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.

[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.

[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.

[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.

[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.

[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.

[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.

[Nodular antritis and Helicobacter pylori infection in children]. / Antritis nodular e infección por Helicobacter pylori en el niño.

We have studied 115 patients infected with Helicobacter pylori (HP). Endoscopic nodular antritis and histologic lymphonodular hyperplasia was noted. The frequency of nodular antritis was compared between the 115 HP patients and a HP-negative age-matched control group. Nodular antritis was observed in 70.4% of the HP-positive patients and 11.3% of the HP-negative group (p <0.001). The specificity of nodular antritis was 86.1%. A significant association was found between the existence of nodular antritis and histologic lymphonodular hyperplasia (67.9%; p < 0.001). In conclusion, the endoscopic nodular antritis is a frequent and specific finding in children with HP infection and is associated with histologic lymphonodular hyperplasia.

[Histological lesions and Helicobacter pylori colonization in children]. / Lesión histológica y colonización por Helicobacter pylori en el niño.

We have assessed the relationship between histologic findings and the Helicobacter pylori (HP) colonization in 115 patients (age: 9.8 +/- 3.5 years) with HP infection. Normal antral histology was found in 8.7% of the patients. Signs of histological activity were observed in 58.2% of the patients and glandular lesions in 3.5%. A significantly greater HP colonization was observed in patients with signs of histological activity (p < 0.001). A significant correlation was found between the HP colonization score and histological score (rs = 0.62) with a significant association between the degree of HP colonization and the histologic categories (p < 0.001).

[Colloidal bismuth subcitrate and amoxicillin in the treatment of Helicobacter pylori infection in children]. / Tratamiento con subcitrato de bismuto coloidal más amoxicilina en la infección por Helicobacter pylori del niño.

We have analyzed the effect of treatment with colloidal bismuth subcitrate (CBS) and amoxicillin in a series of 60 children (age range: 2.9-18 years) infected by Helicobacter pylori (HP). The following results were found: clinical remission 70%; endoscopic normalization 55%; histological normalization 61.6% and HP eradication 53.3%. The improvement of histological activity (100% vs 11.7%) and histological normalization (90.6% vs 28.5%) were significantly more frequent in the HP eradicated group as compared with the persistent HP group (p < 0.001). The present study suggests that combined therapy with CBS and amoxicillin is not the most adequate treatment to eradicate HP in children and supports the role played by HP in the development of antral histologic lesions.

[Inflammatory disease of intestine in the child]. / Enfermedad inflamatoria intestinal en el niño.

We review 28 patients with IBD (14 UC and 14 CD) diagnosed in a period of eight years (1982-1990). The age at the onset of symptoms was similar in both groups (8.1-8.7 years), but the delay in diagnosis was significantly higher in CD (16.2 months). Fever, growth retardation and oral and perianal lesions were more frequent in CD. Articular, mucocutaneous and hepatic were the more usual extraintestinal manifestations. All of them were more frequent in CD. The same comment can be made with respect to the abnormal laboratory test results. Colonoscopy including histological studies was the useful diagnostic method. An increase of the incidence of IBD it has been observed.

[Primary peptic ulcer in infancy and childhood. Personal experience]. / Ulcera péptica primaria en la infancia. Nuestra experiencia.

We report a retrospective review of primary peptic ulcer peptic disease in 61 children. The follow-up period ranged from 1 month to 6 years. All cases were confirmed by endoscopic examination and related with radiological studies result. The number of ulcer peptic disease detected increased as much as the number of endoscopies were performed annually. In the last 20 patients pinch biopsies from antrum were taken looking for Campylobacter pylori, encountering 10 positives. The relation male:female were 2:1. Gastrointestinal bleeding had occurred in children below 6 years and abdominal pain was seen in older children. All of patients who had C. pylory infection had had recurrent abdominal pain. Three peptic ulcer were documented in patients with cystic fibrosis, which had the worse response to medical treatment. Others, had a satisfactory response.

[Results of the treatment of gastroesophageal reflux and peptic esophagitis using cisapride]. / Resultados del tratamiento del reflujo gastroesofágico y la esofagitis péptica con Cisapride.

Cisapride is a new prokinetic agent that acts at gastric emptying, esophagic peristalsis and the pressure of the low esophagic sphincter. In the present study we grave Cisapride for 12 weeks to 34 patients with severe pathologic gastroesophageal reflux and/or peptic esophagitis. The results show an important improvement of the clinic, pH monitoring, endoscopic and histologic alterations.

[Primary saccharose-isomaltose deficit: a 20-year case load]. / Déficit primitivo de sacarasa-isomaltasa: observaciones en veinte años.

In the last twenty years we have diagnosed 9 cases of congenital sucrase-isomaltase deficiency. In all the cases the diagnosis was made before 9 months of age and was confirmed by quantitative determination of sucrase-isomaltase activity in jejunal mucosal homogenates. Malnutrition and dehydration were frequent findings. In 3 cases there was clinical intolerance to dextrinomaltose and to glucose polymers. In the 6 cases in which were performed, abnormal breath H2 test after an oral sucrose load was found. Lactase activity was above the mean in all cases and an important decrease of maltase activity was demonstrated. The enzymatic deficiency persisted even though the clinical tolerance to sucrase improved with age.