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.

Portal Vein Thrombosis: A Rare Complication of Nephrotic Syndrome.

Deep vein thrombosis, renal vein thrombosis, and cerebral venous sinus thrombosis in children are frequently described complications of nephrotic syndrome (NS). Early diagnosis and treatment with anticoagulants is the key for a good outcome. There are a few reported cases of portal vein and superior mesenteric thrombosis in adults in association with NS. Here, we describe two cases of portal vein thrombosis with variable extent of involvement of superior mesenteric vein in association with relapse of NS. A high degree of suspicion, ultrasonography of the abdomen along with Doppler study of abdominal vessels, and computed tomography angiography can only pick up such unusual sites of thrombosis and facilitate early management.

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.

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.

Chronic type B aortic dissection in association with Hemolyticuremic syndrome in a child.

Aortic dissection (AD) is a potentially life-threatening medical emergency usually encountered in the elderly. Here, we report a 9-year-old child who was incidentally detected to have asymptomatic chronic type B dissecting aneurysm of aorta when he presented with relapse of Hemolytic uremic syndrome (HUS) without any genetic abnormalities like Marfan or Ehler-Danlos syndrome. To the best of our knowledge, this is the first case of AD associated with HUS in a child without any known associated genetic or inherited risk factors.

Intercity deceased donor renal transplantation: a single-center experience from a developing country.

In a developing country such as India, deceased donor renal transplantation (DDRTx) accounts for only about 1% of all renal transplants (RTx). Our institute initiated an intercity DDRTx in the year 2006, which significantly increased the number of RTx. We retrieved 74 kidneys from 37 deceased donors from various cities of Gujarat from January 2006 to December 2009. We transplanted the allografts in 66 recipients and a retrospective analysis of the donor profile and management and recipient outcome was performed. The mean age of the donors was 43.3 ± 18.8 years. The causes of death included road traffic accident in 51.35% of the donors and cerebrovascular stroke in 48.65% of the donors; 83.78% of the donors required ionotropes for hemodynamic stability in addition to vigorous intravenous fluid replacement. The average urine output of the donors was 350 ± 150 mL. The organs were perfused and stored in HTK solution. The mean cold ischemia time (CIT) was 9.12 ± 5.25 h. The mean anastomosis time in the recipient was 30.8 ± 8.7 min. 57.6% of the recipients established urine output on the operating table and 42.4% developed delayed graft function. At the end of 1 month after transplantation, the mean serum creatinine was comparable to the Ahmadabad city DDRTx, although the CIT was significantly longer in the intercity patients. Intercity organ harvesting is a viable option to increase the donor pool. Distance may not be an impediment, and good recipient outcome is possible in spite of prolonged CIT in case of proper harvesting and preservation.

Primary renal carcinoid tumor.

Primary renal carcinoid tumor is extremely rare and, therefore, its pathogenesis and prognosis is not well known. We report a primary renal carcinoid in a 26-year-old man treated by radical nephrectomy.

Successful renal transplantation from a brain-dead deceased donor who died from snakebite: a case report.

Even though India is the country with the highest annual number of deaths (50,000) from snakebite, there is contradictory evidence regarding acceptance of deceased donors (DD) who died from this cause. We present 2 successful renal transplantations (RTx) from a brain-dead DD who died from a neurotoxic snakebite without manifestations of a viper bite. We accepted the donor as he exhibited no evidence of hematoxic snakebite. Rather the findings were consistent with a neurotoxic bite (probably krait), which can cause hypoxic brain injury. Both recipients established good diuresis intraoperatively and did not require hemodialysis. The patients were discharged with good diuresis and normal serum creatinines. After 3-month follow-up, both recipients show normal graft function. According to our experience of favorable RTx outcomes from a brain-dead DD who died from neurotoxic snakebite may expand the donor pool.

Outcome of renal transplantation from deceased donors after cardiac death: a single-center experience from a developing country.

BACKGROUND: Limited information is available in the literature about the use of organs from donation after cardiac death (DCD) renal transplantation (RTx) from a developing country. MATERIAL AND METHODS: We report RTx outcome between DCD donors ≥70 years (Group 1; n = 14; mean age, 75.7 ± 5.81) and DCD donors <70 years (Group 2; n = l9; mean age, 51.7 ± 10.1) between January 1999 and January 2012. The mean age of recipients was 39.5 ± 14.7 years, 24 of whom were males. The mean donor age was 61.9 ± 14.6 years, 21 of whom were males. All recipients received single-dose thymoglobulin induction followed by immunosuppression with a steroid, a calcineurin inhibitor, and mycophenolate mofetil or azathioprine. Statistical analysis used chi-square test and unpaired Student t test. Kaplan-Meier curves were used for survival analysis. RESULTS: Over a mean follow-up of 3.21 ± 3.46 years, one-, five-, and ten-year, patient survival rates were 77%, 67.4%, and 67.4%, respectively, and death-censored graft survival rates were 85.7% for one, five, and ten years. Delayed graft function (DGF) was observed in 36.4% (n = 12) with 12.1% (n = 4) biopsy-proven acute rejection (BPAR). Patient survival (P = .27), graft survival (P = .20), DGF (P = .51), and BPAR (P = .74) were similar in 2 groups. A total of 27.2% (n = 9) of patients died, mainly due to infections (n = 5). CONCLUSION: Given the widespread organ shortage, outcomes of controlled DCD renal transplantation has a potential to expand the donor pool and shorten the waiting list for RTx, encouraging the use of this approach even in low-income countries.

Outcome of second kidney transplant: a single center experience.

Nowadays, a repeat transplantation is considered to confer a better survival advantage to patients over dialysis. The cost-effectiveness of transplantation for end-stage renal disease patients shows benefits over dialysis even for re-transplanted patients. This retrospective single center ten-year study was undertaken to evaluate patient/graft survival, function vis-à-vis serum creatinine (SCr) and rejection episodes in 62 re-transplanted patients. Sixty-two patients underwent a second renal transplant (24 living related, 38 deceased donors) at our center between 2000 to 2009. The mean recipient age was 41.9 ± 12.27 years. Fifty-three recipients were male and nine recipients were female. Recipients had negative acceptable lymphocyte cross-matching using anti-human globulin complement-dependent cytotoxicity tests and flow cytometric cross-match before transplant. All recipients except those who were hepatitis C virus or hepatitis B surface antigen positive received single-dose rabbit-anti-thymocyte globulin induction and steroids, calcineurin inhibitor ± mycophenolate mofetil/azathioprine for maintenance immunosuppression. Of the 62 patients, 38 patients received kidneys from deceased donors and 24 patients received kidneys from live donors. Over the mean follow-up of 4.03 ± 2.93 years, the 1-year, 5-year and 10-year patient survival rates were 85.33%, 66.7% and 66.7%, respectively, and the graft survival rates were 96.7%, 79.7% and 79.7%, respectively. The acute rejection rates were 17.6%, with a mean SCr of 1.92 ± 0.5 mg/dL. There was unexplained interstitial fibrosis with tubular atrophy in 11.2% patients (n = 7), all leading to graft loss eventually. Overall, 25% (n = 16) of the patients were lost, mainly to infectious complications. Re-transplantation has acceptable graft and patient survival over a ten-year follow-up period and should be encouraged for better quality of life as compared with dialysis.