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Most patients with chronic kidney disease (CKD) require renal replacement therapy. Majority of hemodialysis initiation is done through a dialysis catheter access. The use of catheters double lumen (CDL) for hemodialysis has several potential complications, including infection. Catheter related bloodstream infection (CRBSI) is associated with increased morbidity and mortality in CKD patients. CRBSI therapy should not be delayed, including delays due to waiting for culture results to confirm the diagnosis. Several studies have reported the use of clinical findings and routine blood count parameters to predict CRBSI. This case series reported three cases with suspected CRBSI consisting of one woman and two men with mean age of 44.67 ± 8.21 years, mean catheter days 13 ± 6.97, and mean hemodialysis duration of 10.5 ± 13.79 month. Diagnosis CRBSI was suspected from clinical clue, i.e symptoms such as fever and chills, pain at the CDL site, and physical examination i.e body temperature and local CDL site signs. Laboratory parameters in this case series reported elevations for PLR, NLR and SIRI supporting the suspicion of bloodstream infection. All patients in this case series improved after CDL removal and antibiotics therapy. Investigation of clinical findings and routine blood count parameters can be an alternative diagnosis of CRBSI that enables prompt treatment in areas with limited facilities. Keywords: chronic kidney disease, catheter related bloodstream infection, diagnosis

Advances in the treatment of chronic kidney disease (CKD) resulted in expanding therapy goals from simple prolongation of life to a return to normal social functioning and having an active and satisfactory life after reaching adulthood. Objectives. The aim of the study was to evaluate life activity, disease acceptance (DA) and quality of life (QOL) in patients with end-stage renal disease (ESRD) treated with renal replacement therapy (RRT) since childhood. Material and methods. We surveyed 117 patients aged ≥16 years on RRT since childhood. The control group included 25 healthy subjects. We used questionnaires that included a sociodemographic questionnaire (questions regarding education, work, family, and offspring), Acceptance of Illness Scale (AIS), Satisfaction With Life Scale, and Kidney Disease Quality of Life (KDQOL). Results. A completed survey was returned by 45 respondents aged 27.16 ±6.78 years, among whom 82.2% had a transplanted kidney and 17.8% were on hemodialysis (HD). Higher education was reported by 18.18% of respondents, secondary and primary by 63.64% and 18.18%, respectively. Employment was reported by 46.67% of the respondents. A family was started by 35% of women and 4% of men. Good DA was found in 28.9% of the respondents. Satisfaction with life was lower in the study group compared to the control group. We found statistically significant correlations between the age when the kidney disease was diagnosed and satisfaction with life (r = 0.33), and between the time since the last change of RRT modality and emotional well-being (r = 0.34). The number of kidney transplantations correlated negatively with emotional component of QOL (r = -0.66) and emotional well-being (r = -0.73). Conclusions. Patients treated with RRT were quite well adapted to their chronic disease but showed less ability to live independently. Young age at the diagnosis of kidney disease, loss of kidney transplant and living on social security benefit had a negative effect on their emotional well-being. [ABSTRACT FROM AUTHOR]

Background: Leigh syndrome (LS) is a rare neurodegenerative mitochondrial disorder which typically presents in childhood but has a varied clinical course. Renal involvement such as proximal tubulopathy in patients with mitochondrial disorders has been described. However, end stage renal disease (ESRD) is uncommon and literature regarding patients undergoing kidney transplantation is limited. Successful deceased donor renal transplant has not been previously described in a patient with Leigh Syndrome.Case Presentation: We report a 21-year-old Han Chinese man who presented with limb weakness and unsteady gait, which progressed rapidly over a period of months until he was wheelchair-bound. He subsequently developed ESRD and was commenced on hemodialysis. Investigations revealed a m.13513G > A mutation with clinical and radiological features consistent with LS. His mitochondrial disease stabilised and he underwent a multidisciplinary assessment for deceased donor kidney transplantation to identify and minimise the LS-associated perioperative risks and potential negative effects of immunosuppressants on his LS. Successful kidney transplantation followed with excellent graft function three and a half years post-transplant and improvement in the patient's physical function.Conclusion: This case highlights the importance of careful pre-transplant perioperative risk assessment and post-transplant care in a rare and heterogeneous neurological disease to achieve an ultimately excellent clinical outcome. To our knowledge, this is the first report of successful deceased donor kidney transplant in a patient with known LS. [ABSTRACT FROM AUTHOR]

Polycystic kidney disease (PKD) is a systemic disorder which adds majority of renal patients to end stage renal disease. Autosomal dominant polycystic kidney disease (ADPKD) is more prevalent and leading cause of dialysis and kidney transplant. Linkage analysis revealed some closely linked loci, two of which are identified as PKD1, PKD2 and an unidentified locus to ADPKD. This study was performed using PCR and automated DNA sequencing in 84 cases and 80 controls to test potential candidature of PKD2 as underlying cause of PKD by in silico and statistical analyses. Two associated symptoms, hypertension (19%) and liver cyst (31%) havemajor contribution to PKD. Gender-based analysis revealed that familial female patients (27%) and familialmale patients (33%) are more hypertensive. Liver cyst, the second major contributing symptom presented by large percentage of sporadic males (46%). Genetic screening of all 15 exons of PKD2 revealed eight pathogenic (c.854_854delG, c.915C>A, c.973C>T, c.1050_1050delC, c.1604_1604delT, c.1790T>C, c.2182_2183delAG, c.2224C>T) and eight likely pathogenic (g.11732A>G, c.646T>C, c.1354A>G, g.39212G>C, c.1789C>A, c.1849C>A, c.2164G>T, c.2494A>G)DNA sequence variants. In our study, 27.38% (23/84) cases shown pathogenic / likely pathogenic variants in PKD2 gene. Some regions of PKD2 prone for genetic variation suggested to be linked with disease pathogenesis. This noticeable hot spot regions hold higher frequency (50%) of pathogenic / likely pathogenic genetic variants constituting single nucleotide variants than large deletion and insertion that actually represents only 41.08% of coding sequence of PKD2. Statistically significant association for IVS3-22AA genotype was observed with PKD, while association of IVS4+62C>Twas found insignificant. [ABSTRACT FROM AUTHOR]

Aims: End-stage renal disease (ESRD) increases the risk of implantable cardioverter-defibrillator (ICD) infection. We sought to define outcomes of lead extraction in patients with ESRD.Methods and Results: Implantable cardioverter-defibrillator lead extractions at our institution from January 2006 to March 2014 were stratified by absence (Control-Ex, n = 465) or presence (ESRD-Ex, n = 43) of ESRD. Procedural outcomes and survival were determined by medical records review. Survival in the ESRD-Ex group was compared with a contemporaneous cohort with ESRD undergoing ICD lead implantation (ESRD-I, n = 127). Among extraction patients, those with ESRD were more likely to be extracted for infection (74.4% vs. 28.6%, P < 0.001). Extraction procedure success (Control-Ex: 97% vs. ESRD-Ex: 93%, P = 0.17) and procedural deaths (Control-Ex: 1.1% vs. ESRD-Ex: 2.3%, P = 0.413) were similar. Survival 1 year following extraction was worse in the ESRD-Ex group compared with the Control-Ex, with a survival rate of 65.6% vs. 92.6% (P < 0.001); these curves continued to diverge through year 3. One-year survival in the ESRD-Ex group was worse than among ESRD patients undergoing ICD implant (ESRD-I), but these curves converged and survival was similar by year 3.Conclusions: Implantable cardioverter-defibrillator lead extraction can be performed safely and effectively in patients with ESRD. However, despite high rates of procedural success, long-term mortality following extraction in ESRD patients is substantial. Much of the long-term mortality risk appears to be accounted for by the presence of ESRD and an indication for an ICD. [ABSTRACT FROM AUTHOR]

Background: Chronic kidney disease (CKD) has become a major public health issue worldwide in the past few years. Pruritus is a common, well-recognized complication often seen in patients with chronic renal failure. For assessment of pruritus, different tools are used but these tools are unable to identify the changes and variations in the severity of pruritus. The aim of our study was to validate the Urdu-version of the Urdu-version of the 5D itch scale among patients suffering from chronic kidney disease in Pakistan.Method: The 5D itch scale was translated from English into Urdu following translation guidelines for translation. Face and content validity was determined by a panel of experts and piloted. For retest, the Urdu version of the 5D itch scale was administered at baseline and two weeks.Results: A total of 50 participants with end stage renal disease were recruited, and of these, 64% were males. Exploratory factor analysis revealed that the 5D-IS had 2-factor loadings: "Pattern and activity" and "Distribution" with Kaiser-Mayer-Olkin (KMO) = 0.802, Bartlett's test of sphericity was significant (df = 28, p < 0.001). At test re-test, Cronbach's alpha was 0.914, while the intra class correlation was 0.9160 (95% confidence interval 0.941-0.975), which is a highly significant correlation (p < 0.0001).Conclusion: The Urdu version of the 5D itch scale was found to be a valid and reliable instrument for assessing pruritus and its severity in patients with chronic kidney disease in Pakistan. [ABSTRACT FROM AUTHOR]

Background: Since diabetes mellitus (DM) is the leading cause of end stage renal disease (ESRD), this study aimed to develop a 5-year ESRD risk prediction model among Chinese patients with Type 2 DM (T2DM) in primary care.Methods: A retrospective cohort study was conducted on 149,333 Chinese adult T2DM primary care patients without ESRD in 2010. Using the derivation cohort over a median of 5 years follow-up, the gender-specific models including the interaction effect between predictors and age were derived using Cox regression with a forward stepwise approach. Harrell's C-statistic and calibration plot were applied to the validation cohort to assess discrimination and calibration of the models.Results: Prediction models showed better discrimination with Harrell's C-statistics of 0.866 (males) and 0.862 (females) and calibration power from the plots than other established models. The predictors included age, usages of anti-hypertensive drugs, anti-glucose drugs, and Hemogloblin A1c, blood pressure, urine albumin/creatinine ratio (ACR) and estimated glomerular filtration rate (eGFR). Specific predictors for male were smoking and presence of sight threatening diabetic retinopathy while additional predictors for female included longer duration of diabetes and quadratic effect of body mass index. Interaction factors with age showed a greater weighting of insulin and urine ACR in younger males, and eGFR in younger females.Conclusions: Our newly developed gender-specific models provide a more accurate 5-year ESRD risk predictions for Chinese diabetic primary care patients than other existing models. The models included several modifiable risk factors that clinicians can use to counsel patients, and to target at in the delivery of care to patients. [ABSTRACT FROM AUTHOR]

Background/Aims: In hemodialysis (HD) patients the endothelial and erythrocyte glycocalyx is impaired which in turn correlates with elevated erythrocyte sodium sensitivity (ESS). Additionally, apoptotic erythrocyte death (eryptosis), characterized by phosphatidylserine (PS) exposure on the cell surface, is increased in this population. We aimed to explore the relationship of ESS and eryptosis. Methods: Blood samples were collected from 11 healthy controls and 20 chronic HD patients before and after midweek HD. ESS was quantified by the salt blood test. PS-exposure, intracellular reactive oxygen species (ROS) of erythrocytes and reticulocytes were assessed by flow cytometry. Results: Compared to controls ESS was significantly higher in HD patients preHD and did not change during treatment. The percentage of eryptotic cells did not differ between controls and patients preHD. However, eryptosis decreased during HD. ESS and eryptosis were uncorrelated, while eryptosis was positively correlated with intracellular ROS and percent reticulocytes. Conclusions: Higher ESS levels in HD patients indicate a pathologic glycocalyx. ESS and eryptosis were not correlated. The decreased eryptosis postHD may possibly be related to dialytic uremic toxin removal, but is likely multifactorial. The relationship between eryptosis and intracellular ROS warrants further research. [ABSTRACT FROM AUTHOR]

Glomerular filtration rate and/or creatinine are not accurate methods for renal failure prediction. This study tested homocysteine (Hcy) as a predictive and prognostic marker for end stage renal disease (ESRD). In total, 176 subjects were recruited and divided into:healthy normal group(108 subjects);mild-to-moderate impaired renal function group(21 patients);severe impaired renal function group(7 patients); andchronic renal failure group(40 patients) who were on regular hemodialysis. Blood samples were collected, and serum was separated for analysis of total Hcy, creatinine, high sensitive C-reactive protein (CRP), serum albumin, and calcium. Data showed that Hcy level was significantly increased from normal-to-mild impairment then significantly decreases from mild impairment until the patient reaches severe impairment while showing significant elevation in the last stage of chronic renal disease. Creatinine level was increased in all stages of kidney impairment in comparison with control. CRP level was showing significant elevation in the last stage. A significant decrease in both albumin and calcium was occurred in all stages of renal impairment. We conclude Hcy in combination with CRP, creatinine, albumin, and calcium can be used as a prognostic marker for ESRD and an early diagnostic marker for the risk of renal failure. [ABSTRACT FROM AUTHOR]

Background: Chronic kidney disease (CKD) and end-stage-kidney disease (ESKD) continue to be under-diagnosed and a major burden for Aboriginal communities in central Australia. The aim of this study was to examine the risk of poor clinical outcomes associated with elevated albumin-to-creatinine ratio (ACR) among Aboriginal people in central Australia.Methods: Cox proportional hazards models were used to estimate the risk of end stage kidney disease (ESKD), dialysis, CVD (cardiovascular disease) and mortality associated with participants' baseline albuminuria reading from a 10-year cohort study of Aboriginal people (n = 623) from three communities in central Australia. Predictors of progression of albuminuria were also examined in the context of the Kidney Health Australia (KHA) Risk Matrix.Results: A baseline ACR level of ≥3.5 mg/mmol was associated with an almost 10-fold increased risk of ESKD (95%CI 2.07-43.8) and a 15-fold risk of dialysis (95%CI 1.89-121). Albuminuria ≥3.5 mg/mmol was also associated with a borderline 63 % increased risk of CVD (95%CI 0.98-2.71). No significant association was observed with mortality from all-causes or chronic disease. Diabetes and a waist-to-hip ratio ≥0.90 independently predicted a two-fold increased risk of a progression to higher ACR levels.Conclusions: A single measure of moderately increased albuminuria was a strong predictor of renal failure in this population. A single spot urine ACR analysis in conjunction with the KHA Risk Matrix may be a useful and efficient strategy to screen for risk of CKD and progression to dialysis in remote communities. A focus on individuals with diabetes and/or central obesity for strategies to avoid increases in albuminuria may also prevent future CKD and CVD complications. [ABSTRACT FROM AUTHOR]

PurposeDisrupted bone metabolism in patients with chronic kidney disease (CKD) is associated with elevated concentrations of biochemical bone markers. Recently, animal studies show the role of osteocalcin in energy metabolism, which is partially confirmed in humans. The aim of our study was to evaluate the relationships between serum concentrations of bone markers and indices of energy metabolism in CKD patients on maintenance hemodialysis; in particular, the relationship between various forms of osteocalcin and adiponectin.Patients and methodsThe cross-sectional study included 155 hemodialyzed stage 5 CKD patients. Serum concentrations of glucose, insulin, adiponectin, bone alkaline phosphatase (bALP), tartrate resistant acid phosphatase (TRAP), carboxylated (cOC), undercarboxylated (ucOC), and intact osteocalcin (OC) were determined.ResultsIn total cohort, bALP, TRAP, cOC, and ucOC negatively correlated with BMI. All analyzed bone markers positively correlated with adiponectin in total cohort and in men. In multiple linear regression analysis including all patients, log(cOC) and log(intact OC) were the only bone markers that predicted log(adiponectin) (beta = 0.22;p = 0.016 and beta = 0.26;p = 0.010) independently of sex, dialysis vintage, CRP, insulin, iPTH concentrations, BMI, and age.ConclusionsOur data confirm the positive association between cOC, intact OC, and adiponectin concentrations in CKD patients on maintenance hemodialysis. [ABSTRACT FROM AUTHOR]

Background: Within-class comparative effectiveness studies of β-blockers have not been performed in the chronic dialysis setting. With widespread cardiac disease in these patients and potential mechanistic differences within the class, we examined whether mortality and morbidity outcomes varied between cardio-selective and non-selective β-blockers.Methods: Retrospective observational study of within class β-blocker exposure among a national cohort of new chronic dialysis patients (N = 52,922) with hypertension and dual eligibility (Medicare-Medicaid). New β-blocker users were classified according to their exclusive use of one of the subclasses. Outcomes were all-cause mortality (ACM) and cardiovascular morbidity and mortality (CVMM). The associations of cardio-selective and non-selective agents on outcomes were adjusted for baseline characteristics using Cox proportional hazards.Results: There were 4938 new β-blocker users included in the ACM model and 4537 in the CVMM model: 77 % on cardio-selective β-blockers. Exposure to cardio-selective and non-selective agents during the follow-up period was comparable, as measured by proportion of days covered (0.56 vs. 0.53 in the ACM model; 0.56 vs 0.54 in the CVMM model). Use of cardio-selective β-blockers was associated with lower risk for mortality (AHR = 0.84; 99 % CI = 0.72-0.97, p = 0.0026) and lower risk for CVMM events (AHR = 0.86; 99 % CI = 0.75-0.99, p = 0.0042).Conclusion: Among new β-blockers users on chronic dialysis, cardio-selective agents were associated with a statistically significant 16 % reduction in mortality and 14 % in cardiovascular morbidity and mortality relative to non-selective β-blocker users. A randomized clinical trial would be appropriate to more definitively answer whether cardio-selective β-blockers are superior to non-selective β-blockers in the setting of chronic dialysis. [ABSTRACT FROM AUTHOR]

Introduction: The global prevalence of chronic kidney disease (CKD) is estimated to be 8-16%. Studies have shown that the increased mortality in patients with CKD is due to anemia that leads to cardiovascular disease (CVD), also known as "Cardio renal anemia syndrome". The present study was undertaken to look into the laboratory profiles of end stage renal disease (ESRD) patients. Aim: To study the laboratory profiles of End stage renal disease (ESRD) patients coming for hemodialysis. Materials and Methods: The study was a retrospective, crosssectional study done by collecting data from the medical case records of all patients during a period of one year from January 1st 2014 to December 31st 2014. Records of a total of 140 patients who underwent hemodialysis during this period were taken. The laboratory profiles that was recorded included haemoglobin, serum sodium, potassium, chloride, fasting glucose, calcium and phosphorus. Results: The mean age of the subjects was 53.5±14.5 yrs. All the patients had moderate anaemia. There was a significant difference in the mean systolic and diastolic blood pressure, serum creatinine and serum urea values between males and females. The mean serum calcium levels were low. Conclusion: The present study is the first such study in this rural area and shows evidence of a relatively young population with ESRD having moderate anaemia and hypertension. There is evidence of hypocalcaemia and serum phosphorus is on the higher end of the normal range. These findings are usually associated with a higher risk of mortality. With the explosion of diabetes and hypertension in India, chronic kidney disease should be diagnosed and managed as early as possible if not prevented. [ABSTRACT FROM AUTHOR]

Background: Calciphylaxis is a rare, yet life-threatening disease mainly occurring in dialysis patients. Traditional options of treatment remain unsatisfactory. Methods: Here we present a novel, combined approach, treating calciphylaxis with IV sodium thiosulfate, cinacalcet and sevelamer. In a case series five hemodialysis patients, have been successfully treated with this regimen. Treatment and survival data were analyzed using descriptive statistics. Results: In all patients, a rapid decrease in pain, improvement of general condition and wound healing within six months occurred. Side effects were low. Drug dosages: IV sodium thiosulfate initial dose 119.4 +/- 84.9 g/m2/week, maintenance dose 40.6 +/- 9 g/m2/week; cinacalcet: maintenance dose 36 +/- 32.9 mg/d and sevelamer maintenance dose 3320 +/-1671 mg/d. One and two year survivals were 100 % and 80 %, respectively. We also report on long-term application of IV sodium thiosulfate of up to 52 months. Patient survival after diagnosis was 52, 84, 21, 36 and 30 months, respectively. Survival since initiation of hemodialysis was 76, 136, 89, 36 and 35 months, respectively. Conclusion: This novel combined approach, a multi-modal treatment of calciphylaxis with persistent hyperparathyroidism, using IV sodium thiosulfate, cinacalcet and sevelamer seems to improve the outcome of this devastating disease. © 2013 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Background: Hyperuricemia has been associated with the development of high blood pressure (BP). We studied the effects of allopurinol therapy in hyperuricemic hemodialysis (HD) patients with high BP. Materials and Methods: This single-blind, randomized cross-over clinical study involved 55 HD patients with serum uric acid level > 6.5 (men) and > 5.5 mg/dL (women). They were randomly divided in two groups, each of which went through two phases. Group-1 in phase-1 received 100 mg/day orally of allopurinol for three months; while Group-2 was given whatever medication they received prior to the study. After two months of washing period, the groups were crossed-over. The BP levels were measured before and after HD during the eight months study period. Results: Fifty-three patients completed the study (33 men and 20 women, with mean age of 55.8 years). Uric acid levels decreased significantly during the12 weeks of allopurinol therapy (7.71 ± 1.53 to 5.2 ± 1.2 P < 0.005). Overall, after the 12 weeks of allopurinol therapy, systolic and diastolic BP also significantly decreased in allopurinol group, 15.8% (139 to 117, P < 0.0005) and 8.6% (81 to 74, P < .0005), respectively. There were not significant changes in body mass index, blood urea nitrogen, creatinine, albumin, cholesterol, triglyceride, hemoglobin, liver enzymes and serum electrolytes level after treatment. Patients treated with allopurinol had a significant increase in the quality of dialysis (KT/V) (P: 0.043). Conclusions: In HD patients, allopurinol treatment reduced BP. The results indicate a new potential therapeutic approach for controlling BP in HD patients. [ABSTRACT FROM AUTHOR]

This open label, multicentric, comparative clinical trial was done to compare the efficacy and tolerability of two sevelamer formulations, sevelamer carbonate, and sevelamer hydrochloride, in the treatment of hyperphosphatemia in Indian end stage renal disease (ESRD) patients. A total of 97 ESRD patients on hemodialysis, were enrolled. Patients were randomized to receive either sevelamer carbonate or sevelamer hydrochloride. All patients were evaluated every week for 6 weeks for efficacy and safety variables. Total 88 patients completed the study. After 6 weeks of therapy, there were similar reductions (P<0.0001) in mean serum phosphorus and the CaxP product both the groups. The responder rates for test and reference groups were 75%, 68.18% respectively (P=0.3474). The adverse events reported were nausea, abdominal pain/discomfort, heartburn, constipation, diarrhea, increased prothrombin time, and severe arthritis. No serious adverse events were reported. There was no significant difference between the groups for adverse events and the laboratory parameters. From the results of this multicentric, comparative, randomized clinical study on sevelamer carbonate we can recommend that sevelamer carbonate may be used as a phosphate binder in Indian chronic kidney disease patients. [ABSTRACT FROM AUTHOR]

Purpose: To review the association of an abnormal prenatal sonogram with most recent serum creatinine in patients with proven posterior urethral valves (PUV). Methods: Since 1992, all live-born patients between 1992-2004 with clinically proven PUV, with postnatally proven PUV, from 2 pediatric tertiary care centers, were reviewed for age at diagnosis, most recent serum creatinine, presence of chronic renal failure (CRF) (serum creatinine >2 standard deviations above normal for age), or end stage renal disease (dialysis or transplant). Available antenatal reports from the 2 centres and surrounding community hospitals were reviewed for gestational age (GA) at the time of ultrasound, volume of amniotic fluid, and urinary-tract abnormality. Results: Thirty-four patients with proven PUV and prenatal sonograms were identified (1992-2004). Eighteen patients had abnormalities on their prenatal sonogram, with poor outcome in 5 (mean follow-up, 8 years [1-13 y]). No specific features were identified on prenatal sonogram. Sixteen patients had normal prenatal sonograms, with poor outcomes in 2 (mean follow-up, 8 years [3-13y1]). There is an increased risk of an abnormal serum creatinine among those patients with an abnormal prenatal study, odds ratio (OR) 2.6 (95% confidence interval, 0.35-32). Conclusions: PUV represents a spectrum of disease severity. A normal prenatal ultrasound does not preclude PUV. The majority of patients with a normal prenatal examination have good outcomes. The OR suggests that there may be increased risk for poor outcome in those with an abnormal prenatal examination. A multicenter study is necessary to obtain a larger sample size and more precise ORs. [ABSTRACT FROM AUTHOR]

We report two end stage renal disease (ESRD) patients, who rapidly developed neurotoxicity after taking baclofen, a centrally acting gamma.aminobutyric acid agonist. They presented to our hospital in a state of confusion. On physical examination, there were no focal neurological deficits and the remainder of the examination was also not diagnostic. Laboratory data were unremarkable. The consciousness improved with supportive treatment and intensive hemodialysis over 3-4 days. In conclusion, physicians should be aware of the possibility of baclofen-induced neurotoxicity in patients with renal disease, especially in ESRD patients and it is necessary to avoid or reduce baclofen dosage in these patients. [ABSTRACT FROM AUTHOR]

Background: Up to one third of patients on renal replacement programmes have an unknown cause of kidney disease, and the diagnosis may only be established following renal transplantation when the disease recurs or if new extra-renal symptoms develop.Case Presentation: We present two patients who presented with progressive chronic kidney disease of unknown cause. Both patients underwent successful renal transplantation but subsequently developed multisystem abnormalities, and were ultimately diagnosed with mitochondrial cytopathy 10-15 years following transplantation.Conclusions: Mitochondrial cytopathies are rare inborn errors of metabolism that should be considered in adults with renal impairment, especially in those with a family history of kidney or other multisystem disease. The widespread availability of genetic testing provides the potential for earlier diagnoses, thereby enhancing management decisions, anticipation of complications, avoidance of mitotoxic drugs, and informed prognosis prediction. [ABSTRACT FROM AUTHOR]

Background: End stage renal disease (ESRD)/ end stage renal failure (ESRF) is on the rise globally and especially in Singapore. Varicella or chickenpox is not uncommon among adults especially ESRD/ESRF patients. It has been reported to cause complications and even death among immunocompetent adults.Methods: A retrospective data collection on patients with varicella infection and ESRD in Singapore General Hospital (SGH) from the year 2005 to 2016 was performed. Continuous data and categorical data were summarized as median (range) and count (%) respectively. The association of health care utilization (total length of hospital stay, readmission related to varicella, intensive care unit admission) and mortality with complication due to varicella were tested using chi-square and Mann-Whitney test for categorical and continuous outcomes respectively.Results: Sixty-six patients with ESRD developed varicella during the study period (2005-2016). The case incidence rates for varicella among ESRD ranges from 97 to 267 per 100,000 populations with ESRD yearly. There were 9 deaths (13.6%). Mortality was higher among the ESRD patients with one or more varicella complications compared to patients without complications ((25% vs 7.1%, 95% CI for difference: - 1.1%, 36.9%, p = .063). Likewise, utilisation of intensive or high dependency units were higher among patients with complications compared to those without (20.8% vs 2.4%, 95% CI for difference: 1.6%, 35.3%, p = .012). Length of stay was twice as long in the group with complications compared to patients without (median (IQR) days: 14 (8, 21) vs 7 (5, 14), p = .065), although it did not reach statistical significance.Conclusions: Varicella is associated with high morbidity and significant mortality rate in ESRD patients. Varicella vaccination is recommended for seronegative ESRD patients. [ABSTRACT FROM AUTHOR]