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1/520. Management of a premature infant with moderate haemophilia A using recombinant factor viii.

    The birth of a very premature infant with haemophilia A is a rare event. In this case report the problems posed in the management of a child with a factor viii level of 0.03 IU mL-1 born at 28 weeks of gestation and weighing 1590 g are considered. The value of recombinant factor viii, the pharmacokinetics of factor VIII in this situation and the importance of close cooperation between paediatricians and haematologists are discussed.
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ranking = 1
keywords = gestation
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2/520. A case of aplastic anaemia in pregnancy.

    Aplastic anaemia in pregnancy is an extremely rare condition with high maternal morbidity and mortality rates. Intensive haematological support remains the mainstay of therapy and a successful obstetric outcome can be best accomplished with the close clinical collaboration of the haematologist and the obstetrician as occurred with our patient reported here.
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ranking = 133.62037378483
keywords = pregnancy
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3/520. 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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ranking = 26.724074756967
keywords = pregnancy
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4/520. Serial serum thrombopoietin levels in a pregnant woman with essential thrombocythaemia.

    thrombopoietin (TPO) is a ligand for c-mpl, which regulates the differentiation and maturation of megakaryocytes. Essential thrombocythaemia (ET) is a clonal myeloproliferative disorder. It has been reported that the platelet count declines during pregnancy in ET patients. We examined serial changes in the serum TPO level during the course of pregnancy in a patient with ET. The serum TPO level showed significant negative correlation with the platelet count. Although it mimicked the normal feed back system, the TPO levels were consistently higher than the normal upper limit. Accumulation of these data will be helpful in revealing the pathogenesis of ET and the decline in the platelet count during pregnancy.
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ranking = 80.172224270901
keywords = pregnancy
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5/520. Management of chronic myeloid leukemia during pregnancy with leukapheresis.

    We describe the successful treatment of a pregnant patient with chronic myelogenous leukemia in chronic phase by using only leukapheresis. Following 20 leukapheresis procedures initiated during the 13th week of gestation and performed over approximately 7 weeks, the patients white blood cell count dropped from 242,000/microl to 19,300/microl. The WBC remained stable over the ensuing 17 weeks until the time of delivery. The patient gave birth by cesarean section to a healthy 2,640 g boy at 37.5 weeks of gestation. This is the second report of the successful use of leukapheresis alone for chronic myelogenous leukemia in chronic phase during the first half of pregnancy. We conclude that where leukapheresis is available, it may provide an alternative treatment to chemotherapy or alpha-interferon, especially in light of their potential teratogenic and leukemogenic side-effects.
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ranking = 135.62037378483
keywords = pregnancy, gestation
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6/520. Spontaneous intrahepatic hemorrhage and hepatic rupture in the hellp syndrome: four cases and a review.

    Subcapsular hemorrhage and hepatic rupture are unusual catastrophic complications of the HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome. A high index of suspicion and prompt recognition are keys to proper diagnosis and management of affected patients. The optimal management of these patients is evolving. An aggressive multidisciplinary approach has considerably improved the morbidity and mortality associated with these complications. We present our experience with four cases of hepatic hemorrhage occurring in association with the hellp syndrome and review the literature on this subject. All of our patients were multiparous, and three had a history of eclampsia/preeclampsia in a previous pregnancy. All four patients developed intrahepatic hemorrhage; two developed hepatic rupture requiring surgical intervention. Three patients developed disseminated intravascular coagulation and acute renal failure. Two patients developed pericardial effusion, pleural effusions, and ascites. One patient died of septic complications after multiple surgical interventions.
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ranking = 26.724074756967
keywords = pregnancy
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7/520. Small for gestational age infant in association with maternal prothrombin gene variant (nt 20210A).

    Most of disproportionate infants born small for gestational age (SGA) have an history of placental dysfunction with no explained cause. We report a case of an unexplained SGA infant with placental infarctions and thrombosis. Maternal thrombophilic disorder tests revealed that the patient was heterozygous for the A20210 prothrombin gene variant a newly identified thrombotic risk factor. It may be suggest that prothrombin gene variant, as factor V Leiden, could be a genetic risk factor for placental insufficiency.
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ranking = 5
keywords = gestation
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8/520. Extensive spontaneous retroperitoneal hemorrhage: an unusual complication of heparin anticoagulation during pregnancy.

    A 27-year-old patient at 13 weeks' gestation maintained on subcutaneous heparinization due to hemoglobin S and hemoglobin c (SC) sickle cell disease and previous splenic vein thrombosis presented with spontaneous acute onset of severe left lower abdominal and groin pain. The pain, which radiated to the anterior aspect of the thigh, was associated with nausea and vomiting and was exacerbated by extension of the left lower extremity. The patient was hemodynamically stable, yet during the first 24 h of hospitalization a marked decrease in hematocrit from 29% to 22% occurred. Contrast computed tomography (CT) revealed an extensive abdominal-pelvic, retroperitoneal hematoma extending approximately 15 cm in length from above L5 cephalad to below the greater trochanter of the left femur caudally. The retroperitoneal hemorrhage self-tamponaded and did not require surgical management. The dosage of heparin was decreased and maintained with appropriate activated partial prothrombin (aPTT) levels. To our knowledge, this is the first report of a spontaneous retroperitoneal hemorrhage complicating heparin anticoagulation in pregnancy. Unusual hemorrhagic complications of anticoagulation therapy are discussed.
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ranking = 134.62037378483
keywords = pregnancy, gestation
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9/520. Death due to thrombotic thrombocytopenic purpura in pregnancy: case report with review of thrombotic microangiopathies of pregnancy.

    maternal death during pregnancy, although uncommon, may result from a broad range of conditions. In this paper, a case of thrombotic thrombocytopenic purpura diagnosed by postmortem examination is presented. Thrombotic thrombocytopenic purpura is one of a subset of diseases that result in the formation of microthrombi within the vasculature, either as a primary or secondary manifestation. Other conditions included in the differential diagnosis during pregnancy are hemolytic uremic syndrome, systemic lupus erythematosus, preeclampsia-eclampsia and the hellp syndrome, acute fatty liver of pregnancy, antiphospholipid antibody syndrome, and disseminated intravascular coagulation. The histologic manifestations of these diseases can be similar and in most cases do not provide adequate information to accurately differentiate these diseases in the postmortem period. This paper addresses the need for clinical history (i.e., symptomatology, trimester of onset) and antemortem laboratory testing in addition to a thorough autopsy to accurately differentiate among the conditions named previously. In the absence of an adequate clinical history and antemortem laboratory testing, the more general diagnosis of "thrombotic microangiopathy of pregnancy" is acceptable.
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ranking = 320.6888970836
keywords = pregnancy
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10/520. Spinal haematoma following epidural anaesthesia in a patient with eclampsia.

    A patient with a twin pregnancy required a Caesarean section for severe pre-eclampsia. Her platelet count was 71 x 10(9).l-1. Epidural anaesthesia was performed after platelet transfusion. A spinal epidural haematoma was diagnosed postoperatively. A generalised tonic-clonic seizure sparing the lower limbs enabled early diagnosis to be made. The patient recovered with no permanent neurological damage after laminectomy and clot removal. The risks and benefits of regional techniques require careful consideration, and postoperative monitoring for recovery of neural blockade is essential.
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ranking = 26.724074756967
keywords = pregnancy
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