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]
In a poster presented at the World Federation of Hemophilia, results were presented of a study to quantify the amount of daily physical activity performed by adults with hemophilic arthropathy receiving secondary prophylaxis under supervision of the treatment [rarebleedingdisorders.com]
Entire Body System
- Pain
Before and 12 weeks after treatment, pain assessment was performed using the visual analog scale (VAS). The pain status assessment included the general status, pain of all joints affected, and pain of the 3 mostly involved joints. [ncbi.nlm.nih.gov]
- Swelling
His knee radiography revealed subchondral cysts, widening of intercondylar notch, soft tissue swelling, flattened intercondylar surfaces, erosions, generalized osteopenia on anteroposterior view, and squaring of patella on lateral view (Fig.1 and 2). [chanrejournals.com]
Joint swelling was measured prior to injury, the following 7 days and upon euthanasia. [ncbi.nlm.nih.gov]
Causes - Hemophilic arthropathy * Difficulty moving joints * Joint pain * Joint swelling Prevention - Hemophilic arthropathy Not supplied. [checkorphan.org]
It causes pain, stiffness, and swelling. Over time, the joint can become severely damaged. [icdlist.com]
- Surgical Procedure
The most common surgical procedures used to manage hemophilic arthropathy are synovectomy, joint débridement, fusion, and joint arthroplasty. [journals.lww.com]
The most common surgical procedures used to manage hemophilic arthropathy are synovectomy, joint debridement, fusion, and joint arthroplasty. [ncbi.nlm.nih.gov]
From anatomical and biomechanical foundations, to revision total knee replacement, this authoritative reference provides the most up-to-date and complete guidance on cutting-edge surgical procedures, the largest collection of knee videos in one knee textbook [books.google.com]
[…] the tendency of the coagulated areas to slough after surgery; Wound suction in deep wounds for a minimum of 24 hours; No aspirin or other medications postoperatively that inhibit platelet As many procedures in one single surgical session No intramuscular [orthopaedicprinciples.com]
procedures • Radionuclide synovectomy • Chemical synovectomy – Rifampacin/oxytetracycline • Arthroscopic/open synovectomy 38. [slideshare.net]
Hematological
- Hemophilia A
AIM: : The purpose of this study was to determine the correlation between these tools when used to assess hemophilia patients. METHODS: This cross-sectional study enrolled 38 hemophilia patients between 2 and 18 years of age. [ncbi.nlm.nih.gov]
MD Articles Abstract Author Information Authors The most common clinical manifestation of hemophilia is arthropathy secondary to recurrent hemarthroses and chronic synovitis. [journals.lww.com]
- 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]
They may experience joint bleeds or easy bruising. [20] Mild haemophilia may only be discovered later, usually after an injury or a dental or surgical procedure. [en.wikipedia.org]
Gastrointestinal
- Nausea
It can cause disorientation, nausea, loss of consciousness, brain damage, and death. [en.wikipedia.org]
Cardiovascular
- Thrombosis
© 2010 International Society on Thrombosis and Haemostasis. [Indexed for MEDLINE] Free full text [ncbi.nlm.nih.gov]
Thrombosis and Haemostasis. 110 (6): 1114–20. CiteSeerX 10.1.1.684.7962. doi : 10.1160/TH13-05-0363. [en.wikipedia.org]
Recommendation of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 2001; 85 : 560. 5. [err.eg.net]
Musculoskeletal
- Arthritis
Hemophilic arthropathy shares some injury characteristics with rheumatoid arthritis, although the degree of analogy is a matter of some debate. [ncbi.nlm.nih.gov]
Joint ankylosis- One close Differential diagnosis would be JRA but unlike in JRA bony ankylosis and growth retardation does not occur in haemophilic arthritis. [orthopaedicprinciples.com]
Septic Arthritis Until the early 1980s, septic arthritis rarely occurred in hemophilic patients. [musculoskeletalkey.com]
ICD-9-CM Volume 2 Index entries containing back-references to 713.2 : Arthritis, arthritic (acute) (chronic) (subacute) 716.9 due to or associated with acromegaly 253.0 [ 713.0 ] actinomycosis 039.8 [ 711.4 ] amyloidosis 277.39 [ 713.7 ] bacterial disease [icd9data.com]
- Ankylosis
Joint ankylosis- One close Differential diagnosis would be JRA but unlike in JRA bony ankylosis and growth retardation does not occur in haemophilic arthritis. [orthopaedicprinciples.com]
[…] abnormalities), I (minimal amount of hemosiderin), II (large amount of hemosiderin and isolated cartilaginous erosion), III (cartilage destruction, bone erosions, and subchondral cysts) to IV (large internal joint derangement, secondary osteoarthritis and/or ankylosis [ncbi.nlm.nih.gov]
RADIOLOGIC MANIFESTATIONS OF CHRONIC HEMOPHILIC ARTHROPATHY Specific • Femoral intercondylar notch widening • Squaring of distal patellar margin (lateral view) • Proximal radial enlargement • Talar flattening ± ankle ankylosis 23. [slideshare.net]
Stage V Total loss of joint space with fibrous ankylosis of the joint. There is marked incongruity of the articular structures with severe irregular hypertrophy of the epiphysis. [boneandspine.com]
Furthermore, in severe forms of hemophilic arthropathy, ankylosis, fusion of the bones can occur; a phenomenon also observed in severe osteoarthritis [7], [8]. [err.eg.net]
- Joint Effusion
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]
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]
This image shows an opaque joint effusion due to the presence of iron in the synovium (arrows). [emedicine.medscape.com]
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]
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
The late sequelae of joint hemorrhage appear in adolescence or adulthood as a joint deformity, contracture, and/or degenerative arthritis. [emedicine.medscape.com]
deformity, decreased range of motion, and unequal extremity lengths. [appliedradiology.com]
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]
The objective is to correct joint deformity and to restore function. [boneandspine.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]
- Joint Swelling
Causes - Hemophilic arthropathy * Difficulty moving joints * Joint pain * Joint swelling Prevention - Hemophilic arthropathy Not supplied. [checkorphan.org]
Treatment with rhFVIII reduced or prevented swelling and degenerative joint changes, returning the F8(-/-) animals to a wild-type phenotype. [ncbi.nlm.nih.gov]
Joints can be damaged by many types of injuries or diseases, including Arthritis - inflammation of a joint. It causes pain, stiffness, and swelling. Over time, the joint can become severely damaged. [icdlist.com]
As chondrocytes rarely proliferate, apoptosis leads to long lasting and irreversible damage to the cartilage matrix, or permanent cartilage loss. 2 Stage 1: Soft tissue swelling of the joint and adjacent tissues. [now.aapmr.org]
In an acute joint bleed, patients may report sensations of tingling and warmth, followed by pain, swelling and decreased motion. [physio-pedia.com]
Neurologic
- Headache
The most common adverse experiences were upper respiratory infection and headache. [ncbi.nlm.nih.gov]
Haug Browse recently published Learning/CME Learning/CME View all learning/CME CME Case 3-2019: A 70-Year-Old Woman with Fever, Headache, and Progressive Encephalopathy Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura Randomized [nejm.org]
[…] of bleeding inside joints or the brain. [1] Those with a mild case of the disease may have symptoms only after an accident or during surgery. [1] Bleeding into a joint can result in permanent damage while bleeding in the brain can result in long term headaches [en.wikipedia.org]
- Seizure
[…] inside joints or the brain. [1] Those with a mild case of the disease may have symptoms only after an accident or during surgery. [1] Bleeding into a joint can result in permanent damage while bleeding in the brain can result in long term headaches, seizures [en.wikipedia.org]
- Encephalopathy
Haug Browse recently published Learning/CME Learning/CME View all learning/CME CME Case 3-2019: A 70-Year-Old Woman with Fever, Headache, and Progressive Encephalopathy Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura Randomized [nejm.org]
Workup
X-Ray
- Joint Space Narrowing
Abnormal articular cartilage was demonstrated in 26 joints; bone lesions, fluid collections, and joint space narrowing could also be seen. MR imaging appears to be useful in depicting the components of hemophilic arthropathy. [ncbi.nlm.nih.gov]
The images also revealed significant degenerative changes of the knee joint, including widening of the intercondylar notch and joint space narrowing (Figures1 and 2). [appliedradiology.com]
[…] of patella; inter-condylar notch of distal femur or trochlear notch of the ulna widened no significant narrowing of joint space; joint shows signs of disorganization III NA progression of bone changes in Stage III joint space narrowed with destruction [meducator3.net]
Treatment
The association between hyperemia (pathologic BPI) and treatment outcome was examined using nonparametric tests for independent samples. [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]
Prognosis - Hemophilic arthropathy Not supplied. Treatment - Hemophilic arthropathy Not supplied. Resources - Hemophilic arthropathy Not supplied. [checkorphan.org]
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]
[…] radiosynoviorthesis radioisotopes can be injected therapeutically into a joint to decrease bleeding and synovitis Rhenium 186 is emerging as the preferred isotope over Phosphorus 32 and Yttrium 90 particularly in medium-sized joints 5 Treatment and prognosis [radiopaedia.org]
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]
Two other major causes of death include hepatitis infections causing cirrhosis and obstruction of air or blood flow due to soft tissue haemorrhage. [16] Epidemiology [ edit ] Haemophilia is rare, with only about 1 instance in every 10,000 births (or 1 [en.wikipedia.org]
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]
[…] 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]
Pathophysiology of Hemophilic Arthropathy. J. Clin. Med. 2017, 6, 63. Show more citation formats Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here. [Return to top] [mdpi.com]
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]