[Twin pregnancy in a patient with Glanzmann disease (congenital thrombasthenia) (author's transl)]. 1978

H Riedel, and M Barthels, and E Lingelbach, and H Weitzel

Report on a 30-year old primigravida with a family history of bleeding and a personal history of nasopharyngeal bleeding since age four. Severe menorrhagia since her menarche. First seen at 20 weeks gestation with naso-pharyngeal bleeding and a twin pregnancy. The patient was found to have Glanzmann's disease (congenital thrombasthenia) with an excessive lack of factor XIII. Ambulatory treatment with packed cell transfusions until 35 weeks gestation. The thrombelastogram did not improve with transfusions of platelet concentrates. In-patient treatment from 35 to 37 weeks gestation, no vaginal bleeding throughout the pregnancy. Elective Caesarean hysterectomy at 37 weeks gestation with lo units fresh blood, 3 units platelet concentrate and 10 ampules factor XIII concentrate in readiness. Delivery of twin girls 3180 and 3050 g without signs of Glanzmann's disease. Treatment of the maternal bleeding by replacement. Factor XIII 80% of normal 8 weeks postpartum. This appears to be the first case of twin pregnancy in the presence of Glanzmann's disease and the fourth case in the world literature.

UI MeSH Term Description Entries
D007231 Infant, Newborn An infant during the first 28 days after birth. Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D011247 Pregnancy The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH. Gestation,Pregnancies
D011250 Pregnancy Complications, Hematologic The co-occurrence of pregnancy and a blood disease (HEMATOLOGIC DISEASES) which involves BLOOD CELLS or COAGULATION FACTORS. The hematologic disease may precede or follow FERTILIZATION and it may or may not have a deleterious effect on the pregnant woman or FETUS. Complications, Hematologic Pregnancy,Hematologic Pregnancy Complications,Pregnancy Complications, Hematological,Pregnancy, Hematologic Complications,Complication, Hematologic Pregnancy,Complication, Hematological Pregnancy,Complications, Hematological Pregnancy,Hematologic Pregnancy Complication,Hematological Pregnancy Complication,Hematological Pregnancy Complications,Pregnancies, Hematologic Complications,Pregnancy Complication, Hematologic,Pregnancy Complication, Hematological
D011696 Purpura, Thrombocytopenic Any form of purpura in which the PLATELET COUNT is decreased. Many forms are thought to be caused by immunological mechanisms. Purpura, Thrombopenic,Purpuras, Thrombocytopenic,Purpuras, Thrombopenic,Thrombocytopenic Purpura,Thrombocytopenic Purpuras,Thrombopenic Purpura,Thrombopenic Purpuras
D001803 Blood Transfusion The introduction of whole blood or blood component directly into the blood stream. (Dorland, 27th ed) Blood Transfusions,Transfusion, Blood,Transfusions, Blood
D002585 Cesarean Section Extraction of the FETUS by means of abdominal HYSTEROTOMY. Abdominal Delivery,Delivery, Abdominal,C-Section (OB),Caesarean Section,Postcesarean Section,Abdominal Deliveries,C Section (OB),C-Sections (OB),Caesarean Sections,Cesarean Sections,Deliveries, Abdominal
D005177 Factor XIII Deficiency A deficiency of blood coagulation FACTOR XIII or fibrin stabilizing factor (FSF) that prevents blood clot formation and results in a clinical hemorrhagic diathesis. Deficiency, Factor 13,Deficiency, Factor Thirteen,Deficiency, Factor XIII,Factor 13 Deficiency,Factor Thirteen Deficiency,Deficiencies, Factor 13,Deficiencies, Factor Thirteen,Deficiencies, Factor XIII,Factor 13 Deficiencies,Factor Thirteen Deficiencies,Factor XIII Deficiencies
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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