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Carlos E Rodriguezmerchan - One of the best experts on this subject based on the ideXlab platform.

  • curcumin hopeful treatment of Hemophilic Arthropathy via inhibition of inflammation and angiogenesis
    Expert Review of Hematology, 2020
    Co-Authors: Fatemeh Norooznezhad, Carlos E Rodriguezmerchan, Soheila Asadi, Amir Hossein Norooznezhad
    Abstract:

    ABSTRACTIntroduction: Hemophilic Arthropathy (HA) is a serious complication among Hemophilic patients causing a wide range of morbidity due to the inflammatory reactions followed by repeated episod...

  • risks and patient outcomes of surgical intervention for Hemophilic Arthropathy
    Expert Review of Hematology, 2019
    Co-Authors: Carlos E Rodriguezmerchan
    Abstract:

    Introduction: Surgical interventions usually needed for Hemophilic Arthropathy include arthroscopic synovectomy, alignment osteotomy, arthroscopic joint debridement, radial head removal, arthrodesi...

  • management of Hemophilic Arthropathy of the ankle
    Cardiovascular and Hematological Disorders - Drug Targets, 2017
    Co-Authors: Carlos E Rodriguezmerchan
    Abstract:

    BACKGROUND: Hemophilic Arthropathy (HA) of the ankle is prevalent in people with hemophilia (PWH). It is frequently severe and incapacitating, due to recurrent bleeding into the ankle articulation during infancy. Around 50% of Hemophilic patients suffer from ankle pain and radiological signs of HA. OBJECTIVE: To review current treatment of HA of the ankle in PWH. METHOD: A literature review of Hemophilic ankle Arthropathy in PWH was performed utilizing MEDLINE (PubMed) and the Cochrane Library. RESULTS: Primary hematologic prophylaxis could keep away from the development of ankle HA if the level of the patient's deficient factor is prevented from dropping below 1% of normal. Recurrent articular bleeding can be prevented by the intravenous infusion of clotting factor concentrates (prophylaxis). Major articular bleeds and chronic Hemophilic synovitis should be managed fiercely to prevent ankle HA. In the circumstance of advancing articular involvement, some noninvasive and invasive procedures can procure symptomatic mitigation and ameliorate the patient's function and quality of life. CONCLUSION: The ideal treatment for the Hemophilic ankle when hematologic prophylaxis fails includes physical medicine and rehabilitation, orthoses, radiosynovectomy, arthroscopic ankle debridement (in the initial stages of cartilage degeneration), and ankle distraction, ankle fusion or total ankle replacement (in advanced stages of cartilage degeneration).

  • synovium and cartilage biomarkers in Hemophilic Arthropathy
    Expert Review of Hematology, 2016
    Co-Authors: Carlos E Rodriguezmerchan
    Abstract:

    Some promising cartilage and synovium biomarkers are at various stages of development and awaiting further validation in larger patient populations with osteoarthritis (OA). Various reports have shown increased levels of inflammatory biomarkers, both locally (synovial fluid) and systemically (serum and plasma) in such patients. The clinical value of these parameters in combination with imaging biomarkers in order to predict early onset and the burden of OA is being investigated. This review article aims to describe the potential usefulness of synovial and cartilage biomarkers for the diagnosis and prognosis of Hemophilic Arthropathy (HA) by using the existing literature on OA as an applicable model. A systematic review found that serum cartilage oligomeric matrix protein (sCOMP) is elevated in patients with knee OA and is sensitive to OA disease progression.

Bernhard F Morrey - One of the best experts on this subject based on the ideXlab platform.

  • Hemophilic Arthropathy of the elbow treated by total elbow replacement
    Journal of Bone and Joint Surgery American Volume, 2004
    Co-Authors: Srinath Kamineni, Robert A Adams, Shawn W Odriscoll, Bernhard F Morrey
    Abstract:

    Hemophilic Arthropathy most commonly affects the knee, with the elbow being the second most frequently involved joint1. The recurrent intra-articular hemorrhages that cause this form of inflammatory Arthropathy are a feature of severe hemophilia in which <1% of the normal clotting factor titers are present. Whereas total joint replacement is a well-documented treatment for Hemophilic hip and knee Arthropathy2-5, there are very few reports of total elbow replacements in patients with hemophilia. Possible reasons for this lack of published data include (1) the predominant and successful medical management of most patients with hemophilia, (2) less functional impairment of the elbow compared with the hip and knee in Hemophilic Arthropathy, and (3) the more recent successful evolution of total elbow arthroplasty compared with the more established hip and knee arthroplasties. Our aim is to share our experience with total elbow arthroplasty in patients with Hemophilic Arthropathy and to review the cases reported in the literature. We retrospectively reviewed the records on 3100 patients with hemophilia who had presented to our institution and on 1358 total elbow replacements performed at our institution between 1979 and 2001. Five patients had had total elbow replacement for the treatment of Hemophilic Arthropathy (Table I), and we further analyzed those cases. View this table: TABLE I Data on Five Patients with Hemophilia and a Total Elbow Arthroplasty The mean age of the five patients was thirty-nine years (range, twenty-five to fifty-eight years). The elbow on the dominant side was involved in one patient, the elbow on the nondominant side was involved in one patient, and both elbows were involved in three patients. The primary hematological abnormality was hemophilia A (factor-VIII deficiency) in two patients and hemophilia A and B (factor-VIII and IX deficiency), hemophilia C (von Willebrand disease), and hemophilia A with factor-VIII inhibitor in …

  • Hemophilic Arthropathy of the Elbow Treated by Total Elbow Replacement
    Journal of Bone and Joint Surgery American Volume, 2004
    Co-Authors: Srinath Kamineni, Robert A Adams, Shawn W. O'driscoll, Bernhard F Morrey
    Abstract:

    Hemophilic Arthropathy most commonly affects the knee, with the elbow being the second most frequently involved joint1. The recurrent intra-articular hemorrhages that cause this form of inflammatory Arthropathy are a feature of severe hemophilia in which

C J K Bulstrode - One of the best experts on this subject based on the ideXlab platform.

  • total hip arthroplasty for Hemophilic Arthropathy
    Clinical Orthopaedics and Related Research, 1992
    Co-Authors: I W Nelson, Siva Sivamurugan, P D Latham, J Matthews, C J K Bulstrode
    Abstract:

    : From 1969 until 1985, 39 total hip arthroplasties (THAs) were performed in 38 patients for Hemophilic Arthropathy. The median age of patients at operation was 48.1 years. Twenty-two of these hip replacements in 21 patients were reviewed clinically and roentgenographically with a median follow-up study of 7.6 years. Five of the 22 hips have been revised and three are likely to require revision in the near future. The incidence of revision is compared to other studies of THA in young patients and the influence of human immunodeficiency virus infection is examined. Total hip arthroplasty in the hemophiliac is believed to be an appropriate operation for disabling Hemophilic Arthropathy.

Hoda Hassab - One of the best experts on this subject based on the ideXlab platform.

  • quality of life and clinical assessment of joint health in children with Hemophilic Arthropathy
    Blood, 2016
    Co-Authors: Hoda Hassab, Hayam Abdel M Ghany, Hany Rizk
    Abstract:

    Hemophilic Arthropathy results in pain, deformity, and disability with severe impairments, activity limitation, and thus productivity loss. Also patterns of treatment interfere with patients9 life, so quality of life should be assessed when evaluating treatment. The aim of this work was to assess the quality of life of children with Hemophilic Arthropathy and its relation to clinical joint health if any. The study was carried out on fifty boys aged 4 to 16 years, with Hemophilic Arthropathy. All patients were subjected to thorough local clinical assessment of the most affected and/or target joint using Haemophilia Joint Health Score (HJHS 2.1), assessment of quality of life using Haemo-Qol questionnaire kids9 and parents9 versions for three age groups: Group I: 4-7 years (21 items covering 8 dimensions), Group II: 8-12 years (64 items covering 10 dimensions), Group III: 13-16 years (77 items covering 12 dimensions) and complete blood count measurement. Among the fifty Hemophilic patients, 36 (72%) patients were hemophilia A and 14 (28 %) patients were hemophilia B. All patients were receiving on demand replacement therapy using plasma derived Factor concentrate or fresh frozen plasma (FFP) according to availability. The age at first joint bleeding ranged from 1-8 years with a mean of 2.40±1.78. While the number of joints affected in the studied patients ranged from 2-13 joints with a mean of 7±3.25, and the most common target joint being the knee (72%), followed by the ankle (10%) and then the elbow (4%). Twenty three patients (46%) had severe, and 27 (54 %) had moderate hemophilia. The results of the present study showed that there was significant difference between moderate and severe Hemophilic patients as regards age of onset of joint bleeding (z = -2.747, p = 0.006) and number of joints affected (t = -3.855, p There was statistical significant difference between the three studied age groups as regards HJHS (F= 9.843, P Forty four (88%) patients had different degrees of anemia. There was significant correlation between kid Haemo-QOL with degree of anemia (r = 0.291, p = 0.040), mainly with view and school dimensions. There were significant correlations between kid and parent Haemo-QOL and HJHS with each of the following: factor activity level, duration of the disease, duration of joint disease, number of bleeding attacks last year and number of joints affected. Five (10%) of the studied patients did synovectomy. The Haemo-QOL score was statistically significantly lower after the intervention (p=0.043) mainly with physical health, feeling, view, family, school and sports, treatment and dealing dimensions. In the current study there were significant positive correlations between kid and parent Haemo-QOL with global gait (r = 0.671, p Several factors affect the quality of life in patients with Hemophilic Arthropathy including synovectomy, anemia ,mode of treatment and joint health. Disclosures Hassab:Eli Lilly and Company: Research Funding.

  • Serum cartilage oligomeric matrix protein reflects radiological damage and functional status in Hemophilic Arthropathy patients
    The Egyptian Rheumatologist, 2016
    Co-Authors: Hoda Hassab, Wessam M. El-gendy, Khaled I. El-noueam, Hayam M Abdel Ghany, Mysoon M.a. Elwan
    Abstract:

    Abstract Aim of the work The objective of our study was to determine the utility of serum cartilage oligomeric matrix protein (COMP) as a serum biomarker for Hemophilic Arthropathy and to evaluate the degree of joint damage radiologically using plain X-ray and functionally using functional independence score of hemophilia (FISH) and to study their relation with COMP. Patients and methods The study was carried out on 30 boys with Hemophilic Arthropathy (group I) and 20 healthy boys as control (group II). All hemophiliacs patients were scored for FISH and radiological changes (Pettersson’s score). Factor activity level was measured in group I while COMP was measured in both groups. Results The patients’ age ranged from 6 to 16 years (mean 10.6 ± 2.7 years). The knee was the most commonly affected joint (83.3%). Fifteen patients (50%) had severe hemophilia, 7 had moderate and 8 had mild hemophilia. Mean serum levels of COMP in Hemophilic patients (529 ± 288.1 ng/ml) were significantly higher than in control (285 ± 63.2 ng/ml) ( p  = 0.014). The COMP level was significantly higher in patients with severe hemophilia compared to those with moderate or mild disease ( p r  = 0.64, p r  = 0.42, p  = 0.02) and negatively with the FISH score ( r  = −0.44, p  = 0.016). Conclusions Serum COMP level is indicative of the amount of joint damage in patients with Hemophilic Arthropathy. The combined scoring of functional independence and Pettersson score in addition to serum levels of COMP give a good overview of the degree of Hemophilic Arthropathy.

  • serum cartilage oligometric matrix protein and pettersson scoring system as an index for Hemophilic Arthropathy
    Blood, 2014
    Co-Authors: Hoda Hassab, Hayam M Abdel Ghany, Wessam M Elgendy, Khaled I Elnoueam, Mysoon M.a. Elwan
    Abstract:

    The pathogenesis of Hemophilic Arthropathy is multifactorial, with changes occurring in the synovium, bone, cartilage, and blood vessels. Recurrent joint bleeding causes synovial proliferation and inflammation (Hemophilic synovitis) that contributes to end stage degeneration (Hemophilic Arthropathy); with pain and limitation of motion that severely affects patients’ quality of life. The aim of the present study was to evaluate the degree of joint damage in boys with hemophilia using plain x-ray, and to measure serum level of human cartilage oligomeric matrix protein, to determine its relation to the degree of joint damage. The study was carried out on thirty boys with Hemophilic Arthropathy (group I) and ten healthy boys were included as control (group II). All Hemophilic patients were scored for Functional independence score (FISH score) in hemophilia and radiological changes (Pettersson’s score) using conventional frontal and lateral radiographs of the most affected joint. Factor activity level was measured for all Hemophilic patients while serum cartilage oligomeric matrix protein (COMP) was measured for Hemophilic patients and control group. Among the thirty Hemophilic patients, 26 (86.7%) patients were hemophilia A and 4 (13.3%) patients were hemophilia B All patients were receiving on demand replacement therapy using plasma derived Factor concentrate or fresh frozen plasma (FFP) according to availability. Fifteen patients (50%) had severe hemophilia, 7 (23.3%) had moderate and 8(26.72%) had mild hemophilia. A higher serum level of COMP with a mean of 757± 211.30 in the severe hemophiliacs, and a mean of 403.57 ± 86.49 and 211.25 ± 74.26 in the moderate and mild hemophiliacs respectively the difference was statistically significant (p Serum level of COMP in group I was significantly higher than in group II (p=0.004) with significant negative correlation with FISH score (r=-0.435 p=0.016). COMP correlated positively with joint space narrowing of the Pettersson score (r=0.421 p The number of joints affected (during life) of Hemophilic patients ranged between 1-12 with a mean of 5.50 ± 2.46 Joints. A significant positive correlation between serum level of COMP and number of joints affected (r = 0.487, p = 0.006). Joint space narrowing is the most important indicator of cartilage loss. Serum COMP level is indicative of the amount of joint damage in patients with Hemophilic Arthropathy. The combined scoring of functional independence and Pettersson in addition with serum levels of COMP give a good overview of the degree of Hemophilic Arthropathy Disclosures No relevant conflicts of interest to declare.

Mysoon M.a. Elwan - One of the best experts on this subject based on the ideXlab platform.

  • Serum cartilage oligomeric matrix protein reflects radiological damage and functional status in Hemophilic Arthropathy patients
    The Egyptian Rheumatologist, 2016
    Co-Authors: Hoda Hassab, Wessam M. El-gendy, Khaled I. El-noueam, Hayam M Abdel Ghany, Mysoon M.a. Elwan
    Abstract:

    Abstract Aim of the work The objective of our study was to determine the utility of serum cartilage oligomeric matrix protein (COMP) as a serum biomarker for Hemophilic Arthropathy and to evaluate the degree of joint damage radiologically using plain X-ray and functionally using functional independence score of hemophilia (FISH) and to study their relation with COMP. Patients and methods The study was carried out on 30 boys with Hemophilic Arthropathy (group I) and 20 healthy boys as control (group II). All hemophiliacs patients were scored for FISH and radiological changes (Pettersson’s score). Factor activity level was measured in group I while COMP was measured in both groups. Results The patients’ age ranged from 6 to 16 years (mean 10.6 ± 2.7 years). The knee was the most commonly affected joint (83.3%). Fifteen patients (50%) had severe hemophilia, 7 had moderate and 8 had mild hemophilia. Mean serum levels of COMP in Hemophilic patients (529 ± 288.1 ng/ml) were significantly higher than in control (285 ± 63.2 ng/ml) ( p  = 0.014). The COMP level was significantly higher in patients with severe hemophilia compared to those with moderate or mild disease ( p r  = 0.64, p r  = 0.42, p  = 0.02) and negatively with the FISH score ( r  = −0.44, p  = 0.016). Conclusions Serum COMP level is indicative of the amount of joint damage in patients with Hemophilic Arthropathy. The combined scoring of functional independence and Pettersson score in addition to serum levels of COMP give a good overview of the degree of Hemophilic Arthropathy.

  • serum cartilage oligometric matrix protein and pettersson scoring system as an index for Hemophilic Arthropathy
    Blood, 2014
    Co-Authors: Hoda Hassab, Hayam M Abdel Ghany, Wessam M Elgendy, Khaled I Elnoueam, Mysoon M.a. Elwan
    Abstract:

    The pathogenesis of Hemophilic Arthropathy is multifactorial, with changes occurring in the synovium, bone, cartilage, and blood vessels. Recurrent joint bleeding causes synovial proliferation and inflammation (Hemophilic synovitis) that contributes to end stage degeneration (Hemophilic Arthropathy); with pain and limitation of motion that severely affects patients’ quality of life. The aim of the present study was to evaluate the degree of joint damage in boys with hemophilia using plain x-ray, and to measure serum level of human cartilage oligomeric matrix protein, to determine its relation to the degree of joint damage. The study was carried out on thirty boys with Hemophilic Arthropathy (group I) and ten healthy boys were included as control (group II). All Hemophilic patients were scored for Functional independence score (FISH score) in hemophilia and radiological changes (Pettersson’s score) using conventional frontal and lateral radiographs of the most affected joint. Factor activity level was measured for all Hemophilic patients while serum cartilage oligomeric matrix protein (COMP) was measured for Hemophilic patients and control group. Among the thirty Hemophilic patients, 26 (86.7%) patients were hemophilia A and 4 (13.3%) patients were hemophilia B All patients were receiving on demand replacement therapy using plasma derived Factor concentrate or fresh frozen plasma (FFP) according to availability. Fifteen patients (50%) had severe hemophilia, 7 (23.3%) had moderate and 8(26.72%) had mild hemophilia. A higher serum level of COMP with a mean of 757± 211.30 in the severe hemophiliacs, and a mean of 403.57 ± 86.49 and 211.25 ± 74.26 in the moderate and mild hemophiliacs respectively the difference was statistically significant (p Serum level of COMP in group I was significantly higher than in group II (p=0.004) with significant negative correlation with FISH score (r=-0.435 p=0.016). COMP correlated positively with joint space narrowing of the Pettersson score (r=0.421 p The number of joints affected (during life) of Hemophilic patients ranged between 1-12 with a mean of 5.50 ± 2.46 Joints. A significant positive correlation between serum level of COMP and number of joints affected (r = 0.487, p = 0.006). Joint space narrowing is the most important indicator of cartilage loss. Serum COMP level is indicative of the amount of joint damage in patients with Hemophilic Arthropathy. The combined scoring of functional independence and Pettersson in addition with serum levels of COMP give a good overview of the degree of Hemophilic Arthropathy Disclosures No relevant conflicts of interest to declare.