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.

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.

A Case of Sepsis and Acute Renal Failure Associated with Salmonella Enterocolitis / 대한소화기학회지

Salmonella infection can cause an asymptomatic intestinal carrier state or clinical diseases such as enterocolitis presenting abdominal pain, fever, vomiting, or diarrhea. Salmonella usually invades Peyer's patch of terminal ileum or ascending colon. Sepsis is not common and acute renal failure secondary to rhabdomyolysis is rare. The causes of rhabdomyolysis are trauma, excessive exercise, alcohol, seizure, metabolic abnormality, and infection. Infections account for less than 5% of the reported causes of rhabdomyolysis and resulting acute renal failure. The mechanisms underlying rhabdomyolysis due to infection are direct muscle invasion, toxin production, and nonspecific effects that can occur with infections such as fever, dehydration, acidosis, and electrolyte imbalance. We report a case of sepsis and acute renal failure secondary to rhabdomyolysis associated with Salmonella infection.

[Erythema nodosum: profile in a Tunisian teaching hospital]

Background: Erythema nodosum [EN] is the most frequent acute nodular hypodermatitis. Etiologies of EN are varied although a similar clinical presentation

The aim of our study is to establish epidemio-clinical characteristics of EN in 103 patients

Methods: [62 in care [January 1980-december 2005] were seen on the dermatology unit of the Rabta hospital. We have only included patients that had done: blood count, chest X-Ray, ASLO, tuberculin intradermoreaction, transaminase, inflammation marker

Results: We had 86 women and 17 men with a mean age of 36,5 years. Lesions were located in lower limbs in 88 cases. The man etiologies were: post streptococcal infections [42 cases], tuberculosis [8 cases], Crohn disease [8 cases], Behcet disease [7 cases] and sarcoidosis [5 cases]. EN was idiopathic in 23 patients. The most common provoking agent of EN varies from a country to another and even in the same country from a study to another. In Tunisia, post streptococcal infection and Behcet disease still frequent etiologies for EN, sarcoidosis and inflammatory colites are more and more found unlike tuberculosis which is less incriminated than before. In case of EN it is reasonable to begin etiological enquiry by common causes

A Case of Severe Protein-losing Enteropathy as a Late Complication of Pelvic Irradiation

Department of Protein-losing enteropathy is the manifestation of a diverse set of disorders, and it is characterized by the excessive loss of plasma proteins into the affected portions of the gastrointestinal tract, and this results in hypoalbuminemia. We report here on a case of severe protein-losing enteropathy with the typical clinical features of hypoalbuminemia, dependent edema and increased alpha 1-antitrypsin (alpha1-AT) clearance, as measured by using 24hr stool testing. The associated disorder with the protein-losing enteropathy of our case was radiation enterocolitis and lymphatic obstruction that was due to radiation treatment and lymph node dissection in the remote past for the treatment of uterine cervical carcinoma. Our case suggests that chronic radiation enterocolitis can result in irreversible injury to the intestinal mucosa and a protein-losing enteropathy, which can bring about a very poor quality of life and even the loss of life.

A Case of Methicillin Resistant Staphylococcus Aureus Enterocolitis Presenting with Massive Diarrhea / 대한소화기학회지

Methicillin resistant Staphylococcal aureus (MRSA) enterocolitis is characterized by high fever, abdominal distension, and watery diarrhea that leads to severe dehydration, shock, a sharp decrease in the white cell counts and sometimes multiple organ failure. Clinically, it can be an another cause of nosocomial diarrhea. If MRSA enteritis is suspected from the clinical symptoms, prompt treatment and strict prophylactic measures including vancomycin, are most important for its management. We recently observed a case of MRSA enterocolitis as a nosocomial infection in a patient with acute pancreatitis. This patient showed uncontrolled massive diarrhea, fever, and multiple organ failure. We report a case of MRSA enterocolitis with a review of literatures.

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

Newer diarrheal syndromes.

Aetiological diagnosis of protracted diarrhoea remains obscure in as many as 30% of cases despite extensive investigations. A number of newer syndromes have been recognized amongst this "idiopathic group" which includes microvillous inclusion disease, "tufting" enteropathy and epithelial dysplasia, autoimmune enteropathy and "syndromic" immunodeficiency with characteristic facial abnormalities, woolly hair and intractable diarrhea. The molecular basis of some of these syndromes has been reviewed but in only a small series of patients has the functional defect been characterized. If a case is suspected the antenatal history, family history and history of consanguinity should be sought. Extra-intestinal manifestations, presence of gut or other auto-antibodies, together with phenotypic abnormalities should be looked for. Careful light and electron microscopy is done of small bowel biopsies, although microvillous inclusion disease can be usually suspected on PAS staining. Large bowel biopsy may be needed to exclude an unsuspected microscopic colitis. The prognosis of this group of conditions is poor with an overall 50-85% mortality. Although successful gut transplantation has been reported, genetic counselling may be one of the more important aspects of the clinicians' role.

Enterocolitis necrosante en el recien nacido / Necrotizing enterocolitis in the newborn

Determinar las características de neonatos con riesgo a presentar enterocolitis necrosante y los factores que influyen en la elevada mortalidad relacionada con esta patología. Estudio prospectivo, longitudinal y sin exclusión de 47 neonatos con diagnóstico de enterocolitis necrosante. La enterocolitis necrosante se presenta con más frecuencia entre la población de prematuros pequeños para su edad de gestación. La mortalidad fue del 70,21 por ciento significativamente mayor que la mortalidad neonatal institucional. En el grupo de los a término murieron el 100 por ciento y se correlacionó con antecedente de asfixia perinatal. La enterocolitis necrosante es una entidad usualmente fatal, excepto en aquellos casos catalogados como estadio I. No encontramos relación significante entre los factores de riesgo analizados y mortalidad. La prevención es el mejor método para disminuir su incidencia. En nuestro hospital se deben establecer pautas de su asistencia médico-quirúrgica

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