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.

[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.

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.

Vibrio parahaemolyticus serovar O3:K6 gastroenteritis in northeast Brazil.

AIMS: To examine the virulence factors and the genetic relationship isolates of the serogroup O3 of Vibrio parahaemolyticus in outbreaks of diarrhoea in the northeast region of Brazil. METHODS AND RESULTS: Eighteen samples of the O3:K6 and O3:KUT serotypes of V. parahaemolyticus were analysed by multiplex polymerase chain reaction (m-PCR) for detection of the tl, tdh and trh genes, by random-amplified polymorphic DNA (RAPD) using two primers, and by amplification of the rDNA 16S-23S region. The gene tl was amplified in all the samples, tdh in 16 while trh in none; amplification of rDNA 16S-23S generated only one profile; each RAPD primer produced two amplification patterns allowing grouping two tdh(-) Kanagawa-negative isolates. CONCLUSIONS: V. parahaemolyticus with characteristics of the pandemic clone appears to be widely disseminated in the studied region. Because of the genetic uniformity of the isolates, elucidation of outbreaks or tracking the source of contamination by the present molecular techniques seems useless. SIGNIFICANCE AND IMPACT OF THE STUDY: Detection of V. parahaemolyticus with virulence potential of pandemic clone from two outbreaks and from several isolated gastroenteritis cases points out the need for inclusion of this micro-organism in the Brazilian routine monitoring of the diarrhoeas for elucidation of their aetiology.

Surgery of liver tumors in children in the last 15 years.

AIM: Aim of the study was to review our experience in the management of liver tumors in children over the last 15 years. PATIENTS AND METHODS: A cohort of 78 children with liver tumors managed in our institution between 1991 and 2006 was retrospectively reviewed. There were 45 males and 33 females with a mean age of 32 +/- 41 months at diagnosis. Most tumors were malignant (n = 57); the most frequently occurring tumor was hepatoblastoma (n = 47), followed by hepatocarcinoma (n = 5), sarcoma (n = 4), and lymphoma (n = 1). Vascular tumors (n = 12) predominated among the benign tumors followed by mesenchymal hamartoma (n = 4), focal nodular hyperplasia (n = 3), adenoma (n = 1), and inflammatory pseudotumor (n = 1). We reviewed the epidemiologic features, clinical presentation, diagnosis, treatment and outcomes. We employed MRI and angio-CT for SIOPEL PRETEXT staging and selected the management accordingly for malignant tumors. We analyzed the long-term survival using Kaplan-Meier curves. RESULTS: Benign tumors had an excellent outcome with both medical or surgical management. Of the malignant tumors 4 were PRETEXT I and were treated by left lateral segmentectomy with 100 % survival; 20 were PRETEXT II (12 left and 8 right lobe) and were treated by lobectomy of the corresponding side, except for 1 case which required OLT (90 % survival); 9 children had PRETEXT III tumors requiring trisegmentectomy or extended lobectomies with OLT in 1 case (77.7 % survival). Fourteen children had PRETEXT IV tumors: 10 received OLT and 9 of them are still alive (64.2 % survival). Overall survival was 80.8 %, and actuarial survival at 6 years was 82.2 %. Other malignant tumors had variable results. CONCLUSIONS: Outcomes have improved much in the last years. Surgical removal is necessary in most cases. Transplantation is a very useful adjunct. Treatment of these tumors should be concentrated in centers with expertise.

[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.

[Multidisciplinary approach in the management of intestinal failure]. / Tratamiento multidisciplinario del fallo intestinal.

OBJECTIVE: [corrected] Our aim was to analyze our results in the management of intestinal failure with a multidisciplinary approach including optimized parenteral nutrition, reconstructive surgery and intestinal transplantation (ITx). MATERIAL AND METHODS: We included all patients evaluated by our team with the diagnosis of IF. We assessed outcome, mortality and complications in children that achieved adaptation and those listed for ITx. RESULTS: Seventy one children (40 boys, 31 girls) were evaluated between 1997 and 2006 because of IF. Forty eight (76%) were referred from other institutions. In 56 cases (80%) IF began in the newborn period. Causes of IF were: short bowel syndrome (52) intestinal motility disorders (16) and intestinal epithelial disorders (3). Median birth weight in the group of SBS was 2.2 Kg and prematurity was an associated condition in 15% of them. Overall, fourteen patients (20%) achieved intestinal adaptation with progressive weaning from PN, the management of these children consisted of optimized parenteral and enteral nutrition and autologous intestinal reconstructive surgery. Nine (13%) are stable under home parenteral nutrition regimen. Eight children (11%), all of them listed for liver and small bowel transplantation, died in the waiting list after a mean waiting time of more than 300 days, with a median of 4 laparotomies and 4 episodes of catheter related sepsis. Four children (5.6%) died in the adaptation process or before their inclusion on the waiting list. Finally, twenty five (35,2%) children underwent 28 intestinal transplantation: 9 isolated small bowel transplantation (SBTx), 16 combined liver and small bowel (CLSB) and 3 multivisceral (MVTx). Among transplanted patients, 9 (36%) died, (3 MVTx, 1 SBTx and 8 CLSB) and four were retransplanted. CONCLUSIONS: Intestinal Transplantation is an established alternative to parenteral nutrition in the treatment of IF, although complications and mortality rates are still considerable, especially MVTx and CLSBTx. Mortality in children listed for intestinal transplantation remains also high. Intestinal adaptation can be achieved with adequate rehabilitation therapy even in some cases with apparently irreversible intestinal transplantation. Early referral before liver failure or other complications arise is crucial is crucial in order to improve the outcome of these patients.

Screening for drugs in oral fluid: illicit drug use and drug driving in a sample of Queensland motorists.

Police Services in a number of Australian states have indicated random roadside drug testing will be implemented to target drug driving. This paper outlines research conducted to provide an estimate of the prevalence of drug driving in a sample of Queensland drivers. Oral fluid samples were collected from 781 drivers who volunteered to participate at Random Breath Testing (RBT) sites in a large Queensland regional area. Illicit substances tested for included cannabis (delta 9 tetrahydrocannibinol [THC]), amphetamine type substances, heroin and cocaine. Drivers also completed a self-report questionnaire regarding their drug-related driving behaviour. Samples that were drug-positive at initial screening were sent to a government laboratory for confirmation. Oral fluid samples from 27 participants (3.5%) were confirmed positive for at least one illicit substance. The most common drugs detected in oral fluid were cannabis (delta 9 THC) (n = 13) followed by amphetamine type substances (n = 11). A key finding was that cannabis was also confirmed as the most common self-reported drug combined with driving and that individuals who tested positive to any drug through oral fluid analysis were also more likely to report the highest frequency of drug driving. Furthermore, a comparison between drug vs drink driving detection rates for the study period revealed a higher detection rate for drug driving (3.5%) vs drink driving (0.8%). This research provides evidence that drug driving is relatively prevalent on Queensland Roads. The paper will further outline the study findings and present possible directions for future drug driving research.

[Liver transplatation for malignant tumors in children]. / Trasplante hepático en tumores malignos en el niño.

OBJECTIVE: To analyse our results on liver transplantation (LTX) in primitive malignant unresectable liver tumours in children and discussing its controversial indications in order to our experience. METHODS/PATIENTS: We report 12 patients with ages ranging from 6 months to 14 years old. They had hepatoblastoma (11) and fibrolamellar hepatocelullar carcinoma (1) without cirrhosis. LTX was considered as primary treatment in 10 patients (PRETEXT IV or any grade if extension to retrohepatic cava vein, 3 hepatic veins or porta vein were assessed) and as rescue therapy after recurrence (1) or persistence of unresectable macroscopic rests (2). One of the patients who underwent a LTX as primary therapy had lung metastases previously resolved with chemotherapy. We used entire liver (5), left lateral segment from cadaveric donor (3), live related donor (3, 2 segments II-III and 1 right liver) and left lateral segment from split (1). All children received chemotherapy prior and post-transplantation following SIOPEL protocol. OUTCOMES ANALYSED: Procedure tolerance, survival, recurrence rate, disease-free period and risk factors for adverse evolution. RESULTS: All patients overcame the LTX and no early loss of the graft was assessed. 2 patients died because of tumoral relapse, 1 after primary LTX and 1 after rescue LTX (survival rate of both groups 90% vs 50%). Graft and patients 1-year, 3-year, 5-year and 14-year survival were 91%, 91%, 82% and 82% respectively. The boy who presented lung metastases developed new ones one year after LTX that were removed and he actually is free of disease. The disease-free period has a probability for 1, 3 and 5 years of 91%, 75% and 75%. Tumoral tissue persistence is the only risk factor for an adverse evolution in our series. CONCLUSIONS: LTX is possible therapeutic approach for unresectable malignant liver tumours. It provides better results as a primary treatment than as a rescue one, being these outcomes comparable to those from resectable tumours. A right staging and referring patients to an expertise centre contribute to optimize results. LTX for patients presenting with lung metastases could be a controversial option. Live-related donor transplantation is an excellent alternative to avoid disease progression during cadaveric waiting list.