Summary:A 65-year-old male with poorly controlled type 2 diabetes mellitus presented with 7 months of progressive hoarseness of voice, fever, anorexia, cough, and weight loss. Just before admission, he developed drowsiness and memory impairment. Examination showed a drowsy but stable patient without meningeal signs and hyperpigmentation. Persistent hoarseness prompted laryngoscopy, which revealed a vocal cord nodule. Imaging demonstrated bilateral adrenal enlargement with hypodense lesions in liver, pancreas, spleen, and kidney. Hormonal evaluation showed preserved adrenal function. Histopathological examination of vocal cord tissue and an adrenal biopsy confirmed the diagnosis of disseminated histoplasmosis. The patient was treated with antifungal therapy. Over 18 months of therapy, adrenal function remained normal, renal function stabilized, and urinary histoplasma antigen became undetectable. Glycemic control improved on oral hypoglycemic agents, vocal recovery occurred with therapy, and the patient achieved long-term clinical stability. Learning points:Disseminated histoplasmosis can present with hoarseness, fever, weight loss and multi-organ involvement. Adrenal enlargement is common but may not always cause insufficiency. Histopathology and fungal stains (PAS, GMS, and LPCB) are diagnostic gold standards. High clinical suspicion is needed in endemic regions or immunocompromised hosts.

Background: Atypical hemolytic uremic syndrome (aHUS) is a severe thrombotic microangiopathy predominantly affecting the kidneys, often associated with complement dysregulation. This study is aimed to analyze the clinical characteristics, treatment outcomes, and long-term implications of aHUS in a resource-limited setting. Materials and Methods: A retrospective observational study conducted at an institute between January 2016 and December 2022 included all patients with aHUS, excluding secondary causes and renal transplant recipients. Demographic profiles, clinical features, laboratory parameters, treatment modalities (immunosuppression and plasma exchange), and outcomes were collected. Anticomplement Factor H (anti-CFH) antibody, complement levels, and genetic mutation analysis were performed to ascertain etiological factors. The patient and renal outcomes of anti-CFH positive and negative patients on long-term follow-up were compared. Results: Fifty-seven patients (mean age: 12.5 +/- 4.9 years; 63% males) were analyzed. Among them, 33 (57.9%) tested positive for anti-CFH antibodies and eight presented postpartum. Initial remission was achieved in 42 (73.6%) patients, with 13 (22.8%) partial and 29 (50.9%) complete remission. The median follow-up duration was 24 months [interquartile range (IQR) 8.5-84]; 12 (21%) patients died, with two deaths during the index admission, six among nonresponders, and 4 among responders. Dialysis-free renal survival was superior in anti-CFH seropositive patients (81.2%) compared to seronegative counterparts (55.9%), while patient survival was statistically similar between the two groups. Elevated anti-CFH titers (>4000 AU/ml), age >= 16 years, female gender, and seizures predicted nonresponsiveness. Conclusion: Anti-CFH antibody associated aHUS had better kidney outcomes than the seronegative counterparts. In resource limited settings, a combination of plasma exchange and immunosuppression showed promising results in the short and long term.

Background/Aims:Sofosbuvir (SOF) is a major directly acting antiviral (DAA) drug against hepatitis C virus (HCV) treatment. In patients with chronic kidney disease (CKD) and estimated glomerular filtration rate (eGFR) <30 mL/min, the experience of SOF based regimens is limited. We report real-life experience of treating a large cohort of patients with eGFR <30 mL/min using SOF-based regimens. Methods:We retrospectively reviewed the data of HCV viremic adults with eGFR <30 ml/min who were registered in our out-patient hepatitis clinic between December 2015 and April 2025. They were treated with full-dose SOF (400 mg) in combination with either velpatasvir (VEL) 100 mg or daclatasvir (DAC) 60 mg. Regardless of DAA combination, those without cirrhosis or with decompensated cirrhosis were treated for 12 and 24 weeks, respectively. Those with compensated cirrhosis were treated for 12 (SOF/VEL) or 24 (SOF/DAC) weeks. We studied the proportion of participants who could achieve sustained virological response after 12 weeks (SVR12) of treatment completion. Results:271 infection episodes (262 new; 9 reinfections) in 262 participants (men 74.1%; age 42 [32-52] years; hemodialysis 231/262 [88.2%]; dialysis duration 15 [9-25] months; no-cirrhosis 84.4%; HCV RNAlog10 5.82 [5.04-6.59]) were treated with either SOF/DAC (195; 74.4%) or SOF/Vel (67; 25.6%). In the treatment-naïve group, SVR12 was tested for 220/262 (SOF/DAC 168, SOF/VEL 52) participants (83.9%), and SVR12 was achieved in 210 (intention-to-treat analysis 210/262, 80.2%; per-protocol analysis 210/220, 95.5%). The SVR12 rates were not affected by the presence of cirrhosis, genotype, or type of DAA combination. After achieving SVR12, eight and one patient had second and third episodes of HCV re-infections after 8 (4-13.5) months and 6 months, respectively, and five of them achieved SVR12 following retreatment. Conclusions:Sofosbuvir in combination with DAC or VEL are highly and equally effective against HCV in patients with eGFR below 30 mL/min.

Understanding metabolic alterations in CKD is crucial, as serum creatinine-based diagnosis lacks precision, affecting key clinical decisions. In this study, a 1H NMR-based metabolomics approach was employed to distinguish between advanced-stage CKD (ASCKD) patients and healthy controls (HC), as well as within the ASCKD stages (stage 4 and stage 5). Serum samples from 52 ASCKD (S4, S5) and 25 HC were analyzed. Multivariate and univariate analysis revealed distinct metabolic patterns across groups, providing insights into CKD pathophysiology and associated pathway alterations. Compared to HC, six metabolites were significantly altered in both stage 4 and 5 CKD patients with upregulated creatinine, urea, myoinositol, choline, N,N-dimethylglycine, and downregulated tyrosine, showing potential as biomarkers with AUC above 0.8 in ROC analysis. Additionally, myo-inositol, dimethylamine, N,N-dimethylglycine, and choline correlate positively with creatinine while tyrosine correlates negatively. Amino acid metabolism was downregulated in S5 indicating more severity. Within ASCKD patients, significant alterations were observed in metabolites such as glutamate, glutamine, alanine, threonine, myo-inositol, dimethylamine, citrulline, urea, citrate, and betaine. Pathway analysis identified five distinct metabolic pathways associated with CKD progression. Consequently, we propose a panel of serum metabolites which should be monitored along with creatinine for following CKD progression. Markers of oxidative stress, inflammation, and gut dysbiosis were evident in the perturbed metabolic profile due to the systemic impact of CKD.

Introduction:This study evaluated the efficacy and safety of desmopressin in reducing postbiopsy bleeding in patients undergoing kidney biopsy, a common complication requiring effective prevention strategies. Methods:In this double-blind, randomized, placebo-controlled trial conducted from February 2019 to January 2023 at a teaching institute in Lucknow, India, 203 patients aged 18 to 65 years undergoing indication kidney biopsy were randomized to receive desmopressin (300 μg) or placebo intranasally an hour before the procedure. Outcomes included postbiopsy bleeding (primary) and hemoglobin drop, hypotension, hematuria, hematoma, transfusion need, and radiological or surgical interventions (secondary). Results:Bleeding incidence was significantly lower in the desmopressin group (11.9%) compared with placebo (33.3%, P = 0.0003). Hematoma formation was reduced (11.9% vs. 30.4%, P = 0.001), with a relative risk (RR) of bleeding of 0.356 (95% confidence interval [CI]: 0.196-0.648, P = 0.0007). Stratified analysis showed reduced bleeding across estimated glomerular filtration rate (eGFR) categories (> 30 and < 30 ml/min per 1.73 m2), with numbers needed-to-treat of 5.667 and 3.978, respectively. Hyponatremia and headaches were more frequent in the desmopressin group. Factor VIII and von Willebrand factor (vWF) levels were significantly elevated 2 and 4 hours after administration. No serious adverse events occurred. Conclusion:Desmopressin effectively reduces postbiopsy bleeding and hematoma formation with manageable side effects, supporting its role as a prophylactic option in kidney biopsies. Further studies are needed to confirm its broader clinical applications.

BACKGROUND:Diabetes muscle infarction (DMI) is a common misdiagnosed and under-reported diabetes complication. This causes a delay in diagnosis, which increases the morbidity of the disease. OBJECTIVE:To review all cases of DMI and its pathogenesis, clinical features, prognostic implications, and management. METHODS:We retrospectively analyzed 12 DMI patients diagnosed in the past 15 years. We investigated the disease's clinical characteristics, laboratory results, imaging features, therapies, and prognostic progression. RESULT:DMI patients were diagnosed at a mean age of 50 years (range: 33-78 years), were predominantly males (n = 7), and 11 (92%) had type 2 diabetes. They had diabetes for a long time (mean duration 12.6 years). Almost all the patients had nephropathy, neuropathy, or retinopathy. Before presentation, these patients had been symptomatic for an average of 50 days (range: 10-120 days). Most patients presented with unilateral lower limb swelling, local pain and tenderness, and a raised temperature, as well as severe motion-dependent pain. The quadriceps muscle was the most affected, with only one patient experiencing upper limb involvement. On T2W, MRI revealed heterogeneously enlarged muscles with post-contrast enhancement; post-contrast scans revealed multiple focal hypo-enhancing areas, indicating myonecrosis. All the patients were managed conservatively with a good short-term prognosis. CONCLUSION:When a patient presents with a painful, tender lump in the lower limb without a history of trauma, DMI should be strongly suspected. MRI findings are diagnostic, and no biopsy is required. Due to multiple comorbidities, these patients are typically managed conservatively, with a good short-term but poor long-term prognosis.

The available literature on graft histology and graft outcomes in kidney transplant recipients (KTRs) with acute rejection after ABO-incompatible and ABO-compatible kidney transplant is scarce. Among 100 ABO-incompatible kidney transplants (KTx) performed between 2014 and 2019, 37 (37

Background: Focal segmental glomerulosclerosis (FSGS) is a kidney disease with diverse causes, classified as primary, genetic, or secondary. There is a high recurrence rate of FSGS posttransplant and is associated with a significant risk of graft loss, especially in the primary cases. In this descriptive study, we aimed to analyze the presentation, treatment response, and outcomes of posttransplant recurrent FSGS (rFSGS) in the Indian population. Methodology: In this retrospective study, from a North Indian tertiary care center, we identified 18 cases of posttransplant rFSGS over 12 years. Therapeutic plasma exchange (TPE) was used in most cases, with rituximab (RTX) added for refractory cases. We collected the pretransplant and posttransplant clinical profiles, treatment, and follow-up of these patients including graft and patient survival, hospitalizations, and infectious disease episodes. Results: We identified 18 cases of posttransplant FSGS recurrence over 12 years, predominantly affecting male recipients who had received live-related renal transplants. Overall, 55.6% of recurrences responded to therapy, with varying degrees of remission achieved. The response rate was better in patients with TPE, especially with RTX. At 10-year follow-up, the patient survival at 10 years was 77.7%. Death-censored graft survival at 10 years was 50% with better graft survival in patients receiving additional RTX. Major infection rates were higher in patients who received TPE with RTX. Conclusion: This study highlights the significant challenge of managing rFSGS posttransplant in an Indian population, with TPE and RTX showing varied success in inducing remission. These findings underscore the importance of tailored therapeutic strategies to improve long-term outcomes in managing rFSGS but emphasize the need for larger randomized trials to confirm these findings.

Objectives:We analysed interventions related to TAK, their pre-treatment clinical and angiographic associations, and prognostic relevance from a large ambispective, monocentric cohort of TAK from India. Methods:Information regarding endovascular or open surgical interventions (aortoplasty, nephrectomy for refractory hypertension) was retrieved from a cohort of patients with TAK. Demographic characteristics, clinical features, and angiographic involvement were compared between patients with TAK who had undergone interventions with the rest of the cohort using multivariable-adjusted odds ratios (OR, with 95% CI). Hazard ratios were used to compare the mortality rate among TAK who had undergone interventions. Results:Among 238 patients with TAK in the cohort, 41(17.23%) had undergone 69 interventions related to TAK (64 endovascular procedures, one open surgical aortoplasty, 4 nephrectomies) across 55 sittings (a single intervention sitting in 31, two in seven, three in two, and four in one). The most common arterial territories undergoing intervention were the renal arteries (n=21), subclavian arteries (n=8), and descending thoracic aorta (n=6). Six patients with TAK required repeated interventions in the same arterial territories. Patients with TAK who underwent interventions more frequently had abdominal angina (OR 5.12, 95%CI 1.36-19.26), and less often had constitutional features (OR 0.39, 0.18-0.84) at presentation without significant differences in angiography. Survival was similar in TAK who had undergone interventions to those without (hazard ratio for mortality 0.91, 95%CI 0.23-3.55). Conclusion:About one-sixth of our cohort of TAK had undergone interventions, most often endovascular interventions. One-fourth required multiple interventions. Survival was similar in TAK with or without interventions.

The progressive illness known as chronic kidney disease (CKD) can often be challenging to diagnose in its early stages with conventional diagnostic approaches such as serum creatinine and albumin assessment. Early-stage CKD (stages G1-G3) is defined by a GFR of ≥30 mL min-1/1.73 m2, which indicates normal to moderately reduced kidney function with or without symptoms of impaired kidney function. Identifying possible biomarkers for early detection and personalised treatment, as well as physiological changes linked to early CKD-an area that has not been fully investigated before-is the goal of the study to address this gap. We performed a metabolomic analysis using 1H NMR on 115 human serum samples (24 healthy controls and 91 patients with early-stage CKD). MetaboAnalyst 6.0 was used for data pre-processing and statistical analyses (PCA, PLS-DA, OPLS-DA, ANOVA, and Wilcoxon Mann-Whitney test). Strong differentiation between CKD stages was achieved by random forest modelling. The KEGG database was used to perform pathway enrichment, and ROC analysis was used to evaluate the diagnostic value of important metabolites. Across CKD stages, significant changes were observed in ten different metabolites: myo-Inositol, glycerol, pyruvate, carnitine, phenylalanine, tyrosine, histidine, TMAO, 2-hydroxyisobutyrate, and 3-hydroxyisobutyrate (p < 0.05, VIP > 1). AUC values > 0.7 from ROC curves demonstrated its potential for diagnosis. Pathway analysis revealed significant dysregulation in the metabolism of inositol phosphate, tyrosine, histidine, and pyruvate, and biosynthesis of phenylalanine, tryptophan and tyrosine. This comprehensive metabolomics investigation identified potential early-stage CKD biomarkers in addition to significant metabolic abnormalities. These findings could help provide individualized care for early CKD management.

Acute interstitial nephritis (AIN) is one of the major causes of Acute kidney injury who mainly present with non-oliguria. It is imperative to have tissue diagnosis of AIN in order to decide on therapy in cases who are not showing self-recovery of renal function and prevent them from disease progression. This study focuses on the clinical presentations, causes of AIN, outcomes and prognostic indicators.

Background Renal involvement in sarcoidosis is rare. We evaluated the pattern of renal involvement in sarcoidosis, its clinical course, renal histology, and response to treatment. Materials and Methods We retrospectively analyzed the data of all cases with sarcoidosis exhibiting renal involvement referred to our department between January 2010 and December 2021. Results A total of 33 patients (age: 50.6 ± 12.6 years, males: 57.6%) were analyzed. Common presenting symptoms were weight loss (81.8%; n = 27), fever (75.8%; n = 25), and vomiting (63.6%; n = 21). A total of 14 (42.4%) patients had granulomatous interstitial nephritis (GIN), 13 (39.4%) had isolated hypercalcemia, and six (18.2%) had GIN along with hypercalcemia. Renal biopsy was performed in 20 (60.6%) patients, and all showed GIN, with concomitant glomerular disease in four (12.1%) patients. Mean serum creatinine and 24-h urine protein at presentation were 4.3 ± 2.1 mg/dL and 2.5 ± 0.9 g/day, respectively. All patients received oral prednisolone 1 mg/kg/day with subsequent tapering, concomitantly with azathioprine. Mycophenolate mofetil was used in three (9.1%) patients who developed azathioprine-induced hepatoxicity. After a median follow-up of 24 months (8–120 months), mean serum creatinine and 24-h urine protein improved to 1.9 ± 1.5 mg/dL and 1.1 ± 0.6 g/day, respectively, ( P = 0.005). On follow-up, two patients (6.1%) became dialysis-dependent, and three (9.1%) succumbed: one due to a cardiovascular event and two to sepsis and septic shock. Conclusion Granulomatous interstitial nephritis was the most common diagnosis in sarcoidosis patients with kidney failure. Early steroid treatment improves kidney function.

Background: Chronic kidney disease (CKD) patients are at a high risk of tuberculosis (TB), with a relative risk of developing active TB of 10%–25%. Similarly, glomerular disease increases the risk of TB due to diminished glomerular filtration rate, proteinuria, and immunosuppression use. Further, the first-line anti-TB drugs are associated with acute kidney injury (AKI) even in patients with normal kidney functions. Methods: We retrospectively identified 10 patients hospitalized with unusual adverse effects of antituberculosis therapy (ATT) from 2013 to 2022. Results: We found three cases of AKI caused by rifampicin: acute interstitial nephritis, crescentic glomerulonephritis, and heme pigment-induced acute tubular necrosis. We observed rifampicin-induced accelerated hypertension and thrombocytopenia in two patients on maintenance hemodialysis. Isoniazid caused pancreatitis and cerebellitis in two CKD patients, respectively. In a CKD patient, we detected acute gout secondary to pyrazinamide-induced reduced uric acid excretion. We also observed cases of drug rash with eosinophilia and systemic symptoms and hypercalcemia due to immune reconstitution inflammatory syndrome in patients with glomerular disease on ATT. Immediate discontinuation of the offending drug, along with specific and supportive management, led to a recovery in all cases. Conclusion: The adverse effects of ATT may be unusually severe and varied in kidney patients due to decreased renal elimination. Early recognition of these adverse effects and timely discontinuation of the offending drug is essential to limit morbidity and mortality.

Introduction: Monogenic urinary stone disease (MUSD) tends to be more severe with early onset of symptoms and a higher risk of chronic kidney disease than sporadic USD. The literature on the outcome after renal transplant in patients with certain MUSD is scarce. Materials and Methods: This is a retrospective single-center observational study conducted in a tertiary care renal transplant unit in North India between 2018 and 2021. The renal transplant recipients who developed an end-stage renal disease (ESRD) due to renal calculus disease/nephrocalcinosis were included in the study. All the patients presented to us in an anuric state, and hence, a 24-h urine metabolic profile could not be performed. Ear, nose, and throat and ophthalmological evaluations were done to rule out extrarenal manifestations. These patients were subjected to genetic analysis, i.e., clinical exome sequencing using next-generation sequencing. Results: Out of 283 live renal transplants, 11 patients developed ESRD due to nephrocalcinosis/renal calculus disease. Out of 11, only 4 had genetic mutations and the rest did not have any identifiable genetic mutations. The gene mutations were identified in ADCY10, CLDN16, CaSR, and SLC3A4. The patient with ADCY10 mutation had a strong family history. The clinical phenotype and in silico parameters analysis predicted the variant to be damaging except the one with CaSR mutation which causes Hypocalciuric hypercalcemia syndrome, type 1. Three of four underwent surgical intervention at younger age. All underwent successful live-related renal transplantation, with good graft function on follow-up, without any recurrence of calculus in the allograft. Conclusion: Renal transplantation can be safely proceeded in patients with the above monogenic mutations. Genetic analysis should be a part of pretransplant evaluation in young onset nephrolithiasis and end-stage kidney disease patients to look for a monogenic cause, to assess the risk of recurrence postrenal transplant.

Introduction Nephrotic syndrome, an unusual clinical presentation of IgA nephropathy (IgAN), occurs only in a few cases. The data regarding its clinical characteristics and treatment outcomes are lacking. Material and methods In this retrospective analysis, we reviewed kidney biopsies conducted between January 2007 and December 2018. All patients with biopsy-proven IgAN and clinical presentation of nephrotic syndrome were included. Results Sixty-seven (11.9%) out of 560 patients with primary IgAN had nephrotic syndrome as the first clinical presentation. On MEST-C scoring, the baseline estimated glomerular filtration rate (eGFR) was significantly lower in the presence of segmental sclerosis (S1) (p=0.04) and >25% tubular atrophy/interstitial fibrosis (T1/2) (p=0.004). With immunosuppression, complete remission (CR), partial remission (PR), and no response (NR) occurred in 28 (41.8%), 32 (47.8%), and 7 (10.4%) patients, respectively. During the median follow-up of 190 weeks, the median absolute change in eGFR was significant between CR and NR groups (p=0.002), and PR and NR groups (p=0.02); however, it was not significant between CR and PR groups (p=0.14). In the CR, PR, and NR groups, 9, 15, and 7 patients had eGFR losses of ≥15 ml/min (p=0.004). No patient in the CR group, one in the PR group, and three in the NR group progressed to end-stage kidney disease (ESKD) (p=0.003). Further, none of the MEST-C scores correlated with the clinical outcome parameters. Conclusion Immunosuppression in treating IgAN accompanied by nephrotic syndrome helps in proteinuria remission. Achieving remission, whether complete or partial, delays disease progression and improves renal survival. Moreover, the baseline eGFR was significantly lower when S1 and T1/2 lesions were present in kidney biopsies.

BACKGROUND The study focuses on the use of multi-parametric ultrasound [gray scale, color Doppler and shear wave elastography (SWE)] to differentiate stable renal allografts from acute graft dysfunction and to assess time-dependent changes in parenchymal stiffness, thereby assessing its use as an efficient monitoring tool for ongoing graft dysfunction. To date, biopsy is the gold standard for evaluation of acute graft dysfunction. However, because it is invasive, it carries certain risks and cannot be used for follow-up monitoring. SWE is a non-invasive imaging modality that identifies higher parenchymal stiffness values in cases of acute graft dysfunction compared to stable grafts. AIM To assess renal allograft parenchymal stiffness by SWE and to correlate its findings with functional status of the graft kidney. METHODS This prospective observational study included 71 renal allograft recipients. Multi-parametric ultrasound was performed on all patients, and biopsies were performed in cases of acute graft dysfunction. The study was performed for a period of 2 years at Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, a tertiary care center in north India. Independent samples t -test was used to compare the means between two independent groups. Paired-samples t -test was used to test the change in mean value between baseline and follow-up observations. RESULTS Thirty-one patients had experienced acute graft dysfunction at least once, followed by recovery, but none of them had a history of chronic renal allograft injury. Mean baseline parenchymal stiffness in stable grafts and acute graft dysfunction were 30.21 + 2.03 kPa (3.17 + 0.11 m/s) and 31.07 + 2.88 kPa (3.22 + 0.15 m/s), respectively; however, these differences were not statistically significant (P = 0.305 and 0.252, respectively). There was a gradual decrease in SWE values during the first 3 postoperative months, followed by an increase in SWE values up to one-year post-transplantation. Patients with biopsy-confirmed graft dysfunction showed higher SWE values compared to those with a negative biopsy. However, receiver operating characteristic analysis failed to show statistically significant cut-off values to differentiate between the stable graft and acute graft dysfunction. CONCLUSION Acute graft dysfunction displays higher parenchymal stiffness values compared to stable grafts. Therefore, SWE may be useful in monitoring the functional status of allografts to predict any ongoing dysfunction.