Cases reported "Thrombasthenia"

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1/7. Recombinant activated factor vii combined with local measures in preventing bleeding from invasive dental procedures in patients with Glanzmann thrombasthenia.

    Recombinant activated factor vii (rFVIIa), combined with local measures of fibrin glue and a celluloid splint, preventing bleeding from four invasive dental procedures is reported. A single dose of 180-200 micro g/kg was successfully used in three surgical removals of impacted teeth. Four doses of rFVIIa were required in another full mouth treatment of extraction, pulpotomy, filling and the stainless steel crowning of 13 teeth. The repeated dose of rFVIIa was given whenever the bleeding complication was visualized. It is cost-effective for preventing external bleeding. Additionally, an oral rinsing solution of tranexamic acid (25 mg/kg) was given three times a day for 7 days. In conclusion, rFVIIa has been shown to be an effective alternative to platelet concentrate in patients with Glanzmann thrombasthenia.
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ranking = 1
keywords = extraction
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2/7. Mutations in the beta3 gene giving rise to type I Glanzmann thrombasthenia in two families in portugal.

    Glazzmann thrombasthenia is an inherited bleeding syndrome in which an absence of platelet aggregation is associated with quantitative or qualitative deficiencies of the alphaIIbbeta3 integrin. We now describe biochemical and molecular studies on two Portuguese families where platelets lack both surface and intracellular pools of alphaIIbbeta3. dna extraction was followed by PCR-SSCP analysis of all exons and intronic boundaries in the alphaIIb and beta3 genes. Migration abnormalities were found for PCR fragments encompassing exon 12 (family 1) and exon 10 (family 2). For patient 1, there was a homozygous G to T transition at position 1846 which resulted in a stop codon at codon 616 in the beta3 gene. For patient 2, direct sequencing revealed a homozygous 1347C insert which led to a stop codon at codon 444 in the beta3 gene. For both patients a single mutated allele was inherited from each parent. Evidence is accumulating that nonsense mutations leading to a truncated beta3 may be a frequent cause of type I Glanzmann thrombasthenia in the Iberian peninsula.
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keywords = extraction
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3/7. Surgical and clinical management of a patient with Glanzmann thrombasthenia: a case report.

    A 6-year-old girl with Glanzmann thrombasthenia presented with caries and periapical lesions in the primary mandibular second molars and moderate gingivitis of the maxillary and mandibular anterior teeth. Dental extraction was recommended, and before every surgical intervention, the patient underwent platelet-concentrate transfusion to prevent hemorrhage. Epsilon aminocaproic acid was administered 6 hours before, and 48 hours after every dental procedure to prevent bleeding. In this case, treatment was effective in the prevention of hemorrhagic complications, during the required dental procedures.
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ranking = 1
keywords = extraction
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4/7. Recombinant activated factor vii combined with desmopressin in preventing bleeding from dental extraction in a patient with Glanzmann's thrombasthenia.

    The case of a 41-year-old woman with Glanzmann's thrombasthenia who underwent double dental extraction is presented. In the past, treatments with desmopressin (DDAVP) and tranexamic acid had often unsuccessful efficacy to stop or decrease bleeding. After ineffective DDAVP administration, the removal was performed successfully with recombinant activated factor vii (rFVIIa) infusion. rFVIIa infusion after DDAVP administration could be useful in patients with Glanzmann's thrombasthenia in which DDAVP and tranexamic acid weren't always effective.
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ranking = 5
keywords = extraction
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5/7. Multiple dental extractions in a child with Glanzmann's thrombasthenia: report of case.

    patients with Glanzmann's thrombasthenia have a tendency to hemorrhage. This report shows evidence, however, that extractions in children with the disease can be accomplished without supplemental therapy.
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ranking = 5
keywords = extraction
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6/7. Glanzmann's thrombasthenia: the use of autologous fibrin glue in tooth extractions.

    Three teeth were extracted in three sessions in a 15-year-old girl with Glanzmann's thrombasthenia who suffered in the past severe bleeding and required platelet transfusions. The extraction sites were treated locally with autologous fibrin glue in adjunct of tranexamic acid swish and swallow rinses. No systemic platelet infusion was employed as a preventive measure. No postoperative bleeding occurred.
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ranking = 5
keywords = extraction
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7/7. Dental considerations for a Glanzmann's thrombasthenia patient: case report.

    Glanzmann's thrombasthenia is a qualitative platelet disorder characterized by a deficiency in the platelet membrane glycoproteins (GP) IIb-IIIa. It belongs to a group of hereditary platelet disorders typified by normal platelet numbers and a prolonged bleeding time. The bleeding seen in Glanzmann's thrombasthenia usually includes bruising, epistaxis, gingival hemorrhage, and menorrhagia. Spontaneous, unprovoked bleeding is unusual. The severity of bleeding is unpredictable in thrombasthenia and does not correlate with the severity of the platelet GP IIb-IIIa abnormality. The present case report describes the dental treatment of a patient with Glanzmann's thrombasthenia. A 39-year-old female with a history of Glanzmann's thrombasthenia presented for periodontal therapy for spontaneous gingival hemorrhage. The patient had been sporadically seen in the past and had a record of only returning for appointments on an "emergency" basis. The periodontal findings revealed a diagnosis of moderate to advanced adult periodontitis in all quadrants. After all dental options had been discussed, the treatment of choice was determined to be extraction of the remaining dentition and fabrication of immediate dentures. The patient received a loading dose of 5 grams of aminocaproic acid (EACA) intravenously 3 hours prior to the surgery. At the beginning of the extractions 1 gram of EACA per hour continuous infusion and a 6 pack of platelets was administered. The patient tolerated the extractions well. All sites healed normally. The patient has had no difficulty in adjusting to the dentures. The case report discusses a possible treatment option in a noncompliant patient having Glanzmann's thrombasthenia and briefly discusses other hereditary bleeding disorders with similar presentations.
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ranking = 3
keywords = extraction
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