17) (p13;q24;q21) Found in Acute Promyelocytic Leukemia with Basophilic Differentiation

Acute promyelocytic leukemia is a rare form of acute myeloid leukemia in which immature promyelocytes abnormally proliferate in the bone marrow. In most cases, the disease is characterised by the translocation t(15;17) (q24;q21), which causes the formation of PML::RARA, an oncogenic fusion protein responsible for blocking myeloid differentiation and survival advantage. Here, we present a case of acute promyelocytic leukemia with two unusual features: basophilic differentiation and a three-way translocation involving chromosomes 12, 15 and 17. In the few cases reported, basophilic differentiation was associated with a poor prognosis. In contrast, our patient responded promptly to the standard treatment with all-trans-retinoic acid (ATRA) and arsenic trioxide (ATO) and obtained complete remission. To our knowledge, this is the first report of basophilic acute promyelocytic leukemia with the three-way translocation t(12;17;15) (p13; q24;q21)

A case of high‐risk AML in a patient with advanced systemic mastocytosis

Abstract Aggressive SM + AML has limited therapeutic options. Even a strong combination of decitabine–venetoclax–midostaurin has a transient effect on AML and a mitigated effect on SM. Larger series are required to identify the best therapeutic strategy