1 Department of Surgery III, 2 Department of Gastroenterological Surgery, 3 Department of Pediatric Nephrology, Tokyo Women's Medical University, Shinjuku-ku, Tokyo, and 4 Department of Transplantation Surgery, Kyoto University, Kyoto, Japan
Keywords: chronic renal failure; haemodialysis; liver and kidney transplantation; living related donor; paediatric transplantation; primary hyperoxaluria (type 1)
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Introduction |
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Organ shortages remain the most formidable obstacle to widespread application of organ transplantation. In particular, the problem of paediatric donors shortage has been getting more and more serious. Though it is partially alleviated by the use of living-related donors, this procedure places an otherwise healthy person at risk. The donors of liverkidney transplantation are exposed themselves to danger all the more. On the other hand, paediatric recipients with chronic liver disease complicated by renal failure are extremely malnourished and growth retarded. Such frail patients have a decreased ability to withstand stress of invasive surgery.
In our department, sequential liverkidney transplantations (SeqLKTx) from living-related donors were performed. In fact, in three paediatric patients with chronic liver disease complicated by renal failure, living-related liver transplantation was initially performed, and various proper blood purification therapies were introduced to these patients post-operatively. Subsequently, renal transplantation from the same living donor was performed after the transplanted liver function recovered.
Though this procedure requires appropriate control of perioperative blood purification, it is very beneficial from a practical viewpoint in terms of both the living donors safety and the reduction of the frail recipients surgical stress. We report here on three paediatric patients who underwent SeqLKTx.
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Cases |
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Case 2
A 2-year-old boy (body weight 7.3 kg) received CAPD therapy from the 21st day after birth for renal failure resulting from polycystic kidneys. Several months later, the patient developed jaundice which was caused by congenital hepatic fibrosis. The lateral segment of the liver obtained from the donor (mother) was orthotopically transplanted to the recipient at 2 years of age. He had mild post-operative peritonitis from which he quickly and completely recovered. Subsequently, the left kidney of the mother was transplanted to the recipient on the 107th hospital day after liver transplantation. The renal artery and vein were anastomosed directly to the aorta and inferior vena cava respectively. With no significant post-operative complications except for mild rejection, the patient was discharged on the 162nd hospital day.
Case 3
A 3-year-old boy (body weight 12.3 kg) had chronic renal failure resulting from interstitial nephritis. Haemodialysis (HD) was initiated at the age of 3, and CAPD was started 3 months later. His liver function also deteriorated at approximately 1 year of age. Liver biopsy was performed and showed primary sclerosing cholangitis. When he was 3 years and 4 months old, hepatic cirrhosis developed, and plasmapheresis was occasionally needed to improve persistent hyperbilirubinaemia. The lateral segment of the liver obtained from the donor (mother) was orthotopically transplanted to the recipient. On the 65th hospital day, the left kidney of the donor was transplanted to the recipient. The renal artery and vein were anastomosed to the right common iliac artery and vein respectively. The recipient had no significant post-operative complications and showed no signs of rejection. The patient was discharged on the 82nd hospital day.
In all three cases, ureterocystostomy was performed by the extravesical method. All three patients received immunosuppressive therapy with tacrolimus and corticosteroids. No severe post-operative complications were noted in any of the recipients, and mild rejection occurred only once in one of the cases.
The donors showed relatively immediate improvement in both their liver and renal function. They were discharged within 2 weeks, having no significant complications.
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Discussion |
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Liverkidney transplantation holds a lot of advantages from a practical point of view. For example, not only is it a definitive treatment for multiple organ failure, but it also is particularly effective in improving growth and QOL (quality of life), and reversing or arresting developmental retardation in paediatric patients [4].
The following considerations must be made when selecting between simultaneous and sequential LKTx in patients with combined liver and kidney failure. In cases where renal failure is associated with hepatic disorders, such as primary hyperoxaluria (case 1), SeqLKTx is considered the better procedure, since systemic oxalate deposition could damage grafted kidney function. The possibility of pre-emptive living-related liver transplantation should also be evaluated in the near future [5].
In cases where renal failure is not directly associated with hepatic disease, simultaneous and sequential LKTx may be considered equal. In terms of the severity of surgical stress, the simultaneous procedure is hazardous to patients such as the frail recipients in cases 2 or 3 above, since they are extremely malnourished and physically retarded [4]. This may be one of the main reasons why children with renal failure prior to liver transplantation have an increased mortality [6]. SeqLKTx is able to reduce the surgical stress during the initial liver transplantation.
Furthermore, sequential renal transplantation is performed after the transplanted liver function recovers, which should be the great advantage of this procedure. Renal transplantation is performed after the dosage of immunosuppressants such as tacrolimus and cyclosporin is decreased, which should reduce side-effects of the immunosuppressants, including renal toxicity [7]. In addition, multiple organ transplantation from the same donors may facilitate the induction of immunotolerance [8,9]. Mild rejection occurred only once in one patient among these three paediatric patients during hospitalization. The incidence of acute rejection is expected to be low.
In the case of SeqLKTx, blood purification after liver transplantation is relatively difficult to perform due to an unstable haemodynamic state and the presence of a bleeding tendency. Peri-operative control of fluid and electrolyte balance as well as drug levels is difficult due to the severe degree of surgical stress.
In general, CAPD is employed for blood purification therapy in low-weight paediatric patients. However, in SeqLKTx, haemodialysis or haemodiafiltration must be performed to purify the blood after liver transplantation. It is thought that blood purification methods should be improved in order to stabilize the post-operative haemodynamic state. Initially a double-lumen catheter with a subcutaneous tunnel is implanted pre- or post-operatively to be used for blood access. Low volume circuits (less than 10% of total circulating blood volume) as well as dialysers with small membranes (less than 75% of body surface area) should be used to reduce the blood volume in extracorporeal circulation. The combination of long-term continuous blood purification might be the best way to maintain reduced blood flow (less than 34 ml/kg/min) and stabilize the haemodynamics. The use of blood products such as serum albumin and fresh frozen plasma (FFP) for priming, as well as administration of vasopressors, are also considered effective methods for blood purification.
The continuing shortage of organs for paediatric transplant recipients has generated enthusiasm for living-related transplantation. In our country, cadaveric transplantation is still infrequent, and this is the reason why in Japan living-related donors are used as the main source for transplantation. In the case of liverkidney transplantation, living donors are exposed to a greater hazard than in case of single-organ transplantation. If blood purification is performed under good conditions, SeqLKTx is more advantageous than simultaneous LKTx not only for the malnourished frail paediatric patient but also for the living donors.
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Notes |
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References |
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