Genetic diversity of Echinococcus vogeli in the western Brazilian Amazon

Human polycystic echinococcosis is a parasitic infection caused by the larval stage of Echinococcus vogeli, which occurs in rural areas of Central and South America. Until now, little information on the genetic variability of E. vogeli is available. Here, 32 samples from human-excised E. vogeli cysts had a 396-bp sequence of the mitochondrial cytochrome oxidase I (COI) gene sequenced and compared to another 17 COI sequences representing nine Echinococcus species. A Bayesian COI tree revealed that all E. vogeli sequences formed a monophyletic and well-supported clade with an E. vogeli reference sequence. The occurrence of geographically restricted E. vogeli COI haplotypes suggests retention of ancestral polymorphisms with little migration in Acre, Brazil.

Polycystic echinococcosis in the state of Acre, Brazil: contribution to patient diagnosis, treatment and prognosis

The lack of knowledge regarding polycystic hydatid disease results in delayed or even incorrect diagnosis. The lack of systematic information regarding treatment also makes it difficult to assess the results and prognosis in patients with peritoneal and hepatic lesions caused by Echinococcus vogeli. Here we describe the clinical features of patients, propose a radiological classification protocol and describe a therapeutic option for the treatment of hydatid disease that previously had only been used for cases of cystic echinococcosis (Echinococcus granulosus). A prospective cohort study was initiated in 1999 and by 2009 the study included 60 patients. These patients were classified according to the PNM classification (parasite lesion, neighbouring organ invasion and metastases) and placed in one of three therapeutic modalities: (i) chemotherapy with albendazole at a dose of 10 mg/kg/day, (ii) surgical removal of cysts or (iii) percutaneous puncture of the cysts via puncture, aspiration, injection and re-aspiration (PAIR). The results were stratified according to therapeutic outcome: "cure", "clinical improvement", "no improvement", "death" or "no information". The PNM classification was useful in indicating the appropriate therapy in cases of polycystic hydatid disease. In conclusion, surgical therapy produced the best clinical results of all the therapies studied based on "cure" and "clinical improvement" outcomes. The use of PAIR for treatment requires additional study.

Liver transplantation for neotropical polycystic echinococcosis caused by Echinococcus vogeli: a case report

Neotropical polycystic echinococcosis (NPE) is a parasitic disease caused by cestodes of Echinococcus vogeli. This parasite grows most commonly in the liver, where it produces multiples cysts that cause hepatic and vessel necrosis, infects the biliary ducts, and disseminates into the peritoneal cavity, spreading to other abdominal and thoracic organs. In cases of disseminated disease in the liver and involvement of biliary ducts or portal system, liver transplantation may be a favorable option. We present a report of the first case of liver transplantation for the treatment of advanced liver NPE caused by E. vogeli.

Hidatidose policística no norte do Brasil: abordagem parasitológica e histopatológica de pacas e casos humanos / Polycystic hydatid in northern Brazil: parasitological and histopathological approach from human and pacas cases

A hidatidose policística é uma zoonose causada pelo cestóide Echinococcus vogeli, amplamente distribuído no norte do Brasil. Os hospedeiros definitivos são Speothos venaticus (cachorro-vinagre) e Canis familiaris (cães domésticos), enquanto Agouti paca (paca) é hospedeiro intermediário. Tanto as pacas quanto o homem (hospedeiro acidental) desenvolvem a forma larvar (metacestóide), principalmente na superfície e no interior do fígado. Esta tese tem como objetivo geral estudar as características parasitológicas e histopatológicas de metacestóides de E. vogeli, originários de pacas e humanos da região norte do Brasil, visto o conhecimento insuficiente ou mesmo o seu desconhecimento. Os fígados e mesentérios foram obtidos de oito pacientes com hidatidose policística durante ato cirúrgico na Fundação Hospital Estadual do Acre. Pacas foram capturadas no Município de Bujari, Floresta Estadual do Antimary, Acre. Durante a necropsia das pacas, foram observadas lesões macroscópicas (massas esbranquiçadas ou amareladas, semelhantes a bolhas na superfície dos fígados). Para a análise parasitológica foram aplicadas as microscopias de luz, contraste interferencial de Normaski (DIC) e varredura laser confocal. A análise morfométrica foi realizada com o auxílio do Programa Image Pro Plus Media Cybernetics. Os órgãos de pacas e humanos foram submetidos à análise histopatológica. Os pequenos e grandes ganchos rostelares apresentaram polimorfismo morfológico, enquanto a organização dos protoescólices acompanhou o padrão descrito para Echinococcus sp. Todas as pacas apresentavam cistos hepáticos, porém em apenas duas encontramos líquido hidático, comprovados pela presença dos ganchos e protoescólices. A análise histopatológica dos tecidos hepáticos das pacas confirmou a hidatidose policística e evidenciou, pela presença de agrupamentos de ovos, a coinfecção com Calodium hepaticum. As características morfológicas dos ganchos rostelares dos casos humanos não diferiram...

Polycystic hydatidosis is a zoonotic disease caused by the cestode Echinococcus vogeli, largely distributed in northern Brazil. The definitive hosts are Speothos venaticus (bush dogs) and Canis familiaris (domestic dogs), while Agouti paca (paca) is the intermediate host. Both the pacas and man (accidental host) develop the larval form (metacestodes), mainly on the surface and inside the liver. This thesis aims to study histopathological and parasitological characteristics of metacestodes of E. vogeli, from pacas and humans of the north region of Brazil, seen the insufficient knowledge or lack of it. The mesentery and livers were recovered from eight patients with polycystic hydatidosis during surgery in the Acre State Hospital Foundation. Pacas were captured in the municipality of Bujari, Antimary State Forest, Acre. During the pacas necropsy, macroscopic lesions were observed (whitish or yellowish masses, similar to blisters on the surface of the liver). Identification analyses were applied to light microscopy, interferential contrast microscopy of Normaski (DIC) and confocal laser scanning microscopy. Morphometric analysis was performed by Image Pro Plus Media Cybernetics program. The pacas and human organs were subjected to histopathological analysis. The small and large rostellar hooks presented morphological polymorphism while protoscoleces organisation accompanied the pattern described in Echinococcus sp. All pacas presented liver cysts, however in only two found proven by the presence of hydatid fluid, hooks and protoscoleces. Histopathological analysis of pacas’ hepatic tissues confirmed polycystic hydatidosis and evidenced by the presence of eggs clusters the Calodium hepaticum coinfection. The morphological characteristics of human cases rostellar hooks did not differ from described to the pacas; however, the hooks were greater than the mesenteric ones. Already in relation to protoscoleces, the mesenteric ones were larger than the liver...

Hidatidosis poliquística autóctona en dos pacientes Yanomami en el Alto Orinoco, Amazonas, Venezuela / Polycystic hydatid disease in two patients indigenous Yanomami in the Upper Orinoco, Amazonas, Venezuela

La infección por Echinococcus sp. es hipoendémica en Venezuela. Sólo cuatro casos de hidatidosis autóctona por E. vogeli han sido reportados, tres de ellos en la región de la Guayana venezolana. En Febrero del año 2009 se realizó el diagnóstico clínico-sero-epidemiológico de hidatidosis poliquística en una paciente femenina de la etnia Yanomami, procedente de Parima B, Alto Orinoco, en la Amazonía venezolana. Se resolvió con tratamiento médico y quirúrgico por laparoscopia y se evidenció en el quiste la presencia de ganchos rostelares compatibles con E. vogeli. En Abril del 2009 en una segunda paciente Yanomami de igual procedencia, se le diagnosticó hidatidosis por E. vogeli siendo operada exitosamente por cirugía laparoscópica asistida por robot. Dos casos humanos en una misma población y la presencia de factores de riesgo como la tenencia de perros domésticos y la comunicación por informantes indígenas del hallazgo de quistes en hígados de animales de cacería (Cuniculus paca o lapa y Dasyprocta sp. o picure), hacen pensar en transmisión activa en la cuenca del Alto Orinoco y en zonas selváticas de la Guayana venezolana. El presente, es el primer registro de casos de hidatidosis poliquística en indígenas de la etnia Yanomami.

Infection by Echinococcus sp. is hypoendemic in Venezuela. Only four cases of autochthonous E. vogeli hydatidosis have been reported, including three in the Venezuelan region of Guayana. In February 2009, based on epidemiological data, signs and symptoms and serological tests, a female patient of the Yanomami ethnic group, was diagnosed with a polycystic hydatid disease in Parima B, Alto Orinoco, in the Venezuelan Amazon. Rostellar hooks compatible with E. vogeli were found in the cyst. It was resolved with medical and surgical treatment by laparoscopy. A second Yanomami patient from the same location was diagnosed with E. vogeli hydatidosis in April 2009, being successfully operated with robot-assisted laparoscopy. Two human cases in the same population and the presence of risk factors such as domestic dog ownership and findings of cysts in livers of hunted animals (such as Cuniculus and Dasyprocta sp.) reported by indigenous informants, suggest active transmission in the Upper Orinoco basin and forested areas of the Venezuelan Guayana. These are the first reported cases of polycystic hydatid disease of the Yanomami ethnic group.

Hidatidosis poliquística tropical por Echinococcus vogeli / Polycystic tropical echinococcosis due to Echinococcus vogeli

Antecedentes. Hace 30 años se reconoció que las tumoraciones poliquísticas en 13 pacientes autóctonos de países tropicales sudamericanos eran producidos por una nueva especie de parásitos, el Echinococcus vogeli. Ahora se conocen 200 casos en 12 países (desde Panamá hasta Brasil y Perú). Lugar. Cali, Colombia; Nueva Orleans y Seattle, USA. Diseño. Estudio experimental y retrospectivo. Objetivo. Se presenta esta enfermedad a países con zonas tropicales donde la enfermedad aún es desconocida. Métodos. Se estudiaron 78 pacientes. El curso fue crónico, de quistes abdominales, algunos dolorosos, hepáticos, con o sin cirrosis biliar, asociados a lesiones torácicas y mesentéricas. El 85% se localizó en el hígado y el 14% en el pulmón. El diagnóstico se basó en el examen físico, la radiología, la procedencia rural, el conocimiento de la paca, en la convivencia con perros y en la serológica. Conclusiones. El tratamiento se basa en la cirugía y el albendazol. La equinococosis poliquística tropical por el Echinococcus vogeli representa un problema médico severo en América del Sur, con mortalidad del 29%. Los casos diagnosticados representan una pequeña proporción de las infecciones humanas.

Background. Thirty year ago it was recognized experimentally that polycystic tumors in 13 autochthonous patients from south American countries were produced by a new species: Echinococcus vogeli. As of march 2007, 200 cases were known from 12 countries (from Panamá, Colombia and Venezuela to Brasil and Perú). Places. Cali, Colombia; New Orleans and Seattle, USA. Design. Experimental and retrospective studies. Objective. This disease is presented to the countries with tropical areas, where the illness is steel unknown. Methods. 78 patients were studied. They had cysts, sometime painful, connected with the liver, with or without biliar cirrhosis, associated with pulmonary and mesenteric lesions. 85% in the liver, 14% in the lung. The diagnosis was based in palpation, radiological imaging, rural origin, knowledge of the paca, history of closed contact with dogs and positive serology for echinococcosis. Conclusions. The combination of surgery and albendazol was the most efficient treatment. The tropical polycystic echinococcosis is a severe medical problem in South America with a 29% mortality. The number of diagnosed cases probably only represent a small proportion of the human infections.

Equinococosis hepática poliquística autóctona por Echinococcus vogeli en el amazonas venezolano: Descripción de un caso / Autochthonous polycystic hepatic Echinococcosis due to Echinococcus vogeli at the Venezuelan amazon: Case report

Resumen Echinococcus vogeli y Echinococcus oligarthrus, especies autóctonas de América, producen equinococosis poliquística. El objetivo del trabajo es presentar un caso de equinococosis hepática poliquística por Echinococcus vogeli en una indígena de la etnia Yekuana del área amazónica venezolana. Presentó dolor abdominal punzante, hepatomegalia y eosinofilia marcada. Los estudios por imágenes mostraron lesiones poliquísticas en hígado. La serología para equinococosis resultó positiva. Se inicia tratamiento con albendazol previo a la cirugía, sin mejoría clínica. Se extrajo una gran masa quística del hígado por cirugía. Se determinó el promedio de longitud de los ganchos cefálicos de los protoescólices y las proporciones correspondían a E. vogeli. La paciente evolucionó satisfactoriamente y continuó el tratamiento con albendazol por 6 meses. La zona amazónica de Venezuela es abundante en animales silvestres, como roedores infectados con E. vogeli, Cuniculus paca o lapa y Dasyprocta aguti o picure venezolano. La paca es la principal presa tanto para Speothus venaticus como para los humanos que cazan las lapas por el excelente sabor de su carne. Los cazadores alimentan a los perros domésticos con las vísceras de la paca infectada, traspasando la infección a los humanos a través de los huevos excretados por las heces. Se sugiere pensar en equinococosis poliquística en individuos nativos con dolor abdominal, imágenes poliquísticas, calcificaciones en el hígado y eosinofilia, procedentes de la zona selvática del amazonas venezolano.

Abstract Echinococcus vogeli and Echinococcus oligarthrus, new world autochthonous species are responsible for polycystic echinoco-ccosis. The purpose of this work is the presentation of a polycystic hepatic echinococcosis case due to Echinococcus vogeli in an indigenous inhabitant of the Venezuelan amazon area. The patient presented a penetrating abdominal pain, hepatomegalia and marked eosinophilia. Liver imaging (echosonogram and computerized axial tomography) as well an echinococcosis positive serology (ELISA and Western-blot) contributed to the pre-surgical diagnosis. Treatment with albendazol was initiated previous to surgery. The cephalic hooks of the protoscolexes were studied and it was determined that their mean length, morphology and proportions corresponded to E. vogeli. The patient evolved satisfactorily and continued albendazol treatment during six months. In the Venezuelan amazon area there are still numerous wilds animals which are intermediate and final hosts of this cestode. Cuniculus paca or lapa, Dasyprocta aguti or Venezuelan aguti, Speothus veneticus, wild dogs and wild cats, are natural host of both species. We suggest considering the possibility of polycystic echinococcosis in native individuals with polycystic imagining and liver calcifications (and eosinophilia) who come from Venezuelan tropical forests. Nevertheless, it should be remembered that domestic dogs and cats are responsible of human infections.

Equinococose policística na Amazônia oriental brasileira: atualização da casuística / Polycystic echinococcosis in the eastern brazilian Amazon: an update

Mediante critérios epidemiológicos, clínicos e laboratoriais, foi levantada a casuística de equinococose policística no período de 1962 a 2003, no âmbito da Amazônia oriental brasileira, incluindo casos inéditos e aqueles já publicados. Dessa forma, foram identificados 40 casos da doença no referido período, compreendendo casos procedentes dos estados do Pará e Amapá, Brasil. A amplitude das idades foi de 10 a 72 anos. Do total 47,5% pertenciam ao sexo masculino. O fígado foi o órgão mais acometido (82,5% dos casos). O Echinococcus vogeli (Rausch e Bernstein, 1972), apresentou-se como o principal agente etiológico envolvido. A partir do reconhecimento da importância e das implicações do manejo da equinococose para a região tropical, acredita-se que deverá ocorrer uma implementação do diagnóstico precoce, tratamento adequado e de um melhor registro da doença.

By means of epidemiological and clinical-laboratorial approaches was consolidated an update of polycystic echinococcosis in the Eastern Brazilian Amazon, period from 1962 to 2003, including unpublished cases and those already published. In that way, they were identified 40 cases of the disease in referred period, understanding cases coming from the States of Pará and Amapá, Brazil. The width of the ages went from 10 to 72 years and 47,5% belonged to the masculine sex. The liver was the attacked organ (82,5% of the cases). The Echinococcus vogeli (Rausch and Bernstein, 1972), comes as the main agent involved. Starting from the recognition of the importance and of the implications of the handling of the echinococcosis for the tropical area, it is believed that should happen an improvement of the diagnosis, appropriate treatment and of a better registration of the disease.

Doença hidática policística: análise secundária de dados / Polycystic hYdatid disease: secondary data analysis

OBJETIVO: Rever, usando a metodologia de análise secundária de dados, os casos descritos de doença hidática policística (DHP) pelo Echinococcus vogeli, quanto às características clínico-epidemiológicas, de evolução e procedimentos terapêuticos. MÉTODO: Foram usados cinco bancos eletrônicos; anais de eventos científicos da área de Medicina Tropical; livros textos; consultas aos índices remissivos de revistas não-indexadas e a especialistas. As 52 variáveis estudadas foram categorizadas para cada caso de DHP e registradas em ficha-padrão. Somente foram incluídos os casos com comprovação histológica e/ou parasitológica do E. vogeli. RESULTADOS: Foram recuperados 131 trabalhos publicados e uma comunicação pessoal, sendo grande parte com somente um caso descrito, e entre estes apenas 17 (12,9 por cento) tinham casos com comprovação do agente etiológico, com um total de 44 pacientes: 52,3 por cento do sexo masculino; média de idade de 45,0 (± 16,7) anos; e 50 por cento descritos no Brasil. A presença de massas e a dor abdominal foram registradas em 94,7 por cento (18/19) e 92,6 por cento (25/27), respectivamente. Não houve diferença estatística (p>0,20) entre os resultados do tratamento clínico (albendazol) e cirúrgico, mas as freqüências de "sem êxito" foram, respectivamente, de 0 por cento e 28,6 por cento, e as de óbitos de 0 por cento e 21,4 por cento. CONCLUSÕES: A maioria dos trabalhos sobre a DHP não tem pacientes com comprovação etiológica e, conseqüentemente, é possível que parte do conhecimento clínico atual sofra mudanças significativas por investigações futuras. De outra parte, os dados levantados indicam que a melhor opção terapêutica, nos casos irressecáveis, é o uso de albendazol.

BACKGROUND: We reviewed, using secondary analysis, the described cases of Polycystic Hydatid Disease (PHD) by Echinococcus vogeli, addressing epidemiological and clinical characteristics, therapeutical evolution and procedures. METHODS: Five electronic banks, annals of scientific events, textbooks, remissive index, non-indexed magazines and specialists were consulted. The 52 variables studied had been categorized for each case of PHD and registered in a standard file. Only the cases with histological and/or parasitological evidence of E. vogeli had been enclosed. RESULTS: One hundred and thirty-one published works had been recouped and a personal communication. The majority presents, only a described case, and between them only 17 (12.9 percent) had evidence of the etiologic agent. In a total of 44 patients, 52.3 percent were males, mean age of 45.0 (±16,7) years, and 50 percent were described in Brazil. The presence of masses and abdominal pain had been registered in 94.7 percent (18/19) and 92.6 percent (25/27), respectively, with no statistica difference (p>0.20) in the clinical outcome with the clinical treatment (albendazol) versus the surgical one, but the frequencies of no success had been, respectively, 0 percent and 28.6 percent, and deaths of 0 percent and 21.4 percent. CONCLUSIONS: The majority of studies on PHD does not include patients with etiologic evidence and consequently is possible that part of the current clinical knowledge suffers significant changes for future inquiries. Nevertheless, the raised data indicate that the best therapeutical option, in the non surgical approach, the use of albendazol.