Portopulmonary hypertension: Success with combined medical treatment.

Portopulmonary hypertension is an uncommon disease associated with high morbidity and mortality, so its early diagnosis and treatment are essential. We report here the case of a 57-year-old man with portopulmonary hypertension caused by chronic hepatosplenic schistosomiasis and also liver cirrhosis due to hepatitis C and alcoholism. As well as treating both diseases, portopulmonary hypertension was successfully managed with tadalafil and macitentan as maintenance therapy. This case reminds clinicians that pulmonary hypertension can be multifactorial, a good diagnosis and a multidisciplinary treatment can lead to improved prognosis.

Human anthelminthic vaccines: Rationale and challenges.

Helminth infections are the most common afflictions of humankind, affecting almost every single person living in profound poverty. Through mass drug administration (MDA) we have seen sharp declines in the global prevalence of some helminth infections, including lymphatic filariasis, onchocerciasis, and ascariasis. However, since 1990, there has been no appreciable decrease in the global prevalence of hookworm infection, schistosomiasis, or food-borne trematodiases. Through the activities of a non-profit product development partnerships and two research institutes, a total of five human anthelmintic vaccines for hookworm infection (two) and schistosomiasis (three) have advanced from discovery through manufacture and are now in Phase 1 clinical testing. At least three additional antigens, including two for onchocerciasis and one for schistosomiasis, are also advancing through preclinical development with the intention of moving into the clinic soon. These preventive human anthelmintic vaccines could be used as stand-alone technologies administered to infants as part of the Expanded Program on Immunization (EPI), or together with anthelmintic drugs in programs linked to MDA. Significant hurdles though could hinder the advancement of these vaccines into later-stage clinical and product development and licensure. They include the absence of a major pharma partner (and the resultant access to adjuvants and industrial scale manufacturing expertise), an uncharted roadmap for how to introduce anthelmintic vaccines into appropriate health systems, uncertain global access and regulatory strategies that might need to rely on developing country vaccine manufacturers and national regulatory authorities, and the lack of innovative financing schemes. However, the public health and economic benefits of introducing these vaccines could be massive and therefore deserve international attention and support.

Esquistosomiasis intestinal: reporte de un caso / Intestinal Schistosomiasis: a case report

Introducción: la esquistosomiasis intestinal es una parasitosis ocasionada por helmintos de la clase trematode del género Shistosoma, que se caracteriza por presentar en su fase aguda diarreas, dermatitis y dolores abdominales, con alta incidencia en los países pobres con problemas de insalubridad. El factor desencadenante de esta parasitosis está asociado al contacto con agua contaminada por los huevos de shistosomas. Puede prevenirse con medidas de saneamiento ambiental dirigidas al control de las fuentes de abasto de agua. Existe en la actualidad elevada morbilidad principalmente en el África subsahariana. Se persigue como objetivo explicar la epidemiología de esta enfermedad. Presentación del caso: se presenta el caso de un adulto mayor de 60 años de edad, del sexo femenino, sin antecedentes de esta enfermedad, que se encontraba prestando ayuda colaboración en la provincia de Cunene en la hermana República de Angola. Presentó síntomas clínicos durante los meses de enero a marzo del 2012, siendo confirmado por el laboratorio como esquistosomiasis intestinal. Se aplicó a la paciente el medicamento específico y actualizado: praziquantel, con evolución satisfactoria. Conclusiones: la literatura describe fallos del tratamiento en los que se ha requerido más de un ciclo de praziquantel, lo cual no es muy frecuente, pero ocurrió en el caso que se presenta. Se le orientó a la paciente tomar el medicamento acompañado de alimentos. No refirió presentar efectos secundarios.

Introduction: intestinal schistosomiasis is a parasitic disease caused by helminths of the class trematode and genus Schistosoma, characterized by presenting in its acute stage diarrhea, dermatitis and abdominal pain, with high incidence in poor countries with insalubrity problems. The triggering factor of this parasitosis is associated to exposure to water contaminated by schistosomes eggs. It can be prevented with sanitation measures aimed at controlling sources of water supply. There is now high morbidity mainly in sub-Saharan Africa. The objective aimed at is explaining the epidemiology of this disease. Case report: the case of an adult female aged over 60 years is presented, with no history of the disease, which was on a medical collaboration in the province of Cunene in the sister Republic of Angola. She showed clinical symptoms during the months of January to March 2012, and it was confirmed by the laboratory as intestinal schistosomiasis. The specific and updated medication (praziquantel) was administered to the patient, with satisfactory results. Conclusions: the literature describes treatment failures in which it has been required more than one cycle of praziquantel, which is not very common, but occurred in the case presented. The patient was assigned to take the medication together with food. No side effects were referred as presented.

Carcinoma epidermoide de vejiga: una forma inusual de presentación / Bladder squamous cell carcinoma: an unusual form of presentation

Se presenta el caso de un joven procedente de la provincia de Huambo en Angola, el cual es remitido al Hospital Militar de Luanda por presentar toma del estado general, palidez cutánea mucosa, pérdida de peso, astenia, anorexia y una tumoración exófistica en hipogastrio de aproximadamente unos 10 centímetros de diámetro, de color blanco amarillenta e irregular, con zonas difusas de ulceraciones y salida de líquido claro y en ocasiones rojizo por la misma; a unos cinco centímetros a la izquierda de la línea media abdominal. Se toma muestra de la tumoración para estudio histopatológico y se obtiene como resultado un carcinoma epidermoide infiltrante de vejiga, Shistosomiasis vesical (AU)

This work is about a young man that comes from Huambo (Angola) who is evacuated to the Military hospital of Luanda for presenting mucu-cutaneous paleness, loss of weight, asthenia, anorexia and an exophytic tumor in hypogastrium approximately 10 centimeters in diameter, of whiteyellowish color and irregular with diffuse areas of ulcerations and opening clear liquid and sometimes reddish; to approximately five centimeters to the left of the abdominal halfway line; tumoration sample was taken for histopathologic studies giving as result an invasive bladder Squamous Cell Carcinoma . Vesical Shistosomiasis (AU)

Surveillance of schistosomiasis in national surveillance sites of Jingzhou City / 中国血吸虫病防治杂志

According to the National Scheme of Shistosomiasis Surveillance,the endemic situation in 8 national surveillance sites in Jingzhou City were monitored and analyzed.The results showed that the average human infection rate in 8 sites was 0.93%,and the rates in residents above 40 years old were high.People with positive results in fecal examination were all farmers,with an infection rate of 1.16%.The average infection rate of cattle was 6.26%.The total snail area was 1 511.50 hm~2,among which an area of 0.98 hm~2 was newly discovered and the area with infected snails was 1.17 hm~2.It concludes that the endemie situation in surveillance sites decreases year by year.However,the infection rate of earle still remains high and the snail status is still severe,which suggests that the control should be strengthened further.

Long-term efficacy of splenectomcy for patients with advanced shistosomiasis japonica / 中华肝胆外科杂志

Objective To study the long-term efficacy of splenectomy for patients with advanced shistosomiasis japonica.Methods Levels of WBC,RBC,PLT,EOS,ALT,ALP,GGT,A,TB,HA,LN,Ⅳ-C,PCⅢ,IGG,IGA,C3,C4,CD3,CD4,CD8,CD19 in periphetral venous blood were determined in 239 patients with advanced shistosomiasis.Meanwhile,the liver,gallbladder and spleen were examined with ultrasonography.Results The levels of WBC,PLT,EOS,ALT,ALP,IGG,IGA,LN,Ⅳ-C,CD19 increased in splenectomy group,the levels of A,TB,CD3,CD4,C3,C4 decreased in splenectomy group,while RBC,HA,PCⅢ,CD8 were not changed.Conclusion Splenectomy is a danger to hepatic function.Humoral immunity increases while cellular immunity decreases in splenectomy group.Splenectomy may aggravate the hepatic fibrosis in patients with advanced shistosomiasis.

Granuloma esquistossomótico gigante do cólon com intussuscepção: relato de caso / Giant schistosomal granuloma of the colon with intussusception: case report

A esquitossomose é uma doença parasitária crônica causada por uma espécie de trematódeo, o shistossoma, acometendo 200 milhões de pessosas em todo mundo. No Brasil é provocada pela espécie Shistossoma mansoni, cuja apresentação clínica mais comum é a hepato-intestinal, com sintomas como diarréia, tenesmo, náuseas, meteorismo e hepato-esplenomegalia. Relatamos um caso de paciente masculino de 38 anos com quadro de exteriorização de tumor pelo ânus. A colonoscopia demonstou lesão nodular extra-mucosa em topografia de sigmóide. Submetido a sigmoidectomia, cujo anátomo-patológico mostrou tratar-se de granuloma esquistossomótico gigante, apresentação muito rara da esquistossomose.

Shistosomiasis is a chronic parasitic illness caused by Shistossoma Mansoni, responsible for 200 million cases all over the world. The most common presentation is hepatic-intestinal form with symptoms as diarrhea, tenesmus, hepatomegaly, abdominal pain and nausea. We report a case of a male patient, 38 years with anal polyp prolapse, confirmed by colonoscopy, located in the sigmoid. The patient was submitted to sigmoidectomy, which through a histological study of the surgical specimen a giant shistosomal granuloma of the colon was evidenced. The aim of this report is to describe a rare and atypical manifestation of a case of shistosomosis.

Varizes de fundo gástrico na hipertensão portal esquistossomótica: resultados cirúrgicos / Gastric fundus varices in schistossomotic portal hypertension: surgical results

OBJETIVO: Apresentar dados epidemiológicos de pacientes esquistossomóticos na forma hepatoesplênica com varizes do fundo gástrico, assim como avaliar os resultados de uma estratégia cirúrgica no manuseio destas varizes. MÉTODO: No período de janeiro de 1992 à julho de 2001 foram acompanhados no Serviço de Cirurgia Geral do Hospital das Clínicas da Universidade Federal de Pernambuco 125 pacientes submetidos à esplenectomia com ligadura da veia gástrica esquerda (LVGE), desvascularização da grande curvatura do estômago e esclerose endoscópica pós-operatória, para o tratamento da hipertensão portal esquistossomótica com antecedentes de hemorragia digestiva. Quando da presença de varizes de fundo gástrico (44/125) foi associado ao procedimento cirúrgico, a abertura do estômago e sutura das varizes. RESULTADOS: Varizes de fundo gástrico foram identificadas em 35,2 por cento (44/125) dos pacientes com esquistossomose hepatoesplênica e antecedentes de hemorragia digestiva alta. Durante o seguimento de 26 meses o procedimento cirúrgico erradicou 76,5 por cento das varizes de fundo gástrico. A incidência de trombose da veia porta no período pós-operatório foi maior no grupo de pacientes sem varizes de fundo gástrico (16,3 por cento) quando comparado com os pacientes portadores de varizes de fundo gástrico (8,8 por cento), sem que, no entanto, esta diferença tivesse respaldo estatístico (p = 0,62). Não se identificou correlação entre a presença de varizes do fundo gástrico e o grau de fibrose periportal e o peso do baço. Na análise bioquímica e hematológica, no período pré-operatório dos grupos estudados, o número de leucócitos foi estatisticamente menor no grupo de pacientes que apresentavam varizes de fundo gástrico. CONCLUSÃO: A esplenectomia associada a desvascularização da grande curvatura do estômago, ligadura da veia gástrica esquerda, gastrotomia e sutura da varizes de fundo gástrico, erradicou 76,5 por cento das varizes de fundo gástrico...

BACKGROUND: The aim of this study is to present epidemiological data and evaluate a surgical approach in the treatment of gastric fundus varices in patients with hepatosplenic shistosomiasis. METHODS: During the period of January 1992 and July 2001, 125 patients underwent splenectomy, ligation of the left gastric vein (LLGV), devascularization of the great stomach curvature and post-operative endoscopic sclerotherapy for the treatment of hepatic-splenic schistosomiasis with previous gastrointestinal haemorrhages. In the patients who presented gastric varices in the pre-operative endoscopy (44/125), a gastrotomy and an obliterating running suture were also performed intraoperatively. RESULTS: Gastric fundus varices were observed in 35,2 percent of all patients with hepatic-splenic schistosomiasis with previous gastrointestinal haemorrhages (44/125). The surgical treatment proposed eradicated 76,5 percent of the gastric fundus varices in a mean follow-up period of 26 months. Portal vein thrombosis was higher in the group of patients without fundus grastric varices (16,3 percent) when compared with fundus gastric varices patients (8,8 percent). This difference was not statistically significant (p=0,62). There was no correlation between the presence of fundus gastric varices and the degree of periportal fibrosis or the weight of the spleen. Despite a statistically lower number of white blood cells in the gastric fundus varices, no other differences were identified in the preoperative haematological and biochemical data. CONCLUSIONS: The authors concluded that patients underwent splenectomy, ligation of the left gastric vein, devascularisation of the great stomach curvature, post-operative endoscopic sclerotherapy, gastrotomy and an obliterating running suture of the fundus gastric varices, eradicated 76,5 percent of the fundus gastric varices, in a follow-up of 26 months.

Peso do baço como fator prognóstico do tratamento cirúrgico de pacientes portadores de esquistossomose mansônica / Spleen weight as risk factor in the surgical treatment of patients with schistosoma mansoni

OBJETIVO: Analisar pré-operatoriamente o peso do baço como fator prognóstico do tratamento cirúrgico de pacientes portadores de esquistossomose mansônica. MÉTODOS: Foram analisados 114 pacientes, portadores de esquistossomose mansônica com antecedentes de hemorragia digestiva, submetidos a tratamento cirúrgico. Os pacientes foram divididos em três grupos: Grupo 1 - baço menor que 500 gramas (17); Grupo 2 - baço entre 500 e 1.000 gramas (58); Grupo 3 - baço acima de 1.000 gramas (39). RESULTADOS: No Grupo 1 a recidiva hemorrágica foi de 17,6 por cento, trombose da veia porta de 5,9 por cento e não houve mortalidade. A incidência de hiperesplenismo pré-operatório foi de 29,4 por cento e o calibre da veia porta foi de 1,1cm. No Grupo 2 a recidiva hemorrágica foi de 15,5 por cento, trombose da veia porta de 10,3 por cento e a mortalidade de quatro pacientes (6,9 por cento) (dois pacientes no período pós-operatório e dois no seguimento tardio, hepatocarcinoma e hemorragia digestiva). A incidência de hiperesplenismo foi de 53,4 por cento e o calibre médio da veia porta foi de 1,4cm. No Grupo 3 a recidiva hemorrágica foi de 12,8 por cento, trombose da veia porta de 5,1 por cento e uma mortalidade tardia de dois pacientes (linfoma e infarto agudo do miocárdio). A incidência de hiperesplenismo foi de 76,9 por cento e o calibre da veia porta foi de 1,5cm. CONCLUSÕES: O peso do baço apresenta relação com o hiperesplenismo pré-operatório, calibre da veia porta e permanência hospitalar pós-operatória. Não encontramos relação com a incidência de varizes de fundo gástrico, recidiva de sangramento digestivo, trombose da veia porta, grau de fibrose periportal e dados bioquímicos.

BACKGROUND: Searching for that risk factors that could commit surgical treatment of patient with schistosomiasis, the authors analyzed the spleen as a prognostic factor. METHODS: Between 1992 and 1998, 114 patients underwent splenectomy with ligation of the left gastric vein (LLGV) and devascularisation of the great stomach curvature, followed by post-operative endoscopic sclerotherapy for the treatment of hepatic-splenic schistosomiasis with previous gastrointestinal haemorrhages. A clinical/laboratorial analysis was performed. Patients were divided into 3 groups according to the weight of the spleen, in the moment of the accomplishment of the histologycal cuts: GROUP I - spleen smaller than 500 grams (17); GROUP II - spleen between 500 and 1000 grams (58) and; GROUP III - spleen greater than 1000 grams (39). RESULTS: Patients of GROUP I presented rebleeding rate of 17,6 percent, thrombosis of the portal vein of 5,9 percent and there was not mortality. The incidence of preoperative hipersplenism was 29,4 percent and the caliber of the portal vein was 1,1 cm. In GROUP II the rebleeding rate was of 15,5 percent, thrombosis of the portal vein of 10,3 percent and the mortality incidence of hiperplenism of 4 patient (6,9 percent), 2 patients in the immediate postoperative period and 2 in the late followup (hepatocarcinoma and digestive hemorrhage). The hipersplenism incidence was of 53,4 percent and preoperative mean caliber of the portal vein was 1,4cm. In GROUP III the rebleeding incidence was 12,8 percent, thrombosis of the portal vein 5,1 percent and a late mortality of 2 patient (linfoma and miocardium infarct). The hipersplenism incidence was of 76,9 percent and the caliber of the portal vein was of 1,5 cm. CONCLUSIONS: Weight of the spleen presents relationship with preoperative hipersplenism, with portal vein caliber and postoperative hospitalar stay. There was no relationship with fundus gastric varices, rebleeding rate, portal vein...

Tratamento cirúrgico da hipertensão portal esquistossomótica no hc/ufpe: análise de 131 casos / Surgical treatment of the schistossomotic portal hypertension in the hc/ufpe: analysis of 131 cases

Objetivo: A hipertensão portal esquistossomótica com antecedente de hemorragia digestiva foi tratada com esplenectomia + ligadura da veia gástrica esquerda (LVGE) + desvascularização da grande curvaturado estômago + esclerose endoscópica pós-operatória. Quando da existência de varizes de fundo gástrico, realizamos a abertura do fundo gástrico e sutura obliterante destas varizes. O objetivo deste trabalho foi avaliar a recidiva de hemorragia digestiva, repercussões laboratoriais e mortalidade do tratamento cirúrgico/endoscópico.Métodos: Entre 1992 e 1998, foram operados no HC-UFPE 131 pacientes. O seguimento médio foi de 30 meses, em 111 pacientes, que foram solicitados a retornar ao ambulatório do HC-UFPE para a realização de controle clínico e laboratorial. Resultados: A recidiva hemorrágica foi de 14,4% (16/111) e uma mortalidade de 5,4% (6/111). A recidiva de hemorragia digestiva alta foi exteriorizada através de hematemese em oito pacientes e oito por melena. Dos seis pacientes que foram a óbito, três apresentavam diagnóstico de linfoma, hepatocarcinoma e infarto agudo do miocárdio, respectivamente. Dois pacientes foram a óbito no pós-operatório imediato (sepse e coagulação intravascular disseminada). O sexto paciente foi a óbito por recidiva da hemorragia digestiva alta. Em nove pacientes, 13,2%, foi diagnosticada trombose da veia porta. Os dados laboratoriais, hematológicos e de função hepática também foram analisados. Conclusões: Os autores concluíram que o tratamento cirúrgico da hipertensão portal esquistossomótica, através da esplenectomia + LVGE + desvascularização da grande curvatura do estômago + esclerose endoscópica pós-operatória determina resultados compatíveis com a literatura em relação à recidiva de sangramento, mas preserva a funcionalidade hepática.

Background: At the Clinical Hospital of the Federal University of Pernambuco the surgical treatment of hepatosplenic shistosomiasis has been done with splenectomy + left gastric vein ligature (LGVL) + devascularization of the great curvature of the stomach + postoperative endoscopic sclerosis. If the patient has gastric fundus variceals, the gastric fundus was open and the variceals sutured. The objective of this paper was to evaluate the surgical treatment proposal regarding the re-bleeding rate, mortality and laboratorialschanges. Method: During the period between 1992 and April 1998, 131 procedures in the General Surgery Division of the Clinical Hospital. The patients were asked to return to the Hospital and underwent a clinical/laboratorial analysis. The mean follow-up was 30 months. Results: The re-bleeding rate was 14,4% (16/111) and the mortality rate 5,4% (6/111). In 8 cases of re-bleeding the exteriorization was in form of melena and in 8 as hematemesis. In 3 cases the mortality was resulted of a linfoma, a hepatocarcinoma and a cardiac stroke. In two patients the death was resulted from the immediate postoperative period(sepsis and intravascular disseminated coagulation). The other death was during the late postoperative period as a result of a re-bleeding episode. Nine patients (13,2%) evaluated with portal vein thrombosis and in two a superior mesenteric vein thrombosis was identified. Hematological and biochemical data’s was also analyzed. Conclusions: The authors concluded that the surgical treatment of the hepatosplenic shistosomiasis with splenectomy + LGVL + evascularization of the great curvature of the stomach +postoperative endoscopic sclerosis is a safe procedure and with results comparable with the literature, and a advantage to maintain the liver functionality...