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1/120. Prognostic factors and management in pregnancies complicated with severe kell alloimmunization: experiences of the last 13 years.

    Because of the recent referral of an anti-Kell sensitized pregnant woman, whose fetus became severely anemic despite intensive antepartum surveillance, the prevalence and characteristics of fetal Kell isoimmunization were reviewed and analyzed. Cases with Kell and RhD alloimmunization requiring intrauterine intravascular transfusions (IUT) at the Mount Sinai Medical Center during the 13-year period ending March 1998 were compared. Thirty-six fetuses with RhD and 5 with Kell isoimmunization required IUTs. Lower fetal and neonatal hematocrit levels were observed in the RhD group. A significantly higher incidence of polyhydramnios was found among fetuses with Kell isoimmunization and the maternal serum titers were much lower than those in the RhD group. DeltaOD450 values did not reliably reflect the Kell sensitized fetus's condition. There were no intrauterine deaths or neonatal direct hyperbilirubinemia in the Kell group, and the hemolytic disease of the newborn was more severe in the RhD group. Although the course of the hemolytic disease in our cases of Kell isoimmunization showed a better prognosis than that in the RhD group, the importance of this condition should not be underestimated, and differences in the pathophysiology of Kell and RhD alloimmunization should be taken into consideration during the management of these cases.
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keywords = isoimmunization
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2/120. 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 = 0.0067122084083081
keywords = rh
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3/120. 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 = 0.0067122084083081
keywords = rh
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4/120. factor vii deficiency detected in pregnancy: a case report.

    factor vii deficiency is a rare hereditary coagulation disorder with an incidence estimated at 1 in 500,000 individuals. In this report, we describe the 13th case in pregnancy. The diagnosis of severe factor vii deficiency (factor VII level <5%) was established at 10 weeks' gestation after initial laboratory testing showed a markedly prolonged prothrombin time and a normal activated partial thromboplastin time. There was a history of two preterm deliveries, but there was no evidence of previous bleeding manifestations. Antenatal progress of the index pregnancy was unremarkable. Prophylactic treatment with fresh frozen plasma was started at the onset of labor and the patient had a vaginal delivery of a live girl at 36 weeks' gestation. There was no postpartum hemorrhage and mother and newborn were discharged in good condition. The patient's postpartum level of factor VII remained undetectable. Two aspects are outlined: the absence of any significant increase in factor VII clotting activity during this pregnancy and the need to give replacement therapy at labor in patients with severe factor VII deficiency to decrease the risk of postpartum hemorrhage.
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ranking = 0.0019177738309452
keywords = rh
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5/120. May-Hegglin anomaly and pregnancy.

    A hypertensive patients with thrombocytopenia is reported who had two pregnancies complicated by preeclampsia and cesarean deliveries without hemorrhage. During her first pregnancy corticosteroids were given for presumed autoimmune thrombocytopenia. Thereafter she was splenectomised. Ten years later May-Hegglin anomaly and renal failure were diagnosed. One of her children had easy bruising.
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ranking = 0.00095888691547259
keywords = rh
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6/120. Glanzmann's thrombasthenia in pregnancy: a case and review of the literature.

    Glanzmann's thrombasthenia is a rare autosomal recessive bleeding disorder resulting from a deficiency of glycoprotein IIb-IIIa complex in platelets. The deficient complex normally mediates platelet aggregation by binding adhesive proteins, which form bridges between activated cells. Despite normal platelet counts, morphology, prothrombin, and activated thromboplastin times, Glanzmann's thrombasthenia is characterized by a prolonged bleeding time and a severe hemorrhagic mucocutaneous diasthesis. pregnancy and delivery are rare in these patients and have been associated with a high risk of severe hemorrhage. We present an unusual case in which a primi-gravida patient with Glanzmann's thrombasthenia underwent an uneventful pregnancy and spontaneous vaginal delivery, following intrapartum intravenous administration of single-donor platelets. Subsequent late postpartum hemorrhage required intravenous transfusion of an additional unit of single-donor platelets. In addition, we review the literature pertaining to pregnancy and Glanzmann's thrombasthenia with an emphasis on intrapartum prophylactic management.
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ranking = 0.0028766607464178
keywords = rh
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7/120. Bleeding risk and reproductive capacity among patients with factor xiii deficiency: a case presentation and review of the literature.

    factor xiii deficiency is an uncommon, inherited bleeding disorder that usually manifests in infancy or early childhood, involving both boys and girls. We present the case of a woman who had experienced two previous intracranial bleeding events, and was treated before and during her current pregnancy with factor XIII concentrate. Her pregnancy was successful, and she experienced an uncomplicated vaginal delivery. To better understand the issues surrounding bleeding, reproductive capacity, and management of factor xiii deficiency during pregnancy, we conducted a systematic literature review using medline from 1966 to December 1998. We also examined the bibliographic references from all articles, and included all cases, case reports, or case series of patients with factor xiii deficiency. We retrieved data on 117 patients from 37 articles, the majority of which had type II deficiency. Among untreated patients with type II factor xiii deficiency, the literature suggests an elevated mortality rate due to uncontrolled bleeding and intracranial hemorrhage. Because of its high degree of efficacy, the evidence supports the use of life long prophylactic therapy with at least monthly infusions of factor XIII concentrate, including during pregnancy. The opinion that women with type II factor xiii deficiency have inevitable recurrent abortions, or that men are sterile, is not well substantiated. No data were found on whether treatment alters male reproductive capacity. A policy of universal factor XIII replacement, starting in childhood, will likely enable more patients to attain reproductive status. The development of an international data registry would optimally address both bleeding risk and reproductive capacity among patients with factor xiii deficiency.
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ranking = 0.00095888691547259
keywords = rh
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8/120. 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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ranking = 0.0067122084083081
keywords = rh
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9/120. Acute promyelocytic leukemia and pregnancy.

    In acute promyelocytic leukemia (APL), the use of all-trans-retinoic acid (ATRA) as a differentiating agent induces complete remission in a high percentage of patients. In pregnancy, however, this drug bears the risk of severe teratogenicity to the child. We report the case of a 23-yr-old woman at 21 weeks' gestation suffering from APL. She was treated with ATRA (45 mg/m2) for 40 d and two courses of standard chemotherapy. The mother achieved complete remission within 22 d of treatment. fetal development was normal, and a healthy premature girl was born in the 35th week of pregnancy. In a review of the literature we have identified 14 cases of APL in pregnancy treated with ATRA alone or in combination with chemotherapy. ATRA has been used as early as in the 3rd week of gestation and in no case have malformations or other teratogenic effects occurred. Side-effects, however, ranged from fetal cardiac arrhythmias to induction of labour. Although known to exhibit severe teratogenic effects during the first trimester of pregnancy, ATRA seems to be reasonably safe during the second and third trimesters in the treatment of APL. However, careful obstetric follow-up is mandatory regarding fetal cardiac complications.
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ranking = 0.00095888691547259
keywords = rh
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10/120. Pernicious anemia, gastric carcinoid, and autoimmune thrombocytopenia in a young woman.

    The association between gastric carcinoid tumors and pernicious anemia is well recognized. Such tumors occur in the presence of achlorhydria, chronic atrophic gastritis, hypergastrinemia, and enterochromaffin-like cell hyperplasia. In this case report, a 29-year-old woman with pernicious anemia and autoimmune thrombocytopenia who developed gastric carcinoid tumors of the gastric body is described. This is the second description of pernicious anemia associated with autoimmune thrombocytopenia. This association in a young woman together with the therapeutic options and decisions that were taken in the treatment of the patient are discussed.
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ranking = 0.00095888691547259
keywords = rh
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