Pediatric Plexiform Fibromyxoma: A Case Report.

The plexiform fibromyxoma is a rare mesenchymal tumor in adults that generally originates in the antrum of stomach, being its occurrence in pediatric patients exceptional. It was classified as a distinct entity by World Health Organization in 2010. No recurrences and metastases have been documented in many of the reported patients to date, being the surgical treatment curative. We report the case of a 3-month-old infant who presented to the emergency department with an episode of intestinal subocclusion requiring an emergent surgery. During the surgical intervention, a mass was identified in the jejunum, causing partial occlusion of its lumen. The surgical pathology report revealed an infiltrative tumor composed of spindle-shaped cells disposed in a stroma with a plexiform pattern alternating myxoid areas. These findings and the immunohistochemical characteristics of the neoplastic cells led to classify the tumor as a plexiform fibromyxoma. A description of the immunophenotype of this tumor is made and differential diagnosis with other gastrointestinal tumors is also discussed.

Endoglin, a Novel Biomarker and Therapeutical Target to Prevent Malignant Peripheral Nerve Sheath Tumor Growth and Metastasis.

PURPOSE: Malignant peripheral nerve sheath tumors (MPNST) are highly aggressive soft-tissue sarcomas that lack effective treatments, underscoring the urgent need to uncover novel mediators of MPNST pathogenesis that may serve as potential therapeutic targets. Tumor angiogenesis is considered a critical event in MPNST transformation and progression. Here, we have investigated whether endoglin (ENG), a TGFß coreceptor with a crucial role in angiogenesis, could be a novel therapeutic target in MPNSTs. EXPERIMENTAL DESIGN: ENG expression was evaluated in human peripheral nerve sheath tumor tissues and plasma samples. Effects of tumor cell-specific ENG expression on gene expression, signaling pathway activation and in vivo MPNST growth and metastasis, were investigated. The efficacy of ENG targeting in monotherapy or in combination with MEK inhibition was analyzed in xenograft models. RESULTS: ENG expression was found to be upregulated in both human MPNST tumor tissues and plasma-circulating small extracellular vesicles. We demonstrated that ENG modulates Smad1/5 and MAPK/ERK pathway activation and pro-angiogenic and pro-metastatic gene expression in MPNST cells and plays an active role in tumor growth and metastasis in vivo. Targeting with ENG-neutralizing antibodies (TRC105/M1043) decreased MPNST growth and metastasis in xenograft models by reducing tumor cell proliferation and angiogenesis. Moreover, combination of anti-ENG therapy with MEK inhibition effectively reduced tumor cell growth and angiogenesis. CONCLUSIONS: Our data unveil a tumor-promoting function of ENG in MPNSTs and support the use of this protein as a novel biomarker and a promising therapeutic target for this disease.

Document of standardization of enteral nutrition access in adults.

The group of standardization and protocols of the Spanish Society of Parenteral and Enteral Nutrition (SENPE) published in 2011 a consensus document SENPE/SEGHNP/ANECIPN/SECP on enteral access for paediatric nutritional support. Along the lines of this document, we have developed another document on adult patients to homogenize the clinical practice and improve the quality of care in enteral access in this age group. The working group included health professionals (nurses, dietitians and doctor) with extensive experience in enteral nutrition and access. We tried to find scientific evidence through a literature review and we used the criteria of the Agency for Health-care Research and Quality (AHRQ) to classify the evidence (Grade of Recommendation A, B or C). Later the document was reviewed by external experts to the group and requested the endorsement of the Scientific and Educational Committee (CCE) and the group of home artificial nutrition (NADYA) of the SENPE. The full text will be published as a monograph number in this journal.

El grupo de estandarización y protocolos de la Sociedad Española de Nutrición Parenteral y Enteral (SENPE) publicó en el año 2011 un Documento de Consenso SEN - PE/SEGHNP/ANECIPN/SECP sobre vías de acceso en nutrición enteral (NE) pediátrica. Siguiendo las líneas de este documento, hemos querido realizar un documento similar centrado en los pacientes adultos que sirva para homogeneizar la práctica clínica y mejorar la calidad de los cuidados de las vías de acceso en NE en este grupo de edad. El grupo de trabajo incluyó a profesionales (enfermeras, dietistas y médico) con extensa experiencia en NE y vías de acceso Se intentó buscar la evidencia científica mediante una revisión bibliográfica y se utilizaron los criterios de la Agency for Health-care Research and Quality (AHRQ) para clasificar la evidencia (grados de recomendación A-B-C). Posteriormente el documento fue revisado por expertos externos al grupo y se solicitó el aval del Comité Científico Educacional (CCE) y del Grupo de Nutrición Artificial Domiciliaria y Ambulatoria (NADYA) de la SENPE. El texto completo se publicará como número monográfico.

Documento de estandarización sobre las vías de acceso en nutrición enteral en adultos / Document of standardization of enteral nutrition access in adults

El grupo de estandarización y protocolos de la Sociedad Española de Nutrición Parenteral y Enteral (SENPE) publicó en el año 2011 un Documento de Consenso SEN - PE/SEGHNP/ANECIPN/SECP sobre vías de acceso en nutrición enteral (NE) pediátrica. Siguiendo las líneas de este documento, hemos querido realizar un documento similar centrado en los pacientes adultos que sirva para homogeneizar la práctica clínica y mejorar la calidad de los cuidados de las vías de acceso en NE en este grupo de edad. El grupo de trabajo incluyó a profesionales (enfermeras, dietistas y médico) con extensa experiencia en NE y vías de acceso Se intentó buscar la evidencia científica mediante una revisión bibliográfica y se utilizaron los criterios de la Agency for Health-care Research and Quality (AHRQ) para clasificar la evidencia (grados de recomendación A-B-C). Posteriormente el documento fue revisado por expertos externos al grupo y se solicitó el aval del Comité Científico Educacional (CCE) y del Grupo de Nutrición Artificial Domiciliaria y Ambulatoria (NADYA) de la SENPE. El texto completo se publicará como número monográfico (AU)

The group of standardization and protocols of the Spanish Society of Parenteral and Enteral Nutrition (SENPE) published in 2011 a consensus document SENPE/SEGHNP/ANECIPN/SECP on enteral access for paediatric nutritional support. Along the lines of this document, we have developed another document on adult patients to homogenize the clinical practice and improve the quality of care in enteral access in this age group. The working group included health professionals (nurses, dietitians and doctor) with extensive experience in enteral nutrition and access. We tried to find scientific evidence through a literature review and we used the criteria of the Agency for Health-care Research and Quality (AHRQ) to classify the evidence (Grade of Recommendation A, B or C). Later the document was reviewed by external experts to the group and requested the endorsement of the Scientific Committee Educational (CCE) and the group of home artificial nutrition (NADYA) of the SENPE. The full text will be published as a monograph number in this journal (AU)

Complicaciones de la nutricion enteral / No disponible

No disponible

Locally destructive mandibular pseudotumor as a manifestation of immunoglobulin G4-related disease.

Immunoglobulin G4-related disease is a recently identified entity. The cervicofacial location is not frequent. We report the case of a patient with an inflammatory pseudotumor in the mandible, who had to undergo surgery for diagnosis. We review the clinical manifestations, diagnostic criteria, differential diagnosis, and treatment proposed by recent publications in the literature. This case report represents the first case located in the mandible and the second one with bone destruction.

[Nutrition. Generalities]. / Nutrición generalidades.

A patient must cover all his/her energetic and non-energetic nutritional needs in a sure manner It is essential to keep a patient inside his/her recommended weight ranges, having a balanced body composition which means he/she is well hydrated and has an adequate muscular and fat mass. The objective is to find a patient's optimum nutritional state.

[Intestinal nutrition by means of intravenous feeding, and parenteral feeding]. / Nutrición enteral por sonda, y parenteral.

Intestinal nutrition has an optimal range of applications and a wide variety of methods for use, both in composition and in osmolarity, adapted to different pathologies. This facilitated the use of intestinal nutrition for each specific case, whether the patient be an adult or a child, by means of feeds or catheters of varying calibres or access methods.

Nutrición. Generalidades / Nutrition. Generalities

El paciente debe cubrir de forma segura todas sus necesidades nutricionales energéticas y no energéticas. Es preciso conseguir que el enfermo se encuentre dentro de los rangos de peso recomendados, con una composición corporal equilibrada, es decir, bien hidratado, con una masa muscular y grasa adecuada. En definitiva, se trata de lograr su óptimo estado nutricional(AU)

A patient must cover all his/her energetic and non-energetic nutritional needs in a sure manner. It is essential to keep a patient inside his/her recommended weight ranges, having a balanced body composition which means he/she is well hydrated and has an adequate muscular and fat mass. The objective is to find a patient’s optimum nutritional state(AU)

Nutrición enteral por sonda y parenteral / Intestinal nutrition by means of intravenous feeding, and parenteral feeding

La NE (nutrición enteral) tiene optimizadas sus aplicaciones con un gran abanico de presentaciones, tanto en composición como en osmolaridad, adaptadas a las diferentes patologías. Ello facilita la prescripción en cada caso particular, ya sea en adultos o en pediatría, a través de sondas de diferentes accesos o calibres(AU)

Intestinal nutrition has an optimal range of applications and a wide variety of methods for use, both in composition and in osmolarity, adapted to different pathologies. This facilitated the use of intestinal nutrition for each specific case, whether the patient be an adult or a child, by means of feeds or catheters of varying calibres or access methods(AU)