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Biomedical subjects

U Martinowitz

Publications and source records attributed to U Martinowitz.

At least 37 records · Page 2Linked to original sources

Orthopaedic outcome of total knee replacement in haemophilia A.

A consecutive series of 16 patients with classical Haemophilia underwent 21 total knee replacements between 1989 and 1997 for haemophilic arthropathy. The patients received Factor VIII replacement therapy via continuous infusion, and fibrin glue was used to facilitate haemostasis. Three different types of prostheses were used. A follow-up evaluation was undertaken between 2 and 10 years after the operation (mean 5.6 years) and two patients with infection were excluded. Knee scores averaged 77.5 (pre-operative 24. 1) and functional scores averaged 84.4 (preoperative 23.2). There were no cases with aseptic loosening of the prosthesis. Complications included one early deep infection controlled by conversion of the TKR into an arthrodesis, one case of late septic loosening that had to be re-operated upon, one case of patellar dislocation, two cases of stiff knee (fibro-arthrosis) that required manipulation under anaesthesia, one postoperative hepatitis, one superficial infection treated by incision and drainage and four febrile patients with no clear source of infection, who responded to antibiotics alone. In conclusion, TKR offers haemophilic patients a long-lasting improvement of their quality of life and we therefore advocate its use with the appropriate indications.

Adult↗

Elbow joint, crutches and locomotion: special reference to persons with haemophilia.

A 52-year-old, trans-femoral amputee with haemophilia was hospitalized because of ambulatory problems arising from the osteo-arthropathic involvement of other major articulations. Reduced function in the upper limbs, caused by the effects of recurrent haemarthroses, resulted in additional problems concerning the usage of auxiliary ambulatory aids. The advantages and disadvantages of traditional and experimental crutches highlight the functional problems of ambulation in persons with concomitant upper limb pathologies.

Amputees↗

Drug-induced and traumatic nail problems in the haemophilias.

Many persons with haemophilia suffer from HIV and receive highly active antiretroviral therapy. Three patients received indinavir and required surgery due to ingrown toenails. Two patients suffered from a traumatic subungual haematoma. The treatment protocol is described whereby the pressure exerted onto the germinal layer and the nail bed is relieved in order to alleviate pain and nail matrix damage.

Adult↗

Treatment of bleeding episodes in patients with hemophilia and an inhibitor: comparison of two treatment protocols with recombinant activated factor VII.

Six hemophilia A patients with inhibitors were treated with a continuous infusion of recombinant activated factor VII (rFVIIa) for various bleeding episodes. Bleeding episodes (n = 101) were treated according to a 12 h regular dose protocol or a shortened (6 h) augmented dose protocol. Patient response to therapy was assessed by symptomatic improvement within predefined timeframes. Although patient number was limited, both protocols appeared similar with respect to the proportion of patients responding to therapy; however, the augmented dose protocol appeared to be superior to the regular dose protocol with shorter response time, shorter duration of therapy and possibly lower rFVIIa requirements. Further studies are needed to define the efficacy of rFVIIa augmented dose administered as continuous infusion in treating hemophilia patients with inhibitors during major bleeding episodes, trauma and surgery.

Adolescent↗

Anaerobic power and muscle strength in young hemophilia patients.

PURPOSE: The purpose of this study was to evaluate muscle strength and anaerobic power in young boys with hemophilia compared with healthy boys. METHODS: Thirteen boys with severe hemophilia (H) (mean (+/- SD) age = 12.0 +/- 3.17 yr) and 16 control (C) boys (age = 11.9 +/- 2.8 yr) performed elbow and knee flexion and extension on the Biodex System II dynamometer at two angular velocities. They also performed a Wingate Anaerobic Test (WAnT) for the legs and for the arms. All H subjects received prophylactic factor VIII treatment in the 24 h pretesting, and no test was performed in the presence of hemorrhage. RESULTS: C were consistently stronger than H in all dynamic strength measures (e.g., elbow flexors: 0.47 +/- 0.15 vs 0.36 +/- 0.08 N x m x kg(-1) for C and H, respectively, P < 0.05). Anaerobic mean power was also higher in C compared with H in both upper and lower extremities (arms: 3.08 +/- 0.99 vs 2.22 +/- 0.46 W x kg(-1) for C and H, respectively; legs: 6.94 +/- 1.62 vs 5.54 +/- 1.03 W x kg(-1) for C and H, respectively, P < 0.05). Upper and lower extremity strength, as well as anaerobic power, increased with age in C but not in H. By using the Godin Leisure-Time Exercise Questionnaire, H were found to be much less active, especially in intense activities, compared with C. CONCLUSION: Children and adolescents with hemophilia are characterized by lower muscle strength and anaerobic power compared with age-matched controls. This may be related to their lower leisure-time activity.

Adolescent↗

Factor V Leiden and antiphospholipid antibodies are significant risk factors for ischemic stroke in children.

BACKGROUND AND PURPOSE: The association between ischemic childhood stroke and thrombophilia has been debated. We studied the prevalence of thrombophilia risk factors in 65 unrelated children with ischemic stroke compared with 145 control subjects. METHODS: Patients and control subjects were tested for antithrombin protein C and protein S deficiencies, the presence of antiphospholipid antibodies (APLA), factor V Leiden (FVL), G20210A polymorphism of factor II gene (FII G20210A), and C677T polymorphism of 5,10-methylenetetrahydrofolate reductase gene (C677T MTHFR). RESULTS: Of 65 children, 7 had a stroke in the neonatal/perinatal period and therefore were analyzed separately. Thirty-one of the remaining 58 patients with pediatric stroke (53.4%) were found to have at least 1 thrombophilia marker compared with only 25.5% of control subjects. None of the patients or control subjects had protein S or antithrombin III deficiency. The prevalence of protein C deficiency was higher among pediatric stroke patients than among control subjects, but the difference was not statistically significant (OR=7, 95% CI 0.75 to 65.1). Heterozygous FII G20210A and homozygous MTHFR 677T were not associated with an increased risk for stroke (OR=1.29, 95% CI 0.2 to 8.2; and OR=1.06, 95% CI 0.4 to 2.7, respectively). In contrast, the presence of APLA was associated with a >6-fold risk of stroke (OR=6. 08, 95% CI 1.5 to 24.3), and the heterozygosity for FVL increased the risk of stroke by almost 5-fold (OR=4.82, 95% CI 1.4 to 16.5). Five patients with pediatric stroke had a combination of > or =2 thrombophilia markers, whereas none of the control subjects had a combination of the markers. Most of the patients with neonatal/perinatal stroke were found to have at least 1 thrombophilia marker. CONCLUSIONS: These data suggest that the prevalence of thrombophilia markers is increased in children with stroke compared with control subjects and, specifically, that FVL and APLA contribute significantly to stroke occurrence.

3' Untranslated Regions↗

Extensive venous and arterial thrombosis associated with an inhibitor to activated protein C.

Activated protein C resistance (APCR) in the absence of alterations in the factor V gene has been observed during pregnancy, in patients on oral contraceptives, in the presence of antiphospholipid antibodies, and in patients with ischemic stroke. We report a 49-year-old woman with recurrent major venous and arterial thromboses who displayed pronounced APCR, yet no changes in the activated protein C (APC) cleavage sites of factor V. The APCR values determined by four different assays were similar to those obtained in plasma from a homozygote for factor V Q506. Addition of IgG isolated from the patient's serum to normal plasma lowered the APCR ratio from 2.4 to 1.6. Incubation of patient's IgG with normal APC resulted in a profound change in the mobility of APC in crossed immunoelectrophoresis. APC was also shown to bind to patient's IgG immobilized on a protein A agarose column. Factor Va inactivation by APC was inhibited by patient's IgG, but not by control IgG in the presence or absence of either phospholipids or protein S. These results provide evidence for the existence of an acquired antibody against APC in the patient's plasma, which gave rise to the APCR phenotype and was probably responsible for the major thrombotic events. We suggest that acquired APCR due to anti-APC antibodies be considered a potential cause for severe venous and arterial thromboses.

Autoantibodies↗

Dental extractions in patients maintained on continued oral anticoagulant: comparison of local hemostatic modalities.

OBJECTIVE: The purpose of this study was to evaluate postoperative bleeding in patients treated with oral anticoagulant drugs who underwent dental extractions without interruption of the treatment and to compare the effect of 3 different hemostatic modalities. STUDY DESIGN: A total of 150 patients who underwent dental extractions were divided into 3 groups. Local hemostasis was carried out as follows: group 1 (119 extractions), with gelatin sponge and sutures; group 2 (117 extractions), with gelatin sponge, sutures, and mouthwash with tranexamic acid; group 3 (123 extractions), with fibrin glue, gelatin sponge, and sutures. RESULTS: Of 150 patients, 13 (8.6%) presented with postoperative bleeding: 3 patients from group 1, 6 patients from group 2, and 4 patients from group 3. CONCLUSIONS: Dental extractions can be performed without interruption in patients treated with oral anticoagulant. Local hemostasis with gelatin sponge and sutures is sufficient.

Administration, Oral↗

Expanding portal haematomata as a complication of knee arthroscopies in persons with haemophilia.

Recurrent haemarthroses stimulate the hypertrophy of synovial tissues that if left in situ will eventually cause joint destruction. Synovectomies have been the cornerstone of joint preservation and a number of different methods exist. We report two patients who suffered complications after an arthroscopic procedure. No previous complications of this nature have been reported in the literature.

Adult↗

The role of orthoses in the management of elbow joints in persons with haemophilia.

The elbow joint is extremely prone to haemarthroses, and when these occur the elbow requires support and rest. Supporting the limb with a simple collar-and-cuff sling, a triangular bandage either with or without a shoulder immobilization device will provide an analgesic effect without adding an unnecessary heavy orthosis. As the swelling subsides and the pain lessens a dynamic orthosis aids in the support and compression. When considering functional branching of the elbow joints there are a number of basic principles to be followed. One requires to consider problems of orthosis suspension, two dimensional motion and stabilization in the functional range of motion. This article provides the rationale for the special needs of persons with haemophilia who suffer from involvement of their elbow joints.

Contracture↗

Characteristics of septic arthritis in human immunodeficiency virus-infected haemophiliacs versus other risk groups.

The cases are presented of four haemophiliacs infected with human immunodeficiency virus (HIV) and with septic arthritis among the 340 patients followed at our centre. The data of these cases and 39 additional HIV-infected haemophiliacs with septic arthritis, identified in a literature search, are reviewed. The spectrum of bacterial pathogens is limited and somewhat different from that in other risk groups. The localization is exclusively to joints affected by haemophilic arthropathy. The laboratory picture is characterized by the absence of peripheral leucocytosis, varying CD4-helper cell counts, a high erythrocyte sedimentation rate and fever. The clinical picture mimics that of haemarthrosis, often causing a delay in diagnosis. Treatment with systemic antibiotics is often sufficient, obviating the need for arthrotomy and open drainage. Prognosis related to the joint function is relatively good, but poor when related to the medium- to long-term survival of the patient.

Adult↗

Single and combined prothrombotic factors in patients with idiopathic venous thromboembolism: prevalence and risk assessment.

The inherited thrombophilias--deficiencies of protein C, protein S, and antithrombin III--and the prothrombotic polymorphisms factor V G1691A and factor II G20210A predispose patients toward venous thromboembolism (VTE). The aim of this study was to determine the prevalence of single and combined prothrombotic factors in patients with idiopathic VTE and to estimate the associated risks. The study group consisted of 162 patients referred for work-up of thrombophilia after documented VTE. The controls were 336 consecutively admitted patients. In all subjects factor V G1691A, factor II G20210A, and methylenetetrahydrofolate reductase (MTHFR) C677T were analyzed by specific polymerase chain reactions and restriction enzymes. Activities of antithrombin III and protein C, free protein S antigen, and lupus anticoagulant were determined in a subset of 109 patients who were not receiving oral anticoagulants. The prevalences of heterozygotes and homozygotes for factor V G1691A and factor II G20210A among patients and controls were 40.1% versus 3.9% and 18.5% versus 5.4%, respectively (P=0.0001). The prevalence of homozygotes for MTHFR C677T in patients was 22.8% and in controls, 14.3% (P=0.025). Heterozygous and homozygous factor V G1691A, factor II G20210A, and homozygous MTHFR C677T were found to be independent risk factors for VTE, with odds ratios of 16.3, 3.6, and 2.1, respectively. Two or more polymorphisms were detected in 27 of 162 patients (16.7%) and in 3 of 336 controls (0.9%). Logistic regression analysis disclosed odds ratios of 58.6 (confidence interval [CI], 22.1 to 155.2) for joint occurrence of factor V and factor II polymorphisms, of 35.0 (CI, 14.5 to 84.7) for factor V and MTHFR polymorphisms, and of 7.7 (CI, 3.0 to 19.6) for factor II and MTHFR polymorphisms. Among 109 patients in whom a complete thrombophilic work-up was performed, 74% had at least 1 underlying defect. These data indicate that in most patients referred for evaluation of thrombophilia due to idiopathic VTE, 1 or more underlying genetic predispositions were discernible. The presence of >1 of the prothrombotic polymorphisms was associated with a substantial risk of VTE.

Adult↗

The use of fibrin tissue adhesive to reduce blood loss and the need for blood transfusion after total knee arthroplasty. A prospective, randomized, multicenter study.

BACKGROUND: Total knee arthroplasty is associated with major postoperative blood loss of approximately 800 to 1200 milliliters, and blood transfusion is frequently required. With the increased concern about the risks of blood transfusion, various methods of blood conservation in orthopaedic surgery have been studied. The most appropriate solution, however, is to reduce the loss of blood during and after an operation. The present prospective, controlled, randomized study was designed to evaluate the hemostatic efficacy of the use of fibrin tissue adhesive in patients managed with total knee arthroplasty. METHODS: Fifty-eight patients who were scheduled to have a total knee arthroplasty were randomly divided into two groups: a control group, in which the standard means of hemostasis were applied, and a treatment group, in which the standard means to control local bleeding were applied and a fibrin tissue adhesive was sprayed on the internal aspects of the operative field before skin closure. All operations were performed in a bloodless field with use of a pneumatic tourniquet. All patients received low-molecular-weight heparin as thromboprophylaxis twelve hours before the operation and every twelve hours postoperatively. Blood loss during the operation was evaluated by measuring the volume in the suction apparatus and by estimating the amount of lost blood in the swabs at the end of the operation. The apparent postoperative lost blood was determined by measuring the volume in the suction-drain bottles. All blood transfusions were recorded. RESULTS: The mean apparent postoperative blood loss (and standard deviation) in the fibrin-tissue-adhesive group was 360+/-287.7 milliliters compared with 878+/-403.0 milliliters in the control group, with a mean difference of 518 milliliters (p<0.001). The decrease in the level of hemoglobin was 25+/-10 grams per liter in the treatment group compared with 37+/-12 grams per liter in the control group (p<0.001). Sixteen patients (55 percent) in the control group required a blood transfusion and eight (28 percent) required two units of blood, whereas only five (17 percent) of the patients in the fibrin-tissue-adhesive group required a blood transfusion and only one (3 percent) required two units (p = 0.004). The number of adverse events was comparable between the two groups. None of the adverse events were considered to be related to the use of fibrin tissue adhesive. One death, which was due to massive pulmonary embolism, was reported in the control group. No seroconversion was reported at three and six months after the operation. CONCLUSION: The use of fibrin tissue adhesive in total knee arthroplasty seems to be an effective and safe means with which to reduce blood loss and blood-transfusion requirements. Furthermore, the importance of these findings was enhanced by a significant reduction in blood loss, in the postoperative decrease in the level of hemoglobin, and in blood-transfusion requirements despite preoperative thromboprophylaxis with low-molecular-weight heparin.

Aged↗

Safety and efficacy of continuous infusion of a combined factor VIII-von Willebrand factor (vWF) concentrate (Haemate-P) in patients with von Willebrand disease.

We studied the safety and efficacy of treatment with continuous infusion of a von Willebrand factor (vWF) concentrate Haemate-P in patients with von Willebrand disease (vWD). Three patients with mild and 5 patients with severe forms of vWD, were treated with continuous infusion of Haemate-P by minipump. The indications for treatment were: to prevent bleeding during 9 surgical procedures or 1 vaginal delivery in 6 patients and to treat 2 bleeding episodes in 2 patients. The patients were monitored daily for factor VIII (FVIII:C) and ristocetin cofactor (vWF: RCo) levels and the infusion rate was adjusted to maintain the desired therapeutic level of vWF:RCo. The treatment was effective in preventing surgical bleeding and controlling bleeding episodes. All factor VIII:C and most of the vWF:RCo levels measured during the study period were above the target therapeutic levels. A significant decrease in clearance of FVIII:C and vWF:RCo was observed over the treatment period. Haemate-P consumption averaged 24.3+/-7.9 vWF:RCo U/kg/day which is approximately half the expected dose had intermittent bolus injections been used. We suggest that continuous Haemate-P infusion is superior to intermittent bolus injections for the treatment of vWD patients by virtue of its efficiency, simplicity and considerable savings.

Adolescent↗

Low cost locally prepared fibrin glue for clinical applications: reported of 145 cases. Committee of Bangkok International Hemophilia Training Center.

Fibrin glue (FG) is one of the blood products known to be very useful for local hemostatic measure and as a medically valuable tool for adhesion, sealing, anastomosis, repair microvascular and nerve grafts in medical and surgical procedures. Before 1996, FG was used to a limited extent in Thailand due to the high cost. Technology for locally prepared FG was transferred to Bangkok International Hemophilia Training Center of the World Federation of Hemophilia (IHTC-WFH) in July 1996 by Prof. Uri Martinowitz and the late Prof. Henri Horoszowski. Since then FG has been widely used and proved to be very useful in Thailand. This paper reports 145 cases using low cost locally prepared FG at Ramathibodi Hospital during November 1996 to December 1997. A total of 145 cases with age range from 5 months to 73 years, which included 55 pediatrics and 90 adults, 100 males and 45 females. The amount of FG used was 1-80 ml per case. Clinical procedures included dental surgery (46), open heart surgery (35), ENT (28), orthopedic (13) including 2-3 joint correction in one session in 2 hemophiliacs, neurology (11), plastic repair (7), liver (2) and severe bleeding in dengue hemorrhagic fever (3). Forty-seven cases had hemostatic disorders. The result of local hemostatic, adhesive and sealant effect of FG was satisfactory with no complications. In open heart surgery, the amount of content in chest drain decreased and none required reopen-surgery to stop bleeding. Dental surgery was performed in 43 patients with bleeding disorders i.e. hemophilia, idiopathic thrombocytopenic purpura, leukemia, severe thrombocytopenia, patients on anticoagulant, etc. Only 3 cases (7%) required blood component compared to all of the 50 no-FG controlled cases (100%) that required blood component therapy. FG has proved to be very useful in many aspects i.e. minimizing blood product usage, decreasing medical workload, reducing medical cost and increasing patients' convenience and satisfaction in particular.

Adolescent↗

Cone and platelet analyser (CPA): a new test for the prediction of bleeding among thrombocytopenic patients.

The risk of bleeding among thrombocytopenic patients was evaluated using our new cone and platelet analyser (CPA) test. Using this test, adherence of platelets was quantitated on extracellular matrix and expressed as percent of surface coverage (SC) and the average size (AS) of aggregates. 42 thrombocytopenic patients with ITP (n=23), post chemotherapy (n= 12) and others (n= 7) were tested over a total of 82 visits. On each visit, complete blood count and CPA tests were performed and patients were evaluated for evidence of bleeding (found in 40 visits). Bleeding patients had significantly lower platelet counts (27.4 +/- 22.0 v 47.1 +/- 21.0 x 10(9)/l), lower haematocrit values (30.2 +/- 8.1 v 35.2 +/- 6.6%), lower MPV (6.83 +/-1.89 v 8.98 +/- 1.13 fl), and lower SC (4.87 +/- 3.95 v 10.33 +/-5.48%) and AS (33.99 +/- 14.94 v 52.9 +/- 24.34 microm2). Univariate analysis yielded platelet count < or =20.0 x 10(9)/l, MPV < or =8 fl, haematocrit <35%, SC <5%, AS< or =40 microm2 as significantly associated with bleeding, whereas only MPV and SC were associated with bleeding (OR 6.95, CI 2.25-21.46 and OR 4.27, CI 1.29-14.16, respectively) by multivariate analysis. When taken together, 21/22 of patients (95%) with both low SC (<5%) and low MPV (<8.0 fl) had bleeding symptoms, whereas only 9/43 (21%) patients with both these parameters above these values experienced bleeding symptoms. We conclude that the CPA test and the parameter SC (<5%) together with MPV (< or =8 fl) might be used as independent predictors of bleeding in the management of thrombocytopenic patients.

Adolescent↗

The management of a person with haemophilia who has a fixed flexed hip and intractable pain.

The clinical picture of a fixed flexed hip associated with pain in a person with haemophilia is suggestive of a haemorrhage in that area. Sonography facilitates differentiation between a haemarthrosis, intraperitoneal haemorrhage, subperiosteal bleed, a bleed into the soft tissue around the hip joint or a psoas haematoma. All these aforementioned causes may result in the same clinical presentation. Two cases are described in which coxhaemarthrosis resulted in a flexion contracture of the joint associated with severe intractable pain. Narcotic drugs failed to alleviate the severe pain. Joint aspiration produced dramatic pain relief and early joint rehabilitation.

Adult↗