Anaesthetic management of a patient with erythropoietic protoporphyria for ventricular septal defect closure.

Erythropoietic protoporphyria (EPP) is due to a deficiency in ferrochelatase required for haem synthesis. We describe the anaesthetic management of a seven-year-old with EPP undergoing closure of a haemodynamically significant ventricular septal defect. Photosensitivity in EPP patients is triggered at wavelengths near 400 nm and light-excited porphyrins generate free radicals and singlet oxygen that lead to erythrocyte deformity and haemolysis. Stimuli that could trigger a porphyric crisis during anaesthesia and surgery were reduced by avoiding exposure to the sensitive 400 nm spectrum and using light sources covered with yellow acrylate filters in the operating room. Anaesthetic agents not previously associated with porphyric crisis were chosen. Whole blood priming of the extracorporeal circuit was performed to ensure adequate haemoglobin concentrations during the perioperative period.

Identical twins with malrotation and type IV jejunal atresia.

Identical twins are reported, who associated with maternal anaphylactic shock at 101/2 weeks gestation, acquired a type IV jejunal atresia with total absorption of the small bowel beyond the third portion of the duodenum and persistence of the most terminal length of ileum in the ileocolic artery in an apple peel configuration. Both infants survived a tapered jejunoileostomy and have undergone a successful transition to a regular diet. This accidental occurrence in two infants at the same time and in an identical fashion is a rare event.

Fetal movement, biochemical and biophysical parameters, and the outcome of pregnancy.

Fetal movements, fetal heart rate patterns, and serum estriol levels were prospectively monitored in 91 high-risk and normal pregnant women. In four women, there was cessation of fetal movements. In three of these cases, there were pathologic changes in fetal heart rate patterns, with one neonatal death. Of the 87 women who did not demonstrate cessation of fetal movements, one had changes in fetal heart rate patterns. The difference between the two groups was statistically significant (Z test: p less than 0.001). No correlation was found between fetal movements and serum estriol levels in this study. Daily recording of fetal movements can be used as an effective screening test, with high sensitivity and specificity for the fetus in utero. Cessation of fetal movement places the fetus in a high-risk category and should alert the physician to the need for non-stress tests.

Observations on maternal preference for rooming-in facilities.

To determine the length of time mothers prefer their infants with them, 1,000 mothers at North Shore University Hospital (NSUH) and 435 mothers at Kings County Hospital (KCH) were asked postpartum whether they would prefer their infants with them every four hours for 1/2 hour for feeding or rooming-in with their infants during the daytime only or 24 hours a day. Approximately one third of the mothers at NSUH preferred rooming-in whereas approximately three quarters of the mothers at KCH preferred rooming-in. Significantly more mothers at KCH preferred 24-hour rooming-in than at NSUH. Factors such as primiparity, attendance at Lamaze classes, and breast-feeding were significantly associated with the mother's preference to room-in at NSUH, but there was no such association at KCH. This study suggests that: (1) the desire to room-in is not universal; (2) each institution must individualize rooming-in facilities according to the needs of the population; (3) although the demand for rooming-in varies, more mothers prefer rooming-in than there are accommodations presently available; and (4) factors that may be associated with rooming-in are not the same in all populations. It appears that it is as inappropriate to impose rooming-in as it is to deprive mothers of rooming-in.

Kernicterus 1980: problems and practices viewed from the perspective of the practicing clinician.

An understanding of the pathophysiologic factors causing kernicterus, the determination of infants at greatest risk for developing kernicterus, even the cornerstone of therapy for kernicterus--controlling the bilirubin level--are in a current (and continuous) state of revision. These changing concepts are discussed in light of recent research.