RESUMEN
The reduction of the startle response to an auditory stimulus caused by the presentation of another stimulus of lower intensity closely preceding it, a phenomenon known as prepulse inhibition (PPI), can be modulated by changes in dopaminergic activity. Schmajuk, Larrauri, De la Casa, and Levin (2009) demonstrated that this dopaminergic modulation of PPI in rats can be influenced by manipulating the experimental context, specifically by introducing changes in the ambient lighting condition that include novel elements. In this paper we analyze the effects of introducing changes in context illumination on PPI in male rats (Experiment 1) and humans (Experiment 2). The results with rats showed a reduction of PPI when the illumination condition switched from dark to light, but not from light to dark. In the experiment with human participants the reduction of PPI occurred for both changes in illumination conditions. The animal experiment results are interpreted in terms of competing exploratory behavior that appear when the context is illuminated after the dark-light transition; while in the case of human participants a perceptual and/or attentional mechanism after both illumination transitions is proposed, which may result in a reduced processing of the prepulse and subsequent lower PPI.
Asunto(s)
Ambiente , Reflejo de Sobresalto/fisiología , Estimulación Acústica , Análisis de Varianza , Animales , Oscuridad , Electromiografía , Conducta Exploratoria , Femenino , Humanos , Luz , Masculino , Estimulación Luminosa , Ratas , Ratas Wistar , Especificidad de la Especie , Adulto JovenRESUMEN
Prepulse inhibition (PPI) refers to the process wherein startle responses to salient stimuli (e.g., startling sound pulses) are attenuated by the presentation of another stimulus (e.g., a brief pre-pulse) immediately before the startling stimulus. Accordingly, deficits in PPI reflect atypical sensorimotor gating that is linked to neurobehavioral systems underlying responsivity to emotionally evocative cues. Little is known about the effects of changes in visual contextual information in PPI among humans. In this study, the effects of introducing unexpected changes in the visual scenes presented on a computer monitor on the human auditory startle response and PPI were assessed in young adults. Based on our animal data showing that unexpected transitions from a dark to a light environment reduce the startle response and PPI in rats after the illumination transition, it was hypothesized that novel changes in visual scenes would produce similar effects in humans. Results show that PPI decreased when elements were added to or removed from visual scenes, and that this effect declined after repeated presentations of the modified scene, supporting the interpretation that the PPI reduction was due to novel information being processed. These findings are the first to demonstrate that novel visual stimuli can impair sensorimotor gating of auditory stimuli in humans.
Asunto(s)
Estimulación Luminosa/métodos , Reflejo de Sobresalto/fisiología , Filtrado Sensorial/fisiología , Percepción Visual/fisiología , Estimulación Acústica , Adolescente , Atención/fisiología , Femenino , Respuesta Galvánica de la Piel/fisiología , Frecuencia Cardíaca/fisiología , Humanos , Masculino , Adulto JovenRESUMEN
Surgery for chronic inflammatory bowel disease (IBD) is increasingly often necessary in children. This study aimed at assessing the results of these operations in order to facilitate adequate preoperative counseling. We reviewed patients treated from 1992 to 2009. The operations, complications and functional outcome were recorded. For those with preserved rectal defecation, continence (Koivusalo score) and quality of life (standardized questionnaire) were assessed in the long term. Eighty five of 192 patients had Crohn disease (CD), 107 of 192 had ulcerative colitis (UC), and 3 of 192 had indeterminate colitis (IC). 12 of 85 CD patients (15%) aged 14 (12-19) years required 13 resections, 1 stricturoplasty, 1 transplantation and 6 other operations including 3 permanent enterostomies for anorectal involvement. Removal of the involved bowel led to significant improvement of nutritional status, growth and quality of life. The transplanted patient had a striking recovery but eventually died 1 year later of unrelated complications. 29 of 107 UC patients (26%) aged 11 (2-15) years required 87 operations. Nine had emergency colectomy for toxic megacolon (3, one death) or severe hemorrhage (6). 28 had restorative proctocolectomy and ileoanostomy (RPCIA) without (16) or with (12) J-pouch under protective ileostomy. Complications were frequent (40%). Permanent ileostomy was required in five children (17%). Twelve months postoperatively, RPCIA patients had 6.5 (2-13) stools/day; all were continent during daytime, and 25% have nocturnal leaks. Mean Koivusalo score (5-12) was 8.8 ± 2. Quality of life was good in all. All attended normal school and 7 the university, 4 work and 60% of those older than 18 years have sexual partners. Three of 107 children treated as UC with RPCIA had ultimately IC (3%) and were permanently diverted. The nature of IBD involves frustrating surgery. However, it may change life for CD patients and provide a reasonably good quality of life for UC after the first year. Pediatric surgeons should be able to provide adequate preoperative counseling to patients and families.
Asunto(s)
Procedimientos Quirúrgicos del Sistema Digestivo , Enfermedades Inflamatorias del Intestino/cirugía , Adolescente , Biopsia , Niño , Enfermedad Crónica , Consejo , Femenino , Humanos , Ileostomía , Enfermedades Inflamatorias del Intestino/mortalidad , Masculino , Complicaciones Posoperatorias/epidemiología , Calidad de Vida , Encuestas y Cuestionarios , Resultado del Tratamiento , Adulto JovenRESUMEN
BACKGROUND: Gastric adenocarcinoma (GA) has been considered a disease of elderly age and has been rarely reported in patients younger than 35 years of age. The aim of thisΩ demographic, clinicopathological and prognosis of gastric cancer in young patients and to compare their features with the behavior in elder adults. METHODS: Between 1993 and 2008, 1536 patients with GA were enrolled in a retrospective database. Clinical and pathologic features of thirty patients aged 35 years or less (young group) were compared with those of 458 aged 75 years or more (elder group). RESULTS: Mean patient age was 31 and 80-years old in the young and elder groups, respectively, with a predominance of females in the last group (61%). Lauren diffuse type carcinoma was more frequent in people younger than 35 years (70%) than in older patients (17.4%). Main symptoms were dyspepsia (40%) and hemorrhage (20%). The most common T stage in young and elder patients was T3 (52.9% and 56.7% respectively). Surgical resection was performed in 68% of cases and the rest received only systemic chemotherapy. CONCLUSION: Gastric adenocarcinoma is rare in young patients and most cases presented at advanced clinical stage similar to elderly patients, so the prognosis in both age groups is poor. For this reason is important to be aware of alarm symptoms and risk factors in order to perform an early endoscopic diagnosis and a treatment with curative intent.
Asunto(s)
Adenocarcinoma/epidemiología , Neoplasias Gástricas/epidemiología , Adenocarcinoma/patología , Adenocarcinoma/terapia , Adulto , Factores de Edad , Anciano , Anciano de 80 o más Años , Terapia Combinada , Bases de Datos Factuales , Femenino , Humanos , Masculino , México/epidemiología , Persona de Mediana Edad , Estudios Retrospectivos , Neoplasias Gástricas/patología , Neoplasias Gástricas/terapia , Análisis de Supervivencia , Adulto JovenRESUMEN
AIM: The treatment of Ulcerative Colitis (UC) unresponsive to current medical treatment involves total proctocolectomy and ileanostomy. The aim of this study was to assess the long term results in such patients. MATERIAL AND METHODS: We reviewed children treated from 1992 to 2008. The operations, complications and functional outcome were recorded. For those with preserved rectal defecation, continence (Holschneider score) and quality of life (standardized phone questionnaire) were assessed in the long term. RESULTS: 104 patients had Ulcerative Colitis (UC) and 3 undetermined colitis (excluded from the study). 26/104 UC patients (26%) aged 10.7 +/- 4.1 years required 82 operations. Eight had emergency colectomy for toxic megacolon (3, one death) or severe hemorrhage (5). 25 had restorative proctocolectomy and ileoanostomy (RPCIA) without (16) or with (9) J-pouch under protective ileostomy. Complications were frequent (40%). Permanent ileostomy was required in 5 children (20%). Twelve months postoperatively, RPCIA patients had 6.3 +/- 3 stools/day, all were continent during daytime and 25% have nocturnal leaks. Mean Holschneider score (0-12) was 8.8 +/- 2. Quality of life was good in all with normal school (all) or university attendance (7), 4 work and 60% of those older than 18 have sexual partners. CONCLUSIONS: The nature of UC involves risky and frustrating surgery. However, it may provide a reasonably good quality of life for UC patients after the first year. Pediatric surgeons should be able to provide adequate preoperative counseling to patients and families.
Asunto(s)
Canal Anal/cirugía , Colitis Ulcerosa/cirugía , Ileostomía , Proctocolectomía Restauradora , Niño , Humanos , Factores de Tiempo , Resultado del TratamientoRESUMEN
Objetivo. La colitis ulcerosa (CU) refractaria al tratamiento médico actual requiere una proctocolectomía total con ileoanostomía. El objetivo de nuestro estudio es analizar el resultado a largo plazo en estos pacientes. Material y métodos. Revisamos los niños operados por CU entre1992 y 2008 con atención a las complicaciones y el resultado a largo plazo. En aquellos en los que se restableció la defecación se evaluaron la continencia (escala de Holschneider modificada) y la calidad de vida (encuesta telefónica estandarizada)Resultados. De 107 pacientes diagnosticados de CU, 3 tenían colitis indeterminada y se excluyeron, 26/104 se operaron (26%) con una edad media de 10,7±4.1 años. Ocho necesitaron una colectomía de urgencia (3 por megacolon tóxico y 5 por hemorragia grave). A 25 se les realizó una proctocolectomía e ileoanostomía (PCIA), con (AU)
Aim. The treatment of Ulcerative Colitis (UC) unresponsive to current medical treatment involves total proctocolectomy and ileanostomy. The aim of this study was to assess the long term results in such patients. Material and methods. We reviewed children treated from 1992to 2008. The operations, complications and functional outcome were recorded. For those with preserved rectal defecation, continence(Holschneider score) and quality of life (standardized phone questionnaire) were assessed in the long term. Results. 104 patients had Ulcerative Colitis (UC) and 3 undetermined colitis (excluded from the study). 26/104 UC patients (26%) aged10.7±4.1 years required 82 operations. Eight had emergency colectomy for toxic megacolon (3, one death) or severe hemorrhage (AU)
Asunto(s)
Humanos , Masculino , Femenino , Niño , Colitis Ulcerosa/cirugía , Proctocolectomía Restauradora/métodos , Ileostomía/métodos , Calidad de VidaRESUMEN
BACKGROUND: The purpose of this study was to identify clinical and pathological parameters to improve prediction of disease-free survival (DFS) and overall survival (OS) in patients treated with neoadjuvant chemoradiotherapy for rectal cancer. METHODS: Between July 1995 and May 2007, 148 patients with primary rectal adenocarcinoma received neoadjuvant chemoradiotherapy followed by mesorectal excision. Preoperative treatment included various protocols, UFT and leucovorin (28%) and oxaliplatin-based chemotherapy (72%). Clinical and pathological variables were evaluated in relation to patient outcomes. RESULTS: Thirteen percent of patients achieved a complete pathologic response. No response or minimal response as defined by Dworak (Tumor Regression Grade 0/1) was observed in 30 patients (20%). At a median follow-up of 37 months, the 3-year DFS and OS were 64% and 83%, respectively. Pre-treatment serum carcinoembryonic antigen (CEA) level = 2.5 ng/ml was associated with higher DFS (74 vs. 53%; p = 0.018), higher complete pathologic responses (21 vs. 9%; p = 0.05), and less recurrences (24 vs. 44%; p = 0.014). CONCLUSION: The data suggest that a CEA level = 2.5 ng/ml might be a predictor not only of tumor response, as has been suggested before, but also of DFS. This finding could be useful in the future to predict individual risk and to develop more aggressive or alternative strategies.
Asunto(s)
Antígeno Carcinoembrionario/sangre , Terapia Neoadyuvante , Neoplasias del Recto/diagnóstico , Neoplasias del Recto/terapia , Adulto , Anciano , Anciano de 80 o más Años , Supervivencia sin Enfermedad , Femenino , Humanos , Masculino , Persona de Mediana Edad , Análisis Multivariante , Pronóstico , Neoplasias del Recto/sangre , Neoplasias del Recto/patología , Recurrencia , Resultado del TratamientoRESUMEN
We investigated the role of dopaminergic mechanisms in the attenuation of the acoustic startle response and prepulse inhibition (PPI) in rats by the introduction of unexpected changes in environment illumination. Experiment 1 showed that Dark-to-Light transitions robustly reduce startle responses and PPI. Experiment 2 showed that this phenomenon habituates across repeated testing sessions and reappears after an interval without testing. Experiment 3 demonstrated that haloperidol blocks the startle and PPI-reducing effect of the Dark-to-Light transition. We show how a computational model of acoustic startle response and prepulse inhibition can be extended to incorporate the empirical effects demonstrated in this study. We conclude that sensory gating as measured by prepulse inhibition is markedly attenuated in situations where novel stimuli are introduced during a test session and that dopaminergic systems may be involved in the dynamic changes evoked by the onset of illumination.
Asunto(s)
Parpadeo/efectos de los fármacos , Dopamina/fisiología , Haloperidol/farmacología , Reflejo de Sobresalto/fisiología , Estimulación Acústica/métodos , Animales , Parpadeo/fisiología , Dopamina/metabolismo , Antagonistas de Dopamina/farmacología , Femenino , Ratas , Ratas Sprague-Dawley , Reflejo de Sobresalto/efectos de los fármacos , Literatura de Revisión como AsuntoRESUMEN
BACKGROUND AND AIM: Eosinophilic esophagitis can be associated with a wide range of endoscopic patterns. The aim of the present case series report is to describe and classify endoscopic appearances before and after corticoid therapy in relation to histopathology and manometry. PATIENTS AND METHODS: In 30 patients (m : f, 27 : 3; mean age 36.2 years) with eosinophilic esophagitis, endoscopic findings were prospectively classified according to luminal diameter and mucosal pattern. Manometric and bioptic histopathologic findings were also recorded. Endoscopy was repeated following a 3-month course of steroid therapy. RESULTS: In total, 20 % of patients showed a concentric esophageal stricture, and in 57 % simultaneous contraction rings were visible. Mucosal alterations consisted of granular mucosa (20 %), longitudinal furrows (33 %) and transversal undulations (3 %). Lower esophageal sphincter dysfunction and distal esophageal dysfunctional manometry were seen in 73 % and 57 % of cases, respectively. Following steroids, the esophagus showed a normal caliber in 97 % of patients, and 63 % of patients had normal mucosa. CONCLUSIONS: The most frequent findings were narrowing of the esophageal lumen, which returned to normal following steroid treatment to a larger extent than mucosal alterations.
Asunto(s)
Androstadienos/administración & dosificación , Esofagitis/diagnóstico , Glucocorticoides/administración & dosificación , Administración Tópica , Adulto , Eosinófilos , Esofagitis/tratamiento farmacológico , Esofagitis/fisiopatología , Esofagoscopía , Femenino , Fluticasona , Humanos , Masculino , Manometría , Estudios ProspectivosRESUMEN
BACKGROUND: Lafora disease (LD; progressive myoclonus epilepsy type 2; EPM2) is an autosomal recessive disorder caused by mutations in the EPM2A and EPM2B genes. LD is characterized by the presence of strongly PAS-positive intracellular inclusions (Lafora bodies) in several tissues. Glycogen storage disease type IV (GSD-IV; Andersen disease) is an autosomal recessive disorder characterized by cirrhosis leading to severe liver failure. GSD-IV has been associated with mutations in the glycogen branching enzyme gene (GBE). Histopathologic changes of the liver in both diseases show an identical appearance, although cirrhosis has never been described in patients with LD. We report a LD family in which the proband presented severe liver failure at onset of the disease. METHODS: Clinical histories, physical and neurologic examination, laboratory tests, EEGs, MRI of the brain, and liver or axillary skin biopsies were performed in the two affected siblings. The diagnosis was confirmed by molecular genetic analysis of the EPM2A, EPM2B, and GBE genes and loci. RESULTS: During the first decade of life, abnormalities in liver function tests were detected in the two affected siblings. The proband's liver dysfunction was severe enough to require liver transplantation. Subsequently, both sibs developed LD. Mutation analysis of EPM2A revealed a homozygous Arg241stop mutation in both patients. CONCLUSIONS: This is the first description of severe hepatic dysfunction as the initial clinical manifestation of LD. The phenotypic differences between the two affected siblings suggest that modifier genes must condition clinical expression of the disease outside the CNS.
Asunto(s)
Enfermedad de Lafora/diagnóstico , Fallo Hepático/etiología , Proteínas Tirosina Fosfatasas/genética , Enzima Ramificadora de 1,4-alfa-Glucano/genética , Biopsia , Encéfalo/patología , Proteínas Portadoras/genética , Niño , Codón sin Sentido , Diagnóstico Diferencial , Progresión de la Enfermedad , Enanismo/etiología , Electroencefalografía , Exones/genética , Enfermedad del Almacenamiento de Glucógeno Tipo IV/diagnóstico , Humanos , Lactante , Enfermedad de Lafora/complicaciones , Enfermedad de Lafora/genética , Hígado/patología , Cirrosis Hepática/etiología , Fallo Hepático/cirugía , Trasplante de Hígado , Imagen por Resonancia Magnética , Masculino , Repeticiones de Microsatélite , Mutación Missense , Linaje , Reacción del Ácido Peryódico de Schiff , Fenotipo , Mutación Puntual , Polimorfismo Conformacional Retorcido-Simple , Proteínas Tirosina Fosfatasas no Receptoras , Piel/patología , España , Ubiquitina-Proteína LigasasRESUMEN
Schmajuk and Larrauri [Schmajuk NA, Larrauri JA. Neural network model of prepulse inhibition. Behav Neurosci 2005;119:1546-62.] introduced a real-time model of acoustic startle, prepulse inhibition (PPI) and facilitation (PPF) in animals and humans. The model assumes that (1) positive values of changes in noise level activate an excitatory and a facilitatory pathway, and (2) absolute values of changes in noise level activate an inhibitory pathway. The model describes many known properties of the phenomena and the effect of brain lesions on startle, PPI, and PPF. The purpose of the present study is to (a) establish the magnitude of startle and PPI as a function of pulse, prepulse, and background intensity, and (b) test the model predictions regarding an inverted-U function that relates startle to the intensity of the background noise.
Asunto(s)
Simulación por Computador , Modelos Biológicos , Inhibición Neural/fisiología , Ruido , Reflejo Acústico/fisiología , Reflejo de Sobresalto/fisiología , Estimulación Acústica/métodos , Animales , Conducta Animal , Relación Dosis-Respuesta en la Radiación , Femenino , Habituación Psicofisiológica , Masculino , Ratones , Ratas , Ratas Sprague-Dawley , Reflejo Acústico/efectos de la radiaciónRESUMEN
Different authors have investigated the immunohistochemical expression of some proteins in the adenocarcinoma of the stomach, including cell cycle regulators proteins like p53 and Bcl-2; growth factors (c-erb-B2 and EPO-R); angiogenesis-related markers such as COX-2 and cellular adhesion molecules (beta-catenin and E-cadherin). While these proteins have been studied in gastric adenocarcinoma, their immunophenotyping in non tumoral gastric mucous membrane remains unexplored. In the present study, we investigated the expression, function and behavior of these proteins in normal gastric mucous membrane to contribute to gain further knowledge on the significance of their loss or overexpression in malignant gastric tumors.
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Mucosa Gástrica/metabolismo , Proteínas/metabolismo , Biomarcadores/metabolismo , Cadherinas/metabolismo , Ciclooxigenasa 2/metabolismo , Femenino , Mucosa Gástrica/anatomía & histología , Humanos , Inmunohistoquímica , Masculino , Proteínas de la Membrana/metabolismo , Persona de Mediana Edad , Proteínas Proto-Oncogénicas c-bcl-2/metabolismo , Receptor ErbB-2/metabolismo , Receptores de Eritropoyetina/metabolismo , Proteína p53 Supresora de Tumor/metabolismo , beta Catenina/metabolismoRESUMEN
Still, very little is known about the precise pathogenetic mechanisms, the triggering events and in particular, the evolution and treatment of nonalcoholic steatohepatitis (NASH). It is part of the broad spectrum of nonalcoholic fatty liver diseases (NAFLDs). Mainly, it has been reported as a benign disease, associated with metabolic disorders commonly occurrence en the general population. Nevertheless, the syndrome can lead to cirrhosis, liver failure or hepatocellular carcinoma, requiring liver transplantation. We present one patient with diagnosis of NASH, who was treated initially for overweight, HTA and hyperlipaemia with incompleted response and who showed a quickly progress to cirrhosis but no cause of liver decompensated disease could be identified. Currently she is at end-stage waiting a liver transplantation. Controlled and multicentric studies with the same definition of NASH and the study end-points are needed, and will provide information about diagnosis features and novel therapies to early management of the disease.
Asunto(s)
Hígado Graso/complicaciones , Adulto , Progresión de la Enfermedad , Hígado Graso/patología , Femenino , HumanosRESUMEN
Hasta ahora poco se sabe acerca de la patogenia, los desencadenantes y en particular de la evolución y terapia de la esteatohepatitis no alcohólica (EHNA). Incluída en el amplio espectro de enfermedades del hígado graso no alcohólicas. Se ha considerado una enfermedad mayoritariamente benigna y asociada a alteraciones metabólicas muy prevalentes en la población general. Sin embargo no es raro que progrese a cirrosis, insuficiencia hepática o hepatocarcinoma que precise transplante hepático. Presentamos el caso de una paciente que diagnosticamos de EHNA, al inicio con respuesta parcial y tardía al tratamiento para el sobrepeso, la HTA y la hiperlipidemia, pero con una evolución rápida a cirrosis hepática sin identificarse ningún factor desencadenante. Actualmente es candidata a transplante hepático.Son necesarios estudios multicéntricos y controlados, con la misma definición de EHNA y objetivos comunes que aportarán información sobre datos diagnósticos y nuevas terapias para el manejo precoz de la enfermedad
Still, very little is known about the precise pathogenetic mechanisms, the triggering events and in particular, the evolution and treatment of non-alcoholic steatohepatitis (NASH). It is part of the broad spectrum of nonalcoholic fatty liver diseases ( NAFLDs). Mainly, it has been reported as a benign disease, asociated with metabolic disorders commonly occurrence en the general population. Nevertheless, the syndrome can lead to cirrhosis, liver failure or hepatocellular carcinoma, requiring liver transplantation. We present one pacient with diagnosis of NASH, who was treated initially for overweight, HTA and hyperlipaemia with incompleted response and who showed a quickly progress to cirrosis but no cause of liver descompensated disease could be identified. Currently she is at end-stage waitting a liver transplantation. Controlled and multicentric studies with the same definition of NASH and the study end-points are needed, and will provide informationabout diagnosis features and novel therapies to early management of the disease
Asunto(s)
Femenino , Adulto , Humanos , Hígado Graso/complicaciones , Cirrosis Hepática/etiología , Obesidad/complicaciones , Hipertensión/complicaciones , Hiperlipidemias/complicacionesRESUMEN
RATIONALE: It has been suggested that, in classical conditioning, dopamine (DA) codes for (a) attention to the conditioned stimulus (CS) or (b) the intensity of the unconditioned stimulus. OBJECTIVES: To clarify the role of DA in pre-clinical classical conditioning studies. METHODS: An existing model of classical conditioning presented by Schmajuk, Lam, and Gray (J Exp Psychol Anim Behav Process 22:321-349, 1996) suggests that DA cells in the ventral midbrain area code for the attentionally modulated internal representation of the CS. It is assumed that this representation is increased by dopaminergic agonists and decreased by dopaminergic antagonists. Computer simulations with the model describe the effect of nicotine and haloperidol on latent inhibition. RESULTS: Simulations replicate experimental results demonstrating that both nicotine and haloperidol affect latent inhibition when administered during the pre-exposure phase. In addition, the model reproduces data showing that administration of nicotine or haloperidol results in the impairment or facilitation of latent inhibition depending on the duration of CS or the number of CSs. CONCLUSIONS: The model demonstrates that pre-clinical experimental results, including cell activity and pharmacological data, are consistent with an attentional role for DA in classical conditioning.
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Agonistas de Dopamina/administración & dosificación , Evaluación Preclínica de Medicamentos/métodos , Inhibición Psicológica , Simulación por Computador , Condicionamiento Clásico/efectos de los fármacos , Condicionamiento Clásico/fisiología , Agonistas de Dopamina/farmacocinética , Esquema de Medicación , Evaluación Preclínica de Medicamentos/tendencias , Haloperidol/farmacología , Humanos , Modelos Neurológicos , Nicotina/farmacología , Psicofarmacología/métodos , Psicofarmacología/tendencias , Factores de TiempoRESUMEN
UNLABELLED: The hepatic multicentric haemangioma is defined by its extension, affecting all the mass of the liver. The high mortality associated with it is mostly related with the complications produced by its enormous size (haemodynamic, platelet trapping, spontaneous rupture and bleeding). There is a general belief that is a benign tumor with possibility of spontaneous regression and cure. AIM: Retrospective analysis of our recent cases of MHH with the purpose of: 1 degrees) To show the evolution and results. 2 degrees) To realize if the "benign character" of the tumor is real or if some cases may be considered as malignant tumors. MATERIAL AND METHODS: 10 cases of MHH treated in the last 10 years. In 9 the age of presentation was less than 6 months and one patient was diagnosed at 3 and half years. The diagnosis was confirmed by image techniques in 7 cases and by biopsy in 3. In 7 patients extrahepatic vascular lesions were associated prior to the treatment. Methylprednisolone was given to all the cases and alpha-2-interferon was administered to the patients that not responded to the steroids. Vincristine was added to 2 patients. In two cases the hepatic artery embolization was tried and one patient had a liver transplant. RESULTS: Four children had at least one episode of congestive cardiac insufficiency, two patients suffered a consumption coagulopathy (Kasabach Merrit syndrome), and one presented acute hepatic failure. In six children it has been complete regression of the tumor, one more is still under treatment and three died. The dead were produced by the malignant behavior of the tumor in one case (tumoral rupture of a MHH recurrence in the transplanted liver), and possibly in other (intracranial haemorrhage and hepatic failure in a liver transplantation candidate without demonstrated extrahepatic extension in the previous studies, but with multiorgan dissemination at autopsy. In both cases it was impossible to discover signs of histologic or biologic malignancy neither in the primitive lesion nor in the metastasis. CONCLUSIONS: 1a) The regression of the MHH, spontaneous or induced by the treatment is frequent. 2a) Some cases of MHH are aggressive and develop local recurrences and distant metastasis. 3a) The discrimination between MHH of "benign" or "malignant" behaviour is not possible. 4a) Despite of the unpredictable biological conduct of the tumor, the liver transplantation must be considered as an option in the symptomatic cases that not respond to the conventional treatment.
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Hemangioendotelioma/patología , Neoplasias Hepáticas/patología , Preescolar , Hemangioendotelioma/terapia , Humanos , Lactante , Neoplasias Hepáticas/terapia , Estudios RetrospectivosRESUMEN
AIM: To analyze the outcome of children with intestinal failure (IF) included as candidates for intestinal transplantation (IT). Patients, Eight out of 23 children with IF assessed since July 1997 met criteria for IT and were included on the waiting list. The causes of IT were Short Bowel Syndrome (SBS) in 6 and Microvillus Inclusion Disease (MID) in 2. The indication of IT were end stage liver disease (ESLD) in 5 (related to total parenteral nutrition administration, TPN), progressive hepatic fibrosis in 2 and loss of venous access in 1. The patients with ESLD were included for combined liver-small bowel transplantation (LSBT) and the remaining for isolated intestinal transplantation (IIT). RESULTS: Two children died waiting for LSBT, 4 patients are on the waiting list, 2 for LSBT and 2 for IIT (length of stay: 4-11 months). Two children were transplanted, one with IIT in a 2.5 years old boy with MID, and one with LSBT in a 22 months girl with SBS and ESLD. Both patients recovered intestinal function after transplantation and are a live (follow-up of 19 and 10 months respectively). The LSBT's patient is under treatment for postransplant lymphoproliferative disease (PTLD). CONCLUSIONS: The lack of suitable donors for the small children candidate to IT explains the long period of stay on the waiting list and the high pretransplant mortality. Two strategies are possible; early referral of children with IF to a transplant center and surgical techniques like ex vivi-hepatic reductions of the LSB graft.
Asunto(s)
Intestinos/trasplante , Pediatría/organización & administración , Niño , Servicios de Salud del Niño/organización & administración , Humanos , Selección de Paciente , España , Trasplantes , Resultado del Tratamiento , Listas de EsperaRESUMEN
Objetivos. 1°. Analizar la evolución de los niños con fallo intestinal (FI) valorados y aceptados como candidatos a trasplante intestinal (TI). 2°. Discutir estrategias para evitar la mortalidad pretrasplante en lista de espera de los niños candidatos al procedimiento. Material y métodos. Desde julio de 1997 se han evaluado 23 niños con FI como potenciales candidatos a TI; ocho fueron incluidos en lista para trasplante y constituyen el material de este estudio. Las causas de FI fueron síndrome de intestino corto (SIC) en seis, y enfermedad de inclusión a microvilli (EIM) en dos. El FI se asoció en cinco niños a enfermedad hepática terminal y fueron incluidos, por tanto, para trasplante combinado de hígado-intestino (THI); los tres restantes fueron incluidos para trasplante de intestino aislado (TIA), indicado por pérdida de accesos venosos profundos en uno; y por fibrosis hepática progresiva en dos, con el objeto de evitar posteriormente un eventual THI; uno de ellos asociaba además pérdida importante de acceos venosos profundos. Al incluirse en lista de trasplante, la media de edad y peso fue de 11 meses y 8,1 kg, respectivamente (rangos de 4 meses-25 meses y 4 kg-16 kg, respectivamente), Resultados. Dos niños candidatos ambos a THI fallecieron sin poderse trasplantar, tras un tiempo en lista de espera para trasplante de 5 y 14 meses, respectivamente. Cuatro están actualmente activos en lista, dos para THI y dos para TIA; el tiempo de espera oscila entre 2 y 10 meses. Otros dos han sido trasplantados, uno con TIA en un caso de displasia microvellositaria (variante de la EIM) y otro con THI en una niña con SIC asociado a cirrosis; el tiempo de evolución postrasplante es de 18 y 8 meses, respectivamente. Tras el TI, ambos consiguieron una autonomía digestiva completa, quedando libres de NP. Conclusiones. El tiempo de espera en lista de los niños candidatos a TI es prolongado, y se debe a la escasez de donantes adecuados. Dos estrategias permiten disminuir la mortalidad pretrasplante: 1ª. Referencia precoz del niño con FI a un centro en donde se ofrezca la posibilidad de TI. 2ª. Aplicación de técnicas quirúrgicas que permiten usar donantes de peso varias veces superior al del receptor (utilizadas en nuestro caso de THI y discutidas en la comunicación) y que incrementan, por tanto, las posibilidades de acceso al trasplante (AU)
Asunto(s)
Niño , Humanos , España , Listas de Espera , Selección de Paciente , Trasplantes , Resultado del Tratamiento , Pediatría , Intestinos , Servicios de Salud del NiñoRESUMEN
To study the cost-benefit of the histological examination of tonsilar samples, we evaluated 567 cases (547 routine and 20 nonroutine cases) of patients under 14 years of age, operated between 1st January 1996 and 30th November 2000. There were 2 routine cases (0.3%), and 6 nonroutine cases (30%) with a diagnosis different to follicular hyperplasia. In this way, our clinical preoperative sensitivity was 75% and specificity 97%. The average cost per case at our Centro to study the tonsilar samples was 30$. We conclude that the histological examination of tonsilar specimens is economically worth only in nonroutine cases, although additional factors (e.g. training of residents of Pathology and the quality control of the institution must be taken into consideration.
Asunto(s)
Tonsila Palatina/patología , Servicio de Patología en Hospital/economía , Patología Clínica/economía , Tonsilectomía , Adolescente , Niño , Preescolar , Análisis Costo-Beneficio , Pruebas Diagnósticas de Rutina/economía , Femenino , Costos de Hospital , Humanos , Lactante , Masculino , Estudios Retrospectivos , Sensibilidad y Especificidad , EspañaRESUMEN
Con el objeto de estudiar la relación costo-beneficio del examen histopatológico de las piezas quirúrgicas de amígdala palatina, se evaluaron 567 amigdalectomías (547 rutinarias y 20 no rutinarias) realizadas en menores de 14 años, entre el 1 de enero de 1996 y el 30 de noviembre de 2000. En 2 casos (0,3 por ciento) de las rutinarias y 6 (30 por ciento) de las no rutinarias se encontró un diagnóstico distinto del de hiperplasia folicular linfoide. Así, la sensibilidad de nuestra valoración clínica preoperatoria resultó ser del 75 por ciento, y la especificidad fue del 97 por ciento. El costo medio por caso en nuestra Institución para el estudio histológico de los especímenes de amígdala resultó ser de 5.955 pesetas. Como conclusión, el análisis sistemático de las amígdalas sólo es económicamente beneficioso en los casos no rutinarios, aunque han de tenerse en cuenta factores adicionales como son la docencia de los residentes de Anatomía Patológica y el control de calidad de la Institución (AU)
To study the cost-benefit of the histological examination of tonsilar samples, we evaluated 567 cases (547 routine and 20 nonroutine cases) of patients under 14 years of age, operated between 1st January 1996 and 30th November 2000. There were 2 routine cases (0.3%), and 6 nonroutine cases (30%) with a diagnosis different to follicular hyperplasia. In this way, our clinical preoperative sensitivity was 75% and specificity 97%. The average cost per case at our Centro to study the tonsilar samples was 30$. We conclude that the histological examination of tonsilar specimens is economically worth only in nonroutine cases, although additional factors (e.g. training of residents of Pathology and the quality control of the institution must be taken into consideration (AU)