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1/117. 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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2/117. 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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3/117. pregnancy in bone marrow failure syndromes: diamond-Blackfan anaemia and Shwachman-diamond syndrome.

    pregnancy in bone marrow failure syndromes has risk to mother and fetus. There are fewer than 30 reports of cases with diamond-Blackfan anaemia (DBA), and none with Shwachman-diamond syndrome (SD). We report two DBA and one SD cases. One DBA mother received transfusions intra-partum, and the other only post-partum. Both required caesarean sections (C-sections) for failure of labour to progress and severe pre-eclampsia respectively. Both subsequently resumed pre-pregnancy steroid-induced control of anaemia. approximately 40% of DBA pregnancies required maternal transfusions; 25% delivered by C-section. The SD patient also had Ehlers-Danlos (ED) syndrome and urticaria pigmentosa (UP). Her blood counts were adequate until week 38, when the platelet count dropped and a C-section was performed. pregnancy management in marrow failure disorders requires obstetricians with expertise in high-risk pregnancies, and haematologists with experience with marrow failure syndromes.
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4/117. 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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5/117. Effective treatment of beta-thalassemia intermedia during pregnancy with rHuEpo. A case report.

    The anemia of pregnancy in presence of beta-thalassemia intermedia usually aggravates pregnancy procedure. In the present study we investigated whether the administration of recombinant human erythropoietin (rHuEpo) recombinant human erythropoietin in combination with iron and folic acid may ameliorate blood indices as an alternative choice to blood transfusion.
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6/117. Umbilical artery regression: a rare complication of intravascular fetal transfusion.

    BACKGROUND: Intravascular fetal transfusion is an important therapeutic advance but is associated with several complications. Regression of an umbilical artery associated with transfusion is rare. CASE: A case of red blood cell alloimmunization managed by serial transfusions was complicated by functional loss of an umbilical artery during pregnancy. Refractory fetal bradycardia occurred during the last transfusion procedure, requiring emergency preterm cesarean delivery. Coagulative necrosis, thrombosis, and focal calcification of one umbilical artery was confirmed after delivery. CONCLUSION: Umbilical artery regression associated with transfusion therapy is rare and may complicate subsequent fetal transfusions.
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7/117. Angiographic embolisation in the management of pelvic haemorrhage.

    Haemorrhage from pelvic veins during obstetric and gynaecological surgery is a major cause of morbidity and mortality. In a recent commentary entitled The Seven Surgeons of King's: a fable by Aesop(1), surgeons from different specialties used techniques peculiar to their own practice to treat a woman with intractable haemorrhage. Could transcatheter arterial embolisation be the 'eighth surgeon'? We describe two cases where embolisation was used to control bleeding when local surgical measures had failed and discuss the use of embolisation in obstetrics and gynaecology.
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ranking = 0.43625712749387
keywords = haemorrhage
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8/117. pregnancy, labour and delivery in a Jehovah's Witness with esophageal varices and thrombocytopenia.

    PURPOSE: An increasing number of women with cirrhosis are conceiving and carrying their pregnancies to term. However, the maternal mortality rate remains high (10-61%). This case report describes the management of a parturient with esophageal varices and thrombocytopenia. She was also a Jehovah's Witness. CLINICAL FEATURES: A 25-yr-old Jehovah's Witness parturient with portal hypertension and esophageal varices secondary to cryptogenic cirrhosis was referred to our obstetrical unit at eight weeks gestation. In addition she was thrombocytopenic with platelet counts ranging from 42,000-67,000 x microl(-1). Her esophageal varices were banded prophylactically on three occasions during her pregnancy. magnetic resonance imaging at 32 wk gestation showed extensive caput medusa and dominant midline varix. Therefore, the planned mode of delivery was changed from cesarean section which could result in massive hemorrhage, to elective induction of labour with an assisted second stage. The patient refused any blood product transfusion except acute hemodilution and cell saving if necessary during labour and delivery. Despite elaborate preparations for a planned vaginal delivery, she underwent an unanticipated rapid labour. Spinal analgesia was provided to facilitate smooth assisted vacuum delivery. CONCLUSION: Multidisciplinary care is the key for a successful outcome in parturients with cirrhosis. Periodic examination and banding of esophageal varices is recommended during pregnancy. Active consideration should be given to availing of the benefits of regional anesthesia.
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keywords = transfusion
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9/117. Hyperhemolytic transfusion reaction in sickle cell disease.

    BACKGROUND: An atypical form of life-threatening hemolytic transfusion reaction (HTR) in patients with sickle cell disease (SCD) has been well described in the literature. Continuation of blood transfusion may be lethal, as it can further exacerbate hemolysis. The pathophysiologic mechanism of HTR is not well understood. case reports: Two cases of severe HTR in SCD after the transfusion of compatible RBC units are reported. hemolysis of both autologous and transfused cells was documented in Case 1 by urine Hb high-performance liquid chromotography. Multispecific HLA antibodies were identified in Case 1. Reticulocytopenia was noted in both cases during the acute hemolytic process. This was followed by a rise in reticulocyte count during receipt of IVIG and steroid therapy. bone marrow examination during reticulocytopenia in Case 2 showed erythroid hyperplasia. CONCLUSION: In SCD, both mature sickle cells and sickle reticulocytes adhere more readily to macrophages. In view of the bone marrow aspiration results, it appears that the recipients' HbS cells are destroyed by hyperactive macrophages and that the reticulocytopenia observed during HTR is likely to be due to peripheral consumption (i.e., destruction by macrophages), rather than suppression of erythropoiesis. Cessation of hemolysis during IVIG and steroid treatment may be due to IVIG's blocking of the adhesion of sickle cells and reticulocytes to macrophages, together with steroid suppression of macrophage activity.
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ranking = 7
keywords = transfusion
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10/117. Fetal splenic rupture following transfusion.

    BACKGROUND: splenic rupture in the newborn is a rare complication in erythroblastosis fetalis. There are no reports of splenic rupture in the fetus affected by hemolytic disease of the newborn. CASE: A 41-year-old gravida 3, para 2-0-0-2 with severe rhesus alloimmunization was managed with serial intrauterine transfusions resulting in fetal death after the fourth procedure. autopsy findings revealed intra-abdominal clotted blood and splenic capsular defects consistent with splenic rupture. CONCLUSION: Fetal splenic rupture might occur in hemolytic disease of the newborn associated with splenomegaly. Acute hemodynamic changes with increased intra-abdominal pressure from intrauterine transfusion might precipitate splenic rupture. (Obstet Gynecol 2001;97:824-5.
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ranking = 6
keywords = transfusion
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