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Objective To investigate the clinical manifestations, treatment and prognosis of primary hyperoxaluria type 1 (PH1). Methods Relevant literature review was conducted from Chongqing VIP, CNKI, Wanfang Data, PubMed, Web of Science, Embase and Cochrane databases. Clinical data of 57 patients with PH1 were collected, and the clinical manifestations, diagnosis and treatment and prognosis were analyzed. Results A total of 35 eligible studies were searched, including 57 patients with PH1, 39 male and 18 female, aged 0.2-57.0 years old, and the age of onset was from date of birth to 42 years old. The specificity of clinical symptoms of 57 patients with PH1 was relatively low, including 41 cases of renal stones, 21 cases of renal calcification and/or calcium deposition, 12 cases of oxalic acid deposition outside the urinary system, 12 cases of lumbago, backache and abdominal pain, and 8 cases of ureteral stones. Besides, alternative symptoms, such as decreased urine output, metabolic acidosis, disorder of water and electrolyte, anemia and gross hematuria were also reported. Thirty-three patients were diagnosed with end-stage renal disease (ESRD) upon admission. Twenty-six patients received transplantation. Among them, 17 cases underwent kidney transplantation (2 cases repeatedly received combined liver-kidney transplantation due to recurrence of stones and resumption of dialysis, and 1 case repeatedly received liver transplantation due to resumption of dialysis), 7 cases received combined liver-kidney transplantation, 2 cases underwent liver transplantation, and 3 cases received sequential liver-kidney transplantation, respectively. Thirty-one patients did not undergo transplantation. Significant differences were observed in the survival rate between patients treated with and without transplantation (85% vs. 58%, P < 0.05). Conclusions Clinical manifestations of PH1 are diverse and lack of specificity. A majority of PH1 patients are diagnosed with ESRD upon admission. Clinical prognosis of patients undergoing transplantation is better than that of those counterparts without transplantation. Prior liver transplantation or combined liver-kidney transplantation is recommended.
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Objective To evaluate the outcome of liver transplantation (LT) or combined liver-kidney transplantation (CLKT) for acute-on-chronic liver failure (ACLF) patients with renal dysfunction.Method From January 2001 to December 2009,133 patients underwent LT for ACLF at our center.Among them,30 had both ACLF and renal dysfunction.Of the 30 patients,12 underwent CLKT for end-stage renal disease (ESRD),and the other 18 with hepatorenal syndrome type 1 (HRS1) underwent LT alone.Their clinical data were reviewed and their survival outcomes were compared.Result The median model for end-stage liver disease scores (MELD) of the patients with ACLF were 28.133 patients received deceased donor liver grafts and 12 patients also received the same deceased donor kidney grafts,The hospital mortality rate was 21.8％ for all patients with ACLF.The 5-year survival rates were 72.8％ for patients without renal dysfunction and 70％ for patients with renal dysfunction.The curative effectiveness of the patients with ESRD who underwent CLKT was better than that of the patients without renal dysfunction or the patients with HRS1 who underwent LT alone.Conclusion LT alone improved renal function in most patients with HRS1.Simultaneous liver-kidney transplantation is an excellent strategy in patients with both ACLF and ESRD.It provides protection to kidney allograft in liver-based metabolic diseases affecting the kidney.
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Objective: To establish a method of reversal phase-high performance liquid chromatography (RP-HPLC) for determining the mass concentration of oxalate in human plasma and urine and to monitor the variation of mass concentration of oxalate in the patients with primary hyperoxaluria (PH) before and after combined liver-kidney transplantation. Methods: Agilent XDBC18 (150 mm × 4.6 mm, 5 μm) column and Agilent Zorbax extend-C18 (12.5 mm × 4.6 mm, 5 μm) guard column were used. Methyl alcohol and aqueous solution containing 0.1 mol/L ammoniom acetate (15∶85) were used as mobile phase. The flow rate was at 1.2 mL/min, ultraviolent determination wavelength was 314 nm, column temperature was at 26.3 ℃, and injection volume was 50 μL. o-phenylenediamine was used as derivating agent, reacted with oxalate in human plasma and urine so as to obtain the compound with better ultraviolet absorption-2, 3-dyhydroxy quinoxaline. Results: The detection limit in human plasma was 0.3 mg/L, the linear range was 1.953-125 mg/L, the average recovery was 94.89%, and its RSD was 4.1%; The detection limit in urine was 0.5 mg/L, the linear range was 1.953-125 mg/L, the average recovery was 94.31%, and its RSD was 3.2%. Conclusion: The method is believable for determining the mass concentration of oxalate with its simplicity, sensibility, repeatability, and better recovery rate.
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ObjectiveTo compare orthotopic liver transplantation (OLT)and combined liverkidney transplantation (CLKT) in the treatment of severe hepatitis B.MethodsIn this study 52 patients of severe hepatitis B were allocated to OLT (40 cases) or CLKT( 12 cases) at our department from Jan.2001 to Sep.2005.The perioperative complications and the result of follow-up were analyzed.ResultsThe preoperative renal functions in CLKT cases were severer than that in OLT cases.Postoperative severe infection was more common in CLKT cases than that in OLT cases.In OLT group 28 patients (70％)suffered from early posttransplant renal dysfunction,among them 11 patients needed dialysis,whilst there were 2 (16.7％ ) patients who needed dialysis in CLKT group (P ＜0.01 ).The posttransplant mortality in OLT group was 40％ ( n =16),significantly higher than that in CLKT ( 16.7％,n =2) ( P ＜ 0.01 ).In OLT group,9 cases developed severe renal failure and died.No one died of renal failure in CLKT group.ConclusionsThe prognosis is more favorable to perform CLKT in patients who suffered from severe hepatitis B with chronic renal dysfunction before transplantation.
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Combined liver-kidney transplantation has gained increasing popularity and applied for the patient with end-stage liver and kidney disease. There are some controversies about the sequence of transplantation. In general, the liver allograft is temporary placed before the kidney allograft. This method may provide some immunologic advantages that liver allograft may protect concomitantly transplantated kidney from rejection. In our opinion, kidney-liver sequence may provide several benefits in hemodynamic stability, safer monitoring and planned fluid replacement by urine flow assessment. Combined liver-kidney transplantation requires only conventional immunosuppressive drug dosage as in kidney transplantation alone. And combined liver-kidney transplantation can be performed with acceptable morbidity and mortality, and have exellent long term result. We present two cases of combined liver-kidney transplantation performed by kidney- liver sequence for complex end organ failure.
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Humanos , Aloenxertos , Hemodinâmica , Nefropatias , Transplante de Rim , Rim , Fígado , Doadores Vivos , MortalidadeRESUMO
We here report two cases of combined liver-kidney transplantation in patients with both end stage renal disease and hepatitis B related liver cirrhosis. The first case was a 55-year-old man with hepatitis B related liver cirrhosis and chronic renal failure, who received cadaveric liver and kidney transplantation. Immunosuppressants were cyclosporine, prednisolone, and mycophenolate mofetil. Clinical course was uneventful except for hemolytic anemia due to alloimmunization that occurs after ABO-mismatched solid organ transplantation. Hemoglobin level became stable after plasmapheresis. His renal and hepatic function is maintained up to the present time. The second case was a 42-year-old man with nephrotic syndrome and liver cirrhosis. The patient underwent living related-combined liver-kidney transplantation. Donors were his son and brother. Blood type of the patient and donors were identical and the result of HLA crossmatch was negative. On the 14th postoperative day, stenosis at anastomotic site of hepatic artery was detected. After balloon angioplasty hepatic function was normalized. At 8 months after the transplantation, the patient is stable without adverse events.