Peter F. Whitington

Effect of taurine supplementation on hepatic function during short-term parenteral nutrition in the premature infant.

To evaluate the potential role of taurine deficiency in the pathogenesis of parenteral nutrition-induced cholestasis, 20 premature (<34 weeks AGA) infants were randomized to receive parenteral nutrition with and without taurine (10.8 mg/kg/day) during the first 10 days of life. Birth weight, gestational age, and protein and caloric intake were similar in both groups. Plasma taurine levels and hepatic function were assessed before the study began (3 ± 1 days of age), at 5 ± 1 days of age, and at 9 ± 1 days of age. Although plasma taurine levels were significantly greater at 5 ± 1 and 9 ± 1 days of age (p = 0.009) in the group receiving supplementation, no differential effect on hepatocellular function could be detected during this short period of time. A decrease in plasma ammonia (p = 0.001), alanine aminotransferase (ALT) (p = 0.036), γ-glutamyltranspeptidase (GGTP) (p = 0.05), 5-nucleotidase (5N) (p = 0.001), and bile salt concentrations was noted in both groups, indicating the rapid maturation of hepatic function even in the presence of parenteral nutrition during the first 10 days of life.

Gastroduodenal endoscopic-histologic correlation in pediatric patients.

The histopathologic abnormalities in endoscopic biopsies from the stomach and duodenal bulb were correlated with the visual findings of upper gastrointestinal endoscopy in 94 children (mean age, 8.4 years; 51 boys and 43 girls) with a variety of complaints. Histology was graded by observers blinded to the endoscopic findings, and both endoscopy and histology were graded using scales reflecting increasing severity with increasing grade. In all three locations studied (gastric body, antrum, and duodenal bulb), endoscopic grade was significantly higher than the histologic grade. Correlation was especially poor with mild endoscopic findings such as erythema and granularity/nodularity, which had little predictive value for histologic inflammation. Few patients had severe disease with eight of 94 having ulcer by endoscopy and seven of 94 having greater than grade 2 histologic disease in any location. We conclude that endoscopy without biopsy should not be used to diagnose gastroduodenal inflammation in pediatric patients.

Rectal gastric mucosa heterotopia as a cause of hematochezia in an infant.

J.S.C. was a 6-month-old white male when first admitted to LeBonheur Childrens Hospital for evaluation of rectal bleeding. He had a history of intermittent passage of dark blood since the age of 10 weeks. This bleeding was associated with symptoms of colic, but with no abdominal distention, vomiting, or bleeding from other sites. Although his stools were normal between attacks, during one they became loose and frequent with passage of clots of blood. He had been evaluated in other hospitals with colonoscopy, proctoscopy, upper gastrointestinal series, two 99Tc pertechnetate scans for ectopic gastric mucosa within a Meckels diverticulum, 15 barium enemas, and laparotomy without a diagnosis. During one episode of bleeding, he developed hypovolemic shock requiring resuscitation. Many formula changes had been tried without effecting relief from the symptoms. Gestational, perinatal, and neonatal histories were normal. Family history revealed that both the patients father and paternal grandfather had intermittent painless rectal bleeding throughout childhood, ending spontaneously in late adolescence. In addition, the father had digital anomalies like those of the patient. Because of an injury which required resection of his rectum, proctoscopy on the father was impossible. At the time of evaluation he was a white male at the 90th percentile for weight and the 50th percentile for

Manometric guidance in suction biopsy of the esophagus in children

Esophageal histological observation is the most reliable indicator of esophagitis. Biopsy specimens obtained by endoscopic grasp forceps are sometimes inadequate for histologic evaluation, and established methods for determining the location of suction biopsies have been imprecise or inconvenient. A method was developed employing manometric control to locate the site of suction biopsies performed with the Quinton multipurpose suction biopsy instrument. With the use of this technique, biopsies were performed in 40 children without failure and without complication.

Alterations in reserve bilirubin binding capacity of albumin by free fatty acids. II. In vitro and in vivo studies using difference spectroscopy.

The effects of free fatty acids (FFA) on reserve bilirubin binding capacity of solutions of human serum albumin (Alb) were determined in vitro using difference spectroscopy. Additionally, sera from 15 infants requiring parenteral nutrition were obtained before, during, and after the administration of a 1.0 g/kg intravenous dose of safflower oil emulsion, and determinations of FFA/Alb molar ratios and reserve bilirobin binding capacities were made for in vivo confirmation. The in vitro experiments demonstrated that increasing the linoleic acid/Alb molar ratio from 0 to 3.5 produced a 37% increase in binding capacity, but further increases to 9 ultimately resulted in a 32% decrease. Similar results were obtained using Alb at pH 7.4 and using defatted whole human serum. Using salicylate to block secondary binding sites demonstrated that linoleic acid enhanced binding at primary sites. In contrast to linoleic acid, octanoic acid did not alter binding at primary sites but lowered total binding capacity when its concentration exceeded 2.5 times that of the Alb in the sample. The in vivo measurements demonstrated that the total reserve bilirubin binding capacity dropped to 30% below base line only when the FFA/Alb molar ratio was greater than 7:1. Increases in total reserve bilirubin binding capacity above 30% over base line occurred in the range of FFA/Alb molar ratios of 1.6:4.0. Binding at primary sites was enhanced in a like manner at moderate FFA concentrations and did not fall below base line even at very high concentrations. These data support the concept of a positive cooperative binding effect between fatty acids and bilirubin at moderate fatty acid/Alb molar ratios and reconfirm the negative effect of fatty acids on bilirubin binding at high molar ratios. These data suggest that hyperbilirubinemic infants may be offered the nutritional benefits of intravenous safflower oil emulsion without additional risk of kernicterus as long as the FFA/Alb molar ratio is kept below 4 or the reserve bilirubin binding capacity is not decreased.

Esophageal stricture dilatation in awake children

Dilatation of esophageal strictures in children has generally been performed in the hospital under general anesthesia, or under deep sedation. We report here a series of 211 dilatations in 13 pediatric patients who had these procedures performed in the outpatient department, 72% of them without any sedation, during a 4-year period. This report characterizes the children, the method of outpatient dilatation, and our results. At the end of the study period, six of the patients no longer required dilatation after five to 14 procedures; four patients still required dilatation at twice-yearly intervals; and three patients remained on an active schedule of dilatations at intervals less than 5 months. Esophageal lumen diameter increased and symptoms improved in all of the children. Catch-up weight gain occurred in one-half of those initially below the 10th percentile. Dilatation without anesthesia or sedation was preferred by all of the parents, and by those children old enough to express a preference. Estimated reduction of medical costs by more than

Triglyceride and Fatty Acid Clearance in Neonates Following Safflower Oil Emulsion Infusion

100,000 was realized, compared with bouginage using general anesthesia. No episode of perforation, significant hemorrhage, documentable aspiration, or neurologic complication occurred. Outpatient dilatation without anesthesia is an effective method of treating esophageal strictures in children.

Correctable Plasma Lipoprotein Abnormalities in Infants with Choledochal Cysts

Fifteen neonates requiring parenteral nutrition with lipid emulsion were given a 1.0 g/kg dose of safflower oil emulsion to evaluate triglyceride (TGY) and free fatty acid clearance. The dose was infused intravenously over 4 hr, and serum was obtained at 0, 2, 4, 6, and 8 hr. Peak serum TGY averaged 592 mg/dl for the appropriate for gestational age newborns and 606 mg/dl for the small for gestational age babies. The small for gestational age neonates had significantly higher serum free fatty acids at 2 and 4 hr into the infusion than did the appropriate for gestational age infants. Peak serum free fatty acids ranged from 0.915 to 3.233 mM in the appropriate for gestational age babies and 2.518 to 3.586 mM in the small for gestational age infants. In contrast to previous work with soybean oil emulsion, we did not demonstrate differences in TGY clearance between small for gestational age and appropriate for gestational age neonates and did demonstrate a markedly elevated serum TGY in these critically ill newborns. Serum TGY and free fatty acids must be monitored in newborns administered intravenous fat emulsion to avoid the complications of iatrogenic hyperlipemia.

Colonic Stricture Complicating Formula Protein Intolerance Enterocolitis

ABSTRACT: Plasma lipoproteins from two female patients—patient A, 4 wk and patient B, 19 months—were examined prior to and at 1 and 5 wk after surgical correction of biliary obstruction due to choledochal cyst. The findings were correlated with standard indices of hepatic function, namely SGPT, GGTP, 5′nucleotidase, serum bile salts, and total and conjugated bilirubin. Prior to surgery in both patients plasma cholesterol, phospholipid, and triglyceride were elevated; cholesterol esters were low; high-density lipoprotein (HDL) cholesterol and apolipoprotein A-I, the major protein constituent of HDL, were subnormal; most of the plasma lipids were contained in the low-density lipoprotein density region; lipoprotein-X was present. Patient B had had a relatively brief obstruction and suffered little secondary hepatic injury. One week after surgery, plasma lipid concentrations returned to normal; apoliprotein A-I increased in the HDL density region and a concomitant rise in cholesterol esters to near normal, 65%, was observed; plasma lipids were contained predominantly in HDL; hepatic function improved markedly. Patient A had had intrauterine obstruction and suffered major hepatic injury with cirrhosis. One week after surgery, plasma lipid concentrations, cholesterol esters, low-density lipoprotein lipid predominance, and hepatic function remained essentially unchanged. Five weeks after surgery, the lipoprotein levels and composition and hepatic function were near normal. In conclusion, children with biliary obstruction have lipoprotein abnormalities similar to those seen in adult patients. These alterations are rapidly reversible with surgical relief and may be used as prognostic indicators of outcome.

Reserve bilirubin binding capacity determined by difference spectroscopy. I. Modifications and performance of the difference spectroscopy assay.

Cases of formula protein intolerance (FPI), in which many of the features of neonatal necrotizing enterocolitis (NEC) have been exhibited (vomiting, diarrhea, hematochezia, abdominal distention, and pneumatosis intestinalis). have been reported (1–6). This case of combined cows milk and soy protein intolerance illustrates further the difficulty in distinguishing clinically between NEC and FPI. Moreover, it demonstrates that intestinal stricture is a potential long-term complication of FPI. as it is also of NEC, and suggests that significant morbidity may result when the diagnosis of FPI is not considered as a cause of the NEC syndrome.