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Examination of Knee Joint in a Child
Published in Nirmal Raj Gopinathan, Clinical Orthopedic Examination of a Child, 2021
Knee cruciates are strong cord-like structures present in the intercondylar region of the tibia and femur, preventing their movements on each other in the anteroposterior direction. The ACL passes backward and outwards from the anterior intercondylar region of the tibia to attach to the posterior medial aspect of the lateral condyle of the femur. The PCL, however, originates from the posterior intercondylar region and adjacent popliteal surface of the tibia and passes inwards and forwards to insert on the anterior aspect of the lateral surface of the medial condyle of the femur. With an acute injury, the child may be in pain and muscle guarding may be present. The knee may be swollen because of hemarthrosis. In such circumstances, the various physical examination tests for anterior and posterior instability may be of less value. These tests have a greater value for chronically deficient knees.
Erupting tooth hemophilia
Published in Alisa McQueen, S. Margaret Paik, Pediatric Emergency Medicine: Illustrated Clinical Cases, 2018
Clinical manifestations will vary based on the severity and can range from intracranial hemorrhage in the newborn, epistaxis, easy bruising intramuscular hematomas, and gastrointestinal bleeding. Hemarthrosis is a hallmark presentation. Administration of factor is based on the severity and location of the bleeding.
Genetics
Published in Manoj Ramachandran, Tom Nunn, Basic Orthopaedic Sciences, 2018
Peter Calder, Harish Hosalkar, Aresh Hashemi-Nejad
Haemophilia. Haemophilia is the oldest known hereditary bleeding disorder with an X-linked recessive inheritance. Haemophilia A has abnormal factor VIII and haemophilia B (Christmas disease) has abnormal factor IX. Because of deficiency of the clotting factor, patients present with abnormal bleeding spontaneously or following trauma. Typically, bleeds occur deep within muscles and in joints. The iliacus is the most commonly involved muscle, presenting with abdominal or hip pain, flexion deformity and swelling (pseudotumour) of the hip. Ultrasonography is very helpful in delineating the cause. Haemarthrosis presents with pain, swelling and restricted motion (most commonly in the knee). Acute medical management of the disease often includes factor replacement and splinting of the affected joints. It is important to determine the factor inhibitor level so that factor levels can be raised to at least one-fourth of the baseline in major bleeds. Some of the radiological features include osteopenia, thinning of the bones, overgrowth of bones and squaring of the patella.
Applying World Health Organization 2020 guidelines on physical activity and sedentary behavior to people with hemophilia
Published in Expert Review of Hematology, 2021
Hortensia De la Corte-Rodriguez, E. Carlos Rodriguez-Merchan, M. Teresa Alvarez-Roman, Victor Jiménez-Yuste
Thanks to advances in hematological treatments and prophylactic modalities, physical activity has been made possible for PWH, to help maintain their health [3]. Physical activity confers several benefits to the PWH such as improved muscle strength, joint stability and flexibility, decreased pain, increased bone density and prevention of falls, reduced risk of bleeding [4]. Other physical benefits include the prevention of obesity, heart disease, high blood pressure and diabetes. Physical activity also confers several mental health benefits, such as improved emotional regulation, self-efficacy and social interactions [3]. Physical inactivity is more harmful in PWH than in the general population; thus, the WHO recommendations are especially important in hemophilia [3]. Without proper treatment, people with hemophilia will suffer multiple joint and muscle bleeds. Joint bleeding (hemarthrosis) is the most frequent clinical manifestation in hemophilia. Repeated hemarthroses will lead to a process of inflammation and synovial hypertrophy, as well as progressive deterioration of the osteochondral structures, known as hemophilic arthropathy [5]. This arthropathy usually affects several joints, mainly ankles, knees and elbows. The degree of arthropathy will condition the pain, the functional deficit and the detriment in the quality of life of the patients.
Incapacitating pain from Tenofovir Induced Hypophosphatemic Osteomalacia in a Hemophilia Patient – A Case Report
Published in Canadian Journal of Pain, 2020
Emma Woo, Dinesh Kumbhare, Paul Winston
Pain is commonplace in patients with hemophilia and often arises as the result of hemarthrosis, joint bleeds that may cause progressive joint damage and have a significant effect on quality of life and mobility.1 However, it cannot be assumed that severe pain is due to hemarthrosis-related joint damage. Clinicians should consider a wide range of etiologies for pain in patients with hemophilia. Acute causes include infection, osteoporotic fractures, bleeds, with chronic causes including arthritis, and osteomalacia. A comprehensive medical history may reveal unanticipated causes, including side effects from medications such as tenofovir, specifically tenofovir disoproxil fumarate. This case demonstrates a rare instance of poorly localized back and hip pain, severe enough to prevent ambulation in a patient with hemophilia A caused by hypophosphatemic osteomalacia due to tenofovir treatment for blood transfusion–acquired human immunodeficiency virus (HIV). In the early 1980s, before blood products were tested or heated, nearly 50% of the U.S. population with hemophilia had become infected with HIV from blood transfusions.2 Among the aging population of people with hemophilia, there is a significant likelihood of an HIV infection.2 Other uses of tenofovir include pre-exposure prophylaxis (PrEP) and treatment of chronic hepatitis B infections.
Joint lavage followed by intra-articular injection of hyaluronic acid and/or corticosteroids in patients with severe hemophilic arthropathy of the knee: Is this intervention really effective?
Published in Expert Review of Hematology, 2018
E. Carlos Rodriguez-Merchan, Leonard A. Valentino
Hemophilia is a congenital bleeding disorder due to the deficiency of blood coagulation factor VIII or IX leading to hemophilia A or B, respectively. Bleeding and in particular joint bleeding or hemarthrosis is the hallmark of the disease. Recurrent hemarthrosis or even a single severe joint hemorrhage may lead to hemophilic arthropathy, the most common clinical manifestation of hemophilia. Bleeding into the joint results in an inflammatory and proliferative process termed hemophilic synovitis. Although the precise biochemical and molecular mechanisms underlying this disorder remain somewhat speculative, it is clear that bleeding into the joint is the trigger of an inflammatory and proliferative process that involves an aggressive vascularization of the synovial membrane.