Amiloidose nodular - relato de rara apresentação da amiloidose localizada cutânea / Nodular amyloidosis - report of a rare presentation of cutaneous localized amyloidosis

Contexto: Amiloidose é um grupo de doenças caracterizadas pelo depósito de proteínas fibrilares, denominadas substância amiloide. Podem ser divididas em formas localizadas ou sistêmicas, sendo que dentre as localizadas, a forma nodular é a mais rara. Descrição do caso: Relatamos o caso de amiloidose primária localizada cutânea nodular que se apresentou com nódulos violáceos no dorso, e placas acastanhadas na região cervical há 8 anos sem evidências de envolvimento sistêmico. Discussão: Como cerca de 1% a 7% dos casos de amiloidose nodular localizada cutânea podem evoluir com envolvimento sistêmico, o seguimento dos pacientes faz-se necessário. O tratamento não é obrigatório, a retirada das lesões pode ser feita se o paciente o desejar, contudo as recidivas são frequentes. Conclusões: Mesmo possuindo baixa prevalência, a amiloidose nodular deve ser reconhecida pelo risco de progredir para acometimento sistêmico e associação com discrasias plasmocitárias, como mieloma múltiplo.

Evaluación de la formación de biopelículas en aislamientos bacterianos y fúngicos por el método semicuantitativo de microtitulación con cristal violeta y el cualitativo de agar con rojo Congo / Assessment of biofilms formation of bacterial and fungal isolates using qualitative Congo red agar and semiquantitative crystal violet microtiter methods

Introducción. El 65 % de las infecciones humanas son producidas por bacterias o levaduras, cuya capacidad de formar biopelículas las hace más resistentes a los antimicrobianos y antifúngicos. Objetivo. Determinar la capacidad de formación de biopelículas en aislamientos bacterianos y fúngicos por medio de los métodos cuantitativo de microtitulación con cristal violeta y cualitativo de cultivo en agar con rojo Congo. Materiales y métodos. Con el método cuantitativo, se utilizaron los medios de cultivo infusión cerebro-corazón, tripticasa de soya y Müeller-Hinton para aislamientos bacterianos; para levaduras, se usaron caldo infusión cerebro-corazón y Sabouraud dextrosa. Para el método cualitativo de cultivo en agar, se utilizaron los mismos medios de cultivo más una solución con 3 % de rojo Congo y 10 % de dextrosa. Cómo método de referencia, se utilizó la propuesta de Stepanovic et al. Resultados. Se evaluaron 103 aislamientos bacterianos y 108 de levaduras. No es recomendable sustituir el caldo infusión cerebro-corazón por los caldos tripticasa de soya y Müeller-Hinton en el método cuantitativo, para evaluar la formación de biopelículas en los aislamientos bacterianos. El medio Sabouraud dextrosa, en caldo y agar, puede sustituir al de infusión de cerebro-corazón para evaluar la formación de biopelículas en levaduras, tanto por el método cuantitativo como por el cualitativo. Conclusión. El estudio de las biopelículas en el laboratorio de microbiología, a partir del método cualitativo de cultivo en agar con rojo Congo, es un procedimiento sencillo, rápido y de bajo costo, que proporciona información útil para el diagnóstico y la terapéutica de infecciones persistentes causadas por bacterias y levaduras.

Introduction. Sixty-five percent of human infections are caused by bacteria or yeasts able to form biofilms. This feature makes them more resistant to antimicrobials and antifungals. Objective. To determine biofilm formation capacity of bacterial and fungal isolates by quantitative crystal violet microtiter and qualitative Congo red agar methods. Materials and methods. Brain-heart infusion, trypticase soy broth and Müeller-Hinton culture media were used in bacterial isolates for the quantitative method; brain-heart infusion broth and Sabouraud dextrose were used for yeasts. The same culture media plus 3% Congo red and 10% dextrose were used to apply the qualitative method in agar. The proposal by Stepanovic, et al. was used as a reference method. Results. We evaluated 103 bacterial isolates and 108 yeasts isolates. We did not recommend substitute brain-heart infusion broth for trypticase soy and Müeller-Hinton broths for biofilm formation assessment in bacterial isolates using the quantitative method. Sabouraud dextrose medium, both broth and agar, can replace brain-heart infusion to assess biofilm formation in yeasts, quantitatively and qualitatively. Conclusion. The study of biofilms in the microbiology laboratory, using Congo red agar qualitative method, is a simple, fast, and inexpensive procedure that provides precise information for the diagnosis and treatment of persistent infections caused by bacteria and yeasts.

Decolorization of the benzidine-based azo dye Congo red by the new strain Shewanella xiamenensis G5-03 / Descoloração do corante azo derivado da benzidina vermelho Congo por uma nova linhagem de Shewanella xiamenensis G5-03

Shewanella xiamenensis G5-03 was observed to decolorize the azo dye Congo red in synthetic wastewater. The influence of some factors on the dye decolorization efficiency was evaluated. The optimal decolorization conditions were temperature 30-35 °C, pH 10.0, incubation time 10 h, and static condition. The kinetic of Congo red decolorization fitted to the Michaelis­Menten model (Vmax = 111.11 mg L-1 h-1 and Km = 448.3 mg L-1). The bacterium was also able to degrade benzidine, a product of azo bond breakage of the Congo red, which contributed to reduce the phytotoxicity. The ability of S. xiamenensis G5-03 for simultaneous decolorization and degradation of Congo red shows its potential application for the biological treatment of wastewaters containing azo dyes.

Shewanella xiamenensis G5-03 foi capaz de descolorir o corante azo vermelho Congo em água residuária sintética. A influência de alguns fatores na eficiência da descoloração do corante foi avaliada. As condições ótimas de descoloração foram temperatura de 30-35 °C, pH 10,0 e condições estáticas. A cinética de descoloração do vermelho Congo se ajustou ao modelo de Michaelis­Menten (Vmax = 111,11 mg L-1 h-1 and Km = 448,3 mg L-1). A bactéria também foi capaz de degradar a benzidina, um produto da quebra da ligação azo do vermelho Congo, o que contribuiu para a redução da fitotoxicidade. A habilidade da S. xiamenensis G5-03 em simultaneamente descolorir e degradar o vermelho Congo demostra seu potencial de aplicação no tratamento de águas residuárias contendo corantes azo.

Amiloidosis cardiaca secundaria a macroglobulinemia de Waldenström / Cardiac amyloidosis secondary to Waldenström macroglobulinemia

Introducción: La macroglobulinemia de Waldenström constituye una neoplasia hematológica del grupo de las gammapatías monoclonales, que incluye síntomas sistémicos y relacionados al incremento de la paraproteína M. Objetivo: Describir un caso de amiloidosis cardiaca asociada a macroglobulinemia. Caso clínico: Paciente masculino que fue admitido por astenia, disfonía, y durante su evolución desarrolló disnea progresiva, insuficiencia cardiaca y efusión pleural. Adicionalmente, la ecocardiografía mostró patrón granular miocárdico, y la biopsia pleural resultó positiva para la tinción rojo congo. Posteriormente, recibió tratamiento con bortezomib, dexametasona y rituximab con evolución favorable. Conclusiones: En esta enfermedad el diagnóstico temprano es una ventaja importante para la supervivencia. Es por esa razón, que su manejo es paliativo de las manifestaciones cardiacas. El presente caso pone en manifiesto un reto diagnóstico, en el cual se deben tomar en cuenta las etiologías menos frecuentes de insuficiencia cardiaca(AU)

Introduction: Waldenström's macroglobulinemia is a hematological neoplasm belonging to the group of monoclonal gammopathies, which includes systemic symptoms and those related to an increase in M paraprotein. Objective: To describe a case of cardiac amyloidosis associated with macroglobulinemia. Clinical case: Male patient who was admitted for asthenia, dysphonia, and who, during his evolution, developed progressive dyspnea, heart failure and pleural effusion. Additionally, echocardiography showed myocardial granular pattern, while pleural biopsy was positive for Congo red staining. Subsequently, he received treatment with bortezomib, dexamethasone and rituximab, with favorable evolution. Conclusions: In this disease, early diagnosis is an important advantage for survival. Therefore, its management is palliative of cardiac manifestations. The present case shows a diagnostic challenge, in which the less frequent etiologies of heart failure must be taken into account(AU)

Assessment of different methods for the detection of biofilm production in coagulase-negative staphylococci isolated from blood cultures of newborns

Abstract INTRODUCTION: Coagulase-negative staphylococci (CoNS) are a frequent cause of bacteremia, especially in neonates. The major virulence determinant in CoNS is the ability to produce biofilms, which is conferred by the icaADBC genes. This study aimed to assess different methods for the detection of biofilm formation in 176 CoNS isolates from blood cultures of newborns. METHODS: The presence of the icaACD genes was assessed by polymerase chain reaction (PCR), and biofilm formation was assessed on congo red agar (CRA), by the tube method (TM), and on tissue culture plates (TCP). RESULTS: Of the 176 CoNS isolates, 30.1% expressed icaACD and 11.4% expressed icaAD. The CRA assay and TM showed that 42% and 38.6% of the isolates were biofilm producing, respectively. On TCP, 40.9% of the isolates produced biofilms; 21% were weakly adherent and 19.9% were strongly adherent. When compared to the gold standard technique (PCR), the CRAassay showed 79% sensitivity and 84% specificity (kappa = 0.64), TM showed 78% sensitivity and 89% specificity (kappa = 0.68), and TCP showed 99% sensitivity and 100% specificity (kappa = 0.99). CONCLUSIONS: In this study, ~42% of CoNS isolates produced biofilms, and the presence of icaACD was associated with a greater capacity to form biofilms. Compared to the other phenotypic methodologies, TCP is an ideal procedure for routine laboratory use.

Amiloidosis AL: reporte de caso clínico en adulto mayor / Amyloidosis AL: clinical case report in elderly

Se presenta el caso clínico de una paciente de 63 años, con indicación de tiroidectomía total, a causa de un bocio multinodular con infiltrado neoplásico folicular. La evolución posquirúrgica fue tórpida y se caracterizó por la presencia de insuficiencia cardiaca aguda, hepatomegalia, neuropatía periférica y equimosis periorbitaria, condiciones clínicas sugerentes de amiloidosis, la que fue confirmada por medio de estudios como amiloidosis de cadenas ligeras (AL).

We present the clinical case of a 63-year-old patient, with indication of total thyroidec-tomy, due to a multinodular goiter with a follicular neoplastic infiltrate. Postoperative evolution was torpid and was characterized by the presence of acute heart failure, hepatomegaly, peripheral neuropathy and periorbital ecchymosis, clinical conditions suggestive of amyloidosis, which was confirmed by studies such as amyloidosis of light chains (AL).

Optimization of phenoxazinone synthase production by response surface methodology and its application in Congo red decolourization

Background: Enzymatic decolourization has been recently proposed as a promising and eco-friendly method for treatment of synthetic dye-contaminated wastewaters. However, the processes require large quantities of enzymes, attracting significant attention in developing efficient methods for mass production of multifunctional enzymes. Several methods such as response surface methodology (RSM) and orthogonal experiment have been applied to optimize the parameters in bioprocesses for enzyme production. Results: In the present study, a laccase-like enzyme, phenoxazinone synthase (PHS) originated from Streptomyces antibioticus was recombinantly expressed in Escherichia coli BL21 (DE3). The production of PHS in E. coli BL21 was optimized by response surface methodology based on Box-Behnken design. A full third-order polynomial model was generated by data analysis with Statistica 8.0 in which the optimal conditions for PHS production were calculated to be 1.525 mM CuSO4 and 16.096 hrs induction at temperature of 29.88ºC. The highest PHS production under optimal conditions was calculated to be 4098.51 U/l using the established model. Average PHS production obtained from actual production processes carried out under the calculated optimal conditions was 4052.00 U/l, very close to the value predicted by the model. Crude PHS was subsequently tested in Congo red decolourization which exhibited a low decolourization rate of 27% without mediator. Several mediators were found to improve PHS-catalyzed Congo red decolourization, with the highest rate of 73.89% obtained with 2,2’-azinobis-3-ethylbenzothiazoline-6-sulfonic acid (ABTS) as mediator under optimized conditions of 4000 U/l PHS activity, 10 μM ABTS, 100 μM Congo red, and 8 hrs reaction time. Conclusion: Our results indicated that PHS recombinantly produced in E. coli BL21 was a prospective enzyme for decolorizing reactive dye Congo red.

Comparison of methods for the detection of biofilm formation by Staphylococcus aureus isolated from bovine subclinical mastitis

Biofilm formation is considered to be a selective advantage for Staphylococcus aureus mastitis isolates by facilitating bacterial persistence in the udder. It requires attachment to mammary epithelium, proliferation and accumulation of cells in multilayers. The objective of this study was to determine the sensitivity and specificity of three techniques for the detection of S. aureus biofilm-positive strains. Two phenotypic tests, including growth on microtitre plates and Congo red agar, were compared with a PCR technique using 94 S. aureus strains obtained from cows with subclinical mastitis from two farms in the state of São Paulo. These strains were characterised by in vitro slime production on Congo red agar, biofilm formation on microtitre plates and the presence of the icaA and icaD genes. The results revealed that 85% of the isolates tested produced slime on the Congo red agar, 98.9% of the isolates produced biofilms in vitro by adhering to sterile 96-well "U" bottom polystyrene tissue culture plates, and 95.7% of the isolates carried the icaA and icaD genes. The results of the phenotypic tests for biofilm formation were compared with those of the molecular analysis, and the sensitivity and specificity of the Congo red agar test were 88.9% and 100%, respectively, while those of the microtitre plate test were 100% and 25%, respectively. When the phenotypic methods for the detection of biofilm producers, namely growth on microtitre plates and Congo red agar, were compared, the sensitivity and specificity were 86% and 100%, respectively. Therefore, growth on Congo red agar and the microtitre plate test are methods that could be used to determine whether an isolate has the potential for biofilm production.

Antimicrobial resistance of Staphylococcus spp. from small ruminant mastitis in Brazil / Resistência antimicrobiana de Staphylococcus spp. de mastite de pequenos ruminantes no Brasil

The study aimed to determine the antimicrobial resistance patterns and to identify molecular resistance markers in Staphylococcus spp. (n=210) isolated from small ruminant mastitis in Brazil. The antimicrobial resistance patterns were evaluated by the disk diffusion test and by detection of the presence of mecA, blaZ, ermA, ermB, ermC and msrA genes by PCR. The efflux pump test was performed using ethidium bromide and biofilm production was determined by Congo red agar test along with PCR for detection of the icaD gene. The isolates were most resistant to amoxicillin (50.0%), streptomycin (42.8%), tetracycline (40.4%), lincomycin (39.0%) and erythromycin (33.8%). Pan-susceptibility to all tested drugs was observed in 71 (33.8%) isolates and 41 Staphylococcus isolates were positive for the efflux pump. Although phenotypic resistance to oxacillin was observed in 12.8% of the isolates, none harbored the mecA gene. However, 45.7% of the isolates harbored blaZ indicating that beta-lactamase production was the main mechanism associated with staphylococci resistance to beta-lactams in the present study. The other determinants of resistance to antimicrobial agents ermA, ermB, ermC, and msrA were observed in 1.4%, 10.4%, 16.2%, and 0.9% of the isolates, respectively. In addition, the icaD gen was detected in 32.9% of the isolates. Seventy three isolates (54 from goats and 19 from sheep) were negative for all resistance genes tested and 69 isolates presented two or more resistance genes. Association among blaZ, ermA, ermB, ermC and efflux pump were observed in 17 isolates, 14 of which originated from goats and three from sheep. The data obtained in this study show the resistance of the isolates to beta-lactamics, which may be associated with the use of antimicrobial drugs without veterinary control.

O presente trabalho teve como objetivo determinar os padrões de resistência a agentes antimicrobianos e identificar marcadores moleculares de resistência em Staphylococcus spp. (n=210) isolados de mastite de pequenos ruminantes no Brasil. Os padrões de resistência a agentes antimicrobianos foram avaliados pelo teste de difusão em disco e pela detecção da presença dos genes mecA, blaZ, ermA, ermB, ermC e msrA via PCR. O teste da bomba de efluxo foi realizado utilizando brometo de etídio e a produção de biofime foi determinada pelo teste do vermelho congo em paralelo com o PCR para detecção do gene icaD. Os isolados foram mais resistentes a amoxicilina (50,0%), estreptomicina (42,8%), tetraciclina (40,4%), lincomicina (39,0%) e eritromicina (33,8%). Setenta e um (33,8%) isolados foram sensíveis a todas as drogas testadas e 41 foram positivos para a bomba de efluxo. Embora a resistência fenotípica a oxacilina tenha sido observada observada em 12,8% dos isolados, nenhum possuiu o gene mecA. Entretanto, 45,7% dos isolados continham a gene blaZ, indicando que a produção de beta-lactamases foi o principal mecanismo associado com a resistência dos Staphylococcus aos beta-lactâmicos. Os outros determinantes de resistência a agentes antimicrobianos ermA, ermb, ermC e msrA foram observados em 1,4%, 10,4%, 16,2% e 0,9% dos isolados respectivamente. Além disso, o gene icaD foi detectado em 32,9% dos isolados. Setenta e três isolados (54 de cabras e 19 de ovelhas) foram negativos para todos os genes de resistência testados e 69 isolados apresentaram dois ou mais genes de resistência. A associação entre blaZ, ermA, ermB, ermC e bomba de efluxo foi observada em 17 isolados dos quais 14 eram oriundos de cabras e três de ovelhas. Os dados obtidos no presente estudo indicam a resistência dos isolados aos beta-lactâmicos, o que pode estar associado ao uso sem controle veterinário destas drogas nos animais.

Primary systemic amyloidosis associated with multiple myeloma / Amiloidose sistêmica primária associada ao mieloma múltiplo

This case report is about a 48-year-old female patient with systemic amyloidosis and multiple myeloma simultaneously. Amyloid cutaneous infiltrative lesions like papules, nodules, or plaques with a serous-hemorrhagic aspect were found in the eyelids, neck and retroauricular region, among others. She had presented intermittent papular lesions on the upper eyelids one year before, which worsened following local trauma. A local skin biopsy showed amorphous and eosinophilic substance in the dermis. Congo red staining confirmed the amyloid deposits. Abnormal exams: proteinuria (570mg/24h), Bence-Jones proteinuria and clonal plasma cells (70%) found in myelogram. Following the diagnosis of multiple myeloma based on amyloid skin lesions, the patient was referred to the Hematology service and died 5 months after the diagnosis.

Relatamos um caso de uma paciente de 48 anos com amiloidose sistêmica associada a mieloma múltiplo. Lesões infiltrativas cutâneas como pápulas, nódulos ou placas com aspecto sero-hemorrágico podem ser localizados nas pálpebras, pescoço, região retroauricular dentre outras. No presente caso, as pálpebras foram acometidas por pápulas, há 1 ano, de caráter intermitente e piora após trauma local. Biópsia local evidenciou material amorfo e eosinofílico na derme. A coloração vermelho do Congo confirmou presença de substância amiloide. Exames anormais: proteinúria de 570mg/24 horas, proteinúria de Bence-Jones positiva e mielograma com 70% de plasmócitos atípicos. Assim, realizou-se o diagnóstico de mieloma múltiplo a partir de manifestações cutâneas de amiloidose. Paciente encaminhada ao serviço de hematologia e foi a óbito em 5 meses.

A simple and efficient diffusion technique for assay of endo b-1, 4-xylanase activity

Endo-β-1, 4-xylanases is thought to be of great significance for several industries namely paper, pharmaceuticals, food, feed etc. in addition to better utilization of lignocellulosic biomass. The present investigation was aimed to develop an easy, simple and efficient assay technique for endo-β-1, 4-xylanases secreted by the aerobic fungi. Under the proposed protocol, 9 g/L xylan containing agar was prepared in 100 mM phosphate buffer at different pH (4.5, 5.5 and 6.5). The sterilized xylan agar was dispensed in 90 mm petri dishes. 100 µl of culture supernatant of 12 fungal isolates was added to the wells and left overnight at 31±10C. The petri dishes were observed for zone of clearance by naked eye and diameter was measured. Congo red solution (1 g/L) was applied over the petri dishes as per the established protocol and thereafter plates were flooded with 1M Sodium chloride solution for the appearance of zone of clearance. The diameter for zone of clearance by the proposed method and the established protocol was almost identical and ranged from 21 to 42 mm at different pH depending upon the activity of endo-β-1, 4-xylanases. Change of pH towards alkaline side enabled similar or marginal decrease of diameter for the zone of clearance in most of the fungal isolates. The specific activities of these fungal isolates varied from 1.85 to 11.47 IU/mg protein. The present investigation revealed that the proposed simple diffusion technique gave similar results as compared to the established Congo red assay for endo-β-1, 4-xylanases. Moreover, the present technique avoided the cumbersome steps of staining by Congo red and de-staining by sodium chloride.

Mycoremediation of Congo red dye by filamentous fungi

Azo, anthroquinone and triphenylmethane dyes are the major classes of synthetic colourants, which are difficult to degrade and have received considerable attention. Congo red, a diazo dye, is considered as a xenobiotic compound, and is recalcitrant to biodegradative processes. Nevertheless, during the last few years it has been demonstrated that several fungi, under certain environmental conditions, are able to transfer azo dyes to non toxic products using laccases. The aim of this work was to study the factors influencing mycoremediation of Congo red. Several basidiomycetes and deuteromycetes species were tested for the decolourisation of Congo red (0.05 g/l) in a semi synthetic broth at static and shaking conditions. Poor decolourisation was observed when the dye acted as the sole source of nitrogen, whereas semi synthetic broth supplemented with fertilizer resulted in better decolourisation. Decolourisation of Congo red was checked in the presence of salts of heavy metals such as mercuric chloride, lead acetate and zinc sulphate. Decolourisation parameters such as temperature, pH, and rpm were optimized and the decolourisation obtained at optimized conditions varied between 29.25- 97.28 percent at static condition and 82.1- 100 percent at shaking condition. Sodium dodecyl sulphate polyacrylamide gel electrophoretic analysis revealed bands with molecular weights ranging between 66.5 to 71 kDa, a characteristic of the fungal laccases. High efficiency decolourisation of Congo red makes these fungal forms a promising choice in biological treatment of waste water containing Congo red.

Amiloidose macular de localização atípica / An unusual presentation of macular amyloidosis

A amiloidose macular é uma forma cutânea primária de amiloidose, caracterizada tipicamente por máculas acastanhadas, acometendo predominantemente a região interescapular. O presente caso é o de uma mulher de 45 anos, há um ano com manchas hipercrômicas, rendilhadas e não pruriginosas, em membros superiores, região cervical, sacral e membros inferiores. No estudo histopatológico da biópsia de pele, foi evidenciada substância amiloide amorfa no topo das papilas dérmicas, que se tornou mais evidente após a coloração pelo vermelho Congo. Não havia alterações sistêmicas associadas. Além da localização atípica e extensa da amiloidose cutânea macular, o presente caso procura acrescentar dados para esclarecimento do tema.

Macular amyloidosis is a form of cutaneous amyloidosis characterized by dusky-brown lesions usually located on the upper back between the shoulder blades. This report describes the case of a 45year-old female presenting with hyperpigmented macules and lace-like, non-pruritic erythema in the sacral and cervical region as well as on both arms and legs. Histology revealed amyloid deposits in the papillary dermis which exhibited apple-green birefringence after Congo red staining. There were no systemic findings. This is a case of macular amyloidosis with an unusual presentation. The literature on the subject is also reviewed.

Adherence assays and Slime production of Vibrio alginolyticus and Vibrio parahaemolyticus / Ensaios de adesão e produção de muco por Vibrio alginolyticus e Vibrio parahaemolyticus

In this study we investigated the phenotypic slime production of Vibrio alginolyticus and Vibrioparahaemolyticus strains, food-borne pathogens, using a Congo red agar plate assay. Furthermore, westudied their ability to adhere to abiotic surfaces and Vero cells line. Our results showed that only V.alginolyticus ATCC 17749 was a slime-producer developing almost black colonies on Congo red agar plate.Adherence to glace tube showed that all V. alginolyticus strains were more adherent than V. parahaemolyticus.Only V. alginolyticus ATCC 17749 was found to be able to form biofilm on polystyrene microplate wells (OD570= 0.532). Adherence to Vero cells showed that all tested strains were non adherent after 30 min, however after60 min all the studied strains become adherent. The percentage of adherence ranged from1.23% to 4.66%.

Neste estudo, investigou-se a produção de muco por cepas de Vibrio alginolyticus e Vibrio parahaemolyticus através do teste em placa de ágar com vermelho congo. Estudou-se também a capacidade de adesão à superfícies abióticas e células Vero. Os resultados indicaram que somente V. alginolyticus ATCC 17749 produziu muco, formando colônias quase negras nas placas de ágar com vermelho congo. O teste de adesão a tubos de vidro indicou que as cepas de V. alginolyticus foram maisaderentes do que as de V. parahaemolyticus. Somente V. alginolyticus ATCC 17749 foi capaz de formar biofilme nos poços das microplacas de poliestireno (OD570=0,532). Testes de adesão a células Vero mostraram que nenhuma das cepas apresentou adesão em 30 min, mas todas aderiram após 60 min. Aporcentagem de adesão variou de 1,23% a 4,66%.

Amiloidosis: enfermedad desafiante en su diagnóstico y tratamiento / Amyloidosis: diagnosis and treatment

La amiloidosis es una enfermedad sistémica caracterizada por el depósito de fibrillas amiloídeas en diversos órganos, lo que lleva a un deterioro y falla progresiva de éstos, afectando sustancialmente la sobrevida del paciente. Se analizaron los registros médicos de 34 pacientes egresados con diagnóstico de amiloidosis del Hospital Clínico de la Universidad de Chile en un período de 15 años. El promedio del tiempo entre la aparición de síntomas y el diagnóstico fue aproximadamente de seis meses. Los compromisos sistémicos de mayor relevancia fueron el renal y cardíaco, influyendo este último notablemente en la sobrevida. El laboratorio inmunológico tuvo un rol fundamental en identificar a los pacientes portadores de una paraproteína y de esta manera apoyar el diagnóstico. Es necesario plantearse este diagnóstico diferencial en todo paciente con compromiso sistémico de causa no clara y más aún cuando está asociado un trastorno mieloproliferativo.

Amyloidosis is a systemic disease characterized by the deposition of amyloid fibrils in many organs, leading to a progressive deterioration and failure of these, substantially affecting the survival of the patient. We present 34 medical records of patients that were hospitalized with the diagnosis of amyloidosis in the Hospital of the Universidad de Chile, over a period of fifteen years. The average time between the onset of symptoms and diagnosis was approximately six months. The most important systemic affectations were to the kidney and heart, the latter significantly influences survival. The immunology laboratory plays a fundamental role in identifying patients with paraprotein, thus supporting diagnosis. It is necessary to consider this differential diagnosis in all patients with systemic symptoms of no clear cause, especially when it is associated to a myeloproliferative disorder.

Evaluación de la produccion de beta - amiloide por la mutación E280A en el gen de la presenilina 1 / Evaluation of amyloid - beta by the E280A mutation in presenilin gene

Introducción. La mutación E280A en el gen de presenilina 1 se encuentra asociada al grupo familiar más grande del mundo con enfermedad de Alzheimer. La presenilina 1 es un componente esencial del complejo g-secretasa, responsable de la producción del péptido beta-amiloide, considerado clave en la fisiopatogenia de la enfermedad. Objetivo. Determinar si la mutación E280A en presenilina 1 incrementa la producción de beta-amiloide, como reflejo de la ganancia de función g-secretasa. Materiales y métodos. Se evaluaron, por la tinción con rojo congo e inmunohistoquímica, depósitos sistémicos de beta-amiloide en tejidos de cadáveres afectados, portadores de la mutación E280A en la presenilina 1 y cadáveres de no afectados. Se cuantificó por la técnica de ELISA el beta-amiloide de 40 y 42 aminoácidos en cultivos de células CHO que expresan la proteína precursora de amiloide, transfectadas con los cADN de presenilina 1 portando las mutaciones M146L, E280A, DE9 y L392V. Resultados. Se encontraron depósitos proteicos en todos los tejidos estudiados y escasos depósitos de beta-amiloide. No se encontró diferencia en la cantidad de depósitos, ni en su localización. No se observó incremento en la producción de beta-amiloide en las células transfectadas con los cADN de presenilina 1 con las mutaciones comparadas con los controles. Conclusiones. La mutación E280A en presenilina 1 no aumentó la producción de beta-amiloide en tejidos periféricos no neuronales o en el modelo in vitro, contrario a lo que sucede en una ganancia de función de g-secretasa.

Introduction. The E280A mutation of the presenilin 1 gene has been found to be the most common associate in Alzheimer’s patients with a family history of this disease. Presenilin 1 is a critical component of the g-secretase complex and plays an essential role in the production of amyloid-b peptide. This peptide has been strongly associated with the physiopathology of the disease. Objective. The E280A mutation in the presenilin 1 was investigated for increased production of amyloid-b, as a response to gain in g-secretase function. Materials and methods. Levels of systemic amyloid-b were measured with congo red staining and immuno-histochemistry of the tissues of affected cadavers, compared with non-affected cadavers. The 40 and 42 amino acid amyloid-b levels were quantified by ELISA assay in CHO cell cultures. The amyloid precursor protein expressed by the cultures was detected by transfection with the cDNAs of presenilin 1 carrying the M146L, E280A, DE9 y L392V mutations. Results. Protein deposits were found in all tissues investigatged, but only a few with b-amyloid deposition. No differences were observed in the amount or location of amyloid-b between affected and unaffected cadavers. Not increase was noted in the production of amyloid-b from the CHO cells transfected with cDNA from any of the mutations of presenilin 1. Conclusions. The E280A mutation in the presenilin 1 gene was not associated with the increased production of amyloid-b in non-neuronal peripheral tissues, or in the in vitro model. This is in contrast to the expectation in a g-secretase gain of function.

Diagnóstico de amiloidosis mediante biopsia de glándula salival menor / Diagnosis of amiloidosis biopsy of minor glands

Objetivo: diagnosticar amiloidosis biopsia de glándula salival menor. Material y método: se utilizó el método de observación, retrospectivo y descriptivo. El estudio consistió en obtener resultados de 60 pacientes que se realizaron biopsias de glándula salival menor para descartar amiloidosis. Los pacientes estaban hospitalizados por otras patologías y tenían sospecha clínica de amiloidosis; por esa razón todos los pacientes traían una hoja de interconsulta. Resultados: de las 60 biopsias, 38 resultaron negativas y 22 resultaron positivas para amiloidosis. De los 60 pacientes, 13 tuvieron antecedentes de TBC, de los cuales 7 hicieron la enfermedad de amiloidosis. Conclusiones: la biopsia de glándula salival es útil para diagnosticar amiloidosis; además, es más accesible en comparación con las biopsias de riñón, mucosa rectal y grasa abdominal, que también se realizan para diagnosticar amiloidosis. De 16 pacientes con biopsia que tenían síndrome nefrótico, todos resultaron negativos para amiloidosis.

Objective: to diagnose amyloidosis biopsy of minor salivary gland. Material and Method: I used the method of observation, retrospective and descriptive. The study consisted of results of 60 patients who were minor salivary gland biopsied to rule out amyloidosis. Patients were hospitalized for other ailments and had clinical suspicion of amyloidosis; that is why all patients brought a sheet interconsultation. Results: Of the 60 biopsies, 38 were negative and 22 were positive for amyloidosis. Of the 60 patients, 13 had a history of TB, of which 7 were disease amyloidosis. Conclusions: The biopsy of the salivary gland is useful for diagnosing amyloidosis; moreover, they are more accessible compared to the biopsies of Kidney, rectal mucosa and abdominal fat, which also are made for diagnosing amyloidosis. Biopsies of 16 patients who had nephrotic syndrome, all were negative for amyloidosis.

Amiloidosis endobronquial / Tracheobronchial amyloidosis: case report

Amyloidosis is a generic term for a group of diseases that share the common feature of extracelular deposit of pathologic proteins. Systemic and localized forms are recognized. Both can produce pulmonary involvement. The current classification is based on the nature of the precursor of the amyloid. It is an infrequent condition, in USA the incidence is 5.1 to 12.8 per million of people. We present a case of a 32 years old male, obese, light smoker with Diabetes Mellitus 2, asymptomatic, with a pneumonia and poor response to treatment. The first diagnostic approach was a malignant disease and the histological study showed Amyloidosis. The analysis of the case suggest the diagnosis of tracheobronchial amyloidosis.

La amiloidosis es un término genérico para un conjunto de enfermedades que comparten como hecho común el depósito extracelular de proteínas patológicas. A grandes rasgos se divide en sistémica y localizada. Ambas pueden presentar compromiso pulmonar. Actualmente, su clasificación, se basa en las proteínas precursoras del amiloide. Es una patología poco frecuente, reportándose en USA una incidencia de 5,1 a 12,8 por millón de personas. Presentamos un caso de un enfermo de 32 años de edad obeso con antecedentes de tabaquismo y Diabetes Mellitus 2, asintomático que frente a una neumonía con mala respuesta al tratamiento se plantea una patología maligna y el estudio histológico demuestra amiloidosis. El análisis del caso clínico configura el diagnostico de Amiloidosis localizada endobronquial.

Amiloidosis, Comunicación de 11 casos y revisión de la literatura / Clinical features of patients with the pathological diagnosis of amyloidosis

Background: Amyloidosis is characterized by the extracellular deposit of an insoluble fibrillar protein that leads to tissue atrophy and necrosis. Aim: To report the clinical features of cases of amyloidosis diagnosed in a public hospital in Santiago, Chile, from 2000 to 2004. Material and methods: Retrospective review of all pathology reports of biopsies obtained from 2000 to 2004. In all cases reported as "amyloidosis", the clinical features of such patients were obtained from their medical records. Results: The medical records of 11 patients with amyloidosis were obtained (aged 35 to 71 year old, seven females). Seven had a systemic and four a localized disease. Six patients had primary amyloidosis and in one, it was secondary to a disseminated tuberculosis. Five patients with the generalized disease consulted for anarsarca, three for weight loss and 2 for chronic diarrhea. Patients with localized disease consulted for tonsil enlargement, dysphonia and skin lesions. Five patients with generalized disease had renal involvement and five had cardiac involvement. Three patients had malabsorption. Conclusions: The most common presentation of systemic amyloidosis is anasarca and renal involvement is common.