Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019045 (hemoglobinopathies)
 2,704 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The efficacy of correlating the L/S ratio in the amniotic fluid with fetal lung maturity has been substantiated in normal pregnancies. In gestations complicated by fetomaternal diseases, however, the assay is less reliable. This study involves 555 pregnancies in which there was a significant maternal, fetal, or placental disorder. The L/S ratio was related to fetal respiratory maturity as measured by Dubowitz criteria and the occurrence of RDS. The results show that pre-eclampsia, chronic hypertension, diabetes (Class D, E, F), significant cardiovascular disease, severe hemoglobinopathies, various congenital anomalies, chronic placental insufficiency, and prolonged ruptured membranes accelerated the L/S ration. Conversely, mild diabetes (Class B, C), intrinsic renal disease, hepatitis, collagen disease, hydrops fetalis, syphilis, and toxoplasmosis were associated with a delay in the L/S ratio. A significant increase in erroneous responses was noted in these patients when the L/S ratio was correlated to infant maturity and to the incidence of RDS. Possible mechanisms for these findings are discussed.
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PMID:The lecithin/sphingomyelin ratio in cases associated with fetomaternal disease. 57 73

A study of 20 patients with hemoglobin E trait abnormality and 8 patients with hemoglobin E disease in pregnancy is presented. The hematologic abnormality in HbE hemoglobinopathy is mild though hemoglobin values are significantly lower than in HbA patients. Fetal wastage is no increased and the salvage rate is high (live births over 95 per cent). The mean birthweight of the baby in HbE patients is significantly lower than in HbA patients, but the incidence of prematurity is not significantly increased in HbE patients. Blood loss at delivery is not markedly affected by hemoglobin type. The incidences of postpartum hemorrhage, preeclampsia, and malaria are not significantly increased in HbE patients; the reason for the significant increase in incidence of abruptio placentae in HbE is not known. No maternal deaths or congenital fetal abnormalities were recorded. It is concluded that the course of pregnancy in HbE trait abnormality and HbE disease is relatively benign.
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PMID:Maternal and fetal outcome associated with hemoglobin E trait and hemoglobin E disease. 107 30

To evaluate recent management of pregnancy in women with sickle cell hemoglobinopathy, pregnancy outcomes of 29 women with homozygous (HbSS) disease, 52 with double heterozygous (HbSC) disease, and 51 normal (HbAA) women were assembled. The case series was 132 singleton births to 132 Ghanaian women, 81 of whom were from the Korle-bu Teaching Hospital, Accra, from June 1988 to October 1991. Controls had no history of hypertension, diabetes, rhesus isoimmunization, or pre-eclampsia, and their infants with significantly lower birth weight (mean 2.59 kg), at lower gestational age (35.2 weeks) than controls. Doubly heterozygous sickle cell mothers had infants with a shorter gestational age (36.4 weeks) than controls (37.25 weeks), yet averaging the same birthweight as controls (3.10 kg). The placental weights were not significantly different between the 3 groups. The results were interpreted to suggest that children of HbSC mothers were large for gestational age (LGA). Continuous sickling of the mother's red blood cells is thought to be the cause of poor outcomes in homozygous sickle cell pregnancy.
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PMID:Comparative studies of live neonates in maternal sickle cell haemoglobinopathy in Ghana. 129 39

This study analyzes the obstetric cases seen by 1 consultant firm at the Riyadh Military Hospital in Saudi Arabia between December 4, 1978-July 1, 1979. Of 487 patients referred to the booking clinic by primary care physicians, only 384 patients attended. Many patients were advanced in pregnancy when seen in the primary care department and were seen by the obstetricians only when admitted to the labor floor. A retrospective analysis of the notes was made and the results assessed of those patients under the care of 1 consultant firm. Patients attending the antenatal clinic underwent the routine investigations and clerking procedures. Iron deficient anemia was rarely seen (2.6%), and the need for iron or vitamin replacements was based on hematological indices. Megaloblastic anemia was detected in only 1 case. Very few abnormal hemoglobinopathies (1.6%) were found. Diabetes and rheumatic and congenital cardiac disease were occasionally seen. Grand multiparity was common, although very few problems arose. The incidence of mild preeclampsia was 2.6%, compared with 20-30% for the U.K. It was rarely necessary to resort to surgical induction for suspected post-maturity. The incidence of preterm infants was between 4.7-5%. Salbutamol was used in the obstetric unit, being the only available beta-adrenergic agonist for the suppression of uterine activity. Patients were not adverse to taking tablets during pregnancy, but they were aware that drugs can reach the baby through the mother's milk. There was no major problem with the predominantly male medical staff. Contraception was freely discussed in the clinics and wards. Patients in higher social classes found it fully acceptable, but those in lower classes always insisted on discussions with the male partner and rarely was it accepted. Hospital delivery was acceptable to many patients, yet others gave birth at home and were ignorant of the hospital facilities available. A reasonable perinatal mortality rate is reported (15/1000) but the study does not consider those patients not attending the new hospital for delivery.
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PMID:Initial findings in a new obstetric unit in Saudi Arabia. 708 Jan 67

Retrospective analysis was made of office and hospital records of patients with sickle cell hemoglobinopathies. Blood products were transfused only when indicated for symptomatic anemia, severe anemia with a hematocrit less than 18%, sickle crisis, cardiovascular instability, and preoperatively. The Fisher exact test and the Student t test were used for statistical analysis; P < 0.05 was considered significant. All mean values are reported +/- 1 standard deviation. From 1981 to 1991, 40 patients with sickle cell hemoglobinopathies had a total of 61 singleton pregnancies: 36 were complicated by SS disease (SSD), 22 by sickle cell disease (SCD), two by sickle-thalassemia, and one had CC disease (CCD). Only patients with SSD and SCD are reported here. The mean maternal age was 24.3 +/- 5.3 and 19.5 +/- 0.6 years in patients with SSD and SCD, respectively. There was a high occurrence of preterm labor (45% and 20%), preeclampsia (20% and 8.7%), pain crisis (50% and 34.2%), pulmonary complications (25% and 16.7%), and cesarean sections (52.6% and 37.1%) in SSD and SCD, respectively. An average of two units of blood was required by 43.1% of the patients. Two patients with SSD had unpreventable deaths. The mean gestational age at delivery was 35.5 +/- 4.3 and 37.0 +/- 3.7 weeks (P < 0.05), and the mean birthweight was 2443 +/- 926 and 2997 +/- 807 g (P < 0.05), respectively. There were two intrauterine fetal deaths and one neonatal death in the SSD group and one neonatal death in the SCD group. The perinatal mortality was 10.5% and 2.9%, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Outcome of pregnancies complicated by sickle cell and sickle-C hemoglobinopathies. 804 82

Sickle cell disease (SCD) is the most common inherited hemoglobinopathy and is associated with increased risk of complications and early mortality. Nowadays, with improved health care facilities, antibiotic prophylaxis, vaccination, and availability of drugs like hydroxyurea, the life expectancy of SCD patients has improved. More women are reaching reproductive age group and are expressing their desire to reproduce. Though SCD adversely affects pregnancy, leading to increased incidence of maternal and perinatal complications like pre-eclampsia, preterm labor, IUGR, abortions etc., adequate care throughout pregnancy ensures a better outcome. Also, recent advancements in the fields of prenatal diagnosis and preimplantation genetic diagnosis, help couples suffering from SCD to have a healthy baby. This paper focuses on the effects of SCD on pregnancy outcomes and effective management of complications during pregnancy, also comparing maternal and perinatal outcomes in studies conducted in different countries. The second part of the paper summarizes pregnancy management in SCD for better maternal and fetal outcomes.
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PMID:Sickle Cell Disease and Pregnancy. 3130 16