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Fabry disease may be treated by enzyme replacement therapy (ERT), but the impact of chronic kidney disease (CKD) on the response to therapy remains unclear. The aim of the present study was to analyse the incidence and predictors of clinical events in patients on ERT., Study Design: Multicentre retrospective observational analysis of patients diagnosed and treated with ERT for Fabry disease. The primary outcome was the first renal, neurological or cardiological events or death during a follow-up of 60 months (24-120)., Results: In 69 patients (42 males, 27 females, mean age 44.6±13.7 years), at the end of follow-up, eGFR and the left ventricular septum thickness remained stable and the urinary albumin: creatinine ratio tended to decrease, but this decrease only approached significance in patients on agalsidase-beta (242-128mg/g (p=0.05). At the end of follow-up, 21 (30%) patients had suffered an incident clinical event: 6 renal, 2 neurological and 13 cardiological (including 3 deaths). Events were more frequent in patients with baseline eGFR≤60ml/min/1.73m 2 (log Rank 12.423, p=0.001), and this remained significant even after excluding incident renal events (log Rank 4.086, p=0.043) and in males and in females. Lower baseline eGFR was associated with a 3- to 7-fold increase the risk of clinical events in different Cox models., Conclusions: GFR at the initiation of ERT is the main predictor of clinical events, both in males and in females, suggesting that start of ERT prior to the development of CKD is associated with better outcomes., (Copyright © 2021. Published by Elsevier España, S.L.U.)

Objectives: Antibiotic treatment is vital in patients with severe sepsis/septic shock. The objectives were to assess the degree of concordance between antibiotic prescribed in emergencies and post requirements; to relate it to health outcomes (mortality) and to analyze the reasons for disagreement., Material and Methods: Retrospective descriptive study of antibiotic treatment prescribed in emergencies and the subsequent treatment in patients with criteria of severe sepsis/septic shock in 2013. We collected patient demographic characteristics, infectious focus, antibiotic prescribed from emergencies and subsequent changes. It was considered concordant if there were no changes, if there were changes, but the initial antibiotic was right and suspensions for end of treatment. Mortality and evolution were analyzed., Results: Six hundred patients were included. A 60% experienced changes respect to the antibiotic treatment initiated in emergencies (87.6% justified), with a degree of overall antibiotic concordance of 47.5% The mortality rate at end-point was 9.83%, with no statistically significant relationship with the degree of concordance (OR=0.864 (0.503-1.484)/χ2=0.28; p=0.597). Reasons for change of antibiotic: clinical outcome (17.96%), change of spectrum (35.03%), de-escalation (41.32%), sequential therapy (8.68%). An 11% required ICU admission. Clinical outcomes: resolution of the disease (79.2%), readmission after 30 days (7.7%) and transfer to health centers (4.5%). The median hospital stay was 7 days., Conclusions: The degree of concordance antibiotic was quite high, and the mortality rate was lower than that described in the literature, without relating to the discordance. The presence of concordance was associated with fewer readmissions and ICU admissions. The main reasons for disagreement were inadequate spectrum selection and change after microbiological crops.

There have been biliary complications since the beginning of liver transplants, and is a topic of great interest due to its high incidence, as well as their influence on morbidity and mortality. The biliary fistula is currently uncommon and its management is straightforward. Anastomotic stenosis continues to have an incidence of 10-15%. Although the current treatment of choice is endoscopic retrograde cholangiopancreatography (ERCP), surgical treatment (hepatico-jejunostomy) continues to have an important role. Non-anastomotic stenosis has an incidence of 5-10%, and is associated with ischaemic or immunological factors, and usually involves a re-transplant. Choledocholithiasis has an incidence of 5-10%, with the treatment of choice being ERCP. However the treatment of biliary complications should be individualised. We must take into account, liver function, the general health status of the patient, and the availability and experience of the team in the different therapeutic options., (Copyright © 2011 AEC. Published by Elsevier Espana. All rights reserved.)

Introduction: Cephalic duodenopancreatectomy (CDP) is the treatment of choice in cancer of the head of the pancreas. However, it continues to have a high post-surgical morbidity and mortality. The aim of this article is to define variables that influence post-surgical morbidity and mortality after cephalic duodenopancreatectomy due to pancreatic adenocarcinoma (PA) cancer of the head of the pancreas (CHP)., Material and Methods: The variables were prospectively collected form patients operated on between 1991 and 2007, in order to investigate the factors of higher morbidity., Results: A total of 204 patients had been intervened due to PA, of whom 57 were older than 70 years. Of these patients, 119 had a CPD, 11 extended lymphadenectomy, 66 with pyloric conservation, and 8 with extension to total pancreatectomy due to involvement of the section margin. Portal or mesenteric vein resection was included in 35 cases. Post-surgical complications were detected in 45% of cases, the most frequent being: slow gastric emptying (20%), surgical wound infection (17%), pancreatic fistula (10%), and serious medical complications (8%). Further surgery was required in 13%, and the over post-surgical mortality was 7%. A patient age greater than 70 years, post-surgical haemoperitoneum, gastroenteric dehiscence, and the presence of medical complications were post-surgical mortality risk factors in the multivariate analysis. Pancreatic fistula was not a factor associated with post-surgical mortality., Conclusions: Cephalic duodenopancreatectomy is a safe technique but with a considerable morbidity. Patients over 70 years of age must be carefully selected before considering surgery. Serious medical complications must be treated aggressively to avoid an unfavourable progression., (Copyright © 2010 AEC. Published by Elsevier Espana. All rights reserved.)

Background and Objectives: Fabry disease is a hereditary disorder. Clinical manifestations are multisystemic. The majority of the patients remain undiagnosed until late in life, when alterations could be irreversible. Early detection of cardiac symptoms is of major interest in Fabry's disease (FD) in order to gain access to enzyme replacement therapy. Echo-Doppler tissular imaging (TDI) has been used as a cardiologic early marker in FD., Objectives: This study is intended to determine whether the cardiac magnetic resonance is as useful tool as TDI for the early detection of cardiac affectation in FD., Patients and Methods: Echocardiography, tissue Doppler and Cardio magnetic resonance was performed in 20 patients with confirmed Fabry Disease. Left ventricular hypertrophy was defined as septum and left ventricular posterior wall thickness ≥12 mm. An abnormal TDI velocity was defined as (Sa), (Ea) and/or (Aa) velocities <8 cm/s at either the septal or lateral corner. Late phase gadolinium-enhanced images sequences were obtained using magnetic resonance., Results: Twenty patients included in the study were divided into three groups: 1. Those without left ventricular hypertrophy nor tissue Doppler impairment 2. Those without left ventricular hypertrophy and tissue Doppler impairment 3. Those with left ventricular hypertrophy and Tissue Doppler impairment. Late gadolinium enhancement was found in only one patient, who has already altered DTI and LVH., Conclusion: Tissue Doppler imaging (TDI) is the only diagnostic tool able to provide early detection of cardiac affectation in patients with FD. Magnetic resonance provides information of the disease severity in patients with LVH, but can not be used as an early marker of cardiac disease in patients with FD. However MRI could be of great value for diagnostic stratification., (Copyright © 2009 Elsevier España, S.L. All rights reserved.)

Unlabelled: Management of biliary tract complications (BTC) after liver transplantation (LT) has progressed in recent years. The aims of this study were, to analyse the incidence and management in our institution of BTC after 1000 LT; and to study the management of patients with anastomotic strictures (AS)., Results: The incidence of BTC was 23%. There were 76 cases of bile leak, 106 cases of anastomotic strictures, 46 non-anastomotic strictures, 42 choledocolithiasis and 19 other complications. Among 106 cases of anastomotic strictures, radiological treatment, either PTC or ERCP, was initially indicated in 62. The AS of 38 patients (33%) were resolved with surgical treatment, 18 of them after a previous attempt at radiological treatment. Patients who were treated initially by radiologically required more procedures. Morbidity and mortality related to BTC were slightly higher in the group of patients treated by radiology (morbidity: surgical: 4 (18%) vs. radiological: 20 (32%); p=0.2 and mortality: surgical: 0% vs. radiological: 8 (11%); p=0.23). Among 46 patients with non-anastomotic strictures, 29 were resolved with retransplantation (63%)., Conclusions: Surgery has a significant role in the management of BTC, and is the treatment of choice in some cases of anastomotic strictures. Retransplantation may be the preferred option in patients with non-anastomotic strictures., (Copyright (c) 2009 AEC. Published by Elsevier Espana. All rights reserved.)

Management of the cystic lesions of the pancreas is of interest to general and pancreatic surgeons and physicians of other disciplines: gastroenterology, internal medicine, endoscopy, radiology, pathology, etc. The majority of cystic lesions are inflammatory pseudo-cysts. Cystic neoplasms represents only 10% of cystic lesions of the pancreas and 1% of pancreatic tumours. Preoperative diagnosis is crucial given the differences in natural history of the spectrum of benign, malignant, and borderline lesions. Serous cystadenoma is a benign lesion that requires non-surgical management if there are no symptoms. Mucinous neoplasms are premalignant lesions that mainly require pancreatic resection. Despite improved radiographic imaging techniques, definitive diagnosis is only made after studying the resection sample. The pancreatic surgical risk is a problem for the appropriate management of these patients.

Objective: The reported prevalence rate of bile duct cysts is very low. However, the clinical presentation of bile duct cysts is common to other hepatobiliary diseases. In this article, we report on a series of patients who have been surgically treated over the last 15 years., Material and Method: All the patients who had undergone bile duct cyst-related surgery at this hospital had their clinical history reviewed retrospectively from 1990 to 2002. Data were obtained prospectively from 2002 to 2005. The following variables were taken into account in our analysis: diagnosis data, surgical procedure, morbidity, post-surgery mortality rates, and follow-up., Results: Over the last 15 years, 18 patients have undergone surgery at our hospital (6 male, 12 female). The most common clinical presentation was that of abdominal pain and the usual symptoms associated with acute cholangitis. As for surgical procedure, a complete cyst resection with biliary derivation was performed in all 15 cases. The histopathological diagnosis was choledochal cyst in 12 cases, Caroli's disease in 5 cases and a malignant choledochal cyst (adenocarcinoma) in 1 case. The most frequent post-surgical complication was bile leak (3 cases, 16.6%). There was no post-surgical mortality (0%). There were no relapses in the subsequent follow-up, Conclusions: Our preferred surgical procedure is that of complete cyst resection with biliary derivation. Our overall results are similar to those of medical teams who practise a radical resective procedure, and better than those who practise partial resections.

Background: Unsuspected intrahepatic and extrahepatic metastases are frequently discovered at laparotomy in patients with resectable colorectal liver metastases (CLM), and 60% of these patients will develop a recurrent tumour within 3 years, after a "curative" liver resection. These findings strongly support the need for more effective preoperative staging. The combined positron emission tomography-computed tomography technique (PET-CT) has emerged as a promising diagnostic modality for determining whether patients with recurrent disease in the liver are suitable candidates for curative resection., Objective: The aim of this study was to assess the additional value of information provided by PET-CT compared to that of conventional radiological studies (CT and MR) in patients with resectable CLM., Patients and Method: Between June 2006 and August 2007, 63 patients evaluated for a first resection of CLM were entered into a prospective database. Each patient received a CT-MR and a PET-CT. Forty-three patients underwent a laparotomy and 42 a hepatectomy. The main end point of the study was to assess the impact of the PET-CT findings on the therapeutic strategy., Results: New findings in the PET-CT resulted in a change in the therapeutic strategy in 9 (14%) of the patients. However, PET-CT provided additional information was true positive by revealing abdominal extrahepatic metastases only in 4 (6.4%) patients, and falsely over-staged four patients and under staged one patient. Lesion-by-lesion sensitivity and predictive positive value for liver lesions were 78,4% and 96% for CT-MR alone, and 55% and 100% for PET-CT respectively. PET-CT was superior to CT-MR for the detection of local recurrence at the site of the initial colorectal surgery., Conclusions: In the selection of patients with CLM being considered for surgical therapy, PET-CT provided useful information only in 6.4% of cases. Possibly longer follow-up will increase this percentage. Our findings support the use of PET-CT mainly in patients with high risk of local recurrence.

Objective: The objective of the study is to review our experience in the surgical treatment of Klatskin tumours, after the systematic application of the current concepts of radicalism. Sixty-one patients resected using these criteria are presented., Patients and Method: We have studied 154 patients. Surgery was ruled out in 59 (41%) of them, and a liver transplant was performed on 9; of the 86 patients operated on, 25 were resectable. Resectability was 71% (of the 86 patients operated on) and was 39% of the total patients. The results during two periods are compared, 1989-1998 (pre-99) and 1999-2007 (post-99)., Results: On comparing the two periods, resectability increased from 26% to 53% (p = 0.01), the percentage of exploratory laparotomies decreasing (pre: 45% vs post: 22%; p = 0.04). Hepatectomy was performed in 53 cases (87%), being most frequent post-99 (pre: 66% vs post: 91%; p = 0.02). Resection of the caudate was performed in 48 cases (90%), being most frequent in the post-99 period (pre: 40% vs pos: 89%; p = 0.005). Post-operative morbidity was 77%, with 28% the patients being re-operated on, and the post-operative mortality was 16.4%, with no significant differences between the periods. Actuarial survival at 5 years increases in the post-99 period (pre: 26% vs post: 51%; p = 0.06)., Conclusions: Adequate staging, associated with an aggressive surgical strategy can achieve a greater than 50% resectability rate. The post-operative morbidity and mortality of this strategy is high, but the survival that it achieves justifies this.

Extrahepatic complications from hepatic cystic echinococcosis are rare and may be life threatening. Although the prevalence of echinococcosis in Spain had decreased, the number of cases of this disease and its severity has risen again due to immigration. We report the case of a patient with a giant hepatic echinococcus cyst diagnosed during investigation of an abdominal mass. The mass was associated with three other cysts: the first cyst was fistulized to the biliary tract, without clinical or laboratory alterations, the second cyst was fistulized to the right pleural cavity, leading to pleural echinococcosis and respiratory distress due to massive pleural effusion, and the third cyst was calcified. The challenge in this case layed in its diagnosis and treatment. The clinical presentation was unusual due to the extension of the disease, hampering complete cystic excision.

Introduction: Surgery that preserves the duodenopancreatic region has become well-established in chronic pancreatitis (CP) and some groups have begun to use these techniques to treat benign tumors and even those with uncertain potential malignancy. However, the technical complexity of this type of intervention may be greater than that of cephalic duodenopancreatectomy and complications may be even more frequent and consequently the indications for these procedures are debated. The aim of this study was to evaluate the experience accumulated at our center over the past few years in the use of pancreatic surgery preserving the duodenopancreatic region (PS). MATERIAL AND METHODS. Between 1996 and 2006, we carried out PS in 24 patients with disease localized in the head of the pancreas. PS was defined as any of the following techniques: resection of the head of the pancreas with duodenal preservation (RHPDP), uncinatectomy (UC) and cystic tumor enucleation (EN)., Results: RHPDP was performed in 20 patients (83%), UC in 1 (4%) and EN in 3 (13%). Surgery was performed for CP in 11 patients, serous cystoadenoma in 4, intraductal papillary mucinous tumor in 5 and miscellaneous injuries in the four remaining patients. Overall, the series showed 54% morbidity with no post-operative mortality. The median length of postoperative hospital stay was 11 days (7-43)., Conclusion: After analyzing the experience accumulated over the years, showing nil mortality and acceptable morbidity, we believe that the use of these 3 techniques for preserving the pancreatic parenchyma is useful when their suitability is rigorously indicated. Subsequent studies should look in depth at improving quality of life and physiological effects, depending on the technique used.

Objective: To describe the characteristics of patients requiring hospitalization for burns produced by lighter flame and to review current knowledge about their prevention., Patients and Methods: We performed a retrospective, descriptive study through a review of the medical records of children (0-14 years) admitted to our hospital for more than 24 hours with burns and/or smoke inhalation from 2000-2004. Of these, accidents related to the handling of lighters were selected. The variables studied were: age, sex, mechanism, extent of body surface area (BSA) burned, degree of burn, length of hospital stay, the need for skin grafts, mortality, and sequelae., Results: Twenty-two patients were evaluated. Seventy-six percent were boys. Age ranged from 1.5 years to 14 years. Ten patients (45 %) were aged less than 6 years old and 12 were aged 6 years old or older (55 %). The percentage of BSA burned varied: 65 % presented burns of less than 10 % BSA. Twenty-seven percent (6 patients) suffered major burns (> 20 %BSA). The mean length of hospital stay was 25 days (range:4-58 days). Seventy-seven percent of patients required surgery, mainly wound debridement and skin autografting and 33 % showed sequelae. The most severe was hypoxic-ischemic encephalopathy due to smoke and CO inhalation in 1 patient, who developed severe psychomotor sequelae and died 3 months later from infectious complications related to severe encephalopathy. Overall, 2 patients (9 %) died., Conclusions: Because of the frequency and severity of burns associated with lighter handling by children, pediatricians should increase their efforts to educate families. Effective legislation should be passed in Spain aimed at incorporating childproof safety devices in lighters.

Objective: To describe the characteristics of patients with tap-water scalds admitted to our hospital and review current knowledge on their prevention., Patients and Methods: We performed a retrospective study of admissions for tap-water scalds. The medical records codified as water-tap scalds (MBDS; CIE-9-CM, 940.0-949.5, E.924.0, E.924.2) of patients admitted to our hospital for more than 24 hours from January 1 to December 31, 2003 were reviewed. The following variables were studied: age, sex, mechanism, extent and degree of burn, localization, length of hospital stay, treatment, requirement for skin grafting, mortality, and sequelae., Results: Twenty-nine patients were treated in our hospital. Sixty-two percent were boys. Age ranged from 3 days to 9 years. Six patients (20.6 %) were aged less than 1 year, 19 (63 %) were aged between 1 and 3 years and four (13.8 %) were aged from 3 to 9 years. Body surface area was 10 % or less in 25 patients and more than 20 % in four. Five patients required skin autografting. In two patients, the scald was produced in the neonatal area of our hospital. The mean length of hospital stay was 12 days, ranging from 1 to 38 days. None of the patients died. One patient suffered severe sequelae., Conclusions: Because of the frequency and severity of the burns reported in this study, pediatricians should increase their efforts in educating families about this type of burn. In addition, effective legislation should be implemented in Spain.

Non-intentional burns are still a frequent cause of morbidity in children despite the implementation of preventive campaigns. Children with severe burns (more than 10 % of the body surface) may require iron supplementation, especially in the post-stress or recovery phase. We report the case of a 15-month-old boy, who suffered polytrauma and burns to 20 % of the body surface. A dermal graft was performed eight days after his admission to the pediatric burns unit, which required transfusion of packed red blood cells. On day 15 hemoglobin value was 9.3 g/dL, the reticulocyte hemoglobin content was reduced to 26.2 pg (normal value: 27.2-30.3 pg) and the reticulocyte mean corpuscular value was increased to 124 fl (normal value: 101-108 fl). These findings were interpreted as a functional iron insufficiency, despite the increased ferritin value (219 ng/mL). We highlight the satisfactory outcome of the different hematologic values, with the development of reticulocyte crisis and normalization of hematological parameters after administration of 0.5 mg/kg/day of ferrous sulfate, plus iron corresponding to the follow-up milk formula and a regular diet for the child's age.

Background: In domino liver transplantation (LT), the explanted liver of a patient with familial amyloidotic polyneuropathy (FAP) is donated to another patient., Patients and Method: Between February 1999 and March 2001 we performed 131 LT with 121 cadaveric donors in our unit. Ten domino LTs were performed., Results: Patients with FAP were younger (37 years) than recipients of the second LT (64 years). The evolution of patients undergoing transplantation for FAP was excellent and all are currently alive and without complications. Among recipients of the second LT, one patient died in the postoperative period. A further two patients died from tumoral recurrence and hepatitis C virus recurrence 18 months and 9 months after transplantation, respectively. The remaining patients have shown no symptoms of FAP during the follow-up., Conclusion: The results of this study show that domino LT is technically feasible. The technique increases the number of grafts without apparent risk either to the recipient with FAP or to the recipient of the latter's explanted liver.

Background: Surgical treatment for hepatocellular carcinoma remains controversial due to a lack of prospective randomized studies., Material and Method: Between January 1990 and December 2000, 121 liver transplantations (group 1) and 52 hepatectomies (group 2) were performed for hepatocellular carcinoma. Each surgical treatment was carried out depending on patients' and tumor's characteristics., Results: Patients from group 1 had a more advanced tumoral grade, with higher involvement of two lobes (19 vs 4%; p = 0.015) and higher number of nodules (1.9 DE [2] vs 1.2 [0.6]; p = 0.001); yet the mean tumor size was lower (3 cm [1.5] vs 4.2 [3.2]; p = 0.006). Operative mortality (4% vs 2%; p = 0.66) and 5- and 10-years survival (68% and 42% vs 63% and 45%; p = 0.23) were similar between both groups. Nevertheless, 5- and 10-years recurrence rates (10.6% and 10.6% vs 50% and 65.5%; p < 0.0001) were more favourable in group 1. Prognostic factors of recurrence included microscopic vascular invasion (RR = 12.12; CI, 2.02-75.52) and alpha-fetoprotein levels higher than 300 ng/mL (RR = 7.12; 95% CI, 1.08-47.02) in group 1, and the pT3-4 stage (RR = 3.86; 95% CI, 1.06-14.03) in group 2. Mean time on waiting lists for liver transplantation was 3.06 (2.66) months and it has increased significantly in last years, especially among blood group 0 patients. However, this fact has not been associated with a worsening of survival rates (p = 0.98)., Conclusions: After a good patient selection, either liver transplantation or hepatectomy achieve excellent long term survival rates in patients with hepatocellular carcinoma, though the former allows a better control of the tumoral disease. The increase of mean time on waiting lists for liver transplantation during the last years has not led to a worsening of survival results.

Background: The aim of this prospective study was to analyze the risk of liver resection in unselected patients., Patients and Method: From 1990 to 2000, 437 consecutive hepatectomies were performed in our center. Most frequent indications were liver metastases (n = 288), hepatocellular carcinoma (n = 62), Klatskin tumor (n = 17), gallblader carcinoma (n = 139) and other malignant tumors (n = 6). The indication was a benign tumor in 51 patients. In 357 cases the liver parenchyma was normal, 51 patients had an underlying cirrhosis and 17 patients had an obstructive jaundice., Results: Overall mortality was 3.6% (15 cases). Mortality in benign tumors was lacking. The prevalence of postoperative complications was 43.9%, which was mainly influenced by malignancy (46.9% vs 21.6%, p = 0.001) and type of tumor (Klastkin tumor, p # 0.001). Major liver resection (p < 0.001), blood transfusion (p < 0.001), age over 60 years (p = 0.001) and the type of hepatectomy (p < 0.001) also increased significantly the morbidity. The prevalence of biliary fistula was 11.2%, which was mainly related to the type of hepatectomy (major hepatectomy; p = 0.002) and a biliary-enteric anastomosis (p < 0.001). The prevalence of hepatic insufficiency was 3.6%, and chief risk factors for its development were underlying liver disease and major liver resection (p = 0.017)., Conclusions: Mortality after hepatectomy in experienced centers is low. Morbidity is mainly related to the amount of parenchyma resected, type of hepatectomy, underlying liver disease and associated procedures. Liver resection should be performed preferentially in centers with high volume by specialized surgeons.

Background: Domino or sequential liver transplantation (DTXL) is a kind of living donor transplant, which was proposed in 1993 and performed for the first time in 1995; later on, more than 45 have been reported. The liver from a patient with familial amyloidotic polyneuropathy(FAP) is used to another patient aged more than 60 with hepatic disease generally cancer, because FAP livers are anatomically and functionally normal except for the synthesis of the systemic TTR variant which only could generate FAP in the recipient after more than 8 years., Patients and Method: The three first cases of DTXL performed in Spain are presented. The donors were FAPTTRMet30 patients from the Major can focus. The first recipient showed severe hyperinsulinism due to metastatic liver from malignant insulinoma; the others had hepatocellular carcinoma on a cirrhotic liver., Results: During the post operatory period liver function of recipients was perfect,and hyperinsulinism disappeared in the first; this patient died after 10 days by sepsis whereas the others showed normal liver function, no recurrent cancer nor onset of FAP. The donors outcome was normal with perfect liver function., Conclusions: Based on our results, in agreement with previous reports, we conclude that DTXH is valid procedure for a selected patient group. In addition they increase the pool of liver donors and therefore diminish the overloaded waiting lists.

Introduction: The increase in indications for liver transplantation has meant that waiting lists are growing ever longer. For this reason, broadening the donor pool is a priority for most groups., Objective: The objective of this study was to analyze the predictive value of post-reperfusion biopsy in the evolution of graft function after liver transplantation., Patients: One hundred and forty-eight liver biopsies, obtained after graft reperfusion, were analyzed. Eight pathological variables and thirty-seven clinical variables of the donors were recorded. Risk factors for presenting primary graft non-function or dysfunction were studied with logistic regression models. Factors associated to the long-term graft failure were studied using Cox analysis and actuarial survival curves., Results: Microvesicular steatosis greater than 50% was the only risk factor associated to graft dysfunction in the multivariate logistic regression model. Microvesicular steatosis greater than 30%, severe hepatocyte necrosis and presence of abundant neutrophilic leukocytes were risk factors associated to graft failure in the univariate study. Only steatosis remained as an independent risk factor in the multivariate study. These grafts also presented poorer long-term survival. Abundant polymorphonuclear infiltrate was associated to a higher frequency of biliary complications., Conclusions: Microvesicular steatosis implies a better evolution than macrovesicular steatosis. Neutrophilic infiltrate and hepatocellular necrosis lead to poorer initial graft function and reduced long-term survival.

Background: We present the experience of the liver transplantation program at the Hospital of Bellvitge with 500 transplantations performed during 15 years, to describe changes in liver transplantation observed throughout the time and to analyze the long term results., Patients and Method: Five groups each one including 100 consecutive transplantations are studied., Results: The main indications were hepatocellular carcinoma (23%), alcoholic cirrhosis (22.8%), and post-hepatitis C cirrhosis (18.8%). Sixty-five retransplantations were performed in 59 patients (13%), being the more frequent indications arterial thrombosis (13 patients) and primary nonfunction of graft (10 patients). In 10 patients a hepatorenal transplantation was performed. In group I, the most frequent donor cause of death was cranial traumatism (80%), while in group V it was the vascular pathology (52%). There were other significative differences between these groups of patients (I vs V): patients with stage 2 or 3 from UNOS status (45 vs 19%), blood use (29.6 [26] vs 4.6 [5.3] PRBC), ICU stay (13 [13] vs 7.4 [11] days), hospital stay (40 [52] vs 23.7 [17] days), rejection rate (46 vs 20%) and primary graft nonfunction (9 vs 3%). However, the infection rates (48 vs 54.5%) and biliary tract complications (26 vs 20%) have not shown statistically significant differences. Actuarial one and 5-year survival are 83 and 70% respectively., Conclusions: An important and progressive improvement of liver transplantation results has been observed. However, de novo tumours, hepatitis C virus recurrence and chronic rejection can limit long term results.

Introduction: Liver retransplantation is the only alternative to irreversible graft failure. However, it remains a controversial treatment. The aim of this study was to analyze the clinical course and the results of liver retransplantation in our center., Patients and Methods: The actuarial survival in a series of 54 retransplantations in 49 patients between February 1984 and December 1997 was analyzed. The retransplantations were grouped according to period: group A (n = 16) 1984-1992, group B (n = 22) 1993-1995 and group C (n = 16) 1996-1997., Results: The actuarial survival per group according to year was: 31.25%, 54.55% and 62.50% for groups A, B, and C, respectively, which shows a clear improvement with time, although differences were not statistically significant., Conclusions: The results of liver transplantation in our series show a lower actuarial survival rate than those of primary transplantation but these results have improved in recent years.

Germ-line mutations in the RET proto-oncogene are associated with multiple endocrine neoplasia type 2A (MEN 2A) and with familial medullary thyroid carcinoma (FMTC). Detection of these mutations allows the identification of the affected kindred members, who will develop medullary thyroid carcinoma (MTC) in 100% of cases. We studied 24 patients of two kindreds (MEN 2A and FMTC). Basal calcitonin levels and pentagastrin-stimulated calcitonin were measured in all patients. The RET mutations were detected by DNA analysis. The RET mutations were identified in 14 patients. Two of them had been operated in the past, 2 refused operation and 4 were living abroad. In the 6 remaining, only one showed a thyroid mass, basal calcitonin was normal in all patients except one, and pentagastrin-stimulated calcitonin was negative in 2 patients. Total thyroidectomy was performed in all cases. Histology showed C-cell hyperplasia in all patients and MTC in 5 of them. In MEN 2A and FMTC DNA analysis allows the identification of RET mutation carriers, in which presymptomatic thyroidectomy allows and improvement in survival.

Introduction: Orthotopic liver transplantation (OLT) is the only curative treatment for hepatic cirrhosis and is the most effective in the control of portal hypertension. The aim of this study was to analyze whether greater morbi-mortality is observed in patients undergoing liver transplantation with previous surgery for portal hypertension with respect to patients not having undergone this surgery., Materials and Methods: Different variables were analyzed in 2 groups of transplanted patients: one of 18 patients who had previously undergone surgery for portal hypertension and another group of 54 patients without this previous surgery., Results: The following factors were studied: mean operative time and length of anahepatic phase, intraoperative consumption of concentrates of erythrocytes, fresh frozen plasma, units of platelets and cryoprecipitates, days of mechanical ventilation, stay in the ICU and total postoperative stay. No significant differences were observed (p < 0.05) in any of these factors or in survival., Discussion: On analysis of the difficulty of surgical technique, postoperative evolution and survival and based on the variables described it may be concluded that previous surgery for portal hypertension does not only not contraindicate posterior liver transplantation, but rather may be useful in patients with an adequate hepative reserve presenting variceal hemorrhage since posterior transplantation does not present a worsened prognosis.

A retrospective analysis of our experience in the treatment of hiliary cholangiocarcinoma or Klatskin tumor was performed with the aim of evaluating the morbi-mortality and prognosis of its treatment to thereby determine the usefulness of the different therapeutic options. From 1989 to 1997, 51 patients diagnosed with hiliary cholangiocarcinoma were treated in our hospital. Surgery was indicated in 16 with curative aims (group I) while palliative treatment with percutaneous biliary drainage was indicated in 35 (group II). Biliary resection was carried out in 8 patients being associated with hepatic resection in 4 (group IA) and in 8 patients undergoing liver transplantation (group IB). Clinico-epidemiologic data and hospital stay were similar in all the groups. The frequency of complications was similar in groups I and II although the frequency of cholangitis (49%) in group II was noticeable. The percentage of readmissions was also greater in group II (12 vs 46%, respectively; p = 0.03) with prosthesis obstruction being the most frequent cause. Accumulated survival at 1, 2, and 3 years in group I was 84, 64 and 48% with a median survival of 33 months, while in group II the median survival was of 6 months with no patient surviving more than 2 years (p = 0.0001). When groups IA and IB were compared, greater frequency of complications in groups IA (100 vs 37%; p = 0.002), similar frequency of readmissions (87 vs 75%; p = NS), median survival greater in group IB (12.5 months vs 48 months) and significantly higher actuarial survival in group IB (48% in 2 years vs 83% to 2 years; p = 0.02) was observed. In conclusion, surgery is the treatment of choice in hiliary cholangiocarcinoma whenever possible, given the greater survival without a significant increase in morbimortality. Likewise, we consider that liver transplantation is a useful option in the treatment of patients with cholangiocarcinoma type IV of Bismuth.

Purpose: Study of the effectiveness and morbidity of palliative treatment of malignant obstructive jaundice with metallic biliary endoprosthesis compared to surgical palliation., Design: Retrospective review., Patients: 35 patients with non-resectable neoplasms causing jaundice were treated with percutaneous stent (pancreatic carcinoma, n = 11; cholangiocarcinoma, n = 11; gallbladder carcinoma, n = 4; extrahepatic metastases of various malignancies, n = 8)., Control Group: 23 patients with malignant jaundice treated with palliative surgery., Results: Most frequent complications were cholangitis and stent obstruction. The mean hospital stay after the stent placement was 6.8 days, longer in patients with complications (p = 0.035). Recurrence of jaundice was seen in 22.9% of the patients and the rate of readmission was 42.9%. The mean survival was 163.33 days (range 19-522). Reduction in serum bilirubin after BE was significant (215 vs. 82 mmol/l, p < 0.001)., Conclusions: Comparing to our previous experience with surgical palliative treatment, there was no significant difference neither in morbidity-mortality, nor recurrence or readmission. Patients with pancreatic cancer and cholangiocarcinoma benefit from a shorter hospital stay.

Background: The aim of this study was to know the clinical, etiopathogenic and microbiologic characteristics of pyogenic liver abscesses (PLA)., Methods: A retrospective analysis of the cases of PLA diagnosed from 1978 to 1992 in the Internal Medicine, Infectious Disease, and Gastrointestinal Surgery Departments of the Hospital de Bellvitge in Barcelona, Spain was performed., Results: A total of 63 cases of PLA (43 males and 20 females, mean age 54 +/- 19 years) were analyzed. The most frequent clinical and analytical data included fever (92%), leucocytosis (84%) and abnormal levels of alkaline phosphatase (81%). The PLA were single in 65% and multiple in 35%. Echography was diagnostic in 91% of the cases. A positive culture of the abscess was obtained in 40 cases, being monomicrobial in 27 cases (67.5%). Eleven of the 13 polymicrobial cultures were from single PLA. The most frequent bacteria found were the enterobacteria (44%) followed by the microaerophilic streptococci (28%) and the anaerobes (17%). The PLA was of biliary origin in 31.8%, contiguous in 12.7% and unknown in 38%. Percutaneous drainage was performed in 34 patients (54%). Mortality attributable to the abscess was 3%., Conclusions: The clinical presentation of pyogenic liver abscess has not varied over time. There has, however, been a change with respect to its epidemiology and therapeutic management. At present, the possibility of rapid diagnosis and image guided percutaneous drainage offers a better prognosis for this disease.

Transcatheter arterial chemoembolization with lipiodol and adriamycin was performed in 46 patients with hepatocellular carcinoma (HCC). In 27, this procedure was followed by selective arterial embolization using gelatin sponge particles. Surgical resection was carried out in 5 cases and 13 patients were transplanted (OLT). The aim of the study was to analyze the survival and degree of tumor necrosis. In the nonsurgical group the overall survival was 67% after 24 months in the OKUDA I stage, 31% after 20 months in the OKUDA II stage and 25% after 6 months in the OKUDA III stage. For the patients who underwent surgery, survival was 38% after 20 months in 5 patients who were resected and 72% after 24 months in 13 patients who were transplanted. Transcatheter arterial chemoembolization improves survival in patients who are not operated on, and can be used as a complementary treatment for patients who undergo surgery. A full tumor necrosis was observed in well encapsulated tumors.

Soft-Tissue Retroperitoneal Sarcomas (STRP) are rare tumors. Late diagnostic and extreme technical difficulties to obtain total resection with margins free of tumor, are associated to a high relapse rate. Eighteen patients with STRP were retrospectively reviewed, evaluating: Clinical onset, histology, treatment-type performed, mortality and survival rate. Total resection was performed in eleven patients (61%), with inclusion of neighbor organs or structures in five of them (45%). In one case partial exeresis associated with left hemicolectomy was done, and only biopsy was performed in six cases. After total exeresis, seven patients showed local relapse (64%) and four of these patients (57%) underwent surgery again. Global surgery mortality was one patient. Survival at five years in resected patients was 57%. In patients with biopsy only, mean survival was 16.6 months, patients with total or partial resection, survival was 79.1 months. An aggressive surgical approach, with tumor total exeresis and a block resection of neighbor affected organs or structures, is the best alternative in the treatment of STRP.

Venous pressure between the external jugular vein (EJV), and the superior caval vein (SCV) was compared in 15 patients, free of cardiorespiratory disease and undergoing abdominal surgery. In each patient data was taken in four different positions: A) anaesthetized patient in supine position and mechanical ventilation; B) anaesthetized patient in Trendelenburg position and mechanical ventilation; C) anaesthetized patient in anti-Trendelenburg and mechanical ventilation, and D) awake patient in supine position and spontaneous ventilation. A short, thick catheter with unique end lumen was used for EJV, and a long catheter from the basilic vein, to the SCV. Significant differences between mean and standard deviations of EJV and SCV were not founded. Linea correlation for the 4 positions was performed (r = 0.97, 0.91, 0.88 and 0.60 respectively) being significant for A-B y C positions. 4.5 cm H2O was the maximal difference obtained between SCV and EJV for A, B and C positions, and 10 cm H2O the maximal difference for the D position. The mean changes between the positions A-B, A-C and A-D was compared and significant differences (p less than or equal to 0.001) for A-C were seen, whereas A-B did not attain significant differences. We conclude that the EJV cannulation, with a short and thick catheter, allow the central venous pressure control during anaesthesia, in supine position, Trendelenburg and anti-Trendelenburg, but not during the recovery.