bluuebunny/crossref_metadata_2025_split huggingface dataset:
α‐Amylase in Preterm Human Milk
Authors: Jones Jay B. , Mehta Nitin R. , Hamosh Margit 
Published: February 2024

SummaryWe have measured α‐amylase activity in the milk of 18 women who delivered at 25–40 weeks of pregnancy: 25–30 weeks (n = 7), 31–35 weeks (n = 5), and 36–40 weeks (n = 6). In each subject, α‐amylase activity in milk was measured 3 days postpartum (colostrum) and at 1. 3, and 6 weeks of lactation. In each of these three groups, α‐amylase activity was high in colostrum (mean values of 8,973 ± 697, 4.422 ± 1.000, and 3,550 ± 889 U/L. respectively) and decreased gradually during the following 6 weeks of lactation (mean values of 2.007 ± 413. 1,462 ± 465, and 1,373 ± 400 U/L, respectively. after 6 weeks). The decrease in total α‐amylase activity during this time period was not accounted for by a similar decrease in total milk protein since the specific α‐amylase activity in colostrum (142.3 ± 15.9 U/g protein) was substantially greater than that measured in milk obtained 6 weeks postpartum (103.4 ± 12.6 U/g protein). The α‐amylase in pooled preterm human milk was relatively stable (22°r loss of activity) when preincubated at a pH of 3.0 for 2 h at 37°C. The activity of the enzyme in preterm human milk had a broad pH optimum in the range of 5.0–7.0. Thus, the enzyme can pass through the stomach of the infant after a milk meal without substantial inactivation because of acidity (pH 3.5–6.5). and has maximum catalytic activity at a pH in the range of that found in the duodenum (pH 6.0–7.0).
Cardiac Arrest in Two Children with Nonfamilial Chronic Intestinal Pseudoobstruction on Total Parenteral Nutrition
Authors: Anuras Sinn , Mitros Frank A. , Shirazi Siroos S. , Soper Robert T. , Younoszai Kabir , Green Jennifer B. 
Published: February 2024

SummaryTwo children with nonfamilial chronic intestinal pseudoobstruction are reported. Both had no family history. They had an exploratory laparotomy to rule out mechanical intestinal obstruction, and required long‐term parenteral nutrition to obtain sufficient nutrients. These two children had dilatation of the whole length of the small intestine, which differs from short segmental dilatation (megaduodenum) in patients with familial chronic intestinal pseudoobstruction. There were also differences in the histology of the gastrointestinal tract between these two patients although they had similar clinical manifestations. Both patients died from cardiac arrest, one after 2 years and the other after 4 months on long‐term parenteral hyperalimentation. At autopsy, heart examination was normal in one patient, and a small infarction (0.4 mm diameter) was found in the other. Although mild hypokalemia was found in one case, and mild hyperkalemia in the other, the cause of cardiac arrest in these two children is not known.
Progressive Esophageal Dysfunction in Chronic Granulomatous Disease
Authors: Markowitz James F. , Aronow Eugene , Rausen Aaron R. , Aiges Harvey , Silverberg Mervin , Daum Fredric 
Published: February 2024

SummaryChronic granulomatous disease of childhood (CGD), a hereditary disorder of neutrophil function, affects the gastrointestinal tract in a variety of ways. Esophageal involvement has only rarely been reported. An 11‐year‐old boy with CGD and progressive esophageal dysmotility is described. Repeated radiographic, endoscopic, and motility studies revealed a markedly atonic esophagus with varying function of the lower esophageal sphincter. Pharmacologic therapy and esophageal dilatations were unsuccessful in establishing adequate esophageal function. A feeding gastrostomy was required for nutritional support.
Changes in the Lipids of Human Milk from 2 to 16 Weeks Postpartum
Authors: Clark Richard M. , Ferris Ann M. , Fey Mark , Brown Peter B. , Hundrieser Kenneth E. , Jensen Robert G. 
Published: February 2024

SummaryChanges in total lipid, fatty acids, total cholesterol, free cholesterol, and lipid phosphorus in mature milk with time were investigated. Milk samples were collected from 10 mothers at 2, 6, 12, and 16 weeks postpartum. During 1 day, each mother donated two complete breast expressions. Expressions were taken using an electric breast pump 1 h after the previous a.m. and p.m. nursing. For each mother, the a.m. and p.m. samples were pooled for analysis. It was observed that the amount of total lipid increased significantly (p < 0.05) from 3.9g/100 ml at 2 weeks to 5.2 g/100 ml at 16 weeks postpartum. The total fatty acid composition remained uniform during the investigation. Average total cholesterol and free cholesterol in the milk were 10.3 mg/100 ml and 8.3 mg/100 ml, respectively. These concentrations did not change significantly with time postpartum. Average lipid phosphorus was 3.9 mg/100 ml and also remained constant throughout. We conclude that the fatty acid pattern, lipid phosphorus, total cholesterol, and free cholesterol of mature milk to 16 weeks postpartum remains relatively constant while total lipid concentration increases.
Lipases in Preterm Human Milk
Authors: Mehta Nitin R. , Jones Jay B. , Hamosh Margit 
Published: February 2024

SummaryTerm human milk contains two lipases: Bile salt stimulated lipase (BSSL—this lipase also has bile salt stimulated esterolytic activity, BSSE) and lipoprotein lipase (LPL). We have measured the activity of these lipases in preterm (25–36 weeks' gestation) and term (37–40 weeks' gestation) human milk collected from the time of parturition until 3 months of lactation. BSSL levels are similar in preterm and term milk and do not vary significantly as a function of length of lactation. In the three groups of women who delivered at 26–30, 31–36, and 37–40 weeks of gestation, BSSL levels were 22.5 ± 6.6, 21.0 ± 3.6. and 32.3 ± 7.6 U/ml in colostrum and 26.3 ± 1 1.4, 31.1 ± 3.4, and 33.3 ± 10.7 U/ml in milk collected at 3 months of lactation, respectively. These observations suggest that preterm milk has the same fat digesting potential as term milk. BSSE activity decreased as a function of length of gestation and lactation, 61.2 ± 8.78, 44.4 ± 4.6, and 44.9 ± 13.3 U/ml in colostrum and 40.8 ± 5.0, 35.6 ± 9.5, and 23.3 ± 3.4 U/ml in milk collected after 3 months of lactation in the three groups studied. In all milks examined, the esterase lost its bile salt dependency upon storage at ‐ 10°C for periods of 2 weeks, suggesting that spontaneous hydrolysis of milk fat and accumulation of free fatty acids can occur in milk stored at ‐ 10°C. LPL activity increased as a function of length of gestation and lactation. LPL activity increased from 12.2 ± 3.3 U/ml in colostrum to 101.6 ± 12.6 U/ml in milk collected at 6 weeks of lactation from the preterm mothers and from 17.0 ± 9.7 U/ml in colostrum to 270 ± 15.5 U/ml in milk collected at 3 weeks of lactation from mothers of term infants. Our data suggest: (a) Milk secreted by women who deliver premature infants (26–36 weeks' gestation) has the same fat digesting potential as milk secreted by mothers of term infants, (b) The esterolytic component of the BSSL develops earlier than the lipolytic activity, (c) “Drip ” milk has higher LPL activity than milk pumped concomitantly from the other breast, suggesting that there is no relationship between mammary cell damage and LPL release, (d) Since BSSL and BSSE activities are 200− to 3,000‐fold higher in human milk than LPL activity, and since BSSE loses its bile salt dependency during storage at −10°C, the rise in free fatty acid levels in banked milks could be related to BSSE rather than to LPL.
Use of Psyllium in the Management of Chronic Nonspecific Diarrhea of Childhood
Authors: Smalley James R. , Klish William J. , Campbell Mary Ann , Brown Marilyn R. 
Published: February 2024

SummaryThe etiology and treatment of chronic nonspecific diarrhea of childhood is still poorly understood. We evaluated 23 children with this disorder in whom other etiologies of chronic diarrhea had been ruled out. The children were treated first with an unrestricted diet for 1 week, and if no response was obtained they were then treated with psyllium bulk agents for 2 weeks (1 tablespoon b.i.d.). Eighty‐seven percent of the patients responded to therapy. Seven patients responded to an unrestricted diet only, and 13 responded to psyllium. Only three patients (13%) did not respond. Most children were on a restricted diet at presentation, but the amount of dietary fat intake did not correlate with the response, contrary to other reports. Treatment with normalization of the diet and psyllium bulk agents seems to be an effective mode of therapy for chronic nonspecific diarrhea of childhood.
Gastroschisis
Authors: Kekomäki Martti , Savilahti Erkki , Louhimo Ilmo 
Published: February 2024

SummaryThirty‐nine newborns were treated for gastroschisis at the Children's Hospital. Helsinki University Central Hospital from 1964 to 1980. Sixteen infants died during the first hospital admission. Of the 23 survivors, II were two years or older, and these 11 children were subjected to a follow‐up examination. The incidence of the lesion increased markedly during the period of observation. Dysmaturity (birth weight at or below the 10th percentile of normal intrauterine growth) accompanied gastroschisis in 17 cases, and a low birth weight (below 2,500 g) in 23 cases. The mortality of gastroschisis was clearly and constantly decreasing, being 54% for the first decade, 23% for the last 5 years, and 41% for the total series. The age of the mothers at parturition, 20.9 years, was significantly (p < 0.001) lower than the Finnish average. The extensive clinical and laboratory analysis of the 11 oldest children with completed reconstruction of the abdominal wall indicated good physical condition and, specifically, normal absorptive function of the intestine.
Nonspecific Suppressor Cell Activity and Lymphocyte Response to β‐Lactoglobulin in Cow's Milk Protein Hypersensitivity
Authors: Weil S. , Kuperman O. , Ilfeld D. , Finelt M. , Freier S. 
Published: February 2024

Summaryβ‐Lactogloblin (BLG) is clinically the most frequent allergen in cow's milk protein hypersensitivity (CMPH). We therefore assessed its in vitro blastogenic effect on lymphocytes of patients suffering from CMPH. Twenty‐two patients aged from 1 to 20 months were investigated, β‐Lactoglobulin produced a significantly (p < 0.00003) higher blastogenic response in lymphocytes of patients with CMPH (mean stimulation index 7.7 ± 0.7 SEM) than in 26 age‐matched controls (2.7 ± 0.4). This response was age related, being most marked in infants up to 5 months of age. Lymphocyte proliferation following stimulation with BLG can be a useful in vitro test in the diagnosis of CMPH. Because of this augmented lymphocyte response, we considered the possibility of a related defect in immune suppression. Nonspecific concanavalin A‐induced suppressor cell activity was assessed in the above patient and control populations. Concanavalin A‐induced suppressor cell activity was significantly (p < 0.05) reduced in patients with CMPH. This too was most marked in the first few months of life. We suggest that the development of CMPH may be due to delayed maturation of a suppressor cell population.
Maturational Effect of Triiodothyronine and Its Analog 3,5‐Dimethyl‐3'‐isopropyl‐L‐thyronine on Sucrase Activity in the Small Intestine of the Developing Rat
Authors: Vaucher Yvonne E. , Anna Judy A. , Lindberg Robert F. , Jorgensen Eugene C. , Krulich Ladislav , Koldovsk Otakar 
Published: February 2024

SummaryIn this study we compared the effect of 3,5‐dimethyl‐3'‐isopropyl‐L‐thyronine (DIMIT) with that of triiodothyronine (T3) on sucrase activity in the small intestine of the fetal and suckling rat. DIMIT (5 μg/100 g BW) was injected into pregnant Sprague—Dawley rats on days 17–20 of gestation and DIMIT (10 and 50 μg/100 g BW) and T3 (10 and 50 μg/100 g BW) were given subcutaneously to two groups of sucklings on days 4–7 and 11–14. Fetal animals were sacrificed on day 21 of gestation; sucklings on days 8 and 15. Sucrase activity in the proximal, middle, and distal small intestine was assayed. DIMIT and T3 suppressed serum thyroid‐stimulating hormone (TSH) in the fetus and suckling. Sucrase activity was increased in all small intestinal segments in the fetal and older suckling groups. In the younger sucklings the effect was seen only in the middle and distal segments. In both fetal and suckling groups the sucrase response was dose related. Thus, DIMIT has a thyromimetic effect on development of sucrase activity. The lack of sucrase reactivity in the proximal segment of the younger (8‐day‐old) sucklings to the administration of either DIMIT or T3 is interesting but unexplained.
Characteristics of Intact Lactose Transport in Rat Jejunum In Vitro
Authors: Oyesiku J. E. J. , Muller D. P. R. , Harries J. T. 
Published: February 2024

SummaryThe characteristics and kinetics of intact transmucosal transport of lactose have been studied in the rat jejunum using an everted sac preparation. Lactose transport displayed linear kinetics up to the maximum concentration (150 m M) of lactose studied. Ouabain (1 m M) when placed in the serosal solution, and phlorhizin (0.1 m M) when placed in the mucusal solution, had no significant effect on lactose transport. In contrast to glucose, transport of intact lactose did not occur against a concentration gradient. These results strongly suggest that intact transmucosal transport of lactose occurs by a process of simple passive diffusion, and that it is independent of the sodium‐coupled glucose transport system.
Calcium Metabolism in Young Rats During Early Dietary Phosphate Deprivation
Authors: Urban Ernest , Beck Nama , Reineck H. John , Lee Scott 
Published: February 2024

SummaryA series of in vivo experiments were designed to study the early effects of phosphate depletion on calcium metabolism in young adult (8–10 week old) rats with intact thyroid‐parathyroid glands. Hypercalciuria occurred within 24 h of dietary phosphate deprivation. It was the consequence of mobilization of calcium stores from the exchangeable calcium pool of the diaphysis of bone. Increased net intestinal absorption of calcium also occurred early. This was at least in part due to decreased net intestinal secretion of calcium into the gut lumen. Thus the early onset of hypercalciuria and hypercalcemia associated with Pi deprivation in normal growing young rats is the combined result of increased net intestinal calcium absorption and increased calcium mobilization from bone.
Failure of p‐Aminobenzoic Acid Screening Test to Diagnose Pancreatic Insufficiency in Shwachman's Syndrome
Authors: Kai Hiroshi , Nose Osamu , Harada Tokuzo , Maki Ichiro , Tajiri Hitoshi , Yabuuchi Hyakuji 
Published: February 2024

SummaryThe 6‐h urine recovery of p‐aminobenzoic acid (PABA) following the administration of a standard dose of N‐benzoyl‐L‐tyrosyl‐p‐aminobenzoic acid (BTPABA) was performed in 13 control subjects and two siblings with Shwachman“s syndrome. The control subjects showed a recovery of 67 ± 12.1% (mean ± 1 SD) of the administered dose, consistent with previously reported values. Unexpectedly, the recovery of PABA in two siblings with Shwachman's syndrome was found to be 67 and 63%, respectively. The values are well within the normal range. In these siblings, fecal chymotrypsin activities were very low when measured with N‐acetyl‐L‐tyrosyl‐ethyl ester (ATEE) as substrate, but were normal when BTPABA was the substrate. The duodenal juice of the younger affected child following pancreozymin‐secretin stimulation showed very low chymotrypsin activity against ATEE, BTPABA, and N‐benzoyl‐L ‐tyrosyl‐ethyl ester. These findings suggest that there may be BTPABA‐splitting activity in the lower bowel of these siblings with Shwachman's syndrome. This activity might be that of enteric bacteria or of the intestinal mucosa.
Changes in Body Composition and Muscle Protein Degradation During Nutritional Supplementation in Nutritionally Growth‐Retarded Children with Cystic Fibrosis
Authors: Shepherd R. W. , Thomas B. J. , Bennett D. , Cooksley W. G. E. , Ward L. C. 
Published: February 2024

Summary:Changes in body composition and muscle protein degradation were studied in seven nutritionally growth‐retarded children with cystic fibrosis (CF) before and after nutritional supplementation and in eight healthy children who served as controls. Supplemental feedings consisted of a peptide formula that increased dietary protein and energy intakes approximately 20‐40% over a 6‐month period, delivered either as oral supplement or overnight intragastric feeding. Body composition was assessed by anthropometric data and measurements of whole body potassium (40K) and creatinine excretion. Muscle protein degradation was measured by urinary 3‐methyl‐histidine excretion, an index of myofibrillar protein catabolism. Compared with controls, CF children had significantly reduced body mass, body fat, and muscle mass, and a significantly increased rate of myofibrillar protein degradation. With nutritional supplementation, significant catch‐up weight gain and improved linear growth were observed with evidence of accretion of lean body mass and muscle mass, and in all but one severely malnourished patient with progressive disease, there was normalization of the high rate of muscle protein degradation. Thus, this form of nutritional therapy has significant benefits in terms of body protein accretion and myofibrillar protein degradation.
Metal‐Binding Ligands in Viokase
Authors: Martin Mark T. , Jacobs Francis A. , Brushmiller George J. 
Published: February 2024

Summary:The object of this study was to detect low molecular weight zinc binders in Viokase®, a porcine pancreatic preparation used in the treatment of variant acrodermatitis enteropathica (AE). Extracts were centrifuged and ultrafiltered to yield a soluble fraction containing solutes of molecular weights ≤500 daltons. These were studied by modified gel chromatography (MGC) using sealed Sephadex G‐15‐120 columns equilibrated with buffered solvent systems containing either 5 ppm copper (II) or 5 ppm zinc (II). Eluted fractions were assayed for these metals by atomic absorption and for amino acids by various techniques. MGC profiles of ultrafiltrates resulted in a peak consisting of glutamic acid and another peak consisting of several amino acids. A third peak was due to metal ions. Citrate and picolinate have been claimed to be important zinc‐binding ligands in Viokase, but neither was detectable by MGC, although small amounts of citrate or similar compounds were detected by the Furth and Herrmann reaction. Since amino acid ligands are easily detectable by MGC, and metal distribution among ligands is competitive in nature, the possibility of a role for citrate or picolinate as a zinc transporter in Viokase is seriously reduced. Relief to the variant AE patient was probably not due to citrate‐ or picolinate‐enhanced zinc uptake.
Influence of Genetic Factors on the Serum Levels of Antigliadin Antibodies in Celiac Disease
Authors: Mearin Maria Luisa , Koninckx Carmen Ribes , Biemond Izak , Polanco Isabel , Peña A. Salvador 
Published: February 2024

SummaryThe relationship between titers of antigliadin antibodies (IgA class and total immunoglobulins IgA, IgG, and IgM) and HLA‐DR antigens was studied in two random samples of unrelated Spanish children with celiac disease. One of these groups had been on a gluten‐free diet for at least 6 months and the other had been given a similar regimen followed by at least 6 months on a gluten‐containing diet. Children with celiac disease who had the DR7 antigen but not DR3 showed significantly higher antigliadin antibody titers on a gluten‐containing than on a gluten‐free diet, whereas those with DR3 but not DR7 did not show this difference. With respect to DR phenotypes, the children with DR3/DR7 and DR7/DR5 phenotypes had significantly higher antibody titers while consuming gluten than when on a gluten‐free diet. However, children with celiac disease with the DR31DR3 phenotype showed similar antigliadin antibody titers on both diets. The present results suggest that genetic factors in the DR region influence the humoral immune response to gliadin in celiac disease.
Fecal and Biliary Bile Acid Patterns in Children with Bile Acid Malabsorption
Authors: Tazawa Yusaku , Yamada Masaaki , Nakagawa Michiko , Suzuki Hiroshi , Igarashi Yutaka , Konno Tasuke , Tada Keiya 
Published: February 2024

SummaryBile acid metabolism was examined in two children with bile acid malabsorption, who were being treated with intravenous hyperalimentation. Fecal bile acid excretion was 1,261 μmol/m2/day in a child with bile acid malabsorption of unknown origin, and 1,877 μmol/ m2/day in a child with secondary bile acid malabsorption after an operation for long‐segment aganglionosis. These values were approximately 10 times higher than those in diarrheal or nondiarrheal children without apparent abnormalities in bile acid metabolism. Fecal bile acids in these patients with bile acid malabsorption were almost completely conjugated, with little unconjugated bile acid present. It is possible that the disturbed bile acid deconjugation in the intestine might be caused by a rapid intestinal transit time, which was found in our patients with bile acid malabsorption. In the analysis of biliary lipid composition, children with bile acid malabsorption were shown to have a chenodeoxycholate‐dominant pattern, an increased glycine‐ to taurine‐conjugated bile acid ratio, and markedly supersaturated cholesterol. Such profiles may be related not only to bile acid malabsorption but also to cholestasis, presumably due to intravenous hyperalimentation.
Plasma Selenium Concentration in Healthy Japanese Children and Adults Determined by Flameless Atomic Absorption Spectrophotometry
Authors: Hatano Shuichi , Nishi Yoshikazu , Usui Tomofusa 
Published: February 2024

SummaryThis study showed a rapid and direct method for determining selenium concentration in plasma by flameless atomic absorption spectrophotometry, and differences in plasma selenium concentration in healthy children and adults. A direct method is possible, since selenium is heat stable in the presence of nickel. With this method, the recovery of selenium added to plasma was 100.3 ± 5.7%, and the relative standard deviation in repeated determinations of pooled plasma selenium was 3.0% and 6.8%. The plasma selenium concentration in adults was 99.4 ± 12.5 ng/ml, lower than reported concentrations from the United States and Canada, and higher than those from New Zealand. These variations may reflect dietary habits, bioavailability of selenium compounds in diet, racial difference, or different analytical methods. The mean concentration of plasma selenium at 1 to 6 months of age (51.0 ± 13.1 ng/ml) was significantly lower than in adults (p < 0.001); it increased gradually and steadily to the adult level with age. This age‐related difference of plasma selenium level is similar to that reported previously.