Refractory seizures in a dialysis patient and a vitamin consigned to oblivion.

Intradialytic breakthrough seizures refractory to multiple classes of antiepileptic medications are not common and can be due to many different reasons. Pyridoxine deficiency is an under-recognized cause of such seizures and frequently missed in clinical practice. Many factors specifically related to dialysis can lead to pyridoxine deficiency and in turn can contribute to refractory seizures. Herein, we report one of the very few cases of intradialytic breakthrough refractory seizures secondary to pyridoxine deficiency recognized in the literature.

Characteristics and Outcome of Biopsy-proven Malignant Hypertension with Severe Kidney Injury: A Retrospective Study.

BACKGROUND: Although malignant hypertension begets multiple target organ damage, there is limited data on patients with severe renal injury and evident malignant hypertension in renal histopathology. METHODS: We assessed the baseline demographic, histopathological findings and clinical outcomes in this retrospective analysis of patients with biopsy-proven malignant hypertension. RESULTS: Thirty cases were analysed, the mean age of patients was 40 ± 11.5 years, 28 (93.3%) were males and the average systolic and diastolic blood pressures at hospitalisation were 197.04 ± 24.14 and 117.41 ± 18.31 mmHg, respectively. Severe retinopathy was seen in 10 (33.3%). The median eGFR at admission was 6.3 (IQR 4.4-9.15) mL/min and 21 (72.4%) needed dialysis. Nine (30%) cases with glomerular crescents were having the primary glomerular disease (7 IgAN, 1 C3 glomerulonephritis, 1 membranoproliferative glomerulonephritis) and 17 (56.6%) had thrombotic microangiopathy. Three-month ESRD free survival was 34.5% (n = 10) and the ESRD cohort had more incidence of dialysis requiring kidney injury at presentation (94.4% vs. 40% in the non-ESRD cohort). Patient survival at 1 year was 50%. Isolated malignant hypertension, differed from others with regard to lesser incidence of severe retinopathy, less glomerular sclerosis (29.61 ± 15.86 vs. 48.45% ± 30.78; P = 0.03), absence of crescents (P = 0.02), more incidence of tuft wrinkling (100% vs. 35%, P = 0.00) and total vessel occlusion (P = 0.02). CONCLUSION: Clinicopathologically, accelerated essential hypertension differs from hypertension of glomerular disease. Degree of kidney injury at presentation is risk predictor for long-term morbidity in malignant hypertension.

Chronic Kidney Disease after Snake Envenomation Induced Acute Kidney Injury.

Snake bite is an important cause for acute kidney injury (AKI) in the tropics and the victims are often otherwise healthy young adults without conventional risk factors for chronic kidney disease (CKD). Available literature on long-term outcomes of AKI -snake envenomation is limited, with only two small retrospective studies with follow-up periods of two years or less. In a hospital-based prospective and retrospective follow-up study, all consecutive adult patients who developed AKI and discharged alive were recruited in the prospective group and for retrospective analysis, we retrieved medical records of patients hospitalized with snake bite from the digitized medical records. Clinical and biochemical characteristics during hospitalization and on follow-up were collected. We recruited 193 patients with snake bite and AKI in a prospective (140) and a retrospective (53) group. Of the 193 AKI patients, 162 (84%) had estimated glomerular filtration rate (eGFR) <60 mL/min/1.73 m2 at the time of discharge from hospital and at follow-up (n = 171) 29 (16.9%) had eGFR<60 mL/min/1.73 m2. Of the 116 patients with follow-up beyond one year, 30 (26%) had CKD. Overall 26 to 28% of patients went on to develop CKD depending on the duration of follow-up. Only very few (1.16%) patients progressed to ESRD. This is the largest follow-up study so far on snake bite-related AKI and provides further evidence that snake bite-associated AKI causes CKD on long-term follow-up. Older age, diabetes, severe AKI, and inadequate anti-snake venom administration were risk factors for the development of CKD on follow-up.

Erratum: Prognosis and long-term outcomes of acute kidney injury due to snake envenomation.

[This corrects the article DOI: 10.1093/ckj/sfz055.][This corrects the article DOI: 10.1093/ckj/sfz055.].

A Newly Recognized Endemic Region of CKD of Undetermined Etiology (CKDu) in South India-"Tondaimandalam Nephropathy".

INTRODUCTION: Chronic kidney disease (CKD) is being increasingly recognized as a public health problem in India. The entity of CKD of undetermined etiology (CKDu) is increasingly being reported globally. Here we describe the burden of CKDu in a heretofore undescribed population in South India. METHODS: We prospectively enrolled all patients with CKD referred to the nephrology department in an observational registry. We analyzed their sociodemographic and clinical features over 4 years. The diagnosis of CKD and its etiology was determined using predefined criteria. Geolocalization of CKD patients was performed. Subsequently, CKD screening was conducted in a village located in an area of CKDu clustering. RESULTS: A total of 2424 patients were analyzed; the median age was 52 years and 75.3% were male. Seventy-five percent had advanced CKD. CKDu was the most common (51.7%) etiologic category. This is the highest proportion of CKDu reported among all published CKD studies to date from India. The clinical and demographic profile of this patient population match that of CKDu patients reported from Sri Lanka and Central America, where CKDu is endemic. A clustering of cases of CKDu was noted in specific districts using a geographic information system software. Screening of 447 people in an outreach program at a village located in an area identified to have clustering of CKDu showed a CKD prevalence of 19%. CONCLUSION: We report a previously unrecognized endemic area of CKDu among the underprivileged population engaged in agricultural labor in coastal southeastern India in the states of Tamil Nadu and Puducherry (Tondaimandalam) in India.

Prognosis and long-term outcomes of acute kidney injury due to snake envenomation.

BACKGROUND: Snakebite is a common occupational hazard in tropical countries. To date, the literature on snakebite-related acute kidney injury (AKI) has been limited by retrospective study designs, lack of uniformity in case definitions of AKI and limited follow-up. This study aims to identify the in-hospital outcomes and long-term changes in kidney function that follow haemotoxic envenomation. METHODS: All adult patients admitted with AKI following haemotoxic envenomation from January 2016 to June 2017 were recruited and followed up until July 2018. Predictors of in-hospital mortality was assessed. Long-term follow-up data on kidney function were collected from survivors. RESULTS: In total, 184 patients with haemotoxic envenomation and AKI were recruited. The mean age of the subjects was 42.2 years [95% confidence interval (CI) 40.3-44.7]. The majority were male (71.2%). The mortality of patients with haemotoxic envenomation was 21.5%. The mortality was considerably higher in patients with Kidney Disease: Improving Global Outcomes (KDIGO) Stage 3 AKI [relative risk (RR) 4.45 (95% CI 1.14-17.42)] and those who met KDIGO urine output criteria [RR 20.45 (95% CI 2.84-147.23)]. A Cox regression model identified mechanical ventilation [odds ratio (OR) 5.59 (95% CI 2.90-10.81)], hypotension [OR 2.48 (95% CI 1.31-4.72)] and capillary leak syndrome [OR 2.02 (95% CI 1.05-3.88)] as independent predictors of mortality. Long-term follow-up data were available for 73 patients. A total of 21 patients (28.7%) developed adverse renal outcomes (glomerular filtration rate <60 mL/min/1.73 m2, urine albumin excretion >30 mg/g and new-onset hypertension or prehypertension). CONCLUSIONS: AKI resulting from snake envenomation is associated with considerable risk of mortality. The greater the AKI stage the greater the likelihood of mortality. One-third of patients with AKI developed long-term complications like chronic kidney disease, prehypertension and hypertension over the follow-up period.

Outcome of biopsy-proven lupus nephritis with low glomerular filtration rate at presentation.

OBJECTIVES: Currently, there is limited data regarding the outcomes of lupus nephritis (LN) with moderate to severe renal failure at presentation (defined by low glomerular filtration rate; GFR <30 mL/min). METHODS: Sixty-four patients with biopsy-proven LN and estimated GFR (eGFR) <30 mL/min were prospectively analyzed. Outcome measure of persistently low eGFR, end-stage renal disease (ESRD) or death at 365 days were grouped as Major Adverse Kidney Events (MAKE365). RESULTS: Diagnosis of lupus was simultaneous with onset of renal disease in 60% of cases. Histologically, 82.3% (n = 51) were class IV, the median serum creatinine was 4 mg/dL (interquartile range [IQR] 3.1-5.9 mg/dL), median eGFR was 13.75 mL/min (IQR 9.25-19 mL/min) and 42.2% (n = 27) required dialysis at presentation. Induction regimens included National Institute of Health (68.2%), Eurolupus protocol (10.9%) and mycophenolate mofetil (8%). Over 365 days, 23 (37.5%) subjects died, while 41 (62.5%) survived. The majority of deaths were due to infection and sepsis (14/23). Among the survivors, 70.7% had good renal outcome, 12.1% had persistently low GFR (<30 mL/min), while 17% developed ESRD. In this group, treatment response rate was 84.6% (complete response 25.6%, partial response 59%). Those with a better renal function at presentation had a good treatment response (100% vs. 40%). Altogether, n = 35 (54.6%) were included in the MAKE365 category. Between the renal survival group (n = 29) versus the MAKE365 group (n = 35) there was no difference in clinical or histological parameters. CONCLUSION: The current treatment protocols had a good response rate in patients with LN even with severe kidney injury at presentation. However, the risk of serious infections and subsequent mortality was high.

Reliability of Anthropometry-Based Equations Compared to Dual Energy Absorptiometry for Assessing Body Composition in Predialysis Chronic Kidney Disease-A Longitudinal Study.

OBJECTIVES: Skinfold thickness measurements for assessing body composition are reported to have good reproducibility compared to the reference method of dual energy absorptiometry (DXA). In the current study, we compared the level of agreement between body composition measured with DXA and skinfold thickness (SFT) in CKD Stage 3 and 4, at 2 occasions, 6 months apart. METHODS: Body composition was assessed in 177 Indian patients with CKD Stage 3 and 4 using DXA and anthropometry (SFT). The body fat mass obtained by the 2 methods was compared by paired t-test, intraclass correlation coefficients, regression analysis, and Bland-Altman plots. A linear regression analysis was done to identify the patient-related parameters which would account for the intermethod differences between DXA and SFT. RESULTS: Compared to DXA, SFT underestimated the fat mass at baseline as well as 6 months [DXA vs. SFT at entry: 15.85 kg (95% confidence interval, CI 15.07-16.65) vs. 13.71 kg (95% CI 13.21-14.32), P < .001; at 6 months: 16.13 (95% CI 15.33-16.93) vs. 13.85 (95% CI 13.25-14.45), P < .001]. The intraclass correlation coefficients at entry and 6 months were 0.894 (0.857-0.921) and 0.896 (0.860-0.923), respectively. The intermethod differences between DXA and SFT at baseline and 6 months were comparable: 2.08 kg (95% CI 1.66-2.5) at baseline versus 2.27 kg (95% CI 1.83-2.71) at 6 months, P = 0.200. Gender and body mass index turned out to be the significant predictors of intermethod differences at base line and exit (P < .001). CONCLUSIONS: SFT-based measurements show good reproducibility compared to DXA over a period of 6 months. However, SFT systematically underestimates the fat mass by 2 Kg compared to DXA.

Correction of metabolic acidosis improves muscle mass and renal function in chronic kidney disease stages 3 and 4: a randomized controlled trial.

BACKGROUND: Metabolic acidosis (MA) is associated with a loss of muscle mass and faster deterioration of kidney function in patients with chronic kidney disease (CKD). A few single-centre randomized trials have reported favourable outcomes following correction of MA. Additional good quality evidence on the safety and efficacy of alkali supplementation is required in epidemiologically different patient subsets with CKD. METHODS: A single-centre, open-label, randomized, prospective parallel-group study was conducted to assess the effect of correction of MA on body composition and kidney function. A total of 188 patients with CKD stages 3 and 4, with venous bicarbonate levels <22 mEq/L were randomized. The intervention arm received standard care as per Kidney Disease: Improving Global Outcomes (KDIGO) 2012 guidelines along with oral sodium bicarbonate supplementation to maintain venous bicarbonate levels at 24-26 mEq/L, whereas the control group received standard care alone. The mid-arm muscle circumference (MAMC), lean body mass (LBM) and estimated glomerular filtration rate (eGFR) were compared between the groups at the end of 6 months. RESULTS: The intervention arm showed a higher LBM {36.8 kg [95% confidence interval (CI) 36.5-37.1] versus 36 [35.7-36.4]; P = 0.002} and MAMC [22.9 cm (95% CI 22.8-23) versus 22.6 (22.5-22.7); P = 0.001] when compared with the control group. The GFR in the intervention arm was higher [32.74 mL/1.73 m2 (95% CI 31.5-33.9) versus 28.2 (27-29.4); P ≤ 0.001]. A rapid decline in GFR was documented in 39 (41.5%) patients in the control arm and 19 (20.2%) patients in the intervention arm (P = 0.001). CONCLUSIONS: Alkali supplementation to increase venous bicarbonate levels to 24-26 mEq/L is associated with preservation of LBM and kidney function in patients with CKD stages 3 and 4.

Manifest interstitial and vascular pathology in anti-neutrophil cytoplasmic antibody-associated renal disease.

Rapidly progressive renal failure in anti-neutrophil cytoplasmic antibody (ANCA)- associated renal disease customarily implies crescentic glomerulonephritis with approximately 50% of the glomeruli will have crescents. The tubulointerstitial inflammation is often proportionate to the glomerular inflammation and may have granulomatous pattern adjacent to the glomeruli or an inflamed vessel. A 77-year-old male with rapidly progressive renal failure was myeloperoxidase-ANCA positive, and renal histopathology revealed thrombotic microangio-pathy, significant interstitial inflammation, interstitial granulomas, and arteritis. Pathology is unique for the paucity of the classical crescents and the myriad of extraglomerular features. His renal function improved and stabilized after induction with cyclophosphamide and maintenance with azathioprine.

Functional Status in Hemodialysis - A Comparative Study with FIM, ADLQ and 7D5L Instruments.

There is only limited information on the functional status (FS) of patients receiving hemodialysis (HD) from developing countries where the etiology of chronic kidney disease (CKD) and demographic profile are different. The study aims to assess the FS in patients with CKD using three validated generic instruments. A total of 116 adult patients on HD with a dialysis vintage >3 months were enrolled. FS was assessed using three generic tools - Functional Independence Measure (FIM) (scores 18-126), Seven domains and five levels (7D5L) (scores 0-28), and Activities of Daily Living Questionnaire (ADLQ) (scores 0-6) scales. A higher FIM and ADLQ scores and lower 7D5L score indicated good FS. The mean age of patients was 41.28 ± 15.44 years. About 77.6% were males and 80.2% were unemployed. About 67.2% were receiving twice-weekly HD, and 28.4% were receiving thrice-weekly dialysis. The mean scores were 119.05 ± 11.42 with FIM, 6.44 ± 4.26 with 7D5L, and 5.51 ± 1.19 with ADL instruments. More than 80% showed full functional independence with ADLQ instrument. With FIM, the overall scores showed a tendency for functional independence, but the subdomains involving locomotion/mobility were impaired to a greater extent. The proportion of patients with full independence was less with 7D5L. We observed an inferior FS in individuals <40 years. Patients on HD were functionally independent as assessed by FIM and ADLQ instruments. However, 7D5L appeared to be better in identifying mild to moderate limitations in daily activities. The domains involving motor tasks seem to be affected to a greater extent. The current scales for assessing FS do not incorporate a time-dependent component.

Severe acute kidney injury owing to rhabdomyolysis and intravascular haemolysis in an 11-year-old child with G6PD deficiency.

Glucose-6-phosphate dehydrogenase (G6PD) deficiency leading to acute intravascular haemolysis and acute kidney injury (AKI) is a known clinical presentation. However, there is a paucity of information regarding the occurrence of rhabdomyolysis and myoglobinuria in G6PD-deficient individuals, especially children. An 11-year-old south Indian Tamil girl presented with severe anaemia and anuric AKI following a short febrile illness. Investigations demonstrated evidence of intravascular haemolysis and rhabdomyolysis, and on histopathology myoglobin deposits (casts) were detected in the renal tubules. She was successfully managed with repeated sessions of haemodialysis and blood transfusions. Follow-up estimation of G6PD levels after 3 months confirmed severe G6PD deficiency (0.003 nkat/g haemoglobin). Although there are anecdotal reports of myoglobinuria in G6PD-deficient individuals, the occurrence of severe anuric AKI in this clinical setting has not been reported. It can be speculated that myoglobinuria (in addition to haemoglobinuria) can contribute towards jeopardising renal function in G6PD deficiency-related acute haemolytic crisis.

Dietary and Fluid Regime Adherence in Chronic Kidney Disease Patients.

Introduction: Patients with Chronic Kidney Disease (CKD) needs to modify their lifestyle chiefly focusing on diet and fluid intake as the prognosis of these patients largely depends on adherence to the recommended nutritional regime. Non adherence to the suggested diet and fluids regime leads to rapid worsening of the condition. Methods: Cross sectional survey was conducted to determine the level of adherence to the dietary and fluids restriction among CKD patients. Inclusion criteria's was, age between 18- 65 years, patients with CKD for at least 6 months and received dietary counseling. Consecutive sampling technique was used to select 100 patients. Data was collected with self-reported Dialysis Diet and Fluid non adherence Questionnaire (DDFQ). Results: Majority (73%) of the subjects was males, 64% belongs to 40-60 years age, majority of them were unemployed. Mean Body Mass Index (BMI) was 20.52 kg/m2, the mean duration of the treatment is 2.15 years and mean fluid intake was 2153ml ml/day and inter-dialytic weight gain was 1.48 kg. Regarding adherence, 20% of them had mild deviation and 69% of them had moderate deviation from dietary restrictions and similarly 69 % of the participants had moderate deviation, and 22% of them had mild deviation from fluid restriction guidelines. Low level of adherence to fluid and diet restrictions was noted in illiterate patients which was significant P<0.05. Conclusion: In spite of the dietary counseling, considerable proportions of the patients were non adherent to the diet and fluid restrictions which necessitate regular counseling to patient and family members.

Acenocoumarol as an alternative anticoagulant in a patient with warfarin-related nephropathy.

ADVERSE EVENT: Warfarin-related nephropathy. DRUG IMPLICATED: Warfarin. THE PATIENT: A 31-year-old female, managed with warfarin for rheumatic heart disease with atrial fibrillation. EVIDENCE THAT LINKS THE DRUG TO THE EVENT: There were no alternative causes of nephropathy that could have caused the adverse event in this patient. MANAGEMENT: Shifting the drug from warfarin to acenocoumarol. MECHANISM: Difference in renal elimination between warfarin and acenocoumarol. IMPLICATION FOR THERAPY: Clinicians should be aware of this rare adverse effect of warfarin, and acenocoumarol can be considered as an alternative therapy for this condition. HYPOTHESES TO BE TESTED: Further prospectively designed studies are needed to consider acenocoumarol as an alternative therapy in warfarin-related nephropathy.

Subcutaneous phaeohyphomycosis in kidney transplant recipients: A series of seven cases.

BACKGROUND: Superficial and deep fungal infections are more frequent in transplant recipients primarily because of the failure of cell-mediated immunity and lesser amount of antigen-presenting Langerhans cells in their epidermis. Here, we report seven cases of post-renal transplant subcutaneous phaeohyphomycosis, all of which manifested within 1 year after transplantation and were unresponsive to prolonged courses of itraconazole. This is the first case series, to our knowledge, of phaeohyphomycosis in transplant recipients in India. METHOD: We performed a retrospective review of cases of phaeohyphomycosis among kidney transplant recipients for type of transplant, immunosuppression, histopathology, and treatment, with prospective follow-up of healed lesion. RESULTS: An overall incidence of 8.3% was noted, with a median duration of approximately 6 months post transplant to the onset of skin lesion. None of the lesions responded to itraconazole alone and 6/7 lesions were surgically excised. Histopathology showed various lesions and culture could isolate Neocytalidium and Exophiala jeanselmi in two cases. CONCLUSION: Dematiaceous fungi are increasingly implicated in cutaneous lesions in transplant recipients. Histopathology and surgical excision are the appropriate tools for diagnosis and treatment, respectively.