Cases reported "Hematoma"

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1/40. A case of factor v deficiency presenting as menorrhagia.

    factor v deficiency is a rare hereditary disorder. We report a patient with factor v deficiency who presented with menorrhagia and pelvic haematoma. The Haematology Department at the Royal Brisbane Hospital performed the definitive factor assays leading to the diagnosis. The challenges of her management were obtaining adequate supplies of factor V and her socioeconomic circumstances. The main future challenge will be the supervision of her pregnancies.
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2/40. Clinical efficacy and recovery levels of recombinant FVIIa (NovoSeven) in the treatment of intracranial haemorrhage in severe neonatal FVII deficiency.

    The use of replacement FVII is critical to the successful treatment of life-threatening bleeds in newborns and infants with severe FVII deficiency (<1%). However, the clinical efficacy, optimum dosage and pharmacologic recovery of rFVIIa in such children has not been studied systematically. This report is a case of an infant with severe FVII deficiency (FVII:C at 0%) and massive intracranial haemorrhage in which successful use of rFVIIa (NovoSeven) was carefully monitored. The drug was administered by intravenous bolus through a central line every 4 h at each of three dose levels: 15 microg kg-1, 22 microg kg-1 and 30 microg kg-1. FVII:C was >100% between 30 and 180 min after each infusion with mean trough levels above 25% for all three dose levels. There was no evidence of hyper-coagulation as indicated by measurements of the platelet count, D-dimer, plasma protamine paracoagulant and fibrinogen levels in spite of high FVII:C concentration. In this infant, rFVIIa was well-tolerated, maintained effective haemostasis with good clinical outcome, and produced consistent therapeutic mean trough levels above 25% FVII:C even at 15 microg kg-1 every 4 h.
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3/40. Spontaneous epidural hematoma following a shunt in an infant with congenital factor x deficiency. Case report and literature review.

    The authors describe a case of an infant with congenital factor x deficiency. The patient presented with a central nervous system hemorrhage followed by hydrocephalus. He underwent a ventriculoperitoneal shunt and, during the postoperative period, developed a spontaneous epidural hematoma, which was evacuated. The clinical and pathophysiological aspects of this case are discussed based on a literature review.
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4/40. Intraosseous hematoma in a newborn with factor viii deficiency.

    We present an unusual case of an intraosseous hematoma in a newborn with a known bleeding disorder. This cephalohematoma was diagnosed shortly after birth, was entirely within the bony skull, and was in fact determined to be an intraosseous hematoma. The initial CT scans showed the unusual appearance and location of the lesion; later scans showed a significant amount of remodeling, with resolution of the hematoma. Although the coagulopathic diagnosis was independent of this finding, a bleeding disorder might be considered in other patients with similar CT findings.
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5/40. Hereditary prothrombin deficiency presenting as intracranial haematoma in infancy.

    Hereditary deficiency of prothrombin is a rare autosomal recessive bleeding disorder, with severe bleeding diathesis in homozygotes, but rarely resulting in intracranial haematoma. We describe two infants of consanguineous parents, presenting with acute subdural haematoma. Because such haematomas in infancy are highly indicative of trauma caused by child battering and because the socio-economic status of the family was unstable, there was a suspicion of child battering. However, further investigations revealed a bleeding diathesis due to a prothrombin deficiency. dna analysis of the prothrombin gene showed homozygosity for a novel mutation, substituting Lys for Glu at codon 7 and resulting in decreased specific clotting activity. We discuss the probability of bleeding diathesis versus child battering in the aetiology of intracranial haematoma.
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6/40. wiskott-aldrich syndrome: life-threatening haemorrhage from aneurysms within the liver, small bowel mesentery and kidney, requiring both surgical and radiological intervention.

    wiskott-aldrich syndrome (WAS) is a rare, generally X-linked recessive condition, originally described by Wiskott in 1937 as a triad of discharging ears, eczema and thrombocytopoenia. Aldrich included bloody diarrhoea in his report of 1954, with severe immunodeficiency and predisposition to malignancy being recognised subsequently. The incidence currently quoted is approximately 4 per million live male births, although there is some regional variation. We report the case of a long-term survivor who had massive haemorrhage from an intrahepatic aneurysm and, on a separate occasion, the right kidney.
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7/40. Recurrent haemoperitoneum in a mild von Willebrand's disease combined with a storage pool deficit.

    Haemoperitoneum secondary to haemorrhagic corpus luteum has been described in severe bleeding disorders such as afibrinogenaemia, type 3 von Willebrand's disease and patients under oral anticoagulation. We have studied one patient who presented three episodes of severe bleeding at ovulation, requiring surgery twice, with the diagnosis of mild von Willebrand's disease and mild storage pool deficiency. Mild von Willebrand's disease (associated with other thrombopathies or coagulopathies) should be considered in this pathology, although physicians would prefer to find a severe haemorrhagic disorder as the underlying condition in these cases.
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8/40. Subperiosteal orbital hemorrhage as initial manifestation of Christmas disease (factor ix deficiency).

    PURPOSE: To report a case of subperiosteal orbital and subgaleal hemorrhage with optic nerve compromise in a patient with a factor ix deficiency. DESIGN: Interventional case report. methods: A 5-year-old male presented 10 days after mild trauma with progressive left-sided scalp swelling, proptosis, and visual loss. RESULTS: The patient had marked proptosis of the left eye, 20/200 visual acuity, and an afferent pupillary defect. magnetic resonance imaging demonstrated a large subgaleal and left subperiosteal orbital hematoma. Quantitative assays of coagulation proteins identified a factor ix deficiency (Christmas disease). CONCLUSION: Delayed-onset subgaleal and subperiosteal orbital hematoma can rarely be an initial manifestation of Christmas disease.
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9/40. Spontaneous bilateral perirenal hematoma as a complication of polyarteritis nodosa in a patient with human immunodeficiency virus infection.

    We present a 29-year-old man with polyarteritis nodosa (PAN) having human immunodeficiency virus (hiv) infection. This patient fulfilled the American College of rheumatology (ACR) 1990 criteria for PAN, and the diagnosis was confirmed by typical arteriographic findings, including microaneurysms. Due to the rupture of microaneurysms, perirenal hematomas occurred in both kidneys. Unilateral nephrectomy was performed, and renal histology confirmed that aneurysm rupture was the etiology of the perirenal hematoma. The occurrence of renal hematomas is a usual complication of PAN. However, bilateral renal hematoma during the course of hiv-associated PAN is quite rare, and to our knowledge, this would be the second case reported in the literature. When compared with other viral agents, the association of hiv with PAN may be considered rare. However, as suggested by various reports in the literature, hiv infection should always be kept in mind while evaluating patients with PAN.
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10/40. Acute onset dysphagia associated with an intramural oesophageal haematoma in acquired haemophilia.

    A 78-year-old man presented with a 5-day history of epistaxis and spontaneous bruising, and a 2-day history of acute dysphagia. barium swallow, computerized tomography scan of the chest and upper gastrointestinal endoscopy were suggestive of an upper oesophageal tumour, although biopsies failed to confirm this. Investigations including a raised activated partial thromboplastin time led to the detection of an inhibitor causing functional factor viii deficiency. Following treatment with intravenous human immunoglobulin, oral prednisolone and oral cyclophosphamide, the patient's dysphagia resolved. There was a resolution of the findings seen at the initial endoscopy and on computerized tomography scan of the chest, consistent with an oesophageal haematoma. Follow-up endoscopy failed to detect recurrence or an aetiological factor.
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