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Connexins are membrane-spanning proteins that allow for the formation of cell-to-cell channels and cell-to–extracellular space hemichannels. Many connexin subtypes are expressed in kidney cells. Some mutations in connexin genes have been linked to various human pathologies, including cardiovascular, neurodegenerative, lung, and skin diseases, but the exact role of connexins in kidney disease remains unclear. Some hypotheses about a connection between genetic mutations, endoplasmic reticulum (ER) stress, and the unfolded protein response (UPR) in kidney pathology have been explored. The potential relationship of kidney disease to abnormal production of connexin proteins, mutations in their genes together with ER stress, or the UPR is still a matter of debate. In this scenario, it is tantalizing to speculate about a possible role of connexins in the setting of kidney pathologies that are thought to be caused by a deregulated podocyte protein expression, the so-called podocytopathies. In this article, we give examples of the roles of connexins in kidney (patho)physiology and propose avenues for further research concerning connexins, ER stress, and UPR in podocytopathies that may ultimately help refine drug treatment.

For a long time now, kidney transplant rejection has been considered the consequence of either cellular or antibody-mediated reaction as a part of adaptive immunity response. The role of innate immunity, on the other hand, had been unclear for many years and was thought to be only ancillary. There is now consistent evidence that innate immune response is a condition necessary to activate the machinery of rejection. In this setting, the communication between antigen-presenting cells and T lymphocytes is of major importance. Indeed, T cells are unable to cause rejection if innate immunity is not activated. This field is currently being explored and several experiments in animal models have proved that blocking innate immunity activation can promote tolerance of the graft instead of rejection. The aim of this review is to systematically describe all the steps of innate immunity response in kidney transplant rejection, from antigen recognition to T-cells activation, with a focus on clinical consequences and possible future perspectives.

Objectives:Potential celiac disease (CD) patients are at an increased risk to developing CD as indicated by positive CD-associated serology. We investigated in duodenal mucosa of such patients the presence of both IL-21 and IL-17A and the role of gliadin peptides and IL-15 in their expression.Methods:Duodenal biopsies from 76 active CD, 90 potential CD, and 58 control patients were analyzed for IL-21 and/or IL-17A production by quantitative real-time PCR, immunohistochemistry, flow cytometry, and ELISA. The presence of IL-21 receptor was investigated by western blot. Potential CD duodenal fragments were cultured with gliadin peptides (PTG) and/or IL-15 and the expression/production of IL-21 and IL-17A assessed by quantitative real-time PCR and by immunohistochemistry.Results:In potential CD, IL-21 was lower than in active CD, in terms of RNA expression (P<0.01), density of lamina propria (LP) IL-21+cells (P<0.05), and protein secretion (P<0.05). Also, IL-21R was weakly detectable in potential CD. Several LP cell types produced IL-21 in CD. In potential CD, CD4+IL-21+cells increased after PMA-ionomycin stimulation and co-produced IFN-γ but not IL-17A. After 24 hours of culture stimulation with PTG, IL-21-producing cells increased but not the ones producing IL-17A. This increase was further enhanced by the addition of IL-15 to culture medium.Conclusions:In potential CD, IL-21 is less expressed than in active CD; however, IL-21-producing cells are present and prone to respond after specific stimuli. This suggests a key role of IL-21 in the progression of mucosal damage in CD.

In recent years, in the Dialysis departments, the need of a team for the construction and the surveillance of vascular access begins to appear. In this article we describe our experience.

With advancing age, the functional reserve of many organs tends to decrease. In particular, the lean body mass, the levels of serum albumin, the blood flow to the liver, and the glomerular filtration rate are reduced in elderly individuals and can be further impaired by the concomitant presence of acute or chronic kidney disease. Moreover, patients with kidney disease are often affected by comorbid processes and are prescribed multiple medications. The aging process also modifies some drug interactions, including the affinity of some drugs for their receptor, the number of receptors, and the cell responses upon receptor activation. Therefore, older patients with kidney disease are particularly susceptible to the risks of adverse drug reactions. Planning a pharmacological regimen in such patients is confounded by the paucity of information available on the pharmacokinetic and pharmacodynamic profiles of a large number of drugs commonly used in this group of patients. Finally, many aged patients suffer from unintentional poor compliance. In this review, the problems physicians face in designing safe and effective medication management in elderly individuals are discussed, paying attention to those more frequently used, which may be potentially harmful in patients with kidney disease. The risks of overdosing and underdosing are outlined, and some recommendations to reduce the risk of adverse drug reactions are provided. A review of the literature covering the field of drug management in older patients with kidney disease was performed by selecting those articles published between January 1, 1990, and December 1, 2014, using PubMed as a search engine with the keywords elderly, kidney disease, drugs, drug interaction, and renal function.

Kidney transplant is the replacement treatment of choice in ADPKD patients with end-stage renal failure. However, the possible coexistence of potential risk factors for serious complications requires careful clinical evaluation and particular preparation of the ADPKD patient who is a candidate for transplantation. This article will take into consideration the main problems an ADPKD patient may present, their possible treatment, and the need for other routine evaluations (clinical, laboratory and instrumental) to exclude the presence of relative or absolute contraindications in kidney transplant candidates.

Introduction:Basiliximabis a chimeric monoclonal antibody directed against the alpha chain of interleukin-2 receptor (IL-2R). When administered intravenously at a dosage of 20 mg at the time of transplantation and 4 days later, basiliximab saturates the alpha chain of IL-2R for 4 weeks.Areas covered:This review evaluates the efficacy and safety of basiliximab in kidney transplantation. Randomized controlled trials showed that basiliximab can significantly reduce the incidence of acute rejection without increasing the risk of adverse events. When compared with other antibodies used for induction, basiliximab showed efficacy and safety profiles similar to daclizumab, another monoclonal antibody directed against the alpha chain of IL-2R. In comparison with rabbit anti-thymocyte globulins (rATG), basiliximab showed a similar efficacy. However, in patients at higher risk of rejection, rATG proved to be more effective. No serious safety problems related to basiliximab have been reported.Expert opinion:There is a solid evidence that basiliximab can significantly decrease the risk of acute rejection in kidney transplant recipients without increasing adverse events. This can allow decreased dosage or avoidance of glucocorticoids and reduced dosage of calcineurin inhibitors. On the basis of efficacy, tolerability, ease of administration, and cost effectiveness, basiliximab may be considered the drug of choice for the prophylaxis of acute rejection in standard renal transplant recipients.

In the last years, the placement of permanent central venous catheters for hemodialysis, infusion, and electrophysiological therapy has widely increased in clinical practice. As a result, the number of central venous obstructions has increased due to thrombosis or reactive stenosis correlated with these devices.This article describes the clinical case of an extensive thrombosis involving the superior vena cava and the anonymous trunks in a patient reliant on a permanent central venous catheter for hemodialysis treatment.

The growing interest in biofuels is a consequence of the need to reduce operating costs of energy systems and the need to reduce emissions of greenhouse gases. The same applies to PV modules, comprising a solution environmentally desirable for power generation. This paper presents a study on the inclusion of biodiesel and PV modules in a system already in operation on a small farm in southern Brazil. The insertion of biodiesel in different blends with diesel reveals that the use of B100 results in a reduction of about 3% in emissions and a reduction of less than 10% in the final cost of energy. A direct comparison between pure diesel, biodiesel produced from waste agricultural industries poultry slaughtering and various blends indicates the use of B40 as the best choice. The inclusion of PV modules requires a cost reduction for at least 30% of the current value.

The use of temporary or tunneled central venous catheters (CVC) is increasing worldwide in hemodialysis patients, but their numerous and potentially severe complications should be taken into account. Among the possible complications, infections are certainly those with the greater impact on morbidity and mortality. Catheter-related infections can affect the CVC exit site, the subcutaneous tunnel or, in the most severe cases, can induce bacteremia and sepsis (CRBSI, catheter-related blood stream infections). CRBSI prevention is of the utmost importance and can be achieved through educational activities for the dialysis personnel and through the use of specific protocols for CVC insertion and management. Indeed, it has been demonstrated that these measures allow a significant reduction of the number of CRBSI, to less than 1 episode per 1,000 catheter days.

A 55-year-old man was admitted to the hospital in November 2010 because of an anemia resistant to erythropoietin treatment and scarcely responsive to blood transfusions. The patient had received a kidney transplant 45 days earlier and he was receiving an immunosuppression treatment with steroids, everolimus and tacrolimus. A secondary anemia was ruled out during his stay in the hospital. The clinical hypothesis was a hyporigenerative anemia due to erythropoietin antibodies. The erythropoietin treatment was stopped, the serum erythropoietin was dosed and its value was higher than normally. A bone marrow needle aspiration showed a pure acquired aplasia of the red cells. The parvovirus B19 was detected both in the serum and in the bone marrow. The patient was treated with corticosteroids (prednisone) and i.v. immunoglobulins. A gradual and slow recovery of the aplasia followed.

AbstractChyluria denotes the urinary excretion of chyle, which is a lymphatic fluid rich in chylomicrons. Chyle flows from the intestinal lacteals to the left subclavian vein through the thoracic duct. When an abnormal connection between these structures and the urinary tract develops, chyluria appears. The syndrome is often associated with a nephrotic-range proteinuria, and this could be a wrong indication to perform renal biopsy. Chyluria is classified as parasitic or nonparasitic, the former being induced by lymphatic filariasis, whereas the latter is caused by medical, traumatic or inherited diseases. The patient usually reports excretion of milky urines, monolateral flank pain, malnutrition, weight loss and weakness. Urinalysis demonstrates lymphocyturia associated with chylomicrons and triglycerides in the supernatant. The diagnostic approach is aimed to define the site of lymphourinary fistula. A selective ureteral catheterization allows to collect urine samples from each kidney, demonstrating a monolateral source of proteins and lipids and making renal biopsy superfluous. Other diagnostic tools include nuclear magnetic resonance urography and lymphoangiography. Many therapeutic options have been proposed. Sclerosing solution instillation into the renal pelvis and laparoscopic renal pedicle disconnection are the invasive procedures most commonly employed. Among the medical alternatives, a low-fat diet supplemented with medium-chain triglycerides is often followed by complete clinical and biochemical remission.Copyright © 2011 S. Karger AG, Basel

AbstractThe Working Group of the European Renal Association-European Nephrology and Dialysis Association (ERA-EDTA) dedicated to immune system involvement in renal disease (Immunonephrology Working Group) was established in 2009 to facilitate exchanges of ideas on basic science research and new treatment protocols among European nephrologists. A section of the ERA-EDTA website describes activities and is open for new applications and proposals. In 2010, the first meeting of this Working Group focused on aspects of immune-mediated renal damage shared by kidney during glomerulonephritides or after kidney transplantation. From the large series of data reported, a previously disregarded presence of pathogenetic and therapeutic aspects common to glomerular diseases and transplanted kidneys was highlighted. Although the antigens involved in transplant rejection and in glomerulonephritis are different, a number of factors involved in the effector response are similar, mainly those based on the interaction between innate and adaptive immune mechanisms and on the strict cooperation between T and B cells. Moreover, the common target for the allo- and autoimmune attacks is represented by the endothelial cells, of which the kidney is particularly rich. These similarities may explain why immunomodulating treatments used in alloimmunity may also be useful in autoimmune diseases and vice versa. Considering renal damage from a holistic point of view may favour exchange of news for two formerly considered non-communicant areas, i.e. glomerular disease and renal transplantation nephrology sections.Copyright © 2011 S. Karger AG, Basel

Some new, interesting, and unknown heterocyclic rings and openchain molecules have been obtained with ease and under mild conditions by reaction of a wide range of aminocarbonyl compounds with acetylenic esters. The reactivity is highly dependent on the substituents and their positions in the starting material, which can be either cyclic pyrazolones or openchain compounds.

DNA synthesis is catalyzed by an ensemble of proteins designated the replicase. The efficient assembly of this multiprotein complex is essential for the continuity of DNA replication and is mediated by clamp-loading accessory proteins that use ATP binding and hydrolysis to coordinate these events. As a consequence, the ability to selectively inhibit the activity of these accessory proteins provides a rational approach to regulate DNA synthesis. Toward this goal, we tested the ability of several non-natural nucleotides to inhibit ATP-dependent enzymes associated with DNA replicase assembly. Kinetic and biophysical studies identified 5-nitro-indolyl-2′-deoxyribose-5′-triphosphate as a unique non-natural nucleotide capable of selectively inhibiting the bacteriophage T4 clamp loader versusthe homologous enzyme from Escherichia coli. Modeling studies highlight the structural diversity between the ATP-binding site of each enzyme and provide a mechanism accounting for the differences in potencies for various substituted indolyl-2′-deoxyribose-5′-triphosphates. An in vivoassay measuring plaque formation demonstrates the efficacy and selectivity of 5-nitro-indolyl-2′-deoxyribose as a cytostatic agent against T4 bacteriophage while leaving viability of the E. colihost unaffected. This strategy provides a novel approach to develop agents that selectively inhibit ATP-dependent enzymes that are required for efficient DNA replication.

AbstractLittle attention has been paid by the renal literature to ureteral endometriosis, a rare and silent disorder that can eventually lead to renal failure. In endometriosis, the ureteral involvement can be limited to a single ureter, more often the left one, or both ureters with consequent urine tract obstruction and ureterohydronephrosis. In most cases, the ureteral obstruction is caused by endometrial tissue surrounding the ureter (extrinsic ureteral endometriosis). In the remaining cases, endometrial cells are located within the ureter (intrinsic ureteral endometriosis). Progressive ureteral obstruction can be insidious in onset and can ultimately lead to renal failure if a correct diagnosis is missed. The true incidence of renal failure caused by endometriosis is completely unknown, although cases have been reported in the literature. The diagnosis of ureteral endometriosis is difficult since the disease may be clinically silent or associated with non-specific symptoms. Only a high index of suspicion and radiological support may help to obtain an early diagnosis. However, while renal imaging is useful in the cases of extrinsic endometriosis, the diagnosis of intrinsic endometriosis often requires ureteroscopy or laparoscopy. The prognosis of ureteral endometriosis depends on the time of diagnosis. In too many cases of bilateral obstruction, the patient is referred to the nephrologist because of an advanced, irreversible renal failure. Although some patients may benefit from progestin or anti-arotamase therapy, in most cases of ureteral endometriosis surgery is needed, laparoscopy surgery being preferred today to laparatomy.Copyright © 2009 S. Karger AG, Basel

Importance of the field:Idiopathic membranous nephropathy (IMN) can have a variable natural course. Treatments able to induce remission can improve the long-term prognosis. However, the optimal therapy for IMN remains controversial.Area covered in this review:We reviewed the historical and current literature from 1979 to 2010 regarding the natural course of IMN and the possible treatments giving special emphasis to randomized controlled trials and to more recent approaches.What the reader will gain:The reader will gain a comprehensive review of the available treatments of IMN. A personal therapeutic algorithm for nephrotic patients with IMN is also provided.Take home message:At least five different treatments showed efficacy in many (but not all) patients with IMN.

Primary nitro compounds have not been employed as nitrile oxide precursors in reactions with active methylene compounds because the reagents commonly used as dehydrating agents also react with these dipolarophiles. However, the CuIIcatalysed cycloadditioncondensation procedure has been shown to be viable with these substrates, leading directly to the expected polyfunctional isoxazoles provided nitro compounds with enhanced acidity “activated” were used. In the absence of added dipolarophiles, these nitro compounds underwent selfcondensation to the corresponding furoxans. However, as well as 3,4dibenzoylfuroxan, benzoylnitromethane predominantly gave the isomer 3benzoyl4nitro5phenylisoxazole, the structure of which was confirmed by crystallographic analysis. © WileyVCH Verlag GmbH & Co. KGaA, 69451 Weinheim, Germany, 2009

In combination with everolimus (EVL), cyclosporine A (CsA) may be used at low exposure, so reducing the risk of renal dysfunction in renal transplant recipients (RTR). We evaluated whether higher exposure of EVL could allow a further reduction of CsA.

A new family of isoxazolopyridobicyclooxacalix[4]arenes was obtained by reaction of dichloroisoxazolopyridines with phloroglucinol. Xray crystallography and density functional calculations were used for their structural determination and evaluation of their chemical properties. Their role as metal chelators was studied by mass spectrometry. This new family of heteracalixarenes is of potential interest for host–guest interactions. © WileyVCH Verlag GmbH & Co. KGaA, 69451 Weinheim, Germany, 2008

Cardiovascular disease is a frequent cause of morbidity after renal transplantation. The aims of this study were to evaluate the incidence of cardiovascular events and to identify the main risk factors for cardiovascular complications and mortality in 2071 white adults with a renal transplant functioning for at least 1 year.

Aims and background In 2006 a survey was performed to define the resources available in Italy for the provision of radiotherapy services to head and neck cancer patients. This was the first initiative of the newly founded Head and Neck Group of the Italian Association for Radiation Oncology.Methods A questionnaire was sent to all 138 radiotherapy centers active in the country. Items investigated included total numbers of head and neck cancer patients treated per year, waiting time before the start of treatment, general technical issues, and integration with surgery and chemotherapy.Results Sixty-nine questionnaires were returned (50% response rate). The total number of patients treated was 4,670, averaging 68 cases per center. The larynx was the primary site most frequently involved. Average waiting time was 30 days and 47 days for nonresected and postoperative cases, respectively. The combination of chemotherapy and radiotherapy was delivered to nonresected and resected patients in 96% and 54% of centers, respectively. Survey response rates, waiting time, and the use of organ preservation protocols were the issues showing more variations across the country.Conclusions This survey provides important data on radiotherapy resources available for head and neck cancer patients in Italy. The evidence of significant differences across the country concerning several relevant issues and the potential for cooperative clinical efforts in this relatively rare group of diseases urge the Group to plan further initiatives.

Little information is available about the long-term outcome of renal transplantation in patients with systemic vasculitis (SV). We compared the outcomes of 19 renal transplant recipients with SV with those of 38 controls matched for time of transplantation, age, gender and source of donor. The mean post-transplant follow-up was 58 ± 57 months for vasculitic patients and 61 ± 49 months for controls. The actuarial 10-year patient survival was 87 in vasculitic patients and 90 in controls, death-censored graft survival were 84 and 100, respectively. The risks of acute and chronic rejection, and arterial hypertension were not significantly different between the two groups. Infection was significantly more frequent in vasculitic patients (74 vs. 34; p 0.01). Seven patients (36.8) had a recurrence of vasculitis in mean 45 months after renal transplant (0.076patientsyear). After recurrence, one patient had an irreversible humoral rejection, another died from hemophagocytosis and another restarted dialysis 1 year later. Long-term patient and renal allograft survival in vasculitic patients was good. Although graft function recovered in most relapsers after reinforcement of immunosuppression, one patient died and two lost graft function.

Background. Rituximab, a chimeric monoclonal antibody, has been successfully given in various diseases including HCV-associated mixed cryoglobulinemia. However, only preliminary data exists on its efficacy and safety after renal transplantation.Methods. We report on a renal transplant recipient with chronic hepatitis C who received rituximab therapy for gastric cancer. Four rituximab infusions of 375 mg/m2were given.Results. Rituximab therapy was complicated by cholestatic hepatitis C with very high HCV RNA levels; liver insufficiency occurred. The patient developed bacterial pneumoniae and respiratory insufficiency was the cause of death. Although other mechanisms cannot be excluded, we found that rituximab therapy was implicated in the pathogenesis of cholestatic hepatitis C in our patient.Conclusions. We suggest that rituximab therapy may be associated with significant side effects. More experience has to be accumulated before any conclusions on efficacy and safety of rituximab therapy after RT can be drawn.

D'Agostino: GSK: Membership on an entity's Board of Directors or advisory committees. Offidani:Celgene: Consultancy, Honoraria; Janssen: Consultancy, Honoraria; BMS: Consultancy, Honoraria. Petrucci:BMS: Honoraria, Membership on an entity's Board of Directors or advisory committees; Takeda: Honoraria, Membership on an entity's Board of Directors or advisory committees; GSK: Honoraria, Membership on an entity's Board of Directors or advisory committees; Sanofi: Honoraria, Membership on an entity's Board of Directors or advisory committees; Janssen: Honoraria, Membership on an entity's Board of Directors or advisory committees; Celgene: Honoraria, Membership on an entity's Board of Directors or advisory committees; Amgen: Honoraria, Membership on an entity's Board of Directors or advisory committees. Liberati:JANSSEN: Honoraria; AMGEN: Honoraria; CELGENE: Honoraria; ROCHE: Honoraria, Research Funding; PFIZER: Honoraria, Research Funding; ONCOPEPTIDES AB: Honoraria, Research Funding; TAKEDA: Honoraria, Research Funding; MORPHOSYS: Honoraria, Research Funding; ONCONOVA: Honoraria, Research Funding; ABBVIE: Honoraria, Research Funding; NOVARTIS: Honoraria, Research Funding; KARYOPHARM: Honoraria, Research Funding; BMS: Honoraria; BEIGENE: Honoraria; ARCHIGEN: Honoraria; BIOPHARMA: Honoraria; FIBROGEN: Honoraria; INCYTE: Honoraria; VERASTEM: Honoraria, Research Funding. Benevolo:Amgen: Honoraria; Celgene: Honoraria; Novartis: Honoraria. Gaidano:Sunesys: Membership on an entity's Board of Directors or advisory committees; Janssen: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Astrazeneca: Membership on an entity's Board of Directors or advisory committees; Abbvie: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau. Galli:Takeda: Honoraria; Janssen: Honoraria; Celgene: Honoraria; BMS: Honoraria. Corradini:Janssen: Consultancy, Honoraria; Incyte: Consultancy; Gilead: Consultancy, Honoraria, Other: Travel and accommodations paid by for; Daiichi Sankyo: Consultancy, Honoraria; Celgene: Consultancy, Honoraria, Other: Travel and accommodations paid by for; Amgen: Consultancy, Honoraria, Other: Travel and accommodations paid by for; Sanofi: Consultancy, Honoraria; Servier: Consultancy, Honoraria; Takeda: Consultancy, Honoraria, Other; BMS: Other; KiowaKirin: Consultancy, Honoraria; Kite: Consultancy, Honoraria; AbbVie: Consultancy, Honoraria, Other: Travel and accommodations paid by for; F. Hoffman-La Roche Ltd: Consultancy, Honoraria; Novartis: Consultancy, Honoraria, Other: Travel and accommodations paid by for. Conticello:Amgen, Takeda, Janssen: Honoraria. Cavo:Novartis: Honoraria, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Janssen: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Other: Travel accomodations, Speakers Bureau; BMS: Honoraria, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Sanofi: Honoraria, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; GlaxoSmithKline: Honoraria, Speakers Bureau; Karyopharm: Honoraria; AbbVie: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees; Amgen: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Celgene: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Other: Travel accomodations, Speakers Bureau. Sonneveld:Skyline Dx: Honoraria, Research Funding; Karyopharm: Consultancy, Honoraria, Research Funding; Amgen: Consultancy, Honoraria, Research Funding; Janssen: Consultancy, Honoraria, Research Funding; Sanofi: Consultancy; Bristol-Myers Squibb: Consultancy, Honoraria, Research Funding; Takeda: Consultancy, Honoraria, Research Funding. Boccadoro:Mundipharma: Research Funding; Janssen: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; Bristol-Myers Squibb: Honoraria, Research Funding; Novartis: Honoraria, Research Funding; Sanofi: Honoraria, Research Funding; Celgene: Honoraria, Research Funding; Amgen: Honoraria, Research Funding; GlaxoSmithKline: Membership on an entity's Board of Directors or advisory committees; AbbVie: Honoraria. Bringhen:Bristol-Myers Squibb: Honoraria; Takeda: Consultancy; Celgene: Honoraria, Membership on an entity's Board of Directors or advisory committees; Karyopharm: Membership on an entity's Board of Directors or advisory committees; Janssen: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees; Amgen: Honoraria, Membership on an entity's Board of Directors or advisory committees.The presentation includes discussion of off-label use of a drug or drugs for the treatment of multiple myeloma (including carfilzomib, cyclophosphamide, dexamethasone, bortezomib, melphalan, prednisone and lenalidomide).

Background. Retroperitoneal fibrosis is a severe disease that affects the ureters, causing renal insufficiency in three-quarters of patients. The optimal treatment is far from being established.Methods. Seventeen patients with idiopathic retroperitoneal fibrosis and ureteral entrapment followed in our unit for at least 1 year were selected for this study. At presentation 13 patients had renal insufficiency.All patients received steroids, associated with ureterolysis in five (group 1), with azathioprine in six (group 2) and with tamoxifen in six (group 3). Four patients of group 2 and five of group 3 received ureteral stenting or nephrostomy. There were no significant differences among the three groups or the clinical and biochemical characteristics at presentation.Results. All patients of groups 1 and 2 entered remission after therapy. One patient from group 3 did not respond to therapy. During a mean follow-up of 56 ± 41 months, three patients (two from group 1, one from group 2, 18%) had a recurrence of the disease, which fully responded to retreatment in all three cases. At the last observation, all patients were alive; three patients (18%) had renal insufficiency, of them one from group 1 had to start dialysis 6 years after ureterolysis, one patient from group 2 and one from group 3 had serum creatinine of 1.5 mg/dl. Renal survival was 100% at 5 years and 80% at 10 years.Conclusions. In most patients, each of the three different therapeutic approaches restored renal function and significantly reduced the fibrotic mass in the short-term and maintained stable serum creatinine in the long-term.