Inferior vena cava filters: Concept review and summary of current guidelines.

Anticoagulation is the first-line approach in the prevention and treatment of pulmonary embolism. In some instances, however, anticoagulation fails, or cannot be administered due to a high risk of bleeding. Inferior vena cava filters are metal alloy devices that mechanically trap emboli from the deep leg veins halting their transit to the pulmonary circulation, thus providing a mechanical alternative to anticoagulation in such conditions. The Greenfield filter was developed in 1973 and was later perfected to a model that could be inserted percutaneously. Since then, this model has been the reference standard. The current class I indication for this device includes absolute contraindication to anticoagulants in the presence of acute thromboembolism and recurrent thromboembolism despite adequate therapy. Additional indications have been more recently proposed, due to the development of removable filters and of progressively less invasive techniques. Although the use of inferior vena cava filters has solid theoretical advantages, clinical efficacy and adverse event profile are still unclear. This review analyzes the most important studies related to such devices, open issues, and current guideline recommendations.

Temporomandibular joint involvement in patients with Juvenile Idiopathic Arthritis: comparison of ultrasonography and magnetic resonance imaging in assessing the periarticular space width.

OBJECTIVES: This study aimed to compare the performance of Ultrasonography (US) and Magnetic Resonance Imaging (MRI) in assessing the Lateral Periarticular Space (LPAS) of Temporomandibular Joints (TMJs) in patients with Juvenile Idiopathic Arthritis (JIA). METHODS: The LPAS width was evaluated in two different patient groups. In the JIA group, including 29 children (13 ± 2.8 years) with JIA, the LPAS width was measured with both MRI and US. In the healthy group, including 28 healthy children (12.6 ± 2.5 years), the LPAS width was measured only with US. Comparisons of LPAS width based on patient groups and TMJ contrast enhancement in MRI were evaluated by applying the Mann-Whitney U test. Correlation and agreement between MRI and US measurements in JIA group were tested using Spearman rank correlation and Bland-Altman method. RESULTS: The LPAS width was significantly greater in the JIA group than in the healthy group. In the JIA group, the LPAS width was significantly greater in TMJs with moderate/severe enhancement than those with mild enhancement. A positive significant correlation between MRI and US measurements of LPAS width was found in the JIA group. In the same group, Bland-Altman method showed a good level of agreement between MRI and US measurements. CONCLUSION: Although, US cannot replace MRI in the evaluation of TMJ in patients with JIA, US could be used as a supplementary imaging method to MRI in assessing the TMJ disease.

Lung Dysfunction and Chronic Kidney Disease: A Complex Network of Multiple Interactions.

Chronic kidney disease (CKD) is a progressive disease that affects > 10% of the total population worldwide or >800 million people. CKD poses a particularly heavy burden in low- and middle-income countries, which are least able to cope with its consequences. It has become one of the leading causes of death worldwide and is one of the few non-communicable diseases where the number of related deaths has increased over the last two decades. The high number of people affected, and the significant negative impact of CKD should be a reason to increase efforts to improve prevention and treatment. The interaction of lung and kidney leads to highly complex and difficult clinical scenarios. CKD significantly affects the physiology of the lung by altering fluid homeostasis, acid-base balance and vascular tone. In the lung, haemodynamic disturbances lead to the development of alterations in ventilatory control, pulmonary congestion, capillary stress failure and pulmonary vascular disease. In the kidney, haemodynamic disturbances lead to sodium and water retention and the deterioration of renal function. In this article, we would like to draw attention to the importance of harmonising the definitions of clinical events in pneumology and renal medicine. We would also like to highlight the need for pulmonary function tests in routine clinical practise for the management of patients with CKD, in order to find new concepts for pathophysiological based disease-specific management strategies.

Lipoprotein(a) Modulates Carotid Atherosclerosis in Metabolic Syndrome.

Background and Aim: High lipoprotein(a) [Lp(a)] is a well-established cardiovascular (CV) risk factor, but the effect of mildly elevated Lp(a) on CV health is largely unknown. Our aim was to evaluate if Lp(a) is associated with the severity of carotid atherosclerosis (CA) in the specific subset of metabolic syndrome (MetS). Patients and Methods: Subjects with diagnosed MetS and ultrasound-assessed CA were enrolled. Those patients were categorized according to the severity of CA (moderate vs. severe), and the circulating levels of Lp(a) alongside with clinical, anthropometric, and biochemical data were collected. Results: Sixty-five patients were finally included: twenty-five with moderate and forty with severe CA (all with asymptomatic disease). Intergroup comparison showed Lp(a) as the only significantly different variable [6 (2-12) mg/dl vs. 11.5 (6-29.5) mg/dl; p = 0.018]. Circulating levels of Lp(a) were also confirmed as the only variable independently associated with severity of CA at logistic regression analysis [OR 2.9 (95% CI 1.1-7.8); p = 0.040]. ROC curve analysis for Lp(a) confirmed a serum level of 10 mg/dl as the best cut-off value [AUC 0.675 (95% CI 0.548-0.786)]. Although sensitivity and specificity were suboptimal (69.0 and 70.4%, respectively)-likely due to the small sample size-this result is in line with those previously reported in the literature. Conclusion: Lp(a) is independently associated with severity of CA in the subgroup of MetS patients.

Unintentional Recovery of Parasitic Diversity Following Restoration of Red Deer (Cervus elaphus) in North-Western Italy.

Red deer (Cervus elaphus) populations in north-western Italy have been remodeled in recent decades. Multiple translocations and the spontaneous migration from Switzerland and France resulted in the successful redistribution of the red deer after human-driven extirpation during the 18th century. The scarcely diverse parasitic community harbored by these cervids has been enriched with two species-specific taxa, Onchocerca jakutensis and Phayigomyia picta, suggesting that the recovery of parasitic biodiversity could be included amongst future conservation goals of this intensively managed game. Nodular onchocercosis was reported in three red deer populations since 2011, while nasal bots were reported since 2018. Hypoderma spp. larvae were identified for the first time in 1989, then a second record was made in 2014 in the province of Biella, where a yearling male in poor condition infested with Hypoderma diana was observed. In the perspective that the restoration of species-specific parasite communities of native mammals in Europe is increasingly perceived as a conservation target, with similar dignity as the conservation of their hosts, baseline data presented in this communication may give new insights for future parasite conservation efforts.

Role of Zinc in Diabetic Kidney Disease.

Diabetic Kidney Disease (DKD) represents the most common cause of Chronic Kidney Disease (CKD) in developed countries. Approximately 30% to 40% of diabetes mellitus (DM) subjects develop DKD, and its presence significantly increases the risk for morbidity and mortality. In this context, Zinc seems to have a potential role in kidney and body homeostasis in diabetic individuals as well as in patients at a high risk of developing this condition. This essential element has functions that may counteract diabetes-related risk factors and complications, which include stabilization of insulin hexamers and pancreatic insulin storage and improved glycemic control. In our review, we analyzed the current knowledge on the role of zinc in the management of renal impairment in course of DM. Several studies underline the critical role of zinc in reducing oxidative stress levels, which is considered the common denominator of the mechanisms responsible for the progression of kidney disease. Reaching and maintaining a proper serum zinc level could represent a valuable target to reduce symptoms related to DM complications and contrast the progression of kidney impairment in patients with the high risk of developing end-stage renal disease. In conclusion, analyzing the beneficial role of zinc in this review would advance our knowledge on the possible strategies of DM and DKD treatment.

Remote digital monitoring during the retention phase of orthodontic treatment: A prospective feasibility study.

Objective: To evaluate if a remote digital monitoring system added at the end of orthodontic treatment could positively influence the retention phase by reducing the occurrence of misfit of removable appliances, number of emergency appointments (EA), and orthodontic relapse. Methods: Twenty-seven patients who completed active orthodontic treatment were divided into the study and control groups. In addition to the standard chairside follow-up appointments at month 1 (T1), month 3 (T2), month 6 (T3), the study group patients were monitored using Dental Monitoring® with monthly intra-oral scans. Occurrence of misfit of removable retainers, number of EAs, and intercanine width change were recorded for both groups. Differences in EAs and retainer fit were assessed using the chi-square test. Intra-group and inter-group differences in the intercanine width were assessed with Friedman test and Mann-Whitney U test, respectively (α = 0.05). Results: The study group showed a significantly lower occurrence of misfit of removable retainers (p = 0.027) compared to the control group. No significant inter- and intra-group difference was found in the EAs and intercanine width change at each time-point. Conclusions: Integrating remote monitoring systems, such as Dental Monitoring®, to the retention phase of the orthodontic treatment may lower the occurrence of misfit of removable retainers. However, a small sample size and a short observation period limit the strength of this evidence. These preliminary results tentatively suggest that remote monitoring technologies may be beneficial, especially during the COVID-19 pandemic, when the regularity of in-office visits might be disrupted.

Proposed parameters of optimal central incisor positioning in orthodontic treatment planning: A systematic review.

OBJECTIVE: Planning of incisal position is crucial for optimal orthodontic treatment outcomes due to its consequences on facial esthetics and occlusion. A systematic summary of the proposed parameters is presented. METHODS: Studies on Google Scholar©, PubMed©, and Cochrane Library, providing quantitative information on optimal central incisor position were included. RESULTS: Upper incisors supero-inferior position (4-5 mm to upper lip, 67-73 mm to axial plane through pupils), antero-posterior position (3-4 mm to Nasion-A, 3-6 mm to A-Pogonion, 9-12 mm to true vertical line, 5 mm to A-projection, 9-10 mm to coronal plane through pupils), bucco-lingual angulation (4-7° to occlusal plane perpendicular on models, 20-22° to Nasion-A, 57-58° to upper occlusal plane, 16-20° to coronal plane through pupils, 108-110° to anterior-posterior nasal spine), mesio-distal angulation (5° to occlusal plane perpendicular on models). Lower incisors supero-inferior position (41-48 mm to soft-tissue mandibular plane), antero-posterior position (3-4 mm to Nasion-B, 1-3 mm to A-Pogonion, 12-15 mm to true vertical line, 6-8 mm to coronal plane through pupils), bucco-lingual angulation (1-4° to occlusal plane perpendicular on models, 87-94° to mandibular plane, 68° to Frankfurt plane, 22-25° to Nasion-B, 105° to occlusal plane, 64° to lower occlusal plane, 21° to A-Pogonion), mesio-distal angulation (2° to occlusal plane perpendicular on models). CONCLUSIONS: Although these findings can provide clinical guideline, they derive from heterogeneous studies in terms of subject characteristics and reference methods. Therefore, the optimal incisal position remains debatable.

Appropriateness of standard cephalometric norms for the assessment of dentofacial characteristics in patients with cleidocranial dysplasia.

OBJECTIVES: Cleidocranial dysplasia (CCD) is a rare skeletal syndrome affecting craniofacial and dental development. As a consequence, conventional cephalometric landmarks may not be valid for CCD patients, and the appropriateness of norms used for the general population should be critically discussed. METHODS: Five patients 9- to 22-year-old (three females, two males) with CCD were included. Lateral-cephalograms, orthopantomographies, and intra-oral photos were retrospectively analysed. Lateral-cephalograms of 50 normal controls (ten for each CCD patient) matched for age and sex were selected from an online database. Cephalometric measurements of each CCD patients were compared with average values of matched controls using Wilcoxon signed-rank test for paired values (α = 0.05). RESULTS: In CCD patients, a shortening of the cranial base was present (ΔSN = -17.1 mm, p = 0.043). Thus, the mandible (ΔSNPg = +9.5°, p = 0.043) and the maxilla (ΔSNA = +11.2°, p = 0.043) showed protrusion compared to the cranial base, despite a reduced maxillary (ΔCo-A = -15.1 mm, p = 0.043) and mandibular (ΔCo-Gn = -15.2 mm, p = 0.080) length. The mandibular divergence was reduced (ΔSN/GoGn = -6.4°, p = 0.043), a reduced overbite was present (ΔOverbite = -2.9 mm, p = 0.043), and the interincisal angle was increased (ΔInterincisalAngle = +13.7°, p = 0.043), mainly due to retro-inclination of lower incisors. CONCLUSIONS: Standard cephalometric norms for the assessment of horizontal jaw position may not be applicable to CCD patients because of a reduced anterior cranial base length compared to normal subjects. Vertical relationships may not be affected, and mandibular hypodivergency was confirmed.

Potential Role of Copper in Diabetes and Diabetic Kidney Disease.

Copper is a fundamental element for the homeostasis of the body. It is the third most abundant essential transition metal in humans. Changes in the concentration of copper in the blood are responsible for numerous diseases affecting various organs, including the heart, brain, kidneys, and liver. Even small copper deficiencies can lead to the development and progression of several pathologies. On the other hand, excessive exposure to copper can cause toxicity in many human organs, leading to various systemic alterations. In the kidney, increased copper concentration in the blood can cause deposition of this element in the kidneys, leading to nephrotoxicity. One of the most interesting aspects of copper balance is its influence on diabetes and the progression of its complications, such as Diabetic Kidney Disease (DKD). Several studies have shown a close relationship between copper serum levels and altered glycemic control. An imbalance of copper can lead to the progression of diabetes-related complications and impaired antioxidant homeostasis. A high Zinc/Copper (Zn/Cu) ratio is associated with improved renal function and reduced risk of poor glycemic control in patients with type two diabetes mellitus (T2DM). Furthermore, the progression of DKD appears to be related to the extent of urinary copper excretion, while regulation of adequate serum copper concentration appears to prevent and treat DKD. The aim of this review is to evaluate the possible role of copper in DKD patients.

Cardiorenal Syndrome: New Pathways and Novel Biomarkers.

Cardiorenal syndrome (CRS) is a multi-organ disease characterized by the complex interaction between heart and kidney during acute or chronic injury. The pathogenesis of CRS involves metabolic, hemodynamic, neurohormonal, and inflammatory mechanisms, and atherosclerotic degeneration. In the process of better understanding the bi-directional pathophysiological aspects of CRS, the need to find precise and easy-to-use markers has also evolved. Based on the new pathophysiological standpoints and an overall vision of the CRS, the literature on renal, cardiac, metabolic, oxidative, and vascular circulating biomarkers was evaluated. Though the effectiveness of different extensively applied biomarkers remains controversial, evidence for several indicators, particularly when combined, has increased in recent years. From new aspects of classic biomarkers to microRNAs, this review aimed at a 360-degree analysis of the pathways that balance the kidney and the heart physiologies. In this delicate system, different markers and their combination can shed light on the diagnosis, risk, and prognosis of CRS.

An ultrasound protocol for temporomandibular joint in juvenile idiopathic arthritis: a pilot study.

OBJECTIVES: As it is well known, the diagnosis of temporomandibular joint (TMJ) involvement in patients affected by Juvenile Idiopathic Arthritis (JIA) is important to avoid the impairment of mandibular growth. In this context, Magnetic Resonance Imaging (MRI) is the gold-standard for detection of TMJ involvement, however it is expensive and requires patients' collaboration. The aim of this study was to evaluate if ultrasound may be used as an alternative tool to investigate the acute signs of TMJ involvement in JIA patients. METHODS: Lateral periarticular space (LPAS) and joint effusion were evaluated by ultrasound in a study Group A of 8 JIA children (11.6±3.5 years old) with 14 TMJs involved, as confirmed by MRI, and in a control Group B of 7 healthy children (9.3±1.2 years old) without temporomandibular disorders (TMD). The LPAS width values were compared between the two groups using the Mann-Whitney test. The ultrasound images of the JIA group were then matched with the corresponding MR images; the Spearman Rank Correlation test and the Bland-Altman test were used to evaluate the differences. RESULTS: The LPAS values in Group A were statistically significantly higher than those in Group B (p < 0.001). There was no overlap of the LPAS values confidence intervals (CIs) between the two groups. No signs of joint effusion were identified in groups A and B. The Spearman test applied to the values of LPAS measured in ultrasound and the corresponding MR images showed a proportional positive correlation with a ρ of 0.623 and a p < 0.05. CONCLUSIONS: Ultrasound can detect differences in the TMJ features between JIA patients and healthy patients and it might be used as a follow-up tool in the assessment of TMJ involvement in subject affected by JIA.

Attitude towards Telemonitoring in Orthodontists and Orthodontic Patients.

The purpose of this study was to analyze the attitude of dentists and patients towards the use of Dental MonitoringTM (DM), an orthodontic telemonitoring software. Thus, two different specially prepared specific questionnaires were administered to 80 dentists (40 were general dentists and 40 orthodontists) and 80 orthodontic patients. All dentists judged positively telemonitoring, as 96.25% of them considered telemonitoring indicative of high tech and high-quality treatment; 100% considered it a way to reduce the number of in-office visits; 17.5% agreed on a weekly telemonitoring frequency, 40% on a biweekly, and 42.5% on a lower frequency. Further, 97.5% of patients judged positively telemonitoring; 81.25% of them considered telemonitoring indicative of high-tech treatment; 81.25% declared to be interested in reducing the number of in-office visits through telemonitoring; 27.5% agreed on taking self-picture every week, 57.5% every two weeks, and 15% on a lower frequency. Both patients and dentists positively judged telemonitoring, considering it a technologically advanced tool increasing the perception of quality and accuracy of the treatment. Both groups were interested in reducing the number of in-office visits, although not all of them revealed to be ready to invest more money and time in it.

Treatment of ankylosed deciduous molars with or without permanent successors in children and adolescents: a systematic review.

INTRODUCTION: The aim of this systematic review was to provide guidelines for decision-making during orthodontic treatment planning of infra-occluded deciduous molars with or without their successors in children and adolescents. EVIDENCE ACQUISITION: Computerized search was conducted on Medline via PubMed, and Cochrane Library. Articles published until 2020 in English language were analyzed following the Preferred Reporting Items for Systematic Reviews (PRISMA) Checklist. Observational and interventional longitudinal studies reporting the treatment of ankylosed deciduous molars with or without successor tooth in 3 to 15-year-old patients were included. EVIDENCE SYNTHESIS: In case of ankylosis with presence of successor, exfoliation took place in 77% of teeth, while extraction involved 23%. Infra-occlusion happened in 53% of teeth (worsening in 52%), alveolar bone loss in 37%, mesial tipping of first permanent molar in 5%, and over-eruption of antagonist in no cases (after exfoliation and eruption of successor). In case of ankylosis without successor, exfoliation took place in 1% of teeth, progression of infra-occlusion in 42%, progression of root resorption in 58%, development of mesial tipping of first permanent molars in 25%, while no case of antagonist over-eruption was reported. CONCLUSIONS: When the permanent tooth is present and the ankylosed tooth is slightly or moderately infra-occluded, observation is appropriate. In case of severe infra-occlusion or absence of successor, tooth extraction may be considered together with orthodontic space closure, transplantation, or prosthetic replacement. Alternatively, nonextraction and a prosthetic build-up may be considered.

Evaluation of a Digital Protocol for Pre-Surgical Orthopedic Treatment of Cleft Lip and Palate in Newborn Patients: A Pilot Study.

The aim of this study was to evaluate the accuracy, invasiveness and impact on clinical results of a digital oral impression protocol in the pre-surgical orthopedic treatment (PSOT) of newborn cleft lip and palate (CLP) patients undergoing primary alveolar surgical repair. Six patients were divided, according to impression technique used, into a digital (intraoral scanner (IOS)) and a non-digital (tray and putty (T&P)) group. Parents considered IOS impressions to be less invasive, compared to T&P impressions. The clinician that took all the impressions considered the IOS to be less stressful compared to the T&P method. In two T&P patients, the impression was repeated because some important anatomical details were missing, in one case due to patient regurgitation during the first attempt. No impression was repeated, and any adverse event was reported in the IOS group. There were no significant differences between these two protocols in pre-surgical alveolar gap reduction and surgical challenge. The study results indicate that this digital protocol can accelerate the production process of the passive molding plate with an instantaneous transmission of the digital impression to the dental lab, maintaining the same accuracy level and clinical outcomes of classical techniques and reducing the invasiveness of impression taking, avoiding any risk of impression material ingestion or inhalation.

Treatment of recurrent aphthous stomatitis (RAS; aphthae; canker sores) with a barrier forming mouth rinse or topical gel formulation containing hyaluronic acid: a retrospective clinical study.

BACKGROUND: Use of hyaluronic acid-based products has become a valuable alternative to drug-based approaches in the treatment of recurrent aphthous stomatitis (RAS). The presented study aimed to investigate the effect of a barrier forming hyaluronic acid containing mouth wash or a topical gel formulation on the healing of RAS and patient's quality of life. METHODS: For this single-center retrospective study, medical records of the Dental School of the University of Brescia were screened for adult and systemically health patients suffering from minor recurrent aphthous stomatitis (RAS) and treated with either a barrier forming, hyaluronic acid containing mouth wash (GUM® AftaClear® rinse) or a topical gel (GUM® AftaClear® gel) in 2015. All patients fulfilling the in-/exclusion criteria and presenting full data sets on lesion diameter, lesion color, as well as pain perception for baseline (day 0) and 4 and 7 days after treatment were enrolled into the presented study. RESULTS: Out of 60 screened patients, a total of 20 patients treated with the Rinse formulation and 25 treated with the Gel formulation were eligible for the enrollment into this study. Both groups showed equal distribution in patient's age, sex and presented a similar mean lesion size (3.0 ± 1.0 mm), lesion color distribution as well as pain perception at baseline. All patients showed significant normalization of lesion color, reduction of pain, and lesion dimension within the course of their treatment. After 7 days, the mean percentage of lesion reduction was highly significant for both groups attaining 77.4 ± 30.1% in the Rinse group and 81.2 ± 23.1% in the Gel group with a complete lesion closure obtained in 60 and 56% of the cases, respectively. However, a significant (p < 0.05) higher percentage of lesions in the Gel group (72%) compared to the Rinse group (40%) showed an improvement in lesion size already after 3 days. CONCLUSIONS: Within the limitation of retrospective design, it can be concluded that both the barrier forming hyaluronic acid containing mouth rinse as well as the topical gel formulation are effective in the treatment of minor recurrent aphthous stomatitis (RAS), with a trend for an earlier healing onset for the topical Gel formulation.

Erythropoiesis and chronic kidney disease-related anemia: From physiology to new therapeutic advancements.

Erythropoiesis is triggered by hypoxia and is strictly regulated by hormones, growth factors, cytokines, and vitamins to ensure an adequate oxygen delivery to all body cells. Abnormalities in one or more of these factors may induce different kinds of anemia requiring different treatments. A key player in red blood cell production is erythropoietin. It is a glycoprotein hormone, mainly produced by the kidneys, that promotes erythroid progenitor cell survival and differentiation in the bone marrow and regulates iron metabolism. A deficit in erythropoietin synthesis is the main cause of the normochromic normocytic anemia frequently observed in patients with progressive chronic kidney disease. The present review summarizes the most recent findings about each step of the erythropoietic process, going from the renal oxygen sensing system to the cascade of events induced by erythropoietin through its own receptor in the bone marrow. The paper also describes the new class of drugs designed to stabilize the hypoxia-inducible factor by inhibiting prolyl hydroxylase, with a discussion about their metabolism, disposition, efficacy, and safety. According to many trials, these drugs seem able to simulate tissue hypoxia and then stimulate erythropoiesis in patients affected by renal impairment. In conclusion, the in-depth investigation of all events involved in erythropoiesis is crucial to understand anemia pathophysiology and to identify new therapeutic strategies, in an attempt to overcome the potential side effects of the commonly used erythropoiesis-stimulating agents.

Convective Dialysis Reduces Mortality Risk: Results From a Large Observational, Population-Based Analysis.

According to many studies, extracorporeal dialysis with convective methods is associated with better clinical outcomes and a survival benefit compared to diffusive techniques. However, there is no full agreement on the actual superiority of this kind of renal replacement therapy on hard end-points such as mortality. We performed a retrospective epidemiological cohort study to provide "real-world" evidence on the impact of convective and non-convective dialysis techniques on all-cause and cardiac mortality and biochemical outcomes among dialysis patients in Sicily, the southernmost region of Italy. Data of all incident adult patients (N = 6529) who have started chronic extracorporeal dialysis over the period 2009-2015 were retrieved from the Sicilian Registry of Nephrology, Dialysis and Transplantation. There were 1558 patients receiving convective techniques (23.86%). Overall mortality rate was 45.21% with a significant difference between convective (31.39%) and non-convective (49.55%) groups (P < 0.0001). After adjustment for potential confounders in multiple Cox regression models of increasing complexity, the mortality risk remained significantly lower for patients treated with convective methods (HR, 0.581; 95%CI, 0.525 to 0.643; P < 0.0001). Moreover, the convective group had a better blood chemistry profile, improved dialysis efficacy, and reduced mortality rate from cardiac diseases compared to the non-convective group. As a sensitivity analysis, patients were categorized according to propensity score quartiles and the hazard ratio for both all-cause and cardiac mortality was significantly lower for the convective group in each quartile. In conclusion, despite the observational and retrospective design, the results of the present study further support the use of convective therapies for the treatment of end-stage renal disease.

The Myth of Water and Salt: From Aquaretics to Tenapanor.

The impact of water intake has been studied in several renal diseases. For example, increasing water intake is useful to prevent primary and secondary nephrolithiasis. In autosomal dominant polycystic kidney disease, arginine vasopressin (AVP) is involved in the progression of the disease, and water intake could play a therapeutic role by inhibiting the synthesis of AVP, but its efficacy is still controversial. Conversely, the use of aquaretics, which are antagonists of AVP V2 receptors, results in the reduction of the increase rate of total kidney volume with a slower decline of glomerular filtration rate. In chronic kidney disease, AVP contributes to glomerular hyperfiltration, arterial hypertension, and synthesis of renin, resulting in renal sclerosis. Increased water intake could reduce AVP activation determining a potential protective effect on the kidney, but its efficacy has not yet been clearly demonstrated. On the other side, sodium and potassium play an important role in the control of arterial blood pressure and are involved in the development and progression of chronic kidney disease. Reduction of sodium intake and increase of potassium intake determine a decrease of arterial blood pressure with a beneficial effect on the kidney; however, adherence to sodium restriction is very poor. Regarding this, sodium-hydrogen exchanger isoform 3 inhibitors may reduce sodium absorption in the gut. The most recent sodium-hydrogen exchanger isoform 3 inhibitor, known as tenapanor, reduces extracellular fluid volume, left ventricular hypertrophy, albuminuria, and blood pressure in experimental studies and increases fecal loss of sodium in humans.

[Complement factor B mutation in atypical hemolytic uremic syndrome. Rare cause of rare disease].

Hemolytic uremic syndrome (HUS) is a rare disease characterized by microangiopathic hemolysis, platelet consumption and multiple organ failure with predominant renal involvement. In the most of cases (85-90%), it is associated with enteric infection due to Shiga-toxin or verocytotoxin (STEC-VTEC)-producer Escherichia coli. Rarely, in about 10-15% of cases, HUS develops in the presence of a disorder of alternative complement pathway regulation and it is defined atypical (aHUS). We describe the case of a 65-year-old man who came to our attention with a clinical presentation of aHUS and a clinical course characterized by rapidly progressive acute renal failure (ARF), which required renal replacement treatments, and by a stable clinical picture of hematological impairment as a marker of a non-severe and self-limiting form. The clinical and laboratory course allowed us not to perform specific therapies such as plasma exchange and/or block of the complement with eculizumab. Less than two weeks after hospital admission, there was a gradual recovery of renal function with spontaneous diuresis and hematological remission. Genetic screening has revealed a heterozygous mutation in the complement factor B (CFB) that is not described in the literature and therefore not yet characterized in the genotype/phenotype correlation, also for the extreme rarity of the forms associated with CFB alteration. In conclusion, the presence of a new mutation in the CFB, such as the one described in our case, is probably associated with the development of aHUS but has not led to a poor prognosis, as generally reported in the literature for known variants of the CFB.