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      Síndrome hemolítico urémico Translated title: Uremic haemolytic syndrome

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          Abstract

          Se realiza una revisión de los aspectos generales del síndrome hemolítico urémico (SHU): epidemiología, patogenia, manifestaciones clínicas y tratamiento. El SHU es el resultado de la acción de numerosos factores etiológicos y patogénicos, causado principalmente por una verotoxina producida por diferentes cepas de Escherichia coli. En esta entidad se produce un daño endotelial que genera una serie de fenómenos tales como: adhesión, agregación plaquetaria y depósitos de fibrina, que llevan a la formación de trombos en la microcirculación: la microangiopatía trombótica. El SHU se caracteriza por la combinación de insuficiencia renal aguda, trombocitopenia y anemia hemolítica microangiopática. Las diarreas y las infecciones del tracto respiratorio superior son los factores precipitantes más comunes. La plasmaféresis y la diálisis son actualmente el tratamiento de elección

          Translated abstract

          It was made a review of the general aspects of the uremic haemolytic syndrome (UHS): epidemiology, pathogeny, clinical manifestations and treatment. UHS results from the action of numerous ethiological and pathogenic factors It is mainly caused by a verotoxin produced by different Escherichia coli strains. In this entity, it is produced an endothelial damage that generates a series of phenomena, such as adhesion, platelet aggregation and deposits of fibrin, leading to the formation of thrombi in the microcirculation: thrombotic microangiopathy. UHS is characterized by a combination of acute renal failure, thrombocytopenia and microangiopathic haemolytic anemia. Diarrheas and upper respiratory tract infections are the most common precipitating factors. Plasmaphaeresis and dialysis are nowadays the election treatment

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          Most cited references56

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          Quinolone antibiotics induce Shiga toxin-encoding bacteriophages, toxin production, and death in mice.

          Shiga toxin-producing Escherichia coli (STEC) cause significant disease; treatment is supportive and antibiotic use is controversial. Ciprofloxacin but not fosfomycin causes Shiga toxin-encoding bacteriophage induction and enhanced Shiga toxin (Stx) production from E. coli O157:H7 in vitro. The potential clinical relevance of this was examined in mice colonized with E. coli O157:H7 and given either ciprofloxacin or fosfomycin. Both antibiotics caused a reduction in fecal STEC. However, animals treated with ciprofloxacin had a marked increase in free fecal Stx, associated with death in two-thirds of the mice, whereas fosfomycin did not. Experiments that used a kanamycin-marked Stx2 prophage demonstrated that ciprofloxacin, but not fosfomycin, caused enhanced intraintestinal transfer of Stx2 prophage from one E. coli to another. These observations suggest that treatment of human STEC infection with bacteriophage-inducing antibiotics, such as fluoroquinolones, may have significant adverse clinical consequences and that fluoroquinolone antibiotics may enhance the movement of virulence factors in vivo.
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            The molecular basis of familial hemolytic uremic syndrome: mutation analysis of factor H gene reveals a hot spot in short consensus repeat 20.

            The aim of the present study was to clarify whether factor H mutations were involved in genetic predisposition to hemolytic uremic syndrome, by performing linkage and mutation studies in a large number of patients from those referred to the Italian Registry for Recurrent and Familial HUS/TTP. PCR and Western blot analyses were conducted to characterize the biochemical consequences of the mutations. Five mutations in the factor H gene were identified. Three, identified in two families and in a sporadic case, are heterozygous point mutations within the most C-terminal short consensus repeat 20 (SCR20) of factor H, resulting in single amino acid substitutions. The other two mutations introduce premature stop codons that interrupt the translation of factor H. A heterozygous nonsense mutation was identified in SCR8 in one family, and a homozygous 24-bp deletion within SCR20 was identified in a Bedouin family with a recessive mode of inheritance. Reverse transcription-PCR analysis of cDNA from peripheral blood leukocytes from the Bedouin family showed that the deletion lowered factor H mRNA levels. Although heterozygous mutations were associated with normal factor H levels and incomplete penetrance of the disease, the homozygous mutation in the Bedouin family resulted in severe reduction of factor H levels accompanied by very early disease onset. These data provide compelling molecular evidence that genetically determined deficiencies in factor H are involved in both autosomal-dominant and autosomal-recessive hemolytic uremic syndrome and identify SCR20 as a hot spot for mutations in the disease. The mutations identified here give an important hint to the relevance of the C-terminus of factor H in the control of the alternative complement activation pathway.
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              A new biological agent for treatment of Shiga toxigenic Escherichia coli infections and dysentery in humans.

              Gastrointestinal disease caused by Shiga toxin-producing bacteria (such as Escherichia coli O157:H7 and Shigella dysenteriae) is often complicated by life-threatening toxin-induced systemic sequelae, including hemolytic-uremic syndrome. Such infections can now be diagnosed very early in the course of the disease, but at present no effective therapeutic intervention is possible. Here, we constructed a recombinant bacterium that displayed a Shiga toxin receptor mimic on its surface, and it adsorbed and neutralized Shiga toxins with very high efficiency. Moreover, oral administration of the recombinant bacterium completely protected mice from challenge with an otherwise 100%-fatal dose of Shiga toxigenic E. coli. Thus, the bacterium shows great promise as a 'probiotic' treatment for Shiga toxigenic E. coli infections and dysentery.
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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Journal
                hih
                Revista Cubana de Hematología, Inmunología y Hemoterapia
                Rev Cubana Hematol Inmunol Hemoter
                Editorial Ciencias Médicas (Ciudad de la Habana )
                1561-2996
                December 2003
                : 19
                : 2-3
                : 0
                Affiliations
                [1 ] Instituto de Hematología e Inmunología Cuba
                [2 ] Hospital Docente José Ramón Martínez Cuba
                [3 ] Hospital General Universitario Comandante Pinares Cuba
                [4 ] Hospital Pediátrico William Soler Cuba
                Article
                S0864-02892003000200002
                fee611cc-28e3-43b0-9647-5f302b88f44c

                http://creativecommons.org/licenses/by/4.0/

                History
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                SciELO Cuba

                Self URI (journal page): http://scielo.sld.cu/scielo.php?script=sci_serial&pid=0864-0289&lng=en
                Categories
                HEMATOLOGY

                Hematology
                HEMOLYTIC-UREMIC SYNDROME,ESCHERICHIA COLI,KIDNEY FAILURE, ACUTE,TROMBOCYTOPENIA,ANEMIA, HEMOLYTIC,CHILD,SINDROME HEMOLITICO- UREMICO,SINDROME HEMOLITICO-UREMICO,INSUFICIENCIA RENAL AGUDA,TROMBOCITOPENIA,ANEMIA HEMOLÍTICA,NIÑO

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