RESUMEN
Two alkalinizing mechanisms coexist in cardiac myocytes to maintain intracellular pH: sodium/bicarbonate cotransporter (electroneutral isoform NBCn1 and electrogenic isoform NBCe1) and sodium/proton exchanger (NHE1). Dysfunction of these transporters has previously been reported to be responsible for the development of cardiovascular diseases. The aim of this study was to evaluate the contribution of the downregulation of the NBCe1 to the development of cardiac hypertrophy. To specifically reduce NBCe1 expression, we cloned shRNA into a cardiotropic adeno-associated vector (AAV9-shNBCe1). After 28 days of being injected with AAV9-shNBCe1, the expression and the activity of NBCe1 in the rat heart were reduced. Strikingly, downregulation of NBCe1 causes significant hypertrophic heart growth, lengthening of the action potential in isolated myocytes, an increase in the duration of the QT interval and an increase in the frequency of Ca2+ waves without any significant changes in Ca2+ transients. An increased compensatory expression of NBCn1 and NHE1 was also observed. We conclude that reduction of NBCe1 is sufficient to induce cardiac hypertrophy and modify the electrical features of the rat heart.
Asunto(s)
Bicarbonatos , Simportadores de Sodio-Bicarbonato , Ratas , Animales , Simportadores de Sodio-Bicarbonato/genética , Simportadores de Sodio-Bicarbonato/metabolismo , Bicarbonatos/metabolismo , Cardiomegalia/genética , Cardiomegalia/metabolismo , Sodio/metabolismo , Isoformas de Proteínas/metabolismo , Concentración de Iones de HidrógenoRESUMEN
Rhabdomyosarcoma (RMS) is the most frequent soft tissue sarcoma (STS) in children and adolescents. In Spain the annual incidence is 4.4 cases per million children < 14 years. It is an uncommon neoplasm in adults, but 40% of RMS are diagnosed in patients over 20 years of age, representing 1% of all STS in this age group. RMS can appear anywhere in the body, with some sites more frequently affected including head and neck, genitourinary system and limbs. Assessment of a patient with suspicion of RMS includes imaging studies (MRI, CT, PET-CT) and biopsy. All patients with RMS should receive chemotherapy, either at diagnosis in advanced or metastatic stages, or after initial resection in early local stages. Local control includes surgery and/or radiotherapy depending on site, stage, histology and response to chemotherapy. This guide provides recommendations for diagnosis, staging and treatment of this neoplasm.
Asunto(s)
Tomografía Computarizada por Tomografía de Emisión de Positrones/métodos , Guías de Práctica Clínica como Asunto/normas , Rabdomiosarcoma/terapia , Niño , Terapia Combinada , Humanos , Incidencia , Rabdomiosarcoma/diagnóstico por imagen , Rabdomiosarcoma/epidemiología , Rabdomiosarcoma/patología , España/epidemiologíaRESUMEN
Resumen Introducción: El envejecimiento poblacional ha impulsado a que su abordaje se convierta en un tema prioritario a nivel mundial, sobre la cual aún existen mitos y discriminación, siendo las personas adultas mayores en privación de libertad en un sistema penitenciario, una población con mayores riesgos de exclusión y que cuentan con características neuropsicológicas que ameritan su atención. Por lo tanto, se realizó en Costa Rica un primer abordaje en el Centro de Atención Institucional Adulto Mayor desde la neuropsicología forense, para mejorar los protocolos disponibles de evaluación de la población adulta mayor. Procedimiento: Se llevó a cabo un trabajo final de graduación en formato de práctica dirigida durante 858 horas tanto de forma presencial como a distancia, en el período de abril del 2017 a abril del 2018, participando de los procesos de evaluación neuropsicológica forense, elaboración de un protocolo de evaluación neuropsicológica forense y participación en procesos psicoeducativos sobre consumo de sustancias y violencia sexual. Resultados: En total se atendieron 40 personas hombres, entre 58 y 85 años de edad. Con variables sociodemográficas y de salud como una baja escolaridad (45%), con antecedentes de trauma craneoencefálico (67%) y en su mayoría cometieron abuso sexual (55%). Se elaboró un protocolo de evaluación neuropsicológica que incluye un módulo de entrevista y observación, evaluación del estado de ánimo y evaluación del funcionamiento cognitivo.
Abstract Introduction: Population ageing has become a priority between the attention and care systems but still deals with myths and discrimination, being the older adults incarcerated in the prison system a population with a greater risk of exclusion and that have neuropsychological characteristics that need attention. Therefore, a first approach was carried out in Costa Rica in the Institutional Adult Care Center from the forensic neuropsychology, to improve the available protocols for the evaluation of the older adult population. Method: A final graduation work was carried out in a guided practice format for 858 hours both in person and remotely, in the period from April 2017 to April 2018, participating in the processes of forensic neuropsychological evaluation, preparation of a protocol of forensic neuropsychological evaluation and participation in psychoeducational processes on substance use and sexual violence. Results: In total 40 male people, between 58 and 85 years of age, were attended. With sociodemographic and health variables such as low schooling (45%), with a history of cranioencephalic trauma (67%) and most of them committed sexual abuse (55%). A neuropsychological evaluation protocol was elaborated that includes an interview and observation module, evaluation of mood and evaluation of cognitive functioning module.
Asunto(s)
Humanos , Anciano , Anciano de 80 o más Años , Prisiones , Prisioneros , Anciano , Estado de Salud , Costa Rica , Psicología Forense , NeuropsicologíaRESUMEN
INTRODUCTION: Endocarditis is a rare disease in children, especially in those without previous heart disease, and Kingella Kingae (KK) is rarely identified as the cause. Extracorporeal membrane oxyge nation (ECMO) is a support for both heart and respiratory failure. OBJECTIVE: To report the first case of infectious endocarditis (IE) due to KK which required ECMO support secondary to refractory cardiogenic shock. CLINICAL CASE: 19-months-old previously healthy female patient, with a 2-day his tory of fever, and diagnosed with hand-foot-and-mouth disease. The patient developed refractory cardiogenic shock, multiorgan failure, acute respiratory distress syndrome, and deep hemodynamic compromise that required veno-arterial ECMO support. The echography showed an image compa tible with mitral valve vegetation, confirming IE with transthoracic echocardiography. Blood culture was positive for KK. She had an ischemic stroke and required two heart surgeries, the first one for the mass resection and the second one for mitral valve repair, which had a posterior ring pseudoa neurysm. The patient had a favorable evolution and was discharged 73 days after admission. At one year of follow-up, she had no cardiological symptoms, but a mild right brachial-crural hemiparesis persisted. CONCLUSION: This is the first reported case of IE due to KK that required extracorporeal life support. KK endocarditis is an uncommon pathology that can cause multiorgan failure, which can be successfully supported with ECMO.
Asunto(s)
Endocarditis Bacteriana/complicaciones , Oxigenación por Membrana Extracorpórea , Kingella kingae , Infecciones por Neisseriaceae/complicaciones , Choque Cardiogénico/terapia , Femenino , Humanos , Lactante , Choque Cardiogénico/microbiologíaRESUMEN
Resumen: Introducción: La endocarditis es una enfermedad poco frecuente en niños, especialmente en los sin patología cardiaca previa, y de manera extraordinaria se identifica a Kingella Kingae (KK) como la causa. La oxigenación por membrana extracorpórea (ECMO) es una forma de soporte tanto para fa lla cardiaca como respiratoria. Objetivo: Reportar el primer caso de endocarditis infecciosa (EI) por KK que requiere soporte con ECMO por shock cardiogénico refractario. Caso clínico: Lactante de 19 meses, previamente sana, que consultó por cuadro de 2 días de fiebre, diagnosticándose síndrome pie mano boca. Evolucionó con shock, falla multiorgánica, síndrome de distress respiratorio agudo y compromiso hemodinámico profundo, por lo que se le dio soporte con ECMO veno arterial. La ecoscopía mostró imagen compatible con vegetación en válvula mitral, confirmando EI con ecocardiografía transtorácica. El hemocultivo fue positivo a KK. Presentó accidente cerebrovascular isquémico. Requirió dos cardiocirugías -la primera para resección de la masa y la segunda para la reparación de la válvula mitral, que había quedado con un pseudoaneurisma del anillo- velo posterior. La paciente tuvo una evolución favorable, siendo dada de alta a los 73 días desde el ingreso. Al año de seguimien to se encontraba asintomática cardiaca, pero persistía una hemiparesia braquiocrural derecha leve. Conclusión: Este es el primer caso reportado de EI por KK que requirió soporte vital extracorpóreo. La EI por KK es una patología infrecuente, que puede provocar falla orgánica múltiple, la que puede ser soportada exitosamente con ECMO.
Abstract: Introduction: Endocarditis is a rare disease in children, especially in those without previous heart disease, and Kingella Kingae (KK) is rarely identified as the cause. Extracorporeal membrane oxyge nation (ECMO) is a support for both heart and respiratory failure. Objective: To report the first case of infectious endocarditis (IE) due to KK which required ECMO support secondary to refractory cardiogenic shock. Clinical case: 19-months-old previously healthy female patient, with a 2-day his tory of fever, and diagnosed with hand-foot-and-mouth disease. The patient developed refractory cardiogenic shock, multiorgan failure, acute respiratory distress syndrome, and deep hemodynamic compromise that required veno-arterial ECMO support. The echography showed an image compa tible with mitral valve vegetation, confirming IE with transthoracic echocardiography. Blood culture was positive for KK. She had an ischemic stroke and required two heart surgeries, the first one for the mass resection and the second one for mitral valve repair, which had a posterior ring pseudoa neurysm. The patient had a favorable evolution and was discharged 73 days after admission. At one year of follow-up, she had no cardiological symptoms, but a mild right brachial-crural hemiparesis persisted. Conclusion: This is the first reported case of IE due to KK that required extracorporeal life support. KK endocarditis is an uncommon pathology that can cause multiorgan failure, which can be successfully supported with ECMO.
Asunto(s)
Humanos , Femenino , Lactante , Choque Cardiogénico/terapia , Oxigenación por Membrana Extracorpórea , Infecciones por Neisseriaceae/complicaciones , Kingella kingae , Endocarditis Bacteriana/complicaciones , Choque Cardiogénico/microbiologíaRESUMEN
Soft-tissue sarcomas are uncommon and heterogeneous tumors of mesenchymal origin. A soft-tissue mass that is increasing in size, greater than 5 cm, or located under deep fascia are criteria for suspicion of sarcoma. Diagnosis, treatment, and management should preferably be performed by a multidisciplinary team in reference centers. MRI and lung CT scan are mandatory for local and distant assessment. A biopsy indicating histological type and grade is needed previous to the treatment. Wide surgical resection with tumor-free tissue margin is the primary treatment for localized disease. Radiotherapy is indicated in large, deep, high-grade tumors, or after marginal resection not likely of being improved with reexcision. Neoadjuvant and adjuvant chemotherapy improve survival in selected cases, usually in high-grade sarcomas of the extremities. In the case of metastatic disease, patients with exclusive lung metastasis could be considered for surgery. First-line treatment with anthracyclines (or in combination with ifosfamide) is the treatment of choice. New drugs have shown activity in second-line therapy and in specific histological subtypes.
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Guías de Práctica Clínica como Asunto , Sarcoma/diagnóstico , Sarcoma/terapia , Neoplasias de los Tejidos Blandos/diagnóstico , Neoplasias de los Tejidos Blandos/terapia , Humanos , Clasificación del Tumor , Metástasis de la Neoplasia , EspañaRESUMEN
KEY POINTS: Mice with Ca(2+) -calmodulin-dependent protein kinase (CaMKII) constitutive pseudo-phosphorylation of the ryanodine receptor RyR2 at Ser2814 (S2814D(+/+) mice) exhibit a higher open probability of RyR2, higher sarcoplasmic reticulum (SR) Ca(2+) leak in diastole and increased propensity to arrhythmias under stress conditions. We generated phospholamban (PLN)-deficient S2814D(+/+) knock-in mice by crossing two colonies, S2814D(+/+) and PLNKO mice, to test the hypothesis that PLN ablation can prevent the propensity to arrhythmias of S2814D(+/+) mice. PLN ablation partially rescues the altered intracellular Ca(2+) dynamics of S2814D(+/+) hearts and myocytes, but enhances SR Ca(2+) sparks and leak on confocal microscopy. PLN ablation diminishes ventricular arrhythmias promoted by CaMKII phosphorylation of S2814 on RyR2. PLN ablation aborts the arrhythmogenic SR Ca(2+) waves of S2814D(+/+) and transforms them into non-propagating events. A mathematical human myocyte model replicates these results and predicts the increase in SR Ca(2+) uptake required to prevent the arrhythmias induced by a CaMKII-dependent leaky RyR2. ABSTRACT: Mice with constitutive pseudo-phosphorylation at Ser2814-RyR2 (S2814D(+/+) ) have increased propensity to arrhythmias under ß-adrenergic stress conditions. Although abnormal Ca(2+) release from the sarcoplasmic reticulum (SR) has been linked to arrhythmogenesis, the role played by SR Ca(2+) uptake remains controversial. We tested the hypothesis that an increase in SR Ca(2+) uptake is able to rescue the increased arrhythmia propensity of S2814D(+/+) mice. We generated phospholamban (PLN)-deficient/S2814D(+/+) knock-in mice by crossing two colonies, S2814D(+/+) and PLNKO mice (SD(+/+) /KO). SD(+/+) /KO myocytes exhibited both increased SR Ca(2+) uptake seen in PLN knock-out (PLNKO) myocytes and diminished SR Ca(2+) load (relative to PLNKO), a characteristic of S2814D(+/+) myocytes. Ventricular arrhythmias evoked by catecholaminergic challenge (caffeine/adrenaline) in S2814D(+/+) mice in vivo or programmed electric stimulation and high extracellular Ca(2+) in S2814D(+) /(-) hearts ex vivo were significantly diminished by PLN ablation. At the myocyte level, PLN ablation converted the arrhythmogenic Ca(2+) waves evoked by high extracellular Ca(2+) provocation in S2814D(+/+) mice into non-propagated Ca(2+) mini-waves on confocal microscopy. Myocyte Ca(2+) waves, typical of S2814D(+/+) mice, could be evoked in SD(+/+) /KO cells by partially inhibiting SERCA2a. A mathematical human myocyte model replicated these results and allowed for predicting the increase in SR Ca(2+) uptake required to prevent the arrhythmias induced by a Ca(2+) -calmodulin-dependent protein kinase (CaMKII)-dependent leaky RyR2. Our results demonstrate that increasing SR Ca(2+) uptake by PLN ablation can prevent the arrhythmic events triggered by SR Ca(2+) leak due to CaMKII-dependent phosphorylation of the RyR2-S2814 site and underscore the benefits of increasing SERCA2a activity on SR Ca(2+) -triggered arrhythmias.
Asunto(s)
Arritmias Cardíacas/metabolismo , Proteínas de Unión al Calcio/deficiencia , Proteína Quinasa Tipo 2 Dependiente de Calcio Calmodulina/metabolismo , Canal Liberador de Calcio Receptor de Rianodina/metabolismo , Potenciales de Acción/fisiología , Animales , Arritmias Cardíacas/genética , Arritmias Cardíacas/fisiopatología , Calcio/metabolismo , Proteínas de Unión al Calcio/genética , Proteína Quinasa Tipo 2 Dependiente de Calcio Calmodulina/genética , Técnicas de Sustitución del Gen , Masculino , Ratones , Ratones Endogámicos C57BL , Ratones Noqueados , Ratones Transgénicos , Miocitos Cardíacos/metabolismo , Miocitos Cardíacos/fisiología , Fosforilación/fisiología , Canal Liberador de Calcio Receptor de Rianodina/genéticaRESUMEN
PURPOSE: To evaluate the efficacy and toxicity of docetaxel regimen as second-line after failure of a platinum-based chemotherapy. METHODS: Between May 2005 and June 2008, we retrospectively analyzed the data of 22 patients who had evidence of disease progression after one prior platinum-based regimen for metastatic urothelial carcinoma. Patients were treated with two different docetaxel dose schedules: (1) docetaxel 60 mg/m(2) every 21 days for unfit patients or (2) docetaxel 75 mg/m(2) every 21 days for fit patients. RESULTS: Median number of docetaxel cycles was three. Overall disease control rate was 18 %. Of the 22 patients, no patient achieved complete or partial response and four patients had stable disease. Median progression-free survival was 1.67 months and median overall survival was 3.12 months. Neutropenia was the most common adverse event. CONCLUSIONS: This study identifies that docetaxel as second-line chemotherapy has low activity and was associated with significant toxicity.
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Antineoplásicos/uso terapéutico , Carcinoma de Células Transicionales/tratamiento farmacológico , Taxoides/uso terapéutico , Neoplasias de la Vejiga Urinaria/tratamiento farmacológico , Anciano , Anciano de 80 o más Años , Carcinoma de Células Transicionales/mortalidad , Supervivencia sin Enfermedad , Docetaxel , Femenino , Humanos , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Terapia Recuperativa , Neoplasias de la Vejiga Urinaria/mortalidadRESUMEN
INTRODUCTION: In 1994 our group began its experience with pediatric liver transplantation. The experience gained during this period is the largest in the country, positioning the Hospital Luis Calvo Mackenna and Clinica Las Condes as major referral centers in the public and private sectors. The aim of this study was to report our experience of our pediatric liver transplantation program during this period. METHODS: The liver transplantation database of Hospital Luis Calvo Mackenna and Clinica Las Condes between January 1994 and July 2011 was reviewed recording age, gender, indications for transplantation, surgical technique, complications, and survival. Survival rates were calculated using Kaplan-Meier analysis. RESULTS: During the period described 230 transplantations were performed in 189 pediatric patients. Fifty-five percent were male patients. The average age was 5 years. The main causes of transplantation were biliary atresia (50%), fulminant hepatic failure (25%), and other cholestatic diseases by 10%. Vascular and biliary complications were the leading cause of graft loss and retransplantation. The overall rate of retransplantation at 5 years was 20%. The technique of living donor was used in 28% of the cases. The 1-year patient actuarial survival rate was 80%, 73% at 5 years, and 68% at 10 years. In the last 3 years the survival rate at 1 year exceeds 90%. DISCUSSION: Our program includes more than 90% of the national liver experience. The incorporation of living donor is a milestone that has enabled us to save many patients who previously died while waiting for an organ. Its use in cases of full acute liver failure has allowed us to dramatically reduce mortality on the waiting list. Our results in the last 3 years reflect the experience that results in a significant decrease in mortality, comparing favorably to other series published in the international literature.
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Trasplante de Hígado , Factores de Edad , Preescolar , Chile , Femenino , Supervivencia de Injerto , Humanos , Estimación de Kaplan-Meier , Trasplante de Hígado/efectos adversos , Trasplante de Hígado/mortalidad , Donadores Vivos/provisión & distribución , Masculino , Complicaciones Posoperatorias/mortalidad , Complicaciones Posoperatorias/cirugía , Evaluación de Programas y Proyectos de Salud , Reoperación , Factores de Riesgo , Tasa de Supervivencia , Factores de Tiempo , Resultado del Tratamiento , Listas de EsperaRESUMEN
To explore whether CaMKII-dependent phosphorylation events mediate reperfusion arrhythmias, Langendorff perfused hearts were submitted to global ischemia/reperfusion. Epicardial monophasic or transmembrane action potentials and contractility were recorded. In rat hearts, reperfusion significantly increased the number of premature beats (PBs) relative to pre-ischemic values. This arrhythmic pattern was associated with a significant increase in CaMKII-dependent phosphorylation of Ser2814 on Ca(2+)-release channels (RyR2) and Thr17 on phospholamban (PLN) at the sarcoplasmic reticulum (SR). These phenomena could be prevented by the CaMKII-inhibitor KN-93. In transgenic mice with targeted inhibition of CaMKII at the SR membranes (SR-AIP), PBs were significantly decreased from 31±6 to 5±1 beats/3min with a virtually complete disappearance of early-afterdepolarizations (EADs). In mice with genetic mutation of the CaMKII phosphorylation site on RyR2 (RyR2-S2814A), PBs decreased by 51.0±14.7%. In contrast, the number of PBs upon reperfusion did not change in transgenic mice with ablation of both PLN phosphorylation sites (PLN-DM). The experiments in SR-AIP mice, in which the CaMKII inhibitor peptide is anchored in the SR membrane but also inhibits CaMKII regulation of L-type Ca(2+) channels, indicated a critical role of CaMKII-dependent phosphorylation of SR proteins and/or L-type Ca(2+) channels in reperfusion arrhythmias. The experiments in RyR2-S2814A further indicate that up to 60% of PBs related to CaMKII are dependent on the phosphorylation of RyR2-Ser2814 site and could be ascribed to delayed-afterdepolarizations (DADs). Moreover, phosphorylation of PLN-Thr17 and L-type Ca(2+) channels might contribute to reperfusion-induced PBs, by increasing SR Ca(2+) content and Ca(2+) influx.
Asunto(s)
Arritmias Cardíacas/enzimología , Arritmias Cardíacas/etiología , Proteína Quinasa Tipo 2 Dependiente de Calcio Calmodulina/metabolismo , Daño por Reperfusión Miocárdica/complicaciones , Daño por Reperfusión Miocárdica/enzimología , Transducción de Señal , Potenciales de Acción , Sustitución de Aminoácidos , Animales , Arritmias Cardíacas/genética , Arritmias Cardíacas/prevención & control , Bencilaminas/farmacología , Proteína Quinasa Tipo 2 Dependiente de Calcio Calmodulina/antagonistas & inhibidores , Proteína Quinasa Tipo 2 Dependiente de Calcio Calmodulina/genética , Corazón/efectos de los fármacos , Corazón/fisiopatología , Masculino , Ratones , Ratones Transgénicos , Mutación , Daño por Reperfusión Miocárdica/genética , Fosforilación/efectos de los fármacos , Inhibidores de Proteínas Quinasas/farmacología , Ratas , Ratas Wistar , Retículo Sarcoplasmático/efectos de los fármacos , Retículo Sarcoplasmático/metabolismo , Sulfonamidas/farmacologíaRESUMEN
BACKGROUND: The incidence of testicular germ cell tumours (TGCT) is increasing and the improvement in survival may lead to an increased incidence of bilateral tumours. We examined the incidence, prognosis, clinical and histological characteristics, treatment and outcome of patients with bilateral TGCTs based on 15 years of experience from a single institution. MATERIAL AND METHODS: We reviewed the charts from all patients treated for a testicular tumour germ cell at Hospital Vall d'Hebron in Barcelona, Spain. The information was retrospectively obtained from the patients' hospital. All the patients were evaluated with clinical history, physical exam, serum markers (alphaFP, LDH and betahCG), ultrasonographic evaluation of the testicles, computed tomography (CT) scans of the chest, abdomen and pelvis, surgery, location and histology of first and second tumour, treatment after the surgery and follow-up. RESULTS: Of 151 patients with TGCT, 8 (5.3%) developed bilateral tumours, seven (4.6%) were metachronous and one (0.7%) synchronous tumours. Two patients underwent testis-sparing surgery for the second tumour. All the patients are alive without evidence of disease based on physical exam, tumour markers and CT scan. CONCLUSIONS: Survival in patients with bilateral testicular germ cell tumours (BTGCT) is similar to that of patients with unilateral TGCT. There is no standard therapy to treat BTGCT and each patient requires a tailored therapeutic treatment.
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Neoplasias de Células Germinales y Embrionarias/patología , Neoplasias Testiculares/patología , Adulto , Humanos , Incidencia , Masculino , Neoplasias de Células Germinales y Embrionarias/epidemiología , Neoplasias de Células Germinales y Embrionarias/terapia , Neoplasias Primarias Múltiples/epidemiología , Neoplasias Primarias Múltiples/patología , Neoplasias Primarias Múltiples/terapia , Neoplasias Primarias Secundarias/epidemiología , Neoplasias Primarias Secundarias/patología , Neoplasias Primarias Secundarias/terapia , Pronóstico , Neoplasias Testiculares/epidemiología , Neoplasias Testiculares/terapia , Adulto JovenRESUMEN
BACKGROUND: Acute liver failure (ALF) in children is a life-threatening condition, associated with high mortality, and in almost one third of the cases, with no other therapeutic option than orthotopic liver transplant (OLT). The aim of this study was to present our experience with OLT for ALF in pediatric patients in Chile. Patients fulfilling the criteria for ALF who were transplanted in our centers were prospectively included in an excel Microsoft database. We analyzed demographics, etiology, surgical techniques, complications, and long-term results. PATIENTS AND METHODS: Between 1994 and 2009, we transplanted 52 pediatric patients with ALF. The most frequent known etiology was acute hepatitis A in 9 cases (18%), but in 26 cases (50%) it was impossible to determine the etiology. Thirty- one patients were males (63%). The overall mean age was 7.5 years and the mean weight, 28.1 kg. Thirty-five (67%) received a cadaveric graft. Among them in 18 cases (34%) the liver had to be reduced but 17 (33%) received whole livers. There were 17 (33%) recipients of living-related livers. Twenty-two patients needed reoperation, including 13 due to surgical complications (59%) and 9 (41%) as planned interventions. Ten patients were retransplanted. RESULTS: Actuarial survival of patients at 1 year was 80% and at 5 and 10 years, 72%. Graft survival at 1 year was 79%, at 5 years 69%, and at 10 years 50%. CONCLUSION: We have reported a series of pediatric liver transplant patients due to ALF whose results were comparable to other reported series. Living donor transplantation for ALF should be considered and offers a low morbidity rate without mortality.
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Fallo Hepático Agudo/epidemiología , Trasplante de Hígado/efectos adversos , Cadáver , Niño , Chile , Femenino , Estudios de Seguimiento , Hepatitis A/complicaciones , Hepatitis Viral Humana/complicaciones , Humanos , Fallo Hepático Agudo/cirugía , Fallo Hepático Agudo/virología , Trasplante de Hígado/mortalidad , Donadores Vivos , Masculino , Enfermedades Metabólicas/complicaciones , Tasa de Supervivencia , Donantes de TejidosRESUMEN
Pigeon pea (Cajanus cajan var. aroíto) seeds were fermented in order to remove antinutritional factors and to obtain functional legume flour to be used as pasta ingredients. Fermentation brought about a drastic reduction of alpha-galactosides (82%), phytic acid (48%), and trypsin inhibitor activity (39%). Fermented legume flours presented a notable increase of fat and total soluble available carbohydrates, a slight decrease of protein, dietary fiber, calcium, vitamin B2, vitamin E, and total antioxidant capacity, and a decrease of soluble dietary fiber, Na, K, Mg, and Zn contents. No changes were observed in the level of starch and tannins as a consequence of fermentation. The fermented flour was used as an ingredient to make pasta products in a proportion of 5, 10, and 12%. The supplemented pasta products obtained had longer cooking times, higher cooking water absorptions, higher cooking loss, and higher protein loss in water than control pasta (100% semolina). From sensory evaluations, fortified pasta with 5 and 10% fermented pigeon pea flour had an acceptability score similar to control pasta. Pasta supplemented with 10% fermented pigeon pea flour presented higher levels of protein, fat, dietary fiber, mineral, vitamin E, and Trolox equivalent antioxidant capacity than 100% semolina pasta and similar vitamins B1 and B2 contents. Protein efficiency ratios and true protein digestibility improved (73 and 6%, respectively) after supplementation with 10% fermented pigeon pea flour; therefore, the nutritional value was enhanced.
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Cajanus/metabolismo , Fermentación , Alimentos , Semillas/química , Semillas/metabolismo , Fibras de la Dieta/análisis , Galactósidos/análisis , Calor , Valor Nutritivo , Ácido Fítico/análisis , Proteínas de Plantas/análisis , Inhibidores de Tripsina/análisis , Vitaminas/análisisRESUMEN
Extubation, like intubation, is a critical moment in general anesthesia. There are no algorithms or ordered sequences of steps for extubation. Rather, the approach to take is strict observation of the patient in a setting equipped with monitors, material for managing the difficult airway, and experienced staff who should be able to establish access immediately, provide oxygen, and facilitate gas exchange, keeping the airway open and safeguarding it in case of a failed extubation attempt. This review will analyze the clinical conditions and pathophysiology associated with extubations at high risk of complications. We will describe strategies for extubating in situations in which a difficult airway is known or suspected.
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Intubación Intratraqueal/métodos , Algoritmos , Diseño de Equipo , Humanos , Intubación Intratraqueal/efectos adversos , Intubación Intratraqueal/instrumentaciónRESUMEN
A 56-year-old man recovering from a glossectomy and radical neck dissection presented severe oral bleeding, tracheal deviation with an asphyxiating hematoma and cyanosis. When 2 attempts at orotracheal intubation with the patient awake failed, transtracheal jet ventilation was used temporarily until a definitive airway could be established. Transtracheal jet ventilation is highly useful for managing an airway and maintaining gas exchange in life-threatening situations in which intubation and ventilation has become impossible, yet it is rarely used for that purpose. An easy, fast procedure that has not been widely used in Spain, this technique provides effective ventilation and oxygen while a definitive resolution of the emergency is sought.
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Ventilación con Chorro de Alta Frecuencia/métodos , Intubación Intratraqueal , Diseño de Equipo , Ventilación con Chorro de Alta Frecuencia/instrumentación , Humanos , Masculino , Persona de Mediana Edad , Respiración Artificial/métodosRESUMEN
Conversion of T4 to T3 is the first step in TH action and deiodinases are the major determinants of TH tissue availability and disposal. We here report the kinetic characterization of the outer-ring deiodinating (ORD) enzymes in the liver, gill and retina of sea water-adapted killifish, by using both rT3 and T4 as substrates. In liver, by using rT3, we detected a high Km (84 nM) and a low Km (1.3 nM) component with kinetic characteristics similar to mammalian deiodinases DI and DII. In contrast, T4-ORD only generated a low Km (0.5 nM) component. As judged by its Vmax (920 fmol 125I/mg per h) this DII enzyme is very abundant, approximately five and 20 times higher than that found in trout liver and hypothyroid rat, respectively. Kinetic analysis in killifish gill showed only one enzymatic component, with a high rT3 Km (430 nM) and a relatively low Vmax (4.3 pmol 125I/mg per h). Our results in killifish retina show the expression of a T4-low Km (0.6 nM) deiodinase with high cofactor requirements akin to the mammalian DII. The Vmax value for this enzyme is 182 fmol 125I/mg per h, five times lower than the one found in killifish liver, but comparable to that in hypothyroid rat pituitary. The biochemical similarities between fish and mammalian deiodinases could reflect their high conservation during vertebrate evolution and thus their importance in the regulation of thyroid hormone action.
Asunto(s)
Yoduro Peroxidasa/metabolismo , Peces Killi/metabolismo , Animales , Activación Enzimática , Branquias/enzimología , Cinética , Hígado/enzimología , Especificidad de Órganos , Ratas , Retina/enzimología , Especificidad por SustratoAsunto(s)
Plaguicidas/envenenamiento , Estricnina/envenenamiento , Animales , Niño , Chile , Perros , Humanos , Masculino , Intoxicación/terapiaRESUMEN
Neck located hydatid cysts are of rare occurrence. We report two patients with such condition. A 66 years old male with a slowly progressive painless 4 cm nodule located in the right thyroid. It did not concentrate 131I and a fine needle aspiration cytology was informed as an acute thyroiditis. A 5 years old boy presented with a 5 cm painless right submaxillary cyst. Ultrasound examination showed that it was unilocular, and fine needle aspiration biopsy disclosed unspecific findings. In both cases surgical findings and the pathological study showed hydatid cysts. Both patients had normal chest x ray and abdominal ultrasound examinations. They had an uneventful postoperative evolution. Echinococcosis must be considered in the differential diagnosis of cervical cysts in endemic area.