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Underlying comorbid illness is a known risk factor for severe coronavirus disease-2019 (COVID-19). Clinical course of COVID-19 in children with primary kidney disease is not well understood. We present the clinical profile and management of COVID-19 in three children at a COVID hospital in India. These children had nephrotic syndrome, hemolytic uremic syndrome, and chronic kidney disease, respectively. The first two were immunosuppressed, mandating to stop their immunosuppressive medications temporarily. Both had mild course of illness. Third child presented with respiratory distress requiring oxygen support, falling into moderate disease. Renal functions were normal in all of them. They all responded well to oral azithromycin and supportive management. None of them received chloroquine, corticosteroids, or monoclonal antibodies. All three recovered without complications.

Clinical and biochemical manifestations of lecithin-cholesterol acyltransferase (LCAT) deficiency include an abnormal lipid profile (characterized by hypercholesterolemia with markedly decreased high-density lipoprotein cholesterol [HDL-C] and hypertriglyceridemia), corneal opacities, hematologic abnormalities (normochromic anemia of varying intensity), splenomegaly, variable early coronary artery disease and nephropathy (initially proteinuria followed by progressive deterioration of renal function). We presented a patient with nephrotic syndrome, which renal biopsy revealed classic features of LCAT deficiency. To our knowledge, the present case is the first reported case of LCAT deficiency presenting with symptoms related to nephrotic syndrome in a patient with no obvious family history without any corneal deposits and normal HDL-C levels.

To determine the knowledge, attitudes and practices regarding organ donation in western India.Convenience sampling was used to generate a sample of 250; 200 interviews were successfully completed and used for analysis. Data collection was carried out via face to face interviews based on a pre-tested questionnaire in selected public areas of Ahmedabad, Gujarat state of India. Data entry was made in excel software in codes and analysis was done by SPSS software.About 86% of participants were aware of the term organ donation but knowledge about its various aspects was low. About 48% aware people heard about organ donation through medical fraternity, whereas only about 21% became aware through mass media. About 59% of aware people believed there is a potential danger of donated organs being misused, abused or misappropriated. About 47% of aware people said they would consider donating organs, while only 16% said they would definitely donate irrespective of circumstances. Around 97.67% participants said they would prefer to donate to nonsmokers. About 74.41% participants were unaware about any legislation regarding organ donation. About 77% participants showed their will to donate to mentally sound persons, and 42.04% participants showed their will to donate even physically challenged people. Around 78 participants felt that they would donate organs to persons irrespective of their religion. About 81% of aware people were of the opinion that consent for organ donation after death should be given by family members. None of the interviewed participants had a donor card.Better knowledge and awareness will help in promoting organ donation. Effective campaign needs to be driven to educate people with relevant information with the involvement of media, doctors and religious scholars.

Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiographic entity of heterogenous etiologies, which are grouped together because of similar findings on neuroimaging studies, associated with similar symptom complex of headache, vision loss, altered mentation, and seizures. In this report, we describe a case of PRES in setting of postobstructive diuresis in a 5-year-old male child, whose solitary functioning kidney was obstructed by a 1.6-cm radio-opaque stone, who after percutaneous nephrostomy (PCN) diversion developed persistent hypocalcemia which persisted despite maximum replacement by iv calcium gluconate drip, and the child developed repeated generalized tonic clonic convulsions and became unconscious for 4 days. Computerized tomography (CT) scan of the brain showed typical hypodensities in bilateral occipitoparietal regions suggesting PRES. Ultimately, over a period of 4 days, his hypocalcemia could be corrected and the child was neurologically normal on the 5th day. CT scan of the brain after a month was free of any hypodensities.

Plasmodium vivax infection is increasingly a major public health burden and the second most frequent human malaria. Higher levels of clinical severity and chloroquine resistance are major factors responsible for such increases. Malarial glomerular injury is uncommon and mainly observed in Plasmodium malariae-infected patients. Occasionally, transient immune complex-mediated glomerulonephritis is associated with Plasmodium falciparum infection. Coexistent crescentic glomerulonephritis and vivax malaria have not previously been reported. We report a fatal case of P. vivax malaria, who presented with acute renal failure. P. vivax monoinfection status was diagnosed with peripheral blood smear and rapid antigen test. Further evaluation for renal failure related to systemic illness and immunological markers were inconclusive. He was treated with antimalarial drugs, hemodialysis, and supportive therapy. Renal biopsy performed for nonrecovering renal failure reveled crescentic glomerulonephritis. This case highlights the need to thoroughly search for malaria-associated crescentic glomerulonephritis using renal biopsy after nonrecovering renal failure.

Kute VB, Trivedi HL, Vanikar AV, Shah PR, Gumber MR, Patel HV, Munjappa BC, Modi PR, Gera DN. Long-term outcome of deceased donor renal transplantation in pediatric recipients: A single-center experience from a developing country Abstract: RTx is best treatment for children with ESRD. Data scarcity on DDRTx outcome in children prompted us to review our experience. This study was undertaken to evaluate patient/graft survival, function vis-a-vis SCr, rejection episodes, and mortality in DDRTx performed in 37 children between 1998 and 2011. The most common recipient diseases leading to ESRD were congenital anomalies of kidney and urinary tract (48.6%) and chronic glomerulonephritis (18.9%). Mean recipient age was 13.8 ± 3.1 yr; 67.5% (n = 25) were men. Mean donor age was 38.8 ± 18.6 yr; 48.5% (n = 18) were men. Mean dialysis duration pre-transplantation was 15.5 ± 3.5 months. All recipients received r-ATG, and triple immunosuppression. Over a mean follow-up of 3.93 ± 3.5 yr, patient and graft survival rates were 72.9% (n = 27) and 83.7% (n = 31), respectively, with a mean SCr of 1.1 mg/dL; 21.6% (n = 8) of patients had acute rejection episodes; 24.3% (n = 9) of patients had DGF. A total of 27% (n = 10) patients died, mainly owing to infections (n = 6) and cardiovascular disease (n = 3). DDRTx is a viable option for children and achieves acceptable graft function with patient/graft survival over long-term follow-up, encouraging use of this approach.

Background: Renal biopsy is a well-established diagnostic modality for the assessment of kidney diseases in children. It can provide diagnostic precision and prognostic value and guide in therapeutic options for many renal diseases. Objectives: This report describes the indication, histopathological patterns, and epidemiology of renal diseases in children in India. Patients and Methods: This is a single-center study on renal biopsies performed between January 2008 and December 2013 in 346 children (age ≤ 14 years). Results: Eleven (3.17%) biopsies were inadequate, and 335 biopsies were considered for analysis. The mean age was 7.91 ± 3.04 years with a predominance of males (68.1%). Nephrotic syndrome (46.2%) was the most common indication, followed by urinary abnormality (41.19%), acute nephritic syndrome (10.74%), and chronic renal failure (1.79 %). Primary glomerulonephritis (GN) was predominant (81.79%), and secondary GN constituted 16.12% of the biopsies. Primary GN included mesangial proliferative GN (MePGN), IgM nephropathy, focal segmental glomerulosclerosis, minimal change disease, IgA nephropathy, membranoproliferative GN, membranous nephropathy, crescentic GN, and post-infectious GN. Secondary GN revealed lupus nephritis, hemolytic uremic syndrome, amyloidosis, and hypertensive nephropathy. Tubulointerstitial nephritis was observed in 2.08%. The most common histological pattern of primary GN was MePGN (20%) and in secondary GN it was lupus nephritis (7.76%). Conclusions: The present study provides data on the epidemiology of renal diseases in children in India and will be helpful for developing a national registry and devising therapeutic guidelines.

Background and Objectives: This observational study was conducted to evaluate and compare the efficacy of calcineurin inhibitors in frequently relapsing nephrotic syndrome (FRNS) and steroid-resistant nephrotic syndrome (SRNS). Subjects and Methods: Each group comprised 15 patients who were studied prospectively. These patients were treated with tacrolimus (Tac) or cyclosporine (CSA) concomitant with prednisone, which was subsequently tapered off and stopped. The primary outcome variable was the number of patients who attained a complete remission (CR) or partial remission (PR). Results: Out of 15 patients with FRNS, 11 were children and 4 were adults. In SRNS, out of total 15 patients 7 were children and 8 were adults. There was male preponderance in FRNS children, whereas in SRNS, gender distribution was almost equal. Hypertension was more common in steroid-resistant group (80%) as compared to FRNS (33%). In FRNS, focal segmental glomerulosclerosis (FSGS), and immunoglobulin M variant of minimal change disease were the most common cause, each accounting for 5 patients, whereas FSGS (8 patients) was the most common etiology in SRNS. In FRNS, out of total 4 patients who were treated with CSA, 2 achieved CR and 2 achieved PR. Mean time to achieve remission was 3.2 months. In FRNS, out of 13 patients who were treated by Tac, 7 patients achieved CR and 6 patients achieved PR. Mean time to achieve remission was 2.1 months. In SRNS, out of 3 patients treated with CSA, 1 achieved PR whereas 2 patients did not respond. Time to achieve remission was 5 months. In SRNS, 11 of 14 patients achieved CR and 2 achieved PR. Mean time to achieve remission was 2.5 months. Among 2 patients resistant to CSA 1 achieved CR with Tac and one did not respond to Tac also. Conclusion: Thus, in FRNS patients, both CSA and Tac are almost equally effective whereas in SRNS patients Tac was more effective than CSA and was also effective in 1 patient resistant to CSA.

Introduction: The kidneys are involved in significant number of patients with multiple myeloma (MM) who can present with acute or chronic renal failure, nephritic syndrome, non-nephrotic proteinuria or tubular function defects. Objectives: To assess the clinical profile of kidney involvement preceding diagnosis of multiple myeloma Patients and Methods: Renal involvement in 29 cases with MM admitted over an 18-month period to our tertiary care center was retrospectively examined. Diagnosis of MM was confirmed by two or more of the following four features: lytic bone lesions, serum or urine monoclonal peak, Bence-Jones proteinuria, and greater than 20% plasma cells in bone marrow. Results: Renal disease was present in all patients before MM was diagnosed. Non-steroidal anti-inflammatory drugs (NSAIDs) was the most common precipitating factor for acute kidney injury (AKI). All 29 patients received combination chemotherapy of bortezomib, dexamethasone and thalidomide. More than half of the total number of patients did not complete chemotherapy because of death or lost to follow-up. Twenty-two of 29 patients required hemodialysis support. AKI was the most common renal presentation of MM. Four patients with AKI had complete renal recovery. Eleven patients who required hemodialysis support initially later on recovered to non-dialyzable range of renal failure. Seven patients became hemodialysis dependent. Twelve patients died from infection, uremia or hyperkalemia. Nine patients lost to follow up. Remission of MM was seen in 8 patients who completed chemotherapy. Conclusion: In our study AKI is the most common renal presentation preceding the diagnosis of MM. Reversal of renal function was achieved with chemotherapy and high flux hemodialysis in few cases.

We report a case of diabetic end-stage renal disease patient who presented with a right common carotid artery jugular arteriovenous fistula as a complication of the insertion of a polyurethane double-lumen hemodialysis catheter into the right internal jugular vein .On physical examination of the neck, a pulsating mass with a palpable thrill and a bruit was noted in the right subclavicular region. The diagnosis was confirmed by color doppler ultrasonography of the neck and carotid angiography. The review of the literature suggests the occurrence of this complication as rather rare. The fistula was successfully repaired surgically. It is emphasized that while securing the access, a thorough physical examination with a special emphasis on seeking any neck swellings, thrill, and bruit along with routine use of vascular doppler for securing dialysis access is recommended.

Despite heightened international interest in performing living donor kidney paired donation (KPD) transplantation after the publication of a research protocol by Ross and colleagues in 1997, only a few hundred have been performed worldwide. The major obstacle is that many individuals in end-stage renal disease are of blood type O and can only receive an organ from a donor of blood type O, whereas blood type O donors are "universal donors" and will be able to donate directly with an intended recipient of any blood type unless there is a positive crossmatch. To overcome this, patients with compatible but non-HLA identical donors over 45 years of age should be approached for inclusion in KPD program especially O blood group donors. Inclusion of all these additional pairs into the algorithm greatly increases chances of possible matches for O blood group recipients. We report successful three-way KPD transplantation resulting in transplantation of O blood group patient using compatible O blood group donor from India. None of the patients had delayed graft function or rejection and all had stable graft function on discharge without any medical and surgical complications. We need to allocate O blood group kidneys from compatible donors to overcome the barrier of HLA, non-HLA antibodies and other donor related factors to improve transplant quality and long term outcomes. This will increase transplantation of O blood group patients.

Tacrolimus, a calcineurin inhibitor, is a potent immunosuppressive agent used by a majority of transplanters across the globe. Its adverse effects include nephrotoxicity, neurotoxicity, new onset diabetes after transplant, gastro-intestinal toxicity, hepatotoxicity, and thrombotic microangiopathy. Tacrolimus-induced hepatotoxicity is a very uncommon side effect. We report a case of tacrolimus-induced hepatotoxicity in the form of cholestatic hepatitis a renal transplant recipient. Hepatotoxicity did not decrease after dose reduction; however, normalization of liver enzymes occurred after discontinuing tacrolimus.

RTx is best treatment for children with ESRD. Data scarcity on DDRTx outcome in children prompted us to review our experience. This study was undertaken to evaluate patient/graft survival, function vis-a-vis SCr, rejection episodes, and mortality in DDRTx performed in 37 children between 1998 and 2011. The most common recipient diseases leading to ESRD were congenital anomalies of kidney and urinary tract (48.6%) and chronic glomerulonephritis (18.9%). Mean recipient age was 13.8 ± 3.1 yr; 67.5% (n = 25) were men. Mean donor age was 38.8 ± 18.6 yr; 48.5% (n = 18) were men. Mean dialysis duration pre-transplantation was 15.5 ± 3.5 months. All recipients received r-ATG, and triple immunosuppression. Over a mean follow-up of 3.93 ± 3.5 yr, patient and graft survival rates were 72.9% (n = 27) and 83.7% (n = 31), respectively, with a mean SCr of 1.1 mg/dL; 21.6% (n = 8) of patients had acute rejection episodes; 24.3% (n = 9) of patients had DGF. A total of 27% (n = 10) patients died, mainly owing to infections (n = 6) and cardiovascular disease (n = 3). DDRTx is a viable option for children and achieves acceptable graft function with patient/graft survival over long-term follow-up, encouraging use of this approach.

Guillain–Barre syndrome is acute, frequently severe fulminant polyradiculoneuropathy characterized by areflexic motor paralysis with/without sensory disturbance. We report a rare case of post-renal transplant (RT) Guillain–Barre syndrome (GBS) in a 34-year-old male, who presented with sudden onset of ascending pattern paralysis of lower limbs (LL) without bowel/bladder involvement. The diagnosis was confirmed by neurological examination and nerve conduction velocity (NCV) studies. He was treated with IV immunoglobulin and recovered completely.

We report a case of diabetic end-stage renal disease patient who presented with a right common carotid artery jugular arteriovenous fistula as a complication of the insertion of a polyurethane double-lumen hemodialysis catheter into the right internal jugular vein .On physical examination of the neck, a pulsating mass with a palpable thrill and a bruit was noted in the right subclavicular region. The diagnosis was confirmed by color doppler ultrasonography of the neck and carotid angiography. The review of the literature suggests the occurrence of this complication as rather rare. The fistula was successfully repaired surgically. It is emphasized that while securing the access, a thorough physical examination with a special emphasis on seeking any neck swellings, thrill, and bruit along with routine use of vascular doppler for securing dialysis access is recommended.

We designed and implemented a clinical trial to achieve zero-rejection status in pediatric renal allograft recipients, using granulocyte-macrophage colony-stimulating factor (GM-CSF)-stimulated peripheral blood stem cell (PBSC) infusion. We studied 44 consecutive patients: 24 volunteers in a treated group (Tn) and 20 in a control group (Cn). Both groups were comparable with respect to clinical and laboratory parameters. The Tn group had 70.8% one haplo-match donors and the Cn group had 80% one haplo-match donors. Patients in the Tn group received cyclosporin A (CsA) and 0.4 mg/kg body weight prednisolone as immunosuppressants; azathioprine was added for patients of the Cn group, who received 1 mg/kg body weight prednisolone together with CsA. Living-related donors (LRD) of patients in the Tn group received GM-CSF 450 microg on four consecutive days followed by leucopheresis and immediate transfusion of unmodified PBSC into the recipient. This procedure was repeated once/twice, with one portal and one/two systemic infusions. Our aim was to maximize the dose of PBSC. The total average dose was 22 x 10(8) cells/kg body weight. Lymphocyte cross-match (LCM) was performed before GM-CSF injection and after the last PBSC infusion. Follow-up over an 18-month period revealed 100% graft survival with sustained low serum creatinine (SCr) values in patients of the Tn group as compared with 80% graft survival in patients of the control group who had marginally higher SCr levels. Absence of graft vs. host disease (GvHD), acute rejection episodes, and low incidence of cytomegalovirus (CMV) disease were the principal benefits of this protocol.