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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.

This chapter presents general and specialty-specific issues leading to malpractice litigation. Strategies for decreasing medical error and preventing malpractice litigation are outlined with emphasis on accurate documentation, review of clinical information, selection and appropriate use of consultants, and above all, communication to the patient and family. The need to continue learning from national care guidelines and specialty-specific publications is emphasized.

Article-at-a-Glance Background Smoking during pregnancy has been linked with such negative outcomes as increased risk for spontaneous abortions, low birth weight, and perinatal and neonatal mortality. In spring 1998 three leading health care systems in San Diego initiated the Trilateral Partnership ("the Partnership"), whose mission is to improve the health and well-being of children. The Partnership chose tobacco control in pregnant women and their families as its first initiative. Program components—year one (1999) Three interventions were developed: intervention by the prenatal care provider, initiation of a referral process to telephone counseling for pregnant women, and intervention for women reporting spontaneously quitting smoking. To date, 83% of the more-than 20,000 women who have been seen in prenatal screening in 28 months counted themselves as nonsmokers. Eleven percent of the women reported they independently stopped smoking once they learned they were pregnant. Six percent reported that they were still smoking. Twenty-three percent of the women reported living in a household with other smokers. Program components—year two (2000) Activity focused on continuing the previous components, hospital intervention for all new mothers at the time of delivery, pediatric intervention at the newborn's visits at 2 and 6 months of age, and development and refinement of a telephone protocol for new parents. Elements of success The noncontroversial topic of encouraging smoking cessation during pregnancy was one that enhanced immediate buy-in by most individuals contacted to support and engage in the program. Strong commitment and financial support from three health care systems opened doors for the Smoke-Free Families staff and increased the program's visibility in the community.

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.

Amniotic fluid bubble stability and creatinine concentration are analyzed in relation to the L/S ratio and clinical outcome. Bubble stability by the standard method showed 20.5 per cent (31/151) initial disagreement between two laboratories, 53.6 per cent (81/151) correlation with L/S ratio, and did not correlate well with patient outcome. However, a "foamy" test, i.e., foam noticeable at a distance, in both 1:1 and 1:2 dilutions, indicated an elevated L/S ratio and mature pulmonary outcome. Amniotic fluid creatinine levels showed an estimated 5 to 6 per cent risk for RDS at levels we formerly thought to be safe (greater than or equal to 2.0 mg. per 100 ml. with maternal serum less than or equal to 0.9 mg. per 100 ml.), and has many false-negative values. Fetal maturity can be estimated with clinical data and amniotic fluid analysis. Amniotic fluid sampling is necessary in all instances where the clinical data leave a reasonable doubt of actual gestational age and in all cases for elective preterm delivery. We recommend a sequential approach to amniotic fluid analysis. First a bubble stability test (a "foamy" at both 1:1 and 1:2 dilutions indicates pulmonary maturity). If the bubble stability test is not "foamy", an L/S ratio. The risk of RDS associated with creatinine concentration makes this test unacceptable in any case of elective delivery; however, it may be useful as a weighing factor in emergency situations where phospholipid analysis is unavailable.

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...

A new reflectance photometer (Glucometer, Ames Division, Miles Laboratories, Elkhart, Indiana) has been developed for use with the Dextrostix reagent strip. The device measures 8 × 17 cm, weighs 330 g, uses AA batteries, and requires no warm-up. High (330 mg/dl) and low (50 mg/dl) calibrations are stored in memory and need be repeated only when: (1) a control solution (100 mg/dl) reading is in error, (2) ambient temperature changes by more than 5°C, (3) a new bottle of Dextrostix is used, and (4) batteries are changed. A 60-s timer with alarm helps make timing of the procedure consistent. Nineteen insulin-dependent and one non-insulin-dependent diabetic subjects each performed a minimum of 20 blood glucose determinations over a 3-mo period in the investigators' laboratories. Simultaneous capillary blood samples were obtained for plasma glucose analysis by a comparative method. A total of 602 patient-determined glucose values ranging from 30 to 400 mg/dl were compared with simultaneous laboratory values. These values correlated significantly (r = 0.96, P < 0.0001, y = 2.6 + 0.92x). The Glucometer-Dextrostix System is an accurate and convenient method for home blood glucose monitoring by diabetic patients.

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.

In 1971 and 1975, the Committee on Drugs provided information about marijuana for the Academy membership.1,2 Since that time, marijuana use by children and adolescents has continued to increase. Investigations of the biologic effects of the agent have expanded, and there is justification for concern about frequent, chronic use by youth. In addition, marijuana use and experimentation with other drugs are now being promoted to children in subtle and insidious ways which need to be identified (Figs 1 to 5). Pediatricians should be aware of the changing pattern of marijuana use in this country. Whereas occasional use by an adult may have little health consequence for the individual, frequent use by large numbers of children and adolescents raises different concerns. The National Institute on Drug Abuse (NIDA) conducts annual surveys of marijuana use in this country and reported in a recent publication3 that 10% of all high school seniors smoke marijuana daily. In the 12- to 17-year old group, 16% of those surveyed in 1977 used marijuana in the month preceding the survey. Even if the incidence figures are not entirely accurate, an increase in use by these two groups from 1976 to 1977, as determined by similar annual sampling techniques, is clear. Nationwide data since 1977 are not yet available, but in two states surveyed in 1978, 16% of high school seniors smoked marijuana daily (R. C. Peterson, PhD, personal communication, 1979). This information supports the conclusion that use in youth had increased each year since 1975. Extrapolation of additional data collected by NIDA revealed that in 1977 about 16 million Americans were current users of marijuana (ie, used the drug during the month preceding the survey).3

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.

Cerebral blood flow and the distribution of cephalic flow were measured by the microsphere technique in 12 fetal lambs in utero one hour postoperatively and in 11 fetal lambs in utero 24 to 72 hours postoperatively. The mean fetal cerebral blood flow for the two groups was 111.0 ± 7.8 and 120.6 ± 8.7 ml. per 100 Gm. per minute, respectively. The mean distribution of cephalic flow 24 to 72 hours postoperatively was brain 37.6 per cent ± 1.8, eyes 4.0 per cent ± 0.3, skin 12.3 per cent ± 0.7, and remainder of tissue 46.1 per cent ± 1.5. In 8 fetuses, the mean cerebral oxygen consumption was 4.04 ± 0.17 ml. per 100 Gm. per minute 24 to 72 hours postoperatively. Simultaneous fetal cerebral glucose and oxygen consumptions were estimated in 4 fetuses. The mean cerebral glucose consumption was 5.31 ± 0.42 mg. per 100 Gm. per minute, and the mean glucose-oxygen quotient was 1.10 with 95 per cent confidence limits 0.95 and 1.25. The results indicate that these uptakes are comparable to those observed in the brains of adult animals.

Glucose has been considered the principal metabolic fuel of the mammalian fetus, but sound experimental evidence is lacking. In this study, the relative contribution of glucose to aerobic metabolism across the umbilical and cerebral circulations of fetal lambs was evaluated. The fetal vessels catheterized were : femoral artery and vein, umbilical vein, subclavian artery and periorbital vein. The arteriovenous differences in glucose and oxygen contents (Δ glucose and Δ oxygen) were determined from simultaneous samples across both circulations. The Δ glucose was expressed as the O2 stoichiometrically required to metabolize glucose to CO2 and H2O. In six chronic, unstressed, unanesthtized animals, the Δ glucose/Δ oxygen ratio across the umbilical circulation during control periods was 0.47 ± 0.04 (mean ± s.e.m.). Fetal infusions of porcine insulin increased the ratio by 90%. We have attempted to compare metabolism in a single organ of the fetus, the brain, with that of the whole fetus (i.e. umbilical circulation) in acute experimetns in seven fetuses. The ratio across the cerebral circulation was 1.5 ± 0.03 and across the umbilical circulation was 0.38 ± 0.03. These data indicate that (a) under normal conditions, glucose supplies a maximum of 50% of the metabolic fule of the fetal lamb, (b) exogenous insulin does affect fetal glucose uptake, and (c) in the acute preparation, the fetal brain utilizes more glucose than can be accounted for by aerobic metabolism.