Clinicopathological Study of Males with Lupus Nephritis: Pathologist's Experience at a Tertiary-Care Center.

Background: Systemic lupus erythematosus (SLE) is an autoimmune systemic disorder, more common in females of reproductive age-group as compared with males. There are very few studies regarding lupus nephritis (LN) in males. Hence, we decided to study the clinical and pathological findings of LN in males. Materials and Methods: We carried out a retrospective study over a period of 5 years (January 2014-December 2018) on indicated native renal biopsies from male patients with LN. We analyzed the clinical, laboratory, and histological findings of these patients. Results: Renal biopsies were performed on 228 patients with LN, of which 29 (12.72%) biopsies were in male patients. The mean age at presentation was 28.3 ± 12.98 years. Edema (65.5%) was the most common clinical feature followed by arthritis (27.58%), fever (27.58%), and skin rash (24.1%). The mean values for 24 hours urinary protein, serum double-stranded DNA, serum antinuclear antibody, and serum complement C3 were 4.98 ± 2.91 g, 137.7 ± 91.93 IU/mL, 2.96 ± 1.78, and 65.07 ± 36.30 mg/dL, respectively. On histology, the most common class of LN was Class IV (34.48%) followed by Class V (20.68%), combined Class IV + V (20.68%), Classes II, III, and III + V. Conclusion: LN can affect males, although the prevalence is lower than in females. The incidence of LN in our study was 12.7% with the most common histological class being diffuse proliferative LN.

Anti-phospholipase A2 Receptor Antibody in Differentiation and Prognostication of Membranous Nephropathy.

Anti-M-type phospholipase A2 receptor (anti-PLA2R) antibody is believed to be associated with primary membranous nephropathy (pMN) and absent in secondary MN (sMN). There are few data regarding utility of anti-PLA2R antibody as a prognosticator. Our study aimed to compare the incidence of positive serum anti-PLA2R antibody titer in pMN versus sMN and correlation with clinical outcome. From August 2015 to July 2019, patients with biopsy-proven MN were evaluated for serum anti-PLA2R antibody titers by the enzyme-linked immunosorbent assay. The subset of cases was repeated to monitor the clinical response in terms of 24 h proteinuria. A total of 169 patients, 65 pMN and 104 sMN were studied. Anti-PLA2R antibody was found in 41 (63.08%) pMN with mean titer, 232.62 RU/mL, and 40 (38.46%) sMN with mean titer 253.59 RU/mL. Out of positive antiPLA2R antibody titer in pMN cases, 15 were retested twice to 5 times with mean titers of 78.95, 36.27, 13.9, and 15.45 RU/mL, respectively. Out of positive anti-PLA2R antibody in sMN cases, 11 were retested twice to five times with mean titers of 104.42, 122.49, 12.33, and 17.2 RU/mL, respectively. All patients with decreasing anti-PLA2R antibody titer in both groups had clinical remission, with a decrease in mean 24 h proteinuria from 7.11 g to 3.36 g in pMN and 5.97 g to 3.41 g in sMN. Ten pMN and 11 sMN patients without remission showed persistent positive anti- PLA2R antibody titer. Anti-PLA2R antibody titer may be elevated in pMN/sMN. It can also be used as a noninvasive prognostic marker for MN.

Ten-year appraisal of pediatric renal allograft biopsies: Points to ponder.

There is paucity of literature on pediatric renal allograft biopsy (RAB) evaluation. We present RAB findings of pediatric renal transplantation (RT) and correlate with outcome. This is a 10-year retrospective study of diagnostic RAB of children <12 years divided in to three groups: Group 1 (n = 9): less than haplo-match living donor RT (LDRT), Group 2 (n = 32): greater than or equal to haplo-match LDRT, and Group 3 (n = 7): deceased donor RT. Demographics, biopsy findings, survival, and serum creatinine (SCr) were evaluated. Statistical analysis was performed using IBM SPSS Statistics version 20.0. The most common findings were antibody-mediated rejection (ABMR) observed in 77.7%, 45%, and 71.5% and T-cell-mediated rejections (TCMRs) in 33.3%, 52.5%, and 42.9% in Groups 1, 2, and 3, respectively. Recurrent oxalosis was seen in 5% in Group 2. Death-censored graft survival was 100% at 1 year and 43.8% from 5 to 9 years in Group 1; 93.5%, 76.6%, 56.5%, and 14.4% at 1, 5, 10, and 15 years in Group 2; 100% at one year; and 71.4% from 5 to 12 years in Group 3. No patient appeared after 9 years in Group 1 and after 12 years in Group 3. In Group 1, the mean SCr (mg/dL) was 1.06 ± 0.45, 2.12 ± 1.87, and 1.39 at 1, 5, and 9 years; 1.35 ± 0.97, 1.73 ± 1.15, and 2.49 ± 1.64 in Group 2; and 1.15 ± 1.24, 1.43 ± 0.1, and 1.18 ± 0.06, respectively, in Group 3 at 1, 5, and 10 years posttransplant. ABMR followed by TCMR was the most common injury in all the groups. Group 1 had more rejections than others.

Thrombotic microangiopathy in a renal allograft: Single-center five-year experience.

Thrombotic microangiopathy (TMA) is devastating for renal transplantation (RT) causing graft/ patient loss. We present 5-year experience of TMA in RT in retrospective study of indicated renal allograft biopsies with TMA. Patient-donor demographics and associated histological findings with respect to transplants under tolerance induction protocol (Group 1) were compared with patients transplanted under triple immunosuppression (Group 2). Statistical analysis was performed using IBM SPSS Statistics version 20. Sixty-one (4.1%) of 1520 biopsies [Group 1:17 (1.9%)/882, Group 2:44 (6.9%)/638] revealed TMA. Tacrolimus trough levels were normal. There was no evidence of systemic involvement in any patient. Mean age was 36.8 years with 70.6% males, HLA-match, 2.6/6, and the most common original disease unknown (41.2%) in Group 1, and 35.9 years with 86.4% males, HLA-match, 2.1/6, and the most common original disease unknown (50%) in Group 2. Biopsies were performed at mean 5.1-year posttransplant in Group 1 and 2.3 years in Group 2. Acute TMA constituted 47% Group 1 and 43.2% Group 2 biopsies; of these, antibody-mediated rejections were observed in 58.8%, T-cell mediated rejections in 11.8%, tacrolimus toxicity in 76.5%, and other findings in 35.3% Group 1; and 61.4%, 25%, 50%, and 18.2%, respectively, in Group 2 biopsies. Higher rejection activity scores were more in Group 2. Postbiopsy 1- and 5- year patient survival was 94.1%, 86.9% in Group 1 and 92.1%, 88.3% in Group 2; 1- and 4-year graft survival was 52.9%, 15.9% in Group 1 and 20.3%, 5.4% in Group 2. TMA was poor prognosticator for RT, especially under triple immunosuppression. Antibody- mediated rejection and tacrolimus toxicity were more prone to TMA.

C4d-negative antibody-mediated rejection: A pathologist's perspective and clinical outcome.

Banff'13 update included C4d-antibody-mediated rejection (ABMR) as a separate entity responsible for graft dysfunction with limited clinical/prognostic implications. We present a retrospective study to determine the incidence and outcome of C4d-negative ABMR. A total of 987 renal allograft (RA) biopsies obtained from 987 RA recipients were studied from January 2013 to January 2016. All samples were subjected to light microscopy using standard stains and C4d immunohistochemistry on paraffin sections and reported according to modified Banff's criteria. Adequate biopsies with immunological injuries were categorized as Group 1: pure ABMR, Group 2: combined ABMR with concurrent T-cell-mediated rejection (TCR), and Group 3: pure TCR. Groups 1 and 2 were further subgrouped as C4d positive (Group 1a and 2a) or C4d negative (Group 1b and 2b). Graft function was measured by serum creatinine (SCr) level (mg/dL). Of the 987 biopsies, 43.3% (404) biopsies revealed immunological injury. Of these, 27.7% of the biopsies revealed pure ABMR (Group 1), 60.6% revealed combined ABMR with TCR (Group 2), and 11.3% revealed pure TCR (Group 3). The overall incidence of ABMR (pure ABMR + ABMR with TCR) was 36.27%, of which C4d-negative rejections were 18.48% and 18.7% in Group 1 and Group 2, respectively. The mean SCr at the end of three years follow-up in patients with C4d-negative rejections was comparatively higher. C4d-negative ABMR, recently included in Banff'13, has a low incidence, usually presents early after transplantation but carries better outcome than C4d-positive ABMR. However, further long-term studies are still required for knowing the clinical course over years.

Urinary Screening for Early Detection of Kidney Diseases.

OBJECTIVE: Urinary screening is a simple inexpensive tool to evaluate kidney functions. The authors carried out urinary screening of school children for early detection of kidney diseases. METHODS: Children in the age group 5-15 y were screened for urinalysis. They were divided in 2 groups; group-1 included 5-10 y and group-2 included >10-15 y old children. RESULTS: Urine samples of 3340(78%) out of 4283 enrolled children were tested. Abnormal samples were found in 5.75%; with proteinuria in 4.59%, pyuria in 3.29% and hematuria in 4.31%. Males constituted 47.71% in group-1 and 54.64% in group-2. Low body mass index was found in 94.1% group-1 and 78.99% group-2 children. Mild proteinuria was found in 1.2% group-1 and 2.56% group-2 children. Severe proteinuria was more in group-2 (0.77% vs. 0.06%) with female preponderance. Glucosuria was found in 1 boy of group-2. Urobilinogen was more in group-2 (0.65% vs. 0.24%) with male preponderance. Nitrituria was found in 9 girls. Pyuria (2.02% vs. 1.27%) and hematuria were more in group-2 (3.04% vs. 1.87%) with female preponderance. Combined proteinuria and hematuria (0.42% vs. 0.24%) as well bacteruria and fungaluria were more in group-2 (4.11% vs. 1.39%). Six of 192 children with abnormal urinary findings were treated; 1 for urinary calculus and 5 for urinary tract infection. CONCLUSIONS: Abnormal urinary findings were more common in children >10 y of age. Thus urinary screening program of children can become useful for early detection of kidney diseases and contribute towards building up of a healthy nation.

Clinicopathological study of nondiabetic renal disease in type 2 diabetic patients: A single center experience from India.

Diabetic nephropathy (DN) is a major complication of diabetes mellitus (DM), leading to chronic kidney disease/end-stage renal disease. Wide spectrum of nondiabetic renal diseases (NDRD) is reported in type-2 diabetes (type-2 DM). We carried out this single-center study to find clinical, laboratory, and histological features of NDRD in type-2 DM patients and to assess the prevalence of NDRD in India. A single-center retrospective study which included analysis of renal biopsies from patients with type-2 DM, performed between January 2008 and September 2016. Biopsy findings were categorized into three groups, Group-I (isolated NDRD); Group-II (NDRD superimposed on underlying DN); and Group-III (isolated DN). Out of 152 diabetic patients (111 males and 41 females), 35 (23.03%) patients were of Group-I (isolated NDRD), 35 (23.03%) of Group-II (NDRD superimposed on underlying DN), and 82 (53.95%) of Group-III (isolated DN). The mean age (in years) was 55.08 ± 10.71, 55.65 ± 8.71, and 54.45 ± 9.01 respectively in Group-I, II, and III. Nephrotic syndrome (NS) was the most common clinical presentation in all groups. Duration of DM was significantly shorter in Group-I than in Group-II. Diabetic retinopathy was absent in Group-I. Proteinuria was more in Group-III than Group-I. Low serum C3 and/or C4 levels was observed in five (14.29%) cases of Group-I and Group-II each and two (2.43%) cases of Group-III. Nearly, 70 (46.05%) patients were found to have NDRD either in isolated form or as combined lesions. The most common histological types of NDRD were acute tubulointerstitial nephritis (38.57%) followed by benign nephrosclerosis (15.72%), membranous nephropathy (10%), IgA nephropathy (7.14%), and membranoproliferative glomerulonephritis (7.14%). The incidence of NDRD (with/without DN) in type-2 DM is very high. Shorter duration of diabetes, hematuria, absence of retinopathy, low serum complement levels, and nephrotic range proteinuria are predictors of NDRD.

Repercussions of eosinophils in a renal allograft - Predictor of early graft loss!

We present 5-year experience of renal transplantation (RT) with tissue eosinophilia (TE) in renal allograft biopsy (RAB) and its repercussions on the outcome. In total, 1217 recipients underwent RT from 2011 to 2015, and they were evaluated for the presence of ≥4% TE. Group 1 consisted of RT with RAB showing TE, Group 2 consisted of RT with RAB with rejections without TE, and Group 3 consisted of RT without rejections. Group 1 had 27 recipients, Group 2 had 395, and Group 3 had 795 recipients. The outcome in terms of graft function, patient and graft survival were evaluated and compared between three groups. All recipients received standard triple immunosuppression. One-year patient and death-censored graft survival were 80.7% and 82.7% in Group 1, 87.2% and 95.1% in Group 2, and 92.6% and 99.6%, respectively in Group 3 and corresponding mean serum creatinine (SCr, mg/dL) was 1.60 ± 0.45 in Group 1, 1.63 ± 0.58 in Group 2, and 1.19 ± 0.39 Group three, respectively. Five-year patient and death-censored graft survival were 72.9 % and 71.1% for Group 2 and 87% and 98.2% for Group 3 with SCr of 1.63 ± 0.38 and 1.25 ± 0.4, respectively. Group 1 recipients did not appear at five years. At four years posttransplant, patient and death-censored graft survival were 71.7% and 59.5% in Group 1 with SCr of 1.55 ± 0.65 mg/dL. In conclusion, the presence of eosino-phils in a renal allograft is an impending sign of graft damage and eventual graft loss.

De Novo Focal Segmental Glomerulosclerosis in Renal Allograft-Histological Presentation and Clinical Correlation: Single Centre Experience.

INTRODUCTION: Recurrent or de novo glomerulonephritis are one of the well-known causes for renal allograft dysfunction in early and late period after renal transplantation. Focal Segmental Glomerulosclerosis (FSGS) is a devastating lesion of the renal allograft. De novo FSGS is uncommon compared to recurrent FSGS. AIM: To find out the incidence of de novo FSGS. MATERIALS AND METHODS: A retrospective evaluation of renal allograft biopsies was performed from 2007 to 2015, by light microscopy and immunohistochemistry including patient-donor demographics. Graft function status in terms of serum creatinine (SCr) and proteinuria were evaluated. RESULTS: Out of 2,599 renal allograft biopsies performed, 1.6% biopsies were reported as de novo FSGS. Majority were live related females donors with mean age of 43.8 years. Mean time of biopsy was 1.1 years post-transplant with proteinuria of 2.95 grams/24 hours and SCr of 2.24 mg/dL. Histopathological variants were collapsing 47.6%, Not Otherwise Specified/ classical 35.7%, cellular 9.5% and perihilar 7.1% biopsies. Associated Antibody Mediated Rejection (AMR) with T-Cell Rejection (TCR) was observed in 35.7% biopsies, acute on chronic CNI toxicity (calcineurin inhibitor) in five biopsies. Majority of the patients were on CNI based maintenance immunosuppression regimen. Total 28.6% patients and 23.8% grafts were lost over a mean follow up of 2.40 years. The mean SCr of remaining patients was 1.98 mg/dL. CONCLUSION: De novo FSGS can occur after the first year of renal transplant with related Human Leukocyte Antigen (HLA)matched donors leading to poor allograft survival. Close monitoring of urinary proteinuria and evaluation of allograft biopsy help in appropriate therapeutic modification to improve long term outcome of graft function.

Invasive fungal infections in renal transplant patients: a single center study.

BACKGROUND: Timely diagnosis of invasive fungal infections (IFI) in renal transplant (RT) patients on immunosuppression is often difficult, jeopardizing their life and graft. We reported IFI and their causative fungal agents in post-RT patients. MATERIALS AND METHODS: This was a retrospective 6-year clinical study carried out from 2010 to 2015 on 1900 RT patients. Clinical data included patient-donor demographics, time to onset of infection, risk factors and graft function in terms of serum creatinine (SCr). To identify IFI, we examined bronchoalveolar lavage (BAL), blood, tissue, and wound swab samples by conventional mycological methods. RESULTS: IFI were diagnosed in 30 (1.56%) patients on triple immunosuppression, mainly males (n = 25) with mean age of 36.57 ± 11.9 years at 13.12 ± 18.35 months post-RT. Aspergillus species was identified in 11 BAL, one tissue, and one wound specimen each, 30.76% of these were fatal and 15.38% caused graft loss; Candida albicans was in nine BAL, four blood, two wound swab, and one tissue specimens, 25% of these were fatal and 25% had graft loss and one mucor in BAL which was fatal. Seven patients were diabetic, 10 had superadded cytomegalovirus infection, and 15 were anti-rejected. CONCLUSION: IFI are associated with increased morbidity and mortality in RT patients. Triple immunosuppression, broad spectrum antibiotics for ≥ two weeks, diabetes and superadded infection are added risks for these patients. Prevention, early diagnosis, and appropriate management are necessary to improve their prognosis.

Persistent mullerian duct syndrome presenting as retractile testis with hypospadias: A rare entity.

A rare entity of persistent mullerian duct syndrome usually presents with a common symptom of undescended testis (UDT) or hernia. Male pseudo-hermaphroditism with persistent internal mullerian duct structures can present with a 46, XY karyotype with normal external genitalia and. It arises due to deficiency of anti-mullerian substance, resulting from reduced production/responsiveness to mullerian duct, leading to persistence of mullerian duct along with normal development of Wolffian duct structures. Presence of mullerian structure prevents testicular descent increasing the risk of testicular vanishing syndrome. The authors here report a case of 16 years old phenotypical male who came with retractile right sided testis and left side UDT in the urology out-patient department. Explorative laparotomy was performed and an ill-defined mass was excised and sent for histopathological examination. Histopathology revealed presence of mullerian structures. The serum testosterone level was normal, buccal smear cytology and karyotyping revealed a 46, XY genotype of the patient.

Collapsing Glomerulopathy: A Single Centre Clinicopathologic Study of Seven Years.

INTRODUCTION: Collapsing Glomerulopathy (CG) is recognized as distinct pattern of proliferative parenchymal injury with poor response to empirical therapy. AIM: A single center retrospective study was carried out to find out clinicopathological features of idiopathic CG. MATERIALS AND METHODS: A total of 3335 native renal biopsies were analyzed retrospectively which were performed from 2008 to 2014 with emphasis on clinicopathological correlation and histopathological presentation. RESULTS: Idiopathic CG constituted 0.75% incidence (25 out of 3335 biopsies) of all biopsies, adults constituting major study part with 88%. The duration of the symptoms at the time of biopsy was 34.12±26.09 days and 35±22.91 days respectively in adults and children. Hypertension was noted in 9(40.9%) and oliguria in 8(36.4%) in adults. Urinalysis revealed microscopic haematuria 12(54.5%) in adults. Nephrotic range proteinuria was reported in 10 (45.5%) adult patients. Glomerular collapse with hyperplasia/ hypertrophy of podocytes was seen in 4.54±3.11 glomeruli. Tubular microcystic dilation was seen in 16(64%) patients. Tubular atrophy involving mild (t1) in 15(60%), moderate (t2) in 4(16%) and severe (t3) in 6(24%) patients. Interstitial fibrosis was mild (i1) in 17(68%), moderate (i2) in 2(8%) and severe (i3) in 6(24%) patients. CONCLUSION: Idiopathic CG is a morphological pattern of grave podocyte injury with poor prognosis. However, there are chances of remission/ recovery if the tubular atrophy and interstitial fibrosis are of grades ≤ t1 i1.

Renal Myxoma- A Rare Variety of Benign Genitourinary Tumour.

Renal myxomas are rare neoplasms and very few cases have been reported in literature. Here we report a renal myxoma in a 48-year-old lady with hypothyroidism who presented with abdominal pain. She was found to have a mass lesion of 67 x 61 x 74 mm with exophytic component in the right kidney on ultrasonography and computed tomography. Right radical nephrectomy was performed due to suspicion of malignancy. Grossly the resected kidney showed a gelatinous, semi-translucent mass involving the mid and lower poles. Microscopy revealed marked hypocellular appearance of loose myxoid tissue with foamy histiocytes. Tumour cells were reactive for vimentin. At eight months of follow-up, patient is doing well.

Pediatric Renal Biopsies in India: A Single-Centre Experience of Six Years.

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.

Acute Kidney Injury and Quadriparesis Due to Rosuvastatin Induced Rhabdomyolysis- A Case Report.

Statins as lipid lowering drugs, are safe and effective in reducing cardiovascular disease risk, but rarely produce myopathy like myalgia, myositis or rhabdomyolysis. We report the case of Rosuvastatin induced rhabdomyolytic acute renal failure and quadriparesis in a 67-year old male, a known case of type-2 diabetes mellitus and with a history of coronary angioplasty four months back. He was on antihypertensive, oral hypoglycemic and antiplatelet medications with Rosuvastatin 40mg/day. He was admitted with altered sensorium, breathlessness, vomiting, muscle weakness and decreased urine output and had raised serum creatinine, creatinine phosphokinase and myoglobin. After ruling out all other causation for rhabdomyolysis, we stopped Rosuvastatin and started supportive management and hemodialysis. Patient showed gradual recovery in renal function and quadriparesis. Patient was discharged with good urine output and on antihypertensive, hypoglycemic drug and diet restrictions for lipid control. He recovered completely and had normal renal function with well controlled lipid level on follow up of 6 months after discharge. Thus, prompt diagnosis of Rhabdomyolysis due to Rosuvastatin in absence of other aetiology and the multidisciplinary management can prevent further complication with favorable outcome.

Diagnosis and management of atypical hemolytic uremic syndrome in children: single centre experience.

Atypical hemolytic uremic syndrome (aHUS) although rare is the commonest cause of acute renal failure (ARF) in children and has poor prognosis. We present single centre experience of aHUS. Thirty six children (29 males, 7 females) with mean age, 7.9 years presented with ARF, 2 children also had tonic-clonic type convulsions. Their hematology examination revealed hemolytic anemia with s. creatinine (SCr), 5.54 mg/dl. Acute HUS was observed in 75 %, acute on chronic HUS in 19.4 % and patchy cortical necrosis (PCN) in 5.6 % biopsies. Mean 5.4 plasma exchanges (PE) were carried out. Supportive management of anti-hypertensives and prednisone was also given. Recovery end points were establishment of urine output, improvement of SCr and hematological profile. Hematology and renal function profile improved variably in all children, 5.6 % died, relapse was observed in 80.5 % over mean 70 days; 13.9 % children are doing well over mean follow-up of 268.8 days. Thus poor prognosis was observed in 86.1 % children. Children with acute on chronic HUS and PCN did not recover. Six children who recovered had acute HUS. aHUS in Indian children occurs at an older age of around 8 years and chronic/irreversible changes on histopathology examination are harbingers of poor prognosis. PE is life-saving however further research for developing strategies to improve long-term survival is needed.

Hemolytic uremic syndrome in laboratory medicine: single centre experience in children.

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS), although rare, is the most common cause of acute renal failure (ARF) in children and has poor prognosis. We present a single centre experience of aHUS. METHODS: Thirty six children (29 males, 7 females), with mean age 7.9 years, presented with ARF, 2 children also had tonic-clonic type convulsions. Their hematology examination revealed hemolytic anemia with serum create-nine (SCr) of 5.54 mg/dL. Acute HUS was observed in 75%, acute-on-chronic HUS in 19.4%, and patchy cortical necrosis (PCN) in 5.6% biopsies. A mean of 5.4 plasma exchanges (PE) were carried out. Supportive management of anti-hypertensives and prednisone was also given. Recovery end points were establishment of urine output and improvement of SCr and hematological profile. RESULTS: Hematology and renal function profile improved variably in all children, 5.6% died, relapse was observed in 80.5% over a mean of 70 days; 13.9% children are doing well over a mean follow-up of 268.8 days. Thus, poor prognosis was observed in 86.1% children. Children with acute or chronic HUS and PCN did not recover. Six children who recovered had acute HUS. CONCLUSIONS: aHUS in Indian children occurs at an older age of around 8 years and chronic/irreversible changes on histopathology examination are harbingers of poor prognosis. PE is life-saving; however, further research for developing strategies to improve long-term survival is needed.

IgM nephropathy in India: a single centre experience.

OBJECTIVE: To find out the incidence and natural history of IgMN in India. METHODS: Renal biopsies of children ≤12 y age of last 6 y were retrospectively evaluated. Their clinical and biochemical presentation were correlated. Patients with systemic diseases/disorders were excluded from the study. RESULTS: Immunoglobulin M nephropathy (IgMN) constituted 11.9% (n = 28) of 236 renal biopsies. Mean age was 10 y, predominant in boys (n = 24), most of the patients presented with proteinuria and edema. The most common associated histopathological finding was mesangial proliferative glomerulonephritis (MePGN) in 60.7% (n = 17) followed by minimal change disease (MCD) in 28.6% (n = 8) and focal segmental glomerulosclerosis (FSGS) in 10.7% (n = 3). In 85.7%, IgM appeared as the sole immunoglobulin deposit mainly in mesangial regions, followed by accompanied C3 in 3.6% (n = 1) and C1q + C3 in 10.7% (n = 3) biopsies. CONCLUSIONS: IgMN was observed in 11.9% biopsies with commonest morphology of MePGN followed by MCD and FSGS; proteinuria was bad prognosticator in addition to FSGS and co-deposition of other immunoglobulins had no significance.

Desensitization protocol for highly sensitized renal transplant patients: a single-center experience.

Highly sensitized patients are destined to remain untransplanted for long. Early transplantation results in cost-saving, reduced morbidity/mortality and improved quality of life. We carried out a prospective study to evaluate the efficacy and safety of desensitization protocol vis-à-vis patient/graft survival in living donor renal transplantation in highly sensitized patients. Between December 2008 and April 2010, 34 renal transplant (RTx) patients underwent desensitization protocol. An anti-human globulin-enhanced lymphocytotoxicity crossmatch assay (AHG-CDC) ≥25% and T-cell median channel shift (MCS) >50, B-cell MCS >100 [flow crossmatch (FXM)] were considered crossmatch (XM) positive. All patients were administered bortezomib (1.3 mg/m 2 , days 1, 4, 8, 11), plasmapheresis, rabbit-anti-thymocyte globulin (r-ATG), mycophenolate mofetil (MMF) and intravenous immunoglobulins (IVIg). LCXM and FXM were repeated post-protocol. In the event of persistent sensitization, additional bortezomib cycle was repeated along with plasmapheresis, IVIg, calcineurin inhibitors (CNI) and rituximab. If the cross match (CMX) was negative or acceptable, patients underwent RTx. Post-transplant immunosuppression consisted of prednisone, CNI and MMF. Biopsy was performed in the event of graft dysfunction and treated accordingly. There were 18 males and 16 females, with a mean age of 37.4 years. Mean dialysis duration was 14.9 ± 17.6 months. Average third party transfusions were 6.2 ± 4.5, 17.6% had autoimmune diseases, 20.6% were multi-para. Pre-protocol AHGXM was 55.3 ± 24.5%, T-cell crossmatch (TCXM) was 122.4 ± 91.4 MCS and B-cell crossmatch (BCXM) was 279 ± 142.9 MCS. Totally, 85.3% responded within 1 month with reduction in AHG-CDC to 19.9 ± 5.2%, TCXM to 24.7 ± 19.4 MCS and BCXM to 74.7 ± 34.8 MCS. Side effects noted in 38.2% were manageable. Over follow-up of 0.92 ± 0.8 years, patient/graft survival was 100%/88.2% and mean serum creatinine was 1.27 ± 0.32 mg/dL. Acute rejections were noted in 24.1%, who responded to steroids + rabbit antithymocyte globulin (rATG). Five (14.7%) patients were transplanted after changing donors. Our desensitization protocol seems to be safe and effective. Bortezomib may offer new possibilities in desensitization protocols.