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Hemophilic Arthropathy

Arthrohemorrhagic Syndrome


Presentation

  • The musculoskeletal manifestations of hemophilia A and B are some of the most common presenting symptoms and continue to be challenging to practitioners.[ncbi.nlm.nih.gov]
  • A case of temporomandibular joint hemophilic arthropathy in a patient with a factor XI deficiency will be presented.[ncbi.nlm.nih.gov]
  • Indications for surgery are discussed and a case study that incorporates both conservative and surgical management options is presented.[ncbi.nlm.nih.gov]
  • A 24-year-old patient with advanced HA presented multiple joint pain. Physical examination revealed joint tenderness, swelling, and limited activity.[ncbi.nlm.nih.gov]
  • Over the last 20 years at the present authors' institution, 168 such procedures have been performed. Of these, 141 were primary procedures and 27 were reoperations for persisting or recurring symptoms, failed prostheses, and infection.[ncbi.nlm.nih.gov]
Easy Bruising
  • The main symptoms of hemophilia are excessive bleeding and easy bruising. Blood tests can tell if you have it. The main treatment is injecting the missing clotting factor into the bloodstream.[icdlist.com]
  • DIAGNOSIS  Suspected in patients presenting with history of Easy bruising in early child hood Spontaneous bleeds Excessive bleeding following trauma or surgery 5.[slideshare.net]
Arthritis
  • BACKGROUND: Ankle arthrodesis is an accepted treatment for patients with advanced disabling tibiotalar arthritis, mostly in osteoarthritis, rheumatoid, and posttraumatic arthritis.[ncbi.nlm.nih.gov]
  • Hemophilic arthropathy shares some injury characteristics with rheumatoid arthritis, although the degree of analogy is a matter of some debate.[ncbi.nlm.nih.gov]
  • Septic Arthritis Until the early 1980s, septic arthritis rarely occurred in hemophilic patients.[musculoskeletalkey.com]
  • Vascular architecture changes and pronounced expression of α-SMA appeared unique to hemophilia, as these were not found in joint tissue obtained from mouse models of rheumatoid arthritis and osteoarthritis and from patients with the same conditions.[ncbi.nlm.nih.gov]
  • HA resembles much more to the rheumatoid arthritis because a presence of thick synovial membrane and large lymphocytes infiltration. However, in its final stages, HA resembles OA and that is when IA injections of MSCs are commonly used.[ncbi.nlm.nih.gov]
Joint Effusion
  • In the present review, we summarize major literature evidence on the use of joint US for the evaluation of markers of disease activity (joint effusion and synovial hypertrophy) and of degenerative damages (osteochondral changes) in patients with hemophilia[ncbi.nlm.nih.gov]
  • Radiographic findings at this stage are generally limited to a dense joint effusion (hemarthrosis). Following an acute episode of hemarthrosis,the synovium must absorb the blood degradation products.[appliedradiology.com]
  • Large joints are most commonly involved in the following order of frequency 2 : knee elbow ankle hip shoulder Plain radiograph joint effusion is seen in the setting of hemarthrosis periarticular osteoporosis: from hyperaemia epiphyseal enlargement with[radiopaedia.org]
  • This image shows an opaque joint effusion due to the presence of iron in the synovium (arrows).[emedicine.medscape.com]
  • Joint effusions (haemarthrosis) 2. Articular erosions 3. Periarticular osteopenia 4. Multiple subchondral cysts 5. Enlarged epiphysis 6. Narrowing of joint space 7. Gross incongruence of articulating bone ends 8.[orthopaedicprinciples.com]
Joint Deformity
  • deformity, decreased range of motion, and unequal extremity lengths.[appliedradiology.com]
  • The objective is to correct joint deformity and to restore function.[boneandspine.com]
  • The late sequelae of joint hemorrhage appear in adolescence or adulthood as a joint deformity, contracture, and/or degenerative arthritis.[emedicine.medscape.com]
  • In many cases, joint deformities have to be treated by open orthopedic surgery. State-of-the-art treatment of patients with hemophilia requires a multidisciplinary team.[jahjournal.org]
  • EARLY INTERVENTION - SPLINTING, ORTHOTICS, AND ASSISTIVE DEVICES Splints • To immobilize a bleeding or painful joint • To prevent deformity in chronic bleeding joints and/or to prevent postsurgical deformity • To correct joint deformity if a contracture[slideshare.net]
Ankle Pain
  • 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.[ncbi.nlm.nih.gov]
  • 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.[eurekaselect.com]
  • CASE NUMBER 118 Note This is a patient who presents with chronic ankle pain. Frontal and lateral radiographs of the ankle were obtained. The red arrows on the frontal radiograph demonstrate severe narrowing of the tibiotalar joint space.[ctisus.com]
  • This may exceed that of rheumatoid arthritis. [36] Characteristics/Clinical Presentation The characteristics and clinical presentation of ankle arthropathies such as different forms of arthritis can be described as followed: Ankle pain Stiffness Swelling[physio-pedia.com]
Knee Pain
  • The patient followed up data was recorded, which included the information of hospital for special surgery knee score(HSS), range of motion(ROM), post-operative complication, and anterior knee pain.[ncbi.nlm.nih.gov]
  • Total Joint Replacement Total joint replacement in bilateral knees is indicated with Stage IV or Stage V arthropathy when persistent knee pain is present.[boneandspine.com]
Distractibility
  • 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[ncbi.nlm.nih.gov]
  • 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[eurekaselect.com]
Impulsivity
  • INTERVENTIONS: The hemophilic group followed a surface electrical stimulation program (frequency 45 Hz, impulse 200 micros, 10s on/10s off) over an 8-week period on the biceps brachii of both arms. The control group did no training of any kind.[ncbi.nlm.nih.gov]
Unable to Walk
  • He goes in wheelchair unable to walk since the previous five months. Radiographs confirm a grade III hemophilic arthropathy in the right knee and grade II for the left elbow.[medcraveonline.com]

Workup

Lymphocytic Infiltrate
  • HA resembles much more to the rheumatoid arthritis because a presence of thick synovial membrane and large lymphocytes infiltration. However, in its final stages, HA resembles OA and that is when IA injections of MSCs are commonly used.[ncbi.nlm.nih.gov]

Treatment

  • The association between hyperemia (pathologic BPI) and treatment outcome was examined using nonparametric tests for independent samples.[ncbi.nlm.nih.gov]
  • The mean pain score was reduced from 2.62 (range, 2-3; SD, 0.49) before treatment to 0.79 (range, 0-2; SD, 0.65) after treatment.[ncbi.nlm.nih.gov]
  • No haemarthrosis was recorded during treatment or during the follow-up period.[ncbi.nlm.nih.gov]
  • No detailed reports have been published regarding the use of arthroscopy for the treatment of the end-stage hemophilic ankle.[ncbi.nlm.nih.gov]
  • We conclude that etoricoxib provided superior efficacy versus placebo for the treatment of hemophilic arthropathy and was generally safe and well tolerated.[ncbi.nlm.nih.gov]

Prognosis

  • 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.[ncbi.nlm.nih.gov]
  • While the advent of factor replacement therapy has dramatically changed the course of treatment and prognosis for patients with hemophilia, the authors argue that the economic burden of treating these patients is still very high.[ncbi.nlm.nih.gov]
  • Prognosis - Hemophilic arthropathy Not supplied. Treatment - Hemophilic arthropathy Not supplied. Resources - Hemophilic arthropathy Not supplied.[checkorphan.org]
  • Hemophilic pseudotumors are of two types: the adult type, which occurs proximally, usually in the pelvis or femur; and the childhood type, which occurs distal to the elbows or knees and carries a better prognosis. 16, 17 Conservative early management[musculoskeletalkey.com]
  • What is the Prognosis of Hemophilic Pseudotumor? (Outcomes/Resolutions) The prognosis of hemophilic Pseudotumor is usually good, with early diagnosis and appropriate treatment.[dovemed.com]

Etiology

  • Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10coded.com]
  • Applicable To Hemarthrosis in hemophilic arthropathy Code First Code First Help Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10data.com]
  • Collection of cytokine and growth factor samples from subjects on prophylaxis and with joint disease should be considered as a first step to more clearly define the etiology of hemophilic arthropathy.[bloodjournal.org]
  • For example, a traumatic effusion or pigmented villonodular hyperplasia is more likely than hemophilia as an etiology for a bleeding disorder in a female.[emedicine.medscape.com]

Epidemiology

  • Covering how to assess both bleeding children and adults, Haemophilia A and B, molecular basis of the disease, the role of factors in coagulation, epidemiology, pharmacokinetics, and treatment of inhibitors.[books.google.com]
  • Global epidemiology of gout: prevalence, incidence and risk factors. Nature Reviews Rheumatology 2015;11(11):649-662. 28.0 28.1 Kim KY., et al. A literature review of the epidemiology and treatment of acute gout.[physio-pedia.com]
Sex distribution
Age distribution

Pathophysiology

  • A brief review of factor XI deficiency and a hypothetical pathophysiological mechanism for internal derangement induced by myofascial pain dysfunction are included.[ncbi.nlm.nih.gov]
  • Thorough comprehension of the pathophysiology, diagnosis, and both medical and surgical interventions is critical in establishing an appropriate treatment regimen for these patients.[ncbi.nlm.nih.gov]
  • Although histological confirmation was not possible, this study demonstrated that magnetic resonance imaging allows a better understanding of the pathophysiology and the natural evolution of the subchondral cysts in hemophilic arthropathy.[ncbi.nlm.nih.gov]
  • […] of Hemophilic Arthropathy. ( 28672826 ) Melchiorre D....Matucci-Cerinic M. 2017 21 A Combined Ultrasonographic and Conventional Radiographic Assessment of Hemophilic Arthropathy. ( 28824241 ) Chung S.W....Kim J.H. 2017 22 Pathophysiology of hemophilic[malacards.org]
  • Blood 2011 117:2302-2303; doi: In this issue of Blood, Acharya and colleagues provide evidence for the role of angiogenesis in the pathophysiology of hemophilic joint disease. 1 Is this the linchpin that unravels this important clinical condition or merely[bloodjournal.org]

Prevention

  • 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.[ncbi.nlm.nih.gov]
  • When severe joint bleeds have caused synovitis and arthropathy, chemical or radioactive synovectomy and surgical joint procedures seem feasible to prevent further joint damage.[ncbi.nlm.nih.gov]
  • This article highlights issues surrounding hemophilic arthropathy of the elbow and focuses on preventive measures, management strategies of the hemophilic elbow, and treatment options for established arthropathy. Copyright 2011 Elsevier Inc.[ncbi.nlm.nih.gov]
  • Treatment with rhFVIII reduced or prevented swelling and degenerative joint changes, returning the F8(-/-) animals to a wild-type phenotype.[ncbi.nlm.nih.gov]

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