Atypical hemolytic uremia syndrome in a young adult male

Atypical hemolytic uremia syndrome (aHUS) is a rare and life-threatening disease, characterized by the same triad of hemolytic anemia, thrombocytopenia, and renal failure as seen in HUS. It differs in its etiology, being caused by a dysregulation of the complement pathway rather than Shiga-like toxin-producing Escherichia coli. Prognosis is poor, with 50% of cases progressing to end-stage renal disease (ESRD) and 25% succumbing in the acute phase. The treatment of choice is therapeutic plasma exchange which can lower mortality. Monoclonal antibody drugs such as eculizumab, which suppress the dysregulated complement pathway, help to prevent complement-mediated kidney injury. We report the case of a young adult male who presented with thrombocytopenia and worsening acute kidney injury and was diagnosed with aHUS based on high lactic dehydrogenase, low complement C3, and haptoglobin, as well as renal biopsy showing thrombotic microangiopathy

An Stx-EAEC O59:NM[H19] strain isolated from a hemolytic uremic syndrome case in Argentina / Cepa EAEC-Stx O59:NM[H19] aislada de un caso de síndrome urémico hemolítico en Argentina

Abstract Shiga toxin-producing Escherichia coli (STEC) are a heterogeneous group of foodborne pathogens causing a broad spectrum of human disease, from uncomplicated diarrhea to hemolytic uremic syndrome (HUS). In this study, we report an HUS case associated with an O59:NM H19 mstrain, harboring stx2a, iha, lpfAO26, lpfAO113 genes associated with STEC, and aatA, aap, pic, sigA, agg4A genes associated with enteroaggregative E. coli (EAEC), named Stx-EAEC. The strain showed low toxicity on Vero cells, and was resistant to streptomycin and trimethoprim/sulfonamides. The child carried the bacteria for more than 100 days. Since the large outbreak associated with Stx-EAEC O104:H4, many strains with similar profiles have been described. In Germany, an O59:NM[H19] strain, with comparable characteristics to the Argentine strain, was isolated from a bloody diarrhea case. In Argentina, this is the first report of an HUS case associated with a Stx-EAEC infection, and represents a new challenge for the surveillance system. © 2019 Published by Elsevier Espana, S.L.U. on behalf of Asociacion Argentina de Microbiolog´a. This is an open access article under the CC BY-NC-ND license (https://creativecommons.org/ licenses/by-nc-nd/4.0/).

Resumen Escherichia coli productor de la toxina Shiga (STEC) es un grupo heterogéneo de patógenos transmitidos por alimentos que causan un amplio espectro de enfermedades humanas, desde diarrea no complicada hasta síndrome urémico hemolítico (SUH). Nosotros informamos de un caso de SUH por O59:NM[H19], que portaba los genes stx2a, iha, lpfAo26, lpfAoii3 asociados con STEC, y los genes aatA, aap, pic, sigA, agg4A de E. coli enteroagregativo (EAEC), llamado EAEC-Stx. La cepa mostró baja citotoxicidad en las células Vero, y fue resistente a estreptomicina y trimetoprima/sulfonamidas. El niño excretó la bacteria durante más de 100 días. Desde el brote asociado con EAEC-Stx O104:H4, se describieron muchas cepas con perfiles similares. En Alemania se aisló una cepa O59:NM[H19] de una diarrea sanguinolenta, con características comparables a la cepa argentina. Este es el primer informe de un caso de SUH asociado a una infección por EAEC-Stx, y representa un nuevo desafío para el sistema de vigilancia. © 2019 Publicado por Elsevier Espana, S.L.U. en nombre de Asociación Argentina de Microbiología. Este es un artículo Open Access bajo la licencia CC BY-NC-ND (http://creativecommons. org/licenses/by-nc-nd/4.0/).

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.

Detección y caracterización de Escherichia coli productor de toxina Shiga en ninos atendidos en un hospital pediátrico interzonal de la ciudad de La Plata / Detection and characterization of Shiga toxin-producing Escherichia coli in children treated at an inter-zonal pediatric hospital in the city of La Plata

Escherichia coli productor de toxina Shiga (STEC) es un patógeno transmitido por alimentos que puede causar diarrea acuosa, diarrea sanguinolenta (DS) y síndrome urémico hemolítico (SUH). El objetivo de este estudio fue determinar las características fenotípicas y genotípicas de cepas STEC aisladas de niños con DS y SUH atendidos en un hospital pediátrico de la ciudad de La Plata en el período 2006-2012 y establecer la relación clonal de los aislamientos O157: H7 mediante electroforesis de campo pulsado. El porcentaje de muestras positivas fue de 4,9 y 39,2% en los pacientes que presentaron DS y SUH, respectivamente. Se aislaron 77 cepas STEC de 10 serotipos distintos, con el 100% de recuperación de colonias. El serotipo más frecuente fue O157: H7 (71,4%), seguido por O145: NM (15,6%). El 98,2% de los aislamientos O157: H7 correspondió al biotipo C y fue sensible a los antibióticos ensayados. Todos esos aislamientos presentaron el genotipo stx2, eae, fliC H7, ehxA, iha, efa, toxB, lpfA1-3 y lpfA2-2.Al estudiar la relación clonal de las cepas O157: H7, se identificaron un total de 42 patrones con al menos un 88% de similitud y se establecieron 6 clústeres que agruparon cepas con perfiles idénticos. Los aislamientos eae negativos pertenecieron a los serotipos O59: H19, O102: H6, O174: NM y O174: H21. Las cepas O59: H19 y O174: H21 fueron positivas para el gen aggR. Este estudio muestra que en la ciudad de La Plata y alrededores circulan STEC de diferentes serotipos y genotipos. A pesar de la diversidad genética observada entre los aislamientos O157: H7, algunos fueron indistinguibles por las técnicas de subtipificación utilizadas.

Shiga toxin-producing Escherichia coli (STEC) is a foodborne pathogen that can cause watery diarrhea, bloody diarrhea (BD), and hemolytic uremic syndrome (HUS). The objective of this study was to determine the phenotypic and genotypic profiles of STEC strains isolated from children with BD and HUS treated at a pediatric hospital in the city of La Plata in the period 2006-2012, and to establish the clonal relationship of O157: H7 isolates by pulsed field electrophoresis. The percentage of positive samples was 4.9% and 39.2% in patients with BD and HUS, respectively. Seventy-seven STEC strains from 10 different serotypes were isolated, with 100% colony recovery, O157: H7 being the most frequent (71.4%) serotype, followed by O145: NM (15.6%). An average of 98.2% of O157: H7 isolates belonged to biotype C and were sensitive to all the antibiotics tested. All of them (100%) carried genotype stx2, eae, fliC H7, ehxA, iha, efa, toxB, lpfA1-3 and lpfA2-2. When the clonal relationship of the O157: H7 strains was studied, a total of 42 patterns with at least 88% similarity were identified, and 6 clusters with identical profiles were established. The eae-negative isolates belonged to serotypes O59: H19, O102: H6, O174: NM and O174: H21. The strains O59: H19 and O174: H21 were positive for the aggR gene. This study shows that STEC of different serotypes and genotypes circulate in the city of La Plata and surroundings. Despite the genetic diversity observed between the O157: H7 isolates, some were indistinguishable by the subtyping techniques used.

El síndrome urémico hemolítico y manejo renal de proteínas / Hemolytic uremic syndrome and renal handling of proteins

El síndrome urémico hemolítico (SUH) está definido por la tríada de anemia hemolítica microangiopática, trombocitopenia e insuficiencia renal aguda. En Argentina constituye la primera causa de insuficiencia renal aguda en pediatría. Aproximadamente, del 2% al 4% de los pacientes mueren durante la fase aguda de la enfermedad, y solo un tercio del 96% restante que sobrevive lo hace con secuelas renales, como la persistencia de la proteinuria. Un individuo adulto sano filtra alrededor de 5000 mg/día de proteínas, si bien la excreción en orina es escasa (150 mg/día). La escasa cantidad de proteínas excretadas indica la presencia de un mecanismo de reabsorción a nivel del túbulo proximal. Por lo tanto, la reabsorción tubular renal desempeña un papel muy importante ya que, ante una función glomerular normal, es el principal mecanismo encargado de evitar la depleción proteica corporal. Desde hace aproximadamente 30 años se sabe que la albúmina es reabsorbida en el túbulo proximal. La reabsorción proteica se produce por un mecanismo de endocitosis mediada por el receptor dependiente de clatrina y por endocitosis de fase líquida. Clásicamente se ha descrito que el mecanismo básico del daño renal en el SUH típico y en el atípico es una microangiopatía trombótica, pero de diferentes causas. Sin embargo, debe tenerse en cuenta que la fisiopatología de esta enfermedad es más compleja de lo que se creía, ya que la alteración tubular que surge va a evolucionar en fallas en el mecanismo de endocitosis de proteínas que se suman a las eliminadas por las alteraciones a nivel de la barrera de filtración glomerular.

Hemolytic uremic syndrome (HUS) is defined by the triad of hemolytic anemia microangiopathic, thrombocytopenia and acute renal failure. In Argentina it constitutes the first cause of acute renal failure in Pediatrics. Approximately 2-4% of patients die during the acute phase of the disease, and only a third of the remaining 96% survive with renal sequelae, such as the persistence of proteinuria. A healthy adult filters around 5000 mg/day of proteins, with an excretion in urine of 150 mg/day. The little quantity of proteins excreted indicates the presence of a reabsorption mechanism at the level of the proximal tubule. Therefore, the tubular reabsorption plays a very important role since it is the main mechanism responsible for preventing the depletion of protein. For approximately 30 years, it has been known that albumin is reabsorbed in the proximal tubule. Protein reabsorption occurs by a clathrin-dependent receptor mediated endocytosis mechanism and by fluid phase endocytosis. The basic mechanism of renal damage in typical and atypical HUS has been described as a thrombotic microangiopathy, but of different causes. However, the pathophysiology of this disease is more complex than what was believed since the emerging tubular alteration will ewvolve into failures of the protein endocytosis mechanism that are added to the alterations at the level of the glomerular filtration barrier.

Kidd Phenotypes in Multiple Ethnicities in Hospital Umum Sarawak, Malaysia

@#Introduction: Kidd blood group system is distributed differently within populations. In Malaysia, the prevalence of Kidd phenotypes have been reported but not in Hospital Umum Sarawak (HUS).We characterised Kidd phenotypes among regular blood donors in HUS. Methods: A cross-sectional study was done from 1st September 2015 to 10th September 2015. Blood samples were collected from 250 regular blood donors of different ethnicities in HUS. Samples were then investigated for Kidd blood group phenotypes by utilising Seraclon anti-Jka and anti-Jkb reagents employing the Diamed-ID gel card system. Results: Phenotype Jk(a+b+) was found in 110 out of 250 (44.0%) and phenotype Jk (a-b-) phenotype in seven out of 250 (2.8%) blood donors. Jk(a+b-) was detected in 60 out of 250 (24.0%) and Jk(a-b+) in 73 out of 250 (29.2%) donors. Kidd phenotype was detected in four ethnics; Chinese 50.8%, Malays 38.4%, Bidayuh 10.0% and Iban 0.8%. Jk(a-b-) phenotype was present only in the Malays; seven out of 250 (2.8%) but not found in other ethnicities. Conclusion: Jk(a+b+) is the most common Kidd phenotype found in regular blood donors in HUS in the four ethnicities studied. Only Malays exhibit the Jk(a-b-) phenotype which is a rare phenotype. The results of this study may serve as a preliminary database for Kidd blood group profile of regular blood donors in HUS.

Diretrizes gerais para uma política nacional de inclusão das ações de diagnóstico no processo de atenção à saúde: a atenção em análises clínicas e toxicológicas como um dos eixos estruturantes / General guidelines for a national policy for the inclusion of diagnosis actions in the process of health care: attention in clinical and toxicological analysis as one of the structuring axes

As políticas públicas voltadas para atenção à saúde dependem da participação e envolvimento político, exigem o aprimoramento científico e técnico, além de ferramentas adequadas para a gestão, incorporação de tecnologias e a aplicação de estratégias para a superação das fragmentações dos serviços, na perspectiva concreta das efetivas universalidade e integralidade das ações de saúde, com equidade. O objetivo deste documento foi propor diretrizes de uma política voltada às análises clínicas e toxicológicas alinhada com as atuais iniciativas do Ministério da Saúde. Foi utilizada como metodologia a análise das discussões e demandas extraídas dos fóruns das Comissões de Análises Clínicas dos Conselhos de Farmácia do Sul do Brasil, tendo como pressupostos as questões conceituais e estratégicas elencadas no presente documento. O resultado deste documento gerou de um conjunto de diretrizes gerais e seis eixos estratégicos, quais sejam, os conceitos fundamentais, o acesso e segurança do paciente, o financiamento e estruturação da rede, a incorporação de tecnologias, o reconhecimento do trabalho na atenção em análises clínicas e a regulação dos serviços. Espera-se que estes eixos possam colaborar com as iniciativas em andamento no Ministério da Saúde, especialmente no tocante à estruturação da rede de diagnóstico e suas interfaces com todas as ações de atenção e cuidado em saúde, incluindo aqueles de caráter complementar às ações desenvolvidas no contexto dos serviços públicos de atenção.

Public policies for health care depend on the participation and political involvement, requiring scientific and technical improvement, as well as appropriate tools for management, incorporating technologies and implementation strategies to overcome the fragmentation of services with a concrete view of the actual universality and comprehensiveness of health actions with equity. The purpose of this study is to propose guidelines for a policy aimed at clinical and toxicological analysis in line with the current Ministry of Health initiatives. It used the analysis of discussions and demands extracted from the forums of Clinical Analysis Committees of South Pharmacy Councils Brazil as its methodology, taking the conceptual and strategic issues listed in this document as assumptions. The result of this document has generated a set of general guidelines and six strategic axes, namely, the fundamental concepts, patient access and safety, network financing and structuring, technology incorporation, work recognition in clinical analysis care, and service regulation. It is expected that these axes can collaborate with ongoing initiatives at the Ministry of Health, especially regarding the structure of the diagnostic network and its interfaces with all care and health care actions, including those of complementary character to the actions developed in the context of public health care services.

Síndrome urémico hemolítico con compromiso renal leve debido a una cepa de Escherichia coli productora de toxina Shiga [Shiga-toxin-producing Escherichia coli (STEC)] 0145 / Hemolytic uremic syndrome with mild renal involvement due to Shiga toxin-producing Escherichia coli (STEC) 0145 strain

El síndrome urémico hemolítico (SUH) es una afección caracterizada por la presencia de la tríada clásica: anemia hemolítica microangiopática, trombocitopenia y compromiso renal agudo. Los casos de SUH sin insuficiencia renal pueden confundirse con otras enfermedades hematológicas. Presentamos un caso de SUH pediátrico causado por una cepa de Escherichia coli productora de toxina Shiga Shiga-toxin-producing Escherichia coli (STEC) O145 con el genotipo stx2, ehxA, eae subtipo ?1. El niño no requirió diálisis durante la etapa aguda del SUH, evolucionó favorablemente y no tuvo recurrencias hasta el último control; además, mantuvo cifras normales de presión arterial y función renal normal. Esto puede deberse a varios factores: características de la cepa STEC infectante y susceptibilidad del hospedero al daño renal, entre otros. Este hallazgo destaca la participación regional de STEC no-O157 en enfermedades de la infancia y la importancia de realizar una vigilancia activa de todas las formas de SUH

Hemolytic uremic syndrome (HUS) is a disorder characterized by the presence of the classic triad: microangiopathic hemolytic anemia, thrombocytopenia and acute renal injury. HUS without acute renal failure can be confused with other hematologic diseases. An infantile HUS caused by a Shiga-toxin-producing Escherichia coli (STEC) O145 strain carrying genotype stx2, ehxA, eae subtype ?1 is herein reported. The infant did not require dialysis during the acute stage of HUS, evolved favorably, maintained normal blood pressure and normal renal function and had no recurrence until the last control. This could be due to several factors, such as the characteristics of infecting STEC strain and a reduction in host susceptibility to renal injury. This report highlights the regional participation of non-O157 STEC in childhood diseases and the importance of performing active surveillance for all forms of HUS

Virulence profiles of Shiga toxin-producing Escherichia coli and other potentially diarrheagenic E.coli of bovine origin, in Mendoza, Argentina

This study described a group of strains obtained from a slaughter house in Mendoza, in terms of their pathogenic factors, serotype, antibiotype and molecular profile. Ninety one rectal swabs and one hundred eight plating samples taken from carcasses of healthy cattle intended for meat consumption were analyzed. Both the swab and the plate samples were processed to analyze the samples for the presence of virulence genes by PCR: stx1, stx2, eae and astA. The Stx positive strains were confirmed by citotoxicity assay in Vero cells. The isolates were subsequently investigated for their O:H serotype, antimicrobial susceptibility and molecular profile by Random Amplification of Polymorphic DNA (RAPD). Twelve E.coli strains were identified by their pathogenicity. Nine were from fecal origin and three from carcasses. Three strains carried the stx1 gene, three the stx2 gene, two carried eae and four the astA gene. The detected serotypes were: O172:H-; O150:H8; O91:H21; O178:H19 and O2:H5. The strains showed a similarity around 70% by RAPD. Some of the E.coli strains belonged to serogroups known for certain life-threatening diseases in humans. Their presence in carcasses indicates the high probability of bacterial spread during slaughter and processing.

Epidemiología del síndrome urémico hemolítico en dos regiones de la Provincia de Buenos Aires / Epidemiology of hemolytic uremic syndrome in two regions of Buenos Aires Province

Los objetivos de este estudio fueron describir y comparar la frecuencia del síndrome urémico hemolítico (SUH), registrada durante el periodo 2005-2010 en pobladores rurales y urbanos de la región centro-sur de la provincia de Buenos Aires y caracterizar la distribución de factores hipotéticos asociados al SUH en ambas poblaciones. Se caracterizaron 82 casos de SUH clínica y epidemiológicamente. Para el análisis estadístico de los datos se utilizó el test de Chi² o test exacto de Fisher cuando fue necesario, y el test de la mediana. La incidencia específica fue 12.7 casos cada 100 000 habitantes rurales (IC 0-23.5) y 7.1 casos cada 100 000 habitantes urbanos (IC 0-9.5). La mediana de la edad en el total de los pacientes estudiados fue de 27 meses (5-139 meses), siendo significativamente más baja en los niños del área rural. Estos resultados podrían explicarse por el contacto más frecuente con materia fecal de bovinos, el mayor consumo de leche sin pasteurizar y la mayor proporción de familiares que realizan actividades de riesgo, encontrados en la subpoblación rural. Aunque el SUH es una enfermedad que se asocia frecuentemente con el consumo de carne picada, la mayor parte de los niños que enfermaron no presentaron este antecedente. Las manifestaciones clínicas fueron semejantes en ambas subpoblaciones. Un tercio de los pacientes urbanos había recibido tratamiento con antibióticos previo al desarrollo del SUH.

The objectives of this study were (a) to describe and estimate the frequency of hemolytic uremic syndrome (HUS) in rural and urban populations in two regions of Buenos Aires Province, and (b) to compare the presentation and distribution of factors hypothetically associated with HUS. A total of 82 HUS cases, recorded during the years 2005-2010 in rural and urban areas of the south-central region of Buenos Aires Province, were clinically and epidemiologically characterized. Statistical data analysis included Chi square or Fisher test and median test. The incidence rate of HUS was significantly higher in the rural population, being 12.7 cases per 100 000 (CI 0-23.5) in rural inhabitants vs. 7.1 cases per 100 000 (CI 0-9.5) in urban inhabitants. The median age of the patients was 27 months (5-139 months), significantly lower in children from the rural area. This could be explained by a more frequent contact with bovine feces, the consumption of raw milk and a higher proportion of relatives who work in risk labors found in the rural population. Although HUS is often associated with the consumption of undercooked minced meat, most of the children cases here included did not present this antecedent. Clinical manifestations were similar in both subpopulations. One-third of urban patients had received antibiotics prior to HUS development.

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.

Síndrome urémico hemolítico atípico de presentación neonatal / Atypical hemolytic uremic syndrome of neonatal presentation

El Síndrome urémico hemolítico (SUH) tiene formas típicas y atípicas. Se describe una variedad de formas genéticas con pobre pronóstico. Presentamos un bebé prematuro de 36 semanas, de bajo peso al nacer, quien a las 2 semanas de vida cursó con sepsis y necrosis intestinal siendo sometido a cirugía para realizarle ileostomía. Evolucionó con hipertensión arterial, hematuria, falla renal aguda y proteinuria persistente. A los 2 meses de vida, posterior al cierre de ileostomía, cursó con shock séptico y falleció. La biopsia renal post mortem mostró cuadro compatible de SUH. Dos años después, un hermano debutó a los 2 días de vida con síndrome nefrótico congénito. El estudio genético reveló que la madre era portadora del gen NPHS1 y el padre, del Factor I de complemento. El segundo hijo era portador de ambos genes.

Hemolytic Uremic Syndrome (HUS) has typical and atypical presentations. A variety of genetic forms, with poor prognosis are described. We report a 36 week premature baby, low birth weight, who at 2 weeks of life evolved with sepsis and intestinal necrosis undergoing surgery for ileostomy, hypertension, hematuria, acute renal failure and persistent proteinuria. At 2 months, after ileostomy closure, developed irreversible septic shock and died. Postmortem renal biopsy was compatible with HUS. Two years later, a brother presented after 2 days of birth with congenital nephrotic syndrome. Genetic studies revealed that the mother was carrying the gene NPHS1 and the father, factor I of complement. The second child was a carrier of both genes.

Diagnosing Small Hepatic Cysts on Multidetector CT: an Additional Merit of Thinner Coronal Reformations

OBJECTIVE: We wanted to validate the additional merit of the thinner coronal reformation images from multidetector CT (MDCT) for making the diagnosis of hepatic cysts. MATERIALS AND METHODS: For the 90 benign hepatic cysts confirmed on MRI, the transverse (5-mm thickness) and additional coronal (2-mm thickness) reformation images from MDCT were compared with each other in terms of the Hounsfield units (HUs) and the size of each hepatic cyst. RESULTS: The attenuations (mean: 17.2 HUs, standard deviation: +/- 14.4) on the thinner coronal images were significantly lower than those (mean: 40.7 HUs; standard deviation: +/- 20.6) on the thicker transverse images for the small hepatic cysts (< or = 10 mm on the transverse image, p < 0.01). Twenty-three (79%) of the 29 cysts between 5 mm and 10 mm and 21 (51%) of 41 lesions up to 5 mm showed a mean HU value of 20 or less on the coronal reformation images. CONCLUSION: By reducing the partial volume effect, routine coronal reformation of MDCT with a thinner section thickness can provide another merit for making a confidential diagnosis of many small sub-centimeter hepatic cysts, and these small cysts are not easily characterized on the conventional transverse images.

Antibody responses elicited in mice immunized with Bacillus subtilis vaccine strains expressing Stx2B subunit of enterohaemorragic Escherichia coli O157: H7 / Resposta de anticorpos obtidas em camundongos imunizados com linhagens vacinais de Bacillus subtilis expressando a subunidade B da Stx2 de Escherichia coli O157: H7 enterohemorrágica

No effective vaccine or immunotherapy is presently available for patients with the hemolytic uremic syndrome (HUS) induced by Shiga-like toxin (Stx) producedbyenterohaemorragic Escherichia coli (EHEC) strains, such as those belonging to the O157:H7 serotype. In this work we evaluated the performance of Bacillus subtilis strains, a harmless spore former gram-positive bacterium species, as a vaccine vehicle for the expression of Stx2B subunit (Stx2B). A recombinant B. subtilis vaccine strain expressing Stx2B under the control of a stress inducible promoter was delivered to BALB/c mice via oral, nasal or subcutaneous routes using both vegetative cells and spores. Mice immunized with vegetative cells by the oral route developed low but specific anti-Stx2B serum IgG and fecal IgA responses while mice immunized with recombinant spores developed anti-Stx2B responses only after administration via the parenteral route. Nonetheless, serum anti-Stx2B antibodies raised in mice immunized with the recombinant B. subtilis strain did not inhibit the toxic effects of the native toxin, both under in vitro and in vivo conditions, suggesting that either the quantity or the quality of the induced immune response did not support an effective neutralization of Stx2 produced by EHEC strains.

Até o presente o momento, não há vacina ou imunoterapia disponível para pacientes com Síndrome Hemolítica Urêmica (SHU) induzida pela toxina Shiga-like (Stx) produzida por linhagens de Escherichia coli entero-hemorragica (EHEC), tais como as pertencentes ao sorotipo O157:H7. Neste trabalho, avaliamos a performance de Bacillus subtilis, uma espécie bacteriana gram-positiva não-patogênica formadora de esporos, como veículo vacinal para a expressão da subunidade B da Stx2B (Stx2B). Uma linhagem vacinal recombinante de B. subtilis expressando Stx2B, sob o controle de um promoter induzível por estresse, foi administrada a camundongos BALB/c por via oral, nasal ou subcutânea usando células vegetativas e esporos. Camundongos imunizados com células vegetativas e esporos pela via oral desenvolveram títulos anti-Stx2B baixos, mas específicos, de IgG sérico e IgA fecal, enquanto camundongos imunizados com esporos recombinates desenvolveram resposta anti-Stx2B apenas após a administração pela via parenteral. No entanto, anticorpos produzidos em camundongos imunizados com a linhagem recombinante de B. subtilis não inibiram os efeitos tóxicos da toxina nativa em condições in vitro e in vivo, sugerindo que a quantidade e/ou a qualidade da resposta imune gerada não suportam uma neutralização efetiva da Stx2 produzidas por linhagens de EHEC.

Detección y caracterización de Escherichia coli productor de toxina Shiga a partir de casos clínicos y de alimentos en Uruguay / Detection and characterization of Shiga toxin - producing Escherichia coli from clinical cases and food in Uruguay

Establecimos la frecuencia de aislamiento de Escherichia coli productor de toxina Shiga (STEC) a partir de muestras clínicas y de alimentos, así como las características fenotípicas y genotípicas de las cepas recuperadas. Se analizaron 198 muestras fecales de niños con diarrea sanguinolenta (DS), 14 muestras fecales de niños con síndrome urémico hemolítico (SUH) y 220 muestras de carne picada. También se estudiaron 4 cepas STEC aisladas de alimentos embutidos. Se recuperó STEC de 3 (1,5%) de los niños con DS, de 1 (7%) niño con SUH y de 4 (1,8%) de las muestras de carne picada. Todas las cepas fueron eae y ehxA positivas. Los serotipos detectados fueron: O157:H7 (9 cepas), O26:H11 (2 cepas), O111:NM (1 cepa) y O145:HNT (1 cepa). Todas las cepas O157:H7 portaron el subtipo eae-g1; las cepas O26:H11 y O145:HNT portaron el subtipo eae-b1 y la cepa O111:NM portó el subtipo eae-g2/q. Las cepas STEC del mismo serogrupo mostraron alta diversidad genética. En Uruguay STEC no sería agente frecuente de diarrea con sangre en niños. Sin embargo, las cepas recuperadas presentaron los genes asociados con enfermedad severa y 2 de los 3 niños infectados con STEC evolucionaron a SUH. La carne picada y otros alimentos serían vehículos importantes de O157:H7.

We have assessed the frequency of Shiga toxin-producing Escherichia coli (STEC) in clinical and food samples as well as studied the genotypic and phenotypic characteristics of the recovered strains. One hundred ninety eight fecal samples from children with bloody diarrhea (BD), 14 from children with hemolytic uremic syndrome (HUS), 220 ground beef samples and 4 STEC isolates from other beef-derived products were analyzed. The STEC strains were isolated from 3 (1.5%) children with bloody diarrhea, 1 (7%) from a child with HUS and 4 (1.8%) from ground beef samples. All strains were eae and ehxA positive. The serotypes found were: O157:H7 (9 strains), O26:H11 (2), O111: NM (1) and O145:HNT (1). All O157:H7 STEC strains harbored the eae subtype g1, O26:H11 and O145:HNT strains, subtype b1 and O111:NM strain, subtype g2/q. The STEC strains of the same serogroup showed high genetic diversity. In Uruguay, STEC is not frequently isolated from cases of bloody diarrhea in children. However, all the recovered STEC strains carried the genes associated with severe disease and 2 out of 3 children infected with STEC developed HUS. Ground beef and other food products might be important vehicles for O157:H7 strains.

Plasma Exchange in a Patient with Post-abortion Hemolytic Uremic Syndrome / 대한수혈학회지

Hemolytic uremic syndrome is characterized by microangiopathic hemolytic anemia, thrombocytopenia and severe renal failure. Microangiopathic thrombosis, thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) seem to occur under the condition of certain stresses, including pregnancy. Therapeutic plasma exchange (TPE) has proven to be an effective treatment for HUS and it has decreased the mortality of this illness. We experienced a case of a 34-year-old woman who was admitted for renal failure to our hospital immediately after an abortion. She presented with anuria, hemolytic anemia and thrombocytopenia. Plasma exchange was started and substitution was performed with fresh frozen plasma and four consecutive plasmapheresis sessions were administered. After about five days from the beginning of her illness, the signs of active hemolysis disappeared and her renal function partially recovered.

ADAMTS13 Activity in Childhood Hemolytic Uremic Syndrome(HUS)

PURPOSE: HUS usually occurs in children after infection with shiga toxin-producing microorganism(D+HUS). In contrast, non-postdiarrheal(D-) HUS occurs at any age and has a high rate of relapse and a poor prognosis. The clinical presentation of D-HUS is similar to that of thrombotic thrombocytopenic purpura(TTP). Recently severe deficiencies of ADAMTS13 were reported not only in TTP and D- HUS but also in D+ HUS during their acute phase. The purpose of the study is to evaluate the plasma ADAMTS13 activity in D+ and D-HUS. METHODS: Nineteen children with HUS(D+ HUS 12 and D- HUS 7) were enrolled. The assays of plasma ADAMTS13 activity were performed during the acute stage in the D+ HUS and at various stages of relapsing courses in the D- HUS patients by multimer assay, based on electrophoresis. RESULTS: The median plasma activity of ADAMTS13 in D+ HUS and D- HUS were 80.9%(37.8-132.4%) and 53.9%(1.0-94.1%), respectively, which were not statistically significantly different from control(86.4%, 34.2-112.3%)(P>0.05). One boy with D- HUS had severe deficiency of ADAMTS13(1.0%). His platelet count was normalized temporarily by fresh frozen plasma infusion. CONCLUSION: We have demonstrated that there is no significant difference of the plasma ADAMTS13 activity between D+ HUS, D- HUS and control. We detected severe deficiency of ADAMTS13 in one boy who presented with relapsing episodes of D- HUS. ADAMTS13 deficiency should be considered in the subgroup of D- HUS especially with early onset and recurrent courses. Plasma therapy can be beneficial in this subgroup.

A Case of Hemolytic Uremic Syndrome Induced by Pneumococcal Infection

Hemolytic Uremic Syndrome (HUS) is the most common cause of acute renal failure in children and is comprised of the combination of hemolytic anemia, thrombocytopenia, and acute renal failure. Atypical HUS, rare in childhood, has worse prognosis than that of typical HUS and is associated with chemotherapy drug, other bacterial (especially Streptococcus pneumoniae) or viral infections, and so on. We report a case of HUS caused by pneumococcal infection in 4-year-old boy. While he was admitted with pneumonia and pleural effusion, pneumococcal infection could be revealed. Although HUS progressed rapidly, he immediately received 3-time hemodialysis and recovered completely after two weeks.

Clinical Feature and Response to Plasma Exchange in Adult Patients with Diarrhea-associated Hemolytic Uremic Syndrome (D+HUS) / 대한신장학회잡지

Diarrea-associated hemolytic uremic syndrome (HUS) is very rare in adults. Few reports are available on clinical features and plasma exchange in adult patients with diarrhea-associated HUS in Korea. We retrospectively examined the records of five adult patients with diarrhea-associated HUS admitted to Samsung Seoul Hospital between January 1995 and December 1997. If the patient had neurologic abnormalities, or there was rapid clinical deterioration, with the hematocrit decreasing below 20%, the platelet count falling below 10,000/mm3, the creatinine concentration increasing above 5.0 mg/dl, plasma exchange was begun. There were 4 females and 1 male. Patients ranged in age from 16 to 61 years. All patients presented with diarrhea and abdominal pain, and 3 patients had bloody diarrhea. The mean time between the onset of diarrhea and thrombocytopenia was 4.4+/-1.9 days (range, 1 to 6). All patients received 7 to 24 plasma exchanges. The mean exchanged plasma volume was 1.1+/-0.2 times of patients own plasma volume. The pattern of clinical response to plasma exchange was initial normalization of platelet count (8.0+/-3.8 days), followed by normalization of LDH level (20.2+/-14.5 days) and creatinine concentration (25.8+/-13.8 days). Metabolic alkalosis developed in two patients undergoing daily plasma exchange. We successfully managed the metabolic alkalosis with continuous venovenous hemofiltration. The mean duration of hospitalization was 28.8+/-11.2days (range, 20 to 42). All patients successfully recovered without any sequale. Although this study is based on small case series, we suggested that plasma exchange may improve the outcome in adult diarrhea-associated HUS.

Immune Hemolytic Anemia and Acute Tubular Necrosis by Rifampicin Dependent Antibodies that Mimicked Hemolytic Uremic Syndrome / 대한신장학회잡지

A 52-year-old male admitted the hospital because of the spontaneous pneumothorax. About twenty years ago, he had experienced the pulmonary tuberculosis but he did not medicine the antituberculosis regulary. The pleural biopsy during right lobectomy and closed thoracotomy revealed the pleural tuberculosis and we instituted the re-trial of antituberculosis medication Acute oliguric renal failure occurred after the medications for seven days. The blood smear showed the evidence of microangiopathic hemolysis. We regarded the HUS deveoloped in this patient and four times of plasma exchage was accomplished. But the renal biopsy revealed the acute tubular necrosis due to the pigment nephropathy with interstitial inflammation and the careful laboratory evaluation showed the massive hemolysis with the compensated DIC. The rifampicin dependent antibodies were identified by indirect antiglobulin test. The microangiopathic finding would be due to DIC. We regarded that the renal failure was due to the hemolysis and, in minor part, was due to interstitial nephritis. Total plasma exchage, hemodialysis and prednisolone teatment resolved the hemolysis and the renal failure in this patient.