Abdominal angiostrongyliasis, report of two cases and analysis of published reports from Colombia. / Abdominal angiostrongyliasis, report of two cases and analysis of published reports from Colombia

Abdominal angiostrongyliasis is a parasitic zoonosis, endemic in the American continent. Its etiological agent is Angiostrongylus costaricensis, a nematode whose definitive hosts are rats and other rodents and the intermediate hosts, slugs. Mammals acquire the infection by consuming vegetables contaminated with L3 larvae. The disease shows a heterogeneous clinical spectrum and given its low incidence its diagnosis is a great challenge. In Colombia, the first case was reported in 1979 and until 1998, only five additional cases have been reported. However, in the last two decades, no new cases were reported. Here we discuss two cases of children from Huila and Caquetá departments who developed the disease. Both cases required long in-patient care and multiple surgical interventions. The diagnosis was achieved by histopathological observation of parasitic elements inside the mesenteric arteries. One of the children died while the other fully recovered. We discuss the epidemiology, pathogenic cycle, clinical presentation, diagnosis, and prevention strategies of this disease paying particular attention to our patients' features and the Colombian context.

La angiostrongiloidiasis abdominal es una zoonosis parasitaria endémica en el continente americano. Su agente etiológico es el nematodo Angiostrongylus costaricensis, cuyos huéspedes definitivos son los roedores y, los intermediarios, los caracoles y las babosas, por lo que se adquiere al consumir vegetales contaminados con larvas en estadio 3. La presentación clínica es muy variada y, dada su rareza, su diagnóstico es un desafío. En Colombia el primer caso se reportó en 1979 y, desde entonces hasta 1998, se han informado cinco casos más, aunque en las últimas dos décadas no se había reportado ningún caso. Se describen aquí dos casos de angiostrongiloidiasis en niños provenientes de los departamentos de Huila y Caquetá que requirieron una larga hospitalización y múltiples intervenciones quirúrgicas. El diagnóstico se logró al observar en los especímenes quirúrgicos larvas and huevos cuya morfología sugería una infección por nematodos; uno de los pacientes murió y el otro se recuperó satisfactoriamente. Se discuten la epidemiología, la patogenia, la presentación clínica, el diagnóstico y las estrategias de prevención de esta parasitosis, con énfasis en las características particulares de los casos descritos y en el contexto colombiano.

Abdominal angiostrongyliasis, report of two cases and analysis of published reports from Colombia / Angiostrongiliasis abdominal, reporte de dos casos y análisis de los casos publicados en Colombia

Abdominal angiostrongyliasis is a parasitic zoonosis, endemic in the American continent. Its etiological agent is Angiostrongylus costaricensis, a nematode whose definitive hosts are rats and other rodents and the intermediate hosts, slugs. Mammals acquire the infection by consuming vegetables contaminated with L3 larvae. The disease shows a heterogeneous clinical spectrum and given its low incidence its diagnosis is a great challenge. In Colombia, the first case was reported in 1979 and until 1998, only five additional cases have been reported. However, in the last two decades, no new cases were reported. Here we discuss two cases of children from Huila and Caquetá departments who developed the disease. Both cases required long in-patient care and multiple surgical interventions. The diagnosis was achieved by histopathological observation of parasitic elements inside the mesenteric arteries. One of the children died while the other fully recovered. We discuss the epidemiology, pathogenic cycle, clinical presentation, diagnosis, and prevention strategies of this disease paying particular attention to our patients' features and the Colombian context.

La angiostrongiloidiasis abdominal es una zoonosis parasitaria endémica en el continente americano. Su agente etiológico es el nematodo Angiostrongylus costaricensis, cuyos huéspedes definitivos son los roedores y, los intermediarios, los caracoles y las babosas, por lo que se adquiere al consumir vegetales contaminados con larvas en estadio 3. La presentación clínica es muy variada y, dada su rareza, su diagnóstico es un desafío. En Colombia el primer caso se reportó en 1979 y, desde entonces hasta 1998, se han informado cinco casos más, aunque en las últimas dos décadas no se había reportado ningún caso. Se describen aquí dos casos de angiostrongiloidiasis en niños provenientes de los departamentos de Huila y Caquetá que requirieron una larga hospitalización y múltiples intervenciones quirúrgicas. El diagnóstico se logró al observar en los especímenes quirúrgicos larvas and huevos cuya morfología sugería una infección por nematodos; uno de los pacientes murió y el otro se recuperó satisfactoriamente. Se discuten la epidemiología, la patogenia, la presentación clínica, el diagnóstico y las estrategias de prevención de esta parasitosis, con énfasis en las características particulares de los casos descritos y en el contexto colombiano.

Primary cardiac tumors: 32 years of experience from a Spanish tertiary surgical center.

INTRODUCTION: Primary cardiac tumors are rare entities with an autopsy frequency ranging between 0.001% and 0.3%. Our objective was to review 32 years of experience from a Spanish tertiary surgical center. METHOD: We collected data on 73 patients with a histopathological diagnosis of a primary cardiac tumor in a retrospective analysis from 1979 to 2012. Tissue samples were obtained either at surgery or from necropsy and subsequently divided into benign and malignant groups. RESULTS: Benign neoplasms accounted for 84.9% of cases. The average age at diagnosis was 61 years, and tumors were twice as frequent among women. It was an incidental finding in one quarter of patients. In symptomatic patients, the typical presentation was of cardioembolic stroke or of congestive symptoms. Myxoma (93.5%) was the most common diagnosis, typically affecting the left atrium (74.2%). Surgical resection was curative for 95% of these patients. Malignant tumors represented 15.1% of cases with an average age at diagnosis of 50 years. Over 90% of these patients were symptomatic at presentation with the cardinal symptom being heart failure. Undifferentiated sarcoma was the most frequent malignant tumor (36.4%). Less than 20% of patients were alive 1 year after diagnosis despite the treatment. CONCLUSIONS: In our study, primary cardiac tumors were insidious. The most common was left atrial myxoma in which surgical resection was considered curative. Undifferentiated sarcoma was the most frequent malignant tumor. Malignant neoplasms carried a worse prognosis with unsuccessful total excision and the presence of metastatic disease being markers of an adverse outcome.

Adaptor protein complex 2-mediated, clathrin-dependent endocytosis, and related gene activities, are a prominent feature during maturation stage amelogenesis.

Molecular events defining enamel matrix removal during amelogenesis are poorly understood. Early reports have suggested that adaptor proteins (AP) participate in ameloblast-mediated endocytosis. Enamel formation involves the secretory and maturation stages, with an increase in resorptive function during the latter. Here, using real-time PCR, we show that the expression of clathrin and adaptor protein subunits are upregulated in maturation stage rodent enamel organ cells. AP complex 2 (AP-2) is the most upregulated of the four distinct adaptor protein complexes. Immunolocalization confirms the presence of AP-2 and clathrin in ameloblasts, with strongest reactivity at the apical pole. These data suggest that the resorptive functions of enamel cells involve AP-2 mediated, clathrin-dependent endocytosis, thus implying the likelihood of specific membrane-bound receptor(s) of enamel matrix protein debris. The mRNA expression of other endocytosis-related gene products is also upregulated during maturation including: lysosomal-associated membrane protein 1 (Lamp1); cluster of differentiation 63 and 68 (Cd63 and Cd68); ATPase, H(+) transporting, lysosomal V0 subunit D2 (Atp6v0d2); ATPase, H(+) transporting, lysosomal V1 subunit B2 (Atp6v1b2); chloride channel, voltage-sensitive 7 (Clcn7); and cathepsin K (Ctsk). Immunohistologic data confirms the expression of a number of these proteins in maturation stage ameloblasts. The enamel of Cd63-null mice was also examined. Despite increased mRNA and protein expression in the enamel organ during maturation, the enamel of Cd63-null mice appeared normal. This may suggest inherent functional redundancies between Cd63 and related gene products, such as Lamp1 and Cd68. Ameloblast-like LS8 cells treated with the enamel matrix protein complex Emdogain showed upregulation of AP-2 and clathrin subunits, further supporting the existence of a membrane-bound receptor-regulated pathway for the endocytosis of enamel matrix proteins. These data together define an endocytotic pathway likely used by ameloblasts to remove the enamel matrix during enamel maturation.