ABO Incompatible Kidney Transplant in a Patient With Atypical Hemolytic Uremic Syndrome: Case Report.

Kidney transplant (KT) or renal transplant is 1 of the preferred treatment options for patients with end-stage renal disease, but the presence of atypical hemolytic uremic syndrome (aHUS) further increases the risk of reoccurrence with graft rejection, and poor outcomes. ABO incompatibility further adds to the rejection risk. Here, we present a case of a young adult with a history of aHUS undergoing a successful ABO-incompatible (ABOi) renal transplant. ABO incompatibility desensitization was carried out, and the antibody titer was reduced to nullify the risk of rejection. Graft acceptance was facilitated by triple immunosuppression (steroid, tacrolimus, and mycophenolate mofetil), and 4-month serum creatinine follow-up indicated the absence of antibody-mediated rejection and recurrence of aHUS. This case demonstrates that in patients with aHUS, ABOi renal transplant can be performed successfully.

Kidney Transplant Outcomes in Patients with Atypical Hemolytic Uremic Syndrome.

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare disorder with a high probability of recurrence after a kidney transplant and can adversely affect the graft outcome. Our objective was to assess the transplant outcome of patients with aHUS who had undergone a kidney transplant. METHODS: We retrospectively included patients who had undergone a kidney transplant and been diagnosed with aHUS based on an anti-complement factor H (AFH) antibody level >100 AU/mL and the presence of a genetic abnormality in complement factor H (CHF) or CHF-related (CFHR) genes. Data were analyzed with descriptive statistics. RESULTS: Among 47 patients with AFH antibody levels >100 AU/mL, 5 (10.6%) had undergone a kidney transplant. The mean age was 24.2 years, and all were male. Atypical hemolytic uremic syndrome was diagnosed before transplant in 4 (80.0%) cases, whereas 1 was diagnosed after transplant owing to disease recurrence in the transplanted graft. Genetic analysis of all cases revealed one or more abnormalities in CFH and CFHR genes 1 and 3. With an average of 5 sessions of plasma exchange and the use of rituximab in 4 cases, there was a reduction in the disease severity with no recurrences in the post-transplant period. At the latest follow-up of 223 days, the mean serum creatinine level was 1.89 mg/dL, indicating good graft function. CONCLUSIONS: Among patients diagnosed with aHUS, the use of pre-transplant plasma exchange and rituximab can be beneficial in terms of preventing graft dysfunction and reducing disease recurrence in the post-transplant period.

Profile and Post-Kidney Retrieval Outcomes in Asymptomatic Kidney Donors With Genetic Mutations in Complement Factors-Related Genes.

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare disorder triggered by various stressors. Most of the time, stressors may not be identified in patients with aHUS. The disease may remain quiescent without manifestation throughout life. BACKGROUND: To assess the outcome of an asymptomatic carrier of genetic mutations of patients with aHUS who had undergone donor kidney retrieval surgery. METHODS: We retrospectively included the patients diagnosed with a genetic abnormality in complement factor H (CHF) or CHF-related (CFHR) genes without manifestation of the aHUS and who had undergone donor kidney retrieval surgery. The data were analyzed with descriptive statistics. RESULTS: Among patients who were the kidney recipients from the prospective donors, 6 donors were screened for genetic mutations in CFH and CFHR genes. Four donors showed positive mutation for CFH and CFHR. The mean age was 54.5 years (range, 50-64 years). After over a year since donor kidney retrieval surgery, all prospective mother donors are alive without aHUS activation and with a normal kidney function on a single kidney. CONCLUSION: Asymptomatic carriers of genetic mutations in CFH and CFHR can be the prospective donors for their first-degree family member who have active aHUS. A genetic mutation in an asymptomatic donor should not be a contraindication for refuting the prospective donor.

CYP3A5 Polymorphism in Renal Transplantation: A Key to Personalized Immunosuppression.

BACKGROUND: Tacrolimus is essential for the maintenance of immunosuppression after a kidney transplant. CYP3A5 is the gene that metabolizes tacrolimus, and polymorphism in this gene affects the metabolizing status. AIM: To assess the genetic polymorphism status of patients undergoing kidney transplantation and determine its impact on graft function and complications in the post-transplant period. METHODS: We retrospectively included the patients who had undergone a kidney transplant and had positive genetic polymorphism of the CYP3A5 gene. Based on loss of alleles, patients were categorized as non-expresser (loss of both alleles), intermediate expresser (loss of one allele), and expresser (no loss of allele) denoted by CYP3A5*3/*3, CYP3A5*1/*3, and CYP3A5*1/*1, respectively. Data were analyzed with descriptive statistics. RESULTS: Of 25 patients, 60%, 32%, and 8% were non-expressers, intermediate-expressers, and expressers, respectively. The mean tacrolimus trough concentration to dose ratio after 6 months of the transplant was higher in non-expressers than intermediate-expressers and expressers (213 vs 85 and 46 ng/mL/mg/kg/d, respectively). The graft function was normal in all 3 groups except for graft rejection 1 patient in the expresser group. Compared with expressers, urinary tract infections (42.9% and 62.5%) and new-onset diabetes after transplantation (28.6% and 12.5%) were more frequent in non-expresser and intermediate expressers, respectively. The proportion of patients developing new-onset diabetes after transplantation was lower with the pre-transplant diagnosis of CYP3A5 polymorphism (16.7% vs 23.1%). CONCLUSION: Genotype-based dosing of tacrolimus helps achieve the desired therapeutic concentrations that can help to optimize graft outcomes and reduce the tacrolimus-related adverse effects. Pre-transplant evaluation of CYP3A5 can be more helpful in planning treatment strategies for optimized outcomes after kidney transplantation.

Humoral Response After Two Doses of COVISHIELDTM Vaccine in Patients Undergoing Maintenance Hemodialysis.

OBJECTIVES: Maintenance hemodialysis (MHD) patients are at increased risk of contracting coronavirus disease 2019 (COVID-19). Vaccine against COVID-19 offers the benefit of protection from severe illness. In this study, we assessed the humoral response after two doses of the COVISHIELDTM vaccine in MHD patients. MATERIALS AND METHODS: In a prospective cohort study, the humoral response with two doses of the COVISHIELDTM vaccine was assessed after 14 ± 2 days of the second dose. The COVIPROTECT antibody titers against the spike protein were measured using the electrochemiluminescence immunoassay (ELECSYS, Roche Diagnostics International Ltd.). Data were analyzed to determine the predictors of antibody response. RESULTS: Between February and October 2021, 50 MHD patients were assessed. The mean age was 55.8 ± 10.8 years, and 72% were males. A total of 48 (96%) MHD patients have seropositivity. The median level of spike protein antibody was 579 U/mL [interquartile range (IQR25-75) 166-1852.75]. Compared to patients with no COVID-19 infection history, the median levels of antibodies were significantly higher in those with a history of COVID-19 (1047 vs 297 U/mL, p = 0.011). The antibody titers did not differ by age (p = 0.269), presence of comorbidities such as hypertension (p = 0.341), diabetes mellitus (p = 0.719) or ischemic heart disease (IHD) (p = 0.695), dialysis vintage (p = 0.660), and timing of diagnosis of COVID-19 in relation to vaccination (p = 0.261). Adverse events (AEs) occurred in one-third of patients that were mild and self-limiting. No serious AEs were observed in any patient. CONCLUSION: In MHD patients, two doses of the COVISHIELDTM vaccine induced a substantial humoral response. Prior history of COVID-19 resulted in a higher antibody response. Thus, the COVISHIELDTM vaccine is efficacious and safe for use in patients with MHD. How to cite this article: Balwani MR, Pasari AS, Bawankule C, et al. Humoral Response After Two Doses of COVISHIELDTM Vaccine in Patients Undergoing Maintenance Hemodialysis. J Assoc Physicians India 2023;71(9):56-60.

Spectrum of Biopsy-proven Native Kidney Disease in Central India.

Spectrum of native renal biopsy reports varies geographically. Here, we tried to determine the prevalence of renal biopsy disorders and compare it with other studies. Retrospective study was performed at Saraswati Kidney Care Center, Nagpur and Jawaharlal Nehru Medical College, Sawangi, India. All the native kidney biopsies from January 2017 to March 2020 were included in the analysis. Demographic details of all the patients were recorded. Renal diseases were classified as glomerular, tubulo-interstitial, predominant vascular involvement and other disease categories. Total 347 native kidney biopsies were performed during the study period. Mean age of the patients at the time of biopsy was 41.41 ± 15.75 years. Majority of patients were males (58.5%). Most common indication for kidney biopsy was nephrotic syndrome (36.3%) followed by nephritic syndrome (19.9%). Among the glomerular diseases (GDs), 69% were primary glomerulopathies and 31% were secondary GDs. Immunoglobulin (IgA) nephropathy (30.85%) was the most common primary GD followed by membranous nephropathy (MN) (26.59%), focal segmental glomerulosclerosis (FSGS) (17.02 %) and minimal change disease (14.36 %). Among secondary glomerulopathies, lupus nephritis was the most common histopathological diagnosis (31.8%) followed by diabetic nephropathy (26.1%), amyloidosis (17%), infection related glomerulonephritis (11.3%), light chain deposition disease (4%) and anti-neutrophil cytoplasmic antibody associated vasculitis (3.4%). In tubulointerstitial disease, 33.3% had acute tubulointerstitial nephritis, whereas each 26.6% had acute tubular injury and cast nephropathy. The most prevalent diagnosis in our only study from central India was IgA nephropathy followed by MN and FSGS. Data analysis at regular intervals helps in understanding the changing trend of prevalence of native kidney disease and also gives understanding of geographical variations.

Clinical and Histopathological Profile of Immunoglobulin A Nephropathy Patients in Central Part of India: A Single-Center Study.

The objective of the study was to assess clinical and histopathological profile of patients who were diagnosed as immunoglobulin A nephropathy (IgAN) on renal biopsy. Medical data were collected for this retrospective study at a single center from patients with biopsy-proven IgAN, from those biopsied between January 2017 and September 2020. A total of 347 renal biopsies were performed during the study. There were 52 patients with primary IgAN who met our inclusion criteria. Males were more commonly affected (61.5%). The mean age at the time of kidney biopsy was 35.26 ± 10.39 years. Hypertension was present in 84.5% of patients. Median serum creatinine and estimated glomerular filtration rate (eGFR) at presentation were 3.58 mg/dL and 15.8 mL/min/1.73 m2, respectively. Mesangial hypercellularity (M1), endocapillary hypercellularity (E1), segmental glomerulosclerosis (S1), tubular atrophy/ interstitial fibrosis (T1/T2), and crescents (C1/C2) were present in 46.2%, 38.5%, 88.5%, 75% and 36.6% of patients respectively. Thrombotic microangiopathy (TMA) and hypertensive vasculopathy were seen in 38.5% and 86.5% of patients respectively. The presence of tubular atrophy (T1/T2), hypertensive vasculopathy, and TMA on renal biopsy was significantly associated with low eGFR at presentation whereas no such correlation could be established with segmental glomerulosclerosis (S1), crescents (C1/C2), mesangial (M1) and endocapillary hypercellularity (E1). The presence of hypertensive vasculopathy and TMA on renal biopsy was associated with poor renal function at presentation. The most common clinical presentation of IgAN was hypertension and so we suggest patients with hypertension should be screened for microscopic dysmorphic hematuria and proteinuria, if present, should undergo a renal biopsy to diagnose this disease in early stages.

Anti-phospholipase A2 Receptor Antibody-Positive Membranous Glomerulopathy due to Anti-Koch's Therapy in a Tuberculosis Patient.

Tuberculosis (TB)-associated glomerulonephritis is difficult to diagnose that usually presents with hematuria, proteinuria, edema, hypertension, or renal insufficiency, which is similar to symptoms of primary glomerulonephritis. Membranous nephropathy (MN) is uncommonly seen in TB patients. We report a case of a 30-year-old female with Koch's chest who developed anti-phospholipase A2 receptor antibody-positive MN after initiation of anti-Koch's therapy and resolved after completion of anti-Koch's therapy.

Diabetic muscle infarction: An unusual cause of acute limb pain in patients on maintenance hemodialysis.

Diabetic muscle infarction is underdiagnosed complication occurring in dialysis patients with advanced diabetes mellitus. Atherosclerotic vascular disease and long-standing diabetes are risk factors for this painful condition. Most common presenting symptom is localized pain in the affected limb. We present here a case of muscle infarction occurring in a diabetic patient on maintenance hemodialysis (HD). Our patient had low-grade fever and pain in right thigh which restricted his movements for one month. His pain worsened during and post-HD. External examination of right lower limb was normal except for tenderness in the right thigh region. Laboratory examination showed leukocytosis with normal serum creatine phosphokinase levels. Magnetic resonance imaging of the thigh was suggestive of muscle infarction. Patient was treated with bed rest, analgesics, antiplatelets and blood transfusion. HD prescription was changed to sustained low-efficiency dialysis with reduced ultrafiltration. Gradually, in a week, his fever and pain subsided and he was able to walk on his own. Thus, it is important to identify this clinical condition early in the course of illness to further prevent its progression.

Heterozygous laminin ß2 mutation in C3 glomerulopathy.

C3 glomerulopathy is usually seen with the presence of C3 nephritic factor, homozygous or heterozygous mutations in the regulatory complement proteins factor H, factor I, or C3. We describe the presence of heterozygous laminin ß2 mutation in a patient of C3 glomerulonephritis with ocular and central nervous system involvement, the significance of which is unknown.

Knowledge about COVID-19 and Practices among Hemodialysis Technicians in the COVID-19 Pandemic Era.

INTRODUCTION: Hemodialysis technicians play a crucial role in infection control practices in hemodialysis units. Thus, it is important to assess the knowledge and attitude towards COVID-19 among hemodialysis technicians in this pandemic situation. MATERIALS AND METHODS: An online survey composed of 22 closed-ended questions using Google Forms was conducted in the month of April (13th to 19th) 2020. The survey consisted of questions regarding the knowledge of COVID-19 and current hemodialysis practice among hemodialysis technicians. The study was approved by the institutional ethics board. The survey was administered online through a mobile phone invitation. Basic statistics (mean and standard deviation or total number and percent) were computed for all covariates. RESULTS: Out of 150, 115 technicians participated in the survey. 80.9% of the participants were males. The mean age of respondents was 28.22 + 6.97 years. Most of the respondents could correctly identify fever (87.8%), breathlessness (86.08%), and dry cough (81.7%) as the symptoms of COVID-19 infection. 75.7% of the technicians were aware that it can be transmitted by asymptomatic persons. 61.1% of the technicians were segregating patients who had symptoms such as fever and cough to the last shift of the day. 81.1% of the technicians read the guidelines issued by the Indian Society of Nephrology-COVID-19 working group. But, only 25.5% of the respondents could rightly identify to keep a minimum distance of two meters between two beds while dialyzing a suspected patient of COVID-19 along with other patients to minimise risk of COVID-19 transmission. 60% of the technicians have received hydroxychloroquine as prophylaxis against coronavirus infection. CONCLUSION: Our study shows a significant knowledge gap among hemodialysis technicians about COVID-19. Effective COVID-19 education campaigns should be carried out intensively with relevant information among hemodialysis technicians to address the knowledge gap. A well-informed hemodialysis technician can prove to be a great tool to spread the right infection control practices among dialysis-dependent patients.

Hospitals may Become "Disease Hotspots" for COVID-19 amid Shortage of Personal Protective Equipment.

How to cite this article: Patel MP, Kute VB, Goswami J, Balwani MR. Hospitals may Become "Disease Hotspots" for COVID-19 amid Shortage of Personal Protective Equipment. Indian J Crit Care Med 2020;24(11):1145-1146.

Awareness about kidney and its related function/dysfunction in school going children: A survey from the Central India.

In the absence of a national registry, the exact incidence and burden of chronic kidney disease in children in India is not known. The most common diagnosis for which children are likely to see a pediatric nephrologist is nephrotic syndrome constituting almost 40% of cases. Considering all the renal manifestations in the pediatric age group, we designed simply survey to find out the awareness among school going children about the kidney's function and dysfunction in our Indian scenario. More than 95% of children knew that normally humans have two kidneys. Around 50% of school children on an average were not aware of normal location of their kidneys. About 60%-75% of school going children was unaware of all the functions of a normal kidney. More than half of school children had no idea that even one normal kidney was sufficient to lead a normal life. Again more than half of the participant children were unaware of the basic symptoms of the kidney failure. Around 8%-9% of students reported a positive family history of kidney disease in their family. Pediatric population, especially the school going students should be educated from their early years about the basics of any vital organ like the kidney. Here, in the present study, we found that there is the scope of improvement in making children aware of normal functions of a kidney and the abnormalities that occur when the kidneys are malfunctioning. Early diagnosis will lead to reduced kidney-related morbidity and mortality.

Minimal change disease and Kimura's disease responding to tacrolimus therapy.

Kimura's disease (KD) usually presents with a subcutaneous swelling and associated lymphadenopathy in the periauricular area. KD has a tendency to involve the kidneys. Proteinuria is reported in 12%-16% of cases, and around 60%-70% of them develop nephrotic range proteinuria. We are reporting a case of KD which developed around 12 years later in a patient of biopsy-proven steroid responsive minimal change disease. Recurrent swellings of KD and subnephrotic range proteinuria responded to low-dose tacrolimus therapy (0.05 mg/kg).

Widening spectrum of renal involvement in psoriasis: First reported case of C3 glomerulonephritis in a psoriatic patient.

Renal involvement in psoriasis is usually seen as mesangioproliferative glomerulonephritis (GN), IgA nephropathy, and focal segmental glomerulosclerosis. Microscopic hematuria is not uncommon in a patient of psoriasis with above-mentioned disorders. We found C3 GN as a cause when evaluated for macroscopic and persistent microscopic hematuria in a patient of psoriasis.

An initial evaluation of hypokalemia turned out distal renal tubular acidosis secondary to parathyroid adenoma.

Primary hyperparathyroidism (PHPT) usually presents with hypercalcemia related symptoms and signs. Kidneys play an important role in calcium homeostasis. PHPT has been reported to be associated with hyperchloremia, defective urinary acidification, and renal tubular acidosis (RTA). The dysfunction of distal renal tubules is proposed to be secondary to calcium deposition in distal tubules. This case report highlights an initial presentation of parathyroid adenoma as hypokalemia due to distal RTA secondary to medullary nephrocalcinosis.

An uncommon cause of rapidly progressive renal failure in a lupus patient: Pauci-immune crescentic glomerulonephritis.

We report a case of systemic lupus erythematosus (SLE) who presented with rapidly progressive renal failure (RPRF) with positive antinuclear antibody (ANA) and anti-double-stranded DNA (dsDNA) antibody and active urinary sediment in the form of microscopic hematuria and proteinuria. Provisional clinical diagnosis of lupus nephritis was made. Renal biopsy showed pauci-immune crescentic glomerulonephritis, the diagnosis of which was supported by positive serum anti-MPO antibody. Renal biopsy in SLE patients can sometimes reveal varied pathological entities such as antinuclear cytoplasmic antibodies (ANCAs) positive vasculitis, as in our case, which modified our treatment protocol. Thus, in a patient with SLE presenting with RPRF with active urinary sediments, ANCA serology, and renal biopsy with immunofluorescence examination should be performed always.

Scoring systems and outcome of chronic kidney disease patients admitted in intensive care units.

The outcome of chronic kidney disease (CKD) patients admitted to the Intensive Care Unit (ICU) is difficult to predict. This study assessed the outcome of CKD patients admitted to the ICU and evaluated prediction of 30-day mortality using the Acute Physiology and Chronic Health Evaluation (APACHE II), Simplified Acute Physiology Score (SAPS II), and Sequential Organ Failure Assessment (SOFA) score. One hundred consecutive CKD patients admitted to the ICU at a tertiary care hospital, Ahmedabad between 2011 and 2013 were included prospectively. Data on demographics, indication for admission, cause of CKD, use of vasoactive drugs and mechanical ventilation (MV), mode of renal replacement therapy (RRT), and 30-day mortality were recorded. The APACHE II, SAPS II, and SOFA scores were calculated based on the admission characteristics. The mean APACHE II, SAPS II, and SOFA scores were 28.22 ± 7.53, 43.04 ± 16.40, and 10.39 ± 5.20, respectively, and area under receiver operating characteristics curve in predicting 30-day mortality were 0.961, 0.994, and 0.950, respectively. The scores were significantly higher in 30-day nonsurvivors as compared to survivors (P = 0.001). During the ICU stay, MV and vasoactive drugs were required in 57% and 67% of the patients, respectively, and the requirement was significantly greater in nonsurvivors as compared to survivors (P = 0.001). About 85% of patients were on intermittent hemodialysis and 15% of patients were on continuous venovenous hemodiafiltration. Sepsis was the main reason for hospital admission, and the mean length of stay in the ICU was 7.74 ± 5.34 days. The study indicates that all three scores (APACHE II, SAPS II, and SOFA) perform equally well and have equal diagnostic utility in predicting 30-day mortality.

Primary Renal Echinococcosis.

Echinococcosis is a parasitic infection caused by the larval stage of a cestode Echinococcus granulosus and is endemic in sheep farming regions of developing countries. It manifests as hydatid cyst and most commonly is found in liver followed by lungs. Renal hydatid cyst is rare and amounts for 2% of all cases. There are no specific clinical manifestations, and hence diagnosis of renal hydatid disease is missed out easily without imaging. We report a case of 50-year-old female who had 6 months history of lower abdominal pain with hematuria, found to have right renal hydatid cyst on imaging which was treated with right nephrectomy with pre- and post-operative albendazole treatment.