Biplot analysis of strawberry genotypes recommended for the State of Espírito Santo.

Most strawberry genotypes grown commercially in Brazil originate from breeding programs in the United States, and are therefore not adapted to the various soil and climatic conditions found in Brazil. Thus, quantifying the magnitude of genotype x environment (GE) interactions serves as a primary means for increasing average Brazilian strawberry yields, and helps provide specific recommendations for farmers on which genotypes meet high yield and phenotypic stability thresholds. The aim of this study was to use AMMI (additive main effects and multiplicative interaction) and GGE biplot (genotype main effects + genotype x environment interaction) analyses to identify high-yield, stable strawberry genotypes grown at three locations in Espírito Santo for two agricultural years. We evaluated seven strawberry genotypes (Dover, Camino Real, Ventana, Camarosa, Seascape, Diamante, and Aromas) at three locations (Domingos Martins, Iúna, and Muniz Freire) in agricultural years 2006 and 2007, totaling six study environments. Joint analysis of variance was calculated using yield data (t/ha), and AMMI and GGE biplot analysis was conducted following the detection of a significant genotypes x agricultural years x locations (G x A x L) interaction. During the two agricultural years, evaluated locations were allocated to different regions on biplot graphics using both methods, indicating distinctions among them. Based on the results obtained from the two methods used in this study to investigate the G x A x L interaction, we recommend growing the Camarosa genotype for production at the three locations assessed due to the high frequency of favorable alleles, which were expressed in all localities evaluated regardless of the agricultural year.

c-Abl links APP-BACE1 interaction promoting APP amyloidogenic processing in Niemann-Pick type C disease.

BACKGROUND: Niemann-Pick type C (NPC) disease is characterized by lysosomal accumulation of cholesterol. Interestingly, NPC patients' brains also show increased levels of amyloid-ß (Aß) peptide, a key protein in Alzheimer's disease pathogenesis. We previously reported that the c-Abl tyrosine kinase is active in NPC neurons and in AD animal models and that Imatinib, a specific c-Abl inhibitor, decreased the amyloid burden in brains of the AD mouse model. Active c-Abl was shown to interact with the APP cytosolic domain, but the relevance of this interaction to APP processing has yet to be defined. RESULTS: In this work we show that c-Abl inhibition reduces APP amyloidogenic cleavage in NPC cells overexpressing APP. Indeed, we found that levels of the Aß oligomers and the carboxy-terminal fragment ßCTF were decreased when the cells were treated with Imatinib and upon shRNA-mediated c-Abl knockdown. Moreover, Imatinib decreased APP amyloidogenic processing in the brain of an NPC mouse model. In addition, we found decreased levels of ßCTF in neuronal cultures from c-Abl null mice. We demonstrate that c-Abl directly interacts with APP, that c-Abl inhibition prevents this interaction, and that Tyr682 in the APP cytoplasmic tail is essential for this interaction. More importantly, we found that c-Abl inhibition by Imatinib significantly inhibits the interaction between APP and BACE1. CONCLUSION: We conclude that c-Abl activity facilitates the APP-BACE1 interaction, thereby promoting amyloidogenic processing of APP. Thus, inhibition of c-Abl could be a pharmacological target for preventing the injurious effects of increased Aß levels in NPC disease.

Viability of Yersinia pestis subcultures in agar stabs.

UNLABELLED: Since its identification as the causative agent of plague in 1894, thousands of Yersinia pestis strains have been isolated and stored. Here, we report the ability of Y. pestis to survive up to 47 years in agar stabs, in rubber-stoppered tubes, under refrigeration (+4 to +10°C), although overall subculture recovery rates were poor and inversely related to the length of time stored. Genetic characterization of virulence gene presence among these subcultures was suggestive of significant variation in the genomic stability of Y. pestis subcultures stored under these conditions. Specifically, we found variation in the presence of plasmid and chromosomal virulence markers (genes pla, lcrV, caf1 and irp2) among multiple subcultures of Y. pestis strains in the 'Collection of Yersinia pestis' (Fiocruz-CYP) maintained by the SRP of FIOCRUZ-PE in Brazil. This variation, together with all of the inherent temporal, geographic and other genetic variation represented by all of the recoverable strains in this historical collection was preserved in new frozen culture stocks stored at -70°C as a result of this study. These frozen culture stocks represent a valuable resource for future comparative studies of Y. pestis. SIGNIFICANCE AND IMPACT OF THE STUDY: We report the ability of Yersinia pestis to survive up to 47 years in agar stabs, in rubber-stoppered tubes, under refrigeration (+4 to +10°C), although overall subculture recovery rates were poor and inversely related to the length of time stored. Genetic characterization of virulence gene presence among these subcultures was suggestive of significant variation in the genomic stability of Y. pestis subcultures stored under these conditions. This variation, together with all of the inherent temporal, geographic and other genetic variation represented by all of the recoverable strains in the historical 'Collection of Yersinia pestis' (Fiocruz-CYP) maintained by the SRP of FIOCRUZ-PE in Brazil was preserved in new frozen culture stocks stored at -70°C as a result of this study. These frozen culture stocks represent a valuable resource for future comparative studies of Y. pestis.

Preservation of the native spleen in multivisceral transplantation.

The native spleen is usually removed in patients undergoing MTV. The consequential asplenic state is associated with a high risk of sepsis, especially in immunosuppressed children. In contrast, the inclusion of an allogeneic spleen in multivisceral grafts has been associated with a high incidence of GVHD. We propose an alternative technique for patients undergoing MTV, consisting of the preservation of the native spleen. This approach avoids the additional risk of infection that characterizes the asplenic state without the detrimental side effects of the allogeneic spleen.

Genetic diversity of Yersinia pestis in Brazil.

Plague outbreaks are occasionally reported in Brazil. Unfortunately, due to great genetic similarity, molecular subtyping of Yersinia pestis strains is difficult. Analysis of multiple-locus variable number of tandem repeats (VNTR), also known as MLVA, has been found to be a valuable tool to discriminate among strains. To check for genetic differences, strains obtained from two different ecological complexes in Brazil collected during two different epidemiological events, an epizootic in Sítio Alagoinha in 1967 and an outbreak in Planalto da Borborema in 1986, were subtyped through MLVA using 12 VNTR loci. Three clusters (A, B and C) were observed. Of the 20 strains from the epizootic, 18 fit into cluster A. Cluster A was divided into two subgroups: A(1) (15 strains) and A(2) (3 strains). Of the 17 strains from the outbreak, 15 fit into cluster B. Cluster B was divided into three subgroups: B(1) (4 strains), B(2) (4 strains) and B(3) (7 strains). Cluster C is a singleton with one epizootic strain. The external standards, Y. pestis CO92 and Y. pseudotuberculosis IP32953, formed two clusters of singletons. The stability of 12 VNTR loci of three unrelated cultures included in this study was assessed. The 12 VNTR loci were stable through multiple serial subcultures in the laboratory. MLVA revealed that Y. pestis populations in Brazil are not monomorphic, and that there is intraspecific genetic diversity among Brazilian plague strains. We conclude that there is some correlation among genetic groups of this species, related to the temporal and geographic origin of isolates.

High-risk hepatoblastoma: results in a pediatric liver transplantation center.

AIM: Aim of the study was assess the results of the treatment of High-Risk Hepatoblastoma (HRH) in a tertiary center where all liver surgery facilities, including pediatric transplantation (LT), are available. METHODS: 91 primary liver tumors treated between 1991 and 2009 were retrospectively reviewed. HRHs as defined by the SIOP criteria (PRETEXT IV or any stage with venous involvement, extrahepatic disease, tumor rupture and <100 ng/ml serum AFP) were identified and imaging and biopsies were reviewed. The treatment consisted of total removal of the tumor, involving extended hepatectomies and LT if necessary, together with SIOPEL-guided chemotherapy. RESULTS: 23/57 hepatoblastomas were HRH (11F/12M). 17 were considered unresectable by standard techniques, 3 had extrahepatic disease, and 3 fulfilled both criteria. Mean age at diagnosis was 2.3 ± 2.4 years. 3 children (referred after chemotherapy) died without surgery. 4 had resections (2 left and 2 right trisegmentectomies). Primary LT was required in 15 children (7 cadaveric donors and 8 living related donor transplantations (LRDT), 2 of them with retrohepatic vena cava replacement), and 1 patient had rescue LT after recurrence. Mean follow-up was 4.8 ± 2.9 years. 2 children who had undergone liver resection developed pulmonary metastases at 1.7 and 1.6 years postoperatively and survived after surgical treatment. 2 children with LT developed EBV-related lymphoma and leukemia respectively but survived. Event-free survival (EFS) at 1, 5, and 10 years was 78.3 ± 8.6%, 63.1 ± 10.5%, and 63.1 ± 10.5%, respectively. 6 children died (3 without surgery, 1 after liver resection, 1 after primary LT and 1 after rescue LT). Overall survival at 1, 5 and 10 years was 78.3 ± 21.7%, 73.2 ± 26.8% and 73.2 ± 26.8%. Of those with primary LT, survival at 1, 5 and 10 years was 93.3 ± 6.4%, 93.3 ± 6.4% and 93.3 ± 6.4%. CONCLUSIONS: Outstanding results in the treatment of HRH are possible in tertiary centers when referral is early (preferably at diagnosis) and specialized liver surgery and transplantation facilities are available.

Pseudoobstrucción intestinal idiopática crónica y síndrome de Berdon: todavía un desafío diagnóstico y terapéutico para el cirujano pediátrico / Chronic idiopathic intestinal pseudoobstruction and berdon syndrome: Still a diagnostic and therapeutic challenge for the pediatric surgeon

Introducción. La pseudoobstrucción intestinal idiopática crónica(POIIC) y el síndrome de Berdon (SB) son trastornos motores de etiopatogenia aún mal conocida, difícil diagnóstico y tratamiento. Pacientes/métodos. Se estudiaron retrospectivamente 26 pacientes (8H/18M) tratados en nuestro hospital por POIIC (21) o SB (5) desde 1982-2009 analizando aspectos clínicos, diagnósticos, terapéuticos y evolutivos. Resultados. El 77% debutaron en los primeros 3 meses de vida(5 tenían diagnóstico prenatal de megavejiga). Los principales signos/síntomas fueron la distensión abdominal (87%), episodios obstructivos recurrentes (70%) y malnutrición (60%), seguidos de vómitos, diarrea crónica, estreñimiento pertinaz y disfagia. Doce tenían afectación urológica (8 megavejiga, 8 RVU/ectasia); 2 asociaban arritmias, 1 sordera, 1 hidrocefalia y 5 mal rotación intestinal. El estudio radiológico fue compatible en todos (1 tenía afectación esofágica exclusiva, 6 presentaban micro colon, 3 dilataciones segmentarias y el resto dilatación generalizada). Se realizó manometría anorrectal en 12 (demostrando RIA+),esofágica en 9 (evidenciando aperistalsis en 4) y antroduodenal en 9(viendo patrón miopático en 4 y neuropático en 5). Las biopsias rectales por succión (16) y musculares (5) fueron normales en todos ellos;las de pared intestinal completa (en 18, tras cirugía) demostraron solo (..) (AU)

Aim/background. Chronic Intestinal Pseudo Obstruction (CIPO)and Berdon Syndrome (BS) are motility disorders with still unclear pathophysiology, and challenging diagnosis and management. Patient and methods. 26 patients (8M/18F) treated of CIPO (21)or BS (5) at our institution between 1982-2009 were retrospectivelyre viewed and clinical, diagnostic, therapeutic and follow-up data were analyzed. Results. 77% had a neonatal onset by the 3rd month of life (5had a prenatal diagnosis of megacystis). Abdominal distension (87%),recurrent suboclusive episodes (70%) and malnourishment (60%)were the main symptoms followed by vomits, chronic diarrhea, constipation and dysfagia. The urinary tract was involved in 12 patients(46%): 8 had megacystis, 8 had vesicoureteral reflux. Arrythmias were seen in 2, deafness in 1, hydrocephalia in 1 and mal rotation in5. Radiological studies were consistent for CIPO in all of them: the (..) (AU)

Necesidad de la bipartición hepática o split en el trasplante en niños / Need of hepatic bipartition or split in the transplant in children

Objetivo. Analizar el beneficio del trasplante hepático (TH) con labipartición adulto-niño. Pacientes /métodos. 1) Análisis de la mortalidad pretrasplante calculadas sobre 228 inclusiones a TH (enero 2004-diciembre 2008).2) Impacto de las técnicas alternativas (donante vivo/bipartición) en la mortalidad pretrasplante de nuestros enfermos. 3) Análisis de los resultados de 33 biparticiones que dieron lugar a 66 trasplantes (1994-2008). Resultados. Referida por 1.000 enfermos y año de exposición, la mortalidad pretrasplante fue de 110 en niños mayores de 5 años, 180en niños de 2 a 5 años, 90 en niños entre 1 y 2 años, y 510 en menores de 1 año (p<0,05 respecto a restantes grupos). 36/66 injertos divididos fueron implantados por nuestro grupo. Cinco se perdieron, 3 por retrasplante, 2 por fallecimiento. La supervivencia actuarial a 10 años fue94,5% (enfermos) y 85,1% (injerto). Los 30 injertos restantes fueron trasplantados en otros hospitales, de los que 4 se perdieron precozmente. En el (..) (AU)

Aim. To analyze the benefits of Split (for adult and for child) in liver transplantation. Patient/methods. 1) Analysis of the waiting list mortality estimated on 228 inclusions for transplant since January 2004 to December2008. 2) Impact of the variant techniques (living-related donor and split)on the waiting list mortality in our patients. 3) Analysis of the outcome of 33 split livers which allowed to perform 66 transplants (1994-2008). Results. Estimated as number of patients by 1,000 candidates by year of exposure, the waiting list mortality was 110 in children older than5 year old, 180 in children from 2 to 5 year-old, 90 in children between1 and 2 year-old and 510 in younger than 1 year (p<0.05 for the last group). 36/66 split grafts were implanted by our group. Five grafts were (..) (AU)

Trasplante intestinal: ¿en qué fase estamos? / Intestinal transplant: in what phase are we?

Objetivo. Analizar la evolución del trasplante intestinal (TI) desde el inicio del programa .Material y métodos. Estudiamos retrospectivamente todos los niños con TI (1997-2009): epidemiología, situación previa, técnica quirúrgica, inmunosupresión, resultados, supervivencia y calidad de vida .Resultados. Realizamos 52 TI (20 intestinal aislado, 20 hepatointestinal, 12 multiviscerales) en 46 niños, edad mediana 32m (rango7m-19a); peso 12,3 kg (rango 3,9-60); 31 tenían intestino corto, 8 dismotilidad, 5 diarrea intratable y 2 misceláneos. Veintiséis se intentaronadaptar inicialmente, 20 se incluyeron directamente como candidatos. La modalidad de trasplante se modificó durante su espera en 18. Todos recibieron tacrolimus y corticoides requiriendo 5 conversión a sirolimus posteriormente. Seis fallecieron el primer mes por sepsis/fallo multiorgánico (mala situación basal); 13 fallecieron tardíamente; observamos rechazo agudo en 20, rechazo crónico en 3, síndrome linfoproliferativo en 8 (fallecieron 6), y EICH en 5 (fallecieron 3). La supervivencia tras 5 años es del 65,2 % (51,7% el injerto). Desde 2006-2008,la supervivencia a los 6m, 1 y 3 años del paciente/injerto es 88,7/84,1,81,2/81,2 y 81,2/71,1%, respectivamente. Tras un seguimiento medio de 39 ± 29 meses, todos los pacientes vivos (n=27, 59%) son autónomos digestivos (70% ya sin estoma), están escolarizados, con mínimosingresos y buena calidad de vida. Conclusiones. El TI se afianza como alternativa de tratamiento en (..) (AU)

Objective. To analyze the evolution of Small Bowel Transplantation program since the beginning of the program. Matherial and methods. All children who underwent intestinal transplantation between 1997 and 2009 were retrospectively reviewed: epidemiological data, status before transplant, surgical technique, immunosupression, results, survival and long. term quality of life were analysed. Results. Fifty-two intestinal transplants were performed in 46 children (20 isolated bowel, 20 combined liver and intestine, and 12 multivisceral); median age was 32m (range 7m-19a); weight 12,3 kg (range3,9-60); 31 had short gut syndrome, 8 dismotility, 5 intractable diarrhea, and two were miscellaneous. Intestinal adaptation was initially attempted in 26 patients, without success, 20 were directly listed for transplant. The modality of transplant was modified in 17 while listed. Baseline immune supression consisted of tacrolimus and steroids, although 5 required conversion to Sirolimus later. Six died during the first month, due to sepsis/multiorganic failure (poor status at transplant);13 died during the long-term follow-up. Acute rejection was seen in 20,chronic rejection in 3, PTLD in 8 (6 died) and GVHD in 5 patients (3died). Overall survival after 5 years of follow-up is 65,2 % (51,7% for (..) (AU)

[Chronic idiopathic intestinal pseudoobstruction and Berdon syndrome: still a diagnostic and therapeutic challenge for the pediatric surgeon]. / Pseudoobstrucción intestinal idiopática crónica y síndrome de berdon: todavía un desafío diagnóstico y terapéutico para el cirujano pediátrico.

AIM/BACKGROUND: Chronic Intestinal Pseudo Obstruction (CIPO) and Berdon Syndrome (BS) are motility disorders with still unclear pathophysiology, and challenging diagnosis and management. Patient and methods. 26 patients (8M/18F) treated of CIPO (21) or BS (5) at our institution between 1982-2009 were retrospectively reviewed and clinical, diagnostic, therapeutic and follow-up data were analyzed. RESULTS: 77% had a neonatal onset by the 3rd month of life (5 had a prenatal diagnosis of megacystis). Abdominal distension (87%), recurrent suboclusive episodes (70%) and malnourishment (60%) were the main symptoms followed by vomits, chronic diarrhea, constipation and dysfagia. The urinary tract was involved in 12 patients (46%): 8 had megacystis, 8 had vesicoureteral reflux. Arrythmias were seen in 2, deafness in 1, hydrocephalia in 1 and malrotation in 5. Radiological studies were consistent for CIPO in all of them: the disease was limited to the esophagus in 1, 3 had segmentary involvement of the small bowel, 6 had microcolon, and the rest had all the digestive tract involved. Anorectal manometry ruled out aganglionosis in 12, esophageal manometry showed aperistalsis in 9 and antro-duodenal manometry confirmed the diagnosis in 9 (visceral myopathy in 4 y neuropathy in 5). Rectal biopsies (16) and muscular biopsies (5) were normal in all of them. Full thickness biopsies (in 18, after surgery) showed myopathy in 12 and neuropathy in 6. Prokinetics and antibiotics for bacterial overgrowth were employed in 100%, 17 required long-time parenteral nutrition (PN), 21 required surgery and 7 were transplanted (4 isolated small bowel, 3 multivisceral). Symptoms improved in 9/15 with an ileostomy. 19 weaned from PN. After a median follow-up of 7.9 years (range 5m-17a), 3 were lost, 17/23 patients are alive and only 2 on home PN. Six died, 3 after being transplanted. CONCLUSIONS: CIPO and/or BS have a wide clinical spectrum and a complex diagnosis; however, the knowledge of the disease and an appropriate treatment, generally aggressive, allows the patients with CIPO to enjoy a long-term acceptable quality of life.

[Need of hepatic bipartition or split in the transplant in children]. / Necesidad de la bipartición hepática o split en el trasplante en niños.

AIM: To analyze the benefits of Split (for adult and for child) in liver transplantation. PATIENT/METHODS: 1) Analysis of the waiting list mortality estimated on 228 inclusions for transplant since January 2004 to December 2008.2) Impact of the variant techniques (living-related donor and split) on the waiting list mortality in our patients. 3) Analysis of the outcome of 33 split livers which allowed to perform 66 transplants (1994-2008). RESULTS: Estimated as number of patients by 1,000 candidates by year of exposure, the waiting list mortality was 110 in children older than 5 year old, 180 in children from 2 to 5 year-old, 90 in children between 1 and 2 year-old and 510 in younger than 1 year (p<0.05 for the last group). 36/66 split grafts were implanted by our group. Five grafts were lost, 3 due to retransplantation and 2 due to death. Overall patient/graft survival alter 10 years of follow-up was 94.5% and 85.1%, respectively. The rest of the grafts (n=30), were used in other hospitals, and 4 were lost in the early postoperative period. Since the beginning of the study, 85.4% of children between 1 and 2 years, received a living-donor or a split graft, as only 59.9% in the younger than 1 year-old group. CONCLUSION: Our results absolutely justify the ethics of split liver transplantation for an adult and a child. Despite other factors, the benefits of the variant techniques in the 1-2 year-old group are obvious. Up to 60% optimization with these techniques in children younger than 1 year would not be yet enough in order to decrease the mortality waiting list down to that of the rest of the groups.

[Intestinal transplant: in what phase are we?]. / Trasplante intestinal: ¿en qué fase estamos?

OBJECTIVE: To analyze the evolution of Small Bowel Transplantation program since the beginning of the program. MATERIAL AND METHODS: [corrected] All children who underwent intestinal transplantation between 1997 and 2009 were retrospectively reviewed: epidemiological data, status before transplant, surgical technique, immunosupression, results, survival and long.term quality of life were analysed. RESULTS: Fifty-two intestinal transplants were performed in 46 children (20 isolated bowel, 20 combined liver and intestine, and 12 multivisceral); median age was 32m (range 7m-19a); weight 12,3 kg (range 3,9-60); 31 had short gut syndrome, 8 dismotility, 5 intractable diarrhea, and two were miscellaneous. Intestinal adaptation was initially attempted in 26 patients, without success, 20 were directly listed for transplant. The modality of transplant was modified in 17 while listed. Baseline immunosupression consisted of tacrolimus and steroids, although 5 required conversion to Sirolimus later. Six died during the first month, due to sepsis/multiorganic failure (poor status at transplant); 13 died during the long-term follow-up. Acute rejection was seen in 20, chronic rejection in 3, PTLD in 8 (6 died) and GVHD in 5 patients (3 died). Overall survival after 5 years of follow-up is 65,2 % (51,7% for the graft). From 2006 to 2008, overall patient/graft survival at 6 m, 1 and 3 years after transplant is 88,7/84,1, 81,2/81,2 and 81,2/71,1%, respectively. After a median follw-up of 39 +/- 29 months, 27 patients are alive (59%), off TPN, (70% had their ostomy taken down), go to school, are scarcely hospitalized and enjoy a good quality of life. CONCLUSIONS: Intestinal transplantation has consolided itself as a good choice for irreversible intestinal failure, being feasible to achieve a normal life. Although overall survival diminishes over time, the center experience has improved the results. These patients need a very close follow-up, once transplant is over, in order to get an early diagnose of immunological complications.

Characterization of potentially virulent non-O1/non-O139 Vibrio cholerae strains isolated from human patients.

Traditional methods of typing Vibrio cholerae define virulent strains according to their recognition by sera directed against the known epidemic serogroups O1 and O139, overlooking potentially virulent non-O1/non-O139 strains. Here, we have undertaken the characterization of eight clinical isolates of non-O1/non-O139 V. cholerae, collected during cholera outbreaks in Brazil. Seven of these were typed as O26 and one, 17155, was defined as non-typable. A PCR-based approach has previously detected in these strains several virulence genes derived from the CTXvarphi prophage and generally associated with pathogenic strains. Here, the presence of the O1-specific wbeN gene was investigated through PCR and found to be restricted to strain 17155, as well as one of the O26 strains, 4756, although neither strain was recognized by O1-specific antisera. The same two isolates were the only strains able to express the cholera toxin in culture, assayed by western blotting. They also possessed four repeats of the heptanucleotide TTTTGAT upstream of the ctxAB genes encoding the cholera toxin. The remaining strains possessed only two intact repeats, whereas pathogenic O1 possessed four to six repeats. To define their evolutionary relationships, selected 16S-23S intergenic rRNA spacer regions were sequenced from the various strains and the resulting sequences used to build phylogenetic trees. Strains 4756 and 17155 always clustered with control O1 strains, whereas the remaining O26 strains clustered separately. These results confirm that, despite their serological phenotype, these two strains are genotypically related to O1 strains and potentially able to produce epidemic cholera.

[Liver transplant from living donor]. / Trasplante hepático de donante vivo.

AIM: Even though Spain has the highest donation rate in the world, our needs cannot be satisfied, specially in younger children. Living-related donor transplant is an alternative in those cases. PATIENTS AND METHOD: We performed a retrospective study of 57 living-related donor transplants performed in our hospital between June 1993 and December 2007. Median age and weight were 1.2 years old (0.5-14.8) and 8.5 kg (5-62). Indications for transplant were as follow: biliary atresia in 42 cases (73.7%), hepatic tumor in 8 (14%) and others in 7 patients. Type of graft was: monosegment (n=1), left lateral segment (n=45), extended left lateral segment (n=5), left liver (n=4), right liver (n=2). We studied the following factors: graft and patient survival (Kaplan Meier), perioperative conditions, complications, causes of graft lost, donor complications and technique difficulties. RESULTS: Patient survival at 3 months, 1 year, 5 years and 10 years was 98.2%, 98.2%, 95% and 95% respectively. Three grafts werelost due to arterial thrombosis, two due to rejection, one due to portal thrombosis and three due to other causes. Complications were as follow: biliary fistula in the cut surface (6), biliary anastomosis complications (6), cut surface abcess (1), portal stenosis (2), suprahepatic stenosis (1) and intestinal perforation (2). Most common complication in donors was biliary leak (4). Among the technique difficulties, 8 patients needed major reconstruction of suprahepatic vein; 4 needed complex portal reconstruction, 6 patients had double biliary tract and 4 patients needed multiple arterial anastomosis. Wall closure was delayed (Goretex) in 35% of cases (20). CONCLUSIONS: Despite technical complications, results after living-related donor transplantation are excellent. It is particularly favourable for children with low weight, since Spanish policy for organ allocation does not make easy to find an adecuate donor in short periods of time. Without living-related donor transplantations, mortality pretransplant would be much higher.

[Liver bipartition as an alternative to the transplant]. / Bipartición hepática como alternativa en el trasplante.

AIM: Liver pediatric transplantation finds in the lack of donors its main limitation. An alternative in those cases is split liver grafts from bigger donors. PATIENTS AND METHOD: We performed a retrospective study of 56 hepatic split transplants performed between 1994 and 2007. Twenty-nine children were transplanted with a median age and weight of 1.8 years old (0.3-9) and 9.7 kg (6.2-23). In 16 cases (53.3%) liver transplant was performed in emergency situation. In one patient we performed a combined transplant (liver-kidney) and in another patient it was a second transplant due to primary graft failure after receiving an hepatointestinal allograft. Type of grafts used were: lateral left segment (n=26), extended lateral left segment (n=1) and extended right liver (n=3). Median donor age and weight were 20 years old (8-44) and 60 kg (24-80). We studied patient and graft survival (Kaplan Meier), perioperative factors, complications and net rate of early complications in adults recipients. RESULTS: Patient survival was 96.7% after 6 months, 1 year, 5 years and 10 years. Id for grafs 86.7%. Two grafts were lost due to arterial thrombosis, one due to primary non function and another due to recipient death secondary to a sepsis. Five children had major biliary complications and 2 of them developed multiple intrahepatic stenoses, one of them being on waiting list for retransplant. Early graft lost (retransplant or death before leaving the hospital) occurred in 4 out of the 25 grafts transplanted in other centers (25 adults, 1 kid); all of them occurred in the initial period (1994-2001). CONCLUSIONS: Even though it is clearly documented that benefit of transplant (measured in years of life won) is very good after split transplantation, nowadays criteria for organ allocation in Spain do not allow a more extensive diffusion of this technique and it is confined to urgent transplant. Even in those cases, results after split transplantation are excellent. Without this possibility our pretransplant mortality would be much higher.

Trasplante hepático de donante vivo / Liver transplant from living donor

Objetivo. Pese a la elevada tasa de donaciones en España, las necesidades de trasplante hepático, especialmente en niños de bajo peso, no están cubiertas. Una alternativa en estos casos es recurrir a los donantes vivos. Pacientes y método. Análisis retrospectivo de los 57 trasplantes hepático con injerto de donante vivo (THDV), realizados en nuestro hospital entre junio de 1993 y diciembre de 2007. La mediana de edad y peso fue de 1,2 años (0,5 – 14,8) y 8,5 Kg (5 – 62) respectivamente. Las indicaciones de trasplante fueron atresia biliar en 42 casos (73,7%),tumor hepático irresecable en 8 (14,0%) y otras en 7 pacientes. El tipo de injerto fue: monosegmento (n=1); segmento lateral izquierdo (n =45), segmento lateral izquierdo extendido (n = 5), hígado izquierdo(n=4), hígado derecho (n = 2). Las variables analizadas fueron: supervivencia del enfermo y del injerto (Kaplan Meier), variables (..) (AU)

Aim. Even though Spain has the highest donation rate in the world, our needs cannot be satisfied, specially in younger children. Living related donor transplant is an alternative in those cases. Patients and method: We performed a retrospective study of 57living-related donor transplants performed in our hospital between June1993 and December 2007. Median age and weight were 1.2 years old(0.5-14.8) and 8.5 kg (5-62). Indications for transplant were as follow: biliary atresia in 42 cases (73.7%), hepatic tumor in 8 (14%) and others in 7 patients. Type of graft was: monosegment (n=1), left lateral segment (n=45), extended left lateral segment (n=5), left liver (n=4),right liver (n=2). We studied the following factors: graft and patient survival (Kaplan Meier), perioperative conditions, complications, causes of graft lost, donor complications and technique difficulties. Results. Patient survival at 3 months, 1 year, 5 years and 10 years was 98.2%, 98.2%, 95% and 95% respectively. Three grafts were lost due (..) (AU)

Bipartición hepática como alternativa en el trasplante / Liver bipartition as an alternative to the transplant

Objetivo. El trasplante hepático pediátrico, encuentra en la falta de donantes su principal limitación. Una alternativa en estos casos es recurrir a la bipartición (split) de hígados procedentes de donantes de mayor peso. Pacientes y método. Análisis retrospectivo de 56 trasplantes hepáticos con injerto procedente de bipartición entre 1994-2007. Fueron implantados a 29 niños con una mediana de edad y peso de 1,8 años (0,3-9,0) y 9,7 Kg (6,2-23). En 16 casos (53,3%) el TH fue realizado en situación de emergencia. En un caso el trasplante fue combinado (hígado riñón) y en otro se trataba de un retrasplante por fallo primario del injerto hepático tras haber recibido un aloinjerto hepatointestinal. El tipo de injerto fue: segmento lateral izquierdo (n = 26), segmento lateral izquierdo extendido (n = 1), hígado derecho extendido (n = 3). La mediana de (..) (AU)

Aim. Liver pediatric transplantation finds in the lack of donors its main limitation. An alternative in those cases is split liver grafts from bigger donors. Patients and method. We performed a retrospective study of 56hepatic split transplants performed between 1994 and 2007. Twenty-nine children were transplanted with a median age and weight of 1.8 years old (0.3-9) and 9.7 kg (6.2-23). In 16 cases (53.3%) liver transplant was performed in emergency situation. In one patient we performed a combined transplant (liver-kidney) and in another patient it was a second transplant due to primary graft failure after receiving an hepatointestinal allograft. Type of grafts used were: lateral left segment (n=26), extended lateral left segment (n=1) and extended right liver (n=3). Median donor (..) (AU)

Variant techniques for liver transplantation in pediatric programs.

INTRODUCTION: Several variant techniques have been developed as alternatives to whole liver transplantation to improve size matching, timing, or simply to increase the pool of donors. The aim of this study was to assess the requirements of these techniques and their outcomes in a pediatric transplant program. PATIENTS AND METHOD: A retrospective analysis of children on the waiting list in the last 4 years was carried out. Data of patients who died while on the waiting list (WL) were recorded. Transplanted patients were divided according to the type of graft: whole liver, split, living donor and reduced liver. The analyzed outcome variables were: age, weight, UNOS status, cause of liver failure, complications and graft and patient survival. Comparisons between types of graft were performed by using Kaplan-Meier, log-rank, chi (2) and Kruskal-Wallis tests. RESULTS: During the period studied, 116 children were listed for liver transplantation. Of these 116 children, nine (7.7 %) died after a mean period of 40.5 (5-175) days waiting for a suitable graft. Their median age at inclusion was 214 (35-1607) days, and median weight was 7.2 (12.3-3.6) kg. The cause of liver failure in this group was: 1 hemochromatosis, 1 hepatoblastoma, 2 biliary atresia, 2 acute liver failure, 2 primary non-function (PNF) and 1 chronic rejection. Liver transplantation was performed in 103 children: 25 (24 %) whole livers, 17 (16.5 %) split, 29 (28 %) living donor, 32 (31 %) reduced and 4 remain on the waiting list. Recipient age and weight were significantly lower in those receiving split and living donor than in those who given whole livers. Patient and graft survival were similar in all groups although there was a trend to lower graft survival in patients receiving whole livers. More than 50 % of patients with UNOS status I received a split graft and 5/6 children with hepatoblastoma underwent living donor transplantation. There were no differences in the rate of acute vascular complications, but long-term biliary complications were more frequent in split and living donor grafts. CONCLUSIONS: As long as the goal of zero mortality for children on the waiting list is not achieved, variant techniques will be necessary in pediatric liver transplantation programs. Split and living donor were employed mostly to treat younger children and particularly those with a higher UNOS status. Children with tumors were treated mainly with living donor grafts. Variant techniques, which are absolutely necessary in a pediatric program, need to be improved in order to avoid long-term biliary complications.