[Cuff Shaving in Recurrent Exit-Site Infections in a Patient on Peritoneal Dialysis].

In patients on peritoneal dialysis, the cutaneous emergency (exit-site) represents a potential access route to the peritoneum; consequently, it can become a site for microbial infections. These infections, initially localized to the exit-site, may spread to the peritoneum causing peritonitis, which is the most common cause of drop-out from peritoneal dialysis and transition to hemodialysis. Peritoneal catheters have dacron caps which have the function of counteracting the traction of the catheter itself and at the same time acting as a barrier for microorganisms, preventing the spread towards the peritoneum.

[Omentopexy in Peritoneal Catheter Malfunction].

Among the various problems associated with peritoneal dialysis, besides infectious causes, the risk of catheter malfunction plays a significant role in conditioning the continuation of the method, accounting for up to 15-18% of the total causes of dialysis drop-out. When non-invasive maneuvers, such as the use of laxatives to stimulate intestinal peristalsis or heparin and/or urokinase have no effect, videolaparoscopy is the only method that directly detects the precise causes of peritoneal catheter malfunction. Those found are, with decreasing frequency, the winding of the catheter between the intestinal loops and the omentum (wrapping), the dislocation of the catheter, the combination of wrapping and dislocation, the occlusion of the catheter by a fibrin plug, the adhesions between the intestine and abdominal wall, the occlusion of the catheter by epiploic appendages or adnexal tissue and, occasionally, the presence of a new formation of endoperitoneal tissue enveloping and obstructing the peritoneal catheter.

[A case of AL amyloidosis with fulminant evolution].

Amyloidosis represents a heterogeneous group of pathologies characterized by the deposit, in the form of fibrils, in the various organs and tissues of the body, of abnormal proteins; the deposits made up of these fibrils are called amyloid or amyloid substance. AL amyloidosis, also called "light chains", is a primary form characterized by deposits of light chains of monoclonal immunoglobulins, proteins that are produced by the bone marrow with the aim of protecting the body from pathological processes; for unknown reasons, these immunoglobulins, once fulfilled their function, do not dissolve but, on the contrary, they transform into amyloid fibrils and accumulate progressively, transported by the bloodstream, in the various organs and tissues. Below we report the case of a 77-year-old Caucasian male patient hospitalized at our Operative Unit for nephrotic syndrome and creatinine increase in the last couple of months, compared to previous normal tests.

Contraception in chronic kidney disease: a best practice position statement by the Kidney and Pregnancy Group of the Italian Society of Nephrology.

Even though fertility is reduced, conception and delivery are possible in all stages of CKD. While successful planned pregnancies are increasing, an unwanted pregnancy may have long-lasting deleterious effects, hence the importance of birth control, an issue often disregarded in clinical practice. The evidence summarized in this position statement is mainly derived from the overall population, or other patient categories, in the lack of guidelines specifically addressed to CKD.

Correction to: A best-practice position statement on pregnancy after kidney transplantation: focusing on the unsolved questions. The Kidney and Pregnancy Study Group of the Italian Society of Nephrology.

In the original publication of the article, the first name and last name of the authors were interchanged.

A best-practice position statement on pregnancy after kidney transplantation: focusing on the unsolved questions. The Kidney and Pregnancy Study Group of the Italian Society of Nephrology.

Kidney transplantation (KT) is often considered to be the method best able to restore fertility in a woman with chronic kidney disease (CKD). However, pregnancies in KT are not devoid of risks (in particular prematurity, small for gestational age babies, and the hypertensive disorders of pregnancy). An ideal profile of the potential KT mother includes "normal" or "good" kidney function (usually defined as glomerular filtration rate, GFR ≥ 60 ml/min), scant or no proteinuria (usually defined as below 500 mg/dl), normal or well controlled blood pressure (one drug only and no sign of end-organ damage), no recent acute rejection, good compliance and low-dose immunosuppression, without the use of potentially teratogen drugs (mycophenolic acid and m-Tor inhibitors) and an interval of at least 1-2 years after transplantation.

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.

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.

[A case of acute renal failure secondary to late-onset McArdle's disease].

Introduction: Mc Ardles disease, also known as Type V glycogen storage disease, is a rare deficiency of the enzyme glycogen phosphorylase in muscle cells, inherited as an autosomal recessive trait. In the absence of this enzyme, muscles cannot break down glycogen during exercise, so in patients affected by McArdles disease even moderate physical activity produces cramps, pain and fatigue. Anaerobic activity leads to severe fixed contractures and rhabdomyolisis with myoglobinuria and raised serum creatine-kinase, which, in turn, can lead to acute renal failure.

[Escherichia coli-induced emphysematous pyelonephritis in a diabetic patient with polycystic kidney disease].

Emphysematous pyelonephritis is a rare, necrotizing infection of the kidney and the perirenal space resulting in the formation of gas in both structures and associated with a high mortality rate. In 90% of cases it affects one kidney only; in the remaining 10% with bilateral emphysematous pyelonephritis aggressive surgical intervention may be required. Women are much more frequently affected than men, with diabetes mellitus (in 70-90% of cases) and urinary tract obstruction being common predisposing conditions.

Pregnancy and progression of IgA nephropathy: results of an Italian multicenter study.

Background: Whether pregnancy impacts on the long-term outcome of immunoglobulin A (IgA) nephropathy is unknown. This study aims to compare the long-term outcome of kidney disease in women with IgA nephropathy and preserved kidney function who did and did not become pregnant.

Study Design: Multicenter longitudinal cohort study.

Pregnancy in CKD stages 3 to 5: fetal and maternal outcomes.

Background: Prognostic criteria to inform women with moderate to severe renal insufficiency who wish to bear children are not well established.

Study Design: Longitudinal multicenter cohort study.

Settings & Participants: Nondiabetic white women with pregnancies proceeded beyond the 20th week and estimated glomerular filtration rate (GFR) less than 60 mL/min/1.

Effect of protein leaking BK-F PMMA-based hemodialysis on plasma pentosidine levels.

Background: Advanced glycation end-products (AGEs) are now considered to contribute to the middle molecule toxicity of uremia and, because they are not cleared by conventional low-flux hemodialysis, alternative strategies are needed to improve their removal.

Methods: In a prospective cross-over trial involving 18 adult chronic hemodialysis subjects, we evaluated the intradialytic removal and the long-term effect on predialysis levels of Protein-bound (PBPe) and Free (FPe) pentosidine by high-pore, protein-leaking BK-F Polymethylmethacrylate-based hemodialysis (BK-F-HD), by comparing it to hemodialysis using low-flux dialyzers (LF-HD).

Results: A single BK-F-HD session removed more PBPe, but not FPe, than LF-HD.

Central role of vasopressin in sodium/water retention in hypo- and hypervolemic nephrotic patients: a unifying hypothesis.

The underfill and overflow hypotheses are usually held as mutually exclusive mechanisms for explaining sodium/water retention in nephrotic syndrome, but neither of them is entirely convincing. In this paper, we will briefly summarize the experimental and clinical evidence in favor of and against each hypothesis. Based on our personal observations, we propose a unifying hypothesis in which underfill and overflow are subsequent stages of the disease.

Diagnostic accuracy of ultrasound dilution access blood flow measurement in detecting stenosis and predicting thrombosis in native forearm arteriovenous fistulae for hemodialysis.

Background: Vascular access surveillance by ultrasound dilution blood flow rate (Qa) measurement is widely recommended; however, optimal criteria for detecting stenosis and predicting thrombosis in arteriovenous fistulae (AVFs) are still not clearly defined.

Methods: In a blinded trial, we evaluated the accuracy of single Qa measurement, Qa adjusted for mean arterial pressure (Qa/MAP), and decrease in Qa over time (dQa) in detecting stenosis and predicting thrombosis in an unselected population of 120 hemodialysis subjects with native forearm AVFs (91 AVFs, located at the wrist; 29 AVFs, located at the midforearm). All AVFs underwent fistulography, which identified greater than 50% stenosis in 54 cases.

A prospective controlled trial on effect of percutaneous transluminal angioplasty on functioning arteriovenous fistulae survival.

Balloon angioplasty (PTA) is an established treatment modality for stenosis in dysfunctional arteriovenous fistulae (AVF), although most studies showing efficacy have been retrospective, uncontrolled, and nonrandomized. In addition, it is unknown whether correction of stenosis not associated with significant hemodynamic, functional, and clinical abnormality may improve survival in AVF. This study was a prospective controlled open trial to evaluate whether prophylactic PTA of stenosis not associated with access dysfunction improves survival in native, virgin, radiocephalic forearm AVF.

Potassium-based dilutional method to measure hemodialysis access recirculation.

Background: Assessment of access recirculation (AR) is crucial to dialysis efficiency and there is thus a need for a method yielding a highly accurate, fast, easy and economical measurement that can be applied in any busy dialysis clinic. Non-urea based dilutional methods are more accurate than urea based methods and avoid problems with cardiopulmonary recirculation, but they require expensive specialized devices, which limit their applicability.

Methods: We developed a simple dilutional method of AR which does not require any specific device, based on the determination of serum potassium [K+] in two samples.

Severe preeclampsia in antithrombin III deficiency with no history of venous thromboembolism.

Complications of pregnancy, such as preeclampsia, placental abruption, fetal growth retardation, still-birth and fetal death are associated with an increased frequency of pro-thrombotic abnormalities. We describe a case of severe preeclampsia and multiple placental infarctions in a 28-year-old woman at 31 weeks' gestation. Despite a negative personal history for venous thromboembolism, coagulation screening for thrombophilia detected an isolated antithrombin III deficiency.

The role of iron status markers in predicting response to intravenous iron in haemodialysis patients on maintenance erythropoietin.

Background: Iron deficiency (ID) is the main cause of hyporesponsiveness to erythropoietin in haemodialysis patients and its detection is of value since it is easily corrected by intravenous iron. Markers of iron supply to the erythron, including erythrocyte zinc protoporphyrin (Er-ZPP), percentage of hypochromic erythrocytes (Hypo), reticulocyte haemoglobin content (CHr) and soluble transferrin receptor (sTfR), may be more accurate predictors of ID than ferritin (Fer) and transferrin saturation (TSat), but relative diagnostic power and best threshold values are not yet established.

Methods: In 125 haemodialysis patients on maintenance erythropoietin, the diagnostic power of the above parameters was evaluated by ROC curve, multivariate regression, and stepwise discriminant analyses.

Identification of the gene for oral-facial-digital type I syndrome.

Oral-facial-digital type 1 syndrome (OFD1 [MIM 311200]) is transmitted as an X-linked dominant condition with lethality in males and is characterized by malformations of the face, oral cavity, and digits, and by a highly variable expressivity even within the same family. Malformation of the brain and polycystic kidneys are commonly associated with this disorder. The locus for OFD1 was mapped by linkage analysis to a 12-Mb interval, flanked by markers DXS85 and DXS7105 in the Xp22 region.