共查询到20条相似文献,搜索用时 31 毫秒
1.
Eline D. P. van Bergen Flora H. P. van Leeuwen Wouter Foppen Merel A. Timmer Roger E. G. Schutgens Simon C. Mastbergen Floris P. J. G. Lafeber Pim A. de Jong Kathelijn Fischer Lize F. D. van Vulpen 《Haemophilia》2023,29(6):1580-1588
Aim
Subclinical bleeding and inflammation play a role in progression of haemophilic arthropathy. Synovial proliferation is predictive of joint bleeding and its early detection may guide treatment changes and prevent arthropathy progression. This study evaluated the prevalence of active and inactive subclinical synovial proliferation and investigated potential biochemical blood/urine markers to identify patients with active subclinical synovial proliferation.Methods
This cross-sectional study included patients with severe haemophilia A born 1970–2006 who were evaluated during routine clinic visits. Patients with (a history of) inhibitors or recent joint bleeding were excluded. Elbows, knees and ankles were examined for subclinical synovial proliferation by ultrasound and physical examination. Active synovial proliferation was distinguished from inactive synovial proliferation using predefined criteria. Blood/urine biochemical markers (serum osteopontin, sVCAM-1, Coll2-1, COMP, CS846, TIMP, and urinary CTX-II) were compared individually and as combined indexes between patients with and without active synovial proliferation.Results
This cohort consisted of 79 patients with a median age of 31 years (range 16.5–50.8 years) with 62/79 (78%) of the patients using continuous prophylaxis. The annualized joint bleeding rate over the last 5 years was .6 (.2–1.1). Active (17/79, 22%) and inactive subclinical synovial proliferation (17/79, 22%) were both prevalent in this cohort. Biochemical markers were not correlated with active subclinical synovial proliferation.Conclusion
Subclinical synovial proliferation, both active and inactive, was prevalent in patients with severe haemophilia A with access to prophylaxis and would be overlooked without routinely performed ultrasounds. Biochemical markers were unable to identify patients with active subclinical synovial proliferation. 相似文献2.
Olav Versloot Ellen Kemler Johan Blokzijl Merel Timmer Marleen Schuuring Karin P. M. van Galen Idske Cornelia L. Kremer Hovinga Paul R. van der Valk Lize F. D. van Vulpen Roger E. G. Schutgens Casper F. van Koppenhagen Janjaap van der Net Kathelijn Fischer 《Haemophilia》2023,29(4):1013-1023
Background
Improved treatment options for people with haemophilia (PWH) have increased the possibilities for sports participation, but the risk of sports-induced bleeding (SIB) is still considered considerable by many.Aim
To assess sports associated injury- and bleeding risk in PWH and to assess clotting levels associated with safe sports participation.Methods
Sports injuries and SIBs were prospectively collected for 12 months in PWH aged 6–49 without inhibitors playing sports at least once weekly. Injuries were compared according to factor levels, severity, joint health, sports risk category and sports intensity. Factor activity at the time of injury was estimated using a pharmacokinetic model.Results
125 participants aged 6–49 (41 children, 90% haemophilia A; 48% severe, 95% severe on prophylaxis) were included. Sports injuries were reported by 51 participants (41%). Most participants (62%) reported no bleeds at all and only 16% reported SIBs. SIBs were associated with factor levels at time of injury (OR: 0.93/%factor level (CI 0.88–0.99); p = .02), but not with haemophilia severity (OR: 0.62 (CI 0.20–1.89); p = .40), joint health, sports risk category or sports intensity. PWH with factor levels <10% during sports injury had a bleeding risk of 41% versus 20% in those with higher (>10%) factor levels.Conclusion
The results of this study emphasize the importance of clotting factor levels in prevention of bleeds. This information is vital for patient counselling and tailoring prophylactic treatment with clotting factors and non-replacement therapy. 相似文献3.
Flora H. P. van Leeuwen Kathelijn Fischer Wouter Foppen Lize F. D. van Vulpen Merel A. Timmer 《Haemophilia》2023,29(3):883-891
Aim
Traditionally, recovery after a joint bleed in people with bleeding disorders is evaluated by clinical symptoms. Following a bleed, however, asymptomatic joints may still show synovial hypertrophy and effusion on ultrasound. We evaluated the duration of full recovery from a joint bleed. Additionally, we determined how recovery differed when assessed by physical examination and ultrasound.Methods
In this retrospective cohort study, we investigated joint bleeds in elbows, knees and ankles of people with haemophilia or Von Willebrand disease who attended the Van Creveldkliniek between 2016 and 2021. Physical examination (warmth, swelling, range of motion and gait) and ultrasound (effusion and synovial hypertrophy) were performed within 7 days after the onset of the bleed, 1 week after the first examination and monthly thereafter until patients had recovered fully. Joint bleeds were treated in line with the current international treatment guidelines.Results
We evaluated 30 joint bleeds in 26 patients. The median recovery time was 1 month (range 0.3-5 months). In 47% of the joint bleeds, the recovery took longer than 1 month. The moment of recovery based on physical examination and ultrasound differed in 27% of bleeds. Both persistent abnormalities at physical examination in joints with normalized ultrasounds and persistent ultrasound findings in clinically recovered joints occurred.Conclusion
Joint bleed recovery can take long and recovery times differed per bleed. Recovery differed when assessed by physical examination or ultrasound. Therefore, both should be used to closely monitor recovery of joint bleeds and offer personalized care. 相似文献4.
Summary. The incidence of haemarthrosis as a result of a spontaneous periarticular aneurysm in haemophilia is very low. In these circumstances, angiographic embolization might be considered as a promising therapeutic and coagulation factor saving option in joint bleeds not responding to replacement of coagulation factor to normal levels. Moreover, embolization should be considered as a possible treatment for postoperative pseudoaneurysms complicating total knee arthroplasty in haemophilia. However, the pathological process of aneurysmal bleeding and clotting factor replacement is entirely different. While embolization is the treatment of choice for some periarticular complications that may occur, it is by no means a panacea for all resistant periarticular bleeds in haemophilia or for postoperative bleeding which usually settles with clotting factor replacement. Another use of arterial embolization is for the treatment of haemophilic tumours of the pelvis, because they can act as a focus for infection and cause cutaneous fistulas. When they present perforations and infections of endogenous origin, their course is usually fatal. Suitable treatment has been investigated on numerous occasions, most of the literature agreeing that the only curative treatment is surgical resection. However, surgical resection after performing arterial embolization to reduce the vascularization of the pseudotumour is a good alternative, thereby reducing the size of the pseudotumour and the risk of bleeding complications during surgery. It is important to bear in mind that despite its efficacy, arterial embolization is an invasive procedure with a reported rate of complications up to 25% (16% minor, 7% serious, 2% death). 相似文献
5.
Fukae J Isobe M Kitano A Henmi M Sakamoto F Narita A Ito T Mitsuzaki A Shimizu M Tanimura K Matsuhashi M Kamishima T Atsumi T Koike T 《Arthritis care & research》2011,63(9):1247-1253
Objective
To investigate the relationship between synovial vascularity and progression of structural bone damage in each finger joint in patients with rheumatoid arthritis (RA) and to demonstrate synovial vascularity as a potential therapeutic marker.Methods
We studied 250 metacarpophalangeal (MCP) and 250 proximal interphalangeal (PIP) joints of 25 patients with active RA who were administered adalimumab or tocilizumab. Patients were examined with clinical and laboratory assessments. Power Doppler sonography was performed at baseline and at the fourth and eighth weeks. Synovial vascularity was evaluated according to quantitative measurement. Hand and foot radiography was performed at baseline and the twentieth week.Results
Clinical indices such as the 28‐joint Disease Activity Score, the Clinical Disease Activity Index, and the Simplified Disease Activity Index were significantly decreased by biologic agents. The MCP and PIP joints with no response in synovial vascularity between baseline and the eighth week (vascularity improvement of ≤70% at the eighth week) showed a higher risk of radiographic progression compared with responsive joints (vascularity improvement of >70% at the eighth week; relative risk 2.33–9). Radiographic progression at the twentieth week was significantly lower in responsive joints than in nonresponsive joints.Conclusion
The improvement of synovial vascularity following treatment with biologic agents led to suppression of radiographic progression of RA in each finger joint. The alteration in synovial vascularity numerically reflected therapeutic efficacy. Using vascularity as a marker to determine the most suitable therapeutic approach would be beneficial for patients with active RA. 相似文献6.
Jun Fukae Yujiro Kon Mihoko Henmi Fumihiko Sakamoto Akihiro Narita Masato Shimizu Kazuhide Tanimura Megumi Matsuhashi Tamotsu Kamishima Tatsuya Atsumi Takao Koike 《Arthritis care & research》2010,62(5):657-663
Objective
To investigate the relationship between synovial vascularity assessed by quantitative power Doppler sonography (PDS) and progression of structural bone damage in a single finger joint in patients with rheumatoid arthritis (RA).Methods
We studied 190 metacarpophalangeal (MCP) joints and 190 proximal interphalangeal (PIP) joints of 19 patients with active RA who had initial treatment with disease‐modifying antirheumatic drugs (DMARDs). Patients were examined by clinical and laboratory assessments throughout the study. Hand and foot radiography was performed at baseline and the twentieth week. Magnetic resonance imaging (MRI) was performed at baseline. PDS was performed at baseline and the eighth week. Synovial vascularity was evaluated according to both quantitative and semiquantitative methods.Results
Quantitative PDS was significantly correlated with the enhancement rate of MRI in each single finger joint. Comparing quantitative synovial vascularity and radiographic change in single MCP or PIP joints, the level of vascularity at baseline showed a significant positive correlation with radiographic progression at the twentieth week. The change of vascularity in response to DMARDs, defined as the percentage change in vascularity by the eighth week from baseline, was inversely correlated with radiographic progression in each MCP joint. The quantitative PDS method was more useful than the semiquantitative method for the evaluation of synovial vascularity in a single finger joint.Conclusion
The change of synovial vascularity in a single finger joint determined by quantitative PDS could numerically predict its radiographic progression. Using vascularity as a guide to consider a therapeutic approach would have benefits for patients with active RA. 相似文献7.
Successful treatment of severe bleeding in hemophilic target joints by selective angiographic embolization 总被引:2,自引:0,他引:2
下载免费PDF全文
![点击此处可从《Blood》网站下载免费的PDF全文](/ch/ext_images/free.gif)
Mauser-Bunschoten EP Zijl JA Mali W van Rinsum AC van den Berg HM Roosendaal G 《Blood》2005,105(7):2654-2657
Bleeding into the joints is common in patients with hemophilia. After total knee or elbow replacement, profuse intraarticular bleeding unresponsive to high-dose clotting factor replacement sometimes occurs. In some patients who have severely damaged elbow or knee joints the same profuse bleeding pattern can be seen. To control bleeding in these patients, selective catheterization with a microcatheter and therapeutic embolization with microcoils was performed whenever a severe blush or microaneurysm was observed on angiography. Over 12 years, in 23 cases of massive joint bleeding in 18 patients with hemophilia selective catheterization was performed. In 15 cases the bleeding was postoperative and in 8 spontaneous. Results of angiographic imaging revealed vascular blush, false aneurysm, true aneurysm, and arteriovenous shunt in combination with an aneurysm as cause of bleeding. In 2 patients, the cause of bleeding was not found. In 21 cases an embolization procedure was performed, in which the bleeding was completely controlled by a single procedure in 14 cases. Recurrence of the bleeding occurred in 7 cases and required a second embolization procedure; in one patient even a third embolization was required to stop the bleeding completely. No difference in the outcome, that is, clinical end of bleeding and joint range of motion, was observed, when comparing postoperative and spontaneous bleeding. 相似文献
8.
9.
Esperanza Naredo Lara Valor Inmaculada De la Torre Julia Martínez‐Barrio Michelle Hinojosa Francisco Aramburu Juan Gabriel Ovalles‐Bonilla Diana Hernández María Montoro Carlos Manuel González Javier López‐Longo Indalecio Monteagudo Luis Carreño 《Arthritis care & research》2013,65(4):512-517
Objective
To investigate the sensitivity for detecting subclinical synovitis of different reduced joint ultrasound (US) assessment models as compared with a comprehensive US assessment in rheumatoid arthritis (RA) patients in clinical remission.Methods
Sixty‐seven RA patients (50 women, 17 men) in clinical remission as judged by their consultant rheumatologist and treated with methotrexate were prospectively recruited. Patients were evaluated for disease activity according to the Disease Activity Score in 28 joints (DAS28) and the Simplified Disease Activity Index (SDAI) by the same investigator. Each patient underwent a 44‐joint B‐mode and power Doppler (PD) assessment by a rheumatologist blinded to the clinical and laboratory data. B‐mode synovial hypertrophy (SH) and synovial PD signal were scored from 0–3 at each joint. Global indices for SH and PD signal were calculated for the 44‐joint and different joint combination models for each patient.Results
SH was detected in 87.8% of patients with a DAS28 <2.6 and in 81.8% of patients with an SDAI <3.3. Synovial PD signal was detected in 46.3% of patients with a DAS28 <2.6 and in 36.4% of patients with an SDAI <3.3. Wrist, second through fifth metacarpophalangeal (MCP), ankle, and second through fifth metatarsophalangeal (MTP) joint and 12‐joint US assessments showed the highest correlations with the comprehensive US assessment. The wrist, MCP, ankle, and MTP joint US assessment showed the highest sensitivity for detecting SH and synovial PD signal in patients in remission according to the DAS28 and SDAI as compared to the comprehensive US assessment.Conclusion
US assessment of the wrist, MCP, ankle, and MTP joints can be highly sensitive for detecting residual B‐mode and Doppler joint inflammation in RA patients. 相似文献10.
E. P. MAUSER-BUNSCHOTEN N. W. D. JANSEN† A. S. DORIA‡ J. OLDENBURG§ 《Haemophilia》2008,14(S3):147-152
Summary. New imaging techniques are valuable for the care of patients with haemophilia. On angiography it is shown that some bleedings in severely damaged joints or after implantation of prostheses are arterial. Effect of clotting factor is often poor. Selective catherization with embolization of the bleeding artery stops the bleed and is clinically effective. From 31 patients with severe haemophilia A or B, 62 knee radiographs were scored according to the Pettersson-scoring system as well as with Knee Digital Image Analysis (KIDA). Using KIDA, good correlation was found for osteoporosis, irregular subchondral surface, narrowing of the joint space, deformity and incongruence. For each of the parameters within one point in the Pettersson score a large variation existed in KIDA grading. MRI is accurate for diagnosis of soft and osteochondral tissue. Nevertheless, it is costly and not very accessible. The use of parallel imaging is more feasible for assessment of multiple joints within a relatively short period of time. Although ultrasonography also holds the potential for being an adjunct to MRI it has the disadvantage that it is operator-dependent. In a cohort of 124 chronically HCV infected haemophilia patients transient elastography was performed to measure liver stiffness. 57 (46%) had no or mild fibrosis, 18 (14.5%) moderate fibrosis and 49 severe or cirrhotic fibrosis. Transient elastography is safe and helpful to refer patients to antiviral therapy. 相似文献
11.
12.
13.
Real‐world comparative analysis of bleeding complications and health‐related quality of life in patients with haemophilia A and haemophilia B
下载免费PDF全文
![点击此处可从《Haemophilia》网站下载免费的PDF全文](/ch/ext_images/free.gif)
Jason Booth Abiola Oladapo Shaun Walsh Jamie O'Hara Liz Carroll Daniel‐Anibal Garcia Diego Brian O'Mahony 《Haemophilia》2018,24(5):e322-e327
Introduction
Clinical severity and impact of haemophilia on quality of life have been generally considered to be lower for haemophilia B (HB) compared with haemophilia A (HA) patients.Aims
To compare annual bleeding rate (ABR), target joint development and health‐related quality of life (HRQoL) between adult (≥18 years) severe HA and HB patients using recent data from the Cost of Haemophilia in Europe: a Socioeconomic Survey (CHESS) study.Methods
Multivariate generalized linear models (GLM) were constructed to assess the relationship between haemophilia type, ABR, HRQoL (derived from EQ‐5D index scores) and the presence of target joints while controlling for covariates.Results
Of the 1225 patients included, 77% (n = 949) had HA and 23% (n = 278) had HB. Of the 514 patients who completed the EQ‐5D, 78% (n = 405) had HA, and 22% (n = 110) had HB. Unadjusted mean ABR was 3.79 in HA and 4.60 in HB. The presence of ≥1 target joint was reported in 59% and 54% of patients with HA and HB, respectively. Unadjusted mean EQ‐5D index score was 0.78 in HA and 0.76 in HB. Haemophilia type was not a significant predictor of ABR, target joints or HRQoL when adjusted for confounding factors such as BMI, age and replacement therapy regimen.Conclusion
Data suggest comparable ABR, incidence of target joints and HRQoL between patients with HB and HA indicating comparable clinical severity and disease impact on patient quality of life. 相似文献14.
Silvia Magni‐Manzoni Oscar Epis Angelo Ravelli Catherine Klersy Chiara Veisconti Stefano Lanni Valentina Muratore Carlo Alberto Sciré Silvia Rossi Carlomaurizio Montecucco 《Arthritis care & research》2009,61(11):1497-1504
Objective
To compare clinical evaluation and ultrasonography (US) in the assessment of joint synovitis in children with juvenile idiopathic arthritis (JIA).Methods
Thirty‐two patients underwent clinical evaluation of 52 joints by 2 pediatric rheumatologists. Joints were assessed for swelling, tenderness/pain on motion, and restricted motion. The same joints were scanned independently by an experienced sonographer for synovial hyperplasia, joint effusion, and power Doppler (PD) signal.Results
In total, 1,664 joints were assessed both clinically and with US. On clinical examination, 98 joints (5.9%) were swollen, 59 joints (3.5%) were tender, and 40 joints (2.4%) had restricted motion. On US evaluation, 125 joints (7.5%) had synovial hyperplasia, 153 joints (9.2%) had joint effusion, and 53 joints (3.2%) had PD signal. A total of 104 (6.3%) and 167 (10%) joints had clinical and US synovitis, respectively. Of the 1,560 clinically normal joints, 86 (5.5%) had subclinical synovitis (i.e., had synovitis on US). US led to classifying 5 patients as having polyarthritis who were classified as having oligoarthritis or were found to have no synovitis on clinical evaluation. US variables were moderately correlated with clinical measures of joint swelling, but poorly correlated with those of joint tenderness/pain on motion and restricted motion. Overall, correlations were lower for PD signal than for synovial hyperplasia and joint effusion.Conclusion
We found that subclinical synovitis as detected by US is common in children with JIA. This finding may have important implications for patient classification and may affect the choice of the optimal therapeutic strategy in individual patients. 相似文献15.
David C. Wilson Anthony D. Marinov Alisha Decewicz Patrick Grof‐Tisza David Kirchner Brendan Giles Paul R. Reynolds Michael N. Liebman V. S. Kumar Kolli Susan D. Thompson Raphael Hirsch 《Arthritis \u0026amp; Rheumatology》2010,62(6):1813-1823
Objective
Juvenile idiopathic arthritis (JIA) is a heterogeneous group of inflammatory diseases, and no clinically useful prognostic markers to predict disease outcome in children with JIA are currently available. Synovial fluid likely reflects the proteins present in the inflamed synovium. The purpose of this study was to delineate the synovial fluid proteome and determine whether protein expression differs in the different subtypes of JIA.Methods
Synovial fluid samples obtained from children with oligoarticular JIA, polyarticular JIA, or systemic JIA were compared. Two‐dimensional gel electrophoresis for protein separation and matrix‐assisted laser desorption ionization−time‐of‐flight mass spectrometry and quadripole time‐of‐flight mass spectrometry for protein identification were used for this study. Synovial fluid cells were analyzed by polymerase chain reaction (PCR) for the presence of haptoglobin messenger RNA (mRNA).Results
The synovial fluid proteome of the samples was delineated. The majority of proteins showed overexpression in JIA synovial fluid as compared with noninflammatory control samples. There were 24 statistically significantly differentially expressed spots (>2‐fold change; P < 0.05) between the subtypes of JIA. PCR analysis revealed haptoglobin mRNA, suggesting that haptoglobin is locally produced in an inflamed joint in JIA.Conclusion
Despite the similar histologic appearance of inflamed joints in patients with different subtypes of JIA, there are differences in protein expression according to the subtype of JIA. Haptoglobin is differentially expressed between the subtypes of JIA and is locally produced in an inflamed joint in JIA. Haptoglobin and other differentially expressed proteins may be potential biomarkers in JIA.16.
Alfredo Dragani Raniero Malizia Ornella Iuliani Ilaria Di Marzio Giovanni Dav�� 《Trasfusione del sangue》2008,6(3):136-142
Backgorund
Inherited bleeding disorders registries can be useful to improve health care of these rare disorders and document their natural history.Material and methods
We analysed the epidemiological, diagnostic and therapeutic aspects of patients managed at an Italian Regional Haemophilia Centre, based in Pescara (in the Region of Abruzzo).Results
This Regional Haemophilia Centre currently follows 376 patients: 248 with rare clotting factor defects, 33 with von Willebrand’s disease, 75 with haemophilia A and 20 with haemophilia B. Three patients with severe haemophilia A have developed inhibitors. Among all the haemophiliacs, the prevalence of hepatitis C virus infection is 21% while the prevalence of human immunodeficiency virus infection is 5.3%. Among the whole haemophilic population referring to the Pescara Haemophilia Centre, 87.4% are treated with recombinant factors while 12 patients with severe haemophilia are receiving primary prophylaxis.Conclusion
In brief, an analysis of the epidemiological, clinical and therapeutic data collected at the Regional Haemophilia Centre of Pescara is a useful tool for monitoring and continuously improving the quality of care of patients with inherited bleeding disorders. 相似文献17.
Benedikt Ostendorf Ralf Peters Paul Dann Arnd Becker Axel Scherer Freya Wedekind Johannes Friemann Klaus‐Peter Schulitz Ulrich Mdder Matthias Schneider 《Arthritis \u0026amp; Rheumatology》2001,44(11):2492-2502
Objective
To evaluate and characterize magnetic resonance imaging (MRI) findings in the metacarpophalangeal (MCP) joints of rheumatoid arthritis (RA) patients macroscopically, using miniarthroscopy (MA; needle arthroscopy).Methods
The second MCP joint of the dominant hand of 22 RA patients (13 with various RA activities/stages; 9 with early RA [≤1.5 years' duration]) was examined by MRI followed by MA. Findings were evaluated by standardized semiquantitative measures of synovial and bony pathologic changes of the MCP joint, and were compared with the clinical and conventional radiologic findings.Results
Erosions and pre‐erosions were detected in 17 of 22 patients by MRI; 2 of the other 5 patients (all early RA) displayed bony changes on MA. All 10 joints with pre‐erosions on MRI (grade I bony alterations on MRI) exhibited significant cartilaginous and bony pathology on MA. Synovial membrane pathology was detected in all but 1 patient by MRI and in all patients by MA, although findings of plain radiography were normal in 6 of the 22 patients and another 9 patients had a Larsen score of 1. Semiquantitative analysis of synovial findings of MRI revealed gadolinium diethylenetriaminepentaacetic acid enhancement as a significant marker of macroscopically varied synovial vascularity and hyperemia, both of which strongly correlated with clinical activity (as measured by the Disease Activity Score). The extent of synovitis/synovial proliferation shown by MA and MRI were significantly correlated with each other, but not with any other activity or damage parameter analyzed.Conclusion
In RA, both MRI and MA findings support early detection and staging of synovial changes. Ongoing longitudinal studies are aimed at evaluating the value of synovial proliferation as visualized by both methods.18.
Shelby Addison R. Edward Coleman Sheng Feng Gary McDaniel Virginia Byers Kraus 《Arthritis \u0026amp; Rheumatology》2009,60(11):3366-3373
Objective
To evaluate the association of serum and synovial fluid cartilage oligomeric matrix protein (COMP) with systemic and local measures of osteoarthritis (OA) activity by bone scintigraphy.Methods
Samples of serum and knee joint synovial fluid (275 knees) were obtained from 159 patients with symptomatic OA of at least 1 knee. Bone scintigraphy using 99mTc‐labeled methylene diphosphonate was performed, and early‐phase knee scans and late‐phase whole‐body bone scans of 15 additional joint sites were scored semiquantitatively. To control for within‐subject correlations of knee data, generalized linear modeling was used in the correlation of the bone scan scores with the COMP levels. Principal components analysis was used to explore the contribution of each joint site to the variance in serum COMP levels.Results
The correlation between synovial fluid and serum COMP levels was significant (r = 0.206, P = 0.006). Synovial fluid COMP levels correlated most strongly with the early‐phase knee bone scan scores (P = 0.0003), even after adjustment for OA severity according to the late‐phase bone scan scores (P = 0.015), as well as synovial fluid volumes (P < 0.0001). Serum COMP levels correlated with the total‐body bone scan scores (r = 0.188, P = 0.018) and with a factor composed of the bone scan scores in the shoulders, spine, lateral knees, and sacroiliac joints (P = 0.0004).Conclusion
Synovial fluid COMP levels correlated strongly with 2 indicators of knee joint inflammation: early‐phase bone scintigraphic findings and synovial fluid volume. Serum COMP levels correlated with total‐body joint disease severity as determined by late‐phase bone scintigraphy, supporting the hypothesis that whole‐body bone scintigraphy is a means of quantifying the total‐body burden of OA for systemic biomarker validation.19.
Ingrid den Uijl Douwe Biesma Diederick Grobbee Kathelijn Fischer 《Trasfusione del sangue》2013,11(3):364-369
Background
Since the introduction of prophylaxis, physicians have tried to convert the clinical phenotype of severe haemophilia (SH) into that of moderate haemophilia (MH), but the outcome of patients with SH has never been compared to that of patients with MH.Material and methods
The outcome of 80 patients with SH on long-term, intermediate dose prophylaxis was compared to that of 40 patients with MH in a single-centre study. Data on treatment history, activities (assessed by the IPAQ and HAL), quality of life (assessed by the SF-36 and EQ5D), and 5-year bleeding and clotting factor consumption were collected for patients born between 1970–1995.Results
The median age of the patients was 24 years (IQR 18–30). All patients with SH received long-term prophylaxis, which was started at a median age of 4.8 years (IQR 3.2–6.2). Among the patients with MH, ten (25%) received prophylaxis, starting at a median age of 10.8 years (IQR 3.8–13.8). The annual number of bleeds, including joint bleeds, was significantly higher in patients with SH (median 2.0 joint bleeds/year, IQR =0.8–3.7) than in patients with MH (median 0.8 joint bleeds/year, IQR =0–1.2). Due to greater use of prophylaxis, the annual clotting factor consumption of SH patients (median 2,120 IU/kg; IQR 1,514–2,768), was higher than that of MH patients (median 133 IU/kg; IQR 49–468). Patients with SH showed slightly but significantly more loss of clinical function (assessed by the Haemophilia Joint Health Score): a median of 8 points (IQR 3–15) vs a median of 2 points, IQR 0–6). Quality of life, as measured by the SF-36, EQ5D and physical activity, was similar between patients with disease of different severity, as well as compared to that of the general population.Discussion
When comparing unselected cohorts, the bleeding pattern of patients with SH does not appear to be fully converted to that of the milder bleeding pattern of MH by long-term, intermediate-dose prophylaxis, although activities and quality of life were similar. 相似文献20.
A. V. Perruccio J. D. Power H. M. K. Evans S. R. Mahomed R. Gandhi N. N. Mahomed A. M. Davis 《Arthritis care & research》2012,64(6):838-846