An elderly man with cholestatic jaundice due to biliary hydatidosis

Hydatid disease is found throughout the world, with a higher prevalence in the Mediterranean area. In Spain, the most endemic regions are Rioja and Aragon, with rates above 10 cases/100,000 inhabitants, followed by Castilla-La Mancha and Castilla y Leon (5-10 cases/100,000 inhabitants). This parasitic disease is caused by the larval stage of Echinococcus granulosus (EG) and the main organs affected are the liver and the lung (85 % cases). We present a case of obstructive jaundice and secondary cholangitis due to a biliary hydatidosis. Abdominal computed tomography scan showed dilatation of the entire biliary tract. The technique used for diagnosis and treatment was endoscopic retrograde cholangiopancreatography.     

Surgical alternatives in the treatment of hepatic hydatidosis]

Echinococcosis is an endemic zoonosis in the south of Chile, so we have the opportunity to treat many patients especially the liver location which is the most common situation of this disease. Hepatic hydatid cyst presents its own morbidity and mortality due its complications and associated with surgical procedures. Morbidity has rates between 11 and 86% and is related with previous surgical interventions, cysts complications, the necessity of perform additional procedures during surgery and the treatment of other disease locations. Mortality by otherwise has rates below 5%. But both, morbidity and mortality persist high in spite of technological advances and therapeutical improvement. The surgical treatment of hepatic hydatidosis can be divided in four phases: surgical area isolation, cysts evacuation, treatment of cyst complications (biliary tract rupture, hepatothoracic transit, etc.), and residual cavity treatment. For each one exist different alternatives. On the other hand, hydatid cyst surgery can be classified in conservative and resective procedures. In the first group we consider marsupialization, cystostomy, Knowsly or Posadas operation and cystojejunostomy. In the resective group we include pericystectomy, partial or subtotal cystectomy and the different types of hepatectomies. Finally, some other surgical procedures are under evaluation. These include the laparoscopic approach of liver echinoccosis with few studies in the field, but with hopeful results.     

Outcomes of patients with benign liver diseases undergoing living donor versus deceased donor liver transplantation

Background/Aims

The number of people undergoing living donor liver transplantation (LDLT) has increased rapidly in many transplant centres. Patients considering LDLT need to know whether LDLT is riskier than deceased donor liver transplantation (DDLT). The aim of this study was to compare the outcomes of patients undergoing LDLT versus DDLT.

Methods

A total of 349 patients with benign liver diseases were recruited from 2005 to 2011 for this study. LDLT was performed in 128 patients, and DDLT was performed in 221 patients. Pre- and intra-operative variables for the two groups were compared. Statistically analysed post-operative outcomes include the postoperative incidence of complication, biliary and vascular complication, hepatitis B virus (HBV) recurrence, long-term survival rate and outcomes of emergency transplantation.

Results

The waiting times were 22.10±15.31 days for the patients undergoing LDLT versus 35.81±29.18 days for the patients undergoing DDLT. The cold ischemia time (CIT) was 119.34±19.75 minutes for the LDLT group and 346±154.18 for DDLT group. LDLT group had higher intraoperative blood loss, but red blood cell (RBC) transfusion was not different. Similar ≥ Clavien III complications, vascular complications, hepatitis B virus (HBV) recurrence and long-term survival rates were noted. LDLT patients suffered a higher incidence of biliary complications in the early postoperative days. However, during the long-term follow-up period, biliary complication rates were similar between the two groups. The long-term survival rate of patients undergoing emergency transplantation was lower than of patients undergoing elective transplantation. However, no significant difference was observed between emergency LDLT and emergency DDLT.

Conclusions

Patients undergoing LDLT achieved similar outcomes to patients undergoing DDLT. Although LDLT patients may suffer a higher incidence of early biliary complications, the total biliary complication rate was similar during the long-term follow-up period.     

Complications of cardiac catheterization: one centre's experience.

Data on complication rates in a cardiac catheterization laboratory were prospectively gathered over a 6-year period. During this time 7960 catheterizations were performed. Death occurred in seven (0.1%) of the cases. The difference between the mortality rates for procedures performed with and without systemically administered heparin (0.04% and 0.2% respectively) was barely statistically significant (p less than 0.05). A significant complication occurred in 1.5% of the cases; however, most did not have long-term sequelae. No significant change in the annual rate of such complications was seen during the study period. Such a tabulation permits audit of quality of care, points out changing trends in morbidity and offers meaningful information on the safety of cardiac catheterization to referring physicians and their patients.     

Analysis of some variables in patients with hydatidosis surgically treated at the Regional Hospital of Valdivia, Chile, 1976-1986

A survey of surgically treated cases of hydatidosis in the province of Valdivia, Chile, was done. Data were provided by the Regional Hospital of Valdivia, and a period of eleven years was covered (1976-1986). Disease, treatment and related data from each patient were consigned. For the analysis, the information was grouped according to: sex, age, cyst location, condition of the cyst, concomitance with another pathology, condition at time of discharge, hospitalization period and number of repeat hospitalizations. In the period studied, 197 cases of hydatidosis were surgically treated. The sex distribution was 53.3% for males and 46.7 for females; the age group 10-19 years was the most affected by the disease (19.3%). The most frequent locations of hydatid cysts were the liver (58.9%) and lungs (36.5%); the 38.6% of the patients showed complicated cysts, mainly with bacterial infections (11.7%); also, 20% of the cases presented a concomitant liver-related pathology. After the operation, 153 (77.7%) clinically recovered and 27 (13.7%) non-recovered patients left the hospital and 7 (3.5%) cases died while hospitalized. The average hospitalization period was 29 days. During 1976-1986, 24 patients were rehospitalized.     

肝内外胆管多发结石肝部分切除术后肝功能衰竭的诊治

目的：探讨肝内外胆管多发结石术后肝功能衰竭的预防、诊断及治疗。方法：我院2011年1 月~2013 年12 月收治肝内外胆 管多发结石行手术治疗患者共126 例,术后发生肝功能衰竭者6 例,均是合并肝叶切除患者。及时准确诊断肝功能衰竭后予抗 炎、护肝、止血、输血、糖皮质激素、抑酸、人血白蛋白、利尿、降血氨、血浆置换及对症支持等治疗。结果：6 例患者出院前复查总胆 红素28.3~ 58.7 mmol/L,谷丙转氨酶16~ 62 U/L,谷草转氨酶12~ 85 U/L,血浆白蛋白32.1~ 37.8 g/L,凝血功能基本正常,腹水消 失,血氨正常,上消化道出血停止。术后12~ 35 d出院,平均18 d。6例患者术后长期随访,目前均存活。结论：肝功能衰竭是肝脏 及胆道术后最为严重的并发症之一,充分的术前准备及评估,术后的及时诊断及治疗,可明显降低其死亡率。     

Observations on clinical efficacy of albendazole emulsion in 264 cases of hepatic cystic echinococcosis

Two regimens of albendazole emulsion (AbzE), a novel formulation, were used in the treatment of 264 cases of hepatic cystic echinococcosis. AbzE 10 mg/kg per day (calculated by albendazole base) was administered orally to 71 cases for 6 months to over 1 year. Imaging evaluation at the end of courses showed overall efficacy in 97.2%, (cure rate 60.6%, and inefficacy rate 2.8%); The follow-up study on 62 cases 3-4 years post therapeutic courses showed overall efficacy in 92.0% (cure rate 83.9%, ineffective rate 1.5% and recurrence rate 6.5%); Abz 12.5 mg/kg per day was administered orally to 193 cases for 3 months to over 1 year, resulting in an overall efficacy of 97.9%, (cure rate 75.1% and inefficacy rate 2.1%). The follow-up study in 139 cases 2-4 years post treatment demonstrated efficacy in 89.2%, (cure rate 84.2% and recurrence rate 10.8%); Mild reversible adverse reactions were observed in 14.4% of the patients. Retreatment of recurrent hydatidosis patients with AbzE provided promising results. AbzE is considered to be superior to the albendazole tablet or capsule formulations currently used in treatment of liver cystic hydatid disease.     

Serologic longitudinal follow-up of surgically treated patients for hydatidosis]

Beginning with the register of human hydatidosis cases, kept by hydatidosis struggle programs of Rio Negro Province, was done the serologic longitudinal follow-up in patients operated on in the period 1980-1987, in the influence area of Ingeniero Jacobacci, Rio Negro, Argentina. The local Supervision Program obtained blood samples of 36 patients during 1990 (59% of them were operated on in the considered period), five patients died corresponding the remaining to persons to whom did not live in Ingeniero Jacobacci or to whom were not possible to evaluate. The blood samples were studied by DD5, confirming the disease immunologically in 18 cases (50%). In this work are evaluated possible causes of reinfection: surgery technic, individual susceptibility, immunological factors.     

麻疹77例临床分析

目的 分析2005年77例麻疹病人的发病与流行病学特征。方法 回顾性分析我院2005年收治的77例麻疹病人的年龄、预防接种史、发病时间、临床表现、并发症、实验室检查及预后等资料。结果 麻疹发病高峰时间集中在3～6月份,发病年龄主要在20～49岁(94．8％),高热持续1周的病人数多(62．3％),有扁桃体炎(10．9％)、血尿(8．2％)、腹泻(5．5％)、口腔溃疡(4．1％)、心悸(2．7％)等临床表现;并发症表现为:肝功能异常(63．6％)和肺部感染(16．9％);77例麻疹患者中75例麻疹抗体IgM阳性;预后良好。结论 麻疹该年出现的高峰时间在3～6月份,以成人发病为主,并以外来人群居多,高热持续时间长,并发症以肝功能异常最多见,麻疹抗体IgM仍是实验室诊断的主要指标。     

A Nationwide Cohort Study of Mortality Risk and Long-Term Prognosis in Infective Endocarditis in Sweden

Objectives: Infective endocarditis (IE) remains a serious disease with substantial mortality. In this study we investigated the incidence of IE, as well as its associated short and long term mortality rates.

Methods

The IE cases were identified in the Swedish national inpatient register using ICD-10 codes, and then linked to the population register in order to identify deaths in the cohort. Crude mortality rates among IE patients were obtained for different time intervals. These rates were directly standardized using sex- and age-matched mortality in the general population.

Results

The cohort consisted of 7603 individuals and 7817 episodes of IE during 1997–2007. The 30 days all-cause crude mortality rate was 10.4% and the standardized mortality ratio (SMR) was 33.7 (95% confidence interval [CI]: 31.0–36.6). Excluding the first year of follow-up, the long term mortality (1–5 years) showed an increased SMR of 2.2 (95% CI: 2.0–2.3) compared to the general population. Significantly higher SMR was found for cases of IE younger than 65 years of age with a 1–5 year SMR of 6.3, and intravenous drug-users with a SMR of 19.1. Native valve IE cases, in which surgery was performed had lower crude mortality rates and Mantel-Haenzel odds ratios of less than one compared to those with medical therapy alone during 30-day and 5-years follow-up.

Conclusions

The 30-days crude mortality rate for IE was 10.4% and long-term relative mortality risk remains increased even up to 5 years of follow-up, therefore a close monitoring of these patients would be of value.     

Reconstruction with Modular Hemipelvic Endoprosthesis after Pelvic Tumor Resection: A Report of 50 Consecutive Cases

PurposeTo evaluate the effectiveness of reconstruction with a modular hemipelvic endoprosthesis after pelvic tumor resection.MethodsWe retrospectively studied 50 consecutive patients diagnosed with pelvic tumor from 2003 to 2013. All patients received limb-salvage surgery and reconstruction with modular hemipelvic endoprosthesis.ResultsPatients were followed for an average of 54 months. At the most recent follow-up, 32 patients were alive with an estimated three-year and five-year survival rate of 66.3% and 57.5% according to the Kaplan-Meier survival analysis. Eighteen patients died from the tumor, with a mean survival of 28 months, and 9 patients experienced local recurrence at an average of 19.6 months after surgery. Patients with marginal or intracapsular surgical margins had a significantly higher recurrence rate than those with wide margins (p=0.02). Metastasis occurred in 12 cases at an average of 16 months after surgery. The perioperative complication rate was 48.0%, and the most common complications were wound healing disturbance (28.0%) and deep infection (14.0%). The endoprosthetic complication rate was 16.0%, and breakage of the pubic connection plate was the most common complication. The mean Musculoskeletal Tumor Society score was 61.4%.ConclusionReconstruction with a modular hemipelvic endoprosthesis after pelvic tumor resection can improve function, with an acceptable complication rate.     

Intraductal Radiofrequency Ablation Followed by Locoregional Tumor Treatments for Treating Occluded Biliary Stents in Non-Resectable Malignant Biliary Obstruction: A Single-Institution Experience

Objectives

To determine the safety and feasibility of intraductal radiofrequency ablation (RFA) followed by locoregional tumor treatments in patients with non-resectable malignant biliary obstruction and stent re-occlusion.

Methods

Fourteen patients with malignant biliary obstruction and blocked metal stents were studied retrospectively. All had intraductal RFA followed by locoregional tumor treatments and were monitored clinically and radiologically. The practicality, safety, postoperative complications, jaundice remission, stent patency and survival time were analyzed.

Results

Combination treatment was successful for all patients. There were no severe complications during RFA or local treatments. All patients had stent patency restored, with a decline in serum bilirubin. Three patients had recurrent jaundice by 195, 237 and 357 days; two patients underwent repeat intraductal RFA; and one required an internal-external biliary drain. The average stent patency time was 234 days (range 187-544 days). With a median follow-up of 384 days (range 187-544 days), six patients were alive, while eight had died. There was no mortality at 30 days. The 3, 6, 12 and 18 month survival rates were 100%, 100%, 64.3% and 42.9%, respectively.

Conclusion

Intraductal RFA followed by locoregional tumor treatments for occluded metal stents is safe and practically feasible and potential increase stent patency and survival times.     

ALPPS Procedure for Extended Liver Resections: A Single Centre Experience and a Systematic Review

Aim

To report a single-centre experience with the novel Associating Liver Partition and Portal vein ligation for Staged hepatectomy (ALPPS) technique and systematically review the related literature.

Methods

Since January 2013, patients with extended primary or secondary liver tumors whose future liver remnant (FLR) was considered too small to allow hepatic resection were prospectively assessed for the ALPPS procedure. A systematic literature search was performed using PubMed, Scopus and the Cochrane Library Central.

Results

Until July 2014 ALPPS was completed in 9 patients whose mean age was 60±8 years. Indications for surgical resection were metastases from colorectal cancer in 3 cases, perihilar cholangiocarcinoma in 3 cases, intrahepatic cholangiocarcinoma in 2 cases and hepatocellular carcinoma without chronic liver disease in 1 case. The calculated FLR volume was 289±122 mL (21.1±5.5%) before ALPPS-1 and 528±121 mL (32.2±5.7%) before ALLPS-2 (p<0.001). The increase in FLR between the two procedures was 96±47% (range: 24–160%, p<0.001). Additional interventions were performed in 4 cases: 3 patients underwent Roux-en-Y hepaticojejunostomy, and one case underwent wedge resection of a residual tumor in the FLR. The average time between the first and second step of the procedure was 10.8±2.9 days. The average hospital stay was 24.1±13.3 days. There was 1 postoperative death due to hepatic failure in the oldest patient of this series who had a perihilar cholangiocarcinoma and concomitant liver fibrosis; 11 complications occurred in 6 patients, 4 of whom had grade III or above disease. After a mean follow-up of 17.1±8.5 months, the overall survival was 89% at 3–6 and 12 months. The recurrence-free survival was 100%, 87.5% and 75% at 3-6-12 months respectively. The literature search yielded 148 articles, of which 22 articles published between 2012 and 2015 were included in this systematic review.

Conclusion

The ALPPS technique effectively increased the resectability of otherwise inoperable liver tumors. The postoperative morbidity in our series was high in accordance with the data from the systematic review. Age, liver fibrosis and presence of biliary stenting were predisposing factors for postoperative morbidity and mortality.     

肝动脉栓塞化疗术对原发性肝癌伴 有不同类型肝炎后肝硬化患者预后的影响

目的:探讨肝动脉栓塞化疗(TACE)对原发性肝癌伴有不同肝炎后肝硬化类型患者术后肝功能、凝血功能及其对远期预后的影响。方法:2007年8月至2009年8月,131例曾行TACE的伴有不同肝炎后肝硬化原发性肝癌患者,以肝炎后肝硬化类别(乙肝、丙肝)分类,乙肝后肝硬化组为组1,丙肝后肝硬化组为组2,随访观察术后一年肝功能、凝血功能、血小板等的变化以及预后。两组研究因素采用SPSS17.0进行卡方检验,随访预后采用Kaplan-Meier方法计算生存率,Log-rank法检验生存差异。以P<0.05为差异有统计学意义。结果:随访统计结果显示术后半年和一年AST、ALT、ALP、GGT、PT、PLT在两组间均无统计学差异,(P>0.05);组一半年、一年远处转移率同组二差异间无统计学意义;组一半年、一年生存率分别为70.1%,48.1%;组二半年、一年生存率分别为68.5%,58.9%,两组间同期生存率差异无统计学意义。(X2=0.039,P=0.884;X2=0.183,P=0.669)。结论:TACE治疗PHC安全可靠,对于伴有乙肝或丙肝后肝硬化患者术后肝功能、凝血功能、疗效及预后效果相当。     

Epidemiology of Echinococcus granulosus in Arbil province, northern Iraq, 1990-1998

During the period 1990-1998, 99 cases of human cystic hydatidosis (12.4 cases per year) were surgically treated at the two main hospitals in Arbil province, northern Iraq, and from this the human occurence for the province was estimated to be 2 per 100,000 inhabitants. In the same area, 1270 sheep, 550 goats and 320 cattle were examined at slaughter for hydatid cysts and prevalence rates were found to be 15.0%, 6.2% and 10.9%, respectively. A decreasing tendency in livestock prevalences was found towards the end of the study period. As in humans, most of the hydatid cysts in livestock were located in the liver. Fertility of sheep cysts, i.e. those containing protoscoleces, was found to be significantly higher (64%) than that of goats (35.7%) and cattle (29.8%). The percentage of fertile cysts containing viable protoscoleces varied between 63 and 82% in the livers and between 72 and 79% in the lungs of the different animal species. A total of 97 stray dogs were examined post-mortem in the years 1991, 1992 and 1998, and Echinococcus granulosus worms were found in the intestines of 48 dogs (49.5%). High worm burdens (> 1000) were observed in 37% of the dogs, medium worm burdens (200-1000) in 41%, and low worm burdens (< 200) in 22%. In 1998, the prevalence of canine echinococcosis (24.3%) was found to be significantly lower than in 1991 (70.4%) and 1992 (60.6%). The prevalence of human hydatidosis did not differ significantly over the years, but the study confirmed that hydatidosis is endemic in northern Iraq, and that housewives, labourers and farmers appear to be at the greatest risk of infection.     

肝部分切除治疗肝内胆管结石患者术后并发症及影响因素分析

目的:分析肝部分切除术治疗肝内胆管结石患者术后并发症及影响因素。方法:选取我院收治的肝内胆管结石患者117例,均采取肝部分切除术治疗,对其临床资料进行回顾性分析,研究术后并发症的发生情况,并对影响因素进行分析。结果:本组117例患者,并发症发生率35.04%,其中肝功能衰竭1例,胆道出血2例,消化道出血6例,腹腔感染6例,胆瘘6例,胸腔积液8例,切口感染12例。并发症组患者术前白蛋白、手术时间、既往胆道手术史水平与非并发症组比较,差异均有统计学意义(P0.05)。肝部分切除术后的并发症多因素Logistic回归分析结果显示,手术时间、既往胆道手术史均是术后并发症独立风险因素。结论:肝部分切除术治疗肝内胆管结石患者术后并发症发生率较高,以切口感染和胸腔积液为最,患者的既往手术史以及手术时间均是影响并发症发生的重要危险因素,做好针对性预防可预防并发症的发生。     

Serum Antigen and Antibody Detection in Echinococcosis: Application in Serodiagnosis of Human Hydatidosis

Diagnosis of hydatidosis is based on immunodiagnostic methods along with radiological and ultrasound examinations. The objectives of the present study were to develop a specific and simple antigen-based ELISA method for diagnosis of hydatidosis and compare it with antibody detection method. The subjects in this study included 89 patients in the following groups: surgically confirmed hydatidosis patients (35 cases), control with other parasitic diseases (29 cases), and healthy controls (25 cases). Hyperimmune serum was raised against hydatid cyst fluid in rabbits. Anti-hydatid cyst IgG was purified by affinity chromatography using protein A column and labeled with horseradish peroxidase. Collected sera were assessed for hydatid cyst antigens and antibody by ELISA. Circulating hydatid antigen was found in 9 out of 35 patients with surgically confirmed hydatidosis. A sensitivity of 25.7% and a specificity of 98.0% were calculated for the antigen detection assay. Antibody detection by indirect ELISA, using antigen B, showed that 94.2% of patients (33 cases) have anti-hydatid cyst antibodies in their serum while cross reaction was noted in a few of non-hydatidosis patients. A sensitivity of 94.2% and specificity of 81.6% were found for the antibody detection assay. Findings of this study indicated that antibody detection assay is a sensitive approach for diagnosis of hydatid cyst while antigen detection assay might be a useful approach for assessment of the efficacy of treatment especially after removal of the cyst.     

Treatment of hepatic hydatid cysts by percutaneous aspiration and hypertonic saline injection: results of a cooperative work

Percutaneous puncture-aspiration-injection-reaspiration (PAIR) of hydatid liver cysts, was performed in 38 patients 14-80 years old, with a total of 60 liver hydatid cysts. After aspiration under computed tomography guidance, hypertonic saline was injected into the cystic cavities of patients as a scolecidal agent. No major complications were associated with the procedures. In the follow-up period of 18 months, control CT scans of 35 cysts revealed a gradual decrease in cyst size with a mean volume reduction of 66%. Complications included two cases of urticaria, one case of anaphylaxis and one subcapsular hematoma. No mortality occurred. It is concluded that percutaneous aspiration and hypertonic saline injection for liver hydatid cysts appears to be an effective form of treatment and may eventually prove to be an alternative to surgical intervention.     

Assessment of prevalence of hydatidosis in slaughtered Sawakny sheep in Riyadh city,Saudi Arabia

Hydatidosis, or echincoccosis, is a serious medical and veterinary problem in many countries, particularly those with rural communities where there is a greater contact between dogs and domestic animals. Domestic livestock act as intermediate hosts which are the main reservoir for the disease in humans. It is therefore very important to estimate the prevalence of hydatid cysts in slaughtered animals since it can be transmitted to humans through dogs, which act as the final host for the disease. From this point of view, the present study was suggested to determine the prevalence of hydatidosis in Sawakny sheep slaughtered in Riyadh city, Saudi Arabia. During the course of the study 12,569 Sawakny sheep were inspected for hydatidosis infection. An overall prevalence of 1.06% was detected among the examined sheep, with the highest prevalence occurring in winter (1.38%) and lowest prevalence in summer (0.67%). Sheep aged 6–12 months had a higher rate of infection than older animals, and males were the predominant carriers of infection (97.7%) compared to females (2.3%). The liver was the most infected organ (79.1%), followed by the lungs (14.6%), while concurrent infections of both the liver and the lungs occurred in 6% of cases. The fertility and viability rates of hydatid cysts in the liver (70.1% and 85.1% respectively) were higher than that in any other organs. In conclusion, it is evident that fertile cysts in slaughtered sheep could have an important role in the continuation of hydatid cyst transmission to humans through dogs. Considerable effort should be devoted to controlling the transmission of cysts from abattoirs by the secure disposal of infected offal. In addition, plans are required for further epidemiological studies and control programs.     

Cirrhotic changes in livers from children undergoing transplantation. Image analysis.

The pattern and extent of disorganization of the liver architecture were studied in 25 children undergoing orthotopic liver transplantation for cirrhosis. Image analysis techniques based on mathematical morphology were used to define seven parameters for each case, including fibrosis index (percentage of Sirius-red-stained areas), three categories of regenerative nodules (< 0.8, 0.8-1.6 and > 1.6 mm in diameter) and three categories of fibrous septa (< 0.4, 0.4-1.2 and > 1.2 mm in width). Fibrosis index ranged from 10.2% to 51.9%. Percentage of small nodules of infralobular size (< 0.8 mm in diameter) varied from 31.4% to 98.2%. Percentage of large nodules, > 1.6 mm in diameter, in only four cases was > 15%. Multivariate clustering analysis classified the cases into three main groups. One of them included only cases of cirrhosis secondary to parenchymal disease or to inborn errors of metabolism. In their pattern, tiny nodules predominated, and the percentage of slender septa, < 0.4 mm in diameter, was very high, although the overall fibrosis index was relatively low. Patients with biliary cirrhosis were classified into two groups. In one the fibrosis index was low and the size of the nodules variable, with 12% large nodules. In the other the fibrosis index was high, and small nodules, < 0.8 mm in diameter, predominated. No relationship was found with age at transplantation or previous portoenterostomy. A presumptive explanation for this divergent evolution might be the occurrence of cholangitic episodes. Overall fibrosis that the liver can sustain without failure is apparently limited.(ABSTRACT TRUNCATED AT 250 WORDS)