C1q Nephropathy in a Patient of Neurofibromatosis Type 1: A Rare Case Report.

C1q nephropathy is a rare glomerular disease defined by the presence of characteristic mesangial dominant or codominant C1q deposition on immunofluorescence microscopy. Neurofibromatosis type 1 (NF-1) is an autosomal dominant syndrome caused by a mutation of a gene located on chromosomal segment 17q11.2. Nephrotic syndrome has rarely been reported in patients of NF-1, and the relation of NF-1 with nephrotic syndrome is unclear. Here, we present a rare case of C1q nephropathy in a patient of NF-1.

Collapsing Glomerulopathy- A Troublemaker for the Renal Allograft: Lessons Learnt.

Collapsing glomerulopathy (CG) is a well-recognized distinct morphological pattern of proliferative parenchymal injury leading to rapid graft failure. We conducted a single-center retrospective study to evaluate the prevalence, clinicopathological features, and prognosis of CG in renal transplant recepient. We analyzed 2518 renal allograft biopsies performed from 2007 to 2015 and correlated their clinicopathological features. The prevalence of CG was 0.83% (21 out of 2518) of allograft biopsies with a higher prevalence of 1.4% during the period from 2012 to 2015. Out of 21 patients, 18 (85.71%) patients had undergone live donor and 3 (14.28%) patients had undergone deceased donor renal transplant. Hypertension was observed in 3 (14.28%) patients. The mean duration of diagnosis for CG was 1.85 ± 1.91 years. Urinalysis revealed microhematuria in 5 (23.8%) patients. The mean 24 h urinary protein excretion was 4.77 ± 5.3 g and serum creatinine was 2.12 ± 1.5 mg/dl. The predominant native kidney diseases in recipients were chronic glomerulonephritis of unknown etiology in 12 (57.14%) patients and hypertensive nephropathy in 3 (14.28%) patients. CG was associated with rejection in 9 (42.85%), calcineurin-inhibitor toxicity in 2 (9.5%), and BK virus nephropathy in 1 patient. All patients received standard triple immunosuppression. Eleven (52.38%) patients developed graft failure over a mean period of 2.2 ± 1.7 years and 6 (28.57%) patients recovered with stable graft function. CG can coexist with viral infection, drug toxicity, rejection, microvascular injury, etc. CG usually presents with moderate to severe proteinuria and may lead to rapid graft dysfunction and subsequent graft failure in most of the patients.

De Novo Collapsing Glomerulopathy in Renal Allograft in Association with BK Virus Nephropathy in a Child and Stabilization of Renal Function by Elimination of Viremia.

Well-recognized association between HIV 1 infection and collapsing glomerulopathy (CG) raises the possibility that intrarenal infection by other viruses may also contribute to the development of this lesion in native or post-transplant kidneys. There is evidence in literature about association of these lesions with cytomegalovirus, Epstein-Barr virus, hepatitis C virus, and parvovirus B19 infections. Here, we present a case report of post-transplant BK virus nephropathy in a male child who was found to have CG in subsequent biopsy 2 months later. His renal function and proteinuria were stabilized on elimination of viremia.

The Learning Curve of Pure Retroperitoneoscopic Donor Nephrectomy.

BACKGROUND: Retroperitoneoscopic donor nephrectomy (RDN) is a well-established modality for the procurement of kidneys for renal transplantation. However the learning curve of pure RDN is not yet defined. Defining the learning curve will help in proper mentorship of the new donor surgeons besides providing safety to the donors. OBJECTIVE: To define the learning curve of pure RDN. METHODS: We analyzed the prospectively collected data of 102 voluntary kidney donors who underwent RDN by a single surgeon between August 2012 and April 2015 at our center. The donors were classified into group A (1-34), group B (35-68), and group C (69-102) according to the chronological order of their surgery. Left RDN was performed in 28 (82%), 25 (74%), and 28 (82%) donors of group A, B, and C, respectively. Right RDN was performed in 6 (18%), 9 (26%), and 6 (18%) donors of group A, B, and C, respectively. The clinical data were analyzed for each group. RESULTS: Statistically significant difference was observed for the mean operative time (p<0.01) and warm ischemia time (p<0.04). The operative time remained around 200 minutes after the initial 35 cases. CONCLUSION: The learning curve of pure RDN was 35 cases, although the mastery requires more number of cases to be performed.

Buccal Mucosal Graft Urethroplasty in Patients Awaiting Renal Transplantation.

OBJECTIVE: To assess the perioperative morbidity and early outcome of buccal mucosal graft (BMG) urethroplasty in patients with urethral stricture awaiting renal transplantation. METHODS: Thirteen patients awaiting renal transplantation underwent BMG urethroplasty for long anterior urethral stricture between June 2011 and March 2013. The management issues, complications, and outcome of the BMG urethroplasty in this cohort of patients were studied. RESULTS: Mean age of the patient was 38.7 ± 12.7 years. History of urethral manipulation was present in 8 patients. Mean stricture length was 6.92 ± 2.90 cm. Mean serum creatinine of the patient was 8.1 ± 3.6 mg%. Three patients required oral exploration for bleeding. Two patients had urinary extravasation, 3 patients had infected hematoma, and 1 patient developed dry gangrene of the glans. One patient had sepsis due to pyonephrosis in the postoperative period and succumbed to it. Mean follow-up of the patients was 34.54 ± 6.46 months. Three patients underwent VIU for recurrence of the stricture in the follow-up. At 3-month follow-up mean Qmax was 23.8 mL/sec, whereas at 6-month and 1-year follow-up, Qmax was 23.6 and 23.4 mL/sec, respectively. CONCLUSION: This study shows a relatively higher complication rate of urethroplasty in prerenal transplant patients. Although the number of cases is too small to arrive at any definite conclusion, this study does gives an insight into the management issues, complications, and success of urethroplasty in this group of patients.

Crescentic Glomerulonephritis Associated with Pulmonary Tuberculosis.

Tuberculosis of kidney and urinary tract is caused by members of the Mycobacterium tuberculosis complex. Kidney is usually infected by haematogenous spread of bacilli from focus of infection in the lungs. Glomerular involvement in tuberculosis presenting as a rapidly progressive glomerulonephritis is a rare entity. We report a rare case of crescentic glomerulonephritis associated with pulmonary tuberculosis in a 26-year-old man. Patient was treated with corticosteroids, haemodialysis, intravenous immunoglobulin and four cycles of plasmapheresis. He did not respond to 4-drug anti-tuberculosis treatment for renal pathology and was switched over to maintenance haemodialysis. However, he responded to pulmonary TB.

Histological and Clinicopathological Evaluation of Liver Allograft Biopsy: An Initial Experience of Fifty Six Biopsies.

INTRODUCTION: Liver biopsy is gold standard for diagnosis of allograft dysfunction. AIM: The aim of study was to evaluate liver allograft biopsies performed for graft dysfunction, study the pattern of injury and intensity, and timeline of occurrence of graft dysfunction. MATERIALS AND METHODS: Retrospective study was carried out of 56 liver allograft biopsies and their histological findings with clinical presentation were correlated. Totally 56 needle liver allograft biopsies from January 1210 to July 2014, obtained from 35 patients were studied for histological and clinicopathological evaluation. RESULTS: The mean age was 53.2±5.48 years. The most common original disease was alcoholic cirrhosis. The most common histological lesion was acute cellular rejection (ACR) in 31 (55.36%) biopsies followed by preservation-reperfusion injury (PRI) in 10 (17.86%) biopsies and drug toxicity in 8 (14.29%) biopsies. Chronic rejection was reported in 2 (3.57%) and recurrence of HCV in 3 (5.36%). Ischemic coagulative necrosis and acute cholangitis were seen in 1 (1.79 %) case each. CONCLUSION: Alcoholic cirrhosis was the most common etiology for end stage liver disease. ACR and PRI were the major complications in liver allograft biopsies at our centre.

C1q nephropathy in India: a single-center study.

C1q nephropathy (C1qN) is defined by conspicuous C1q deposits in the glomerular mesangial regions of patients who do not have any evidence of systemic lupus erythematosus (SLE). We present our experience with C1qN over the last three years. In total, 1775 native renal biopsies were reviewed and dominant/co-dominant C1q mesangial deposits in patients with absence of clinical and/or serological evidence of SLE were considered as C1qN. Their clinical profile and renal function status were studied and correlated. C1qN was observed in 11 patients (0.61%), and included eight males and three females; the mean age was 36.6 years. The most common presentation was nephrotic syndrome. Hematuria was noted in eight patients (72%). The mean serum creatinine was 2.78 mg/dL. Hypertension was seen in two patients (18%). Mesangial proliferative glomerulonephritis (MePGN) was the most common histological pattern, followed by focal and segmental glomerulosclerosis and other lesions. The common codeposits along with C1q were IgM, followed by C3 and others. MePGN had better prognosis than others. To conclude, C1qN was noted in 0.61% of all renal biopsies with bimodal age distribution and may present as podocytopathy or non-podocytopathy. The prognosis depends on the morphological pattern and C1q deposits per se are not prognostic indicators.

Non-Functional Adrenal Gland Ganglioneuroma Masquerading as Chronic Calculus Cholecystitis.

Adrenal ganglioneuromas in young adults are rare and ill-understood. We report an incidentally detected adrenal gland tumor diagnosed as ganglioneuroma (mature type) in 33 years old man who presented with vomiting and epigastric pain for 2 months. Histopathology examination revealed a well-encapsulated benign tumor of mature ganglion cells and Schwann-like cells arranged in fascicles, staining strongly with NSE and s-100 proteins, with adjacent unremarkable adrenal cortex and medulla.

Rare Co-existence of Squamous Cell Carcinoma with Infiltration of Renal Vein and Xanthogranulomatous Pyelonephritis.

Primary renal squamous cell carcinoma is a very rare malignancy of the upper urinary tract. Most patients have history of chronic urolithiasis, analgesics abuse, radiotherapy or infection. Co-existence of SCC with xanthogranulomatous pyelonephritis is exceedingly rare with only few reports in the literature. We report a case of a 60-year-old male presented with right flank pain and mild tenderness of abdomen. Computed tomography of the abdomen revealed gross hydronephrosis with parenchymal thinning and irregular thick enhancing wall of pelvicalyceal system with multiple calculi in right kidney. Right renal vein appeared distended, filled with hypo dense material. Right nephrectomy was performed and sent for pathological examination. Histological evaluation revealed keratinizing squamous cell carcinoma with infiltration of renal vein and xanthogranulomatous pyelonephritis.

Novel therapy for insulin-dependent diabetes mellitus: infusion of in vitro-generated insulin-secreting cells.

Insulin-dependent diabetes mellitus (IDDM) is a metabolic disease usually resulting from autoimmune-mediated ß-cell destruction requiring lifetime exogenous insulin replacement. Mesenchymal stem cells (MSC) hold promising therapy. We present our experience of treating IDDM with co-infusion of in vitro autologous adipose tissue-derived MSC-differentiated insulin-secreting cells (ISC) with hematopoietic stem cells (HSC). This was an Institutional Review Board approved prospective non-randomized open-labeled clinical trial after informed consent from ten patients. ISC were differentiated from autologous adipose tissue-derived MSC and were infused with bone marrow-derived HSC in portal, thymic circulation by mini-laparotomy and in subcutaneous circulation. Patients were monitored for blood sugar levels, serum C-peptide levels, glycosylated hemoglobin (Hb1Ac) and glutamic acid decarboxylase (GAD) antibodies. Insulin administration was made on sliding scale with an objective of maintaining FBS < 150 mg/dL and PPBS around 200 mg/dL. Mean 3.34 mL cell inoculums with 5.25 × 10(4) cells/µL were infused. No untoward effects were observed. Over a mean follow-up of 31.71 months, mean serum C-peptide of 0.22 ng/mL before infusion had sustained rise of 0.92 ng/mL with decreased exogenous insulin requirement from 63.9 international units (IU)/day to 38.6 IU/day. Improvement in mean Hb1Ac was observed from 10.99 to 6.72%. Mean GAD antibodies were positive in all patients with mean of 331.10 IU/mL, which decreased to mean of 123 IU/mL. Co-infusion of autologous ISC with HSC represents a viable novel therapeutic option for IDDM.

Mesangial proliferative glomerulonephritis with acute tubule interstitial nephritis leading to acute kidney injury in influenza A (H1N1) infection.

Respiratory complications and renal failure are the leading causes for morbidity and mortality due to influenza (H1N1) virus infection. There has been limited information on histopathology of H1N1 influenza-related acute kidney injury (AKI). We describe AKI with H1N1 infection in a 52-year-old female. Renal biopsy showed mesangial proliferative glomerulonephritis with acute tubule interstitial nephritis. Her condition improved rapidly with oseltamivir, fluid replacement, steroid and dialysis. Our case suggests that H1N1 infection may have a causative link to the development of mesangial proliferative glomerulonephritis with acute tubulointerstitial nephritis.

Outcome of live and deceased donor renal transplantation in patients aged ≥55 years: A single-center experience.

Renal transplantation (RTx) has now become an accepted therapeutic modality of choice for elderly ESRD patients. This single-center study was undertaken to evaluate the outcome of RTx in ESRD patients ≥55 years. A total of 103 patients underwent RTx 79 living related living donors [LD], 24 deceased donors [DD]) at our center. Post-transplant immunosuppression consisted of calcineurin inhibitor-based regimen. The mean donor age was 58.3 years in the LD group and 59.5 years in the DD group. Male recipients constituted 92% in LD and 75% in DD group. In living donor renal transplantation, 1- and 5-year patient survival was 93% and 83.3% respectively and death-censored graft survival was 97.3% and 92.5% respectively. There were 12.6% biopsy proven acute rejection (BPAR) episodes and 12.6% patients were lost, mainly due to infections. In deceased donor renal transplantation, 1- and 5-year patient survival was 79.1% and 74.5% respectively and death-censored graft survival was 95.8% and 85.1% respectively. There were 12.5% BPAR episodes and 25% of patients were lost, mainly due to infections. RTx in ESRD (≥55 years) patients has acceptable patient and graft survival if found to have cardiac fitness and therefore should be encouraged.

Posterior reversible encephalopathy syndrome in children with kidney disease.

Posterior reversible encephalopathy syndrome (PRES) is a clinic-radiographic entity of heterogeneous etiologies that are grouped together because of similar findings on neuro-imaging and associated symptom complex of headache, vision loss, altered mentation, and seizures. Although usually considered benign and reversible, characteristics of this syndrome in pediatric patients remain obscure. This case series included 11 patients (8 males, 3 females, age 3-15 years) of PRES during September 2010 to February 2012 out of a total 660 renal pediatric patients (1.66%). We studied their clinical profile, contributory factors, and outcome. Presenting symptoms were headache in 73%, dimness of vision or cortical blindness in 36%, seizures in 91%, and altered mentation in 55%. The associated renal diseases were acute renal failure (55%), chronic renal failure (9%), and 36% had normal renal function. The contributory factors were uncontrolled hypertension (100%), severe hypoproteinemia (9%), persistent hypocalcemia (9%), hemolytic uremic syndrome (36%), cyclosporine toxicity (9%), lupus nephritis (9%), high hematocrit (9%), and pulse methylprednisolone (9%). Brain imaging showed involvement of occipito-parietal area (100%) and other brain areas (63%). All but one patient of hemolytic uremic syndrome had complete clinical neurological recovery in a week, and all had normal neurological imaging after 4-5 weeks. PRES is an underdiagnosed entity in pediatric renal disease patients. Associated hypertension, renal disease, and immunosuppressive treatment are important triggers. Early diagnosis and treatment of comorbid conditions is of prime importance for early reversal of syndrome.

Successful three-way kidney paired donation transplantation: The first Indian report.

Providing transplantation opportunities for patients with incompatible live donors through kidney paired donation (KPD) is an important strategy for easing the crisis in organ availability. KPD is can overcome the barriers when the only living potential donors are deemed unsuitable owing to an incompatibility of blood type, of human leukocyte antigen cross-match, or both. In KPD, the incompatibility problems with two donor recipient pairs can be solved by exchanging donors. In the absence of well-organized deceased donor program, or transplantation with desensitization protocol and ABO incompatible transplantation, living donor KPD promises hope to the growing number of patients suffering from end-stage renal disease in India. We report our first successful three-way KPD transplantation from India. In an era of organ shortage, this approach is relevant to encourage wider participation from KPD donors and transplant centers to prevent commercial transplantation.

Successful renal transplantation from a brain-dead deceased donor with head injury, disseminated intravascular coagulation and deranged renal functions.

Deceased donors (DDs) with the brain death due to head injury are the major source of organs for transplantation. The incidence of post-head injury disseminated intravascular coagulation (DIC) ranges from 24% to 50%. Many centers do not accept organs from donors with DIC due to increased risk of primary graft non-function and/or high chances of morbidity/mortality. We performed two successful renal transplants from a DD with head injury with DIC and deranged renal function. One of the recipients developed transient thrombocytopenia, but there was no evidence of DIC or delayed graft functions in either of the recipients. Over a follow-up of 1 month, both are doing well with stable graft function and hematological profile. Thus, a carefully selected DD with severe DIC even with deranged renal function is not a contraindication for organ donation if other risk factors for primary non-function are excluded. This approach will also help in overcoming organ shortage.