bluuebunny/crossref_metadata_2025_split huggingface dataset:
Prevalence of Lactose Malabsorption in Galicia
Authors: Leis Rosaura , Tojo Rafael , Pavón Pilar , Douwes Adriaan 
Published: February 2024

Background:The aim of the current study was to evaluate the prevalence of lactose malabsorption (LM) in Galicia (NW Spain) in order to design nutritional intervention and/or public education strategies for high risk groups.Methods:We conducted a study of LM by breath‐hydrogen carbohydrate absorption test (BH2 test) in 850 healthy subjects. All subjects underwent BH2 tests following ingestion of a aqueous solution of 2 g lactose/kg body weight up to a maximum of 50 g. Subjects with LM were retested after ingesting 250 ml of milk and/or 250 ml of yogurt.Results:The frequency of LM in the subjects who ingested 2g lactose/kg body weight was 32.5%. This percentage decreased significantly with a decrease in the quantity of administered lactose and the vehicle was milk or yogurt‐only 13.7% was LM after 250 ml of milk and 3.8% after 250 ml of yogurt. Gastrointestinal symptoms also depend on dosage of lactose and vehicle, decreasing from 54.3% after 2g lactose/kg to 18.5% after milk and to 0% after yogurt. The frequency and number of gastrointestinal symptoms were significantly higher (p < 0.001) in LM than in lactose absorption (LA).Conclusions:Lactose malabsorption is prevalent in the population of Galicia. An important number of subjects identified as LM with usual clinical testing become LA when the ingestion of dairy products is limited so that the amount of lactose consumed is similar to that contained in a usual serving. Our results suggest the importance of BH2 testing following ingestion of usual consumed amounts of lactose per serving.
Sacrosidase Therapy for Congenital Sucrase‐Isomaltase Deficiency
Authors: Treem William R. , McAdams Luanne , Stanford Lesley , Kastoff Geraldine , Justinich Christopher , Hyams Jeffrey 
Published: February 2024

ABSTRACTBackground:The purpose of this study was to determine if sacrosidase, a liquid produced from Saccharomyces cerevisiae containing 6000 IU of sucrase activity per mg protein, prevented symptoms of diarrhea, abdominal cramps, gas, and bloating in patients with congenital sucrase‐isomaltase deficiency (CSID) consuming a normal sucrose and carbohydrate‐containing diet.Methods:Twenty‐eight children (aged 5 months to 11 years) underwent a randomized, double‐blind trial consisting of two phases: 1) three sucrose breath H2 tests with three single‐dose treatments (placebo, sacrosidase, and sacrosidase plus milk), and 2) a dose‐response phase consisting of four multidose treatments, each for 10 days of full‐strength sacrosidase, 1:10 dilution, 1:100 dilution, and 1:1000 dilution. Patients who weighed less than or equal to 15 kg received a dose of sacrosidase and those who weighed more than 15 kg received 2 ml. For the dose‐response phase each patient consumed a normal diet. The number of stools and severity of symptoms were recorded daily for each concentration of sacrosidase administered and compared to a baseline period during which the patient took no sacrosidase and consumed a sucrose/starch‐free diet. Data were analyzed using an ANOVA model and the nonparameter Wilcoxon signed‐rank test.Results:Breath H2 excretion decreased significantly when patients received sacrosidase or sacrosidase plus milk compared to placebo during sucrose breath tests. During the dose‐response phase significant treatment differences were observed between the two higher concentrations and the two lower concentrations of sacrosidase for both total stools (p < 0.001) and total symptom score (p = 0.003). Higher concentrations of sacrosidase were associated with fewer stools and a greater number of formed or hard stools compared to lower concentrations and compared to the baseline period. Higher concentrations were also associated with fewer symptoms of gas, abdominal cramps, or bloating, but no differences in vomiting. The only significant adverse event was wheezing in one child with a history of asthma.Conclusions:Sacrosidase is a safe, effective, well‐accepted treatment to prevent gastrointestinal symptoms in patients with CSID consuming a normal diet.
Increased Concentrations of Eosinophilic Cationic Protein in Whole‐Gut Lavage Fluid From Children With Inflammatory Bowel Disease
Authors: Troncone Riccardo , Caputo Nives , Esposito Valeria , Campanozzi Angelo , Campanozzi Francesca , Auricchio Renata , Greco Luigi , Cucchiara Salvatore 
Published: February 2024

ABSTRACTBackground:Eosinophils may be involved in the pathogenesis of inflammation in inflammatory bowel disease. The purpose of this study was to verify whether concentrations of eosinophilic cationic protein in gut lavage fluid from children with inflammatory bowel disease correlate with clinical and laboratory indexes of disease activity.Methods:Twenty‐three children with Crohn's disease, 14 with ulcerative colitis, and 22 age‐matched control subjects entered the study. Radioimmunoassay and sandwich enzyme‐linked immunosorbent assay techniques were used to measure eosinophilic cationic protein, total immunoglobulin G and interleukin‐1β, respectively.Results:Gut lavage eosinophilic cationic protein levels were significantly (p < 0.005) higher in patients with Crohn's disease and ulcerative colitis than in control subjects. Intestinal eosinophilic cationic protein levels decreased in three of four children with Crohn's disease who were fed an elemental diet. There was a significant (p < 0.001) correlation between eosinophilic cationic protein concentrations and immunoglobulin G and interleukin‐1β levels in gut lavage fluid.Conclusions:Elevated intestinal eosinophilic cationic protein levels in inflammatory bowel disease suggest that eosinophils are involved in the gastrointestinal inflammation in this disease. Intestinal eosinophilic cationic protein concentration is another marker with which to discriminate between active and inactive inflammatory bowel disease.
Nitrous Oxide Sedation in Pediatric Patients Undergoing Gastrointestinal Endoscopy
Authors: Michaud Laurent , Gottrand Frédéric , Ganga‐Zandzou Patrice S. , Ouali Mohamed , Vetter‐Laffargue Anne , Lambilliotte Anne , Dalmas Serge , Turck Dominique 
Published: February 2024

ABSTRACTBackground:The ideal medication to administer to children before gastrointestinal endoscopy procedures has yet to be found. The efficacy of using inhaled nitrous oxide during endoscopy in children was assessed in a pilot study.Methods:Patients aged 5 to 17 years, referred to our hospital for diagnostic upper gastrointestinal endoscopy or rectosigmoidoscopy procedures, were eligible for enrollment in this study. All received 50% nitrous oxide in oxygen (Entonox; AGA, Rueil‐Malmaison, France) before endoscopy and some of them again during endoscopy. The pediatric endoscopist and the nurse performing the procedure were separately asked to rate cooperation, emotional state, drowsiness and overall efficacy of sedation. Oxygen saturation and adverse effects were recorded throughout the procedure. After endoscopy, children scored their degree of pain during the procedures on a visual analog scale (0, no pain; 100, agony) and on a body outline (projective method). Any adverse effects were noted.Results:Thirty‐seven patients were enrolled in the study. Thirty patients underwent diagnostic upper gastrointestinal endoscopy and seven diagnostic rectosigmoidoscopy. The median time from administration of nitrous oxide until insertion of the endoscope was 5 minutes (range, 3‐8 minutes). Good or excellent efficacy of the sedation was noted in 92% of children by the endoscopist and in 89% by the nurses. Good or excellent cooperation was noted in 92% of the children by the endoscopist and in 78% by the nurses. The children's pain score on the visual analog scale ranged from 5 to 100 (median, 20) for upper gastrointestinal endoscopy and from 0 to 30 (median, 0) for rectosigmoidoscopy. The time of discharge after endoscopy, defined as the time elapsed between the end of the endoscopy and discharge from the endoscopy suite, varied from 1 to 7 minutes (median, 1.5 minutes).Conclusion:Entonox provides rapid and effective analgesia without heavy sedation, leads to adequate relaxation and cooperation, and facilitates quick and efficient endoscopy. The effect of Entonox was of short duration, allowing the children to leave the endoscopy unit without need for a long recovery period. The adverse effects of Entonox appeared to be minor, and their duration was always brief. Nitrous oxide‐oxygen inhalation may provide a valuable alternative to conventional sedation regimens during gastrointestinal endoscopy in children, but randomized and prospective studies comparing nitrous oxide sedation and conventional sedation regimens are necessary.
Coagulation and Fibrinolysis in Children, Adolescents, and Young Adults with Inflammatory Bowel Disease
Authors: Weber Peter , Husemann Stephanie , Vielhaber Heinrich , Zimmer Klaus‐Peter , Nowak‐Göttl Ulrike 
Published: February 2024

ABSTRACTBackground:Patients with Crohn's disease and ulcerative colitis have an increased risk of thromboembolic events.Methods:Data were collected from 24 patients aged 4.5 to 23 years who had inflammatory bowel disease. Platelet count, antithrombin, fibrinogen, prothrombin fragment F1+2, soluble thrombomodulin, tissue plasminogen activator, D‐dimer, and plasminogen activator inhibitor‐1 antigen were investigated. In addition the response to activated protein C, the factor V R506Q mutation, protein C, free protein S antigen, and lipoprotein (a) were analyzed. These data were compared with medical treatment, duration, and disease activity, estimated with the Pediatric Crohn's Disease Activity Index or the Clinical Colitis Activity Index.Results:Forty‐five percent of our patients showed an increase in fibrinogen, 29% in prothrombin fragment F1+2, and 20% in platelet count, plasminogen activator inhibitor‐1 antigen, and soluble thrombomodulin. Thrombomodulin was higher in active disease than in inactive disease and in Crohn's disease than in ulcerative colitis. Fibrinogen was also higher with Crohn's disease and tended to be higher in active disease than in ulcerative colitis and inactive disease. Plasminogen activator inhibitor‐1 antigen was significantly higher in patients with Crohn's disease than in those with ulcerative colitis and was higher in the patient group treated with steroids.Conclusion:As has been shown in adults, young patients with active and inactive inflammatory bowel disease were found to have abnormal coagulation and fibrinolysis. The relevance as a thromboembolic risk factor is discussed.
Reproducibility of the 13C‐Octanoic Acid Breath Test for Assessment of Gastric Emptying in Healthy Preterm Infants
Authors: Barnett Christopher , Snel Antonie , Omari Taher , Davidson Geoff , Haslam Ross , Butler Ross 
Published: February 2024

ABSTRACTBackground:The 13C‐octanoic acid breath test has been used to measure gastric emptying in preterm infants, but the reproducibility of the test has not been evaluated in this population.Methods:Fifty‐six paired breath test analyses were performed on 28 healthy preterm infants 1 to 5 days apart using the same food type, volume, and energy content for each paired sample. Breath samples were taken before the feeding, at 5‐minute intervals after feeding for 30 minutes, then each 15 minutes for 4 hours. Samples were analyzed using an isotope‐ratio mass spectrometer, and 13C recovery was used to calculate values for gastric‐emptying coefficient and gastric half‐emptying time.Results:There was no significant difference between test results on different days in the paired samples studied. Gastric‐emptying coefficients for the first and subsequent samples were 2.6 ± 0.1 (mean ± SEM) and 2.7 ± 0.1, respectively, and half‐emptying times were 44.5 ± 3.7 minutes and 41.4 ± 3.2 minutes.Conclusion:The 13C‐octanoic acid breath test is a reliable, noninvasive, and reproducible measure of gastric emptying in preterm infants that should have wide application for use in this population.
Serum Type III Procollagen in Children with Type I Hereditary Tyrosinemia
Authors: Pitkänen Sari , Salo Matti K. , Vettenranta Kim , Näntö‐Salonen Kirsti , Heikinheimo Markku 
Published: February 2024

ABSTRACTBackground:Type I hereditary tyrosinemia leads to hepatic dysfunction and fibrosis and is associated with a high risk of hepatic malignancy. Serum N‐terminal propeptide of type III procollagen is a sensitive marker of organ fibrosis of diverse origins. The current study was conducted to determine whether analysis of serum levels of type III procollagen in hereditary tyrosinemia would be useful in the follow‐up of the progressive liver disease and eventually in detecting hepatic malignancy.Methods:Serum N‐terminal propeptide of type III procollagen was sequentially studied in 10 children with type I hereditary tyrosinemia.Results:At diagnosis of type I hereditary tyrosinemia, serum N‐terminal propeptide of type III procollagen ranged from 0.6 to 2.9 multiples of age‐related median. During follow‐up, serum N‐terminal propeptide of type III procollagen decreased, yet remained elevated 0.2 to 2.6 years after diagnosis. Children with the acute type of the disease tended to have higher serum N‐terminal propeptide of type III procollagen than did those with the chronic type. Porphyria crises were associated with elevated serum type III procollagen. The one patient receiving 2‐(2‐nitro‐4‐trifluoromethyl‐benzoyl)‐1,3‐cyclohexanedione (NTBC) did not differ from the other ones in serum type III procollagen levels. Serum N‐terminal propeptide of type III procollagen did not increase with developing hepatocellular carcinoma.Conclusions:Serum N‐terminal propeptide of type III procollagen may be useful in monitoring the hepatopathy in type I hereditary tyrosinemia but is not useful in detecting malignant transformation in the liver.
Effect of Continuous Glucose Therapy Begun in Infancy on the Long‐Term Clinical Course of Patients with Type I Glycogen Storage Disease
Authors: Wolfsdorf Joseph I. , Crigler John F. 
Published: February 2024

ABSTRACTBackground:To evaluate the effects of continuous glucose therapy on metabolic control, occurrence of severe hypoglycemia, physical growth and development, and complications of glycogen storage disease type I (GSD‐I).Methods:Seventeen patients (11 males) with GSD‐I were studied, mean age 14.6 ± 5.0 (SD) years, in whom continuous glucose therapy was begun at 0.8 ± 0.4 years. At the time of this study, subjects had received continuous glucose therapy for a total duration of 13.9 ± 5.0 years. Uncooked cornstarch was used as the method of providing glucose continuously for 10.2 ± 3.2 years. Subjects were admitted to the Clinical Research Center and followed their usual home dietary regimens, which included cornstarch supplements at 2‐ to 4‐hour intervals during the day and at 4‐ to 8‐hour intervals during the night. Plasma glucose, blood lactate, and glucoregulatory hormones were measured hourly for 24 hours.Results:During a 24‐hour period of biochemical monitoring, mean hourly plasma glucose concentrations for the group of 17 subjects ranged from 76 ± 17 (SD) mg/dl (4.2 ± 0.9 mmol/l) to 108 ± 16 mg/dl (6.0 ± 0.9 mmol/l), and blood lactate concentrations ranged from 2.1 ± 1.2 mmol/l to 3.8 ± 2.8 mmol/l. Four subjects had transient plasma glucose levels of 50 mg/dl (2.8 mmol/l) or less in the interval between midnight and 8:00 AM. Mean blood lactate levels were highest (≥3 mmol/l) between 2:00 and 09:00 AM. Mean height standard deviation score for chronological age (SDSCA) was ‐0.8 ± 1.1, significantly (p < 0.01) less than the mean target height SDS of ‐0.1 ± 1.1; mean weight SDS was 0.3 ± 1.3. Six (35%) subjects (12.2‐21.4 years of age) had anemia with hemoglobin concentrations of 10.6 to 11.6 g/dl. Ultrasound examination showed one or more focal hepatic lesions, consistent with an adenoma in 5 (29%) subjects (10.4 to 21.4 y); 16 subjects had glomerular hyperfiltration; and urinary albumin excretion was increased in 2 subjects, ages 15.9 and 21.1 years.Conclusions:Long‐term continuous glucose therapy with cornstarch, begun in infancy, resulted in mean height 0.7 SDS less than target height. Optimal biochemical control of GSD‐I requires meticulous adherence to an individualized dietary regimen that is based on the results of periodic metabolic evaluation and home blood glucose monitoring. Renal glomerular dysfunction and formation of hepatic adenomata remain serious long‐term complications.
Interferon‐α Treatment of Chronic Hepatitis B in Childhood: A Consensus Advice Based on Experience in European Children
Authors: Jara Paloma , Bortolotti Flavia 
Published: February 2024

ABSTRACTBackground:The efficacy of interferon (IFN) in children with chronic hepatitis B has been evaluated in randomized controlled trials over the past decade, but recommendations for treatment based on this experience have not been published yet. The purpose of this workshop, held in Madrid in October 1997, was to provide pediatricians with guidelines for practical use of IFN in hepatitis B.Methods:Eighteen European pediatricians and hepatologists agreed to report and discuss their experience on 1,122 treated children, 40% of whom were considered responders.Results:Agreement was obtained on the following main items: 1) rationale for treatment is to accelerate hepatitis B early antigen (HBeAg) clearance in a subgroup of patients; 2) candidates for treatment are children with HBeAg and HBV DNA positivity, with low‐intermediate HBV DNA levels and abnormal alanine aminotransferase values, aged 2 years or more; 3) IFN is contraindicated in children with decompensated liver disease, cytopenia, severe renal or cardiac disorder, and autoimmune disease; 4) the standard treatment regimen is 5 mU/m2 thrice weekly for 6 months. Retreatment in nonresponders is not indicated.Conclusions:A consensus was obtained on the use of IFN in children with hepatitis B, based on its short‐term efficacy. The long‐term clinical and virological effects of the drug, however, remain to be evaluated.
Analysis of Anti‐Gliadin Antibodies by Immunoblot Analysis and Enzyme‐Linked Immunosorbent Assay Using Gliadin Fractions As Antigens
Authors: Chirdo Fernando G. , Rumbo Martín , Carabajal Patricia , Castagnino Norma , Mavromatopulos Elizabeth , Cirincione Vilma , Añón María‐Christina , Fossati Carlos Alberto 
Published: February 2024

ABSTRACTBackground:Anti‐gliadin antibody (AGA) determination has been widely used in the screening test to detect celiac patients in the general population and in risk groups. Serological assays present variable efficiency, probably caused by differences in the antigenic mixtures employed as antigen. The objective of this work is to evaluate the use of purified gliadin fractions in an enzyme‐linked immunosorbent assay (ELISA) test.Methods:Anti‐gliadin antibody reactivity was characterized in the sera of patients with celiac disease, and AGA levels were determined by immunoblot analysis using purified gliadin fractions after separation of wheat proteins by sodium dodecyl sulfate‐polyacrylamide gel electrophoresis (SDS‐PAGE) and acid‐PAGE and after indirect ELISA. Seven antigenic mixtures were tested: commercial gliadin, ethanolic wheat extract, and five fast protein liquid chromatography‐purified fractions (ω‐gliadins, two mixtures of α‐/β‐ and β‐/γ‐gliadins). Immunoblot analysis after A‐PAGE separation showed that immunoglobulin (Ig)A reactivity was frequently more restricted than that of IgG. Serum IgA in 15 of 23 patients showed intense reactivity against ω‐gliadins.Results:In seven cases, only ω‐gliadins were detected. To compare the efficiency of ELISA tests, serum samples of 28 patients with celiac disease and 31 control subjects were tested against the seven gliadin fractions. Immunoglobulin G AGAs demonstrated similar levels against the different gliadin fractions, whereas IgA AGAs showed a heterogeneous reactivity that depended on the fraction tested. The lowest number of false‐positive and false‐negative results was obtained when the ω‐gliadin fraction was used. Parameters for ELISA showed that the ω‐gliadin fraction elicited the highest assay efficiency for determinations of both IgA and IgG AGAs. A good correlation was found between IgG and IgA anti‐ω‐gliadin and anti‐endomysial antibody determinations. Of the 28 biopsy‐confirmed patients with celiac disease, 26 samples (23 positive and 3 negative) were found to have concordant results among the three determinations.Conclusions:In this study, an intense and, in many cases, selective recognition of ω‐gliadins was observed. Results suggest that a higher performance in AGA determination could be achieved using ω‐gliadin as an antigen in indirect ELISA.
Normalizing Results of 13C‐Urea Breath Testing for CO2 Production Rates in Children
Authors: Klein Peter D. , Malaty Hoda M. , Czinn Steven J. , Emmons Steven J. , Martin Robert F. , Graham David Y. 
Published: February 2024

ABSTRACTBackground:The 13C‐urea breath test detects the presence of Helicobacter pylori from an enrichment of breath 13CO2, which, in turn, is critically dependent on the amount of dilution by endogenous CO2 production. The production of CO2 differs according to age (adults > children), sex (male > female) weight, and height. The cutoff value of 2.4 Δ‰ (delta over baseline, DOB) for the 13C‐urea breath test, defined in adults, does not take into account actual CO2 production. Therefore, this cutoff value (2.4 Δ‰) may or may not be appropriate for children. The purpose of this study was to determine a cutoff value that would provide accurate results in pediatric patients, independent of their differences in anthropometric parameters.Methods:Estimates of CO2 production were combined with DOB values to calculate the host‐dependent urea hydrolysis rate.Results:Calculated as urea hydrolysis rate, the cutoff range for adults was 10.4 to 10.9 µg/min. Individual ranges were concentric (men, 9.6‐10.9 µg/min; women, 8.5‐12.2 µg/min). Results in studies of 312 children show that a urea hydrolysis rate of more than 10 µg/min may also be appropriate to predict H. pylori infection.Conclusion:Calculating 13C‐urea breath test values as urea hydrolysis rate removes the effect of individual anthropometric differences on test outcome and provides a single cutoff value for pediatric patients of all ages.