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Congenital hypoprothrombinemias are very rare, inherited disorders in which factor II (prothrombin) levels and/or activity are extremely low or absent. We report eight pregnancies in a patient with this disorder. Obstetric complications attributed to the coagulation disturbance included first-trimester bleeding in each pregnancy, miscarriage in four of the pregnancies, spontaneous maternal subarachnoid hemorrhage in one, and postpartum hemorrhage in one of four term pregnancies despite administration of clotting factor concentrate. The management of pregnancy in congenital hypoprothrombinemia, and issues of coagulation factor replacement, are discussed.

We report a subgroup of patients with fulminant hemolysis, elevated liver enzymes, low platelet count (HELLP) syndrome, manifesting extreme elevation of aspartate aminotransferase (AST; SGOT) and lactate dehydrogenase (LDH) levels and abnormal mental status. These gravidas are at high risk for mortality. Only four patients treated by the authors over a 10-year period have had AST more than 2000 IU/L and LDH more than 3000 IU/L in the HELLP syndrome. This report is based on retrospective chart review. All patients manifested disordered mental status, jaundice, intense hemolysis, and extreme hypertension. One patient had developed multiple organ system failure, was moribund at initial perinatal consultation, and died. The three others were treated with aggressive afterload reduction and plasma infusion or plasmapheresis; two survived. Fulminant HELLP syndrome occurs rarely, but marks a group of patients at high risk for mortality. Optimal therapy is unclear; early intervention, including afterload reduction, volume expansion, and consideration of plasma infusions or plasmapheresis, is recommended.

In a multicentered, randomized trial of maternal administration of corticosteroids to prevent neonatal RDS, 626 singleton pregnancies were treated either with dexamethasone (N=315) or placebo (N=311). Among these parturients, 6.9% had pregnancy-induced hypertension (PIH) and 6.4% had chronic hypertension. Maternal corticosteroid administration to those with PIH revealed a trend toward reduction in the rate of neonatal RDS. Normotensive women did not more frequently develop de novo hypertension regardless of treatment group. Those with hypertension did not demonstrate aggravation of this maternal condition more often upon treatment with dexamethasone compared to patients receiving the placebo. Neither hypertensive condition was associated with an increase in neonatal neurologic dysfunction such as intraventricular hemorrhage, hydrocephalus, seizures or apnea when the placebo and treatment groups were compared. The incidence of perinatal mortality, particulary fetal death, was not increased among those part...

Ninety-eight insulin-dependent diabetic patients were cared for during 108 pregnancies following an ambulatory program aimed at close control of blood sugar levels. The incidence of respiratory distress syndrome (RDS) in these patients was not different from that in nondiabetic patients. Fetal pulmonary maturity seemed to progress at a normal rate as evidenced by an equal incidence of mature lecithin/sphingomyelin (L/S) ratios at various stages of gestation in diabetic and nondiabetic patients. There was no evidence that Class B and C diabetic patients have delayed fetal lung maturation or that Class E, F, and R patients have accelerated maturation. It is concluded that careful metabolic control of the pregnant diabetic patient results in a normal process of fetal pulmonary maturation without increased risk of RDS.

Adolescent pregnancy is associated with a high degree of risk for the mother and her baby which may be mitigated by good prenatal care, intensive nutritional counseling, and attention to social problems. A study of 222 predominately black, single adolescents who delivered at the University of Tennessee Center for the Health Sciences during the 2-month period between December 1977 and February 1978 revealed an increased incidence of hypertension and convulsive disorders as well as a high rate of toxemia and fetal distress during labor and delivery. Birth weights of less than 2500 gm were associated with a 12% incidence of prematurity, and nearly 15% of the newborns required intensive care (23 had high-risk Apgar scores at 1 minute and 18 at 5 minutes). Perinatal mortality was 54/1000. In this population, teenage pregnancy is viewed as an accepted and even welcome occurrence, supporting the finding that poor neonatal care is a prime cause of the increased complications of adolescent pregnancy. Comprehensive personal services are required to achieve patient compliance with prenatal medical care.

Because of the impression obtained from our population with sickle cell anemia that there were no cases of diabetics, a study was conducted to investigate the prevalence of diabetes mellitus in: 1) 186 cases of pregnant black patients with sickle cell disease (HbS-S, S-C, or S-Thal) or HgA-S with random and 2-h postprandial blood glucose (compared to 2263 matched control patients); 2) a study of the hemoglobin pattern in 280 consecutive cases of nonpregnant black patients with confirmed diabetes mellitus in our outpatient clinic; and 3) a review of the records of 711 patients with sickle cell disease in our Sickle Cell Center with performance of a 100-g oral glucose tolerance test on 5 patients with random plasma glucose greater than 120 mg/dl. These studies showed: 1) there were no diabetics in pregnant black patients as compared to a 4% incidence of abnormal glucose tolerance in a control, matched population; 2) there were no cases of sickle cell disease in the diabetic population; and 3) there...

One hundred four postdate pregnancies were managed according to a well-defined protocol calling for weekly oxytocin challenge tests and urinary estriols three times per week. Although the perinatal mortality rate in these patients was not increased there was a significant increase in the incidence of neonatal morbidity and complications. The clinical syndrome of dysmaturity was seen in 20 per cent of the neonates. When meconium was present in the amniotic fluid the incidence of neonatal and fetal complications was higher. The cesarean section rate was twice the normal rate, with nonprogression of labor being the commonest indication. It is recommended that: (1) pregnancies carried beyond 42 weeks do not require termination simply because they are post dates; (2) all postdate patients should be monitored during labor; (3) trained personnel to initiate neonatal resuscitation should be present at each postdate delivery.

Two groups of patients are summarized. The first group demonstrates contemporary perinatal outcome using a predominantly outpatient management plan. The second group demonstrates extension of the outpatient management using a reflectance meter for in-the-home blood glucose assessments. The financial savings of outpatient management is emphasized.

Intravenous sodium tolbutamide tests were performed in patients with 1) proven insulomas, 2) functional hyperinsulinism, 3) mild diabetes mellitus and associated postprandial hypoglycemia, 4) severe adrenal insufficiency, and 5) liver disease. Healthy subjects were used as controls. Administration of sodium tolbutamide to patients with functioning islet-cell adenomas usually produced a greater degree of hypoglycemia than was observed in the other subjects studied. Of much greater significance, however, were the prolonged periods of hypoglycemia in the insuloma patients. After removal of the insulomas the response to tolbutamide became normal. Persistence of tolbutamide-induced hypoglycemia: 1) is a valuable adjunct in the diagnosis of insuloma, 2) is due, in part, to release of excessive amounts of insulin from the insuloma, and 3) is supportive evidence that the primary mechanism of action of the sulfonylureas is release of pancreatic insulin. From a clinical point of view the intravenous tolbutamide tes...

Extract: The relative utilization rates of glucose and oxygen by the fetal brain were evaluated by measuring simultaneously arteriovenous differences of glucose (Δ glucose) and oxygen (Δ oxygen) across the cerebral circulation of 10 fetal sheep. The mean ± 1 SEM Δ glucose was 0.197 ± 0.012 mM, and the Δ oxygen was 1.11 ± 0.08 mM. Preductal arterial blood was obtained from the brachiocephalic artery; cerebral venous blood was obtained from the fetal sagittal sinus up to 6 days following surgery. A glucose/oxygen quotient was calculated to determine the fraction of cerebral oxygen consumption required to metabolize the glucose utilized by the brain to CO2 and H2O. This quotient was 1.06 with 95% confidence limits of 1.12 and 1.02. These data indicate that glucose can supply all the aerobic metabolic requirements of the fetal brain. Speculation: Although glucose seems to be the primary substrate of cerebral metabolism, the amount of glucose acquired by the fetal lamb via the umbilical circulation can satisfy, at most, one-half of the total fetal aerobic needs. Thus the fetal brain seems much more dependent on glucose than do other fetal organs, and may be particularly vulnerable to periods of hypoglycemia.