bluuebunny/crossref_metadata_2025 huggingface dataset:
Treatment of Cholestatic Children with Water‐soluble Vitamin E (α‐Tocopheryl Polyethylene Glycol Succinate): Effects on Serum Vitamin E, Lipid Peroxides, and Polyunsaturated Fatty Acids
Authors: Socha Piotr , Koletzko Berthold , Pawlowska Joanna , Proszynska Krystyna , Socha Jerzy
Published: February 2024

Background:

Treatment of vitamin E‐deficient cholestatic children with water‐soluble α‐tocopherol polyethylene glycol succinate (TPGS) was previously shown to normalize vitamin E status and to improve neurological outcome.

Methods:

Because vitamin E plays an important role as a free‐radical scavenger, we studied the effects of long‐term TPGS supplementation on lipid peroxidation and polyunsaturated fatty acid status in 15 children ages 9 months‐3.4 years (median, 1.3 years) with chronic cholestasis with low serum vitamin E concentrations [1.95 (0.8‐3.7) mg/L; median (1st‐3rd quartile)]. The previous supplementation of α‐tocopherol was replaced by a 20% solution of TPGS in one daily dose of 20 IU/kg. Serum α‐tocopherol, plasma lipid peroxides expressed as thiobarbiturate reactive substance concentration (TBARS) and plasma phospholipid fatty acid profile were estimated at baseline and again after 1 month in all 15 patients, and after 1 year of TPGS therapy in 11 patients.

Results:

α‐Tocopherol was significantly increased after 1 month [6.9 (4.4‐8.4) mg/L; p = 0.008] and rose further after 1 year [9.7 (7.2‐14.9) mg/L]; similar results were obtained for the ratio vitamin E/total lipids. TBARS concentrations were significantly higher in cholestatic children at baseline [2.9 (1.5‐3.32) nmol/ml] than in a control group [1.2 (1.1‐1.3) nmol/ml; p = 0.0006], but were not changed significantly during TPGS therapy [after 1 year 2.34 (1.9‐3.0) nmol/ml]. Compared with controls, the contributions of polyunsaturated fatty acids to total phospholipid fatty acids were markedly decreased in cholestatic patients at baseline [27.7 (22.4‐31.5)% versus 36.9 (34.5‐39.0)%; p = 0.001] and did not show major changes after 1 year of TPGS supplementation.

Conclusions:

We conclude that oral TPGS supplementation of cholestatic children can quickly normalize serum vitamin E levels but does not improve the increased lipid peroxidation and poor polyunsaturated fatty acid status.
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

Background:

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

Background:

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

Background:

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

Background:

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

Background:

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

Background:

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

Background:

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

Background:

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

Background:

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

Background:

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.
Evaluation of a Long‐Chain Polyunsaturated Fatty Acid Supplemented Formula on Growth, Tolerance, and Plasma Lipids in Preterm Infants up to 48 Weeks Postconceptional Age
Authors: Vanderhoof Jon , Gross Steven , Hegyi Thomas , Clandinin Thomas , Porcelli Peter , DeCristofaro Joseph , Rhodes Torunn , Tsang Reginald , Shattuck Karen , Cowett Richard , Adamkin David , McCarton Cecilia , Heird William , Hook‐Morris Brenda , Pereira Gilberto , Chan Gary , Van Aerde John , Boyle Frances , Pramuk Kathryn , Euler Arthur , Lien Eric L.
Published: February 2024

Background:

The last trimester of pregnancy is a period of rapid accretion of long‐chain polyunsaturated fatty acids, both in the central nervous system and the body as a whole. Human milk contains these fatty acids, whereas some preterm infant formulas do not. Infants fed formulas without these fatty acids have lower plasma and erythrocyte concentrations than infants fed human milk. Preclinical and clinical studies have demonstrated that single‐cell sources (algal and fungal) of long‐chain polyunsaturated fatty acids are bioavailable. A balanced addition of fatty acids from these oils to preterm formula results in blood fatty acid concentrations in low birth weight infants comparable to those of infants fed human milk.

Methods:

In the present study the growth, acceptance (overall incidence of discontinuation, reasons for discontinuation, overall incidence and type of individual adverse events), and plasma fatty acid concentrations were compared in three groups of infants fed a long‐chain polyunsaturated fatty acid‐supplemented preterm infant formula, an unsupplemented control formula, or human milk. The study was prospective, double‐blind (formula groups only), and randomized (formula groups only). Two hundred eighty‐eight infants were enrolled (supplemented formula group, n = 77; control formula group, n = 78; human milk group, n = 133).

Results:

Anthropometric measurements at enrollment, at first day of full oral feeding, and at both 40 and 48 weeks postconceptional age did not differ between the formula groups, whereas the human milk‐fed group initially grew at a lower rate. The incidence of severe adverse events was rare and not significantly different between formula groups. The groups fed either human milk or supplemented formula had long‐chain polyunsaturated fatty acid concentrations higher than those in the control formula group.

Conclusions:

The results of this study demonstrate the safety and efficacy of a preterm formula supplemented with long‐chain polyunsaturated fatty acids from single‐cell oils.
Fruit Juice Consumption and the Prevalence of Obesity and Short Stature in German Preschool Children: Results of the DONALD Study
Authors: Alexy Ute , Sichert‐Hellert Wolfgang , Kersting Mathilde , Manz Friedrich , Schöch Gerhard
Published: February 2024

Background:

In recent years, a possible association between excessive consumption of fruit juice (≥12 fl oz per day) and short stature and/or obesity has been discussed. The association among the consumption of fruit juice, anthropometric indices, and the overall diet was examined during a 3‐year period in a sample of healthy preschool children participating in the Dortmund Nutritional and Anthropometrical Longitudinally Designed (DONALD) Study.

Methods:

Two hundred five children were examined annually at the ages of 3, 4, and 5 years. Dietary intake was calculated from 3‐day weighed diet records. Height was measured using a stadiometer. Weight was measured using an electronic scale.

Results:

Five children consumed excessive fruit juice continually in all three records, 10 children in two records, and 23 children in one record. None of the five children with repeatedly excessive fruit juice consumption was obese or short. Growth velocity, body mass index, and height standard deviation score were not correlated with fruit juice consumption. Consumption of fruit juice was inversely correlated with the consumption of all other beverages and the total consumption of all other food. The intake of protein, fat, and carbohydrates of children consuming excessive fruit juice was closer to the international dietary preventive guidelines than the intake of children consuming low amounts of fruit juice.

Conclusions:

In the study sample, even repeatedly excessive fruit juice consumption had no influence on anthropometric indices. The results do not justify a general warning or a general promotion regarding high fruit juice consumption in preschool children's diets.
Protein Hydrolysate Formula Maintains Homeostasis of Plasma Amino Acids in Preterm Infants
Authors: Mihatsch Walter A. , Pohlandt Frank
Published: February 2024

Background:

Plasma amino acid concentrations were measured in preterm infants who were fed either a new hydrolyzed cow's milk protein formula or a standard preterm infant formula. It was hypothesized that feeding with the hydrolysate results in preprandial amino acid concentrations that are significantly different from the concentrations found when feeding with the standard formula.

Methods:

Fifteen preterm infants, median gestational age, 29 weeks (range, 24‐32 weeks); birth weight, 1241 g (range, 660‐1900 g); and postnatal age, 18 days (range, 7‐54 days) receiving full enteral feedings (>150 ml/kg·day), were enrolled. The intervention was randomized allocation to the formula with hydrolyzed or natural cow's milk protein (the whey/casein ratio was 60:40 in both formulas). In a crossover design, each formula was fed for 5 days, and plasma amino acids were analyzed on day 4 or 5 of each 5‐day period.

Results:

In spite of the 12% higher amino acid intake with hydrolysate formula, the median individual plasma amino acid concentrations were virtually identical with both formulas, and they were within the 10th and the 90th percentile of the reference of levels in the umbilical cord artery after elective cesarean delivery or of breast‐fed newborn infants. The median concentrations of lysine and aspartic acid were higher with hydrolyzed formula feeding (p < 0.05; two‐tailed Mann‐Whitney test). With both formulas, single amino acid concentrations were out of the reference values.

Conclusion:

Virtually identical plasma amino acid concentration patterns were measured with the new hydrolyzed preterm infant formula and the standard preterm infant formula, but longitudinal studies are required before the studied protein hydrolysate can be recommended for preterm feeding in general.
Are Endoscopic Biopsies of Small Bowel as Good as Suction Biopsies for Diagnosis of Enteropathy?
Authors: Thomson Mike , Kitching Paul , Jones Alison , Walker‐Smith John A. , Phillips Alan
Published: February 2024

Background:

Endoscopy is increasingly used in paediatric practice for diagnosis of enteropathy, although the quality of grasp forceps‐obtained biopsy specimens required for reliable diagnosis has been questioned in comparison with suction capsule biopsy specimens. This study prospectively compared the diagnostic suitability of grasp forceps biopsy versus suction biopsy in the same patient during the same procedure.

Methods:

A double‐port paediatric suction biopsy capsule was front‐loaded onto an endoscope and directed to the fourth part duodenum‐proximal jejunum for biopsy sampling. Subsequently, three grasp biopsy specimens were taken from the same region. All biopsies were coded, photographed, and measured for area, using computed morphometry. A single blinded histopathologist assessed sample adequacy for diagnosis. Twenty‐nine patients were enrolled (age range, 8‐185 months).

Results:

On three occasions the suction capsule failed to fire, and on four occasions only one sample was obtained. Three grasp biopsy specimens were obtained on each occasion by endoscopy, and the first two were used for comparison with suction biopsy samples. Median total area of individual biopsy samples obtained by the two procedures was not different (21.3 vs. 22.5 mm2; P = 0.027). Muscularis mucosae was obtained more commonly with grasp biopsies (P < 0.001), and no difference was observed for the presence of three or more villuscrypt units, degree of haemorrhage, or optimal orientation. Two suction biopsy procedures and one grasp biopsy procedure were inadequate for diagnosis.

Conclusions:

Endoscopic grasp biopsies are perfectly adequate for the assessment of small intestinal histology. In addition, endoscopy affords advantage in diagnosis of other upper gastrointestinal disease with avoidance of radiologic screening involved with the suction capsule technique.
Colon Involvement in Langerhans' Cell Histiocytosis
Authors: Nanduri Vasanta Rao , Kelly Kara , Malone Marian , Milla Peter , Pritchard Jon
Published: February 2024

Background:

Langerhans' cell histiocytosis (LCH), a granulomatous disorder of unknown cause, most often affects the bony skeleton and skin. Obvious gut involvement is uncommon, and colon involvement has been cited in only a small number of case report, probably because most patients who have LCH with diarrhoea and/or failure to thrive are not investigated by colonoscopy and biopsy. The current study was conducted to determine the incidence of symptomatic colon involvement among patients with multisystem LCH treated at a single institution.

Method:

A retrospective review of cases in hospital records and the literature.

Results:

Of the 275 children with LCH in the database, 5 were identified as having biopsy‐confirmed colonic involvement. Another 14 cases were identified by a literature review.

Conclusion:

Colonic involvement in patients with multisystem LCH is probably more common than currently recognised. In addition to the other investigations recommended by the Writing Group of the Histiocyte Society, it is recommended that patients with any symptoms suggestive of gut involvement undergo colonoscopy and biopsy of the colonic mucosa.
The Caffeine Metabolic Ratio as an Index of Xanthine Oxidase Activity in Clinically Active and Silent Celiac Patients
Authors: Boda Márta , Németh Ilona , Boda Domokos
Published: February 2024

Background:

The xanthine oxidoreductase system has been identified as one of the main sources of free radicals responsible for various forms of tissue injury. Because the intestinal villi are an important location of this enzyme, it was of interest to study the role of xanthine oxidase in gluten‐sensitive celiac enteropathy, associated with characteristic villous atrophy. Measured by a noninvasive method, the ratio of caffeine metabolites excreted in the urine after a caffeine challenge had previously been shown to be indicative of the total xanthine oxidase activity of the patient.

Methods:

The study involved 22 children with gluten‐challenged celiac disease, exhibiting subtotal villous atrophy in specimens from the third intestinal biopsy in accordance with ESPGHAN criteria. Ten of the patients displayed overt clinical symptoms (active form), whereas 12 had no symptoms (silent form). Urinary caffeine metabolites were determined by high‐pressure liquid chromatography. The total in vivo xanthine oxidase activity was expressed as the caffeine metabolite index.

Results:

In patients with active celiac disease the xanthine oxidase activity index was considerably higher, whereas in those with silent disease it was significantly lower than the control value. A significant negative correlation was shown between the index indicative of xanthine oxidase activity and the serum iron level of the patients.

Conclusions:

Activation of xanthine oxidase may play a role in the pathogenesis of active celiac disease with definite malabsorption, gastrointestinal symptoms, and anemia. The caffeine test reflects the difference in the pathogenetic mechanism leading to the mucosal lesion and clinical symptoms of active and silent forms of celiac disease.
Double‐Blind Randomized Controlled Trial of Glutamine‐Enriched Polymeric Diet in the Treatment of Active Crohn's Disease
Authors: Akobeng Anthony K. , Miller Victor , Stanton John , Elbadri Abubaker M. , Thomas Adrian G.
Published: February 2024

Background:

Glutamine is traditionally considered a nonessential amino acid but may be conditionally essential in patients with catabolic conditions. Glutamine‐supplemented foods in these patients have been shown to prevent deterioration of gut permeability, protect against the development of intestinal mucosal atrophy, and improve nitrogen balance. Animal models of inflammatory bowel disease suggest that glutamine‐enriched enteral diets may lead to less severe intestinal damage, less weight loss, improved nitrogen balance, and reduced disease activity. The purpose of the current study was to compare the efficacy of a glutamine‐enriched polymeric diet with a standard low‐glutamine polymeric diet in the treatment of active Crohn's disease.

Methods:

Eighteen children with active Crohn's disease were randomly assigned to receive a 4‐week course of either a standard polymeric diet with a low glutamine content (4% of amino acid composition; group S) or a glutamine‐enriched polymeric diet (42% of amino acid composition; Group G). The two diets were isocaloric and isonitrogenous with an identical essential amino acid profile. Remission rates were analysed on an intent‐to‐treat basis. Changes in clinical and laboratory parameters of disease activity were also compared after 4 weeks of nutritional treatment.

Results:

Two of the children, both in group G, were withdrawn from the trial because of nontolerance of the diet. There was no difference between the two groups in proportion of patients achieving remission (intent‐to‐treat basis): 5 (55.5%) of 9 in group S versus 4 (44.4%) of 9 in group G (p = 0.5). Improvement in mean paediatric Crohn's disease activity index (PCDAI) was significantly more in group S (p = 0.002) but changes in orosomucoid level, platelet count, and weight were not different between the groups.

Conclusions:

The findings suggest that a glutamine‐enriched polymeric diet offers no advantage over a standard low‐glutamine polymeric diet in the treatment of active Crohn's disease. Rather, it appears to be less effective in improving PCDAI. The reported beneficial effects of glutamine seen in many catabolic states must be viewed with caution when extrapolating to the management of Crohn's disease.
Electrogastrography Versus Gastric Emptying Scintigraphy in Children With Symptoms Suggestive of Gastric Motility Disorders
Authors: Barbar Maha , Steffen Rita , Wyllie Robert , Goske Marilyn
Published: February 2024

Background:

Cutaneous electrogastrography is a method of recording gastric electrical activity. Abnormalities of the electrogastrogram have been described in a variety of disorders. The purpose of the study was to correlate the electrogastrograms of children with vomiting and dyspepsia with the results of radionucleotide gastric emptying studies.

Methods:

Nine patients (5–16 years old) with gastrointestinal symptoms of vomiting and/or abdominal pain were studied. The electrogastrogram was recorded using surface electrodes for 30 minutes in the fasting state and for 120 minutes after a radioisotope‐labeled solid meal. Gastric emptying was simultaneously monitored for 120 minutes. The postprandial change in dominant power (power ratio: postprandial/fasting dominant power), percentages of normal slow wave, bradygastria, and tachygastria were recorded and analyzed.

Results:

The patients were divided into two groups. The first group (four patients; five studies) had normal gastric emptying, whereas the second group (five patients) had delayed emptying (half‐life, >90 minutes). The median power ratio in the first group was 1.69 and in the second group was 2.78; the difference was not statistically significant (P = 0.90). The median difference in slow wave percentages in the fasting and postprandial periods was 0.99 in the first group and 0.73 in the second group; again, the difference was not statistically significant (P = 0.27).

Conclusions:

Although it is a method of assessing gastric myoelectrical activity and gastric motility disorders, electrogastrogram does not correlate with nuclear scintigraphic gastric emptying studies in children.
Inflammatory Bowel Disease in Children and Adolescents in Sweden, 1984–1995
Authors: Lindberg Eva , Lindquist Bo , Holmquist Lars , Hildebrand Hans
Published: February 2024

Background:

A prospective study of inflammatory bowel disease (IBD) in Sweden was performed to investigate whether the incidence and morbidity have changed from 1984 through 1995.

Methods:

Children 15 years of age or less with IBD were included—i.e., those with a definite diagnosis of ulcerative colitis (UC) and Crohn's disease (CD) and those classified as having indeterminate colitis (IC) and probable Crohn's disease (PCD). The study covered 56.5% of the pediatric population of Sweden.

Results:

The diagnosis of IBD was made in 639 children, which corresponds to a mean annual incidence of 5.8 per 100,000. The incidence increased from 4.6 per 100,000 per year from 1984 through 1986 to 7.0 from 1993 through 1995. It reflected an increase in UC from 1.4 to 3.2 per 100,000 per year, which is a significant yearly percentage of increase (8%; confidence interval, 2–14%;P < 0.05). In contrast, no change occurred in the incidence of CD (1.2–1.3 per 100,000). The incidence of IC and PCD also remained fairly stable. The percentages of children who underwent surgery decreased from 17.3% in the first 6 years to 4.6% in the last 6 years (P < 0.001). Surgery was performed in 27.7% of CD and 5.3% of UC cases. The median age at diagnosis was 12.2 years for UC, 13.0 years for CD, 11.2 for IC, and 11.2 for PCD. At diagnosis, 48 children (7.5%) were 5 years of age or less, whereas most of the patients were 11 years of age or more (398 children, 62.3%).

Conclusions:

In Sweden, the incidence of UC has increased, whereas that of CD remains the same. A significant number of children were classified with IC and PCD. In most children, IBD was diagnosed when they were 11 years old or more, but some cases were detected even in those below 6 years of age. A decrease in the frequency of surgery occurred during the study.
Intestinal Colonization Leading to Fecal Urobilinoid Excretion May Play a Role in the Pathogenesis of Neonatal Jaundice
Authors: Vítek Libor , Kotal Petr , Jirsa Milan , Malina Jiří , Černá Marcela , Chmelař Dittmar , Fevery Johan
Published: February 2024

Background:

Neonatal hyperbilirubinemia remains of concern because of the potential danger for the central nervous system. Because urobilinogen is a nontoxic derivative of bilirubin, the current study was conducted to examine the fecal excretion of urobilinoids and bilirubin in healthy newborns and infants, as well as their intestinal bacteria capable of reducing bilirubin, to assess a possible relation to serum bilirubin levels during the first weeks of life.

Methods:

Bilirubin pigments, urobilinoids, and porphyrins were measured in stools of infants during the first week (group A, n = 60) and between the second week and the first 6 months of life (group B, n = 64). Microbiologic analysis of stools was performed in selected cases and bilirubin‐converting activity of isolated bacteria was determined in vitro.

Results:

Urobilinoids were detectable in stools of 57% of the neonates at day 5, but not before. However, fecal urobilinoid production on that day was only a fraction of that observed in adults (0.07 vs. 0.7–3.6 mg/kg per day), whereas at week 6 it increased significantly to an average of 0.9 mg/kg per day. Microbiologic analysis of neonatal stools revealed two novel bacterial strains of Clostridium perfringens and Clostridium difficile capable of reducing bilirubin to urobilinoids.

Conclusions:

Urobilinoids can be detected in stools of 57% of newborns at day 5 after delivery. However, the urobilinoid production during the first week of life is quantitatively insufficient to contribute significantly to the removal of bilirubin. Enhancement of the microbial conversion of bilirubin could decrease the intestinal concentration of bilirubin and may decrease the degree or enhance the removal of neonatal hyperbilirubinemia.
Screening by Anti‐Endomysium Antibody for Celiac Disease in Diabetic Children and Adolescents in Austria
Authors: Schober Edith , Bittmann Barbara , Granditsch Gerhard , Huber Wolf‐Dietrich , Hüppe Amelie , Jäger Andrea , Oberhuber Georg , Rami Birgit , Reichel Gerlinde
Published: February 2024

Background:

Unrecognized celiac disease (CD) may be found in a substantial proportion of patients with type 1 diabetes mellitus.

Methods:

A cohort of 403 Austrian children and adolescents with type 1 diabetes mellitus (210 males and 193 females; age range, 1–22 years) was screened for celiac disease using the IgA anti‐endomysium antibody test (EMA) and the immunoglobulin (Ig)G anti‐gliadin (AGA‐IgG) and IgA anti‐gliadin (AGA‐IgA) antibody test.

Results:

Twelve patients' sera (2.98%) yielded positive EMA results and two patients' sera (0.49%) with IgA deficiency had high AGA‐IgG values. All but one of these patients underwent intestinal biopsy. Six (1.49%) had clear histologic evidence of CD (flat mucosa), whereas three (0.74%) showed minor histologic changes (increase in intraepithelial lymphocytes) and four (0.99%), including the EMA‐negative patients with IgA deficiency, had a normal mucosa. When the cases with silent and potential CD were combined, the overall prevalence in the current cohort was 2.98%. There was no difference in the hemoglobin (Hb)A1c level between antibody‐positive and ‐negative patients, and subsequent gluten‐free diet did not change this metabolic parameter.

Conclusion:

The prevalence of clinically unrecognized CD, found by EMA screening, is much higher in Austrian children with diabetes than in a comparable population without diabetes. The prevalence of CD in diabetic children in Austria is distinctly lower, however, than in several other countries.
Normal Growth Velocity Before Diagnosis of Celiac Disease
Authors: Lejarraga Horacio , Caíno Silvia , Salvador Alexander , De Rosa Susana
Published: February 2024

Background

Clinical experience leads us to believe there may be patients with celiac disease who have not yet been treated, with a normal physical growth.

Methods

To evaluate height velocity of patients with confirmed celiac disease before their diagnosis and treatment, anthropometric measurements taken by the general pediatricians in charge of the primary care of the patients before they were sent to the authors' hospital were studied. Forty‐two growth periods (available velocities) were measured at varying intervals (ranging from 6 to 27 months) in 23 patients aged 0.1 to 10.66 years were analyzed.

Results

All patients studied during the first semester of life (n = 5) showed normal growth velocity, and 6 of 10 patients showed normal growth velocity during the second semester of life. Ten of 12 patients between the ages of 1.0 and 1.99 years of age showed normal height velocity, and 7 of 9 patients aged 2.0 to 10.66 years showed normal height velocity. Normal height velocities were found not only during the first year of life, but also in children aged 1 to 8 years.

Conclusions

Results should alert pediatricians and those in gastroenterology and growth clinics. In the latter case, norms for studying children who have short stature but are growing at a normal rate should not be a condition for excluding a child from screening for celiac disease.