Natural History and Outcome of Hepatic Vascular Malformations in a Large Cohort of Patients with Hereditary Hemorrhagic Teleangiectasia

BACKGROUND: Hereditary hemorrhagic telangiectasia is a genetic disease characterized by teleangiectasias involving virtually every organ. There are limited data in the literature regarding the natural history of liver vascular malformations in hemorrhagic telangiectasia and their associated morbidity and mortality. AIM: This prospective cohort study sought to assess the outcome of liver involvement in hereditary hemorrhagic telangiectasia patients. METHODS: We analyzed 16 years of surveillance data from a tertiary hereditary hemorrhagic telangiectasia referral center in Italy. We considered for inclusion in this study 502 consecutive Italian patients at risk of hereditary hemorrhagic telangiectasia who presented at the hereditary hemorrhagic telangiectasia referral center and underwent a multidisciplinary screening protocol for the diagnosis of hereditary hemorrhagic telangiectasia. Of the 502 individuals assessed in the center, 154 had hepatic vascular malformations and were the subject of the study; 198 patients with hereditary hemorrhagic telangiectasia and without hepatic vascular malformations were the controls. Additionally, we report the response to treatment of patients with complicated hepatic vascular malformations. RESULTS: The 154 patients were included and followed for a median period of 44 months (range 12-181); of these, eight (5.2%) died from VM-related complications and 39 (25.3%) experienced complications. The average incidence rates of death and complications were 1.1 and 3.6 per 100 person-years, respectively. The median overall survival and event-free survival after diagnosis were 175 and 90 months, respectively. The rate of complete response to therapy was 63%. CONCLUSIONS: This study shows that substantial morbidity and mortality are associated with liver vascular malformations in hereditary hemorrhagic telangiectasia patients

Anticorpos anti-Toxoplasma gondii e anti-Neospora caninum em cães da zona rural do município de Botucatu, São Paulo, Brasil

Toxoplasma gondii and Neospora caninum are protozoa and important agents of systemic disease in several animal species, including dogs. The aim of this study was to determine the frequency of IgG antibodies anti-N. caninum and T. gondii in serum samples of dogs from rural area of Botucatu-SP. The serological titers were correlated with age, sex and breed. A total of 689 healthy dogs, 298 (43.3%) females and 391 males (56.7%) were randomly selected for the study. Animals were divided into three age groups: younger than 12 months (11.32%), aged between one and five years (73.58%) and more than five years old (15.09%). A total of 180 (26.1%) were breed and 509 (73.9%) mongrel. Of total samples, 144 (21%) samples were positive for the presence of anti-T. gondii and 78 (11.3%) for anti-N. caninum antibodies. Correlation was found between antibody titles and age, for the two infections, which did not occur with respect to gender and breed.Toxoplasma gondii y Neospora caninum son protozoários importantes que causan enfermedad sistémica en muchas especies animales, incluyendo perros. El objetivo de este estudio fue determinar la frecuencia de anticuerpos IgG contra N. caninum y contra T. gondii en muestras de suero de los perros de las zonas rurales de Botucatu-SP, la correlación serológica con la edad, sexo y raza. Un total de 689 perros sanos, 298 hembras (43,3%) y 391 machos(56,7%) fueron seleccionados al azar para participar del estudio. Se agruparon en tres grupos de edad: menores de 12 meses (11,32%), con edades comprendidas entre uno y cinco años (73,58%) y más de cinco años (15,09%). 180 (26,1%) eran de raza definida y 509 (73,9%) mestizo. Del total de muestras, 144 (21%) fueron positivas para la presencia de anticuerpos contra T. gondii y 78 (11,3%) para anticurpos contra N. caninum. Se encontró correlación entre los títulos de anticuerpos y la edad, para ambas infecciones, lo que no ocurrió con respecto al género y la raza.Toxoplasma gondii e Neospora caninum são protozoários importantes causadores de doença sistêmica em muitas espécies animais, inclusive os cães. O objetivo do presente estudo foi determinar a frequência de anticorpos da classe IgG anti-N. caninum e anti-T. gondii em amostras de soros de cães da zona rural do município de Botucatu-SP, correlacionando os títulos sorológicos com a idade, sexo e raça dos animais. Um total de 689 cães hígidos, 298 (43,3%) fêmeas e 391 (56,7%) machos foram selecionados, aleatoriamente, para participarem do estudo. Estes, foram agrupados em três faixas etárias: menores de 12 meses (11,32%), com idades entre um e cinco anos (73,58%) e maiores de cinco anos (15,09%), sendo que 180 (26,1%) tinham raça definida e 509 (73,9%) não. Do total de amostras, 144 (21%) foram positivas para a presença de anticorpos anti-T. gondii e 78 (11,3%) para anticorpos anti-N. caninum. Foi observada correlação estatística entre os títulos de anticorpos e idade, para as duas infecções, o que não ocorreu com relação ao sexo e a raça

Active surveillance of canine visceral leishmaniasis and american trypanossomiasis in rural dogs from non endemic area

A leishmaniose visceral canina (LVC) e a tripanossomíase americana são importantes zoonoses para a saúde pública que encontram no cão o principal reservatório doméstico para o homem. O trabalho procurou estimar a prevalência de anticorpos circulantes anti-Trypanosoma cruzi e anti-Leishmania spp., em amostras de cães provenientes da zona rural do município de Botucatu, SP. Durante a campanha de vacinação antirrábica canina da zona rural do município, foram coletadas 689 amostras de soro e processadas pela técnica de imunofluorescência indireta. Os testes sorológicos revelaram a ausência de anticorpos anti-Leishmania spp. e, na pesquisa dos anticorpos anti-T. cruzi, foram detectados 3 (0,4%) cães.The canine visceral leishmaniasis (CVL) and american trypanosomiasis are important zoonoses in public health and dogs are the main domestic reservoir of the parasite for humans. The goal of this study was to estimate the prevalence of circulating antibodies anti-Trypanosoma cruzi and anti-Leishmania sp. in sera of dogs from the rural area of Botucatu, SP, Brazil. During the annual vaccination campaign against canine rabies in rural area, 689 blood samples were taken and processed by indirect immunofluorescent antibody test. The serological tests revealed the absence of antibodies anti-Leishmania spp., but anti-T. cruzi antibodies were detected in 3 (0.4%) dogs