Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0030552 (paresis)
 5,831 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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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PMID:Intraspinal hematomas in hemophilia. 153 Jan 22

Reports of intracerebral hemorrhage (ICH) in patients with hemophilia B are relatively rare. We describe the first clinical results of the use of a monoclonal antibody purified factor IX (FIX) concentrate (Mononine) after an ICH and the long-term outcome of prophylaxis with this product to prevent recurrences. A 44-year-old male with severe hemophilia B was referred to our department because of nausea, vomiting, left lower limb hemiplegia, and left arm paresis. Computed tomography (CT) revealed a right frontal intraparenchymal bleed. The patient was treated with replacement therapy with FIX for 40 days. Computed tomography scans performed on day 40 after the event showed complete disappearance of the cerebral hematoma from the parenchymal tissue. Subsequently, the patient received 25.6 IU/kg(-1) of FIX twice a week. At the 48-month follow-up visit, no more major or minor bleeding events had occurred. Long-term prophylaxis after ICH is recommended.
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PMID:Outcome of long-term prophylaxis after cerebral hemorrhage in a patient with severe hemophilia B. 2107 18