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2.
Niger J Clin Pract ; 22(11): 1617-1620, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31719286

RESUMO

Budd-Chiari syndrome (BCS) is a rare disease characterized by obstruction of hepatic venous outflow tract with diversified etiologies. Sea-blue histiocytosis (SBH) is a kind of storage diseases defined by the deposition of abundant sea-blue histiocytes in various organs and can lead to hepatosplenomegaly, cirrhosis, or even liver failure. The association between BCS and SBH has never been reported before. Here, we report a patient with BCS presenting with hepatosplenomegaly, portal hypertension, and pancytopenia who was later confirmed to also have SBH.


Assuntos
Síndrome de Budd-Chiari/complicações , Hepatomegalia/diagnóstico por imagem , Hipertensão Portal/complicações , Pancitopenia/complicações , Síndrome do Histiócito Azul-Marinho/diagnóstico , Esplenomegalia/diagnóstico por imagem , Adulto , Síndrome de Budd-Chiari/diagnóstico , Síndrome de Budd-Chiari/etiologia , Hepatomegalia/complicações , Humanos , Masculino , Doenças Raras , Esplenomegalia/complicações , Veia Cava Inferior
3.
Blood ; 134(22): 1902-1911, 2019 11 28.
Artigo em Inglês | MEDLINE | ID: mdl-31778549

RESUMO

Thrombotic and hemorrhagic complications are prevalent in patients with essential thrombocythemia, polycythemia vera, and myelofibrosis. Given the impact on morbidity and mortality, reducing the risk of thrombosis and/or hemorrhage is a major therapeutic goal. Historically, patients have been risk stratified on the basis of traditional factors, such as advanced age and thrombosis history. However, multiple factors contribute to the thrombotic tendency, including gender, mutational profile, inflammatory stress, and abnormal cell adhesion. Management includes cardiovascular risk reduction and use of antiplatelet therapy, depending on myeloproliferative neoplasm subtype and mutational status. Anticoagulation is a mainstay of therapy for those with venous thrombosis, but practice patterns remain heterogeneous. Cytoreduction is indicated for higher-risk patients, but efficacy may depend on the involved vascular bed. Management of special situations, such as unusual site thrombosis, bleeding, the perioperative period, and pregnancy, are especially challenging. In this article, risk factors and treatment strategies for myeloproliferative neoplasm thrombosis and bleeding, including special situations, are reviewed. Insights gained from recent studies may lead to the development of a more precise risk classification and tailored therapy.


Assuntos
Anticoagulantes/uso terapêutico , Síndrome de Budd-Chiari , Neoplasias Hematológicas , Hemorragia , Transtornos Mieloproliferativos , Doenças de von Willebrand , Adulto , Síndrome de Budd-Chiari/sangue , Síndrome de Budd-Chiari/tratamento farmacológico , Síndrome de Budd-Chiari/etiologia , Síndrome de Budd-Chiari/genética , Feminino , Neoplasias Hematológicas/sangue , Neoplasias Hematológicas/complicações , Neoplasias Hematológicas/tratamento farmacológico , Neoplasias Hematológicas/genética , Hemorragia/sangue , Hemorragia/tratamento farmacológico , Hemorragia/etiologia , Hemorragia/genética , Humanos , Masculino , Transtornos Mieloproliferativos/sangue , Transtornos Mieloproliferativos/complicações , Transtornos Mieloproliferativos/tratamento farmacológico , Transtornos Mieloproliferativos/genética , Fatores Sexuais , Doenças de von Willebrand/sangue , Doenças de von Willebrand/tratamento farmacológico , Doenças de von Willebrand/etiologia , Doenças de von Willebrand/genética
4.
Indian Pediatr ; 56(11): 965-967, 2019 11 15.
Artigo em Inglês | MEDLINE | ID: mdl-31729328

RESUMO

We performed a review of case records of children diagnosed with hepatic venous outflow tract obstruction at our center in last 10 years. Out of 11 cases identified, 6 had variable blocks in the hepatic venous system and 4 had combined hepatic venous and inferior vena cava (IVC) block. One child with paroxysmal nocturnal hemoglobinuria (PNH) had isolated IVC involvement. Angioplasty was attempted in 3 patients; among them 2 had successful outcome. Seven children with advanced liver disease underwent transplantation, which was successful in six. With availability of modalities like interventional radiology and transplantation, the overall prognosis of hepatic venous outflow tract obstruction seems to be good when managed in a well-equipped center.


Assuntos
Angioplastia/métodos , Síndrome de Budd-Chiari , Doença Hepática Terminal , Veias Hepáticas , Transplante de Fígado , Síndrome de Budd-Chiari/etiologia , Síndrome de Budd-Chiari/fisiopatologia , Síndrome de Budd-Chiari/cirurgia , Criança , Doença Hepática Terminal/complicações , Doença Hepática Terminal/fisiopatologia , Doença Hepática Terminal/cirurgia , Feminino , Hemoglobinúria Paroxística/complicações , Hemoglobinúria Paroxística/fisiopatologia , Veias Hepáticas/diagnóstico por imagem , Veias Hepáticas/cirurgia , Humanos , Índia/epidemiologia , Transplante de Fígado/métodos , Transplante de Fígado/estatística & dados numéricos , Masculino , Avaliação de Processos e Resultados em Cuidados de Saúde , Prognóstico , Radiografia Intervencionista/métodos
5.
Reumatismo ; 71(3): 163-165, 2019 Oct 24.
Artigo em Inglês | MEDLINE | ID: mdl-31649380

RESUMO

Behçet's disease is a chronic autoinflammatory disorder that usually presents with recurrent oral and genital ulcers, uveitis, skin lesions and arthritis. Any organ, however, can be involved and in a subgroup of patients the large vascular lesions are the predominant feature (vasculo-Behçet disease). We report a case of a 27-yearold man with Behçet's disease who developed Budd-Chiari syndrome while on immunosuppressive therapy. Infliximab resulted in a rapid and total clinical remission. Literature data have shown that antitumor necrosis factor alpha (anti-TNF-α) agents including infliximab can suppress disease activity but there is no long-term experience on tapering and/or discontinuation of treatment under clinical remission. Our case supports the use of infliximab in vasculo-Behçet patients.


Assuntos
Síndrome de Budd-Chiari/tratamento farmacológico , Infliximab/uso terapêutico , Adulto , Síndrome de Behçet/complicações , Síndrome de Budd-Chiari/etiologia , Humanos , Masculino , Indução de Remissão
6.
Med Ultrason ; 21(3): 344-348, 2019 Aug 31.
Artigo em Inglês | MEDLINE | ID: mdl-31476215

RESUMO

Budd Chiari syndrome defines an obstruction of the hepatic venous outflow. Primary causes include pro-coagulant states resulting in venous thrombosis, while secondary Budd Chiari syndrome appears in conditions associated with extrinsic compression of the hepatic veins or tumor invasion. Clinical presentation is greatly varied, from incidentally discovered asymptomatic thrombosis to fulminant liver failure due to hepatic congestion. Abdominal ultrasonography is the key diagnostic tool of Budd Chiari syndrome. This pictorial essay aims to show the ultrasonographic aspect of Budd-Chiari syndrome associated with other medical conditions (abdominal malignancy, hematologic disorders and abdominal surgery).


Assuntos
Neoplasias Abdominais/complicações , Síndrome de Budd-Chiari/diagnóstico por imagem , Síndrome de Budd-Chiari/etiologia , Doenças Hematológicas/complicações , Complicações Pós-Operatórias/diagnóstico por imagem , Ultrassonografia/métodos , Neoplasias Abdominais/diagnóstico por imagem , Doenças Hematológicas/diagnóstico por imagem , Humanos
7.
Medicine (Baltimore) ; 98(22): e15821, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31145318

RESUMO

RATIONALE: Supradiaphragmatic inferior vena cava (IVC) injury due to blunt thoracic trauma is extremely rare. Budd-Chiari syndrome (BCS) is also rare and presents with ascites, abdominal pain, hepatomegaly, leg swelling, and jaundice. Its etiology is diverse, and it is rarely caused by trauma. PATIENT CONCERNS: A 36-year-old man with blunt trauma from a traffic accident presented with chest pain. Chest computed (CT) and emergency surgery with CPB revealed completely transected supradiaphragmatic inferior vena cava (IVC), which reconstruction was essential. DIAGNOSES: BCS caused by impaired hepatic venous drainage through a reconstructed neo-IVC after severe blunt trauma injury to the supradiaphragmatic IVC was diagnosed. INTERVENTION: Hepatic failure, ascites, leg swelling, and jaundice were resolved post-insertion of a veno-venous extracorporeal membrane oxygenator (V-V ECMO) for hepatic venous drainage, but these clinical symptoms reappeared after ECMO removal. OUTCOME: The patient died from rapidly progressing sepsis, pneumonia, and acute renal failure during repeated insertion of ECMO and weaning off ECMO. LESSONS: Reconstructing and improving the patency of the supradiaphragmatic IVC is essential for successful hepatic venous drainage. Additionally, a surgical strategy focused on graft selection can prevent kinking stenosis, and possibly BCS, especially in emergency surgeries. A ring-supported synthetic graft should be considered an alternative to improve long-term patency and survival rate.


Assuntos
Síndrome de Budd-Chiari/etiologia , Traumatismos Torácicos/cirurgia , Procedimentos Cirúrgicos Vasculares/efeitos adversos , Veia Cava Inferior/lesões , Veia Cava Inferior/cirurgia , Ferimentos não Penetrantes/cirurgia , Adulto , Humanos , Masculino , Procedimentos Cirúrgicos Vasculares/métodos
8.
Hepatol Int ; 13(2): 214-221, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30617764

RESUMO

INTRODUCTION: Anticoagulation is universally recommended in Budd-Chiari syndrome [BCS]. Vitamin K epoxide reductase complex 1 (VKORC1) and CYP2C9 are involved in the metabolism of warfarin. The present study was done to assess whether these mutations are associated with the risk of bleeding in patients with BCS receiving warfarin. PATIENTS AND METHODS: Patients diagnosed with BCS underwent genotyping for three single nucleotide polymorphisms [SNPs]-two for the CYP2C9 and one for the VKORC1 haplotype. The patients were followed up for at least 12 months and all bleeding episodes were recorded. Patients with and without mutations were compared for bleeding complications and a crude odds ratio [crude OR] was derived for the association between bleeding and presence or absence of mutant alleles. RESULTS: Eighty patients [mean (SD) age 27.47 (8.93) years, 35 male] with BCS underwent genetic testing. 37/80 (46.2%) patients had mutation of CYP2C9 and/or VKORC1; 22/80 (27.5%) had either of the mutant alleles of CYP2C9 and, similarly, 22/80 (27.5%) had the VKORC mutation. Over a median follow-up of 20 (range 12-96) months, 21/80 (26.3%) patients had bleeding complications. Patients with mutant SNPs had a higher risk of bleeding than those without [14/37 vs. 7/43, p = 0.04, crude OR (95% CI) 3.13 (1.1-8.9)]. CONCLUSION: The presence of mutations in VKORC1 or CYP2C9 is associated with increased risk of bleeding in patients with BCS on warfarin. Such patients with SNPs of CY2C9 or VKORC1 haplotype should be monitored intensively while receiving warfarin.


Assuntos
Anticoagulantes/efeitos adversos , Síndrome de Budd-Chiari/tratamento farmacológico , Citocromo P-450 CYP2C9/genética , Hemorragia/genética , Vitamina K Epóxido Redutases/genética , Varfarina/efeitos adversos , Adolescente , Adulto , Alelos , Síndrome de Budd-Chiari/etiologia , Feminino , Haplótipos , Hemorragia/induzido quimicamente , Humanos , Masculino , Pessoa de Meia-Idade , Mutação , Polimorfismo de Nucleotídeo Único , Índice de Gravidade de Doença , Adulto Jovem
9.
Ann Vasc Surg ; 57: 274.e1-274.e3, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30500636

RESUMO

A case with an extremely rare intravenous cystic lesion in the suprahepatic inferior vena cava was reported, which originated from the lymphatic system and had induced Budd-Chiari syndrome. To the best of our knowledge, this is the first report of a benign cystic lesion originating from the wall of a suprahepatic inferior vena cava which results in Budd-Chiari syndrome.


Assuntos
Síndrome de Budd-Chiari/etiologia , Linfocele/complicações , Veia Cava Inferior , Biópsia , Síndrome de Budd-Chiari/diagnóstico por imagem , Síndrome de Budd-Chiari/patologia , Síndrome de Budd-Chiari/cirurgia , Angiografia por Tomografia Computadorizada , Humanos , Linfocele/diagnóstico por imagem , Linfocele/patologia , Linfocele/cirurgia , Masculino , Pessoa de Meia-Idade , Flebografia/métodos , Resultado do Tratamento , Veia Cava Inferior/diagnóstico por imagem , Veia Cava Inferior/patologia , Veia Cava Inferior/cirurgia
10.
Eur J Haematol ; 102(1): 53-62, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30267448

RESUMO

Thrombosis of unusual venous sites encompasses a large part of consultative hematology and is encountered routinely by practicing hematologists. Contrary to the more commonly encountered lower extremity venous thrombosis and common cardiovascular disorders, the various thromboses outlined in this review have unique presentations, pathophysiology, workup, and treatments that all hematologists should be aware of. This review attempts to outline the most up to date literature on cerebral, retinal, upper extremity, hepatic, portal, splenic, mesenteric, and renal vein thrombosis, focusing on the incidence, pathophysiology, provoking factors, and current recommended treatments for each type of unusual thrombosis to provide a useful and practical review for the hematologist.


Assuntos
Trombose Venosa/diagnóstico , Trombose Venosa/terapia , Síndrome de Budd-Chiari/diagnóstico , Síndrome de Budd-Chiari/etiologia , Síndrome de Budd-Chiari/terapia , Veias Cerebrais/patologia , Gerenciamento Clínico , Humanos , Veias Mesentéricas/patologia , Veia Porta/patologia , Veias Renais/patologia , Veia Retiniana/patologia , Veia Esplênica/patologia , Extremidade Superior/patologia , Trombose Venosa/etiologia
11.
J Visc Surg ; 155(6): 513-515, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30420262

RESUMO

Asymptomatic right diaphragmatic rupture with liver and gallbladder herniation and secondary Budd-Chiari syndrome is a rare complication of abdominal trauma. In this setting, the management of gallbladder stones remains poorly described and may require a thoracic approach.


Assuntos
Colecistectomia/métodos , Vesícula Biliar , Hérnia Diafragmática/cirurgia , Fígado , Pancreatite/complicações , Traumatismos Abdominais/complicações , Síndrome de Budd-Chiari/etiologia , Feminino , Vesícula Biliar/diagnóstico por imagem , Cálculos Biliares/cirurgia , Hérnia Diafragmática/diagnóstico por imagem , Humanos , Fígado/diagnóstico por imagem , Pessoa de Meia-Idade , Esfinterotomia Endoscópica , Toracotomia/métodos , Tomografia Computadorizada por Raios X
12.
Transplant Proc ; 50(9): 2715-2717, 2018 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-30401383

RESUMO

BACKGROUND: Our aim in this study was to evaluate long-term efficiency of hepatic venous balloon angioplasty (BA) and stent placement (SP) for hepatic venous outflow obstruction (HVOO) in pediatric liver transplantation (LT). METHODS: From January 1999 to September 2016, 262 pediatric patients underwent LT at our hospital. Ten were diagnosed with HVOO, which included 8 living donor grafts and 2 split liver grafts. BA and SP were used in management of these 10 patients with HVOO. After intervention, Doppler ultrasound (DUS) was the major follow-up modality for comparing efficiency of BA and SP. RESULTS: The incidence of HVOO was 3.8% (10 of 262) in our pediatric LTs. Of the 10 HVOO cases, 5 had SP, 3 had BA once, 1 had BA twice, and 1 had BA twice along with SP. The patent hepatic vein was maintained after a mean follow-up of 7.4 (range, 0.04-17) years. Recurrent rate of HVOO after BA was 42%. Neither recurrent HVOO nor stent migration occurred after SP and throughout long-term follow-up. CONCLUSION: Hepatic venous SP was found to be more effective and safe than BA for treatment of HVOO in pediatric LT for long-term follow-up.


Assuntos
Angioplastia com Balão/métodos , Síndrome de Budd-Chiari/etiologia , Síndrome de Budd-Chiari/cirurgia , Procedimentos Cirúrgicos do Sistema Digestório/instrumentação , Transplante de Fígado/efeitos adversos , Stents , Adolescente , Angioplastia com Balão/mortalidade , Síndrome de Budd-Chiari/epidemiologia , Criança , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Feminino , Veias Hepáticas/cirurgia , Humanos , Incidência , Doadores Vivos , Masculino , Recidiva , Resultado do Tratamento
13.
Rev Med Liege ; 73(11): 557-561, 2018 Nov.
Artigo em Francês | MEDLINE | ID: mdl-30431244

RESUMO

Rarely encountered in the Western countries, membranous occlusion of the inferior vena cava results from a fibrous thickening of the intima and is commonly located at the orifices of the hepatic veins. To date, two etiopathogenic hypotheses have been formulated: the first, dealing with the embryological aspect, the second, arguing for a thrombotic origin. Nevertheless, several studies are still underway to understand the exact physiopathological mechanism of this obstruction. Among these studies, some suspect the predisposing role of anatomical and infectious factors. On average, it takes 6 years between the beginning of the symptoms and the diagnosis. The later the diagnosis is settled, the higher is the risk of encountering a hepatocellular carcinoma. The most relevant additional examinations to visualize the membrane are the abdominal ultrasound and the cavography. Computed tomography allows to highlight the effects of obstruction on the hepatic parenchyma. Concerning the treatment, many studies showed that angioplasty has an excellent immediate and late efficacy. A few surgical techniques also exist but those are less exploited because of the non invasive aspect and lower morbidity-mortality of the endovascular treatment.


Assuntos
Síndrome de Budd-Chiari/etiologia , Veia Cava Inferior , Trombose Venosa/complicações , Humanos
14.
BMJ Case Rep ; 20182018 Sep 28.
Artigo em Inglês | MEDLINE | ID: mdl-30269090

RESUMO

Leiomyosarcoma (LMS) of primary vascular origin is a rare entity with only potentially curative option being complete surgical resection; despite which the prognosis remains dismal. Tumour recurrence is very common, and the benefits of adjuvant therapy are undefined. A 39-year-old woman presented with 6 months' history of abdominal pain, abdominal distension and pedal oedema. On evaluation, she was diagnosed to have chronic Budd-Chiari syndrome (BCS) secondary to a tumour arising from the inferior vena cava (IVC) on evaluation. Her liver decompensation included jaundice, gastrointestinal bleed and ascites. Following a detailed multidisciplinary team discussion, she underwent complete excision of the tumour along with a segment of the IVC with living donor liver transplantation. She remains disease-free 24 months following surgery. This is the first reported case of liver transplantation for IVC LMS causing chronic BCS.


Assuntos
Leiomiossarcoma/cirurgia , Transplante de Fígado/métodos , Neoplasias Vasculares/cirurgia , Veia Cava Inferior , Adulto , Síndrome de Budd-Chiari/diagnóstico , Síndrome de Budd-Chiari/etiologia , Feminino , Humanos , Leiomiossarcoma/complicações , Leiomiossarcoma/patologia , Doadores Vivos , Tomografia Computadorizada por Raios X , Neoplasias Vasculares/complicações , Neoplasias Vasculares/patologia , Veia Cava Inferior/diagnóstico por imagem , Veia Cava Inferior/patologia
16.
Clin Immunol ; 197: 54-59, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30125675

RESUMO

OBJECTIVE: To describe the outcome and tolerance in patients treated with anti-TNFα in severe and refractory major vessel disease in Behçet's disease (BD). METHODS: A multicenter study evaluating 18 refractory BD patients with major vessel involvement [pulmonary artery (n = 4), aorta (n = 4) or peripheral artery aneurysm (n = 1) and/or pulmonary artery (n = 7), inferior vena cava (n = 5), or intra-cardiac (n = 3) thrombosis or Budd Chiari Syndrome (n = 2)] treated with anti-TNFα agents. RESULTS: Vascular remission was achieved in 16 (89%) patients. The 9 months risk of relapse was significantly higher with conventional immunosuppressants used prior anti-TNFα agents as compared to anti-TNFα therapy [OR = 8.7 (1.42-62.6), p = 0.03]. The median daily dose of corticosteroids significantly decreased at 12 months. Side effects included infection (n = 4) and pulmonary edema (n = 1). CONCLUSION: TNFα-antagonists are safe and might be associated with a decreased risk of relapse at 9 months compared to conventional immunosuppressants in BD patients with major vessels disease.


Assuntos
Adalimumab/uso terapêutico , Antirreumáticos/uso terapêutico , Síndrome de Behçet/tratamento farmacológico , Infliximab/uso terapêutico , Trombose/fisiopatologia , Adulto , Doenças da Aorta/etiologia , Doenças da Aorta/fisiopatologia , Síndrome de Behçet/complicações , Síndrome de Behçet/fisiopatologia , Síndrome de Budd-Chiari/etiologia , Síndrome de Budd-Chiari/fisiopatologia , Feminino , Cardiopatias/etiologia , Cardiopatias/fisiopatologia , Humanos , Imunossupressores/uso terapêutico , Infecções , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Artéria Pulmonar/fisiopatologia , Edema Pulmonar , Recidiva , Indução de Remissão , Estudos Retrospectivos , Índice de Gravidade de Doença , Trombose/etiologia , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Doenças Vasculares/etiologia , Doenças Vasculares/fisiopatologia , Veia Cava Inferior/fisiopatologia , Adulto Jovem
17.
J Gastroenterol Hepatol ; 33(12): 2015-2021, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-29851172

RESUMO

BACKGROUND AND AIM: The aim of this study is to compare long-term outcomes of retrievable stents (RSs) and permanent stents (PSs) for Budd-Chiari syndrome (BCS) due to long-segment obstructive inferior vena cava (IVC). METHODS: Between July 2000 and August 2016, 42 patients with BCS due to long-segment obstructive IVC were treated with RSs, and 41 patients were treated with PSs. The RSs were removed eventually after thrombus disappeared. Patients were subsequently followed up by color Doppler sonography or computed tomography scanning. RESULTS: All RS placements were successful, and 37 RSs were retrieved 8 to 29 days later. Forty-two stents were implanted in PS group. One failure retrieval of RSs occurred, and two failures of cannulations were found in PS group. Two deaths may be procedure related and died from acute pulmonary thromboembolism perioperatively. One patient developed acute cerebral infarction and recovered after treatment. In PS group, minor complications were found in three patients. The length of IVC lesion segment and length and thickness of IVC thrombus decreased significantly, and diameter of retrocaval IVC and diaphragm IVC increased significantly in both groups. During follow-up, three patients died from liver failure in RS group, and two patients died in PS group. RS group showed a significantly higher primary patency rate than PS group. Cumulative 1-, 3-, and 5-year secondary patency rates were 95.2%, 89.6%, and 89.6% in RS group and 100%, 96.6%, and 96.6% in PS group (P = 0.7109). CONCLUSIONS: Retrievable stents are effective for BCS because of long-segment obstructive IVC, with a higher primary patency rate.


Assuntos
Síndrome de Budd-Chiari/terapia , Remoção de Dispositivo , Procedimentos Endovasculares/instrumentação , Stents , Veia Cava Inferior , Adulto , Idoso , Síndrome de Budd-Chiari/diagnóstico por imagem , Síndrome de Budd-Chiari/etiologia , Síndrome de Budd-Chiari/fisiopatologia , Angiografia por Tomografia Computadorizada , Constrição Patológica , Procedimentos Endovasculares/efeitos adversos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Flebografia/métodos , Desenho de Prótese , Estudos Retrospectivos , Terapia Trombolítica , Fatores de Tempo , Resultado do Tratamento , Ultrassonografia Doppler em Cores , Grau de Desobstrução Vascular , Veia Cava Inferior/diagnóstico por imagem , Veia Cava Inferior/fisiopatologia
19.
Gynecol Obstet Invest ; 83(4): 404-409, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29886492

RESUMO

Stroke and hepatic vein thrombosis are highly associated with neoplasia but are extremely rare events in young, pregnant women. Rare and recurrent thrombotic events in pregnancy increase the suspicion for occult malignancy. We describe the case of a healthy 31-year-old G2P1 who presented with visual changes and dysarthria during pregnancy. Imaging showed cerebral infarcts. Her thrombophilia evaluation was negative. During delivery, she was diagnosed with fulminant Budd-Chiari Syndrome. Hepatic ultrasound suggested malignancy or metastasis, and postpartum CT scan and biopsy confirmed the diagnosis of Stage IV pancreatic cancer. Although rare in pregnancy, a new diagnosis of malignancy should be considered in patients with recurrent unexplained hypercoagulable complications. We propose an evidence-based algorithm for evaluation of occult malignancy in pregnancy based upon this case and review of the literature.


Assuntos
Síndrome de Budd-Chiari/etiologia , Disartria/etiologia , Neoplasias Pancreáticas/complicações , Complicações Neoplásicas na Gravidez , Diagnóstico Pré-Natal/métodos , Adulto , Biópsia , Síndrome de Budd-Chiari/diagnóstico , Disartria/diagnóstico , Feminino , Humanos , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/patologia , Período Pós-Parto , Gravidez , Complicações Neoplásicas na Gravidez/diagnóstico , Complicações Neoplásicas na Gravidez/patologia , Tomografia Computadorizada por Raios X
20.
Ann Hepatol ; 17(4): 638-644, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29893705

RESUMO

INTRODUCTION AND AIM: Budd-Chiari syndrome (BCS) is caused by hepatic venous outflow obstruction. This work aims to analyze the pattern of vascular involvement in Egyptian patients with BCS, demonstrates its relation to etiology and shows its impact on clinical presentation. MATERIAL AND METHODS: The current retrospective study was conducted at The Tropical Medicine Department, Ain Shams University on one hundred Egyptian patients with confirmed diagnosis of primary BCS who were presented to the Budd-Chiari Study Group (BCSG) from April 2014 to May 2016 by collecting clinical, laboratory and radiological data from their medical records. RESULTS: Isolated hepatic vein occlusion (HVO) was the most common pattern of vascular involvement (43%), followed by combined HVO and inferior vena cava (IVC) compression by enlarged caudate lobe (32%), then combined HVO and IVC stenosis/webs (21%), and lastly isolated IVC occlusion (4%). Ascites was more significantly encountered in BCS patients with HVO than in those with isolated inferior vena cava (IVC) occlusion and patent HVs (P = 0.005). Abdominal pain was significantly encountered in patients with occluded three major HVs (P = 0.044). Behcet's disease was significantly detected in isolated IVC occlusion. Protein C deficiency was significantly detected in patients with combined HVO and IVC compression. CONCLUSION: Isolated HVs occlusion was the most common pattern of vascular involvement in Egyptian patients with primary BCS. Vascular pattern of involvement affected the clinical presentation and was related to the underlying thrombophilia in those patients.


Assuntos
Síndrome de Budd-Chiari/etiologia , Veias Hepáticas , Hepatopatia Veno-Oclusiva/etiologia , Veia Cava Inferior , Adolescente , Adulto , Síndrome de Budd-Chiari/diagnóstico por imagem , Constrição Patológica , Egito , Feminino , Veias Hepáticas/diagnóstico por imagem , Hepatopatia Veno-Oclusiva/diagnóstico por imagem , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Veia Cava Inferior/diagnóstico por imagem , Adulto Jovem
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