Enuresi: del ritual màgic fins als tractaments actuals / Enuresis: desde el ritual mágico hasta los tratamientos actuales / Enuresis: From magic rituals to current treatments

Introducció. L’enuresi és un trastorn greu, per la gran prevalença i la repercussió que té sobre la qualitat de vida dels pacients i les seves famílies. Al llarg de la història s’han emprat diferents teràpies, i fins avui no es disposa d’una solució definitiva. Objectiu. Examinar l’evolució del concepte d’enuresi, les causes atribuïdes i els tractaments aplicats des de les primeres referències fins a l’actualitat. Mètode. Revisió bibliogràfica del concepte, les causes atribuïdes i els tractaments aplicats contra l’enuresi, estructurada en etapes històriques. Resultats. Al llarg de la història s’han donat diferents explicacions sobre l’origen de l’enuresi, des de la invasió del cos per animals fins a càstigs divins o debilitat de l’individu. La ciència ha descartat diferents teories fins a conèixer els veritables mecanismes fisiopatològics, dels quals encara queden molts aspectes per aclarir. Al llarg dels segles s’han aplicat càstigs, s’han elaborat pocions màgiques, dispositius complexos que inhibeixen la micció, teràpies conductuals, intervencions quirúrgiques i tractaments tòpics i sistèmics. També les alarmes d’enuresi evolucionen, des de les que aplicaven descàrregues elèctriques fins a les actuals que desperten el pacient mitjançant senyals acústics o vibratoris. Conclusions. Els tractaments aplicats per a l’enuresi al llarg de la història han estat relacionats amb el concepte de malaltia de cada època. Conèixer els mecanismes fisiopatològics ha permès trobar tractaments efectius que han millorat la qualitat de vida dels enurètics. Avançar en aquest coneixement és clau per trobar la solució definitiva. (AU)

Introducción. La enuresis es un trastorno grave por su gran prevalencia y repercusión sobre la calidad de vida de los pacientes y sus familias. A lo largo de la historia se han utilizado diferentes terapias y, hasta hoy, no se dispone de solución definitiva. Objetivo. Examinar la evolución del concepto, causas atribuidas y tratamientos aplicados para la enuresis, desde las primeras referencias hasta la actualidad. Método. Revisión bibliográfica del concepto, las causas atribuidas y tratamientos aplicados contra la enuresis, estructurada en etapas históricas. Resultados. A lo largo de la historia se han dado diferentes explicaciones sobre el origen de la enuresis, desde la invasión del cuerpo por animales hasta castigos divinos o debilidad del individuo. Diferentes teorías han sido descartadas por la ciencia hasta el conocimiento de los verdaderos mecanismos fisiopatológicos, de los cuales aún quedan muchos aspectos por aclarar. A lo largo de los siglos se han aplicado castigos, se han elaborado pociones mágicas, complejos dispositivos que inhiben la micción, terapias conductuales, intervenciones quirúrgicas y tratamientos tópicos y sistémicos. También las alarmas de enuresis evolucionan, desde las que aplicaban descargas eléctricas hasta las actuales que despiertan al paciente mediante señales acústicas o vibratorias. Conclusiones. Los tratamientos aplicados para la enuresis a lo largo de la historia se han relacionado con el concepto de enfermedad de cada época. Conocer los mecanismos fisiopatológicos ha permitido encontrar tratamientos efectivos que han mejorado la calidad de vida de los enuréticos. Avanzar en este conocimiento es clave para hallar la solución definitiva. (AU)

Background. Enuresis is a severe disorder because of its high frequency and impact on the quality of life of affected children and their families. Although until today there is no established treatment for enuresis, diverse therapies have been applied over history. Objective. To examine the historic evolution of the concept, presumed causes, and treatments of enuresis from first references until today. Method. Bibliographical review of presumed causes and treatments applied in the treatment of enuresis, structured in historical stages. Results. Throughout history, enuresis has been considered as the invasion of human body by animals, divine punishments, or weakness. Different theories have been rejected by science until the knowledge of the true pathophysiological mechanisms. Punishments, potions and magic, complex devices inhibiting the urination, behavioral therapies, surgical trials, and topical and systemic treatments have been applied throughout history. In addition, different “alarm systems” have been used, evolving from applying electric shocks to wake patients by acoustic or vibrating signals. Conclusions. Treatments applied in the management of enuresis throughout the centuries have been closely related to the understanding of its cause over time. Knowing the pathophysiological mechanisms has made it possible to find effective treatments that have improved the quality of life of affected children. However, to establish a definitive treatment for enuresis, it is necessary to advance in the understanding the diverse mechanisms involved in its etiopathogenesis. (AU)

Hipogammaglobulinemia crónica postrasplante de precursores hematopoyéticos y su tratamiento con gammaglobulina subcutánea en pacientes pediátricos / Chronic hypogammaglobulinemia after allogeneic stem cell transplantation and their treatment with subcutanaeos inmunoglobulin in pediatric patients

Introducción: La hipogammaglobulinemia en los primeros meses postrasplante de progenitores hematopoyéticos (TPH) es común en pacientes pediátricos. Durante esta fase se debe administrar tratamiento sustitutivo con inmunoglobulina humana por vía parenteral para la prevención de infecciones. En algunos casos, esta hipogammaglobulinemia persiste en el tiempo, lo que obliga a prolongar el tratamiento cuando el paciente ya no suele ser portador de una vía central, por lo que son candidatos ideales para el tratamiento de reemplazo por vía subcutánea. Existe escasa bibliografía publicada que describa el uso de esta vía en pacientes pediátricos sometidos a TPH; sin embargo, está ampliamente descrita y con muy buenos resultados en el tratamiento de reemplazo en los niños con inmunodeficiencias primarias. Pacientes y métodos: Se realiza un estudio observacional, descriptivo y longitudinal de carácter retrospectivo. Durante los años 2008-2019 se evalúan a todos los pacientes pediátricos sometidos a TPH en nuestro centro que presentan una hipogammaglobulinemia crónica persistente (superior a un año). Se evalúa la fase de tratamiento con inmunoglobulina intravenosa (Privigen®) y los primeros 4 años de tratamiento con inmunoglobulina subcutánea (Hizentra®) mediante un cuestionario. Resultados: Durante los años 2008-2019 se han realizado en nuestro centro 175 trasplantes de precursores hematopoyéticos, de los cuáles 143 (82%) superaron los 3 meses postrasplante. De estos, 3 (2%) pacientes presentaron una hipogammaglobulinemia persistente. Los 3 comparten factores descritos en la bibliografía involucrados en la reconstitución inmune. Mediante el cuestionario se observa que el cambio de gammaglobulina intravenosa a subcutánea ha supuesto una gran mejoría en la calidad de vida de los pacientes. (AU)

Introduction: Hypogammaglobulinemia in the first months after allogeneic hematopoietic stem cell transplantation (HSCT) is common in pediatric patients. During this phase, replacement therapy with human immunoglobulin must be administered parenterally to prevent infections. In some cases, this hypogammaglobulinemia persists over time, which forces further treatment when the patient is usually no longer a carrier of a central line, making them ideal candidates for subcutaneous replacement therapy. There is little published literature describing the use of this method in pediatric patients undergoing HSCT, widely described in replacement treatment in children with primary immunodeficiencies with very good results. Patients and methods: An observational, descriptive, longitudinal and retrospective study is carried out. During the years 2008–2019, we evaluated all pediatric patients undergoing HSCT in our center with persistent chronic hypogammaglobulinemia (for over a year). The treatment phase with intravenous immunoglobulin (Privigen®) and the first four years of treatment with subcutaneous immunoglobulin (Hizentra®) are evaluated using a questionnaire. Results: During the years 2008-2019, 175 patients underwent HSCT, 143 (82%) of whom exceeded three months after transplantation. 3 (2%) of them had persistent hypogammaglobulinemia. All three share factors described in the literature involved in immune reconstitution. After analyzing the questionnaire, it is observed that switching from intravenous to subcutaneous gammaglobulin has involved a great improvement in their quality of life. (AU)

Chronic hypogammaglobulinemia after allogeneic stem cell transplantation and their treatment with subcutaneous immunoglobulin in pediatric patients.

INTRODUCTION: Hypogammaglobulinemia in the first months after allogeneic hematopoietic stem cell transplantation (HSCT) is common in paediatric patients. During this phase, replacement therapy with human immunoglobulin must be administered parenterally to prevent infections. In some cases, this hypogammaglobulinemia persists over time, which forces further treatment when the patient is usually no longer a carrier of a central line, making them ideal candidates for subcutaneous replacement therapy. There is little published literature describing the use of this method in paediatric patients undergoing HSCT, widely described in replacement treatment in children with primary immunodeficiencies with very good results. PATIENTS AND METHODS: An observational, descriptive, longitudinal and retrospective study is carried out. During the years 2008-2019, we evaluated all paediatric patients undergoing HSCT in our center with persistent chronic hypogammaglobulinemia (for over a year). The treatment phase with intravenous immunoglobulin (Privigen®) and the first four years of treatment with subcutaneous immunoglobulin (Hizentra®) are evaluated using a questionnaire. RESULTS: During the years 2008-2019, 175 patients underwent HSCT, 143 (82%) of whom exceeded three months after transplantation. Three (2%) of them had persistent hypogammaglobulinemia. All three share factors described in the literature involved in immune reconstitution. After analysing the questionnaire, it is observed that switching from intravenous to subcutaneous gammaglobulin has involved a great improvement in their quality of life. CONCLUSIONS: The origin of chronic hypogammaglobulinemia in our patients shows different factors and cannot be attributed to a single cause. Due to the limited number of patients no conclusions can be drawn at the population level. We have been able to observe that replacement treatment with Hizentra 20% has been as effective as the intravenous administration without evidence of an increase in bacterial infections. Furthermore, it has also led to an improvement in quality of life and increased comfort, as the patients themselves have stated.

Experience of the Spanish Group for Hematopoietic Transplantation (GETMON-GETH) in allogenic Hematopoietic stem cell Transplantation in Philadelphia acute lymphoblastic leukemia.

INTRODUCTION: Outcomes in patients diagnosed of acute lymphoblastic leukemia with Philadelphia chromosome (Ph-ALL) remains unfavourable compared to other subtypes of acute lymphoblastic leukemia despite improvements in drug treatments as well as advances in hematopoietic stem cell transplantation (HSCT). PATIENTS AND METHODS: The role of allogeneic HSCT in Ph-ALL patients has been analysed through a multicentric study where data belonging to 70 patients diagnosed of this entity in different centers that received HSCT between years 1998 and 2014, were reported by the Grupo Español de Trasplante Hematopoyético (GETH). RESULTS: The performance of HSCT from year 2004, in first complete remission (CR) status with thymoglobulin (ATG) based conditioning had a favorable impact on overall survival (OS). HSTC performance from year 2004, in first CR with ATG-based conditioning in addition to acute graft versus host disease (aGvHD) development, increased event free survival (EFS). Treatment with imatinib as well as undetectable minimal residual disease (MRD) prior to HSCT, combined with aGvHD, reduced risk of relapse (RR). Patient age less than 10 years when HSCT, first CR and ATG-based conditioning were associated to a lower transplant related mortality (TRM). CONCLUSIONS: Patients that could achieve first CR that also received ATG-based conditioning had a better OS and EFS, so HSCT should be considered for this group of patients.

Experiencia del Grupo Español de Trasplante Hematopoyético (GETMON-GETH) en el trasplante alogénico de progenitores hematopoyéticos en leucemia aguda linfoblástica Philadelphia / Experience of the Spanish Group for Hematopoietic Transplantation (GETMON-GETH) in allogenic hematopoietic stem cell transplantation in Philadelphia acute lymphoblastic leukemia

Introducción: Los resultados de los pacientes con diagnóstico de leucemia linfoblástica aguda con cromosoma de Philadelphia (LLA-Ph) continúan siendo desfavorables comparados con los otros tipos de leucemias linfoblásticas agudas, pese a las mejoras en los tratamientos farmacológicos y los avances del trasplante de progenitores hematopoyéticos (TPH).Pacientes y métodos: Se ha analizado el papel del TPH alogénico en pacientes diagnosticados de LLA-Ph mediante un estudio multicéntrico donde se recogen datos pertenecientes a 70 pacientes reportados por el Grupo Español de Trasplante Hematopoyético (GETH), diagnosticados de esta enfermedad trasplantados en distintos hospitales españoles entre los años 1998 y 2014.Resultados: La realización del TPH a partir del año 2004, en primera remisión completa (RC) y con el empleo de timoglobulina (ATG) como parte del acondicionamiento, impactó favorablemente en la supervivencia global (SG). El TPH a partir del año 2004 en primera RC, así como el tratamiento con ATG y el desarrollo de enfermedad de injerto contra receptor aguda (EICRa), aumentaron la supervivencia libre de eventos (SLE). La administración de imatinib, así como la ausencia de enfermedad mínima residual previas al TPH, junto con la EICRa redujeron la probabilidad de recaída. La edad del paciente inferior a 10 años, el estado de primera RC y el empleo de ATG en el acondicionamiento disminuyeron la mortalidad relacionada con el TPH.Conclusiones: Los pacientes en primera RC que han recibido ATG durante el acondicionamiento presentan mayores SG y SLE. La indicación de TPH debería considerarse en estas situaciones. (AU)

Introduction: Outcomes in patients diagnosed of acute lymphoblastic leukemia with Philadelphia chromosome (Ph-ALL) remains unfavourable compared to other subtypes of acute lymphoblastic leukemia despite improvements in drug treatments as well as advances in hematopoietic stem cell transplantation (HSCT).Patients and methods: The role of allogeneic HSCT in Ph-ALL patients has been analysed through a multicentric study where data belonging to 70 patients diagnosed of this entity in different center that received HSCT between years 1998 and 2014, were reported by the Grupo Español de Trasplante Hematopoyético (GETH).Results: The performance of HSCT from year 2004, in first complete remission (CR) status with thymoglobulin (ATG) based conditioning had a favorable impact on overall survival (OS). HSTC performance from year 2004, in first CR with ATG-based conditioning in addition to acute graft versus host disease (aGvHD) development, increased event free survival (EFS). Treatment with imatinib as well as undetectable minimal residual disease (MRD) prior to HSCT, combined with aGvHD, reduced risk of relapse (RR). Patient age less than 10 years when HSCT, first CR and ATG-based conditioning were associated to a lower transplant related mortality (TRM).Conclusions: Patients that could achieve first CR that also received ATG-based conditioning had a better OS and EFS, so HSCT should be considered for this group of patients. (AU)

[Experience of the Spanish Group for Hematopoietic Transplantation (GETMON-GETH) in allogenic hematopoietic stem cell transplantation in Philadelphia acute lymphoblastic leukemia]. / Experiencia del Grupo Español de Trasplante Hematopoyético (GETMON-GETH) en el trasplante alogénico de progenitores hematopoyéticos en leucemia aguda linfoblástica Philadelphia.

INTRODUCTION: Outcomes in patients diagnosed of acute lymphoblastic leukemia with Philadelphia chromosome (Ph-ALL) remains unfavourable compared to other subtypes of acute lymphoblastic leukemia despite improvements in drug treatments as well as advances in hematopoietic stem cell transplantation (HSCT). PATIENTS AND METHODS: The role of allogeneic HSCT in Ph-ALL patients has been analysed through a multicentric study where data belonging to 70 patients diagnosed of this entity in different center that received HSCT between years 1998 and 2014, were reported by the Grupo Español de Trasplante Hematopoyético (GETH). RESULTS: The performance of HSCT from year 2004, in first complete remission (CR) status with thymoglobulin (ATG) based conditioning had a favorable impact on overall survival (OS). HSTC performance from year 2004, in first CR with ATG-based conditioning in addition to acute graft versus host disease (aGvHD) development, increased event free survival (EFS). Treatment with imatinib as well as undetectable minimal residual disease (MRD) prior to HSCT, combined with aGvHD, reduced risk of relapse (RR). Patient age less than 10 years when HSCT, first CR and ATG-based conditioning were associated to a lower transplant related mortality (TRM). CONCLUSIONS: Patients that could achieve first CR that also received ATG-based conditioning had a better OS and EFS, so HSCT should be considered for this group of patients.

Executive Summary of the Consensus Document on the Diagnosis and Management of Patients with Primary Immunodeficiencies.

Primary immunodeficiencies (PIDs) are rare, undiagnosed and potentially fatal diseases. Clinical manifestations of PID can be fatal or leave sequelae that worsen the quality of life of patients. Traditionally, the treatment of PIDs has been largely supportive, with the exception of bone marrow transplantation and, more recently, gene therapy. The discovering of new affected pathways, the development of new molecules and biologics, and the increasing understanding of the molecular basis of these disorders have created opportunities in PIDs therapy. This document aims to review current knowledge and to provide recommendations about the diagnosis and clinical management of adults and children with PIDs based on the available scientific evidence taking in to account current practice and future challenges. A systematic review was conducted, and evidence levels based on the available literature are given for each recommendation where available.

Executive Summary of the Consensus Document on the Diagnosis and Management of Patients with Primary Immunodeficiencies.

Primary immunodeficiencies (PIDs) are rare, undiagnosed and potentially fatal diseases. Clinical manifestations of PID can be fatal or leave sequelae that worsen the quality of life of patients. Traditionally, the treatment of PIDs has been largely supportive, with the exception of bone marrow transplantation and, more recently, gene therapy. The discovering of new affected pathways, the development of new molecules and biologics, and the increasing understanding of the molecular basis of these disorders have created opportunities in PIDs therapy. This document aims to review current knowledge and to provide recommendations about the diagnosis and clinical management of adults and children with PIDs based on the available scientific evidence taking in to account current practice and future challenges. A systematic review was conducted, and evidence levels based on the available literature are given for each recommendation where available.

Executive Summary of the Consensus Document on the Diagnosis and Management of Patients with Primary Immunodeficiencies / Resumen ejecutivo del Documento de consenso sobre diagnóstico y manejo de pacientes con inmunodeficiencias primarias

Primary immunodeficiencies (PIDs) are rare, undiagnosed and potentially fatal diseases. Clinical manifestations of PID can be fatal or leave sequelae that worsen the quality of life of patients. Traditionally, the treatment of PIDs has been largely supportive, with the exception of bone marrow transplantation and, more recently, gene therapy. The discovering of new affected pathways, the development of new molecules and biologics, and the increasing understanding of the molecular basis of these disorders have created opportunities in PIDs therapy. This document aims to review current knowledge and to provide recommendations about the diagnosis and clinical management of adults and children with PIDs based on the available scientific evidence taking in to account current practice and future challenges. A systematic review was conducted, and evidence levels based on the available literature are given for each recommendation where available

Las inmunodeficiencias primarias (IDP) son unas enfermedades raras, frecuentemente infradiagnosticadas y potencialmente fatales. Las manifestaciones clínicas de las IDP pueden ser muy graves y ocasionar secuelas que empeoran la calidad de vida de los pacientes. Tradicionalmente, el tratamiento de las IDP ha sido fundamentalmente de soporte, con excepción del trasplante de progenitores hematopoyéticos y, más recientemente, la terapia génica. El descubrimiento de nuevos mecanismos patogénicos, el desarrollo de nuevas moléculas y fármacos biológicos y los avances en el conocimiento de las bases moleculares de estas enfermedades han abierto oportunidades para el tratamiento de esta afección. El objetivo de este documento es revisar el conocimiento actual y aportar recomendaciones para el diagnóstico y el tratamiento clínico de los pacientes adultos y pediátricos con IDP basado en la evidencia científica disponible y teniendo en cuenta la actual práctica y los retos futuros. Se realizó una revisión sistemática, que justifica los niveles de evidencia para cada recomendación

Genetic analyses of aplastic anemia and idiopathic pulmonary fibrosis patients with short telomeres, possible implication of DNA-repair genes.

BACKGROUND: Telomeres are nucleoprotein structures present at the terminal region of the chromosomes. Mutations in genes coding for proteins involved in telomere maintenance are causative of a number of disorders known as telomeropathies. The genetic origin of these diseases is heterogeneous and has not been determined for a significant proportion of patients. METHODS: This article describes the genetic characterization of a cohort of patients. Telomere length was determined by Southern blot and quantitative PCR. Nucleotide variants were analyzed either by high-resolution melting analysis and Sanger sequencing of selected exons or by massive sequencing of a panel of genes. RESULTS: Forty-seven patients with telomere length below the 10% of normal population, affected with three telomeropathies: dyskeratosis congenita (4), aplastic anemia (22) or pulmonary fibrosis (21) were analyzed. Eighteen of these patients presented known pathogenic or novel possibly pathogenic variants in the telomere-related genes TERT, TERC, RTEL1, CTC1 and ACD. In addition, the analyses of a panel of 188 genes related to haematological disorders indicated that a relevant proportion of the patients (up to 35%) presented rare variants in genes related to DNA repair or in genes coding for proteins involved in the resolution of complex DNA structures, that participate in telomere replication. Mutations in some of these genes are causative of several syndromes previously associated to telomere shortening. CONCLUSION: Novel variants in telomere, DNA repair and replication genes are described that might indicate the contribution of variants in these genes to the development of telomeropathies. Patients carrying variants in telomere-related genes presented worse evolution after diagnosis than the rest of patients analyzed.

Solid organ transplantation after hematopoietic stem cell transplantation in childhood: A multicentric retrospective survey.

We report data obtained from a retrospective multicenter pediatric survey on behalf of the European Society for Blood and Marrow Transplantation (EBMT). Information on solid organ transplantation (SOT) performed in pediatric recipients of either autologous or allogeneic hematopoietic stem cell transplantation (HSCT) between 1984 and 2016 was collected in 20 pediatric EBMT Centers (25.6%). Overall, we evaluated data on 44 SOTs following HSCT including 20 liver (LTx), 12 lung (LuTx), 6 heart (HTx), and 6 kidney (KTx) transplantations. The indication for SOT was organ failure related to intractable graft-vs-host disease in 16 children (36.3%), acute or chronic HSCT-related toxicity in 18 (40.9%), and organ dysfunction related to the underlying disease in 10 (22.8%). The median follow-up was 10.9 years (95% confidence interval: 1.7-29.5). The overall survival rate at 1 and 5 years after SOT was 85.7% and 80.4%, respectively: it was 74% and 63.2% after LTx, 83.2% after HTx, and 100% equally after LuTx and KTx. This multicenter survey confirms that SOT represents a promising option in children with severe organ failure occurring after HSCT. Additional studies are needed to further establish the effectiveness of SOT after HSCT and to better understand the mechanism underlying this encouraging success.

Complicacions en els dispositius d'accés venós implantables subcutanis en una localització alternativa a l'habitual / Complicaciones de los dispositivos de acceso venoso subcutáneo / Complications related to subcutaneous venous access devices

Fonament: els dispositius d'accés venós implantables sub-cutanis (Port-a-Cath) eviten puncions venoses repetides i milloren la qualitat de vida. La seva col•locació a nivell infraclavicular evidencia i recorda que es pateix una malaltia. Es presenta una localització alternativa que estètica-ment pot ser més acceptada i que no incrementa les possibles complicacions associades. Objectiu: determinar les complicacions dels Port-a-Cath a la paret lateral del tòrax (PLT). Mètode: es realitza un estudi retrospectiu dels pacients in-tervinguts per cirurgia pediàtrica (2013-2015) amb Port-a-Cath a PLT. Resultats: s'insereix el Port-a-Cath en 52 pacients (me-diana d'edat: 42 mesos), majoritàriament a l'hemitòrax dret (80,8%). La durada mitjana del dispositiu és de 17,7 mesos, tenint en compte vuit èxitus (malaltia de base) i cinc pèrdues de seguiment. Com a complicacions, s'identifiquen set infeccions de catèter (13,5%), una fuga (1,9%), una desconnexió (1,9%), tres mal funcionaments (5,7%) i tres exterioritzacions del portal (5,7%); no hi ha migracions. En relació amb les complicacions, tretze pacients requereixen recanvi de Port-a-Cath. Es realitza una prova de comparació de proporcions d'infecció de Port-a-Cath respecte una mostra bibliogràfica (10,1% infecció de Port-a-Cath infraclavicular), amb Z=0,62 i P-valor: 0,732, de manera que no podem rebutjar la hipòtesi nul•la d'igualtat o menor proporció d'infecció del dispositiu de la nostra mostra. Conclusions: no trobem evidència científica per afirmar que la proporció de complicacions infeccioses de la nostra mostra sigui més gran que la dels altres estudis publicats. L'estudi demostra que la PLT del Port-a-Cath és una alternativa vàlida, amb un avantatge estètic i sense incrementar les complicacions infeccioses

Fundamento. Los dispositivos de acceso venoso implantables subcutáneos (Port-a-Cath) evitan punciones venosas repetidas y mejoran la calidad de vida. Su colocación a nivel infraclavicular evidencia y recuerda que se sufre una enfermedad. Se presenta una localización alternativa que estéticamente puede ser más aceptada y que no incrementa las complicaciones asociadas. Objetivo. Determinar las complicaciones de los Port-a-Cath en la pared lateral del tórax (PLT). Método. Se realiza un estudio retrospectivo de pacientes intervenidos por cirugía pediátrica (2013-2015) que presentan Port-a-Cath en la PLT. Resultado. Se inserta el Port-a-Cath en 52 pacientes (mediana de edad: 60 meses), mayoritariamente en el hemitórax derecho (80,8%). La duración media del dispositivo es de 17,7 meses, teniendo en cuenta ocho éxitus (enfermedad de base) y cinco pérdidas de seguimiento. Como complicaciones, se identifican siete infecciones de catéter (13,5%), una fuga (1,9%), una desconexión (1,9%), tres mal funcionamientos (5,7%) y tres exteriorizaciones del portal (5,7%); no hay migraciones. En relación con las complicaciones, trece pacientes requieren recambio de Port-a-Cath. Se realiza una prueba de comparación de proporciones de infección de Port-a-Cath respeto una muestra bibliográfica (10,1% infección Port-a-Cath infraclavicular) siendo Z=0,62 y P-valor: 0,732, con lo que no se puede rechazar la hipótesis nula de igualdad o menor proporción de infección del Port-a-Cath de nuestra muestra. Conclusión. No encontramos evidencia científica para afirmar que la proporción de complicaciones infecciosas de nuestra muestra sea mayor a la de otros estudios publicados. El estudio demuestra que la localización en la PLT de Port-a-Cath es una alternativa válida, con una ventaja estética sin incrementar las complicaciones infecciosas (AU)

Background. The use of subcutaneous implantable venous devices (Port-a-Cath) allows for avoiding repeated venipunctures and improving quality of life. However, placement of these devices under athe clavicle creates a visual evidence and reminder of an underlying disease. We present an alternative location that can be esthetically more acceptable without leading to an increase in complication rates. Objective. To evaluate the complications associated with the placement of a Port-a-Cath in the lateral chest wall. Method. Retrospective study of patients who underwent placement of a Port-a-Cath in the lateral chest wall between 2013 and 2015. Results. Fifty-two patients (median age 60 months) had a Port-a-Cath placed in the lateral chest wall, mostly on the right side (80.8%). The mean duration of the device was 17.7 months (eight patients died due to underlying disease and five were lost to follow-up). The following complications occurred: Seven catheter infections (13.5%), one leak (1.9%), one catheter disconnection (1.9%), three malfunctions (5.7%), and three exposures (5.7%), with no cases of device migrations. Thirteen patients required replacement of their Porta- Cath due to complications. A review of the literature showed an infection rate of 10.1% for infraclavicular devices (Z=0.62, p value: 0.732). The null hypothesis of equality or lower infection rate for the lateral chest wall location could not be rejected. Conclusions. No scientific evidence was found to accept that the proportion of infectious complications in our sample was higher than in the reported studies. Our study demonstrates that the placement of a Port-a-Cath in the lateral chest wall is a valid alternative to the infraclavicular location, providing an esthetic advantage without an associated increase in infectious complications (AU)