[Survey on the Prevalence of Rare Kidney Diseases in Sicily].

Renal Diseases represent almost 6% of all Rare Diseases but are often misdiagnosed. In a survey made in Sicily in 2016, based on cases reported from all public hospitals according to a list of rare kidney diseases, we were able to collect 337 cases (199 males and 138 females). The highest prevalence was detected in children: 13.9 cases in 100.000 children; the mean age was 10, and the median 5 years, at the time of the diagnosis. Comparing our data with those available in the Sicilian Register of Rare Diseases we found that only 141 cases (54%) were present in the register. Promoting regional registries of rare kidney diseases in Italy may be useful for epidemiologic studies.

[Methodology for determining workforce in health nephrologic unit in ITALY. Update of lows].

Nephrology continues to be in transition. While rates of kidney diseases and injury continue to rise, changes in the general health care system and the delivery of kidney care make it unclear how increases in need will be translated into demand for nephrologists. The changes in the delivery system also raise questions as to the future roles and career paths for nephrologists. There a major interrelated workforce issues to be watched closely : how many nephrologists are needed ? The supply of nephrologists does not reflect the distribution of patients with kidney diseases or the activity and job description related to end stage renal disease (ESRD) patients. Looking forward, more needs to be done to systematically measure need and access, and to identify clinical areas and activity of high need for nephrologists. This review examines the laws that govern the measure of work and the needs of personnel of the Italian state and in particular in health care. Therefore, once the method is accepted and established, it will be possible communicate those findings to policy makers and fellows and to involve the politicians.

[Nephrologic workforce. Who we are? Where do we go? A project by SIN]. / La Workforce nefrologica in Italia. Chi siamo e dove andiamo: un progetto SIN.

The SIN Workforce Working Group has carried out an initial demographic study project that highlights the situation of nephrological workforce in European and non-European countries, noting in particular the apparent discrepancies between the number of nephrologists in other health systems and in the Italian one. Italy seems to have the highest number of nephrologists per capita: in fact, the number of nephrologists has decreased in recent years due to the number of retirements far higher than the entry of new specialists. The project arises from the need to define the actual number of nephrologists in Italy in relation to the population and the epidemiology of chronic renal disease, taking into account the trends of ageing and feminization of our specialty. The tools used to collect data on the demographics of Italian nephrologists are a database for demographic data collection by presidents of SIN regional sections and a questionnaire for a survey to describe demography, workload, the adhesion to the discipline and the recruitment and retirement programs of the Italian nephrological community.

A best practice position statement on the role of the nephrologist in the prevention and follow-up of preeclampsia: the Italian study group on kidney and pregnancy.

Preeclampsia (PE) is a protean syndrome causing a transitory kidney disease, characterised by hypertension and proteinuria, ultimately reversible after delivery. Its prevalence is variously estimated, from 3 to 5% to 10% if all the related disorders, including also pregnancy-induced hypertension (PIH) and HELLP syndrome (haemolysis, increase in liver enzyme, low platelets) are included. Both nephrologists and obstetricians are involved in the management of the disease, according to different protocols, and the clinical management, as well as the role for each specialty, differs worldwide. The increased awareness of the role of chronic kidney disease in pregnancy, complicating up to 3% of pregnancies, and the knowledge that PE is associated with an increased risk for development of CKD later in life have recently increased the interest and redesigned the role of the nephrologists in this context. However, while the heterogeneous definitions of PE, its recent reclassification, an emerging role for biochemical biomarkers, the growing body of epidemiological data and the new potential therapeutic interventions lead to counsel long-term follow-up, the lack of resources for chronic patients and the increasing costs of care limit the potential for preventive actions, and suggest tailoring specific interventional strategies. The aim of the present position statement of the Kidney and Pregnancy Study Group of the Italian Society of Nephrology is to review the literature and to try to identify theoretical and pragmatic bases for an agreed management of PE in the nephrological setting, with particular attention to the prevention of the syndrome (recurrent PE, presence of baseline CKD) and to the organization of the postpartum follow-up.

Outcomes of Pregnancies After Kidney Transplantation: Lessons Learned From CKD. A Comparison of Transplanted, Nontransplanted Chronic Kidney Disease Patients and Low-Risk Pregnancies: A Multicenter Nationwide Analysis.

BACKGROUND: Kidney transplantation (KT) may restore fertility in chronic kidney disease (CKD). The reasons why maternofetal outcomes are still inferior to the overall population are only partially known. Comparison with the CKD population may offer some useful insights for management and counselling.Aim of this study was to analyse the outcomes of pregnancy after KT, compared with a large population of nontransplanted CKD patients and with low-risk control pregnancies, observed in Italy the new millennium. METHODS: We selected 121 live-born singletons after KT (Italian study group of kidney in pregnancy, national coverage about 75%), 610 live-born singletons in CKD, and 1418 low-risk controls recruited in 2 large Italian Units in the same period (2000-2014). The following outcomes were considered: maternal and fetal death; malformations; preterm delivery; small for gestational age (SGA) baby; need for the neonatal intensive care unit; doubling of serum creatinine or increase in CKD stage. Data were analyzed according to kidney diseases, renal function (staging according to CKD-epidemiology collaboration), hypertension, maternal age, parity, ethnicity. RESULTS: Maternofetal outcomes are less favourable in CKD and KT as compared with the low-risk population. CKD stage and hypertension are important determinants of results. Kidney transplantation patients with estimated glomerular filtration rate greater than 90 have worse outcomes compared with CKD stage 1 patients; the differences level off when only CKD patients affected by glomerulonephritis or systemic diseases ("progressive CKD") are compared with KT. In the multivariate analysis, risk for preterm and early-preterm delivery was linked to CKD stage (2-5 vs 1: relative risk 3.42 and 3.78) and hypertension (RR 3.68 and 3.16) while no difference was associated with being a KT or a CKD patient. CONCLUSIONS: The maternofetal outcomes in patients with kidney transplantation are comparable with those of nontransplanted CKD patients with similar levels of kidney function impairment and progressive and/or immunologic kidney disease.

[Census of the renal and dialysis units by Italian Society of Nephrology: nephrologist's workload for renal patient assistance in Italy (2014-2015)]. / Censimento a cura della Società Italiana di Nefrologia delle strutture nefrologiche e della loro attività in Italia nel 2014-2015: il lavoro del nefrologo.

BACKGROUND: Given the public health challenge and burden of chronic kidney disease, the Italian Society of Nephrology (SIN) promoted acensusof the renal and dialysis units to analyse structural and human resources, organizational aspects, activities and workload referring to theyear 2014. METHODS: An online questionnaire, including 64 items exploring structural and human resources, organization aspects, activities and epidemiological data referred to 2014, was sent to chiefs of any renal or dialysis unit. RESULTS: Renal and dialysis activity was performed by over 2718 physicians (45 pmp). The management of the acute renal failure was one of the most frequent activities in the public renal units (12,206 patients in ICU and 140.00 dialysis sessions). There were performed about 9000 AV fistulas and 1700 central vascular catheters insertions. In the census, there are a lot of data regarding organization, workforce and workload of the renal unit in Italy. The benchmark data derived from this census show interesting comparisons between centres, regions and groups of regions. These data realised the clinical management of renal disease in Italy.

[Census of the renal and dialysis units by Italian Society of Nephrology: structure and organization for renal patient assistance in Italy (2014-2015)]. / Censimento a cura della Società Italiana di Nefrologia delle strutture nefrologiche e delle loro attività in Italia nel 2014-2015: l'organizzazione.

BACKGROUND: Given the public health challenge and burden of chronic kidney disease, the Italian Society of Nephrology (SIN) promoted a census of the renal and dialysis units to analyse structural and human resources, organizational aspects, activities and workload referring to the year 2014. METHODS: An online questionnaire, including 64 items exploring structural and human resources, organization aspects, activities and epidemiological data referred to 2014, was sent to chiefs of any renal or dialysis unit. RESULTS: 615 renal units were identified. From these 615 units, 332 were public renal centres (of which 318 centres answered to the census) and 283 were private dialysis centres (of which 113 centres answered to the census). The results show 6 public renal units pmp. Renal biopsies were 4624 (81 pmp). The nephrology beds are about 41 pmp. There are 7.304 nurses working in HD wards, 1.692 in the nephrology wards and only 613 for outpatients clinics. The benchmark data derived from this census show interesting comparisons between centres, regions and groups of regions. These data realised the clinical management of renal disease in Italy.

Pregnancy outcomes after kidney graft in Italy: are the changes over time the result of different therapies or of different policies? A nationwide survey (1978-2013).

BACKGROUND: Kidney transplantation is the treatment of choice to restore fertility to women on renal replacement therapy. Over time, immunosuppressive, support therapies and approaches towards high-risk pregnancies have changed. The aim of this study was to analyse maternal-foetal outcomes in two cohorts of transplanted women who delivered a live-born baby in Italy in 1978-2013, dichotomized into delivery before and after January 2000. METHODS: A survey involving all the Italian transplant centres was carried out, gathering data on all pregnancies recorded since the start of activity at each centre; the estimated nationwide coverage was 75%. Data on cause of ESRD, dialysis, living/cadaveric transplantation, drug therapy, comorbidity, and the main maternal-foetal outcomes were recorded and reviewed. Data were compared with a low-risk cohort of pregnancies from two large Italian centres (2000-14; Torino and Cagliari Observational Study cohort). RESULTS: The database consists of 222 pregnancies with live-born babies after transplantation (83 before 2000 and 139 in 2000-13; 68 and 121 with baseline and birth data, respectively), and 1418 low-risk controls. The age of the patients significantly increased over time (1978-99: age 30.7 ± 3.7 versus 34.1 ± 3.7 in 2000-13; P < 0.001). Azathioprine, steroids and cyclosporine A were the main drugs employed in the first time period, while tacrolimus emerged in the second. The prevalence of early preterm babies increased from 13.4% in the first to 27.1% in the second period (P = 0.049), while late-preterm babies non-significantly decreased (38.8 versus 33.1%), thus leaving the prevalence of all preterm babies almost unchanged (52.2 and 60.2%; P = 0.372). Babies below the 5th percentile decreased over time (22.2 versus 9.6%; P = 0.036). In spite of high prematurity rates, no neonatal deaths occurred after 2000. The results in kidney transplant patients are significantly different from controls both considering all cases [preterm delivery: 57.3 versus 6.3%; early preterm: 22.2 versus 0.9%; small for gestational age (SGA): 14 versus 4.5%; P < 0.001] and considering only transplant patients with normal kidney function [preterm delivery: 35 versus 6.3%; early preterm: 10 versus 0.9%; SGA: 23.7 versus 4.5% (P < 0.001); risks increase across CKD stages]. Kidney function remained stable in most of the patients up to 6 months after delivery. Multiple regression analysis performed on the transplant cohort highlights a higher risk of preterm delivery in later CKD stages, an increase in preterm delivery and a decrease in SGA across periods. CONCLUSIONS: Pregnancy after transplantation has a higher risk of adverse outcomes compared with the general population. Over time, the incidence of SGA babies decreased while the incidence of 'early preterm' babies increased. Although acknowledging the differences in therapy (cyclosporine versus tacrolimus) and in maternal age (significantly increased), the decrease in SGA and the increase in prematurity may be explained by an obstetric policy favouring earlier delivery against the risk of foetal growth restriction.

A best practice position statement on pregnancy in chronic kidney disease: the Italian Study Group on Kidney and Pregnancy.

Pregnancy is increasingly undertaken in patients with chronic kidney disease (CKD) and, conversely, CKD is increasingly diagnosed in pregnancy: up to 3 % of pregnancies are estimated to be complicated by CKD. The heterogeneity of CKD (accounting for stage, hypertension and proteinuria) and the rarity of several kidney diseases make risk assessment difficult and therapeutic strategies are often based upon scattered experiences and small series. In this setting, the aim of this position statement of the Kidney and Pregnancy Study Group of the Italian Society of Nephrology is to review the literature, and discuss the experience in the clinical management of CKD in pregnancy. CKD is associated with an increased risk for adverse pregnancy-related outcomes since its early stage, also in the absence of hypertension and proteinuria, thus supporting the need for a multidisciplinary follow-up in all CKD patients. CKD stage, hypertension and proteinuria are interrelated, but they are also independent risk factors for adverse pregnancy-related outcomes. Among the different kidney diseases, patients with glomerulonephritis and immunologic diseases are at higher risk of developing or increasing proteinuria and hypertension, a picture often difficult to differentiate from preeclampsia. The risk is higher in active immunologic diseases, and in those cases that are detected or flare up during pregnancy. Referral to tertiary care centres for multidisciplinary follow-up and tailored approaches are warranted. The risk of maternal death is, almost exclusively, reported in systemic lupus erythematosus and vasculitis, which share with diabetic nephropathy an increased risk for perinatal death of the babies. Conversely, patients with kidney malformation, autosomal-dominant polycystic kidney disease, stone disease, and previous upper urinary tract infections are at higher risk for urinary tract infections, in turn associated with prematurity. No risk for malformations other than those related to familiar urinary tract malformations is reported in CKD patients, with the possible exception of diabetic nephropathy. Risks of worsening of the renal function are differently reported, but are higher in advanced CKD. Strict follow-up is needed, also to identify the best balance between maternal and foetal risks. The need for further multicentre studies is underlined.

Best practices on pregnancy on dialysis: the Italian Study Group on Kidney and Pregnancy.

BACKGROUND: Pregnancy during dialysis is increasingly being reported and represents a debated point in Nephrology. The small number of cases available in the literature makes evidence-based counselling difficult, also given the cultural sensitivity of this issue. Hence, the need for position statements to highlight the state of the art and propose the unresolved issues for general discussion. METHODS: A systematic analysis of the literature (MESH, Emtree and free terms on pregnancy and dialysis) was conducted and expert opinions examined (Study Group on Kidney and Pregnancy; experts involved in the management of pregnancy in dialysis in Italy 2000-2013). Questions regarded: timing of dialysis start in pregnancy; mode of treatment, i.e. peritoneal dialysis (PD) versus haemodialysis (HD); treatment schedules (for both modes); obstetric surveillance; main support therapies (anaemia, calcium-phosphate parathormone; acidosis); counselling tips. MAIN RESULTS: Timing of dialysis start is not clear, considering also the different support therapies; successful pregnancy is possible in both PD and HD; high efficiency and strict integration with residual kidney function are pivotal in both treatments, the blood urea nitrogen test being perhaps a useful marker in this context. To date, long-hour HD has provided the best results. Strict, personalized obstetric surveillance is warranted; therapies should be aimed at avoiding vitamin B12, folate and iron deficits, and at correcting anaemia; vitamin D and calcium administration is safe and recommended. Women on dialysis should be advised that pregnancy is possible, albeit rare, with both types of dialysis treatment, and that a success rate of over 75% may be achieved. High dialysis efficiency and frequent controls are needed to optimize outcomes.

Women and the sciences.

The education level of women has increased significantly in recent decades. However, although there is a continued positive trend overall, women remain underrepresented (or misrepresented?) in the main fields of science. In Europe the proportion of women in scientific research is growing faster than that of men, but women are more likely than men to choose education, arts and humanities, health and welfare. Moreover, of the total number of women graduating in all faculties (55%), the percentage of women graduating in medicine is 65%-68%, in Europe as in the United States. As far as nephrology is concerned, unpublished data from the Italian Society of Nephrology indicate that female nephrologists make up almost 30% of the total number in the age group between 40 and 55, and this proportion is even higher in the age group younger than 40 years. In comparison with the past, there are some hints that things are going to change, but the path is still a difficult one, much effort is needed and there is a long way ahead.