Cases reported "Protein S Deficiency"

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21/179. Moyamoya syndrome with protein s deficiency.

    moyamoya disease is a cerebrovascular disease with progressive occlusion of both internal carotid arteries and of their branches and formation of a new vascular network at the base of the brain. Because of the angiographic appearance, it is named as moyamoya. The clinical features are cerebral ischaemia, recurrent transient ischaemic attacks, sensorimotor paralysis, convulsions and migraine-like headaches. A 10-year-old child who acutely developed hemiparesis, weakness and aphasia was found to have moyamoya disease and heterozygous protein S deficiency. This case shows us that during the thromboembolic events the coexistence of protein s deficiency and moyamoya should be investigated.
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22/179. thrombophilia and dental surgery: a report of dental extraction in a patient with protein s deficiency.

    Most patients with thrombophilia are asymptomatic. A case is presented here of a young woman with protein s deficiency, one of the thrombophilias, who required dental extraction. protein s deficiency predisposes a very small number of those affected to life-threatening thromboses and emboli, for which they are required to take lifelong prophylactic anticoagulation. This report emphasizes the need to liaise closely with haematology departments when deciding whether heparinization is required for patients already taking warfarin. The role of low-molecular-weight heparins is highlighted, a brief review of thrombophilia is given and the management of patients who are taking warfarin and need dental surgery is discussed.
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23/179. Multiple aortic thrombi associated with protein c and S deficiency.

    We describe a woman with an unusual case of thromboembolism of the mesenteric artery in whom multiple thrombi were subsequently found in the aorta and right heart chambers on transesophageal echocardiography. Further evaluation revealed a deficiency of protein c and S plasma proteins, inhibitors of the clotting system. The patient was treated successfully with systemic anticoagulation. Aortic thrombus is common in the setting of underlying atherosclerosis. However, the association of aortic thrombus with a deficiency of protein c and S is rare. To our knowledge, this is the first reported case of mural thrombus of the thoracic aorta associated with combined protein c and S deficiency. Our report underscores the important role of transesophageal echocardiography in the evaluation of patients with arterial thromboembolism.
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24/179. Idiopathic pylephlebitis and idiopathic sclerosing peritonitis in a man with protein s deficiency.

    Idiopathic pylephlebitis and primary sclerosing peritonitis are two highly unusual entities. To our knowledge, the association of the two diseases has not been described previously. We report a 42-year-old patient with a protein s deficiency who presented with fever and chills, in whom idiopathic pylephlebitis was diagnosed. A year later, the patient was readmitted because of recurrent vomiting and weight loss. An exploratory laparotomy yielded diagnosis of sclerosing peritonitis, which resolved after surgery. The short time interval between the processes suggests that they were related to each other, and also to the protein s deficiency.
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25/179. anterior spinal artery syndrome in an adolescent with protein s deficiency.

    The diagnosis of anterior spinal artery syndrome can be made with high accuracy by thorough clinical examination in combination with typical magnetic resonance imaging findings. Sudden onset of tetra- or paraparesis and dissociated sensory loss with bladder dysfunction are the leading clinical signs. We discuss clinical and radiologic findings in an adolescent presenting with anterior spinal artery syndrome. The laboratory results showed a hereditary protein s deficiency.
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26/179. A novel splice acceptor site mutation of protein S gene in affected individuals with type I protein s deficiency: allelic exclusion of the mutant gene.

    Sequencing studies of the protein S gene (PROS1) in a Japanese patient suffering from recurrent thrombosis revealed the following. The proband and his first daughter, but not the second daughter, were having the type I protein S (PS) deficiency due to a novel point mutation from A to G at the intronic acceptor splice site in intron 13 of the PROS1. In the affected daughter, exclusion of the aberrant allele was assessed by the BstX1 dimorphism of PROS1 at Pro626 (CCG/CCA). The reduced PS activities in the proband and his first daughter were apparently due to defective production of mRNA from the mutant allele.
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27/179. Unusual retinal vasculitis in a patient with protein s deficiency and systemic toxoplasmosis: a case report.

    A 28-year old female patient presented with unilateral dropped visual acuity and a central scotoma. Fundus examination and a fluorescein angiogram were suggestive for central retinal vein prethrombosis. Further tests revealed an isolated protein s deficiency for which oral anticoagulation therapy was initiated. Six months later she presented with new symptoms of a right retrobulbar pain on ocular movements. The retinal vessels had normalized with improved visual acuity. Re-evaluation of the fluorescein angiogram taken six months earlier showed an image compatible with unilateral vasculitis. During this six month period persisting high levels of IgG and IgM antibodies against toxoplasma were observed while no signs of other active infection could be found. This report illustrates that in cases of prethrombosis with vasculitis in a young patient, a full medical examination and a thorough investigation of infectious diseases are warranted.
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28/179. Lupus anticoagulant and protein s deficiency in a child who developed disseminated intravascular coagulation in association with varicella.

    Varicella is not always a benign disease it may cause serious complications. We report a two-year-old boy with disseminated intravascular coagulation in association with varicella. The patient had the lupus anticoagulant, the antiphospholipid antibody, acquired free protein s deficiency, and increased concentrations of the prothrombin F 1 2 fragment. Intravenous immunoglobulin was administered due to its potential antibody-blocking activity, and the patient responded well. We recommend that children with varicella and disseminated intravascular coagulation should be examined for the lupus anticoagulant, the free protein S antigen, the prothrombin fragment F 1 2 and the other coagulation parameters. Intravenous immunoglobulin administration could be useful in such conditions because of its antibody-blocking activity.
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29/179. heparin-induced skin necrosis in a patient with end-stage renal failure and functional protein s deficiency.

    skin ulceration is a well-characterized thrombotic complication of the heparin-induced thrombocytopenia (HIT) syndrome. We present the case of a 73-year-old diabetic woman nearing end-stage renal failure who developed extensive upper thigh, abdominal and buttock ulceration following initiation of subcutaneous heparin for prophylaxis against deep vein thrombosis. A preliminary diagnosis of calciphylaxis was made based on the classical distribution and macroscopic appearance of the ulceration in a patient with end-stage renal failure and secondary hyperparathyroidism. However skin biopsy showed complete absence of calcium deposits in the dermal microvasculature. The presence of extensive microthrombi within dermal vessels prompted serologic testing to detect a prothrombotic state. We identified the combined presence of heparin-dependent platelet activating (HIT) antibodies and functional protein s deficiency. To our knowledge this is the first reported case of a dialysis patient presenting with skin ulceration induced by heparin and protein s deficiency. This case highlights the importance of a skin biopsy and testing for a hypercoaguable state in patients with end-stage renal disease and skin ulceration. We suggest that HIT antibodies should be requested in all dialysis patients presenting with skin ulceration.
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30/179. Internal jugular vein thrombosis following in-vitro fertilization in a woman with protein s deficiency and heterozygosity for the prothrombin 3' UTR mutation, despite anticoagulation with heparin.

    We report the case of a 31-year-old woman with protein s deficiency and heterozygosity for the prothrombin 3' UTR mutation who developed an internal jugular vein thrombosis despite therapeutic anticoagulation with a low molecular weight heparin, following in-vitro fertilization. This case indicates that the stimulus to thrombosis in such women is intense and can occur despite apparent therapeutic anticoagulation. Close attention should, therefore, be paid to any women with a personal or family history of thrombosis and the potential thrombotic risks associated with assisted conception must be discussed.
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