儿童肝脏移植术后感染现状分析

自1989年首例儿童活体肝脏移植手术成功以来,肝脏移植术逐渐成为治疗儿童终末期和代谢性肝脏疾病的有效手段.近20年来,随着外科技术的日益完善及免疫抑制剂的合理应用,儿童肝脏移植术后的短期、长期存活率明显提高,但感染仍是肝脏移植术后最常见的并发症,严重影响预后.该文对儿章肝脏移植术后感染现状及合理用药作一概述.     

Biliary atresia and liver transplantation: results and thoughts for primary liver transplantation in select patients

Biliary atresia (BA) is one of the most common indications for liver transplantation in children. Despite advances in biliary atresia surgical techniques, most children will ultimately require liver transplantation. Possible pre-operative predictors of outcome after the Kasai operation are: 1. Age at operation 2. Presence of the biliary atresia splenic malformation syndrome (BASM) 3. Center specific factors 4. Liver histology and 5. Anatomic pattern of bile ducts found at surgery.Age at surgery is considered a strong predictor of success after portoenterostomy. In a recent study, age of 75 days or more at surgery was associated with less frequent resolution of jaundice and decreased transplant free survival. Similarly, the Ohi type II or III anatomy was associated with a higher risk of transplantation or death than type I. Inflammatory findings on pre-operative biopsy predicted a pooreroutcome after a Kasai procedure than obstructive changes. Nodularity of the liver at surgery as well as ascites was associated with a poorer prognosis.Primary transplantation is rarely done despite excellent outcome. Deaths on the waiting list also have improved with routine use of split and live donor transplantation. The Kasai operation has the highest failure rate in its stated objective than any other operation in pediatric surgery. Failure to achieve any improvement in jaundice occurs in over 30% of all cases, even in the best of hands, and transplantation or listing for transplantation occurs in over half the children with type II and III BA by one year of age in countries where liver transplantation is readily available.There are almost no studies in children with BA that compare the outcome after liver transplantation for BA with or without a prior Kasai procedure. It is postulated that a prospective trial in children predicted to have a poor prognosis after the Kasai procedure based on anatomic pattern, liver histology and presence of BASM, would yield improved care, spare some infants needless surgery, and quite possibly result in diminished morbidity and mortality following liver transplant.     

Pediatric liver transplantation

PURPOSE OF REVIEW: Pediatric liver transplantation is a challenging and exciting field for all healthcare providers involved with children who have end-stage liver disease. Graft and patient survival continue to improve due to improvements in medical, surgical, and anesthetic management, organ availability, immunosuppression, and identification and treatment of postoperative complications. This review will describe recent advances in pediatric liver transplantation. RECENT FINDINGS: Although pediatric cases only represent approximately 10% of the total patients on the waiting list, the number of deaths on the waiting list increased from 196 to 1753 between 1988 and 1999. Recently, a new pediatric liver allocation policy was instituted. The utilization of cut down "reduced" livers, split liver grafts, and living-related donors has provided more organs for pediatric patients. Newer immunosuppression regimens, including induction therapy, continue to have a significant impact on graft and patient survival. Excellence in peri-operative management and identification and treatment of complications or infections also has had an impact on graft and patient survival. Finally, investigation and analysis of the postoperative quality of life, for both the patient and parents, is being conducted. SUMMARY: Pediatric liver transplantation is a challenging and rewarding field with continued improvements in patient and graft survival. A multidisciplinary team approach coupled with improvements in organ availability, immunosuppression, and peri-operative management has had a dramatic impact on survival.     

Hepatic cell transplantation in childhood. State of the art

To date, children who suffer from a certain type of illness such as hepatic failure, could benefit with a non conventional functional replacement as an alternative to liver transplantation. Deterioration and death of patients on waiting list encourage the search for alternatives methods within transplantation. Liver cell transplantation has become a potential alternative treatment whose validation as an alternative or as a bridge until the donor appears, will probably contribute to improve the quality of life and survival of the patients. The aim of this review is to describe the state of the art of hepatocyte transplantation in pediatric patients.     

Pediatric liver transplantation

Considerable strides have been made over the last several decades toward improving outcomes in pediatric liver transplantation. Refinements in surgical technique has allowed for the use of living donor and deceased donor split-liver grafts, thus expanding the pool of available organs and reducing waitlist mortality. The use of a multidisciplinary team continues to be paramount in the care of the transplant recipient. With improvements in overall graft and survival, indications for liver transplantation have also broadened. Currently, pediatric transplant patients have a 5-year survival of over 85%. Long-term morbidity is mainly associated with complications from immunosuppression and chronic rejection. Here we review indications for liver transplantation in children, surgical considerations, post-operative complications, and long-term outcomes.     

Hepatitis C in children: a quaternary referral center perspective

INTRODUCTION: Chronic hepatitis C virus (HCV) infection affects 0.3% of children in the United States, and the general impression is that it has a benign course in childhood. We analyzed a pediatric population with chronic HCV in a quaternary referral center. MATERIAL AND METHODS: This is a retrospective clinical review comprising all patients with chronic HCV referred to the Pediatric Liver/Liver Transplant Program between January 1999 and December 2004. RESULTS: Ninety-one patients (52% female; mean age, 9 years) were assessed. Eight-three percent of the patients were genotype 1. Twenty-one patients received/are receiving interferon and ribavirin for chronic HCV (treatment indications--advanced disease, 9; clinical trial, 6; genotype 2, 2; social, 2; prerenal transplant, 1). Eight (53%) of 15 patients, who have completed therapy and follow-up, achieved sustained viral response. Seven of 91 patients had cirrhosis at presentation (mean age, 11.7 years). Four underwent liver transplantation, all experienced HCV recurrence, 2 died, 1 was retransplanted, and 1 has compensated cirrhosis. CONCLUSION: Although, in general, HCV in children has a slow progression, there are cases with an accelerated course and early development of cirrhosis requiring liver transplant. Hepatitis C virus recurs universally after transplant, and its prognosis is usually poor; therefore, the most promising long-term approach is to clear this infection before transplantation.     

Liver transplantation from living related donors: Review of world experience and its implications for India

Living related liver transplantation (LRLT) was made possible because of a better understanding of the anatomy of the liver and advances made in hepatic surgical techniques. It was developed to reduce the waiting period for pediatric recipients. In countries like Japan, which do not have brain stem death legislation, LRLT is the only modality available for treating end stage liver disease. The world experience has shown that LRLT has been successfully performed in a variety of conditions leading to acute and chronic liver failure not only in children, but in young adults as well. The initial results of LRLT appear to be better than liver transplantation from cadaveric organs in terms of graft survival and function. Donor safety has been of prime concern. LRLT has tremendous petential in India with or without the brain stem death legislation. Liver transplantation has not been performed in India although the need and expertise for it exists.     

Pediatric liver transplantation review of the literature 2007–2008

Abstract: Several hundred publications in the years 2007 and 2008 elucidate the ongoing progress in pediatric liver transplantation. Compared with previous reviews (2005–2006 and 2006–2007), the trend of reducing the initial loss of patients and grafts is stabilized and reaches more than 90% one‐yr cumulative patient survival. Given this remarkable progress, the issue of QoL after pediatric liver transplantation becomes even more important and will be addressed with special emphasis. Long‐term side effects of calcineurin inhibitors, growth, food allergy, adherence, pregnancy, and sexual function will be discussed in this context. Apart from these topics, papers reporting new insights in immunosuppression and ways to achieve immunotolerance will be reviewed. New trends in setting the indication for liver transplantation in children will be referred. This review will only reflect these aspects of pediatric liver transplantation.     

Outcomes after liver transplantation: keep the end in mind

Since the advent of transplantation as a life-saving procedure for patients with end-stage liver disease, more than 15,000 children and adolescents have received liver transplants. With the improvements in long-term posttransplant survival offered by advances in medical and surgical therapy, the concept of transplantation outcome has expanded beyond simple patient and graft survival rates. The quality of the life years restored, the long-term complications of transplant immunosuppression, and the overall cost of care have been increasingly recognized as important components of liver transplantation outcome. This review focuses on the efforts of a single pediatric transplant center to examine the incidence of, and risk factors for, common posttransplantation complications, to characterize posttransplantation health-related quality of life, to describe the cost of posttransplant care, and to implement novel programs to improve health care delivery. Together, these projects set the future course for research and care improvement initiatives in this population and encourage us to "keep the end in mind" when considering pediatric liver transplantation.     

Nitric oxide for post‐liver‐transplantation hypoxemia in pediatric hepatopulmonary syndrome: Case report and review

Schiller O, Avitzur Y, Kadmon G, Nahum E, Steinberg RM, Nachmias V, Schonfeld T. Nitric oxide for post‐liver‐transplantation hypoxemia in pediatric hepatopulmonary syndrome: Case report and review.
Pediatr Transplantation 2011: 15: E130–E134. © 2010 John Wiley & Sons A/S. Abstract: HPS is rare in the pediatric population. Liver transplantation is the ultimate treatment for severe HPS. There are only a few case reports and one series of children in whom HPS was the main indication for liver transplantation. Outcome was good in most of them, with full regression of the pulmonary process. However, hypoxemia in the early post‐operative course can have severe consequences, and effective treatment modalities are needed. There are rare instances of the use of iNO for the treatment of post‐operative hypoxemia. We describe a 10.5‐yr‐old boy with severe HPS owing to chronic liver disease after bone marrow transplantation. Liver transplantation from a living related donor (the same sister who donated the bone marrow) was complicated by severe hypoxemia on POD 2. iNO was administered via the ventilator circuit and, after extubation, through nasal prongs. It was slowly tapered down and stopped on POD 10. The child had an otherwise uneventful course and was discharged home on POD 21 with normal oxygen saturation. Liver transplantation should be offered to children with severe HPS. iNO can reverse the hypoxemia that may occur after the operation.     

Orthotopic liver transplantation in children: two-year experience with 47 patients

During a 24-month period (May 1981 to May 1983), 47 pediatric patients (ranging in age from 7 months to 18 years) underwent orthotopic liver transplantation using cyclosporine and prednisone. Major indications were biliary atresia/hypoplasia, and metabolic liver disease. Thirty-two of 138 patients evaluated for the procedure died prior to transplantation. Thirty patients are alive from 6 to 29 months later including 7/15 patients who required retransplantation. Twenty-one of 32 patients are alive at 1 year following initial transplantation. All 30 survivors are clinically well and living at home; only one has an abnormal bilirubin level. Serious, life-threatening medical and surgical complications were common during the early months following transplantation. With one exception, deaths and major rejection episodes occurred early (before 120 days). All survivors are relieved of the stigmata of chronic liver disease, and many have demonstrated catch-up growth. Liver transplantation is an effective treatment for end-stage pediatric liver disease.     

The role of liver transplantation for hepatic adenomatosis in the pediatric population: Case report and review of the literature

Wellen JR, Anderson CD, Doyle M, Shenoy S, Nadler M, Turmelle Y, Shepherd R, Chapman WC, Lowell JA. The role of liver transplantation for hepatic adenomatosis in the pediatric population: Case report and review of the literature.
Pediatr Transplantation 2010: 14: E16–E19. © 2009 John Wiley & Sons A/S. Abstract: Hepatic adenomas are benign lesions often found in young women during childbearing age. These tumors are often solitary but can also be multiple in which case this is referred to as hepatic adenomatosis (HA). HA is defined as having greater than or equal to ten adenomas within an otherwise normal liver. We present a case of a teenager with HA who underwent an orthotopic liver transplant for complications of her HA. To date there are only four reports of teenagers, without an underlying glycogen storage disease, who have undergone a liver transplant for HA. Liver transplantation within the pediatric population is an acceptable treatment for HA that are deemed unresectable.     

Progress in surgical techniques in pediatric liver transplantation

Bartlett A, Rela M. Progress in surgical techniques in paediatric liver transplantation.
Pediatr Transplantation 2010: 14: 33–40. © 2009 John Wiley & Sons A/S.
Abstract  Pediatric LT has evolved into an accepted treatment modality for children with end-stage liver disease with excellent long-term graft and patient survival. A number of factors have been critical in the improvement in recipient outcomes including a change in organ donation from donation after cardiac death, to donation after brain death, and more recently live donor LT and a better appreciation of hepatic anatomy allowing for split LT, LDLT, auxiliary partial orthotopic LT, and laparoscopic donor hepatectomy. In this review we summarize the surgical advances that have occurred in pediatric LT that have been important in shaping what is now considered by many to be a routine operation.     

Liver transplantation for progressive familial intrahepatic cholestasis: clinical and histopathological findings, outcome and impact on growth

In this study, we analyze the demographic features, clinical and histopathological findings in patients who underwent liver transplantation for progressive familial intrahepatic cholestasis. We also analyze outcome and impact of liver transplantation on growth and bone mineral content. Most of the patients were presented with jaundice mainly beginning within the first six months. At the time of initial admission; eight patients had short stature (height SD score<2), and four patients had weight SD score<2. Liver transplantation were performed at the age of 43.2+/-27 months (range 9 to 96 months), 6.5+/-3.5 months later after the first admission. Infection, surgical complications and osmotic diarrhea associated with severe metabolic acidosis were noted in 41.4%, 16.6% and 33.3%, respectively. One patient developed posttransplant lymphoproliferative disorder. Overall; 1 year graft and patient survival was 69.2% and 75%, respectively. At the end of the 1st year only 2 patients had height SD score<2. Linear regression of height gain against increase in total body BMD measured at the time of transplantation and 1 year after liver transplantation gave a coefficient r=0.588 (p=0.074). No correlation was found between the height gain and age and PELD score at time of transplantation, and no difference was noted between the sexes and donor type. Liver transplantation is effective treatment modality with good outcome and little morbidity, and increases the growth acceleration in patients with PFIC associated with cirrhosis.     

The current status of pediatric liver transplantation in Mainland China

Zhou J, Shen Z, He Y, Zheng S, Fan J. The current status of pediatric liver transplantation in Mainland China.
Pediatr Transplantation 2010: 14:575–582. © 2010 John Wiley & Sons A/S. Abstract: The aim of this article is to study the current status of pediatric liver transplantation in Mainland China. A total of 337 cases of pediatric liver transplantation enrolled in CLTR between 1993 and May 2009 were analyzed retrospectively. The median transplant age was 8.7 yr (64 day–17.8 yr), and Wilson’s disease was the most common indication (35.4%). Liver transplantation for biliary atresia accounted for 49.3% and 54.2% in 2008 and 2009 and had become the most common indication nowadays. One‐ and three‐yr survival rates of children transplanted at age<1 yr were 69.2% and 59.3%, respectively, and were significantly worse than those transplanted at age≥1 yr (83.9% and 76.6%, p < 0.05).In 63.8% (208/326) of the patients, LDLT was used with an overall one‐ and three‐yr survival rates of 87.5% and 84.4%, respectively. The one‐ and three‐yr survival for DDLT was significantly lower (66.7% and 52.2%, p < 0.05). The one‐ and three‐yr survival rates for those transplanted in era 1993–2000 were 63.6% and 36.4%, respectively, and the one‐ and two‐yr survival rates in the latest era (2007‐2009) were markedly improved (81.9% and 79.0%, p < 0.05). Cox’s analysis identified DDLT (HR = 2.90, CI 95% 1.5–5.6), being transplanted in era 1993–2000(HR = 3.4, CI 95% 1.1–10.2), fulminant liver failure (HR = 6.0, CI 95% 2.0–17.5), and malignancy (HR = 3.8, CI 95% 1.4–10.3) as predictors of increased mortality, and children transplanted at age 8–17 yr have an better survival (HR = 0.2, CI 95% 0.1–0.6). We concluded that pediatric liver transplantation is gradually developing and would probably be a promising therapy for pediatric end‐stage liver diseases in Mainland China.     

Liver transplantation in children with glycogen storage disease: controversies and evaluation of the risk/benefit of this procedure

Abstract:  GSD-I, III, and IV are congenital disorders of glycogen metabolism that are commonly associated with severe liver disease. Liver transplantation has been proposed as a therapy for these disorders. While liver transplantation corrects the primary hepatic enzyme defect, the extrahepatic manifestations of GSD often complicate post-transplantation management. Upon review of the English-language literature, 42 children <19 yr of age were discovered to have undergone liver transplantation for complications associated with GSD (18 patients with GSD-Ia, six with GSD-Ib, one with GSD-III, 17 with GSD-IV). An additional two children followed at our institution have undergone liver transplantation for GSD complications (one with GSD-Ia and one with GSD-III) and are included in this review. The risks and benefits of liver transplantation should be considered prior to performing liver transplantation in these metabolic disorders, particularly in GSD-Ia. As liver pathology is not the major source of morbidity in GSD-Ib and GSD-IIIa, liver transplantation should only be performed when there is high risk for HCC or evidence of substantial cirrhosis or liver dysfunction. Liver transplantation remains the best option for treatment of GSD-IV.     

Pediatric heart transplant

Pediatric heart transplantation has additional and unique aspects from standard pediatric heart surgery and adult heart transplantation. The purpose of this article is to review pediatric heart transplantation and special surgical considerations. The methods used by the authors involved reviewing the literature and surgical techniques surrounding this patient population and procedure. The article presents a general review of the topic including the history, current state, surgical approaches, post-operative management, and outcomes in this patient population.     

Long-term survival after liver transplantation in children with metabolic disorders

BACKGROUND: Liver transplantation for inherited metabolic disorders aims to save the patient's life when the disorder is expected to progress to organ failure, and to cure the underlying metabolic defect. METHODS: We retrospectively analyzed 146 pediatric liver transplants (28 metabolic; 118 non-metabolic) performed between 1986 and 2000. RESULTS: Twenty-eight transplants were performed in 24 children with metabolic disease (8 females; 16 males; age range 3 months to 17 yr). Indications included alpha-1-antitrypsin deficiency (n = 8), two cases each of hyperoxaluria type 1, Wilson's disease, hereditary tyrosinemia type I, citrullinemia, methylmalonic acidemia, and one case each of propionic acidemia, Crigler-Najjar syndrome type I, neonatal hemachromatosis, hemophilia B, Niemann-Pick disease type B, and cystic fibrosis. Eighteen transplants were whole organ grafts and 10 were lobar or segmental. Auxiliary liver transplants were performed in two patients and three received combined liver-kidney transplants. There were three deaths from sepsis, two from chronic rejection, and one from fulminant hepatitis. Seven of 10 patients currently of school age are within 1 yr of expected grade and three who had pretransplant developmental delay have remained in special education. Actuarial survival rates at 5 and 10 yr are 78% and 68%, respectively, with mean follow-up in excess of 5 yr. These results compare favorably to 100 pediatric patients transplanted for non-metabolic etiologies (65% and 61%, respectively) (p= NS). CONCLUSIONS: Pediatric liver transplantation for metabolic disorders results in excellent clinical and biochemical outcome with long survival and excellent quality of life for most recipients.     

Current development and future outlook of pediatric liver transplantationCSCD

It has been over 60 years since the first global case of pediatric liver transplantation. With continuous refinements of surgical techniques and post-surgical management of pediatric liver transplantation, the number of surgeries is steadily rising while surgical quality is gradually greater. Such developed countries as Europe, USA, Japan and South Korea have enjoyed an early start and accumulated rich experiences of treatment discipline and long-term management of patients and grafts. Pediatric liver transplantation in Chinese mainland began late but has been rapidly developing in recent years. This review summarized the overall development and focused upon key issues of domestic and foreign pediatric liver transplantations. © 2022, Science and Technology Association of Hunan Province. All rights reserved.