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Obstetric complications in a patient with Bernard-Soulier syndrome

Article Abstract:

The Bernard-Soulier syndrome is a genetic disorder characterized by the absence of a specific membrane glycoprotein (a molecule containing carbohydrate and protein, located in the cell membrane) called IB/IX. This results in impaired function of the platelets, cells involved in blood coagulation. Patients with the Bernard-Soulier syndrome have an increased risk of spontaneous bleeding or bleeding during medical procedures, including childbirth. If a pregnant woman with the Bernard-Soulier syndrome is exposed to the blood of a normal fetus, she will produce antibodies directed against the normal platelets of the fetus. This results in thrombocytopenia, a decreased number of blood platelets, in the fetus. A case is described of a 35-year-old woman with Bernard-Soulier syndrome who was immunized to platelet glycoprotein IB/IX, causing thrombocytopenia in her third pregnancy. Her first pregnancy was normal, but she required blood transfusions for bleeding after childbirth. Her second pregnancy resulted in fetal death at 38 weeks of pregnancy and was associated with severe bleeding from the gastrointestinal tract during the last three months of pregnancy. The patient was given blood transfusions and aminocaproic acid for vaginal bleeding, which developed two weeks after the second childbirth. In her fourth pregnancy, the patient was given gamma globulin, a blood protein important in resistance against infections, at the start of labor. She delivered an infant without thrombocytopenia 28 hours later, and was given aminocaproic acid, blood transfusions, and methergine to treat vaginal bleeding. This case demonstrate the complications associated with pregnancy in a patients with Bernard-Soulier syndrome, including severe bleeding during and after childbirth and risk of thrombocytopenia in the fetus. (Consumer Summary produced by Reliance Medical Information, Inc.)

Author: Bell, William R., Kickler, Thomas S., Peng, Thomas C., Haller, Emily
Publisher: Elsevier B.V.
Publication Name: American Journal of Obstetrics and Gynecology
Subject: Health
ISSN: 0002-9378
Year: 1991
Evaluation, Causes of, Thrombocytopenia, Pregnancy, Complications of, Pregnancy complications

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Fetal and neonatal echocardiographic findings in twin-twin transfusion syndrome

Article Abstract:

Serial Doppler echocardiography was conducted with 17 monochorionic twin pregnancies to determine kinds of cardiac involvement associated with twin-twin transfusion syndrome. Cardiovascular state of the donor twins was normal, while the recipient twins exhibited variable severity of biventricular hypertrophy and dilation with tricuspid regurgitation. Cardiac impairments were reversible after the fluid imbalances were corrected by decompressive amniocenteses or after birth.

Author: Nicolini, Umberto, Mosca, Fabio, Fesslova, Vlasta, Villa, Laura, Nava, Simon
Publisher: Elsevier B.V.
Publication Name: American Journal of Obstetrics and Gynecology
Subject: Health
ISSN: 0002-9378
Year: 1998
Research, Complications and side effects, Echocardiography, Twins, Fetal heart

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Obstetric and perinatal outcomes from the Australian and New Zealand Twin-Twin Transfusion Syndrome Registry

Article Abstract:

Twin-twin transfusion syndrome can cause illness and even death even when it is treated. A survey of 112 treated cases revealed a 63% survival rate and 37% of those who survived had brain abnormalities.

Author: Dickinson, Jan E., Evans, Sharon F.
Publisher: Elsevier B.V.
Publication Name: American Journal of Obstetrics and Gynecology
Subject: Health
ISSN: 0002-9378
Year: 2000
Prognosis, Fetofetal transfusion

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