Biomaterial Database

Pregnancy and paroxysmal nocturnal hemoglobinuria. 1994

J Bais, and M Pel, and A von dem Borne, and H van der Lelie

Department of Obstetrics and Gynecology, Academic Medical Center, Amsterdam, The Netherlands.

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.

UI	MeSH Term	Description	Entries
D007751	Labor, Induced	Artificially induced UTERINE CONTRACTION.	Induced Labor,Induction of Labor,Labor Induced,Labor Induction,Induced, Labor,Induction, Labor,Inductions, Labor,Labor Inductions
D007752	Obstetric Labor, Premature	Onset of OBSTETRIC LABOR before term (TERM BIRTH) but usually after the FETUS has become viable. In humans, it occurs sometime during the 29th through 38th week of PREGNANCY. TOCOLYSIS inhibits premature labor and can prevent the BIRTH of premature infants (INFANT, PREMATURE).	Preterm Labor,Labor, Premature,Premature Labor,Premature Obstetric Labor,Labor, Premature Obstetric,Labor, Preterm
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
D011248	Pregnancy Complications	Conditions or pathological processes associated with pregnancy. They can occur during or after pregnancy, and range from minor discomforts to serious diseases that require medical interventions. They include diseases in pregnant females, and pregnancies in females with diseases.	Adverse Birth Outcomes,Complications, Pregnancy,Adverse Birth Outcome,Birth Outcome, Adverse,Complication, Pregnancy,Outcome, Adverse Birth,Pregnancy Complication
D005260	Female		Females
D005322	Fetal Membranes, Premature Rupture	Spontaneous tearing of the membranes surrounding the FETUS any time before the onset of OBSTETRIC LABOR. Preterm PROM is membrane rupture before 37 weeks of GESTATION.	Premature Rupture of Membrane (Pregnancy),PROM (Pregnancy),Premature Rupture of Fetal Membranes,Preterm PROM (Pregnancy),Preterm Premature Rupture of Fetal Membranes,Membrane Premature Rupture (Pregnancy),Membrane Premature Ruptures (Pregnancy),PROM, Preterm (Pregnancy)
D006457	Hemoglobinuria, Paroxysmal	A condition characterized by the recurrence of HEMOGLOBINURIA caused by intravascular HEMOLYSIS. In cases occurring upon cold exposure (paroxysmal cold hemoglobinuria), usually after infections, there is a circulating antibody which is also a cold hemolysin. In cases occurring during or after sleep (paroxysmal nocturnal hemoglobinuria), the clonal hematopoietic stem cells exhibit a global deficiency of cell membrane proteins.	Paroxysmal Cold Hemoglobinuria,Paroxysmal Nocturnal Hemoglobinuria,Marchiafava-Micheli Syndrome,Paroxysmal Hemoglobinuria,Paroxysmal Hemoglobinuria, Cold,Paroxysmal Hemoglobinuria, Nocturnal,Cold Paroxysmal Hemoglobinuria,Hemoglobinuria, Cold Paroxysmal,Hemoglobinuria, Nocturnal Paroxysmal,Hemoglobinuria, Paroxysmal Cold,Hemoglobinuria, Paroxysmal Nocturnal,Marchiafava Micheli Syndrome,Nocturnal Paroxysmal Hemoglobinuria,Syndrome, Marchiafava-Micheli
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
D000925	Anticoagulants	Agents that prevent BLOOD CLOTTING.	Anticoagulant Agent,Anticoagulant Drug,Anticoagulant,Anticoagulant Agents,Anticoagulant Drugs,Anticoagulation Agents,Indirect Thrombin Inhibitors,Agent, Anticoagulant,Agents, Anticoagulant,Agents, Anticoagulation,Drug, Anticoagulant,Drugs, Anticoagulant,Inhibitors, Indirect Thrombin,Thrombin Inhibitors, Indirect