ABSTRACT
Purpose: Limited data exist regarding the safety and efficacy of bevacizumab in pediatric patients under the age of 4 years. Here, we report a large cohort of pediatric patients under 4 years of age treated with bevacizumab. Methods: The primary objective was to document adverse events with a possible relationship to bevacizumab. Patients (n = 16) were identified through retrospective chart review and harbored a variety of conditions (44 % central nervous system (CNS) tumors, 31 % vascular anomalies, 13 % neuroblastoma, 12 % other). Results: The median age was 34.3 months (range 4.9-47.3), including five patients years of age. Patients received bevacizumab for a median duration of 6.2 months, alone or with chemotherapy, and a median dose of 9.25 mg/ kg (range 7.0-11.8). Partial responses were seen in 19 % of patients, and clinical improvements were seen in 69 %. Adverse events known to be associated with bevacizumab occurred in 37 %. Outcomes observed in this population resemble those reported for bevacizumab in older pediatric patients. The overall pattern and frequency of adverse events observed was similar to those seen in reports of older pediatric patients with a variety of conditions. The highest level of efficacy observed was seen among patients with vascular malformations or with low-grade CNS tumors. Conclusions: Our results suggest that the use of bevacizumab is safe for the youngest children (AU)
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Subject(s)
Humans , Male , Female , Child, Preschool , Bevacizumab/adverse effects , Bevacizumab/therapeutic use , Neuroblastoma/complications , Neuroblastoma/drug therapy , Angiogenesis Inhibitors/adverse effects , Angiogenesis Inhibitors/analysis , Angiogenesis Inhibitors/therapeutic use , Patient Safety/standards , Treatment Outcome , Cohort Studies , Retrospective Studies , Central Nervous System/pathology , Endothelial Growth Factors/adverse effectsABSTRACT
El angiosarcoma hepático es una neoplasia de estirpe mesenquimal de baja frecuencia y difícil diagnóstico por su forma inespecífica de manifestarse clínica y radiológicamente. Tanto es así que muchos diagnósticos se obtienen mediante necropsia, no siendo posible poner de manifiesto la enfermedad durante su curso. Se asocia a diferentes agentes etiológicos, pero en la mayoría de los casos no es posible establecer una exposición concreta a ninguno de ellos. Cuando comienza a manifestarse, la evolución suele ser rápida y las opciones de tratamiento curativo son escasas. Presentamos en nuestro trabajo dos casos de angiosarcoma hepático. En el primero, el paciente sufre en principio una evolución insidiosa, presentando al fin, y de forma abrupta, un cuadro de insuficiencia hepática seguido de distrés respiratorio, falleciendo por este motivo. El diagnóstico se alcanza en la necropsia. En el segundo caso se inicia un estudio de hepatopatía en el cual se indica una PAAF. Esta es informada como hallazgos compatibles con angiosarcoma hepático. El paciente presenta en las semanas ulteriores una evolución tórpida, falleciendo en fracaso multiorgánico(AU)
Hepatic angiosarcoma is a rare primary tumor of the liver with a mesenchymal origin. Diagnosis is difficult because clinical manifestations and imaging studies are inconclusive. In many cases a diagnosis is obtained during necropsy, not being apparent during the course of disease. It is associated with several risk factors, but these contribute to explaining only a few of all reported cases. When clinical manifestations begin progression is often fast, and possibilities for curative treatment are limited. We report two cases of hepatic angiosarcoma. In the first one, our patient had an insidious initial course, and then suddenly presented with hepatic failure followed by acute respiratory distress. A diagnosis was reached during necropsy. In the second case, we initiated the study of a chronic liver disease using fine-needle aspiration biopsy, which showed findings suggestive of hepatic angiosarcoma. In the following weeks the patient started on a torpid clinic course, and died from multiple organ failure(AU)
Subject(s)
Humans , Male , Middle Aged , Hemangiosarcoma/diagnosis , Liver Neoplasms/diagnosis , /methods , Fatal Outcome , Hemangiosarcoma/physiopathology , Hemangiosarcoma/chemically inducedABSTRACT
Objetivo: evaluar la eficacia de los diferentes indicadores pronósticosde supervivencia a corto y largo plazo en pacientes concirrosis y hemorragia digestiva alta por hipertensión portal (HTP).Material y métodos: calculamos los indicadores pronósticosen una cohorte de 201 pacientes con cirrosis y hemorragia digestivapor HTP ingresados en el Hospital General Universitario deAlicante. Las variables a estudio fueron: edad, sexo, etiología de lacirrosis, hallazgos endoscópicos, episodios previos de hemorragiadigestiva por HTP. Infección por el virus de la inmunodeficienciahumana (VIH), hepatocarcinoma (HCC), infección bacteriana duranteel episodio de hemorragia digestiva y clasificación de Child-Turcotte-Pugh (CTP) y el modelo para enfermedades terminalesdel hígado (MELD score) calculados dentro de las primeras 24 horasdel inicio de la hemorragia. Los pacientes fueron seguidos almenos 6 meses hasta su muerte, trasplante hepático o final del seguimiento.Resultados: la mediana de seguimiento fue de 66,85 semanas(rango 0-432,4). La mortalidad a las 6 semanas, 3 meses, 12meses y 36 meses fue de 22,9, 24,9, 34,3 39,8%, respectivamente.La edad ≥ 65 años, la presencia de HCC, una clasificaciónde CTP ≥ 10 y un MELD score ≥ 18 fueron las variables asociadasa la mortalidad en el estudio multivariante. La precisión delMELD score como predictor de mortalidad a las 6 semanas, 3meses, 12 meses y 36 meses fue superior a la de la clasificaciónde CTP (valor c-estadístico: 6 semanas MELD 0,804, CTP 0,762;3 meses MELD 0,794, CTP 0,760; 12 meses MELD 0,766, CTP0,741; 36 meses MELD 0,737, CTP 0,717).Conclusión: el MELD score y la clasificación de CTP, juntocon la edad y la presencia de HCC, son marcadores útiles en lavaloración pronóstica de supervivencia a corto y largo plazo de lospaciente con cirrosis y hemorragia digestiva por HTP(AU)
Objective: to evaluate the efficacy of various indicators in predicting short- and long-term survival in patients with cirrhosis and acute variceal bleeding. Material and methods: prognostic indicators were calculated for a cohort of 201 cirrhotic patients with acute variceal bleeding hospitalized in our center, a third-level teaching hospital. The studied variables were: age, sex, etiology of cirrhosis, endoscopic findings, previous variceal bleeding episodes, human immunodeficiency virus (HIV) infection, hepatocellular carcinoma (HCC), infection during episode, and Child-Turcotte-Pugh (CTP) and Model for End-stage Liver Disease (MELD) scores within 24 hours of bleeding onset. Patients were followed up for at least 6 months until death, liver transplantation, or end of observation. Results: median follow-up was 66.85 weeks (range 0-432.4). The 6-week, 3-month, 12-month and 36-month mortality rates were 22.9, 24.9, 34.3, and 39.8%, respectively. Age ≥ 65 years, presence of HCC, CTP score ≥ 10, and MELD score ≥ 18 were the variables associated with mortality in the multivariate analysis. The accuracy of MELD scores as predictors of 6-week, 3-month, 12-month, and 36-month mortality was better than that of CTP scores (c-statistics: 6 week MELD 0.804, CTP 0.762; 3-month MELD 0.794, CTP 0.760; 12-month MELD 0.766, CTP 0.741; 36 month MELD 0.737, CTP 0.717). Conclusion: MELD and CTP scores together with age and a diagnosis of hepatocellular carcinoma are useful indicators to assess the short- and long-term prognosis of patients with acute variceal bleeding(AU)
Subject(s)
Humans , Male , Female , Esophageal and Gastric Varices/etiology , Esophageal and Gastric Varices/mortality , Gastrointestinal Hemorrhage/etiology , Gastrointestinal Hemorrhage/mortality , Liver Cirrhosis/complications , Liver Cirrhosis/mortality , Acute Disease/epidemiology , Prognosis , Retrospective Studies , Survival Rate , Time FactorsABSTRACT
La linitis plástica rectal es una entidad poco frecuente con gran dificultad en su diagnóstico, debido a la falta de lesión mucosa en la endoscopia, la baja rentabilidad de las biopsias y los hallazgos inespecíficos en la radiología baritada y la tomografía computarizada. La ultrasonografía endoscópica rectal ha supuesto una revelación en el diagnóstico diferencial de las lesiones estenosantes de recto, entre ellas la linitis plástica, y ha permitido el diagnóstico de esta entidad incluso en pacientes con biopsias negativas
Linitis plastica of the rectum is an uncommon entity that is difficult to diagnose due to the lack of mucosal lesions on endoscopy, the low diagnostic yield of biopsy and non-specific findings of barium radiology and computerized tomography. Rectal endoscopic ultrasonography has had a radical impact on the differential diagnosis of stenosing lesions of the rectum, among them linitis plastica, allowing diagnosis of this lesion even in patients with negative results of biopsy
