Síndrome urémico hemolítico del adulto / Adult hemolytic uremic syndrome

El síndrome urémico hemolítico (SUH), descripto en 1955, se caracteriza por la tríada de anemia hemolítica no inmunomediada, trombocitopenia y lesión renal aguda. En su patogenia interviene la toxina Shiga, producida con mayor frecuencia por E. coli O157:H. Puede manifestarse a cualquier edad, aunque es infrecuente en adultos, y se desarrolla en forma esporádica o en brote. Se presenta con un cuadro de dolor abdominal, diarrea, fiebre y vómitos. Puede afectar el sistema nervioso central, pulmones, páncreas y corazón. En adultos, el síndrome evoluciona tras un período de incubación de 1 semana posterior a la diarrea y tiene alta morbimortalidad, a diferencia de los casos pediátricos. Presentamos el caso de una paciente adulta, que cursó internación por síndrome urémico hemolítico. (AU)

Hemolytic uremic syndrome (HUS), described in 1955, is characterized by the triad of non-immune mediated hemolytic anemia, thrombocytopenia, and acute kidney injury. Shiga toxin, produced most frequently by E coli O157:H, is involved in its pathogenesis. Hus can manifest at any age, although it is rare in adults and develops sporadically or in outbreaks. HUS presents with a picture of abdominal pain, diarrhea, fever and vomiting. It can affect the central nervous system, lungs, pancreas, and heart.In adults, the syndrome evolves after an incubation period of 1 week after diarrhea, with high morbidity and mortality, unlike pediatric cases.We present the case of an adult patient who was hospitalized for hemolytic uremic syndrome. (AU)

Síndrome urémico hemolítico: reporte de dos casos pediátricos con nexo epidemiológico / Hemolytic uremic syndrome: report of two pediatric clinical cases with epidemiologic link / Síndrome urémico hemolítico: reporte de casos pediátricos con nexo epidemiológico

Introducción: el síndrome hemolítico urémico (SHU) es en muchos países, de las causas más frecuentes de insuficiencia renal aguda. La mayoría de los casos ocurre luego de un episodio de gastroenteritis aguda (GEA) por Escherichia coli productora de toxina Shiga (STEC). En Uruguay a pesar de ser una enfermedad de notificación obligatoria, existe subregistro. Objetivo: describir dos casos clínicos de SHU asociados a GEA con nexo epidemiológico. Casos clínicos: se trata de dos varones de 4 y 5 años, sanos. En los días previos, ingesta de carne en el mismo local comercial. Consultaron por dolor abdominal, deposiciones líquidas y vómitos reiterados. El niño de 4 años presentaba fiebre y deposiciones líquidas con sangre. El niño de 5 años dolor abdominal. El estado de hidratación y las constantes vitales eran normales en ambos. Fueron admitidos a cuidados moderados. A las 48 horas y a los 5 días, respectivamente, agregan palidez cutáneo-mucosa intensa, edemas y oliguria. Estudios complementarios: anemia, plaquetopenia e insuficiencia renal. Ingresaron a cuidados intensivos y se realizó diálisis peritoneal. La investigación de STEC fue negativa y la evolución favorable. Conclusiones: en menores de 5 años el SHU asociado a GEA es la forma de enfermedad más frecuente. En Uruguay predominan las cepas STEC no-O157. En estos casos no se pudo identificar el agente. La existencia de un nexo epidemiológico alerta sobre la necesidad de extremar los cuidados en la preparación y cocción de la carne. Debido a la asociación con una enfermedad prevalente, es necesario tener presente esta complicación para poder sospecharla e iniciar el tratamiento en forma precoz y oportuna.

Introduction: hemolytic uremic syndrome (HUS) is one of the most frequent causes of acute renal failure in many countries. Most cases occur after an episode of acute gastroenteritis (GEA) due to the Shiga toxin producing Escherichia Soli (STEC). In Uruguay, despite being a disease that requires mandatory notification, it is under reported. Objective: to describe two clinical cases of HUS associated with GEA with an epidemiological link. Clinical cases: these are two healthy boys aged 4 and 5 years. In the previous days, they reported meat intake in the same commercial premises. They consulted for abdominal pain, liquid stools and repeated vomiting. The 4 year old boy had a fever and bloody stools. The 5 year old boy had abdominal pain. They both showed normal hydration levels and vital signs. They were admitted to moderate care. At 48 hours and 5 days, respectively, they showed intense skin and mucosal paleness, edema and oliguria. Complementary studies: anemia, thrombocytopenia and renal failure. They were admitted to intensive care and peritoneal dialysis was performed. The STEC's investigation was negative and the evolution favorable. Conclusions: in children under 5 years of age, HUS associated with GEA is the most frequent form of the disease. In Uruguay, non-O157 STEC strains predominate. In these cases, the agent could not be identified. The existence of an epidemiological link warns us about the need for extreme care in the preparation and cooking of meat. Due to the association with a prevalent disease, it is necessary to keep this complication in mind in order to suspect it and initiate early and timely treatment.

Introdução: a síndrome hemolítico urêmica (SHU) é uma das causas mais frequentes de insuficiência renal aguda em muitos países. A maioria dos casos ocorre após um episódio de gastroenterite aguda (GEA) devido à Escherichia Coli, a toxina produtora de Shiga (STEC). No Uruguai, apesar de ser uma doença de notificação compulsória, há subnotificação. Objetivo: descrever dois casos clínicos de SHU associada à AGE com vínculo epidemiológico. Casos clínicos: dois meninos saudáveis com idades entre 4 e 5 anos. Nos dias anteriores, eles reportaram consumo de carne nos mesmos estabe- lecimentos comerciais. Eles consultaram para dor abdominal, fezes líquidas e vômitos repetidos. O menino de 4 anos teve febre e fezes com sangue. O menino de 5 anos teve dores abdominais. O estado de hidratação e os sinais vitais foram normais em ambos meninos. Foram internados em cuidados moderados. Às 48 horas e 5 dias, respectivamente, apresentaram aliás palidez intensa da pele e mucosas, edema e oligúria. Realizaramse estudos complementares: anemia, trombocitopenia e insuficiência renal. Eles foram internados em terapia intensiva e realizouse diálise peritoneal. A investigação do STEC foi negativa e a evolução favorável. Conclusões: em crianças menores de 5 anos, a SHU associada à GEA é a forma mais frequente da doença. No Uruguai, predominam cepas STEC não-O157. Nesses casos, o agente não pôde ser identificado. A existência de um nexo epidemiológico alerta para a necessidade de extremo cuidado no preparo e cozimento da carne. Devido à associação com doença prevalente, é necessário considerar essa complicação para suspeitar e iniciar o tratamento precoce e oportunamente.

Research Advances on the Pathogenesis and Treatment of Hemolytic Uremic Syndrome --Review / 中国实验血液学杂志

Hemolytic uremic syndrome (HUS) is clinically rare, with high mortality and case fatality rates. In recent years, the research on HUS has been intensified and the pathophysiological mechanism has been continuously improved. At present, the main mechanism of pathogenesis is the excessive activation of complement alternative pathways mediated by complement-related gene mutations or the existence of antibodies. The treatment methods and strategies are also constantly updated, mainly including complement-blocking drugs such as Eculizumab, Lavalizumab, and Ravulizumab. In this review, the new developments in the pathogenesis and treatment of HUS is summarized, and provide references for the clinical treatment of HUS.

Hemolytic uremic syndrome associated with streptococcus pneumoniae in pediatrics: a case series / Síndrome hemolítico-urêmica associada com streptococcus pneumoniae em pediatria: série de casos

ABSTRACT Objective: To describe a case series of four (4) patients with hemolytic uremic syndrome due to Streptococcus pneumoniae in a level four complexity institution in the city of Bogotá, D.C., Colombia. Cases description: We describe cases of four patients who presented respiratory symptoms and fever. All four patients were in regular conditions on hospital admission, after which they required intensive care and ventilatory support. Upon admission, three cases showed evidence of pleuropulmonary complication. Penicillin-sensitive Streptococcus pneumoniae was isolated in all cases. All patients presented anemia, severe thrombocytopenia, schistocytes on peripheral blood smear, and hyperazotemia. They required blood transfusion and renal replacement therapy during their hospitalization. The patients were diagnosed with hemolytic uremic syndrome due to S. pneumoniae. Three of the four patients had a progressive recovery of the renal function and were discharged after an average of 36 days of hospital stay. The remaining patient had two amputations in the extremities due to thrombotic vascular complications and was discharged after 99 days of hospital stay, requiring hemodialysis every other day. Comments: Hemolytic uremic syndrome due to Streptococcus pneumoniae is a rare but severe complication of invasive pneumococcal disease. Complicated pneumonia is the main condition associated with this entity. It is noteworthy the short period in which these cases were presented, considering the low annual incidence of the disease.

RESUMO Objetivo: Descrever uma série de casos de quatro pacientes com síndrome hemolítico-urêmica por pneumococo em uma instituição de referência em Bogotá, Colômbia. Descrição dos casos: Descrevemos os casos de quatro pacientes que apresentaram sintomas respiratórios e febre. Todos estavam em estado geral regular à admissão hospitalar e necessitaram de cuidados intensivos e suporte ventilatório. Na admissão, em três dos casos foi evidenciada a complicação pleuropulmonar. Isolamento de Streptococcus pneumoniae sensível à penicilina foi realizado em todos os casos. Os quatro pacientes precisaram de transfusão sanguínea e terapia de reposição renal durante a hospitalização. Nos testes laboratoriais, observou-se anemia, trombocitopenia grave, presença de esquizócitos em esfregaço de sangue periférico e hiperazotemia. Com esse quadro, o diagnóstico foi de síndrome hemolítico-urêmica associada à infecção por S. pneumoniae. Houve recuperação progressiva da função renal em três dos quatro pacientes, que tiveram alta após 36 dias de internação hospitalar, em média. Um paciente teve complicações vasculares trombóticas, resultando em duas amputações nas extremidades, e teve alta após 99 dias de internação, com necessidade de hemodiálise em dias alternados. Comentários: A síndrome hemolítico-urêmica por Streptococcus pneumoniae é uma complicação rara, mas grave, da doença invasiva pneumocócica. A pneumonia complicada é a principal condição associada a essa entidade. Destaca-se o curto período em que esses casos foram apresentados, levando em conta a baixa incidência anual de síndrome hemolítico-urêmica.

Síndrome hemolítico urémico asociado a Shigatoxina: ¿Cómo prevenirlo? / Shiga-toxin associated hemolytic uremic syndrome: how to prevent it?

Resumen: El síndrome hemolítico urémico (SHU) asociado a infección intestinal por bacterias productoras de Shigatoxina, que afecta principalmente a población infantil, puede causar morbilidad aguda grave, secuelas crónicas en varios órganos, y la muerte prematura en algunos de ellos. Dado su carácter zoonótico, adecuadas medidas de manejo agropecuario y correcta higiene de lo que consumimos es indispensable a la hora de prevenir la infección. Actualmente, una vez gatillado el SHU el manejo es médico y, principalmente, de soporte. En los últimos años diversas estrategias terapéuticas se han ido desarrollando para evitar que esta enfermedad ocurra, o, al menos, que pueda ser atenuada en sus consecuencias de morbi-mortalidad. El presente artículo describe acciones específicas a diferentes niveles de prevención de esta patología.

Abstract Hemolytic uremic syndrome (HUS) associated with intestinal infection by Shiga toxin-producing bacteria, which mainly affects children, can cause severe acute morbidity, chronic sequelae in seve ral organs, and premature death in some of them. Given its zoonotic nature, adequate measures of agricultural management and proper hygiene of what we consume are essential to prevent infection. Once the HUS is triggered, medical management is currently mainly supportive. In recent years, va rious therapeutic strategies have been developed to prevent this disease from occurring or, at least, to mitigate its morbidity and mortality consequences. This article describes specific actions at different levels of prevention of this pathology.

Síndrome urémico hemolítico atípico asociado a neumococo / Pneumococcal hemolytic uremic syndrome

RESUMEN El síndrome urémico hemolítico (SUH) es una entidad caracterizada por la tríada de insuficiencia renal aguda, anemia hemolítica no inmune y trombocitopenia. Se distinguen 2 tipos: el SUH típico o D (+) asociado a toxina Shiga y el atípico o D(-) entre los que se encuentra el SUH asociado a neumococo. Ésta es una patología poco común con alta morbimortalidad. Se presenta el caso clínico de un lactante que desarrolló un SUH asociado a neumococo en el marco de enfermedad neumocóccica invasiva

ABSTRACT Hemolytic uremic syndrome (HUS) is an entity characterized by the triad of acute renal failure, nonimmune hemolytic anemia and thrombocytopenia. Two types are distinguished: the typical HUS or D (+) related to Shiga toxin and the atypical or D (-) among which the HUS associated with pneumococcus. This is an uncommon pathology with high morbidity and mortality. We present the case of an infant who developed an HUS associated with pneumococcus in the contex of invasive pneumococcal disease

Microalbuminuria en pacientes pediátricos con diagnóstico de síndrome hemolítico urémico / Microalbuminuria in pediatric patients diagnosed with hemolytic uremic syndrome

Introducción: El síndrome hemolítico urémico (SHU) se caracteriza por la presencia de anemia hemolítica microangiopática, trombocitopenia y afectación renal aguda. Es la principal causa de falla renal aguda en niños menores de 3 años. Un número variable de pacientes evoluciona con afectación renal a largo plazo con proteinuria, hipertensión arterial e insuficiencia renal crónica. Objetivo: Evaluar la afectación renal mediante el índice microalbuminuria/creatininuria en pacientes pediátricos con diagnóstico de SHU. Pacientes y Método: Estudio descriptivo de cohorte concurrente que analizó la presencia de microalbuminuria en pacientes diagnosticados de SHU entre enero de 2001 y marzo de 2012, que evolucionaron sin hipertensión y con función renal normal (clearance mayor de 90 ml/min medido por fórmula de Schwartz). Se evaluaron factores demográficos (edad, sexo), presentación clínica en el momento del diagnóstico, uso de antibióticos previo al ingreso y requerimiento de terapia de reemplazo renal. Resultados: Se estudiaron 24 pacientes, el 54% varones; la edad promedio en el momento del diagnóstico fue de 2 años; un 45% requirió diálisis peritoneal; un 33% evolucionó con microalbuminuria persistente; cuatro pacientes recibieron tratamiento antiproteinúrico con buena respuesta. El promedio de seguimiento fue de 6 años (rango: 6 meses a 11 años); todos los pacientes durante el seguimiento evolucionaron con creatinina plasmática normal. Conclusiones: En nuestro grupo, el porcentaje de microalbuminuria persistente en pacientes con diagnóstico previo de SHU fue similiar a lo descrito en la literatura; el tratamiento con antiproteinúricos podría retrasar el daño renal, pero es necesario realizar estudios prospectivos multicéntricos.

Introduction: Hemolytic uremic syndrome (HUS) is characterized by the presence of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney failure. It is the leading cause of acute kidney failure in children under 3 years of age. A variable number of patients develop proteinuria, hypertension, and chronic renal failure. Objective: To evaluate the renal involvement in pediatric patients diagnosed with HUS using the microalbumin/creatinine ratio. Patients and Methods: Descriptive concurrent cohort study that analyzed the presence of microalbuminuria in patients diagnosed with HUS between January 2001 and March 2012, who evolved without hypertension and normal renal function (clearance greater than 90 ml/min using Schwartz formula). Demographic factors (age, sex), clinical presentation at time of diagnosis, use of antibiotics prior to admission, and need for renal replacement therapy were evaluated. Results: Of the 24 patients studied, 54% were male. The mean age at diagnosis was two years. Peritoneal dialysis was required in 45%, and 33% developed persistent microalbuminuria. Antiproteinuric treatment was introduce in 4 patients, with good response. The mean follow-up was 6 years (range 6 months to 11 years). The serum creatinine returned to normal in all patients during follow up. Conclusions: The percentage of persistent microalbuminuria found in patients with a previous diagnosis of HUS was similar in our group to that described in the literature. Antiproteinuric treatment could delay kidney damage, but further multicenter prospective studies are necessary.

Tos convulsa, causa extraña de síndrome hemolítico urémico / Whooping cough, unusual cause of hemolytic uremic syndrome

La tos convulsa es una enfermedad infecto-contagiosa producida por Bordetella pertussis y Parapertussis. El principal reservorio son los adultos jóvenes. Los pacientes más susceptibles de presentar una forma grave de esta enfermedad son los lactantes menores de 6 meses. El síndrome urémico hemolítico es una complicación rara de la tos convulsa. La patogenia del mismo no ha sido aclarada, planteándose que el mismo es secundario al efecto de las toxinas producidas por Bordetella pertussis y por fenómenos inflamatorios. En la literatura internacional son escasos los reportes de síndrome urémico hemolítico asociado a tos convulsa y no se ha podido establecer cuáles son los factores de riesgo para el desarrollo del mismo. Se presenta el caso de una lactante que desarrolló insuficiencia renal aguda secundaria a un síndrome urémico hemolítico en el curso de una infección aguda por Bordetella pertussis que requirió terapia de sustitución de la función renal.

Whopping cough is an infectous disease caused by Bordetella pertussis and Parapertussis. The main reservoir of Bordetella are adolescents and adults. Infants younger than 6 months have increased risk for developing a severe illnes with a lethal evolution. Hemolytic uremic syndrome is an unusual complication of whopping cough. The pathogenic mechanisms have not been clarified. One of the hypothesis has established the role of Bordetella toxins and inflammatory mediators. There are few cases published of hemolytic uremic syndrome associated to whopping cough and risk factors for its development have not been established. In the present article we report the case of a breastfeeding who developed an acute renal failure secondary to a hemolytic uremic syndrome during the hospitalization in the Intensive Care Unit.

Síndrome hemolítico urémico en niños en los últimos 10 años en el hospital Gustavo Fricke / Hemolytic Uremic Syndrome in Children in the last 10 years at hospital Gustavo Fricke

El síndrome hemolítico urémico se caracteriza por la presencia de anemia hemolítica microangiopática, trombocitopenia e injuria renal aguda. Es una de las causas más frecuentes de falla renal aguda en pacientes pediátricos. Objetivo: Conocer las características clínicas y la evolución de los pacientes con SHU hospitalizados en nuestro hospital. Material y Método: Se revisaron 55 historias clínicas de los pacientes egresados con el diagnostico de SHU en el Hospital Dr. Gustavo Fricke entre el año 2001 y 2011 y se extrajo la información más relevante sobre la presentación clínica y la evolución de esta enfermedad durante la hospitalización. Resultados: 4 pacientes fallecieron (5,7 por ciento). Un 62 por ciento presentó una diarrea aguda disentérica; 30,9 por ciento hipertensión arterial y 11 por ciento convulsiones. Un 84 por ciento fue transfundido con glóbulos rojos, 45 por ciento requirió terapia de sustitución renal. La duración de la hospitalización fue de 14 días en promedio. Al año solo un 66 por ciento permanecían en control médico. Conclusiones: El SHU continúa siendo una de las causas más frecuentes de injuria renal aguda con requerimiento de diálisis en nuestro hospital. La mayoría de los pacientes sufre anemias severas con necesidad de trasfusión de glóbulos rojos. La mortalidad es similar a la reportada en otros centros...

Hemolytic Uremic Syndrome (HUS) is characterized by the presence of hemolytic microangiopathic anemia, thrombocytopenia and acute renal failure. Is one of the most frequent causes of acute renal failure in pediatric patients. Objective: Know clinical characteristics and evolution of patients with HUS hospitalized at Hospital Dr. Gustavo Fricke. Material and Methods: 55 medical records of discharged patients with the diagnosis of HUS in Dr. Gustavo Fricke Hospital between 2001 and 2011 were reviewed. We extracted the most relevant information on clinical presentation and evolution of this disease during hospitalization. Results: 4 patients died (5.7 percent). 62 percent presented an acute dysenteric diarrhea; 30.9 percent evolved with hypertension and 11 percent presented seizures. 84 percent were transfused with red blood cells, 45 percent required renal replacement therapy. The hospital stay was 14 days on average. After one year, only 66 percent remained in medical control. Conclusions: HUS remains one of the most frequent causes of acute kidney injury who required dialysis at our hospital. Most patients have severe anemia requiring transfusion of RBCs. Mortality is similar to that reported in other centers...

Síndrome Hemolítico-Urêmica na infância / Hemolytic-Uremic Syndrome in childhood

O grupo de doenças que podem manifestar-se com MAT podem apresentar superposição clínica, dificultando o diagnóstico diferencial. Entre estas ressaltamos a PTT e SHU, sendo que esta última pode ocorrer pela ação de toxinas, doenças sistêmicas, hiperativação da via alterantiva descontrolada por alterações nas proteínas reguladoras desta via (SHUa) e por fim, idiopática. Deve-se proceder a uma série de exames para diferenciá-las. Ressaltando que SHUa é um diagnóstico de exclusão de outras causas de MAT. O tratamento da SHUa com infusão de plasma ou plasmaferese, resulta na maior parte dos casos com boa resposta, especialmente hematológica a curto-prazo, porém é uma doença grave e devastadora e, pode levar a óbito e doença renal crônica terminal. Tratamento com plasma apresenta grande recorrência da doença a longo-prazo e evolução renal desfavorável. Eculizumab, um anticorpo monoclonal anti-C5, tem surgido como uma esperança no prognóstico a curto e a longo-prazo nestes pacientes.

There is a group of diseases that may manifest with thrombotic microangiopathy and present clinical overlap. Among these we emphasize the thrombotic thrombocytopenic purpura and Hemolytic Uremic Syndrome, and the latter can occur by the action of toxins, systemic diseases, overactivation of the alternative complement system pathway, which can occur due to changes in regulatory proteins (atypical HUS) and finally, idiopathic. You must carry out a series of tests to differentiate them. aHUS is a diagnosis of exclusion of other causes of MAT. The treatment of aHUS with plasma therapy, results in most cases with good shortterm response, especially hematological; however, it is a progressive and devastating disease and can lead to death and terminal chronic renal disease. Treatment with plasma displays great recurrence of long-term disease and renal insufficiency. Eculizumab, a monoclonal antibody anti-C5, has been associated with hematological remission, benefits on renal function and no need of plasma therapy.

Seguridad y eficacia de la colocación del catéter de diálisis peritoneal aguda por punción en pacientes con síndrome urémico hemolítico asociado a diarrea / Safety and efficacy of percutaneous acute peritoneal dialysis catheter placement in patients with post-diarrheal hemolytic uremic syndrome

Introducción: La diálisis peritoneal aguda (DPA) es la modalidad dialítica preferentemente seleccionada para niños con injuria renal aguda por síndrome urémico hemolítico postdiarreico (SUH D+). Evaluamos la seguridad y eficacia de la colocación por punción percutánea del catéter de DPA con anestesia local en niños con SUH D+. Pacientes y métodos: Se revisaron las historias clínicas de todos los pacientes con SUH D+ internados entre el 1 de enero de 1998 y el 31 de diciembre de 2008 en el Hospital de Pediatría Prof. Dr. Juan P. Garrahan. La seguridad se evaluó por la presencia de eventos adversos mayores relacionados con la colocación del catéter (per foración de vísceras y/o vasos mayores abdominales, sangrado que requiera transfusión) y menores (infección del sitio de salida y peritonitis dentro de las 48 hs del procedimiento). La eficacia se evaluó a través de la colocación exitosa del catéter y su buen funcionamiento. Además se registró la necesidad de recambio luego de su uso por mal funcionamiento. Resultados: Identificamos 149 pacientes que realizaron DPA, edad de 20.2 meses (rango 2,9-111) y peso de 11,35 kg (rango 5-24.4). Recuento de plaquetas previo al procedimiento de 89000 (22000-148000) mm3. Seguridad: el único efecto adverso detectado fue el desarrollo de peritonitis en un paciente. No se registró perforación de órganos ni de vasos mayores abdominales, ni sangrado severo, ni infección del sitio de salida. Eficacia: en todos los casos el catéter fue colocado exitosamente y en 48 pacientes (32.2%) hubo que recambiarlo por mal funcionamiento. Tanto la colocación como el recambio fueron realizadas en todos los casos por el nefrólogo al pie de la cama. Conclusión: la colocación del catéter de DPA por punción es un procedimiento seguro y eficaz.

ntroduction: Acute peritoneal dialysis (DPA) is the dialytictreatment of choice for children with acute kidney injury dueto post-diarrheal hemolytic uremic syndrome (D+HUS). In thisstudy safety and efficacy of percutaneous placement of anAPD catheter under local anesthesia in children with D+HUSwas assessed. Patients and methods: We reviewed the cli-nical charts of all patients with D+HUS admitted to thePediatric Hospital Prof. Dr. Juan P. Garrahan betweenJanuary 1, 1998 and December 31, 2008. Safety was eva-luated based on the presence of major (perforation of theviscera and/or major abdominal vessels, bloody dialysaterequiring red-blood-cell transfusion) and minor (exit-siteinfection and peritonitis within 48 hs of the procedure) adverse events associated with catheter insertion. Efficacy was assessed based on successful catheter insertion and func-tioning. Additionally, the need for catheter replacement dueto malfunction was recorded. Results: We identified 149patients with a mean age of 20.2 months (range, 2.9-111)and weight of 11.35 kg (range, 5-24.4) who underwent APD.Median platelet count previous to the procedure was 89000(range, 22000-148000) mm3. Safety: The only adverse eventfound was the development of peritonitis in one patient.Organ or major vessel perforation, severe bleeds, or exit-site infection were not observed. Efficacy: In all patients the catheter was successfully inserted and in 48 patients (32.2%) the catheter had to be replaced due to malfunctioning. Both placement and replacement were performed by a nephrologist at the bedside in all cases. Conclusion: Percutaneous APD catheter insertion is a safe and efficacious procedure.

Actualización en el tratamiento del síndrome urémico hemolítico endémico: Patogénesis y tratamiento de la complicación sistémica más grave de las infecciones por Escherichia coli productor de toxina Shiga / Update on the treatment of endemic hemolytic uremic syndrome: Pathogenesis and treatment of the most severe systemic complication of infections by Shiga toxin-producing Escherichia coli

La forma típica o post-diarreica del síndrome urémico hemolítico (SUH) es la complicación más grave de las infecciones por cepas de Escherichia coli productoras de toxina Shiga (STEC). En la Argentina el SUH es un problema crítico de salud pública, ya que representa la principal causa de falla renal aguda en la infancia, la segunda causa de falla renal crónica, y aporta el 20% de los casos de transplante renal durante la infancia y la adolescencia. A pesar de los avances en el conocimiento de su patogénesis, el único tratamiento actual de los pacientes con SUH es de sostén, y no existen terapias específicas ni preventivas. En la presente revisión expondremos los conocimientos básicos de los mecanismos patogénicos y discutiremos los enfoques terapéuticos tradicionales e innovadores, con especial foco en la situación nacional y los aportes hechos por grupos de la Argentina.

The typical form of hemolytic uremic syndrome (HUS) is the major complication of Shiga toxin-producing Escherichia coli (STEC) infections. HUS is a critical health problem in Argentina since it is the main cause of acute renal failure in children and the second cause of chronic renal failure, giving account for 20% of renal transplants in children and adolescents in our country. In spite of the extensive research in the field, the mainstay of treatment for patients with HUS is supportive therapy, and there are no specific therapies preventing or ameliorating the disease course. In this review, we present the current knowledge about pathogenic mechanisms and discuss traditional and innovative therapeutic approaches, with special focus in national status and contributions made by Argentinean groups.

Medicina transfusional basada en la evidencia / Evidence-based transfusional medicine

La idea de publicar este trabajo, es estimular la utilización de preguntas surgidas de la práctica médica, para obtener respuestas con base en la mejor evidencia publicada. La medicina basada en la evidencia, es una herramienta fundamental para la toma de decisiones, no sólo en lo que respecta a un paciente o caso puntual, sino también para la salud y la sociedad en general. El caso que motiva esta publicación es una de las entidades que, como equipo dedicado a la medicina transfusional más nos convoca, ya que no sólo es una patología que requiere de nuestra participación activa como integrantes de un equipo médico, sino que además depende en gran medida del desarrollo de los bancos de sangre como servicios activos y pensados para dar respuesta a este tipo de problemática.

Publishing this paper aims to encourage doctors to take questions that arise from their medical practice to be answered basing their research on the best evidence published. Evidence-based medicine is an essential tool when making decisions not only when it comes to a patient or a particular case but also when these decisions have an impact on health and societies in general. The case that inspires this publication is one that concerns us the most because it consists in a pathology that requires our whole active participation as members of a transfusion medicine team. Furthermore, it heavily depends on the development of blood banks as an active service that is conceived to salve this type of problem.

Childhood hemolytic uremic syndrome in Jordan

Hemolytic Uremic Syndrome [HUS] is the most common cause of Acute Kidney Injury [AKI] in the developed countries. It consists of Microoangiopathic Hemolytic Anemia [MAHA], AKI and thrombocytopenia. To review the outcome of childhood Diarrhea-associated Hemolytic Uremic Syndrome [D+HUS] presenting to the pediatric department at Jordan University Hospital [JUH]. In this retrospective study war reviewed the medical rectors of children presenting to JUH between January 1977 and January 2008 with D+HUS. There were 21 patients [15 girls and 6 boys]. Age ranged from 6 months to 11 years. 8 children [38%] had Entamoeba histolytica infection. 57% needed peritoneal dialysis. Central nervous system manifestations included drowsiness in 8 patients [38.1%], limb weakness in 2 patients [9.5%], seizures in 9 patients [43%], irritability 3 patients [14%], transient blindness in 2 patients [9.5%], and uremic encephalopathy in 1 patient [4.8%]. Complete recovery in 11 patients [52.4%], chronic kidney disease in 6 patients [28.6%], central nervous system deficit in 2 patients, and death in 2 patients [9.5%]. There was no correlation between the outcome and the presence of leukocytosis, thrombocytopenia, severity of renal failure, hyponatremia, or hypertension [p<0.05]. Our data highlights the importance of D+HUS in the pediatric age group. In addition, it emphasizes its manifestations, complications, and outcome

Un caso de diarrea aguda sanguinolenta / A case of acute bloody diarrhea

Este artículo presenta un caso de diarrea aguda sanguinolenta y sus aspectos clínicos, con especial énfasis en la forma adecuada de manejo.

Síndrome hemolítico urémico en Chile: presentación clínica, evolución y factores pronósticos / Hemolytic-uremic syndrome in Chile: clinical features, evolution and prognostic factors

Background: Hemolytic-uremic syndrome (HUS) is characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Aim: To describe the characteñstics ofpatients with the diagnosis ofHUS in Chile, and to identify the most reliable early predictors oímorbidity and moñality. Material and methods: The clinical records ofpatients with HUS aged less than 15 years, attended between January 1990 and December 2003 in 15 hospitals, were reviewed. Demographic, clinical, biochemical, hematological parameters, morbidity and mortality were analyzed. Results: A cohort of 587 patients aged 2 to 8 years, 48 percent males, was analyzed. Ninety two percent had diarrhea. At the moment of diagnosis, anuria was observed in 39 percent of the patients, hypertension in 45 percent and seizures in 17 percent. Forty two percent required renal replacement therapy (RRT) and perítoneal dialysis was used in the majoríty of cases (78 percent). The most frequently isolated etiological agentwas Escherichia coli. Mortality rate was 2.9 percent in the acute phase of the disease and there was a positive correlation between mortality and anuria, seizures, white blood cell count (WCC) >20.000/mm³ and requirements of renal replacement therapy (p <0.05). Twelve percent of patients evolved to chronic renal failure and the risk factors during the acute phase were the need for renal replacement therapy, anuria, WCC >20.000/mm³, seizures and hypertension. Conclusions: The present study emphasizes important clinical and epidemiological aspeets ofHUSin a Chilean pediatricpopulation.