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]
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]
Hematological
- 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]
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]
- 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]
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]
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 late sequelae of joint hemorrhage appear in adolescence or adulthood as a joint deformity, contracture, and/or degenerative arthritis. [emedicine.medscape.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]
- 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]
- Arthralgia
Response to RSO was evaluated 6 months post-treatment by measuring changes in intensity of arthralgia according to the visual analog scale (VAS), bleeding frequency, and range of motion. [ncbi.nlm.nih.gov]
If the patient later suffers from chronic arthralgias, corticosteroids should be avoided [24]. Steroids are administered when inflammatory symptoms are resistant to NSAIDs [75]. [physio-pedia.com]
Psychiatrical
- 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 [eurekaselect.com]
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]
- 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]
Workup
Other Pathologies
- 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
Articular range of movement was assessed before treatment and during follow up in short and midterm. All treated knees showed grade III arthropathy before and after treatment. Elbow arthropathy was classified as grade II before and after treatment. [medcraveonline.com]
Keywords: Hemophilia, ankle, hemophilic arthropathy, treatment, hemorrhages, radiosynovectomy. Title: Management of Hemophilic Arthropathy of the Ankle VOLUME: 17 ISSUE: 2 Author(s): E. [eurekaselect.com]
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]
[…] 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]
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]
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]
Prevention of bleeding episodes through early treatment will prevent accumulation of blood in the joint and the subsequent inflammation and potential haemophilic arthropathy. [jcomorbidity.com]