ABSTRACT
Introducción: los tumores del estroma gastrointestinal (GIST)son neoplasias mesenquimales del tubo digestivo que generalmenteexpresan el receptor KIT (CD117) y muestran mutaciones enlos genes KIT o PDGFRA. Aunque la forma de presentación clínicahabitual es como una neoplasia mural solitaria, excepcionalmentepueden presentarse formas múltiples en el mismo o diferenteórgano.Objetivo: revisar las características morfológicas, inmunohistoquímicasy moleculares de las formas de GIST múltiples no metastásicos.Fuentes: revisión de la literatura en Medline y la propia experiencia.Conclusiones: los GIST múltiples pueden presentarse en trescontextos diferentes: lesiones espontáneas (del adulto o de la edadinfantil); síndrome familiar propio (transmitido con herencia autosómicadominante); y lesiones asociadas a síndromes específicos(tríada de Carney, síndrome de Carney-Stratakis, y neurofibromatosistipo I). Fuera de estos ámbitos, se interpreta que todo GISTmúltiple es el resultado de siembras tumorales metastásicas y, portanto, corresponde a enfermedad avanzada. Estas variantes debenser conocidas por el clínico dado las connotaciones pronósticas yterapéuticas que ello conlleva(AU)
Introduction: gastrointestinal stromal tumors (GISTs) are specific,generally KIT (CD117)-positive, mesenchymal tumors of thedigestive tract displaying KIT or PDGFRA gene mutations. Clinically,they tend to present as solitary tumors of the intestinal wall;more rarely, multiple tumors may occur in one or more organs.Objective: to review the morphological, immunohistochemicaland molecular features of multiple, non-metastatic forms ofGIST.Sources: review of the literature on Medline, and authorsown experience.Conclusions: multiples GISTs may occur in three differentcontexts: as spontaneous lesions (in both adults and children); dueto familial GIST syndrome (autosomal dominant inheritance); orin association with specific syndromes (e.g. Carneys triad, Carney-Stratakis syndrome, type I neurofibromatosis). Outside thesecontexts, the existence of multiple GISTs is deemed to be the resultof tumor metastasis, and therefore indicative of advanced-stagedisease. Clinicians need to be aware of these variants, whoseprognosis and treatment differ(AU)
Subject(s)
Humans , Male , Female , Gastrointestinal Stromal Tumors/complications , Gastrointestinal Stromal Tumors/diagnosis , Neurofibromatosis 1/complications , Neurofibromatosis 1/diagnosis , Immunohistochemistry/methods , Immunohistochemistry , Gastrointestinal Stromal Tumors/physiopathology , Gastrointestinal Stromal Tumors , Neurofibromatosis 1/physiopathology , Neurofibromatosis 1ABSTRACT
INTRODUCTION: gastrointestinal stromal tumors (GISTs) are specific, generally KIT (CD117)-positive, mesenchymal tumors of the digestive tract displaying KIT or PDGFRA gene mutations. Clinically, they tend to present as solitary tumors of the intestinal wall; more rarely, multiple tumors may occur in one or more organs. OBJECTIVE: to review the morphological, immunohistochemical and molecular features of multiple, non-metastatic forms of GIST. SOURCES: review of the literature on Medline, and authors own experience. CONCLUSIONS: multiples GISTs may occur in three different contexts: as spontaneous lesions (in both adults and children); due to familial GIST syndrome (autosomal dominant inheritance); or in association with specific syndromes (e.g. Carney s triad, Carney-Stratakis syndrome, type I neurofibromatosis). Outside these contexts, the existence of multiple GISTs is deemed to be the result of tumor metastasis, and therefore indicative of advanced-stage disease. Clinicians need to be aware of these variants, whose prognosis and treatment differ.
Subject(s)
Gastrointestinal Stromal Tumors , Adult , Child , Gastrointestinal Stromal Tumors/genetics , Gastrointestinal Stromal Tumors/pathology , HumansSubject(s)
Angiodysplasia/complications , Angiodysplasia/drug therapy , Angiogenesis Inhibitors/therapeutic use , Gastrointestinal Hemorrhage/etiology , Hypertension, Portal/complications , Thalidomide/therapeutic use , Compassionate Use Trials , Female , Humans , Ileum/pathology , Middle Aged , Protein-Losing Enteropathies/complicationsABSTRACT
AIM: Doppler-ultrasound assessment of the splanchnic hemodynamic effects of intravenous somatostatin and octreotide administration. MATERIAL AND METHOD: Forty-five cirrhotic patients with esophageal varices were randomized to receive 1-hour intravenous somatostatin (SOM, 250 mg), octreotide (OCT, 50 mg), or placebo (PLA). In baseline and at 15, 30, 45 and 60 minutes of infusion, mean velocity, congestion index, flow volume and diameter of the portal vein, as well as the superior mesenteric artery resistivity index, were measured. Plasma bradykinine and vasoactive intestinal peptide (VIP) concentrations were also measured at baseline and at 30 and 60 minutes. RESULTS: While placebo caused no changes in any of the venous and arterial parameters, SOM and OCT caused a sustained decrease in portal vein velocity (-19.41 vs. -11.19%) and flow (-22.79 vs. -12.33%), and an increase in the congestion index (+17.5 vs. +7.5%) and resistivity index of the superior mesenteric artery (+7.18 vs. +6.16%) with respect to baseline (p < 0.05). These changes were already evident at 15 minutes and remained unchanged during the time of the study period. With respect to OCT, SOM caused a higher reduction in mean velocity and flow of the portal vein, with no significant differences for congestion index and mesenteric artery resistivity index, both increased by SOM and OCT. Plasma bradykinine and VIP concentrations remained unchanged in the three groups. CONCLUSIONS: At therapeutic doses, intravenous somatostatin and octreotide reduce portal vein velocity and flow, and increase portal vein congestion index and superior mesenteric artery resistivity index. Somatostatin causes a higher portal flow reduction than octreotide in spite of a similar splanchnic arterial effect.
Subject(s)
Gastrointestinal Agents/therapeutic use , Hemodynamics/drug effects , Hypertension, Portal/drug therapy , Liver Cirrhosis/complications , Octreotide/therapeutic use , Portal Vein/drug effects , Portal Vein/physiology , Somatostatin/therapeutic use , Splanchnic Circulation/drug effects , Ultrasonography, Doppler , Aged , Blood Flow Velocity/drug effects , Female , Humans , Hypertension, Portal/etiology , Hypertension, Portal/physiopathology , Infusions, Intravenous , Male , Middle Aged , Regional Blood Flow/drug effectsABSTRACT
Objetivo: valoración ultrasonografica Doppler del efecto hemodinámicode la administración intravenosa de somatostatina yoctreótido.Material y método: aleatorizamos a 45 cirróticos con varicesesofágicas para recibir en una hora una infusión intravenosa de somatostatina(SOM, 250 mg), octreotido (OCT, 50 mg) o placebo (PLA).Pretratamiento y a 15, 30, 45 y 60 minutos medimos velocidad media,índice de congestión, volumen de flujo y diámetro de la vena portaademás del índice de resistencia en arteria mesentérica superior.Analizamos las concentraciones séricas de bradicinina y péptido intestinalvasoactivo (VIP) en situación basal y a 30 y 60 minutos.Resultados: respecto de los valores basales tanto SOM comoOCT provocaron un descenso significativo en la velocidad (-19,41vs. -11.19%) y flujo portal (-22,79 vs. -12,33%), con aumento delíndice de congestión (+17,5 vs. +7,5%) y del índice de resistenciaarterial (+7,18 vs. +6,16%) respecto de sus valores basales (p <0,05). Estos efectos eran ya evidentes a los 15 minutos y se mantuvierondurante todo el tiempo del estudio. Los cambios inducidosen la velocidad y flujo portal eran más pronunciados con SOMque con OCT, sin diferencias en el índice de congestión o en el índicede resistencia arterial. Los niveles plasmáticos de bradicininay VIP no experimentaron cambios respecto de sus niveles pretratamientoen ninguno de los grupos.Conclusiones: a dosis terapeúticas, somatostatina y octreotidoreducen la velocidad y flujo portales, incrementando el índicede congestión y el índice de resistencia en la arteria mesentéricasuperior. La somatostatina determina una reducción del flujo venosoportal más marcada que la inducida por octreótido a pesarde un efecto similar sobre la resistencia arterial esplácnica
Aim: Doppler-ultrasound assessment of the splanchnic hemodynamiceffects of intravenous somatostatin and octreotide administration.Material and method: forty-five cirrhotic patients withesophageal varices were randomized to receive 1-hour intravenoussomatostatin (SOM, 250 mg), octreotide (OCT, 50 mg), orplacebo (PLA). In baseline and at 15, 30, 45 and 60 minutes ofinfusion, mean velocity, congestion index, flow volume and diameterof the portal vein, as well as the superior mesenteric arteryresistivity index, were measured. Plasma bradykinine and vasoactiveintestinal peptide (VIP) concentrations were also measured atbaseline and at 30 and 60 minutes.Results: while placebo caused no changes in any of the venousand arterial parameters, SOM and OCT caused a sustained decreasein portal vein velocity (-19.41 vs. 11.19%) and flow (-22.79vs. 12.33%), and an increase in the congestion index (+17.5 vs.+7.5%) and resistivity index of the superior mesenteric artery (+7.18vs. +6.16%) from baseline (p < 0.05). These changes were alreadyevident at 15 minutes and remained unchanged over the study period.With respect to OCT, SOM caused a higher reduction in meanvelocity and flow in the portal vein, with no significant differences forcongestion index and mesenteric artery resistivity index, both increasedby SOM and OCT. Plasma bradykinine and VIP concentrationsremained unchanged in the three groups.Conclusions: at therapeutic doses, intravenous somatostatinand octreotide reduce portal vein velocity and flow, and increaseportal vein congestion index and superior mesenteric artery resistivityindex. Somatostatin causes a higher portal flow reductionthan octreotide in spite of a similar splanchnic arterial effect
Subject(s)
Humans , Male , Female , Middle Aged , Aged , Gastrointestinal Agents/therapeutic use , Hemodynamics , Hypertension, Portal/drug therapy , Liver Cirrhosis/complications , Octreotide/therapeutic use , Portal Vein , Portal Vein/physiology , Somatostatin/therapeutic use , Splanchnic Circulation , Ultrasonography, Doppler , Blood Flow Velocity , Hypertension, Portal/etiology , Hypertension, Portal/physiopathology , Infusions, Intravenous , Regional Blood FlowSubject(s)
Liver Abscess, Amebic/diagnosis , Adult , Alkaline Phosphatase/blood , Amebicides/therapeutic use , Animals , Antibodies/analysis , Cecum/pathology , Cecum/surgery , Colon, Ascending/pathology , Colon, Ascending/surgery , Colonoscopy , Drainage , Female , Follow-Up Studies , Humans , Leukocytosis , Liver Abscess, Amebic/diagnostic imaging , Liver Abscess, Amebic/drug therapy , Liver Abscess, Amebic/pathology , Liver Function Tests , Paromomycin/therapeutic use , Radiography, Thoracic , Time Factors , Treatment Outcome , UltrasonographySubject(s)
Esophageal and Gastric Varices/complications , Factor VII/therapeutic use , Gastrointestinal Hemorrhage/drug therapy , Blood Coagulation Factors/metabolism , Drug Evaluation , Factor VII/adverse effects , Factor VIIa , Gastrointestinal Hemorrhage/blood , Gastrointestinal Hemorrhage/etiology , Gastrointestinal Hemorrhage/therapy , Hemostatic Techniques , Humans , Liver Failure/metabolism , Pilot Projects , Prothrombin Time , Recombinant Proteins/adverse effects , Recombinant Proteins/therapeutic use , Thrombophilia/chemically inducedABSTRACT
No disponible
Subject(s)
Humans , Esophageal and Gastric Varices/complications , Factor VII/therapeutic use , Gastrointestinal Hemorrhage/drug therapy , Blood Coagulation Factors/metabolism , Drug Evaluation , Factor VII/adverse effects , Gastrointestinal Hemorrhage/blood , Gastrointestinal Hemorrhage/etiology , Gastrointestinal Hemorrhage/therapy , Hemostatic Techniques , Hepatic Insufficiency/metabolism , Pilot Projects , Prothrombin Time , Recombinant Proteins/adverse effects , Recombinant Proteins/therapeutic use , Thrombophilia/chemically inducedSubject(s)
Cocaine-Related Disorders/complications , Pancreatitis/complications , Purpura, Thrombotic Thrombocytopenic/etiology , Acute Disease , Adult , Alanine Transaminase/blood , Amylases/blood , Aspartate Aminotransferases/blood , Glucocorticoids/therapeutic use , Humans , Male , Prednisone/therapeutic use , Purpura, Thrombotic Thrombocytopenic/blood , Purpura, Thrombotic Thrombocytopenic/drug therapy , Treatment OutcomeABSTRACT
AIMS: To report a new strategy for the detection of hepatotoxic adverse drug reactions (ADRs) in hospitalized patients improving the results obtained with other methods. DESIGN: The model is based on the identification of a single alert signal in various target clinical departments over a 12-month period. Each patient was later interviewed following a set protocol. The main results analyzed were the drugs suspected of ADR; causal relationship between suspected drugs and ADRs; ADR severity, and incidence of hepatotoxic ADR/100,000 inhabitants. SUBJECTS: Population served by a university-affiliated urban teaching hospital (519,381 inhabitants). RESULTS: The overall ratio of confirmed/suspected ADRs was high (35/80). The most commonly reported drug was amoxicillin-clavulanic acid (4 cases). With regard to causality, 2 suspected cases were classified as definite and 14 as probable. The distribution according to the severity of hepatotoxicity was 6 severe and 29 mild cases. The incidence of hepatotoxic ADRs/100,000 inhabitants as revealed by our method was much higher versus voluntary report (6.74 and 1.79, respectively). CONCLUSIONS: Our method has proven effective for improving the detection of hepatotoxic ADRs, and may be extended to other types of adverse reactions.
Subject(s)
Adverse Drug Reaction Reporting Systems , Chemical and Drug Induced Liver Injury/diagnosis , Medication Systems, Hospital/standards , Adult , Aged , Chemical and Drug Induced Liver Injury/prevention & control , Female , Hospitals, Teaching , Hospitals, Urban , Humans , Male , Middle Aged , Pilot Projects , Quality Assurance, Health Care , SpainABSTRACT
Objetivos: comunicar una nueva estrategia para la detecciónde reacciones hepatotóxicas por medicamentos que mejora los resultadosobtenidos con otros métodos utilizados.Diseño: el modelo se basa en la identificación de una señal dealerta simple en los pacientes de varios servicios diana, durante12 meses. Cada paciente fue posteriormente entrevistado siguiendoun protocolo específico. Se analizaron: los fármacos sospechososde producir hepatotoxicidad, la relación de causalidad entre elfármaco sospechoso y la hepatotoxicidad, la gravedad y la incidenciade hepatotoxicidad medicamentosa/100.000 habitantes.Pacientes: la población del área de influencia de nuestro hospital(519.381 habitantes).Resultados: se encontraron 80 sospechas de reacciones hepatotóxicasa medicamentos. La relación de reacciones confirmadas/sospechadas fue de 35/80. El fármaco imputado con mayorfrecuencia fue amoxicilina/clavulánico (4 casos). En relación algrado de imputabilidad, 2 sospechas fueron calificadas como definitivasy 14 como probables. Según la gravedad, se encontraron6 reacciones graves y 29 leves. La incidencia de reacciones hepatotóxicaspor medicamentos/100.000 habitantes (6,74) fue muchomayor que las obtenidas con otros métodos.Conclusiones: la aplicación de este método mejora la detecciónde reacciones hepatotóxicas por medicamentos y podría serempleado en otros tipos de reacciones adversas
Aims: to report a new strategy for the detection of hepatotoxicadverse drug reactions (ADRs) in hospitalized patients improvingthe results obtained with other methods.Design: the model is based on the identification of a singlealert signal in various target clinical departments over a 12-monthperiod. Each patient was later interviewed following a set protocol.The main results analyzed were the drugs suspected of ADR;causal relationship between suspected drugs and ADRs; ADRseverity, and incidence of hepatotoxic ADR/100,000 inhabitants.Subjects: population served by a university-affiliated urbanteaching hospital (519,381 inhabitants).Results: The overall ratio of confirmed/suspected ADRs washigh (35/80). The most commonly reported drug was amoxicillinclavulanicacid (4 cases). With regard to causality, 2 suspected caseswere classified as definite and 14 as probable. The distributionaccording to the severity of hepatotoxicity was 6 severe and 29mild cases. The incidence of hepatotoxic ADRs/100,000 inhabitantsas revealed by our method was much higher versus voluntaryreport (6.74 and 1.79, respectively).Conclusions: our method has proven effective for improvingthe detection of hepatotoxic ADRs, and may be extended to othertypes of adverse reactions
Subject(s)
Adult , Aged , Humans , Adverse Drug Reaction Reporting Systems , Chemical and Drug Induced Liver Injury/diagnosis , Medication Systems, Hospital/standards , Chemical and Drug Induced Liver Injury/prevention & control , Hospitals, Teaching , Hospitals, Urban , Pilot Projects , Quality Assurance, Health Care , Spain/epidemiologyABSTRACT
No disponible
Subject(s)
Male , Humans , Adenocarcinoma/secondary , Duodenal Neoplasms/secondary , Jejunal Neoplasms/secondary , Lung Neoplasms/pathologyABSTRACT
The therapeutic approach in post-ERCP bleeding depends on the severity of the episode. In most instances early bleeding is self-limited, but when it is severe enough endoscopic injection of epinephrine (EP) is the usual treatment. Nevertheless, in some cases bleeding relapses, whereas in between 5% and 10% of patients the refractoriness to endoscopic management may even be fatal and other therapeutic alternatives would be needed. Otherwise, in a small subgroup of cases the bleeding becomes massive, the vision is obscured, and the injection may be very difficult in a situation of hemodynamic instability. We here report a case of refractory post-endoscopic sphincterotomy (ES) bleeding in a patient without preexisting coagulopathy, successfully treated with a single injection of rFVII. This novel experience suggests that rFVIIa, besides its actual high costs, might be useful and safe as a second-line, noninvasive, therapeutic tool in selected cases of massive, or refractory, post-ES bleeding.