The Experts below are selected from a list of 21 Experts worldwide ranked by ideXlab platform

Kjell Haram - One of the best experts on this subject based on the ideXlab platform.

  • Paroxysmal nocturnal hemoglobinuria in pregnancy.
    Acta obstetricia et gynecologica Scandinavica, 2003
    Co-Authors: Line Bjørge, Peter Ernst, Kjell Haram
    Abstract:

    Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia in which a defect of glycophosphatidylinositol (GPI)-anchored proteins in the cell membrane of bone marrow stem cells leads to increased sensitivity of the red cells to complement, causing intravascular hemolysis and hemoglobinuria. Other clinical features of this disease are cytopenia and an increased frequency of thrombotic events. We report a case of a pregnant woman with PNH on high-dosage anticoagulation therapy, the follow-up during the pregnancy, the deLivery and the postpartum period. The obstetric literature on women with PNH is reviewed, the maternal and fetal risks are evaluated and the management of pregnancies and deLiveries in such patients are discussed. During the pregnancy our patient was hypertransfused and used anticoagulation treatment. A healthy child was deLivered in week 37 by cesarean section because of premature rupture of the membranes, unsuccessful induction and intrauterine infection. Because of bleeding problems a hysterectomy also had to be performed. In the postpartum period the patient developed her second episode of a Liver Vein Thrombosis. She recovered gradually and 18 months after the deLivery her disease is now in a stable phase. The literature shows a high maternal morbidity and mortality among pregnant PNH patients. Fetal wastage and prematurity rate are also high. Pregnancy in patients with PNH represents a high-risk situation for both the mother and the child and should not be recommended. A pregnant PNH woman should be followed closely by both obstetricians and hematologists.

  • Paroxysmal nocturnal hemoglobinuria in
    2003
    Co-Authors: Peter Ernst, Kjell Haram
    Abstract:

    Acta Obstet Gynecol Scand 82 2003Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia in which adefect of glycophosphatidylinositol (GPI)-anchored proteins in the cell membrane of bonemarrow stem cells leads to increased sensitivity of the red cells to complement, causingintravascular hemolysis and hemoglobinuria. Other clinical features of this disease arecytopenia and an increased frequency of thrombotic events. We report a case of apregnant woman with PNH on high-dosage anticoagulation therapy, the follow-up duringthe pregnancy, the deLivery and the postpartum period. The obstetric literature on womenwith PNH is reviewed, the maternal and fetal risks are evaluated and the management ofpregnancies and deLiveries in such patients are discussed. During the pregnancy ourpatient was hypertransfused and used anticoagulation treatment. A healthy child wasdeLivered in week37 by cesarean section because of premature rupture of the membranes,unsuccessful induction and intrauterine infection. Because of bleeding problems a hyster-ectomy also had to be performed. In the postpartum period the patient developed hersecond episode of a Liver Vein Thrombosis. She recovered gradually and 18months after thedeLivery her disease is now in a stable phase. The literature shows a high maternalmorbidity and mortality among pregnant PNH patients. Fetal wastage and prematurityrate are also high. Pregnancy in patients with PNH represents a high-risk situation forboth the mother and the child and should not be recommended. A pregnant PNH womanshould be followed closely by both obstetricians and hematologists.Keywords: PNH; pregnancySubmitted 21 February, 2003Accepted 3 June, 2003

Line Bjørge - One of the best experts on this subject based on the ideXlab platform.

  • Paroxysmal nocturnal hemoglobinuria in pregnancy.
    Acta obstetricia et gynecologica Scandinavica, 2003
    Co-Authors: Line Bjørge, Peter Ernst, Kjell Haram
    Abstract:

    Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia in which a defect of glycophosphatidylinositol (GPI)-anchored proteins in the cell membrane of bone marrow stem cells leads to increased sensitivity of the red cells to complement, causing intravascular hemolysis and hemoglobinuria. Other clinical features of this disease are cytopenia and an increased frequency of thrombotic events. We report a case of a pregnant woman with PNH on high-dosage anticoagulation therapy, the follow-up during the pregnancy, the deLivery and the postpartum period. The obstetric literature on women with PNH is reviewed, the maternal and fetal risks are evaluated and the management of pregnancies and deLiveries in such patients are discussed. During the pregnancy our patient was hypertransfused and used anticoagulation treatment. A healthy child was deLivered in week 37 by cesarean section because of premature rupture of the membranes, unsuccessful induction and intrauterine infection. Because of bleeding problems a hysterectomy also had to be performed. In the postpartum period the patient developed her second episode of a Liver Vein Thrombosis. She recovered gradually and 18 months after the deLivery her disease is now in a stable phase. The literature shows a high maternal morbidity and mortality among pregnant PNH patients. Fetal wastage and prematurity rate are also high. Pregnancy in patients with PNH represents a high-risk situation for both the mother and the child and should not be recommended. A pregnant PNH woman should be followed closely by both obstetricians and hematologists.

Peter Ernst - One of the best experts on this subject based on the ideXlab platform.

  • Paroxysmal nocturnal hemoglobinuria in pregnancy.
    Acta obstetricia et gynecologica Scandinavica, 2003
    Co-Authors: Line Bjørge, Peter Ernst, Kjell Haram
    Abstract:

    Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia in which a defect of glycophosphatidylinositol (GPI)-anchored proteins in the cell membrane of bone marrow stem cells leads to increased sensitivity of the red cells to complement, causing intravascular hemolysis and hemoglobinuria. Other clinical features of this disease are cytopenia and an increased frequency of thrombotic events. We report a case of a pregnant woman with PNH on high-dosage anticoagulation therapy, the follow-up during the pregnancy, the deLivery and the postpartum period. The obstetric literature on women with PNH is reviewed, the maternal and fetal risks are evaluated and the management of pregnancies and deLiveries in such patients are discussed. During the pregnancy our patient was hypertransfused and used anticoagulation treatment. A healthy child was deLivered in week 37 by cesarean section because of premature rupture of the membranes, unsuccessful induction and intrauterine infection. Because of bleeding problems a hysterectomy also had to be performed. In the postpartum period the patient developed her second episode of a Liver Vein Thrombosis. She recovered gradually and 18 months after the deLivery her disease is now in a stable phase. The literature shows a high maternal morbidity and mortality among pregnant PNH patients. Fetal wastage and prematurity rate are also high. Pregnancy in patients with PNH represents a high-risk situation for both the mother and the child and should not be recommended. A pregnant PNH woman should be followed closely by both obstetricians and hematologists.

  • Paroxysmal nocturnal hemoglobinuria in
    2003
    Co-Authors: Peter Ernst, Kjell Haram
    Abstract:

    Acta Obstet Gynecol Scand 82 2003Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia in which adefect of glycophosphatidylinositol (GPI)-anchored proteins in the cell membrane of bonemarrow stem cells leads to increased sensitivity of the red cells to complement, causingintravascular hemolysis and hemoglobinuria. Other clinical features of this disease arecytopenia and an increased frequency of thrombotic events. We report a case of apregnant woman with PNH on high-dosage anticoagulation therapy, the follow-up duringthe pregnancy, the deLivery and the postpartum period. The obstetric literature on womenwith PNH is reviewed, the maternal and fetal risks are evaluated and the management ofpregnancies and deLiveries in such patients are discussed. During the pregnancy ourpatient was hypertransfused and used anticoagulation treatment. A healthy child wasdeLivered in week37 by cesarean section because of premature rupture of the membranes,unsuccessful induction and intrauterine infection. Because of bleeding problems a hyster-ectomy also had to be performed. In the postpartum period the patient developed hersecond episode of a Liver Vein Thrombosis. She recovered gradually and 18months after thedeLivery her disease is now in a stable phase. The literature shows a high maternalmorbidity and mortality among pregnant PNH patients. Fetal wastage and prematurityrate are also high. Pregnancy in patients with PNH represents a high-risk situation forboth the mother and the child and should not be recommended. A pregnant PNH womanshould be followed closely by both obstetricians and hematologists.Keywords: PNH; pregnancySubmitted 21 February, 2003Accepted 3 June, 2003

Hans Van Der Lelie - One of the best experts on this subject based on the ideXlab platform.

  • Pregnancy and paroxysmal nocturnal hemoglobinuria
    European journal of obstetrics gynecology and reproductive biology, 1994
    Co-Authors: Joke M.j. Bais, Maria Pel, Albert E. G. Kr. Von Dem Borne, Hans Van Der Lelie
    Abstract:

    A patient is described who developed symptoms of paroxysmal nocturnal hemoglobinuria (PNH) in her first pregnancy. This was uneventful except for a spontaneous preterm deLivery. The second pregnancy was complicated by severe anemia and a hemolytic crisis with Budd-Chiari syndrome at 31 weeks' amenorrhoea. DeLivery was again preterm and was the result of induced labour after premature rupture of membranes at 34 weeks. Literature shows a high maternal mortality among PNH patients (5.8%). The most common cause of death is Liver Vein Thrombosis (Budd-Chiari syndrome). Fetal wastage (30%) and prematurity rate (16%) are also high. Recommendations for follow-up and therapy are given such as anticoagulation therapy, platelets and washed erythocytes transfusions, screening for Budd-Chiari syndrome and infections.

Tor Egge - One of the best experts on this subject based on the ideXlab platform.

  • Hepatic Vein Thrombosis. Diagnostic and therapeutic difficulties
    Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin ny raekke, 1997
    Co-Authors: Kristian Bjøro, J. P. Blomhoff, Erik Schrumpf, Frank Brosstad, Anstein Bergan, Tor Egge
    Abstract:

    Budd Chiari syndrome (Liver Vein Thrombosis) may be a diagnostic and therapeutic problem. On the basis of four different cases we review the major diagnostic and therapeutic principles involved. Imaging techniques are necessary in order to establish the diagnosis. Ultrasound examination with Duplex doppler is usually sufficient, but MR angiography is also useful. Treatment options are thrombolysis, surgery or Liver transplantation. What treatment is selected will depend on the clinical situation and the prognosis.