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.

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

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.

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.

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

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.

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.

The comparative pathology of enterocolitis caused by Clostridium perfringens type C, Clostridioides difficile, Paeniclostridium sordellii, Salmonella enterica subspecies enterica serovar Typhimurium, and nonsteroidal anti-inflammatory drugs in horses.

To determine if there were significant differences produced by 5 of the most prevalent causes of equine enterocolitis, we studied retrospectively the gross and microscopic pathology of 90 cases of enterocolitis submitted to the San Bernardino laboratory of the California Animal Health and Food Safety Laboratory. Included were cases caused by Clostridium perfringens type C (CP; n = 20), Clostridioides difficile (CD; n = 20), Paeniclostridium sordellii (PS; n = 15), Salmonella enterica subspecies enterica serovar Typhimurium (ST; n = 20), and NSAID intoxication (NS; n = 15). Grossly, necrotizing hemorrhagic typhlocolitis was seen most frequently in cases of CD, ST, and NS disease. Cases of CP and PS had enteritis or colitis in similar percentages. Congestion, hemorrhage, and pleocellular inflammatory infiltrates followed by mucosal and submucosal necrosis were the main lesions found in horses with enteritis or colitis produced by any of the etiologic agents investigated. Severe lesions were more frequent in cases of CD and CP than in cases associated with any of the other 3 etiologies. Pseudomembranes were observed with similar prevalence in the small intestine and colon affected by all agents studied. Thrombosis of the lamina propria and/or submucosa was observed in ~50% of the cases of enteritis and colitis by all etiologies, except for PS, in which the majority of the cases had thrombosis. Gross and microscopic lesions of enterocolitis were not sufficiently specific for any of these etiologic agents to enable these enteritides to be distinguished by gross and/or histologic examination.

[Food protein-induced enterocolitis syndrome in a patient with pseudo-Bartter syndrome associated with cystic fibrosis. A case report]. / Enterocolitis inducida por proteínas alimentarias en un paciente con síndrome de pseudo-Bartter asociado a fibrosis quística. Reporte de un caso.

INTRODUCTION: Food protein-induced enterocolitis is a non-immunoglobulin E-mediated food allergy with acute manifestations like recurrent vomiting, dehydration, and shock. It is a rare pathology that requires a high index of suspicion. Pseudo-Bartter syndrome (metabolic alkalosis, hypokalemia and hypochloremia in the absence of tubulopathy) is an infrequent complication of cystic fibrosis. CASE REPORT: A 5-month-old boy with recurrent vomiting, dehydration, and shock; who had been breastfed and had consumed baby formula three hours prior to the onset of symptoms. Laboratory tests confirmed hyponatremia, hypochloremic metabolic alkalosis, and hypokalemia in absence of tubulopathy; two iontophoresis showed altered results, stool elastase was decreased, and genetic sequencing confirmed the diagnosis of cystic fibrosis. The provocation test confirmed food protein-induced enterocolitis syndrome. CONCLUSION: Recurrent vomiting and dehydration after the intake of milk formula must lead to suspicion of food protein-induced enterocolitis syndrome. If pseudo-Bartter syndrome is found, cystic fibrosis must be ruled out.

Introducción: La enterocolitis inducida por proteínas alimentarias es una alergia alimentaria no mediada por inmunoglobulina E, manifestada en forma aguda por vómito recurrente, deshidratación y choque. Es una patología inusual que requiere alto índice de sospecha. El pseudo-Bartter (alcalosis metabólica, hipocaliemia e hipocloremia en ausencia de tubulopatía) es una complicación infrecuente de fibrosis quística. Reporte de caso: Niño de cinco meses de edad con vómito recurrente, deshidratación y choque, alimentado con lactancia materna, pero que consumió fórmula tres horas previas al inicio de síntomas. Los exámenes de laboratorio confirmaron hiponatremia, alcalosis metabólica hipoclorémica e hipocalemia sin tubulopatía; dos iontoforesis mostraron resultados alterados; la elastasa en materia fecal se encontró disminuida y la secuenciación genética confirmó el diagnóstico de fibrosis quística. La prueba de provocación confirmó enterocolitis inducida por proteínas alimentarias. Conclusión: El vómito recurrente y la deshidratación tras la ingesta de fórmula láctea deben hacer sospechar un enterocolitis inducida por proteínas alimentarias. Ante el hallazgo de pseudo-Bartter se debe descartar fibrosis quística.

Fatal nosocomial hemorrhagic enterocolitis probably caused by adenovirus. Report of one case.

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.

[Food Protein Induced Enterocolitis Syndrome due to ingestion of kiwi causing potentially severe unexplained event in a child]. / Síndrome de enterocolitis inducida por proteínas alimentarias por ingesta de kiwi como causa de evento potencialmente grave inexplicado en una niña.

Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE food allergy manifesting as profuse, repetitive vomiting, sometimes with diarrhea, leading to dehydration and lethargy that can be severe and lead to shock. Despite the potential severity, awareness of FPIES is low and diagnosis is often delayed, especially in those triggered by solid foods. Presence of vomits and duration of more than 1 minute are the key differential factors to distinguish FPIES from brief resolved unexplained events. We report a case of a 6-month-old infant finally diagnosed as having kiwi induced FPIES.

El síndrome de enterocolitis inducida por proteínas alimentarias es una alergia alimentaria no mediada por inmunoglobulina E que se manifiesta clínicamente con vómitos profusos y repetitivos, en ocasiones, asociados a diarrea, y puede llegar a asociar deshidratación y letargia, con riesgo de desarrollo de shock. A pesar de su potencial gravedad, el índice de sospecha de este síndrome es bajo, lo que demora su diagnóstico, especialmente, en aquellos casos que son desencadenados por alimentos sólidos. La presencia de vómitos y la duración de más de un minuto son los datos clave que pueden diferenciarlo de los episodios breves, resueltos e inexplicados. Se presenta el caso de una lactante de 6 meses de vida con diagnóstico final de síndrome de enterocolitis inducida por proteínas alimentarias por ingesta de kiwi.

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.

Food Protein-Induced Enterocolitis Syndrome Food Challenges: Experience from a Large Referral Center.

BACKGROUND: Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated food allergy that is diagnosed based on clinical findings, but can be confirmed with oral food challenge (OFC). OFC is more often performed to assess the development of tolerance. Most studies describing OFCs in FPIES are limited in size. OBJECTIVE: We sought to describe our experience with OFCs using our FPIES protocol. Patients were given one-third of serving size with a 4-hour observation period, followed by home titration to full dose. METHODS: We conducted a retrospective chart review of patients who underwent OFC via the FPIES protocol from 2014 to 2017. Data regarding the history of reaction, age at the time of challenge, and reactions during challenge or with home introduction were collected. RESULTS: A total of 169 OFCs were completed under the FPIES protocol, in 119 patients to 19 different foods. Thirty challenges (18%) were positive, with 17 challenges (10%) during initial challenge and 13 (7.7%) during home dosing. Most reactions during initial challenge required intravenous fluids (IVF), but hypotension was uncommon. One hundred thirty-nine (82%) OFCs were negative with home introduction, indicating tolerance to the challenged foods. The mean age of passing a challenge to milk, soy, and grain was earlier than that of other solid foods. CONCLUSIONS: Our data suggest that our FPIES OFC protocol is safe. Early administration of IVF may prevent the development of hypotension. It is difficult to stratify the risk of severe or delayed reaction based on patient characteristics, and more data are needed to identify those appropriate for home introduction.

Food Protein-Induced Enterocolitis Instead of Necrotizing Enterocolitis? A Neonatal Intensive Care Unit Case Series.

Necrotizing enterocolitis is an important disease in infants born premature. However, other disease entities present with similar signs and symptoms. This series reviews 5 atypical cases initially diagnosed as necrotizing enterocolitis that may be more consistent with food protein-induced enterocolitis. Food protein-induced enterocolitis may be underdiagnosed in this population.

Severe forms of food allergy.

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.

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.

Growth and developmental outcomes of infants with hirschsprung disease presenting in the neonatal period: a retrospective study.

OBJECTIVES: To describe the presentation and progress over the first year of life of neonates with Hirschsprung disease, to describe their physical and developmental outcomes at 12 months of age, and to compare the outcomes of infants with short- vs long-segment Hirschsprung disease. STUDY DESIGN: A retrospective study of neonates born with Hirschsprung disease in Western Australia between January 1, 2001, and December 31, 2010, to review their presentation, progress, growth, and development at 12 months of age. RESULTS: Fifty-four infants were identified (40 with short and 11 with long segment and 3 with total colonic aganglionosis); 9 infants had a recognized syndrome and 1 infant died, unrelated to Hirschsprung disease. A primary pull-through procedure was performed in 97% and 21% of neonates with short- and non-short-segment Hirschsprung disease, respectively; 17 (31%) infants developed anal stenosis requiring dilatations. Enterocolitis occurred in 14 (26%) infants. Griffiths Mental Development Scale scores (1 year) were available in 31 of 45 nonsyndromic survivors: mean general quotient (94.2, SD 8.89) was significantly less than the population mean (P = .007), but the number of infants with developmental delay was within the expected range. Physical growth, except length, appeared adequate in nonsyndromic infants. There were no significant differences in the outcomes of infants with short- vs non-short-segment Hirschsprung disease. CONCLUSIONS: At 1 year of age, many infants with Hirschsprung disease have ongoing gastrointestinal problems. Their overall growth appears satisfactory, and most infants are developing normally; however, their mean general quotient appears shifted to the left. Longer-term studies will better define developmental outcomes.

Granulomatous enterocolitis secondary to Yersinia in an 11-year-old boy from Puerto Rico, confirmed by PCR: a case report.

We report the case of an 11-year-old boy without any history of systemic illness and who developed fever and chills and experienced a 10 lbs. weight loss 3 weeks prior to admission. Two days before admission, he experienced abdominal pain that became localized to the right lower quadrant within 24 hours. A non-enhanced abdominal computed tomography scan revealed a 5 mm, amorphous, hyperdense, right lower quadrant calcification associated with a 9 mm fluid-filled structure. An exploratory laparotomy revealed thickening of the proximal vermiform appendix extending to the cecum, with mesenteric adenopathy. An en-bloc excision of the distal ileum, cecum, mesocolon, and vermiform appendix with an end-to-end ileo-ascending colon anastomosis was performed because of a suspected neoplasm. Pathologic examination of the specimen revealed granulomatous enterocolitis with associated acute suppurative appendicitis and a cecum abscess. Polymerase chain reaction analysis of a paraffin-embedded tissue block confirmed Yersinia enterocolitica DNA in the tissue, pointing to Yersinia as being the etiologic microbe. To our knowledge, this is the first case of yersiniosis to be reported in Puerto Rico.

Food protein-induced enterocolitis syndrome: insights from review of a large referral population.

BACKGROUND: Food protein-induced enterocolitis (FPIES) is a rare non-IgE mediated disease. Most studies have been limited in nature, with the largest cohort being 66 patients. The most common foods that have been reported are milk and soy. OBJECTIVE: A retrospective chart review of patients seen in the Allergy Section at The Children's Hospital of Philadelphia with International Classification of Diseases Ninth Revision code of 558.3 (Allergic Gastroenteritis and Colitis) between 2007 and 2012 was conducted to identify patients with suspected FPIES. Diagnosis of FPIES was confirmed based on meeting clinical criteria of delayed reaction with pronounced vomiting and/or diarrhea. Data regarding patient characteristics and features of their reactions were collected for analysis and comparison with existing studies. RESULTS: A total of 462 cases were identified in our chart review. Patients had a similar demographic profile to the normal allergy patients seen in our clinic. The most common foods identified were milk (67%), soy (41%), rice (19%), oat (16%), and egg (11%). Patients had onset of FPIES to milk and soy around 7 months of age compared with 12 months of age for solid foods. FPIES reactions were identified to meats, tree nuts, peanuts, fruits, and vegetables; 70% of the patients reacted to one or two foods. Skin prick testing and atopy patch testing were not helpful in identifying the foods. CONCLUSION: FPIES reactions were seen more frequently than previously described. However, the presentation and clinical features were similar to previous reports. Milk- and soy-triggered FPIES were common, and 43.5% of patients who had a milk trigger reacted to soy. There is no laboratory test to identify foods that cause FPIES, and clinician-supervised oral food challenge is the only definitive test available.

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.