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1.
Medicine (Baltimore) ; 99(9): e19339, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32118768

RESUMO

Ageing people with hemophilia (PWH) have a higher prevalence of hypertension than the general population. This study aimed to determine whether macroscopic hematuria was associated with hypertension in PWH in a post hoc analysis using data from a cross-sectional study conducted by the ADVANCE Working Group (the H3 study), which included PWH ≥ 40 years of age. Data from 16 contributing centers, located in 13 European countries and Israel, were analyzed using logistic regression models. Of 532 recruited PWH in the H3 study, 117 had hypertension and a positive family history of hypertension (hypertension FH+), 75 had hypertension and a negative family history of hypertension (hypertension FH-), 290 had no diagnosis of hypertension, and the remaining 50 had missing hypertension data. Logistic regressions showed that macroscopic hematuria was associated with hypertension FH+, both in the univariate (OR = 1.84 [1.17-2.90], P = .01) and in the multivariate model (OR = 1.80 [1.03-3.16], P = .04). Macroscopic hematuria was not associated with hypertension FH-. Moreover, in a multivariate logistic regression the odds of hypertension FH+ were increased with the number of macroscopic hematuria episodes. The association between macroscopic hematuria and hypertension was significant for PWH with a family history of hypertension.


Assuntos
Hematúria/etiologia , Hemofilia A/complicações , Hipertensão/complicações , Adulto , Idoso , Idoso de 80 Anos ou mais , Envelhecimento , Estudos Transversais , Feminino , Hematúria/epidemiologia , Hematúria/fisiopatologia , Hemofilia A/epidemiologia , Hemofilia A/fisiopatologia , Humanos , Hipertensão/epidemiologia , Hipertensão/fisiopatologia , Israel/epidemiologia , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Fatores de Risco
4.
N Engl J Med ; 382(1): 29-40, 2020 01 02.
Artigo em Inglês | MEDLINE | ID: mdl-31893514

RESUMO

BACKGROUND: Adeno-associated virus (AAV)-mediated gene therapy is under investigation as a therapeutic option for persons with hemophilia A. Efficacy and safety data include 3 years of follow-up after a single administration of AAV5-hFVIII-SQ. METHODS: We report durable efficacy, long-term safety, and clinical and biologic results in 15 adults with severe hemophilia A (factor VIII level, ≤1 IU per deciliter) who had received a single infusion of AAV5-hFVIII-SQ at various dose levels. We evaluated the factor VIII level, annualized rate of bleeding events, use of factor VIII, safety, expression kinetics, and biologic markers of AAV transduction for up to 3 years. RESULTS: Three years after infusion, two participants (one who had received 6×1012 vector genomes [vg] per kilogram of body weight and one who had received 2×1013 vg per kilogram) had factor VIII expression of less than 1 IU per deciliter, as assessed on chromogenic assay. Seven participants (who had received 6×1013 vg per kilogram) had a median factor VIII expression of 20 IU per deciliter; the median number of annualized treated bleeding events was 0, and the median use of exogenous factor VIII was reduced from 138.5 infusions to 0 infusions per year. Bleeding in all target joints (major joints with ≥3 bleeding events within 6 months) in this cohort resolved (≤2 bleeding events within 12 months). Two years after infusion, six participants (who had received 4×1013 vg per kilogram) had a median factor VIII expression of 13 IU per deciliter; the median annualized rate of bleeding events was 0, and the median use of factor VIII was reduced from 155.5 infusions to 0.5 infusions per year. Bleeding in target joints resolved in five of six participants. The factor VIII pharmacodynamic profiles reflected cellular turnover in the blood and molecular events leading to episomal DNA stabilization for persistent expression, findings that are consistent with previous observations in two model systems. Transgene-derived human factor VIII (hFVIII) protein activity mirrored native hFVIII in hemostatic ability. No inhibitor development, thromboses, deaths, or persistent changes in liver-function tests were observed. CONCLUSIONS: Gene therapy with AAV5-hFVIII-SQ vector in participants with hemophilia A resulted in sustained, clinically relevant benefit, as measured by a substantial reduction in annualized rates of bleeding events and complete cessation of prophylactic factor VIII use in all participants who had received 4×1013 vg per kilogram or 6×1013 vg per kilogram of the gene therapy. (Funded by BioMarin Pharmaceutical; ClinicalTrials.gov number, NCT02576795; EudraCT number, 2014-003880-38.).


Assuntos
Dependovirus , Fator VIII/genética , Terapia Genética , Vetores Genéticos , Hemofilia A/terapia , Adulto , Biomarcadores , Coagulantes/uso terapêutico , Fator VIII/uso terapêutico , Seguimentos , Terapia Genética/efeitos adversos , Hemofilia A/complicações , Hemorragia/etiologia , Hemorragia/prevenção & controle , Humanos , Infusões Intravenosas , Masculino , Pessoa de Meia-Idade , Transgenes , Adulto Jovem
5.
World Neurosurg ; 133: 112-120, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31562972

RESUMO

BACKGROUND: Retroclival hematomas are a rare entity and may occur in 3 compartments, namely the epidural, subdural, and subarachnoid spaces. They are frequently secondary to trauma. Hemophilia is a clinical syndrome affecting usually men and characterized by the inherited tendency to bleed excessively after slight injury. Hemophilia is caused by a specific defect of coagulation factor VIII. The main concern associated with the disease is bleeding, especially after trauma and surgeries. The most serious site of bleeding is the central nervous system. CASE PRESENTATION: An 11-year-old boy diagnosed with hemophilia presented after sustaining a fall. On arrival to the emergency department, his vitals where within normal range and he was fully conscious. Neurologic examination was significant for bilateral abducens nerve palsy; the rest of the examination was unremarkable. Imaging studies with computed tomography (CT) scan and brain magnetic resonance imaging showed subacute retroclival subdural hemorrhage with left cerebellar and upper cervical spine extension. Follow-up imaging with CT scan showed progressive resolution of the hematoma, and the patient had a stable clinical course while receiving factor VII replacement. CONCLUSIONS: Retroclival subdural hematomas are rare and may present either spontaneously or after trauma. Conservative treatment is the usual course of treatment. Patients with hemophilia A are under a constant threat from bleeding, either spontaneous or after trivial injury. The most common cause of death in this patient population is intracranial hemorrhage. The most important aspect of intracranial hematoma management is the early replacement therapy of deficient coagulation factors in patients with hemophilia.


Assuntos
Fossa Craniana Posterior/diagnóstico por imagem , Hematoma Subdural Intracraniano/complicações , Hemofilia A/complicações , Criança , Hematoma Subdural Intracraniano/diagnóstico por imagem , Hemofilia A/diagnóstico por imagem , Humanos , Masculino , Tomografia Computadorizada por Raios X
6.
Rinsho Ketsueki ; 60(11): 1567-1572, 2019.
Artigo em Japonês | MEDLINE | ID: mdl-31839636

RESUMO

A 72-year-old man was hospitalized because of thrombocytopenia (0.5×104/µl) and anemia. The bone marrow test result revealed excessive numbers of megakaryocytes and no platelet adhesion. Furthermore, platelet-associated immunoglobulin G levels were high, and he was tested positive for Helicobacter pylori antibody. On the basis of these findings, immune thrombocytopenia was diagnosed. The patient was initially treated with eradication therapy; prednisolone, 20 mg/day (0.5 mg/kg) and a thrombopoietin receptor agonist 12.5 mg/day. During the course of treatment, the anemia worsened. Detailed examination revealed marked prolongation of activated partial thromboplastin time and inhibition of factor VIII activity. Therefore, he was diagnosed with acquired hemophilia A. Although extensive muscle hemorrhage had occurred, hemostatic therapy comprising intensification of the immunosuppressive therapy and administration of recombinant activated factor VII resulted in successful hemostasis. As the treatment progressed, inhibition of factor VIII recurred temporarily; however, immunosuppressive therapy was continued. No recurrence was observed even after 1 year of the onset of both diseases.


Assuntos
Hemofilia A , Hemostáticos , Trombocitopenia , Idoso , Hemofilia A/complicações , Hemorragia , Humanos , Masculino , Trombocitopenia/complicações
7.
Medicine (Baltimore) ; 98(46): e17998, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31725667

RESUMO

Giant abdominal hemophilic pseudotumor is exceedingly rare, thus may bring great challenges to the timely and proper diagnosis and treatment of clinicians. The only definitive management is complete removal of the abdominal hemophilic pseudotumor. The objective of this article is to report surgical treatment and follow-up outcomes of three unusual cases with giant abdominal hemophilic pseudotumor.We describe 3 patients with giant hemophilic pseudotumor involving the abdomen who were successfully treated with tumor resection. On presentation to our institution, the patients all had signs of giant cystic lesions in abdomen, and the patients' most outstanding complaints were aggravated abdominal pain. All of three patients underwent complete excision of abdominal hemophilic pseudotumor. The patients showed adequate pain relief compared with the previous status.Surgical resection is the most effective treatment option for patients with giant abdominal hemophilic pseudotumor who can undergo appropriate surgical treatment. This represents a safe and reasonable approach to sustainably relieve pain and other symptoms with giant hemophilic pseudotumor in the abdomen. Perioperative coagulation factor replacement therapy is also of great significance in reducing the risks and complications.


Assuntos
Hematoma/complicações , Hematoma/cirurgia , Hemofilia A/complicações , Adulto , Humanos , Masculino , Pessoa de Meia-Idade
10.
Blood Coagul Fibrinolysis ; 30(1S Suppl 1): S11-S13, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31517710

RESUMO

: The role of the orthopedic surgeon is to use invasive and/or surgical methods to treat the musculoskeletal disorders suffered by persons with hemophilia, always within the context of a multidisciplinary team. Muscle hematomas must be diagnosed as early as possible and be subjected to continuous treatment until full resolution, as they are associated with the risk of severe complications (compartment syndromes and pseudotumors). Arthrocentesis (extraction of intra-articular blood) is recommended in cases of acute and profuse hemarthrosis. Synovectomy is mandatory in the case of synovitis. Radiosynovectomy plays a key role as it has been shown to reduce bleeding by 65%. Our department uses Yttrium-90 in knees and Rhenium-186 in elbows and ankles. Radiosynovectomy is our treatment of choice for synovitis whereas arthroscopic synovectomy is resorted to as second-line treatment. Total knee replacement (TKR) has shown itself to be effective for treating severe hemophilic arthropathy, although the infection risk in patients with hemophilia is higher than in patients with osteoarthritis (1-2 vs. 7%).


Assuntos
Hemofilia A/complicações , Artropatias/etiologia , Artropatias/cirurgia , Artroplastia do Joelho/métodos , Hemartrose/etiologia , Hemartrose/cirurgia , Humanos , Radioisótopos/uso terapêutico , Rênio/uso terapêutico , Sinovectomia/métodos , Sinovite/etiologia , Sinovite/cirurgia , Radioisótopos de Ítrio/uso terapêutico
11.
Blood Coagul Fibrinolysis ; 30(1S Suppl 1): S19-S21, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31517712

RESUMO

: Prophylaxis has helped improve patients' perception of their quality of life, enabling them to lead a more normal life. For these reasons prophylactic treatment is nowadays considered a gold standard in the treatment of severe hemophilia A or B. Despite its benefits in terms of preventing bleeding and preserving patients' health, this intensive treatment is not always adhered to by patients with hemophilia - promotion of adherence should involve a multidisciplinary team which addresses not only the clinical aspects of a condition but also the different psychosocial aspects affecting patients and their (social, family and healthcare) environment.


Assuntos
Hemofilia A/prevenção & controle , Hemofilia B/prevenção & controle , Hemorragia/prevenção & controle , Gerenciamento Clínico , Hemofilia A/complicações , Hemofilia B/complicações , Hemorragia/etiologia , Humanos , Estilo de Vida , Cooperação do Paciente , Qualidade de Vida
12.
Wien Klin Wochenschr ; 131(21-22): 558-566, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31535221

RESUMO

BACKGROUND: Hemophilia is a congenital bleeding disorder with an estimated frequency of 1:10,000 births. Repeated joint bleeding is a hallmark of the disorder and leads to painful hemophilic arthropathy. Regular exercise can help improve joint stability and function, reduce the risk of injury and bleeding and improve physical fitness and quality of life. This method paper describes an online training concept aiming to offer access to appropriate exercise instructions for people with hemophilia who are not able to attend regular training at a hemophilia center. METHODS: The online exercise program is accessible through the homepage of the Department of Physical Medicine, Rehabilitation and Occupational Medicine of the Medical University Vienna as well as through scanning a QR code printed on information material using a smart phone or tablet. RESULTS: The program contains exercises to improve mobility, coordination, muscular strength and flexibility. A brief introduction is given by a hematologist, a pediatrician and a physiatrist. An introductory video informs about contraindications and essential precautions, such as medical attendance and sufficient factor therapy to consider before starting the training. Another video gives advice on the exercise composition. The demonstrated exercises are explained by a physician and are available for adults and children. To individualize training recommendations and offer further diagnostic tools and physical treatment options as necessary, the Department of Physical Medicine, Rehabilitation and Occupational Medicine of the Medical University of Vienna will establish consultation hours for people with hemophilia. CONCLUSION: As hemophilia is an orphan disease, patients are mainly treated in specialized centers. For patients who live far from these centers or have limited access to a training there for other reasons, the physical medicine consultation hour and the implementation of online exercise instructions offer individually adapted exercise information for a regular home-based training to benefit from increased physical fitness and joint stability.


Assuntos
Artralgia/terapia , Hemofilia A , Aptidão Física , Adulto , Artralgia/etiologia , Criança , Exercício/fisiologia , Terapia por Exercício , Hemofilia A/complicações , Humanos , Qualidade de Vida
13.
Medicina (Kaunas) ; 55(10)2019 Sep 24.
Artigo em Inglês | MEDLINE | ID: mdl-31554178

RESUMO

Introduction: Concern about weight gain among people has been high due to negative health consequences in addition to the increasing prevalence of the problem. Overweight and obesity also occur in patients with hemophilia. Analysis of literature shows that increased body weight might have a biomechanical effect on the spatial orientation of the pelvis and the lumbar spine. The aim of this study was to determine the correlation between body mass index (BMI) and the parameters characterizing the alignment of the sacrum (SS, sacral slope), the pelvis (PT, pelvic tilt; PI, pelvic incidence) and the angle value of lumbar lordosis (LL, lumbar lordosis) assessed in the sagittal plane among patients with hemophilia. Materials and methods: A total of 49 patients were subjected to the study, 23 of whom met the inclusion criteria. Body weight and height were measured. Measurement of the angle values of indicators characterizing the position of the lumbar-pelvic complex was established based on X-ray imaging analysis. Results: Analysis of the correlation between the BMI and sacral, pelvic, and lumbar indicators evaluated in the sagittal plane in the study group of patients with hemophilia showed a correlation between BMI and SS (r = 0.48). SS values were significantly and positively related to PI (r = 0.6; p = 0.002) and LL (r = 0.46; p = 0.02). The results obtained indicate the BMI relationship with the setting of the sacrum in the sagittal plane (SS). After adjusting for the knee flexion contracture, the correlation on the border of significance (b = 0.73, p = 0.07) between the body mass index and the spatial orientation of the pelvis and the spine was revealed. Conclusion: We hypothesize that increased body weight among people with hemophilia might have an effect on the positioning of the lumbosacral region. Therefore, it is believed that preventing obesity among people with hemophilia can contribute to a smaller number of intra-articular hemorrhages and better orthopedic condition of the limb joints, and thus could avoid changes in the lumbosacral region as well as their consequences.


Assuntos
Índice de Massa Corporal , Hemofilia A/complicações , Hemofilia B/complicações , Vértebras Lombares/patologia , Região Lombossacral/patologia , Obesidade/complicações , Curvaturas da Coluna Vertebral/etiologia , Adulto , Humanos , Região Lombossacral/anatomia & histologia , Região Lombossacral/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Obesidade/patologia , Adulto Jovem
14.
BMC Musculoskelet Disord ; 20(1): 402, 2019 Sep 04.
Artigo em Inglês | MEDLINE | ID: mdl-31481049

RESUMO

BACKGROUND: The lower limb joints, including hip and knee, are the most commonly involved joints in haemophilic arthropathy. With a higher risk of transfusion, total hip and knee arthroplasty (THA and TKA) are still the first choice after failure of conservative treatment. In the present study, we aimed to analyze clinical outcomes and complications rate after total joint arthroplasty of the lower limbs using tranexamic acid (TXA) or not. METHODS: Thirty-four patients with haemophilia A undergoing 24 TKA and 18 THA were evaluated in this retrospective study (No. 201302009). Based on using TXA or not, they were divided into either TXA (12 knees and 10 hips) or Non-TXA groups (12 knees and 8 hips). Total blood loss, intraoperative blood loss, total amount of FVIII usage, range of motion, inflammatory biomarkers, joint function, pain status, complication rate and patient satisfaction were assessed and compared at a mean follow-up of 68 months. RESULTS: Usage of TXA can decrease not only the perioperative blood loss (p = 0.001), transfusion rate (p = 0.017) and supplemental amount of FVIII (p < 0.001) but also swelling ratio, surgical joint pain. Moreover, compared with non-TXA group, the patients in TXA group had a lower level of inflammatory biomarkers and better joint function. CONCLUSION: The hemophiliacs treated with TXA had less perioperative blood loss, hidden blood loss, transfusion rate, a lower ratio of postoperative knee swelling, less postoperative joint pain, lower levels of inflammatory biomarkers and better joint function. Further studies need performing to assess the long-term effects of TXA in these patients.


Assuntos
Antifibrinolíticos/administração & dosagem , Artroplastia de Quadril/efeitos adversos , Artroplastia do Joelho/efeitos adversos , Hemofilia A/complicações , Ácido Tranexâmico/administração & dosagem , Administração Intravenosa , Adulto , Antifibrinolíticos/efeitos adversos , Artralgia/epidemiologia , Artralgia/etiologia , Artralgia/prevenção & controle , Perda Sanguínea Cirúrgica/prevenção & controle , Perda Sanguínea Cirúrgica/estatística & dados numéricos , Transfusão de Sangue/estatística & dados numéricos , Fator VIII/administração & dosagem , Feminino , Hemofilia A/diagnóstico , Hemofilia A/tratamento farmacológico , Articulação do Quadril/fisiologia , Articulação do Quadril/cirurgia , Humanos , Articulação do Joelho/fisiologia , Articulação do Joelho/cirurgia , Masculino , Pessoa de Meia-Idade , Osteoartrite do Quadril/complicações , Osteoartrite do Quadril/fisiopatologia , Osteoartrite do Quadril/cirurgia , Osteoartrite do Joelho/complicações , Osteoartrite do Joelho/fisiopatologia , Osteoartrite do Joelho/cirurgia , Dor Pós-Operatória/epidemiologia , Dor Pós-Operatória/etiologia , Dor Pós-Operatória/prevenção & controle , Hemorragia Pós-Operatória/epidemiologia , Hemorragia Pós-Operatória/etiologia , Hemorragia Pós-Operatória/prevenção & controle , Cuidados Pré-Operatórios/métodos , Estudos Prospectivos , Amplitude de Movimento Articular , Recuperação de Função Fisiológica , Estudos Retrospectivos , Ácido Tranexâmico/efeitos adversos , Resultado do Tratamento
15.
Acta Med Port ; 32(9): 614-617, 2019 Sep 02.
Artigo em Inglês | MEDLINE | ID: mdl-31493366

RESUMO

Intramural hematoma of the colon is very rare, particularly when associated with the development of autoantibodies against factor VIII.We report a case of a 66-year-old man with abdominal pain, hematochezia and clots in the left colon, without any radiologic signs of active bleeding or bowel occlusion or analytical changes in routine coagulation screening, but with positive autoantibodies against factor VIII. The clinical instability prompted surgical exploration. An intramural hematoma of the left colon was found, and a left colectomy was performed. The patient was treated with hemoderivatives and corticosteroids with clinical improvement. The diagnosis of spontaneous intramural hematoma might be a challenge, particularly in the absence of clinical suspicion. An early recognition is essential for a positive outcome. This case highlights a rare cause of bleeding and intestinal obstruction, but also the difficulty and relevance of establishing a clinical diagnosis when diagnostic tests are not completely informative.


Assuntos
Dor Abdominal/etiologia , Autoanticorpos/análise , Doenças do Colo/etiologia , Fator VIII/imunologia , Hematoma/etiologia , Hemofilia A/complicações , Idoso , Doenças do Colo/diagnóstico , Doenças do Colo/cirurgia , Colonoscopia , Fator VIII/análise , Hemorragia Gastrointestinal/etiologia , Hematoma/diagnóstico , Hematoma/cirurgia , Hemofilia A/diagnóstico , Humanos , Obstrução Intestinal/etiologia , Masculino
16.
BMJ Case Rep ; 12(9)2019 Sep 06.
Artigo em Inglês | MEDLINE | ID: mdl-31494593

RESUMO

A 55-year-old man with a history of haemophilia A and bilateral haemophilic arthropathy of the elbows presented with an enlarging left elbow mass and worsening paresthesias in the ulnar distribution of the left hand. The mass, originally thought to be olecranon bursitis and treated as such, was found to be due to a haemophilic pseudotumour. The patient underwent successful excision of the haemophilic pseudotumour with concomitant ulnar nerve decompression and had sustained resolution of the pseudotumour and symptoms at 10 years of follow-up. This case demonstrates the need for consideration of haemophilic pseudotumour in the differential diagnosis for olecranon masses and cubital tunnel compressive neuropathy in patients with haemophilia, and highlights the viability of surgical excision as a therapeutic option for the treatment of haemophilic pseudotumours in the elbow.


Assuntos
Granuloma de Células Plasmáticas/fisiopatologia , Hemofilia A/complicações , Olécrano/patologia , Síndromes de Compressão do Nervo Ulnar/etiologia , Descompressão Cirúrgica , Granuloma de Células Plasmáticas/diagnóstico por imagem , Granuloma de Células Plasmáticas/cirurgia , Hemofilia A/patologia , Hemofilia A/cirurgia , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Olécrano/diagnóstico por imagem , Resultado do Tratamento , Síndromes de Compressão do Nervo Ulnar/fisiopatologia , Síndromes de Compressão do Nervo Ulnar/cirurgia
17.
Haemophilia ; 25(5): e315-e321, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31393063

RESUMO

INTRODUCTION: Haemophilia patients may exhibit lower levels of bone mineral density (BMD) than the general population for a variety of reasons. AIM: We aimed to investigate decreased BMD in people with severe adult haemophilia A (PWH) living in eastern Turkey, and to evaluate the related potential risk factors. METHODS: The study included 41 PWH and 40 healthy volunteers. Dual-energy x-ray absorptiometry (DXA) was used to measure the BMD. Blood tests and body mass index (BMI) were recorded. The Functional Independence Score in Hemophilia (FISH) test was used to measure functional ability status. RESULTS: There was a significant difference between the PWH and control groups with respect to femoral neck and total hip BMD (in g/cm2 ), but the difference for lumbar spine was not significant (P = .017, P < .001, P = .071, respectively). In PWH, patients under 50 years of age, 19.4% were found to have "lower than expected" BMD levels for their age, while 27.8% showed "low normal" levels. In PWH, osteoporosis was found in 60% of the patients over 50 and osteopenia in 20%. Vitamin D insufficiency and deficiency were present in 63.4% of the PWH, significantly higher than the control group (37.5%; P < .001). CONCLUSION: The results indicated that the decrease in BMD was significantly greater in patients with severe haemophilia A than in the normal healthy population. This reduction was correlated with BMI, vitamin D and low functional ability status. However, in multivariate analysis, none of these was a strong independent risk factor.


Assuntos
Densidade Óssea/genética , Hemofilia A/complicações , Osteoporose/complicações , Adolescente , Adulto , Estudos de Casos e Controles , Humanos , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Adulto Jovem
18.
Thromb Res ; 181: 106-111, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31386935

RESUMO

INTRODUCTION: Data on the prevalence of hypertension and cardiovascular diseases (CVD) among persons with haemophilia (PWH) vary. Sweden has a long tradition of maintaining population-based data registries, and there is extensive follow-up of haemophilia patients due to the use of prophylaxis over decades. We evaluated the prevalence of these diseases among Swedish PWH compared to matched controls using a longitudinal study design. METHODS: Data were obtained from the National Patient Registry and linked to records of persons with haemophilia enrolled in the haemophilia centres. For each subject, five gender and age matched controls were identified. RESULTS: We identified 193 (19.7%) diagnoses of hypertension in PWH born in 1978 or earlier over ≥30 years compared with 550 (11.2%) among controls. The median ages and interquartile ranges were 60.0 (42.8, 69.9) and 57.2 (42.6, 70.6) years. The hazard rate (HR) for hypertension, PWH vs. controls, was 2.1, 95% CI: [1.8; 2.5], p < 0.001. The findings were similar in subgroup analyses of patients with non-severe and severe haemophilia with or without HIV and/or viral hepatitis. Angina pectoris was diagnosed in 69 (4.8%) of patients censored at age 75 compared with 311 (4.3%) in controls, and myocardial ischemia in 84 (5.9%) compared with 442 (6.2%). As a cause of death, the HR for myocardial ischemia, comparing PWH and controls, was 0.58, 95% CI: [0.42, 0.80], p = 0.001. CONCLUSION: Our data support an increased prevalence of hypertension among persons with haemophilia. The prevalence of CVD seems to be similar to that of controls, but with lower mortality.


Assuntos
Doenças Cardiovasculares/etiologia , Hemofilia A/complicações , Hipertensão/etiologia , Adulto , Idoso , Feminino , Hemofilia A/patologia , Humanos , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Sistema de Registros , Fatores de Risco , Suécia , Adulto Jovem
19.
Clin Appl Thromb Hemost ; 25: 1076029619862052, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31298044

RESUMO

Hemophilic arthropathy from joint bleeding remains a complication with major morbidity in the increasingly aging patients with hemophilia. Prophylactic clotting factor infusions, based on pharmacokinetic dosing to reduce bleeding rates, are being explored more and more. However, there is little evidence on the benefits of pharmacokinetic dosing in direct association with bleeding events. Here, we prospectively followed a cohort of adult patients with hemophilia A and B (n = 26) and arthropathic joints on various clotting factor products over a period of 2 years with clinical and radiographic joint health assessments, frequent joint ultrasound, and pharmacokinetic studies. Joint bleeds and synovitis with synovial vascularity changes were objectively diagnosed by musculoskeletal ultrasound and power Doppler and analyzed in relation to pharmacokinetic, joint- and patient-specific parameters. Results revealed that, contrary to common beliefs, bleeding episodes were not readily explained by pharmacokinetic features, as they were not associated with more time spent below certain clotting factor thresholds. Joint bleeding was found to be associated with prominent vascularity changes, suggesting that vascular remodeling and leakiness may contribute to joint bleeding that cannot be prevented by clotting factor replacement alone.


Assuntos
Fatores de Coagulação Sanguínea/farmacocinética , Vasos Sanguíneos/patologia , Hemartrose/etiologia , Hemartrose/prevenção & controle , Hemofilia A/complicações , Hemofilia B/complicações , Adulto , Fatores de Coagulação Sanguínea/análise , Fatores de Coagulação Sanguínea/uso terapêutico , Vasos Sanguíneos/diagnóstico por imagem , Fragilidade Capilar , Hemartrose/diagnóstico por imagem , Hemartrose/tratamento farmacológico , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Ultrassonografia/métodos , Remodelação Vascular
20.
Haemophilia ; 25(5): 782-788, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31291048

RESUMO

INTRODUCTION: Haematuria is a recognized complication of haemophilia A and B (HA, HB). Adult persons with haemophilia (PWH) have a higher prevalence of renal disease than the general population. There is limited literature investigating the prevalence of haematuria in paediatric PWH. AIM: Our paediatric haemophilia treatment centre (HTC) had previously used quality improvement methods to increase the frequency of screening urinalyses at annual comprehensive visits. We retrospectively reviewed the data collected to determine the prevalence of haematuria and explore for associations in those with haematuria. METHODS: Retrospective chart review to identify the frequency of haematuria on screening urinalysis in all male PWH ≥2 years old. Haematuria was defined as ≥3 red blood cells (RBCs) per high power field. Univariate logistic regression was performed to evaluate for associations with haematuria. RESULTS: A total of 93 patients met eligibility criteria. Sixty-seven with HA (11 mild, 17 moderate, 39 severe) and 26 with HB (three mild, 16 moderate, seven severe). Forty-two of ninety-three (45%) patients were identified as having haematuria (median RBCs 7, mean RBCs 332). Of those with haematuria, 76% were identified by screening UA, as opposed to clinical symptoms, and 52% had recurrent haematuria. Older age and HA were associated with an increased likelihood of haematuria. CONCLUSIONS: Our study demonstrated that the prevalence of haematuria was high in PWH treated at our paediatric HTC. Future investigation is needed to determine the population-wide prevalence of haematuria in paediatric PWH and its impact on renal function.


Assuntos
Hematúria/etiologia , Hemofilia A/complicações , Hemofilia B/complicações , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Estudos Retrospectivos , Fatores de Risco , Adulto Jovem
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