Two Synchronous Neonatal Tumors: An Extremely Rare Case.

We report a case of a newborn with two synchronous tumors-sialoblastoma and hepatoblastoma-diagnosed at 20 weeks of gestation by magnetic resonance imaging (MRI) and ultrasonography (US). The aim of this study was to describe the management of this case together with a review of the literature. Our patient had a large facial tumor associated with extremely high alpha-fetoprotein levels. Diagnosis of the tumors was made by surgical biopsy, showing typical features in both. Sialoblastoma is a potentially aggressive tumor. In our case, the Ki67 index in the sialoblastoma was between 20 and 30%, indicating a possibly unfavorable behavior. The infant underwent surgery and chemotherapy in different steps. Complete surgical resection with clean margins is considered to be the best treatment option for sialoblastoma. Only four similar cases were previously reported. Timely management by a multidisciplinary team is essential in these difficult cases. In our patient, outcome was good at the time of this report.

Adherence of gastroenterologists to European Crohn's and Colitis Organisation consensus on Crohn's disease: a real-life survey in Spain.

BACKGROUND: There is no information as to the extent by which Spanish gastroenterologists adhere to Crohn's disease (CD) management guidelines. The objective of this study was to evaluate the degree of adherence of Spanish gastroenterologists to the European Crohn's and Colitis Organisation (ECCO) guidelines and to determine whether differences in adherence exist between gastroenterologists specialized in inflammatory bowel diseases (GSIBDs) and general gastroenterologists (GGs). METHODS: This was a prospective, nation-wide, questionnaire-based survey covering aspects related to diagnosis, treatment, follow-up, and safety considered by the physicians in their daily management of CD, as well as demographic traits seen in clinical practice. RESULTS: The overall degree of adherence to guidelines by both GSIBDs and GGs was high. However, the use of imaging techniques in diagnosis, follow-up, and in relapsed patients differed between the two groups. In the diagnosis of perianal disease, GSIBDs used magnetic resonance and surgical exploration under anesthesia more frequently than GGs. In terms of therapeutic choices, the adherence to guidelines was good in both groups. However, GSIBDs showed significantly higher adherence in some areas: thiopurines were used less in refractory cases and methotrexate was used more commonly in corticoid-dependent, azathioprine-intolerant patients, and in patients under biological treatment. Request for infection studies and vaccinations at diagnosis or prior to treatment was more common among GSIBDs. CONCLUSIONS: Guideline adherence among Spanish gastroenterologists is high. However, there are significant differences between IBD-specialized (more adherent in general) and non-specialized gastroenterologists.

Adherence of gastroenterologists to European Crohn's and Colitis Organisation consensus on ulcerative colitis: a real-life survey in Spain.

BACKGROUND & AIMS: A European consensus on the management of ulcerative colitis (UC) was recently published; however, there is no adequate evidence about adherence to such guidelines among gastroenterologists. This knowledge would allow the local evaluation of the situation and the adoption of actions to reduce the existent clinical variability. METHODS: A cross-sectional survey was conducted in Spain to assess the adherence to the European Crohn's and Colitis Organisation (ECCO) guidelines on mild to moderate UC. We surveyed 700 gastroenterologists, and finally a total of 530 gastroenterologists specialised in inflammatory bowel disease (GSIBDs) and general gastroenterologists (GGs), responded to the survey (76%). RESULTS: Agreement with the guidelines was high; discrepancies included that only 25% of the GGs used the combination of oral and topical 5-aminosalycilic acid (5-ASA) for treating extensive UC vs 45% of the GISBDs. In addition, topical rectal steroids were considered as effective as topical mesalazine by 48% of the GGs vs 31% of the GSIBDs, indefinite treatment with 5-ASA was prescribed by only 26% of the GGs vs 41% of the GSIBDs, and the once daily dosing of 5-ASA was generally used by 46% of the GGs vs 51% of the GSIBDs. CONCLUSIONS: The questionnaire showed a high degree of agreement between GGs and GSIBDs. Nevertheless, the GSIBD group showed closer agreement with the ECCO guidelines. Furthermore, some shortcomings were found in the GG group, in which increased maintenance treatment with 5-ASA, the use of a single daily dose of 5-ASA, and the use of combined oral and topical treatment for distal colitis should be advised.

Clinical outcome of newly diagnosed Crohn's disease: a comparative, retrospective study before and after infliximab availability.

BACKGROUND: Infliximab (IFX) could change the course of Crohn's disease (CD) by reducing steroid use, surgery or prompting earlier introduction of immunomodulators (IMM). AIM: To evaluate the impact of IFX availability on the course of early CD. METHODS: Two cohorts of newly diagnosed CD patients were identified: The first cohort included patients diagnosed from January 1994 to December 1997 and the second from January 2000 to December 2003. All patients were diagnosed, treated and followed up in the same centre until December 1999 (first cohort) or December 2005 (second cohort). Development of disease-related complications, steroid, IMM or IFX requirements and intestinal resections during follow-up were registered. RESULTS: A total of 328 patients were included (146 first cohort, 182 second cohort). A similar proportion of patients in both cohorts received steroids, but steroid exposure resulted significantly more intense in the first cohort (P = 0.001). In the second cohort, 14% of patients received IFX. Thiopurines were used more (P = 0.001) and earlier (P = 0.012) in the second cohort. No differences in surgical requirements or the development of disease-related complications were found. CONCLUSIONS: Following a step-up therapeutic algorithm, IFX availability did not reduce surgical requirements or the development of disease-related complications.

Therapeutic strategies after coronary stenting in chronically anticoagulated patients: the MUSICA study.

OBJECTIVES: To identify the therapeutic regimens used at discharge in patients receiving oral anticoagulant therapy (OAT) who undergo stenting percutaneous coronary intervention and stent implantation (PCI-S), and to assess the safety and efficacy associated with different therapeutic regimens according to thromboembolic risk. DESIGN: A prospective multicentre registry. SETTING: In hospital, after discharge and follow-up by telephone call. PATIENTS AND METHODS: 405 patients (328 male/77 female; mean (SD) age 71 (9) years) receiving OAT who underwent PCI-S between November 2003 and June 2006 from nine catheterisation laboratories of tertiary care teaching hospitals in Spain and one in the United Kingdom were included. RESULTS: Three therapeutic regimens were identified at discharge: triple therapy (TT) -- that is, any anticoagulant (AC) plus double antiplatelet therapy (DAT; 278 patients (68.6%); AC and a single antiplatelet (AC+AT; 46 (11.4%)) and DAT only (81 (20%)). At 6 months, patients receiving TT showed the greatest rate of bleeding events. No patients receiving DAT at low thromboembolic risk presented a bleeding event (14.8% receiving TT, 11.8% receiving AC+AT and 0% receiving DAT, p = 0.033) or cardiovascular event (6.7% receiving TT, 0% receiving AC+AT and 0% receiving DAT, p = 0.126). The combination of AC+AT showed the worst rate of adverse events in the whole cohort, especially in patients at moderate-high thromboembolic risk. CONCLUSIONS: In patients receiving OAT, TT was the most commonly used regimen after PCI-S. DAT was associated with the lowest rate of bleeding events and a similar efficacy to TT in patients at low thromboembolic risk. TT should probably be restricted to patients at moderate-high thromboembolic risk.

Adalimumab en la enfermedad de Crohn: los datos de la vida real / No disponible

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Toxicidad hepática recurrente secundaria a metilprednisolona intravenosa / No disponible

Las reacciones adversas hepáticas relacionadas con la administraciónde fármacos (hepatotoxicidad) son cuadros relativamentefrecuentes que presentan una amplia variabilidad clínica e histológica.La identificación precoz de estos cuadros es fundamental enla práctica clínica debido a su potencial gravedad. En la mayoríade los casos la suspensión del fármaco desencadenante es suficientepara la resolución del cuadro clínico.A pesar de que los esteroides son utilizados en una amplia variedadde situaciones clínicas, la notificación de cuadros de hepatotoxicidadsecundaria a esteroides intravenosos es excepcional.Presentamos el caso clínico de una mujer diagnosticada de esclerosismúltiple, que recibió metilprednisolona a altas dosis enforma de “pulsos” intravenosos como tratamiento de las reagudizacionesde su enfermedad y presentó 3 brotes recurrentes de hepatitisde predominio hepatocelular con un patrón clínico, analíticoe histológico compatible con toxicidad hepática agudasecundaria a metilprednisolona intravenosa. En el tercer episodiose realizó una biopsia hepática que demostró un patrón de hepatitisaguda con necrosis líticas confluentes, histología no descritapreviamente en pacientes tratados con esteroides intravenosos

Adverse drug reactions (hepatotoxicity) are a frequent cause ofacute liver injury with a wide clinical and histological spectrum. Anearly recognition of drug-related liver disease has been consideredessential in clinical practice due to potential risks. In most casesexposure discontinuation improves the clinical picture.Steroids are used in a variety of clinical settings. However, intravenoussteroids have rarely been associated with hepatotoxicity.We report the case of a middle-aged woman with multiple sclerosiswho received a bolus of methylprednisolone on threeoccasions for the management of relapsing disease, with the developmentof repeated episodes of elevated liver enzymes aftercorticoid administration. In the third episode a liver biopsy wasperformed, which showed acute hepatitis with bridging necrosis;such histological picture has not been described before in patientstreated with intravenous steroids

Hairy esophagus: una complicación de la cirugía reconstructiva faringoesofágica / No disponible

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[Recurrent acute liver toxicity from intravenous methylprednisolone]. / Toxicidad hepática recurrente secundaria a metilprednisolona intravenosa.

Adverse drug reactions (hepatotoxicity) are a frequent cause of acute liver injury with a wide clinical and histological spectrum. An early recognition of drug-related liver disease has been considered essential in clinical practice due to potential risks. In most cases exposure discontinuation improves the clinical picture.Steroids are used in a variety of clinical settings. However, intravenous steroids have rarely been associated with hepatotoxicity. We report the case of a middle-aged woman with multiple sclerosis who received a bolus of methylprednisolone on three occasions for the management of relapsing disease, with the development of repeated episodes of elevated liver enzymes after corticoid administration. In the third episode a liver biopsy was performed, which showed acute hepatitis with bridging necrosis; such histological picture has not been described before in patients treated with intravenous steroids.

“Enfermedad de las tres mentiras”: síndrome de la úlcera rectal solitaria / “The three-lies disease”: Solitary rectal ulcer syndrome

El síndrome de la úlcera rectal solitaria es una entidad benignapoco común cuyos síntomas más frecuentes son la rectorragia y eldolor anal. Los hallazgos anatomopatológicos son típicos. Se sueleevidenciar engrosamiento de la mucosa, elongación y distorsiónde las glándulas, una lámina propia edematosa con gran cantidadde colágena y engrosamiento de la muscularis mucosae. El diagnósticose realiza mediante endoscopia con toma de biopsias. Nosiempre se trata de lesiones ulceradas. Se suele localizar preferentementeen la cara rectal anterior y/o lateral, aunque hasta un30% de las lesiones son múltiples, existiendo casos de afectacióndel colon sigmoide y descendente. Por tanto, la presentación puedeser heterogénea y este es el motivo por el que esta entidad estambién conocida como la “enfermedad de las tres mentiras”.Presentamos un caso de síndrome de la ulcera rectal solitaria manifestadoendoscópicamente como una placa eritematosa localizadaen la cara lateral izquierda del recto

Solitary rectal ulcer syndrome is an uncommon benign conditioncharacterized by rectal bleeding, passage of mucus, and pain.Histological features are well established as obliteration of thelamina propria by fibrosis and smooth-muscle fibers extendingfrom a thickened muscularis mucosa to the lumen. Diagnosis canusually be made on sigmoidoscopy, and biopsies should always betaken. Ulceration is not universally present, and polypoid, non-ulceratedlesions and erythematous areas are also seen. The lesionor lesions are most often found on the anterior or anterolateralwall of the rectum, although they can also be located in the leftcolon and be more extensive or even circumferential. Lesions aremultiple in 30 percent of cases. These are the reasons why thisentity is also known as “the disease of three lies”. We report acase of solitary rectal ulcer syndrome presenting at endoscopywith an erythematous area on the left side wall of the rectum (AU)

[Current status of inflammatory bowel disease treatment]. / Estado actual del tratamiento de la enfermedad inflamatoria intestinal.

Treatment of inflammatory bowel disease should take some general considerations into account, standing out among them the importance of nutrition, antithrombotic prophylaxis in certain situations, prevention of osteoporosis and prevention of colorectal cancer by endoscopic screening in extensive ulcerous colitis or Crohn's colitis. Ulcerous colitis is still effectively treated with salicylates and steroids in its mild and moderate forms. Severe forms require parenteral steroids, cyclosporine or infliximab, and these are successfully used with immunosuppressants (azathioprine or mercaptopurine) in steroid dependence. Maintenance with salicylates, that should be adequately complied with, is an important point. Crohn's disease is treated with steroids in its mild and moderate forms, and again in corticodependent ones with immunosuppressants, which we are using increasingly sooner. Refractory forms or fistulous forms benefit from the use of biological treatments (infliximab) generally accompanied by immunosuppressants.

Estado actual del tratamiento de la enfermedad inflamatoria intestinal / Current status of inflammatory bowel disease treatment

El tratamiento de la enfermedad inflamatoria intestinal debe tener en cuenta unas consideraciones generales, entre las que destacan la importancia de la nutrición, de la profilaxis antitrombótica en determinadas situaciones, de la prevención de la osteoporosis y de la prevención del cáncer colorrectal mediante cribado endoscópico en la colitis ulcerosa o la colitis de Crohn extensas. La colitis ulcerosa se sigue tratando eficazmente con salicilatos y esteroides en las formas leves y moderadas. Las formas graves requieren esteroides parenterales, ciclosporina o infliximab, y se emplean con éxito inmunosupresores (azatioprina o mercaptopurina) en la dependencia a esteroides. El mantenimiento con salicilatos, que debe ser cumplido adecuadamente, es un punto importante. La enfermedad de Crohn se trata con esteroides en sus formas leves y moderadas, y en las corticodependientes de nuevo con inmunosupresores, que cada vez usamos más pronto. Las formas refractarias o las formas fistulosas se benefician del uso de tratamientos biológicos (infliximab), por lo general acompañados de inmunosupresores (AU)

Treatment of inflammatory bowel disease should take some general considerations into account, standing out among them the importance of nutrition, antithrombotic prophylaxis in certain situations, prevention of osteoporosis and prevention of colorectal cancer by endoscopic screening in extensive ulcerous colitis or Crohn's colitis. Ulcerous colitis is still effectively treated with salicylates and steroids in its mild and moderate forms. Severe forms require parenteral steroids, cyclosporine or infliximab, and these are successfully used with immunosuppressants (azathioprine or mercaptopurine) in steroid dependence. Maintenance with salicylates, that should be adequately complied with, is an important point. Crohn's disease is treated with steroids in its mild and moderate forms, and again in corticodependent ones with immunosuppressants, which we are using increasingly sooner. Refractory forms or fistulous forms benefit from the use of biological treatments (infliximab) generally accompanied by immunosuppressants (AU)

[A prospective study about the usefulness of ultrasonographic monitoring after invasive liver procedures--liver biopsy and fine-needle aspiration (FNA)]. / Estudio prospectivo sobre la utilidad de la ecografía de control tras la realización de pruebas invasivas hepáticas: biopsia hepática y punción aspiración con aguja fina (PAAF).

OBJECTIVE: To determine the need to perform ultrasound scans to all patients after liver biopsy or fine-needle aspiration (FNA) in order to detect complications with or without symptoms. MATERIAL AND METHODS: After liver biopsy or FNA using a regular protocol the patient is observed for 24 hours at the hospital, and all patients undergo an abdominal sonography at that time even in the absence of evident complications. RESULTS: 298 liver biopsies and 98 FNAs were performed. There were complications in 37 patients (9.34%): 36 (9.09%) were minor complications such as pain, vasovagal episodes, or small bleeding, and 1 (0.25%) was a major complication with severe hemorrhage. Only 1 out of all 396 procedures had a complication detected by ultrasounds (intrahepatic hematoma) while the patient was asymptomatic. CONCLUSIONS: The low incidence of complications occurring without symptoms, and their favorable course suggest that routine ultrasonography is not necessary after these techniques, and that it should be only performed when a complication is suspected.

Estudio prospectivo sobre la utilidad de la ecografía de control tras la realización de pruebas invasivas hepáticas: biopsia hepática y punción aspiración con aguja fina (PAAF) / A prospective study about the usefulness of ultrasonographic monitoring after invasive liver procedures –liver biopsy and fine-needle aspiration (FNA)

Objetivo: establecer si es necesario realizar una ecografía de control a todos los pacientes sometidos a una biopsia hepática o una punción aspiración con aguja fina, para detectar posibles complicaciones con o sin repercusión clínica. Material y métodos: tras la realización de una biopsia hepática o una punción aspiración con aguja fina según el protocolo habitual, se mantiene al paciente en observación durante 24 horas, realizándose en ese momento una ecografía a todos los pacientes aunque no presenten datos clínicos de complicación. Resultados: se llevaron a cabo 298 biopsias hepáticas y 98 punciones mediante aguja fina. Presentaron complicaciones un total de 37 pacientes (9,34%), de las cuales 36 (9,09%) fueron complicaciones menores en forma de dolor, síncope vasovagal o hemorragia leve y 1 (0,25%) complicación mayor en forma de hemorragia grave. De las 396 exploraciones tan sólo uno de los casos presentó una complicación detectada en la ecografía (hematoma intraparenquimatoso) encontrándose asintomático. Conclusiones: la baja incidencia de complicaciones, que cursan de forma asintomática, y la buena evolución de las mismas hacen poco rentable la realización de ecografía de control tras la realización de dichas técnicas diagnósticas, siendo necesaria tan sólo en el caso de sospecha clínica de complicación

Objective: to determine the need to perform ultrasound scans to all patients after liver biopsy or fine-needle aspiration (FNA) in order to detect complications with or without symptoms. Material and methods: after liver biopsy or FNA using a regular protocol the patient is observed for 24 hours at the hospital, and all patients undergo an abdominal sonography at that time even in the absence of evident complications. Results: 298 liver biopsies and 98 FNAs were performed. There were complications in 37 patients (9.34%): 36 (9.09%) were minor complications such as pain, vasovagal episodes, or small bleeding, and 1 (0.25%) was a major complication with severe hemorrhage. Only 1 out of all 396 procedures had a complication detected by ultrasounds (intrahepatic hematoma) while the patient was asymptomatic. Conclusions: the low incidence of complications occurring without symptoms, and their favorable course suggest that routine ultrasonography is not necessary after these techniques, and that it should be only performed when a complication is suspected