Diagnostic Dilemma: Cardiorenal Syndrome As an Unusual Presentation of IgG4-Related Disease.

IgG4-related kidney disease (IgG4 RKD) is a rare clinical entity characterized by lymphoplasmacytic infiltration rich in IgG4-positive plasma cells along with fibrosis affecting several organs. Tubulointerstitial nephritis is commonly the predominant finding on kidney biopsy. Our patient was admitted with a provisional diagnosis of cardiorenal syndrome of unknown etiology. The patient was dialysis dependent for around 45 days following which kidney biopsy revealed features of acute tubulointerstitial nephritis (ATIN) with IgG4-positive plasma cells and no glomerular involvement. Positron emission tomography-computed tomography was supportive of findings of sialadenitis along with myocarditis. Our patient responded to treatment with steroids with definitive improvement in both renal and cardiac functions. This case highlights the importance of IgG4 RKD as an important differential in patients with ATIN presenting as a clinical syndrome.

ABO-Incompatible Kidney Transplantation in India: A Single-Center Experience of First Hundred Cases.

Aim: ABO-incompatible (ABOi) kidney transplantation overcomes immunological barrier of blood group incompatibility. There have been very few published experiences of ABOi kidney transplantation from India. We present our single-center experience of the first hundred ABOi kidney transplants. Material and Methods: This is a single-center retrospective study of consecutive first hundred ABOi kidney transplant with at least 6 months of follow-up. Results: During the study period (2011-2020), a total of 121 ABOi kidney transplants were performed. Of these, first hundred patients were analyzed. Median follow-up duration was 33 (10-101) months. Mean recipient and donor age were 41.5 ± 13 and 47.68 ± 11.25 years, respectively. Mean HLA mismatch was 4 ± 1.5. Median baseline anti-blood group antibody titer was 128 (2-1024). Most common recipient blood group was O. Patient and death censored graft survival was 93% and 94%, respectively, at median follow-up of 33 months. Biopsy-proven acute rejection (BPAR) rate was 17% with acute antibody-mediated rejection being 3%. Rate of infection was 37%, most common being urinary tract infection. Conclusion: ABOi kidney transplant patients had acceptable patient and graft survival as well as BPAR rates. With current preconditioning protocol, infection rate was high.

Impact of COVID-19-associated Mucormycosis in Kidney Transplant Recipients: A Multicenter Cohort Study.

BACKGROUND: COVID-19-associated mucormycosis (CAM) is a recently emerging entity. There is a lack of reports of CAM in organ transplant recipients. METHODS: We conducted a multicenter (n = 18) retrospective research in India during November 2020 to July 2021. The purpose of this study was to explore the clinical spectrum, outcome and risk factors for mortality of CAM in kidney transplant recipients (KTRs). RESULTS: The incidence of CAM was 4.4% (61/1382 COVID-19-positive KTRs) with 26.2% mortality. The median age of the cohort was 45 (38-54) y. Twenty (32%) were not hospitalized and 14 (22.9%) were on room air during COVID-19. The proportion of postdischarge CAM was 59.1%, while concurrent CAM was reported in 40.9%. The presentation of CAM was 91.8% rhino-orbital-cerebral mucormycosis and 8.2% pulmonary with 19.6% and 100% mortality, respectively. In the univariable analysis, older age, obesity, difficulty of breathing, high-flow oxygen requirement, and delay in starting therapy were significantly associated with mortality. In the multivariable logistic regression analysis, patients requiring high-flow oxygen therapy [odds ratio (95% confidence interval) = 9.3 (1.6-51); P = 0.01] and obesity [odds ratio (95% confidence interval) = 5.2 (1-28); P = 0.05] was associated with mortality. The median follow-up of the study was 60 (35-60) d. CONCLUSIONS: We describe the largest case series of CAM in KTRs. Morality in pulmonary CAM is extremely high. Severe COVID-19 pose extra risk for the development of CAM and associated mortality. Our report will help in better understanding the conundrum and management of CAM.

Grafalon® vs. Thymoglobulin® as an Induction Agent in Renal Transplantation - A Retrospective Study.

INTRODUCTION: Antihuman thymocyte immunoglobulin, used as an induction agent in renal transplantation, is of two types - thymoglobulin and grafalon (formerly ATG-Fresenius). In this study, we compared outcomes with these two agents. METHODS: This was a single-center retrospective study of patients transplanted from January 2017 to October 2019, who received either grafalon or thymoglobulin induction. Grafalon or thymoglobulin was given at 6 and 3 mg/kg, respectively, followed by standard triple immunosuppression of tacrolimus, MMF, and prednisolone. RESULTS: Median follow up was 22 (3-36) months. Thymoglobulin was given to 255 patients, whereas 78 patients received grafalon. Baseline demographics were similar between the two groups although significantly more patients in the grafalon group received ABO incompatible transplant (15% vs. 4.3%; P = 0.002). Patient survival was similar between the two groups (99% in grafalon vs. 98.8% in thymoglobulin; P = 1.0). Death censored graft survival was also similar (99% in grafalon vs. 100% in thymoglobulin; P = 0.23). Biopsy proven acute rejection (BPAR) was significantly higher in the grafalon group (12.8% vs. 5.1%, P = 0.04). The significance persisted after multivariable regression analysis (P = 0.02). Other outcomes such as infection rate and estimated glomerular filtration rate on last follow up were comparable between the two groups. CONCLUSIONS: Grafalon (6 mg/kg dose) when used as an induction agent was associated with significantly higher rate of BPARs as compared to thymoglobulin (3 mg/kg dose) although with comparable short-term patient and death censored graft survival, graft function, and infection rates.

A retrospective multi-center experience of renal transplants from India during COVID-19 pandemic.

INTRODUCTION: Coronavirus disease 2019 (COVID-19) pandemic led to a sudden drop in renal transplant numbers across India in the initial months of 2020. Although the transplant numbers increased with easing of lockdown, the outcome of these transplants remains unknown. METHODS: This was a retrospective, observational, multi-center study done across eight different transplant centers in India. All the transplants done from January 30, 2020 to December 31, 2020 were included. The primary outcomes studied were patient and death censored graft survival as well as incidence of COVID-19 infection and its outcomes. RESULTS: During the study period a total of 297 kidney transplants were done. After a median follow up of 265 days the patient and death censored graft survival was 95.3% and 97.6%, respectively. Forty-one patients (13.8%) developed COVID-19 post-transplant. Majority (58.5%) were asymptomatic to mildly symptomatic and the case fatality ratio was 14.6%. On multivariable logistic regression analysis older age was associated with higher likelihood of COVID-19 infection (odds ratio 1.038; CI 1.002-1.077). CONCLUSIONS: Patient and graft outcome of kidney transplants done during the COVID-19 pandemic in India was acceptable. The incidence of COVID-19 was 13.8% with a high case fatality ratio.

Microbiological Spectrum and Outcomes of Acute Pyelonephritis in North Indian Population.

A wide range of causative organisms can cause acute pyelonephritis (APN). However, in recent times, these pathogens have increasingly become resistant to most of the antibiotics making treatment difficult. This was a prospective observational single-center study with a aim to study the microbiological spectrum, resistance patterns, and clinical outcome of patients with APN conducted in a private tertiary care hospital in India. All adult patients hospitalized in the department of nephrology at our institute with a diagnosis of APN from February 2016 to May 2017 were included. Patients <18 years of age, kidney-transplant recipients, and pregnant patients were excluded. Demographic details, clinical symptoms, signs, and radiological and laboratory data including urine and blood cultures of all patients were recorded. The details of treatment received and outcomes in hospital and after discharge were noted. Patients were followed up three months post discharge. Decision of antibiotic and duration of antibiotics was documented by treating nephrologists. Quantitative data were presented in terms of means and standard deviation. Student's "t" test was used for comparison of quantitative outcome parameters. P <0.05 is considered statistically significant. SPSS software version 23.0 was used for statistical analysis. A total of 89 patients with a mean age of 50.33 ± 13.9 years, of which 61.8% were males and were studied; 82/89 had complicated pyelonephritis. The most common risk factor for APN was diabetes mellitus in 64 (72%). Most common symptom was fever in 80 (90%). A triad of fever, flank pain, and dysuria was present only in 27 (30.33%). Overall, 15 patients (16.8%) had severe pyelonephritis requiring intensive care unit admission. The most common organism isolated was Escherichia coli in 26/49 (53%), followed by Klebsiella pneumoniae in 12 (24.40%). Twenty-two (58%) isolates were extended-spectrum beta lactamase producers. Six (12.20%) were resistant to carbapenems and two (4%) were pan-resistant. All 89 were treated with intravenous antibiotics. Older patients, those with diabetes, with poor glycemic control, and with emphysematous pyelonephritis and patients in whom ESBL organisms were grown had poor outcome. Piperacillin tazobactam, aminopenicillins, cefoperazone sulbactam, and carbapenems (in severe pyelonephritis) can be considered as the empirical antibiotic of choice.

Post kidney transplant histoplasmosis: An under-recognized diagnosis in India.

Histoplasmosis is an invasive mycosis caused by fungus Histoplasma capsulatum. Clinical features of histoplasmosis are often nonspecific, but patients with disseminated infection may present with severe manifestations posing an increasing threat to patients with various immunocompromised conditions. It is often misdiagnosed as tuberculosis in endemic regions leading to high mortality. There is under-reporting of histoplasmosis in solid organ transplant from India undermining its actual incidence and impact. As a result of the potentially fatal nature of the disease, careful evaluation with tissue diagnosis is recommended. We present a series of five cases of disseminated histoplasmosis in renal transplant recipients from our centre, highlighting its significance as differential diagnosis in this population. To our knowledge, this is the largest case series reported from India in renal transplant patients.

Heparin free dialysis in critically sick children using sustained low efficiency dialysis (SLEDD-f): A new hybrid therapy for dialysis in developing world.

BACKGROUND: In critically sick adults, sustained low efficiency dialysis [SLED] appears to be better tolerated hemodynamically and outcomes seem to be comparable to CRRT. However, there is paucity of data in critically sick children. In children, two recent studies from Taiwan (n = 11) and India (n = 68) showed benefits of SLED in critically sick children. AIMS AND OBJECTIVES: The objective of the study was to look at the feasibility and tolerability of sustained low efficiency daily dialysis-filtration [SLEDD-f] in critically sick pediatric patients. MATERIAL AND METHODS: Design: Retrospective study Inclusion criteria: All pediatric patients who had undergone heparin free SLEDD-f from January 2012 to October 2017. Measurements: Data collected included demographic details, vital signs, PRISM III at admission, ventilator parameters (where applicable), number of inotropes, blood gas and electrolytes before, during, and on conclusion of SLED therapy. Technical information was gathered regarding SLEDD-f prescription and complications. RESULTS: Between 2012-2017, a total of 242 sessions of SLEDD-f were performed on 70 patients, out of which 40 children survived. The median age of patients in years was 12 (range 0.8-17 years), and the median weight was 39 kg (range 8.5-66 kg). The mean PRISM score at admission was 8.77±7.22. SLEDD-f sessions were well tolerated, with marked improvement in fluid status and acidosis. Premature terminations had to be done in 23 (9.5%) of the sessions. There were 21 sessions (8.6%) terminated due to hypotension and 2 sessions (0.8%) terminated due to circuit clotting. Post- SLEDD-f hypocalcemia occurred in 15 sessions (6.2%), post- SLEDD-f hypophosphatemia occurred in 1 session (0.4%), and post- SLEDD-f hypokalemia occurred in 17 sessions (7.0%). CONCLUSIONS: This study is the largest compiled data on pediatric SLEDD-f use in critically ill patients. Our study confirms the feasibility of heparin free SLEDD-f in a larger pediatric population, and even in children weighing <20 kg on inotropic support.

Pediatric ABO-incompatible kidney transplantation: Evolving with the advancing apheresis technology: A single-center experience.

Recent literature has endorsed favorable outcomes following ABOi kidney transplantation in pediatric population. Nevertheless, reluctance to pursue an ABOi still remains pervasive. This could be ascribed to various legitimate reasons, namely less extensive pediatric ABOi data, technical difficulties encountered during PP, cost restraints, and concerns regarding higher rates of antibody-mediated rejection, infectious complications, and post-transplant lymphoproliferative disorder as compared to adults. However, given the similar excellent outcomes of both ABOi and ABOc kidney transplantation, clinicians should consider this option sooner if a compatible donor or swap is not available. Here, we describe the outcomes of three pediatric ABOi performed at our institute in India (from 2014 till now), wherein distinct apheresis modalities had been employed in each desensitization protocol, and our techniques evolved with advancing science in apheresis. This case series includes India's first published pediatric ABO-incompatible transplant (Case 2) and the youngest child to undergo ABO-incompatible renal transplant in SAARC nations (Case 3).

Management of acute kidney injury in cirrhosis.

Acute kidney injury (AKI) is a relatively frequent problem, occurring in approximately 20 % of hospitalized patients with cirrhosis. Although serum creatinine (S Cr) is the most commonly used method to determine AKI because of easy availability and low cost, practically it underestimates the extent of kidney injury in patients with chronic liver disease. AKI is defined as an abrupt rise in S Cr of 0.3 mg/dl or more (>26.4 mmol/l) or an increase of 150 % or more (1.5-fold) from baseline. The cause of AKI in cirrhosis is multifactorial and is unique in terms of pathogenesis. The most common causes of AKI in cirrhosis can be subdivided into either functional or structural. The functional group includes volume-responsive (prerenal azotemia) and volume-unresponsive states (hepatorenal syndrome). Volume responsive is the most common type of AKI due to frequent use of diuretics, large volume abdominal paracentesis and gastrointestinal bleeding in patients with liver disease. The structural causes include acute tubular necrosis, tubulointerstitial and glomerular diseases. Patients with decompensated cirrhosis are in a vasodilatory state leading to a decrease in effective arterial blood volume, predisposing to AKI. Therefore, management of AKI depends on the underlying cause, and therapy should be directed toward removal of the cause. The outcome in cirrhosis when patients are on dialysis is very dismal. Every effort should be made to prevent AKI.