Up-regulation of the monocyte chemotactic protein-3 in sera from bone marrow transplanted children with torquetenovirus infection

Torquetenovirus (TTV) represents a commensal human virus producing life-long viremia in approximately 80% of healthy individuals of all ages. A potential pathogenic role for TTV has been suggested in immunocompromised patients with hepatitis of unknown etiology sustained by strong proinflammatory cytokines

Distinct Changes in cAMP and Extracellular Signal-Regulated Protein Kinase Signalling in L-DOPA-Induced Dyskinesia

Background: In rodents, the development of dyskinesia produced by L-DOPA in the dopamine-depleted striatum occurs in response to increased dopamine D1 receptor-mediated activation of the cAMP- protein kinase A and of the Rasextracellular signal-regulated kinase (ERK) signalling pathways. However, very little is known, in non-human primates, about the regulation of these signalling cascades and their association with the induction, manifestation and/or maintenance of dyskinesia. Methodology/Results: We here studied, in the gold-standard non-human primate model of Parkinson’s disease, the changes in PKA-dependent phosphorylation of DARPP-32 and GluR1 AMPA receptor, as well as in ERK and ribosomal protein S6 (S6) phosphorylation, associated to acute and chronic administration of L-DOPA. Increased phosphorylation of DARPP-32 and GluR1 was observed in both L-DOPA first-ever exposed and chronically-treated dyskinetic parkinsonian monkeys. In contrast, phosphorylation of ERK and S6 was enhanced preferentially after acute L-DOPA administration and decreased during the course of chronic treatment. Conclusion: Dysregulation of cAMP signalling is maintained during the course of chronic L-DOPA administration, while abnormal ERK signalling peaks during the initial phase of L-DOPA treatment and decreases following prolonged exposure

The role of echocardiography in diagnosing carditis in the setting of acute rheumatic fever

n/

Cuore e Turner: cosa c'\ue8 oltre la coartazione?

A young woman affected by Turner syndrome undergoes periodic cardiac monitoring. Her last ECG documented QT prolongation. QT prolongation is reported in patients with Turner syndrome. This electrophysiological abnormality is probably due to an intrinsic defect in the regulation of the cardiovascular system. Although a long QT is more likely to be found in these patients, no severe life-threatening tachyarrhythmias have been described so far. Paediatricians should reassure Turner patients about the prognosis of this electrophysiological abnormality while recommending the cardiologic follow up

Miocardite: la grande simulatrice

Myocarditis is a rare, but life threatening disease in childhood. It is most often due to common viral infections; less commonly, it may result from bacterial infections, immune mediated diseases or chemotherapy. Myocarditis may present with unspecific symptoms, ranging from respiratory to gastrointestinal ones; a clear hypomobility is the typical sign of myocarditis (\u201cthe immobile child\u201d). The diagnosis is based on electrocardiogram and echocardiography, which are always pathologic but unspecific; an X-chest is useful to identify cardiomegaly. Among laboratory tests, the most sensitive element is an increased level of aspartate aminotransferase, while troponin dosage has low specificity and not absolute sensitivity. Endomyocardial biopsy is the gold standard diagnostic test, but it should be performed only in patients who do not respond to usual treatment, because of the high risk of side effects. The mainstay of therapy is supportive therapy for left ventricular dysfunction. The fulminant viral forms usually have initial significant cardiovascular impairment, followed by a complete resolution. On the other hand, a subacute disease might have less initial cardiovascular impairment, but more often can evolve to chronic dilated cardiomyopathy. In this case immunosuppressive therapy could be useful

La sindrome del QT lungo

The long QT syndrome (LQTS) is an arrhythmogenic syndrome due to cardiac ion channel disorders characterized by prolonged QT interval on ECG (QTc >440 ms for male, >460 ms for female) and the most common presentations are syncope, seizures, cardiac arrest and sudden death. Many different congenital forms have been identified but also an acquired form due to specific drugs, hypokalaemia, or hypomagnesemia is known. Familiarity is the leading risk factor. LQTS should be suspected in case of any syncope in order to perform ECG and start proper therapy. To identify and remove risk factors it is necessary to avoid potentially life-threatening arrhythmia in these patients

Meninges harbor cells expressing neural precursor markers during development and adulthood

Brain and skull developments are tightly synchronized, allowing the cranial bones to dynamically adapt to the brain shape. At the brain-skull interface, meninges produce the trophic signals necessary for normal corticogenesis and bone development. Meninges harbor different cell populations, including cells forming the endosteum of the cranial vault. Recently, we and other groups have described the presence in meninges of a cell population endowed with neural differentiation potential in vitro and, after transplantation, in vivo. However, whether meninges may be a niche for neural progenitor cells during embryonic development and in adulthood remains to be determined. In this work we provide the first description of the distribution of neural precursor markers in rat meninges during development up to adulthood. We conclude that meninges share common properties with the classical neural stem cell niche, as they: (i) are a highly proliferating tissue; (ii) host cells expressing neural precursor markers such as nestin, vimentin, Sox2 and doublecortin; and (iii) are enriched in extracellular matrix components (e.g., fractones) known to bind and concentrate growth factors. This study underlines the importance of meninges as a potential niche for endogenous precursor cells during development and in adulthood

Echocardiographic screening for the anomalous aortic origin of coronary arteries

Aims We sought to determine the diagnostic performance, clinical profiles and outcomes of anomalous aortic origin of coronary arteries (AAOCA) using a standardised echocardiographic approach in young adults and athletes.Methods In 2015–2019, we screened 5998 outpatients (age 16 years (Q1–Q3: 11, 36)), referred for routine echocardiography, using four specific echocardiographic windows: parasternal short/long axis and apical 4/5-chambers view. Coronary CT confirmed AAOCA. For the performance analysis, 300 coronary-CT scans were available; two independent and double-blinded physicians retrospectively reviewed echocardiographic images.Results A total of 47 AAOCA was diagnosed; the overall prevalence was 0.0078%. Over 5 years, we found a significant increment of AAOCA diagnostic rate (P for trend=0.002). Syncope (n=17/47) and palpitations (n=6/47) were prevalent symptoms. All patients suspended sports activity at the diagnosis. Twenty-seven patients underwent surgery, while 20 underwent a conservative medical treatment. All patients are alive at a median follow-up of 3±1.6 years; only surgical repairs restarted their activity. Our method showed better sensitivity than traditional short-axis evaluation: 93% vs 83%, p=0.0030 (AUC 0.96 (95% CI 0.92, 0.99) and AUC 0.89 (95% CI 0.83, 0.95), respectively), with a good interobserver agreement (95%, k=0.83, p<0.001).Conclusions The application of a standardised echocardiographic approach for AAOCA detection led to a significantly increased rate of identified anomalies. This approach demonstrated higher sensitivity than the traditional echocardiographic assessment. Implementing this protocol in clinical practice may help improve the AAOCA diagnosis in young adults and athletes.Trial registration number NCT04224090