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Objective: This study aimed to analyze early and midterm results of custom-made proximal scallop and fenestrated stent grafts for thoracic endovascular aortic repair (TEVAR) with a proximal landing zone (PLZ) in the aortic arch., Methods: All consecutive patients treated with the custom made proximal scalloped and fenestrated Relay stent grafts (Terumo Aortic Bolton Medical Inc.) in 10 Italian centers between January 2014 and December 2022 were included. The primary end points were technical success, incidence of intraoperative major adverse events, deployment accuracy, and rate of early neurological complications, endoleaks (ELs) and retrograde aortic dissection., Results: During the study period, 49 patients received TEVAR with Relay custom-made endograft in Italy were enrolled. The median patient age was 70.1 years (interquartile range, 23-86 years) and 65.3% were male. The indication for treatment was atherosclerotic aneurysms in 59.2% of cases and penetrating aortic ulcer in 22.4%. The endograft configuration was proximal fenestration in 55.1% and scallop in 44.9%. The proximal landing zone was zone 0 in 25 cases (51%), zone 1 in 14 cases (28.6%), and zone 2 in 10 cases (20.4%). The supra-aortic debranching procedures were 38 (77.5%). Technical success was 97.9% (48/49) owing to one case (2.0%) of inaccurate deployment. Intraoperatively, one (2.0%) type Ia and one (2.0%) type III EL were detected. There were no cases of in-hospital mortality, major adverse events, or retrograde dissection. Three minor strokes (6.1%) (National Institutes of Health Stroke Scale score of ≤4) were observed. At a mean follow-up time of 36.3 ± 21.3 months the rate of types I to III ELs and reintervention was 4.1%, respectively. Four patients (8.2%) died during the follow-up period, one (2.1%) from abdominal aortic rupture and three (6.1%) from nonaortic causes., Conclusions: Our early and midterm outcomes suggest that scalloped and fenestrated TEVAR may provide an acceptable alternative treatment option for aortic arch pathologies. Large-scale studies are needed to assess the long-term durability of this technique., Competing Interests: Disclosures None., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Thrombotic microangiopathies represent a group of particularly serious pathologies that can cause a rapid worsening of renal function, especially in young subjects. Through the clinical case described, we will focus our attention on the clinical and laboratory manifestations of the pathology, on the diagnostics and on the therapies to be used. Recent therapeutic innovations for the treatment of this pathology will also be analysed., (Copyright by Società Italiana di Nefrologia SIN, Rome,Italy.)

Objective: The aim of this study was to investigate the mid-term results of stented-assisted balloon-induced intimal disruption and relamination (STABILISE) in patients with aortic dissection with the implementation of volumetric analysis., Methods: This was a single-center retrospective analysis of prospectively collected data. From May 2017 to September 2022, 42 patients underwent STABILISE for acute complicated or subacute high-risk aortic dissection. STABILISE was completed with distal extended endovascular aortic repair in 24 patients. A computed tomography scan was performed at baseline, before hospital discharge, and at 1, 3, and 5 years. Perfused total aortic, true lumen, and false lumen volumes were assessed for thoracic, visceral, and aorto-iliac segment. The ratio between false lumen and total volume was named perfusion dissection index (PDI). Complete remodeling was defined as PDI = 0, and positive remodeling as PDI ≤0.1., Results: Technical success was 97.6%. No 30-day deaths, spinal cord injuries, or retrograde dissections were observed. Mean follow-up was 44 ± 19.4 months. Thoracic diameter was lower at last available computed tomography scan (36.7 vs 33.0 mm; P = .01). Aortic growth >5 mm was observed in 9.5% of the patients. Thoracic and visceral aortic complete remodeling were 92.8% and 83.3%, respectively, with no difference between acute and subacute group. Distal extended endovascular aortic repair significantly increased complete remodeling in the aorto-iliac segment, compared with STABILISE alone (69.6% vs 21.4%; P < .001). Freedom from vascular reinterventions at 3 years was 83.1% (95% confidence interval, 71.5%-96.6%). Total PDI ≤0.1 at first postoperative control was a predictor of vascular reinterventions (P < .0001)., Conclusions: STABILISE is a safe and feasible technique associated with high mid-term rates of complete remodeling in the thoracic and visceral aorta. Volumetric analysis allows the quantification of aortic remodeling and represents a predictor of aortic reinterventions., Competing Interests: Disclosures None., (Copyright © 2024 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Cytopenia is a common finding in patients admitted to internal medicine wards and the clinical workup may be long and time-consuming. In this single-center observational study, we analyzed a series of 151 inpatients who received hematologist referral due to cytopenia observed during hospital admission. Patients were mainly elderly (median 71 years, 15-96) and 87% had at least one comorbidity. Anemia was the most common cytopenia (91%), followed by thrombocytopenia (51%), and neutropenia (22%); 73 (48%) patients had a bicytopenia and 5 (3%) pancytopenia. Cytopenias were mainly severe, 66% of cases required RBC transfusions, and 21% platelet pools. During a median hospital stay of 15 days (1-166), 53 subjects (35%) received a hematologic discharge diagnosis, whilst the two-thirds had secondary cytopenia mainly due to associated comorbidities. Only about 34% of 2,728 diagnostic tests performed (including laboratory, imaging, and histology) clearly informed the discharge diagnosis in this heterogenous setting. Specifically, bone-marrow evaluation indicated in 46 (30%) patients, was diagnostic in 32 (69.6%). Eleven percent of patients died due to progression of the oncohematologic disease (29%), sepsis (24%), and solid tumor progression (24%). In conclusion, cytopenias in the internal medicine setting are mainly severe, more frequently secondary to associated comorbidities (2/3 of patients) and deserve proper workup before second/third-level tests (immune-hematological assays and CT scan or PET and bone-marrow evaluation, respectively)., (© 2024. The Author(s).)

The number of vascular centers performing endovascular repair of ascending aortic disease is constantly increasing. Accordingly to the guidelines, open surgical repair remains the gold standard for these pathologies. However, approximately one quarter of patients are deemed unfit for open surgery. In this study, we describe three cases of ascending thoracic endovascular aortic repair (TEVAR) performed at our center. All the patients were deemed unfit for open surgery by the aortic team. Two patients had an ascending aortic pseudoaneurysm, and the third had a focal type A aortic dissection. In two cases, we used two abdominal aortic cuffs deployed from zone 0B to zone 0C, with no need for supra-aortic trunk debranching. In one case, we performed a "reverse" extrathoracic debranching, and we deployed a thoracic endograft from zone 0B to zone 2. Complications included one minor stroke and one inguinal hematoma. In one patient with an infected pseudoaneurysm, we performed ascending TEVAR as a bridge strategy for open repair. This patient developed a type Ia endoleak; however, clinical stabilization and infection control were obtained, and he was able to undergo heart surgery successfully. He underwent a second reintervention to treat superior mesenteric embolic occlusion. At 2 years of follow-up, all three patients were alive. Our preliminary experience demonstrates the technical feasibility and clinical appropriateness of ascending TEVAR using standard, commercially available endografts. However, no consensus has been reached regarding some critical aspects, such as the development of a standardized technique or the efficacy of the currently available devices. The improvements in graft design and the adoption of the "aortic team" approach could help in the near future to standardize the procedure, establish appropriate indications, and ensure good clinical outcomes., Competing Interests: None., (© 2024 The Authors.)

Background: Since birth, during the exploration of the environment to interact with objects, we exploit both the motor and sensory components of the upper limb (UL). This ability to integrate sensory and motor information is often compromised following a stroke. However, to date, rehabilitation protocols are focused primarily on recovery of motor function through physical therapies. Therefore, we have planned a clinical trial to investigate the effect on functionality of UL after a sensorimotor transcranial stimulation (real vs sham) in add-on to robot-assisted therapy in the stroke population., Methods: A randomised double-blind controlled trial design involving 32 patients with a single chronic stroke (onset > 180 days) was planned. Each patient will undergo 15 consecutive sessions (5 days for 3 weeks) of paired associative stimulation (PAS) coupled with UL robot-assisted therapy. PAS stimulation will be administered using a bifocal transcranial magnetic stimulator (TMS) on the posterior-parietal cortex and the primary motor area (real or sham) of the lesioned hemisphere. Clinical, kinematics and neurophysiological changes will be evaluated at the end of protocol and at 1-month follow-up and compared with baseline. The Fugl-Meyer assessment scale will be the primary outcome. Secondly, kinematic variables will be recorded during the box-and-block test and reaching tasks using video analysis and inertial sensors. Single pulse TMS and electroencephalography will be used to investigate the changes in local cortical reactivity and in the interconnected areas., Discussion: The presented trial shall evaluate with a multimodal approach the effects of sensorimotor network stimulation applied before a robot-assisted therapy training on functional recovery of the upper extremity after stroke. The combination of neuromodulation and robot-assisted therapy can promote an increase of cortical plasticity of sensorimotor areas followed by a clinical benefit in the motor function of the upper limb., Trial Registration: ClinicalTrials.gov NCT05478434. Registered on 28 Jul 2022., (© 2023. The Author(s).)

Background: Fragile X syndrome (FXS) is the leading cause of genetic intellectual disability. Among the neurobehavioral dysfunctions in FXS individuals, language development and literacy are compromised. Recent evidence hypothesized that the disruption of excitatory glutamatergic and GABAergic inhibitory neurotransmission balance might be responsible for impairment in cognitive function. In this study, we evaluated for the first time, the safety, tolerability, and efficacy of anodal prefrontal transcranial direct current stimulation (tDCS) combined with standard speech therapy to enhance language function in FXS patients., Methods: In total, 16 adult FXS patients were enrolled. Participants underwent 45 min of anodic tDCS combined with speech therapy for 5 weeks (3 times per week). Language function was evaluated using the Test for Reception of Grammar-Version 2 (TROG-2) and subtests of the Italian Language Examination (Esame del Linguaggio - II, EDL-II). Right and left dorsolateral prefrontal cortex transcranial magnetic stimulation and concurrent electroencephalography (TMS-EEG) recordings were collected at baseline and after the treatment to evaluate cortical reactivity and connectivity changes., Results: After 5 weeks of combined therapy, we observed a significant improvement in the writing (7.5%), reading (20.3%), repetition (13.3%), and TROG-2 (10.2%) tests. Parallelly with clinical change, TMS-EEG results showed a significant difference in TMS-evoked potential amplitude over the left frontal cortex after treatment (-0.73 ± 0.87 μV) compared to baseline (0.18 ± 0.84 μV)., Conclusion: Our study provides novel evidence that left anodal prefrontal tDCS combined with standard speech therapy could be effective in enhancing language function in FXS patients, mainly by inducing a rebalance of the dysfunctional prefrontal cortical excitability., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Picciuca, Assogna, Esposito, D’Acunto, Ferraresi, Picazio, Borghi, Martino Cinnera, Bonnì, Chiurazzi and Koch.)

Hemoglobinopathies, including thalassemias and sickle cell disease, are the most common monogenic diseases worldwide, with estimated annual births of more than 330,000 affected infants. Hemoglobin disorders account for about 3.4% of deaths in children under 5 years of age. The distribution of these diseases is historically linked to current or previously malaria-endemic regions; however, immigration has led to a worldwide distribution of these diseases, making them a global health problem. During the last decade, new treatment approaches and novel therapies have been proposed, some of which have the potential to change the natural history of these disorders. Indeed, the first erythroid maturation agent, luspatercept, and gene therapy have been approved for beta-thalassemia adult patients. For sickle cell disease, molecules targeting vaso-occlusion and hemoglobin S polymerization include crizanlizumab, which has been approved for patients ≥ 16 years, voxelotor approved for patients ≥ 12 years, and L-glutamine for patients older than 5 years.    Conclusion: We herein present the most recent advances and future perspectives in thalassemia and sickle cell disease treatment, including new drugs, gene therapy, and gene editing, and the current clinical trial status in the pediatric populations. What is Known: • Red blood cell transfusions, iron chelation therapy and hematopoietic stem cell transplantation have been the mainstay of treatment of thalassemia patients for decades. • For sickle cell disease, until 2005, treatment strategies were mostly the same as those for thalassemia, with the option of simple transfusion or exchange transfusion. In 2007, hydroxyurea was approved for patients ≥ 2 years old. What is New: • In 2019, gene therapy with betibeglogene autotemcel (LentiGlobin BB305) was approved for TDT patients ≥ 12 years old non β0/β0 without matched sibling donor. • Starting from 2017 several new drugs, such as L-glutamine (approved only by FDA), crizanlizumab (approved by FDA and EMA for patients ≥ 16 years), and lastly voxelotor (approved by FDA and EMA for patients ≥ 12 years old)., (© 2023. The Author(s).)

Purpose: This study investigated the long-term outcomes of patients treated with fenestrated and branched endovascular aneurysm repair (F-BEVAR) or open surgical repair (OSR) for complex abdominal aortic aneurysms (c-AAAs). Complex abdominal aortic aneurysms are defined as aneurysms that involve the renal or mesenteric arteries and extend up to the level of the celiac axis or diaphragmatic hiatus but do not extend into the thoracic aorta. This study compares with a propensity-score matching the outcome of these procedures from 2 high-volume aortic centers., Materials and Methods: All patients with c-AAAs undergoing repair at 2 centers between January 2010 and June 2016 were included. The long-term imaging follow-up consisted in a yearly computed tomography angiography (CTA) in the F-BEVAR group. Yearly abdominal ultrasound examination and 5-year CTA were performed in the OSR group. The primary endpoints were long-term mortality, aneurysm-related mortality, and chronic renal decline (CRD), defined as estimated glomerular filtration rate reduction to <60 mL/min/1.73 m 2 or >20%/de novo dependence on permanent dialysis in patients with normal or abnormal preoperative renal function, respectively. Secondary endpoints included aortic-related reinterventions, target vessel occlusion, proximal aorta degeneration, access-related complications, graft infection, and the composite endpoint of clinical failure during follow-up., Results: After 1:1 propensity matching, 102 consecutive patients who underwent F-BEVAR and OSR, respectively, were included. The median follow-up was 67 months. There was no significant difference in long-term overall mortality (40.2% vs 36.3%; p=0.40) and aneurysm-related mortality (6.8% vs 5.8%; p=0.30), in the F-BEVAR and OSR groups, respectively. During follow-up, late renal function decline occurred in 27 (27.8%) versus 46 patients (47.4%) in the F-BEVAR and OSR groups, respectively (p<0.01). During follow-up, 23 reinterventions (23.5%) were performed in the F-BEVAR group, and 5 (5.1%) in the OSR group (p<0.01)., Conclusions: No differences in overall and aneurysm-related mortality were observed. Chronic renal decline was significantly higher after OSR, while the reintervention rate was higher in the F-BEVAR group. These long-term results reflect the outcomes of a complex procedure performed by a single experienced operator in 2 high-volume centers, and followed with a strict surveillance imaging follow-up., Clinical Impact: Nowadays, F-BEVAR and OSR are considered two established techniques for the treatment of c-AAA. However, long-term comparative outcomes are not well studied, and concerns may rise in terms of durability of the repair, risk of reinterventions and late chronic renal decline. The present study showed, with a median follow-up > 5 years, no differences in overall and aneurysm-related mortality. Chronic renal decline was significantly higher after OSR, while the reintervention rate was higher in the endovascular group. To achieve the best possible long-term outcomes, both techniques should be performed in high volume aortic centres, tailored to the patient, and with an adequate surveillance imaging.

This study investigated the long-term outcomes of patients treated with open surgical repair for complex abdominal aortic aneurysms (c-AAAs). A total of 119 patients with c-AAAs undergoing repair between January 2010 and June 2016 in a high-volume aortic center were included. The long-term imaging follow-up consisted of yearly abdominal ultrasound examinations and 5-year computed tomography angiography. At a median follow-up of 76 months (IQR 38 months), forty-three deaths (37%) and three (2.5%) aortic-related deaths were observed. Long-term chronic renal decline was observed in fifty (43.8%) patients, significantly correlated with post-operative acute kidney injury. During the follow-up, five reinterventions (4.3%) were performed. The present study suggests that open c-AAA repair can be performed with acceptable operative risk with durable results. To achieve the best possible long-term outcome, the open surgery repair of complex AAA should be performed in high-volume aortic centers and tailored to the patient.

We rely on a periodic public opinion poll indicator of the performance of mayors collected for 103 large cities in Italy and in three waves (2015, 2017, and 2020) to examine whether and to what extent the exogenous shift in policy-making decisions induced by the COVID-19 pandemic has affected citizens' perceptions regarding attributions of responsibility. We leverage the variation in political alignment between central and local governments and implement a difference-in-differences research design, finding that when decisions are fully centralised (during the lockdown), voter approval for the mayor of an aligned city decreases by around 7%. Further analyses suggest that our results are more marked (i) during pre-electoral years and (ii) in cities with a lower level of social capital. Lastly, we document that the decrease in the approval ratings of aligned mayors is entirely guided by cities not severely hit by the pandemic, thereby reflecting a sense of 'discontent' in these areas for the policy decisions adopted by the central government to tackle the pandemic., Competing Interests: The authors declare that they have no relevant or material financial interests that relate to the research described in this paper., (© 2021 The Authors.)

This paper presents first-hand evidence of the impact of Covid-19 on the re-allocation of migrants. I use monthly data on the migrants in reception centres and on daily arrivals in Italy during the period from October 2017 to October 2020, combined with information on Covid-19 cases across Italian regions. I employ a difference-in-differences design, finding that the presence of migrants decreased approximately 7% points more in regions highly exposed to the pandemic as compared to those less affected by Covid-19. In practice, migrants in second-line reception centres are reduced by approximately 381 units when considering a region less affected by the pandemic, and by around 2150 units in regions severely hit by the Covid-19 outbreak. Finally, back-of-the-envelope calculations suggest that in more affected regions, such an unusual reallocation of migrants implies potential savings in the range of 60-94 million euros, corresponding to about a 30-90% reduction in spending on migrant, refugee, and asylum seekers in these regions, whereas the reduction is of roughly 3-6% in less exposed areas., Supplementary Information: The online version contains supplementary material available at 10.1007/s40888-022-00262-y., (© Springer Nature Switzerland AG 2022.)

Objective: The aim of this study was to evaluate the peri-operative and short term results of institutional experience with the Distal Extended EndoVascular Aortic Repair PETTICOAT (DEEVAR PETTICOAT) procedure., Methods: This was a single centre, observational study. From January 2015 to December 2019, 53 patients were admitted to the institution for treatment of acute and subacute complicated type B aortic dissection. Among them, data on 16 patients deemed suitable for a PETTICOAT procedure extended at infrarenal and iliac level by means of a bifurcated endograft were prospectively collected. Patients with persistent pain, visceral or lower limb malperfusion, true lumen collapse, and distal extension with computed tomography angiography (CTA) evidence of entry tears in the infrarenal or iliac zone were included. A CTA scan was performed in all patients at the baseline, before hospital discharge and then at six and 12 months post-operatively to assess aortic remodelling. Complete aortic remodelling was defined as stable aortic size (maximum enlargement < 5 mm) with complete true lumen re-expansion and complete false lumen exclusion or disappearance., Results: Technical success was obtained in all patients. One patient (7%) had intra-operative infrarenal aortic rupture during balloon dilation of the distal end of the bare stent, successfully treated by deployment of a bifurcated endograft. Additional covered stenting of the visceral arteries was necessary in nine patients (56%) and malperfusion resolved immediately in all cases. There were no spinal cord ischaemia or other peri-procedural complications, and no post-operative death, stroke, paraplegia/paraparesis, or acute renal failure was observed. Follow up ranged from one month to 24 months (median 7.5 months). One patient (7%) with a history of drug abuse died from sepsis. At the last CTA check, all patients showed complete remodelling of the thoraco-abdominal aorta and iliac arteries without vessel enlargement., Conclusion: The present findings suggest that the DEEVAR PETTICOAT procedure provides effective sealing of all distal tears without increased risk of major peri-procedural complications. Further studies with larger number of patients and longer follow up are needed to confirm the safety and durability of this technique., (Copyright © 2021 European Society for Vascular Surgery. Published by Elsevier B.V. All rights reserved.)

Congenital anemias may be complicated by immune-mediated hemolytic crisis. Alloantibodies are usually seen in chronically transfused patients, and autoantibodies have also been described, although they are rarely associated with overt autoimmune hemolytic anemia (AIHA), a serious and potentially life-threatening complication. Given the lack of data on the AIHA diagnosis and management in congenital anemias, we retrospectively evaluated all clinically relevant AIHA cases occurring at a referral center for AIHA, hemoglobinopathies, and chronic hemolytic anemias, focusing on clinical management and outcome. In our cohort, AIHA had a prevalence of 1% (14/1410 patients). The majority were warm AIHA. Possible triggers were recent transfusion, infection, pregnancy, and surgery. All the patients received steroid therapy as the first line, and about 25% required further treatment, including rituximab, azathioprine, intravenous immunoglobulins, and cyclophosphamide. Transfusion support was required in 57% of the patients with non-transfusion-dependent anemia, and recombinant human erythropoietin was safely administered in one third of the patients. AIHA in congenital anemias may be challenging both from a diagnostic and a therapeutic point of view. A proper evaluation of hemolytic markers, bone marrow compensation, and assessment of the direct antiglobulin test is mandatory.

Background: Despite the great evolution of endograft devices for thoracic endovascular aortic repair (TEVAR), threatening related complication such as graft migration and endoleaks still occur during follow up. The Drag Forces (DF), that is the displacement forces that play a role in graft migration and endoleaks caused by the blood flow against the thoracic graft, can be studied by means of Computational Fluid Dynamics (CFD)., Method: A general review of papers found in current literature was performed. CFD studies available on the topic of thoracic aortic diseases and DF were analyzed. All anatomic, hemodynamics or graft related factors which could have an impact on DF were reported., Results: Different factors deeply influence DF magnitude in the different site of the Ishimaru's zones classification: angulation, tortuosity and length of the landing zone, graft diameter, length and deployment position, blood pressure, pulse waveform, blood viscosity and patient heart rate have been related to the magnitude of DF. Moreover, also the three-dimensional orientation of DF is emerging as a fundamental issue from CFD studies. DF can be divided in sideways and upward components. The former, even of higher magnitude in zone 0, maintain always an orthogonal orientation and does not change in any type of aortic arch; the latter result strictly related to the anatomic complexity of the aortic arch with values up to four times higher in zone 3., Conclusion: Different DF magnitude and orientation could explain how TEVAR have higher rate of migration and endoleaks when we face with more complex aortic anatomies. All these aspects should be foreseen during the planning of TEVAR procedure. In this field, collaboration between physicians and engineers is crucial, as both parts have a primary role in understanding and describing hidden aspects involved in TEVAR procedures., (Copyright © 2021 Elsevier Inc. All rights reserved.)

We use hand-collected data on penalty kicks in the top-level football competitions across France, Germany, Italy, Spain, and the United Kingdom over the 2019/2020 season to analyse how social environment affects the performance of individuals. We exploit the Covid-19 outbreak to induce a plausible source of variation in the supporters' attendance. We find that for home teams the probability of missing a penalty increases when matches are forced to be played behind closed doors, while visiting teams are less likely to choke on a penalty kick, with these effects being more pronounced when the level of attendance (measured before the pandemic) was high. Taken together, these findings indicate that not only a supportive audience, but also the size of the support plays a key role for success of skill tasks., (© 2021 The Author(s).)

Background: The aim of the present study was to evaluate early results of thoracic endovascular aortic repair (TEVAR) using the Valiant Navion™ stent graft in a "real-world" scenario., Methods: All patients who underwent TEVAR with the Valiant Navion™ endograft between November 2018 and November 2019 were included in this retrospective multicenter study (six European centers). The primary endpoints were technical success, incidence of major adverse events (MAEs), access failure, deployment failure, deployment accuracy, and rate of intraoperative endoleaks (ELs)., Results: One hundred-sixteen patients with varying thoracic aortic diseases were included. Eighteen patients (15.5%) were treated for an off-label condition. The technical success rate was 100%, without any access or deployment failures. The proximal and distal deployment accuracy rates were 99.1% and 97.4%, respectively. There were no intraoperative MAEs, including death. Two (1.7%) type Ib ELs were detected at the first postoperative CTA, all of which were in off-label procedures and related to the short length of the sealing neck. No type III ELs were detected. The median hospitalization time was 8 days (IQR 4-12), including a median intensive care unit stay of 1 day (IQR 1-2). The in-hospital mortality rate was 4.3%. At a median follow-up time of 98 days (IQR 39-187), there were no aortic-related mortalities or new onset of endoleaks., Conclusion: Our initial experience with the Valiant Navion™ endograft in a wide variety of aortic diseases showed safe early outcomes, especially for on-label procedures., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Hemophagocytic lymphohistocytosis (HLH) is a rare hyperinflammatory condition which may be primary or secondary to many diseases, including hematologic malignancies. Due to its life-threatening evolution, a timely diagnosis is paramount but challenging, since it relies on non-specific clinical and laboratory criteria. The latter are often altered in other diseases, including autoimmune cytopenias (AIC), which in turn can be secondary to infections, systemic autoimmune or lymphoproliferative disorders. In the present article, we describe two patients presenting at the emergency department with acute AICs subsequently diagnosed as HLH with underlying diffuse large B cell lymphoma. We discuss the diagnostic challenges in the differential diagnosis of acute cytopenias in the internal medicine setting, providing a literature review of secondary HLH and AIC.

Since COVID-19 was declared a pandemic, countries on the same pandemic trajectory have adopted very different lockdown strategies. Using data for over 132 countries, and employing an event-study design, this paper identifies the role of political, economic and institutional factors in explaining the differential timing and intensity of stringency measures undertaken., (© 2020 Published by Elsevier B.V.)

Objective: The aim of this study was to evaluate early and late results of hybrid repair techniques for Kommerell's diverticulum (KD)., Methods: All patients who underwent hybrid repair (thoracic endovascular aortic repair + supra-aortic debranching) for KD between 2009 and 2018 were included in this retrospective multicenter study (three Italian centers). A proximal landing zone (PLZ) of at least 2 cm of healthy aorta was considered adequate for the deployment of a standard thoracic stent graft. The early end points were technical success, in-hospital mortality, and cerebrovascular events. Late outcomes included survival, reintervention, and patency of supra-aortic debranching. We used an embryogenetic anomaly based aortic arch classification for PLZ evaluation to identify the most appropriate hybrid adjunct., Results: Sixteen patients with KD were included. According to the aforementioned classification, stent graft deployment was required in six patients (37.5%) in PLZ 0, nine patients (56.3%) in PLZ 1, and one patient (6.3%) in PLZ 2. Technical success was achieved in all patients. One patient (6.3%) died in the hospital because of posterior cerebral hemorrhage after total debranching (PLZ 0). No further cerebrovascular events were observed. One patient (6.3%) had an asymptomatic left subclavian artery-right left subclavian artery bypass occlusion and required early reintervention. The 30-day secondary patency of supra-aortic debranching was 100%. Two type II endoleaks (12.5%) were detected at 1 month through computed tomography angiography. Further transient complications were found in three cases: hemidiaphragm paralysis in one patient and recurrent laryngeal nerve paralysis in two patients. At a mean follow-up of 48 months, four patients had died because of nonaortic reasons, and one RCCA-right subclavian artery bypass had lost its patency. None of the patients reported any growth of KD after hybrid repair. Ten patients (62.5%) showed aneurysmal sac shrinkage of at least 5 mm., Conclusions: Hybrid repair is confirmed to be a safe and effective approach for KD. Operative risk is associated primarily with the invasiveness of the hybrid adjunct., (Copyright © 2019 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

High-technology medicine saves lives and produces waste; this is the case of dialysis. The increasing amounts of waste products can be biologically dangerous in different ways: some represent a direct infectious or toxic danger for other living creatures (potentially contaminated or hazardous waste), while others are harmful for the planet (plastic and non-recycled waste). With the aim of increasing awareness, proposing joint actions and coordinating industrial and social interactions, the Italian Society of Nephrology is presenting this position statement on ways in which the environmental impact of caring for patients with kidney diseases can be reduced. Due to the particular relevance in waste management of dialysis, which produces up to 2 kg of potentially contaminated waste per session and about the same weight of potentially recyclable materials, together with technological waste (dialysis machines), and involves high water and electricity consumption, the position statement mainly focuses on dialysis management, identifying ten first affordable actions: (1) reducing the burden of dialysis (whenever possible adopting an intent to delay strategy, with wide use of incremental schedules); (2) limiting drugs and favouring "natural" medicine focussing on lifestyle and diet; (3) encouraging the reuse of "household" hospital material; (4) recycling paper and glass; (5) recycling non-contaminated plastic; (6) reducing water consumption; (7) reducing energy consumption; (8) introducing environmental-impact criteria in checklists for evaluating dialysis machines and supplies; (9) encouraging well-planned triage of contaminated and non-contaminated materials; (10) demanding planet-friendly approaches in the building of new facilities.

Background: Data from the literature suggest that in patients with acute, complicated type B aortic dissection (cTBAD), initial successful treatment with thoracic endovascular aneurysm repair (TEVAR) is not necessarily associated with favorable remodeling of the dissected aorta during follow-up, and long-term results indicate that TEVAR failed to completely suppress false lumen patency. Sealing of all relevant distal reentries, infrarenal and/or iliac, seems to be the key issue to induce total false lumen thrombosis in abdominal aorta as well as the iliac arteries, especially in complicated patients presenting with malperfusion or complete true lumen collapse., Materials and Methods: Of the 34 consecutive patients diagnosed with cTBAD at our hospital from January 2015 to April 2018, 30 had complicated dissections receiving endovascular treatment according to the standard Provisional ExTension To Induce COmplete ATtachment (PETTICOAT) technique and were excluded from this study, whereas 4 patients with radiologic evidence of multiple reentry tears at detached lumbar arteries and iliac bifurcation with complete true lumen collapse and clinical evidence of malperfusion were treated with a modified PETTICOAT technique with distal extension of the aortic stent, balloon expansion of the stented true lumen, and use of the AFX bifurcated endograft system to preserve the natural aortic bifurcation and provide complete distal sealing of reentry tears., Results: Primary technical success was achieved in all patients. No postoperative deaths were observed, but 1 patient experienced an hemorrhagic shock on the second postoperative day. No patient suffered postoperative stroke, paraplegia, paraparesis, or acute renal failure., Conclusions: Using an abdominal aortic bifurcated endograft with PETTICOAT to treat acute cTBAD seems to be a feasible approach in high-risk patients to improve aortic remodeling. The AFX bifurcated endograft system meets the requirements of anatomical fixation and sealing of distal tears., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Connective tissue disease (CTD) represents a group of genetic conditions characterized by disruptive matrix remodeling. When this process involves aortic and vascular wall, patients with CTD have a high risk of developing arterial aneurysms, dissections and ruptures. Open surgical repair is still the gold standard therapy for patients with CTD with reasonable morbidity and mortality risk. The surgical treatment of CTD often requires multiple operations. In the endovascular era, fenestrated and branched stent grafts may play a role in reducing the complications of multiple open operations. Although the long-term results of endovascular treatment in the setting of CTD are unknown, it is generally accepted that endovascular treatment is restricted to selected patients with high surgical risk. In an emergency setting, endovascular intervention can serve as a lifesaving bridge to elective open aortic repair. Aortic centers performing a large volume of complex open and endovascular aortic repairs have started to combine these two techniques in a staged fashion. The goal is to reduce the morbidity and mortality associated with extensive aortic repairs in CTD patients. For this reason, recommend endovascular therapy when a "graft-to-graft" approach is possible. In this scenario, the surgeon who performs the open repair must take into consideration future interventions. Surgical repair in any aortic segment should allow creation of proximal and distal landing zones over 4 cm to secure the sealing of a future stent graft. Connective tissue disease should be treated with a multidisciplinary approach, in high volume centers. Endovascular treatment represents a potential option in patients at high risk for open repair. Staged hybrid procedures have emerged as a way to reduce spinal cord ischemia and avoid multiple open surgeries. The aim of this article is to discuss the management of aortic diseases in CTD, focusing on to the role of standard open surgery and emerging endovascular treatment, and to give an overview of the few series published regarding this topic with a small number of patients.

Chronic aortic dissecting aneurysms (TAAD) presenting after acute Stanford type A or B dissection includes both arch and/or thoracoabdominal aortic aneurysms (TAAA). Approximately 60% of patients who survive surgical treatment of acute type A aortic dissections will require another aortic procedure. Similarly, more than 70% of patients with chronic type B aortic dissections will experience false lumen dilation at 5-year follow-up, often requiring intervention. Open or hybrid aortic repairs of complex TAAD involving the arch and the TAAA are very demanding procedures for both patients and clinicians. Open surgery remains the first line therapy in fit patients. Recent development of branched arch devices has offered an alternative option for high-risk patients. Technical challenges associated with the endovascular management of these complex aneurysms include proximal sealing zone often located in the aortic arch or the ascending aorta, narrow true lumen working space, and aortic branch perfusion by either the true or false lumen, or both. Recent studies have reported encouraging results with endovascular treatment of these complex dissecting aneurysms, especially following open ascending aortic repair. The aim of this review was to describe the available strategies for arch repair in the setting of a chronic TAAD and to determine the subset of patients that can benefit from of a totally endovascular approach.

Aim: Nephrocalcinosis is a clinical-pathological entity characterized by the deposition of calcium salts within the kidney parenchyma. Both the protean presentation and multiple causes may explain the lack of data regarding its prevalence. The aim of this study is to report the prevalence and main clinical features of nephrocalcinosis diagnosed in a newly opened nephrology outpatient unit., Methods: Analysis on the data we prospectively gathered from the start of activity (2007-2013) was carried out. Clinical and laboratory data were collected from the medical records and from the general laboratory; diagnosis was based upon imaging data reviewed by the same radiologists., Results: Sixty-five of 2695 patients referred to our unit were diagnosed with nephrocalcinosis (2.4%). The affected patients were younger than the overall out-patient population (median: 37.7 (min-max: 8-82) vs 63 years (2-102) P < 0.001), with higher female prevalence (68% vs 51.4%: P < 0.05) and better preserved kidney function (CKD-EPI 103 (23-165) vs 60 mL/min (3.2-169) P < 0.001). Kidney stones were the main reason for referral (35.4%), followed by electrolyte disturbances (22.7%), acute pyelonephritis (4.6%), AKI or CKD (4.6%). Nephrocalcinosis was associated with autoimmune diseases in 29% and with microcythaemia in 23%, while positive family history was present in 23% of patients. Various electrolyte disturbances were observed, with hypercalciuria being the hallmark of beta thalassaemic patients., Conclusions: Nephrocalcinosis is a rare, but not exceptional disease in nephrology. In Mediterranean countries, microcythaemia would appear to be a major cause of this disease. Greater awareness of nephrocalcinosis is needed for an integrated approach involving various branches of internal medicine and radiology., (© 2015 Asian Pacific Society of Nephrology.)

Severe hyperparathyroidism is a challenge on hemodialysis. The definition of dialysate calcium (Ca) is a pending issue with renewed importance in cases of individualized dialysis schedules and of portable home dialysis machines with low-flow dialysate. Direct measurement of calcium mass transfer is complex and is imprecisely reflected by differences in start-to-end of dialysis Ca levels. The study was performed in a dialysis unit dedicated to home hemodialysis and to critical patients with wide use of daily and tailored schedules. The Ca-phosphate (P)-parathyroid hormone (PTH) profile includes creatinine, urea, total and ionized Ca, albumin, sodium, potassium, P, PTH levels at start, mid, and end of dialysis. "Severe" secondary hyperparathyroidism was defined as PTH > 300 pg/mL for ≥3 months. Four schedules were tested: conventional dialysis (polysulfone dialyzer 1.8-2.1 m(2) ), with dialysate Ca 1.5 or 1.75 mmol/L, NxStage (Ca 1.5 mmol/L), and NxStage plus intradialytic Ca infusion. Dosages of vitamin D, calcium, phosphate binders, and Ca mimetic agents were adjusted monthly. Eighty Ca-P-PTH profiles were collected in 12 patients. Serum phosphate was efficiently reduced by all techniques. No differences in start-to-end PTH and Ca levels on dialysis were observed in patients with PTH levels < 300 pg/mL. Conversely, Ca levels in "severe" secondary hyperparathyroid patients significantly increased and PTH decreased during dialysis on all schedules except on Nxstage (P < 0.05). Our data support the need for tailored dialysate Ca content, even on "low-flow" daily home dialysis, in "severe" secondary hyperparathyroid patients in order to increase the therapeutic potentials of the new dialysis techniques., (© 2015 International Society for Hemodialysis.)

Background: Approximately 2 million chronic haemodialysis patients produce over 2,000,000 tons of waste per year that includes about 600,000 tons of potentially hazardous waste. The aim of the present study was to analyse the characteristics of the waste that is produced through chronic haemodialysis in an effort to identify strategies to reduce its environmental and financial impact., Methods: The study included three dialysis machines and disposables for bicarbonate dialysis, haemodiafiltration (HFR) and lactate dialysis. Hazardous waste is defined as waste that comes into contact with bodily fluids. The weight and cost of waste management was evaluated by various policies of differentiation, ranging from a careful-optimal differentiation to a careless one. The amount of time needed for optimal management was recorded in 30 dialysis sessions. Non-hazardous materials were assessed for potential recycling., Results: The amount of plastic waste that is produced per dialysis session ranges from 1.5 to 8 kg (from 1.1 to 8 kg of potentially hazardous waste), depending upon the type of dialysis machine and supplies, differentiation and emptying policies. The financial cost of waste disposal is high, and is mainly related to hazardous waste disposal, with costs ranging from 2.2 to 16 Euro per session (2.7-21 USD) depending on the waste management policy. The average amount of time needed for careful, optimal differentiation disposal is approximately 1 minute for a haemodialysis session and 2 minutes for HFR. The ecological cost is likewise high: less than one-third of non-hazardous waste (23-28%) is potentially recyclable, while the use of different types of plastic, glues, inks and labels prevents the remaining materials from being recycled., Conclusion: Acknowledging the problem of waste management in dialysis could lead to savings of hundreds of millions of Dollars and to the reuse and recycling of hundreds of tons of plastic waste per year on a world-wide scale with considerable financial and ecological savings., (© The Author 2015. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.)

In the medical field, the attention to the environmental impact of industrial processes and products is still limited. In recent years there has been an increased sensitivity towards the environment; meanwhile, the economic burden of hazardous waste disposal is becoming evident. Dialysis is a "big producer" of waste and it has been estimated that disposal costs can be up to 10-40% of the cost of disposables. So there are several reasons of interest on "ecodialysis": the high amount of waste defined as "potentially hazardous", which requires a very expensive management and the recyclability potential of the non-contaminated waste, that has not yet been fully explored in dialysis. This primary study has been performed in collaboration with the Politecnico di Torino. Its aim has been to define a schedule of activities by a few brainstorming sessions. This schedule is to be readily performed or it should be developed in detail to optimize, by reducing and recycling, the waste production during the dialysis session. The discussion identified seven basic points for the eco-sustainability of haemodialysis to: [1] reduce packaging; [2] facilitate separation of materials, and [3] their discharge; [4] differentiate materials; [5] clearly highlight the potentially hazardous materials; [6] improve the recyclability of plastic products; [7] propose a path of recovery and reuse. Although a full optimization requires a close cooperation with the manufacturers and is achievable only in the long term, the reduction of one pound of potentially contaminated materials could presently lead, on a national scale, to a saving of several million euros, which can be better employed in investments to improve our treatments.

Objectives: Low protein diets (LPDs) are milestones in chronic kidney disease (CKD). Concerns over compliance and safety limit their use. The aim of this study was to test the feasibility and main results of a multiple-choice approach to LPDs, adapted to patient preferences., Methods: From December 2007 to January 2013, all CKD patients (stages 4/5; progressive stage 3) without contraindications (malnutrition, short life expectancy), were offered two main LPDs (proteins 0.6 g/kg daily): Vegan supplemented (LPD-KA) or with "aproteic" commercial food (LPD-ACF). LPDs followed a qualitative approach based on forbidden and allowed food; one to three free meals per week, and flexible control policy (1-3 mo). Start of dialysis, death, and combined outcome (death-dialysis) were analyzed by Kaplan-Meier curves and Cox model. Comparison with dialysis in patients with glomerular filtration rate (GRF) <15 mL/min, (corresponding to "early" dialysis start) employed standardized mortality rates, with respect to the Italian and the United States Dialysis Registry., Results: One hundred eighty-five patients (222 patient-years) started at least a trial of LPD-KA, 122 (177 patients-years) LPD-ACF; only 3 patients with GFR <30 mL/min denied an LPD trial. Patients who chose LPD-KA were younger than those on LPD-ACF (63 versus 74 y), had less comorbidity (82% versus 93%), higher proteinuria (1.4 versus 0.7 g/d) and lower GFR (17 versus 23 mL/min) (P < 0.001). Median daily protein intake was 0.7 g/kg on both diets (Maroni-Mitch formula). The combined outcome (death or dialysis) was not influenced by the diet chosen (Cox analysis). Relative risk for death on the diet (patients with GFR <15 mL/min) was 0.5 with respect to the Italian Registry and 0.3 to the United States Dialysis Registry. The diets had comparable costs (1 y on dialysis: 50 patient-years on LPD)., Conclusions: The choice of diet is strictly linked to patient characteristics, thus supporting a multiple-choice offer. Once corrected for baseline data, both LPDs led to similar results, suggesting at least survival equivalence with dialysis, at lesser cost., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background and Objectives: Women affected by CKD increasingly choose to get pregnant. Experience with low-protein diets is limited. The aim of this study was to review results obtained from pregnant women with CKD on supplemented vegan-vegetarian low-protein diets., Design, Setting, Participants, & Measurements: This was a single-arm, open intervention study between 2000-2012 of a low-protein diet in pregnant patients with stages 3-5 CKD or severe proteinuria (>1 g/d in the first trimester or nephrotic at any time). Stages 3-5 CKD patients who were not on low-protein diets for clinical, psychologic, or logistic reasons served as controls. The setting was the Obstetrics-Nephrology Unit dedicated to kidney diseases in pregnancy. The treated group included 24 pregnancies--21 singleton deliveries, 1 twin pregnancy, 1 abortion, and 1 miscarriage. Additionally, there were 21 controls (16 singleton deliveries, 5 miscarriages). The diet was a vegan-vegetarian low-protein diet (0.6-0.8 g/kg per day) with keto-acid supplementation and 1-3 protein-unrestricted meals allowed per week., Results: Treated patients and controls were comparable at baseline for median age (35 versus 34 years), referral week (7 versus 8), eGFR (59 versus 54 ml/min), and hypertension (43.5% versus 33.3%); median proteinuria was higher in patients on the low-protein diet (1.96 [0.1-6.3] versus 0.3 [0.1-2.0] g/d; P<0.001). No significant differences were observed in singletons with regard to gestational week (34 versus 36) or Caesarean sections (76.2% versus 50%). Kidney function at delivery was not different, but proteinuria was higher in the diet group. Incidence of small for gestational age babies was significantly lower in the diet group (3/21) versus controls (7/16; chi-squared test; P=0.05). Throughout follow-up (6 months to 10 years), hospitalization rates and prevalence of children below the third percentile were similar in both groups., Conclusion: Vegan-vegetarian supplemented low-protein diets in pregnant women with stages 3-5 CKD may reduce the likelihood of small for gestational age babies without detrimental effects on kidney function or proteinuria in the mother.

Background: Low-protein diets are often mentioned but seldom used to slow chronic kidney disease (CKD) progression. The aim of the study was to investigate the potential for implementation of a simplified low-protein diet supplemented with alpha-keto analogues (LPD-KA) as part of the routine work-up in CKD patients., Methods: In an implementation study (December 2007-November 2011), all patients with CKD Stages IV-V not on dialysis, rapidly progressive Stage III and/or refractory proteinuria, were offered either a simplified LPD-KA, or commercially available low-protein food. LPD-KA consisted of proteins 0.6 g/kg/day, supplementation with Ketosteril 1 pill/10 Kg, 1-3 free-choice meals/week and a simplified schema based on 'allowed' and 'forbidden' foods. 'Success' was defined as at least 6 months on LPD-KA. Progression was defined as reduction in glomerular filtration rate (GFR)[(Chronic Kidney Disease Epidemiology Collaboration) formula CKD-EPI] in patients with at least 6 months of follow-up., Results: Of about 2500 patients referred (8% CKD Stages IV-V), 139 started LPD-KA; median age (70 years) and prevalence of comorbidity (79%) were in line with the dialysis population. Start of dialysis was the main reason for discontinuation (40 cases, unplanned in 7); clinical reasons were recorded in 7, personal preference in 14 and improvement and death in 8 each. The low gross mortality (4% per year) and the progression rate (from -8 to 0 mL/min/year at 6 months) are reassuring concerning safety. None of the baseline conditions, including age, educational level, comorbidity or kidney function, discriminated the patients who followed the diet for at least 6 months., Conclusions: Our data suggest a wider offer of LPD-KA to patients with severe and progressive CKD. The promising results in terms of mortality and progression need confirmation with different study designs.

Background: Complementary and Alternative Medicines (CAMs) are increasingly practiced in the general population; it is estimated that over 30% of patients with chronic diseases use CAMs on a regular basis. CAMs are also used in hospital settings, suggesting a growing interest in individualized therapies. One potential field of interest is pain, frequently reported by dialysis patients, and seldom sufficiently relieved by mainstream therapies. Gentle-touch therapies and Reiki (an energy based touch therapy) are widely used in the western population as pain relievers.By integrating evidence based approaches and providing ethical discussion, this debate discusses the pros and cons of CAMs in the dialysis ward, and whether such approaches should be welcomed or banned., Discussion: In spite of the wide use of CAMs in the general population, few studies deal with the pros and cons of an integration of mainstream medicine and CAMs in dialysis patients; one paper only regarded the use of Reiki and related practices. Widening the search to chronic pain, Reiki and related practices, 419 articles were found on Medline and 6 were selected (1 Cochrane review and 5 RCTs updating the Cochrane review). According to the EBM approach, Reiki allows a statistically significant but very low-grade pain reduction without specific side effects. Gentle-touch therapy and Reiki are thus good examples of approaches in which controversial efficacy has to be balanced against no known side effect, frequent free availability (volunteer non-profit associations) and easy integration with any other pharmacological or non pharmacological therapy. While a classical evidence-based approach, showing low-grade efficacy, is likely to lead to a negative attitude towards the use of Reiki in the dialysis ward, the ethical discussion, analyzing beneficium (efficacy) together with non maleficium (side effects), justice (cost, availability and integration with mainstream therapies) and autonomy (patients' choice) is likely to lead to a permissive-positive attitude., Summary: This paper debates the current evidence on Reiki and related techniques as pain-relievers in an ethical framework, and suggests that physicians may wish to consider efficacy but also side effects, contextualization (availability and costs) and patient's requests, according also to the suggestions of the Society for Integrative Oncology (tolerate, control efficacy and side effects).

Background: Chronic kidney disease (CKD) and pre-eclampsia (PE) occur in 3-5% of pregnancies. They often share hypertension and proteinuria and a differential diagnosis may be impossible. However, in PE, the pathogenesis is related to abnormal placentation, which can be detected by abnormal uterine and umbilical Doppler flow velocities, while in CKD, an intrinsic kidney disease is present. We hypothesize that Doppler studies can help to differentiate PE from CKD, as the flow velocities are altered in PE and normal in CKD., Methods: We retrospectively selected patients who were followed in our Materno-Foetal Unit (2005-10) and had at least one flow measurement in our setting. CKD patients were included in the presence of proteinuria (≥ 300 mg/day) and hypertension, mimicking PE. The clinical charts were reviewed by the same operators; the clinical diagnoses were taken as reference. Three flow patterns were considered: alteration of both flow velocity waveforms (FVWs) (uterine and umbilical arteries), hypothesized as predictive of PE; normal FVWs at both levels, hypothesized as predictive of CKD; altered FVW in either artery, considered 'mixed'. Uterine FVWs were considered pathological according to the classical cut-point (RI > 0.58). Umbilical flows were evaluated according to standards adjusted for gestational age. Statistical analysis was performed in SPSS., Results: The analysis included 61 cases. The presence of normal FVWs was significantly associated with the diagnosis of CKD (P = 0.0018). Conversely, the presence of both altered flows was significantly associated with PE (P = 0.0233)., Conclusions: In the presence of proteinuria and hypertension, normal flows suggest CKD altered flows PE. Prospective studies are needed to refine this hypothesis based on the first Doppler criteria supporting the differential diagnosis between CKD and PE.

Background: Kidney diseases, which have a prevalence of 3% in women of childbearing age, are increasingly encountered in pregnancy. Glomerulonephritis may develop or flare up in pregnancy, and a differential diagnosis with pre-eclampsia may be impossible on clinical grounds. Use of kidney biopsy is controversial, but a systematic review has not been carried out to date., Objectives: To review the literature on kidney biopsy in pregnancy, with a focus on indications, risks and timing., Search Strategy: Medline, Embase, CHINAL and the Cochrane Library were searched in September 2012, with 'pregnancy' and 'kidney biopsy' used as MESH and free terms, for the period 1980-2012. Results were filtered for 'human' if this option was available., Selection Criteria: Biopsies during pregnancy and within 2 months after delivery. Case reports (fewer than five cases) and kidney grafts were excluded. Paper selection was performed in duplicate., Data Collection and Analysis: Data were extracted in duplicate. The high heterogeneity in study design necessitated that the review be narrative, except for data on adverse events, which were analysed with regard to the timing of kidney biopsy., Main Results: Of 949 references, 39 were selected, providing data on 243 biopsies in pregnancy and 1236 after delivery (timing was unclear in 106 women). The main aims of the studies were to define morphology in pre-eclampsia (23 studies), to carry out a risk-benefit analysis of kidney biopsy (11 studies), and to investigate pregnancy-related acute kidney injury (five studies). Four cases of major bleeding complications occurred at 23-26 weeks of gestation. Relevant complications were observed in 7% of women during pregnancy and 1% after delivery (P = 0.001). Kidney biopsy performed for the diagnosis of glomerulonephritis or pre-eclampsia led to therapeutic changes in 66% of cases., Authors' Conclusions: The evidence on kidney biopsy in pregnancy is heterogeneous, but a significantly higher risk of complications (relative to postpartum biopsy) was found, with a possible peak at around 25 gestational weeks., (© 2013 The Authors BJOG An International Journal of Obstetrics and Gynaecology © 2013 RCOG.)

Background: In the last decade, significant improvements have been achieved in maternal-fetal and diabetic care which make pregnancy possible in an increasing number of type 1 diabetic women with end-organ damage. Optimal counseling is important to make the advancements available to the relevant patients and to ensure the safety of mother and child. A systematic review will help to provide a survey of the available methods and to promote optimal counseling., Objectives: To review the literature on diabetic nephropathy and pregnancy in type 1 diabetes., Methods: Medline, Embase, and the Cochrane Library were scanned in November 2012 (MESH, Emtree, and free terms on pregnancy and diabetic nephropathy). Studies were selected that report on pregnancy outcomes in type 1 diabetic patients with diabetic nephropathy in 1980-2012 (i.e. since the detection of microalbuminuria). Case reports with less than 5 cases and reports on kidney grafts were excluded. Paper selection and data extraction were performed in duplicate and matched for consistency. As the relevant reports were highly heterogeneous, we decided to perform a narrative review, with discussions oriented towards the period of publication., Results: Of the 1058 references considered, 34 fulfilled the selection criteria, and one was added from reference lists. The number of cases considered in the reports, which generally involved single-center studies, ranged from 5 to 311. The following issues were significant: (i) the evidence is scattered over many reports of differing format and involving small series (only 2 included over 100 patients), (ii) definitions are non-homogeneous, (iii) risks for pregnancy-related adverse events are increased (preterm delivery, caesarean section, perinatal death, and stillbirth) and do not substantially change over time, except for stillbirth (from over 10% to about 5%), (iv) the increase in risks with nephropathy progression needs confirmation in large homogeneous series, (v) the newly reported increase in malformations in diabetic nephropathy underlines the need for further studies., Conclusions: The heterogeneous evidence from studies on diabetic nephropathy in pregnancy emphasizes the need for further perspective studies on this issue.

Background: Proteinuria and dilatation of the urinary tract are both relatively common in pregnancy, the latter with a spectrum of symptoms, from none to severe pain and infection. Proteinuria is a rare occurrence in acute obstructive nephropathy; it has been reported in pregnancy, where it may pose a challenging differential diagnosis with pre-eclampsia.The aim of the present study is to report on the incidence of proteinuria (≥ 0.3; ≥ 0.5 g/day) in association with symptomatic-severe urinary tract dilatation in pregnancy., Methods: Case series., Setting: Nephrological-Obstetric Unit dedicated to pregnancy and kidney diseases (January 2000-April 2011)., Source: database prospectively updated since the start of the Unit. Retrospective review of clinical charts identified as relevant on the database, by a nephrologist and an obstetrician., Results: From January 2000 to April 2011, 262 pregnancies were referred. Urinary tract dilatation with or without infection was the main cause of referral in 26 cases (predominantly monolateral in 19 cases): 23 singletons, 1 lost to follow-up, 1 twin and 1 triplet. Patients were referred for urinary tract infection (15 cases) and/or renal pain (10 cases); 6 patients were treated by urologic interventions ("JJ" stenting). Among them, 11 singletons and 1 triple pregnancy developed proteinuria ≥ 0.3 g/day (46.1%). Proteinuria was ≥ 0.5 g/day in 6 singletons (23.1%). Proteinuria resolved after delivery in all cases. No patient developed hypertension; in none was an alternative cause of proteinuria evident. No significant demographic difference was observed in patients with renal dilatation who developed proteinuria versus those who did not. An association with the presence of "JJ" stenting was present (5/6 cases with proteinuria ≥ 0.5 g/day), which may reflect both severer obstruction and a role for vescico-ureteral reflux, induced by the stent., Conclusions: Symptomatic urinary tract dilatation may be associated with proteinuria in pregnancy. This association should be kept in mind in the differential diagnosis with other causes of proteinuria in pregnancy, including pre-eclampsia.

Preeclampsia, affecting 5-8% of pregnancies, is the main cause of fetal-maternal mortality and morbidity. The differential diagnosis with chronic kidney disease (CKD) is a challenge owing to the overlapping clinical features. No biomarker has been found to discriminate between the two conditions. Here, we tested whether maternal serum levels of placental growth factor (PlGF) and soluble FMS-like tyrosine kinase-1 (sFlt-1), markers of preeclampsia, could be used to discriminate between 34 patients with preeclampsia, 23 patients with CKD during pregnancy, and 38 healthy pregnant women. Serum levels of PlGF and sFlt-1 were determined during the third trimester by commercially available immunoassays. In preeclampsia, sFlt-1 levels were significantly increased in comparison with that in CKD and in the control women. Serum levels of PlGF in preeclampsia were significantly decreased relative to both controls and patients with CKD. The sFlt-1 to PlGF ratio was significantly increased in preeclampsia (median 436) compared with controls (median 9.4) and CKD (median 4.0). No differences were found between controls and patients with CKD. Thus, our study suggests that it is possible to discriminate between preeclampsia and CKD during pregnancy by determining maternal serum levels of sFlt-1 and PlGF and their ratio.

Background and Objectives: CKD and multiple pregnancies bear important risks for pregnancy outcomes. The aim of the study was to define the risk for adverse pregnancy-related outcomes in multiple pregnancies in CKD patients in comparison with a control group of "low-risk" multiple pregnancies., Design, Setting, Participants, & Measurements: The study was performed in the Maternal Hospital of the University of Turin, Italy. Of 314 pregnancies referred in CKD (2000-2011), 20 were multiple (15 twin deliveries). Control groups consisted of 379 low-risk multiple pregnancies (314 twin deliveries) and 19 (15 twin deliveries) cases with hypertension-collagen diseases. Baseline data and outcomes were compared by univariate and logistic regression analyses., Results: The prevalence of multiple pregnancies was relatively high in the CKD population (6.4%); all referred cases were in early CKD stages (I-II); both creatinine (0.68 to 0.79 mg/dl; P=0.010) and proteinuria (0.81 to 3.42 g/d; P=0.041) significantly increased from referral to delivery. No significant difference in demographic data at baseline was found between cases and low-risk controls. CKD was associated with higher risk of adverse pregnancy outcomes versus low-risk twin pregnancies. Statistical significance was reached for preterm delivery (<34 weeks: 60% vs 26.4%; P=0.005; <32 weeks: 53.3% vs 12.7%; P<0.001), small for gestational age babies (28.6% vs 8.1%; P<0.001), need for Neonatal Intensive Care Unit (60% vs 12.7%; P<0.001), weight discordance between twins (40% vs 17.8%; P=0.032), and neonatal and perinatal mortality (6.6% vs 0.8%; P=0.032)., Conclusion: This study suggests that maternal-fetal risks are increased in multiple pregnancies in the early CKD stages.

Background: The resurgence of home hemodialysis (HHD) underlines the importance of educational programs. Brainstorming is a powerful tool for innovation, widely employed in industry but seldom used in medicine. The aim of this study was to define an e-learning Web platform for HHD patients via a brainstorming approach., Methods: Four brainstorming sessions were held 2-6 weeks apart. Twelve people were involved: 2 dialysis physicians, 2 nurses, 2 HHD patients, 2 caregivers, a filmmaker, 2 computer experts (1 with a psychology degree) and a senior engineer. Each session was summarized as the starting point for the following one. The topics discussed were the platform structure and its logo., Results: For the platform, the following requirements were defined: teaching should be extensive and tailored to different levels of knowledge; all available teaching tools (tutorials, demonstrations, recorded and written materials) should be used; films enhance emotional participation and can be used to reduce fears; the contents should include general information on chronic kidney disease, details of all types of renal replacement therapy (RRT) (how and why), dialysis accidents, blood and imaging tests, laws and reimbursements, direct experiences and history of RRT. Remote monitoring and visual interactions are important for reassurance about HHD and should be provided. The requirements for the logo were that it be innovative, related to daily life, representative of a holistic approach and convey happiness. The logo "Hom-e-hem" was created, playing on the assonance between the religious term Om and the word home, with the e of electronic linking it with hem, short for hemodialysis., Conclusion: Brainstorming sessions can be used to design patient-tailored educational interventions. The key message, "self-care is a bridge from illness back to life," may apply to a wider context.

Background: Chronic kidney disease (CKD) has a high prevalence in pregnancy. In a period of cost constraints, there is the need for identification of the risk pattern and for follow-up., Methods: Patients were staged according to K-DOQI guidelines. The analysis was prospective, January 2000-June 2011. Two hundred and forty-nine pregnancies were observed in 225 CKD patients; 176 singleton deliveries were recorded. The largest group encompasses stage 1 CKD patients, with normal renal function, in which 127 singleton deliveries were recorded. No hard outcomes occurred (death; dialysis); therefore, surrogate outcomes were analysed [caesarean section, prematurity, need for neonatal intensive care unit (NICU)]. Stage 1 patients were compared with normal controls (267 low-risk pregnancies followed in the same setting) and with patients with CKD stages 2-4 (49 singleton deliveries); two referral patterns were also analysed (known diagnoses; new diagnoses)., Results: The risk for adverse pregnancy rises significantly in stage 1 CKD, when compared with controls: odds ratios were caesarean section 2.73 (1.72-4.33); preterm delivery 8.50 (4.11-17.57); NICU 16.10 (4.42-58.66). The risks rise in later stages. There is a high prevalence of new CKD diagnosis (overall: 38.6%; stage 1: 43.3%); no significant outcome difference was found across the referral patterns. Hypertension and proteinuria are confirmed as independent risk factors., Conclusions: CKD is a risk factor in pregnancy; all patients should be followed within dedicated programmes from stage 1. There is need for dedicated interventions and educational programmes for maximizing the diagnostic and therapeutic potentials in early CKD stages.

Background: Home hemodialysis (HHD) has met with alternating fortunes. The present revival of interest is due to lower costs and more frequent/efficient treatments. HHD is underdeveloped, and a marketing approach may help in defining development strategies. The aim of this study was to systematically review the recent literature (2000-2010) according to a marketing approach, defining the potential of HHD according to the classical marketing items: market size, growth rate, profitability, trends, keys for success, needs for structures and distribution channels., Methods: A Medline search was conducted for 2000-2010. The analysis took into account the recent trends in publication as a measure of interest, size and trends, while survival and costs were analyzed as keys for success. The issues of structures and distribution channels were arbitrarily considered as equivalent to the overall hemodialysis market., Results: Interest in HHD is growing, as shown by the increasing number of published papers (9 in 2000, 52 in 2010); yet, clinical studies accounted for less than half of the papers. In the 138 clinical studies, quality of life (33 papers) and metabolism (16 papers) were the most studied topics. Survival and cost analyses were highly heterogeneous (the broad inclusion of nocturnal or quotidian dialysis has to be mentioned). Overall, survival was equal to, or better than, that for other modalities, including transplantation and peritoneal dialysis; costs compared favorably with hospital dialysis and were equivalent to those of peritoneal dialysis., Conclusion: The small "market" of HHD is increasing, with potential for further growth, the keys for success being equivalence or superiority of survival at equivalent or lower costs.

The evolution of home dialysis marked the main steps in the progress of renal replacement therapy. From the origins when home hemodialysis was often the only alternative to death, to the advent and widespread use of peritoneal dialysis, the dream of kidney transplant as a solution to all problems (at least in the young), and ultimately the profound social and organizational changes that have led to a drastic reduction of home hemodialysis, we arrive at the present with the rediscovery of the clinical, rehabilitative and economic advantages of home dialysis. Seven experts from five different centers with different expertise in home dialysis report their opinions on the future of home dialysis in a ''noncontroversial controversy''. Beyond the sterile competition between peritoneal dialysis and home hemodialysis, the shared opinion is that the two methods may complement each other, allowing a tailored treatment for each patient and a tailored organization in each setting. The organizational solutions are many; the authors underline the importance of longer survival and better rehabilitation, and the ethical need of offering each patient a choice among all available treatments. Add to this the importance of dedicated educational programs targeted to physicians, nurses and patients alike and focused on self-care and patient empowerment. A new generation of dialysis machines, easier technical solutions, and financial incentives may strengthen motivations and simplify problems; all these elements may in the near future be combined in a joint effort to increase peritoneal dialysis and revive home hemodialysis in Italy.

Background: MELAS syndrome (MIM ID#540000), an acronym for Mitochondrial Encephalopathy, Lactic Acidosis and Stroke-like episodes, is a genetically heterogeneous mitochondrial disorder with protean manifestations and occasional kidney involvement. Interest in the latter is rising due to the identification of cases with predominant kidney involvement and to the hypothesis of a link between mitochondrial DNA and kidney neoplasia., Case Presentation: We report the case of a 41-year-old male with full blown MELAS syndrome, with lactic acidosis and neurological impairment, affected by the "classic" 3243A > G mutation of mitochondrial DNA, with kidney cancer. After unilateral nephrectomy, he rapidly developed severe kidney functional impairment, with nephrotic proteinuria. Analysis of the kidney tissue at a distance from the two tumor lesions, sampled at the time of nephrectomy was performed in the context of normal blood pressure, recent onset of diabetes and before the appearance of proteinuria. The morphological examination revealed a widespread interstitial fibrosis with dense inflammatory infiltrate and tubular atrophy, mostly with thyroidization pattern. Vascular lesions were prominent: large vessels displayed marked intimal fibrosis and arterioles had hyaline deposits typical of hyaline arteriolosclerosis. These severe vascular lesions explained the different glomerular alterations including ischemic and obsolescent glomeruli, as is commonly observed in the so-called "benign" arteriolonephrosclerosis. Some rare glomeruli showed focal segmental glomerulosclerosis; as the patient subsequently developed nephrotic syndrome, these lesions suggest that silent ischemic changes may result in the development of focal segmental glomerulosclerosis secondary to nephron loss., Conclusions: Nephron loss may trigger glomerular sclerosis, at least in some cases of MELAS-related nephropathy. Thus the incidence of kidney disease in the "survivors" of MELAS syndrome may increase as the support therapy of these patients improves.

A total of 72 patients (73 hips) who had been submitted to Girdlestone arthroplasty to treat aseptic (63%) and septic (37%) loosening of total hip replacement, and who had been followed-up after an average of 6.8 years, were reviewed. Good pain control was obtained in nearly 60% of the patients. Functional results were poor, but 52% of the patients said that they were satisfied. Mean score based on the Harris Hip Score was 54. Infection was dominated in 92.6% of the cases. The best results were obtained in younger patients, while we did not observe any differences based on the cause of loosening (aseptic or septic). Despite the limits of the method, Girdlestone arthroplasty continues to constitute an effective indication for the treatment of loosening in total hip replacement, when the choice of reimplantation exposes the patient to a high risk of further failure.