Acidosis metabólica neonatal por síndrome de enterocolitis inducido por proteína de alimentos: reporte de caso / Neonatal metabolic acidosis caused by food proteininduced enterocolitis syndrome: a case report

El síndrome de enterocolitis inducido por proteínas de los alimentos (FPIES, por su sigla en inglés) es una reacción alérgica no mediada por inmunoglobulina E (IgE) con síntomas gastrointestinales, como vómitos y diarrea. El diagnóstico se basa en criterios clínicos y en una prueba de provocación para confirmarlo. Es una enfermedad desconocida en las unidades neonatales, debido a la inespecificidad de los síntomas en los recién nacidos. La cifra de metahemoglobina elevada es una opción sencilla de aproximación diagnóstica. Se describe el caso clínico de un recién nacido que ingresa al servicio de urgencias por deshidratación, letargia, vómitos, diarrea y acidosis metabólica grave con elevación de metahemoglobina, con mejora clínica y recuperación total tras el inicio del aporte de fórmula elemental. La sospecha diagnóstica se confirmó tras la prueba de provocación positiva.

Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE mediated allergic reaction with gastrointestinal symptoms, such as vomiting and diarrhea. FPIES diagnosis is based on clinical criteria and on a food challenge test. It is an unknown disease in neonatal units due to its nonspecific symptoms in newborn infants. An elevated methemoglobin level is a simple way to approach diagnosis. Here we describe a clinical case of a newborn admitted to the emergency department because of dehydration, lethargy, vomiting, diarrhea, severe metabolic acidosis, and a high methemoglobin level. Clinical improvement and complete recovery was achieved after initiation of elemental formula. The diagnostic suspicion was confirmed after a positive challenge test.

Recomendaciones para el diagnóstico y el tratamiento de los síntomas posquirúrgicos persistentes en la enfermedad de Hirschsprung / Recommendations for the diagnosis and treatment of persistent postsurgical symptoms in Hirschsprung disease

La enfermedad de Hirschsprung ocurre en 1 de cada 5000 nacimientos. La falla de migración de las células ganglionares desde la cresta neural en dirección cefalocaudal genera su ausencia en parte o todo el colon. Se manifiesta con falta de eliminación de meconio, distensión abdominal y dificultades en la evacuación. Luego del tratamiento quirúrgico, existen complicaciones a corto y largo plazo. El objetivo de esta publicación es describir las principales causas de síntomas persistentes en los pacientes operados por enfermedad de Hirschsprung y presentar un algoritmo diagnóstico-terapéutico factible de ser realizado en nuestro medio

Hirschsprung disease is characterized by the lack of migration of intrinsic parasympathetic ganglia from neural crest and consequently absence of them at varying length of the bowel, resulting in functional obstruction. The incidence is 1 per 5000 births. After surgery, short term and long term comorbidity commonly occurs. The aim of this article is to revise the main causes of ongoing symptoms after surgery in Hirschsprung disease patients and to show a diagnostic and therapeutic algorithm that can be developed in our community

Fatal nosocomial hemorrhagic enterocolitis probably caused by adenovirus: report of one case / Enterocolitis hemorrágica fatal nosocomial probablemente causada por adenovirus: informe de un caso

ABSTRACT Adenovirus (ADV) is a recognized cause of severe disease among immunocompromised patients. We report a previously healthy 39-year-old female, admitted with influenza pneumonia and evolving with lung hemorrhage and acute renal failure requiring mechanical ventilation and hemodialysis. She received high corticosteroid doses due to an initial suspicion of alveolar hemorrhage. Lymphopenia already present before steroid use (567/μL), was maintained during the whole hospital stay (mean 782/μL). From the second week of admission she presented a high-volume diarrhea (mean 2.5 L/day) associated to intermittent bloody stools. An ulcerative enterocolitis was confirmed by CT images and colonoscopy. ADV was detected in a colonic tissue sample by real time PCR but not by a commercial filmarray test. Cidofovir-probenecid and racecadotril therapy were indicated without changing the clinical course of diarrhea and the patient finally died.

Adenovirus (ADV) es una causa reconocida de enfermedades graves en pacientes inmunocomprometidos. Informamos el caso de una mujer de 39 años, previamente sana, que ingresó por neumonía grave por influenza, evolucionando con hemorragia pulmonar y falla renal aguda, requiriendo ventilación mecánica y hemodiálisis. Recibió altas dosis de corticoides por la sospecha inicial de una hemorragia alveolar. Tuvo linfopenia durante toda su estadía (promedio 782/μL), la que ya estaba presente antes del uso de los corticoides (567/μL). Desde la segunda semana de hospitalización, presentó una diarrea de alto volumen (promedio 2,5 L/día) asociada a la presencia de sangre en deposiciones en forma intermitente. Se confirmó una enterocolitis ulcerativa por tomografía computada y colonoscopía. Se detectó ADV en muestras de biopsia colónica por PCR en tiempo real pero no por un test de PCR múltiples automatizado comercial. Fue tratada con cidofovir-probenecid y racecadrotrilo sin impacto clínico y la paciente finalmente falleció.

Un caso inusual de síndrome de enterocolitis inducida por proteínas alimentarias / Unusual case of food protein-induced enterocolitis syndrome

El síndrome de enterocolitis inducido por proteínas alimentarias es un síndrome de hipersensibilidad gastrointestinal a alimentos no mediado por inmunoglobulina E, que, en su forma aguda, se manifiesta con vómitos repetitivos, palidez e hipotonía, que puede acompañarse o no de diarrea y producir un cuadro grave de deshidratación y letargia. Una prueba de provocación oral controlada es, en ocasiones, realizada para confirmar el diagnóstico y el tratamiento consiste en la eliminación del alimento causante. Se presenta el caso de un lactante de 3 meses con varios episodios de síndrome de enterocolitis tras la toma de biberón de leche de fórmula de inicio con tolerancia de otra marca comercial. Se encontraron diferencias en los ingredientes de su composición que podrían ser el origen de la sensibilización.

Food protein-induced enterocolitis syndrome is a nonimmunoglobulin E mediated gastrointestinal food hypersensitivity that manifests as profuse, repetitive vomiting, pallor and hypotonia, often with diarrhea leading to severe dehydration and lethargy (sepsis-like symptoms) in the acute setting. An oral food challenge is sometimes performed to confirm the diagnosis and treatment consists of elimination of the food trigger(s) from the diet. We report a case of a 3-months-old infant with several episodes of food protein-induced enterocolitis syndrome after taking infant formula milk with tolerance of another trademark. Differences in the composition of its ingredients could be the cause of the sensitization.

Severe forms of food allergy / Formas graves de alergia alimentar

Abstract Objectives: To guide the diagnostic and therapeutic management of severe forms of food allergy. Data sources: Search in the Medline database using the terms "severe food allergy," "anaphylaxis and food allergy," "generalized urticaria and food allergy," and "food protein-induced enterocolitis syndrome" in the last ten years, searching in the title, abstract, or keyword fields. Summary of data: Food allergy can be serious and life-threatening. Milk, eggs, peanuts, nuts, walnuts, wheat, sesame seeds, shrimp, fish, and fruit can precipitate allergic emergencies. The severity of reactions will depend on associated cofactors such as age, drug use at the onset of the reaction, history and persistence of asthma and/or severe allergic rhinitis, history of previous anaphylaxis, exercise, and associated diseases. For generalized urticaria and anaphylaxis, intramuscular epinephrine is the first and fundamental treatment line. For the treatment in acute phase of food-induced enterocolitis syndrome in the emergency setting, prompt hydroelectrolytic replacement, administration of methylprednisolone and ondansetron IV are necessary. It is important to recommend to the patient with food allergy to maintain the exclusion diet, seek specialized follow-up and, in those who have anaphylaxis, to emphasize the need to carry epinephrine. Conclusion: Severe food allergy may occur in the form of anaphylaxis and food-protein-induced enterocolitis syndrome, which are increasingly observed in the pediatric emergency room; hence, pediatricians must be alert so they can provide the immediate diagnosis and treatment.

Resumo Objetivos: Abordar o manejo diagnóstico e terapêutico das formas graves de alergia alimentar. Fontes dos dados: Busca ativa na base de dados Medline dos termos severe food allergies, anaphylaxis and food allergy e food protein-induced enterocolitis nos últimos dez anos e com busca nos campos título, resumo ou palavra-chave. Síntese dos dados: A alergia alimentar pode ser grave e ameaçadora à vida. Leite, ovo, amendoim, castanha, noz, trigo, gergelim, crustáceo, peixe e frutas podem precipitar emergências alérgicas. A gravidade das reações vai depender de fatores associados, tais como idade, uso de medicamentos no início da reação, persistência de asma e/ou rinite alérgica grave, história de prévia anafilaxia, exercício e doenças intercorrentes. Para anafilaxia, a adrenalina intramuscular é uma indicação bem estabelecida. Para o tratamento da síndrome da enterocolite induzida pela proteína alimentar na fase aguda no setor de emergência, fazem-se necessárias a pronta reposição hidroeletrolítica e a administração de metilprednisolona e odansetrona IV. Importante recomendar ao paciente com o diagnóstico de alergia alimentar grave que mantenha a dieta de exclusão, procure acompanhamento especializado e, naqueles que apresentaram anafilaxia, enfatizar a necessidade de portar adrenalina. Conclusão: Alergia alimentar grave pode se manifestar como anafilaxia ou síndrome da enterocolite induzida por proteína alimentar em fase aguda, as quais, por serem condições cada vez mais presentes e reconhecidas no setor de emergência pediátrica, demandam diagnóstico e tratamento imediatos.

Colonic complications following human bone marrow transplantation / Complicações do cólon após transplante de medula óssea humana

BACKGROUND: Human bone marrow transplantation (BMT) becomes an accepted treatment of leukemia, aplastic anemia, immunodeficiency syndromes, and hematologic malignancies. Colorectal surgeons must know how to determine and manage the main colonic complications. OBJECTIVE: To review the clinical features, clinical and pathological staging of graft vs host disease (GVHD), and treatment of patients suffering with colonic complications of human bone marrow transplantation. PATIENTS AND METHODS: We have reviewed the records of all patients that received an allogeneic bone marrow transplant and were evaluated at our Colon and Rectal Surgery department due to gastrointestinal symptoms, between January 2007 and January 2012. The study was carried out in patients who developed colonic complications, all of them with clinical, histopathological or laboratory diagnosis. RESULTS: The study group was constituted by 77 patients, 43 male and 34 female patients. We identified colonic complications in 30 patients (38.9%); five patients developed intestinal toxicity due to pretransplant chemotherapy (6.4%); graft vs. host disease was present in 16 patients (20%); 13 patients (16.8%) developed acute colonic GVHD, and 3 (3.8%) chronic GVHD. Infection was identified in 9 patients (11.6%). CONCLUSIONS: The three principal colonic complications are the chemotherapy toxicity, GVHD, and superinfection; the onset of symptoms could help to suspect the type of complication (0-20 day chemotherapy toxicity, 20 and more GVHD), and infection could appear in any time of transplantation. (AU)

EXPERIÊNCIA: O transplante de medula óssea humana (MOH) passou a ser um tratamento adotado para leucemia, anemia aplástica, síndromes de imunodeficiência e neoplasias hematológicas. Cirurgiões colorretais devem saber como determinar e tratar as principais complicações do cólon. OBJETIVO: Revisar as características clínicas, estadiamentos clínico e patológico da doença do enxerto versus hospedeiro (DEVH) e o tratamento de pacientes padecendo com as complicações colônicas do transplante de medula óssea humana. PACIENTES E MÉTODOS: Revisamos os registros de todos os pacientes que receberam um transplante de medula óssea alogênica e foram avaliados em nosso Departamento de Cirurgia do Cólon e Reto em função de sintomas gastrointestinais, entre janeiro de 2007 e janeiro de 2012. O estudo teve por base os pacientes que desenvolveram complicações do cólon, todos com diagnóstico clínico, histopatológico ou laboratorial. RESULTADOS: O grupo de estudo foi constituído por 77 pacientes, sendo 43 homens e 34 mulheres. Identificamos complicações do cólon em 30 pacientes (38,9%); cinco pacientes exibiam toxicidade intestinal por quimioterapia antes do transplante (6,4%); DEVH estava presente em 16 pacientes (20%), 13 pacientes (16,8%) foram acometidos por DEVH colônica aguda três pacientes (3,8%) DEVH crônica. Infecção foi detectada em 9 pacientes (11,6%). CONCLUSÕES: As três principais complicações do cólon são: toxicidade por quimioterapia, DEVH e superinfecção. O surgimento dos sintomas poderia ajudar a levantar suspeitas sobre o tipo de complicação (0-20 dias, toxicidade por quimioterapia; 20 ou mais dias, DEVH). Infecções podem ocorrer em qualquer momento do transplante. (AU)

Efecto de la temperatura de refrigeración y calentamiento de fórmulas lácteas Infantiles en el crecimiento de cronobacter sakazakii / Effect of temperature cooling and heating of powered infant formula on the growth of cronobacter sakazakii

Cronobacter sakazakii (C. sakazakii) es un microorganismo perteneciente a la familia Enterobacteriaceae asociado con patologías como meningitis y septicemia en recién nacidos. Las fórmulas lácteas infantiles (FLI) han sido reconocidas como el vehículo más importante de transmisión de C. sakazakii, por condiciones inadecuadas durante su producción o preparación. El objetivo de la presente investigación fue evaluar el efecto de la temperatura de refrigeración y el calentamiento de las FLI en el crecimiento de C. sakazakii. Las muestras de FLI reconstituidas se contaminaron con 102, 104 y 106 UFC/mL de C. sakazakii y se refrigeraron durante cuatro horas a 4°C, 6°C y 10°C, posterioriormente, se calentaron en baño hidrotérmico hasta 37°C. Las muestras fueron sembradas al tiempo “0” (postrefrigeración) y luego del calentamiento hasta 37°C en placas con agar cromogénico y se incubaron a 35°C por 24h. Para el análisis de los datos se utilizó la prueba de Wilcoxon. Según los datos obtenidos no hubo variación significativa en la población de C. sakazakii en las FLI luego de 4 horas en refrigeración a 4°C, 6°C y 10°C; sin embargo, al calentar las fórmulas hasta 37°C, hubo en todos los casos una reducción de un ciclo logarítmico en la población de C. sakazakii con un valor de p = 0.0056 y Z= 2.752. Se concluye que el calentamiento a 37°C post-refrigeracion de las muestras, disminuye la población de C. sakazakii en FLI (p<0,05)(AU)

Cronobacter sakazakii (C. sakazakii) is a microorganism belonging to the family Enterobacteriaceae associated with diseases such as meningitis and sepsis in newborns. The powdered infant formula (PIF) have been recognized as the most important vehicle of transmission of C. sakazakii, by inadequate conditions during production or during its preparation. The aim of this investigation was to assess the effect of the cooling temperature and the heating of PIF in the growth of C. sakazakii. For this, PIF reconstituted samples were contaminated with 102, 104 and 106 CFU / mL of C. sakazakii and refrigerated for four hours at 4 ° C, 6 ° C or 10 ° C after this hydrothermal bath heated to 37 ° C. Samples were cultured at time “0” (after- cooling) and then heating to 37 ° C in chromogenic agar plates and incubated at 35 ° C for 24h . For data analysis we used the Wilcoxon test. According to the data there was no significant variation in the population of C. sakazakii in PIF after 4 hours in the refrigerator at 4 ° C, 6°C and 10 ° C , however , by heating the formulas to 37 ° C , in all cases there was a reduction of one log cycle in population C. sakazakii with a value of p = 0.0056 and Z = 2.752 . We conclude that heating at 37°C post- cooling of the samples, decreases the population of C. sakazakii in FLI ( p < 0,05 )(AU)

Milk-induced enterocolitis in monozygotic twin neonates.

Reports of milk protein-induced enterocolitis are increasing, but few describe the condition in twins. Twin Japanese girls developed bloody diarrhea on day 4 and were transferred to the NICU in our hospital. Surgical disorder and infection were initially suspected, but the correct diagnosis was reached after 12 days of hospitalization.

Antemortem diagnosis of gastrointestinal mucormycosis in neonates: report of two cases and review of literature.

Gastrointestinal mucormycosis has been reported more frequently in adults than in children. Due to high mortality diagnosis is usually made at autopsy. Only 3 cases of neonates surviving this disease have been described. We report two cases of preterm neonates presenting clinically with symptoms of necrotizing enterocolitis (NEC), but lacking radiological characteristics of NEC where histopathology confirmed the diagnosis of gastrointestinal mucormycosis involving caecum and appendix.

Enfermedad injerto versus huesped: manifestación-enterocólica y diagnóstico diferencial / Graft versus host disease: gastrointestinal manifestation and differential diagnosis

La enfermedad de injerto contra huésped es un síndrome que ocurre en pacientes inmuno-deprimidos generalmente post-trasplante de médula ósea, caracterizado en su etapa aguda por manifestaciones dermatológicas, hepáticas y gastrointestinales. El diagnóstico oportuno es fundamental, ya que puede disminuir significativamente la morbimortalidad. Dentro del diagnóstico diferencial de las manifestaciones gastrointestinales se incluye enterocolitis neutropénica y colitis pseudomembranosa. Mediante tomografía computada, hay signos que apuntan a su diagnóstico, como dilatación focal de asas, y refuerzo significativo de la mucosa enterocólica.

Enterocolitis neutropénica / Neutropenic enterocolitis

Se presenta caso de un paciente de 18 años de edad con leucemia limfoblástica aguda que recibió tratamiento quimioterápico con vincristina, metotrexato, asparginasa y aracytina en 2 bloques de 5 días cada uno y con una diferencia de un mes. Posteriormente el esquema se debió repetir por presentar recaída de su enfermedad. Durante el segundo bloque de terapia de su recaída, presenta cuadro de dolor abdominal en fosa ilíaca derecha (FID), compromiso de estado general, fiebre hasta 39ºC y deposiciones líquidas abundantes, sin elementos patológicos, Al examen su abdomen era sensible en FID con irritación peritoneal. El recuento de glóbulos blancos era de 400/mm3. Se realiza laparotomía de Mc Burney y apendicectomía, evidenciando ciego de paredes engrosadas y adermatosa. Tomografía axial computada postoperatoria, reveló compromiso inflamatorio del colon derecho, transverso y escaso líquido libre. Evoluciona séptico, grave. Se realizó tratamiento médico intensivo que incluyó régimen o y antibióticos con buena respuesta clínica y de imagenes. Sin embargo, desarrolló fístula cecal a los 20 días de tratamiento que se manejó conservadoramente

importance of pathological findings in identification of patients at risk of developing Hirschsprung's disease associated enterocolitis

Enterocolitis remains the most serious complication of Hirschsprung's disease, and has been reported in 15% to 50% of cases. Patients are prone to develop relapses despite a defunctioning colostomy and after definitive pull-through procedures. There are several risk factors as delayed diagnosis of Hirschsprung's disease, long spastic segment, trisomy 21 and .family history of Hirschsprung's disease. Currently no clinical factor or test is available to predict patients prone to develop postoperative enterocolitis. Thirty-two Hirschsprung's disease patients were studied in an attempt to correlate the histologic changes of the colonic mucosa with the clinical and post-operative course to outline a histological guide line of value in prediction of patients prone to develop clinical enterocolitis

Hospital based incidence of clostridium difficile enterocolitis and cross reactivity of the latex agglutination test

In order to determine the prevalence and characteristics of antibiotic-associated Clostridium difficile enterocolitis, a total of 2476 specimens from 2356 patients were examined. Of these 224 [9.5%] were positive, 158 [70%] of them occurring in organ transplant, bone marrow transplant or neutropenic patients. Antibiotic-associated colitis due to enterotoxigenic C. difficile was diagnosed by use of cytotoxin assay [CTA]. During a previous study, CTA was compared with a commercial latex agglutination [LA] test and gave a high frequency of false positives. Sixteen of these 31 false positive specimens also had a bacterial pathogen or parasite present. Since a wide range of microbial pathogens/parasites are found in Saudi patients, we also tested stool supernatants from a further 107 patients harboring enteric microbial pathogens/parasites to determine the cross-reactivity of a rapid latex agglutination kit [LA] with these organisms. Only one specimen which grew Salmonella enteritidis gave a false-positive reaction. Since the reagents in the LA test also react with non-toxigenic strains of C. difficile and other anaerobes, the method could be used only as a rapid screening procedure and positive findings should be confirmed by a specific test. Running title: C. difficile toxin enterocolitis

Cancer del cuello uterino: tratamiento radiante y complicaciones / Cervix uteri cancer: radiation therapy and coomplications

Se estudiaron 114 pacientes con cancer de cuello uterino entre 1988 y 1991, los cuales fueron tratados en el servicio de Oncologia del Hospital Provincial Docente Clinicoquirurgico de Santa Clara mediante tratamiento radiante con cobalto-60 en forma de teleterapia y braquiterapia. El grupo mas afectado se encontro entre los 50-59 anos, la variedad histica mas frecuente correspondio al carcinoma epidermoide, y la etapa clinica mas comun fue la II. En la mayoria de los casos se aplicaron dosis de 2001 a 3000 cGy con teleterapia externa en los puntos A y B, previo a la braquiterapia; despues se completo los puntos B con teleterapia. La mayoria de los pacientes recibieron 80 Gy en el punto A y mas de 60 en el B. Las complicaciones inmediatas mas frecuentes fueron la proctitis y la enterocolitis, y entre las tardias, las fistulas y la estenosis vaginal

Aeromonas spp en niños con síndrome diarreico agudo / Aeromonas spp in children with acute diarrheal disease

El interés por el género Aeromonas es creciente, tanto como causante de enfermedades diarreicas como extraintestinales. Produce cuadros intestinales a través de acción citotóxica y enterotóxica. Empleando agar sangre con 30 mg/l de ampicilina (ASA) se aislaron Aeromonas spp en 8 de 400 niños diarreicos y en ninguno de 230 controles no diarreicos. El aislamiento fue muy inferior en los medios tradicionales del coprocultivo, agar lactosa y agar Shigella-Salmonella, así como agar sangre sin ampicilina, en el que hubo sobredesarrollo de flora entérica. Cinco cepas aisladas fueron A. hydrophila, 2 A. caviae y 1 A. veroni biotipo sobria. Cuatro de cinco cepas probadas resultaron enterotoxigénicas en asa de conejo. Todas las cepas fueron hemolíticas; 6 fueron Voges Proskauer (+), lisina decarboxilasa (+) y glucosa con gas (+), características que han sido relacionadas con la virulencia. Todos los aislados resultaron resistentes a las aminopenicilinas con o sin sulbactama y sensibles a cloranfenicol, colistin, gentamicina, neomicina y norfloxacina. Dos cepas resultaron resistentes a sulfametoxazol-trimetoprima

Acometimento gastrointestinal pelo citomegalovírus na síndrome de imunodeficiência adquirida / Gastrointestinal disorders due to cytomegalovirus in the acquired immunodeficiency syndrome

Descrevemos quatro casos de hemofílicos A com AIDS que desenvolveram gastroenterocolite pelo citomegalovírus com ulceraçöes e perfuraçöes que conduziram a quadros abdominais agudos de urgência e de elevada mortalidade. A média de idade foi de 18 anos (10-36 anos). Todos foram transfundidos com concentrados locais de Fator VIII, obtidos de doadores voluntários de sangue. Os pacientes possuíam anti-HIV positivo (Elisa), com inversäo significativa de OKT4; OKT8. Clinicamente a doença se manifestou por dor abdominal em cólica e hemorragia digestiva alta e/ou baixa, seguidas de perfuraçöes de vícera oca (três casos). O primeiro caso apresentou quadro idêntico ao da retocolite ulcerativa. Nos casos revistos foram observadas inclusöes intranucleares e intracitoplasmáticas de CMV, envolvendo as células endoteliais e epiteliais, dentro e em tomo das lesöes ulceradas. Comcluímos que (1) o comprometimento gastrointestinal citomegalovírus em pacientes com AIDS é freqüente e com elevada mortalidade; (2) o diagnóstico precoce da infecçäo pelo citomegalovírus näo muda o prognóstico e a evoluçäo destes pacientes