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4.
Cir Pediatr ; 35(3): 146-148, 2022 Jul 01.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-35796088

RESUMO

INTRODUCTION: Perineal groove is an infrequent midline malformation. It is a humid, mucosal, non-keratinized groove located at the perineal midline, extending from the vulvar fourchette to the anterior anal border. It is rare and usually asymptomatic, and it heals spontaneously in most cases. It is frequently mistaken for other malformations, which means correctly identifying it is essential to avoid iatrogenesis. CLINICAL CASE: We present the case of a female newborn with an asymptomatic lesion at the perineal midline consistent with anorectal malformation. Following assessment by the Pediatric Surgery Department, she was diagnosed with perineal groove. DISCUSSION: Perineal groove is a little known malformation among healthcare professionals as it is infrequent and there are not many publications in the literature about it. This case demonstrates how important it is to keep this abnormality in mind to avoid erroneous diagnoses, unnecessary treatments, and family stress.


INTRODUCCION: El surco perineal es una malformación infrecuente de la línea media. Se trata de un surco húmedo, mucoso y no queratinizado localizado en la línea media del periné desde la horquilla vulvar hasta el borde anal anterior. Es una malformación infrecuente, usualmente asintomática y de resolución espontánea en la mayoría de los casos. Esta anomalía es frecuentemente confundida con otras malformaciones por lo que su reconocimiento es fundamental para evitar yatrogenia. CASO CLINICO: Se presenta el caso de una recién nacida con una lesión asintomática en la línea media del periné sospechosa de malformación anorrectal. Tras valoración por el Servicio de Cirugía Pediátrica se diagnosticó de surco perineal. COMENTARIOS: Debido a la infrecuencia y escasa documentación bibliográfica del surco perineal, esta malformación es desconocida para muchos sanitarios. Este caso expone la importancia de tener presente esta anomalía para evitar diagnósticos erróneos, tratamientos innecesarios y estrés familiar.


Assuntos
Canal Anal , Períneo , Criança , Feminino , Humanos , Recém-Nascido
5.
Cir. pediátr ; 35(3): 146-148, Jul 2022. ilus
Artigo em Espanhol | IBECS | ID: ibc-206105

RESUMO

Introducción: El surco perineal es una malformación infrecuente dela línea media. Se trata de un surco húmedo, mucoso y no queratinizado localizado en la línea media del periné desde la horquilla vulvar hastael borde anal anterior. Es una malformación infrecuente, usualmente asintomática y de resolución espontánea en la mayoría de los casos. Esta anomalía es frecuentemente confundida con otras malformaciones por lo que su reconocimiento es fundamental para evitar yatrogenia. Caso clínico: Se presenta el caso de una recién nacida con una lesión asintomática en la línea media del periné sospechosa de malformación anorrectal. Tras valoración por el Servicio de Cirugía Pediátrica se diagnosticó de surco perineal. Comentarios: Debido a la infrecuencia y escasa documentación bibliográfica del surco perineal, esta malformación es desconocida paramuchos sanitarios. Este caso expone la importancia de tener presente estaanomalía para evitar diagnósticos erróneos, tratamientos innecesarios y estrés familiar.(AU)


Introduction: Perineal groove is an infrequent midline malformation. It is a humid, mucosal, non keratinized groove located at theperineal midline, extending from the vulvar fourchette to the anterioranal border. It is rare and usually asymptomatic, and it heals spontane-ously in most cases. It is frequently mistaken for other malformations,which means correctly identifying it is essential to avoid iatrogenesis. Clinical case: We present the case of a female newborn with anasymptomatic lesion at the perineal midline consistent with anorectal malformation. Following assessment by the Pediatric Surgery Department, she was diagnosed with perineal groove. Discussion: Perineal groove is a little known malformation amonghealthcare professionals as it is infrequent and there are not many publications in the literature about it. This case demonstrates how importantit is to keep this abnormality in mind to avoid erroneous diagnoses, unnecessary treatments, and family stress.(AU)


Assuntos
Humanos , Feminino , Recém-Nascido , Períneo/lesões , Malformações Anorretais , Períneo/anormalidades , Períneo/diagnóstico por imagem , Erros de Diagnóstico , Anormalidades Congênitas/prevenção & controle , Saúde da Criança , Pediatria , Cirurgia Geral
6.
Cir Pediatr ; 34(1): 43-46, 2021 Jan 01.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-33507644

RESUMO

INTRODUCTION: Enterocutaneous fistula treatment in patients undergoing multiple surgeries is complex and requires creative solutions. We present the case of an enterocutaneous fistula managed with laser diode and cyanoacrylates. CLINICAL CASE: 15-year-old patient, diagnosed with ulcerative colitis at 12 years of age, undergoing full colectomy with urgent ileostomy as a result of a flare-up refractory to medical treatment. Five months later, an ileoanal pull-through with pouch was carried out, leaving a protection ileostomy in place. However, postoperative evolution was poor, with pelvic infection, so two further urgent open surgeries were required for lavage and hemostasis purposes. Six months later, anastomotic stricture was noted. It was healed following various pneumatic dilations under ultrasound vision and at-home dilations using Hegar dilators. One year following this, ileostomy was closed, but one month later, abdominal distension occurred. It was associated with a fistula in the abdominal midline, which could be endoscopically guided, with its origin being located at the ileoanal anastomosis. Laser diode sessions were applied for treatment purposes, with partial improvement, but still with gas emission. One year later, embolization was performed by placing platinum coils and lipiodol-diluted cyanoacrylates, and clinical signs disappeared. 17 months following this surgery, the patient has no symptoms, with full day and night fecal continence and 3 daily stools, and the fistula is completely closed. CONCLUSION: Combined treatment with laser diode and platinum coil and cyanoacrylate embolization proves effective in the management of enterocutaneous fistula, with low morbidity.


INTRODUCCION: El tratamiento de las fístulas enterocutáneas establecidas en pacientes multioperados es complejo y requiere de soluciones creativas. Presentamos la resolución de un caso con láser diodo y con cianoacrilatos. CASO CLINICO: Paciente de 15 años, diagnosticado a los 12 de colitis ulcerosa y sometido a pancolectomía con ileostomía urgente por brote refractario al tratamiento médico. Cinco meses después se realizó un descenso ileoanal con reservorio dejando ileostomía de protección, pero presentó un posoperatorio tórpido con infección pélvica, precisando dos nuevas laparotomías urgentes, para lavado y hemostasia. Seis meses después, se evidenció una estenosis de la anastomosis que se resolvió tras varias dilataciones, neumáticas bajo visión endoscópica y domiciliarias con tallos de Hegar. Un año después se cerró la ileostomía, presentando al mes una distensión abdominal y aparición de fístula en línea media abdominal, que pudo tutorizarse endoscópicamente, observando su origen en la anastomosis ileoanal. Se trató mediante sesiones de láser diodo, con mejoría parcial, aunque persistía la salida de gases. Un año después se embolizó implantando espirales de platino y cianoacrilatos diluidos con lipiodol, resolviéndose totalmente el cuadro. Transcurridos 17 meses de esta última intervención, el paciente se encuentra asintomático, con continencia fecal total diurna y nocturna, tres deposiciones al día y la fístula está totalmente cerrada. CONCLUSION: El tratamiento con láser diodo, combinado con embolización con espirales de platino y cianoacrilatos, puede ser un tratamiento eficaz con baja morbilidad de las fístulas enterocutáneas establecidas.


Assuntos
Fístula Intestinal , Complicações Pós-Operatórias , Adolescente , Colectomia , Humanos , Ileostomia , Fístula Intestinal/etiologia , Fístula Intestinal/terapia , Lasers , Complicações Pós-Operatórias/cirurgia
7.
Cir Pediatr ; 30(1): 22-27, 2017 Jan 25.
Artigo em Espanhol | MEDLINE | ID: mdl-28585786

RESUMO

BACKGROUND: Extrahepatic portal vein obstruction (EPVO) is the principal cause of portal hypertension in children. The objective of this study was to analyze the capacity of the surgical technique that creates a mesoportal shunt to treat changes caused by EPVO. METHODS: Retrospective review of patients with idiopathic EPVO who underwent a mesoportal shunt and analysis of the changes in the number of leucocytes, platelets, prothrombin time and spleen size one year after the surgery. RESULTS: Twelve patients underwent surgery, out of which 10 had prior leukopenia, 11 thrombopenia, 9 longer prothrombin times and all had hypersplenism. One patient suffered a postoperative shunt thrombosis, was reoperated and underwent a change in the operative technique. The remaining patients (92%) have functioning shunts 4.3 ± 2.5 years after surgery, and none have suffered any episode of gastrointestinal bleeding. One year after surgery, there were significant changes in the number of platelets, prothrombin time and spleen size, with no significant changes in the number of leukocytes. However, the number of patients who went from a leukopenic to a normal state was significant, as happened with changes in prothrombin time. CONCLUSIONS: Mesoportal Rex shunt improves some of the disorders caused by portal hypertension in children suffering EPVO, with a high rate of surgical success. This technique should be of first choice in these patients.


OBJETIVOS: La trombosis portal extrahepática (TPEH) es la causa más frecuente de hipertensión portal en el niño. El objetivo de este estudio es analizar la utilidad de la técnica quirúrgica que crea un shunt mesoportal para mejorar o revertir las alteraciones causadas por la TPEH. MATERIAL Y METODOS: Revisión retrospectiva de los pacientes con TPEH idiopática sometidos al shunt mesoportal y análisis de los cambios en la cifra de leucocitos, plaquetas, tiempo de protrombina y tamaño del bazo al año de la cirugía. RESULTADOS: De los 12 pacientes intervenidos, 10 tenían leucopenia, 11 plaquetopenia, 9 un tiempo de protrombina alargado y todos presentaban hiperesplenismo. Una paciente sufrió una trombosis postoperatoria del shunt por la que fue reoperada con cambio de la técnica quirúrgica. El resto de pacientes (92%) tienen un shunt funcionante con un seguimiento 4,3 ± 2,5 años y no han sufrido ningún sangrado gastrointestinal. Al año de la cirugía, observamos cambios significativos en el número total de plaquetas, tiempo de protrombina y tamaño del bazo, no siendo estos cambios significativos para la cifra de leucocitos. Sin embargo, el número de pacientes que pasó de estar leucopénico a tener cifras normales de leucocitos, sí fue significativo, hecho que también ocurrió en el caso de las alteraciones en el tiempo de protrombina. CONCLUSIONES: El shunt mesoportal de Rex mejora de manera efectiva las alteraciones asociadas a la hipertensión portal por TPEH, con una alta tasa de éxito, por lo que debería ser la técnica de elección en estos pacientes.


Assuntos
Hipertensão Portal/cirurgia , Veia Porta/fisiopatologia , Derivação Portossistêmica Cirúrgica/métodos , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Hipertensão Portal/etiologia , Masculino , Veia Porta/cirurgia , Complicações Pós-Operatórias/epidemiologia , Estudos Retrospectivos , Resultado do Tratamento
8.
Cir. pediátr ; 30(1): 22-27, ene. 2017. tab, graf
Artigo em Espanhol | IBECS | ID: ibc-163329

RESUMO

Objetivos. La trombosis portal extrahepática (TPEH) es la causa más frecuente de hipertensión portal en el niño. El objetivo de este estudio es analizar la utilidad de la técnica quirúrgica que crea un shunt mesoportal para mejorar o revertir las alteraciones causadas por la TPEH. Material y métodos. Revisión retrospectiva de los pacientes con TPEH idiopática sometidos al shunt mesoportal y análisis de los cambios en la cifra de leucocitos, plaquetas, tiempo de protrombina y tamaño del bazo al año de la cirugía. Resultados. De los 12 pacientes intervenidos, 10 tenían leucopenia, 11 plaquetopenia, 9 un tiempo de protrombina alargado y todos presentaban hiperesplenismo. Una paciente sufrió una trombosis postoperatoria del shunt por la que fue reoperada con cambio de la técnica quirúrgica. El resto de pacientes (92%) tienen un shunt funcionante con un seguimiento 4,3 ± 2,5 años y no han sufrido ningún sangrado gastrointestinal. Al año de la cirugía, observamos cambios significativos en el número total de plaquetas, tiempo de protrombina y tamaño del bazo, no siendo estos cambios significativos para la cifra de leucocitos. Sin embargo, el número de pacientes que pasó de estar leucopénico a tener cifras normales de leucocitos, sí fue significativo, hecho que también ocurrió en el caso de las alteraciones en el tiempo de protrombina. Conclusiones. El shunt mesoportal de Rex mejora de manera efectiva las alteraciones asociadas a la hipertensión portal por TPEH, con una alta tasa de éxito, por lo que debería ser la técnica de elección en estos pacientes (AU)


Background. Extrahepatic portal vein obstruction (EPVO) is the principal cause of portal hypertension in children. The objective of this study was to analyze the capacity of the surgical technique that creates a mesoportal shunt to treat changes caused by EPVO. Methods. Retrospective review of patients with idiopathic EPVO who underwent a mesoportal shunt and analysis of the changes in the number of leucocytes, platelets, prothrombin time and spleen size one year after the surgery. Results. Twelve patients underwent surgery, out of which 10 had prior leukopenia, 11 thrombopenia, 9 longer prothrombin times and all had hypersplenism. One patient suffered a postoperative shunt thrombosis, was reoperated and underwent a change in the operative technique. The remaining patients (92%) have functioning shunts 4.3 ± 2.5 years after surgery, and none have suffered any episode of gastrointestinal bleeding. One year after surgery, there were significant changes in the number of platelets, prothrombin time and spleen size, with no significant changes in the number of leukocytes. However, the number of patients who went from a leukopenic to a normal state was significant, as happened with changes in prothrombin time. Conclusions. Mesoportal Rex shunt improves some of the disorders caused by portal hypertension in children suffering EPVO, with a high rate of surgical success. This technique should be of first choice in these patients (AU)


Assuntos
Humanos , Masculino , Feminino , Criança , Adolescente , Trombose Venosa/cirurgia , Hipertensão Portal/fisiopatologia , Derivação Portossistêmica Cirúrgica/métodos , Veia Porta/fisiopatologia , Leucopenia/complicações , Transtornos Plaquetários/complicações , Estudos Retrospectivos
9.
Cir Pediatr ; 29(2): 72-76, 2016 Apr 10.
Artigo em Espanhol | MEDLINE | ID: mdl-28139106

RESUMO

Patients with recto vestibular fistula may have gynecological malformations that could be unnoticed at the initial examination. The aim of this paper is to demonstrate the incidence of these malformations and propose a study method to help diagnose these malformations, avoiding unnecessary surgeries. We reviewed the records of patients treated with rectovaginal fistula (RVF) in the last 18 years and studied their gynecological malformations, time at diagnosis and treatment received. Of the 39 patients treated, 5 of them (13.1%) demonstrated 9 gynecological malformations: Hemivaginas (2), hemiuteros (2), uterine agenesis (2), vaginal agenesis (2) and vaginal septum (1). The diagnosis was made after the posterior sagittal approach (PSA) in two patients (acute abdomen and hydrometrocolpos), during the PSA in 2 patients and only one of them was diagnosed before the PSA. The 2 patients with hemivaginas and hemiuterus underwent a hemihysterosalpinguectomy and a vaginoplasty later in adolescence. The patient with vaginal and uterine agenesis diagnosed prior to PSA underwent a posterior sagittal anorectoplasty and a vaginoplasty with colon in the same procedure. The patient with vaginal and uterine agenesis (age 13 months) is waiting for vaginal replacement. The patient with vaginal septum (intraoperative finding) underwent a septum resection during the PSA. Gynecological defects are part of RVF spectrum. Girls with RVF require a complete gynecological examination prior to the definitive repair. Preoperative examinations assist in the timing and type of repair, and ultimately avoids complications and unnecessary interventions.


La fístula recto-vestibular se puede asociar a anomalías ginecológicas que, en numerosas ocasiones, pasan inadvertidas en la exploración inicial. Su reconocimiento en el momento adecuado puede cambiar el plan terapéutico y el pronóstico. Se revisan las historias de las pacientes con fístula recto-vestibular de los últimos 18 años y se describen las malformaciones ginecológicas asociadas, el momento del diagnóstico, el tratamiento y resultado postoperatorio. De 39 pacientes, en 5 (12,8%) se observaron 12 malformaciones ginecológicas: hemivaginas (2), hemiúteros (2), agenesia uterina (2), agenesia vaginal (2) y tabique vaginal (3). En dos pacientes el diagnóstico se realizó durante la anorrectoplastia, en otras dos después de la anorrectoplastia sagital posterior a causa de hidrometrocolpos y solo en una de ellas, antes de la intervención. En las pacientes con hemivagina y hemiútero se realizó, en una, la extirpación del hemiútero y la trompa y, en la otra, plastia vaginal transformándola en una única vagina. Cuando el diagnóstico se hizo antes de la intervención se pudo planificar la plastia adecuadamente. En la paciente con tabique vaginal y hallazgo intraoperatorio la anomalía genital fue tratada durante la anorrectoplastia. La paciente con agenesia vaginal y uterina aún no ha sido intervenida. Las malformaciones ginecológicas se asocian con relativa frecuencia a la fístula recto-vestibular. Por ello se requiere una exploración ginecológica adecuada antes de la anorrectoplastia para poder planificar de manera correcta el momento de la reparación, evitando complicaciones e intervenciones innecesarias. intraútero, la invaginación intestinal postnatal en el prematuro y la invaginación intestinal postnatal en el neonato a término.


Assuntos
Fístula Retovaginal/cirurgia , Útero/anormalidades , Vagina/anormalidades , Adolescente , Feminino , Humanos , Lactente , Fístula Retovaginal/etiologia , Útero/cirurgia , Vagina/cirurgia
10.
Cir Pediatr ; 29(4): 166-170, 2016 Oct 10.
Artigo em Espanhol | MEDLINE | ID: mdl-28481070

RESUMO

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.


Assuntos
Fístula Cutânea/terapia , Fístula Intestinal/terapia , Tratamento de Ferimentos com Pressão Negativa/métodos , Complicações Pós-Operatórias/terapia , Criança , Fístula Cutânea/etiologia , Humanos , Fístula Intestinal/etiologia , Laparotomia , Estudos Retrospectivos
11.
An. pediatr. (2003, Ed. impr.) ; 79(4): 218-223, oct. 2013. graf
Artigo em Espanhol | IBECS | ID: ibc-116358

RESUMO

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)


Assuntos
Humanos , Masculino , Feminino , Lactente , Citrulina/sangue , Síndrome do Intestino Curto/fisiopatologia , Intestinos/transplante , Biomarcadores/análise , Enterócitos/fisiologia
12.
An Pediatr (Barc) ; 79(4): 218-23, 2013 Oct.
Artigo em Espanhol | MEDLINE | ID: mdl-23528708

RESUMO

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.


Assuntos
Citrulina/sangue , Intestinos/fisiopatologia , Intestinos/transplante , Complicações Pós-Operatórias/sangue , Complicações Pós-Operatórias/fisiopatologia , Síndrome do Intestino Curto/sangue , Síndrome do Intestino Curto/fisiopatologia , Adolescente , Biomarcadores/sangue , Criança , Pré-Escolar , Enterócitos/fisiologia , Feminino , Humanos , Lactente , Masculino
13.
Cir. pediátr ; 23(3): 177-183, jul. 2010. ilus, tab
Artigo em Espanhol | IBECS | ID: ibc-107269

RESUMO

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


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


Assuntos
Humanos , Masculino , Feminino , Criança , Síndrome do Intestino Curto/cirurgia , Intestinos/transplante , Enteropatias/cirurgia , Tacrolimo/uso terapêutico , Sirolimo/uso terapêutico , Nutrição Parenteral
14.
Cir Pediatr ; 23(3): 177-83, 2010 Jul.
Artigo em Espanhol | MEDLINE | ID: mdl-23155666

RESUMO

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.


Assuntos
Enteropatias/cirurgia , Intestino Delgado/transplante , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Adulto Jovem
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