Hemophagocytic syndrome in pregnancy.

BACKGROUND: Hemophagocytic syndrome is characterized by nonmalignant histiocytes that undergo uncontrolled phagocytosis of normal hematopoietic cells. Clinical severity ranges from complete recovery to rapid deterioration and death. CASE: Thrombocytopenia was discovered upon routine initial prenatal evaluation of a 24-year-old, gravida 2, para 1, at 29 weeks' gestation with a history of necrotizing lymphadenitis. Cytopenia and elevated transaminases developed, followed by hyperpyrexia. The patient delivered and her postpartum course was complicated by coagulopathy, multiorgan failure, and death. Bone marrow biopsy confirmed hemophagocytic syndrome. CONCLUSION: Early diagnosis of hemophagocytic syndrome during pregnancy might be helped by recognizing symptoms and signs, including a history of necrotizing lymphadenitis, and obtaining a bone marrow biopsy.

Impaired ability of patients with familial isolated vitamin E deficiency to incorporate alpha-tocopherol into lipoproteins secreted by the liver.

Plasma and lipoprotein alpha-tocopherol concentrations of four patients with familial isolated vitamin E deficiency and six control subjects were observed for 4 d after an oral dose (approximately 15 mg) of RRR-alpha-tocopheryl acetate labeled with six deuterium atoms (d6-tocopherol). Chylomicron d6-tocopherol concentrations were similar in the two groups. d6-Tocopherol concentrations of plasma, very low (VLDL), low (LDL), and high (HDL) density lipoproteins were similar in the two groups only during the first 12 h; then these were significantly lower, and the rate of disappearance faster, in the patients. The times (tmax) of the maximum chylomicron d6-tocopherol concentrations were similar for the two groups, but tmax values in the controls increased in the order: chylomicrons less than VLDL less than or equal to LDL approximately HDL, while the corresponding values in the patients were similar to the chylomicron tmax. Thus, plasma d6-tocopherol in controls increased during chylomicron and VLDL catabolism, whereas in patients it increased only during chylomicron catabolism, thereby resulting in a premature and faster decline in the plasma tocopherol concentration due to a lack of d6-tocopherol secretion from the liver. We suggest that these patients are lacking or have a defective liver "tocopherol binding protein" that incorporates alpha-tocopherol into nascent VLDL.

Total complement activity in maternal sera, amniotic fluids and cord sera in women with premature labor, premature rupture of membranes or chorioamnionitis.

Total hemolytic complement activity (CH50) was determined in maternal sera, amniotic fluids or cord sera, or all, from 119 patients with preterm uterine contractions, premature rupture of membranes or chorioamnionitis, or all, at 24 to 40 weeks of gestation. The mean CH50 of maternal sera exceeded the mean CH50 of both amniotic fluids and cord sera. The mean CH50 of amniotic fluids exceeded that of cord sera and increased significantly at 32 weeks. This rise preceded that of the mean CH50 of cord sera, which occurred at a fetal weight of approximately 2,500 grams. The mean CH50 of amniotic fluids varied significantly and inversely with that of cord sera. The levels of CH50 in these three fluids did not distinguish between patients with preterm uterine contractions who delivered prematurely and those who delivered at term. The CH50 in patients with premature rupture of membranes did not differ from a control population of women with uncomplicated pregnancies. The mean CH50 of maternal sera was increased in patients with chorioamnionitis but was not predictive of chorioamnionitis. The mean CH50 of maternal sera was decreased in patients who smoked cigarettes and in patients who received intravenous ritodrine.

Acute pulmonary edema resulting from treatment for premature labor.

The development of acute pulmonary edema in four pregnant women being treated for premature labor with betamimetic and glucocorticoid therapy is described. The radiological features and pertinent obstetrical literature concerning this adverse drug reaction are discussed.

The effect of dopamine, atropine, phenylephrine and cardiac pacing on oxygen consumption during fentanyl-nitrous oxide anaesthesia in the dog.

In animals deeply anaesthetized with fentanyl and nitrous oxide the arterial blood pressure and heart rate were increased using dopamine, atropine, electrical pacing and phenylephrine in order to study the accompanying change in whole body oxygen consumption. Seven dogs (16-24 kg) were anaesthetized with fentanyl 1 microgram . kg-1 . min-1. After completing instrumentation a dopamine infusion was started at a rate of 39 micrograms . kg-1 .min-1. After the mean blood pressure reached 18.6 kPa the infusion was reduced to 10 micrograms . kg-1 . min-1 and maintained for 10 minutes. After waiting 45 minutes an infusion of atropine 20 micrograms . kg-1 . min-1 was started and when the heart rate reached 120 b/min the infusion was slowed to 1.25 micrograms . kg-1. min-1 and maintained for 10 minutes. Twenty-five minutes later the heart rate was increased to 150 beats/min and maintained at that level for 10 minutes using electrical pacing. The pacing was removed and an infusion of phenylephrine 5 micrograms . kg-1 . min-1 was started. When the blood pressure reached 21.3 kPa the infusion was reduced to 2.5 micrograms . kg-1 . min-1 and maintained for 10 minutes. The results show increases in oxygen consumption of 14 per cent with dopamine, 19 per cent with atropine, 16 per cent, and 14 per cent with phenylephrine. All changes were significantly different from the control values. The magnitude of change in whole body oxygen consumption was best predicted by either the cardiac output X blood pressure product or by the cardiac output alone.