Infectious Complications Following Small Bowel Transplantation.

Microbiological spectrum and outcome of infectious complications following small bowel transplantation (SBT) have not been thoroughly characterized. We performed a retrospective analysis of all patients undergoing SBT from 2004 to 2013 in Spain. Sixty-nine patients underwent a total of 87 SBT procedures (65 pediatric, 22 adult). The median follow-up was 867 days. Overall, 81 transplant patients (93.1%) developed 263 episodes of infection (incidence rate: 2.81 episodes per 1000 transplant-days), with no significant differences between adult and pediatric populations. Most infections were bacterial (47.5%). Despite universal prophylaxis, 22 transplant patients (25.3%) developed cytomegalovirus disease, mainly in the form of enteritis. Specifically, 54 episodes of opportunistic infection (OI) occurred in 35 transplant patients. Infection was the major cause of mortality (17 of 24 deaths). Multivariate analysis identified retransplantation (hazard ratio [HR]: 2.21; 95% confidence interval [CI]: 1.02-4.80; p = 0.046) and posttransplant renal replacement therapy (RRT; HR: 4.19; 95% CI: 1.40-12.60; p = 0.011) as risk factors for OI. RRT was also a risk factor for invasive fungal disease (IFD; HR: 24.90; 95% CI: 5.35-115.91; p < 0.001). In conclusion, infection is the most frequent complication and the leading cause of death following SBT. Posttransplant RRT and retransplantation identify those recipients at high risk for developing OI and IFD.

Fracaso intestinal y trasplante en la enfermedad por inclusiones microvellositarias / Intestinal failure and transplantation in microvillous inclusion disease

INTRODUCCIÓN: La enfermedad por inclusiones microvellositarias es una entidad rara, de herencia autosómica recesiva y caracterizada por una diarrea grave de carácter secretor que produce un fracaso intestinal permanente dependiente de nutrición parenteral. Habitualmente se inicia en el período neonatal y el único tratamiento posible en el momento actual es el trasplante intestinal. PACIENTES Y MÉTODOS: Se revisa, de forma retrospectiva, a 6 pacientes (3 varones y 3 mujeres), diagnosticados entre 1998 y 2013 de enfermedad por inclusiones microvellositarias. RESULTADOS: Todos comenzaron en el primer mes de vida, con una mediana de edad de tres días (rango: 3-30 días) y presentaron diarrea secretora dependiente de nutrición parenteral, con un volumen fecal en ayunas de 150-200ml/kg/día. La microscopia óptica de muestras biópsicas duodenales mostró grados variables de atrofia vellositaria sin hiperplasia críptica, con acumulación de material PAS positivo en el citoplasma de los enterocitos del borde en cepillo y la inmunotinción anti-CD10 fue indicativa de inclusiones intracitoplasmáticas. La confirmación diagnóstica se realizó con microscopia electrónica. En 2 de ellos se realizó estudio genético que demostró mutaciones en el gen MYO5B. Evolutivamente, 3 fallecieron y 3 se encuentran vivos; 2 de ellos portadores de trasplante intestinal y el tercero en espera de trasplante multivisceral

INTRODUCTION: Microvillous inclusion disease is a rare autosomal recessive condition, characterized by severe secretory diarrhea that produces a permanent intestinal failure and dependency on parenteral nutrition. It usually begins in the neonatal period, and the only treatment at present is intestinal transplantation. PATIENTS AND METHODS: A retrospective review was conducted on 6 patients (three males and three females) diagnosed with microvillous inclusion disease between 1998 and 2013. RESULTS: All debuted in the first month of life, with a median age of three days (range, 3-30 days), and had secretory diarrhea dependent on parenteral nutrition, with fasting fecal volume of 150-200ml/kg/day. Light microscopy of duodenal biopsy samples showed varying degrees of villous atrophy without cryptic hyperplasia, accumulation of PAS positive material in the cytoplasm of enterocytes brush border, and anti-CD10 immunostaining was suggestive of intracytoplasmic inclusions. Diagnostic confirmation was performed with electron microscopy. Two of them had a genetic study, and showed mutations in MYO5B gene. Three died and three are alive; two of them with an intestinal transplantation and the third waiting for a multivisceral transplantation

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.

[Intestinal failure and transplantation in microvillous inclusion disease]. / Fracaso intestinal y trasplante en la enfermedad por inclusiones microvellositarias.

INTRODUCTION: Microvillous inclusion disease is a rare autosomal recessive condition, characterized by severe secretory diarrhea that produces a permanent intestinal failure and dependency on parenteral nutrition. It usually begins in the neonatal period, and the only treatment at present is intestinal transplantation. PATIENTS AND METHODS: A retrospective review was conducted on 6 patients (three males and three females) diagnosed with microvillous inclusion disease between 1998 and 2013. RESULTS: All debuted in the first month of life, with a median age of three days (range, 3-30 days), and had secretory diarrhea dependent on parenteral nutrition, with fasting fecal volume of 150-200ml/kg/day. Light microscopy of duodenal biopsy samples showed varying degrees of villous atrophy without cryptic hyperplasia, accumulation of PAS positive material in the cytoplasm of enterocytes brush border, and anti-CD10 immunostaining was suggestive of intracytoplasmic inclusions. Diagnostic confirmation was performed with electron microscopy. Two of them had a genetic study, and showed mutations in MYO5B gene. Three died and three are alive; two of them with an intestinal transplantation and the third waiting for a multivisceral transplantation.

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.

Infección por citomegalovirus como causa de diarrea grave rebelde en un lactante inmunocompetente / Cytomegalovirus infection causing protracted diarrhea in an immunocompetent child

La infección por citomegalovirus (CMV) es una causa frecuente de complicaciones en pacientes inmunodeprimidos. En huéspedes sanos, la infección por CMV cursa generalmente de forma asintomática. A continuación se presenta el caso de un paciente de 3 meses de edad inmunocompetente, en el que se desarrolló un cuadro de diarrea grave que precisó nutrición parenteral. El cuadro se resolvió sin secuelas tras tratamiento con ganciclovir (AU)

Cytomegalovirus infection is a frequent cause of complications in immunodeficient patients. In healthy hosts, cytomegalovirus infection is usually asymptomatic. We describe a case of a 3 month old immunocompetent boy who suffered from protracted diarrhoea that required parenteral nutrition. After treatment with ganciclovir he recovered without any after effects (AU)

[Cytomegalovirus infection causing protracted diarrhea in an immunocompetent child]. / Infección por citomegalovirus como causa de diarrea grave rebelde en un lactante inmunocompetente.

Cytomegalovirus infection is a frequent cause of complications in immunodeficient patients. In healthy hosts, cytomegalovirus infection is usually asymptomatic. We describe a case of a 3 month old immunocompetent boy who suffered from protracted diarrhoea that required parenteral nutrition. After treatment with ganciclovir he recovered without any after effects.

Endoscopia digestiva diagnóstica / Diagnostic digestive endoscopy

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Síndrome de megavejiga-microcolon-hipoperistaltismo intestinal. A propósito de un caso de supervivencia prolongada / Megacystis-microcolon-intestinal hypoperistalsis syndrome: a propos of a case of prolonged survival

No disponible

No disponible

[Cystic fibrosis: nutritional considerations]. / Fibrosis quística: aspectos nutricionales.

During the last few decades, improved treatment measures and nutritional support in cystic fibrosis have increased survival and quality of life in these patients. There is a clear relationship between the development of malnutrition and worsening in respiratory function and both factors have been related to poor disease outcome. Malnutrition is a very frequent complication of this disease and is found in 20% of patients, due to negative energy-proteic balance. This disequilibrium can be explained by increased energy expenditure, high nutritional requirements and decreased oral intake. Periodic monitoring of clinical, anthropometrical and functional nutritional status is mandatory. Intake must be greater than requirements and specific nutritional support should be established when required. Patients with cystic fibrosis must receive a hypercaloric and hyperproteotic diet, with a high fat content, a normal quantity of carbohydrates and with pancreatic and liposoluble vitamin supplements in case of pancreatic insufficiency.

Fibrosis quística: aspectos nutricionales / Cystic fibrosis: nutritional considerations

La mejora durante las últimas décadas de las técnicas de tratamiento y de soporte nutricional de los pacientes con fibrosis quística ha permitido prolongar la supervivencia y aumentar la calidad de vida de estos pacientes. Existe una clara relación entre el desarrollo de la malnutrición y la disminución de los parámetros de función respiratoria y ambos se asocian con un peor pronóstico de la enfermedad. La malnutrición es una complicación muy frecuente, presente al menos en el 20% de los pacientes. Esto se debe a que, con frecuencia, se establece un balance energético y proteico negativo originado por el aumento del gasto energético y el aumento de las necesidades nutricionales junto con una disminución de los aportes ingeridos. Debe monitorizarse de forma periódica el estado nutricional desde el punto de vista clínico, antropométrico y analítico, asegurando que la ingesta alcance los requerimientos y realizando un soporte nutricional más específico en aquellas situaciones en las que se produzca un fracaso nutricional. La dieta del paciente con fibrosis quística debe ser hipercalórica e hiperproteica, rica en grasa, con aporte normal de hidratos de carbono y con aporte de suplementos pancreáticos y de vitaminas liposolubles cuando exista insuficiencia pancreática (AU)

[Diagnosis and therapeutic options in chronic idiopathic intestinal pseudo-obstruction: review of 16 cases]. / Diagnóstico y opciones terapéuticas en la pseudoobstrucción intestinal idiopática crónica: revisión de 16 casos.

Chronic idiopathic intestinal pseudo-obstruction (CIPS) is a rare condition in which there is a defective motility of the gastrointestinal tract of unknown cause leading to repeated bouts of intestinal obstruction without organic explanation. This syndrome groups several ill-defined varieties of motor disorders that can sometimes be classified according to the presence of familial incidence and to the presence of muscular or nervous lesions. Nevertheless, a considerable proportion of cases cannot be ascribed to either type. CIPS is a very difficult challenge for pediatric surgeons because our role is never curative and because when we are involved in it is usually as a result of a false diagnosis. We present herein the experience of 2 Pediatric Surgery Departments in this entity. In the last 30 years we have been involved in the management of 16 children with CIPS. Male-to-female ratio was 5:11 and all but 3 patients had symptoms before 6 months of life. Thirteen had abdominal distension, 10 maldevelopment, 9 recurrent bouts of intestinal obstruction, 8 chronic diarrhea, 7 vomiting, 2 dysphagia and 2 constipation. Seven out of the 16 had urinary tract involvement and in three prenatal diagnosis of megacysts was made. The mean delayed time for diagnosis was 3.08 years. Esophageal or antroduodenal manometry was performed in 8 patients and it was abnormal in 7. Histologic and histochemical samples were available in 8 patients, but only in 4 was enough to make a diagnosis of myopathy. Twelve patients underwent 41 surgical procedures. Three are currently included in a program of home parenteral nutrition. Only three have died, and the mean age of the survivors is 13.9 years. In most of the patients with CIPS surgery is only useful for nutritional purposes, for diversion procedures or for intestinal transplantation in extreme cases. Every effort should be made to avoid unnecessary explorations, misdiagnosis and delay in the identification of the syndrome.

[Colitis induced by a food allergen. A report of 20 cases]. / Colitis inducida por alergeno alimentario. Presentación de 20 casos.

In the last years, we have appreciated a descending number of classic enteral food-protein intolerance and cases of colitis related to food proteins are increasing. In this study, 20 cases of food protein colitis (mainly related to cow's milk) are reviewed and clinical, endoscopic and histological aspects are analyzed. We found that the age at the time of the first consultation was approximately 1.5 months, with males predominating. The main symptom was bloody diarrhoea. Other causes such as perianal fissure or infectious gastroenteritis were excluded. The infants were fed breast milk and/or cow's milk formula. Some patients had low hemoglobin, but low serum albumin was infrequent. The colonoscopy showed erythema and, in some cases, loss of vascular pattern, ulcerations or bleeding. The main histological finding was an increased number of eosinophils. There was a favorable response to the dietary change. After 6 months, all of the provocations done were negative. We conclude that food-protein intolerance is one of the most probable causes of bloody stools in a young infant. Colonoscopy and a histopathological study of at least three biopsy pieces are necessary for the diagnosis. The treatment is the exclusion of the offending protein from the diet.