Eight grafts in seven patients from a single cadaver donor: a case report.
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Biomedical subjects
Publications and source records attributed to J B Otte.
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The study assessed psychological and social adjustment to pediatric liver transplantation in 101 patients aged 4-16 years who had undergone transplantation 2-10 years earlier. Each parent completed the Child Behavior Check List (CBCL) independently and answered a transplantation questionnaire assessing psychosocial issues. Most children scored within the normal range for both parts of the CBCL (i.e. Behavioral Problems and Social Competences). The impact of age at transplantation and of time elapsed since transplantation were examined. Earlier transplantation was associated with subsequent higher scores for Aggressive Behavior and Sex Problems, with lower scores for Activities and Competences, and with more parental anxiety concerning their child's health. The longer the time elapsed since transplantation, the more mothers rated high levels on the Somatic complaints, Anxiety/depression, Competence, and Social activity CBCL scales for their child. Parents reported talking more about the transplantation with their children with increasing time following transplantation. This suggests that transplantation remains an important event even after long periods of time from the transplant event. Finally, despite the difficulties encountered, more than 80% of the parents considered the transplantation had been beneficial to their child.
Growth failure is a common observation in children with end-stage liver disease (ESLD). The liver is an important endocrine organ producing potent anabolic growth factors such as IGF-I (insulin-like growth factor-I) and its major binding-proteins, called IGFBP-1, -2 and -3. Circulating IGF-I and IGFBP-3 levels are low in children with chronic liver failure despite increased GH secretion. This discrepancy suggests that GH resistance is present in chronic liver failure and is mainly due to the associated malnutrition. The advent of orthotopic liver transplantation (OLT) has dramatically improved the life expectancy of children with ESLD. Nevertheless, the growth of 15-20% of the children in recent studies remains poor after successful transplantation. Several factors such as age and height deficit at the time of OLT, etiology of the liver disease and graft function as well as the dose and mode of administration of glucocorticoids have been implicated in the lack of complete catch-up growth following surgery. Few studies have explored the possibility that anomalies in the GH-IGF-I cascade could explain growth retardation. However, it is unlikely that major anomalies of the GH-IGF-I axis contribute to impaired growth. Treatment with GH alone or in combination with IGF-I before or after OLT may improve the growth of children. Randomized multi-center studies are needed to address this issue.
The aim of this literature review is to evaluate current data on laparoscopic and open appendicectomy in order to establish a new gold standard in surgery. The authors have reviewed 4190 appendicectomies, 3322 laparoscopic appendicectomies (LA) and 868 open appendicectomies (OA), from 22 studies published between February 1992 and June 1994. Four studies dealt exclusively with pediatric patients (n = 1558). The analysis compares surgical technique, operating time, pathological findings, major and minor complications, postoperative pain and costs. The strongest arguments against LA are the increased rate of major complications, the increase of overall cost and the negative effect of the learning curve. The argument in favour of LA are the significant reduction of minor complications, the shortening of the postoperative hospitalization and of the time to resume full activity. The benefit of LA regarding the amount of analgesia requirement and the time needed to resume normal feeding after surgery is still controversial. The authors have reached the conclusion that LA might emerge as the first choice for appendicectomy. More studies are necessary to confirm this trend.
Encouraging results of alternative techniques used for liver transplantation in children (liver reduction) and the persistent lack of a sufficient number of cadaver donors has favored the development of living related donor liver transplantation. This program, which began after a long preparative period concerning the ethical questions involved, has included 32 children during the first 30 months. Results have been excellent. All children who underwent elective transplantations (n = 18) are still living. Among the 14 patients whose condition required hospitalization before transplantation, 86% have survived. Vascular complications and graft loss due to primary dysfunction or chronic rejection have been reduced, but 22% of the patients have biliary stenosis. In the donors, there has been no severe complication or sequelae. Use of related living donor livers has increased the number of grafts available for children on the waiting list for cadaver livers. The resulting gain in waiting time has also reduced the risk of death before transplantation. In our experience, the combination of the two transplantation programs using living donors and cadaver livers has had a positive impact on global management of children referred for liver transplantation, whatever the option chosen by the parents.
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The shortage of cadaveric liver grafts is very detrimental for young children because of the scarcity of small size-matched pediatric donors. Living related liver transplantation combined with innovative techniques using a partial cadaveric liver graft (reduced liver, split liver) gives excellent results and has a positive impact on the overall management of children needing a liver transplant.
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A prospective trial was conducted in 129 recipients of primary liver transplantation, to compare induction immunosuppression using triple drug therapy (cyclosporine, steroids, and azathioprine; group 1, n = 42), versus triple drug therapy with a 10-day course of OKT3 (group 2, n = 44) or of the anti-interleukin-2 receptor monoclonal antibody LO-Tact-1 (group 3, n = 43). Two-year actual patient survival rates were 64%, 79%, and 93% in groups 1, 2, and 3, respectively (1 vs. 2, NS; I vs. III, P = 0.003; 2 vs. 3, NS). Up to 2 years after transplantation, 18%, 44%, and 53% of the grafts in groups 1, 2, and 3, respectively, had not experienced steroid-resistant acute rejection (1 vs. 2, P = 0.002; 1 vs. 3, P = 0.007; 2 vs. 3, NS). The overall incidence of chronic rejection was 4%. OKT3 therapy, but not LO-Tact-1, significantly increased the incidence of cytomegalovirus infections (P = 0.019). In conclusion, immunoprophylaxis with LO-Tact-1 seemed to provide a liver graft acceptance rate at least as satisfactory as that with OKT3, without an increase in the incidence of infections.
The oral bioavailability of Sandimmun can be impaired by cholestasis, external biliary diversion, and diarrhea. We report two cases of pediatric liver transplant recipients who experienced chronic rejection and diarrhea secondary to proximal bowel resection. These conditions resulted in poor oral absorption of Sandimmun; the children were converted to the new oral microemulsion formulation Neoral, which significantly improved oral absorption, allowing intravenous cyclosporine weaning and patient discharge. Comparative pharmacokinetic studies were performed in both cases, and the relative Neoral/Sandimmun bioavailabilities were 32.9 and 5.4, respectively. Accordingly, Neoral may constitute to good alternative to ensure the effectiveness of oral cyclosporine administration, particularly in liver-transplanted children with severe cholestasis or shortened small bowel.
This study represents a multicenter survey on the management of patients with Crigler-Najjar syndrome (CNS) type 1. The aim of the survey was to find guiding principles for physicians in the care of these patients. Fifty-seven patients were included. At the time of inclusion, 21 patients had received a liver transplant (37%). The average age at transplantation was 9.1 +/- 6.9 years (range, 1-23 years); the age of the patients who had not been transplanted at the time of inclusion was 6.9 +/- 6.0 years (range, 0-23 years). Brain damage had developed in 15 patients (26%). Five patients died, and 10 are alive with some degree of mental or physical handicap. In 2 patients, ages 22 and 23 years, early signs of bilirubin encephalopathy could be reversed, in 1 by prompt medical intervention followed by liver transplantation and in the other by prompt liver transplantation. Seven patients underwent transplantation with some degree of brain damage at the time of the surgery; 1 of these died after retransplantation, 2 improved neurologically, and 4 remained neurologically impaired. The age of 8 patients with and 13 without brain damage at or before transplantation was 14.3 +/- 5.9 and 5.9 +/- 5.4 years (P < .01), respectively. Therapy of CNS type 1 consists of phototherapy (12 h/d), followed by liver transplantation. Phototherapy, although initially very effective, is socially inconvenient and becomes less efficient in the older age group, thus also decreasing compliance. Currently, liver transplantation is the only effective therapy. This survey shows that, in a significant number of patients, liver transplantation is performed after some form of brain damage has already occurred. From this, one must conclude that liver transplantation should be performed at a young age, particularly in situations in which reliable administration of phototherapy cannot be guaranteed.
Transjugular intrahepatic portosystemic stent shunting (TIPSS) appears to be an attractive, nonsurgical procedure to overcome complications of end-stage liver disease. During the period August 1992 to February 1995, 23 adults who had previously undergone TIPSS received liver transplants. These patients were compared to 36 cirrhotic patients, grafted during the same time period, in relation to the implantation technique, the intraoperative use of blood products, and the length of their hospital stay. These groups were comparable for previous right upper quadrant surgery, splanchnic vein modifications, and Child-Pugh classification. Liver transplantation was performed electively in all TIPSS patients. Ten patients (43.4%) presented with a significant shunt stenosis at a median follow-up time of 4.5 months (range 2.5 to 30 months). At transplantation 8 of the 23 TIPSS patients (34.8%) had specific TIPSS-related modifications i.e., extrahepatic portal vein aneurysm formation (n = 2), dislocation of the distal end of the stent into the inferior vena cava (n = 4) or into the main portal vein trunk (n = 1), bilioportal fistula (n = 1), and pronounced phlebitis of the inferior vena cava and hepatic veins due to redilation of shunt stenosis (n = 4). The intraoperative blood product requirement at transplantation was similar in the 23 TIPSS-patients and in the 36 cirrhotic patients who received transplants without the TIPSS procedure during the same time period [median 800 ml (range 0-20300 ml) vs median 620 ml (range 0-7600 ml), respectively]. There was also no difference between the two groups in length of hospital stay [median 18 days (range 0-34 days) vs median 19 days (range 0-66 days), respectively]. We conclude that TIPSS plays an important role in the management of life-threatening complications of end-stage liver disease arising in potential liver transplant candidates. TIPSS should be considered as a temporary, effective bridge to elective transplantation and not as a means to lower the blood product requirement at transplantation. Specific TIPSS-related modifications should be recognized early by the transplant surgeon in order to adapt the technique of graft implantation.
Calcification within active post-transplantation lymphoproliferative disorders has, to our knowledge, never been described. We report the case of a 14-month-old boy who presented 4 months after orthotopic liver transplantation with a primary calcification in a lymphoproliferative nodule involving the hepatic graft. This calcification was detected by routine ultrasonography, visible on plain radiographs, and the post-transplantation lymphoproliferative disorder was histologically proved.
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BACKGROUND: Open surgery is the standard approach for splenectomy in hematologic disorders, but a few cases of successful laparoscopic splenectomy have been reported. METHODS: Thirty-one patients (18 adults, group 1; and 13 children, group 2) underwent laparoscopic splenectomy. Indications for surgery included idiopathic thrombocytopenic purpura (25 patients), congenital spherocytosis (4 patients), and hemolytic anemia (2 patients). In 97% of the patients the diameter of the spleen was less than 15 cm. RESULTS: Laparoscopic splenectomy was successful in 94% of the patients; conversion to open surgery was mainly related to hemorrhage. Accessory spleen was found in 39% in group 1 and 8% in group 2. Two adults received intraoperative autotransfusion. Postoperative morbidity was minimal. The median postoperative stay was 3 days (range, 2 to 12 days) in group 1 and 2 days (range, 2 to 5 days) in group 2. CONCLUSIONS: Laparoscopic splenectomy is safe in both adults and children. Adequate selection of patients (small-size spleen, splenic destruction on preoperative scanning of platelets), appropriate preparation in patients with idiopathic thrombocytopenic purpura (immunoglobulin G), and meticulous surgical technique (with routine opening of the gastrocolic ligament to search for accessory spleen) are key factors in obtaining the same long-term results as with open surgery.
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Hyperbilirubinemia in Crigler-Najjar disease type I (CN) can be partially controlled by daily phototherapy, but these children remain at permanent risk of developing brain damage due to kernicterus. Because liver transplantation is the only available curative treatment for liver-based inborn errors of metabolism, orthotopic liver transplantation (OLT) was performed in six patients with CN. Mean age at surgery was 52.5 months (range 27 to 100). Despite a mean daily phototherapy of 12.4 +/- 0.8 hr, mean bilirubin of the 6 patients was 388 microM/L (range 175 to 703) before OLT; one of them was also being treated with tin-protoporphyrin. All 6 had elevated AST/ALT, ranging from 1.4 to 6 times upper normal values. Complications occurred in three patients after OLT, including miliary tuberculosis in one, graft rejection and retransplantation in one, and hepatic artery thrombosis in one. All patients survive with normal serum bilirubin level (follow up 6 to 116 months). Four have normal enzymes on post-OLT follow-up (30 to 95 months), follow a normal education program, and have a normal social life. One recently transplanted patient has progressively normalizing liver function tests 6 months after OLT. One patient transplanted at 8 y.o. (now 116 months post-OLT) has moderate neurological delay due to pretransplant kernicterus, and posttransplant chronic persistent hepatitis. Our series shows that OLT cures hyperbilirubinemia in CN patients, with an excellent survival prospect. The procedure should be decided upon before neurological sequelae occur, since these persist after transplantation.