Absence of thrombocytopaenia and/or microangiopathic haemolytic anaemia does not reliably exclude recurrence of atypical haemolytic uraemic syndrome after kidney transplantation.

A 54-year-old man was diagnosed with atypical haemolytic uraemic syndrome (aHUS) with confirmed complement H mutation in 2012, requiring ongoing dialysis. He was commenced on eculizumab in 2014 once the pharmaceutical board approved this drug. After 4 months, he received a live unrelated donor renal transplant from his wife and continued eculizumab post-transplant. Three months later, there was a rise in his creatinine with no laboratory features of haemolysis and a kidney biopsy confirmed rejection, which was treated with increased immunosuppression. After completing 12 months of treatment with eculizumab, he opted for close monitoring rather than continuation with therapy. Five months post-cessation of the drug, there was a rise in creatinine, and once again, haematological parameters remained within reference range; however, his kidney biopsy showed features consistent with recurrence of aHUS; hence, eculizumab was recommenced with good effect. While there was no evidence of haemolysis, there was a gradual rise in LDH level and a drop in platelet count, although the parameters remained within the normal range. This suggests that aHUS can recur in the allograft in the absence of haematological abnormalities. Clinicians should have a low threshold for allograft biopsy if haematological parameters are not just outside the reference range, but possibly also if there are changes of at least >25% from baseline in platelet count and LDH levels, particularly in those patients who are no longer eligible for eculizumab.

Appropriate Assessment of Symptom Burden and Provision of Patient Informatio.

Patients with ESKD, with or without RRT, are heavily burdened with symptoms which may interact and compound each other. The burden of symptoms experienced by patients on dialysis is rarely mentioned in patient information sheets despite being well documented in research data. There are significant barriers to medication use in ESKD including a lack of knowledge of pharmacokinetics in dialysis and conflicting information about drug dose and safety. There is a growing body of literature on the symptom management of patients with ESKD Patients need clear information about the potential effects dialysis and non-dialysis pathways on symptom burden and how this can change with time Standardisation of tools used to collate information about symptoms can assist in the provision of information to patients. We recommend the POS-S (Renal) tool (accessible via the kcl.ac.uk website) for assessing symptom burden.

Inappropriate interventions in the dying patient.

A core competency of Nephrology should be the capacity to diagnose dying. Withdrawal of dialysis is ethically and legally valid.

Pharmacokinetics and safety of deferasirox in subjects with chronic kidney disease undergoing haemodialysis.

AIM: Treatment of chronic kidney disease (CKD) includes parenteral iron therapy, and these infusions can lead to iron overload. Secondary iron overload is typically treated with iron chelators, of which deferasirox is one of the most promising. However, it has not been studied in patients with CKD and iron overload. METHODS: A pilot study was conducted to evaluate the pharmacokinetics and safety of deferasirox in eight haemodialysis-dependent patients, who were receiving intravenous iron for treatment of anaemia of CKD. Deferasirox was administered at two doses (10 mg/kg and 15 mg/kg), either acute (once daily for 2 days) or steady-state (once daily for 2 weeks). RESULTS: A dose of 10 mg/kg in either protocol was not sufficient to achieve a plasma concentration in the therapeutic range (acute peak 14.1 and steady-state 22.8 µmol/L), while 15 mg/kg in either protocol maintained plasma concentration well above this range (acute peak 216 and steady-state 171 µmol/L). Plasma concentration observed at 15 mg/kg was well above that expected for this dose (40-50 µmol/L), although no adverse clinical events were observed. CONCLUSION: This study highlights the need to profile drugs such as deferasirox in specific patient groups, such as those with CKD and iron overload.

The sensitivity and specificity of ultrasound estimation of central venous pressure using the internal jugular vein.

PURPOSE: The fluid volume status of a patient is difficult to assess clinically. The aim of this study was to compare the ultrasound estimation of the height of the right internal jugular vein (CVP(IJV)) with direct estimation of central venous pressure (CVP) (CVP(CVC)). MATERIALS AND METHODS: A portable ultrasound machine defined the "top" of the right internal jugular vein in 44 patients from a single tertiary hospital. The vertical height from this point to the sternal angle was used to estimate CVP(IJV). A central venous catheter was then inserted and direct measurement of CVP was made with a pressure transducer. A normal CVP was defined as 3 to 6 mm Hg. RESULTS: For overloaded patients, CVP(IJV) correlated well with CVP(CVC), P = .004, sensitivity of 64.3%, specificity of 81.3%, and positive predictive value of 85.7%. The area under the curve for the receiver operating characteristic curve was 0.73 (95% confidence interval, 0.59-0.86). For undervolumed patients, the correlation remained statistically significant, P < .001, sensitivity of 88.9%, specificity of 77.1%, and negative predictive value of 96.4%. The area under the curve was 0.83 (95% confidence interval, 0.70-0.96). CONCLUSION: Ultrasound estimation of CVP using a portable ultrasound machine and the internal jugular vein is simple, noninvasive, and accurate.

End-stage kidney disease due to scleroderma--outcomes in 127 consecutive ANZDATA registry cases.

BACKGROUND: Scleroderma is an uncommon cause of end-stage kidney disease (ESKD) which carries significant morbidity and mortality risks. The aim of this study was to determine the prevalence, treatment and outcomes of scleroderma patients with ESKD. METHODS: A study was conducted of all ESKD patients enrolled in the ANZDATA registry, who commenced dialysis between 15 May 1963 and 31 December 2005, and remained on dialysis for at least 90 days. RESULTS: Of the 40 238 patients who commenced dialysis during the study period, 127 (0.3%) patients had ESKD secondary to scleroderma. Scleroderma ESKD patients were more likely than other ESKD patients to be female (72% versus 43%, P < 0.001), Caucasian (98% versus 79%, P < 0.001) and of lower BMI (22.7 ± 4.7 versus 26.0 ± 5.9, P < 0.001) with a higher prevalence of chronic lung disease (36 versus 14%, P < 0.001) and lower prevalence of diabetes mellitus (10% versus 32%, P < 0.001) and coronary artery disease (23% versus 35%, P = 0.01). Median survival was significantly shorter in scleroderma ESKD (2.43 years, 95% confidence interval (CI) 1.75-3.11 years) than other ESKD (6.02 years, 95% CI 5.89-6.14 years, log-rank score 55.7, P < 0.001). Renal recovery was more likely in scleroderma patients (10% versus 1%, P < 0.001) with a shorter time to recovery. Scleroderma was found to be an independent predictor for mortality (HR 2.47, 95% CI 1.99-3.05) and renal recovery (HR 11.1, 95% CI 6.37-19.4). Five year deceased donor and live donor renal allograft survival rates of recipients with scleroderma were 53 and 100%, respectively. CONCLUSIONS: Scleroderma is an uncommon cause of ESKD, which is associated with increased risks of both spontaneous renal recovery and mortality.

Pseudomonas peritonitis in Australia: predictors, treatment, and outcomes in 191 cases.

BACKGROUND AND OBJECTIVES: Pseudomonas peritonitis is a serious complication of peritoneal dialysis. To date, there as been no comprehensive, multicenter study of this condition. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: The predictors, treatment, and clinical outcomes of Pseudomonas peritonitis were examined by binary logistic regression and multilevel, multivariate Poisson regression in all Australian PD patients in 66 centers between 2003 and 2006. RESULTS: A total of 191 episodes of Pseudomonas peritonitis (5.3% of all peritonitis episodes) occurred in 171 individuals. Its occurrence was independently predicted by Maori/Pacific Islander race, Aboriginal/Torres Strait Islander race, and absence of baseline peritoneal equilibration test data. Compared with other organisms, Pseudomonas peritonitis was associated with greater frequencies of hospitalization (96 versus 79%; P = 0.006), catheter removal (44 versus 20%; P < 0.001), and permanent hemodialysis transfer (35 versus 17%; P < 0.001) but comparable death rates (3 versus 2%; P = 0.4). Initial empiric antibiotic choice did not influence outcomes, but subsequent use of dual anti-pseudomonal therapy was associated with a lower risk for permanent hemodialysis transfer (10 versus 38%, respectively; P = 0.03). Catheter removal was associated with a lower risk for death than treatment with antibiotics alone (0 versus 6%; P < 0.05). CONCLUSIONS: Pseudomonas peritonitis is associated with high rates of catheter removal and permanent hemodialysis transfer. Prompt catheter removal and use of two anti-pseudomonal antibiotics are associated with better outcomes.