bluuebunny/crossref_metadata_2025_split huggingface dataset:
Digestive and Absorptive Phase Anomalies Associated with the Exocrine Pancreatic Insufficiency of Cystic Fibrosis
Authors: Roy Claude C. , Weber Andrée M. , Lepage Guy , Smith Lesley , Levy Emile 
Published: January 2024

SummaryThe pancreas has an enormous reserve capacity, and significant malabsorption usually signals complete absence of exocrine function. However, there is evidence that acid lipases of nonpancreatic origin play an important compensatory role. Complete duodenal hydrolysis of fat requires a series of complex interdependent physicochemical events involving pancreatic lipase, colipase, phospholipase A2, and bile salts in an environment where the pH must be close to neutrality. Lipolytic products must then be shuttled through the unstirred water layer to the surface of the microvillus membrane by ionized bile salts, which must be present in sufficient concentrations to form micelles. In pancreatic insufficiency, there is not only a defective lipolytic phase but also an impaired micellar phase. The output of bile salts is decreased because of increased fecal loss. Furthermore, a significant percentage of bile salts precipitate because the duodenum is acidic and there is a large predominance of glycine conjugates. Although much less work has been done on the absorptive phase of patients with pancreatic insufficiency, there is tentative evidence that defective phospholipid absorption, essential fatty acid deficiency, and protein malnutrition could impair the absorptive phase, particularly chylomicron formation. Although significant advances have been made in our understanding of factors responsible for malabsorption associated with pancreatic insufficiency, much remains to be done for the further delineation of defects. It is hoped that this will lead to further refinements of enzyme preparations and to new strategies of intervention.
Nutritional Requirements in Cystic Fibrosis
Authors: Dodge John A. 
Published: January 2024

SummaryFood intake is often low in cystic fibrosis (CF), although the patient usually needs more than the standard recommended daily allowance (RDA). Clinics giving food supplementation from an early age report improved survival and nutritional status. Nutritional improvement has been facilitated by improved forms of pancreatin. An additional calorie intake in CF is required to compensate for losses due to malabsorption and to allow for catch‐up growth when necessary. With advanced pulmonary disease there are additional requirements for infection and increased work of breathing. There is also evidence for an increased basal metabolic activity in CF, perhaps related to the fundamental intracellular biochemical disorder. Together these factors add to a daily need for 120%‐150% RDA for optimum growth and homeostasis.
Vitamin K1 and K2 in Infant Human Liver
Authors: Kayata Sahar , Kindberg Carl , Greer Frank R. , Suttie John W. 
Published: January 2024

SUMMARYIt is not known to what extent humans store vitamin K in liver. We measured hepatic concentrations of vitamin K1 (phylloquinone) and K2 (menaquinones) in 11 human livers (eight infants and three adults). Relatively small amounts of vitamin K were found in the liver at any age compared to other fat soluble vitamins. Vitamin K1 was the predominant form with much smaller concentrations of vitamin K2. Long‐chain menaquinones (vitamin K2) were readily identified in most liver specimens. Hepatic vitamin K2 concentrations also increased with increasing age. These observations have implications for vitamin K supplementation in infants.
Glucose Intolerance with Low‐, Medium‐, and High‐Carbohydrate Formulas During Nighttime Enteral Feedings in Cystic Fibrosis Patients
Authors: Kane Robert E. , Black Philip 
Published: January 2024

SUMMARYTen young adult cystic fibrosis (CF) patients over 16 years of age (average 21.4 years) began nighttime enteral feedings as a method of nutritional rehabilitation to regain and maintain body weight. Patients received nighttime feedings of 1,000 kcal/M2 of a low‐ (Pulmocare), medium‐ (Ensure Plus), or high‐carbohydrate (Vivonex) formula for at least 2 nights each with pancreatic enzyme therapy. Five of ten young adult CF patients developed nocturnal hyperglycemia (serum glucose >300 mg/dl) and glucosuria (1–3% glucose) with varying degrees of polyuria during enteral feedings. No patient developed ketonuria despite serum glucoses at times >600 mg %. There was no difference between the hyperglycemic and nor‐moglycemic groups in median age, percent of ideal body weight, NIH score, Brasfield scores, pulmonary function tests, or family history of diabetes. All normoglycemic and four of five hyperglycemic patients had normal fasting blood sugars. The percent hemoglobin A1c was greater in the glucose intolerant group than the normoglycemic patients (11.2 ± 0.8% vs. 6.8 ± 1.1%, mean ± SE, p < 0.005). Twelve to 15 units of NPH insulin prior to initiation of feedings provided adequate therapy in most hyperglycemic patients. There was no apparent difference in the elevation of early morning serum glucoses with the low‐ medium‐ and high‐carbohydrate formulas. We concluded that hyperglycemia requiring insulin therapy was common in young adult CF patients using nighttime enteral feedings. A hemoglobin A1c appeared to be a useful screening test before initiating such therapy.
Predictors of Breast Milk Intake in Rural Northern Thailand
Authors: Imong Stella M. , Jackson Dorothy A. , Wongsawasdii Lumduan , Ruckphaophunt Somsri , Tansuhaj Antika , Chiowanich Pien , Woolridge M. W. , Drewett R. F. , Baum J. D. , Amatayakul Kosin 
Published: January 2024

SUMMARYA random sample of 52 mothers and infants from a rural area of Northern Thailand were studied in their homes for 48 h. Infants were breast fed on demand. Daytime feeds were test weighed with electronic averaging balances and nighttime intake estimated using a new method, Indirect Test Weighing (23,24). All feeds, both day and night, were timed to the nearest minute. Other variables measured by direct observation were infant weight, and supplementary food and water intake. Mean 24‐h breast milk intake was 579 g (range 78–1,011 g) with a median between day coefficient of variation of 8.8%. The proportion of 24‐h breast milk intake consumed at night ranged from 8 to 91%, and increased with increasing infant age. Significant positive associations were found between breast milk intake, and sucking time and infant weight. There were significant negative associations between breast milk intake and age, and supplementary food and water intake.
Cerebral Thromboembolic Disease in Pediatric and Adult Inflammatory Bowel Disease
Authors: Markowitz Robert L. , Ment Laura R. , Gryboski Joyce D. 
Published: January 2024

SUMMARYCerebral and retinal vascular disease are rare complications of inflammatory bowel disease. Most reports are of adult patients, with only seven instances described in children. The eighth case, a 14‐year‐old boy with ulcerative colitis and cerebral venous thrombosis is reported in whom the diagnosis was confirmed by magnetic resonance imaging. The adult and pediatric literature is also reviewed.
Intestinal Permeability Changes in Acute Gastroenteritis
Authors: Isolauri E. , Juntunen M. , Wiren S. , Vuorinen P. , Koivula T. 
Published: January 2024

SummaryThe effects of early home management of acute diarrhea followed by rapid in‐hospital realimentation on intestinal permeability were studied in 41 children aged 3–25 months with acute gastroenteritis (73% rotavirus). After oral rehydration, a 100 ml oral load containing 4 g of lactulose and 0.8 g of mannitol was administered, and an aliquot of urine excreted in the subsequent 5 h was analyzed with gas‐liquid chromatography. The mean lactulose/mannitol recovery ratio was significantly higher than in 28 nondiarrheal controls, which was due to decreased mannitol excretion. The gastroenteritis patients who had received uninterrupted feeding in addition to adequate fluid replacement before hospitalization had a normal urinary lactulose/mannitol ratio, with a mean of 0.04, and a 95% confidence interval (CI) of [0.03, 0.07], whereas in fasted children with inadequate or adequate fluid replacement, the respective mean ratios were 0.24, 95% CI of [0.14, 0.43], and 0.14, 95% CI of [0.09, 0.20] (F = 12.63, p < 0.001). The fasting‐associated rise was caused by increased lactulose excretion. At retesting of gastroenteritis patients after 2 days of in‐hospital realimentation, the lactulose/mannitol ratios did not differ significantly from the level on admission. The study indicated that fasting maintains the increased intestinal permeability associated with acute gastroenteritis whereas early feeding at home may promote reduction of permeability and hasten recovery.
Developmental Changes in the Intra‐acinar Distribution of Succinate Dehydrogenase, Glutamate Dehydrogenase, Glucose‐6‐Phosphatase, and NADPH Dehydrogenase in the Rat Liver
Authors: Sokal E. M. , Trivedi P. , Portmann B. , Mowat A. P. 
Published: January 2024

SummaryHepatocytes differ in their metabolism depending on their position in the liver acinus. To assess how such specialization changes during development, we used quantitative cytochemistry to measure succinate dehydrogenase (SDH), glutamate dehydrogenase (GDH), glucose‐6‐phosphatase (G6P), and NADPH dehydrogenase (ND) activities specifically in periportal and perivenular hepatocytes in developing rats, aged between 1–114 days. Important and distinct changes were observed in each zone for each enzyme during development. An intraacinar gradient of distribution was present from day 1 for SDH and G6P and from day 5 for GDH and ND. It was being similar to the adult value for SDH but less pronounced for the remaining enzymes. The SDH and G6P activity was greater in periportal cells, and the GDH and ND activity was greater in perivenular cells. The more pronounced distribution with age was due, for G6P, to an initial specific periportal increase combined with a mild perivenular decrease and for GDH to a greater perivenular than periportal increase. The ND first increased simultaneously in both zones, but from day 20 the perivenular increase became prevalent. The SDH changes were parallel in both zones. All zonal enzyme activities changed distinctly after weaning. To what extent the changes in activities and metabolic zonation observed in our study reflect a response to specific metabolic demands of the liver or can be modified by environmental factors remains to be investigated.
Routine L‐Ascorbic Acid Supplementation Does Not Alter Iron, Copper, and Zinc Balance in Low‐Birth‐Weight Infants Fed a Cows'‐Milk Formula
Authors: Stack T. , Aggett P. J. , Aitken E. , Lloyd D. J. 
Published: January 2024

SummaryThe effect of ascorbic acid (AA) [284 μmol (50 mg) twice daily] on the net intestinal absorption and maximum apparent retention of Fe, Cu, and Zn was investigated by metabolic balance studies in a randomised crossover study of six low‐birth‐weight (LBW) neonates fed a cows'‐milk‐based formula containing (μmol/L) Fe, 126; Cu, 11; Zn, 87; and AA, 400. Absorption ± SD (Fe, −5.0 ± 7.5; Cu, 0 ± 0.4; Zn, −0.8 ± 3.4) (μmol kg‐1 day‐1) was not altered by AA (Fe, −4.1 ± 4.6; Cu, 0.3 ± 0.6; Zn, −1.1 ± 2.7) neither was retention (without AA: Fe, −6.0 ± 8.4; Cu, −0.1 ± 0.3; Zn, −2.4 ± 4.2; with AA: Fe, −4.9 ± 4.7; Cu, 0.1 ± 0.6; and Zn, −2.7 ± 3.1). Supplements of AA administered as in the circumstances of routine care of LBW neonates do not enhance the absorption and retention of Fe, nor do they impair these aspects of the metabolism of Cu and Zn.
Rapid Endoscopy Room Diagnosis of Campylobacter pylori‐Associated Gastritis in Children
Authors: Yeung C. K. , Yuen K. Y. , Fu K. H. , Tsang T. M. , Seto W. H. , Saing Htut 
Published: January 2024

SummaryA modified urease broth test was used in 109 gastric biopsy specimens of children that yielded a 96% sensitivity and a 100% specificity rate for Campylobacter pylori infection. The longest reaction time was ±4 min. The correlation between the degree of C. pylori infection and the reaction time was found to be highly significant (γ = 0.78, p<0.001). Patients were examined as outpatients, and immediate medications were prescribed for eradication of the bacteria on the basis of the urease broth test results. This is the first report on the use of this modified rapid urease test for the diagnosis of C. pylori infection in children.
Plasma Amino Acids in Long‐Term Models for Obstructive Versus Toxic Liver Injury in Developing Rats
Authors: Weisdorf Sally A. , Freese Deborah K. , Radmer William J. , Dehner Louis P. , Cerra Frank B. 
Published: January 2024

SummaryWe evaluated plasma amino acid (AA) concentrations associated with a histologically defined lesion caused by bile duct ligation (BDL) in developing rats. Nineteen rats that underwent BDL at 14 days of age had marked bile duct proliferation with bridging fibrosis, multifocal lobular necrosis, and minimal polymorphonuclear periportal infiltrate in their livers at sacrifice (11–31 days after ligation). These were compared to two age‐matched control groups: 21 nonoperated rats and 22 shamoperated rats; and eight rats with cirrhosis caused by carbon tetrachloride. Signs of liver damage including jaundice, growth failure, bleeding, and ascites were accompanied by elevated bilirubin, ammonia, aspartate aminotransferase (AST), and alkaline phosphatase levels in BDL rats compared to controls. They had higher concentrations of total AAs, phenylalanine, tyrosine, and cyst(e)ine when compared to controls and to CCl4‐treated rats. Micronodular cirrhosis was present in CCl4‐treated rats with elevated AST and alkaline phosphatase levels. Glutamine and glutamate levels were higher in them than in BDL rats or controls, and branched chain AA levels were lower. These two chronic lesions, one obstructive and one hepatotoxic, both result in fibrotic change, but their metabolic abnormalities as reflected in plasma AA levels are distinct. We found that BDL is an appropriate model with which to study metabolic changes and growth failure due to chronic biliary stasis during its progression to frank cirrhosis.
Necrotizing Perianal Infection in Children
Authors: Chuang Jiin‐Haur , Wong Kin‐Sun 
Published: January 2024

SummaryTwo girls aged 1 9/12 years and 5 months were treated for necrotizing perianal infection. In both patients, the necrotizing ulcers around the anus developed following an episode of diarrhea. A poor nutritional status with low body weight (25th percentile) and hypoalbuminemia (2.5 g/L) in the elder patient, as well as a hypogammaglobulinemia in the younger one, may predispose them to bacterial infection and subsequent perianal lesions. Anorectal trauma was denied. The necrotizing infection was confined to the skin and the subcutaneous tissue layer, and both patients had a benign clinical course. We report it and briefly discuss the etiology and treatment of this rare disorder.
Casein and Casein Subunits in Preterm Milk, Colostrum, and Mature Human Milk
Authors: Kunz Clemens , Lönnerdal Bo 
Published: January 2024

SummaryHuman milk proteins have both nutritional and physiological roles for the breast‐fed infant. While the biochemistry and developmental patterns for many whey proteins are well known, our knowledge of human casein and its subunits is still limited. We have recently developed a method to isolate casein from whey proteins in human milk and to separate the casein subunits by fast protein liquid chromatography. In this study we have applied this methodology to study the casein subunit pattern in pretern milk, colostrum, and mature milk. Casein concentration increased with lactation time, largely due to an increase in glycosylated forms of casein (k‐caseins). Thus, the relative proportion of β‐casein to k‐casein decreased during the lactation period. The patterns of phos‐phorylated and glycosylated casein subunits were found to vary during lactation, showing that both synthesis and posttranslational modification of β‐ and k‐casein are regulated by different mechanisms.
Effect of Dietary Nucleosides on Growth and Maturation of the Developing Gut in the Rat
Authors: Uauy Ricardo , Stringel Gustavo , Thomas Rita , Quan Richard 
Published: January 2024

SummaryDietary nucleoside (DN) as a precursor for nucleic acid synthesis may be important for rapidly dividing cells, since gut epithelial cells have limited capacity for de novo purine and pyrimidine synthesis. We evaluated in a controlled blinded study the effect of added nucleosides, 0.8% by weight, given for 2 weeks, on gut growth and maturation in 20 weanling rats. Mucosal protein and DNA in the proximal intestinal segment were 50% and 77% higher, respectively, in the DN‐supplemented group (n = 10; p < 0.05). Villus height based on cell count was 25% greater in the DN group (p < 0.05). Maltase activity was significantly greater in proximal, middle, and distal intestinal segments, and the largest increase, 87%, was seen in the proximal gut mucosa. The maltase/lactase ratio was also higher in this segment. Increases in sucrase were less prominent. Lactase was minimally affected. The pattern of change in disaccharidase activity suggests that DN may enhance gut growth and maturation of the intestine in the weanling rat, the effects being more pronounced in the proximal segment. Diets free of nucleosides and nitrogenous bases may have adverse effects on the gut.
An Acute Form of Tyrosinemia Type I With Multiple Intrahepatic Mass Lesions
Authors: Tazawa Yusaku , Kikuchi Masahiro , Kurobane Ikuo , Watanabe Ayako , Nakai Hiroshi , Narisawa Kuniaki , Tada Keiya 
Published: January 2024

SummaryThe acute form of tyrosinemia type I usually causes severe hepatocellular dysfunction. We report a 4‐month‐old infant with hepatosplenomegaly, ascites, and multiple intrahepatic mass lesions mimicking hepatoma. A marked increase of serum α‐fetoprotein (97.6 μg/ml) and multiple small low‐density lesions in the liver demonstrated by computed tomography suggested the presence of hepatoma. The liver specimens obtained at laparatomy showed mixed nodular cirrhosis with fatty metamorphosis. Serum levels of tyrosine (6.6 mg/100ml) and methionine (5.9 mg/100 ml) were increased. Urinary organic acid analyses disclosed increased excretions of succinylacetone (1,330 mg/g creatinine) and δ‐amino‐levulinic acid (113.6 mg/g creatinine). Histological abnormalities and biochemical evidences led to the correct diagnosis. This case emphasizes the need for complete investigations of puzzling cases and warns against undue reliance on noninvasive imaging techniques.
Congenital Hepatic Fibrosis, Cystic Kidneys, Mental Retardation, and Facial Dysmorphy
Authors: Labrune P. , Lange J. C. , Bedossa P. , Chaussain J. L. , Odievre M. 
Published: January 2024

SummaryThis report concerns a boy with congenital hepatic fibrosis, cystic kidneys, mental retardation, and minor dysmorphic features. These symptoms have rarely been reported before and indicate that congenital hepatic fibrosis may not be a single clinical entity.
Prognostic Implications of Serum α‐Fetoprotein Levels in Neonatal Hepatitis
Authors: Lee Ping‐Ing , Chang Mei‐Hwei , Chen Ding‐Shinn , Hsu Hey‐Chi , Lee Chin‐Yun 
Published: January 2024

SummarySerum β‐fetoprotein (AFP) values in 61 patients with neonatal hepatitis were compared with the normal ranges of AFP in infants and were expressed as standard deviation (SD) scores. The size of SD score indicates how many standard deviations the measured AFP value is away from the mean value of the normal population. An extremely elevated AFP value is arbitrarily defined as a SD score higher than 4.0, i.e., above mean + 4 SD. The result showed that rapid recovery from jaundice before 6 months of age occurred in 16 of 17 (94%) with SD scores of AFP lower than 4.0. By contrast, in 26 with SD scores of AFP higher than 4.0, 17 (65%) has less favorable clinical courses, either slow in recovery or progressing to chronic liver disease and even death. This correlation between AFP level and prognosis was related to the fact that higher AFP values were associated with more severe inflammatory changes in the liver, as assessed from either biochemical parameters (peak total and direct bilirubin levels) or histotogical findings (portal inflammation and giant cell transformation).
Oral Supplementation of Vitamin K for Pregnant Women and Effects on Levels of Plasma Vitamin K and PIVKA‐II in the Neonate
Authors: Motohara Kunihiko , Takagi Satoshi , Endo Fumio , Kiyota Yuji , Matsuda Ichiro 
Published: January 2024

SummaryLevels of plasma vitamin K1 (VK1) and vitamin K2 (VK2) and protein‐induced vitamin K absence‐II (PIVKA‐II) were measured in Japanese mothers and their newborn (N = 33). Twenty milligrams of VK1 (N = 11) or VK2 (N = 12) were given orally to randomly selected mothers 7 to 10 days prior to delivery. Means of plasma VK1 and VK2 concentrations were significantly higher in VK1 (p < 0.01) and VK2 (p < 0.01) treated mothers than in the controls at delivery, respectively. Similarly, these levels were significantly elevated in cord plasma in VK1 (p < 0.05) and VK2 (p < 0.05) treated groups, compared with findings in the control group, although there was a large concentration gradient between maternal and cord plasma (mostly less than one‐tenth). A significant positive correlation was found in VK1 concentration between maternal and cord plasma (N = 33, p < 0.01), and the proportion of PIVKA‐II‐positive infants was significantly lower in the VK treated groups than in the control group at birth (p < 0.05). On the fifth postnatal day, mean levels of VK1 (p < 0.01) and VK2 (p < 0.01) in breast milk were significantly higher in the VK1 and VK2 treated mothers than in the control mothers, respectively. In the control group, 9 of 10 infants had a positive PIVKA‐II, but no one in the treated groups was positive, thereby indicating significant differences between control and treated groups (p < 0.01 and p < 0.01, respectively). The administration of both VK,1 and VK2 to mothers prior to delivery may prevent hypoprothrombinemia in infants, at birth, and during neonatal stages.
Placebo‐Controlled Trial of Misoprostol in Cystic Fibrosis
Authors: Robinson P. , Sly P. D. 
Published: January 2024

SummarySeventeen children with cystic fibrosis were studied in a 6‐week double‐blind, placebo‐controlled, crossover trial to determine the efficacy of misoprostol, 100 μg, four times a day, in improving fat absorption in patients already on pancreatic enzyme therapy. In those patients who had greater than 90% absorption on enzyme therapy alone, no further significant increase in absorption was achieved with misoprostol administration. Those patients who had absorption of less than 90% on standard enzyme therapy showed a significant improvement with misoprostol administration (p < 0.01). One patient had a significant elevation in the eosinophil count during the period of misoprostol administration, but there were no significant changes in any other hematological or biochemical parameter. Misoprostol appears to be of benefit to those children with cystis fibrosis who have residual malabsorption on standard enzyme therapy.
Intravenous Erythromycin for Postpyloric Intubation
Authors: Di Lorenzo Carlo , Lachman Ralph , Hyman Paul E. 
Published: January 2024

SummaryOf 100 consecutive patients referred for antro‐duodenal manometry, we successfully intubated 89 with endoscopic and/or fluoroscopic guidance. In 8 of 8 patients who had failed with our usual techniques, we infused erythromycin 3 mg/kg i.v. over 1 h, and the tube moved through the pylorus and into the duodenum. Erythromycin, which stimulates propagation of 3 high amplitude antral contractions/min, appears to be a safe, noninvasive means to facilitate the postpyloric passage of tubes.