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Autogenous arteriovenous fistulas (AVFs) are considered the most reliable long-term vascular access in patients undergoing haemodialysis. Although AVFs generally require fewer interventions than arteriovenous grafts (AVGs) to maintain patency, some sites of venous stenosis account for a large portion of repeated interventions. A particularly troublesome site within brachiocephalic AVFs is the cephalic arch. Cephalic arch stenosis (CAS) has been implicated in 19–77% of brachiocephalic fistula failures [1]. The cephalic arch has a unique architecture. In addition to containing a high concentration of valves and having a steep angulation, it crosses multiple tissue planes, bridging the superficial and deep venous systems while being tethered to rigid extravascular structures. These factors combine to make the cephalic arch particularly vulnerable to the development of stenotic lesions, as changes in vessel compliance produce turbulent flow. When an anastomosis is created between the brachial artery and the cephalic vein, the flow within the cephalic vein increases and the vessel starts dilating. However, the tissues surrounding the cephalic arch preclude its expansion, so that the cephalic vein narrows as it approaches its junction with the central veins. This sudden reduction in the circumference of a high-flow pathway results in an inflow–outflow mismatch, potentiating turbulence and the increased shear stress associated with it [1, 2]. When a CAS occurs, the management is not easy. Angioplasty of stenotic lesions involving the cephalic arch has limited effectiveness, which led investigators to seek surgical alternatives to treat these lesions and to preserve current fistula [1]. The option for cephalic vein transposition (CVT), described by Chen et al., involves surgical revision to redirect the blood flow to the adjacent patient veins. The surgical procedure entailed transecting the healthy portion of the cephalic vein distal to the stenotic segment in the arch, transposing and anastomosing it to the upper basilic [2] (Figure ​(Figure11). Fig. 1. Illustration of the cephalic vein transposition technique. We present the experience of our centre with upper arm CVT procedures in three haemodialysis patients who underwent haemodialysis treatment in the haemodialysis unit of Centro Hospitalar de Setubal E.P.E. Two of the 3 cases did not have the possibility of endovascular treatment. In these cases CVT to basilic vein made possible the preservation of vascular access. Both accesses are patents to date, 6 and 9 months, respectively. In the other case, the CVT allowed the reduction of percutaneous transluminal angioplasties (PTAs)/access-year in a patient with frequent recurrent CAS (requiring angioplasty in

The haemolytic-uraemic syndrome (HUS), a thrombotic microangiopathy, occurs often secondary to an infection due to Shiga-toxin-producing Escherichia coli (E Coli), the most threatening complication being neurological involvement [1]. Plasmata exchange has been shown to have some efficiency [2, 3]. Use of complement cascade-blocking antibody eculizumab is becoming the first-line treatment in the severest cases [4]. At the onset of spring in Europe in 2011, IgG depletion through immunoadsorption was reported to be effective in treating the severe neurological complications of HUS in adults [5, 6]. In October 2011, a 26-month-old girl presented with HUS requiring dialysis, with hepatic, pancreatic and cardiac involvement, seven days after the onset of E. coli enteritis. Eculizumab was administrated to treat severe neurological symptoms (unresponsiveness, comatose state, hypertonic quadriplegia, Glasgow score 8/15) due to bilateral thalamic infarctions seen on MRI one day after the onset of HUS (Figure 1). On day 3, a session of plasmapheresis was performed followed by a second eculizumab infusion because of the absence of neurological recovery. Despite this treatment and a slight increase in the platelet count, the patient's neurological status worsened, with recurrent occipital seizures and the need for mechanical ventilation. A third eculizumab infusion was carried out on day 8. Diffusion MRI sequences showed new lenticular lesions associated with right occipital-cortex lesions. In this life-threatening situation, we decided to perform IgG depletion by immunoadsorption as a rescue therapy (six sessions over five days, starting on day 9). A moderate clinical improvement (cessation of seizures, extubation on day 14) was associated with a rapid improvement in the disease activity (platelet count >150.109/L on day 16). Dialysis was stopped on day 13. The patient was discharged on day 60 with severe neurological sequelae: absence of eye contact, quadriplegia and major cortical and subcortical atrophy on MRI. The creatinine clearance rate was 45 mL/min, with 2 g/L proteinuria. Screening for complement function abnormalities (CFH, CFI, MCP, C3 and C4) and anti-CFH antibodies was negative. Three months later, surprisingly, the patient recovered her previous level of motor function with an independent walk and previous language skills. The creatinine clearance rate stayed at 45 mL/min. Thus, in this child with severe neurological HUS, eculizumab and immunoadsorption treatment were associated with an unexpectedly favourable evolution in the medium term. Fig. 1. Course of a 26-months-old girl with severe post-diarrhoea HUS treated by administration of eculizumab and by immunoadsorption. (a) day 1 MRI: diffusion-weighted image, axial view: marked bilateral hypersignal in thalami. (b) day 8 MRI: diffusion-weighted ... The effectiveness of plasmapheresis is controversial [2, 3], and despite encouraging results with eculizumab [4], this treatment is not effective in all forms of post-diarrhoea HUS with severe neurological involvement. We suggest that immunoadsorption, considered a therapeutic option in severe neurological HUS in adults, also be used as a rescue treatment in children. However, studies and protocols are needed to determine the relevance of the association between the two treatments and their timing in neurological forms of post-diarrhoea HUS in children.

Anti-glomerular basement membrane (GBM) glomerulonephritis is characterized by rapidly progressive glomerulonephritis (RPGN) with anti-glomerular basement antibodies. Although the underlying pathogenesis is not completely understood, factors such as smoking, cocaine, silica or mechanical stress are known to induce auto-antibody production. Moreover, studies have reported that radiotherapy may trigger auto-immunity: some cases revealed increased immune response to tumour antigens after local radiotherapy. Here, we describe a case of anti-GBM glomerulonephritis which developed post-radiotherapy for early prostate cancer. A 68-year-old Japanese male was admitted to our hospital for acute kidney injury (AKI) with serious rectal bleeding, which was thought to be the adverse effects of previous radiotherapy for prostate cancer. He had been well until he complained of gross haematuria, with increasing prostate-specific antigen (PSA) levels (8.74 ng/mL)(8.74 μg/L).

The incidence of clinically significant thrombosis in a cancer patient varies from 5 to 60%. Venous thrombosis remains more common than arterial thrombosis, and combined arterial and venous thrombosis in cancer patients is rare [1]. We report a rare case of combined pulmonary thromboembolism and complete thrombotic occlusion of the renal artery in a patient with non-small cell lung cancer (NSCLC) while receiving chemotherapy.

Tumor lysis syndrome (TLS) is a group of metabolic abnormalities that are usually seen in cancer patients treated with chemotherapy although it can occasionally occur spontaneously without chemotherapy in patients with a high tumor burden. It is due to the rapid lysis of tumor cells resulting in the release of large amounts of intracellular contents in the blood stream. This results in the rapid development of high levels of phosphate, potassium and uric acid accompanied by a profound decrease in serum calcium. TLS usually occurs in bulky tumors with a high turnover such as leukemias and lymphomas. The malignancy can be undiagnosed at the time of development of TLS [1]. In this report we present a case in which the patient presented with spontaneous TLS (STLS) and was later diagnosed with high-grade B-cell non-Hodgkin's lymphoma (NHL).

Orthostatic proteinuria is characterized by elevated levels of urinary protein excretion while in the upright or lordotic position and normal excretion when in the supine or recumbent position, such as that during bed rest. This type of proteinuria is the most frequent cause of isolated proteinuria in children and adolescents. In adolescents, the prevalence of orthostatic proteinuria is reported to be between 2 and 5% [1]. However, it is an uncommon disorder in individuals over 30 years of age [2]. As it is a benign condition requiring no further evaluation or specific therapy, the differential diagnosis of orthostatic proteinuria is crucial in clinical practice to avoid unnecessary interventions such as a kidney biopsy.

The most frequent complications that occur after the completion of an internal arteriovenous fistula are thrombotic. Other complications, such as infection, cardiac decompensation, aneurysm/pseudoaneurysm or median nerve injury, are less common. Nonetheless, after transplantation, adjuvant immunosuppression has long-term side effects, such as the development of malignancies. Here, we report the case of a patient subjected to renal transplantation who developed an angiosarcoma located in an internal arteriovenous fistula.

Sir, Collapsing glomerulopathy (CG) represents a distinct pattern of renal response to injury characterized by segmental to global collapse of capillaries in association with hyperplasia and hypertrophy of the visceral epithelial cells (VECs) associated with marked tubulointerstitial damage [1]. The reporting of CG in the literature has increased with the growing awareness among the nephrologists and pathologists of its association with disorders other than human immunodeficiency virus-1 infection [1–3]. Currently, it is classified as a variant of focal segmental glomerulosclerosis (FSGS) [3]. However, more recently, some authors have suggested that this relationship with FSGS may not last longer, and sooner or later it may be classified as a separate nosologic entity [2, 3]. With increasing awareness, the vascular lesions and thromboembolic phenomenon with consequent ischaemia have also emerged as important etiopathogenetic mechanisms in the development of CG in both the native and the transplanted kidneys [4–7]. More recently, the direct causal relationship between patchy infarction and de novo CG in transplanted kidneys has been reported [8]. However, no such link with acute cortical necrosis (ACN) secondary to post-partum haemorrhage (PPH) and hypovolaemic shock in native kidneys of young patients with no vasculopathy has been reported till date. We herein report two cases of CG involving the glomeruli in the vicinity of patchy ACN found on biopsies from native kidneys in two patients. Both patients were young females, 17 and 26 years, respectively, and presented with acute renal failure (ARF) following PPH. No history of drug intake or past medical illness of note was elicited. Ultrasound findings were not typical of ACN. Urine analysis was non-contributory. Relevant viral and autoimmune serology was negative. Both patients required dialysis initially, but one is off dialysis and maintaining serum creatinine at 221 μmol/L 8 months post-biopsy, whereas the other is on haemodialysis, waiting for kidney transplantation 10 months after diagnosis. Renal biopsies in both cases showed patchy infarction. In addition, both biopsies showed variable numbers of glomeruli in the vicinity of infarction, with segmental to global collapse of capillaries associated with hyperplasia and hypertrophy of VECs (Figure 1). There was moderate mixed inflammatory cell infiltration in the interstitium. However, no vasculopathy or thrombotic lesions were noted. Immunofluorescence was performed on snap-frozen tissue and showed segmental positivity of immunoglobulin M (IgM) and C3 in areas of collapsed tufts of viable glomeruli, whereas IgG, IgA and C1q were negative. Thus, both cases showed typical glomerular changes of CG involving the glomeruli in close proximity to patchy ACN. Fig. 1. High-power view showing a glomerulus from one of the renal biopsy specimens showing global collapse of capillary tufts associated with marked hypertrophy and hyperplasia of the podocytes. These show marked cytoplasmic vacuolization and protein resorption ... A report of three cases of zonal distribution of CG in the vicinity of patchy infarction secondary to severed accessory renal vessels in the transplanted kidneys has recently been published [8]. Similarly, occasional reports are also available in the literature, in which an association of FSGS and CG in the native kidneys with the vascular lesions has been observed [4–6]. However, this is the first report of CG in association with ischaemic ACN secondary to hypovolaemia resulting from PPH in native kidneys in two young females and provides evidence for the broad etiopathogenetic pathways leading to the final common pattern of CG. We also believe that this lesion is of secondary or reactive nature, rather than primary or idiopathic CG [9]. There is very little information in literature on the clinical behaviour, treatment and prognosis of secondary forms of CG, but these might be determined by the underlying disease. In conclusion, this report highlights the close association of ischaemia with CG and further expands the spectrum of associations of this renal lesion.

Hepatic biotransformation of gut microbiota-derived trimethylamine (TMA) to trimethylamine N-oxide (TMAO) may enhance cardiovascular risk [1, 2]. The source of TMA is fish-derived TMAO [3], meat-derived betaine/choline/phosphatidylcholine [1, 2], and the medicine l-carnitine [4]. There is no clear information on the metabolic fate of TMA/TMAO in patients receiving hemodialysis and treated with l-carnitine orally and intravenously. We report data showing that plasma TMA/TMAO concentrations in hemodialysis patients may be in the same range as those of healthy subjects consuming a typical diet in Japan. Fourteen subjects, randomly selected from a cohort of 18 male and 6 female Japanese patients (47–89 years) with diabetes mellitus or chronic glomerulonephritis who had undergone hemodialysis for several years, were divided into five untreated controls, five treated with 1000 mg of l-carnitine (l-Cartin injection, Otsuka, Tokyo, Japan) intravenously on 3 days (Days 1, 3 and 5) over 1 week, and four treated with 300 mg of oral l-carnitine (l-Cartin tablets, Otsuka) three times per day for 7 days. Ten healthy volunteers (22–59 years) were also enrolled. This study was approved by the ethics committees of Minami Ikebukuro Clinic and Showa Pharmaceutical University. Total carnitine and TMA/TMAO concentrations were determined by Kainos laboratories (Tokyo, Japan) and by gas chromatography [3]. Carnitine concentrations for 2–8 weeks after treatment with l-carnitine were significantly higher than those in the untreated group (Figure 1). Plasma TMA concentrations after oral treatment with l-carnitine were higher than those of the untreated group. TMAO concentrations did not increase significantly after treatments and were in the range of daily interindividual variations. In separate observations in 22 of the 24-patient cohort, pre-dialysis plasma concentrations of carnitine (33 ± 4 µM, mean ± SD), TMA (34 ± 20 µM) and TMAO (189 ± 94 µM) were decreased by hemodialysis (12 ± 8, 25 ± 8 and 140 ± 77 µM, respectively). These concentration ranges were comparable with TMA (26 ± 17 µM) and TMAO (197 ± 100 µM) in healthy volunteers (n = 10). The present plasma levels of TMA and/or TMAO in Japanese patients were one or two orders of magnitude higher than the reported concentrations in Caucasians [1, 2], but were consistent with a review of patients undergoing hemodialysis [4]. Fig. 1. Plasma concentrations of total l-carnitine (A), TMA (B) and TMAO (C) in untreated patients (circles) and in patients treated with l-carnitine intravenously (triangles) and orally (squares). The three bars are averages of the three groups (n = 4 or 5). ... Some TMA formation in a Japanese cohort treated with l-carnitine orally was confirmed, but the levels of TMA/TMAO both pre-/post-dialysis in patients not receiving l-carnitine were in the same ranges as those of the control subjects.

A rise in serum creatinine without acute kidney injury (AKI) is possible. In the last 5 years, three patients presented to us for high serum creatinine levels after binge drinking. We already reported on two of these three patients [1, 2]. For the first one, dialysis was instituted as the initial diagnosis was AKI. With the experience gained, the next two patients were examined specifically for a particular cause. They were not dialyzed. The data of the third patient were as follows: a 44-year-old male, admitted after a fall following binge alcohol consumption. He complained of painful abdominal distension and was anuric. Serum creatinine was 6.5 mg/dL (575 µmol/L), blood urea: 66 mg/dL (23.5 mmol/L), serum sodium 134 mmol/L and serum potassium 4.3 mmol/L. An abdominal ultrasound examination showed two normal kidneys, a normal urinary tract and presence of peritoneal fluid. In view of these findings, a bladder rupture was suspected and confirmed by cystoscopy. With bladder drainage, serum creatinine diminished to 3.5 and then 1.4 mg/dL (309–123.7 µmol/L). Laparotomy was done and a 4-cm tear on the dome of urinary bladder was surgically repaired. All three patients had similar histories: (i) an alcoholic binge, (ii) a trauma, (iii) sudden onset of abdominal pain and progressive distension (iv) anuria (v) elevated serum creatinine (vi) cystography revealing a tear in the bladder and (vii) improvement in serum creatinine on continuous bladder drainage. The data of these patients are given in supplementary Table S1. In the majority of bladder ruptures, there is a history of significant abdominal trauma. Following heavy alcohol intake, the distended bladder is very susceptible to injury and can be ruptured by a minor trauma. Alcohol-induced polyuria and impaired sensorium that removes cues for voiding cause overdistension of the bladder and rupture. The intoxicated patient may fail to recall the trauma. In a review of 20 isolated intraperitoneal bladder ruptures, the mean time between presentation and diagnosis was 5.4 days [3]. The diagnosis was based on a compatible history: suprapubic pain, anuria, hematuria and rapidly increasing ascites. The diagnosis was strengthened by rapidly rising serum creatinine without features of hypercatabolism. Continuous bladder drainage normalized laboratory abnormalities. Urinary bladder injuries after blunt or penetrating trauma are rare, owing to the bladder's anatomical position [4]. Bladder ruptures can be extraperitoneal, intraperitoneal or both, depending on the site of the injury. Intraperitoneal bladder ruptures are usually associated with blunt abdominal trauma (excluding iatrogenic causes), and unlike extraperitoneal leaks, they are not usually associated with pelvic fractures. Intraperitoneal ruptures usually occur at the dome of the bladder in contrast to extraperitoneal leaks which are most often lateral [4]. Non-traumatic rupture is even rarer and causes may be essentially divided as increase in intravesical pressure or weakening of the bladder wall. In most cases, spontaneous rupture occurs in the presence of a urothelial neoplasm or after pelvic radiation therapy [2]. In neonates, peritoneal entry of urine is a rare complication. The major causes are congenital obstructive uropathy with urine leakage into the peritoneal cavity and bladder perforation as a complication of umbilical artery catheterization [5]. The expression ‘reverse peritoneal dialysis’ was first used in 1991 [5]. It is characterized by a flux of small molecules such as creatinine and urea from urine collected in the peritoneum along a concentration gradient opposite to conventional peritoneal dialysis where small molecules move from the blood to peritoneal cavity, a phenomenon mentioned as ‘reverse autoperitoneal dialysis’. As a result, the blood values of creatinine and urea are elevated mimicking renal failure despite normally functioning kidneys. This was described as ‘pseudo acute renal failure’ in the literature.

Rupture of the peritoneal catheter extension is a rare complication in peritoneal dialysis. We report a case of a 75-year-old male with chronic kidney disease of unknown aetiology on continuous ambulatory peritoneal dialysis for 5 months. While manipulating the catheter for the drainage of the first exchange in the morning, the catheter extension broke. In order to not interrupt the drainage, the patient inserted a cylinder of plastic, a straw he had found in his home, as shown in the picture (Figure ​(Figure1).1). The quality of this home remedy showed more than acceptable results, due to the similarity of the cylinder with the catheter in terms of thickness and consistency, so the patient could complete the drainage and the next infusion. Fig. 1. Home remedy with a straw as part of the catheter extension. Once the patient was disconnected, he came to our peritoneal dialysis unit for the replacement of the catheter extension, proud of having solved the problem himself. Prophylactic antibiotic and antifungal treatment was prescribed and the patient, fortunately, did not develop peritonitis. Was this a work of genius or a work of madness?

Sir, The importance of renal function in the prognosis of patients with multiple myeloma (MM) has been well established and rapid reversal of renal failure may offer the best long-term outcome. The use of extracorporeal means of removing immunoglobulin free light chains (FLCs) responsible for cast nephropathy in patients with MM is controversial. Nephrologists are interested in a treatment strategy combining chemotherapy and extracorporeal treatments in patients with biopsy-proven cast nephropathy or a high probability of cast nephropathy (defined as >200 mg/dL on the FLC assay). Some previous studies have shown that the plasma exchange is unable to remove sufficient FLCs for clinical benefits. The issue of whether an extended duration of dialysis with high cut-off (HCO) dialysers is more effective than plasma exchange at removing FLC or reversing renal failure is not settled [1]. Hutchison and Leung reported that a 60% reduction in FLCs by Day 21 is associated with recovery of renal function for 80% of the studied patients [2]. Our experience: a 43-year-old man, who was not complaining of any previous disease and with a previously normal renal function, suffering from biopsy proven lambda myeloma kidney, without any histological sign of chronic renal damage, and renal failure dialysis dependant, in 2 weeks received 10 haemodialysis (HD) treatments with HCO-dialyser (Theralite Gambro). We quantified the concentration of FLCs by nephelometry (Biocite, N latex test; Siemens). Initially, λ FLC concentration was 5500 mg/L. At the end of HCO dialyser HD cycle, the concentration was 94.80 mg/L (Table 1). Table 1. Concentrations and ratios of FLC before and after HCO-dialyser HDa We did not observe any adverse effects. We observed by Day 7 a sustained and >50% reduction of FLCs with dialysis alone before the chemotherapy was initiated. After the third HCO-dialyser HD, the patient started PAD Orlowsky chemotherapy (Bortezomib–Doxorubicin–Dexamethasone) with successful haematological result but with partial renal function recovery. Currently (5 months after HCO-dialyser treatment), the patient is on maintenance HD two times a week. Some reports show a recovery of renal function after several months. If the patient does not recover normal renal function, we think that a new kidney biopsy can help his management.