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1.
Medicina (B Aires) ; 81(2): 286-288, 2021.
Artigo em Espanhol | MEDLINE | ID: mdl-33906149

RESUMO

We report the case of an 86-year-old man presenting with a spontaneous hematoma in the left iliac muscle and previous diagnosis of colon cancer in 1998 (stage pT3N0M0) treated with transverse colectomy and considered in complete remission. After a complete study, it was possible to identify the presence of Factor VIII inhibitors antibodies that confirmed the presence of acquired hemophilia. During hospitalization the patient presented a lower gastrointestinal bleeding leading to the diagnosis of recurrence of a previously treated colorectal adenocarcinoma. He responded to initial therapy with systemic corticoids and anti-inhibitory coagulant complex which includes activated VII Factor [FEIBA].


Assuntos
Neoplasias Colorretais , Hemofilia A , Idoso de 80 Anos ou mais , Neoplasias Colorretais/diagnóstico , Fator VIII , Hematoma , Hemofilia A/complicações , Hemofilia A/diagnóstico , Hemofilia A/tratamento farmacológico , Humanos , Masculino , Recidiva Local de Neoplasia/diagnóstico
2.
J Anesth ; 35(2): 288-302, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33682038

RESUMO

Female carriers are more common than males with hemophilia and unrecognized factor VIII or IX deficiency is associated with intrauterine growth retardation, epidural hematomas, blood transfusion, and peripartum hemorrhage. A review was conducted to assess the evidence for professional society recommendations for > 50% factor levels during labor. Two searches of Pubmed, CINAHL, Cochrane, and Google Scholar were completed in October 2019. The first for case reports and series described neuraxial techniques in patients with hemophilia-regardless of sex, age, or pregnant status. The second for case reports and series described bleeding outcomes of parturients with hemophilia. Primary outcomes were diagnosis of neuraxial hematoma (first search) and postpartum bleeding complications (second search). Thirteen articles (n = 134) described neuraxial techniques in patients with hemophilia. Neuraxial hematoma with paraplegia occurred in 3/134 patients-all had a factor level of 1%. Nineteen articles (2712 deliveries in 2657 women) described bleeding outcomes. Postpartum hemorrhage occurred in 7.1% (193/2712) of deliveries, of which 60% necessitated blood transfusion. Postpartum bleeding complications were twice as likely (51.0% [25/49] vs. 25.6% [52/203], P < 0.001) with factor activity < 50%. Therefore, factor levels should be assessed and increased above 50% prior to neuraxial technique and delivery.Trial registration: PROSPERO 2018 CRD42018110215.


Assuntos
Anestesia Obstétrica , Hemofilia A , Trabalho de Parto , Hemorragia Pós-Parto , Feminino , Hemofilia A/complicações , Humanos , Masculino , Hemorragia Pós-Parto/etiologia , Hemorragia Pós-Parto/terapia , Gravidez
3.
Br J Oral Maxillofac Surg ; 59(3): 341-346, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33685775

RESUMO

Many guidelines and studies describe haemostatic management protocols for patients with haemophilia, but few have evaluated the risk factors for post-extraction bleeding. This retrospective cohort study was performed to investigate these risks among this group of patients. We used medical records to identify patients with haemophilia who underwent tooth extraction(s) between April 2006 and April 2019 in the Department of Oral and Maxillofacial Surgery at Nara Medical University Hospital, Nara, Japan, and conducted logistic regression analyses to identify risk or protective factors for post-extraction bleeding in procedures involving factor replacement therapy. Post-extraction bleeding was defined as bleeding that could not be stopped by biting down on gauze, and that required medical treatment between 30min and 14 days after the extraction. A total of 151 extractions (84 interventions) in 55 patients fulfilled the inclusion criteria (130 extractions (72 interventions) in 48 patients with haemophilia A, and 21 extractions (12 interventions) in seven patients with haemophilia B). Post-extraction bleeding was observed in nine patients (16.3%), 10 interventions (11.9%), and 12 extractions (7.9%). On average, it occurred six days after the intervention, and on the fifth postoperative day after extractions. Use of mouth splints significantly reduced the risk (odds ratio: 0.13; p=0.01) in patients on factor replacement therapy. We will conduct a prospective study to investigate the optimal type of splint and optimal splint-wearing period.


Assuntos
Hemofilia A , Hemofilia A/complicações , Humanos , Japão , Hemorragia Pós-Operatória/etiologia , Estudos Prospectivos , Estudos Retrospectivos , Fatores de Risco , Extração Dentária
4.
Clin Appl Thromb Hemost ; 27: 1076029621989811, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33587652

RESUMO

Antihemophilic factor (recombinant) (rAHF; ADVATE®; Baxalta US Inc., a Takeda company, Lexington, MA, USA) is indicated for the treatment and prevention of bleeding in patients with hemophilia A. We aimed to assess the safety and efficacy of standard prophylaxis versus on-demand treatment with rAHF in previously treated Chinese patients with severe/moderately severe hemophilia A. This open-label, sequential, interventional, postapproval study (NCT02170402) conducted in China included patients of any age with hemophilia A with factor VIII (FVIII) level ≤2%. Patients received 6 months' on-demand rAHF then 6 months' rAHF prophylaxis (20-40 IU/kg every 48 ± 6 hours). The primary objective was percentage reduction in annualized bleeding rate (ABR) in the per-protocol analysis set (PPAS); secondary objectives included ABR by bleeding subtype, hemostatic efficacy, immunogenicity, and safety. Of 72 patients who received ≥1 rAHF dose, 61 were included in the PPAS. Total ABR was lower during prophylaxis (mean 2.5, 95% CI 1.5-3.7; median 0) versus on-demand treatment (mean 58.3, 95% CI 52.5-64.7; median 53.9), representing a 95.9% risk reduction. Similar findings in favor of prophylaxis were observed for all types of bleeding event by cause and location. rAHF hemostatic efficacy was rated as "excellent"/"good" in 96.1% of treated bleeding events. Transient FVIII inhibitors (0.6-1.7 BU) in 4 patients resolved before study end; no unexpected safety issues were observed. rAHF prophylaxis in this study of previously treated Chinese patients with severe/moderately severe hemophilia A resulted in a clear reduction in bleeding events versus rAHF on-demand treatment, with no change in safety profile.


Assuntos
Coagulantes/administração & dosagem , Fator VIII/administração & dosagem , Hemofilia A/tratamento farmacológico , Hemorragia/prevenção & controle , Adolescente , Adulto , Criança , Pré-Escolar , China , Coagulantes/efeitos adversos , Coagulantes/farmacocinética , Esquema de Medicação , Fator VIII/efeitos adversos , Fator VIII/farmacocinética , Hemofilia A/sangue , Hemofilia A/complicações , Hemofilia A/diagnóstico , Hemorragia/etiologia , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Vigilância de Produtos Comercializados , Estudos Prospectivos , Proteínas Recombinantes/administração & dosagem , Índice de Gravidade de Doença , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
5.
BMJ Case Rep ; 14(1)2021 Jan 29.
Artigo em Inglês | MEDLINE | ID: mdl-33514621

RESUMO

Around the world, with the availability of factor concentrates, patients with haemophilia have undergone major and minor surgeries. Inhibitor development in early postoperative period leading to inadequate factor recovery and ongoing bleeding is a nightmare for both operating surgeon as well as haematologists. We describe a case of an elderly man with mild haemophilia A, who was diagnosed with pancreatic carcinoma and underwent Whipple's procedure. After an uneventful procedure, he developed high-titre inhibitors and bleeding a week after surgery posing major challenges in his management. The case highlights the importance of experienced surgeons, trained haematologists, regular monitoring of factor assay/inhibitors, adequate factor and bypassing-agent support while performing such procedures.


Assuntos
Fatores de Coagulação Sanguínea/antagonistas & inibidores , Hemofilia A/imunologia , Neoplasias Pancreáticas/cirurgia , Hemorragia Pós-Operatória/tratamento farmacológico , Idoso , Formação de Anticorpos/imunologia , Fatores de Coagulação Sanguínea/imunologia , Fator VIII/administração & dosagem , Fator VIII/uso terapêutico , Evolução Fatal , Hematologia/normas , Hemofilia A/complicações , Humanos , Fragmentos Fc das Imunoglobulinas/administração & dosagem , Fragmentos Fc das Imunoglobulinas/uso terapêutico , Hemorragias Intracranianas/complicações , Masculino , Neoplasias Pancreáticas/complicações , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/imunologia , Hemorragia Pós-Operatória/etiologia , Proteínas Recombinantes de Fusão/administração & dosagem , Proteínas Recombinantes de Fusão/uso terapêutico , Fatores de Risco , Cirurgiões/estatística & dados numéricos
6.
BMJ Case Rep ; 13(12)2020 Dec 17.
Artigo em Inglês | MEDLINE | ID: mdl-33334751

RESUMO

A 55-year-old male presented to our emergency department with haematuria and abdominal pain. Investigations including a computed tomography (CT) scan revealed an intraluminal filling defect within the left collecting system, consistent in appearance with blood clot. With an initial working diagnosis of upper tract urothelial cell carcinoma, he was discharged with plans for an urgent cystoscopy and ureteroscopy. He subsequently represented with ongoing frank haematuria, anasarca, dropping haemoglobin and new right collecting system blood clot. Subsequent investigations showed that the patient had acquired haemophilia A resulting in the episodes of haematuria, highlighted after an elevated activated partial thromboplastic time prompted a thrombophilia screen. The patient was subsequently treated with factor eight inhibitor bypass activity, corticosteroids and cyclophosphamide.


Assuntos
Dor Abdominal/etiologia , Lesão Renal Aguda/diagnóstico , Hematúria/etiologia , Hemofilia A/diagnóstico , Dor Abdominal/sangue , Dor Abdominal/urina , Lesão Renal Aguda/sangue , Lesão Renal Aguda/etiologia , Lesão Renal Aguda/urina , Fatores de Coagulação Sanguínea/uso terapêutico , Cistoscopia , Fator VIIa/uso terapêutico , Hematúria/sangue , Hematúria/urina , Hemofilia A/sangue , Hemofilia A/complicações , Hemofilia A/tratamento farmacológico , Humanos , Túbulos Renais Coletores/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Tempo de Tromboplastina Parcial , Proteínas Recombinantes/uso terapêutico , Resultado do Tratamento , Ureteroscopia , Urografia
7.
Zhonghua Xue Ye Xue Za Zhi ; 41(11): 903-907, 2020 Nov 14.
Artigo em Chinês | MEDLINE | ID: mdl-33333692

RESUMO

Objective: To study the orthopedic treatment strategy for hemophilia complicated with musculoskeletal disorders as well as the peri-operative consumption of clotting factor. Methods: Total 338 orthopedic surgeries were performed for 261 patients, average age of 30.6 y (6-65 y) , with hemophilia between January 1996 and December 2019 at our institute. Two hundred and twenty-six patients presented with bleeds within the joints. Sixty-one patients presented with intramuscular bleeds, 45 presented with hemophilic pseudotumors, and six presented with miscellaneous complaints. Strategy of clotting factor replacement therapy was designed as per differences in the level of the operation procedure. Information regarding clinical manifestation, operative strategy, clotting factor consumption, and re-operation for complications was retrospectively recorded. The costs for multiple joint procedure and single joint procedure were studied. Results: We found that 270 of the 338 surgical procedures were major surgical procedures (79.9%) . There were 203 procedures of joint arthroplasty (60%) . Fourteen patients underwent reoperations for local recurrence (4.2%) . The average factor Ⅷ consumption before the surgery was 44.4 ± 8.1 IU/kg. The average FⅧ consumption within postoperative 2 weeks was 40 962 IU (647±177 IU/kg) . Seven type A hemophilic patients developed F Ⅷ inhibitor following the surgical procedure, with an average level of 13.7±11.2 BU/mL. Sixty-eight patients underwent multiple joint procedures under one anesthesia session (26%) . There was no significant difference in the factor consumption between the multiple joint procedure and single joint procedure. Conclusions: Surgical treatment was found to be effective for hemophilic arthropathy and lesion of the musculoskeletal apparatus, with the clotting factor replacement therapy. Multiple joint procedures under one anesthesia were more cost effective for patients with hemophilia, with less factor consumption than staged single joint procedure.


Assuntos
Hemofilia A , Doenças Musculoesqueléticas/complicações , Adulto , Artrite , Fatores de Coagulação Sanguínea , Hemofilia A/complicações , Humanos , Manipulação Ortopédica , Estudos Retrospectivos
8.
Rev Med Liege ; 75(10): 665-669, 2020 Oct.
Artigo em Francês | MEDLINE | ID: mdl-33030843

RESUMO

Haemophilic arthropathy affects about half of the patients who suffer from haemophilia. Despite the fact that it's one of the main morbidity factors of haemophilia and that the pathophysiology of its mechanism is slowly better understood, its management is still under discussion. The cases of two men (53 and 54 years old) who suffer from ankle haemophilic arthropathy since several years are reported. For both cases, different aspects of the management are investigated, including a medicated and a physiotherapy approach, and an adequate orthotic. Other treatments are available and sometimes used, such as radio- or arthroscopic synovectomy, corticosteroids or platelet-rich plasma in?ltration or visco-supplementation.


Assuntos
Hemofilia A , Doenças Vasculares , Tornozelo , Articulação do Tornozelo , Hemartrose/etiologia , Hemartrose/terapia , Hemofilia A/complicações , Hemofilia A/terapia , Humanos , Masculino , Pessoa de Meia-Idade
9.
Vnitr Lek ; 66(5): 85-89, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32942877

RESUMO

The authors present clinical case of orthotopic liver transplantation for cirhosis due to chronic viral hepatitis C in a subject with severe hemophilia A. Preoperatively performed pharmacokinetic study with recombinant F VIII confirmed satisfactory in vivo recovery of 2.1 %. A bolus application of 52 units F VIII/kg body weight with target F VIII activity over 100.0 % was administred shortly before the transplantation started. Totally, 30 000 units of recombinant F VIII, 3 thrombocyte concentrates, 2 erythrocyte concentrates, 5 units of virally inactivated plasma, 1 unit of fresh frozen plasma and 3 500 antithrombin units were used. There were no perioperative or postoperative bleeding complications, F VIII substitution was stopped on postoperative day 3. The patient was discharged on twentieth postoperative day.


Assuntos
Hemofilia A , Transplante de Fígado , Fator VIII , Hemofilia A/complicações , Humanos
10.
Expert Rev Hematol ; 13(10): 1073-1079, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32862729

RESUMO

INTRODUCTION: The coronavirus disease 2019 (COVID-19) pandemic represents an unprecedented global health crisis. To combat its effects, many governments have opted for strategies of social isolation that involve a radical change in people's behavior. AREAS COVERED: For patients with hemophilia, the negative consequences of these measures can be greater, given they modify aspects of health care and lifestyles needed to counteract the adverse effects of hemophilia. The long-term consequences of the pandemic on patients with hemophilia are not well known. The aim of this special report is to show what COVID-19 could mean for this population, beyond the risk of infection. EXPERT OPINION: Considerations of the clinical, care, therapeutic, physical, nutritional, mental health, pain, and disability aspects that might be affected are included. Strategies are also suggested to minimize the effects that these issues can have on patients' lives. Patients, health professionals, and society as a whole must work together to mitigate the effects of the pandemic on people with hemophilia.


Assuntos
Infecções por Coronavirus/complicações , Hemofilia A/complicações , Pneumonia Viral/complicações , Betacoronavirus/isolamento & purificação , Infecções por Coronavirus/terapia , Avaliação da Deficiência , Gerenciamento Clínico , Hemofilia A/terapia , Humanos , Saúde Mental , Necessidades Nutricionais , Pandemias , Aptidão Física , Pneumonia Viral/terapia
11.
Tohoku J Exp Med ; 251(4): 327-336, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32788506

RESUMO

After the first cases of COVID-19 appeared in Wuhan, China at the end of 2019, the disease quickly become a pandemic that has seriously affected the economic and health systems in more than 200 countries and territories around the world. Although most patients have mild symptoms or are even asymptomatic, there are patients who can develop serious complications such as acute respiratory distress syndrome or venous thromboembolism requiring mechanical ventilation and intensive care. Hence, it is important to identify patients with a higher risk of complications in a timely manner. Thus, the objective of this paper is to review the hematological laboratory parameters that consistently are altered in COVID-19 and to identify their relationship with the severity of the disease. According to 11 selected reports, the frequency of patients aged > 65 years is higher among subjects severely affected or deceased; likewise, males predominantly suffer from comorbidities such as hypertension, diabetes or obesity. Retrospective studies have identified alterations in various hematological and inflammatory parameters as part of the host's response to infection and a secondary increased risk of different thrombotic events. Among these altered parameters, D-dimer, C-reactive protein, and interleukin-6 have been tested as prognostic biomarkers due to their close relationship with the severity of the disease. Actually, they can reliably indicate the use of antithrombotic therapy at prophylactic or therapeutic doses (mainly D-dimer), as has already been established in those patients who, after an individualized assessment, appear to be at high risk for thrombotic events.


Assuntos
Anticoagulantes/uso terapêutico , Betacoronavirus , Transtornos da Coagulação Sanguínea/etiologia , Infecções por Coronavirus/sangue , Fibrinolíticos/uso terapêutico , Pandemias , Pneumonia Viral/sangue , Fatores Etários , Betacoronavirus/patogenicidade , Betacoronavirus/fisiologia , Biomarcadores , Transtornos da Coagulação Sanguínea/tratamento farmacológico , Transtornos da Coagulação Sanguínea/prevenção & controle , Testes de Coagulação Sanguínea , Comorbidade , Infecções por Coronavirus/complicações , Infecções por Coronavirus/epidemiologia , Gerenciamento Clínico , Produtos de Degradação da Fibrina e do Fibrinogênio/análise , Hemofilia A/complicações , Humanos , Inflamação , Interleucina-6/sangue , Pneumonia Viral/complicações , Pneumonia Viral/epidemiologia , Prognóstico , Risco , Trombofilia/diagnóstico , Trombofilia/tratamento farmacológico , Trombofilia/etiologia , Trombose/etiologia , Trombose/prevenção & controle
12.
Int J Hematol ; 112(5): 621-630, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32748217

RESUMO

Emicizumab shortens activated partial thromboplastin time (aPTT) greater than Factor (F)VIII. Clot waveform analysis triggered by ellagic acid and tissue factor trigger (Elg/TF) provided a useful means of assessing emicizumab activity. Thrombin generation assays (TGA) using this trigger reagent might also overcome the difficulties associated with aPTT by emicizumab. To compare TGA triggered by Elg/TF and other reagents (FXIa, TF) for evaluating emicizumab activity. Emicizumab, FVIII, or FVIII-bypassing agents (BPAs) were incubated with FVIII-deficient plasmas prior to TGA initiated by Elg/TF (0.2 µM/0.5 pM), FXIa (5.21 pM), or TF (PPP-Reagent LOW®). Emicizumab, FVIII, or BPAs increased peak thrombin generation (peak-Th) dose-dependently using Elg/TF-trigger and the other triggers. Low responses were evident with FXIa-trigger and the enhanced effects remained below normal levels with Elg/TF-trigger. Experiments using FVIII with emicizumab demonstrated an additive effect on peak-Th using Elg/TF-trigger, and this effect appeared to be less at FVIII ≥ 40 IU/dl. BPAs with emicizumab appeared to mediate additive effects, although its effects were variable. Parameters of thrombin generation from BPAs and emicizumab with Elg/TF-trigger were improved to normal level compared to low TF-trigger. Elg/TF-TGA could evaluate global coagulation potential during emicizumab prophylaxis including concomitant therapy with FVIII or BPAs.


Assuntos
Anticorpos Biespecíficos/farmacologia , Anticorpos Biespecíficos/uso terapêutico , Anticorpos Monoclonais Humanizados/farmacologia , Anticorpos Monoclonais Humanizados/uso terapêutico , Testes de Coagulação Sanguínea/métodos , Fator VIII/uso terapêutico , Fator X/imunologia , Hemofilia A/tratamento farmacológico , Hemorragia/prevenção & controle , Tempo de Tromboplastina Parcial , Trombina/metabolismo , Relação Dose-Resposta a Droga , Sinergismo Farmacológico , Quimioterapia Combinada , Fator VIII/farmacologia , Hemofilia A/sangue , Hemofilia A/complicações , Hemorragia/etiologia , Humanos
14.
J Thromb Thrombolysis ; 50(4): 795-798, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32676882

RESUMO

The typical symptoms of COVID-19 mimic those of the common season flu. In addition, several changes in the coagulation processes have been observed. To date, it's not fully clear how COVID-19 may affect patients with hereditary bleeding disorders. Anticoagulation in patients with haemophilia is still debated, but in this case could be needed. We are reporting a case of an elderly patient with mild haemophilia A hospitalized for Sars-Cov-2. On the 15th day of hospitalization, we observed an increase of all coagulation parameters. An antithrombotic prophylaxis at low dosage was immediately started, then increased at prophylactic dosage. Even if much more data are needed to ascertain the real thrombotic risk of haemophilia A in COVID-19 patients, it's clear that the FVIII and vWF should be strictly monitored in order to promptly establish an adequate treatment and avoid the onset of thromboembolic events, even fatal, causing many deaths in COVID-19 patients.


Assuntos
Coagulação Sanguínea/efeitos dos fármacos , Infecções por Coronavirus/terapia , Enoxaparina/administração & dosagem , Fibrinolíticos/administração & dosagem , Hemofilia A/complicações , Pneumonia Viral/terapia , Trombose/prevenção & controle , Idoso , Betacoronavirus/patogenicidade , Infecções por Coronavirus/sangue , Infecções por Coronavirus/complicações , Infecções por Coronavirus/virologia , Enoxaparina/efeitos adversos , Feminino , Fibrinolíticos/efeitos adversos , Hemofilia A/sangue , Interações Hospedeiro-Patógeno , Humanos , Pandemias , Pneumonia Viral/sangue , Pneumonia Viral/complicações , Pneumonia Viral/virologia , Trombose/sangue , Trombose/diagnóstico , Trombose/virologia , Resultado do Tratamento
16.
Semin Thromb Hemost ; 46(7): 819-822, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32512586
17.
Rinsho Ketsueki ; 61(5): 451-454, 2020.
Artigo em Japonês | MEDLINE | ID: mdl-32507807

RESUMO

A 72-year-old man developed dipeptidyl peptidase-4 inhibitor-associated bullous pemphigoid (BP) during treatment for type 2 diabetes mellitus and was administered prednisolone (PSL, 0.5 mg/kg). Despite PSL treatment at a daily dose of 19 mg/day, purpura appeared on his bilateral forearms 3 months later. He was diagnosed with acquired hemophilia A (AHA) based on a prolonged activated partial thromboplastin time, decrease in factor VIII activity, and the presence of factor VIII inhibitor. Immunosuppressive therapy (IST) comprising PSL (1 mg/kg) and cyclophosphamide (300 mg/week) did not reduce the inhibitor level, and he subsequently developed the complication of pneumonia caused by a fungal infection. Weekly rituximab (RTX) therapy (375 mg/m2) for 4 weeks not only reduced the inhibitor level, but also enabled a rapid PSL dose reduction. Finally, a coagulative complete remission was achieved with improvements in pneumonia and BP. The prevention of adverse events of IST is particularly important in patients with AHA, who have a high median age. Therefore, RTX-based IST may be safer for AHA patients with complicating infections.


Assuntos
Diabetes Mellitus Tipo 2 , Hemofilia A , Penfigoide Bolhoso , Idoso , Ciclofosfamida , Diabetes Mellitus Tipo 2/tratamento farmacológico , Dipeptidil Peptidases e Tripeptidil Peptidases , Hemofilia A/complicações , Humanos , Masculino , Penfigoide Bolhoso/induzido quimicamente
18.
Surg Today ; 50(10): 1314-1317, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32572584

RESUMO

We herein report an effective procedure for liver transplantation (LT) for severe cirrhotic patients with hemophilia. Three hemophilic patients suffering from liver cirrhosis due to human immunodeficiency virus (HIV)/hepatitis C virus (HCV) coinfection underwent deceased donor LT in our institute. Basic clotting parameters were measured and evaluated during LT to determine the optimal packing procedure. All patients were treated with a gauze packing procedure to ensure stable hemostasis in relation to hemophilia during the peri-transplant period. The graft function of all patients recovered well upon gauze removal (depacking) procedure and the patients were finally discharged to home. The administration of clotting factor was discontinued on day 3 after deceased donor LT. No infectious complications occurred in any of the 3 patients. This technique could be an option for achieving successful LT in these patients. Cooperation between transplant surgeons and anesthesiologists can make this challenging operation possible.


Assuntos
Perda Sanguínea Cirúrgica/prevenção & controle , Coinfecção/complicações , Infecções por HIV/complicações , Hemofilia A/complicações , Hemostasia Cirúrgica/métodos , Hepatite C/complicações , Cirrose Hepática/etiologia , Cirrose Hepática/cirurgia , Transplante de Fígado/métodos , Adulto , Humanos , Masculino , Pessoa de Meia-Idade , Índice de Gravidade de Doença , Resultado do Tratamento
19.
Medicine (Baltimore) ; 99(26): e20851, 2020 Jun 26.
Artigo em Inglês | MEDLINE | ID: mdl-32590782

RESUMO

INTRODUCTION: The Antopol-Goldman lesion (AGL), which expresses subepithelial hemorrhage in the renal pelvis, was first defined by Antopol and Goldman in 1948. The objective of this study is to report the first case of AGL in patients with congenital hemophilia and review the relevant literature. PATIENT CONCERNS: A 32-year-old male patient diagnosed with congenital hemophilia A (FVIII = %4) with high responding inhibitors (7.4 BU) was admitted to our emergency department with gross hematuria and sudden onset flank pain. DIAGNOSIS: Abdominal computed tomography (CT-scan) presented a hyperdense lesion in the left ureteropelvic junction with Hounsfield Units of 56 compatibles with hemorrhage. INTERVENTIONS: The patient was given 4500 IU of factor eight inhibitor bypass activity (FEIBA) intravenously twice daily for 5 days. Subsequently, 4500 IU of FEIBA was administrated once a day for 2 days. OUTCOMES: The patient's complaints disappeared on the fourth day of treatment. Macroscopic and microscopic hematuria was not seen in the following days. Follow-up CT was done 3 months after discharge and showed normal left renal pelvis without hyperdenosis. Follow-up CT was performed 3 months after discharge and presented normal left renal pelvis with no hyperdense lesion. CONCLUSION: Although very rare, AGL should be kept in mind in the differential diagnosis of renal pelvic hemorrhage. In the patient who has an underlying history of coagulopathy nephrectomy can be avoided when there is awareness of AGL.


Assuntos
Hemofilia A/complicações , Hemorragia/etiologia , Administração Intravenosa , Adulto , Fatores de Coagulação Sanguínea/uso terapêutico , Hematúria/etiologia , Hemorragia/tratamento farmacológico , Hemorragia/prevenção & controle , Humanos , Pelve Renal/anormalidades , Pelve Renal/fisiopatologia , Masculino , Tomografia Computadorizada por Raios X/métodos , Ureter/anormalidades , Ureter/fisiopatologia
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