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Aim Percutaneous renal biopsy is often essential for providing reliable diagnostic and prognostic information for people with suspected kidney disease, however the procedure can lead to complications and concerns among patients. This study aims to identify and integrate patient priorities and perspectives into the Kidney Health Australia - Caring for Australasians with Renal Impairment clinical practice guidelines for renal biopsy, to ensure patient-relevance. Methods We convened a workshop, consisting of three simultaneous focus groups and a plenary session, with 10 patients who had undergone a renal biopsy and seven caregivers. Topics and outcomes prioritized by patients and their caregivers were compared to those identified by the guideline working group, which was comprised of seven nephrologists. Transcripts and flipcharts were analyzed thematically to identify the reasons for participants' choices. Results In total, 34 topics/outcomes were identified, 14 of which were common to the list of 28 previously identified by the guideline working group. Most of the new topics identified by patients/caregivers were related to communication and education, psychosocial support, and self-management. We identified five themes underpinning the reasons for topic and outcome selection: alleviating anxiety and unnecessary distress, minimizing discomfort and disruption, supporting family and caregivers, enabling self-management, and protecting their kidney. A new topic on patient care and education was added to the guideline as a result. Conclusions Patient and caregiver involvement in developing guidelines on renal biopsy ensured that their concerns and needs for education, psychosocial support, and self-management were explicitly addressed; enabling a patient-centred approach to renal biopsies.

Background Heart or lung transplantation alone in individuals with significant pre-existing renal impairment results in high mortality and morbidity. Simultaneous heart-kidney (SHK) or simultaneous lung-kidney (SLK) transplantation may be considered in patients with dual organ failure not suitable for single organ transplantation. Aim We aimed to outline the Australian and New Zealand experience of SHK and SLK transplantations, focussing on patient characteristics and survival. Methods We analysed all SHK and SLK transplants performed in four centres across Australia and New Zealand between 1990 and 2014. Results Over the study period, 35 SHK and 3 SLK transplants were performed across 4 transplant centres. Mean age at transplantation for SHK transplants was 45 years, and for SLK transplant was 27 years. The most common aetiology of renal failure was glomerulonephritis. Most SHK transplant patients (77%) required renal replacement therapy prior to transplantation, with only one of the three patients undergoing SLK transplant, dialysis dependent. One-year survival for the cohort was 79%, which is lower than reported for single organ transplantation. However, 5- and 10-year survivals of 76% and 68%, respectively, were comparable. Isolated renal graft loss was seen in five patients, with only one patient successfully re-transplanted and the rest commencing dialysis. Conclusion The Australian and New Zealand experience of SHK and SLK includes 38 patients with a high 1-year mortality, but excellent 5- and 10-year survivals. Based on this, it seems reasonable to continue to offer combined organ transplantation to select patients with dual organ failure.

Summary Various methods of peritoneal dialysis (PD) catheter insertion are available. The purpose of this study was to evaluate a percutaneous insertion technique using ultrasound (US) and fluoroscopy performed under conscious sedation and as day case procedure. Data of 87 percutaneous inserted dialysis catheters were prospectively collected, including patients' age, gender, body mass index, history of previous abdominal surgery and cause of end stage renal failure. Length of hospital stay, early complications and time to first use were also recorded. Institutional review board approval was obtained. A 100% technical success rate was observed. Early complications included bleeding (n = 3), catheter dysfunction (n = 6), exit site infection (n = 1) and exit site leakage (n = 1). All cases of catheter dysfunction and one case of bleeding required surgical revision. Median time of follow-up was 18 months (range 3–35), and median time from insertion to first use was days 14 (1–47). Of the 82 patients who started dialysis, 20 (23%) ceased PD at some stage during follow-up. Most frequently encountered reasons include deteriorating patient cognitive or functional status (n = 5), successful transplant kidney (n = 4) and pleuro-peritoneal fistula (n = 4). Sixty-two (71%) PD catheter insertions were performed as day case. The remaining insertions were performed on patients already admitted to the hospital. Percutaneous insertion of dialysis catheter using US and fluoroscopy is not only safe but can be performed as day case procedure in most patients, even with a medical history of abdominal surgery and/or obesity.

Aims Calcific uraemic arteriolopathy (CUA) or calciphylaxis is most commonly seen in end-stage renal disease and is associated with significant morbidity and mortality. The aim of this study was to determine whether hyperbaric oxygen therapy (HBOT) is effective in healing calciphylaxis lesions and to determine if there are any patient factors that can predict wound healing and patient survival. Methods We identified by retrospective review all cases of CUA referred to our institution for treatment with HBOT. We documented the clinical and biochemical parameters of this patient population, the size and distribution of the lesions as well as wound outcomes and patient survival following treatment. Results A total 46 patients were identified with CUA associated with renal failure. Of the 46 patients, only 34 received a full course of HBOT. The balance was deemed unsuitable for treatment or was unable to tolerate treatment and was palliated. Of the 34 patients that received a full course of HBOT, 58% showed improvement in their wound scores, with more than half of these patients having complete healing of their wounds. The balance did not benefit from the therapy and had a very poor prognosis. Those that benefited from HBOT survived on average for more than 3 years. The only factor significantly associated with improved wound healing and survival was diabetes. Conclusion This retrospective analysis suggests a role for HBOT in the treatment of CUA with more than half of the treated patients benefiting and surviving for an average of more than 3 years.

Background Renal dysfunction is common after lung and heart-lung transplantation (Tx), and it limits the recipient's survival and quality of life. This study analyzed the outcomes of simultaneous and late kidney Tx following lung and heart-lung Tx. Methods From a single-center retrospective chart review of 1031 lung and heart-lung Tx recipients, we identified 13 simultaneous or late kidney Tx cases in 12 patients. Results Three patients underwent simultaneous deceased donor lung and kidney Tx. Eight patients underwent lung and heart-lung Tx, followed by nine living donor kidney Tx (including one ABO-incompatible Tx). One additional patient underwent a late deceased donor kidney Tx following heart-lung Tx. The median time from lung and heart-lung Tx to later kidney Tx was 127 (interquartile range [IQR], 23 to 263) months. Three patients died, 1 of sepsis, 1 of multiple organ failure, and 1 of transplant coronary disease. At a median follow-up of 33 (IQR, 10 to 51) months, 9 patients are alive and well. Eight patients required dialysis before kidney Tx for a median time of 14 months (IQR, 5 to 49). Kidney graft loss occurred in 1 patient at 51 months. After kidney Tx, dialysis was necessary in association with acute allograft dysfunction in 2 patients. No acute kidney rejection has been detected in any patient. Treatable acute lung rejection was seen in 1 patient. Well-preserved pulmonary function was noted in recipients of late kidney Tx. Conclusions Simultaneous kidney Tx and late deceased donor kidney Tx have challenges in the setting of lung Tx. By contrast, late living related kidney Tx after lung Tx is associated with excellent long-term survival and acceptable kidney and lung allograft function.

Background. Peritoneal dialysis (PD) is an important home-based dialysis modality for patients with end-stage kidney disease (ESKD). The initiation of PD requires timely and skilled insertion of a Tenckhoff catheter (TC). At most centres, TCs are inserted laparoscopically by surgeons under general anaesthetic. This requires access to increasingly scarce surgical, anaesthetic and hospital inpatient resources. Radiological insertion of TCs performed as a day procedure under local anaesthetic allows for easier access to the TC insertion with reduced resource requirements. We report our 1-year experience following the introduction of this technique to our PD programme. Methods. This is a retrospective review of the outcomes for all patients who had TCs inserted radiologically (percutaneously with the assistance of ultrasound and fluoroscopy) over the 12-month period from December 2011 to December 2012. Relevant patient demographics collected included age, gender, body mass index (BMI), previous abdominal surgery and cause of ESKD. Extended details of the insertion procedure were also obtained including length of stay, early complications and time to first use of the catheter for PD. Results. Thirty Argyle ™ Swan Neck TCs were inserted under radiological guidance during the study period. The mean age of patients was 56 (SD ± 14). The male-to-female ratio was 2:1. The mean BMI was 25.7 (SD ± 4.8). PD was the initial dialysis modality in 22 (73%) patients. Of the 30 patients, 14 (46.7%) had previously undergone extraperitoneal abdominal surgery. All catheters were inserted successfully as day cases except four patients (13.3%) who had catheters inserted during an inpatient hospital admission. Most catheters were not accessed for a minimum of 10 days to reduce the chance of exit site leakage, in two cases the catheters were used within 5 days without complication. There were no cases of peritonitis or exit site infection during the observation period. Catheter migration occurred in four patients (13.3%) but only one required surgical intervention. Minor pain issues were noted in six patients (20%) and bleeding around the exit site requiring suturing in two patients (6.7%). The introduction of this technique at our institution saw a 67% increase in the number of patients performing PD. Conclusions. Radiological insertion of TCs for PD provided improved access to catheter insertion in a timely manner with reduced resource requirements. Over the 12-month observation period we noted a high technical success rate with very few complications. Our study supports radiological insertion of TCs under local anaesthetic as a viable alternative to catheter insertion in theatre under general anaesthetic. The relative ease of radiological TC insertion has resulted in a significant increase in patient uptake of PD at our centre.

We present management strategies utilised for the first case of an urgent live-donor ABO incompatible B blood group renal transplant, in a patient with a prior A blood group lung transplant for cystic fibrosis. Three years on, renal function is excellent and stable, whilst lung function has improved.

We report a case of steroid- and cyclophosphamide-resistant nephrotic syndrome secondary to minimal-change disease occurring in an otherwise healthy 19-year-old female, responding rapidly to two doses of rituximab therapy. Complete disease remission has been sustained up to last follow-up (32 months) despite CD19 recovery. Literature review suggests emerging evidence that rituximab may have a role to play in recurrent and/or refractory minimal-change disease.

Induction therapy a. Combination treatment with cyclophosphamide plus prednisolone should be used for induction of disease remission when organ function is threatened. Both intravenous (IV) and oral cyclophosphamide show equal clinical efficacy and toxicity. (Level II evidence) b. Methotrexate is an alternative to cyclophosphamide-based induction therapy in patients with milder, early disease (serum creatinine 500 mmol/L). (Level II evidence)

Background The catastrophic variant of the antiphospholipid syndrome (CAPS), also now known as Asherson's syndrome, is defined as a potential life-threatening variant of the antiphospholipid syndrome, which is characterized by multiple small-vessel thrombosis that can lead to multiorgan failure. Relapses in patients with the CAPS are very uncommon. Objective To describe the clinical and laboratory features of patients with relapsing episodes of CAPS. Methods Three patients with relapsing CAPS are presented with their clinical and laboratory features. Results Seven episodes of CAPS that occurred in the 3 patients reported were analyzed. The median time between the episodes of CAPS was 12.5 months (range, 2.5-48). Precipitating factors were identified in 2 episodes only ( Legionella respiratory tract infection and periodontal infection). The most significant manifestations of the episodes were renal involvement (5 episodes), central nervous system and cardiac involvement (4 episodes), and pulmonary and hepatic involvement (3 episodes each). Interestingly, laboratory features of definite microangiopathic hemolytic anemia (MHA) were present in 5 of 7 episodes of relapsing CAPS. The remaining episodes presented with thrombocytopenia, schistocytes, and anemia but data concerning hemolysis and Coombs tests were not reported. Rituximab was used in 2 episodes. Conclusions Relapses occur very infrequently in patients with the CAPS. The presence of MHA is common in these patients, suggesting that an association between MHA and relapses of CAPS could be present and that a "continuum" between various MHAs might exist, as recently suggested.

Chronic renal disease is characterized by declining renal function, loss of intrinsic renal cells, and their replacement with fibrotic tissue. This study investigates apoptosis and its regulation in the context of chronic renal disease. RNA was extracted from renal biopsies from patients with various forms of chronic renal disease. Expression of genes of the Bcl-2 family, death receptor pathway, and growth factors were measured by reverse-transcription real-time polymerase chain reaction. Apoptosis was detected by the terminal deoxynucleotidyl transferase (TdT)-mediated dUTP nick end-labeling technique. Tubulointerstitial apoptosis was positively associated with tubulointerstitial injury and renal dysfunction and increased 2.3-fold per unit (U) increase in transforming growth factor beta(1) (TGFbeta(1)) mRNA (P0.05). Conversely, a 1 U increase in epidermal growth factor (EGF) mRNA was associated with a 47% decrease in tubulointerstitial apoptosis (P0.05). Tubulointerstitial injury was correlated with increased TGFbeta(1) and tumour necrosis factor alpha (TNFalpha) mRNA (P0.005) and decreased EGF mRNA (P0.05). Additionally, for a 10 U decrease in the glomerular filtration rate there was an estimated increase of 5 and 10% in TGFbeta(1) and TNFalpha mRNA, respectively (P0.05), whereas EGF mRNA decreased by an estimated 15% (P0.005). Therefore dysregulation of cytokine/growth factor expression plays a central role in the progression of chronic renal disease through contribution to renal cell loss, tubulointerstitial injury, and renal dysfunction.

Systemic hypertension is common among preterm infants with severe bronchopulmonary dysplasia (BPD); the exact cause is unknown. The objective of this preliminary hypothesis generating study was to examine systemic arterial structure and vasomotor function in a cohort of preterm infants with severe BPD, using a cohort of preterm infants without BPD and a cohort of term infants for comparison.After obtaining informed consent, we measured aortic wall thickness and vasomotor function by ultrasonography in 20 infants with severe BPD, 7 infants with no BPD, and compared them with 20 healthy term infants.Maximum aortic thickness was significantly higher in infants with BPD (827±163 μm) compared to those with no BPD (674±22 μm) and term infants (657±67 μm) (unadjusted P0.0001). The input impedance was similarly elevated in the infants with BPD (574±127 dynes s( )cm(-5)) compared to those with no BPD (325±24 dynes s cm(-)(5)) or term infants (328±113 dynes s cm(-)(5)) (unadjusted P0.0001). Stiffness index was significantly higher in the infants with BPD (3.4±0.6) compared to those with no BPD (2.6±0.3) or term infants (2.3±0.4) (unadjusted P0.0001). Systemic vascular resistance was also significantly elevated in the infants with BPD. The results remained significant even after adjusting for gestational age and birth weight. Measures of vasomotor function significantly correlated with blood pressure.The aortic wall thickness and vasomotor function are significantly altered in preterm infants with severe BPD. These findings may explain the higher incidence of systemic hypertension in this population.

Calcific uraemic arteriolopathy (CUA) or calciphylaxis is most commonly seen in end-stage renal disease and is associated with significant morbidity and mortality. The aim of this study was to determine whether hyperbaric oxygen therapy (HBOT) is effective in healing calciphylaxis lesions and to determine if there are any patient factors that can predict wound healing and patient survival.We identified by retrospective review all cases of CUA referred to our institution for treatment with HBOT. We documented the clinical and biochemical parameters of this patient population, the size and distribution of the lesions as well as wound outcomes and patient survival following treatment.A total 46 patients were identified with CUA associated with renal failure. Of the 46 patients, only 34 received a full course of HBOT. The balance was deemed unsuitable for treatment or was unable to tolerate treatment and was palliated. Of the 34 patients that received a full course of HBOT, 58% showed improvement in their wound scores, with more than half of these patients having complete healing of their wounds. The balance did not benefit from the therapy and had a very poor prognosis. Those that benefited from HBOT survived on average for more than 3 years. The only factor significantly associated with improved wound healing and survival was diabetes.This retrospective analysis suggests a role for HBOT in the treatment of CUA with more than half of the treated patients benefiting and surviving for an average of more than 3 years.

Background Survival after lung transplantation has improved, but with the consequence that long-term toxicities of treatment are of growing importance. In particular, renal impairment is common, has many causes, and carries with it increased morbidity and mortality. Methods We retrospectively analyzed clinical and laboratory data of 136 patients who underwent lung and heart–lung transplantation at our institution between 1990 and 2004 inclusive. Using multivariate analysis we considered the impact of age, gender, pulmonary diagnosis, transplant type (single lung, double lung, heart–lung), hypertension, diabetes mellitus, cigarette smoking, current immunosuppression, duration of calcineurin inhibitor (CNI) exposure and pre-existing renal impairment on renal function. Results At transplantation, creatinine clearance (CrCl) for the patient population was 108 ± 3.28 (mean ± SEM) ml/min/1.73 m2. At end of follow-up (6 ± 0.32 years) there was a significant decline in glomerular filtration rate (GFR) to 56.7 ± 1.78 ml/min/1.73 m2 (p < 0.001). Five of 136 patients (3.7%) developed end-stage renal failure (ESRF). On multivariate analysis, factors most strongly associated with this decline included (in order of significance): CrCl at transplantation; pack-years of cigarette smoking; exposure to sirolimus (SLM); CNI exposure; and age at transplantation. The rate of decline in GFR was linked to CrCl and age at the time of transplantation. Conclusions This analysis has demonstrated that patients with a lower baseline CrCl, older age at transplantation, and a smoking history are at high risk for rapid loss of renal function after transplantation. To best preserve kidney function, these patients should be targeted for aggressive risk factor modification as well as minimization of CNI exposure wherever possible.

Various methods of peritoneal dialysis (PD) catheter insertion are available. The purpose of this study was to evaluate a percutaneous insertion technique using ultrasound (US) and fluoroscopy performed under conscious sedation and as day case procedure. Data of 87 percutaneous inserted dialysis catheters were prospectively collected, including patients' age, gender, body mass index, history of previous abdominal surgery and cause of end stage renal failure. Length of hospital stay, early complications and time to first use were also recorded. Institutional review board approval was obtained. A 100% technical success rate was observed. Early complications included bleeding (n = 3), catheter dysfunction (n = 6), exit site infection (n = 1) and exit site leakage (n = 1). All cases of catheter dysfunction and one case of bleeding required surgical revision. Median time of follow-up was 18 months (range 3-35), and median time from insertion to first use was days 14 (1-47). Of the 82 patients who started dialysis, 20 (23%) ceased PD at some stage during follow-up. Most frequently encountered reasons include deteriorating patient cognitive or functional status (n = 5), successful transplant kidney (n = 4) and pleuro-peritoneal fistula (n = 4). Sixty-two (71%) PD catheter insertions were performed as day case. The remaining insertions were performed on patients already admitted to the hospital. Percutaneous insertion of dialysis catheter using US and fluoroscopy is not only safe but can be performed as day case procedure in most patients, even with a medical history of abdominal surgery and/or obesity.

A 78-year-old man with end-stage kidney disease secondary to autosomal dominant polycystic disease (ADPKD) on haemodialysis, presented to our institution following a myocardial infarction. He was known to have cystic involvement of his liver and spleen, significant mitral regurgitation secondary to mitral valve prolapse, and a distant history of abdominal aortic aneurysm repair. There was no family history of ADPKD or history of cerebral aneurysms. Coronary angiogram revealed widespread large coronary artery aneurysms affecting both branches of the left and right coronary artery, as well as significant atherosclerotic disease (Figure 1). During work up for coronary artery bypass surgery and mitral valve replacement, multiple other sites of aneurysmal disease were identified, including a 5 cm thoracic aortic aneurysm and bilateral femoral artery aneurysms (Figure 2). Fig. 1. Aneurysmal involvement of the mid to distal left anterior descending coronary artery (black arrow). Fig. 2. Bilateral femoral artery aneurysms (white arrows) together with large polycystic kidneys. Note incidental finding of calcified cyst in left polycystic kidney (black arrow). Whilst waiting for cardiac surgery the patient developed septic shock secondary to an infected renal cyst, requiring prolonged intensive care admission and extended rehabilitation. Given his age and frailty a decision was made to manage his widespread aneurysmal disease conservatively with regular radiographic surveillance. Previous case series have suggested a high incidence of coronary artery aneurysms in association with ADPKD. Hadimeri et al. [1] observed coronary aneurysms in 4 out of 30 (13%) ADPKD patients. This is markedly higher than the incidence observed in the general population, of ∼1.5% of patients studied by coronary angiography or autopsy [2]. There is less evidence regarding the link between ADPKD and aortic, thoracic and femoral aneurysms. While Bailey et al. [3] studied the association between aortic aneurysms and ADPKD, the study was unable to conclusively link the two conditions. Evidence linking thoracic or femoral aneurysms with ADPKD appears predominantly limited to case studies [4]. Given that the gene responsible for ADPKD has been linked with abnormalities of vascular smooth muscle, myofibroblasts, extracellular matrix and collagen defects [5], it seems reasonable for there to be widespread vascular abnormalities in this patient population, although it seems to be seldom described. In summary, we present a rare case of a patient with ADPKD and associated coronary artery aneurysms as well as aorto/iliac aneurysms.

A 75-year-old male was admitted for investigation of acute kidney injury. His medical history was significant for hypothyroidism and transfusion-dependent myelodysplastic syndrome. A bone marrow biopsy 1 month prior to admission had categorized him as having refractory anaemia with excess blasts (RAEB) Stage-II. His medications included thyroxine, cholecalciferol and the cytotoxic agent azacitadine. Over the preceding 6 months, he had noticed progressive lethargy, loss of weight and functional decline. Examination was unremarkable. Biochemistry demonstrated a serum creatinine level of 350 µmol/L, which had markedly worsened over 2 months. This was associated with 1.06 g/day proteinuria but no haematuria. Haematology showed normocytic anaemia (Hb 70 g/L), thrombocytopaenia (Plt 48 × 109/L) and marked leucocytosis (WCC 38.8 × 109/L). Dysplastic changes and occasional blasts were noted on the blood film (Figure 1). Serum protein electrophoresis, uric acid, autoimmune studies, hepatitis and HIV serology were unremarkable. Ultrasound demonstrated normal-sized kidneys of normal echogenicity and no evidence of obstruction. Fig. 1. May–Grunwald–Giemsa staining of the peripheral blood film shows pleomorphic population of blasts with an open chromatin and high nuclear:cytoplasmic ratio (thick arrows). Dysplastic neutrophils are also noted with abnormal segmentation ... A renal biopsy was performed, which showed renal cortex with heavy infiltration of atypical cells within the interstitium and lumina of small vessels. These cells had irregular nuclear membranes, prominent nucleoli and granular cytoplasm, and showed positive immunoperoxidase staining with myeloperoxidase, confirming the diagnosis of acute myeloid leukaemia (AML) with renal infiltration (Figures 2 and ​and33). Fig. 2. Highly cellular infiltration in the renal interstitium, atypical myeloid blasts with irregular nuclear membranes, prominent nucleoli and granular basophilic cytoplasm (H&E ×200). Fig. 3. Positive immunohistochemical staining for myeloperoxidase (MPO IHC ×100). A repeat bone marrow biopsy was subsequently performed which showed transformation into AML. The cause of the progressive kidney dysfunction was thus attributed to renal infiltration from AML. In view of the patient's poor prognosis and functional state, community-based palliative care was instituted. He passed away several weeks later. Malignant infiltration of the kidneys as a cause of renal failure has been well described [1–3]. The commonest causes are low-grade non-Hodgkin's lymphoma and acute lymphocytic leukaemia [4]. AKI due to diffuse kidney infiltration from AML has been reported in the setting of other precipitants [5]. Our case is unique, in that it demonstrates acute kidney injury resulting directly from malignant infiltration of renal parenchyma from AML. Conflict of interest statement. None declared.