Aganglionosis of the small intestine: a rare form of Hirschsprung's disease.

Absence of ganglion cells in the small intestine, a rare form of Hirschsprung's disease, is a condition found in newborns and associated with increased morbidity and mortality. Families are faced with numerous challenges, including maintenance of nutrition, the need for surgical interventions, and the potential for complications associated with the medical technologies necessary to maintain children with aganglionosis. Home care needs are not unlike those of other children with severe intestinal dysfunction such as short bowel syndrome. This article gives an overview of Hirschsprung's disease and presents three case studies of small bowel Hirschsprung's. One center's experience with Hirschsprung's disease supports the need for a multidisciplinary approach to long-term management and home care.

Sedation in children: adequacy of two-hour fasting.

OBJECTIVES: (1) To investigate the relationship between the duration of time that children fasted before a procedure and their gastric volume and pH at the time of the procedure. (2) To compare the variables of gastric pH and volume with historical standards. METHODS: We performed 285 gastroscopies for children aged 0.1 to 18.6 years (mean, 7.5 +/- 5.3) between October 1991 and January 1995. Duration of fasting was 0.5 to 24 hours (mean, 6.7 +/- 5.3) after ingestion of clear liquids. Immediately after intravenously administered sedation, the gastric contents were removed endoscopically with suction and direct visualization to ensure complete evacuation. The volume and pH of the gastric contents were measured and analyzed in comparison with the duration of fasting. The values obtained were also compared with historical standards thought to minimize the risk of aspiration pneumonia: gastric volume 0.4 ml or less per kilogram of body weight and pH of 2.5 or greater. RESULTS: There was no significant correlation between duration of fasting and either gastric volume divided by body weight (mean, 0.68 +/- 1.31 ml/kg; range, 0 to 15.23 ml/kg) or pH (mean, 2.03 +/- 1.40; range, 1 to 8). There was less no significant difference in the percentage of children with gastric volume of 0.4 ml/kg or less or with pH of 2.5 or greater between the groups with the following fasting times: 30 minutes to 3 hours, more than 3 hours to 8 hours, and more than 8 hours. CONCLUSIONS: On the basis of the data in this study and a review of the literature, we concluded that (1) fasting longer than 2 hours after ingesting clear liquids does not significantly change gastric volume or pH, (2) there is no advantage in requiring children to fast for longer than 2 hours after clear liquid ingestion before sedation or anesthesia for any procedure, and (3) fewer than half of pediatric patients actually achieve the "desirable" values of a gastric volume of 0.4 ml/kg or less and a pH value of 2.5 pH units or more, regardless of fast duration, even though these values are presented in the literature as a goal to minimize the risk of aspiration pneumonia.

A 3-year survivor of near-total intestinal aganglionosis.

A newborn girl had no ganglion cells beyond 2 cm distal to the ligament of Treitz. The proximal jejunum was dilated. A jejunostomy constructed 46 cm distal to the ligament of Treitz functioned. A 28-cm jejunal segment was myectomized to augment enteral feeding. For almost 1 year she has been on enteral feedings only, and her body size has been in the 75th percentile.

Noncytotoxic inhibition of malignant cell growth by Ulex seed extracts.

A water-soluble, boiled extract produced from the seeds of Ulex europeus (European gorse) was found to inhibit the growth of various reticuloendothelial tumor cell lines. Seven tumor cells, six murine and one human, were investigated and found to be sensitive to the inhibitory activity of such extracts. Ulex seed extracts (USE) reduced the incorporation of tritiated thymidine by tumor cells in an exponential dose - response relationship. In addition, 5- to 8-day cultures of these cell lines grown in the presence of USE had a marked reduction of tumor cell numbers. The reduction in growth exhibited a dose - response relationship. Growth inhibition was not the result of cytotoxicity. Cell viability and washout studies demonstrated the impermanence and easy reversibility of the inhibiting state.

Ulex seed extracts: lymphocyte growth inhibition and the anti-H hemagglutinins.

Extracts produced from the seeds of Ulex europaeus are commonly used for their ability to react with the H basic substance present on erythrocytes and secreted in body fluids. Such extracts were found to also contain a potent inhibitor of human and murine lymphocyte growth. Inhibition of growth does not result from cytotoxicity and is easily reversible. Ulex seed extract (USE) solutions were modified in various ways to produce reagents in which the anti-H hemagglutinins were either retained or removed. The fractionated solutions were then analyzed for hemagglutination and lymphocyte growth-inhibiting activity. Such studies clearly indicated that these two biological functions resulted from the action of different materials. The lymphocyte growth inhibitor is not a glycoprotein lectin. It does not mediate its effect through the H basic substance and is a heat-stable, small molecule. The data suggest that plant seed extracts employed for their lectin content may contain an additional class of biologically active agents potentially useful in man.