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PURPOSE: The purpose of this study was to investigate factors impacting transplant-free survival among infants with biliary atresia. METHODS: A multi-institutional, retrospective cohort study was performed at nine tertiary-level children's hospitals in the United States. Infants who underwent Kasai portoenterostomy (KP) from January 2009 to May 2017 were identified. Clinical characteristics included age at time of KP, steroid use, surgical approach, liver pathology, and surgeon experience. Likelihood of transplant-free survival (TFS) was evaluated using logistic regression, adjusting for patient and surgeon-level factors. Secondary outcomes at 1 year included readmission, cholangitis, reoperation, mortality, and biliary clearance. RESULTS: Overall, 223 infants underwent KP, and 91 (40.8%) survived with their native liver. Mean age at surgery was 63.9 days (± 24.7 days). At 1 year, 78.5% experienced readmission, 56.9% developed cholangitis, 3.8% had a surgical revision, and 5 died. Biliary clearance at 3 months was achieved in 76.6%. Controlling for patient and surgeon-level factors, each additional day of age toward operation was associated with a 2% decrease in likelihood of TFS (OR 0.98, 95% CI 0.97-0.99). CONCLUSION: Earlier surgical intervention by Kasai portoenterostomy at tertiary-level centers significantly increases likelihood for TFS. Policy-level interventions to facilitate early screening and surgical referral for infants with biliary atresia are warranted to improve outcomes.
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Atresia Biliar , Trasplante de Hígado , Atresia Biliar/cirugía , Humanos , Lactante , Portoenterostomía Hepática , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
BACKGROUND: The minimum weight for enterostomy closure (EC) in infants remains debated with the current acceptable cut-off of >2 kg. As enterostomy-related complications or high enterostomy output (>30cc/kg/d) may prohibit a premature infant from reaching 2 kg, additional data is needed to evaluate the safety of EC in infants <2 kg. The objective of this study was to evaluate postoperative outcomes in low body weight (<2 kg) infants undergoing EC compared to larger infants. METHODS: We performed a multi-center retrospective analysis from 1/1/2012-12/31/2022 of all infants (age <1 year) who were <4 kg at time of EC. Primary outcomes included postoperative complications and 30-day mortality. Non-parametric analysis was performed using the Kruskal-Wallis one-way analysis of variance and chi-square tests. Univariable logistic regression was performed to identify factors associated with postoperative complications. RESULTS: Of 92 infants, 15 infants (16.3%) underwent EC at <2 kg, 16 (17.4%) at 2-2.49 kg, 31 (33.7%) at 2.5-2.99 kg, and 30 (32.6%) at ≥3 kg. Infants <2 kg at time of EC exhibited higher rates of hyperbilirubinemia (P = .030), neurologic comorbidities (P = .030), and high enterostomy output (P = .041). There was no difference in postoperative complications (P = .460) or 30-day mortality (P = .460) between the <2 kg group and larger weight groups. Low body weight was not associated with an increased risk for developing a postoperative complication (OR: 1.001, 95% CI: 1.001-1.001; P = .032). CONCLUSION: Our findings suggest that EC in infants <2 kg may be safe with comparable postoperative outcomes to larger weight infants. Thus, the timing of EC should be based on the infant's physiologic status, in contrast to a predetermined minimum weight cut-off.
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Merkel cell carcinoma (MCC) is an aggressive neuroendocrine carcinoma of the skin. Disease progression usually occurs via lymphatic spread to regional lymphatic draining basins, followed by distant metastasis. We report the clinical course, histopathology and genetic analysis of a 69-year-old woman with likely hematogenous spread of cutaneous neuroendocrine carcinoma manifesting as a single metastatic lesion to a distant cutaneous site. Although the possibility of two cutaneous primary MCCs was considered, array comparative genomic hybridization (aCGH) identified identical distal amplification of a region of chromosome 12p, and synchronous loss of chromosomes 8p and 17p, effectively ruling out the possibility of independent primaries. We propose that this represents a primary cheek MCC with rapid, isolated cutaneous metastasis to the contralateral ankle via hematogenous spread. The distinction between a second primary MCC and a distant cutaneous metastasis clearly has important implications with regard to staging, treatment and prognosis. To our knowledge, this represents the first report of the use of aCGH to clarify the relationship of multiple synchronous cutaneous MCCs and the first report of a single distant cutaneous focus of hematogenous spread. Our data calls into question prior reports alleging multiple cutaneous primaries of this very rare tumor.
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Carcinoma de Células de Merkel/patología , Neoplasias Primarias Secundarias/etiología , Neoplasias Cutáneas/patología , Anciano , Carcinoma de Células de Merkel/genética , Carcinoma de Células de Merkel/terapia , Terapia Combinada , Hibridación Genómica Comparativa , ADN de Neoplasias/análisis , Diagnóstico Diferencial , Femenino , Humanos , Neoplasias Cutáneas/genética , Neoplasias Cutáneas/terapiaRESUMEN
BACKGROUND: Plasmalemmal vesicle associated protein-1 (PV-1) is selectively expressed in human brain microvascular endothelial cells derived from clinical specimens of primary and secondary malignant brain tumors, cerebral ischemia, and other central nervous system (CNS) diseases associated with blood-brain barrier breakdown. In this study, we characterize the murine CNS expression pattern of PV-1 to determine whether localized PV-1 induction is conserved across species and disease state. RESULTS: We demonstrate that PV-1 is selectively upregulated in mouse blood vessels recruited by brain tumor xenografts at the RNA and protein levels, but is not detected in non-neoplastic brain. Additionally, PV-1 is induced in a mouse model of acute ischemia. Expression is confined to the cerebovasculature within the region of infarct and is temporally regulated. CONCLUSION: Our results confirm that PV-1 is preferentially induced in the endothelium of mouse brain tumors and acute ischemic brain tissue and corresponds to blood-brain barrier disruption in a fashion analogous to human patients. Characterization of PV-1 expression in mouse brain is the first step towards development of rodent models for testing anti-edema and anti-angiogenesis therapeutic strategies based on this molecule.
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Barrera Hematoencefálica/metabolismo , Isquemia Encefálica/metabolismo , Neoplasias Encefálicas/metabolismo , Proteínas Portadoras/análisis , Endotelio Vascular/metabolismo , Proteínas de la Membrana/análisis , Animales , Biomarcadores/análisis , Modelos Animales de Enfermedad , Masculino , Ratones , Ratones Endogámicos C57BL , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa , Especificidad de la Especie , Regulación hacia ArribaRESUMEN
UNLABELLED: The aim of the present study was to compare two protocols and two information types on detection of concealed knowledge. Four independent groups of subjects were run. Two were tested on probe stimuli of a self-referring (AUTO) nature and two were tested on incidentally acquired details of a mock crime (MOCK). Each pair of groups was run either in a one-probe (1PB) or multiple probe (6PB) block. In the single probe block, which was repeated three times with a different probe on each block, one probe item was randomly repeated multiple times in a Bernoulli series with frequently presented, meaningless or irrelevant items. There was also a rare target item designed to force attention to the stimulus screen. All stimulus types were of the same category within each block. In contrast, in the multiple probe (and category) block, rare probes, rare targets and frequent irrelevant items were repeatedly presented in a Bernoulli series within one block. MAJOR RESULTS: There was a difference in task demand as measured by reaction time between the two protocols (the multiple probe protocol was more demanding), and a difference trend in P300 detection sensitivity between protocols for both information types combined in favor of the 1PB (p<.07). With both protocols combined, there was a trend (p<.07) favoring detection of familiar versus incidentally learned information. In terms of P300 amplitudes, both protocols showed the usual result that P300 to probes was greater than that to irrelevants. Also, as with detection rates, self-referring information was better detected in terms of Probe-Irrelevant P300 amplitude differences than mock crime information, regardless of protocol. There was no effect of time passage across the repeated blocks of the one-probe protocol. Methodological problems with the multiple probe protocol as utilized in most recent publications are discussed.
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Nivel de Alerta/fisiología , Atención/fisiología , Corteza Cerebral/fisiología , Psicología Criminal , Potenciales Relacionados con Evento P300/fisiología , Culpa , Detección de Mentiras , Recuerdo Mental/fisiología , Adolescente , Adulto , Dominancia Cerebral/fisiología , Electroencefalografía , Femenino , Humanos , Masculino , Práctica Psicológica , Tiempo de Reacción/fisiología , Procesamiento de Señales Asistido por ComputadorRESUMEN
PURPOSE: Plasmalemmal vesicle associated protein-1 (PV-1) is up-regulated in the endothelium of human glioblastoma. We sought to further characterize the expression pattern of PV-1 in human brain tumors and interrogate its role in brain tumor angiogenesis. EXPERIMENTAL DESIGN: Quantitative reverse transcription-PCR and in situ hybridization were used to measure PV-1 expression in a panel of 46 human brain tumors and related pathologic states. Matrigel tubulogenesis assays and cell migration assays were used to show function of PV-1 in primary human endothelial cells (HMVEC) under gene knockdown conditions. RESULTS: PV-1 is selectively up-regulated in a variety of high-grade human brain tumors, including glioblastoma and metastatic carcinoma, as well as other cerebral disorders associated with blood-brain barrier disruption, such as acute ischemia. Expression levels were reduced in low-grade neoplasia; however, tumors associated with the ependyma and choroid plexus, known sites of PV-1 expression, also exhibited robust expression. Cerebral expression of PV-1 mRNA was confined to endothelial cells in all cases. PV-1 expression was induced in HMVEC cells in vitro by exposure to medium conditioned by U87MG and U251MG human brain tumor cell lines and by medium supplemented with exogenous vascular endothelial growth factor or scatter factor/hepatocyte growth factor. RNA interference-mediated inhibition of PV-1 induction in HMVEC cells blocked Matrigel-induced tubulogenesis and inhibited cell migration induced by conditioned medium or angiogenic growth factors. CONCLUSIONS: Our results confirm that PV-1 is preferentially induced in the endothelium of high-grade human brain tumors. Inhibition of PV-1 expression is associated with failure of endothelial differentiation in vitro. PV-1 represents a novel marker of brain tumor angiogenesis and integrity of the blood-brain barrier and is a potential therapeutic target.
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Biomarcadores de Tumor , Neoplasias Encefálicas/patología , Proteínas Portadoras/biosíntesis , Regulación Neoplásica de la Expresión Génica , Proteínas de la Membrana/biosíntesis , Neovascularización Patológica , Barrera Hematoencefálica , Western Blotting , Línea Celular Tumoral , Movimiento Celular , Células Cultivadas , Colágeno/química , Medios de Cultivo Condicionados/farmacología , Combinación de Medicamentos , Endotelio Vascular/citología , Endotelio Vascular/patología , Humanos , Hibridación in Situ , Isquemia , Laminina/química , Microcirculación , Metástasis de la Neoplasia , Proteoglicanos/química , ARN/metabolismo , Interferencia de ARN , ARN Mensajero/metabolismo , ARN Interferente Pequeño/metabolismo , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa , Transcripción Genética , Regulación hacia Arriba , Factor A de Crecimiento Endotelial Vascular/metabolismoRESUMEN
Two forms of human tryptophanyl-tRNA synthetase (TrpRS) are produced in vivo through alternative mRNA splicing. The two forms, full-length TrpRS and mini TrpRS, are catalytically active, but are distinguished by the striking anti-proliferative and anti-angiogenic activity specific to mini TrpRS. Here we describe two new splice variants of human TrpRS mRNA. Their production was strongly regulated by gamma-interferon (IFN-gamma), an anti-proliferative cytokine known to stimulate the expression of other anti-angiogenic factors. A new IFN-gamma-sensitive promoter was demonstrated to drive production of these splice variants. In human endothelial cells, both the newly discovered and a previously reported promoter were shown to respond specifically to IFN-gamma and not to other cytokines such as tumor necrosis factor-alpha, transforming growth factor-beta, interleukin-4 or erythropoietin. In addition, both promoters were stimulated by the 'downstream' interferon regulatory factor 1 that, in turn, is known to be regulated by the 'upstream' signal transducer and activator of transcription 1alpha subunit. Thus, the tandem promoters provide a dual system to regulate expression and alternative splicing of human TrpRS in vivo.
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Empalme Alternativo/efectos de los fármacos , Inhibidores de la Angiogénesis/genética , Regulación Enzimológica de la Expresión Génica/efectos de los fármacos , Interferón gamma/farmacología , Regiones Promotoras Genéticas/efectos de los fármacos , Triptófano-ARNt Ligasa/genética , Regiones no Traducidas 5'/genética , Empalme Alternativo/genética , Secuencia de Bases , Células Cultivadas , Proteínas de Unión al ADN/genética , Proteínas de Unión al ADN/metabolismo , Células Endoteliales/efectos de los fármacos , Células Endoteliales/metabolismo , Células HeLa , Humanos , Factor 1 Regulador del Interferón , Isoenzimas/genética , Fosfoproteínas/genética , Fosfoproteínas/metabolismo , Regiones Promotoras Genéticas/genética , ARN Mensajero/genética , ARN Mensajero/metabolismoRESUMEN
INTRODUCTION: Attempts at defining predictors of poor outcome in fetal sacrococcygeal teratoma (SCT) have been hampered by small patient numbers. We sought to validate the utility of tumor volume to fetal weight ratio (TFR) as a predictor of poor prognosis and to identify other morphological outcome predictors in a multicenter series. METHODS: Records of prenatally diagnosed SCT at three fetal centers from 1986 to 2011 were reviewed. Prenatal imaging characteristics including TFR, morphology, hydrops, and placentomegaly were assessed. Poor prognosis was defined as fetal demise, need for fetal intervention, or perinatal death. Receiver operating characteristic (ROC) analysis was used to select a TFR cutoff value. RESULTS: Seventy-nine fetuses with SCT were evaluated. Eleven pregnancies ending in elective termination were excluded. ROC analysis revealed that TFR >0.12 prior to 24 weeks gestation was predictive of poor prognosis (AUC=0.913; Sensitivity=91.7%, Specificity=76.2%, PPV=86.8%; NPV=84.2%). Solid tumor morphology and presence of hydrops were found to be predictors of poor prognosis. None of the factors associated with poor prognosis were independent predictors on multivariate analysis. CONCLUSION: This study validates TFR >0.12 prior to 24 weeks gestation as an objective predictor of outcomes in fetuses with SCT that can be easily applied in most clinical settings.
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Neoplasias de la Médula Espinal/diagnóstico , Teratoma/diagnóstico , Ultrasonografía Prenatal/métodos , Femenino , Muerte Fetal , Edad Gestacional , Humanos , Embarazo , Pronóstico , Región Sacrococcígea , Neoplasias de la Médula Espinal/mortalidad , Teratoma/mortalidad , Estados Unidos/epidemiologíaRESUMEN
PURPOSE: Pulmonary hypertension (pHTN), a main determinant of survival in congenital diaphragmatic hernia (CDH), results from in utero vascular remodeling. Phosphodiesterase type 5 (PDE5) inhibitors have never been used antenatally to treat pHTN. The purpose of this study is to determine if antenatal PDE5 inhibitors can prevent pHTN in the fetal lamb model of CDH. METHODS: CDH was created in pregnant ewes. Postoperatively, pregnant ewes received oral placebo or tadalafil, a PDE5 inhibitor, until delivery. Near term gestation, lambs underwent resuscitations, and lung tissue was snap frozen for protein analysis. RESULTS: Mean cGMP levels were 0.53±0.11 in placebo-treated fetal lambs and 1.73±0.21 in tadalafil-treated fetal lambs (p=0.002). Normalized expression of eNOS was 82%±12% in Normal-Placebo, 61%±5% in CDH-Placebo, 116%±6% in Normal-Tadalafil, and 86%±8% in CDH-Tadalafil lambs. Normalized expression of ß-sGC was 105%±15% in Normal-Placebo, 82%±3% in CDH-Placebo, 158%±16% in Normal-Tadalafil, and 86%±8% in CDH-Tadalafil lambs. Endothelial NOS and ß-sGC were significantly decreased in CDH (p=0.0007 and 0.01 for eNOS and ß-sGC, respectively), and tadalafil significantly increased eNOS expression (p=0.0002). CONCLUSIONS: PDE5 inhibitors can cross the placental barrier. ß-sGC and eNOS are downregulated in fetal lambs with CDH. Antenatal PDE5 inhibitors normalize eNOS and may prevent in utero vascular remodeling in CDH.
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Carbolinas/uso terapéutico , Enfermedades Fetales/tratamiento farmacológico , Terapias Fetales , Hernias Diafragmáticas Congénitas , Óxido Nítrico Sintasa de Tipo III/biosíntesis , Inhibidores de Fosfodiesterasa 5/uso terapéutico , Animales , Carbolinas/administración & dosificación , Carbolinas/farmacología , GMP Cíclico/análisis , Modelos Animales de Enfermedad , Evaluación Preclínica de Medicamentos , Inducción Enzimática/efectos de los fármacos , Femenino , Hernia Diafragmática/complicaciones , Hernia Diafragmática/embriología , Hernia Diafragmática/enzimología , Hernia Diafragmática/prevención & control , Hipertensión Pulmonar/embriología , Hipertensión Pulmonar/enzimología , Hipertensión Pulmonar/etiología , Hipertensión Pulmonar/prevención & control , Hipertrofia Ventricular Derecha/embriología , Hipertrofia Ventricular Derecha/enzimología , Hipertrofia Ventricular Derecha/etiología , Pulmón/química , Pulmón/efectos de los fármacos , Pulmón/embriología , Pulmón/patología , Intercambio Materno-Fetal , Óxido Nítrico Sintasa de Tipo III/genética , Tamaño de los Órganos/efectos de los fármacos , Inhibidores de Fosfodiesterasa 5/administración & dosificación , Inhibidores de Fosfodiesterasa 5/farmacología , Embarazo , Distribución Aleatoria , Sistemas de Mensajero Secundario/efectos de los fármacos , Ovinos , TadalafiloRESUMEN
PURPOSE: Many infants develop a postsurgical chylothorax after diaphragmatic hernia repair. The pathogenesis remains elusive but may be owing to dysfunctional lymphatic development. This study characterizes pulmonary lymphatic development in the nitrofen mouse model of CDH. METHODS: CD1 pregnant mice were fed nitrofen/bisdiamine (N/B) or olive oil at E8.5. At E14.5 and E15.5, lung buds were categorized by phenotype: normal, N/B without CDH (N/B - CDH), or N/B with CDH (N/B+CDH). Anti-CD31 was used to localize all endothelial cells, while anti-LYVE-1 was used to identify lymphatic endothelial cells in lung buds using immunofluorescence. Differential protein expression of lymphatic-specific markers was analyzed. RESULTS: Lymphatic endothelial cells localized to the mesenchyme surrounding the airway epithelium at E15.5. CD31 and LYVE-1 colocalization identified lymphatic endothelial cells. LYVE-1 expression was upregulated in N/B+CDH lung buds in comparison to N/B - CDH and normal lung buds by immunofluorescence. Western blotting shows that VEGF-D, LYVE-1, Prox-1, and VEGFR-3 expression was upregulated in N/B+CDH lung buds in comparison to N/B - CDH or control lung buds at E14.5. CONCLUSIONS: Lung lymphatics are hyperplastic in N/B+CDH. Upregulation of lymphatic-specific genes suggests that lymphatic hyperplasia plays an important role in dysfunctional lung lymphatic development in the nitrofen mouse model of CDH.
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Células Endoteliales/patología , Hernias Diafragmáticas Congénitas , Pulmón/embriología , Tejido Linfoide/anomalías , Animales , Biomarcadores/metabolismo , Western Blotting , Células Endoteliales/metabolismo , Femenino , Técnica del Anticuerpo Fluorescente , Glicoproteínas/metabolismo , Hernia Diafragmática/inducido químicamente , Hernia Diafragmática/embriología , Hernia Diafragmática/metabolismo , Hernia Diafragmática/patología , Hiperplasia/metabolismo , Pulmón/metabolismo , Pulmón/patología , Tejido Linfoide/embriología , Tejido Linfoide/metabolismo , Tejido Linfoide/patología , Proteínas de Transporte de Membrana , Ratones , Éteres Fenílicos , Molécula-1 de Adhesión Celular Endotelial de Plaqueta/metabolismo , Embarazo , Regulación hacia Arriba , Factor D de Crecimiento Endotelial Vascular/metabolismo , Receptor 3 de Factores de Crecimiento Endotelial Vascular/metabolismoRESUMEN
PURPOSE: Some fetuses with sacrococcygeal teratoma (SCT) develop hydrops, but there is no consensus on an appropriate prognostic marker for poor prognosis. The purpose of this study is to establish predictors of poor prognosis in fetuses with SCT. METHODS: A retrospective review of patients with prenatally diagnosed SCT from 1986 to 2011 was performed. Patients with outcome data and ultrasound exams before 32 weeks gestational age (GA) were included (n=37). Tumor volume-to-fetal weight ratio (TFR) and tumor morphology were assessed as sonographic predictors of poor prognosis. RESULTS: Twelve patients (32%) had good prognosis, and twenty-five patients (68%) had poor prognosis. All patients with poor prognosis had a morphology score ≥ 3, which is a significant predictor of poor prognosis (p <0.0001). TFR was assessed, and a receiver operating characteristic (ROC) analysis identified a cutoff value of 0.12 before 24 weeks GA and 0.11 before 32 weeks GA as predictors for poor prognosis. TFR is a significant predictor of poor prognosis (p<0.0001). CONCLUSIONS: Patients with cystic SCT all had good prognosis. TFR >0.12 was validated as a sonographic predictor of poor prognosis. TFR and tumor morphology can be used to counsel expectant families with prenatally diagnosed SCT regarding prognosis.
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Teratoma/patología , Carga Tumoral , Ultrasonografía Prenatal , Femenino , Desarrollo Fetal , Estudios de Seguimiento , Humanos , Hidropesía Fetal/etiología , Recién Nacido , Embarazo , Pronóstico , Curva ROC , Estudios Retrospectivos , Región Sacrococcígea , Tasa de Supervivencia , Teratoma/complicaciones , Teratoma/diagnóstico por imagen , Teratoma/mortalidadRESUMEN
This article reviews fetal intervention for congenital anomalies, which has evolved from a mere concept to a medical specialty over the past 3 decades. Advances in surgical techniques have paralleled developments in fetal imaging, fetal diagnosis, and the advent of maternal tocolysis to prevent preterm labor. Fetal intervention has become an important option for fetuses who would otherwise not survive gestation or who would endure significant morbidity and mortality after birth. However, there were many trials and tribulations as fetal surgery developed into a medical specialty.
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Anomalías Congénitas/cirugía , Feto/cirugía , Anomalías Congénitas/diagnóstico , Cirugía General/tendencias , Humanos , Diagnóstico PrenatalRESUMEN
Congenital diaphragmatic hernia (CDH) is a common birth anomaly. Absence or presence of liver herniation and determination of lung-to-head ratio are the most accurate predictors of prognosis for fetuses with CDH. Though open fetal CDH repair has been abandoned, fetal endoscopic balloon tracheal occlusion promotes lung growth in fetuses with severe CDH. Although significant improvements in lung function have not yet been shown in humans, reversible or dynamic tracheal occlusion is promising for select fetuses with severe CDH. This article reviews advances in prenatal diagnosis of CDH, the experimental basis for tracheal occlusion, and its translation into human clinical trials.
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Oclusión con Balón/métodos , Fetoscopía/métodos , Hernias Diafragmáticas Congénitas , Pulmón/diagnóstico por imagen , Tráquea/diagnóstico por imagen , Femenino , Hernia Diafragmática/diagnóstico , Hernia Diafragmática/terapia , Humanos , Pulmón/fisiopatología , Embarazo , Diagnóstico Prenatal , Pronóstico , Tráquea/cirugía , Ultrasonografía PrenatalRESUMEN
We describe a rare case of multiple intestinal atresias, congenital bilateral perisylvian polymicrogyria, and chronic pulmonary hypertension in a surviving monochorionic twin with co-twin demise. This constellation of congenital anomalies represents a multiple vascular disruption syndrome due to intrauterine vascular compromise in the setting of possible twin-to-twin transfusion syndrome.
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Enfermedades en Gemelos/complicaciones , Hipertensión Pulmonar/etiología , Discapacidad Intelectual/complicaciones , Atresia Intestinal/complicaciones , Malformaciones del Desarrollo Cortical/complicaciones , Enfermedades Vasculares/complicaciones , Anomalías Múltiples , Corion , Enfermedad Crónica , Humanos , Recién Nacido , Atresia Intestinal/clasificación , Masculino , SíndromeRESUMEN
Congenital pulmonary hypoplasia is a devastating condition affecting fetal and newborn pulmonary physiology, resulting in great morbidity and mortality. The fetal lung develops in a fluid-filled environment. In this work, we describe a novel, implantable pressure sensing and recording device which we use to study the pressures present in the fetal pulmonary tree throughout gestation. The system achieves 0.18 cm H2O resolution and can record for twenty one days continuously at 256 Hz. Sample tracings of in vivo fetal lamb recordings are shown.
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Monitoreo Fetal/instrumentación , Feto/cirugía , Pulmón/embriología , Prótesis e Implantes , Transductores de Presión , Animales , Ingeniería Biomédica/instrumentación , OvinosRESUMEN
INTRODUCTION: Fibroinflammatory biliary stricture (FIBS) is a rare benign tumor-like process of the extrahepatic bile duct that masquerades as cholangiocarcinoma. METHODS: In order to distinguish this unusual entity from cancer, we performed a systematic analysis of 11 patients with FIBS. All patients presented with jaundice; six patients had coexisting autoimmune disease. Preoperative evaluation included computed tomography scan and endoscopic retrograde cholangiopancreatography with benign brush cytology. Surgical treatment included nine bile duct resections with five concurrent liver resections and two incisional biopsies. Light microscopy demonstrated fibrous lesions admixed with chronic inflammation. RESULTS AND DISCUSSION: Immunohistochemistry demonstrated smooth muscle actin expression in all lesions except one; five tumors exhibited IgG4 positive plasma cells. The lesions were negative for cytokeratin, ALK1, CD21, S100, Ki67, and p53. Six patients received postoperative immunosuppression. At 41 month median follow-up (range 15-58 months), there was no evidence of recurrent FIBS in ten patients, while one was lost to follow-up. CONCLUSION: FIBS is a rare myofibroblastic lesion with an immunohistochemical profile distinct from other epithelial and stromal neoplasms of the extrahepatic bile duct. A subset of these cases appear to represent IgG4-related sclerosing cholangitis. Because preoperative cytology is not diagnostic of FIBS, surgical resection remains the mainstay of diagnosis and treatment, while immunosuppression may reduce the risk of recurrence.