Metabolism and transport of galactose by rat intestine.

Intestinal uptake and metabolism of galactose were examined in everted jejunal rings from fasted adult rats using 0.2-28 mM sugar. After 60-min incubations, the total uptake (free tissue plus amount metabolized) of galactose ranged from 1.75 mumol/g at 0.2 mM to 21 mumol/g at 28 mM. Free tissue galactose was 17% of the former and 73% of the latter amount while that oxidized to 14CO2 represented only 6-16% of amount taken up. Compared to glucose, similar amounts of galactose are taken up at 0.2-2.0 mM, however, gllcose rtween 0.2 and 2 mM similar amounts of both sugars are metabolized, although a greater portion of the glucose is oxidized to 14CO2. Above 2.0 mM, 2-3 times more glucose is metabolized than galactose. Both uptake and metabolism showed saturability and kinetic analysis revealed two limbed Linweaver-Burk plots, suggesting operation of a high affinity low Km and a low affinity high Km system for sugar transport. In a series of in vivo studies, to assess the role of the intestine in the total body metabolism of galactose, 14C-labeled galactose injected intraperitoneally at a dose of either 50 or 300 mg into fasted normal, sham operated and enterectomized rats, no observable difference in 14CO2 production resulted in between the groups. It would thus appear that although extensive metabolism of galactose may take place in intestinal tissue in vitro, the intestine does not play a significant role in galactose disposition in vivo.

Basal and stimulated serum growth hormone concentrations in inflammatory bowel disease.

Patients with inflammatory bowel disease (IBD) manifest growth failure which may antecede abdominal symptoms by some years. Eight of ten children with documented IBD had records of decreasing growth velocities. Investigation of growth hormone reserves showed excessive rather than impaired responses. Mean basal GH level was 6.2 +/- 0.75 (SEM) ng/ml. During sleep, the mean GH level rose to 26.0 +/- 4.7 ng/ml and following propranolol-glucagon stimulation, to 46.0 +/- 4.5 ng/ml. All values were significantly higher than levels obtained in a control population of 25 children investigated for short stature who were not GH deficient. The mean peak GH response following insulin in the IBD group (10.8 +/- 3.8 ng/ml), however, did not differ from the mean peak response in the control group (13.5 +/- 3.3 ng/ml). Growth failure in patients with IBD is not the result of GH deficiency and is not an irreversible phenomenon. On the contrary, judicious use of glucocorticoids aimed at the control of the disease usually produces compensatory growth acceleration ("catch-up growth").

Galactose and glucose metabolism in the isolated perfused suckling and adult rat liver.

The development of the technique for the perfusion of the immature liver has enabled us to characterize metabolic differences in carbohydrate metabolism in the suckling versus adult rat livers. Livers of fasted suckling and adult rats were perfused with 4 mM galactose or 4 mM glucose. Galactose uptake was the same for both age groups during the first 35 min. The adult liver maintained the initial rate of uptake after this period while the immature liver began to take up galactose more rapidly. By the end of the experimental period, on a weight basis, uptake by the young liver was three times that of the adult. Analysis of the livers at the end of the 90 min perfusion showed hepatic galactose concentrations to be one-half of circulating media levels. Glucose output was observed in each group during perfusion with either galactose or glucose. In the immature liver, galactose perfusion stimulated more glucose output than did the glucose perfusion. In the adult, however, both sugars resulted in the same levels of glucose output. Galactose perfusion resulted in glucose levels in young liver being higher than the media; while in the adult, the level was lower than the media. Galactose perfused livers of the suckling and adult contained significantly more uridine-5'-diphosphogalactose than the glucose perfused livers of each age.

Management of congenital microgastria with a jejunal reservoir pouch.

A 3-mo-old female presented with growth retardation, vomiting, reflux esophagitis, recurrent aspiration pneumonias, and was found to have megaesophagus and microgastria. After the failure of conservative therapy a double-lumen jejunal (Hunt-Lawrence) pouch with distal Roux-en-Y anastomosis was anastomosed to the stomach to increase the gastric reservoir. One year later, there has been progressive weight gain, the megaesophagus and gastroesophageal reflux have lessened significantly, pneumonia has not recurred, and the tracheobronchitis and esophagitis have resolved. This suggests that the gastroesophageal reflux and megaesophagus were due to an inadequate reservoir with a secondary gastric overflow as the esophagus dilated to enlarge the reservoir capacity of the upper gastrointestinal tract. Utilization of a jejunal pouch increased the size of the gastric reservoir, allowed resolution of the secondary esophageal changes, and permitted normal growth to proceed.

Colitis cystica profunda: a pediatric case report.

A case of colitis cystica profunda in a 14-year-old male is presented. The clinical and pathologic management are reviewed. Differentiation from adenocarcinoma by careful histologic evaluation of the epithelium is fundamental in avoiding overtreatment.

Behavior modification in the treatment of rumination.

Rumination is repeated regurgitation without nausea or associated gastrointestinal illness with concomitant weight loss and malnutrition. This paper describes a ruminating infant who was becoming severely malnourished. Hyperalimentation was used to provide necessary nutrition, and a paradigm of aversive behavior modification was instituted. This form of therapy was successful after less than four weeks, with no harmful side effects and no recurrence of the disorder.

Endoscopic balloon dilation of esophageal strictures in children.

A total of 17 patients, ages 3 weeks to 14 1/2 years, had 20 esophageal strictures develop after repair of esophageal atresia (9 strictures); primary gastroesophageal reflux (3 strictures); Nissen fundoplication (4 strictures); epidermolysis bullosa congenita dystrophica (1 stricture); congenital esophageal stenosis (2 strictures); or colonic interposition (1 stricture). These strictures were treated with 132 endoscopic balloon catheter dilations (average, 6.6/patient, range, 1 to 24) during a period of 42 months (average, 8 months; range, 2 to 42 months). Fifteen of the 17 patients are now asymptomatic. Thirteen of the 17 patients had documented reflux esophagitis and were also medically treated. The two residually symptomatic patients included a patient with severe proximal esophagitis secondary to epidermolysis bullosa congenita dystrophica who had only a temporary response to dilations and another patient with a tight Nissen fundoplication who did not improve after dilation but is now asymptomatic after corrective surgery. The 10 patients with tracheoesophageal fistula repair were asymptomatic within 3 to 21 months (average, 10.8 months). Only one patient had perforation develop as a result of the procedure. Sixteen of 17 patients had the procedures performed as outpatients. All patients were given oral feedings throughout the course of dilation and had normal growth.

Clostridium difficile associated with pseudomembranous colitis. Occurrence in a 12-week-old infant without prior antibiotic therapy.

In a previously healthy 12-week-old male infant with a two-week history of poor feeding, colic, and bloody stools, pseudomembranous colitis developed. No prior antibiotics were administered although the child had received dicyclomine hydrochloride. Clostridium difficile and its toxin were detected in the child's stool. Severe disseminated intravascular coagulopathy developed; the patient required total colectomy but eventually recovered. Clostridium difficile colonization has not, to our knowledge, been previously documented in infantile pseudomembranous colitis.

Pulmonary hypertension associated with portal hypertension in a child with Williams syndrome--a case report.

A 14-year-old white female with Williams syndrome and portal hypertension presented in shock; at autopsy she was found to have grade II to VI vascular changes of pulmonary hypertension. This case demonstrates the association of portal hypertension and pulmonary hypertension in a pediatric patient.

Plasma 1-palmitoyl-2-linoleoyl phosphatidylcholine. Evidence for extensive phospholipase A1 hydrolysis and hepatic metabolism of the products.

1-Palmitoyl-2-linoleoyl phosphatidylcholine (PLPC) labeled in either the choline, glycerol, palmitate, or linoleate component in reconstituted rat high density lipoprotein (rHDL), was administered by vein to rats with bile fistula and taurocholate infusion. PLPC disappeared from plasma in a monoexponential fashion with a half-life of 50 min. A small fraction, about 14%, of PLPC disappearance was due to removal of linoleate from the sn-2 ester bond to form plasma cholesterol esters, presumably by lecithin-cholesterol acyltransferase. Otherwise, nearly all of the PLPC components that disappeared from blood in 1 h were recovered in the liver. The choline, glycerol, and linoleate components appeared predominantly in hepatic phosphatidylcholine (PC). These three components remained together in the liver with similar fractions of each in individual PC molecular species, most notably 1-stearoyl-2-linoleoyl-PC and dilinoleoyl-PC as well as PLPC. However, the palmitate component was spread among hepatic triglyceride, free fatty acid, other phospholipids, and all palmitate-containing molecular species of PC. Less than 2% of any administered PLPC component appeared in 1-stearoyl-2-arachidonyl-PC, the major species by mass in the liver. The palmitate component from plasma PLPC appeared in biliary PC at a more rapid rate than glycerol and linoleate components; the latter components appeared in bile in identical fashion. The results show that about two-thirds of plasma PLPC disappearance is due to phospholipase A1 hydrolysis, probably hepatic lipase. The putative produce, 2-linoleoyl-lysoPC, is efficiently reacylated with a saturated fatty acid in the liver, conserving PC.