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2.
Rev. colomb. gastroenterol ; 35(3): 280-286, jul.-set. 2020. tab, graf
Article in Spanish | LILACS | ID: biblio-1138784

ABSTRACT

Resumen El síndrome de Budd-Chiari (Budd-Chiari syndrome, BCS) es una entidad rara, definida por la obstrucción del flujo venoso del hígado y corresponde a una condición heterogénea tanto en presentación como en manejo terapéutico. A continuación, presentamos un estudio retrospectivo de pacientes con diagnóstico de BCS, atendidos por el grupo de hepatología del el Hospital Pablo Tobón Uribe. Los resultados se expresan como proporciones en las variables categóricas y como medias y rangos para las variables continuas. La edad promedio de presentación fue de 37,7 años y no hubo preponderancia por el género. Los principales síntomas fueron dolor abdominal (91,4 %), ascitis (71,4 %) y hepatomegalia (60 %). Asimismo, 11 pacientes (31,4 %) presentaron hipercoagulabilidad primaria, mientras que en 7 (20 %) se encontró algún síndrome mieloproliferativo. Además, 6 (17,1 %) tenían neoplasias extrahepáticas: 3 con compresión mecánica y 3 por hipercoagulabilidad asociada; solo en 1 caso se documentaron membranas en la vena cava inferior. Y en 5 casos no fue posible determinar la causa de trombosis. De igual forma, 32 pacientes (91,4 %) recibieron anticoagulación como primer manejo; solo 4 sujetos fueron remitidos a procedimientos: 2 a angioplastia, 1 a derivación portosistémica intrahepática transyugular (Transjugular Intrahepatic Portosystemic Shunt, TIPS) y 1 a trasplante ortotópico hepático (TOH). Luego de ello, 17 (48,5 %) recibieron procedimientos invasivos como segunda línea: 8 (22,8 %) se condujeron a angioplastia y endoprótesis (stent), 5 (14,29 %) a TIPS y 4 necesitaron TOH. Así, los 6 pacientes que se llevaron a TIPS no necesitaron trasplante hepático. El BCS continúa siendo una condición poco frecuente que afecta la salud de personas jóvenes, tanto hombres como mujeres, con una presentación clínica heterogénea. Sin embargo, en la mayoría de los casos ocurre por una causa desencadenante clara, entre las cuales predominan las trombofilias y los síndromes mieloproliferativos. El uso de medidas terapéuticas invasivas, especialmente de la selección temprana de los enfermos que se benefician de TIPS, ha cambiado la historia natural y el pronóstico de este grupo de pacientes.


Abstract Budd-Chiari syndrome (BCS) is a rare disorder characterized by the obstruction of the veins of the liver. Both its presentation and therapeutic management are heterogenous. The following is a retrospective study of patients diagnosed with BCS treated by the hepatology service at the Hospital Pablo Tobón Uribe. The results of the categorical variables are expressed as proportions and the continuous variables as means and ranges. The average age of onset of this disease was 37.7 years and it was not predominant in neither sex. The most common symptoms were abdominal pain (91.4%), ascites (71.4%) and hepatomegaly (60%). Out of 35 patients, 11 (31.4%) had primary hypercoagulability, 7 (20%) had some myeloproliferative disorder, 6 (17.1%) had extrahepatic malignancies -3 with mechanical compression and 3 due to associated hypercoagulability-, and 1 case had a membranous obstruction of the inferior vena cava. 32 patients (91.4%) received anticoagulation as first-line therapy and only 4 were taken to surgery: 2 to angioplasty, 1 to transjugular intrahepatic portosystemic shunt (TIPS) and 1 to orthotopic liver transplantation (OLT). On the other hand, 17 (48.5%) individuals underwent invasive procedures as a second-line therapy, of which 8 (22.8%) underwent angioplasty and stenting, 5 (14.29%) underwent TIPS and 4 needed OLT. The 6 patients who were taken to TIPS did not need liver transplants. Budd-Chiari syndrome remains a rare condition affecting the health of young men and women alike, with a heterogeneous clinical presentation but, in most cases, with a clear trigger where thrombophilic and myeloproliferative disorders predominate. The use of invasive therapeutic measures, especially the early selection of patients who may benefit from TIPS, has changed the natural course and prognosis of this group of patients.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Patients , Therapeutics , Diagnosis , Budd-Chiari Syndrome , Hospitals , Methods , Ascites , Signs and Symptoms , Abdominal Pain , Hepatomegaly
3.
Rev. colomb. gastroenterol ; 34(3): 297-302, jul.-set. 2019. tab, graf
Article in Spanish | LILACS | ID: biblio-1042818

ABSTRACT

Resumen El síndrome de Budd-Chiari es una entidad definida como la obstrucción al flujo sanguíneo en el tracto de salida hepático. Esta obstrucción se puede localizar desde las pequeñas venas hepáticas hasta la entrada de la vena cava inferior a la aurícula derecha. En la mayoría de los casos, se ocasiona por una trombosis endoluminal conocida como Budd-Chiari primario y secundario, cuando ocurre por una compresión extrínseca asociada a las lesiones que ocupan espacio como malignidad. Por tanto, los estados trombofílicos hereditarios se conocen como los principales factores de riesgo. El embarazo y el puerperio, al ser estados hipercoagulables, se pueden asociar al síndrome de Budd-Chiari, sin embargo, la prevalencia reportada en la literatura es muy variable dependiendo de la población estudiada. En Colombia no hay estudios de incidencia o prevalencia de la enfermedad; existen varios reportes de caso, pero no relacionados con el embarazo. Reportamos el caso de una paciente, que 12 semanas posparto presentó un síndrome de Budd-Chiari; además presentamos el manejo ofrecido, la evolución clínica y una revisión de la literatura de los casos asociados con el embarazo.


Abstract Budd-Chiari syndrome is defined as obstruction of hepatic blood outflow. This obstruction can be located anywhere from the small hepatic veins at the entrance of the inferior vena cava to the right atrium. Most cases are primary Budd-Chiari syndrome which is caused by endoluminal thrombosis. Secondary Budd-Chiari syndrome occurs as the result of extrinsic compression associated with space-occupying lesions such as malignant tumors. Hereditary thrombophilic states are the main risk factors, but since pregnancy and the puerperium are hypercoagulable states, they can be associated with Budd-Chiari syndrome. Nevertheless, the prevalence of this type of case in the literature varies according to the population studied. There have been no studies on the incidence or prevalence of this disease in Colombia. The small number of case reports here have not been related to pregnancy. We report the case of a patient who developed Budd-Chiari syndrome 12 weeks postpartum. Our report includes management and clinical evolution as well as a review of the literature of cases associated with pregnancy.


Subject(s)
Humans , Female , Adolescent , Postpartum Period , Budd-Chiari Syndrome , Thrombosis , Literature
4.
Rev. méd. Urug ; 35(1): 59-65, mar. 2019. tab
Article in Spanish | LILACS | ID: biblio-982060

ABSTRACT

Introducción: el síndrome de Budd-Chiari es una entidad rara definida por la obstrucción al flujo de salida venoso hepático. Se asocia frecuentemente a un estado protrombótico. El diagnóstico y tratamiento oportuno representan un reto para el médico clínico. El objetivo del presente trabajo es reportar un caso clínico de síndrome de Budd-Chiari secundario a síndrome antifosfolípido, asociación infrecuente en la literatura. Caso clínico: mujer de 31 años. Ascitis de seis años de evolución, actualmente refractaria. Repercusión general en el último año. Examen físico: lúcida, ictericia universal, hepatomegalia dolorosa, esplenomegalia y ascitis a tensión. Laboratorio: hepatograma con patrón colestásico. Colinesterasa y tasa de protrombina descendidas. Ecografía Doppler abdominal: ascitis severa, hepatomegalia irregular, ecogenicidad aumentada. Esplenomegalia. Obstrucción de vena suprahepática media, estrechamiento de vena suprahepática derecha. Flujo escasamente fásico de baja velocidad, invertido en algunas ramas, hepatófugo. Fibrogastroduodenoscopía: várices esofágicas grado III. Se planteó ascitis secundaria a hipertensión portal, probable síndrome de Budd-Chiari. Los anticuerpos IgM beta2 glicoproteína 1 fueron positivos. Diagnóstico de síndrome antifosfolípido. Se realizó trasplante hepático con buena evolución posterior. Discusión y conclusiones: el síndrome de Budd-Chiari es más frecuente en mujeres entre los 30 y 50 años. Debe considerarse como causa de enfermedad hepática, fundamentalmente cuando no existe otra causa evidente. Los estados protrombóticos que más se asocian al síndrome de Budd-Chiari en Occidente son las enfermedades hematológicas. El tratamiento debe ser individualizado según la presentación clínica. El trasplante hepático se plantea como medida de salvataje cuando el resto de los tratamientos han fracasado.


Introduction: [/sectitle][p]Budd-Chiari is a rare condition characterized by the occlusion of the hepatic vein flow out. It is frequently associated to a pro-thrombotic state. Diagnosis and treatment constitute a challenge for clinical doctors. This study aims to report a clinical case of Budd-Chiari syndrome secondary to an antiphospholipid syndrome, a rather unusual association in literature.[/p] Clinical case: 31 year old woman, with 6 years of evolution ascites, evidencing a general affection in the last year. Clinical examination: lucid, universal hyperbilirubinemia (jaundice), painful hepatomegaly, splenomegaly and tension ascites. Laboratory tests: hepatogram with cholestatic pattern, cholinesterase, and reduced prothrombin rate. Abdominal Doppler revealed: severe ascites, irregular hepatomegaly, increased echogenicity. Splenomegaly. Occlusion of the middle suprahepatic vein, narrowing of the right suprahepatic vein. Llow speed scarcely phasic flow, inverse in a few branches, hepatofugal. Fibrogastroduodenoscopy: esophagic varices grade III. Ascites secondary to portal hypertension was suspected, probable Budd Chiari syndrome. Antibodies IgM â2glicoprotein 1 were positive. Diagnosis of antiphospholipid syndrome. Liver transplant was performed with a good evolution of the patient. Discussion and conclusions:Budd-Chiari syndrome is more frequent in women between 35 and 50 years old. It needs to be regarded as a cause of liver conditions, in particular when there is no other evident cause. Hematologic diseases are the prothrombotic states more frequently associated to Budd-Chiari syndrome in the western world. Treatment must be based on the clinical presentation. Liver transplant is a rescue/salvage measure when all other treatments fail.


Introdução: a síndrome de Budd-Chiari é uma entidade rara definida pela obstrução do fluxo de saída venoso hepática. Frequentemente está associado a um estado protrombótico. O diagnóstico e o tratamento oportuno são um desafio para o médico clínico. O objetivo deste trabalho é descrever um caso clínico de síndrome de Budd-Chiari secundário à síndrome antifosfolípidica, uma associação pouco frequente na literatura. Caso clínico: mulher de 31 anos. Ascite com seis anos de evolução, atualmente refrataria. Repercussão geral no último ano. Exame físico: lúcida, icterícia universal, hepatomegalia dolorosa, esplenomegalia e ascite sob tensão. Laboratório: hepatograma com padrão colestásico. Colinesterase e taxa de protrombina diminuídas. Ultrassonografia Doppler abdominal: ascite severa, hepatomegalia irregular, ecogenicidade aumentada. Esplenomegalia. Obstrução de veia supra-hepática média, estreitamento de veia supra-hepática direita. Fluxo escassamente fásico de baixa velocidade, invertido em algumas ramas, hematófago. Fibrogastroduodenoscopia: varizes esofágicas de grau 3. Diagnóstico presuntivo: ascite secundaria à hipertensão portal, provável síndrome de Budd-Chiari. Anticorpos IgM alfa 2glicoproteína 1 positivos. Diagnóstico de síndrome antifosfolípido. Foi realizado um transplante hepático com boa evolução.


Subject(s)
Humans , Antibodies, Antiphospholipid , Budd-Chiari Syndrome
5.
Article in English | WPRIM | ID: wpr-741455

ABSTRACT

OBJECTIVE: To assess segmental liver stiffness (LS) with MRI before and after endovascular intervention in patients with Budd-Chiari syndrome (BCS). MATERIALS AND METHODS: Twenty-three patients (13 males and 10 females; mean age, 42.6 ± 12.6 years; age range, 31–56 years) with BCS as a primary liver disease were recruited for this study. Two consecutive magnetic resonance elastography (MRE) examinations were performed before the endovascular treatment. Fifteen patients who underwent endovascular intervention treatment also had follow-up MRE scans within three days after the procedure. LS was measured in three liver segments: the right posterior, right anterior, and left medial segments. Inter-reader and inter-exam repeatability were analyzed with intraclass correlation coefficients (ICCs) and Bland-Altman analysis. Segmental LS and clinical characteristics before and after the intervention were also compared. RESULTS: Within three days of the endovascular intervention, all three segmental LS values decreased: LS of the right posterior segment = 7.23 ± 0.88 kPa (before) vs. 4.94 ± 0.84 kPa (after), LS of the right anterior segment = 7.30 ± 1.06 kPa (before) vs. 4.77 ± 0.85 kPa (after), and LS of the left medial segment = 7.22 ± 0.87 kPa (before) vs. 4.87 ± 0.72 kPa (after) (all p = 0.001). There was a significant correlation between LS changes and venous pressure gradient changes before and after treatments (r = 0.651, p = 0.009). The clinical manifestations of all 15 patients significantly improved after therapy. The MRE repeatability was excellent, with insignificant variations (inter-reader, ICC = 0.839–0.943: inter-examination, ICC = 0.765–0.869). Bland-Altman analysis confirmed excellent agreement (limits of agreement, 13.4–19.4%). CONCLUSION: Segmental LS measured by MRE is a promising repeatable quantitative biomarker for monitoring the treatment response to minimally invasive endovascular intervention in patients with BCS.


Subject(s)
Budd-Chiari Syndrome , Elasticity Imaging Techniques , Female , Follow-Up Studies , Humans , Liver Diseases , Liver , Magnetic Resonance Imaging , Male , Venous Pressure
6.
Rev. colomb. cir ; 34(4): 386-393, 20190000. fig
Article in Spanish | LILACS, COLNAL | ID: biblio-1049206

ABSTRACT

El síndrome de Budd-Chiari consiste en la oclusión de la circulación de salida venosa del hígado, desde las sinusoides hepáticas hasta la unión de la vena cava inferior con la aurícula derecha. Esta entidad es muy rara. Las causas son primarias, obstrucción congénita de la vena cava o trombosis espontánea, o secundarias, compresión extrínseca. Si no se trata la obstrucción esta enfermedad, lleva a cirrosis hepática. Se presenta un caso del síndrome de Budd-Chiari de causa primaria por obstrucción membranosa, tratado por vía endovascular de manera exitosa y con resolución de todos los síntomas. Es el primer reporte en Colombia de este tipo de tratamiento (AU)


Budd-Chiari syndrome refers to the occlusion of the venous outflow of the liver, from the hepatic sinusoids to the junction of the inferior vena cava with the right atrium. This entity is very rare. The causes can be divided in primary (congenital vena cava obstruction or spontaneous thrombosis), and secondary (extrinsic compression). If the obstruction is not treated, leads to liver cirrhosis. In this article, we present a case of Budd-Chiari syndrome, primary due to membranous obstruction, successfully managed by the endovascular route with resolution of all symptoms. To our knowledge, this is the first case reported in Colombia utilizing this type of treatment (AU)


Subject(s)
Humans , Budd-Chiari Syndrome , Vena Cava, Superior , Endovascular Procedures , Liver
7.
Article in English | WPRIM | ID: wpr-715454

ABSTRACT

OBJECTIVE: This study aimed to illustrate the magnetic resonance venography (MRV) manifestations of obstructed hepatic veins (HVs), the inferior vena cava (IVC), and accessory hepatic veins (AHVs) in patients with Budd-Chiari syndrome (BCS) and to evaluate the visualization capacity of MRV in the diagnosis of BCS. MATERIALS AND METHODS: Fifty-two patients with chronic BCS were included in this study. All patients were examined via MRV performed with a 3T system following injections of gadolinium-diethylene triamine pentaacetic acid (Gd-DTPA) or Gd-ethoxibenzyl-DTPA. HV and IVC lesions were classified, and their characteristics were described. HV cord-like occlusions detected via MRV were compared using ultrasonography (US). Digital subtraction angiography (DSA) was performed as a contrast in the MRV detection of IVC lesions. The HVs draining collaterals, mainly AHVs, were carefully observed. HV lesions were classified as segmental stenosis, segmental occlusion, membranous stenosis, membranous occlusion, cord-like occlusion, or non-visualized. Except for patent IVCs, IVC lesions were classified as segmental occlusion, segmental stenosis, membranous occlusion, membranous stenosis, and hepatomegaly-induced stenosis. RESULTS: All patients (52/52, 100%) showed HV lesions of different degrees. MRV was inferior to US in detecting cord-like occlusions (6 vs. 19, χ2 = 11.077, p < 0.001). Dilated AHVs, including 50 (50/52, 96.2%) caudate lobe veins and 37 (37/52, 71.2%) inferior HV and AHV lesions, were well-detected. There were no significant differences in detecting segmental lesions and thrombosis between MRV and DSA (χ2 = 0.000, p1 = 1.000, p2 = 1.000). The capacity of MRV to detect membranous lesions was inferior to that of DSA (7 vs. 15, χ2 = 6.125, p = 0.013). CONCLUSION: In patients with BCS, MRV can clearly display the lesions in HVs and the IVC, as well as in AHVs, and it has diagnostic and therapeutic value.


Subject(s)
Angiography , Angiography, Digital Subtraction , Budd-Chiari Syndrome , Constriction, Pathologic , Diagnosis , Hepatic Veins , Humans , Magnetic Resonance Imaging , Phlebography , Thrombosis , Ultrasonography , Veins , Vena Cava, Inferior
8.
Article in English | WPRIM | ID: wpr-719202

ABSTRACT

PURPOSE: The purpose of this study was to describe the long-term effects of stenting in patients with hepatic venous outflow obstruction (HVOO), who underwent living donor liver transplantation (LDLT). METHODS: Between January 2000 and December 2009, 622 adult patients underwent LDLT at our hospital, and of these patients, 21 (3.3%) were diagnosed with HVOO; among these patients, 17 underwent stenting. The patients were divided into early or late groups according to the time of their HVOO diagnoses (cutoff: 60 days after liver transplantation). RESULTS: The median follow-up period was 54.2 months (range, 0.5–192.4 months). Stent insertion was successful in 8 of 10 patients in the early group and 6 of 7 in the late group. The 5-year primary patency rates were 46% and 20%, respectively. In both groups, patients with recurrent HVOO at the beginning showed kinking confirmed by venography. Patients who carried their stents for more than 3 years maintained long-term patency. There was no significant difference in spleen size between groups; however, when the groups were compared according to whether they maintained patency, spleens tended to be smaller in the patency-maintained group. CONCLUSION: Unlike stenosis, if kinking is confirmed on venography, stenting is not feasible in the long term for patients with LDLT.


Subject(s)
Adult , Budd-Chiari Syndrome , Constriction, Pathologic , Diagnosis , Follow-Up Studies , Hepatic Veins , Humans , Liver Transplantation , Liver , Living Donors , Phlebography , Spleen , Stents
10.
J. bras. nefrol ; 39(1): 91-94, Jan.-Mar. 2017. graf
Article in English | LILACS | ID: biblio-841197

ABSTRACT

Abstract Introduction: Patients with chronic kidney disease (CKD) are at increased risk for thrombotic complications. The use of central venous catheters as dialysis vascular access additionally increases this risk. We describe the first case of Budd-Chiari syndrome (BCS) secondary to central venous catheter misplacement in a patient with CKD. Case report: A 30-year-old female patient with HIV/AIDS and CKD on hemodialysis was admitted to the emergency room for complaints of fever, prostration, and headache in the last six days. She had a tunneled dialysis catheter placed at the left jugular vein. The diagnosis of BCS was established by abdominal computed tomography that showed a partial thrombus within the inferior vena cava which extended from the right atrium to medium hepatic vein, and continuing along the left hepatic vein. Patient was treated with anticoagulants and discharged asymptomatic. Discussion: Budd-Chiari syndrome is a rare medical condition caused by hepatic veins thrombosis. It can involve one, two, or all three of the major hepatic veins. It is usually related to myeloproliferative disorders, malignancy and hypercoagulable states. This case calls attention for inadvertent catheter tip placement into hepatic vein leading to this rare complication. Conclusion: Assessment of catheter dialysis tip location with radiological image seems to be a prudent measure after each procedure even if the tunneled dialysis catheter has been introduced with fluoroscopy image.


Resumo Introdução: Pacientes com doença renal crônica (DRC) apresentam risco aumentado de complicações trombóticas e o uso de cateter venoso central para realização de hemodiálise aumenta este risco. Nós descrevemos um caso de síndrome de Budd-Chiari (SBC) causado pelo mal posicionamento de um cateter de diálise em um paciente com DRC e, para nosso conhecimento, este é o primeiro caso relatado na literatura. Caso clínico: Paciente feminina, 30 anos, com diagnóstico de HIV/SIDA e DRC em hemodiálise foi admitida na emergência com queixas de febre, prostração e cefaleia há 6 dias. Ela apresentava um cateter de diálise tunelizado implantado 7 dias antes na veia jugular esquerda. O diagnóstico de SBC foi realizado por tomografia computadorizada abdominal que mostrava um trombo no interior da veia cava inferior o qual estendia-se desde o átrio direito até a veia hepática esquerda. O cateter foi removido e a paciente foi anticoagulada. A paciente estava assintomática no momento da alta hospitalar. Discussão: SBC é uma condição clínica rara causada por trombose das veias hepáticas, podendo envolver desde uma até todas as três principais veias. Esta síndrome é em geral associada a desordens mieloproliferativas, a malignidades e a situações de hipercoagulabilidade. Este caso demonstra que o mal posicionamento da ponta do cateter no interior da veia hepática causou esta rara complicação. Conclusão: Realização de exame radiológico para avaliar localização da ponta do cateter de diálise é uma medida prudente após cada procedimento, mesmo nos casos de implante de cateter de diálise tunelizados com fluoroscopia.


Subject(s)
Humans , Female , Adult , Thrombosis/complications , Thrombosis/etiology , Vena Cava, Inferior , Central Venous Catheters/adverse effects , Budd-Chiari Syndrome/etiology
11.
Article in English | WPRIM | ID: wpr-787064

ABSTRACT

Klinefelter's syndrome is the most common congenital abnormality that causes primary hypogonadism. It is associated with diseases that predominantly affect women, such as systemic lupus erythematosus (SLE), and it can sometimes cause veno-occlusive disease. We experienced a case of Budd-Chiari syndrome (BCS) in a 33-year-old man with Klinefelter's syndrome presented with hematemesis and edema in both lower extremities. The clinical and laboratory findings were compatible with SLE, antiphospholipid syndrome, and BCS. To the best of our knowledge, this is the first case report to describe a simultaneous presentation of these four clinical syndromes in a single patient.


Subject(s)
Adult , Antiphospholipid Syndrome , Budd-Chiari Syndrome , Congenital Abnormalities , Edema , Female , Hematemesis , Humans , Hypogonadism , Klinefelter Syndrome , Liver Cirrhosis , Lower Extremity , Lupus Erythematosus, Systemic
12.
Rev. colomb. radiol ; 28(4): 4821-4824, 2017. ilus
Article in Spanish | LILACS, COLNAL | ID: biblio-986531

ABSTRACT

El síndrome de Budd-Chiari (SBC) es una patología poco frecuente y potencialmente fatal si no se diagnostica y se trata a tiempo. Se describe un caso de SBC secundario a obstrucción de la vena cava inferior intrahepática y venas suprahepáticas izquierda y media, con manejo intervencionista (colocación de endoprótesis en la vena cava) y posterior complicación con migración de la endoprótesis a la arteria pulmonar


Budd-Chiari syndrome (BCS) is an infrequent and potentially fatal disease if not diagnosed and treated early. We describe a case of BCS secondary to obstruction of intrahepatic inferior cava vein and left and middle suprahepatic veins, with interventional management (stent placement in the cava vein) and subsequent complication with stent migration to the pulmonary artery.


Subject(s)
Budd-Chiari Syndrome , Multidetector Computed Tomography , Liver Cirrhosis
14.
Article in English | WPRIM | ID: wpr-174341

ABSTRACT

Klinefelter's syndrome is the most common congenital abnormality that causes primary hypogonadism. It is associated with diseases that predominantly affect women, such as systemic lupus erythematosus (SLE), and it can sometimes cause veno-occlusive disease. We experienced a case of Budd-Chiari syndrome (BCS) in a 33-year-old man with Klinefelter's syndrome presented with hematemesis and edema in both lower extremities. The clinical and laboratory findings were compatible with SLE, antiphospholipid syndrome, and BCS. To the best of our knowledge, this is the first case report to describe a simultaneous presentation of these four clinical syndromes in a single patient.


Subject(s)
Adult , Antiphospholipid Syndrome , Budd-Chiari Syndrome , Congenital Abnormalities , Edema , Female , Hematemesis , Humans , Hypogonadism , Klinefelter Syndrome , Liver Cirrhosis , Lower Extremity , Lupus Erythematosus, Systemic
15.
Article in English | WPRIM | ID: wpr-18852

ABSTRACT

We report a case of percutaneous transhepatic stent placement for the treatment of hepatic venous outflow obstruction after extracorporeal hepatic resection and autotransplantation. A 63-year-old woman with a large mass in the liver was asymptomatic with no hepatic virus infection. Because the tumor was unresectable by conventional means, we used extracorporeal hepatic resection and autotransplantation for operation. Two days after surgery, hepatic venous outflow obstruction of the right and right inferior hepatic veins was suspected on computed tomography. After failure of the transjugular approach, hepatic venous stenting was performed successfully via the percutaneoustranshepatic approach.


Subject(s)
Autografts , Budd-Chiari Syndrome , Female , Hepatic Veins , Humans , Liver , Middle Aged , Stents , Transplantation, Autologous
16.
Rev. colomb. gastroenterol ; 31(3): 242-252, jul.-set. 2016. tab
Article in Spanish | LILACS | ID: biblio-830334

ABSTRACT

El Síndrome de Budd-Chiari (SBC) es una entidad poco usual; su incidencia se calcula de uno en cien mil. Consiste en una obstrucción completa o parcial del flujo venoso localizada en cualquier lugar desde las pequeñas venas hepáticas hasta la porción suprahepática de la vena cava inferior. De acuerdo con su etiología puede clasificarse en: primario (cuando la obstrucción se debe a una enfermedad venosa), o secundario (cuando se atribuye a una compresión extrínseca o a la invasión por una lesión que se origina fuera de las venas como neoplasias o quistes). En la mayoría de los casos se presenta como un cuadro de inicio súbito de dolor abdominal, ascitis y hepatomegalia, pudiendo ser asintomático. El diagnóstico definitivo se establece por estudios de imagen; si bien deben solicitarse exámenes básicos de laboratorio y estudios de extensión. Las modalidades de imagen incluyen la ultrasonografía Doppler, la tomografía computarizada, la resonancia magnética y la angiografía por sustracción digital, que es considerada el patrón de oro. La primera medida terapéutica que debe emprenderse en estos pacientes es la anticoagulación con heparina de bajo peso molecular, seguida de antagonistas de vitamina K. La mayoría de los pacientes requieren un enfoque multidisciplinario y un tratamiento escalonado que incluye procedimientos radiológicos, como la aplicación de balones y stents y la inserción de shunts portosistémicos transyugulares intrahepáticos (TIPS), procedimientos quirúrgicos de descomprensión y, por último, trasplante hepático


Budd - Chiari Syndrome (BCS) is a rare entity whose incidence is estimated at one in one hundred thousand. It consists of complete or partial obstruction of venous outflow at any location from the small hepatic veins to the hepatic portion of the inferior vena cava. It can be classified according to its etiology into primary BCS when there is venous obstruction and secondary disease when obstruction is attributed to extrinsic compression or invasion due to a lesion outside of the veins such as neoplasms or cysts. In most cases it presents as sudden onset of abdominal pain, ascites and hepatomegaly, but it may be asymptomatic. A definitive diagnosis is established by imaging, but basic laboratory tests and other studies must also be done. Diagnostic imaging techniques include Doppler ultrasonography, computed tomography, magnetic resonance imaging and digital subtraction angiography. The latter is considered to be the gold standard. The first therapeutic measure to be undertaken for these patients is anticoagulation with low molecular weight heparin followed by vitamin K antagonists. Most patients require a multidisciplinary approach and step by step treatment including radiological procedures, balloon enteroscopy, stenting, transjugular intrahepatic portosystemic shunt (TIPS), decompression surgery and finally, liver transplantation


Subject(s)
Humans , Male , Female , Angiography, Digital Subtraction , Budd-Chiari Syndrome , Liver Transplantation , Tomography , Ultrasonography, Doppler
18.
Article in English | WPRIM | ID: wpr-45799

ABSTRACT

Following liver transplantation, a few reports have documented hepatic venous outflow obstruction (HVOO) after inferior vena cava (IVC) stenting for the treatment of IVC stenosis. However, HVOO occurred early after IVC stenting and was mostly associated with living donor liver transplantation. Here, we report a case of HVOO that occurred 31 months after IVC stenting in a man who received deceased donor liver transplantation (DDLT) using a modified piggyback (PB) technique. The cause of HVOO was unclear, but one possible explanation is that the balloon-expandable IVC stent might have compressed the IVC chamber on the donor liver side, which would have changed the outflow hemodynamics, resulting in intimal hyperplasia. Therefore, simultaneous hepatic venous stenting with IVC stent placement could help prevent HVOO in patients receiving DDLT with the modified PB technique.


Subject(s)
Budd-Chiari Syndrome , Constriction, Pathologic , Hemodynamics , Humans , Hyperplasia , Liver Transplantation , Liver , Living Donors , Stents , Tissue Donors , Vena Cava, Inferior
19.
Radiation Oncology Journal ; : 168-176, 2016.
Article in English | WPRIM | ID: wpr-33618

ABSTRACT

PURPOSE: The purpose of current study is to evaluate the response of the patients with portal vein thrombosis (PVT) or hepatic vein thrombosis (HVT) in hepatocellular carcinoma (HCC) treated with three-dimensional conformal radiation therapy (3D-CRT). In addition, survival of patients and potential prognostic factors of the survival was evaluated. MATERIALS AND METHODS: Forty-seven patients with PVT or HVT in HCC, referred to our department for radiotherapy, were retrospectively reviewed. For 3D-CRT plans, a gross tumor volume (GTV) was defined as a hypodense filling defect area in the portal vein (PV) or hepatic vein (HV). Survival of patients, and response to radiation therapy (RT) were analyzed. Potential prognostic factors for survival and response to RT were evaluated. RESULTS: The median survival time of 47 patients was 8 months, with 1-year survival rate of 15% and response rate of 40%. Changes in Child-Pugh score, response to RT, Eastern cooperative oncology group performance status (ECOG PS), hepatitis C antibody (HCVAb) positivity, and additional post RT treatment were statistically significant prognostic factors for survival in univariate analysis (p = 0.000, p = 0.018, p = 0.000, p = 0.013, and p = 0.047, respectively). Of these factors, changes in Child-Pugh score, and response to RT were significant for patients’ prognosis in multivariate analysis (p = 0.001 and p = 0.035, respectively). CONCLUSION: RT could constitute a reasonable treatment option for patients with PVT or HVT in HCC with acceptable toxicity. Changes in Child-Pugh score, and response to RT were statistically significant factors of survival of patients.


Subject(s)
Budd-Chiari Syndrome , Carcinoma, Hepatocellular , Hepatic Veins , Hepatitis C , Humans , Multivariate Analysis , Portal Vein , Prognosis , Radiotherapy , Retrospective Studies , Survival Rate , Thrombosis , Tumor Burden , Venous Thrombosis
20.
Arq. bras. cardiol ; 104(3): 218-225, 03/2015. tab, graf
Article in English | LILACS | ID: lil-742783

ABSTRACT

Background: Burnout Syndrome is the extreme emotional response to chronic occupational stress, manifesting as physical and mental exhaustion. Although associated with higher prevalence of cardiovascular risk factors, no study so far has evaluated whether the Burnout Syndrome could be a prevalent factor in non-elderly individuals active in the labor market, admitted for acute coronary syndrome (ACS). Objective: To evaluate the prevalence of the Burnout Syndrome in non-elderly, economically active patients, hospitalized with ACS. Methods: Cross-sectional study conducted in a tertiary and private cardiology center, with economically active patients aged <65 years, hospitalized with diagnosis of ACS. The Burnout Syndrome was evaluated with the Burnout Syndrome Inventory (BSI), which assesses workplace conditions and four dimensions that characterize the syndrome: emotional exhaustion (EE), emotional distancing (EmD), dehumanization (De) and professional fulfillment (PF). The Lipp’s Stress Symptoms Inventory for Adults (LSSI) was applied to evaluate global stress. Results: Of 830 patients evaluated with suspected ACS, 170 met the study criteria, 90% of which were men, overall average age was 52 years, and 40.5% had an average income above 11 minimum wages. The prevalence of the Burnout Syndrome was 4.1%. When we evaluated each dimension individually, we found high EE in 34.7%, high De in 52.4%, high EDi in 30.6%, and low PF in 5.9%. The overall prevalence of stress was 87.5%. Conclusion: We found a low prevalence of Burnout Syndrome in an economically active, non-elderly population among patients admitted for ACS in a tertiary and private hospital. .


Fundamento: Síndrome de Burnout (SB) é a resposta emocional extrema ao estresse crônico ocupacional, manifestando-se como processo de esgotamento físico e psíquico. Embora associada com maior prevalência de fatores de risco, nenhum estudo avaliou até o momento se a SB poderia ser um fator prevalente em indivíduos não idosos, ativos no mercado de trabalho, admitidos por síndrome coronária aguda (SCA). Objetivo: Avaliar a prevalência da SB em pacientes economicamente ativos, não idosos, hospitalizados com diagnóstico de SCA. Métodos: Estudo transversal realizado em um centro de cardiologia terciário e privado, com pacientes economicamente ativos, com idade < 65 anos, hospitalizados com diagnóstico de SCA. Para avaliação da SB, aplicou-se o Inventário da Síndrome de Burnout (ISB), que avalia as condições do ambiente de trabalho e as dimensões que caracterizam a SB: exaustão emocional (EE), distanciamento emocional (DEm), desumanização (Des) e realização profissional (RP). Aplicou-se ainda o Inventário de Sintomas de Stress para Adultos de Lipp (ISSL) para avaliação de estresse global. Resultados: Dos 830 pacientes avaliados com suspeita de SCA, 170 preencheram os critérios do estudo, sendo 90% homens, com idade média de 52 anos, e rendimento médio acima de 11 salários mínimos em 40,5% da amostra. A prevalência da SB foi de 4,1%. Elevada EE esteve presente em 34,7%, elevado DEm em 52,4%, elevada Des em 30,6% e baixa RP em 5,9%. A prevalência de estresse geral foi de 87,5%. Conclusão: A SB foi pouco prevalente em pacientes ativos no mercado de trabalho, não idosos, e internados por SCA nesta amostra avaliada em um hospital cardiológico privado e terciário. .


Subject(s)
Aged, 80 and over , Humans , Male , Budd-Chiari Syndrome , Carcinoma, Hepatocellular , Heart Failure/etiology , Heart Failure , Liver Neoplasms , Diagnosis, Differential , Echocardiography , Heart Atria , Palliative Care , Thrombosis , Biomarkers, Tumor/analysis , Vena Cava, Inferior , alpha-Fetoproteins/analysis
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