Genetic analysis of human predisposition to hepatosplenic disease caused by schistosomes reveals the crucial role of connective tissue growth factor in rapid progression to severe hepatic fibrosis.

Schistosome worms inhabit mammalian mesenteric veins. Their eggs cause chronic inflammation, which progresses to periportal fibrosis in 5 to 30% of cases, increasing portal blood pressure and leading to esophageal varices. Episodes of bleeding cause hepatic necrosis and may ultimately lead to hepatic failure and the death of the patient. Schistosome infections can also cause pulmonary hypertension and heart failure. The mechanisms of fibrogenesis and fibrolysis are beginning to be unraveled, but it remains unclear why disease occurs only in certain subjects, as also observed for other types of chronic liver inflammation, as in hepatitis C or B. We summarize here the results that showed that fibrosis progression is determined by a genetic locus on chromosome 6. The CCN2 gene at this locus, encodes CTGF that is a crucial regulator of fibrosis. Two groups of CCN2 polymorphisms independently modulate the progression of hepatic fibrosis. These results were obtained in an Asian population, but were extended to humans living in Africa and South America and are presently tested in liver fibrosis of other etiological origins.

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.

Long-term follow-up after liver transplantation in Egyptians transplanted abroad.

OBJECTIVE: To study the long-term outcome after liver transplantation (LT) in Egyptian patients who underwent LT outside Egypt. METHODS: Between May 1993 and February 2004, over 150 Egyptians underwent LT outside Egypt. Data of 67 recipients were collected in Egypt through personal communications with the Overseas Liver Transplant Centers and through the records of the Egyptian Liver Transplant Association. RESULTS: Most patients underwent LT in Europe (73.1%), few in the United State of America (17.9%) and in Japan (9%). Sixty-one patients underwent cadaveric LT and the remaining 6 patients underwent living related liver transplantation (LDLT). The male to female ratio was 58:9. Median age was 45 (3-63 years). Hepatitis C virus (HCV) cirrhosis whether alone or mixed with schistosomiasis was the main indication for LT. Out of those 67 recipients, 52 (77.6%) survived after a median follow-up period of 4.6 years (rang 1-10.5 years). Deaths were due to primary non-function in 3 patients, postoperative bleeding in one patient, recurrent hepatitis C virus (HCV) in 10 patients, and chronic rejection in one patient. CONCLUSION: Egyptians underwent LT abroad showed a good long-term outcome. Due to the high prevalence of HCV, we expect a growing need for LT in Egypt. Although LDLT has been introduced recently in Egypt, cadaveric liver donation is still not legalized by the government. Efforts should be directed to expanding LDLT, legalizing cadaveric LT and also to the prevention and control of HCV infection in Egypt in order to avoid its devastating effect on the society as well as its enormous negative impact on Egypt's economy and future development.

[Pulmonary echinococcosis, hepatic opisthorchiasis and generalized trichinelliasis combined with pregnancy]. / Ekhinokokk legkikh, opistorkhoz pecheni, generalizovannyi trikhinellez v sochetanii s beremennost'iu.

A case is reported of a spontaneous abortion (25 weeks of gestation) in a Nenets woman resulting in thrombohemorrhagic syndrome and profuse uterine bleeding. The abortion is attributed to drastic allergization by three parasites: Echinococcus granulosus, Opisthorchis felineus, Trichinella spiralis.

Forma hepatosplênica da esquistossomose mansônica, em relaçäo à composiçäo racial e ao nível sócio-econômico, em Catolândia-Bahia / Hepatosplenic form of schistosomiasis mansoni, in relation to race and socioeconomic level, in Catolândia-Bahia

De 1125 indivíduos, residentes em área hiperendêmica de esquistosomose mansônica, estudou-se a apresentaçäo clínica, relacionada aos grupos raciais (mestiço de índio, branco, mulato-claro, mulato-médio, mulato-escuro e negro) e ao nível sócioeconômico, conforme o somatório de 16 variáveis. Em 229 indivíduos brancos havia 24 (10,5%) com a forma hepatosplênica, significantemente superior, em comparaçäo com 32 (3,6%) dos 896 näo-brancos; entre os grupos raciais negróides ocorreram freqüentemente semelhantes. Os hepatosplênicos apresentaram nível sócio-econômico mais alto e quando, também, brancos, o risco relativo (2,78) foi marcadamente superior

[Hepatosplenic form of schistosomiasis mansoni, in relation to race and socioeconomic level, in Catolândia-Bahia]. / Forma hepatosplênica da esquistossomose mansônica, em relação à composição racial e ao nível sócio-econômico, em Catolândia-Bahia.

Of 1,125 individuals, residents in an area where mansonic schistosomiasis is hyperendemic, were studied regarding the clinical forms correlated to the racial groups (indian mestizo, white, light mullato, medium mullato, dark mullato and negro) and to their socio-economic level according to a set of 16 variables. The white individuals (10.5%) showed a significantly higher incidence of the hepatosplenic forms; in the other racial groups there were similar frequencies, altogether 3.6%. Those with hepatosplenomegaly had a higher socio-economic level and when they also belonged to the white race the relative risk (2.78) was significantly higher.

Coeficiente de endocruzamento em portadores de esquistossomose mansônica / Imbreeding coefficient in carries of Schistosoma mansoni

O coeficiente de endocruzamento (f ou de Wright) foi calculado em 1123 indivíduos de Catolândia, Bahia, área hiperendêmica da esquistossomose mansônica: 148 (13,2%) tinham o coeficiente f > 0. A forma hepatosplênica foi significantemente maior nos indivíduos com f > 0 (26,8%). Nos brancos com f > 0 o risco relativo foi de 14,1; enquanto, nos brancos com f = 0, a freqüência da hepatosplenomegalia näo diferiu dos näo-brancos com f = ou f > 0. Com este coeficiente estimou-se a probabilidade de genes aléticos iguais, com origem em ancestral comum; os resultados reforçam a hipótese da regulaçäo genética na susceptibilidade à forma hepatosplênica da esquistossomose mansônica

[Inbreeding coefficient in carriers of Schistosoma mansoni]. / Coeficiente de endocruzamento em portadores de esquistossomose mansônica.

The coefficient of inbreeding (for Wright) was studied in Catolândia, in the state of Bahia, Brazil, an area considered hyperendemic for manson schistosomiasis in a population of 1,130 inhabitants. The coefficient of inbreeding was estimated for 1,123 individuals it was classified as f greater than 0 in 13.2% (n = 148). In the hepatosplenic group the frequency of f greater than 0 was 26.8%, and in the hepatointestinal group the frequency was 12.5%. The frequency of the hepatosplenic diagnosis in whites who were f = 0 did not differ from that which was observed in the negroid group. These verifications were confirmed by the Woolf's test; the relative risk of the whites, f greater than 0 in acquiring hepatosplenic schistosomiasis was 14.1. These observations reinforce the influence of the genetic component in the development of the hepatosplenic form of the mansons schistosomiasis.