Cases reported "Hemophilia A"

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1/9. Catheter-related deep venous thrombosis in children with hemophilia.

    central venous catheters (CVCs) are a common adjunct to hemophilia therapy, but the risk of CVC-related deep venous thrombosis (DVT) in hemophiliacs is not well defined. In a previous study, 13 patients with CVCs had no radiographic evidence of DVT. However, recent abstracts and case studies demonstrate that DVT does occur. Therefore, this study sought to determine the frequency of DVT in children with hemophilia and long-term CVCs and to correlate venographic findings with clinical features. All hemophilia patients with tunneled subclavian CVCs in place for 12 months or more were candidates for evaluation. patients were examined for physical signs of DVT and questioned about catheter dysfunction. Contrast venograms were obtained to identify DVT. Fifteen boys with severe hemophilia were evaluated, including 9 from the initially studied group of 13. Eight patients had evidence of DVT, 5 of whom previously had normal venograms. Five of 15 patients had clinical problems related to the CVC, all of whom had DVT. Four of 15 patients had suggestive physical signs; 3 had DVT. The mean duration of catheter placement for all patients was 57.5 months (range, 12-102 months). For patients with DVT, the mean duration was 66.6 /- 7.5 months, compared to 49.5 /- 7.2 months for patients without DVT (P =.06). No patient whose CVC was in place fewer than 48 months had an abnormal venogram. Many hemophilia patients with CVCs develop DVT of the upper venous system, and the risk increases with duration of catheter placement.
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2/9. Successful use of recombinant factor viia for hemostasis during total knee replacement in a severe hemophiliac with high-titer factor viii inhibitor.

    A 32-year-old male patient with severe factor viii (FVIII) deficiency had developed a high-titer FVIII inhibitor at age 13. Recurrent hemarthroses caused bony destruction in both knees, significantly impairing his ability to walk. knee examination revealed 20 degrees of varus, destruction of the medial joint line, and flexion contracture. Total knee arthroplasty was performed using recombinant factor viia (rFVIIa, NovoSeven) for hemostatic control. rFVIIa (85 microg/kg given intravenously over 3-5 minutes) was given just prior to surgery. The dose was repeated every 2 hours during and for the first 48 hours after surgery. When the tourniquet was removed, rFVIIa had not been infused for 1.5 hours, and significant hemorrhage was noted. The hemorrhage responded promptly to rFVIIa infusion. The infusion interval was extended to every 4 hours for an additional 48 hours, and subsequent doses were given every 6 hours until the patient returned to the clinic 2 days postdischarge. Hemoglobin levels dropped from 16.9 gm/dL on admission to 9.1 gm/dL at discharge. After 2 months, the patient returned to work. We recommend that tourniquet release be performed immediately after rFVIIa administration and that aggressive physical therapy be considered in the early postoperative period when rFVIIa infusions are frequent.
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3/9. Severe spontaneous intracranial haematoma in a hiv-negative 66-year-old mild haemophiliac. Complete recovery with the use of 1-month factor viii replacement.

    Intracranial haemorrhage is the most feared manifestation of haemophilia and is usually seen in severe forms. We report herein the case of a 66-year-old hiv-negative patient with mild haemophilia (factor viii: 7%) who presented with a spontaneous and massive intracranial haematoma causing hemiplegia and aphasia. We discuss the management of this peculiar situation emphasizing the need for rapid and adapted FVIII replacement. A complete recovery was obtained using this strategy combined with initial resuscitation measures and subsequent physical therapy.
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4/9. Synovectomy and continuous passive motion (CPM) in hemophiliac patients.

    Synovectomy of the knee is a procedure that has been used traditionally for the management of arthropathies due to intraarticular bleeding diatheses. Although open synovectomy controls recurrent bleeding problems, the success of this procedure has been limited frequently by disabling stiffness of the joint. In an attempt to improve on the results of open synovectomy, arthroscopic synovectomy was combined with continuous passive motion (CPM) in a prospective surgical and rehabilitation program. Five male patients aged 10 to 35 years, with chronic and/or recurrent hemarthroses due to hemophilia, underwent arthroscopic synovectomy of the knee. CPM was begun in the recovery room and continued for 5-7 days. Each patient had mildly restricted range of motion (ROM) immediately postoperatively as compared with preoperative measurements. Both active and passive ROM improved rapidly with CPM and physical therapy. By 3-6 months postoperatively, all patients except one had achieved an active ROM greater than the preoperative range, and all showed a significant reduction in documented bleeding episodes. The combination of arthroscopic techniques, which result in minimal extraarticular trauma, and immediate mobilization using CPM has been successful in maximizing the benefits and minimizing the complications of synovectomy in the patient with hemorrhagic arthropathy.
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5/9. A new modified bovine heterograft for vascular access in haemophiliacs.

    Because of poor venous access and failure of previous arteriovenous shunts due to thrombosis, two patients with severe coagulapathies had an arteriovenous fistula performed using a new chemically treated bovine carotid artery segment (Solcograft-P). To date, no complications have been noted after 16 and 17 months respectively. This treated heterograft maintains many of the physical characteristics observed in a normal bovine carotid artery. The theoretical advantages for using this heterograft in such patients are discussed.
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6/9. hemophilia a in a phenotypic female with normal male karyotype associated with a low factor xii level.

    hemophilia a was detected in a 40-year-old black Gabonese female prior to thoracic surgery for empyema. The diagnosis of mild hemophilia a was supported by the findings of low factor viii coagulant activity (VIII:C 4%), normal levels of factors VIII related antigen (VIIIR:Ag) and VIII von Willebrand (VIIIR:WF), without detectable circulating anticoagulant. Neither the patient nor her immediate relatives had past histories of abnormal bleeding. The physical features were phenotypically female with developed breasts, pubic hair and normal external genitalia: however, she had primary amenorrhea, a blind vagina with no uterus and her karyotype was 46,XY. These findings are consistent with the diagnosis of testicular feminization thereby explaining the apparent contradiction between the phenotype and the known six-linked inheritance of hemophilia a. In addition to factor viii deficiency a low level of factor xii (20%) was detected although it cannot be concluded whether the patient is truly factor xii deficient or whether she represents a low variant of the normal distribution.
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7/9. Hemophiliac knee: rehabilitation techniques.

    Hemophilia is an inherited bleeding disorder which produces its greatest morbidity in the musculoskeletal system. This article reviews current rehabilitation techniques for the hemophiliac knee--including restrictions of physical activity, external support, and corrective orthotic devices--reports our experience using aggressive strengthening techniques in 2 children, and compares the effectiveness of the 2 methods. It is the impression of the authors that strengthening, rather than immobilization, can bring about increased range of motion, increased strength, and decreased bleeding frequency.
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8/9. A 26-year-old hiv-positive hemophiliac with knee pain.

    The following case is presented to illustrate the roentgenographic and clinical findings of a condition of interest to the orthopaedic surgeon. Initial history, physical findings, and roentgenographic examinations are found on the first page. The final clinical and roentgenographic differential diagnoses are presented on the following pages.
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9/9. hypnosis principles and applications: an adjunct to health care.

    hypnosis has existed since the beginning of humankind, and is a part of everyday life. It is a valuable addition to the methods and techniques available to all health care providers, as well as a safe and uncomplicated method used to enhance patient health care. It is simply a state of complete physical and mental relaxation which produces an altered state of consciousness acceptable to suggestions. It is characterized by an increased ability to produce desirable changes in habit patterns, motivation, self-image, lifestyle, and personal health.
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