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The unidirectional flux of 10- and 40-mM lactose was studied in newborn porcine jejunum and colon mounted in Ussing chambers. Polyethylene glycol 400 was used to measure passive paracellular permeability. The mucosal-to-serosal flux and the tissue accumulation of labeled lactose from the colon was similar to that of lactose-derived glucose from the jejunum. However, only jejunum showed a lactose-stimulated increase in short-circuit current. In jejunum, glucose was the sole sugar identified in the serosal bath, whereas in colon, only intact lactose was identified. Despite colonic lactose flux, polyethylene glycol oligomers were not found in the serosal bath, suggesting that they do not share the same route of absorption. Colonic lactose transport was nonsaturable between 1 and 40 mM. Under nongradient conditions, no net colonic lactose transport was observed. Cumulatively, these data suggest that the colon, unlike the jejunum, does not contain a glucose-galactose sodium cotransporter. The colon of the newborn piglet transports intact lactose at a flux equal to that of lactose-derived glucose by the small intestine, but by a different mechanism that is as yet undefined.

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OBJECTIVE: To describe the late manifestation of partial carbamyl phosphate synthetase I deficiency in an adolescent whose previous symptoms were not distinctive enough to suggest the presence of a metabolic disease. RESEARCH DESIGN: Clinical description of one patient. SETTING: Primary care children's hospital. PARTICIPANT: An adolescent boy. SELECTION PROCEDURE: Random observation. INTERVENTIONS: Intravenous sodium benzoate and sodium phenylacetate were more successful in reversing the coma than any other intervention. MEASUREMENTS/MAIN RESULTS: The patient has had no recurrence for 2 years, but he appears to have had a partial impairment of cognitive functioning. CONCLUSIONS: General pediatricians and intensivists should be aware that partial carbamyl phosphate synthetase I deficiency, and other partial urea cycle disorders, may become manifest in adolescence, even though they usually present in neonates or infants. When patients present in hyperammonemic coma, the urea cycle disorders should be considered, especially if no obvious cause is identified.

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Mechanisms for the assimilation of glucose polymers have been inferred from perfusion studies. To further define these mechanisms, the results of measurements of unidirectional glucose fluxes across short-circuited rabbit jejunal segments in vitro are reported. Glucose polymer-stimulated short-circuit current was similar to that of glucose [19 +/- 6.0 microA/cm2 (n = 7) and 26 +/- 5.7 microA/cm2 (n = 13), respectively] and was inhibited by both acarbose and phlorizin. Acarbose, an alpha-glucosidase inhibitor with no effects of glucose transport, was used to uncouple digestion from absorption. Mucosal-to-serosal flux of glucose polymer-derived glucose was lower than that of an equal weight/volume of glucose [124 +/- 62 nmol.h-1.cm-2 (n = 4) vs. 452 +/- 121 nmol.h-1.cm-2 (n = 6); P less than 0.05] and was inhibited by both phlorizin and acarbose. No glucose polymers were detected in the serosal bath solutions by thin-layer chromatography. It is concluded that glucose polymer-derived glucose is transported by a phlorizin-inhibitable process at a rate slower than that of free glucose, a finding that suggests that hydrolysis limits glucose polymer assimilation.

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Considerable discrepancies exist in the literature regarding the rates of glucose absorption from the common dietary disaccharides, lactose, maltose, and sucrose. This study compared the unidirectional flux of glucose derived from dietary disaccharides with that of their constituent monosaccharides in vitro. Lactose-stimulated short-circuit current (Isc) and mucosal-to-serosal flux (Jm----s) were lower than that of an equimolar glucose-galactose mixture and were phlorizin inhibitable. Maltose- and glucose-stimulated Isc were similar, but Jm----s of glucose derived from the hydrolysis of maltose was lower than that of free glucose. Sucrose-stimulated Isc and Jm----s were similar to that of an equimolar glucose-fructose mixture. Isc and Jm----s of glucose from both maltose and sucrose were phlorizin and acarbose inhibitable. We conclude that the rate of glucose uptake from disaccharides is less than or equal to that of free glucose and is dependent on the glucose source. We speculate that regulation of glucose uptake from disaccharides can occur at three sites: the hydrolytic enzyme, the glucose transporter, and the tight junctions.

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Piglets in three age groups (1-3, 9-11, and 16-25 days after birth) were used for in vivo colonic perfusions. Studies compared an isosmolar (312 mosM) with a high osmolar (551 mosM) solution and two equimolar substrates (with hexose concentrations of 73.1 mM), lactose and glucose-galactose. From the isosmolar perfusates, lactose absorption was 0.43 +/- 0.04 in the 18-20 day olds and 1.04 +/- 0.2 mumol.cm-1.min-1 in the 1-3 day olds; absorption from the glucose-galactose solution was negligible in all age groups (less than 0.05 +/- 0.05 mumol.cm-1.min-1). From the high osmolar perfusate, lactose absorption also exceeded that of glucose and galactose. In a third set of perfusion studies, the concentration of lactose was varied between 15 and 240 mM perfusate. Five-day-old animals absorbed 67% more lactose than 18-day-old animals; the right colon absorbed 57% more than the left. Lactose absorption, correlated with its concentration in the perfusate (r = 0.99), was nonsaturable at concentrations up to 240 mM, and was correlated with the uptake both of sodium (r2 = 0.59 for young and 0.64 for older neonates) and of chloride (r2 = 0.55 for young and 0.31 for older neonates). The results suggest that lactose may be removed from the colon without apparent cleavage by beta-galactosidase.

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Freezing is often used to retard bacterial enzymatic activity in fecal specimens collected to quantify specific carbohydrates. The effectiveness of freezer storage on preservation of lactose and glucose polymers was assessed. The data showed that more than 50% of lactose that was added to fecal supernatants that were stored without treatment for more than 50 days at -20 degrees C was lost. Adjustment of pH with HCl (pH 4.9), with HgCl2 (pH 6.3 or 5.85), or with NaOH (pH 10) improved carbohydrate preservation (P less than 0.0004). Storage of the supernatants of fecal homogenates lessened the loss of carbohydrate compared with the total homogenates (P less than 0.001). In supernatants, degradation occurred via simple hydrolysis; in homogenates, degradation occurred by hydrolysis and fermentation to a variety of end-products. Unprocessed fecal specimens that were frozen for months, then retrieved and incubated with lactose or glucose polymers showed extensive fermentative capacity. Cumulatively, the data indicate that enzymatic activity in feces is not halted by storage in the freezer, even if bacteria have been filtered from the stool.

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Our study was designed to determine whether substances that appear in the serum during the course of liver failure have a detrimental effect on the passive permeability of the blood-brain [blood-cerebrospinal fluid (CSF)] barrier. Lactic acid, octanoic acid, and ammonia were infused into rabbits for 4 h. The permeability changes of the blood-brain barrier were quantified by infusing polyethylene glycol 400 (PEG 400) and measuring the quantity and average mol wt of the PEG 400 that entered the CSF. The lipid solubility and effective diffusional radius of the PEG molecules were also quantified to provide greater precision for measurements using this probe. None of the animals receiving toxic infusions became seriously ill during the infusions. Low dose infusions of lactic acid, octanoic acid, and ammonia increased the effective pore diameter of the blood-brain barrier from 7.3 A to an average of 8.5 A. The amount of PEG entering the CSF increased from 1.7 to 4.0 (p less than 0.025), 4.7 (p less than 0.025), and 6.7 (p less than 0.001) mmol/L, respectively. Rabbits with galactosamine-induced liver failure had 10.1 mmol/L PEG 400 in the CSF (P less than 0.001) before any evidence of cerebral edema. These changes occur soon after these toxins accumulate in the plasma and may alone or together with other toxins account for the permeability changes that allow neurotoxic substances to enter the brain during hepatic disease and encephalopathies such as Reye's syndrome.

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To evaluate thiamine status in an urban adolescent population, we performed two investigations. In Study I, we compared whole blood thiamine levels in 101 healthy adolescents from varied racial backgrounds with those that had been obtained previously in 146 healthy white adults from a different geographic locale. Blood thiamine values were significantly lower in the adolescents as a group, but the differences were entirely due to the lower levels in the black adolescents. To explore further these differences (Study II), we compared thiamine status in 34 adolescents with that of their parents using measures of both whole blood thiamine content and of erythrocyte transketolase activity. White adolescents had significantly higher total whole blood thiamine values than black adolescents, and white parents had significantly higher thiamine values than black parents by both total whole blood assay and level of transketolase activity. There were no differences in thiamine status between adolescents and parents of the same race. Racial composition is an important variable to consider in population surveys of thiamine status.

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We measured the activity of several acid hydrolases in oligodendrocyte and mixed glial (predominantly astrocytic) cell cultures prepared from neonatal rat cerebra. When compared with the mixed glial cultures, the cultured oligodendrocytes exhibited higher levels for all the hydrolases when activities were normalized to protein content. When enzymic activities were examined as a function of DNA content, oligodendrocytic alpha-L-fucosidase, beta-D-glucuronidase, arylsulfatase, and N-acetyl-beta-D-glucosaminidase were higher than in mixed glial cultures, whereas the activities of alpha-D-glucosidase, beta-D-galactosidase and acid phosphatase were not elevated. These differences could not be accounted for by the fetal bovine serum present in the culture medium. The enrichment in acid hydrolase specific activities in the oligodendrocytes may be associated with a rapid turnover of at least some of the extensive myelin-like membrane formed by these cultured cells. Alternatively, the enrichment of acid hydrolase activity in the oligodendrocytes may be associated with intracellular vesicles of lysosomal origin which may play a role in myelin-like membrane assembly. Exactly which of the above two processes, or possible combinations thereof, is responsible for the present finding is not known.

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We have studied patients with Duchenne muscular dystrophy (DMD), DMD together with glycerol kinase (GK) deficiency, or DMD together with both GK deficiency and congenital adrenal hypoplasia (AHC). Analysis of deletions in these patients allows the mapping of these mutations in Xp21. The following order is proposed: Xpter - L1 - AHC - GK - DMD - Xcen. One of the boys with DMD, GK, and AHC is shown by pulsed-field-gel electrophoresis to have a deletion which has a proximal endpoint at least 500 kb distal from the pERT87 (DXS164) locus.

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Because mucosal glucoamylase is most active against glucose polymers less than 10 glucose units in length, longer chain polymers may not be completely absorbed by young infants. In order to investigate this possibility, the absorption and oxidation of 13C-rich glucose, short-chain (3 to 8 glucose units in length) and long-chain (average length 43 units) glucose polymers (GP) were compared in 12 healthy, 1-month-old infants. Recovery of the GP and glucose in stool was measured by determining the 13C enrichment of stool. The oxidation of the GP was measured by tracing the increase in breath 13CO2 after GP were fed. Carbohydrate malabsorbed in the small bowel was assessed by measurement of breath H2, a gas formed from the fermentation of carbohydrate in the colon. Analysis of the infants' stools revealed that one infant excreted 9.7% of the dose of glucose, another 6.7% of the dose of short-chain GP, and five infants excreted 2.6 to 18.5% (mean 8.4%) of the dose of long-chain GP. The percent of the administered dose recovered in breath was similar among substrates (mean = 28.7% of the dose fed). A rise in breath H2 greater than 20 ppm was found in four of the 12 infants after the feeding of glucose, in five of 12 after the short-chain GP, and in six of 12 after the long-chain GP. None of the infants developed diarrhea. The results suggest that healthy young infants do not absorb long-chain GP as completely as they absorb short-chain GP.(ABSTRACT TRUNCATED AT 250 WORDS)

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We evaluated the absorption of glucose polymers in canine jejunal Thiry-Vella fistulas proven to be free of pancreatic amylase. Medium-length oligomers with degrees of polymerization of 6 through 10 glucose units (DP 6-10) and long-chain material (DPavg23) were isolated from a cornstarch hydrolysate. We perfused 90, 180, and 360 mg/dl solutions of glucose, DP 6-10, and DPavg23 at 0.4, 1.9, and 3.4 ml/min. At all perfusion rates carbohydrate absorption decreased as the chain length of the oligomers increased, and these differences persisted even at the slowest perfusion rate employed. In two additional animals the fistulas were perfused at 3.4 ml/min with the three test carbohydrates at concentrations of 90, 180, 225, 270, 315, 360, 405, and 450 mg/dl. At this flow rate, the assimilative process of DP 6-10 and the long-chain fraction appeared to be saturated at carbohydrate concentrations above 360 mg/dl, whereas the absorption of glucose was linearly related to concentration throughout the range studied. With both groups of polymers, the fluid emerging from the fistula was virtually free of glucose, a finding that suggests that polymer digestion, not glucose absorption, is the rate-limiting step for polymer assimilation.

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To determine whether salivary amylase of premature infants can function as a surrogate for pancreatic amylase, we evaluated its production in the infant, acid resistance, and hydrolytic potency in a simulated oropharyngeal, gastric, and intestinal environment. The activity of salivary amylase in 11 prematures varied between 1 and 33 U/ml; the isozymic profile and acid resistance of the premature salivary amylase were identical to those of the enzyme of adults. A "modular" formula containing 7 g/dl of a 14C labeled long chain glucose polymer with degrees of polymerization ranging between 18 and 29 glucose units was prepared. Salivary amylase, 1.1 U/ml, was added to this formula. The progressive breakdown of the 14C polymers as the milk was subjected to oropharyngeal, gastric, and intestinal phase environments was evaluated by quantifying the liberation of short-chain oligomers from the 14C labeled substrates. The gastric pH was varied between 2 and 5 and the gastric incubation time was either 5 or 180 min. Substantial gastric phase breakdown only occurred after 3 h of exposure at the higher pHs of 4 (12%) and 5 (32%). During the intestinal phase, salivary amylase activity resumed. Prior gastric phase pH affected ultimate intestinal phase breakdown, p less than 0.001; after 5-min gastric phases at pHs ranging from 2 to 5, the intestinal phase breakdown ranged from 17 to 55%. We conclude that the limited salivary amylase in the saliva of premature infants can produce significant glucose polymer digestion in both the stomach and small intestine but the digestion falls substantially short of that accomplished by usual concentrations of pancreatic amylase.

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We report the results of a randomized comparison of two intravenous safflower oil (fat) emulsions in neonates who required total parenteral nutrition. The fat emulsions differed only in their content of alpha-linolenic acid: in one emulsion the alpha-linolenic acid content of the oil was 0.1% of fatty acids, while in the other emulsion the alpha-linolenic acid content of the oil was 3.0 +/- 1.5% (SD) of fatty acids. Group 1 and 2 patients received the "low" and "high" alpha-linolenic acid emulsions, respectively. Ten patients were studied in each group. The mean daily fat dosage was 1.70 g/kg in patients of Group 1 and 1.56 g/kg in those of Group 2. No significant difference in the clinical effects of either fat emulsion could be detected between the two study groups. Both emulsions prevented or corrected biochemical signs of essential fatty acid deficiency. The major statistically significant difference between study groups was in the level of alpha-linolenic acid and its metabolite, eicosapentaenoic acid. After 2 weeks of fat therapy, these fatty acids were increased in the high alpha-linolenic acid group; however, another metabolite of linolenic acid, docosahexaenoic acid, decreased during intravenous fat therapy in both study groups. Both study groups had significantly decreased arachidonic acid levels and increased linoleic to arachidonic acid ratios.

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