[Orthotopic liver transplantation in children younger than one year]. / Trasplante hepático en menores de un año.

BACKGROUND: Orthotopic liver transplantation (OLT) in children younger than one year is associated to higher waiting list mortality and alternative graft sources are required. We present our experience with this particular group of age. METHODS: Infants younger than one year who received an OLT between 1986 and 2005 were reviewed focused on graft and children survival depending on period and type of graft. Periods were 1:1986-1995; 2:1996-2000 and 3:2001-2005. We also evaluate cold ischemia time (CIT), graft lost causes and differences between CIT and anhepatic time (AT) depending on graft type. RESULTS: Eighty-three children received 103 OLT. Liver transplant indications were 59 (72%) biliary atresia, 8 (10%) metabolic causes, 6 (8%) liver failure, 3 (4%) cirrhosis and 7 (6%) miscelaneous. Patient and graft survival after 5 years was increased depending on period: 45% and 65% on period 1, 70% and 80% on period 2, 94% y 97% on period 3 (p < 0.0198). Thirty-seven grafts were reduced lobes (42%); 8 (21%), 17 (45%) and 12 (35%) during periods 1, 2 and 3 respectively and their 5 years survival rate was 68%. Twenty-four were whole grafts (31%); 11 (45%), 10 (45%) and 3 (14%) during periods 1, 2 and 3 and their 5 years survival rate was 63%. Fourteen grafts were living-related donor (16%); 1 (7%), 2 (14%) and 11 (79%) during periods 1, 2 and 3 and their 5 years survival rate was 93%. Eight (11%) were split; 0, 1 (12%) and 7 (90%) during periods 1, 2 and 3 and their 5 years survival rate was 100%. Average CIT depending on graft was: living donor 5,5 hours (IQR: 4-7), split 6,1 hours (IQR: 5-8), whole 9.2 hours (IQR: 6-11) and reduced 8.5 hours (IQR: 6-11) (p < 0.05). Average AT depending on graft was: living donor 1 hour (IQR: 0.5-1.5), split 1 hour (IQR: 0.5-1.4), whole 1,1 hours (IQR: 0.5-1.5) (p > 0.1). Twenty-four grafts were lost (28%): 10 (41%) were surgical related causes and 6/10 (60%) of them were whole grafts. CONCLUSIONS: Survival rates in children younger than one year are similar to another groups of age. There was a significant increase on graft survival according to transplantation group experience. A higher rate of graft lost is associated to whole grafts. Most frequent reasons of graft lose were related to sepsis and immunosuppresion. A significant shortening of CIT is observed in related living donor and split grafts.

Comparative study between living and cadaveric donors in pediatric liver transplantation.

UNLABELLED: We examined whether the results in living-related hepatic transplantation (LRLT) are better than those from a cadaveric donor (CDLT). MATERIAL AND METHODS: The last 27 consecutive LRLT, performed from 1998 to 2005, were compared with 27 CDLT matched for age, weight, date, and diagnosis. Grafts in LRLT group were left lateral segment (n = 22), left lobe (n = 3), and right lobe (n = 2). In the CDLT group, the grafts were split in situ (n = 10), hepatic reduction (n = 9) and whole liver (n = 8). We analyzed the actuarial survivals (grafts and children), retransplantation, primary nonfunction, initial graft malfunction (liver enzymes >2000 U/L), surgical complications, rejection, and resource consumption. RESULTS: Patient survivals at 6 months, 1 year, and 5 years were 100%, 96%, and 96% in LRLT and 100%, 100%, and 100% in CDLT (P = NS). Graft survivals were 93%, 89%, and 89% versus 96%, 96%, and 96%, respectively (P = NS). Complications were biliary complications (LRLT, 25% vs CDLT, 3%; P = .021); portal vein thrombosis (LRLT, 7% vs CDLT, 3%; NS), and hepatic artery thrombosis (LRLT, 0% vs CDLT, 3%; NS). The overall incidence of acute rejection was slightly higher (NS) in LRLT (LRLT, 18% vs CDLT, 11%; NS). Liver enzyme levels were higher in the CDLT group, but initial malfunction rate was not statistically different. Regarding resource consumption: blood product needs were higher in LRLT (P < .05) and hospital stay and ICU stay were longer, although not significantly, among LRLT. CONCLUSIONS: The results in LRLT among children are similar to those obtained in CDLT. We found a trend towards less initial graft malfunction in LRLT. Blood product needs were higher in LRLT. Hospital and ICU stay were longer, but not significantly different in LRLT. The benefits of LRLT are saving a scarce resource: a cadaveric donor liver graft.

Intestinal transplantation in children: differences between isolated intestinal and composite grafts.

The results of the isolated intestinal grafts were compared with those of composite grafts (intestinal graft + liver) in a series of 18 transplantations performed in 17 children; 5 isolated intestinal grafts, 12 hepatointestinal grafts, and 1 multivisceral graft. Causes of intestinal failure were short bowel syndrome (n = 13), motility disorders (n = 2) and congenital epithelial disorders (n = 2). Transplantation was indicated due to end-stage liver disease (n = 14), loss of venous access (n = 2), untreatable diarrhea (n = 1) and high morbidity associated with a poor quality of life (n = 1). Six children, all with a composite graft, died after transplantation due to lymphoma (n = 2), sepsis (n = 1); intraabdominal bleeding (n = 1); pneumonia (n = 1); and overwhelming adenoviral infection (n = 1). Digestive autonomy was achieved in 16 of 18 grafts, the 11 surviving children are free of parenteral nutrition with a reasonably good quality of life. In conclusion, intestinal transplantation is a viable therapeutic alternative for children with permanent intestinal failure. The results of transplantation with an isolated intestine are clearly better that those with a composite graft.

Pediatric living donor liver transplantation.

AIM: The aim of this study was to analyze the results of living donor in a pediatric liver transplantation program. PATIENTS: Twenty-six living donor liver transplantations were performed in children from 0.5 to 14.8 years of age. The main indication was biliary atresia (72%) followed by tumors (2 hepatoblastomas and 1 hepatocarcinoma). Left lateral segments were used in 23 (1 transformed into a monosegment), 1 left lobe was used in 1, and right lobes were used in 2. Arterial reconstruction employed saphenous venous grafts in the first 3 cases and end-to-end anastomoses with a microsurgical technique in the following 22 cases. RESULTS: There has been no major morbidity in the donors, with a median hospitalization of 6 days. Four grafts have been lost; 2 in the first 3 cases. In only 1 case, the graft loss was related to the procedure saphenous venous graft thrombosis). Early biliary complications were frequent (23%). Six month, 1 year, and 5 year graft and patient survival rates were 91%, 85%, and 85% and 100%, 96%, and 96%, respectively. CONCLUSIONS: Living donor liver transplantation is an excellent option for transplantation in children.

Liver transplantation in small babies.

Pediatric liver transplantation is an effective treatment for end-stage liver disease with 1- and 5-year survivals approaching 90% and 70%, respectively. Survival is influenced by the recipient's age, weight, primary disease, vascular malformations, and nutritional status. Younger patients weighing less than 13 kg are considered to be a high-risk group. The aim of this article is to evaluate the impact of this group of patients on the overall results of our pediatric liver transplant program. From January 1986 through January 1992 we performed 76 liver transplants in 59 pediatric patients. Sixteen received a second graft and a third was required in one. Fourteen patients weighed less than 13 kg (mean, 11 kg; range, 6 to 13 kg). Their mean age was 12 months, with a range of 8 to 36 months. Indications for transplantation were: biliary atresia (9), Byler's disease (1), tyrosinemia (3), and alpha 1-antitrypsin deficiency (1). The incidence of rejection in this group (52%) was not significantly different from that in other patients (61%). Ten episodes of acute rejection required only steroids: in one monoclonal antibodies were added. Five patients had a new graft implanted, four for hepatic artery thrombosis and one for primary liver nonfunction. Nine patients are alive (64%) with the follow-up time ranging from 2 to 56 months (mean, 31). Five patients died of multiorgan failure (3), portal vein thrombosis (1), and primary liver nonfunction (1). Four-year graft and patient survival rates were 47% and 64%, respectively. Small babies are a high-risk group in a pediatric liver transplant program.(ABSTRACT TRUNCATED AT 250 WORDS)

Liver transplantation in patients with homozygotic familial hypercholesterolemia previously treated by end-to-side portocaval shunt and ileal bypass.

Familial hypercholesterolemia is the result of mutations in the gene that encodes the synthesis of the cellular receptor for low density lipoprotein (LDL). In the homozygous form of the disease (HFHC), cellular LDL receptors either do not form, or, when present, cannot bond LDL and mediate its cellular uptake LDL, and the cholesterol that it transports accumulate in plasma, producing severe premature atherosclerosis and death from coronary artery disease usually before the age of 20. Currently, the only effective treatment is liver transplantation, which, alone or in association with medications, normalizes plasma cholesterol levels. The authors report the cases of 2 siblings with HFHC who underwent portocaval shunt at the ages of 2.5 and 1.5 years, respectively. Portocaval shunt produced an immediate, but insufficient decrease in cholesterol (by 40% and 35%, respectively), leaving them with cholesterol concentrations of about 500 mg/dL. One year later they each underwent ileal bypass without obtaining any significant response. Liver transplantation at the ages of 18 and 16 years, respectively, reduced plasma cholesterol concentrations to 129 and 225 mg/dL, respectively. The earlier operations seriously increased the technical difficulty of liver transplantation and did not produce a favorable effect on the natural course of the disease, so portocaval shunt and ileal bypass are not indicated in HFHC, not even for the purpose of delaying liver transplantation.

Long-term survival expectancy after liver transplantation in children.

PURPOSE: The aim of this study was to assess the long-term survival rate in children who have undergone orthotopic liver transplantation (OLT) in the last 13 years. METHODS: The records of 198 consecutive patients under 18 years of age who underwent 249 OLTs between 1986 and 1998 were reviewed. Actuarial patient survival rates were assessed at 1, 3, 5, and 10 years in the whole series, in the last 5 years, and in patients surviving more than 1 year. Age, weight, and indications were analyzed, as well as type and incidence of posttransplant complications. The median follow-up period was 41 months (0 to 154 months). RESULTS: Biliary atresia was the most common indication (41.9%) followed by alpha-1 antitrypsin deficiency (8.1%), Alagille syndrome (7.6%), and fulminant hepatic failure (6.6%). One hundred forty-six patients (58.6%) were below 5 years, and 46 patients were (18.5%) younger than 1 year at operation. Sixty-eight patients (27.3%) weighed less than 10 kg. One hundred seventy whole organs and 70 reduced, 5 living-related donor, and 4 split-liver allografts were used. Hepatic artery thrombosis (n = 18), primary nonfunction (n = 15), and chronic rejection (n = 14) were the most common causes for allograft failure. Fourteen patients (7%) had posttransplant lymphoproliferative disorders (PTLD) at a median time of 28 months (4 to 124 months) postoperation (3 died). The 1-, 3-, 5-, and 10-year actuarial patient survival rates are 80%, 76%, 74%, and 74%, respectively; over the last 5 years it is 88% at 1 year and 82% at 3 and 5 years. For patients surviving more than 1 year, 3-, 5-, and 10-year actuarial survival rates are 95%, 93%, and 93%, respectively. CONCLUSIONS: (1) Overall results of OLT improve with increasing experience. (2) Children who survive more than 1 year after OLT have an excellent prognosis, although long-term complications of immunosuppression can be expected.

Late biliary complications in pediatric liver transplantation.

PURPOSE: The aim of this study was to review the biliary complications occurring in late follow-up after liver transplantation in children. METHODS: The medical records of 135 children who received orthotopic liver transplantations (OLT) and had graft survival of more than 1 year were reviewed. Technical variants using a reduced-size graft were applied in 32 (23.7%). For biliary reconstruction, 15 patients had choledochocholedochostomy and 120 a Roux-en-Y loop. Biliary reoperation in the early post-OLT period was needed in 24 patients (17.7%). Routine checking of liver function and duplex Doppler ultrasonography (DDS) were performed during the follow-up period, which averaged 58 months. Late biliary complication was defined as that occurring after the first hospital discharge. RESULTS: Late biliary complications occurred in 18 children (13.3%); 16 showed symptoms or analytical disturbances in liver function tests. The Diagnoses included uncomplicated cholangitis (n = 6), anastomotic biliary stricture (n = 7), ischaemic damage of the biliary tree (n = 3) including one late (28 months) hepatic artery thrombosis leading to an intrahepatic biloma. and bile leak after T-tube removal (n = 2). The six children with uncomplicated cholangitis had no repeat episodes in follow-up despite persistent aerobilia. Six patients affected by anastomotic strictures were treated successfully with percutaneous dilatation and, if present, stone removal. Persisting dysfunction and cholangitis occurred in one case affected by ischaemic biliary disease. Biliary leaks after T tube removal settled spontaneously. Risk factors for late biliary complications were determined. There was no relation to the cold ischaemia time, type of graft or biliary reconstruction, or previous early post-OLT biliary reoperation. Aerobilia (affecting 21.5% of OLT patients) was related to cholangitis (P = .001). CONCLUSIONS: Anastomotic strictures, reflux of intestinal contents via the Roux-en-Y loop, and residual ischaemic damage led to late biliary complications in 12% of paediatric OLT patients. Evidence of biliary dilatation on DDS may be delayed in anastomotic strictures; in these cases the results of percutaneous treatment were excellent. Children with aerobilia have and increased risk of cholangitis.

Donor vascular grafts for arterial reconstruction in pediatric liver transplantation.

The authors compared the results of 48 orthotopic liver transplantations (OLT) in which revascularization was achieved with a conduit interposed between the receptor aorta and the graft (vascular graft [VG] group) with those obtained for 56 OLT performed during the same period (1991 to 1994) in which end-to-end anastomosis (EEA) of the hepatic arteries or celiac trunk was used (EEA group). In the VG group, the interposed conduits were the cadaveric iliac artery (37) the living-donor saphenous vein (3), or nonthrombosed conduits from previous transplants (8) (7 iliac arteries, 1 saphenous vein). There were significant differences between the two groups with respect to recipient age, recipient weight, the retransplant:first transplant ratio, the number of emergency transplantations, the use of reduced-size grafts, and intraoperative transfusion requirements. Twenty-nine grafts in the VG group (60.4%) and 43 in the EEA group (76.7%) currently are functioning. The actuarial 3-year graft survival rates are 60% and 71.5% for the VG and EEA groups (P < .05), respectively. The rate of arterial thrombosis did not differ between the two groups. The authors conclude that, although EEA of the hepatic artery is still the preferred revascularization technique for OLT, revascularization of the liver graft by conduit interposition is safe when EEA is not possible. Reutilization of the interposed conduit during retransplantation proved to be safe in the absence of hepatic artery thrombosis.

Our first 100 consecutive pediatric liver transplants.

Orthotopic liver transplantation (OLT) is nowadays accepted as the best therapy for end-stage liver disease. The difficulties involved are even greater in children than in adults, and it is debatable whether exclusively pediatric programs are warranted. The aim of this paper is to analyze our experience at the Children's Hospital "La Paz", with the first consecutive 100 OLT in children, 61% of whom weighed less than 20 kg. Since 1986, 220 pediatric patients were evaluated as candidates, 100 OLT were performed in 78 patients and 13 died on the waiting list, currently maintained below 15 cases. Indications were: cholestasis (45), metabolic disease (18), fulminant hepatic failure (3), primary liver tumors (2) and cirrhosis (10). Mean age was 66 months (range = 7 to 216) with a mean weight of 21 kg (range = 6 to 60), twenty patients weighed less than 13 kilograms. OLT was performed by standard technique. Reduced or segmental grafts were necessary in 8 instances. Twenty-two patients were retransplanted and 2 received three grafts. Indications for retransplantation were: hepatic artery thrombosis (8), primary nonfunction (4), chronic rejection (7), portal thrombosis (2) and Budd-Chiari recurrence (1). Acute rejection was observed in 52 patients, and eight cases developed a chronic rejection. These episodes were treated with "bolus" of steroids, monoclonal antibodies (OKT-3) and FK-506. Surgical complications included: hepatic artery thrombosis 12%, portal vein thrombosis 3% and biliary fistula or stenosis 13%. The incidence of primary non-function was 7%. Actuarial survival rate at 5 years was 75%.(ABSTRACT TRUNCATED AT 250 WORDS)

Innovative techniques in pediatric liver transplantation: reduced-, split- and living-donor related liver transplantation.

In spite of the increased number of organ donations, the amount of livers available for pediatric patients does not meet requirements. In an attempt to expand the pool of grafts, several techniques of size reduction (RST) have been developed: reduced-liver transplantation (RLT), liver segment transplantation (LST), "split" liver transplantation (SLT) and living-donor liver transplantation (LDLT). The aim of this work is to study the contribution of these techniques to reduction of mortality in the waiting list and the increased risks that we inflict to our patients by using these procedures. We report our experience on RST in the last nine years (23 RLT, 5 LST, 2 SLT and 3 LDLT). More than a half of them were performed in an emergency. Overall survival in children with RST (67%) is similar to that of patients receiving a "whole graft" (74%). We did not find significant differences in the incidence of graft-related complications, only reintervention for abdominal bleeding was needed more often in the RST group (30%) than in whole graft group (24%). Our data confirm the safety of the RST procedures in the pediatric liver transplantation programs and they decrease the waiting-list mortality (2.5%) among the pediatric patients.

[Reduced-size liver transplants from cadaver and living donors: an alternative to waiting lists]. / Trasplante hepático reducido de cadáver y donantevivo: una alternativa a las listas de espera.

The ever expanding of indications of liver transplantation in children make pediatric donor pool unable to fulfill the needs. The reduced and partial transplants from cadaveric and living related donors reveal an effort to increase the number of pediatric grafts aid to reduce the mortality in the waiting list. Among 126 pediatric transplants performed in a 8-year period, 18 (15%) were reduced (n = 3) and partial livers (with preservation of the recipient vena cava) (n = 15). In 1993, 41% (n = 12) of the 29 liver transplantations performed were partial segments. In two of them the graft was harvested from a living-related donor. Eight transplants were made on an emergency basis and ten were elective. Eight patients were retransplanted. Considering the transplants performed in the Wisconsin era, after completing the "learning curve" the actuarial survival at five years reaches 65% approaching 70% for patients younger than 1 year and/or weighing less than 13 kg. The arterial complications in this group are limited to a single case of thrombosis. Despite our limited experience we conclude that reduced and partial transplants are useful to reduce the mortality in the waiting list with a survival similar to that of the whole grafts and with less vascular complications. The living-related transplant represents another step ahead that allows a further reduction of the shortage of organ available for the small children.

[Hepatic artery thrombosis in the pediatric liver transplant]. / La trombosis de la arteria hepática en el trasplante hepático pediátrico.

The hepatic artery thrombosis (HAT) is the most serious complication after pediatric liver transplantation. Most of the cases will need a retransplantation. Multiple factors are keeping in discussion as causes of this complication: Rejection, two or more arteries, complex vascular reconstruction, etc. This article describes our experience with this complication and the prophylactic treatment. From January 1986 through April 1991, 62 orthotopic liver transplants were performed on 48 pediatric patients with a mean age of seven years. The incidence of hepatic artery thrombosis was 19 per 100, increasing when recipients were less than three years and less than 15 kg (23.8 per 100). Forty six cases were rearterialized with end-to-end anastomoses, ten were rearterialized by directly anastomosing them to the recipient aorta and six with iliac artery graft. We use in the last 38 consecutive patients prophylactic heparin during two weeks after transplantation (300 u/kg/d) following with antiaggregants. There were eleven patients with hepatic artery thrombosis, one of them had two episodes in both grafts. We perform a retransplantation in eight patients, two died waiting a new graft and one exceptional patient is out of the clinic with normal liver function and without regraft. Seventeen grafts had two or more arteries from aorta, ten with branches from superior mesenteric artery and five from gastric artery. One graft had a triple hepatic artery and another one was a common hepatic artery from superior mesenteric artery.(ABSTRACT TRUNCATED AT 250 WORDS)

[Liver transplantation in infants younger than one year of age]. / Trasplante hepático en niños menores de un año.

BACKGROUND: The development of the surgical techniques of hepatic division has maken that the young age (less than one year old) is no longer considered a risk factor in the pediatric liver transplant (TH). AIM: To show our experience with the TH in children younger than one year, comparing these results with the rest of the series and in second place to analyze if a bigger experience improves the results of the TH in this group of patients. METHODS: 44 patients that received a TH with less than a year of age are reviewed. Among them, 27 were in the last five years. The survival indexes of the graft and the patient are determined at 1, 5 and 10 years comparing them with three rest of the series. RESULTS: The grafts and patients survival was slightly inferior in the less than one year old, although in the last five years it improved 71.4% vs 82.1% at one year follow-up, and 61.9 in front of 74.5% at five years. The clinical situation of the patients that were transplanted before the year of life was worse: 43% (UNOS III, IV) in front of 13.1% in the same stadiums in the rest of the serie. In the younger patients, 54% of the grafts were reduced, versus 21% in the older. There were not a higher rate of complications in the young group. CONCLUSIONS: In spite of the difficulties of the TH in children younger than one year of age, the results are not very different from those obtained in the rest of the patients. In these results the experience of the transplant center have a great influence.

[Prognosis in children with biliary atresia successfully treated with Kasai's operation]. / Pronóstico de los niños con atresia biliar tratados con éxito con la operación de Kasai.

BACKGROUND: The Kasai procedure, portoenteroanastomosis (PEA) didn't reach international spreading until the seventy's decade, making difficult to find long-term results from children with ABE successfully treated with this technique. At our institution in the last fifteen years all the therapeutics procedures for these patients can be offered, including the liver transplant. AIM: To show the evolution of our patients with ABE treated with the PEA and that survive long-term without being transplanted. METHODS: The clinical course of 22 patients that survive more than 10 years after the PEA with their own liver is reviewed. The hepatic survival indexes of (success, death or transplant) are beyond the tenth year. The problems raised during the follow-up are analysed. RESULTS: From 99 patients with ABE treated primarily in our center, 22 reached the 10 year-old age after the PEA without a liver transplant. In the follow-up, seven if the these finally needed the transplant. Their median age was 12.2 year-old (range: 10.5-13.8) for a progressive hepatocellular damage in 5 cases associated to syndrome hepatopulmonar in two cases. The other fifteen patients have a compensated hepatopathy. Five of them do not have hyperesplenisme and the serum bilirrubine levels are lower than 1.3 mg/dL. The medium age of these patients at the end of the follow-up was 14.8 years. CONCLUSIONS: In spite of the reestablishment of the biliary flow with the PEA, few are the patients with ABE that preserve their hepatic function lapsed long periods of time. Nevertheless the prognosis of these patients is excellent.

[The pediatric liver transplant. The surgical aspects]. / Trasplante hepático pediatrico. Aspectos quirurgicos.

The Liver Transplant Program was begun at "La Paz" Children's Hospital on January 1986 after a long period of experimental activities. This was the first experience in the Madrid Community. From January 1986 to June 1989 we made 32 orthotopic liver transplants in 25 patients, seven received a second graft and one them received a liver segment because the donor had a large liver. 114 patients were evaluated but only 84 were considered candidates for liver transplantation. The different diseases of the transplants were: biliary atresia (9), Alagille syndrome (4), deficit alpha 1-antitrypsin (3), autoimmune hepatitis (2), neonatal hepatitis (1), Byler disease (1), Wolman disease (1). absent bile ducts (1), Wilson disease (1). Surgical technique was the same that has been described by Starzl using Eurocollins and lactate Ringer. In the 100% we made multiorgan procurement, liver and kidneys, 7% with heart and two heart-lung with hypothermia. In one occasion the donor operation was done out of the country (RFA--Düsseldorf). We never used by-pass during anhepatic phase. Arterial reconstruction was done by end-to-end anastomosis and in five patients we used aortic graft. Our arterial thrombosis rate was 18%. In one patient the portal vein was atrophic and we used a femoral graft between superior mesenteric vein and donor portal vein. For biliary reconstruction we used Roux-en-Y with intraluminal stent in 18 cases and choledocho-choledochus anastomosis in seven cases. Four patients had biliary complications: two biliary fistulas secondary to arterial thrombosis, biliary stenosis and bowel perforation by the intraluminal stent.(ABSTRACT TRUNCATED AT 250 WORDS)

[Infection as a complication of the liver transplant]. / La infección como complicación del trasplante hepático.

Currently, the incidence of one episode of infection after liver transplantation is 83 for 100 and 50 for 100 of them are severe. We have studied 30 patients who underwent liver transplantation, ten of them received a second graft. All patients were treated prophylactic with antibiotics 48 hours and selective bowel decontamination, in 23 we used ganciclovir prophylactic. The incidence of infection was 73 for 100, 40 episodes of bacterial infection, eight fungal and ten viral. The direct relationship of infection and mortality was seen in six patients. We studied different risk factors of infection and we found a correlation between surgical time, prior operations and arterial thrombosis with infections.

[Prognostic factors in pediatric liver transplantation. Multivariate analysis]. / Factores pronósticos del trasplante hepático pediátrico. Análisis multivariante.

AIM: To analyze independent risk factors associated with poor graft and patient survival in a series of 292 pediatric liver transplants (PLT) performed in 234 children during a 15 years period. MATERIAL AND METHODS. 1. Univariate graft and patient survival analysis in 45 variables related to pretransplant patient status, surgical technique and donor conditions. 2. Variables found with univariate analysis to be associated with outcome were entered into a stepwise backward proportional hazard model (Cox), to determine independent prediction of outcome. RESULTS: 11 variables influence the graft survival: recipient age, z-score recipient height, UNOS status, recipient and donor weight, transplant for immune hepatitis, platelet transfusion during the transplant, blood index > 4 during the surgery, type of arterial reconstruction, retransplantation and era of the transplant (first er: 1986-1990; 2nd. era: 1991-1995; 3rd. era: 1996-2000). Four of those variables are independent in the multivariate analysis: UNOS 1 status (Odds Ratio, OR = 2.82, 95% confidence interval = 1.36-5.85), recipient < 3 years (OR = 3.76, 95% CI = 2.13-6.63), transplants for autoimmune hepatitis and era (OR of first and second versus third era respectively 3.93 and 2.81). The independent variables influencing the patient survival were: children receiving more than one graft children less than 3 years old and transplant era. CONCLUSIONS: Liver transplant in small children is associated with an increased risk of graft loss and patient dead. The experience of the hospital in pediatric liver transplantation improves the results, particularly in small children.

[The multifocal hepatic hemangioendothelioma. Is always a benign tumor?]. / Hemangioendotelioma multifocal hepático infantil. Es siempre un tumor benigno?

UNLABELLED: The hepatic multicentric haemangioma is defined by its extension, affecting all the mass of the liver. The high mortality associated with it is mostly related with the complications produced by its enormous size (haemodynamic, platelet trapping, spontaneous rupture and bleeding). There is a general belief that is a benign tumor with possibility of spontaneous regression and cure. AIM: Retrospective analysis of our recent cases of MHH with the purpose of: 1 degrees) To show the evolution and results. 2 degrees) To realize if the "benign character" of the tumor is real or if some cases may be considered as malignant tumors. MATERIAL AND METHODS: 10 cases of MHH treated in the last 10 years. In 9 the age of presentation was less than 6 months and one patient was diagnosed at 3 and half years. The diagnosis was confirmed by image techniques in 7 cases and by biopsy in 3. In 7 patients extrahepatic vascular lesions were associated prior to the treatment. Methylprednisolone was given to all the cases and alpha-2-interferon was administered to the patients that not responded to the steroids. Vincristine was added to 2 patients. In two cases the hepatic artery embolization was tried and one patient had a liver transplant. RESULTS: Four children had at least one episode of congestive cardiac insufficiency, two patients suffered a consumption coagulopathy (Kasabach Merrit syndrome), and one presented acute hepatic failure. In six children it has been complete regression of the tumor, one more is still under treatment and three died. The dead were produced by the malignant behavior of the tumor in one case (tumoral rupture of a MHH recurrence in the transplanted liver), and possibly in other (intracranial haemorrhage and hepatic failure in a liver transplantation candidate without demonstrated extrahepatic extension in the previous studies, but with multiorgan dissemination at autopsy. In both cases it was impossible to discover signs of histologic or biologic malignancy neither in the primitive lesion nor in the metastasis. CONCLUSIONS: 1a) The regression of the MHH, spontaneous or induced by the treatment is frequent. 2a) Some cases of MHH are aggressive and develop local recurrences and distant metastasis. 3a) The discrimination between MHH of "benign" or "malignant" behaviour is not possible. 4a) Despite of the unpredictable biological conduct of the tumor, the liver transplantation must be considered as an option in the symptomatic cases that not respond to the conventional treatment.

[Pediatric liver transplantation: now the patients are different and the problems are different as well]. / Trasplante hepático pediátrico: los enfermos son ahora diferentes y los problemas también son distintos.

AIM: 1. To show how in a program of pediatric liver transplantation (PLT) with 12 years of experience, the continuous use of technical innovations has allowed to improve the results and to treat the most complex cases. 2. To point out that when the immediate results of the transplant improve and the evolution time get longer, the late complications become the main source of concern. MATERIAL AND METHODS: The graft survival and the incidence of early surgical complications was compared between the first 100 PLT and the last 100 PLT (Total 235 PLT). The patients survival was also compared between the first and the last 5 years. In every case it was analyzed the age and weight of the children at the time of the transplant and the type of graft (full or reduced liver). The incidence of late complications in the global series was also analyzed, especially the posttransplant lymphoproliferative disease (PTLD) and the late biliary and vascular complications. RESULTS: There are significant differences between the first 100 PLT and the last 100 PLT in relation with the age of the patients (6.8 +/- 0.6 vs 4.2 +/- 0.5 years), the number of children younger than 1 year (4 vs 28), weight (22.4 +/- 1.2 vs 16.9 +/- 1.7 kg), number of PLT in children under 10 kg (7 vs 43), use of reduction techniques (7 vs 49), rate of hepatic artery thrombosis (12% vs 3%), and rate of early biliary complications (13% vs 4%). The 5 years survival of the graft was 51% in the first 100 PLT vs 65% in the last 100 PLT, and the 5 years patients survival was 70.1% in the first 5 years of the program vs 79.6% in the last 5 years. Among the late complications in the global series, 13 cases of PTLD (2 deaths) have special relevance, 6 cases of late anastomotic biliary stricture, 4 cases of portal stenosis, 1 arterial thrombosis, 1 death due to a fulminant sepsis and another death because of a colitis with multiple hepatic abscess. CONCLUSIONS: 1. The result of PLT improve, despite of the fact that in the last years the difficulty of the surgical procedures have increased (the patients are younger, with less weight and the reduction techniques are used more frequently). 2. The late complications have a significant influence in the prognosis, being that influence not completely established yet.