Children with HSP and pIgAN present with similar signs of engagement of the innate immunity and regulatory T cell depression. The increased immunoproteasome switch, which correlated with TLR2 activation, may suggest an innate immunity pathway peculiar to HSP vasculitic presentation. This research area also deserves further investigation for possible therapeutic applications.

The benefits of tonsillectomy in IgA nephropathy (IgAN) are still debated. Tonsillectomy may remove pathogen sources and reduce the mucosal associated lymphoid tissue (MALT), limiting degalactosylated IgA1 (deGal-IgA1) production, which is considered to be the initiating pathogenetic event leading to IgA glomerular deposition. In the European network VALIGA, 62/1147 IgAN patients underwent tonsillectomy (TxIgAN). In a cross-sectional study 15 of these patients were tested and compared to 45 non-tonsillectomized IgAN (no-TxIgAN) and healthy controls (HC) regarding levels of deGal-IgA1, and markers of innate immunity and oxidative stress, including toll-like receptors (TLR)2, 3, 4 and 9 mRNAs, proteasome (PS) and immunoproteasome (iPS) mRNAs in peripheral blood mononuclear cells (PBMC), and advanced oxidation protein products (AOPP). Levels of deGal-IgA1 were lower in TxIgAN than in no-TxIgAN (p = 0.015), but higher than in HC (p = 0.003). TLR mRNAs were more expressed in TxIgAN than in HC (TLR4, p = 0.021; TLR9, p = 0.027), and higher in TxIgAN than in no-TxIgAN (p ≤ 0.001 for TLR2, 4, 9). A switch from PS to iPS was detected in PBMC of TxIgAN in comparison to HC and it was higher than in no-TxIgAN [large multifunctional peptidase (LMP)2/β1, p = 0.039; LPM7/β5, p < 0.0001]. The levels of AOPP were significantly higher in TxIgAN than HC (p < 0.001) and no-TxIgAN (p = 0.033). In conclusion, the activation of innate immunity via TLRs and ubiquitin-proteasome pathways and the pro-oxidative milieu were not affected by tonsillectomy, even though the levels of aberrantly galactosylated IgA1 were lower in patients with IgAN who had tonsillectomy. The residual hyperactivation of innate immunity in tonsillectomized patients may result from extra-tonsillar MALT.

Neonatal sepsis due to E. coli is often complicated by multiple organ failure (MOF) and a high mortality risk. We report the case of a term newborn discharged in good condition who suddenly fell into septic shock after 11 days and required immediate resuscitation, volume expansion and a high-dosage amine infusion. Extremely severe metabolic acidosis, disseminated intravascular coagulation (DIC) with diffuse bleeding, and unstable hemodynamic status with oliguria turned into strict anuria, and the patient became anuric. The presence of DIC, with gastric and intestinal bleeding, rendered peritoneal dialysis impossible. Continuous renal replacement therapy (CRRT) was started with the new dialysis machine CARPEDIEM® (Cardio-Renal Pediatric Dialysis Emergency Machine), available on a trial-basis in our center, after the surgical placement of jugular double-lumen central venous catheters. A ‘ready to use' neonatal kit with a low-priming volume of the extracorporeal circuit allowed a prompt hemofiltration start. The filtration CRRT was continuously performed for 48 h, then intermittently (12 h/day) for 2 more days and interrupted on day 5 for diuresis reprisal. Acute kidney injury and multi-organ failure resolved after 5 days. The child survived without neurological damage, with a normal renal function and a normal development at 9 months follow-up. In conclusion, a prompt CRRT start with this specifically designed neonatal device allowed a progressive stabilization of hemodynamics, a better control of acidosis, a reduction of amine requirement, a gradual control of fluid overload and a rapid improvement of MOF, DIC as well as a resolution of the acute kidney injury. The device also allowed the extension of CRRT in the neonatal age.

La pre-eclampsia è una complicanza relativamente comune della gravidanza, che interessa circa il 3% delle gravidanze. Attualmente, la patogenesi non è stata ancora completamente chiarita. Comunque, la disfunzione endoteliale materna, specialmente a livello della placenta, sembra essere il fattore chiave per lo sviluppo di questa malattia che clinicamente coinvolge molti organi, come reni, cervello e fegato, e che è caratterizzata da ipertensione, proteinuria ed edema. Lavori recenti suggeriscono il ruolo patogenetico di un'alterata espressione di fattori anti-angiogenici placentari con conseguenti modifiche dello stato redox che provocano stress ossidativo. La risposta all'azione dei fattori anti-angiogenetici produce una disfunzione endoteliale sistemica con ipertensione, proteinuria e altre manifestazioni sistemiche, come, per esempio, l'encefalopatia. In questo articolo verranno descritte le conoscenze più recenti nella fisiopatologia della pre-eclampsia, cercando di dare un'ipotesi patogenetica comune per conciliare le ano-malie a livello feto-placentare e le caratteristiche cliniche della sindrome materna e fornire una spiegazione logica per i potenziali futuri interventi profilattici e terapeutici di questa complicanza della gravidanza.

Pre-eclampsia is a relatively common complication of pregnancy, interesting about a 3% of the pregnancies. Its pathogenesis has been not yet completely clarified. However, a maternal endothelial dysfunction, particularly at the placenta level, seem to be the key factor in the development of this disease that clinically involves many organs such as kidney, brain and liver, characterized by hypertension, proteinuria and oedema. Recent works suggest a pathogenetic role of an altered expression of placental anti-angiogenic factors with consequent modifications in the redox state resulting in an oxidative stress. The effects to these anti-angiogenic factors results in a systemic endothelial dysfunction with hypertension, proteinuria, and the other systemic manifestations, such as encelophalopathy. Here, we will describe the most recent insights into the pathophysiology of preeclampsia attempting to provide a unifying hypothesis to reconcile the abnormalities at the feto-placental level and the clinical features of the maternal syndrome and provide a rationale for potential future prophylactic and therapeutic interventions for this pregnancy complication.