Cases reported "Hemophilia A"

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1/9. Bell's palsy during interferon therapy for chronic hepatitis c infection in patients with haemorrhagic disorders.

    Two adult patients with life-long severe haemorrhagic disorders commenced on interferon-alpha2b therapy for chronic hepatitis c infection. Both developed Bell's palsy several weeks after commencing therapy, They were started on steroids and, in addition, the first patient discontinued interferon-alpha2b therapy while the second patient elected to continue with therapy. In both cases facial paralysis improved over the ensuing weeks. Bell's palsy is often idiopathic but has been reported. in association with herpesviruses. It is not a recognised complication of chronic hepatitis b or C infection, or interferon-alpha2b therapy. However, the interferons are associated with numerous adverse reactions including various neuropsychiatric manifestations and neurological syndromes. There are several reports of nerve palsies, including optic tract neuropathy, occurring during interferon therapy, and immune-based mechanisms are thought to play a role in the aetiopathogenesis. No reports of Bell's palsy in association with interferon therapy were identified in our literature search, although one possible case has been reported to the Committee of safety in medicine. Although Bell's palsy in our patients may have occurred by chance, a neuropathic effect of interferon-alpha2b on the facial nerve cannot be excluded and we urge physicians using interferons to be aware of this potential side-effect.
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2/9. Use of intraligamentary anesthesia in a patient with severe hemophilia and factor viii inhibitor.

    This article reviews the therapeutic problems that arise in the dental management of hemophilia patients with inhibitor and suggests an anesthesia technique that may eliminate the need for factor replacement during restorative procedures. Intraligamentary anesthesia was used during restorative procedures that were performed throughout an 8-year period on a patient with factor viii inhibitor. Multiple restorative procedures were accomplished without the use of replacement factor and with no postoperative bleeding. This technique, in consultation with the patient's physician, may be performed by most general practitioners in their private offices.
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3/9. Effective and safe use of recombinant factor viia (NovoSeven) in elderly mild haemophilia A patients with high-titre antibodies against factor viii.

    Three patients with mild haemophilia A who developed high-titre antibodies against factor viii at high age are reported. These patients had only a limited number of exposure days of FVIII concentrates in the past. The patients had to undergo surgery or presented with recurrent bleeding episodes. Treatment with recombinant FVIIa (rFVIIa) was effective and safe. Despite the high age and the presence of coronary heart disease in one of the patients, no adverse events or thrombotic complications occurred. These cases illustrate that the physician should always be alert on the development of inhibitors, also in elderly patients with mild haemophilia, in whom FVIII inhibitors had never been detectable before and that treatment with rFVIIa was effective and well-tolerated.
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4/9. Intraspinal hematomas in hemophilia.

    We report herein a case of a intraspinal hematoma in a 9-year-old boy with factor ix deficiency. Replacement of factor ix resulted in resolution of symptoms. The most frequent presentations of intraspinal hematomas are neck or back pain, paresis, sensory impairment, and urinary retention. Intraspinal hematomas may have devastating sequelae, including hemiplegia and quadriplegia. The occurrence or development of sequelae are related to the length of time between onset of symptoms and factor replacement. Whenever the physician suspects intraspinal hematoma, immediate replacement should be given to obtain levels of 80-100% prior to any imaging studies. Factor levels should be maintained at 30-50% for 10-14 days while the patient is monitored closely with serial neurological examinations. Most patients respond to factor replacement, but laminectomy should be considered for intractable or progressive cases.
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5/9. Bruising and hemophilia: accident or child abuse?

    Recognition and prompt reporting of suspect child abuse or neglect is necessary to institute programs aimed at preventing further neglect, re-injury or possible death. The families of children with chronic medical conditions, such as hemophilia, may be affected by economic and emotional stresses which may be expressed as abuse or neglect. Because the manifestations of even slight trauma are so common in children with bleeding problems, the physician may not routinely inquire about the cause of injury, delays in seeking medical attention, or accident prevention efforts in the home. Early referral of children with hemophilia to a multidisciplinary team providing medical, psychological, and social care is recommended.
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6/9. Care of the young hemophiliac. New socioeconomic demands and the changing patient-physician relationship.

    Over the past two decades, greater knowledge about hemophilia and vastly improved treatment have led to a veritable revolution in the care of this crippling disease. In prospect is an era in which both short- and long-term sequelae may be avoided altogether. Much of the progress has been effected through novel methods of care delivery in which the relationship of the physician and his patient begins to resemble more a partnership between equals than the traditional dependency of the chronically handicapped on his doctor. Yet with, and because of, these changes ethical and legal aspects are inevitably introduced, some of which are discussed in the article. These deal with issues of public policy, distributive justice, avoidance of harm, risk imposition and risk-taking, confidentiality, and paternalism. When applicable, the discussion surrounds a prototypical case which illustrates the real dilemmas which treaters and patients may have experienced or may encounter. Following each issue is a discussion intended to show how the ethical and legal dimensions may be analyzed in a way which allows certain justifiable decisions. These analyses are themselves based upon ethical premises which are commonly accepted, legal precedent, or both. While some of these considerations are relevant to the older hemophiliac as well, most deal with topics affecting those most likely to benefit from the advances and to contend with the challenges of this new era of hemophilia care: the young hemophiliac and his physician.
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7/9. Rectus sheath hematomas in hematological disorders.

    Rectus Sheath Hematomas are an uncommon cause of abdominal pain. There are many etiologies for this entity. The physician must be aware of this entity and include it in his differential when a patient presents with acute abdominal pain.
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8/9. Management of traumatic oral-facial injury in the hemophiliac patient with inhibitor: case report.

    This report describes identification of factor viii inhibitor in a patient who then received immune tolerance therapy. The precipitating event was a traumatic orofacial injury that was nonresponsive to traditional factor-replacement therapies. An inhibitor complicates medical and dental management of the hemophiliac patient because it counteracts usual techniques of hemorrhage control using coagulation agents derived from factor viii (Monoclate--Armour, Blue Bell, PA). Successful identification and management of the inhibitor patient require communication and consultation between the physician and dentist, up-to-date knowledge regarding the hemophiliac patient's bleeding and infusion history and aggressive application of local adjunctive hemostatic therapies.
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9/9. Acute low back pain secondary to retroperitoneal hemorrhage in an elderly man.

    Acute low back pain is a common complaint heard in the emergency room and in a physiatrist's practice. It is important to rule out occult pathology in patients with an atypical presentation. In the case presented here, the patient was elderly, developed back pain without preceding trauma or lifting, had a history of easy bruisability, had a large ecchymosis, and had worsening back pain with bedrest. An abdominal aortic aneurysm was ruled out and the patient was discovered to have a large retroperitoneal hemorrhage. He was diagnosed with acquired hemophilia secondary to factor viii inhibitors. This has implications for physicians who treat patients with acute low back pain. They must be alert to potentially life-threatening causes of low back pain.
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