A novel Carcinoembryonic Antigen (CEA)-Targeted Trimeric Immunotoxin shows significantly enhanced Antitumor Activity in Human Colorectal Cancer Xenografts.

Immunotoxins are chimeric molecules, which combine antibody specificity to recognize and bind with high-affinity tumor-associated antigens (TAA) with the potency of the enzymatic activity of a toxin, in order to induce the death of target cells. Current immunotoxins present some limitations for cancer therapy, driving the need to develop new prototypes with optimized properties. Herein we describe the production, purification and characterization of two new immunotoxins based on the gene fusion of the anti-carcinoembryonic antigen (CEA) single-chain variable fragment (scFv) antibody MFE23 to α-sarcin, a potent fungal ribotoxin. One construct corresponds to a conventional monomeric single-chain immunotoxin design (IMTXCEAαS), while the other one takes advantage of the trimerbody technology and exhibits a novel trimeric format (IMTXTRICEAαS) with enhanced properties compared with their monomeric counterparts, including size, functional affinity and biodistribution, which endow them with an improved tumor targeting capacity. Our results show the highly specific cytotoxic activity of both immunotoxins in vitro, which was enhanced in the trimeric format compared to the monomeric version. Moreover, the trimeric immunotoxin also exhibited superior antitumor activity in vivo in mice bearing human colorectal cancer xenografts. Therefore, trimeric immunotoxins represent a further step in the development of next-generation therapeutic immunotoxins.

Alcohol binge disrupts the rat intestinal barrier: the partial protective role of oleoylethanolamide.

BACKGROUND AND PURPOSE: Chronic alcohol consumption alters the gut-brain axis, but little is known about alcohol binge episodes on the functioning of the intestinal barrier. We investigated the influence of ethanol binges on bacterial translocation, gut inflammation and immunity, and tight junction (TJ) structure and the ability of the biolipid oleoylethanolamide (OEA) to prevent ethanol binge-induced intestinal barrier dysfunction. EXPERIMENTAL APPROACH: OEA was injected i.p. before repeated ethanol administration by oral gavage. Plasma, spleen, liver and mesenteric lymph nodes (MLN) were collected in sterile conditions for determination of bacterial load. Immune/inflammatory parameters, TJ proteins and apoptotic markers were determined in colonic tissue by RT-PCR and Western blotting. TJ ultrastructure was examined by transmission electron microscopy. KEY RESULTS: Ethanol binges induced bacterial translocation to the MLN (mainly) and spleen. Colonic tissues showed signs of inflammation, and activation of innate (Toll-like receptor-4) and adaptive (IgA) immune systems and TJ proteins (occludin and claudin-3) were decreased after ethanol binges. Pretreatment with OEA reduced intestinal inflammation and immune activation and partially preserved the TJ structure affected by alcohol binges but had no effect on alcohol-induced apoptosis. Ultrastructural analyses of colonic TJs revealed dilated TJs in all ethanol groups, with less electron-dense material in non-pretreated rats. The protective effects of i.p. OEA did not reduce bacterial translocation to the MLN. However, intragastric OEA administration significantly reduced plasma LPS levels and bacterial translocation to the MLN. CONCLUSION AND IMPLICATIONS: OEA-based pharmacotherapies could potentially be useful to treat disorders characterized by intestinal barrier dysfunction, including alcohol abuse.

mTOR mutations in Smith-Kingsmore syndrome: Four additional patients and a review.

Smith-Kingsmore syndrome (SKS) OMIM #616638, also known as MINDS syndrome (ORPHA 457485), is a rare autosomal dominant disorder reported so far in 23 patients. SKS is characterized by intellectual disability, macrocephaly/hemi/megalencephaly, and seizures. It is also associated with a pattern of facial dysmorphology and other non-neurological features. Germline or mosaic mutations of the mTOR gene have been detected in all patients. The mTOR gene is a key regulator of cell growth, cell proliferation, protein synthesis and synaptic plasticity, and the mTOR pathway (PI3K-AKT-mTOR) is highly regulated and critical for cell survival and apoptosis. Mutations in different genes in this pathway result in known rare diseases implicated in hemi/megalencephaly with epilepsy, as the tuberous sclerosis complex caused by mutations in TSC1 and TSC2, or the PIK3CA-related overgrowth spectrum (PROS). We here present 4 new cases of SKS, review all clinical and molecular aspects of this disorder, as well as some characteristics of the patients with only brain mTOR somatic mutations.

Pharmacogenetic biomarkers of response in Crohn's disease.

Crohn's disease (CD) is a chronic condition, which affects the immune system. It can also affect any part of the digestive tract and be associated with external manifestations. The causes of the disease remain unknown, although it seems to be the result of a combination of factors, such as genetic predisposition, environment, lifestyle and the composition of the microbiota, among others. The treatment protocol begins with a change in eating and smoking habits, and is continued with different lines of treatment, including corticosteroids, immunomodulators and biologic therapy (infliximab and adalimumab), which have shown differences in response among patients, especially with biologic treatment. Several studies have considered the possibility that these differences in response are caused by the genetic variability of patients. Many genes have been investigated as potential predictors of response to biological drugs, such as ADAM17, ATG16L1, EMSY, CASP9, CCNY, CNTN5, FASLG, FCGR, NOD2, PTGER4, IL13, IL1B, IL27, IL11, IL17F, TNF and TNFR genes. In this review, we will gather the information on influence of gene polymorphisms investigated to date on response to biological drugs in CD patients.

Clinicobiological features and prognostic impact of diffuse large B-cell lymphoma component in the outcome of patients with previously untreated follicular lymphoma.

BACKGROUND: The co-existence at diagnosis of follicular lymphoma (FL) and diffuse large B-cell lymphoma (DLBCL) components (FL/DLBCL) has been considered a transformed lymphoma and accordingly treated although clinicobiological information on these patients is scarce. The aim of this study was to analyze the initial features and outcome of FL/DLBCL patients in the rituximab era. PATIENTS AND METHODS: All patients consecutively diagnosed at a single institution with FL/DLBCL (n = 40), as well as those with pure FL (n = 328) or de novo DLBCL (n = 510) as controls. RESULTS: The proportion of the DLBCL component was highly variable (median 50%). In 29 FL/DLBCL cases analyzed, the cell of origin was GCB in 86%, ABC in 10% and unclassifiable in 4%. NOTCH1-2 was mutated in 10% of these cases. The proportion of DLBCL component did not impact on overall survival (OS). Regarding initial characteristics, patients with FL/DLBCL were closer to FL in terms of primary nodal origin, good performance status and advanced stage, whereas the other features were intermediate between FL and DLBCL. FL/DLBCL patients were treated as DLBCL with no further intensification. Complete response and primary refractory rates were 65% and 20%, respectively, with these figures being similar to DLBCL and worse than FL. Progression-free survival and OS were intermediate between FL and DLBCL (5-year OS: 85%, 73% and 63% for FL, FL/DLBCL and DLBCL, respectively). FL/DLBCL histology did not reach independent prognostic value for OS in the multivariate analyses. CONCLUSIONS: The outcome of FL/DLBCL patients is not worse than that of de novo DLBCL. These cases should be treated with immunochemotherapy as DLBCL, but intensification with ASCT may not be necessary. The biological insights of FL/DLBCL warrants further genetic and molecular studies.

Broadening the phenotypic spectrum of POP1-skeletal dysplasias: identification of POP1 mutations in a mild and severe skeletal dysplasia.

Processing of Precursor 1 (POP1) is a large protein common to the ribonuclease-mitochondrial RNA processing (RNase-MRP) and RNase-P (RMRP) endoribonucleoprotein complexes. Although its precise function is unknown, it appears to participate in the assembly or stability of both complexes. Numerous RMRP mutations have been reported in individuals with cartilage-hair hypoplasia (CHH) but, to date, only three POP1 mutations have been described in two families with features similar to anauxetic dysplasia (AD). We present two further individuals, one with severe short stature and a relatively mild skeletal dysplasia and another in whom AD was suspected. Biallelic POP1 mutations were identified in both. A missense mutation and a novel single base deletion were detected in proband 1, p.[Pro582Ser]:[Glu870fs*5]. Markedly reduced abundance of RMRP and elevated levels of pre5.8s rRNA was observed. In proband 2, a homozygous novel POP1 mutation was identified, p.[(Asp511Tyr)];[(Asp511Tyr)]. These two individuals show the phenotypic extremes in the clinical presentation of POP1-dysplasias. Although CHH and other skeletal dysplasias caused by mutations in RMRP or POP1 are commonly cited as ribosomal biogenesis disorders, recent studies question this assumption. We discuss the past and present knowledge about the function of the RMRP complex in skeletal development.

Ascorbic acid PEG-2L is superior for early morning colonoscopies in colorectal cancer screening programs: a prospective non-randomized controlled trial.

BACKGROUND: The quality of colon cleansing and the tolerability of anterograde preparation are essential to the success of colorectal cancer screening. AIM: To compare the tolerability and efficacy of low-volume preparations vs the standard regimen in individuals scheduled for an early morning colonoscopy. STUDY: Participants in a population-based colorectal cancer screening program using the fecal immunochemical test who were scheduled for a colonoscopy from 09:00 a.m. to 10:20 a.m. were prospectively included and assigned to: (1) control group (PEG-ELS 4L): PEG 4L and electrolytes; (2) group AscPEG-2L: a combination of PEG and ascorbic acid 2L; and (3) group PiMg: sodium picosulfate and magnesium citrate 500 mL plus 2L of clear fluids. Tolerability was evaluated with a questionnaire and the quality of bowel preparation with the Boston Bowel Preparation Scale. RESULTS: A total of 292 participants were included: 98 in the PEG-ELS 4L control group, 96 in the AscPEG-2L study group and 98 in the PiMg study group. Low-volume treatments were better tolerated than the standard solution (AscPEG-2L 94.8% and PiMg 93.9% vs PEG-ELS 4L 75.5%; p < 0.0001). The effectiveness of AscPEG-2L was superior to that of PEG-ELS 4L and PiMg (p = 0.011 and p = 0.032, respectively). Patient acceptance was higher for single-dose than for split-dose administration but efficacy was higher with the split dose than with other doses. CONCLUSIONS: In early morning colonoscopies, ascPEG-2L appears to be the best option, especially when administered in a split-dose.

The present and future of gene profiling in breast cancer.

Gene signatures can provide prognostic and predictive information to help in the treatment of early-stage breast cancer. Although many of these signatures have been described, only a few have been properly validated. MammaPrint and OncoType offer prognostic information and identify low-risk patients who do not benefit from adjuvant chemotherapy. With regard to prediction of response, molecular subtypes of breast cancer differ in their sensitivity to chemotherapy, although further studies are needed in this field. Cost, small sample size, and the need to use central laboratories are common limitations to the widespread use of these tools.

[Chronic postoperative pain after general anesthesia with or without a single-dose preincisional paravertebral nerve block in radical breast cancer surgery]. / Comparación entre anestesia general con o sin bloqueo paravertebral preincisional con dosis única y dolor crónico postquirúrgico, en cirugía radical de cáncer de mama.

BACKGROUND AND OBJECTIVE: Over 50% of patients still experience pain a year after mastectomy with or without lymphadenectomy. We aimed to determine the association between anesthetic technique, acute postoperative pain intensity, and the development of chronic postoperative pain. PATIENTS AND METHODS: Forty patients were randomly assigned to receive general anesthesia with or without a paravertebral nerve block for modified radical mastectomy. Postoperative pain was assessed on a visual analog scale at 60 minutes and 24 hours; the patients were also asked to respond to a telephone questionnaire on chronic pain 4 to 5 months later. RESULTS: No significant differences in acute pain were observed. Twenty-nine responded to the telephone questionnaire. Only 1 patient in the paravertebral block group reported chronic neuropathic pain and none had phantom breast pain. Only 1 patient (6.7%) in the paravertebral block group reported chronic neuropathic pain and none had phantom breast pain. In the group that received general anesthesia alone, 1 patient reported phantom breast pain and 6 patients had neuropathic pain, associated with phantom breast pain in 2 cases (incidence of chronic pain 50%; P = .01, Fischer exact test; relative risk, 7.5, 95% confidence interval, 1.0-53.5). The incidences of myofascial pain (neck muscle tightness) were similar in the 2 groups. CONCLUSIONS: Four to 5 months after mastectomy, fewer cases of chronic pain developed in the group operated under general anesthesia with a preincisional paravertebral block than in the group that received only general anesthesia, with postoperative morphine chloride for analgesia.

Premio Dr. Abraham Isaías Kohan - XIII Congreso Argentino de Medicina Transfusional. Proceso de colecta de células progenitoras hematopoyéticas de sangre periférica en pediatría / Dr. Abraham Isaías Kohan Award - XIII Argentine Congress of Transfusion Medicine. Stem cell collections and associated processes in a pediatric population

Fundamento: El trasplante de células progenitoras hematopoyéticas, es una terapéutica utilizada para el tratamiento de pacientes con enfermedades hematológicas y oncológicas, entre otras. Las células progenitoras hematopoyéticas de sangre periférica se obtienen mediante leucaféresis, previa movilización del donante con factores de crecimiento hematopoyético. Objetivos: Comunicar la experiencia de colectas de células progenitoras hematopoyéticas y los procesos asociados, en una población pediátrica candidata a trasplante autólogo o alogeneico. Material y Método: Se evaluaron 53 pacientes y/o donantes para realizar colecta de CPH, entre los años 2008 y 2011. Se tomó consentimiento informado para realizar los procedimientos. Todos fueron evaluados clínicamente y mediante estudios de laboratorio. El momento de colecta se determinó por el número de las células CD34+ en sangre periférica (óptimo 10 a 20 CD34+/uL) en los pacientes y/o donantes, la decisión se tomó en equi­po: médico tratante y de hemoterapia. Resultados: Fueron evaluados 53 candidatos, se realizó colecta en 40: Grupo I autólogo 29 (72,5 %) y Grupo II alogeneico 11 (27,5%). Se realizaron 61 colectas, 50 en Grupo I (82%) y 11 en Grupo II (18%). La mediana de la dosis de movilización con G-CSF fue 12,80 ug/ Kg /día (Rango: 10-25) aplicada entre 4 y 6 días. El recuento de CD34+ en los productos obtenidos resultó en una mediana 6,50 CD34+ x10 6/Kg de receptor (Rango: 1,31-38,34). Conclusiones: Los procesos y procedimientos empleados para obtener células progenitoras hematopoyéticas para el trasplante nos permitieron cumplir los objetivos dentro del programa de garantía de la calidad y obtener resultados clínicos deseados comparables a los publicados en la literatura en este campo.

Background: The hematopoietic stem cell transplantation is a therapy used to treat patients with blood diseases and cancer, among others. Hematopoietic progenitor cells from peripheral blood are obtained by leukapheresis after donor mobilization with hematopoietic growth factors. Objectives: Communicating the experience of stem cell collections and associated processes in a pediatric population candidate for autologous or allogeneic transplantation. Methods: 53 patients and / or donors were evaluated for collection between 2008 and 2011. Informed consent was taken. AII were clinically evaluated and we also performed some laboratory testing. The timing of collection was determined by the number of CD34+ peripheral blood (10 - 20 CD34+ cells /uL) and the decision was made as a team integrated by the physician in charge and the Blood Bank physician.Results: Of the 53 candidates, collection was performed in 40. Group I: autologous 29 (72.5%) and Group II allogeneic 11 (27.5%). 61 collections were made, 50 in Group I (82%) and 11 in Group II (18%). The median dosage of G-CSF mobilization was 12.80 ug/kg/day (range: 10-25) was administered for a period of 4 to 6 days. The CD34+ count in the products resulted in a median of 6,50 x 10 6 CD34+ /kg recipient (range: 1.31 to 38.34). Conclusions: The processes employed in obtaining hematopoietic progenitor cells allowed us to meet goals under the Quality Assurance Program and achieve satisfactory clinical results comparable to those reported in the literature of the field.

[Pulmonary torsion complicating lobectomy: report of 2 cases]. / Torsión pulmonar como complicación de una lobectomía pulmonar, descripción de dos casos.

We report 2 cases of pulmonary torsion discovered during the early postoperative recovery of patients who had undergone lobectomy. Early diagnosis, based on chest radiography and confirmed by computed tomography, meant we were able to avoid major surgical resection and the development of further complications. Pulmonary torsion is a rare but potentially serious abnormality. Prompt diagnosis is the key to preventing tissue injury and complications such as necrotizing pneumonitis, thromboembolic disease, or septic shock. Among the diagnostic tests that can be carried out if there is good reason to suspect torsion, we emphasize simple chest radiography and fiberoptic bronchoscopy, supported by computed tomography or arteriography, even though a firm diagnosis requires surgical exploration of the affected lung. Definitive treatments range from reversing the torsion and securing the lung to resecting the lung if the parenchymal tissue has been fully compromised.

[Axillary recurrences after sentinel lymph node biopsy in initial breast cancer]. / Análisis de recurrencias axilares tras biopsia selectiva del ganglio centinela en cáncer de mama inicial.

OBJECTIVE: The aim of our study was to analyze the application of the Selective Sentinel Lymph Node Biopsy (SLNB) in early Breast Cancer of our population, through the analysis of axillary recurrences in patients with false negative sentinel node procedures without complete axillary lymphadenectomy, after a subsequent clinical follow-up. MATERIAL AND METHODS: A total of 218 early Breast Cancer patients who underwent SLNB after being diagnosed of early breast cancer (T1-2N0) with complete axillary dissection only when the SLNB was positive in the histopathological analysis. In every case, a 2-day protocol was used to localize the sentinel node after injection of (99m)Tc-Nanocolloid. RESULTS: The mean subsequent clinical follow-up was 27 months. A total of 413 sentinel nodes were removed with a median of 1.89/p (range 1-5). Infiltration was detected in 33.9% of patients (59.45% macrometastasis, 22.97% micrometastasis and 17.5% Isolated Tumor Cells (ITC)) and negative for the other nodes excised after conventional lymphadenectomy in 60% of cases. In our population, there was only one case of false negative (FN) SLN due to massive lymphatic blockage, and an abnormal lymph node without uptake adjacent to the SLN was identified intraoperatively. No case of axillary recurrence was detected during an average follow-up of 27 months. CONCLUSION: The absence of axillary recurrences in our population with negative SLNB without complete axillary dissection demonstrates the appropriate local control offered by this procedure in early Breast Cancer.

Recambio plasmático terapéutico en enfermedades neurológicas pediátricas / Therapeutic plasma exchange in pediatric neurological diseases

El recambio plasmático terapéutico (RPT) es un procedimiento utilizado en el tratamiento de distintas patologías, especialmente las de etiología autoinmune. La base fisiopatológica del RPT consiste en la eliminación de mediadores inflamatorios a través de la extracción de un volumen variable de plasma del paciente y su sustitución por una solución de reposición, usualmente albúmina al 5%; utilizando separadores celulares. Objetivo: analizar la experiencia de nuestra institución en el tratamiento con RPT de pacientes con enfermedades neurológicas. Material y métodos: estudio retrospectivo, descriptivo sobre una población de 43 pacientes con enfermedad neurológica (Miastenia Gravis, Guillain Barré, Enfermedad de Devic, Encefalomielitis diseminada aguda, Polineuropatía desmielinizante inflamatoria crónica y Encefalitis de Rasmussen), tratados con una serie de RPT entre junio 1994 y junio 2009. Resultados: se pudieron evaluar 38 pacientes, por falta de información sobre los 5 restantes, observándose alguna mejoría del cuadro clínico en el 79% de los mismos. En 68% de los RPT se observó una o más complicaciones (hipocalcemia, hipotensión, parestesias). Conclusiones: en nuestra experiencia el recambio plasmático terapéutico constituye un tratamiento efectivo para las enfermedades neurológicas en las que fenómenos autoinmunes juegan un rol importante en la patogénesis, incluso en aquellas con un bajo nivel de evidencia clínica según la categorización de indicaciones de la ASFA.

Therapuetica plasma exchange (TPE) is a procedure used for the treatment of different diseases, especially those of autoimmune etiology. The pathophysiological basis of the TPE is the removal of inflammatory mediators through the extraction of a variable volume of patient plasma and its replacement by a solution, usually albumin 5%, using cell separators. objective: to analyze our institution's experience in the TPE treatment of patients with neurological diseases. Material and methods: a retrospective, descriptive study of a population of 43 patients with neurological disease ( Myasthenia Gravis, Guillain Barre syndrome, Devic's disease, Acute demyelinating polyneuropathy, Rasmussen's encephalitis) treated with a series of TPE between june 1994 and june 2009. Results: 38 patients were able to assess, for lack of information on the remaining 5. We observed some clinical improvement in 79% of them. In 68% of the TPE one or more complications (hypocalcemia, hypotension, paresthesias) were observed. Conclusions: in our experience the therapeutic plasma exchange is an effective treatment for neurological diseases in which autoimmune phenomena play an important role in pathogenesis, even in those with low levels of clinical evidence according to the categorization of indications of the ASFA.

Outcome of combined liver and kidney transplantation in hepatitis C: a single-center long-term follow-up experience.

INTRODUCTION: Hepatitis C (HCV) cirrhosis is the prevalent liver disease requiring liver transplantation in the United States. Candidates who also have end-stage renal disease, chronic renal disease stage 4, or prolonged hepatorenal syndrome are considered for combined liver and kidney transplantation (CLKT). MATERIALS AND METHODS: We performed a retrospective study of HCV(+) and HCV(-) CLKT patients with more than 12 months of follow-up and HCV(+) patients with isolated liver transplant (OLT) to compare the outcomes of various groups. RESULTS: Since 1988, 2983 OLTs were performed at our institution including 58 CLKTs. Of these, 23 were HCV(+) subjects who were significantly older than HCV(-) CLKT patients. Race, pretransplant dialysis time, renal indication for CLKT, Model for End-stage Liver Disease score, donor age, liver and kidney rejection as well as occurrence of posttransplant hypertension were similar among HCV(+) and HCV(-) CLKT patients. Posttransplant diabetes was observed in 80% of the HCV(+) group and 30% of the HCV(-) group (P = .01). Renal function seemed to be better in HCV(-) when compared with HCV(+) subjects at 5 years (P = .09). Overall patient survival for HCV(+) CLKT, HCV(-) CLKT, and HCV(+) OLT groups at 1, 2, and 5 years were not significantly different (P = .6). CONCLUSION: HCV positivity should not exclude appropriate candidates for CLKT.

Experiencia del programa de trasplante de células progenitoras hematopoyéticas del Hospital de Pediatría Juan P. Garrahan / Experience of the hematopoietic stem-cell transplantation program at the Hospital Garrahan

El trasplante hematopoyético (TH) es la única o la mejor modalidad terapéutica para diversas enfermedades en Pediatría. Objetivos: Describir el programa de TH desarrollado en el Hospital de Pediatria Prof. Dr. Juan P. Garrahan y analizar prospectivamente la evolución de una cohorte de niños y adolescentes que recibieron en forma consecutiva TH en la unidad de TH. Resultados: 261 pacientes recibieron un TH alógenico y 5 pacientes un TH autólogo; 175 pacientes eran varones. La edad mediana fue de 8.2 años, (rango 0.1 -21). Las patologías más frecuentes fueron la Anemia aplástica adquirida severa (25 por ciento) y las leucemias agudas (46 por ciento). La tasa de mortalidad temprana fue 12.3 por ciento (IC95 por ciento: 8.7 -16.7 por ciento) y la tardía de 10.1 por ciento (IC95 por ciento= 6.8 - 14.3 por ciento). La probabilidad de sobrevida global fue de 62 por ciento (IC95 por ciento = 0.3971 - 0.8095) mediana (rango) de seguimiento de 45.6 meses (0.4-183.4). Las cataratas y los trastornos endocrinológicos fueron las complicaciones tardías más frecuentes. Los sobrevivientes presentaron una buena capacidad funcional y calidad de vida. Conclusiones. La selección de los pacientes y donantes, el desarrollo de la infraestructura de apoyo para la preparación de pacientes y donantes, y la capacitación del personal involucrado directa o indirectamente ha permitido alcanzar resultados de mortalidad relacionada al trasplante, sobrevida y calidad de vida comparables a los publicados por centros con vasta experiencia en pacientes pediátricos.

[Spanish Registry of Home-based Parenteral Nutrition during 2006 (NADYA-SENPE Group)]. / Registro de la Nutrición Parenteral Domiciliaria (NPD) en España del año 2006 (Grupo NADYA-SENPE).

OBJECTIVE: To report the data of the Home Parenteral Nutrition (HPN) registry of the NADYA-SENPE working group for the year 2006. METHODOLOGY: We compiled the data from the on-line registry introduced by the responsible Units for the monitoring of HPN from January 1st to December 31st 2006. RESULTS: 103 patients with HPN were registered (47 males and 56 females), belonging to 19 hospitals. Average age for the 91 patients older than 14 years old was 53.3 +/- 14.9 years old, and that for the 12 patients under 14 years old was 2 +/- 0 years old. Most frequent pathology was neoplasm (29%), followed by intestinal motility disorders (13%). The reason for HPN provision was short bowel syndrome (40%), intestinal obstruction (22%), and malabsorption (21%). Tunneled catheters were mostly used (45%), followed by implanted port-catheters (32%). Catheter related infections were the most frequent complications, with a rate of 0.85 episodes / 10(3) days. HPD was provided for more than two years in 50% of the cases. By the end of 2006, 70.9% of the patients remained active; exitus was the most frequent reason to end HPN (53%). Complementary oral or enteral nutrition was provided to 52% of the patients. 48% of the patients had a normal activity level, and a 55% were autonomous. PN formula was supplied by the hospital in 82% of the cases, while this number was 78% for fungible materials. 27% of the patients were eligible for intestinal transplant. CONCLUSIONS: We observe an increase in registered patients with respect to previous years, with a very different prevalence among regions. Neoplasia remains as the main pathology since 2003. We observe a decrease in catheter-related infections with respect to the 2004-2005 period.