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.

Enterocolitis neutropénica en el paciente oncológico pediátrico / Neutropenic enterocolitis in the pediatric cancer patient

Resumen La enterocolitis neutropénica (ECN) es una enfermedad heterogénea de foco digestivo, pero afectación sistémica, que corresponde a una condición clínica grave que amenaza la vida de pacientes inmunocomprometidos, particularmente oncológicos pediátricos. De patogenia aún poco definida y aunque de causa multifactorial, la ECN se asocia a los efectos citotóxicos de la quimioterapia empleada y se caracteriza por la triada clásica que incluye fiebre, neutropenia y dolor abdominal, donde la principal injuria se localiza en la mucosa intestinal, provocando su alteración como barrera y facilitando la invasión bacteriana intramural. La ECN constituye un reto diagnóstico para el equipo tratante, que requiere ser oportuno y contar con apoyo de un óptimo laboratorio general e imagenológico, para iniciar un completo manejo multidisciplinario en unidades y centros de alta complejidad. Se presenta una revisión actualizada del tema incorporando aspectos epidemiológicos, factores de riesgo, elementos de apoyo diagnóstico, consideraciones terapéuticas y medidas de prevención a fin de aportar en el conocimiento de esta patología, y reducir morbimortalidad en estos pacientes.

Abstract Neutropenic enterocolitis (NEC) is a heterogeneous disease of the gastrointestinal tract with systemic response, that corresponds to a severe and life-threatening clinical condition in immunocompromised patients, especially in childhood cancer. The pathologic features are poorly understood, although its multifactorial cause of NEC is well established and it is associated with the cytotoxic effects of the chemotherapy agents used and recognized by the classic triad of fever, neutropenia, and abdominal pain, secondary to gastrointestinal injuries that alters mucosal permeability and helps intramural bacterial invasion. NEC is truly a clinical challenge that requires an early diagnosis and a multidisciplinary approach including basic laboratory and imagological tests in high complexity centers. We present a current review, adding epidemiological aspects, risks factors, diagnostic support elements, therapeutic considerations, and preventive measures in order to provide knowledge of this disease and help to reduce morbidity and mortality associated with it.

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.

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.

Enterocolitis asociada a enfermedad de Hirschsprung / Enterocolitis associated with Hirschsprung's disease

La enterocolitis asociada a enfermedad de Hirschsprung (EAEH) se define como la presencia de diarrea, deposiciones explosivas, distensión abdominal y signos radiológicos de obstrucción intestinal o edema de la mucosa. Es la causa más común de mortalidad en pacientes con enfermedad de Hirschsprung (EH). Puede ocurrir en cualquier etapa de la enfermedad, con una incidencia media del 25 por ciento. La fisiopatología es poco conocida. Etiologías potenciales son la obstrucción mecánica, infecciones, barrera mucosa defectuosa, y un sistema inmunológico deficitario. Microscópicamente la EAEH se caracteriza por criptitis y abscesos en las criptas, seguida de ulceración de la mucosa progresiva conduciendo eventualmente a la necrosis transmural. Los pilares del tratamiento son la reanimación con fluidos, la descompresión del intestino y la terapia antibiótica. A veces se requiere de una estoma cuando estas medidas han fracasado. La mayoría de los pacientes con EAEH siguen teniendo trastornos de la función intestinal muchos años después de la cirugía para la enfermedad de Hirschsprung. Investigaciones en la fisiopatología de la EAEH pueden conducir a medidas preventivas y mejores modalidades de tratamiento.

Hirschsprung’s-associated enterocolitis(HAEC) is defined as the presence of diarrhea, explosive stools, abdominal distension and radiologic evidence of bowel obstruction or mucosal edema. HAEC is the most common cause of mortality in patients with Hirschsprung’s disease. This condition may occur in any stage of the Hirschsprung’s disease, with a mean incidence of 25 percent. The pathophysiology is poorly understood. Potential etiologies are mechanical obstruction, infections, defective mucosal barrier, and impaired immunological system. Microscopically HAEC is characterized by cryptitis and crypt abscesses followed by progressive mucosal ulceration eventually leading to transmural necrosis. The mainstays of treatment are fluid resuscitation, decompression of the bowel and antibiotic therapy. Sometimes a stoma is required when such measures have failed. The majority of patients with HAEC continue to have disturbances of bowel function many years after surgery for Hirschsprung’s disease. Researchs into the pathophysiology of HAEC can lead to better preventative measures and treatment modalities.

The Significance of Gastric Juice Analysis for a Positive Challenge by a Standard Oral Challenge Test in Typical Cow's Milk Protein-Induced Enterocolitis

This study was performed to investigate the significance of gastric juice analysis (GJA) as a diagnostic criterion of a positive challenge in a standard oral cow's milk challenge (OCC) to confirm typical cow's milk protein-induced enterocolitis (CMPIE). Data from 16 CMPIE patients (aged 14 to 44 days) were analyzed. A standard OCC was openly executed using 0.15 g/kg of protein. Three symptoms (vomiting, lethargy, and bloody or pus-like stool), and four laboratory findings (GJA [3 hr], changes in peripheral blood absolute neutrophil count [ANC] [6 hr], C-reactive protein [6 hr], and stool smear test for occult blood or leukocytes) were observed after OCC. Before OCC, baseline studies were conducted; a stool smear test, blood sampling, and GJA. Positive OCC results were; vomiting (87.5%) (observed 1-3 hr after OCC), lethargy (62.5%) (1-3 hr), bloody or pus-like stool (43.8%) (6-10 hr), abnormal GJA (93.8%), an ANC rise >3,500 cells/microliter (93.8%), and an abnormal stool smear test (75.0%). A single GJA test after a standard OCC is a sensitive diagnostic criterion of a positive challenge, and may provide an early confirmatory diagnosis of CMPIE. An investigation of positive OCC outcomes helps to find out a diagnostic algorithm of criteria of a positive challenge in CMPIE.

Indexes of Suspicion of Typical Cow's Milk Protein-Induced Enterocolitis

This study was performed to identify clinical factors that facilitate the diagnosis of typical cow's milk protein-induced enterocolitis (CMPIE). Data from 142 consecutive patients (aged 15 to 45 days, cow's milk formula- or cow's milk and breast milk mixed-fed) admitted due to vomiting and/or diarrhea were retrospectively analyzed. These 142 subjects were divided into three groups: the CMPIE, infection, and non-infection group. Each group was composed of 16 (11.3%), 102 (71.8%), and 24 (16.9%) patients, respectively. On admission, poor weight gain (p=0.003), hypoalbuminemia (p=0.035), peripheral leukocytosis (p=0.012), and metabolic acidosis (p=0.015) were found to be more significant in the CMPIE group than those in other two groups. In CMPIE, serum albumin levels decreased from 3.3+/-0.9 g/dL on admission to 2.6+/-0.3 g/dL during admission (p<0.05), and methemoglobinemia was observed in 3 patients (18.8%) (p=0.012). Multiple logistic regression analysis showed that the independent predictors of CMPIE versus the infection group were failure to gain weight (OR, 10.75 [95% CI, 1.53-66.12]) (p= 0.014) and hypoalbuminemia (OR, 9.53 [95% CI, 1.62-49.01]) (p=0.010). The early recognition of indexes of suspicion for CMPIE may be of help in the diagnosis and treatment of this disorder.

Celiac disease, TB enterocolitis and giardiasis among children with chronic diarrhea: the accuracy of ANT-EMA and tTG expression in the diagnosis of celiac disease

Chronic diarrhea is one of the most common causes of referral to a gastroenterology clinic. Chronic diarrhea may result from many different causes; celiac disease is one of them. Other important causes in our locality are infections such as TB and Giardiasis. This work was planned to: 1-Determine the frequency of celiac disease, TB enteritis and Giardiasis among children referred to the gastroenterology unit with the complaint of chronic diarrhea and to evaluate the different methods used in the diagnosis of each disease. 2- Verifying the diagnostic accuracy of immunohistochemical tTG expression versus serum anti-endomysial antibodies [EMA] in celiac disease [CD] diagnosis. The study included 92 patients with chronic diarrhea. Their ages ranged from 6 months to 15 years. They were 56 males and 36 females, admitted to the Pediatric Gastroenterology Unit, Assiut University Hospital during the period from January 2005 to December 2006. Besides full history and thorough clinical examination, the following investigations were done for all cases: stool analysis for three consecutive days, CBC, ESR, total proteins and serum albumin, tuberculin test, accelerated BCG test [in tuberculin negative cases], serum anti-EMA. Upper GIT endoscopy with duodenal biopsy and aspiration and tissue staining by H and E and by Immunohistochemical [anti-tTG moAbs] to detect tTG antigens in biopsy specimens. Lower GIT endoscopy with biopsy sampling and histopathological examination of biopsy specimens was also done. Out of the total patients, 18 cases [19.5%] were positive for celiac disease AEM antibodies while 16 were positive by tTG immunostaining of biopsy specimens. Fourteen patients [15.2%] had tuberculous enterocolitis while 12 [13%] had biopsy proven Giardiasis. On the other hand 48 patients [52.1%] had other undiagnosed causes of chronic diarrhea. A very high index of suspicion for CD should be maintained for patients who present with chronic diarrhea or iron deficiency anemia. The best method for diagnosis of celiac disease in such patients is serological testing followed by a small-bowel biopsy. The diagnosis of intestinal tuberculosis is difficult due to the lack of specific symptoms and signs. Colonoscopy with ileoscopy is a useful method for diagnosis of intestinal TB. Gastrointestinal endoscopy with biopsy examination is an important method of diagnosis and follows up of children with Giardiasis

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

Perfil clínico da enterocolite por Citomegalovírus (CMV) na síndrome da imunodeficiência adquirida (AIDS) / Clinical profile of Cytomegalovirus (CMV) enterocolitis in acquired immunodeficiency syndrome (AIDS)

Múltiplos agentes estão envolvidos na etiopatogenia da diarréia em Aids. O exame de fezes e a colonoscopia são elementos decisivos para o diagnóstico específico. A enterocolite por CMV pode cursar com febre, emagrecimento, diarréia intermitente e hematoquesia. Outros agentes causadores de diarréia podem ter o mesmo espectro de apresentação. OBJETIVO: Definir o perfil clínico da enterocolite por CMV em pacientes com Aids, comparando os parâmetros clínicos, endoscópicos e de tempo de sobrevida entre dois grupos com diarréia crônica, grupo A com CMV e grupo B sem CMV. MÉTODOS: Foram acompanhados 48 pacientes com Aids e diarréia de duração maior que 30 dias, sendo 27 do grupo A e 21 do grupo B. Os parâmetros analisados foram idade, situação de risco, duração da diarréia, hematoquesia, intervalo de tempo entre diagnóstico da infecção por HIV e início de diarréia, achados endoscópicos e sobrevida. Foram realizados exames parasitológicos, culturas e colonoscopia com biópsias. Foi utilizado o teste "t-student" para amostras não pareadas e o teste Qui-Quadrado com correção de Yates para variáveis não paramétricas. Foram construídas curvas de sobrevida pelo método descrito por Kaplan-Meier e aplicado o teste de Mantel - Haenszel. Foi assumido como nível de significância estatística o valor de P menor que 0.05. RESULTADOS: O padrão endoscópico da infecção por CMV correspondeu a ulcerações associadas a hemorragia de submucosa 14 (51.8 por cento) P < 0.01. O tempo de sobrevida do grupo B após o diagnóstico do HIV e após o diagnóstico específico da diarréia foi maior que o do grupo A (0.005 > P > 0.001). Os outros parâmetros estudados não mostraram significância estatística. CONCLUSÕES: A presença de enterocolite por CMV em Aids é marcador de mau prognóstico e menor sobrevida. Existe um padrão endoscópico sugestivo da infecção por CMV.

Value of clinical features in the diagnosis of enteric fever.

There is no objective data on the value of individual clinical symptoms or signs in the diagnosis of enteric fever in a febrile patient. The purpose of the study was to assess the value of some clinical and simple laboratory features in the diagnosis of enteric fever. One hundred & six patients with microbiologically confirmed enteric fever and 170 patients with other established febrile illnesses were included in the evaluation. History of stepladder pattern of rise of temperature, loose motions, relative bradycardia and coated tongue proved to be powerful markers of enteric fever with high specificity (100%, 94.71%, 94.71%, 94.12% respectively), positive and negative predictive values. Headache, hepatomegaly and splenomegaly were moderately powerful. ESR and WBC count appeared to have little value in the diagnosis of enteric fever. Pattern of onset and loose motions did not discriminate between typhoid and paratyphoid fever. Most of these patients had illness persisting beyond one week by which viral infections and infectious enterocolitides were largely excluded. Elucidation of power of these markers in distinguishing enteric fever from other febrile illnesses with the help of better designed prospective studies would lessen our dependence on expensive and time consuming laboratory investigations.

Tiflitis neutropénica en niños / Neutropenic typhlitis in children

Introducción. La tiflitis es una colitis necrozante con inflamación del ciego en pacientes neutropénicos, usualmente diagnosticada en la autopsia. Material y métodos. identificamos 18 niños hospitalizados en el Hospital Infantil de México Federico Gómez en un período de 10 años, con presencia de fiebre, dolor e hipersensibilidad abdominal sugestiva de tiflitis neutropénica. Resultados. Ocho de los 18 pacientes tenían evidencia radiológica de tiflitis en las radiografías de abdomen. Todos los pacientes presentaron neutropenia al momento del diagnóstico y 9 tenían bacteriemia documentada. Los 18 pacientes fueron tratados con diversos antibióticos, 4 de ellos sometidos a cirugía y 1 a colonoscopia. Once pacientes murieron, 10 se mejoraron médicamente y 1 quirúrgicamente. En orden de frecuencia, la tiflitis fue identificada en 9 pacientes con leucemia aguda no linfoide, en 6 con leucemia aguda linfoide; en 1 con anemia aplásica, neutropenia cíclica y carcinoma renal, respectivamente. Sólo 4 pacientes recibieron factor estimulador de colonias de granulocitos; y en todos los pacientes que sobrevivieron se observó un aumento de leucocitos polimorfonucleares a más de 500/mm3 documentado entre el cuarto y el décimosexto día de manejo médico y/o quirúrgico. Conclusiones. Recomendamos que ante un paciente con fiebre y neutropenia, con dolor e hipersensibilidad abdominal, además de datos como diarrea hematemesis se debe plantear como diagnóstico tiflitis neutropénia e instaurarse el manejo general y específico que incluye la valoración quirúrgica

Sarcocystis infection and actinomycosis in tumorous eosinophilic enterocolitis.

Intramural masses were resected from jejunum and ileocecal portion of a 49-year-old, female patient with partial gut obstruction. Histopathological examination indicated the masses to be tumorous eosinophilic enterocolitis. Recent and late development phases of Sarcocystis in relation to bradyzoite infection have been observed and considered to be responsible for eosinophilic inflammation. Concomitant intestinal actinomycosis, known to produce tumorous lesion without eosinophilia, appears as an attractive natural model in producing tumorous eosinophilic enterocolitis. Pertaining to parasitic development, it is suggested that persisting sporulated oocyst may undergo spontaneous excystation in the host's intestinal wall, along with complex sporogony.

Estudo retrospectivo de 7 casos de enterocolite necrosante (en) em recém-nacidos. Uma proposta de assistência de enfermagem / Retrospective study of 7 cases of necrotizing enterocolitis in newborns: a proposal for nursing assistance

O presente trabalho analisa sete casos de Enterocolite Necrosante (EN) em recém-nascidos (RN). Säo propostos cuidados específicos de enfermagem baseados em dados que caracterizam a populaçäo e os sinais clínicos de riscos. O estudo visa contribuir para o diagnóstico precoce e a prevençäo de complicaçöes