Primary hyperoxaluria diagnosed after kidney transplantation failure: lesson from 3 case reports and literature review.

BACKGROUND: Primary hyperoxaluria (PH) is a rare inborn disorder of the metabolism of glyoxylate, which causes the hallmark production oxalate and forms insoluble calcium oxalate crystals that accumulate in the kidney and other organs. Since the manifestation of PH varies from recurrent nephrolithiasis, nephrocalcinosis, and end-stage renal disease with age at onset of symptoms ranging from infancy to the sixth decade, the disease remains undiagnosed until after kidney transplantation in some cases. CASE PRESENTATION: Herein, we report 3 cases of PH diagnosed after kidney transplantation failure, providing the comprehensive clinical course, the ultrasonic image of renal graft and pathologic image of the biopsy, highlighting the relevance of biopsy findings and the results of molecular genetic testing. We also focus on the treatment and the unfavorable outcome of the patients. Meanwhile, we review the literature and show the additional 10 reported cases of PH diagnosed after kidney transplantation. Additionally, we discuss the progressive molecular understanding of the mechanisms involved in PH and molecular therapy. CONCLUSIONS: Overall, the necessity of preoperative screening of PH in all patients even with a minor history of nephrolithiasis and the importance of proper treatment are the lessons we learn from the 3 cases, which prompt us to avoid tragedies.

Clinical significance of myeloid-derived suppressor cells in human renal transplantation with acute T cell-mediated rejection.

Myeloid-derived suppressor cells (MDSCs) are negative regulators of the immune response and are in part responsible for the inhibition of the T cell-mediated immune response. A recent paper indicated that MDSCs were involved in prolonged allograft survival in animal models of transplantation, but the significance of MDSCs in human renal transplantation is still unknown. In our study, 50 patients with biopsy-proven acute T cell-mediated rejection (ATCMR) were included. The ratio of MDSCs in peripheral blood mononuclear cell (PBMC) was evaluated with FACS, and the patients were divided into the MDSCs high group (MDSCs, >10 %) or the MDSCs low group (MDSCs, <10 %). We compared the allograft function, severity of tissue injury, and long-time survival between the two groups. In the MDSCs high group, allograft function was significantly increased compared with the MDSCs low group. Furthermore, we found that isolated MDSCs from transplant recipients are capable of expanding regulatory T cell (Treg), meanwhile, inhibiting production of IL-17 in vitro. We also found that the ratio between Foxp3(+) and IL-17-producing CD4(+) T cells positively correlated with MDSCs frequency in PBMC. In conclusion, we demonstrated a potential role for MDSCs in prolonging allograft survival after ATCMR, and this was associated with higher CD4(+)Foxp3(+)/CD4(+)IL-17(+) ratio in PBMC.

[Outcome of the kidney transplant recipients with chronic hepatitis B virus].

OBJECTIVE: To investigate the outcome of the kidney transplant recipients with different grades and stages of chronic hepatitis B virus after receiving renal transplantation for 3 years. METHODS: Thirty nine cases of kidney transplant recipients with hepatitis B virus and 20 cases of kidney transplant recipients (control group) between August 2000 and February 2002 were studied. Before the transplantation, the patients were divided into 4 groups: the mild hepatitis group (Group A, n = 8), the middle hepatitis group (Group B, n = 6), the severe hepatitis group (Group C, n =5) according to pathological diagnosis by percutaneous liver biopsy, and the control group (Group D). During the 3 year follow-up, the serum creatinine, alanine aminotransferase, g-Glutamyl transferase (GGT), total bilirubin, direct bilirubin, prothrombin time, cyclosporine trough concentration, urinary protein excretion, the HBV markers, HBV-DNA, albumin (A), globulin (G), the hepatic fibrosis markers and Child-Pugh score were studied at intervals. All patients received ultrasound examination every year. Two patients received repeated liver biopsy at the end of the follow-up in the hepatitis groups. RESULTS: The outcome of Group A and Group D was fine. In Group B, GGT level was significantly elevated (P < 0.05) sixth months after the operation, the Child-Pugh score of 2 patients were B, the liver pathohistological changes in another 2 patients were in severe stage in the endpoint. In Group C, GGT values had higher base-line (P <0.01) during the follow-up. Albumin were lower and globulin were higher than normal at the beginning of the 24th month. At the end of the follow-up, the Child-Pugh scores of all patients were B or C (B = 3, C = 2), 4 patients had end-stage cirrhosis, one died of hepatic cancer and the survival rate was 40% in Group C. CONCLUSION: The outcome of the 3 groups is different. The pathohistological diagnosis by liver biopsy is important for patient selection receiving renal transplantation.