bluuebunny/crossref_metadata_2025_split huggingface dataset:
Histopathologic Features of the Liver in Pediatric Acquired Immune Deficiency Syndrome
Authors: Jonas Maureen M. , Roldan Eneida O. , Lyons Hernando J. , Fojaco Rita M. , Reddy Rajender K. 
Published: February 2024

Summary:Autopsy and liver biopsy specimens from 30 pediatric patients with acquired immune deficiency syndrome (AIDS) or AIDS‐related complex (ARC) were retrospectively reviewed. Of 28 cases with histologic abnormalities, the following findings were noted singly or in combination: giant‐cell transformation, cytomegalovirus inclusions, Kaposi's sarcoma, diffuse lymphoplasmocytic infiltrate, granulomatous hepatitis, mild portal inflammation, necrosis around central veins, steatosis, and cholestasis. For the most part, abnormalities in the liver were not predictive of those in other organs, but the two children with the diffuse parenchymal lymphoplasmocytic infiltrate also had lymphoid interstitial pneumonitis (LIP). Liver histopathology in pediatric patients with AIDS shares some features with that in adults, but appreciable differences are noted. In particular, these differences include the higher frequency of giant‐cell transformation and the lower frequency of granulomas in children and the observation of diffuse lymphoplasmocytic infiltrate associated with LIP.
The Cyclic Vomiting Syndrome Described
Authors: Fleisher David R. 
Published: February 2024

Summary:Cyclic vomiting syndrome (CVS) consists of discrete episodes of nausea and vomiting lasting hours or days, separated by symptom‐free intervals of similar or varying lengths. The disorder is not caused by underlying organic disease. The episodes tend to be similar in time of onset, duration, and symptomatology specific for each patient and are self‐limited. The majority of patients can identify experiences or conditions that may precipitate episodes, the commonest being heightened emotional states and infections. The onset of CVS most often occurs during preschool or early school years, although it may begin at any age, from infancy to mature adulthood. CVS episodes can recur for months or decades, and their subsidence cannot be predicted.
Apolipoprotein B Arg3500Gln Mutation Prevalence in Children With Hypercholesterolemia: A French Multicenter Study
Authors: Viola Sheila , Benlian Pascale , Morali Alain , Dobbelaere Dries , Lacaille Florence , Rieu Daniel , Ginies Jean Louis , Maurage Chantal , Meyer Martine , Lachaux Alain , Larchet Michel , Lenearts Catherine , Goulet Olivier , Sarles Jacques , Mouterde Olivier , Girardet Jean Philippe ,   French‐Speaking Group for Pediatric Hepatogastroenterology Nutrition
Published: February 2024

ABSTRACTBackgroundFamilial defective apolipoprotein B‐100, a dominantly inherited form of hypercholesterolemia caused by a single Arg3500Gln mutation, is silent in childhood but may confer a high risk of cardiovascular disease in adulthood. The objective was to determine the prevalence of familial defective apolipoprotein B‐100 in hypercholesterolemic French children and to provide a basis for targeting screening efforts in this population.MethodsOne hundred ninety children attending 13 pediatric clinics distributed throughout France were included based on the presence of type IIa hypercholesterolemia with a plasma low‐density lipoprotein–cholesterol level of more than 130 mg/dL. The Arg3500Gln mutation was detected in dried blood spots using a polymerase chain reaction assay combined with enzymatic restriction.ResultsThree hyperlipidemia phenotypes were found: monogenic dominant pure hypercholesterolemia (n = 117), polygenic hypercholesterolemia (n = 43), and combined hyperlipidemia (n = 11). Three unrelated children were heterozygous for the Arg3500Gln mutation; all three had monogenic dominant pure hypercholesterolemia (3/94 families; 3.2%), yielding a prevalence of 1.83% (3/164) in hypercholesterolemic children, which is similar to prevalences reported in European adults.ConclusionsThe familial defective apolipoprotein B‐100 mutation was common (1/31) in children with a phenotype of familial hypercholesterolemia, supporting screening in this population with the goal of preventing premature cardiovascular events.
Prevalence and Clinical Picture of Celiac Disease in Italian Down Syndrome Patients: A Multicenter Study
Authors: Bonamico Margherita , Mariani Paolo , Danesi Helene Maria , Crisogianni Massimo , Failla Pinella , Gemme Gerolamo , Quartino Alberto Rasore , Giannotti Aldo , Castro Massimo , Balli Fiorella , Lecora Margherita , Andria Generoso , Guariso Graziella , Gabrielli Orazio , Catassi Carlo , Lazzari Rosanna , Balocco Nicoletta Ansaldi , De Virgiliis Stefano , Culasso Franco , Romano Corrado ,   SIGEP,   Medical Genetic Group
Published: February 2024

ABSTRACTBackgroundA multicenter research study of Down syndrome patients was carried out to estimate the prevalence of celiac disease in patients with Down syndrome and to show clinical characteristics and laboratory data of Down syndrome patients.MethodsThe authors studied 1,202 Down syndrome patients. Fifty‐five celiac disease patients (group 1) were compared with 55 immunoglobulin A antigliadin–positive antiendomysium antibodies–negative patients (group 2) and with 57 immunoglobulin A antigliadin–negative antiendomysium antibodies–negative patients (group 3).ResultsCeliac disease was diagnosed in 55 of 1,202 Down syndrome patients (4.6%). In group 1, weight and height percentiles were shifted to the left, whereas these parameters were normally distributed in groups 2 and 3. In celiac patients, diarrhea, vomiting, failure to thrive, anorexia, constipation, and abdominal distension were higher than in the other two groups. Low levels of hemoglobinemia, serum iron, and calcium were observed more frequently in group 1. The diagnosis of celiac disease was made after a mean period of 3.8 years from the initiation of symptoms. Sixty‐nine percent of patients showed a classic presentation, 11% had atypical symptoms, and 20% had silent celiac disease. Autoimmune disorders were more frequent (30.9%) in group 1 than in the other two groups examined (15%;P < 0.05).ConclusionsThis study reconfirms a high prevalence of celiac disease in Down syndrome. However, the diagnostic delay, the detection of atypical symptoms or silent form in one third of the cases, and the increased incidence of autoimmune disorders suggest the need for the screening of celiac disease in all Down syndrome patients.
Effects of Long‐term Consumption of a Fermented Infant Formula (with Bifidobacterium breve c50 and Streptococcus thermophilus 065) on Acute Diarrhea in Healthy Infants
Authors: Thibault H. , Aubert‐Jacquin C. , Goulet O. 
Published: February 2024

ABSTRACTObjective:To determine whether long‐term consumption of a fermented infant formula could influence the incidence of acute diarrhea and its severity in healthy infants.Method:Nine hundred seventy‐one infants, ranging in age from 4 to 6 months, were included in a randomized, double‐blind, placebo‐controlled trial during a period of 5 months. They consumed daily either a fermented infant formula (FF) (fermentation with Bifidobacterium breve C50 and Streptococcus thermophilus 065) or a standard infant formula (SF) of the same nutritional composition.Evaluation Criteria:Number and duration of acute diarrhea episodes were evaluated. Severity of the episodes was determined by the number of hospital admissions, incidence of dehydration, number of medical consultations, number of oral rehydration solution prescriptions, and number of formula switches.Results:Growth of the infants and acceptability of the formulas were identical in the two groups. Incidence, duration of diarrhea episodes, and number of hospital admissions did not differ significantly between groups. Episodes were less severe in the FF (fermented formula) group. There were fewer cases of dehydration 2.5%versus 6.1% (P = 0.01), fewer medical consultations (46%v 56.6%, P = 0.003), fewer ORS prescriptions 41.9%v 51.9% (P = 0.003) and fewer switches to other formulas (59.5%v 74.9%, P = 0.0001) in FF infants compared to SF.Conclusion:A fermented formula may reduce the severity of acute diarrhea among healthy young infants. This outcome may be linked to the bifidogenic effects of fermentation products and their interactions with the intestinal immune system.
Intestinal Inflammation Measured by Fecal Neopterin in Gambian Children With Enteropathy: Association With Growth Failure, Giardia lamblia, and Intestinal Permeability
Authors: Campbell David I. , McPhail Graham , Lunn Peter G. , Elia Marinos , Jeffries Donald J. 
Published: February 2024

ABSTRACTObjectives:Investigate whether fecal neopterin concentration (a potential marker of gut inflammation) in Gambian children with enteropathy was associated with growth failure. Secondary outcome measures tested the associations between Giardia lamblia infestation, fecal neopterin and lactulose mannitol absorption ratio(L:M), a measure of intestinal permeability.Methods:Seventy‐two children had height and weight measured every 6 to 8 weeks until 15 months of age in a rural Gambian village. L:M ratio, a measure of small intestinal permeability and fecal neopterin were measured at these times. Stool was examined by immunoflorescence and light microscope for Giardia cysts.Results:Long‐term height and weight gains were negatively associated with mean subject fecal neopterin concentration (r = −0.29 and −0.36, respectively; P < 0.001). There was no correlation between fecal neopterin and intestinal permeability or history of diarrhea. Of 72 children studied, 19 had Giardia cysts in stool and 38 had negative stool examinations. Infected children had a mean of 0.7 days of diarrhea/week (95% confidence interval [CI], 0.31–1.03) versus 0.8 days/week (95% CI, 0.71–0.85) in uninfected children. No difference in growth was detected between those with positive or negative fecal smears. Mean L:M ratio was the same in both groups (0.31; 95% CI, 0.26–0.34).Conclusions:Consistent with the theory that intestinal inflammation in tropical infants may impair growth, fecal neopterin concentrations were inversely associated with growth. Factors other than Giardia are causing enteropathy and growth failure in Gambian infants.
Diagnostic Role of Upper Gastrointestinal Endoscopy in Pediatric Inflammatory Bowel Disease
Authors: Castellaneta S. P. , Afzal N. A. , Greenberg M. , Deere H. , Davies S. , Murch S. H. , Walker‐Smith J. A. , Thomson M. 
Published: February 2024

ABSTRACTBackground:Discrimination between ulcerative colitis (UC) and Crohn disease (CD) may be difficult on ileo‐colonoscopy alone because of a lack of definitive lesions. Retrospective studies show upper gastrointestinal endoscopy may be helpful in confirming diagnosis in such cases.Aims:To prospectively determine importance of upper gastrointestinal endoscopy in diagnosis of inflammatory bowel disease (IBD) and assess factors predictive of upper gastrointestinal involvement in IBD.Methods:All pediatric patients were enrolled prospectively and consecutively over a 2‐year period and investigated with an ileo‐colonoscopy and barium meal follow‐through. Children with procto‐sigmoiditis, later confirmed histologically to be typical of UC, were excluded from the study. The remainder underwent upper gastrointestinal endoscopy. The protocol and methodology were determined a priori.Results:65 children suspected of IBD underwent colonoscopy. Of the total, 11 had recto‐sigmoiditis with typical macroscopic appearances of UC; once this was confirmed on histology these patients were excluded from the study. Of the 54 children (males, 31; median age, 11.1 years) remaining, 23 were initially diagnosed with CD on ileo‐colonoscopy and 18 (33%) were diagnosed with UC. The diagnosis remained ambiguous in 13 (six colonic, four ileo‐colonic, three normal colon) on clinical, radiologic and histologic grounds. Upper GI endoscopy helped to confirm CD in a further 11 (20.4%). Two patients were diagnosed with indeterminate colitis.Upper gastrointestinal inflammation was seen in 29 of 54 (22 CD; 7 UC). Epigastric and abdominal pain, nausea and vomiting, weight loss and pan‐ileocolitis were predictive of upper gastrointestinal involvement (P < 0.05). However, 9 children with upper gastrointestinal involvement were asymptomatic at presentation (31%). Overall upper gastrointestinal tract inflammation was most common in the stomach (67%), followed by the esophagus (54%) and duodenum (22%).Conclusions:Upper gastrointestinal tract endoscopy should be part of the first‐line investigation in all new cases suspected of IBD. Absence of specific upper gastrointestinal symptoms do not preclude presence of upper gastrointestinal inflammation.
Preparation and Handling of Powdered Infant Formula: A Commentary by the ESPGHAN Committee on Nutrition
Authors: Agostoni Carlo , Axelsson Irene , Goulet Olivier , Koletzko Berthold , Michaelsen Kim F. , Puntis John W. L. , Rigo Jacques , Shamir Raanan , Szajewska Hania , Turck Dominique , Vandenplas Yvan , Weaver Lawrence T. 
Published: February 2024

ABSTRACTPowdered infant formulae are not sterile and may contain pathogenic bacteria. In addition, milk products are excellent media for bacterial proliferation. Multiplication of Enterobacter sakazakii in prepared formula feeds can cause devastating sepsis, particularly in the first 2 months of life. In approximately 50 published case reports of severe infection, there are high rates of meningitis, brain abscesses and necrotizing enterocolitis, with an overall mortality from 33% to 80%. Breast feeding provides effective protection against infection, one of the many reasons why it deserves continued promotion and support. To minimize the risk of infection in infants not fully breastfed, recommendations are made for preparation and handling of powdered formulae for children younger than 2 months of age. In the home setting, powdered infant formulae should be freshly prepared for each feed. Any milk remaining should be discarded rather than used in the following feed. Infant feeds should never be kept warm in bottle heaters or thermoses. In hospitals and other institutions written guidelines for preparation and handling of infant formulae should be established and their implementation monitored. If formula needs to be prepared in advance, it should be prepared on a daily basis and kept at 4°C or below. Manufacturers of infant formulae should make every effort to minimize bacterial contamination of powdered products.
Nutrition of Very Low Birth Weight Infants Fed Human Milk with or without Supplemental Trace Elements: A Randomized Controlled Trial
Authors: Loui Andrea , Raab Andrea , Wagner Mathias , Weigel Heidrun , Grüters‐Kieslich Annette , Brätter Peter , Obladen Michael 
Published: February 2024

ABSTRACTBackground:Very low birth weight infants (<1500 g) have high nutritional needs. Deficiencies of minerals, trace elements (especially zinc) may develop as a result of rapid growth, low body stores and low content of these substances in human milk We hypothesized that fortification of human milk might prevent deficiencies.Methods:Prospective, randomized trial to evaluate mineral, trace element, thyroid status and growth of infants fed human milk fortified with different amounts of calcium, phosphorus and protein, with (BMF) or without (FM 85) trace elements. Sixty‐two infants, 1000 to 1499 g birth weight, were randomized. Minerals and trace elements in serum, red blood cells and human milk and alkaline phosphatase activity, TSH, T4 and FT4 in serum were measured once until the fifth day and at 3 and 6 weeks of life. Clinical course and anthropometric measurements were recorded.Results:Intake of zinc, copper, manganese, calcium, phosphorus and magnesium was higher in the BMF group (P < 0.001). Serum zinc concentrations <0.49 mg/L occurred in 12% of the FM 85 group and 7% of the BMF group at 6 weeks (not significant). Median alkaline phosphatase activity was 436/379 IU/L in the FM 85/BMF group at 6 weeks (P < 0.01). The FM 85 group showed a higher weight gain (P < 0.05), possibly because of higher caloric (P < 0.01) and protein intake (P < 0.05) at 3 weeks.Conclusions:Zinc deficiency was rare. Elevated intake of calcium, phosphorus and zinc was associated with lower serum alkaline phosphatase activity but did not influence serum zinc concentration.
Distribution of Sialic Acids in the Milk of Spanish Mothers of Full Term Infants During Lactation
Authors: Martín‐Sosa Samuel , Martín María‐Jesús , García‐Pardo Luis A. , Hueso Pablo 
Published: February 2024

Objectives:The protective effect of human milk against infection is well known. Several non‐immunologic components, including complex carbohydrates, have been described. The present study was undertaken to determine the sialic acid distribution in different milk fractions (complex carbohydrates).Methods:Milk samples from 12 Spanish women at three different lactational stages (colostrum, transitional milk and mature milk) were analyzed. Total and glycoprotein‐bound, oligosaccharide‐bound, casein‐bound, and lipid‐bound sialic acids were determined.Results:Sialic acids from human milk are mainly bound to oligosaccharides and only a small amount is present bound to glycoproteins or in the free form. All the fractions analyzed showed a similar trend: sialic acids decrease rapidly along lactation. Casein‐bound sialic acid does not follow this trend. We detected the presence of an O‐acetylated species of N‐acetylneuraminic acid.Conclusions:In human milk from Spanish women we observed slightly different values than those previously reported. This could be a result of population differences but nutritional or methodological aspects can not be discarded.
Comparison of Invasive and Non‐Invasive Tests Diagnosis and Monitoring of Helicobacter Pylori Infection in Children
Authors: Hino B. , Eliakim R. , Levine A. , Sprecher H. , Berkowitz D. , Hartman C. , Eshach‐Adiv O. , Shamir Raanan 
Published: February 2024

ABSTRACTBackground:There are few reports which the tests used for diagnosing Helicobacter pylori infection and monitoring its eradication in children.Study Aims:Prospective evaluation of invasive (gastric histology, rapid urease test [RUT]) and non‐invasive (stool antigen [FemtoLab H. pylori], urea breath test [UBT]) tests in the diagnosis of H. pylori infection and post‐treatment eradication in children and adolescents.Methods:Ninety‐two patients (50 male, 42 female) referred for upper gastrointestinal endoscopy were prospectively enrolled. UBT was performed and stool specimens collected for monoclonal enzyme immunoassay for H. pylori antigen (FemtoLab) 1 to 4 days before endoscopy. H. pylori in gastric biopsies was evaluated by RUT and staining with hematoxylin‐eosin and giemsa. Eradication therapy was given to children with abdominal pain and H. pylori gastritis. FemtoLab H. pylori and UBT were repeated 6 weeks after the end of triple therapy.Results:Histology identified H. pylori in 49 of 92 (53%) subjects. Concordance between histology and RUT was found in 78 of 92 children. FemtoLab H. pylori was positive in 41 of 78 (52.6%) children with sensitivity, specificity, positive and negative predictive values of 97.5%, 94.7%, 95.1% and 97.3%, respectively. For UBT, these values were 100%, 96.9%, 97.5% and 100%, respectively. Twenty‐six of 36 patients who received triple therapy returned for eradication evaluation. Tests for H. pylori antigen in stool were positive in 10 of 26 and for UBT in 11 of 26.Conclusion:Stool antigen (FemtoLab) and UBT were equally effective in diagnosing and confirming eradication of H. pylori infection in children.
Association of Serum Interleukin‐8 Levels with the Degree of Fibrosis in Infants with Chronic Liver Disease
Authors: Nobili Valerio , Marcellini Matilde , Giovannelli Luigi , Girolami Elia , Muratori Flaminia , Giannone Germana , Devito Rita , De Benedetti Fabrizio 
Published: February 2024

ABSTRACTObjective:Biliary atresia is a neonatal obstructive cholangiopathy characterized by a destructive, obliterative process affecting both the intrahepatic and extrahepatic ducts of the biliary tree that uniquely presents in the first months of life. The consequence of progressive inflammatory and sclerotic reaction is the development of obstructive jaundice. To determine the proinflammatory cytokine profile in children with biliary atresia, we measured circulating levels of interleukin (IL)‐1β, IL‐6, tumor necrosis factor‐α and IL‐8.Methods:Twelve children, five males and seven females, with biliary atresia were studied. In addition, four patients with progressive familial intrahepatic cholestasis and three with Alagille syndrome were also included. Five patients with neonatal hepatitis were studied as controls of a liver disease without portal fibrosis. Serum concentration of total and conjugated bilirubin, γ‐glutamyl transferase and glutamic‐pyruvic transaminase were measured by routine methods in all patients at time of sampling for the study. The degree of fibrosis in liver biopsies was scored using the histologic activity index.Results:In our study IL‐8 was detectable in 11 of 12 patients with biliary atresia with a median level of 262 pg/ml and a highly statistically significant difference (P < 0.0001) from controls. In patients with progressive familial intrahepatic cholestasis or with Alagille syndrome serum IL‐8 levels were similarly elevated. In patients with neonatal hepatitis, IL‐8 levels were marginally increased. Serum IL‐8 levels were significantly correlated (Rs = 0.725, P < 0.0001) with the histologic activity index.Conclusions:Although further studies are needed to determine the role of IL‐8 in portal inflammation, our results suggest that increased production of IL‐8 may be a mechanism leading to the progressive portal inflammation and fibrosis in patients with chronic liver disease.
Intestinal Barrier Function and Weight Gain in Malnourished Children Taking Glutamine Supplemented Enteral Formula
Authors: Lima Aldo A. M. , Brito Lúcia F. B. , Ribeiro Hildênia B. , Martins Ma Ceci V. , Lustosa Amália P. , Rocha Edna M. , Lima Noélia L. , Monte Cristina M. G. , Guerrant Richard L. 
Published: February 2024

ABSTRACTObjective:We examined the effect of standard formula and glutamine or glycine supplemented enteral formula on intestinal permeability and weight gain in children with malnutrition.Methods:80 children aged 2 to 60 months with a weight‐for‐age z‐score less than −2 were studied. From December 1996 to April 1999, 27 study patients received nonsupplemented formula. From June 2001 to June 2002 an additional 53 patients were randomly assigned to receive formula supplemented with glutamine or glycine (isosmolar concentrations) for 10 days. Lactulose/mannitol excretion ratio was used as a measure of intestinal permeability and was performed before and after 10 days of nutritional rehabilitation. Weight was measured before and after treatment.Results:Patients were similar on admission with regard to age, sex, nutritional status and lactulose/mannitol ratio. The lactulose/mannitol ratio significantly improved (decreased) in children receiving formula supplemented with glutamine for 10 days but not in those receiving glycine or nonsupplemented formula. Weight gain occurred during therapy in all groups and was not statistically different among groups.Conclusion:Formula supplemented with glutamine improves intestinal barrier function compared with nonsupplemented formula but does not augment weight gain.