Xanthogranulomatous Pyelonephritis in a male child with renal vein thrombus extending into the inferior vena cava: a Case Report

<p>Abstract</p> <p>Background</p> <p>We present a case of Xanthogranulomatous pyelonephritis (XGPN) in a male child with renal vein thrombus extending into the inferior vena cava. This is a rare presentation. XGPN is a rare type of renal infection characterised by granulomatous inflammation with giant cells and foamy histiocytes. The peak incidence is in the sixth to seventh decade with a female predominance. XGPN is rare in children.</p> <p>Case presentation</p> <p>An 11 year old male child presented with a history of high grade fever and chills, right flank pain and progressive pyuria for two months. He had a history of vesical calculus for which he was operated four years back. In our case, a subcapsular right nephrectomy was performed. The surgical specimens were formalin fixed and paraffin embedded. The sections were stained with routine Hematoxylin & Eosin stain. Grossly; the kidney was enlarged with adherent capsule and thickening of the perinephric tissue. The pelvicalyceal system was dilated and was filled with a cast of pus. Histological evaluation revealed diffuse necrosis of the renal parenchyma and perinephric fat. Neutrophils, plasma cells, sheets of foamy macrophages and occasional multinucleate giant cells were seen. The renal vein was partially occluded by an inflammatory thrombus with fibrin, platelets and mixed inflammatory cells. The thrombus was focally adherent to the vein wall with organization.</p> <p>Conclusions</p> <p>The clinical presentation and the macroscopic aspect, together with the histological pattern, the cytological characteristics addressed the diagnosis towards XGPN with a vena caval thrombus. Our case illustrates that the diagnosis of XGPN should be considered even in paediatric age group when renal vein and vena caval thrombi are present.</p

Mixed Chimerism, Lymphocyte Recovery, and Evidence for Early Donor-Specific Unresponsiveness in Patients Receiving Combined Kidney and Bone Marrow Transplantation to Induce Tolerance

Background We have previously reported operational tolerance in patients receiving HLA-mismatched combined kidney and bone marrow transplantation (CKBMT). We now report on transient multilineage hematopoietic chimerism and lymphocyte recovery in five patients receiving a modified CKBMT protocol, and evidence for early donor-specific unresponsiveness in one of these patients. Methods Five patients with end-stage renal disease received CKBMT from HLA-mismatched, haploidentical living related donors following modified non-myeloablative conditioning. Polychromatic flow cytometry (FCM) was used to assess multilineage chimerism where evaluable and lymphocyte recovery post-transplant. Limiting dilution analysis was used to assess helper-T-lymphocyte reactivity to donor antigens. Results Transient multilineage mixed chimerism was observed in all patients but chimerism became undetectable by 2 weeks post-CKBMT. A marked decrease in T and B lymphocyte counts immediately following transplant was followed by gradual recovery. Initially recovering T cells were depleted of CD45RA+/CD45RO− “naïve-like” cells, which have shown strong recovery in two patients and CD4/CD8 ratios increased immediately following transplant but then declined markedly. NK cells were enriched in the peripheral blood of all patients following transplant. For Subject 2, a pre-transplant limiting dilution assay revealed T helper cells recognizing both donor and third-party PBMCs. However, the anti-donor response was completely undetectable by Day 24, while third-party reactivity persisted. Conclusion These results characterize the transient multilineage mixed hematopoietic chimerism and recovery of lymphocyte subsets in patients receiving a modified CKBMT protocol. The observations are relevant to the mechanisms of donor-specific tolerance in this patient group

Control of Herpesvirus Infection in Organ Transplant Recipients

The most important causes of infectious disease morbidity and mortality in organ transplant recipients are the herpesviruses, particularly cytomegalovirus (CMV) and Epstein-Barrvirus (EBV). Because of their properties of latency, cell association, and potential oncogenicity, they are particularly well suited to causing disease in the transplant patient. Antilymphocyte antibody therapies are potent reactivators of these viruses, and cyclosporine, by inhibiting the critical host defense (virus specific cytotoxic T cells) in a dose-dependent fashion, amplifies the extent and effects of the viral replication. This in turn is translated into clinical disease: fever; pneumonia; gastrointestinal ulcerations; broad-based suppression of host defences leading to opportunistic superinfection and, perhaps, allograft injury in the case of CMV; and B cell lymphoproliferative disease in the case of EBV. New approaches to controlling these viruses in which pre-emptive therapy is linked to immunosuppressive therapy, and new diagnostic techniques for viral monitoring, hold promise for limiting clinical disease due to these viruses

Eculizumab as a New Treatment for Severe Acute Post-infectious Glomerulonephritis: Two Case Reports.

Acute post-infections glomerulonephritis (APIGN) is a frequent cause of glomerulonephritis and represents the most common cause of acute glomerulonephritis in children. It can evolve to severe acute renal failure and chronic kidney disease or even end-stage kidney disease. The precise pathophysiological mechanisms of APIGN are still incompletely understood. The implication of the alternative complement pathway and the potential benefits of C5 blockade have been recently highlighted, in particular in the presence of a C3 Nephritic Factor (C3Nef), anti-Factor B or H autoantibodies. We report two children with severe APIGN, successfully treated with eculizumab. The first patient presented a severe form of APIGN with advanced renal failure and anuria, associated with a decreased level of C3 and an increased level of soluble C5b-9, in the presence of a C3NeF autoantibody. The second case had a severe oliguric APIGN associated with low C3 level. Kidney biopsy confirmed the diagnosis of APIGN in both cases. Eculizumab allowed full renal function recovery and the avoidance of dialysis in both cases. In conclusion, the alternative and terminal complement pathways activation might be common in PIGN, and in severe cases, eculizumab might help

Psychiatric disease and cytomegalovirus viremia in renal transplant recipients

Although cytomegalovirus (CMV) is rarely cultured from peripheral-blood leukocytes of immunocompetent patients, it may be cultured from up to 60% of renal transplant recipients, 1 to 4 months after transplantation. During this same period, renal transplant recipients are often referred for psychiatric evaluation. Since CMV may infect the central nervous system, the relationship between isolation of CMV from peripheral-blood leukocytes (viremia) and psychiatric evaluation was investigated in 80 renal allograft recipients at the Massachusetts General Hospital. Five of 16 (31%) patients with viremia and 7 of 64 (11%) patients without viremia required psychiatric consultation (P = 0.04, two-tailed Fisher exact test). CMV viremia may be an important but treatable contributor to psychiatric symptoms in the transplant recipient

Induction of kidney allograft tolerance after transient lymphohematopoietic chimerism in patients with multiple myeloma and end-stage renal disease

Two patients with end-stage renal disease secondary to multiple myelomas were treated with combined kidney and bone marrow transplantation in an effort to achieve donor-specific allotolerance through the induction of mixed lymphohematopoietic chimerism