Orthotics and rehabilitation for chronic hemophilic synovitis of the ankle. An overview

The ankle is the second most affected joint in hemophilia. Recurrent bleeding leads to chronic synovitis. Prevention of chronic synovitis should start with prophylactic replacement therapy or on demand treatment of recurrent hemarthrosis. Attention must be given to the function of the ankle joint. When instability is present, an extensive range of exercises is important. An orthotic or shoe adaption may be useful during the rehabilitation process. Viscoheels worn in shoes seem to reduce the bleeding frequency and pain.     

A clinical study of dialysis-related osteoarthropathy in long-term hemodialysis patients

Amyloid osteoarthropathy has been seen frequently in long-term hemodialysis (HD) patients, in which the bone X-ray examination reveals characteristic cystic radiolucency (CRL) of the carpal bone, shoulder joint, hip joint and knee joint, and destructive spondylarthropathy (DSA) of cervical vertebrae. To clarify the clinico-pathological significance of CRL and DSA in HD-related amyloidosis, we investigated the grade and frequency of CRL or DSA and these relationship with age, HD duration, primary diseases, osteoarticular symptoms and blood analysis in 817 HD patients (492 male and 325 female, age: 52.6 + 15.5 years, dialysis duration: 6.8 + 5.4 years). The number of cases with osteoarticular symptoms increased with the prolongation of HD duration. CRL and DSA were observed even in patients without osteoarticular symptoms: 26.7% for carpal bone, 26.2% for shoulder joint, 17.3% for hip joint and 22.2% for DSA. The grade and frequency of CRL and DSA also increased in accordance with age and HD duration. In patients with CRL of the carpal bone, shoulder CRL was noticed in 39.7%, hip CRL in 25.8%, and DSA in 14.3% of cases, respectively, and these frequencies increased with the prolongation of HD duration. In the carpal CRL negative group, shoulder CRL was noticed in 14.6%, hip CRL in 7.5%, and DSA in 6.0%, respectively. Although there was no relationship between CRL or DSA and serum beta 2-MG level in any of the cases, the serum beta 2-MG level was lower in patients with HD showing shoulder CRL (+2) and DSA (+) for more than 16 years. No significant relationship was noticed between CRL or DSA and serum C-PTH and aluminum level. These results suggested that aging was related to CRL or DSA formation in dialysis-related amyloidosis. The findings also suggested that systemic bone X-ray examination should be considered in patients with carpal bone CRL, high-age patients and long-term HD patients even without osteoarticular symptoms.     

Medical treatment of heart failure at a tertiary hospital of S?o Paulo]

The clinical hemostatic effect of tranexamic acid mouthwash after oral surgery was evaluated in 47 patients receiving oral anticoagulant therapy. Surgery was performed after the anticoagulant medication was reduced in 15 patients (control group) and with no change in anticoagulant therapy in 32 patients (test group). The only statistical difference between the two treatment groups at baseline was the level of anticoagulation, which was significantly higher in the test group. There was no significant difference between the two treatment groups in the incidence of bleeding after oral surgery. The results indicated that a combination of local antifibrinolytic therapy and a local hemostatic agent is effective in preventing postoperative bleeding after oral surgery in patients treated with anticoagulants.     

Traumatic hemarthrosis of the knee joint--clinical significance of nuclear magnetic resonance tomography

A prospective study was performed on 94 patients with hemarthrosis of the knee to assess the value of MR imaging (MRI) in detection of bleeding sources and selection of therapy modalities. Patients were examined clinically within a week after knee trauma; the investigations performed included puncture of the joint to confirm hemarthrosis, a conventional X-ray to exclude fractures, MRI and arthroscopy. MRI was performed for diagnosis of acute lesions, definition of bleeding sources by morphological criteria, and allocation of patients to conservative or surgical therapy. Arthroscopy was performed to control MRI, to visualize bleeding sources, and to maintain therapy if necessary. The 94 patients were found on arthroscopy to have a total of 123 bleeding sources, which were correctly defined by MRI in each of 107 cases as an acute lesion communicating with the joint space; 16 bleeding sources were not found and there were 10 false-positive reports. In keeping with our treatment strategies, arthroscopy disclosed lesions requiring surgical therapy in 77 of 94 patients (82%) and lesions that would be adequately treated by conservative therapy in 17 of 94 patients (18%). MRI predicted surgical or conservative therapy correctly before arthroscopy in 83 of 94 patients (88%). In conclusion, MRI has a high predictive value in definition of acute lesions that will lead to hemarthrosis of the knee. This noninvasive method allows screening out of the relatively small portion of patients without severe lesions, who then do not have to be subjected to further invasive therapy.     

Self management of peroral anticoagulant therapy in patients with artificial heart valves

Twenty-one heart valve operated patients (age 19-70 years) were trained in self-managed oral anticoagulant therapy using a home coagulometer (CoaguChek). Twenty patients accomplished between eight and 29 (median 24) months of self management and were fully capable of self management after 30 weeks of training. No patients experienced major bleeds or thrombo-embolic events. A control group of 20 patients from our department was matched, retrospectively, to the study group. The self-managing patients were within the therapeutic INR range 78% of the study period compared to 54% for the control patients. All self-managing patients had their median INR-value within the therapeutic range, versus only 14 in the control group. Self-management of oral anticoagulant therapy seems feasible for selected patients.     

Treatment of coumarin-induced skin necrosis with a monoclonal antibody purified protein C concentrate

BACKGROUND AND DESIGN--Protein C is a vitamin K-dependent plasma protein that is converted to the serine protease activated protein C by the thrombin-thrombomodulin complex. Activated protein C functions as a natural anticoagulant by inactivating the cofactors of the coagulation cascade, factors Va and VIIIa. Coumarin (warfarin)-induced skin necrosis is thought to be due to a rapid elimination of protein C relative to other vitamin K-dependent factors during the initial phase of oral anticoagulation. We have used a highly purified protein C concentrate to treat a patient with acquired protein C deficiency who developed skin necrosis during the initial phase of oral anticoagulant therapy. OBSERVATIONS AND CONCLUSIONS--During protein C concentrate therapy, no further skin lesions appeared, and the healing process of necrotic areas was facilitated. Replacement therapy with protein C concentrate appears to be safe and effective as an adjunctive treatment for coumarin-induced skin necrosis.     

Efficacy of prothrombin-complex concentrates in hemophiliacs with antibodies to factor VIII: a multicenter therapeutic trial

The therapeutic efficacy of prothrombin-complex concentrates in patients with hemophilia and inhibitors (antibodies) to factor VIII has been increasingly debated. We therefore entered 51 hemophiliacs with factor VIII inhibitors into a double-blind randomized crossover study to compare two commercial prothrombin-complex concentrates (Konyne and Proplex) and an albumin placebo. Acute hemarthrosis of the elbow, knee, or ankle was treated with a single dose of a test preparation and assessed six hours later with objective and subjective criteria. In all measurements the concentrates were significantly more effective than the placebo. The data indicate that although prothrombin-complex concentrates, when used in a single dose, are only partially effective in the treatment of joint hemorrhage in hemophiliacs with inhibitors, their continued use for acute hemarthrosis is justified in the absence of any other effective and readily available therapy for this disorder.     

Time of day variations in driving performance

A 25-year-old woman complained of anasarca and was admitted to Sakura National hospital on the presumptive diagnosis of nephrotic syndrome with 10.7 g of 24-hour urinary protein. At first, lupus nephritis with antiphospholipid antibody syndrome was suspected because of prolongation of APTT, existence of lupus anticoagulant and elevation of serum anticardiolipin antibody titer (IgM) in addition to positive ANA, lymphocytopenia and the biologically false positive test for syphilis (BFPTS). On day 28 of hospitalization, renal biopsy findings revealed severe endocapillary cell damage, such as swelling and proliferation of endothelial cells, fragmentation and double contour of the basement membrane walls, which were located only in the capillary lumens with a few thrombi. Immunofluorescent micrography revealed the absence of specific immunoglobulin or complement deposit. Therefore, the diagnosis of lupus nephritis was negated as these findings were suggestive of characteristic glomerulopathy due to primary antiphospholipid antibody syndrome. She was treated initially with oral prednisolone 60 mg and intravenous infusion of heparin 20,000 units daily. Moreover, cyclophosphamide 750 mg was administered intravenously as pulse therapy on day 13 as her serum level of CH50 had fallen suddenly, and hemodialysis was necessary because her renal function had deteriorated and she was suffering from cough and orthopnea with overhydratin. After the combined therapy, BFPTS disappeared and APTT returned to the normal range: dialysis treatment was not required further after the 4th hemodialysis. Thereafter, renal function improved and complete remission of nephrotic syndrome was obtained. This patient was a case of primary antiphospholipid antibody syndrome in which endothelial cell damage was located exclusively in the capillary lumens and pulse cyclophosphamide therapy in addition to prednisolone and anticoagulant was effective. We present this instructive case to promote understanding of the pathogenesis of primary antiphospholipid antibody syndrome.     

Development of periradicular lesions in immunosuppressed rats

A 53-year old man on long-term hemodialysis (HD) with anticoagulant therapy was scheduled for nephrectomy due to renal cell carcinoma. Two months before surgery, a coronary stent had been placed due to right coronary artery disease. One week before surgery, percutaneous transmural coronary angioplasty (PTCA) was performed for unstable angina. Aggressive oral antiplatelet therapy (aspirin and ticlopidine) was absolutely required to maintain patency. Following withdrawal of the antiplatelets, unfractionated heparin (UFH) was titrated to an activated partial thromboplastin time (APTT) of 1.5 times greater than the control value. Maintenance UFH (800 U.h-1) was continued until the time of arrival in the operation room (activated clotting time (ACT) was 166 seconds). One hour after arrival, reduced dose of UFH (200 U.h-1) was reinfused, and ACT was 121 140 seconds. Hemodynamic change was minimized using balanced general anesthesia (nitrous oxide-isoflurane, fentanyl, midazolam and vecuronium) accompanied by nitroglycerin and diltiazem. There was no ischemic change on ECG or transesophageal echocardiography. Following surgery, the UFH dose was augmented (400 U.h-1), and the maintenance dose was attained 11 hours after surgery. HD on the second postoperative day was performed uneventfully. This hemodynamic stability might be come from the no water removal. Fourteen days after surgery, the patient was discharged without hemorrhagic complications or clinical ischemic events. We conclude that perioperative UFH infusion is not contraindicated for dialysis patient if strict ACT control is maintained.     

Prophylactic transfusion for hypertrophic synovitis in children with hemophilia

A 7- to 9-month protocol of prophylactic transfusion was used to treat 33 joints in 19 children with severe hemophilia (< 1 U/dL Factors VIII or IX) and hypertrophic synovitis. The overall rate of hemarthrosis was reduced, but only 36% (12 of 33 joints) achieved a good result (defined as 0-0.5 bleeding episodes per month and decreased synovial hypertrophy 1 year after completing treatment). Age and severity of arthropathy at initiation of treatment did not affect the result. The degree of synovial hypertrophy and involvement of the knee joint showed an adverse trend, but these factors did not achieve statistical significance. The number of episodes of breakthrough bleeding during the first 6 weeks of therapy was significantly associated with a poor result. Based on the results of this study, a trial of transfusion therapy is recommended for recurrent hemarthroses and synovitis in patients with hemophilia, but the duration of thrice weekly treatment has been increased and the duration of prophylaxis has been reduced in selected cases.     

Physiotherapy for prevention and treatment of chronic hemophilic synovitis

Hemophilia is a hereditary lifelong bleeding disorder affecting males. The nature of the condition predisposes the person to bleed intraarticularly and intermuscularly. Without intervention with replacement factor therapy and physiotherapy, the consequences can lead to chronic synovitis and severe joint hemarthropathy. Physiotherapeutic interventions are available that may help to prevent and treat the sequelae of recurrent hemarthrosis. No particular method of treatment has been shown to eradicate hemorrhages; however, there are protocols that can help. The use of physiotherapy techniques including electrotherapy, joint care, and exercise are extremely important, especially in developing countries where blood products are scarce.     

Hemarthrosis and hip joint pressure in femoral neck fractures

In a prospective clinical study the intraarticular pressure of 55 patients with intracapsular femoral neck fractures was measured intraoperatively with the hip in different positions. Intraarticular hemarthrosis was quantified by a preoperative sonography examination. In 75% of the patients, increased intraarticular pressure caused by the hemarthrosis was found. The spontaneous median pressure increased significantly from 22 mm Hg with extension (28 mm Hg) and internal rotation of the hip joint (56 mm Hg). The lowest pressure was found in 70 degrees flexion (15 mm Hg). The median pressures increased within the first 24 hours after injury from 26 mm Hg in the first 6 hours to 46 mm Hg from 7 to 24 hours. Even in the first and second weeks after trauma, increased median pressures were detected (8.5 mm Hg and 13 mm Hg, respectively). No significant difference was found between undisplaced and displaced fracture types. Because increased joint pressure in other studies correlates with reduced perfusion of the femoral head, it can be deduced that reduction maneuvers without capsulotomy can compromise the circulation of the femoral head. Capsulotomy and osteosynthesis of the femoral neck at the earliest time possible is the best prophylaxis of tamponade. If the osteosynthesis is delayed, a preoperative sonography after admission and a control sonogram after 6 hours is recommended. In the event of relevant hemarthrosis, immediate therapeutic drainage is suggested for patients who will receive joint conserving osteosynthesis.     

Doxorubicin cardiotoxicity in children: comparison of a consecutive divided daily dose administration schedule with single dose (rapid) infusion administration

Kyphoscoliosis surgery is frequently associated with major blood loss and coagulation disorders. A patient with juvenile rheumatoid arthritis, heart valve prosthesis and respiratory restrictive syndrome, was submitted to surgical correction of kyphoscoliosis. Current drug therapy included digitalis, oral anticoagulant and nonsteroidal anti-inflammatory drugs. After careful preoperative evaluation, oral anticoagulant and nonsteroidal anti-inflammatory drugs were discontinued (five and ten days before surgery, respectively), and intravenous heparin was introduced and maintained until two h before surgery. Bacterial endocarditis prophylaxis was obtained with ampicillin (50 mg.kg-1) and gentamicin (1.5 mg.kg-1). Anaesthetic management followed a general, balanced technique and the use of invasive monitoring devices. Clotting times were kept within the normal range--prothrombin time between 13 s and 14 s; partial thromboplastin time between 28 s and 30 s. Surgery was straightforward. The patient remained ventilated for 24 h and intravenous morphine (6 micrograms.kg-1.h-1) was used for nurse controlled analgesia. Afterwards, this was changed for patient controlled analgesia. Intravenous heparin was restarted 12 h after surgery and there were no complications postoperatively. Keeping the patient without anticoagulant therapy during this kind of surgery, was the less harmful option, taking into consideration that haemorrhage is inevitable and thromboembolism is a potential, though serious risk.     

Delayed massive soft tissue uptake of Tc-99m MDP after radiation therapy for cancer of the breast

A patient with a history of breast cancer and known lung metastases was referred for a bone scan to investigate the cause of severe neck and right shoulder pain. The bone scan showed massive uptake of the radiopharmaceutical in the soft tissue surrounding the right shoulder. A review of the patient's history indicated that the patient had undergone radiation therapy to the right upper thorax and breast area 14 months previously and an acute radiation dermatitis of the proximal right arm and should had developed. This had long since resolved. Physical examination and plain radiographs of the right shoulder and humerus failed to demonstrate any abnormality.     

Enhanced expression of bcl-2 inhibits apoptosis in cultured human keratinocytes

Intravenous heparin followed by oral warfarin sodium is effective for preventing recurrent thromboembolism in patients who have pulmonary embolism or proximal vein thrombosis. The effectiveness of intravenous heparin depends on obtaining an adequate anticoagulant response early during therapy. A validated heparin protocol should be used to ensure that an adequate anticoagulant response is obtained as soon as possible. Low molecular weight heparin has the practical advantage that it does not require monitoring and dose finding. If thrombolytic therapy is indicated, it is safer for many patients to base management on the noninvasive diagnosis rather than performing pulmonary angiography. In patients suspected to have pulmonary embolism who have nondiagnostic lung scan and adequate cardiorespiratory reserve, serial noninvasive leg testing is a practical approach that avoids pulmonary angiography, identifies patients who have proximal vein thrombosis requiring treatment, and avoids the risks of anticoagulant treatment in the majority of patients.     

Extracorporeal shock wave treatment for chronic calcific tendinitis of the shoulder

OBJECTIVE: To study the clinical and radiological response of chronic calcific tendinitis of the shoulder to extracorporeal shock wave treatment (ESWT). METHODS: The study included 3 female patients, 42, 48, and 50 years of age, all with calcified tendinitis of the rotator cuff. All had severe shoulder pain and limitation of motion. ESWT was done in one session with an extracorporeal shock wave lithotripter. RESULTS: After 24 hours, a fragmentation of calcification was achieved, and the patients had no pain and had entirely regained their joint movement. After 2 years of followup they were clinically and radiologically asymptomatic, and there were no adverse effects or other complications. CONCLUSION: With its good tolerance, safety, and clinical and radiologic response, ESWT should be considered as an alternative therapy in the treatment of chronic calcific tendinitis of the shoulder refractory to other therapies.     

Neuralgic amyotrophy

Neuralgic amyotrophy is an inflammatory condition of the nerves of the brachial plexus, the cause of which is unknown. Clinically, it presents as acute, severe pain in the shoulder girdle, followed by paresthesia and flaccid paralysis of selected muscles in the upper limb. In its initial stages, the disease may easily be misdiagnosed as a musculoskeletal disorder. We describe a 38-year-old man who experienced acute, severe pain in both shoulders, and hypoesthesia and paresthesias in the right arm without preceeding trauma. The condition was first diagnosed as capsulitis of the shoulder joint, and the patient was treated with naproxen with no effect. Eight days after onset of the disease, flaccid paralysis occurred abruptly in the right biceps muscle and in the left spinati and serratus anterior muscles. Sensibility and muscle strength improved gradually without treatment during the following months.     

Augmentation of the neuromuscular blocking effects of cisatracurium during desflurane, sevoflurane, isoflurane or total i.v. anaesthesia

We recently performed a laparoscopic cholecystectomy on three patients receiving preoperative oral anticoagulant therapy. The patients requiring anticoagulants for pre-existing cardiac conditions have the following risks at surgery: thromboembolism, hemorrhage, endocarditis, and cardiopulmonary dysfunction. In patients receiving anticoagulant therapy, one must thus maintain a balanced international normalized ratio of the prothrombin time to prevent thromboembolism or hemorrhage. Warfarin sodium was discontinued preoperatively in all patients. Heparin sodium was individualized according to each patient's risk of thromboembolism. As a result, these patients all underwent a laparoscopic cholecystectomy without complications. Attention was paid to achieve hemostasis in the operative field and the trocar inserted sites during the procedure. The administration of warfarin sodium was resumed on the first postoperative day in all patients. Restarting warfarin sodium early also helps to simplify postoperative management. A broad spectrum of antibiotic therapy was also used to reduce the risk of endocarditis. Each patient's cardiopulmonary function was carefully monitored. The minimal invasion experienced during a laparoscopic cholecystectomy may thus facilitate the management of gallstones in patients receiving systemic anticoagulation treatment based on the findings of this limited series.     

Superior sagittal sinus thrombosis presenting with subarachnoid hemorrhage in a patient with aplastic anemia

A 54-year-old female, who had been treated for aplastic anemia by metenolone acetate since 1981, developed a sudden unconsciousness in September 1995. On admission, she was drowny, CT showed a subarachnoid hemorrhage (SAH) in the right Sylvian fissure. Angiography demonstrated a complete occlusion of the superior sagittal sinus. The SAH was assumed to be originated from rupture of the right Sylvian vein, which was irregularly dilated on angiography. The dural sinus thrombosis was thought to be caused by a long term use of metenolone acetate, and it was discontinued. But her platelet count dropped due to the aggravation of aplastic anemia, and she developed repeated hemorrhagic infarction. An active anticoagulant therapy for the dural sinus thrombosis was thought to be inappropriate because she had the aplastic anemia and the hemorrhagic infarction recurred. We have successfully treated this case by mild anticoagulant therapy with nafamostat mesilate (Futhan).