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  • 1
    Digitale Medien
    Digitale Medien
    Long-term follow-up after intertrochanteric varus osteotomy for haemophilic arthropathy of the hip (2002)
    Oxford UK : Blackwell Science Ltd
    Haemophilia 8 (2002), S. 0 
    Details
    ISSN: 1365-2516
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: In view of an increasing tendency in prosthetic management of haemophilic arthropathy, we intended to evaluate the efficacy of corrective osteotomy of the hip, specifically taking long-term clinical and radiographic results into consideration. Eleven hips affected by haemarthropathy in nine patients suffering from severe haemophilia A were treated with an intertrochanteric varus osteotomy. The average follow-up period was 15·4 years. The preoperative clinical score of the Advisory Committee of the World Federation of Haemophilia was 5·3 points (range 4–7) and the Pettersson score was 6·4 points (range 2–10). The average WFH score at follow-up had increased to 3·6 points. Seven hips showed clinical improvement, two hips showed a postoperative deterioration while a further two hips remained unchanged. The Petterson score increased to an average of 7·7 points. Here the radiographs of six patients indicated postoperative deterioration, three remained unaltered and two showed improvement. Our study cannot conclusively answer whether intertrochanteric varus osteotomy for haemophilic arthropathy of the hip is always a feasible alternative to joint arthroplasty. The decision for or against this procedure must be individually assessed and the patient must be thoroughly informed about the advantages and disadvantages of both procedures.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1046/j.1365-2516.2002.00593.x
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  • 2
    Digitale Medien
    Digitale Medien
    Haemophilic arthropathy of the hip in children – prognosis and long-term follow-up (2003)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 9 (2003), S. 0 
    Details
    ISSN: 1365-2516
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: Summary.  The aim of this study was to report on the long-term follow-up of haemophilic children with avascular femoral head necrosis and to determine whether radiographic findings at initial diagnosis have any prognostic value. Seven patients with avascular necrosis of the femoral head were clinically and radiographically observed over a period of 5–50 years. The average age of patients at first diagnosis was 7.1 years. At follow-up, three of seven patients claimed to have occasional mild pain in the affected hip, four of seven showed loss of range of motion in the hip joint and two of seven patients showed a limp. Only one patient was clinically completely inconspicuous. The radiographically measured caput–collum–diaphysis angle at follow-up was pathologic in four cases and in one case a lateral subluxation of the femoral head was found. There was marked deformation of the femoral head in three of seven cases and a further two hips showing slight incongruency. Owing to the small patient-number, a statement concerning the prognostic value of defined radiographic signs cannot be made. As expected, the more ‘risk signs’ radiographically found, the higher the likelihood that patients will suffer arthrosis at a later stage. We propose that a clear distinction between haemophilic arthropathy of the hip and Legg–Calvé–Perthes disease should be made. In cases where radiographic changes are also found in the vicinity of the acetabulum, it is indicative for haemophilic arthropathy.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1046/j.1365-2516.2003.00728.x
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  • 3
    Digitale Medien
    Digitale Medien
    Hamstring release and posterior capsulotomy for fixed knee flexion contracture in haemophiliacs (1999)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 5 (1999), S. 0 
    Details
    ISSN: 1365-2516
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: Between 1977 and 1981 hamstring release (n = 27) and posterior capsulotomy (n = 22) were performed on 27 haemophilic patients. The follow-up of 19 patients is documented for at least 5 years after the operation. The mean age was 30 years (15–40 years), the mean follow-up period was 12.5 years (5–20 years). Postoperatively 16 of 19 knee joints achieved immediate full extension, three patients showed 10° extension deficiency. In the long run the original good result could be demonstrated in just 11 patients. Using the Orthopedic Advisory Committee of the World Federation of Hemophilia (OAC) Scores there were three good, 12 satisfactory and four poor results. In minor forms of arthropathy a hamstring release with posterior capsulotomy is the only recommended operation. In severe forms we would actually recommend a combination with other procedures such as arthroscopic synovectomy or joint replacement.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1046/j.1365-2516.1999.0050s1025.x
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  • 4
    Digitale Medien
    Digitale Medien
    Long-term surveillance for human anti-murine antibodies of a group of haemophiliacs treated only with immunoaffinity-purified FVIII concentrates (1995)
    Oxford, UK : Blackwell Publishing Ltd
    Haemophilia 1 (1995), S. 0 
    Details
    ISSN: 1365-2516
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: An immunoaffinity purified, solvent/detergent virally inactivated factor VIII (FVIII) concentrate (Hemofil® M) has been in clinical use in persons with haemophilia A since March 1987 and under clinical trial prior to licencing in the USA, Europe and Japan.The specification set and consistently met for the monoclonal antibody (mAb) content of the final product is 0.1 ng/IU FVIII. This level was considered non-immunogenic, based upon animal studies, recommendations of the European PHarmacopoeia with ovalbumin contamination of influenza vaccine and also the experience during clinical research studies using aAb for diagnostic or other clinical purpose as reported in the medical literature.This research communication documents the surveillance for human anti-murine antibodies (HAMA) in 13 patients with serve haemophilia A. These patients have been treated with a large amount of product (mean 14,026 IU), numerous different lots of Hemofil M (total 45; mean 17) over a substantial period of time (mean 28 months).The data generated from this group of 13 patients before and during therapeutic Hemofil M administration failed to show the development of any HAMA response at any timepoint. Therefore Hemofil M prepared by mAb resin immunoaffinity column chromatography under the currently set specifications for mAb contaminant of 0.1 ng/IU FVIII or less is a safe therapeutic agent in the management and prevention of bleeding episodes in persons with haemophilia A.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1111/j.1365-2516.1995.tb00049.x
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  • 5
    Digitale Medien
    Digitale Medien
    Long-term follow-up after osteotomy for haemophilic arthropathy of the knee (2003)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 9 (2003), S. 0 
    Details
    ISSN: 1365-2516
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: Summary.  In this study the long-term value of corrective osteotomy around the knee was evaluated by means of clinical and radiographic parameters. Between 1974 and 1984 we performed 52 corrective osteotomies in the vicinity of the knee on patients affected by haemophilic arthropathy. Forty-two patients (45 osteotomies) were adequately followed-up at an average 11.6 years postoperatively. Using the clinical score of the Advisory Committee of the World Federation of Haemophilia, 38 patients showed a postoperative improvement, five remained clinically unchanged and two showed deterioration. Range of motion of the knee joint did not significantly improve postoperatively. The radiographic Pettersson score showed only a marginal decrease by an average 0.003 points at the time of follow-up. Only one patient needed subsequent joint replacement of both knees, on the left side 13 years after osteotomy and on the right side 8 years after osteotomy.Even in cases of marked radiographic joint destruction, corrective osteotomy shows acceptable long-term clinical results, underlining the feasibility of this management option in the treatment of haemophilic arthropathy of the knee. Although moderate cartilage degenerations in the femoropatellar complex and in the contralateral compartment can be tolerated, this therapy should primarily be contemplated for those patients where damage is unicompartmental and a corresponding axial deviation is found. Particularly the younger patient can benefit from this treatment option in that joint replacement may possibly wholly be avoided or at least postponed to a later stage of life.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1046/j.1365-2516.2003.00705.x
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  • 6
    Digitale Medien
    Digitale Medien
    Clinical and radiographic scores in haemophilic arthropathies: how well do these correlate to subjective pain status and daily activities? (2002)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 8 (2002), S. 0 
    Details
    ISSN: 1365-2516
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: Summary. Haemophilic patients who reached adulthood before the establishment of prophylactic treatment frequently show multiple and substantial arthropathies. The aim of this study was to determine to what extent haemophiliac's subjective impairment due to arthropathies correlates with objective clinical and radiographic parameters. By means of a questionnaire and a visual analogue scale, we consulted 79 haemophiliacs concerning their joint-pain status, how these were treated and to what extent their daily activities had been affected. Using a scoring system suggested by the Advisory Committee of the World Federation of Haemophilia, clinical evaluation was performed. Radiographs of 60 patients were assessed by means of the Petterson scale. The results were statistically compared. We found a significant correlation between pain intensity and clinical pathology as well as between pain intensity and radiographic joint damage for both knees and for the right ankle. The number of painful joints correlated well with the number of clinically/radiographically affected joints. The more pronounced the objective damage to joints, the more frequently patients claimed to have constant pain, depressive episodes and a dependency on pain-relieving medication. The more pronounced the objectively assessed damage to the knee and ankle joint, the higher the likelihood that the patient suffers from severe joint pain and reduction of activity. Treatment of painful symptoms from arthropathies is often insufficient. Scores and questionnaires may help to define the haemophiliacs pain status more clearly, thereby offering a possibility of assessment and long-term observation.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1046/j.1365-2516.2002.00680.x
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  • 7
    Digitale Medien
    Digitale Medien
    Pain status of patients with severe haemophilic arthropathy (2001)
    Oxford UK : Blackwell Science Ltd
    Haemophilia 7 (2001), S. 0 
    Details
    ISSN: 1365-2516
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: Patients with severe haemophilia A growing up before the establishment of prophylactic treatment frequently developed significant haemarthropathies. The goal of the following study was to clarify the role of haemarthropathic pain for haemophilic patients. Furthermore, we aimed to determine to what degree daily activities are influenced by the impairment and which therapeutic modalities are used in pain management. Using a questionnaire we consulted 71 haemophiliacs concerning their complaints and how they were treated in 1999 (average age 43 years; range 21–63 years). The pain in the large joints and spine and the effect of specific treatment was estimated by a visual analogue scale. On average, there were four joints with major pain and 0.5 with minor pain. The most frequent sources of pain were the ankle joints (45%), followed by the knee (39%), spine (14%) and elbow (7%). Fifty percent of all patients complained of pain throughout the day if no treatment was applied. In 29% of patients, pain persisted after application of factor VIII (FVIII), while 12% claimed that pain still remained after use of FVIII and painkillers. Restriction in activities of daily life was reported by 89% of the group and 85% reported on an impact of pain on their mood. Patients primarily used FVIII to decrease pain, followed in frequency by use of anti-inflammatory drugs, orthopaedic footwear, liniments and bandages. Haemophilic patients with haemarthropathy are chronic pain patients. By means of the questionnaire, it is possible to reveal the ‘silent’ sufferers. Sufficient pain treatment is essential so as to increase the patient’s quality of life and avoid inadvertent abnormal postures possibly resulting in increased loading of joints and subsequent bleeding episodes.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1046/j.1365-2516.2001.00540.x
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  • 8
    Digitale Medien
    Digitale Medien
    Efficacy of a sucrose-formulated recombinant factor VIII used for 22 surgical procedures in patients with severe haemophilia A (2000)
    Oxford UK : Blackwell Science Ltd
    Haemophilia 6 (2000), S. 0 
    Details
    ISSN: 1365-2516
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: A sucrose-formulated recombinant FVIII (rFVIII-SF) was investigated under clinical trial conditions during surgical procedures in previously treated patients (PTPs). Fifteen PTPs with severe haemophilia A (FVIII ≤ 1%) underwent 22 surgical procedures. The procedures performed cover a spectrum from minor to major surgery. Haemostatic outcome was assessed by the investigators to be excellent in 16 procedures and good in the remaining six procedures. It is concluded that rFVIII-SF is efficacious and safe in severe haemophilia A patients undergoing minor or major surgery.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1046/j.1365-2516.2000.00432.x
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  • 9
    Digitale Medien
    Digitale Medien
    Control of the synovium in haemophilia (1998)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 4 (1998), S. 0 
    Details
    ISSN: 1365-2516
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: Summary. Adequate factor substitution is a prerequisite for successful conservative or operative treatment of haemophilic synovitis. Subsequent prophylactic factor substitution can prevent further joint problems in the future. Orthopaedic treatment regimes are dependent on the classification of the synovitis and the stage of the arthropathy. Synovitis can be classified into acute and chronic forms. The arthropathy is classified according to Arnold and Hilgartner. When the synovitis has already become chronic, early arthroscopic synovectomy is recommended in order to prevent the otherwise inexorable progress of the arthropathy. Failure to treat in the early phase of the pathology causes problems in the correction of further stages. The progression of the joint disease can be shown both clincally and radiologically as the arthropathy develops, and influences the treatment.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1046/j.1365-2516.1998.440511.x
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  • 10
    Digitale Medien
    Digitale Medien
    Pharmacokinetic properties of two factor VIII concentrates with single and double viral inactivation (1996)
    Oxford, UK : Blackwell Publishing Ltd
    Haemophilia 2 (1996), S. 0 
    Details
    ISSN: 1365-2516
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: In order to assess whether addition of a modified pasteurization step to the manufacture of a solvent/detergent-treated factor VIII concentrate of high purity has affected its properties, three sets of pharmacokinetic studies were performed. In an open, controlled study with seven patients and a double-blind cross-over study with nine patients, the pharmacokinetic parameters of the double virus-inactivated preparation were compared with those of a mono-inactivated product, using a one-stage clotting assay. In a longitudinal study comparison was made between baseline and 8 months after treatment with the double virus-inactivated product in six patients, using three different factor VIII assays. There were no differences in recovery, clearance or half-life between the single- and double-inactivated products. After 8 months of treatment, the half-life and mean residence time were longer with the one-stage clotting assay, whereas the chromogenic assay showed a lower recovery, a faster cleareance and smaller area under the curve with the double virus-inactivated product. All differences were small and none was consistent in all three methods. In conclusion, the double virus inactivation does not appear to have affected the pharmacokinetic characteristics of this factor VIII preparation.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1111/j.1365-2516.1996.tb00160.x
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