[en] Hemophilia is a primary disorder of coagulation complicated by spontaneous intra-articular hemorrhage. This paper is to describe and analyze the radiographic findings of involved bone and joints of hemophilic patients for past 5 years. The results were as follows: 1. Age distribution of the total 28 patients was ranging from 1 year to 42 years. 86% of patients was below the age of 20 years. 2. The incidence of joint involvement was in next order; knee joint (25), elbow joint (4), ankle joint (2), hip joint (1). 3. The radiologic findings of involved joint were characterized by irregularity of articular cortices (22), joint space change (19), soft tissue swelling (19), subchondral or intra-osseous cyst (12), periarticular osteoporosis (19), degenerative change (10), condylar enlargement (11) intercondylar notch widening (21) and flexion deformity (13)

[en] Even before the discovery of X-rays in 1895 attempts were being made to classify the joint destruction that occurs in hemophilic arthropathy. The advent of radiography added impetus to the search for the optimum classification system. Subsequent attempts have included advanced imaging methods, especially magnetic resonance imaging (MRI). Because of its high spatial resolution and ability to visualize soft tissue abnormalities, MRI is ideally suited as the best way to classify arthropathy. A literature search was carried out to document and list all the imaging methods for hemophilic arthropathy that have been published up to the present. All published classification systems are discussed and listed in tables. MRI has superior imaging capability and will probably become the most important modality for radiological classification of hemophilic arthropathy in the future

[en] In patients with hemophilia, prothrombin complex concentrates (PCCs) have been successfully used to bypass inhibitors to fctor VIII during bleeding episodes. The use of PCCS, including FEIBA (factor eight inhibitor bypassing activity), has been associated with thromboembolic complications. Myocardial infarction (MI) is a rare but serious complication, reported in 13 previous cases, six in the pediatric age group. In all four patients who died during the acute MI, autopsy revealed extensive myocardial hemorrhage. The hearts of three other patients examined at least 5 months after the acute MI showed no evidence of prior hemorrhage. Magnetic resonance (MR) imaging has been shown to be able to evaluate the sequelae of myocardial infarction in adults with coronary artery disease and in children with Kawasaki syndrome. We report the first case of the used of MR imaging in the evaluation of myocardial damage during the acute stage of a FEIBA-associated MI in a 10-year-old boy. (orig.)

[en] Kidneys are the main route of elimination for many antineoplastic drugs and their metabolites. The kidney dysfunction may lead to the drug cumulation in organism with the resulting increased systemic toxicity. A lot of used cytostatics requires a dose modification at different levels of renal insufficiency. Due to the lack of data from clinical trials, the limiting of systemic toxicity is difficult especially in patients with severe renal impairment or patients undergoing chronic hemodialysis. The following article is focused on the preventive strategies dealing with recommended dosing modification of various antineoplastic agents in patients with renal insufficiency. (author)

[en] Sero prevalence of Hepatitis B surface antigen (HbsAg) and anti-HCV IgG was determined in 100 persons with Hemophilia (PWH), registered with Hemophilia Patient Welfare Society (HPWS), Lahore Zone, Pakistan. The study shows that 4% were positive for HBsAg. However, there was a high level of anti-HCV sero positivity (56%) in our PWH, including many patients in younger age groups. When compared with figures from PWH in other regions of Asia like 23% in Western India, 33% in Sri Lanka and 15% of those in Iran, this figure is one of the highest. This rate is a reflection of the same rising trend in our population that is now exceeding 10%. The practice of unscreened blood/blood-products transfusions in the backdrop of high prevalence of HCV in our population is responsible for high figures seen in PWH. The need is to increase awareness amongst the patients, health care workers and policy makers about the transfusion associated viral infections in a group of patients who already had a hereditary disorder of severe nature. (author)

[en] Two intracranial hemorrhages occurred in a young child with hemophilia A. Difficulties in diagnosis and treatment are described. Both incidences ended with a full cure.(author)

[en] Arthropathy is one of the most disabling consequences of haemophilia. The management of this condition has been revolutionized by the introduction of prophylactic treatment with clotting factor replacements. In order for benefits of this treatment to be monitored, the severity of any joint disease needs to be accurately assessed. Diagnostic imaging is used to objectively evaluate haemophilic arthropathy (HA). There are several established scoring systems for grading HA based on conventional radiography and magnetic resonance imaging (MRI). In this review the role of the different imaging methods in the evaluation of HA are discussed, the established staging systems are presented, and an explanation of why MRI is becoming the primary method of joint imaging in HA is provided.

[en] In 1977, Ahlberg saw the need to stop the vicious circle of target joints seen in hemophiliacs which causes lifelong disability and interrupts the physical and social activities of the child by this procedure. Over the decades, it has gained acceptance by providing a simple, economic, and effective therapy for them. Radiosynovectomy by injecting radionuclides intraarticular space is well established as an alternative method to decrease joint hemorrhage episodes and to prevent arthropathy in hemophiliacs. Radiosynovectomy presents with a therapeutic effect of 60%-94% for hemophilia A in several studies; our study showed 80%. Radiosynovectomy is a minimally invasive, well-tolerated procedure that can be done on an outpatient basis

[en] Haemophilia disorders are characterised by a blood coagulation anomaly leading to prolonged and excessive bleeding. Imaging provides an essential role in the investigation of both the musculoskeletal and the non-musculoskeletal complications of haemophilia. Our institution is home to a large tertiary referral centre for haemophilia treatment. Using our broad experience, we present a multi-modality pictorial review of the musculoskeletal manifestations of haemophilia, including haemophilic arthropathy, intra-muscular haemorrhage and haemophilic pseudotumour. The main imaging features of haemophilic arthropathy are described, including synovial hypertrophy, haemosiderin deposition, sub-chondral cyst formation and loss of joint space. (orig.)

[en] Radiosynovectomy has been used as an effective treatment in patients with resistant synovitis after failure of long-term medication and intraarticular steroid injection. Although ⁹⁰Y silicate/citrate, ¹⁸⁶Re sulfide, and ¹⁶⁹Er citrate were approved in Europe for the appropriate radiopharmaceuticals for radiosynovectomy, other radionuclides such as ³²P-chromic phosphate, ¹⁶⁵Dy-ferric hydroxide macroaggregate, ¹⁸⁸Rh-microspheres, ¹⁵³Sm-particulate, and ¹⁶⁶Ho- ferric hydroxide macroaggregate have been used in many countries. Reported success rates range from 40% to 90% for the different joints and underlying disease. In Korea, ¹⁸⁸Re-tin-colloid and ¹⁶⁶Ho-chitosan complex are now using as the major radionuclides in radiosynovectomy with good clinical results. A study on radiation synovectomy using ¹⁸⁸Re-tin-colloid for patients with Korean rheumatoid arthritis shows the treatment resulted in the improvement of arthritis and well tolerated. In our study, the radiosynovectomy with ¹⁶⁶Ho- chitosan complex in 53 hemophilic patients markedly decreased intra-articular bleeding frequency and need for coagulation factor. This review includes general principles in the application of radiosynovectomy and the clinical experience in Korea