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1.
Haemophilia ; 30(2): 513-522, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38282205

RESUMO

AIM: Joint damage due to haemarthrosis can be effectively monitored with point-of care ultrasound using the Haemophilia Early Arthropathy Detection with US (HEAD-US) scoring system. A post hoc comparative analysis of the joint status of patients with severe haemophilia A (HA) or B (HB) was performed. METHODS: The databases of two observational, cross-sectional studies that recruited patients with HA or HB from 12 Spanish centres were analysed to compare the status of the elbows, knees and ankles in patients with severe disease according to treatment modality. The HEAD-US score was calculated in both studies by the same trained operators. RESULTS: Overall, 95 HA and 41 HB severe patients were included, with a mean age of 35.2 ± 11.8 and 32.7 ± 14.2 years, respectively. The percentage of patients who received prophylaxis, over on-demand (OD) treatment, was much higher in HA (91.6%) than in HB (65.8%) patients. With a similar number of target joints, the HEAD-US score was zero in 6.3% HA and 22.0% HB patients (p < .01), respectively. The HA population showed significantly worse HEAD-US scores. Whilst osteochondral damage occurred more frequently in patients OD or tertiary prophylaxis, our data suggest that articular damage is less prominent in primary/secondary prophylaxis, regardless of the type of haemophilia. These latter treatment modalities were also associated with a lower prevalence of synovial hypertrophy, particularly in HB patients. CONCLUSION: This post hoc analysis indicates that joint status seems to be significantly influenced by haemophilia type (HA or HB) and treatment modality in these severe Spanish populations with severe disease. Continuing HEAD-US monitoring for the early detection and management of intra-articular abnormalities, as well as more efficiently tailored therapies should be warranted.


Assuntos
Artrite , Hemofilia A , Artropatias , Humanos , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Hemofilia A/tratamento farmacológico , Espanha , Estudos Transversais , Artropatias/complicações , Hemartrose/complicações , Articulações , Artrite/complicações
2.
Haemophilia ; 30(1): 98-105, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37983883

RESUMO

INTRODUCTION: The impact of moderate haemophilia on health-related quality of life (HRQoL) and physical activity (PA) is not well known. In previous studies, persons with factor VIII/factor IX activity (FVIII/FIX:C) below 3 IU/dL were associated with a more severe bleeding phenotype than predicted. AIM: To explore HRQoL and PA in patients with moderate haemophilia A (MHA) and B (MHB). METHODS: A cross-sectional, multicentre study covering patients with MHA and MHB in Sweden, Finland, and Norway. HRQoL was assessed with the EuroQoL 5-Dimensions (EQ-5D) form and PA with the International Physical Activity Questionnaire among participants aged ≥15 years. RESULTS: We report on 104 patients aged 15-84 years from the MoHem study. Overall, EQ-5D utility was .85 (median) (Q1-Q3 0.73-1.0) with corresponding visual analogue scale (VAS) 80 (70-90), which were similar regardless of treatment modality, FVIII/FIX:C, and MHA or MHB. Pain and mobility were most frequently affected dimensions. Utility (r = -.54), VAS (r = -.42), and PA (r = -.32) correlated negatively with arthropathy (HJHS). Only patients aged 41-50 years displayed lower utility (p = .02) and VAS (p < .01) than the Norwegian population norm. Patients on prophylaxis aged 35-54 years reported higher PA than those treated on-demand (p = .01). CONCLUSION: Haemophilic arthropathy had negative impact on HRQoL and PA in Nordic patients with moderate haemophilia. Middle-aged patients captured lower utility and VAS than observed in the general population. Tailored prophylaxis and improved joint health may influence positively on HRQoL and PA also in moderate haemophilia.


Assuntos
Hemofilia A , Artropatias , Pessoa de Meia-Idade , Humanos , Hemofilia A/tratamento farmacológico , Qualidade de Vida , Estudos Transversais , Artropatias/complicações , Fator IX/uso terapêutico , Exercício Físico
3.
Haemophilia ; 30(1): 204-213, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38082545

RESUMO

INTRODUCTION: In patients with haemophilia, repeated bleeding in large joints leads to chronic haemophilic arthropathy, a rare disease that can be managed surgically with ankle arthrodesis or with total ankle replacement (TAR). TAR has been reported to provide good surgical results in the medium/long-term and allow preservation of joint mobility but the medical therapeutic management of the patients has not been described. AIM: To describe the medical therapeutic management of TAR. METHODS: All patients with haemophilia A/B, with haemophilic ankle arthropathy, and who underwent TAR between April 2006 and October 2019 were retrospectively included. Factor consumption, perioperative and early complications, volume of blood lost, and orthopaedic data were collected. RESULTS: A total of 25 patients underwent 29 TAR (mean age was 44.7 years [range: 26-65]). In the 17 patients with HA without history of anti-FVIII inhibitor, the mean ± SD consumption the day of surgery was 116 ± 16 UI/kg when clotting factors were administered by continuous infusion, 106 ± 13 UI/kg when SHL factors were administered by bolus infusion, and 75 ± 22 UI/kg when EHL factors were administered by bolus infusion. During hospitalisation, the mean factor cost was €38,073 (83.7% of the total cost of surgery). Mean blood loss was significantly lower in patients treated with tranexamic acid (164 mL, range: 40-300) than in those not (300 mL, range: 70-800; p = .01). Six patients had haematoma. The 10-year survival free of any prosthesis removal/arthrodesis was estimated to be 92.2% (95% CI [83; 100]). CONCLUSION: The medical therapeutic management of TAR is complex, carried out by a multidisciplinary team but effective in avoiding the occurrence of complications.


Assuntos
Artrite , Artroplastia de Substituição do Tornozelo , Hemofilia A , Artropatias , Humanos , Adulto , Artroplastia de Substituição do Tornozelo/métodos , Estudos Retrospectivos , Resultado do Tratamento , Articulação do Tornozelo/cirurgia , Hemofilia A/complicações , Hemofilia A/cirurgia , Artropatias/complicações , Artrite/complicações , Artrodese
4.
Instr Course Lect ; 73: 221-230, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38090900

RESUMO

Neuropathic destabilization of the hindfoot and/or ankle is a significant complication for the patient with diabetes/neuropathy. The loss of ligamentous integrity and bony destruction results in a limb that is not stable for weight bearing. This loss of independence adds significant health risks to the patient. Management of this disease process is both time consuming and technically demanding for both the practitioner and the patient. Attention to detail and aggressive decision making is often necessary to salvage the limb. The goal for treatment is to produce a stable, weight-bearing limb that is shoeable and free from soft-tissue ulceration.


Assuntos
Artropatia Neurogênica , Artropatias , Humanos , Tornozelo , , Articulação do Tornozelo/diagnóstico por imagem , Articulação do Tornozelo/cirurgia , Artropatias/complicações , Artropatia Neurogênica/diagnóstico por imagem , Artropatia Neurogênica/etiologia
5.
Ter Arkh ; 95(5): 429-434, 2023 Jul 16.
Artigo em Russo | MEDLINE | ID: mdl-38158997

RESUMO

Most rheumatic diseases are characterized by sexual dimorphism both in prevalence and in the characteristics of the clinical course. Increased production of pro-inflammatory cytokines that accompanies inflammatory joint diseases may be accompanied by a decrease in the level of male sex hormones, and vice versa, the presence of hypogonadism in men increases the risk of developing certain rheumatic diseases. The review presents data on the relationship between testosterone deficiency and major inflammatory joint diseases, as well as the effect of testosterone replacement therapy on their manifestations.


Assuntos
Hipogonadismo , Artropatias , Doenças Reumáticas , Humanos , Masculino , Hipogonadismo/complicações , Hipogonadismo/tratamento farmacológico , Artropatias/complicações , Doenças Reumáticas/complicações , Síndrome , Testosterona/uso terapêutico
6.
BMC Musculoskelet Disord ; 24(1): 916, 2023 Nov 27.
Artigo em Inglês | MEDLINE | ID: mdl-38012586

RESUMO

This report introduces a young adult who has been in bed for more than ten years with end-stage hemophilic arthropathy. He didn't have access to factor VIII (FVIII) in the early stage of hemophilia due to the high costs of clotting replacement therapy. As a result, he is experiencing some difficulties, such as joint contracture, muscular atrophy, severe pain, and poor function of cardiopulmonary. He came to visit us for a comprehensive rehabilitation program, and, finally, he achieved the basic goal of self-care in daily life.


Assuntos
Artrite , Hemofilia A , Artropatias , Masculino , Adulto Jovem , Humanos , Hemofilia A/complicações , Hemofilia A/terapia , Modalidades de Fisioterapia , Artropatias/complicações , Artropatias/diagnóstico por imagem
7.
Haemophilia ; 29(6): 1621-1632, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37861076

RESUMO

INTRODUCTION: Elderly people with haemophilia (PwH) develop haemophilic arthropathy, pain, and reduced health-related quality of life (HR-QoL). The condition of elderly mild haemophilia patients have rarely been evaluated. This study aimed to compare joint status, pain, and HR-QoL between elderly with mild, moderate/severe haemophilia and healthy elderlies. METHODS: Knee/ankle abnormalities were assessed by ultrasound (HEAD-US) and physical examination (HJHS 2.1). Pain severity and pain interference were investigated using the Brief Pain Inventory. Pressure pain thresholds (PPTs) were obtained at knees/ankles and forehead. Functional limitations were evaluated using the 2-Minute-Walking-Test, Timed-Up-and-Go and HAL. The EQ-5D-5L questionnaire evaluated HR-QoL. Healthy controls (HCs) and elderly individuals with moderate/severe and mild haemophilia were compared using Kruskal-Wallis and Mann-Whitney U tests. RESULTS: From the 46 elderly PwH approached, 40 individuals (≥60 years) with haemophilia A/B (17 moderate/severe; 23 mild) and 20 age-matched HCs were recruited. Moderate/severe PwH displayed worse joint status, lower PPTs, and poorer HR-QoL than mild PwH and HCs (p-value = .010-<.001). HEAD-US abnormalities were observed in 100% of knees and 94% of ankles in moderate/severe PwH, versus 50% of knees and 61% of ankles in mild PwH. Pain was reported by 80% and 57% of moderate/severe and mild PwH, respectively. Low PPTs, functional limitations, and poor HR-QoL scores were likewise observed in some mild PwH, yet without significantly differing from HCs. CONCLUSION: This study highlights poor joint/functional status, pain, and HR-QoL outcomes in elderly with moderate/severe haemophilia. A few mild haemophilia subjects presented joint abnormalities, pain, functional limitations, and poor HR-QoL, without significantly differing from HCs. HIGHLIGHTS: Elderly individuals with mild haemophilia have not yet been extensively studied, whereas moderate/severe haemophilia individuals have proven to suffer from haemophilic arthropathy, pain, and poor health-related quality of life (HR-QoL). Using a case-control design, joint status, pain, and HR-QoL outcomes were examined in elderly haemophilia individuals and compared with those of healthy controls (HCs). Elderly moderate/severe haemophilia individuals exhibited worse joint status, increased joint pain sensitivity, and reduced HR-QoL compared with both mild haemophilia subjects and HCs. A subset of mild haemophilia subjects exhibited poor joint status, pain, and HR-QoL outcomes, without any differences noted when compared with HCs.


Assuntos
Artrite , Hemofilia A , Artropatias , Doenças Vasculares , Humanos , Idoso , Hemofilia A/complicações , Qualidade de Vida , Estudos de Casos e Controles , Dor/etiologia , Artropatias/complicações , Artropatias/diagnóstico
8.
Probl Endokrinol (Mosk) ; 69(4): 70-76, 2023 08 30.
Artigo em Russo | MEDLINE | ID: mdl-37694869

RESUMO

The prevalence of obesity in the modern world is increasing. Obesity is an independent risk factor for some rheumatic diseases and also worsens their course. The presence of chronic joint disease can make it difficult for obesity to reduce activity, creating a vicious circle where joint pain makes exercise difficult and being overweight exacerbates joint pain. At the same time, there is a conditionally radical method of treating obesity - bariatric surgery (BS), which is currently used when conservative methods are ineffective. The purpose of this review is to analyze the data presented in the world literature on the impact of BS on the course of the most common rheumatic diseases. The available data show the possibility of a positive effect of BS not only on weight loss, but also on the course of a number of rheumatic diseases.


Assuntos
Cirurgia Bariátrica , Artropatias , Erros Inatos do Metabolismo , Obesidade Mórbida , Doenças Reumáticas , Humanos , Obesidade Mórbida/complicações , Obesidade Mórbida/cirurgia , Artropatias/complicações , Artropatias/cirurgia , Artralgia , Cirurgia Bariátrica/efeitos adversos
9.
Haemophilia ; 29(6): 1604-1610, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37729473

RESUMO

INTRODUCTION: The impact of joint damage on functional capacity in patients with mild haemophilia (PwMH) has yet to be well studied. The primary aim of this study was to investigate the effect of joint impairment on the functional capacity of the lower limb in PwMH. The secondary aim was to identify physical predictors of lower limb functional capacity. METHOD: Forty-nine PwMH were evaluated. Dynamic balance was assessed using Time Up and Go (TUG). Thirty-second sit-to-stand (30-STS) and 60-second-STS (60-STS) were used to assess muscle power and endurance, respectively. Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) was used to assess joint damage. PwMH were divided based on HEAD-US: with joint damage (≥3 points) and without or with very low joint damage (0-2 points). Univariate ANOVA and multiple regression analyses were performed to identify differences in functional capacity and potential physical predictors. RESULTS: Only 30-STS showed significant differences between groups (p = .002). TUG and 60-STS were primarily explained by age (r2  = .21 and r2  = .44, respectively), while for 30-STS, age combined with joint damage and pain level explains 54% of the variance. CONCLUSION: Our findings indicate that the 30-STS is useful for assessing functional deterioration in people with early-stage haemophilia-related arthropathy. Our results also indicate that joint damage, combined with ageing and pain, may impact 30-STS outcomes in PwMH. Furthermore, our findings show that the loss in TUG and 60-STS performance in PwMH is related to ageing.


Assuntos
Artrite , Hemofilia A , Artropatias , Humanos , Hemofilia A/complicações , Artropatias/complicações , Artrite/complicações , Extremidade Inferior , Dor/complicações
10.
Haemophilia ; 29(5): 1359-1365, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-37639381

RESUMO

INTRODUCTION: Joint health is one of the most important factors contributing to a healthy life in patients with haemophilia. Recent study revealed that starting early prophylaxis was not enough to prevent joint disease in most paediatric patients with haemophilia. AIM: In this study, we aimed to determine the age-specific incidence of acute joint disease during childhood at single haemophilia treatment centre (HTC). METHOD: The joint health in 48 patients was evaluated based on consecutive US testing for 5 years at annual multidisciplinary comprehensive care. RESULTS: During the study period, 23 patients (47.9%) had no joint disease since the initial examination, whereas 13 patients (27.0%) showed development from negative to positive findings. The incidence of joint disease increased with age: 0% in preschool, 5.3% in elementary school, 14.3% in junior high school and 35% beyond high school age. Among the 13 patients who developed joint disease, two experienced acquired synovitis that resolved during the follow-up period. Statistical analysis revealed that the patients who routinely underwent follow-up by the HTC exhibited a significantly lower incidence of joint disease than did those followed up at other institutions (p < .001). CONCLUSION: These results indicated that close check-up, including routine joint examination using US as well as frequent assessment of pharmacokinetic profile at the HTC, might play an important role in avoiding joint disease among paediatric patients with haemophilia.


Assuntos
Hemofilia A , Artropatias , Sinovite , Humanos , Criança , Pré-Escolar , Hemofilia A/complicações , Hemofilia A/epidemiologia , Incidência , Artropatias/complicações , Artropatias/epidemiologia , Fatores Etários
11.
Prim Care Diabetes ; 17(6): 548-553, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37643934

RESUMO

The current study ushers in a comprehensive review in clinical research to demonstrate the prevalence of musculoskeletal (MSK) complications in diabetes mellitus and the most relevant clinical aspects. In particular, revealing the early symptoms of the disorders, the pathology lurking behind the complications and their optimal management. In diabetes mellitus, MSK complications are common and are largely due to similar pathogenetic factors responsible for the internal organ complications associated with diabetes leading to chronic low-intensity inflammatory processes. MSK disorders develop by vasculopathy, neuropathy, arthropathy or combinations of the above, which are not specific to diabetes. However, their prevalence is significantly increased in diabetes and contributes to the disability impairing patients' quality of life. Locomotor disease affects approximately 34.4-83.5 % of patients suffering from type-2 diabetes mellitus. Several musculoskeletal abnormalities (cheiroarthropathy, Dupuytren's contracture, trigger finger, ect.) can be diagnosed upon physical examination, although certain symptoms (frozen shoulder, neurogenic arthropathy, septic arthritis, etc.) require differential diagnostic considerations. Early identification regarding characteristic symptoms in the treatment reducing inflammation and pain, followed with increasingly strenuous exercise therapy, aligned with optimal management of carbohydrate metabolism, proves essential in alleviating MSK complications.


Assuntos
Diabetes Mellitus Tipo 2 , Contratura de Dupuytren , Artropatias , Doenças Musculoesqueléticas , Humanos , Qualidade de Vida , Doenças Musculoesqueléticas/diagnóstico , Doenças Musculoesqueléticas/epidemiologia , Doenças Musculoesqueléticas/terapia , Artropatias/complicações , Artropatias/epidemiologia , Contratura de Dupuytren/complicações , Contratura de Dupuytren/epidemiologia , Diabetes Mellitus Tipo 2/diagnóstico , Diabetes Mellitus Tipo 2/epidemiologia , Diabetes Mellitus Tipo 2/terapia
12.
Perm J ; 27(3): 68-78, 2023 09 15.
Artigo em Inglês | MEDLINE | ID: mdl-37417806

RESUMO

Introduction Hemophilic arthropathy affects people with hemophilia (PwH) and causes joint dysfunction and disability. Brazil has a unique situation and implemented policies to improve health care for PwH. The aim of this study was to evaluate the Functional Independence Score in Hemophilia (FISH), Hemophilia Joint Health Score (HJHS), and associated factors among adult PwH attending a Hemophilia Comprehensive Care Center in Brazil. Methods A post hoc analysis was conducted, including 31 patients who had submitted to a physical evaluation during a previously published cross-sectional study performed from June 2015 to May 2016 at the Brasília Blood Center Foundation, Brazil. Results The mean age was 30.8±9.4 years, and 80.6% had severe hemophilia. FISH was 27.0±3.8, and HJHS 18.0±10.8. The ankle was the most often affected joint (25/31, 80.6%). There were significant correlations between FISH and HJHS scores and the Hemophilia Quality of Life Questionnaire for Adults. Patients with severe hemophilia (P = 0.029) and PwH aged ≥ 30 years (P = 0.049) had lower FISH scores. Monthly household income > 2 Brazilian minimum wages was independently associated with improved HJHS (P = 0.033). The factors simultaneously associated with better HJHS and FISH were age < 30 years (P = 0.021) and monthly household income < 2 minimum wages (P = 0.013). Conclusion FISH and HJHS showed favorable scores despite being performed in a country with unfavorable socioeconomic conditions. In addition to hemophilia severity and age, monthly household income was independently associated with functional and articular state of PwH. The results highlight the importance of the free provision of coagulation factors in Brazil.


Assuntos
Hemofilia A , Artropatias , Humanos , Adulto , Adulto Jovem , Hemofilia A/terapia , Hemofilia A/complicações , Qualidade de Vida , Estudos Transversais , Países em Desenvolvimento , Artropatias/complicações
13.
Eur Rev Med Pharmacol Sci ; 27(11): 5059-5069, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-37318480

RESUMO

OBJECTIVE: Comparisons between patellar eversion (PE) and lateral retraction (LR) in total knee arthroplasty (TKA) are still inconclusive. To determine the most suitable procedure, we aimed to evaluate the safety and efficacy of PE and LR in TKA in this meta-analysis. MATERIALS AND METHODS: This meta-analysis complied with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Web-based literature databases, including WANFANG, VIP, CNKI, the Cochrane Library, Embase, and PubMed, were utilized to conduct a comprehensive literature search for studies published until June 2022 that compared PE with LR in primary TKA. The quality of the selected randomized controlled trials (RCTs) was evaluated using guidelines of the Cochrane Reviews Handbook 5.0.2. RESULTS: A total of 10 RCTs, including 782 patients and 823 TKAs, were selected in this meta-analysis. Our results showed that using LR improved postoperative knee extensor function and range of motion (ROM). In addition, PE and LR resulted in similar clinical benefits in terms of Knee Society Function score, pain, length of hospital stay, Insall-Salvati ratio, the occurrence of patella baja, and complications related to the operation. CONCLUSIONS: Existing evidence suggested that using LR in TKA improved early postoperative knee function. Similar clinical and radiographic outcomes were obtained 1 year after the procedures were performed. Based on these findings, we recommended the use of LR in TKA. However, studies with large sample sizes are needed to validate these findings.


Assuntos
Artroplastia do Joelho , Artropatias , Humanos , Artroplastia do Joelho/efeitos adversos , Patela/cirurgia , Articulação do Joelho/cirurgia , Artropatias/complicações , Artropatias/cirurgia , Dor Pós-Operatória/cirurgia , Amplitude de Movimento Articular
14.
Thromb Res ; 226: 86-92, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-37130495

RESUMO

Prophylaxis is the gold standard treatment for children with haemophilia (CWH). MRI studies revealed joint damage, even with this treatment; this suggests the presence of subclinical bleeding. In the case of children with haemophilia, it is relevant to detect early signs of joint damage, as this allows the medical team to provide the appropriate treatment and follow-up, in order to avoid arthropathy development and its consequences. The aim of this study is to detect the hidden joint in children with haemophilia on prophylaxis (CWHP) and analyse, by age group, which joint is the most affected. We define the hidden joint in CWH on prophylaxis as the joint that presents joint damage secondary to repetitive bleeding episodes and is detected in the joint evaluation, despite being asymptomatic or with mild symptoms. It is most commonly caused by repetitive subclinical bleeding. METHODS: This was an observational, analytical, cross-sectional study of 106 CWH on prophylaxis treated in our centre. Patients were divided according to age and type of treatment. Joint damage was defined as a HEAD-US score ≥ 1. RESULTS: Patients' median age was 12 years. All had severe haemophilia. The median age of onset of prophylaxis was 2.7. Forty-seven (44.3 %) patients received primary prophylaxis (PP) and 59 (55.7 %), secondary prophylaxis. Six hundred and thirty-six joints were analysed. Type of prophylaxis and joint involvement showed statistically significant differences (p < 0.001). However, patients on PP had a greater number of damaged joints at older ages. Twenty-two % (140) of the joints scored ≥1 on HEAD-US. Cartilage was most frequently involved, followed by synovitis, and bone damage. We observed a greater frequency and degree of arthropathy in subjects aged 11 and above. Sixty (12.7 %) joints showed a HEAD-US score ≥ 1, with no history of bleeding. The ankle was the most affected joint, representing the hidden joint according to our definition. CONCLUSION: Prophylaxis is the best treatment for CWH. However, symptomatic or subclinical joint bleeding may occur. The routine evaluation of joint health is relevant, particularly, of the ankle. In our study, early signs of arthropathy according to age and type of prophylaxis were detected by HEAD-US.


Assuntos
Hemofilia A , Artropatias , Criança , Humanos , Hemofilia A/complicações , Hemofilia A/tratamento farmacológico , Estudos Transversais , Artropatias/prevenção & controle , Artropatias/complicações , Hemartrose/etiologia , Hemartrose/prevenção & controle , Hemorragia/complicações , Imageamento por Ressonância Magnética
15.
Haemophilia ; 29(4): 1032-1038, 2023 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-37252898

RESUMO

INTRODUCTION: The timing of prophylaxis and F8 genotype can impact treatment outcomes in adults with severe haemophilia A (HA). AIM: To investigate how F8 genotype, timing, and type of prophylaxis influence arthropathy, bleeding rates, factor consumption and health-related quality of life (HRQoL). METHODS: Thirty-eight patients with severe HA were enrolled. Bleeding events were recorded retrospectively during median 12.5 months. F8 gene variants were classified as null or non-null. Joint health and HRQoL were assessed with HJHS and EQ-5D-5L, respectively. RESULTS: The median age at prophylaxis start was 1.25 years in the primary prophylaxis group (N = 15, median age 26 years) and 31.5 years in the secondary group (N = 22, 45 years), respectively. There were significant differences in the medians of HJHS (4 vs. 20, p < .001), EQ-5D-5L index (0.9647 vs. 0.904, p = .022), EQ VAS (87 vs. 75, p = .01) and FVIII consumption (3883 vs. 2737 IU/kg/year, p = .02), between the primary and secondary groups, respectively. Median annualized bleeding rate (ABR) was 0 for both groups. Twenty-five null and thirteen non-null F8 gene variants were identified. In the secondary prophylaxis group, lower median FVIII consumption (1926 vs. 3370 IU/kg/year) was shown for non-null compared to null variants, respectively, with similar ABR and HJHS. CONCLUSION: Delayed prophylaxis start with intermediate dose intensity prevents bleeds but at a cost of more arthropathy and reduced HRQoL, compared to higher intensity primary prophylaxis. Non-null F8 genotype may allow lower factor consumption with similar HJHS and bleeding rates, compared to null genotype.


Assuntos
Hemofilia A , Artropatias , Adulto , Humanos , Hemofilia A/tratamento farmacológico , Fator VIII/genética , Fator VIII/uso terapêutico , Qualidade de Vida , Estudos Retrospectivos , Hemorragia/prevenção & controle , Hemorragia/complicações , Artropatias/complicações , Genótipo
16.
Knee ; 42: 236-245, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-37086540

RESUMO

AIM: To explore the effects and mechanism of millimeter-wave treatment on the development of joint stiffness in the immobilized knee rat model. METHODS: Twenty-four Sprague-Dawley (SD) rats were randomly divided into the control group (O, n = 8), the surgical control group (OC, n = 8), and the millimeter-wave treatment group (MO, n = 8). After immobilized knee modeling, the knee mobility and quadriceps diameter was measured at the 6th week. Hematoxylin and eosin and Masson staining were performed to detect the pathology and fibrous lesions of the knee joint. Furthermore, the expression of TGF-ß1 and Collagen I was quantified by immunohistochemical assay in the knee capsule, and Western blotting was performed to quantify the protein expression of NF-κB and MuRF1 in skeletal muscle. RESULTS: Compared with the O group, knee mobility, and quadriceps diameter was decreased (P < 0.01), and articular capsule fibrosis and quadriceps atrophy occurred in all rats with fixed knee joints. Compared with the OC group, millimeter-wave treatment significantly increased articular mobility and the quadriceps diameter; and improved the fibrotic lesions of the joint capsule and quadriceps atrophy. Moreover, levels of TGF-ß1, Collagen I, and MuRF1 were upregulated (P < 0.01) by knee immobilization, and collagen fiber content in the articular capsule was also increased (P < 0.01). However, millimeter-wave treatment reversed it. The most noteworthy result was that NF-κB expression was not significantly different in all groups. CONCLUSION: Millimeter-wave treatment reversed joint contracture and quadriceps atrophy caused by joint fixation, inhibited TGF-ß1 and Collagen I protein expression of the joint capsule and reduced MuRF1 expression of the quadriceps muscle, thereby inhibiting the development of joint stiffness.


Assuntos
Artropatias , Articulação do Joelho , Animais , Ratos , Atrofia/complicações , Atrofia/metabolismo , Atrofia/patologia , Colágeno Tipo I/metabolismo , Contratura/prevenção & controle , Contratura/etiologia , Cápsula Articular , Artropatias/complicações , Articulação do Joelho/patologia , NF-kappa B/metabolismo , Amplitude de Movimento Articular , Ratos Sprague-Dawley , Fator de Crescimento Transformador beta1/metabolismo
17.
Digestion ; 104(4): 328-334, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36893744

RESUMO

BACKGROUND: Patients with inflammatory bowel diseases (IBD) can develop extraintestinal manifestations (EIMs) during the disease course, which sometimes impact their quality of life. OBJECTIVES: This study aimed to clarify the prevalence and types of EIMs using a hospital-based IBD cohort in Japan. METHODS: A patient cohort with IBD was established in 2019, as participated by 15 hospitals in Chiba Prefecture of Japan. Using this cohort, the prevalence and types of EIMs, which are defined based on previous reports and the Japanese guidelines, were investigated. RESULTS: This cohort enrolled 728 patients, including 542 ulcerative colitis (UC) and 186 Crohn's disease (CD). Of these patients with IBD, 10.0% were identified with one or more EIMs (57 (10.5%) with UC and 16 (8.6%) with CD). Arthropathy and arthritis were the most common EIM in 23 (4.2%) patients with UC, followed by primary sclerosing cholangitis (PSC) (2.6%). Arthropathy and arthritis were also the most common in patients with CD, but no cases of PSC were observed. EIMs were more frequently observed in patients with IBD treated by specialists than in those treated by non-specialists (12.7% vs. 5.5%, p = 0.011). The incidence of EIMs in patients with IBD was not significantly different over time. CONCLUSIONS: The prevalence and types of EIMs in our hospital-based cohort in Japan did not significantly differ from those reported in previous or Western studies. However, the incidence might be underestimated due to the limited ability of non-IBD specialists to discover and describe EIMs in patients with IBD.


Assuntos
Artrite , Colite Ulcerativa , Doença de Crohn , Doenças Inflamatórias Intestinais , Artropatias , Humanos , Artrite/epidemiologia , Artrite/etiologia , Colite Ulcerativa/complicações , Colite Ulcerativa/epidemiologia , Doença de Crohn/complicações , Doença de Crohn/epidemiologia , População do Leste Asiático , Doenças Inflamatórias Intestinais/complicações , Doenças Inflamatórias Intestinais/epidemiologia , Artropatias/etiologia , Artropatias/complicações , Qualidade de Vida
18.
Clin Orthop Relat Res ; 481(8): 1560-1568, 2023 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-36692512

RESUMO

BACKGROUND: The cause of Charcot neuro-osteoarthropathy (CN) is diabetes in approximately 75% of patients. Most reports on the clinical course and complications of CN focus on diabetic CN, and reports on nondiabetic CN are scarce. No study, to our knowledge, has compared the clinical course of patients initially treated nonoperatively for diabetic and nondiabetic CN. QUESTIONS/PURPOSES: Among patients with CN, are there differences between patients with diabetes and those without in terms of (1) the frequency of major amputation as ascertained by a competing risks survivorship estimator; (2) the frequency of surgery as ascertained by a competing risks survivorship estimator; (3) frequency of reactivation, as above; or (4) other complications (contralateral CN development or ulcers)? METHODS: Between January 1, 2006, and December 31, 2018, we treated 199 patients for diabetic CN. Eleven percent (22 of 199) were lost before the minimum study follow-up of 2 years or had incomplete datasets and could not be analyzed, and another 9% (18 of 199) were excluded for other prespecified reasons, leaving 80% (159 of 199) for analysis in this retrospective study at a mean follow-up duration since diagnosis of 6 ± 4 years. During that period, we also treated 78 patients for nondiabetic Charcot arthropathy. Eighteen percent (14 of 78) were lost before the minimum study follow-up and another 5% (four of 78 patients) were excluded for other prespecified reasons, leaving 77% (60 of 78) of patients for analysis here at a mean of 5 ± 3 years. Patients with diabetic CN were younger (59 ± 11 years versus 68 ± 11 years; p < 0.01), more likely to smoke cigarettes (37% [59 of 159] versus 20% [12 of 60]; p = 0.02), and had longer follow-up (6 ± 4 years versus 5 ± 3 years; p = 0.02) than those with nondiabetic CN. Gender, BMI, overall renal failure, dialysis, and presence of peripheral arterial disease did not differ between the groups. Age difference and length of follow-up were not considered disqualifying problems because of the later onset of idiopathic neuropathy and longer available patient follow-up in patients with diabetes, because our program adheres to the follow-up recommendations suggested by the International Working Group on the Diabetic Foot. Treatment was the same in both groups and included serial total-contact casting and restricted weightbearing until CN had resolved. Then, patients subsequently transitioned to orthopaedic footwear. CN reactivation was defined as clinical signs of the recurrence of CN activity and confirmation on MRI. Group-specific risks of the frequencies of major amputation, surgery, and CN reactivation were calculated, accounting for competing events. Group comparisons and confounder analyses were conducted on these data with a Cox regression analysis. Other complications (contralateral CN development and ulcers) are described descriptively to avoid pooling of complications with varying severity, which could be misleading. RESULTS: The risk of major amputation (defined as an above-ankle amputation), estimated using a competing risks survivorship estimator, was not different between the diabetic CN group and nondiabetic CN group at 10 years (8.8% [95% confidence interval 4.2% to 15%] versus 6.9% [95% CI 0.9% to 22%]; p = 0.4) after controlling for potentially confounding variables such as smoking and peripheral artery disease. The risk of any surgery was no different between the groups as estimated by the survivorship function at 10 years (53% [95% CI 42% to 63%] versus 58% [95% CI 23% to 82%]; p = 0.3), with smoking (hazard ratio 2.4 [95% CI 1.6 to 3.6]) and peripheral artery disease (HR 2.2 [95% CI 1.4 to 3.4]) being associated with diabetic CN. Likewise, there was no between-group difference in CN reactivation at 10 years (16% [95% CI 9% to 23%] versus 11% [95% CI 4.5% to 22%]; p = 0.7) after controlling for potentially confounding variables such as smoking and peripheral artery disease. Contralateral CN occurred in 17% (27 of 159) of patients in the diabetic group and in 10% (six of 60) of those in the nondiabetic group. Ulcers occurred in 74% (117 of 159) of patients in the diabetic group and in 65% (39 of 60) of those in the nondiabetic group. CONCLUSION: Irrespective of whether the etiology of CN is diabetic or nondiabetic, our results suggest that orthopaedic surgeons should use similar nonsurgical treatments, with total-contact casting until CN activity has resolved, and then proceed with orthopaedic footwear. A high frequency of foot ulcers must be anticipated and addressed as part of the treatment approach. LEVEL OF EVIDENCE: Level III, prognostic study.


Assuntos
Artropatia Neurogênica , Diabetes Mellitus , Pé Diabético , Artropatias , Doença Arterial Periférica , Humanos , Estudos Retrospectivos , Úlcera/complicações , Amputação Cirúrgica , Pé Diabético/epidemiologia , Pé Diabético/cirurgia , Pé Diabético/complicações , Doença Arterial Periférica/complicações , Progressão da Doença , Artropatias/complicações , Artropatia Neurogênica/complicações , Artropatia Neurogênica/cirurgia , Artropatia Neurogênica/diagnóstico
19.
J Ultrasound Med ; 42(4): 859-868, 2023 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35920366

RESUMO

OBJECTIVES: We aimed to discuss the correlation between the Hemophilia Early Detection Ultrasound in China (HEAD-US-C) score and the Hemophilia Joint Health Score version 2.1 (HJHS 2.1) of the knee joint in patients with hemophilia. METHODS: We included 70 male patients with hemophilia admitted to The Second Hospital of Shanxi Medical University; the patients' bilateral knee joints were evaluated using the HEAD-US-C score and HJHS. We analyzed factors influencing hemophilia arthropathy of the knee and examined the correlation between the HEAD-US-C score and HJHS. RESULTS: The joint injury severity was positively correlated with age and the number of bleeds (P < .001). Further, the HEAD-US-C score and HJHS differed according to the severity (both P < .001), but not type (P = .163 and P = .283, respectively), of hemophilia. There was a significant correlation between the HEAD-US-C score and HJHS (P < .001). CONCLUSIONS: Overall, all joint lesions observed on ultrasound corresponded to clinical joint functional abnormalities. Therefore, the HEAD-US-C is important for hemophilic arthropathy evaluation and is useful in explaining abnormal joint function.


Assuntos
Hemofilia A , Artropatias , Humanos , Masculino , Hemofilia A/complicações , Hemartrose/complicações , Hemartrose/diagnóstico por imagem , Artropatias/complicações , Artropatias/diagnóstico por imagem , Articulação do Joelho/diagnóstico por imagem , Hemorragia , China
20.
Knee Surg Sports Traumatol Arthrosc ; 31(6): 2358-2365, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-36112159

RESUMO

PURPOSE: This study aimed to compare the amount of extrusion of the discoid lateral meniscus (DLM), which was symptomatic and required surgery, with normal meniscuses and asymptomatic DLMs and examine factors associated with the extrusion of symptomatic DLM. METHODS: Medical records of participants with DLM or normal lateral meniscus (LM) were retrospectively reviewed using magnetic resonance imaging (MRI). DLM cases were divided into symptomatic and asymptomatic groups. The midbody meniscal extrusion was measured using mid-coronal MRI. The association between meniscal extrusion and MRI findings, including the meniscofemoral ligament, meniscotibial ligament (MTL), intrameniscal signal intensity of the peripheral rim, meniscal shift, and skeletal maturity, was evaluated. RESULTS: Eighty-six knees with DLM (63 symptomatic) were included. The control group included 31 patients. The symptomatic group showed significantly greater meniscal extrusion (mean ± standard deviation symptomatic DLM: 1.0 ± 1.1 mm, asymptomatic DLM: 0.1 ± 0.4 mm, and normal LM: 0.3 ± 0.6 mm, P < 0.001) and had a significantly higher incidence of MTL loosening (P = 0.02) and intrameniscal signal (P < 0.001) than the other two groups. In the symptomatic group, multivariable linear regression analysis showed that MTL loosening [ß = 1.45, 95% confidence interval (CI) 1.03-1.86, P < 0.001] and intrameniscal signal (ß = 0.49, 95% CI 0.09-0.90, P = 0.002) were independent associated factors. CONCLUSIONS: LM extrusion was significantly more common in patients with symptomatic DLM than in those with asymptomatic DLM or a normal LM. MTL loosening and intrameniscal high-signal intensity on MRI were independently associated with meniscal extrusion. These findings help explain the pathogenesis and diagnosis of symptomatic DLM. LEVEL OF EVIDENCE: III.


Assuntos
Artropatias , Lesões do Menisco Tibial , Humanos , Meniscos Tibiais/diagnóstico por imagem , Meniscos Tibiais/cirurgia , Meniscos Tibiais/patologia , Estudos Retrospectivos , Lesões do Menisco Tibial/complicações , Lesões do Menisco Tibial/diagnóstico por imagem , Lesões do Menisco Tibial/cirurgia , Articulação do Joelho/cirurgia , Imageamento por Ressonância Magnética/métodos , Ligamentos Articulares/patologia , Artropatias/complicações
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