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PURPOSE: To describe our experience in performing transfemoral-transcaval liver biopsy (TFTC) and transjugular liver biopsy (TJLB) in patients with Fontan-associated liver disease (FALD). METHODS: A single-center, retrospective review of 23 TFTC and seven TJLB performed between August 2011 and May 2023 on patients who previously underwent the Fontan procedure (median age 23.1 years, ranging 11-43 years, 48% female). Patient demographics, laboratory values, pathology, radiology, and cardiology reports were reviewed. Liver explants were correlated with histopathological evaluation to determine sampling accuracy when available. RESULTS: All biopsies achieved technical success (accurate targeting and safe tissue sample extraction) and histopathological success (yielding sufficient tissue for accurate diagnosis). Liver biopsies were performed during simultaneous cardiac catheterization in 28 of 30 (93%) procedures. There was no statistically significant change in hemoglobin, hematocrit, platelet count post-procedure, and fluoroscopy times. There was one major complication within the TJLB group and one minor complication within the TFTC group. CONCLUSION: Transvenous liver biopsies, whether via transfemoral or transjugular route, may be safely performed in FALD patients while yielding samples with technical and histopathological success. The transfemoral approach, which is our preferred method; its compatibility with simultaneous cardiac catheterization and its potentially increased safety profile stemming from the avoidance of transversing the Fontan shunt-makes it a particular advantageous option in the management of FALD.
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Procedimiento de Fontan , Venas Yugulares , Hepatopatías , Hígado , Humanos , Procedimiento de Fontan/efectos adversos , Femenino , Masculino , Estudios Retrospectivos , Adolescente , Adulto , Niño , Venas Yugulares/patología , Hígado/patología , Hígado/diagnóstico por imagen , Hepatopatías/patología , Hepatopatías/etiología , Biopsia/métodos , Adulto Joven , Cateterismo Cardíaco/métodos , Arteria Femoral/patología , Arteria Femoral/cirugíaRESUMEN
STUDY OBJECTIVE: Data regarding uterine artery embolization (UAE) to specifically treat anticoagulant-associated iatrogenic abnormal uterine bleeding (AUB-I) are sparse. This manuscript aimed to quantify the effectiveness of UAE in treating this subset of patients. DESIGN: Retrospective case series. SETTING: Academic hospital serving a large minority-majority population. PATIENTS: Twenty-four patients with AUB-I that was provoked or aggravated by the initiation of anticoagulation therapy. INTERVENTIONS: Treatment of anticoagulant-associated AUB-I that failed medical management or was acute with UAE rather than inferior vena cava filter placement and hysterectomy. MEASUREMENTS AND MAIN RESULTS: An imaging database search was performed to identify patients who underwent UAE for anticoagulant-associated AUB-I from May 2011 to July 2020. Medical and radiologic records were reviewed. Short- and long-term outcomes were obtained to date, ranging from 10 months to 10 years after the procedure. In total, 24 patients were identified, ranging in age from 35 to 54 (mean 44.9) years. Venous thromboembolic disease was the most common (92%) indication for anticoagulation. At presentation, 14 patients (58%) were anticipated to require lifelong anticoagulation. Most UAE procedures (54%) occurred within 10 days of anticoagulation initiation. Before UAE, 17 patients (71%) attempted and failed medical management, myomectomy, or endometrial ablation to control bleeding. After UAE, 21 patients (88%) experienced substantial improvement or resolution of AUB and continued anticoagulation therapy. Three patients (14%) did not experience improvement and were treated with hysterectomy. Amenorrhea immediately after UAE occurred in 1 patient at age 45. CONCLUSION: UAE was an effective tool in the management of anticoagulant associated AUB-I in this cohort, resulting in decreased bleeding while allowing the continuation of anticoagulation therapy, with high rates of uterine preservation and preserved menses.
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Embolización de la Arteria Uterina , Neoplasias Uterinas , Adulto , Anticoagulantes/efectos adversos , Femenino , Humanos , Persona de Mediana Edad , Estudios Retrospectivos , Resultado del Tratamiento , Embolización de la Arteria Uterina/efectos adversos , Hemorragia Uterina/inducido químicamente , Hemorragia Uterina/terapia , Neoplasias Uterinas/complicaciones , Neoplasias Uterinas/terapiaRESUMEN
PURPOSE: To assess device and procedural safety and technical success associated with the use of the AngioVac System to remove vascular thrombi and cardiac masses. MATERIALS AND METHODS: The Registry of AngioVac Procedures in Detail (RAPID) study prospectively collected data for 234 patients receiving treatment with AngioVac at 21 sites between March 2016 and August 2019: 84 (35.9%) with caval thromboemboli (CTEs), 113 (48.3%) with right heart masses (RHMs), 20 (8.5%) with catheter-related thrombi (CRTs), and 4 (1.7%) with pulmonary emboli (PEs). Thirteen patients had a combination of procedures during the same admission. RESULTS: Using the AngioVac system, 70%-100% thrombus or mass removal was achieved in 73.6% of patients with CTEs, 58.5% of patients with RHMs, 60% of patients with CRTs, and 57.1% of patients with PEs. Extracorporeal bypass time was < 1 hour for 176 (75.2%) procedures. Estimated blood loss was < 250 mL for 179 procedures (76.5%). Mean hemoglobin decreased from 10.4 g/dL ± 2.9 preoperatively to 9.4 g/dL ± 2.6 postoperatively. Transfusions were administered in 59 procedures (25.2%) with 47 transfusions (78.2%) being ≤ 2 U. There were 36 procedure-related complications, including 1 death. CONCLUSIONS: The RAPID registry data demonstrate that the AngioVac System can be safely and effectively used to remove vascular thrombi and cardiac masses across a broad range of patient populations. The limited use of the device to remove pulmonary emboli in the present series precludes recommending the use of the AngioVac device for this indication.
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Procedimientos Endovasculares/instrumentación , Cardiopatías/terapia , Trombectomía/instrumentación , Tromboembolia/terapia , Trombosis/terapia , Adulto , Anciano , Procedimientos Endovasculares/efectos adversos , Diseño de Equipo , Femenino , Cardiopatías/diagnóstico por imagen , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Sistema de Registros , Medición de Riesgo , Factores de Riesgo , Trombectomía/efectos adversos , Tromboembolia/diagnóstico por imagen , Trombosis/diagnóstico por imagen , Factores de Tiempo , Resultado del TratamientoRESUMEN
Importance: Hepatocellular carcinoma (HCC) is a heterogeneous disease with many available treatment modalities. Transarterial chemoembolization (TACE) is a valuable treatment modality for HCC lesions. This article seeks to evaluate the utility of additional ablative therapy in the management of patients with HCC who received an initial TACE procedure. Objective: To compare the overall survival (OS) and freedom from local progression (FFLP) outcomes after TACE alone with TACE that is followed by an ablative treatment regimen using stereotactic body radiation therapy, radiofrequency ablation, or microwave ablation for patients with HCC. Design, Setting, and Participants: This cohort study of 289 adults at a single urban medical center examined survival outcomes for patients with nonmetastatic, unresectable HCC who received ablative therapies following TACE or TACE alone from January 2010 through December 2018. The Lee, Wei, Amato common baseline hazard model was applied for within-patient correlation with robust variance and Cox regression analysis was used to assess the association between treatment group (TACE vs TACE and ablative therapy) and failure time events (FFLP per individual lesion and OS per patient), respectively. In both analyses, the treatment indication was modeled as a time-varying covariate. Landmark analysis was used as a further sensitivity test for bias by treatment indication. Exposures: TACE alone vs TACE followed by ablative therapy. Main Outcomes and Measures: Freedom from local progression and overall survival. Hypotheses were generated before data collection. Results: Of the 289 patients identified, 176 (60.9%) received TACE only and 113 (39.1%) received TACE plus ablative therapy. Ablative therapy included 45 patients receiving stereotactic body radiation therapy, 39 receiving microwave ablation, 20 receiving radiofrequency ablation, and 9 receiving a combination of these following TACE. With a median (interquartile range) follow-up of 17.4 (9.5-29.5) months, 242 of 512 (47.3%) lesions progressed, 211 in the group with TACE alone and 31 in the group with TACE plus ablative therapy (P < .001). Over 3 years, FFLP was 28.1% for TACE alone vs 67.4% for TACE with ablative therapy (P < .001). The 1-year and 3-year OS was 87.5% and 47.1% for patients with lesions treated with TACE alone vs 98.7% and 85.3% for patients where any lesion received TACE plus ablative therapy, respectively (P = .01), and this benefit remained robust on landmark analyses at 6 and 12 months. The addition of ablative therapy was independently associated with OS on multivariable analysis for all patients (hazard ratio, 0.26; 95% CI, 0.13-0.49; P < .001) and for patients with Barcelona clinic liver cancer stage B or C disease (hazard ratio, 0.31; 95% CI, 0.14-0.69; P = .004). Conclusions and Relevance: Adding ablative therapy following TACE improved FFLP and OS among patients with hepatocellular carcinoma. This study aims to guide the treatment paradigm for HCC patients until results from randomized clinical trials become available.
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Carcinoma Hepatocelular/terapia , Ablación por Catéter/estadística & datos numéricos , Quimioembolización Terapéutica/estadística & datos numéricos , Neoplasias Hepáticas/terapia , Anciano , Carcinoma Hepatocelular/mortalidad , Carcinoma Hepatocelular/patología , Terapia Combinada/métodos , Femenino , Humanos , Neoplasias Hepáticas/mortalidad , Neoplasias Hepáticas/patología , Masculino , Persona de Mediana Edad , Supervivencia sin Progresión , Estudios RetrospectivosRESUMEN
PURPOSE: To compare the safety and efficacy of transfemoral transcaval liver biopsies (TFTC) with that of transjugular liver biopsies (TJLB) at a single tertiary-care institution. MATERIALS AND METHODS: A retrospective review was performed of 500 consecutive transvenous liver biopsies between December 2010 and December 2018. The cases included 286 TFTC patients at a median age of 54 years old (interquartile range [IQR], 42-63 years of age), 37.4% were female; and 214 TJLB patients at a median age of 55 years old (IQR, 46-61 years of age), 45.4% female. Patient demographic and laboratory data and technical and histopathological success, fluoroscopy times, and complications were recorded. Comparative statistical analyses were performed using a 2-sample test or a Wilcoxon ranked sum test for continuous variables and a chi-square test or Fisher exact test for categorical variables when appropriate. RESULTS: TFTC and TJLB data are presented as: technical success rates of 99.3% (283 of 286) and 100% (214 of 214), respectively; histopathologic success rates of 96.5% (275 of 285) and 95.8% (205 of 214), respectively; and major complication rates of 1.4% (4 of 284) and 5.6% (12 of 214), respectively (P = .009). There were no hepatic injuries in the TFTC group, whereas the TJLB group included 6 significant hepatic injuries requiring intervention. Median fluoroscopic times were 5.5 minutes (IQR, 3.9-8.6 minutes) for TFTC and 8.1 minutes (IQR, 5.2-13.1) for TJLB (P < .001). CONCLUSIONS: In this single-institution study, TFTC was associated with a lower major complication rate and lower fluoroscopy times than conventional TJLB with similar technical and histopathologic successes.
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Cateterismo Venoso Central , Cateterismo Periférico , Vena Femoral , Venas Yugulares , Hígado/patología , Adulto , Biopsia , Cateterismo Venoso Central/efectos adversos , Cateterismo Periférico/efectos adversos , Femenino , Vena Femoral/diagnóstico por imagen , Fluoroscopía , Humanos , Venas Yugulares/diagnóstico por imagen , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Punciones , Radiografía Intervencional , Estudios Retrospectivos , Factores de TiempoRESUMEN
BACKGROUND: Coronavirus disease 2019 (COVID-19) is associated with a coagulopathy favouring thrombosis over bleeding that imparts a poor prognosis. Clot in transit (CIT) is considered a rare entity and the most severe form of venous thromboembolism (VTE), carrying a higher mortality than isolated pulmonary embolism (PE). The incidence of this phenomenon in patients with COVID-19 infection is unknown and likely under-recognized. CASE SUMMARY: During the peak of the COVID-19 pandemic in New York City, a 70-year-old Hispanic female presented with syncope due to a saddle PE further complicated by a highly mobile CIT. Polymerase chain reaction was positive for COVID-19 infection, however, there was no evidence of lung parenchymal involvement or hyper-inflammation. Based on consensus from a multidisciplinary team, aspiration thrombectomy was attempted to treat this extreme case of VTE, however, the patient died during the procedure. DISCUSSION: This case raises awareness to the most catastrophic form of VTE, presenting in an early phase of COVID-19 infection without the typical hyper-inflammation and severe lung injury associated with development of COVID-related coagulopathy. It also serves to inform on the critical role echocardiography has in the comprehensive evaluation and re-evaluation of hospitalized patients with COVID-19, and the importance of a multidisciplinary organized approach in clinical decision-making for this complex and poorly understood disease and its sequelae.
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Benign biliary strictures are uncommon in children. Classically, these cases are managed surgically, however less invasive approaches with interventional radiology and or endoscopy may have similar results and improved safety profiles While benign biliary strictures have been described in literature on several occasions in young children, (most older than 1 year and once in an infant 3 months of age), all reported cases were managed surgically. We present two cases of benign biliary strictures in infants less than 6 months of age that were managed successfully with novel non-invasive procedures and a review of all current pediatric cases reported in the literature. Furthermore, we describe the use of a Rendezvous procedure, which has not been reported as a treatment approach for benign biliary strictures.
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Hepatocellular cancer (HCC) is most common primary liver malignancy in adults. Treatment for HCC is a multispecialty undertaking, with surgical, locoregional, and systemic options available. Choice of treatment depends upon patient and disease factors. Surgical therapy, including resection and transplantation, is the primary curative treatment and is best suited to patients with early disease. More advanced disease may be amenable to locoregional therapies to "bridge" to transplantation, downstage disease, or as destination therapy for unresectable cases. These include percutaneous ablation, transarterial therapy, external radiation, and radioembolization with yttrium-90 conjugated beads. Patients with more advanced disease may benefit most from systemic chemotherapeutic or small molecule inhibitor options available, many of which have only been recently FDA approved. Immunotherapy is the newest component of HCC treatment. The Y-90 consultant should be familiar with all modalities of HCC treatment and the interplay between them.
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Neoplasias Hepáticas/tratamiento farmacológico , Neoplasias Hepáticas/cirugía , Microesferas , Radioisótopos de Itrio/química , Radioisótopos de Itrio/uso terapéutico , Humanos , Neoplasias Hepáticas/radioterapiaRESUMEN
PURPOSE: To describe the utility of post-procedure noncontrast cone-beam computed tomography (CT) in identifying cases of incomplete treatment and the need to search for additional vascular supply during uterine artery embolization (UAE). MATERIALS AND METHODS: From June 2013 to June 2018, 427 patients (age, 45 ± 5 years) underwent 430 consecutive UAEs with post-embolization noncontrast cone-beam CT. If noncontrast cone-beam CT showed an area of the uterus lacking contrast retention, aortography was performed to search for collateral supply. Procedures were characterized as suspected complete bilateral UAEs or suspected incomplete UAEs, such as in cases of a unilateral uterine artery or diminutive uterine arteries. Rates of inadequate contrast retention on noncontrast cone-beam CT and discovered collateral artery supply were calculated. In 10 consecutive cases in which both noncontrast cone-beam CT and aortography were performed, dose-area product radiation exposure from noncontrast cone-beam CT and aortography was compared using a 2-sided paired-sample t-test. RESULTS: Of the 411 suspected complete bilateral UAEs, noncontrast cone-beam CT showed an area of the uterus lacking contrast retention in 38 (9.2%) cases. Of the 19 suspected incomplete UAEs, noncontrast cone-beam CT demonstrated incomplete treatment in 6 (31.6%) patients. Aortography was performed in 40 of the 44 cases of incomplete treatment on noncontrast cone-beam CT, and collateral supply was found in 28 (70.0%) cases. In 22 of these cases (5.2% of the 427 patients studied), noncontrast cone-beam CT led to the discovery of significant collateral supply requiring further embolization. Dose-area product radiation exposure from noncontrast cone-beam CT was less than from aortography (P = .007). CONCLUSIONS: Post-UAE noncontrast cone-beam CT can be used to select a subset of patients with a higher likelihood of collateral supply who may benefit from post-embolization aortography.
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Resinas Acrílicas/administración & dosificación , Angiografía por Tomografía Computarizada/métodos , Tomografía Computarizada de Haz Cónico , Gelatina/administración & dosificación , Leiomioma/sangre , Leiomioma/terapia , Embolización de la Arteria Uterina , Neoplasias Uterinas/irrigación sanguínea , Neoplasias Uterinas/terapia , Resinas Acrílicas/efectos adversos , Adulto , Toma de Decisiones Clínicas , Circulación Colateral , Femenino , Gelatina/efectos adversos , Humanos , Leiomioma/diagnóstico por imagen , Leiomioma/patología , Persona de Mediana Edad , Selección de Paciente , Valor Predictivo de las Pruebas , Dosis de Radiación , Estudios Retrospectivos , Factores de Riesgo , Resultado del Tratamiento , Embolización de la Arteria Uterina/efectos adversos , Neoplasias Uterinas/diagnóstico por imagen , Neoplasias Uterinas/patologíaAsunto(s)
Fístula Arteriovenosa/etiología , Arteria Hepática/lesiones , Enfermedad Iatrogénica , Isquemia/etiología , Trasplante de Hígado/efectos adversos , Vena Porta/lesiones , Estómago/irrigación sanguínea , Lesiones del Sistema Vascular/etiología , Fístula Arteriovenosa/diagnóstico por imagen , Fístula Arteriovenosa/fisiopatología , Fístula Arteriovenosa/terapia , Embolización Terapéutica , Endoscopía del Sistema Digestivo , Femenino , Arteria Hepática/diagnóstico por imagen , Arteria Hepática/fisiopatología , Humanos , Isquemia/diagnóstico por imagen , Isquemia/fisiopatología , Circulación Hepática , Angiografía por Resonancia Magnética , Persona de Mediana Edad , Vena Porta/diagnóstico por imagen , Vena Porta/fisiopatología , Resultado del Tratamiento , Lesiones del Sistema Vascular/diagnóstico por imagen , Lesiones del Sistema Vascular/fisiopatología , Lesiones del Sistema Vascular/terapiaRESUMEN
PURPOSE: To describe the technique and outcome of transfemoral transcaval (TFTC) core-needle liver biopsies. MATERIALS AND METHODS: Retrospective chart review was performed on 121 patients who underwent transvenous liver biopsies at a single institution between February 2014 and July 2015, yielding 66 total TFTC liver biopsies for review (65.2% male; mean age, 53.2 y ± 15.0). From August 2014 through July 2015, TFTC biopsies accounted for 64 of 77 (83%) transvenous biopsies. Hepatic tissue was obtained directly through the intrahepatic inferior vena cava from a femoral venous approach. Procedural complications were classified according to Society of Interventional Radiology guidelines. RESULTS: Of the 66 biopsies, technical success was achieved in 64 cases (97.0%). Histopathologic diagnoses were made in 63 cases (95.5%). Fragmented or limited specimens in which a pathologic diagnosis was still made occurred in four cases (6.1%). Complications occurred in two cases (3.0%). Venous pressure measurements were requested in 60 cases, and all were successfully obtained. CONCLUSIONS: TFTC core-needle liver biopsies are feasible and safe as demonstrated in this series of patients.
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Biopsia con Aguja Gruesa/métodos , Cateterismo Periférico/métodos , Vena Femoral , Hepatopatías/patología , Hígado/patología , Vena Cava Inferior , Adulto , Anciano , Biopsia con Aguja Gruesa/efectos adversos , Cateterismo Periférico/efectos adversos , Estudios de Factibilidad , Femenino , Vena Femoral/diagnóstico por imagen , Humanos , Hepatopatías/fisiopatología , Masculino , Persona de Mediana Edad , Flebografía , Valor Predictivo de las Pruebas , Radiografía Intervencional , Estudios Retrospectivos , Tomografía Computarizada por Rayos X , Ultrasonografía Intervencional , Vena Cava Inferior/diagnóstico por imagen , Presión VenosaRESUMEN
Inferior vena cava (IVC) thrombosis, although similar in many aspects to deep venous thrombosis (DVT), has distinct clinical implications, treatments and roles for endovascular management. Etiologies of IVC thrombosis vary from congenital malformations of the IVC to acquired, where indwelling IVC filters have been implicated as a leading cause. With an increasing incidence of IVC thrombosis throughout the United States, clinicians need to be educated on the clinical signs and diagnostic tools available to aid in the diagnosis as well as available treatment options. Untreated IVC thrombus can result in serious morbidity and mortality, both in the acute phase with symptoms related to venous outflow occlusion and embolism, and in the long-term, sequelae of post-thrombotic syndrome (PTS) related to chronic venous occlusion. This manuscript will discuss the clinical presentation of IVC thrombosis, diagnostic and treatment options, as well as the role of endovascular management.
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Lung cancer remains the malignancy with the highest mortality and second highest incidence in both men and women within the United States. Image-guided ablative therapies are safe and effective for localized control of unresectable liver, renal, bone, and lung tumors. Local ablative therapies have been shown to slow disease progression and prolong disease-free survival in patients who are not surgical candidates, either due to local extent of disease or medical comorbidities. Commonly encountered complications of percutaneous ablation of lung tumors include pneumothorax, pleural inflammation, pleural effusions, and pneumonia, which are usually easily managed. This review will discuss the merits of image-guided ablation in the treatment of lung tumors and the underlying mechanism, procedural techniques, clinical utility, toxicity, imaging of tumor response, and future developments, with a focus on radiofrequency ablation.
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Carcinoma de Pulmón de Células no Pequeñas/cirugía , Ablación por Catéter/métodos , Neoplasias Pulmonares/cirugía , Cirugía Asistida por Computador/métodos , Supervivencia sin Enfermedad , Humanos , Procedimientos Quirúrgicos Mínimamente Invasivos , Resultado del TratamientoAsunto(s)
Hipertensión Portal/complicaciones , Hipertensión Portal/diagnóstico , Hemorragia Uterina/diagnóstico , Hemorragia Uterina/etiología , Adulto , Femenino , Humanos , Derivación Portosistémica Intrahepática Transyugular , Vena Esplénica/diagnóstico por imagen , Vena Esplénica/cirugía , Stents , Trombectomía , Trombosis/complicaciones , Trombosis/diagnóstico por imagen , Trombosis/cirugía , Tomografía Computarizada por Rayos X , Resultado del TratamientoRESUMEN
PURPOSE: To retrospectively evaluate the presence and distribution patterns of contrast agent retention in the liver on noncontrast computed tomography (CT) immediately following chemoembolization with drug-eluting beads (DEBs). MATERIALS AND METHODS: From 2008 to 2010, 95 patients with 224 liver lesions had chemoembolization performed with DEBs and a noncontrast CT examination of the liver performed immediately after embolization. Of these, 85 patients with 193 lesions were included. The postembolization CT scan was reviewed by a diagnostic radiologist, and the presence of contrast agent retention within the lesion was assessed. Varying patterns of contrast agent retention were defined. RESULTS: Of the 193 lesions included, 146 (76%) retained contrast medium. Aside from some contrast medium in vessels, very little if any contrast medium was seen in the surrounding liver. Various patterns of contrast agent retention were noted within lesions. In a single case, repeat imaging was obtained 6 hours later, which demonstrated washout of contrast agent in a lesion that had retained contrast agent on the postprocedure CT scan. Of significance, 13 additional foci of contrast agent retention were identified on postchemoembolization CT scans that, on retrospective review of preprocedure imaging, represented enhancing lesions not previously identified. CONCLUSIONS: Noncontrast CT after chemoembolization with DEBs demonstrates contrast agent retention in 76% of cases, without significant contrast medium seen in the adjacent liver parenchyma. The presence or absence of contrast agent retention may prove to be useful in evaluating accurate targeting of a lesion.
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Carcinoma Hepatocelular/diagnóstico por imagen , Carcinoma Hepatocelular/terapia , Quimioembolización Terapéutica , Medios de Contraste , Neoplasias Hepáticas/diagnóstico por imagen , Neoplasias Hepáticas/terapia , Tomografía Computarizada por Rayos X , Antibióticos Antineoplásicos/administración & dosificación , Carcinoma Hepatocelular/irrigación sanguínea , Doxorrubicina/administración & dosificación , Portadores de Fármacos , Aceite Etiodizado/administración & dosificación , Humanos , Neoplasias Hepáticas/irrigación sanguínea , New York , Valor Predictivo de las Pruebas , Estudios Retrospectivos , Factores de Tiempo , Resultado del Tratamiento , Ácidos TriyodobenzoicosAsunto(s)
Antineoplásicos/administración & dosificación , Carcinoma Hepatocelular/diagnóstico por imagen , Carcinoma Hepatocelular/tratamiento farmacológico , Cateterismo , Quimioembolización Terapéutica/instrumentación , Neoplasias Hepáticas/diagnóstico por imagen , Neoplasias Hepáticas/tratamiento farmacológico , Quimioembolización Terapéutica/métodos , Femenino , Humanos , Persona de Mediana Edad , Radiografía Intervencional/instrumentación , Radiografía Intervencional/métodos , Resultado del Tratamiento , Vísceras/diagnóstico por imagenRESUMEN
Gaucher disease is the most common lysosomal storage disease. Enzyme replacement therapy engenders improvement in hematological and visceral parameters; however, improvement in bone density (BMD) with treatment has not been confirmed. This study presents follow-up of BMD in the first ten patients in Israel treated with low-dose recombinant enzyme for up to 108 months. BMD at femoral neck and lumbar spine was determined by dual-energy X-ray absorptiometry (DEXA) at the start of the trial, after 3-6 months, after 18-24 months, and at the most recent follow-up. BMD in all patients was very low at onset and never normalized. There was a decrease in BMD in all patients at 3-6 months. Older patients (four women, two men; >30 years of age) showed some improvement in BMD during treatment. Younger patients (four females; 18-23 years of age) did not show a statistically significant improvement. These findings might reflect the failure of patients with Gaucher disease to achieve expected peak bone density at appropriate chronological milestones despite treatment. Nonetheless, the z-scores of the older patients were better than those of the younger patients, implying some catch-up period. Yet, some patients with Gaucher disease evince rapid onset of osteoporosis in early adulthood. Enzyme treatment per se, as well as attendant improved well-being and increased physical activity, may induce amelioration in BMD at this later stage. One may consider adding anti-osteoporosis therapy in young adults to induce earlier "catch up" to peak bone mass, and then enzyme replacement in later adulthood to prevent decrements in bone mass related to Gaucher cell infiltration.