Comparison of Clinico-pathologic features and outcomes of ANCA negative and ANCA positive pauci immune crescentic glomerulonephritis: A single centre study.

Introduction: Pauci-immune crescentic glomerulonephritis (PICN) is an important cause of rapidly progressive renal failure. 10-40% of PICN cases have ANCA (antineutrophil cytoplasmic antibody) negative serology. The present study compared clinico-pathologic features, Brix's renal risk score, Berden's histopathological classes and differences in outcome between ANCAnegative vs ANCA positive PICN patients. Materials and Methods: Sixty-one patients of biopsy-proven PICN were studied. Biochemical findings and ANCA serology were recorded. Renal biopsy slides were reviewed along with direct immunofluorescence. Clinical and histological features were compared between ANCA negative and positive PICN using the Man Whitney U test and Chi-square test. Patients were compared for distribution in Berden's histological classes and Brix's renal risk categories. Patient and renal survival were compared using Kaplan-Meier survival analysis. Results: ANCA negative PICN patients were younger (44.9 ± 16.5 years vs 53.6 ± 15.1 years, P = 0.049). Nasal (0 vs 18%, P = 0.035) and pulmonary involvement (9% vs 38%, P = 0.014) were lower in ANCA negative group. Both ANCA groups had similar renal biochemical profiles, percentage normal glomeruli, 16.3 ± 18.2 vs 21.7 ± 20.4 and percentage glomeruli with crescents, 64.5 ± 28.1 vs 64.3 ± 27.1. Twenty-seven per cent of ANCA negative cases fell in the sclerotic class in Berden's classification vs just 2.5% in ANCA positive group (p = 0.037) without significant difference in Brix's renal risk categories (p = 0.329). Thirteen per cent of ANCA negative patients achieved complete remission on treatment compared to 33% in ANCA positive patients. Patient survival and overall probability of progressing to ESRD were similar in the two groups. Conclusion: ANCA negative PICN cases present at younger ages. Nasal and pulmonary involvement is uncommon in these patients. Patient survival and progression to ESRD are similar in both ANCA groups.

Variation in performing kidney biopsy amongst nephrologists in the Asia-Pacific region: A multinational survey.

AIM: Kidney biopsy (KB) is the gold standard procedure for diagnosing kidney diseases. Globally, nephrologists are trained to perform KB. However, the past few decades have witnessed a transition where interventional radiologists (IRs) are now preferentially performing the procedure. Our survey-based cross-sectional study aimed to investigate the current trends of KB operators in the Asia-Pacific region (APR) in practicing interventional nephrologists. MATERIAL AND METHODS: The Association of Vascular Access and intervenTionAl Renal Physicians (AVATAR) Foundation from India conducted a multinational online survey among interventional nephrologists from the APR to investigate who does KB, if the nephrology training curriculum includes KB, and whether nephrologists have access to ultrasound. RESULTS: Out of 21 countries from the APR that participated in our survey, 10 countries (47.4%) reported that more than 70% of their nephrologists performed KB, whereas in 11 countries (57.6%), KB was most likely done by an IR. The frequency of nephrologists performing KB ranged from 0% in Afghanistan to 100% in countries such as Pakistan, Singapore, and Thailand. Formal training for KB and access to ultrasound was available to nephrologists in 80% of the responding countries. CONCLUSION: Our study shows that despite the availability of training and access to USG, a significant number of nephrologists are not performing KB in the APR. Similar to the trends observed in Western countries, the observed pattern in the APR could be due to lack of time, less incentive, hospital policy, or interest of nephrologists in other aspects of intervention nephrology.

NOTTO Guidelines for Vaccine Induced Thrombotic Thrombocytopenia in Organ Donation and Transplantation.

From the context of organ donation, COVID-19 vaccine-induced thrombotic thrombocytopenia (VITT) is important as there is an ethical dilemma in utilizing versus discarding organs from potential donors succumbing to VITT. This consensus statement is an attempt by the National Organ and Tissue Transplant Organization (NOTTO) apex technical committees India to formulate the guidelines for deceased organ donation and transplantation in relation to VITT to help in appropriate decision making. VITT is a rare entity, but a meticulous approach should be taken by the Organ Procurement Organization's (OPO) team in screening such cases. All such cases must be strictly notified to the national authorities like NOTTO, as a resource for data collection and ensuring compliance withprotocols in the management of adverse events following immunization. Organs from any patient who developed thrombotic events up to 4 weeks after adenoviral vector-based vaccination should be linked to VITT and investigated appropriately. The viability of the organs must be thoroughly checked by the OPO, and the final decision in relation to organ use should be decided by the expert committee of the OPO team consisting of a virologist, a hematologist, and atreating team. Considering the organ shortage, in case of suspected/confirmed VITT, both clinicians and patients should consider the risk-benefit equationbased on available experience, and an appropriate written informed consent of potential recipients and family members should be obtained before transplantation of organs from suspected or proven VITT donors.

Heparin-Induced Thrombocytopenia in Hemodialysis Patients- The First Study from India.

Heparin-induced thrombocytopenia (HIT), a rare complication of heparin therapy, presents with thrombocytopenia. It leads to paradoxical thromboembolism and has high mortality if untreated. It is less recognized, especially in hemodialysis (HD) patients who are frequently exposed to heparin during dialysis because patients with renal failure may have many other causes of thrombocytopenia. We describe the clinical presentation, diagnosis, and treatment of five cases of confirmed HIT in hemodialysis (HD) patients at our center. The initial suspicion was made based on a high 4T score and positive gel card test followed by confirmation using the functional assay with heparin-induced platelet aggregation. These patients were treated according to the recent American Society of Hematology guidelines 2018 for HIT.

Nocardiosis in Renal Transplantation: Case Series from India.

Nocardiosis is a rare opportunistic infection seen in kidney transplant patients and is caused by aerobic actinomycete. Disease manifestations can vary from a localized infection to multisystem organ failure. In this retrospective case series, we present 16 cases of Nocardiosis. The median age of the patients was 44 years. The median time from transplant to nocardiosis was 21 months. Acute rejection episodes and CMV infection within 6 months of nocardiosis were found in 12.5% and 25%, respectively. The most common organ involvement was the lungs (75%), followed by the brain (12.5%). Only one patient showed cutaneous involvement (6.25%). Mean creatinine at presentation was 0.7 mg/dL (mean eGFR: 92 ± 27 mL/min/1.73 m2). Trimethoprim/sulfamethoxazole resistance was found in 25% of patients. Five patients (31.25%) succumbed to the infection. Nocardiosis has a very low incidence but a high rate of mortality.

Atypical HUS Triggered by COVID-19: A Case Report.

We hereby present a case of an atypical hemolytic uremic syndrome (aHUS) precipitated by coronavirus disease 2019 (COVID-19). A 26-year-old male was diagnosed with COVID-19 and acute kidney injury. His kidney biopsy was suggestive of thrombotic microangiopathy. Five sessions of plasmapheresis were done but were discontinued in view of nonrecovery of kidney function. He was then referred for a kidney transplant. On genetic analysis, he was found to have mutations in the complement system (CFHR1 and CFHR3), which suggested this was a case of aHUS precipitated by COVID-19. In view of the high risk of recurrence of the primary disease in live-related kidney donor transplantation, he was advised for simultaneous liver and kidney transplants.

IgA Nephropathy with Wilson's Disease: A Case Report and Literature Review.

The most common glomerulonephritis seen in the world is immunoglobulin A nephropathy (IgAN). It can be primary or secondary associated with various conditions like Chronic Liver disease, Crohn's disease, neoplasms, etc. However, IgAN secondary to Wilson's disease is very rare. A 9 year old boy presented with gross hematuria and proteinuria. He had a history of recurrent jaundice in the past. Ultrasonography (USG) whole abdomen showed altered echotexture of the liver with normal-sized kidneys. An extended workup for liver disease was done, and the diagnosis of Wilson's disease was confirmed with decreased serum ceruloplasmin levels, increased urinary copper, and the Kayser-Fleischer ring. Urine routine microscopy showed numerous red blood cells, few red blood cell casts, and mild proteinuria. Renal biopsy showed IgAN. The patient was started on D-penicillamine. On follow-up at 3 months, he showed complete resolution of proteinuria and hematuria. Thus, we suggest that Wilson's disease should be considered as one of the causes of secondary IgAN in pediatric patients with hematuria, proteinuria with liver dysfunction.

COVID-19 Infection in Renal Transplant Patients: Early Report From India.

INTRODUCTION: COVID -19 has gripped the whole world and patients with comorbidities especially kidney ailments are at higher risk of developing severe disease. Among kidney disease, transplant patients are the most vulnerable group. Information on coronavirus disease 2019 (COVID-19) in kidney transplant patients is very limited. METHODS: An observational study was conducted on 20 kidney transplant patients who tested positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) by polymerase chain reaction from April to June 2020. RESULTS: The majority of cases were males (85%). The median age of the patients was 50 years (interquartile range [IQR] 40.75-60.75). Diabetes and hypertension were present in 55% and 95% of patients, respectively. Coronary artery disease was present in three patients (15%). The median time from transplant to COVID-19 testing was 54 months (IQR 36-105). Chronic allograft nephropathy was found in 35% of patients. The mean baseline creatinine was 1.71 mg/dL. The most common symptom was fever (80%). Acute Kidney Injury was seen in 60% of patients with a mean creatinine of 2.60 mg/dL. Based on severity, 50% of patients had mild disease, 25% moderate disease, and the remaining 25% had severe disease. All 20 patients were on oral steroids, calcineurin inhibitors (18 on tacrolimus and two on cyclosporine), and antimetabolite (19 on mycophenolate mofetil and one on azathioprine). Antimetabolite agents were stopped in all patients and tacrolimus was stopped in severe cases (25%). Hydroxychloroquine was given in 15 patients (75%). Fifteen patients (75%) recovered while five (25%) died. CONCLUSION: Kidney transplant recipients infected with COVID-19 have high mortality.

INASL-ISN Joint Position Statements on Management of Patients with Simultaneous Liver and Kidney Disease.

Renal dysfunction is very common among patients with chronic liver disease, and concomitant liver disease can occur among patients with chronic kidney disease. The spectrum of clinical presentation and underlying etiology is wide when concomitant kidney and liver disease occur in the same patient. Management of these patients with dual onslaught is challenging and requires a team approach of hepatologists and nephrologists. No recent guidelines exist on algorithmic approach toward diagnosis and management of these challenging patients. The Indian National Association for Study of Liver (INASL) in association with Indian Society of Nephrology (ISN) endeavored to develop joint guidelines on diagnosis and management of patients who have simultaneous liver and kidney disease. For generating these guidelines, an INASL-ISN Taskforce was constituted, which had members from both the societies. The taskforce first identified contentious issues on various aspects of simultaneous liver and kidney diseases, which were allotted to individual members of the taskforce who reviewed them in detail. A round-table meeting of the Taskforce was held on 20-21 October 2018 at New Delhi to discuss, debate, and finalize the consensus statements. The evidence and recommendations in these guidelines have been graded according to the Grading of Recommendations Assessment Development and Evaluation (GRADE) system with minor modifications. The strength of recommendations (strong and weak) thus reflects the quality (grade) of underlying evidence (I, II, III). We present here the INASL-ISN Joint Position Statements on Management of Patients with Simultaneous Liver and Kidney Disease.

Clinical Remission of Immunoglobulin A Nephropathy after Bariatric Surgery in a Young Morbidly Obese Patient.

A 23-year-old girl with morbid obesity, diabetes mellitus, hypertension, obstructive sleep apnea, and immunoglobulin A nephropathy (IgAN) attended a bariatric clinic after multiple failed attempts at weight loss. In the past, she was diagnosed with IgAN with nephrotic syndrome and raised blood pressure at the age of 11 years. Apart from optimization of blood pressure with angiotensin receptor blocker, she required steroid to maintain her remission in initial four years which was later switched to mycophenolate mofetil (MMF). She was diagnosed with diabetes at the age of 13 years; her blood sugars remained poorly controlled despite therapy with oral hypoglycemic agents and insulin. She underwent sleeve gastrectomy with no post-operative complications. During the follow-up, she showed a steady reduction in her weight, along with maintaining normal blood sugars and pressure without medications. At 18 months of follow-up, IgAN remained in remission after stopping MMF at four months after the surgery. Obesity is considered an important cofactor in the progression of IgAN. This case highlights the importance of weight reduction to halt the progression of the disease.

Tacrolimus-induced Epidermoid Cysts in the Renal Transplant Patient.

Epidermal cysts are common benign cystic lesions that occur mostly sporadically. Common sites involved are arms, face, and trunk. The cyst may progress slowly and remain for years. These cysts arise as a result of the plugging of the follicular orifice. Etiology has largely remained unknown although local trauma, ultraviolet rays, and human papilloma virus (HPV) have been implicated in a few cases. Calcineurin inhibitors (CNIs) especially cyclosporine has been discredited for cutaneous side effects such as hirsutism and gingival hyperplasia. Epidermoid cysts have been also associated with patients with solid organ transplant recipients on cyclosporine. Tacrolimus is considered to be free of dermatological side effects. Herein, we report a case of 56-year-old renal allograft recipient on tacrolimus, who develop more than >100 epidermoid cysts over the face, trunk, back, and extremities. The lesions ceased to progress once the tacrolimus was stopped.

Aetiology, practice patterns and burden of end-stage kidney disease in South Asia and South-East Asia: A questionnaire-based survey.

AIM: There is paucity of data on the epidemiology of end-stage kidney disease (ESKD) from South Asia and South-East Asia. The objective of this study was to assess the aetiology, practice patterns and disease burden and growth of ESKD in the region comparing the economies. METHODS: The national nephrology societies of the region; responded to the questionnaire; based on latest registries, acceptable community-based studies and society perceptions. The countries in the region were classified into Group 1 (High|higher-middle-income) and Group 2 (lower|lowermiddle income). Student t-test, Mann-Whitney U test and Fisher's exact test were used for comparison. RESULTS: Fifteen countries provided the data. The average incidence of ESKD was estimated at 226.7 per million population (pmp), (Group 1 vs. Group 2, 305.8 vs. 167.8 pmp) and average prevalence at 940.8 pmp (Group 1 vs. Group 2, 1306 vs. 321 pmp). Group 1 countries had a higher incidence and prevalence of ESKD. Diabetes, hypertension and chronic glomerulonephritis were most common causes. The mean age in Group 2 was lower by a decade (Group 1 vs. Group 2-59.45 vs 47.7 years). CONCLUSION: Haemodialysis was the most common kidney replacement therapy in both groups and conservative management of ESKD was the second commonest available treatment option within Group 2. The disease burden was expected to grow >20% in 50% of Group 1 countries and 78% of Group 2 countries along with the parallel growth in haemodialysis and peritoneal dialysis.

Bilateral Acute Renal Infarction Secondary to Methylene Tetrahydrofolate Reductase A1298C and PAI-1 Mutation.

We present this rare case of hyperhomocysteinemia due to a mutation in methylene-tetrahydrofolate-reductase (MTFHR) combined with plasminogen activator inhibitor deficiency, causing bilateral renal artery thrombosis. This case highlights the importance of genetic screening in individuals with a family history of thrombotic diseases. There seems to be a role of intervention, even in the setting of renal infarction.

What do we know about chronic kidney disease in India: first report of the Indian CKD registry.

BACKGROUND: There are no national data on the magnitude and pattern of chronic kidney disease (CKD) in India. The Indian CKD Registry documents the demographics, etiological spectrum, practice patterns, variations and special characteristics. METHODS: Data was collected for this cross-sectional study in a standardized format according to predetermined criteria. Of the 52,273 adult patients, 35.5%, 27.9%, 25.6% and 11% patients came from South, North, West and East zones respectively. RESULTS: The mean age was 50.1 ± 14.6 years, with M:F ratio of 70:30. Patients from North Zone were younger and those from the East Zone older. Diabetic nephropathy was the commonest cause (31%), followed by CKD of undetermined etiology (16%), chronic glomerulonephritis (14%) and hypertensive nephrosclerosis (13%). About 48% cases presented in Stage V; they were younger than those in Stages III-IV. Diabetic nephropathy patients were older, more likely to present in earlier stages of CKD and had a higher frequency of males; whereas those with CKD of unexplained etiology were younger, had more females and more frequently presented in Stage V. Patients in lower income groups had more advanced CKD at presentation. Patients presenting to public sector hospitals were poorer, younger, and more frequently had CKD of unknown etiology. CONCLUSIONS: This report confirms the emergence of diabetic nephropathy as the pre-eminent cause in India. Patients with CKD of unknown etiology are younger, poorer and more likely to present with advanced CKD. There were some geographic variations.