Angiosarcoma of the Pelvis in a 13-Year-Old Girl.

Angiosarcomas are highly aggressive malignancies of vascular origin and are very rarely found in children. We report a case of a 13-year-old girl with a history of abdominal pain and increased abdominal girth. Radiologic imaging showed significant ascites and large pelvic masses involving bilateral adnexa with abdominal spread. Microscopic examination of a biopsy revealed pleomorphic epithelioid and spindle cells with brisk mitotic activity, intracytoplasmic vacuoles, vascular channels, and large areas of hemorrhage and necrosis. Immunohistochemistry analysis showed strong and diffuse positivity for CD31, D2-40, ERG, FLI-1, and focally for CD34, vWF, and EMA. The diagnosis of metastatic angiosarcoma was rendered. The patient was treated aggressively with systemic chemotherapy, immunotherapy, cytoreductive surgery, and hyperthermic intraperitoneal chemotherapy, with a favorable response after 1-year follow-up. Angiosarcoma should be considered when encountering a vascular tumor with pleomorphism, brisk mitotic activity, and necrosis. Immunohistochemistry studies are necessary for proper diagnosis.

Gestión pública territorial de la seguridad ciudadana en Colombia. Análisis de las inconsistencias normativas y las afectaciones en las relaciones interinstitucionales / Public territorial management of citizen security in Colombia. Analysis of normative inconsistencies and affectations in institutional relationships / Gestão pública territorial da segurança cidadã na Colômbia. A análise das inconsistências normativas e das afetações nas relações interinstitucionais

Servidores públicos que participan en la gestión territorial de la seguridad ciudadana (GTS) advierten que en la normativa existen incoherencias que afectan la coordinación interinstitucional y el funcionamiento mismo de los instrumentos que contiene el modelo de GTS. En este artículo, resultado de investigación, es de interés indagar cuáles son las inconsistencias y afectaciones derivadas de la normativa que regula tres de los instrumentos del modelo GTS, la coordinación, la planeación y las fuentes de recursos. Primero se describieron los instrumentos, luego se analizó la normativa que los regula y, finalmente, se formularon tres rutas de acción para que las iniciativas de reforma normativa se incluyan en la agenda pública. En el método se consideró el desarrollo de la consulta documental, entrevistas, grupos focales y de discusión, así como técnicas de recolección de información. Se diseñaron matrices para realizar análisis de contenido y comparaciones. Mediante la reducción de variables se configuraron seis categorías, en las que se agruparon las inconsistencias normativas de los instrumentos más representativos de planeación, coordinación y fuentes de recursos, inconsistencias cuyo ajuste debe ser una prioridad para la agenda pública, porque afectan el proceso de descentralización, la modernización del Estado y la gestión territorial de los problemas públicos de violencia y delincuencia, de cara a la construcción de paz desde el ámbito territorial.

Public servers taking part in territorial management of citizen security ("Gestión Territorial de la Seguridad Ciudadana" - GTS) have noticed that incoherencies in the normative are affecting inter-institutional coordination, and even the functioning of the instruments containing the GTS model itself. In this article, as the result of a research, it becomes interesting to inquire what these inconsistencies and affectations are that derive from the regulations governing three of the GTS model's instruments, i.e. planning, coordination, and funding sources. In the first place, the instruments were described and, subsequently, their governing normative was analyzed. Finally, three action routes were drawn up in order to include the relevant amendment initiatives in the public agenda. In the method, the development of documentary consultation was taken into consideration together with interviews and focus and discussion groups, as well as information-gathering techniques. Matrices were designed to help carry out content analyses and comparisons. Through the reduction of variables, six categories were set within which the normative inconsistencies of the most representative instruments of planning, coordination and funding sources were grouped, the adjustment of which must become a public agenda priority since they affect both the decentralization and State modernization processes, along with territorial management of public violence and criminality issues, in facing the construction of peace from the territorial scope.

Servidores públicos que participam na gestão territorial da segurança cidadã (GTS) advertem que na normativa existem incoerências que afetam a coordenação interinstitucional e a operação mesma dos instrumentos que contem o modelo de GTS. Neste artigo, o resultado da pesquisa, é do interesse pesquisar quais são as inconsistências e os afetações derivadas da norma que regula três dos instrumentos do modelo GTS, da coordenação, do planejamento e das fontes dos recursos. Os instrumentos foram descritos primeiramente, depois a norma que os regula foi analisada, e finalmente, as três rotas da ação foram formulada de modo que as iniciativas da reforma normativa sejam incluídas na agenda pública. No método considerou-se o desenvolvimento da consulta documental, entrevistas, grupos focais e da discussão, assim como técnicas de coleta de informação. As matrizes foram projetadas para fazer a análise do conteúdo e comparações. Por meio da redução das variáveis seis categorias foram configuradas. Nessas categorias, as inconsistências normativas dos instrumentos os mais representativos do planejamento, da coordenação e das fontes dos recursos foram agrupadas, inconsistências cujo ajuste deve ser uma prioridade para a agenda pública, porque afetam o processo da decentralização, da modernização do Estado e da gestão territorial dos problemas público da violência e da delinquência, a fim de construir a paz do âmbito territorial.

Possible Genetic Origin of Limb-Body Wall Complex.

Limb body wall complex (LBWC) is characterized by multiple severe congenital malformations including an abdominal and/or thoracic wall defect covered by amnion, a short or absent umbilical cord with the placenta almost attached to the anterior fetal wall, intestinal malrotation, scoliosis, and lower extremity anomalies. There is no consensus about the etiology of LBWC and many cases with abnormal facial cleft do not meet the requirements for the true complex. We describe a series of four patients with LBWC and other malformations in an attempt to explain their etiology. There are several reports of fetuses with LBWC and absent gallbladder and one of our patients also had polysplenia. Absent gallbladder and polysplenia are associated with laterality genes including HOX, bFGF, transforming growth factor beta/activins/BMP4, WNT 1-8, and SHH. We postulate that this severe malformation may be due to abnormal genes involved in laterality and caudal development.

Autoimmune hepatitis and primary sclerosing cholangitis in children and adolescents.

UNLABELLED: Clinical presentation and histopathology of autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) overlap syndrome (OS) are similar, but their management is different. We conducted a pediatric retrospective cross-sectional study of 34 patients with AIH and PSC. AIH had female predominance (74%) and was lower in PSC (45%). There was a trend toward higher frequency of blacks in PSC/OS (55%) compared to Caucasians (36%) and Hispanics (9%), but not race differences in AIH. Inflammatory bowel disease (IBD) was present in 75% of PSC/OS. Plasma cells were not specific for AIH (found in 42% of PSC). Concentric fibrosis was not reliable for PSC as was found in 46% of AIH. CONCLUSION: A combination of clinical history, laboratory tests, imaging studies and liver biopsy are required to confirm and properly treat AIH and PSC. Liver biopsy should be used to grade severity and disease progression, but cannot be used alone to diagnose these conditions.