Sistema de presión negativa en el tratamiento de fístulas enterocutáneas en la población pediátrica / Negative pressure system in the treatment of enterocutaneous fistulas in the pediatric population

Objetivos. Las fístulas enterocutáneas (FE) de evolución tórpida y los defectos de pared abdominal (DPA) en niños multioperados son difíciles de manejar y pueden ser causa de fallo intestinal. El objetivo de este estudio fue analizar si el sistema de presión negativa (SPN) que disminuye el edema, favorece la vascularización y la aparición del tejido de granulación, mejora la cicatrización y el pronóstico de las FE. Métodos. Se realizó un estudio retrospectivo de niños multioperados con fístulas enterocutáneas recalcitrantes y de alto débito con o sin defectos de pared abdominal, entre 2008-2014 tratados con SPN. Las variables analizadas fueron el cierre de la fístula y/o defecto abdominal, el tiempo transcurrido y las complicaciones del tratamiento. Resultados. Ocho pacientes cumplieron criterios de inclusión y fueron tratados con SPN con una mediana de 25 días (5-50). La etiología fue vólvulo intestinal (2), enterocolitis necrosante (2), gastrosquisis (2), Blue Rubber Bled Nevus (1) y fístula de anastomosis duodenopancreática en un trasplante hepatopancreático (1). Siete pacientes asociaron defectos de pared abdominal. Dos pacientes se reintervinieron posteriormente por evisceración y ninguno desarrolló nuevas fístulas. Dos pacientes presentaron nueva FE en otra localización y también fue tratada con SPN, resolviéndose. No se identificaron complicaciones derivadas del empleo de la presión negativa. Tras un seguimiento de 5 años, 3 niños recibieron posteriormente un trasplante multivisceral y 4 fallecieron [candidato a trasplante (1), encefalopatía (1), anemia hemolítica (1), sepsis de catéter (1)]. Conclusión. A pesar de nuestra serie limitada de pacientes proponemos este sistema como una herramienta útil en el manejo de FE y/o DPA

Aim of the study. The management of children with enterocutaneous fistulas (EF) along with large abdominal wall defects secondary to multiple surgical interventions can be difficult and sometimes lead to intestinal failure (IF). The aim of this study is to present the results of negative pressure systems and their properties (edema reduction angiogenesis promotion and granulation tissue formation) in children with enterocutaneous fistulas (EF) and their prognosis. Materials and Methods. A retrospective analysis of children with refractory, high output EF treated with NPS between 2008-2014. Outcome variables were duration and effectiveness of treatment as well as complications associated with NPS. Results. Eight patients met inclusion criteria and were treated with NPS during a median of 25 days (range 5-50). The aetiologies were volvulus (2), necrotizing enterocolitis (2), gastroschisis (2), Blue Rubber Bleb Nevus (1) and duodenopancreatic anastomosis fistula following hepatopancreatic transplantation (1). Most patients (n=7) had large abdominal wall defects that closed during treatment, though two patients required further laparotomies due to evisceration. Two patients developed a second EF that was also successfully treated with NPS. No complications were identified arising from the use of NPS. After a 5-yr follow up 3 patients had a multivisceral transplantation and survive, and 4 died due to encephalopathy (1), hemolytic anemia (1), catheter-related sepsis (1) and one while waiting for a multivisceral graft for transplantation. Conclusions. Despite of a limited series of patients we recommend NPS as a useful tool in the management of EF and/ or abdominal wall defects

[Negative pressure system in the treatment of enterocutaneous fistulas in the pediatric population]. / Sistema de presión negativa en el tratamiento de fístulas enterocutáneas en la población pediátrica.

AIM OF THE STUDY: The management of children with enterocutaneous fistulas (EF) along with large abdominal wall defects secondary to multiple surgical interventions can be difficult and sometimes lead to intestinal failure (IF). The aim of this study is to present the results of negative pressure systems and their properties (edema reduction angiogenesis promotion and granulation tissue formation) in children with enterocutaneous fistulas (EF) and their prognosis. MATERIALS AND METHODS: A retrospective analysis of children with refractory, high output EF treated with NPS between 2008-2014. Outcome variables were duration and effectiveness of treatment as well as complications associated with NPS. RESULTS: Eight patients met inclusion criteria and were treated with NPS during a median of 25 days (range 5-50). The aetiologies were volvulus (2), necrotizing enterocolitis (2), gastroschisis (2), Blue Rubber Bleb Nevus (1) and duodenopancreatic anastomosis fistula following hepatopancreatic transplantation (1). Most patients (n=7) had large abdominal wall defects that closed during treatment, though two patients required further laparotomies due to evisceration. Two patients developed a second EF that was also successfully treated with NPS. No complications were identified arising from the use of NPS. After a 5-yr follow up 3 patients had a multivisceral transplantation and survive, and 4 died due to encephalopathy (1), hemolytic anemia (1), catheter-related sepsis (1) and one while waiting for a multivisceral graft for transplantation. CONCLUSIONS: Despite of a limited series of patients we recommend NPS as a useful tool in the management of EF and/ or abdominal wall defects.

OBJETIVOS: Las fístulas enterocutáneas (FE) de evolución tórpida y los defectos de pared abdominal (DPA) en niños multioperados son difíciles de manejar y pueden ser causa de fallo intestinal. El objetivo de este estudio fue analizar si el sistema de presión negativa (SPN) que disminuye el edema, favorece la vascularización y la aparición del tejido de granulación, mejora la cicatrización y el pronóstico de las FE. METODOS: Se realizó un estudio retrospectivo de niños multioperados con fístulas enterocutáneas recalcitrantes y de alto débito con o sin defectos de pared abdominal, entre 2008-2014 tratados con SPN. Las variables analizadas fueron el cierre de la fístula y/o defecto abdominal, el tiempo transcurrido y las complicaciones del tratamiento. RESULTADOS: Ocho pacientes cumplieron criterios de inclusión y fueron tratados con SPN con una mediana de 25 días (5-50). La etiología fue vólvulo intestinal (2), enterocolitis necrosante (2), gastrosquisis (2), Blue Rubber Bled Nevus (1) y fístula de anastomosis duodenopancreática en un trasplante hepatopancreático (1). Siete pacientes asociaron defectos de pared abdominal. Dos pacientes se reintervinieron posteriormente por evisceración y ninguno desarrolló nuevas fístulas. Dos pacientes presentaron nueva FE en otra localización y también fue tratada con SPN, resolviéndose. No se identificaron complicaciones derivadas del empleo de la presión negativa. Tras un seguimiento de 5 años, 3 niños recibieron posteriormente un trasplante multivisceral y 4 fallecieron [candidato a trasplante (1), encefalopatía (1), anemia hemolítica (1), sepsis de catéter (1)]. CONCLUSION: A pesar de nuestra serie limitada de pacientes proponemos este sistema como una herramienta útil en el manejo de FE y/o DPA.

Enfermedades metabólicas congénitas en un servicio de urgencias pediátricas / Inherited metabolic disorders in pediatric emergency services

Introducción: Los avances en el diagnóstico precoz y tratamiento han propiciado una mayor supervivencia, y en mejores condiciones, de los pacientes con enfermedades metabólicas congénitas (EMC). Estos pueden acudir a los Servicios de Urgencias Pediátricas (SUP) por motivos relacionados o no con su enfermedad. El propósito de este trabajo fue revisar las características de las visitas al SUP de estos pacientes, en un hospital de tercer nivel. Material y métodos: Se desarrolló un estudio observacional retrospectivo en el que se analizaron todas las visitas al SUP del Hospital Infantil La Paz durante los años 2011 y 2012 de pacientes con EMC. Se registraron el tipo de EMC, el motivo de consulta, el tiempo de evolución de los síntomas, la necesidad de ingreso hospitalario y la presencia de descompensación metabólica. Resultados: En total, fueron analizadas 107 visitas, siendo el motivo de consulta más frecuente los procesos respiratorios (30,8%). Cuando la consulta fue por vómitos, los pacientes con trastornos relacionados con las proteínas fueron los que menos tardaron en acudir al SUP. Un tercio de las visitas se siguió de ingreso, siendo la mitad de ellas por descompensación metabólica de la patología de base. Conclusiones: Los pacientes con EMC acudieron al SUP por motivos muy diversos, que en algunos casos fueron causa o consecuencia de una descompensación metabólica aguda que motivó el ingreso hospitalario. Al tratarse de enfermedades con baja prevalencia individual, resulta de interés contar con protocolos diagnóstico-terapéuticos que faciliten una atención óptima (AU)

Introduction: Advances in the early diagnosis and treatment have led to improved survival, and a better quality of life for patients with inherited metabolic disorders (IMD). They can go to the Pediatric Emergency Services (PES) for reasons unrelated to their disease. The purpose of this study was to review the characteristics of visitors to the PES of these patients in a tertiary hospital. Material and methods: A retrospective observational study was conducted on all visits from patients with IMD to the PES of Hospital Infantil La Paz over the years 2011 and 2012. IMD type, complaint, duration of symptoms, need for hospitalization, and presence of metabolic decompensation was recorded. Results: A total of 107 visits were analyzed, with the most frequent reason being for consultation of respiratory processes (30.8%). When the consultation was for vomiting, patients with protein-related disorders were those who delayed less in going to PES. One third of visitors were admitted, half of them due to metabolic decompensation of the underlying pathology. Conclusions: Patients with IMD came to PES for many different reasons, which in some cases were the cause or consequence of an acute metabolic decompensation that led to hospitalization. Being diseases with low prevalence, it would be useful to have diagnostic and therapeutic protocols in order to provide optimal care (AU)

Enfermedad de Ménétrier asociada a infección por citomegalovirus / Ménétrier′ s disease associated with cytomegalovirus infection

La enfermedad de Ménétrier es una entidad poco frecuente en el niño, caracterizada por una gastroenteropatía pierde proteínas con engrosamiento de la mucosa gástrica y edemas generalizados. La etiología vírica es la más frecuente, siendo el citomegalovirus el agente infeccioso más habitualmente implicado. A diferencia del adulto, es un trastorno autolimitado y con buen pronóstico en el niño. Se revisa a 4 pacientes (3 varones y una mujer) diagnosticados de enfermedad de Ménétrier en los últimos 5 años. La edad media de presentación fue de 28,7 meses (rango: 10-48 meses). La sintomatología clínica más común fue fiebre, vómitos y edemas. La endoscopia demostró engrosamiento de pliegues gástricos y erosiones en grado variable. Todos los pacientes asociaban infección gástrica por citomegalovirus y presentaron una evolución favorable, con resolución del trastorno en pocas semanas

Menetrier's disease is a rare entity in children, characterized by a protein-losing gastroenteropathy with thickening of the gastric mucosa and generalized edema. The most common etiology is viral, and cytomegalovirus is the agent most frequently implicated. Unlike in the adult, it is a self-limited disorder with a good prognosis in children. Four patients (three boys and one girl) diagnosed with Ménétrier disease in the past five years were reviewed. The mean age at presentation was 28.7 months (range: 10-48 months). The most common clinical symptoms were fever, vomiting, and edema. Endoscopy showed thickened gastric folds and erosions in several stages. All patients had an associated gastric cytomegalovirus infection, and a favorable outcome, with resolution of the disorder,was observed within a few weeks

[Ménétriers disease associated with cytomegalovirus infection]. / Enfermedad de Ménétrier asociada a infección por citomegalovirus.

Menetrier's disease is a rare entity in children, characterized by a protein-losing gastroenteropathy with thickening of the gastric mucosa and generalized edema. The most common etiology is viral, and cytomegalovirus is the agent most frequently implicated. Unlike in the adult, it is a self-limited disorder with a good prognosis in children. Four patients (three boys and one girl) diagnosed with Ménétrier disease in the past five years were reviewed. The mean age at presentation was 28.7 months (range: 10-48 months). The most common clinical symptoms were fever, vomiting, and edema. Endoscopy showed thickened gastric folds and erosions in several stages. All patients had an associated gastric cytomegalovirus infection, and a favorable outcome, with resolution of the disorder,was observed within a few weeks.

[Inherited metabolic disorders in pediatric emergency services]. / Enfermedades metabólicas congénitas en un servicio de urgencias pediátricas.

INTRODUCTION: Advances in the early diagnosis and treatment have led to improved survival, and a better quality of life for patients with inherited metabolic disorders (IMD). They can go to the Pediatric Emergency Services (PES) for reasons unrelated to their disease. The purpose of this study was to review the characteristics of visitors to the PES of these patients in a tertiary hospital. MATERIAL AND METHODS: A retrospective observational study was conducted on all visits from patients with IMD to the PES of Hospital Infantil La Paz over the years 2011 and 2012. IMD type, complaint, duration of symptoms, need for hospitalization, and presence of metabolic decompensation was recorded. RESULTS: A total of 107 visits were analyzed, with the most frequent reason being for consultation of respiratory processes (30.8%). When the consultation was for vomiting, patients with protein-related disorders were those who delayed less in going to PES. One third of visitors were admitted, half of them due to metabolic decompensation of the underlying pathology. CONCLUSIONS: Patients with IMD came to PES for many different reasons, which in some cases were the cause or consequence of an acute metabolic decompensation that led to hospitalization. Being diseases with low prevalence, it would be useful to have diagnostic and therapeutic protocols in order to provide optimal care.

Valor de la citrulina plasmática como biomarcador de función intestinal en el síndrome de intestino corto y en el trasplante intestinal / Plasma citrulline concentration as a biomarker of intestinal function in short bowel syndrome and in intestinal transplant

Introducción: La citrulina es un aminoácido producido exclusivamente por los enterocitos. Se estudió su valor como biomarcador de masa enterocitaria funcionante en pacientes con fracaso intestinal por síndrome de intestino corto (SIC) y su relación con la tolerancia digestiva. Material y métodos: Se determinó la concentración plasmática de citrulina por cromatografía líquida de alta resolución (normal > 15 μmol/L) en 57 pacientes (edad 0, 5-18 años) con fracaso intestinal en distintas situaciones evolutivas. Fueron excluidos pacientes deshidratados, con insuficiencia renal u otras situaciones que pudieran alterar los resultados. Se clasificaron en grupos: I : SIC extremo dependientes de nutrición parenteral (NP); II : SIC en alimentación mixta enteral-parenteral; III : SIC adaptados y autónomos sin NP; IV : trasplantados y autónomos sin NP. Resultados: Los valores medios ± DE de citrulina plasmática fueron: grupo I (n = 15): 7,1 ± 4,1; grupo II (n = 11): 15,8 ± 8,9; grupo III (n = 13): 20,6 ± 7,5; grupo IV (n = 25): 28,8 ± 10,1. Los valores resultaron inferiores en el grupo I comparados con los grupos II - III - IV (p < 0,001) y en el grupo II comparados con los grupos III - IV (p < 0,001). Se observó una fuerte correlación entre la citrulinemia y la longitud del intestino delgado remanente (r = 0,85; p < 0,001). En el grupo IV la citrulina descendió > 50% coincidiendo con rechazo moderado-severo en 3 pacientes y con enteritis viral en un paciente. Conclusiones: 1. La citrulina plasmática puede ser un biomarcador sensible y específico del intestino funcional residual. 2. Se relaciona con la tolerancia a la alimentación enteral. 3. Debe confirmarse su valor pronóstico en el proceso de adaptación intestinal y como marcador de rechazo en pacientes trasplantados (AU)

Introduction: Citrulline is a non-essential amino acid produced solely in the enterocyte. The aim of this study was to analyse the role of serum citrulline as a biomarker of enterocyte load in children with intestinal failure due to short bowel syndrome (SBS) and its relationship to enteral adaptation. Material and methods: Plasma citrulline concentration was determined by chromatography (normal value > 15 mol/L) in 57 patients (age 0.5-18 years) admitted to our Intestinal Rehabilitation Unit with intestinal failure. Those who were dehydrated, with renal insufficiency, or other conditions able to modify the results were excluded. Patients were divided into 4 groups: group I: SBS totally dependent on parenteral nutrition (PN); group II: SBS under mixed enteral parenteral nutrition; group III: IF weaned from PN after a rehabilitation period; group IV: small bowel transplanted patients weaned from PN and taking a normal diet. Results: The mean ± SD plasma citrulline values were: group I (n = 15): 7.1±4.1; group II (n = 11): 15.8±8.9; group III (n = 13): 20.6±7.5; group IV (n = 25): 28.8±10.1. Values were significantly lower in group I in comparison with groups II-III-IV (P <0 .001), and in group II in comparison with groups III-IV (P < 0.001). A low citrulline was associated with remnant small bowel length (P < 0.001, r = 0.85). In group IV citrulline levels decreased > 50% in 3 patients who developed moderates ever e rejection, and in one patient who developed viral enteritis. Conclusions: 1. Plasma citrulline could be a sensitive and specific biomarker of the residual functional enterocyte load. 2. It is related to enteral feeding tolerance. 3. Its prognostic value in the process of intestinal adaptation and as a rejection marker in small bowel transplanted patients needs to be confirmed (AU)

[Plasma citrulline concentration as a biomarker of intestinal function in short bowel syndrome and in intestinal transplant]. / Valor de la citrulina plasmática como biomarcador de función intestinal en el síndrome de intestino corto y en el trasplante intestinal.

INTRODUCTION: Citrulline is a non-essential amino acid produced solely in the enterocyte. The aim of this study was to analyse the role of serum citrulline as a biomarker of enterocyte load in children with intestinal failure due to short bowel syndrome (SBS) and its relationship to enteral adaptation. MATERIAL AND METHODS: Plasma citrulline concentration was determined by chromatography (normal value>15 µmol/L) in 57 patients (age 0.5-18 years) admitted to our Intestinal Rehabilitation Unit with intestinal failure. Those who were dehydrated, with renal insufficiency, or other conditions able to modify the results were excluded. Patients were divided into 4 groups: group i: SBS totally dependent on parenteral nutrition (PN); group ii: SBS under mixed enteral-parenteral nutrition; group iii: IF weaned from PN after a rehabilitation period; group iv: small bowel transplanted patients weaned from PN and taking a normal diet. RESULTS: The mean ± SD plasma citrulline values were: group i (n=15): 7.1 ± 4.1; group ii (n=11): 15.8 ± 8.9; group iii (n=13): 20.6 ± 7.5; group iv (n=25): 28.8 ± 10.1. Values were significantly lower in group i in comparison with groups ii-iii-iv (P<.001), and in group ii in comparison with groups iii-iv (P<.001). A low citrulline was associated with remnant small bowel length (P<.001, r=0.85). In group iv citrulline levels decreased >50% in 3 patients who developed moderate-severe rejection, and in one patient who developed viral enteritis. CONCLUSIONS: 1. Plasma citrulline could be a sensitive and specific biomarker of the residual functional enterocyte load. 2. It is related to enteral feeding tolerance. 3. Its prognostic value in the process of intestinal adaptation and as a rejection marker in small bowel transplanted patients needs to be confirmed.

Enuresis asociada a crisis de ausencia / Enuresis associated with petit mal seizures

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