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  ~ Table of Contents - Current issue
January-February 2021
Volume 31 | Issue 1
Page Nos. 1-78

Online since Tuesday, February 9, 2021

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Enamel renal syndrome: A systematic review p. 1
Maria Luiza Morais Farias, Gabriela Oliveira Ornela, Rodrigo Soares de Andrade, Daniella Reis B. Martelli, Verônica Oliveira Dias, Hercílio Martelli Júnior
The enamel renal syndrome (ERS) is a rare autosomal recessive disease that is associated with mutations in the FAM20A gene. The syndrome is characterized by impaired amelogenesis of the hypoplastic type and nephrocalcinosis, presenting with presence of thin or absence of enamel, late dental eruption, intrapulpal calcifications, bilateral nephrocalcinosis, and normal plasma calcium level. The objective is to characterize ERS by systematically literature reviewing, highlighting the main findings of the syndrome to increase knowledge about this condition in the health professionals. The study is a systematic review of the scientific literature, whose research was developed in the PubMed database in March 2018. A total of 69 articles were found. Two authors analyzed their abstracts and selected, according to the language and main subject, 30 articles to write this study. A total of 69 patients were cited in the studies and their data were analysed. There was gender equivalence and the ages ranged from 1 to 64 years old. There is a clear hereditary relation of the syndrome, since there was consanguinity in 18 cases, indicating a percentage of 26.08% and family history in 30 cases (43.47%). Laboratory changes vary greatly from patient to patient and may even remain unchanged. The relationship between the syndrome and the mutation in the FAM20A gene can be proven from the data, since all patients with ERS screened by the mutation were positive. With the advancement of the ERS studies, some associations with the syndrome are suspected, such as the presence of gingival fibromatosis, hearing loss, and hypertrichosis. Thus, it is noticed that the syndrome does not show a predilection for gender or age and there is a strong hereditary character, marked by the consanguinity and family history of the patients. The association with the FAM20A gene is reinforced, since the mutation was identified in all patients analyzed.
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Effect of chronotherapy of antihypertensives in chronic kidney disease: A randomized control trial Highly accessed article p. 9
Vaibhav Tiwari, Arpita Ray Chaudhary, Sanjay Dasgupta, Smita Divyaveer, Raju Kumar Sahu, Atanu Pal, Rajib Mondal, Kapiljit Chakravarty, Asit Kumar Mandal, Dipankar Sircar, Rajendra Pandey
Introduction: There is a higher prevalence of non-dipping pattern in hypertensive chronic kidney disease (CKD) patients. Nocturnal hypertension has been shown to predict cardiovascular mortality and morbidity and is often superior to daytime blood pressure. We studied the effect of shifting or adding antihypertensive to night time on blood pressure profile of CKD III-IV patients. Methods: In this single-center, prospective, randomized controlled trial, eligible participants were adults from eastern India aged 18–65 years with CKD stages 3 and 4, with a non-dipping pattern on ambulatory blood pressure monitor (ABPM). The intervention group received all the antihypertensives in the night time whereas the standard care group continued to take the medication in the morning. Both groups were followed up for 1 year. The primary outcome was the number of patients changed from non-dippers to dippers in the standard care group and intervention group. Secondary outcomes included a change in estimated glomerular filtration rate (eGFR) and change in the cardiac structure. Results: 39 patients in the intervention group and 36 patients in the standard care group were analyzed. 10 patients (26%) reverted to dipping pattern in the intervention group as compared to none in the standard care group. Mean changes in eGFR were −2.55 and −0.18 mL/min/1.73 m2 in the standard care and intervention group at the end of the study, respectively. Between-group difference in eGFR was significant at 1 year (5.22 [95% CI, 4.3–6.1] ml/min/1.73 m2); (P = 0.03). The cardiac structure showed no significant changes in either group. Conclusions: Bedtime administration of antihypertensives reverted non-dippers to dippers and slowed the decline in eGFR in CKD stages 3 and 4 compared to morning administration of antihypertensives.
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The Utility of Assessing CD68+ Glomerular Macrophages in Assessing Endocapillary Hypercellularity in IgA Nephropathy p. 16
Neha Agrawal, Swarnalata Gowrishankar
Introduction: IgA nephropathy (IgAN) is the most common form of glomerulonephritis across the world. Oxford classification defines criteria and effects of endocapillary hypercellularity on E score but the reproducibility of the same is debatable. Hence, there is a need for an objective marker that could establish a gold standard in assessing endocapillary hypercellularity. Methods: Forty biopsies of proven IgAN were taken and grouped into two groups based on the presence or absence of endocapillary hypercellularity (n = 20 each). These biopsies were then stained by CD68 immune stain and the glomerular macrophages were quantified. Mean serum creatinine, presence of hypertension, degree of proteinuria and haematuria at the time of biopsy were also recorded and the correlation between these parameters and endocapillary hypercellularity was also studied. Results: Mean glomerular CD 68+ cell count was significantly higher in glomeruli showing endocapillary hypercellularity. Utilising the objective cutoff values of 0.6 CD68+ per glomerulus, more than 8 glomerular CD68+ cells in the entire biopsy and/or around 4 CD68+ cells in the most inflamed glomerulus, endocapillary hypercellularity can be predicted with a sensitivity of 70–80% and specificity of 70%. After regrouping the biopsies based on the cutoff values obtained from the receiver operating curve analysis the mean urine RBC count per high power field showed a significant correlation with endocapillary hypercellularity. Conclusion: Glomerular CD68+ macrophage count seems to be a promising approach in assessing endocapillary hypercellularity. Further studies with emphasis on correlation with the clinical outcome are needed to validate its utility as an objective tool.
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Etiological Spectrum and Histopathological Diagnosis of Rhabdomyolysis Associated Myoglobin Cast Nephropathy in South India p. 22
KS Jansi Prema, Anila Abraham Kurien
Background and Aims: Rhabdomyolysis occurs due to injury to skeletal muscle fibers and the release of muscle constituents into the circulation. Myoglobin cast nephropathy leading to acute kidney injury is one of the most severe complications of rhabdomyolysis. This is a retrospective study which aims to analyse the clinicopathological features of myoglobin cast nephropathy. Methods: A total of 57 cases of myoglobin cast nephropathy were identified after performing immunohistochemical staining for myoglobin on all renal biopsies with pigment casts. The clinical, laboratory data, histopathological findings and clinical outcome of these cases were evaluated. Results: The mean patient age was 34.47 years (range 17-77) and the male to female ratio was 6.1:1. All patients presented with acute kidney injury with mean serum creatinine of 8.4 mg/dl (range: 1.7 to 20.8 mg/dl). Rhabdomyolysis was clinically suspected only in 31 patients. Along with myoglobin casts, acute tubular injury was present in all the biopsies. The most frequent conditions associated with myoglobin cast nephropathy in our study were snake envenomation and unaccustomed physical activities. A few activities that caused rhabdomyolysis in our patients were unique to India. Conclusion: Clinicians should be aware of the wide spectrum of causes for rhabdomyolysis. The classical clinical and laboratory findings of rhabdomyolysis may not be present in many of the patients. The pathologist must have a high index of suspicion, and immunohistochemical stain should be used to confirm the diagnosis.
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Is twice-weekly maintenance hemodialysis justified? p. 27
Satish Mendonca, Shweta Bhardwaj, S Sreenivasan, Devika Gupta
Background: The benefits of twice-weekly dialysis at initiation are significant with respect to access longevity, preservation of residual renal function, economic factors, and patient quality of life. It is widely practiced in developing countries due to resource and financial constraints. We present a 3-year follow-up of patients on twice-weekly dialysis and their outcomes. Material and Methods: This was a 3-year observational follow-up study of patients initiated on twice-weekly hemodialysis. Adequacy and basic cost-effective hematological and biochemical parameters were studied monthly. In case of complications, the patient was shifted to thrice-weekly hemodialysis. Results: 88 incident hemodialysis patients were followed up. Total sessions of hemodialysis (HD) studied were 16,406. The mean hemoglobin level was 9.53 g/dl with hyperphosphatemia in 74.88% patients. The mean residual renal function (RRF) at initiation was 5.71 +/- 3.70 ml/min. The mean interdialytic weight gain was 1.91 +/- 1.26 kg with a mean ultrafiltration of 2600 ± 410 ml. The spKt/V and eKt/V were adequate in 68.54% and 48.34% patients; however, the standard Kt/V of 2 was achieved in only 10.51% patients. Emergency HD was done in 41 sessions (0.24%). There were 24 deaths (27.27%) during this period with the mean time to mortality being 503.12 +/- 296.62 days. Conclusion: Initiation at twice-weekly schedules for patients on maintenance hemodialysis is a viable option with increments in case of requirement, more so in patients with good urine output and residual renal function. The biochemical and hematological parameters were stable and within KDOQI guidelines and do not worsen with time.
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Immunoadsorption column reuse p. 33
Vaibhav Tiwari, Anurag Gupta, Smita Divyaveer, Vinant Bhargava, Manish Malik, Ashwani Gupta, Anil K Bhalla, DS Rana
Introduction: Selective immunoadsorption (IA) is a technique to remove preformed Anti-ABO antibodies in ABO-incompatible renal transplants (ABOiRT). Since the cost of a single IA column is high and single use rarely achieves the target anti-ABO titers, its use is not widely spread. We studied the safety and efficacy of the reuse of IA columns in ABOiRT. Methods: Single-center, retrospective analysis of all patients who underwent ABOiRT with IA column reuse from January 2016 to July 2018. The column was reused after sterilization with ethylene oxide and flushed with normal saline before use. Target titers (IgG) were 1:4 preoperatively. Baseline IgG titers, plasma volume processed in each session, postoperative titer rebound were recorded. The primary outcome was IgG titer reduction after each use and adverse reaction during the IA column reuse. Patients were followed up until 1 year. Results: 16 patients underwent ABOiRT using IA columns. Baseline IgG titer ranged from 1:32 to 1:512. Reuse of IA column was done 23 times and underwent 2nd reuse for 9 times. The average plasma volume treated was 22 L. Efficacy of the IA column in log titer reduction of anti-ABO titer was 4 logs after the first use, 3 logs after 1st reuse, and 1.5 logs after 2nd reuse. 12 (75%) patients successfully reached the target IgG titer of ≤1:4 solely with column reuse. One patient received a single session of plasma exchange before transplantation. Postoperatively, one patient received one session of plasma exchange due to a rebound in anti-ABO antibodies. No serious side effects were noted during the reuse. Conclusion: IA column reuse up to two times showed efficacy in the successful reduction of antibody titers. Column reuse was not associated with any significant side effects.
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Body fat in children with chronic kidney disease - A comparative study of bio-impedance analysis with dual energy X-ray absorptiometry p. 39
Arpana Iyengar, Rebecca Kuriyan, Anura V Kurpad, Anil Vasudevan
Introduction: Nutritional impairment in patients with chronic kidney disease (CKD) is due to decreased body stores of both protein and fat. We need a tool that can be used in clinics to determine and monitor fat composition with a special focus on normalizing fat measurements to height in these children. Bio-impedance analysis (BIA), a portable and simple tool, has been used to estimate body fat in children with CKD but needs validation against the reference tool dual energy X-ray absorptiometry (DXA). The purpose of the cross-sectional study was to estimate the prevalence of low body fat in children with stages 2-5 CKD (non-dialysis) and CKD 5D (dialysis), and to compare fat measures from two different methods namely BIA and DXA. Method: Children in stages 2–5 CKD (n = 19) and in CKD 5D (n = 14) were recruited for assessment of fat mass (FM, Kg) by BIA and DXA, from which percent body fat (BF %) and fat mass index (FMI, Kg/M2) were obtained. Low body fat was defined as <5th age and gender centile for BF% or FMI by DXA and BF% by BIA. Results: Low body fat was detected equally using BF% and FMI in 18% of children by DXA while only 12% were detected using BF% by BIA. In children with CKD2–5, a good degree of reliability was found with FMI measurements (ICC 0.76 CI [0.48,0.9]) and poor reliability in children with CKD 5D (ICC 0.58 CI [0.1,0.84]). BF% had poor to fair reliability in the children with CKD 2-5 and CKD 5D (ICC 0.64 [0.28,0.84] and 0.53 [0.02,0.82]), respectively. Comparing BF% and FMI obtained by BIA and DEXA, BIA overestimated BF% by 3.5% in comparison to DXA. Conclusion: In children with CKD, body fat is preserved in the majority. Among the two measures of fat, BF% estimated by BIA did not compare well with DXA while FMI measure was comparable with a lower bias. However, due to lack of reference values in Indian children for FMI obtained by BIA, BIA cannot be used to measure fat in this population.
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Does hemodialysis need to be initiated to improve platelet function in CKD G5 patients? A pilot prospective, observational cohort study p. 43
Vinod B Murakonda, Anjali Mohapatra, Tulasi Geevar, Ramya Vijayan, Shailesh Kakde, Shibu Jacob, Suceena Alexander, Vinoi G David, Sukesh C Nair, Santosh Varughese, Anna T Valson
Introduction: We previously showed that patients with chronic kidney disease (CKD) Stage G4-5 have normal bleeding times. This made us question whether hemodialysis (HD) initiation was really necessary solely to improve platelet function. Methods: In this prospective observational study, two 5 ml citrated blood samples and one 2 ml EDTA blood sample were collected from incident HD patients fulfilling inclusion criteria prior to HD initiation (baseline sample) and after three sessions of short duration, low flow, counter-current HD. In each instance, one sample was used to perform Collagen adenosine diphosphate closure time (CADPCT) using the Platelet function analyzer (PFA 200, normal range 68-142 seconds) and the second for light transmission aggregometry (LTA) with ADP as agonist (normal ≥50%). Results: This study included 20 patients between October 2017 and February 2019. Overall, and in the subgroup with normal baseline CADPCT or LTA, there was no statistically significant improvement after HD. However, of the 30% of patients who had an abnormal baseline CADPCT, 50% attained a normal value after three HD sessions, and the overall reduction in CADPCT in this group was statistically significant (P = 0.02). Of those with a baseline normal CADPCT, 21% developed abnormal prolongation post HD. Conclusion: HD for the sole purpose of improving platelet function is only of benefit in the subgroup of patients with an abnormal CADPCT at baseline, with close to 50% normalizing their platelet function after three sessions of low flow, short duration, counter-current HD.
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Immune Complex Associated Glomerulonephritis in a Patient with Prefibrotic Primary Myelofibrosis: A Case Report p. 50
Mahendra Atlani, Tanya Sharma, Deepti Joshi, Aman Kumar
A case of prefibrotic myelofibrosis with immune complex-mediated glomerulonephritis is presented. A 45-year-old female, with history of right subclavian and axillary vein thrombosis, presented with abdominal distension, facial puffiness, and pedal edema. Evaluation revealed deranged renal functions with nephrotic range proteinuria and acute kidney injury. JAK2 mutation evaluated in view of portal vein thrombosis and splenomegaly was positive. Renal biopsy revealed mesangial proliferative glomerulonephritis with full house immune complex deposition on direct immunofluorescence (DIF). The patient had no signs or symptoms of systemic lupus erythematosus and serological markers for autoimmune or collagen vascular disease were negative. Renal involvement in myeloproliferative neoplasms (MPNs) is uncommon and histological patterns of DIF negative mesangial proliferative glomerulonephritis, focal segmental glomerulosclerosis, and immunoglobulin A nephropathy have been reported.
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Fracture Embolism of In-situ Guidewire–Bewildering Aetiology of Protracted Pyrexia in a Patient with End Stage Renal Disease! p. 54
Vivek Sood, Vivek Kumar, Manish Rathi, Navin Pattanashetty, Raja Ramachandran
Guidewire embolism during venous access for haemodialysis is not uncommon yet potentially avoidable iatrogenic complication. Unrecognised, long-standing in-situ guidewire may predispose to thrombosis and become a nidus for infection. This entity should always be borne in mind and considered as one of the differentials of unexplained pyrexia in patient on maintenance haemodialysis. In this context, we report a patient on maintenance dialysis who presented with fever of 6 weeks duration with no localising history and failed response to empirical antibiotics. On imaging, he was detected to have in-situ guidewire with fracture embolism into inferior vena cava and right external iliac vein and soon patient became afebrile following guidewire retrieval using gooseneck snare device, thereby retrospectively confirming causality.
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A rare case of APRT deficiency with end-stage renal failure and successful renal transplant p. 57
Manish Sharma, Swarnalata Gowrishankar, Tarun K Jeloka
Renal calculus disease is a common cause of renal injury. However, crystal nephropathy (uric acid, oxalate, and dihydroxyadenine) can present as chronic kidney disease without any evidence of renal stones. If left undiagnosed, there is a potential chance of recurrence in the allograft leading to graft failure after transplantation. Pretransplant identification and management can avoid such complications. Here, we describe a case of APRT deficiency leading to crystal nephropathy and end-stage renal failure in a patient who underwent a successful kidney transplant.
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A Case of Joubert Syndrome with Chronic Kidney Disease p. 61
MP Shamsudheen, Uttara Das, Gangadhar Taduri, Swarnalatha Guditi, Raja Karthik, Rajani Thakur
Joubert syndrome is a genetically heterogeneous disorder that belongs to the group of cerebello-oculo-renal syndromes. It is characterised by neurodevelopmental abnormalities and complex midbrain-hindbrain malformation, visible on brain imaging as a molar tooth sign. It is classified as a ciliopathy and has variable renal involvement. Herein, we report a case of a 9-year-old boy with developmental delay, presented as chronic kidney disease and evaluation showed features of Joubert syndrome. Recognition of specific clinical and radiological findings will help in early diagnosis and appropriate care.
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A Case of Hearing Impairment with Renal Dysfunction p. 64
GK Prakash, Sagar C Kulkarni, SR Rashmi, V Mahesha, S Vishwanath, HS Ballal
Hearing impairment in a patient with renal failure is an important clue towards etiologic diagnosis of kidney disease. Variety of hereditary diseases, developmental defects, and toxins involve these two organs. However, additional retinopathy is seen in quite a few diseases which include Alport's syndrome and Muckle–Wells syndrome (MWS). We are reporting a case of middle-aged woman with childhood-onset of hearing impairment who presented with renal failure and was diagnosed to have renal amyloidosis on kidney biopsy but without any light chain restriction. During evaluation for live donor kidney transplant, her brother was also found to have hearing impairment and retinopathy however with normal renal function and urinalysis. Genetic testing of both of them was done for panel of mutations related to hereditary amyloidosis which revealed NLRP3 mutation in both. This mutation is characteristic of MWS which can lead to secondary amyloidosis and renal failure.
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Recurrent seizures in an adolescent female-A daunting puzzle p. 67
Mohanasundaram Subashri, ND Srinivasa Prasad, Edwin Fernando, Yashwanth T Raj
Acute porphyrias are metabolic disorders resulting from deficiency of a specific enzyme involved in heme biosynthetic pathway. These deficiencies also affect normal renal physiology, as kidneys are also involved in heme synthesis. Sometimes, this could even lead to end stage renal disease. Acute Intermittent Porphyria, an autosomal dominant disorder arising from half-normal activity of hydroxymethylbilane synthase, is characterized by occurrence of vague neurovisceral attacks (abdominal pain, nausea, vomiting, constipation and neuropsychiatric symptoms), with urinary excretion of porphyrin precursors, such as 5-Amino-levulinic acid (ALA) and Porphobilinogen (PBG). Acute attacks are triggered by dehydration, diarrhea, steroids, low calorie diets. Treatment includes avoidance of precipitating factors, adequate hydration, high carbohydrate diet and heme replacement. Here, we present an adolescent female who had presented with recurrent abdominal pain, dyselectrolyemia with associated seizures, was diagnosed with Acute Intermittent Porphyria and recovered well with symptomatic management.
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The LE Cell—A Forgotten Entity p. 71
Subrahmanian Sathiavageesan, Suganya Rathnam
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A rare case of kayexalate and CMV colitis in a patient of sarcoidosis and chronic kidney disease p. 73
Nalini Bansal, Kuldeep Kushwaha, Jitendra Rajput, Ajit Singh Narula
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Hyponatremic Hypertensive Syndrome Complicating Unilateral Renal Artery Stenosis: A Rare Manifestation in Childhood p. 75
Annesha Chakraborti, Mukta Mantan, Shasanka S Panda
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Metabolic acidosis and hyperlactatemia requiring continuous venovenous hemofiltration after intentional ingestion of metformin p. 77
Maricel Dela Cruz, Muhammed Ershad, Ahmed Mostafa
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Indian Journal of Nephrology
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