Freeman-Sheldon syndrome. Case contribution and review of the literature]

The paper reports a case of Freeman-Sheldon's syndrome in which a medium-slight degree of mental retardation which was not associated to malformations of the CNS was observed in addition to other typical symptoms. The presence of an hiatal hernia and vesico-ureteral reflow was also noted; to date it is still not possible to establish whether these visceral anomalies are chance findings or whether they are the result of a genetic defect involving tissues of different embryonal origin. Further studies are required to increase our knowledge of Freeman-Sheldon's syndrome and other nosologically related pathologies.     

Fetal vascular responses to prostacyclin

Prostacyclin is a potent vasodilator produced by both maternal and fetal tissues that dilates the umbilical placental vasculature in vitro. To test the hypothesis that prostacyclin dilates the fetal placental circulation in vivo, we measured blood flow by the radioactive microsphere technique in six unanesthetized near-term ovine fetuses before and during prostacyclin infusion. Fetal mean arterial pressure fell 15% from 35 +/- 3 to 31 +/- 3 mm Hg (p less than 0.05) during prostacyclin infusion, and heart rate increased from 182 +/- 6 to 208 +/- 19 beats/min (p less than 0.05). Placental blood flow changed from 240 +/- 58 to 191 +/- 46 ml.min-1.kg-1 fetal weight (p = 0.07), whereas vascular resistance was unchanged (0.16 +/- 0.04 to 0.18 +/- 0.06 mm Hg.ml-1.min.kg fetal weight). Fetal arterial pH decreased from 7.33 +/- 0.03 to 7.28 +/- 0.02 (p less than 0.05) during prostacyclin infusion, with a significant decrease in base excess from -1.2 +/- 1.4 to -3.1 +/- 1.6 (p less than 0.05) and a trend toward hypercarbia (p = 0.07). We conclude that in vivo administration of prostacyclin to the ovine fetus does not cause fetal placental vasodilation and does cause a significant fetal acidemia. The mechanism for these unexpected observations is likely shunting of blood away from the placenta to other organs in the face of systemic vasodilation.     

Current status of and prospects for contraception

It is estimated that at the current rate of growth the world's population will reach 8.5 billion by the year 2025 and 10-11 billion by the end of the 21st century. 90% of this population increase would occur in developing countries, where only 38% of couples used contraceptives during 1980-81 compared to 68% in developed countries. About 300 million couples in the Third World do not use contraceptives, although they do not want more children. Some of these contraceptives include natural steroids, such as progesterone and 17 beta-estradiol that is used for treatment of menopause (1-2 mg daily po). Medroxyprogesterone acetate and norethisterone enanthate depot injections have long-acting properties with low failure rates (3.6% + 0.7 pregnancies/100 women years) if given every 3 months, amenorrhea may occur. RU-486, substance with antiprogesterone activity, inhibits hormonal metabolism during ovulation in a dose of 100 mg/day, just like norgestimate. HRP 102 consists of 50 mg norethisterone enanthate and 5 mg estradiol valerate and cycloprovera contains 25 mg medroxyprogesterone acetate with 5 mg of estradiol cypionate. Both of these agents are effective contraceptives for 2 months. Norplant is implanted subcutaneously in capsule forms. It releases levonorgestrel/LNG for 6-7 years, and in a study of 992 women 2.6 pregnancies occurred for 100 women in the course of 5 years. Vaginal suppositories can release 20 mg/day LNG, or 5-10 mg progesterone/day, and they are considered ideal for nursing mothers. The IUD has been used by 60 million women, however, pelvic inflammatory disease may be associated with its use. Sulprostone and RU-486 (mifepristone) are post ovulatory agents with effectiveness of up to 90 day. Female sterilization has problems of reversibility, male sterilization is less accepted, and other male endocrine approaches producing azoospermia are in the testing phase. The ideal contraceptive with properties of wide acceptability, reversibility, and effectiveness is yet to come.     

Persistent bacteremia due to coagulase-negative staphylococci in low birth weight neonates

During a 6-month period in 1987, 13 low birth weight neonates without indwelling central intravascular catheters had persistent (positive blood cultures for greater than or equal to 6 days) coagulase-negative staphylococcal bacteremia despite adequate antibiotic therapy. Daily blood cultures remained persistently positive for a mean of 13 days (range 6 to 25 days). This group of infants was compared with other low birth weight infants with similar birth weights and nonpersistent coagulase-negative staphylococcal bacteremia, defined as two or more positive blood cultures accompanied by supporting clinical manifestations of sepsis. During this period, coagulase-negative staphylococcal represented 29% of all bacteremias, and 33% of coagulase-negative staphylococcal bacteremias were persistent. Other than soft tissue abscesses, none of the infants with persistent coagulase-negative staphylococcal bacteremia had a defined focus of infection. Abdominal distention (P = .001) and thrombocytopenia (P less than .03) occurred significantly more frequently in the patients with persistent coagulase-negative staphylococcal bacteremia than in those with nonpersistent bacteremia. Of the 13 patients with persistent coagulase-negative staphylococcal bacteremia, 2 received methicillin and 11 received vancomycin. No antibiotic tolerance to either antibiotic could be demonstrated. Serum concentrations of vancomycin far exceeded the minimum bactericidal concentration in all cases in which vancomycin was prescribed. No in vitro differences could be demonstrated between persistent and nonpersistent coagulase-negative staphylococcal strains for slime production, biotype, proteins from modified whole cell lysates developed by sodium dodecyl sulfate-polyacrylamide gel electrophoresis, and opsonophagocytosis by adult neutrophils in the presence of pooled human sera. Additionally, plasmid profile analysis and phage typing revealed no common strain causing the persistent bacteremia.(ABSTRACT TRUNCATED AT 250 WORDS)     

Routine umbilical cord blood gas determinations?

Between 1986 and 1988, 1924 term nulliparous patients with spontaneous onset of labor were studied to assess the importance of obtaining umbilical cord blood gas levels on all deliveries. The umbilical cord arterial and venous pH values (expressed as mean +/- 2 SD) were 7.24 +/- 0.14 (n = 1694) and 7.32 +/- 0.12 (n = 1820), respectively. The incidence of newborn depression (1- or 5-minute Apgar score less than 7) was 14.1%; of these depressed newborns, the incidence of normal umbilical cord arterial pH values (greater than or equal to -2 SD) was 77.8%. Of the vigorous newborns, there was a 2.1% incidence of umbilical cord arterial blood acidemia. Umbilical cord arterial blood acidemia in vigorous newborns was not highly predictive of specific morbidity in the immediate newborn period. Regression analysis demonstrated the umbilical cord arterial pH to correlate best with the Apgar scores when compared with all other arterial or venous blood gas measurements. We reached the following conclusions: (1) that obtaining cord arterial pH values in vigorous newborns should be considered since the values will provide objective documentation or normal fetal acid base balance in 98% of infants. (2) Only a cord arterial pH determination is recommended since it reflects fetal or newborn status more accurately than all other measurements. Additional measurements increase the likelihood of abnormal results and do not contribute to neonatal management. (3) An umbilical cord blood pH value is extremely useful in ruling out the diagnosis of birth asphyxia in the depressed newborn.     

Ovine placental vascular response to the local application of prostacyclin

Rankin et al. have shown that prostacyclin does not dilate the ovine placenta, yet Clark et al. have shown that retrograde infusion of prostacyclin dilates the uterine vasculature. To determine the effects of prostacyclin on the sheep placenta, the two groups have collaborated. In five chronically catheterized near-term sheep, blood flows were measured by the radioactive microsphere technique. Control blood flows were measured and prostacyclin infused into the retrograde uterine artery catheter at 10 micrograms/min. The blood flows were again measured, and the area served by the catheter was then delineated by the injection of a green dye at the time of maternal euthanasia. The placenta and cotyledons were separated and the kidneys were obtained for assay. The systemic effects of prostacyclin were minimal and have been observed previously. The resistance of the myometrium fell from 566 +/- 99.9 to 322 +/- 63.9 mm Hg X ml-1 X min X gm (p less than 0.002). The placental tissue showed no change in resistance. When the tissues were combined, we observed a decrease in resistance from 0.38 +/- 0.18 to 0.32 +/- 0.14 mm Hg X ml-1 X min (p less than 0.004), thereby confirming the observation that there was indeed vasodilation downstream. However, the vasodilation was not in the placenta but in the myometrium, and we reconfirm our previous conclusion that prostacyclin does not dilate the sheep placenta.