ABSTRACT
La enfermedad de Ménétrier es una gastroenteropatía perdedora de proteínas. Definida como una entidad rara y de causa desconocida, la mayoría de los casos reportados la han asociado a infecciones virales. En los pacientes pediátricos, presenta un comienzo agudo con un curso benigno y autolimitado. Se caracteriza por tener pliegues gástricos engrosados que, generalmente, involucran el cuerpo y el fundus gástrico, asociados a hipoalbuminemia, debido a la pérdida de proteína sérica a través de la mucosa. A continuación, se exponen dos casos clínicos de síndrome de Ménétrier infantil asociado a infección por citomegalovirus.
Ménétrier's disease is a protein losing gastroenteropathy. Defined as a rare entity with an unknown cause, most of the reported cases have been associated with viral infections. In pediatric patients, it is characterized by an acute onset with a benign and self-limiting course. It is characterized by thickened gastric folds that generally involve the body and the gastric fundus, associated with hypoalbuminemia due to the loss of serum protein through the mucosa. The following are two clinical cases of infant Ménétrier syndrome associated with cytomegalovirus infection.
Subject(s)
Humans , Male , Infant , Child, Preschool , Protein-Losing Enteropathies , Stomach Diseases , Cytomegalovirus , Gastritis, HypertrophicABSTRACT
lntrodução: a estrongiloidíase tem grande importância médica devido à capacidade de o Strongyloides stercoralis completar seu ciclo de vida no homem e gerar a síndrome de hiperinfecção principalmente em imunocomprometidos. Devido à dificuldade em estruturar a resposta Th2, os pacientes infectados com o Vírus Linfotrópico de Células T Humanas Tipo 1 (HTLV-1) têm maior tendência a apresentar infecção maciça. A leishmaniose visceral, doença relevante em países em desenvolvimento, causa alterações imunológicas semelhantes, porém há poucos relatos de suscetibilidade específica ao Strongyloides stercoralis nos infectados por Leishmania sp. O presente trabalho tem objetivo de relatar um caso de coinfecção HTLV e calazar, que apresentou-se como pancreatite aguda e enteropatia perdedora de proteínas secundárias à estrongiloidíase maciça. Relato de caso: trata-se de um paciente de 34 anos com história de leishmaniose prévia que deu entrada no nosso Serviço com pancreatite aguda idiopática leve, além de história de diarreia crônica há um ano com anasarca e hipoalbuminemia associadas. Apresentou endoscopia digestiva alta com atrofia duodenal importante, tendo sido identificados Strongyloides stercoralis em biópsia, além de sorologia para HTLV positiva. Apresentou translocação bacteriana com sepse grave de foco abdominal, após início do tratamento com ivermectina, tendo posteriormente evoluído com melhora clínica importante e remissão dos sintomas. Fez investigação com punção de medula óssea, em que foram identificadas as formas amastigotas da leishmania. Discussão e conclusão: a presença de HLTV é um fator de risco para a síndrome de hiperinfecção por Strongyloides stercoralis, tendo predisposto o paciente às manifestações graves e raras descritas. A identificação de leishmania na medula óssea, entretanto, é um fator de risco ainda pouco conhecido para estrongiloidíase disseminada, porém com plausibilidade biológica por afetar o sistema imunológico do hospedeiro.(AU)
Introduction: strongyloidiasis has great medical importance because of the ability of the Strongyloides stercoralis to complete its life cycle in man and cause hyperinfection syndrome especially in immunocompromised hosts. Because of the difficulty in triggering The response, Human T-cell lymphotropic virus type 1 (HTLV-1) infected patients has susceptibility for massive infection. Visceral leishmaniasis, a relevant disease in developing countries, causes similar immunological changes, but there are few reports of specific susceptibility to Strongyloides stercoralis on infected by Leishmania sp. This study aimed to report a case of HTLV and kala azar coinfection, presenting as acute pancreatitis and protein losing enteropathy secondary to massive strongyloidiasis. Case report: a 34-year-old patient previously treated for leishmaniasis has presented at our service with idiopathic acute pancreatitis and chronic diarrhea for one year with anasarca and hypoalbuminemia. Upper endoscopy revealed duodenal atrophy in which biopsy identified Strongyloides stercoralis, and HLTV serology was positive. He presented with bacterial translocation and severe sepsis after first dose of ivermectin, but has clinical improvement and remission of symptoms afterwards. Bone marrow aspiration identified amastigote forms of Leishmania. Discussion and Conclusion: the presence of HLTV is a risk factor for Strongyloides stercoralis hyperinfection, and predisposed this patient to the serious and rare events described. The identification of Leishmania in bone marrow, however, is an poorly known risk factor for disseminated strongyloidiasis, but with biological plausibility because it affects the immune system of the host.(AU)
Subject(s)
Humans , Male , Adult , Pancreatitis , Protein-Losing Enteropathies , Strongyloidiasis , Human T-lymphotropic virus 1 , Leishmaniasis, Visceral , Pancreatitis, Acute NecrotizingABSTRACT
Background: Protein-losing enteropathy (PLE) is a known postoperative complication affecting about 10% of patients surgically managed with Fontan procedure. The mortality rate associated with this complication increases to 50%. Objective: To determine the risk factors associated to the development of PLE in patients surgically managed with Fontan procedure. Methods: This was a case-cohort study, and the universe of the trial comprised all patients treated with univentricular surgery. We included male and female patients with congenital heart disease that conditioned a single ventricle syndrome. Those patients with previous intestinal disease causing protein loss, were excluded, cow's milk protein allergy, intestinal resection (previous or after heart surgery), use of cyclic parenteral nutrition or Fontan's dismantlement. Follow-up began immediately after hospital discharge from Fontan procedure. Outcome variable was the development of PLE; independent variables were some before and after surgery hemodynamic and echocardiographic variables, infections and treatment. Statistical analysis: We used measures of statistical dispersion and central tendency. Risk was calculated for each variable estimating the hazard ratio (HR), adjusted for confounding factors; and Kaplan-Meier estimator was used for survival analysis. Results: Eleven (26%) out from patients 42 developed PLE. The median of time between Fontan procedure and the development of this complication was five years. The prognostic variables were: systolic pressure of pulmonary artery between 12-15 mmHg, > 3 years between Glenn and Fontan procedures, aggravated chronic malnutrition, direct bilirubin values > 1.5 mg/dL, pulmonary resistances (APR) between 3-3.5 Wood units, previous hepatomegaly and pleural effusion > 6 day-period. The probability of dying from PLE was 63% in a 10-year period. Conclusions: The prognostic factors associated with PLE are previous hepatic damage and borderlines values of venous pressure.
Antecedentes: La enteropatía perdedora de proteínas (EPP) es una conocida complicación que afecta alrededor del 10% de los sujetos operados con el procedimiento de Fontan. La mortalidad asociada a esta complicación se eleva al 50%. Objetivo: Determinar los factores de riesgo asociados al desarrollo de EPP en pacientes postoperados de procedimiento de Fontan. Métodos: Este es un estudio de caso-cohorte y el universo comprendió a todos los pacientes corregidos con cirugía univentricular. Se incluyeron pacientes de ambos géneros, con cardiopatías que condicionaran síndrome de ventrículo único. Se excluyeron aquellos con enfermedad previa intestinal causante de pérdida de proteínas, alergia a las proteínas de la leche de vaca, resección intestinal (previa o después de la cirugía cardiaca), aquellos con nutrición parenteral cíclica o desmantelamiento del Fontan. El inicio de seguimiento comenzó inmediatamente después del egreso de la cirugía de Fontan. La variable de desenlace fue el desarrollo de enteropatía perdedora de proteínas. Las variables independientes estudiadas fueron algunas variables hemodinámicas y ecocardiográficas pre- y postquirúrgicas, infecciones y tratamiento. Análisis estadístico: Se usaron medidas de dispersión y tendencia central. Se calculó el riesgo por cada variable, estimando el cociente de riesgo (Hazard Ratio, HR en inglés), ajustándose a variables de confusión. Se utilizó el estimador de Kaplan-Meier para el análisis de supervivencia. Resultados: Once de 42 pacientes (26%) desarrollaron EPP. La mediana de tiempo entre la cirugía de Fontan y el desarrollo de esta complicación fue de cinco años. Las variables pronósticas fueron presión sistólica de la arteria pulmonar entre 12-15 mmHg, el tiempo > 3 años entre las intervenciones de Glenn y Fontan, la desnutrición crónica agudizada, una cifra de bilirrubina directa > 1.5 mg/dL, URP entre 3 y 3.5 Unidades Wood, hepatomegalia previa y derrame > 6 días. La probabilidad de mortalidad al desarrollar EPP a 10 años fue de 63%. Conclusiones: Los factores pronósticos asociados a EPP son el daño hepático previo y las variables limítrofes de presión venosa.
ABSTRACT
INTRODU: la enteropatía perdedora de proteínas puede aparecer en la evolución de los pacientes con corazón univentricular que sobreviven a la derivación cavopulmonar total. Una vez que se diagnostica, la mortalidad es alta. OBJETIVO: identificar los posibles factores de riesgo de esta complicación. MÉTODOS: se realizó un estudio de cohorte prospectivo de la evolución en 74 pacientes con derivación cavopulmonar total, intervenidos en el Cardiocentro Pediátrico "William Soler", desde enero de 1992 hasta enero de 2011. RESULTADOS: el tiempo promedio de evolución fue de 8 años. Sufrió enteropatía perdedora de proteínas 8,1 % de los pacientes. Se presentó con mayor frecuencia en los operados con la técnica intratrial, en los operados con más de 6 años de edad, y en quienes sufrieron derrames pleurales persistentes en el posoperatorio inmediato. Se encontró relación significativa entre la enteropatía y la disfunción ventricular posoperatoria, con RR= 11,45 (IC: 95 %: 2,37 a 55,16). El análisis multivariado identificó a la disfunción ventricular como factor de riesgo. CONCLUSIÓN: la detección de disfunción ventricular en la evolución del paciente con derivación cavopulmonar debe orientar el tratamiento, en aras de evitar la aparición de enteropatía perdedora de proteínas.
INTRODUCTION: protein-losing enteropathy may occur in the progression of patients with univentricular heart, who survived total cavopulmonary shunt. Once diagnosed, the mortality rate of the condition is high. ONJECTIVE: to identify the possible risk factor of this complication. METHODS: a prospective cohort study of the progression of 74 patients with total cavopulmonary shunt was conducted from January 1992 through January 2011. They had been operated on at "William Soler" pediatric cardiac center. RESULTS: the average time of progression was 8 years. In this group, 8.1 % of patients suffered protein-losing enteropathy that was more frequently seen in patients operated on by the intraatrial technique, aged over 6 years and in those suffering persistent pleural effusion in the immediate postoperative period. Significant statistical relation was found between enteropathy and postoperative ventricular dysfunction with RR= 11.45 (CI: 95 %: 2.37 to 55.16). The multivariate analysis showed that the ventricular dysfunction was a risk factor. CONCLUSIONS: Detection of the ventricular dysfunction in the progression of a patient with cavopulmonary shunt should guide the treatment to avoid occurrence of protein-losing enteropathy.
Subject(s)
Humans , Protein-Losing Enteropathies/complications , Protein-Losing Enteropathies/etiology , Protein-Losing Enteropathies/prevention & control , Ventricular Dysfunction/prevention & control , Fontan Procedure/adverse effects , Fontan Procedure/methods , Prospective Studies , Cohort StudiesABSTRACT
A propósito del caso de un paciente de 7 meses de vida remitido desde Yopal a la ciudad de Bogotá, se revisa el tema de la linfagiectasia intestinal. Esta es una rara enfermedad que involucra los vasos linfáticos intestinales, y origina hipoproteinemia, edemas, ascitis y enteropatía perdedora de proteínas.
This is the case report of a 7 month old child from Yopal with intestinal lymphangiectasia who was sent to Bogota. We also review the issue of intestinal lymphangiectasia, a rare disease involving intestinal lymphatic vessels which caused hypoproteinemia, edema, ascites and protein-losing enteropathy.
Subject(s)
Humans , Male , Infant, Newborn , Ascites , Hypoproteinemia , Lymphangiectasis, Intestinal , Protein-Losing EnteropathiesABSTRACT
La linfangiectasia intestinal primaria es una enfermedad caracterizada por dilatación de los vasos linfáticos del intestino, manifestándose como una enteropatía perdedora de proteínas. Presentamos un paciente con diarrea crónica, prolapso rectal recurrente y hemihipertrofia de miembro superior izquierdo, asociado a linfopenia e hipoalbuminemia. Por endoscopia digestiva superior y biopsia se diagnostica linfangiectasia intestinal primaria y se inicia tratamiento nutricional exitosamente.
Primary intestinal lymphangiectasia is a disease characterized by dilated intestinal lymph vessels which manifests as a protein losing enteropathy. We present a patient with chronic diarrhea, recurrent rectal prolapse and left upper limb hemihypertrophy associated with lymphopenia and hypoalbuminemia. Primary intestinal lymphangiectasia was diagnosed with upper endoscopy and biopsy. Nutritional treatment was successfully begun.
Subject(s)
Humans , Male , Child, Preschool , Lymphangiectasis, Intestinal , Nutrition Therapy , Protein-Losing EnteropathiesABSTRACT
OBJECTIVE: The aim of the study is to evaluate paediatric patients with protein losing enteropathy (PLE). METHODS: Fourteen cases diagnosed as PLE were evaluated in terms of aetiologies, diagnostic methods, laboratory findings, treatment procedures and longterm prognosis. RESULTS: Four of the cases had coeliac disease, three intestinal lymphangiectasia, three giardia infection, one H pylori infection and three cytomegalovirus (CMV) infection. Histopathological examinations of duodenum specimens revealed total villous atrophy in four cases, lymphatic dilatation in three cases, severe nodular appearance in four cases and no pathology in four cases. All of the cases except patients with intestinal lymphangiectasia were controlled by the appropriate treatment given for the underlying disease. The cases with CMV infection were treated with only supportive treatment and gancyclovir therapy was not needed. CONCLUSION: When proteinuria is not detected in wellappearing children admitted with oedema, PLE must be considered.
OBJETIVO: El objetivo del estudio es evaluar a pacientes con enteropatía perdedora de proteínas (EPP). MÉTODOS: Catorce casos diagnosticados con EPP fueron evaluados en términos de etiologías, métodos de diagnóstico, resultados de laboratorio, procedimientos de tratamiento, y prognósis a largo plazo. RESULTADOS: Cuatro de los casos tenían enfermedad celíaca, tres padecían de linfangiectasia intestinal, tres sufrían de infección por giardias, uno tenía infección por H pylori, y tres presentaba infección por citomegalovirus (CMV). Los exámenes histopatológicos de especímenes duodenales revelaron atrofia de las vellosidades intestinales en cuatro de los casos, dilatación linfática en tres casos, apariencia nodular severa en cuatro casos, y ausencia de patología en cuatro casos. Todos los casos - excepto los pacientes con linfangiectasia intestinal - fueron controlados mediante el tratamiento adecuado para la enfermedad subyacente. Los casos con infección por CMV fueron tratados con tratamiento de apoyo, y no se necesitó terapia con ganciclovir. CONCLUSIÓN: Cuando no se detecta proteinuria en niños con buena apariencia ingresados con edema, hay que considerar principalmente la posibilidad de EPP.