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1/13. Acute pancreatitis and deep vein thrombosis associated with hellp syndrome.

    The hellp syndrome (HS) belongs to the list of obstetric complications believed to be associated with coagulation disorders. It was formerly thought that chronic intravascular clotting (DIC) in the placental vessels was the main cause. A hypercoagulable state has been reported in cases of severe HS associated with microvascular abnormalities that may involve cerebral, placental, hepatic and renal vessels. A case of acute pancreatitis and DVT of inferior cava in a pregnant woman, presenting with HS at 29 weeks, who was found to have a R506Q mutation, is reported. Preeclampsia-associated pancreatitis and DVT have rarely been reported. It is hypothesized that APC-R and factor v Leiden mutation may prove to be new and more important markers capable of predicting a more significant maternal morbidity associated with HS. thrombosis prophylaxis may be considered during pregnancy in order to reduce hazardous multiorgan failure (MOF) in women who are heterozygous for factor v Leiden mutation.
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2/13. Hemorrhagic moyamoya disease during pregnancy.

    BACKGROUND: Intracranial hemorrhage in pregnant patients with moyamoya disease is rare. We review the case of one such patient who presented with pre-eclampsia and a catastrophic intracerebral hemorrhage in order to highlight the associated management difficulties. methods: A case of a pregnant (31 weeks) female brought to the emergency department with hypertension and a progressive decrease in her level of consciousness is presented. She rapidly developed a dilated right pupil and left extensor posturing. A CT scan of her head showed a large putamenal intracerebral hemorrhage. She was intubated, ventilated and given intravenous mannitol and magnesium sulfate. She underwent a simultaneous craniotomy and cesarean section. Post-operatively the patient's ICP and jugular venous saturation were monitored in the intensive care unit. RESULTS: The patient delivered a 1185 g infant who did well. The patient's ICP was well controlled until the tenth post-operative day when she developed malignant brain edema and died. CONCLUSION: This case highlights three important points. First, simultaneous craniotomy and cesarean section can be performed. Second, intraoperative control of bleeding Moyamoya vessels is described. Third, the difficult post-operative management of these cases is highlighted. The literature regarding moyamoya disease and pregnancy is reviewed and some recommendations for the management of this rare but potentially deadly condition are presented.
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3/13. Henoch-Schonlein purpura in pregnancy.

    Henoch-Schonlein purpura (HSP) is an IgA-mediated small vessel vasculitis which commonly involves the skin, gastrointestinal system and kidneys. Numerous HSP triggers have been identified, and pregnancy has been reported as an exacerbating factor. After a pregnant woman had been diagnosed as having new-onset HSP, we reviewed all cases of immunofluorescence-proven HSP evaluated by the Department of dermatology at the Johns Hopkins Hospital between 1990 and 2002, and report three cases of HSP occurring during pregnancy. Two patients developed new-onset HSP, one at 16 weeks gestation and one at 22 weeks, while the third developed a recurrence of HSP at 12 weeks gestation after 19 years of remission. We conclude that pregnancy may be a trigger for HSP onset or recurrence in susceptible individuals.
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4/13. Diffuse alveolar hemorrhage associated with antineutrophil cytoplasmic antibody levels in a pregnant woman taking propylthiouracil.

    propylthiouracil (PTU) is known to be a potential cause of antineutrophil cytoplasmic antibody (ANCA) positive small vessel vasculitis, resulting in glomerulonephritis and diffuse alveolar hemorrhage (DAH). Herein, we describe a 25-year-old pregnant woman who developed a perinulcear ANCA (p-ANCA) and myeloperoxidase ANCA (MPO-ANCA) positive DAH during PTU therapy. The patient improved after corticosteroid therapy and discontinuation of the PTU. methimazole was prescribed in spite of the risk of recurrence of DAH because of the pregnancy. The patient is currently free from pulmonary problems. Our case shows that the alternative agent, methimazole, can be used to treat hyperthyroidism in a pregnant patient with PTU associated DAH.
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5/13. Ocular complications of disseminated intravascular coagulation (DIC) in abruptio placentae.

    A 30 year old white woman presented at term with vaginal bleeding, dizziness and blurred vision due to bilateral serous retinal detachments. Extensive areas of choroidal occlusion with focal leakage of dye from the posterior retinal pigment epithelium and choroid were demonstrated by fluorescein angiography. With the diagnosis of abruptio placentae, a dead fetus and disseminated intravascular coagulation (DIC) the patient underwent removal of the uterine contents by cesarean section. DIC and retinal detachments resolved with improvement of vision, DIC, frequently observed in obstetric complications, has a tendency to occlude the posterior choroidal vessels, which often results in serous retinal detachments. In the setting of pregnancy, especially complicated late pregnancy, the occurrence of serous retinal detachments may be an early ocular sign of DIC.
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6/13. Factors affecting the response to plasma therapy in thrombotic thrombocytopenic purpura.

    Factors affecting the response to plasma therapy (plasma exchange and plasma infusion) were studied in four cases of thrombotic thrombocytopenic purpura (TTP) treated in our hospital. Plasma therapy led to recovery from advanced TTP, but three of the patients exhibited chronic relapse. The longer the period from onset, the more marked was the progression of TTP, from the first stage (thrombocytopenia alone) to the second stage in which thrombocytopenia was accompanied by microangiopathic hemolytic anemia (MHA), and the third stage in which neurological abnormalities appeared. Platelet thrombi, which are thought to be the primary pathogenetic feature, probably enlarge leading to stenosis of the vessels during the progression of TTP. Although the severity of TTP in the early stages could be judged from two markers, LDH and the platelet count, it was impossible to determine the severity of advanced TTP by these two markers. However, the severity of advanced TTP could be judged by including the grade and period of neurological abnormalities. The dose of plasma needed to induce recovery was small in the early stage, but as the period from onset lengthened, the dose had to be increased. Though the early platelet thrombi might be easily eliminated by plasma infusion, larger and well-established platelet thrombi might not be lysed even by massive doses of plasma.(ABSTRACT TRUNCATED AT 250 WORDS)
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7/13. Disseminated intravascular coagulopathy.

    disseminated intravascular coagulation (DIC) is not a disease but a pathological process with widespread thrombus formation in small vessels; it occurs in many systemic conditions that stimulate the intravascular clotting mechanism. There may be widespread tissue involvement, and any tissue in the body may be affected, especially in the kidney, brain, liver, heart, and lungs. This abnormal coagulation is now commonly referred to as disseminated intravascular coagulopathy. It is prone to occur in obstetrical complications, in cancer, after transplantations, and where there has been tissue damage, such as burning, crushing, and surgery, all of which release thromboplastin into the circulation. It may also occur in Gram negative bacterial systemic infections, in antigen-antibody reactions, and in thrombotic thrombocytopenic purpura. When the eye is involved, the thrombi occur in the choriocapillaris, and are usually limited to the submacular and peripapillary choroid. The anterior parts of the eye generally escape involvement. Visual symptoms may be early, and may be due to central choroidopathy or to secondary retinal detachment.
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8/13. Anticardiolipin antibodies during pregnancy.

    A case of antinuclear antibody negative systemic lupus erythematosus, characterized by repeated vessel occlusion and spontaneous abortions, in which anticardiolipin antibodies seemed to be transiently present during early pregnancy is reported. The possible role of the antibodies inducing abortion is discussed.
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9/13. Arterial thrombosis, intrauterine death and "lupus" antiocoagulant: detection of immunoglobulin interfering with prostacyclin formation.

    In a 31-year-old woman with a history of recurrent arterial thrombosis, both of whose pregnancies had resulted in intrauterine death at 23 and 24 weeks, a "lupus" anticoagulant was identified. The patient's IgG fraction, containing the lupus anticoagulant, reduced the release of prostacyclin (PGI2) from rat aorta rings or pregnant human myometrium. This inhibitory effect was abolished in the presence of arachidonic acid. The production of 6-keto-PGF1 alpha by cultured bovine endothelial cells was also decreased in the presence of the patient's IgG fraction. The plasma level of 6-keto-PGF1 alpha was reduced. An antibody in this patient may interfere with the production or release of PGI2 by the vessel wall, possibly by interfering with the availability of arachidonic acid. This mechanism could play a role in this patient's arterial disease and obstetric problems.
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10/13. Thrombotic thrombocytopenic purpura: report of fourteen cases--occurrence during pregnancy and response to plasma exchange.

    Thrombotic thrombocytopenic purpura (TTP), a syndrome of diverse etiology probably related to factors regulating platelet-vessel wall interaction, is predominantly a disorder of women. We report our experience with 14 patients in an 11-year period. Thirteen were female and aged between 25-69 years. Four were postmenopausal, and of the nine premenopausal women three were pregnant, one was immediately postpartum, and three were taking estrogen-containing oral contraceptives. A review of the literature confirms the two to one female/male preponderance and that TTP is reported in 56 women who are pregnant or recently postpartum. While this association with possible hormonal events has been noted, it has previously received little comment. We stress the similarity between TTP and some occurrences of preeclamptic toxemia, and that this may suggest not only a common etiology but that therapeutic attempts should be similar. While no single therapeutic modality is universally successful, our experience is that plasma exchange is the most effective, with five of seven patients so-treated obtaining prolonged remission; four of five patients responded to splenectomy and corticosteroids, but one died of infection postoperatively. Five patients, including two treated exclusively with antiplatelet aggregating agents, died without achieving remission. The frequency of successful therapy is not changed by the concurrent pregnancy, but the fetal loss is high. There does seem to be an increased risk of recurrence of TTP in a subsequent pregnancy, and this might be considered when counseling premenopausal patients who have achieved remission of TTP.
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