bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Impact of Nutrition in Cystic Fibrosis
Authors: Gaskin Kevin John 
Published: January 2024

SummaryA high energy intake, compensating for mal‐absorption, and the energy cost of lung disease, lung infections, and the underlying metabolic abnormality, is required to ensure normal growth in patients with cystic fibrosis. This goal can be readily achieved by adherence to a high quantity, normally balanced diet (with 40% of the energy as triglycerides of long‐chain fatty acids). In contrast, this goal cannot be reached in the majority of patients adhering to low‐fat and thus low‐energy‐containing diets, which almost inevitably lead to malnutrition with wasting and stunting. The prevention of malnutrition may well have considerably enhanced the prognosis of patients at one clinic. Further work is needed to define the interrelationship of nutrition and lung disease, and to define the appropriate nutrient requirements induced by the lung disease per se, recurrent infections, and the underlying disease process.
Efficacy of Pancreatic Enzyme Supplementation in Children with Cystic Fibrosis
Authors: Ansaldi‐Balocco Nicoletta , Santini Bruna , Sarchi Claudio 
Published: January 2024

SummaryTwo studies were performed in children with cystic fibrosis (CF) to compare the efficacy and tolerability of pancreatic enzymes prepared as enteric‐coated microspheres with that of a conventional enzyme preparation. The parameters evaluated included the following: fecal fat excretion, coefficient of fat absorption, daily caloric intake, percent of diet as fats, proteins and carbohydrates, increase in height and weight, frequency and consistency of stools, palatability of the preparation, and patient compliance. The first study was an open randomized crossover trial of an enteric‐coated microsphere preparation (Pancrease) versus conventional pancreatin given alone or with cimetidine. With Pancrease compared to conventional pancreatin, a significant improvement was observed in all the digestive parameters in addition to better patient compliance. In the second study, the response of a group of CF patients given Pancrease for at least 3 months (3–67 months) was retrospectively compared with the response to conventional pancreatin, which had been given to the same patients from 10 months to 8 years earlier. In comparison to conventional pancreatin, Pancrease provided better digestive efficacy and greater increases in the growth rate of teenage patients. With Pancrease, the number of daily dosage units is decreased even when fat intake is increased. No adverse reactions were seen with either of the enzyme preparations used in these studies.
The Effect of Aminophylline on Gastrointestinal Blood Flow and Oxygen Metabolism in the Conscious Newborn Lamb
Authors: Cronin C. M. G. , Canose J. , Buchanan D. , Holzman I. R. 
Published: January 2024

SUMMARYAminophylline, widely used in the neonatal period, has been reported to be associated with necrotizing enterocolitis. We hypothesized that aminophylline might alter gastrointestinal blood flow and oxygen delivery. The effects of this drug on gastrointestinal (GI) blood flow and oxygen delivery, consumption, and extraction were examined using the radioactive microsphere method in ten chronically catheterized, unanesthetized lambs aged 5–12 days. While cardiac output and systemic oxygen consumption did not change, we found statistically significant increases (p < 0.05) in small intestinal blood flow as a function of lamb weight and as a percentage of cardiac output. Splanchnic oxygen consumption also increased significantly. The oxygen reserve available to the GI tract can be compromised by aminophylline at levels similar to those used in the human neonate for treatment of apnea.
Enteropathy and Renal Involvement in an Infant with Evidence of Widespread Autoimmune Disturbance
Authors: Mitton S. G. , Mirakion R. , Larcher V. F. , Dillon M. J. , Walker‐Smith J. A. 
Published: January 2024

SUMMARYAn 8‐month‐old infant presented with a 1 month history of protracted diarrhea, vomiting, and weight loss. Small intestinal biopsy showed a flat mucosa and there was no clinical improvement with gluten, cow's milk protein, and disaccharidase‐free diet. Serial testing for autoantibodies revealed persistent autoantibodies to gut epithelial cells and to renal brush borders; on two occasions, atypical liver‐kidney microsomal antibodies were detected. Treatment with steroids produced clinical improvement but the patient finally succumbed with a combination of gut and renal dysfunction. The widespread nature of the antibodies, with clinical involvement of gut, liver, and kidney, suggest an underlying autoimmune mechanism for the pathogenesis of the condition. Serial autoantibody measurements may provide a means to monitor the disease progress and may be a guide to treatment.
Measurement of Plasma Prednisolone Level to Evaluate a Prednisone Treatment Failure in an Adolescent with Crohn's Disease
Authors: Milov David E. , Hill Malcolm , Andres Joel M. , Cynamon Harry A. , Hendeles Leslie 
Published: January 2024

SUMMARYA 14‐year‐old boy experienced multiple hospitalizations because of symptoms due to Crohn's disease involving the stomach, duodenum, and ileum. He maintained that oral corticosteroids were not effective for control of his symptoms. However, i.v. corticosteroids always relieved his symptoms. To resolve the question of noncompliance versus altered corticosteroid absorption or metabolism, our patient underwent an oral prednisone absorption study. Prednisolone, the active metabolite of prednisone, was measured in his plasma using a high‐pressure liquid chromatography technique. The results led to the discovery of an elaborate deception by the patient and his subsequent need for psychotherapy. This report documents the importance of measuring plasma prednisolone concentrations to diagnose noncompliance, especially in adolescents who are overly concerned about their body image.
Effects of Two Levels of Intake of Chloride, Potassium, and Calcium on Mineral and Acid‐Base Metabolism in Premature Infants
Authors: Mosca F. , Manz F. , Primi D. , Marini A. 
Published: January 2024

SummaryGrowth (weight, length, mid‐upper arm circumference), acid‐base status, serum electrolyte levels, and selected parameters of urine (creatinine and electrolyte levels, urine pH, renal net acid excretion, levels of metabolites of aldosterone) were determined serially from the 10th to the 32nd days for 4 weeks in 21 premature infants (birth weight, 1,100–2,000 g) fed either formula A or formula B (formula B was formula A supplemented with chloride, potassium, and calcium). Premature infants fed formula B showed a higher weight gain (31 versus 28.2 g/day), a higher increment of middle‐upper arm circumference (0.31 versus 0.24 cm/week), and a decreased renal net acid excretion (1.24 versus 1.92 mEq/ kg/day). Almost all premature infants fed formula A and some with a high growth rate receiving formula B showed hypochloruria corresponding to chloride deficiency. Premature infants fed unsupplemented humanized formulas may have an inadequate intake of minerals.
Small Intestinal Development in Growth‐Retarded Fetal Sheep
Authors: Avila C. G. , Harding R. , Rees S. , Robinson P. M. 
Published: January 2024

SummaryThe functional maturity of the gastrointestinal system is essential for the survival of the neonate. The effects of reducing placental size and the ensuing fetal growth retardation on the development of the small intestine (SI) of the sheep fetus were investigated. At 140 days of gestation (term is 147 days), fetal body weight, gastrointestinal weight (from the abomasum to the rectum, i.e., the gut), SI weight, and SI length in the growth‐retarded fetuses (n = 6) were significantly reduced (p < 0.05) when compared with age‐ and breed‐matched control fetuses. The SI weight was disproportionately reduced as a fraction of the gut weight or SI length (p < 0.01). The proximal SI showed marked reductions in the thickness of the wall, muscularis externa, mucosa, villus height, and crypt depth (p < 0.003). Densities of villi and crypts were also reduced (p < 0.02). The crypt‐to‐villus ratio was maintained. Glycogen was accumulated basally in the villus epithelial cells, and their nuclei were located apically. These characteristics are comparable to those of much younger fetuses. In the distal SI, interanimal variation was large; however, the mean thickness of the wall, the muscularis externa, the mucosa, and the villus height were all reduced (p < 0.05). In this region, the density of villi and crypts, the pattern of glycogen accumulation, and the position of the nuclei did not markedly differ between growth‐retarded and control fetuses. Villus cell density was increased (p < 0.01). Thus, fetal growth retardation due to placental insufficiency affects the proximal and distal SI with different degrees of severity, with thinning of the SI wall, a reduction of the surface area available for absorption, reduced epithelial regenerative capacity, and possibly a retardation of normal epithelial maturation.
Electrogenic Sodium Transport in the Developing Rabbit Cecum
Authors: Potter Gregory D. , Burlingame Susan M. , Cragoe Edward J. , Seilin Joseph H. 
Published: January 2024

SummaryThe adult rabbit cecum absorbs Na by an electrogenic, C1‐independent process that is inhibited by the amiloride analogue phenamil. In the colon, this transport system is unique to the cecum. Because the developing colon exhibits many specialized functions, we have now examined the development of electrogenic Na transport in the newborn rabbit. Cecal tissue from animals between 7 and 38 days old was mounted in modified Üssing chambers for measurement of Na and C1 flux (J) and the short‐circuit current (Isc). At 7–10 days, the (Isc) was only 0.8 ± 0.18 μEq cm ‐2 h‐1, but by 35–38 days it had increased to 4.6 ± 0.79 μEq cm ‐2 h ‐1. The Na transport increased in parallel with Isc; JNanet = −0.49 ± 0.33 μEq cm‐2 h‐1 at 7–10 and 4.7 ± 1.6 (μEq cm‐2 h‐1 at 35–38 days. The Na transport was not inhibited by phenamil (10‐4 M) at 7–10 days, but by the 35–38‐day period, phenamil reduced the JNanet to 1.6 ± 0.37 μEq cm‐2 h‐1. The CI secretion was not stimulated by theophylline in the 14–16‐day‐old cecum, nor was Na absorption stimulated by epinephrine. The rabbit cecum does not demonstrate electrogenic Na absorption until after the 4th week of life.
Sulfasalazine‐Induced Hepatotoxicity in Children with Inflammatory Bowel Disease
Authors: Boyer Debra L. , Li B Ulysses K. , Fyda John N. , Friedman Roger A. 
Published: January 2024

SummaryA case of severe sulfasalazine‐induced hepatotoxicity is reported in a 14‐year‐old girl with Crohn's disease. Fourteen days after beginning sulfasalazine, she developed a systemic reaction characterized by high fever, maculopapular rash, lymphadenopathy, abdominal pain, and malaise, with tender hepatomegaly and elevated liver functions, leukemoid reaction, with eosinophilia, and immune complexes. She responded promptly to high‐dose intravenous steroids with complete recovery. This case was compared to the three similar pediatrie cases in the literature. The latent period was 11–19 days between sulfasalazine therapy and the onset of high fever, generalized lymphadenopathy, and pruritic maculopapular rash that spared palms and soles and resolved with desquamation. All children had hepatotoxicity indicated by tender hepatomegaly, elevated liver enzymes, and histologie inflammation on liver biopsy. The similarity between these features and those of other sulfa‐induced hepatotoxic reactions suggests that the sulfapyridine moiety is the etiologic agent in these hypersensitivity reactions. Those children with circulating immune complexes responded well to steroids. Proper therapy for this rare but severe idiosyncratic reaction includes prompt recognition and discontinuation of sulfasalazine, and high‐dose cortico‐steroid therapy.
Milk Lactose Malabsorption in Gabon Measured by the Breath Hydrogen Test
Authors: Gendrel Dominique , Dupont Christophe , Richard‐Lenoble Dominique , Gendrel Christine , Nardou Marline , Chaussain Michèle 
Published: January 2024

SummaryIt is usual to consider that the greatest part of the black African population is lactose intolerant. Also, milk lactose malabsorption was studied by a breath hydrogen technique in 87 Gabonese children and 20 Gabonese adults (central Africa). The prevalence of malabsorption was 64.2% in rural schoolchildren, 65% in the urban hospitalized, and 60% in adults. Twelve children and six adults had clinical symptoms after a lactose load. All subjects were Bantus, with no tradition of consuming dairy products. These data must be considered in programs of nutritional support in Africa.
Phenobarbital Can Aggravate a Cholestatic Bile Acid Pattern in Infants with Obstructive Cholangiopathy
Authors: Nemeth Antal , Wikströ Sten‐Åke , Strandvik Birgitta 
Published: January 2024

SummaryThe effect of phenobarbital on urinary bile acid excretion in intrahepatic cholestasis was studied in four boys 4–43 months of age who received 10 mg/kg of body weight of phenobarbital for a period of 3 weeks‐3 years. One child was observed at two different periods: with and without histologically proven cirrhosis. Before the treatment period, the infants excreted 10‐fold higher amounts of bile acids in urine than healthy children. The primary bile acids predominated, and there were also increased amounts of polyhydroxylated bile acids, 3β‐hydroxy‐5‐cholenoic acid, and ketonic bile acids but small amounts of secondary bile acids. After the phenobarbital treatment, the patients further increased their urinary bile acid excretion, including all kinds of bile acids except the secondary ones. The sulfated fraction did not increase in absolute amounts, and its relative percentage decreased from a mean of 60–33%. Liver function test results generally did not improve, although serum concentration of bilirubin decreased. Most of these changes suggested a worsening of the cholestatic state after phenobarbital treatment. The results indicate that at our present state of knowledge, phenobarbital should not be given routinely to infants or children with intrahepatic cholestasis.
Prostaglandin and Fatty Acid Metabolism in Patients with Extrahepatic Biliary Atresia
Authors: Shimizu Toshiaki , Yamashiro Yuichiro , Yabuta Keijiro , Miyano Takeshi , Hayasawa Hirotoshi 
Published: January 2024

SummaryThe relationship between essential fatty acid (EFA) deficiency and disturbance of prostaglandin (PG) biosynthesis was studied in children after radical operation for extrahepatic biliary atresia (EBA). In addition, to investigate the method for treatment of postoperative EFA deficiency and disturbance of PG biosynthesis, the serum fatty acid and plasma PG levels were determined before and after supplementation of an EFA‐rich powder (38 g of linoeic acid per 100 g of powder) through Suruga II enterostomy. Before administration of the EFA‐rich powder, linoleic acid, arachidonic acid, PGE1, and PGF2α levels were significantly lower in both good bile excretion and poor bile excretion groups than in the control group. After administration, linoleic acid and PGE1 levels significantly increased in the good bile excretion group as compared with the preadministration values. These results suggest that the supplementation of EFA‐rich powder is an effective treatment for linoleic acid deficiency and disturbance of PGE1 biosynthesis in postoperative EBA patients.
Volume and Emptying of the Gallbladder in Patients with Cystic Fibrosis
Authors: Santamaria F. , Vajro P. , Oggero V. , Greco L. , Angelillo M. , Carrillo F. , De Ritis G. 
Published: January 2024

SummaryAs in cystic fibrosis (CF), gallbladder (GB) hypokinesia might play a role in the high incidence of either cholelithiasis or atrophic and hypoplastic GBs. We studied the contractility of the GB by ultrasonography (US) in 57 CF patients and in 25 age‐ and sex‐matched healthy controls. We evaluated the status of the GB, the GB volume after an overnight fasting and 30 min after the ingestion of a standardized milk meal, the GB contractility (the percentage of reduction, (%R) of the GB volume after a meal), the presence of gallstones, and finally, the span of the liver. In addition, liver function test results were evaluated in the CF group. We concluded that the mean values of GB contractility in CF patients do not differ from those of healthy controls, the most hypokinetic GBs (%R < 40%) being observed, however, only in the CF group. Gallstones occur in CF patients with a greater frequency than seen in the normal population, even in early childhood. The CF patients' GB mean volumes in fasting conditions are significantly greater than in healthy controls (13.5 ± 2 versus 8.5 ± 1.5 cc, p < 0.0001).
Disappearance of IgM Antibodies to Hepatitis A Virus After an Acute Infection in Children and Adolescents
Authors: Dollberg Shaul , Kerem Eitan , Klar Aharon , Branski David 
Published: January 2024

SummaryThe kinetics of IgM antibodies to hepatitis A virus (HAV) following an acute infection, were studied in 17 children. Antibodies disappeared in two patterns, one group at 113 ± 18 days after the acute infection in the children, and the second group at 283 ± 90 days. The same two patterns of kinetics were seen in adults. We conclude that IgM anti‐HAV antibodies can be found in the sera of children for over 6 months after the acute infection, as was observed in adults.
Duodenogastric Reflux in Children
Authors: Mouterde Olivier , Foucaud Pierre , Vatier Jacques , Dupont Christophe , Navarro Jean 
Published: January 2024

SummaryThe duodenogastric reflux (DGR) is a suspected cause in some esogastric pathologies in adults: esophagitis, peptic gastric ulcers, stress ulcers, ulcers secondary to drugs, gastric cancer, and gastritis. The toxic substances of the reflux are essentially bile acids, lysolecithin, and trypsin. A number of diagnostic methods have been proposed in the adult. This study suggests a diagnosis technique for DGR in the child. Fasting gastric juice was collected by gastric intubation during 1 h and three substances were measured: phospholipids as markers of biliary reflux, trypsin as a marker of pancreatic reflux, and sialic acid as a marker of the degradation of gastric mucus. The sialic acid enabled us to evaluate some of the toxicity of DGR on the stomach. The study of 49 child subjects permitted us to show the existence, in the normal child, of biliopancreatic markers in the stomach under fasting conditions through a physiological DGR; to define the norms in the child, varying according to three age groups: 0–2 months, 2–12 months, and 1–4 years (the maximum values for an age above 4 years seemed to correspond to those in the adult); and to suggest the existence of a pathological DGR in children with antral gastritis or ulcers.
Protein Turnover in Malnourished Patients with Cystic Fibrosis
Authors: Pelekanos James T. , Holt Terence L. , Ward Leigh C. , Cleghorn Geoffrey J. , Shepherd Ross W. 
Published: January 2024

SummaryTo evaluate the relative efficacy of nonelemental versus semielemental enteral supplements for nutritional rehabilitation of cystic fibrosis (CF) patients, whole‐body protein turnover using the [15N]glycine method was studied in nine malnourished CF patients during enteral feedings, in a block design study comparing a semielemental formula (Criticare), a higher protein density but nonelemental formula (Traumacal) (T), and a nonelemental formula that had been modified to become isocaloric and isonitrogenous to the semielemental formula (modified Traumacal, MT). No significant differences in rates of protein synthesis or catabolism were observed comparing the three formulas. However, the higher protein density nonelemental formula resulted in higher net protein deposition compared to the other two formulas (T + 0.42 g kg‐1 10 h‐1 versus 0.33 g kg‐1 10 h‐1 for Criticare and −0.59 g kg‐1 10 h‐1 for MT), although this was significant (p < 0.05) for the MT versus T comparison only. This study lends support to the use of less expensive nonelemental formulas for the nutritional management of malnourished patients with CF.
The Nutritional Value of Oat Flour for Very Young Children
Authors: Graham George G. , MacLean William C. , Morales Enrique 
Published: January 2024

SummaryWhole groat flour was consumed by nine infants and young children as 22.5, 45, or 67% of total diet energy (one half of 6.4%, all of 6.4%, or all of 9.6% protein energy). Isonitrogenous and isoenergetic casein control diets were given. Apparent absorption of oat nitrogen (N) was consistently around 75% of intake (casein, 87%), but absorptions of oat energy, carbohydrate, and fat, as percentages of intake, decreased disproportionately as oat flour intake was doubled and then tripled. Apparent retentions were 39 ± 5% of mixed oat‐casein protein intake in the 22.5% diet, the preceding and following casein controls being 38 ± 8% (NS) and 44.4% (p<0.05) of the intakes; 32 ± 6% from oats in the 45% diet, controls 38 ± 5 and 46 ± 5% (both p<0.05), and 33 ± 11% from oats in the 67% diet, controls, 36 ± 9% (NS). Fasting plasma free total essential amino acid (TEAA) levels of children consuming 45% oats were low (562 ± 119 μmol of TEAA/L) and did not change significantly after meals. Fasting molar proportions of individual essentials (millimoles of EAA per mole of TEAA) were similar to those from milk protein diets and did not vary significantly 3 and 4 h after feeding, suggesting that no individual amino acid, but rather protein digestibility, was first limiting to N retention. Oats are a satisfactory source of energy, protein, and fat for very young children and many infants.
An Evaluation with Piglets of Bovine Milk, Hydrolyzed Bovine Milk, and Isolated Soybean Proteins Included in Infant Milk Formulas. I. Effect on Organ Development, Digestive Enzyme Activities, and Amino Acid Digestibility
Authors: Moughan Paul J. , Pedraza Maria , Smith William C. , Williams Megan , Wilson Margaret N. 
Published: January 2024

SummaryA study, using the piglet as a model for the human infant, was undertaken to determine the effect of the protein source of a milk formula on organ development, the activity of digestive enzymes, and the absorption of amino acids as measured at the terminal ileum and over the entire digestive tract. Three isocaloric liquid milk formulas containing equal amounts of either intact bovine milk, hydrolyzed bovine milk, or isolated soybean protein as the sole source of this nutrient and with equal levels of fat and carbohydrate were each given to six 14‐day‐old piglets over a 19‐day period in a manner that mimicked human infant feeding practice. Following a 6‐day metabolism study, the piglets were killed, their organs removed, and samples of digesta collected. The protein source of the milk‐based formula did not affect (p > 0.05) the weight/unit body weight of the small intestine, large intestine, liver, or pancreas, but the relative weight of the kidneys was lower (p<0.05) for animals fed the intact bovine milk‐based formula and that of the stomach was higher (p<0.01) for piglets receiving the isolated soybean formula. The activities of pepsin, intestinal trypsin and chymotrypsin and pancreatic chymotrypsin were not influenced (p > 0.05) by protein source, but piglets receiving the bovine milk‐based formula had a lower level of activity (p<0.01) for pancreatic trypsin. The apparent ileal and fecal absorption of nitrogen was similar for the three milk‐based formulas, the overall mean ileal absorption of nitrogen (±SE) being 89.0% (±1.44). Excluding cystine, where ileal absorption was relatively lower (p<0.05) with the isolated soybean formula (86.7%) compared with the intact bovine milk (91.5%), protein source had little effect on the apparent absorption of essential amino acids. It was concluded that the replacement of intact cow's milk protein in human infant formulas by either hydrolyzed cow's milk or isolated soybean protein is unlikely to cause any major disturbance in the digestive process.