Crystalline Nephropathy in Renal Transplant: A Series of 4 Cases.

Crystals are particles of endogenous inorganic or organic composition that can trigger kidney injury when deposited or formed inside the kidney. The most common forms of crystalline nephropathies (CNs) are nephrocalcinosis and oxalate nephropathy. The causes of early allograft dysfunction are changing constantly, and recently calcium oxalate (CaOx) crystal deposition has been added to this list. CaOx deposition in renal allograft is important and probably under-recognized cause of delayed graft function that requires adequate awareness with early intervention to improve the allograft outcome. Here, we describe four cases of irreversible renal graft injury due to CNs.

BK virus-associated hemophagocytic syndrome in a renal transplant recipient.

Hemophagocytic syndrome (HPS) is a life-threatening hematological disorder in immunocompromised patients. Reactive HPS is observed in patients with systemic infection, neoplasia or auto-immune diseases. It is a rare hematological disorder after renal transplantation and must be suspected when fever and pancytopenia are seen in association with viral infections. HPS is usually associated with infection with the Cytomegalovirus and Epstein-Barr viruses. We report here a case of BK-virus-associated HPS.

Secondary oxalosis due to excess vitamin C intake: a cause of graft loss in a renal transplant recipient.

Renal oxalate deposition can be seen with primary hyperoxaluria, malabsorptive states, ethylene glycol toxicity and, rarely, with excessive vitamin C ingestion. We report a case of secondary hyperoxaluria in which the diagnosis was not considered initially because there was no past history of urinary calculi and no evidence of nephrocalcinosis on plain X-ray of the abdomen and ultrasonography. The disease was detected and diagnosed only after kidney transplantation. Secondary oxalosis can cause graft loss or delayed graft function. Biopsy of the allograft should be carefully examined for oxalate deposits even in the absence of a family history. When oxalosis is diagnosed, intensifying hemodialysis (HD) to eliminate calcium oxalate can help in the recovery of renal function in some cases. Systematic vitamin C supplementation in HD patients should be avoided as it can be a cause of secondary oxalosis.

Sirolimus for the treatment of Kaposi sarcoma after renal transplantation: a series of 10 cases.

The incidence of Kaposi sarcoma (KS) has substantially increased among immunocompromised patients, suggesting a role for immunosuppressive drugs. The aim of this study was to evaluate the incidence, features, and outcome of KS among 307 kidney transplantation patients at our center between January 1994 and June 2010. During the study period, the 10 patients who developed KS (3.25%) showed a mean age at transplantation of 35.8 ± 8.7 years (range, 22 to 49 years). The mean interval between transplantation and occurrence of KS was 24.7 ± 21.36 months (range, 6 to 64 months). The mean time of antithymocyte globulin induction was 9.5 days (range, 6 to 13 days). KS was restricted to the skin in 7 cases, among which, one presented with associated Hodgkin lymphoma. Visceral involvement (one lung and one colon) was observed in two cases. One patient presented with a gastric KS without skin lesions. Immunosuppressive treatment was reduced, then withdrawn in three cases, resulting in regression of KS a few weeks later, but with graft loss requiring hemodialysis at 1, 3 and 4 months. Among the remaining 7 cases, we stopped mycophenalate mofetil (MMF) and switched from calcineurin inhibitors to sirolimus. Allograft function remained stable after the switch. Only one patient who already had allograft dysfunction due to biopsy-proven chronic allograft nephropathy. Deteriorated progressively, undergoing hemodialysis at 2 years after KS diagnosis. In conclusion, we observed a relatively high incidence of KS among our cases. The introduction of sirolimus resulted in complete regression of KS lesions with preserved graft function.

Atypical presentation of a brown tumor in a kidney transplant recipient: a case report.

Brown tumor is a rare complication of secondary hyperparathyroidism. It is exceptionally encountered after kidney transplantation. We here report on a 54-year-old male recipient who developed a brown tumor localized in the right forearm, and whose initial presentation was atypical, mimicking a bone tumor. Hence, diagnosis of brown tumors should be suggested by clinicians in a context of hyperparathyroidism.

Evolution of impaired renal function after external continent urinary diversion (Mitrofanoff principle).

OBJECTIVE: To study the evolution of impaired renal function after external continent urinary diversion (Mitrofanoff principle) (ECUD-M) associated with ileocystoplasty. PATIENTS AND METHODS: Over 18 years from 1992 to 2009, ECUD-M with ileocystoplasty was performed in 120 patients with mean age of 25.5 years. Renal impairment was evident in 43 patients (17 children and 26 adults). RESULTS: Ninety percent of patients demonstrated a neurologic bladder and mild to moderate renal failure. Initially, all patients underwent continuous bladder drainage for a mean of 3 weeks. Renal function improved in 35 patients, although with persistent mild renal insufficiency. The other patients demonstrated moderate persistent residual renal insufficiency. During a mean follow-up of 10 years (range, 1-18 years), renal function returned to normal in 13 patients, stabilized at lower values in 15, and remained moderate in 5. After a mean follow-up of 8 years (range, 6-12 years), renal failure gradually worsened, increasing to higher values in 6 patients and leading to hemodialysis in 4. One patient underwent living-donor kidney transplantation, with good evolution. CONCLUSION: ECUD-M with ileocystoplasty can lead to normalization unless stabilizationof impaired residual renal function by eliminating the obstructive factor provides self-adequate management of the diversion. The procedure delays for the need forhemodialysis therapy, and enables patients to prepare for kidney transplantation into a previously reconstructed lower urinary tract.

Primary adenocarcinoma in a kidney allograft: a case report and review of the literature.

De novo tumors are common complications after solid organ transplantation. Lymphoma and skin cancers are the most frequently observed malignancies. However, graft carcinomas can be observed to be five times more frequent after kidney transplantation compared to their incidence in the general population. We report a case of a 49-year-old female who developed an early adenocarcinoma of the graft as revealed by acute renal failure. She underwent transplantectomy and chemotherapy with hemodialysis therapy. Carcinoma of the graft is a rare but serious complication usually occurring late after transplantation. Close monitoring of a kidney recipient using abdominal ultrasound may detect this complication at early stages, which may improve the prognosis. Similarly, good screening of donors may prevent tumor transmission.

Early onset of paucisymptomatic cryptococcal meningitis in a kidney transplant patient: a case report and review of the literature.

Fungal infections of the central nervous system are rare and are more frequently encountered in immunocompromised patients. Cryptococcocal infection is the most common opportunistic fungal infection after Candida and Aspergillus in organ transplant recipients. Atypical manifestations and nonspecific neuroradiological findings due to the lack of inflammatory response in these immunocompromised patients are responsible for a delay in diagnosis. This diagnosis should be considered even in atypical neurological signs, and additional tests (cerebrospinal fluid examination, magnetic resonance, etc) that may help to suggest the correct diagnosis should be used. We report a case of cryptococcal meningitis in a renal transplant recipient, which was misdiagnosed for several months because of an atypical presentation of headaches without fever or neurological signs.

Simultaneous Hodgkin's disease and Kaposi sarcoma in a renal transplant recipient.

A 38-year-old women underwent first cadaver kidney transplantation. Her panel reactive antibody was 0%, and she had never previously been transfused nor pregnant. She received induction therapy with antithymoglobulin (ATG) as standard protocol and maintained on immunosuppressive treatment of cyclosporine A, mycophenolate mofetil (MMF), and prednisone. Nine months after transplantation, she presented with anorexia, asthenia and weight loss. Cutaneous Kaposi's sarcoma and a Hodgkin disease were diagnosed. MMF was discontinued and cyclosporine A was switched to sirolimus. She also received a poly-chemotherapy associated with 4 courses of rituximab. Twelve months later, the patient had normal graft function and both malignancies were in complete remission.

Factors predisposing to post-renal transplant erythrocytosis: a retrospective study.

A retrospective study was conducted on 143 consecutive renal transplant recipients who had a functioning graft for three months or longer, to evaluate the prevalence of post-transplant erythrocytosis (PTE) and its potential risk factors. True PTE was defined as hematocrit (Ht) above 52% and hemoglobin (Hb) above 18 g/dl in males, and Ht above 50% and Hb above 17 g /dl in females. A total of 31 patients (21.6%) developed PTE; none had any evidence of polycythemia vera (PV), or secondary polycythemia due to reduced arterial oxygen, kidney or hepatic tumors, or relative erythrocytosis due to a decrease in plasma volume by overuse of diuretics. Thirty-one non-polycythemic patients (Hb 12.9 +-1.6 g/dl) matched for sex, age and renal function were used as case controls. PTE was more common in males (p= 0.043). The majority of our patients developed PTE within the first year post-transplantation and all had excellent renal function at the time of diagnosis. Also, PTE was found to be related to duration on dialysis prior to transplantation (p= 0.0013) and acute rejection (p= 0.0031).

Autosomal dominant Alport's syndrome: study of a large Tunisian family.

Alport's syndrome is a hereditary nephritis that may lead to end-stage renal disease (ESRD) in early adult life. It is a clinically and genetically heterogeneous nephropathy. Alport's syndrome is often associated with sensorineural deafness and/or ocular abnormalities. In contrast with the well-known X-linked phenotype, very little is known about the autosomal dominant form caused by mutations in COL4A3 and COL4A4 in the chromosome region 2q35-q37. We describe a Tunisian family with autosomal dominant Alport's syndrome in which males and females were equally affected. Two members reached ESRD at age 40 and 53 years, respectively. Three members experienced isolated microhematuria and one member experienced sensorineural deafness. No eye abnormalities were observed. Immunohistochemical studies showed a normal distribution of the alpha5 (type IV collagen) chain in the epidermal basement membrane. Genetic analysis demonstrated that a common haplotype co-segregated with the disease in the heterozygous state in all affected patients, thereby, confirming an autosomal dominant mode of inheritance. The same haplotype was observed in two asymptomatic children. We conclude that autosomal dominant Alport's syndrome, follows a rare mode of inheritance and exhibits a milder phenotype than usually observed in classic X-linked Alport's syndrome. The frequency of this mode of inheritance should be confirmed by a larger study.

Infectious complications in kidney transplant recipients: a single-center experience.

To determine the patterns of infectious complications in renal transplant recipients in our center, we evaluated 48 patients (29 men and 19 women) who were transplanted between 1994 and 2003. The average age of the patients was 29 years. Thirty (62.5%) and 18 (37.5%) transplants were from living related and cadaveric donors, respectively. Posttransplant immunosuppression consisted of azathioprine or mycophenolate mofetil (MMF), prednisone, antithymocyte globulin (ATG), and cyclosporine or tacrolimus. The acute rejection episodes were treated with pulse doses of methylprednisolone; steroid-resistant rejection was treated with ATG or muromonab (OKT3). All patients received prophylaxis with sulfadoxine-pyrimethamine; none received prophylaxis against cytomegalovirus (CMV) infection. Thirty-nine (81%) recipients developed 77 confirmed episodes of infection; 35 (46%) episodes occurred in the early postoperative period, 28 (36%) in the first month and 14 (18%) after 6 months. According to the type of infection, there were 24 urinary tract, 16 CMV, seven herpetic, nine general septic, six fungal, four pneumonia, one disseminated nocardial, and 10 miscellaneous episodes. All 26 (100%) patients who had acute rejection episodes developed infections compared with 13/22 (59%) who did not have rejection (P < .01). There was a significant correlation between CMV disease and acute rejection and/or tacrolimus or MMF use. CMV infection occurred after the additional immunosuppressive treatment for acute rejection in 10 patients or during the use of tacrolimus or MMF in six patients. We conclude that CMV infection was the most frequent opportunistic pathogen in our renal transplant population and related to the intensive antirejection therapy, followed by urinary tract infections within 3 months after surgery.

Asymptomatic gastric angiodysplasia in chronic hemodialysis patients: case reports.

Gastrointestinal (GI) angiodysplasia is a vascular lesion. It is a common cause of GI bleeding in chronic renal failure (CRF). We report three adult chronic hemodialysis patients with asymptomatic angiodysplasia. Over a period of four years, the hemoglobin level was stable and none of our patients received iron supplementation or erythropoietin (EPO) therapy. Incidence of angiodysplasia may be underestimated in the CRF patients. Further studies may be needed.

Anti-major histocompatibility complex antibody removal assay in a swine model.

A swine model of anti-MHC (SLA) immunization by skin grafting was established with the aim of removing preformed anti-MHC antibodies and preventing their resynthesis, in a situation close to that of hyperimmunization in humans. Plasma exchange therapy with or without associated immunosuppressive therapy was used. The feasibility of this animal model in terms of anti-MHC immunization and its therapeutic management have been proven. However, frequent early deaths of animals still mar the experimental protocol. Synchronization of plasma exchange and subsequent cyclophosphamide pulses seemed to abolish the antibody rebound phenomenon and cause marked drop of anti-MHC antibodies. Restimulation by a new skin graft is responsible for an intense polyclonal antibody stimulation, which suggests that we have to be careful in grafting patients with positive historical crossmatches and negative current ones.