bluuebunny/crossref_metadata_2025_split huggingface dataset:
A Prospective Study of the Efficacy and Tolerance of a Chimeric Antibody to Tumor Necrosis Factors (Remicade) in Severe Pediatric Crohn Disease
Authors: Cezard Jean‐Pierre , Nouaili Nizar , Talbotec Cécile , Hugot Jean‐Pierre , Gobert Jean‐Gérard , Schmitz Jacques , Mougenot Jean‐François , Alberti Corinne , Goulet Olivier 
Published: January 2024

ABSTRACTObjectivesTo evaluate the efficacy and toxicity of infliximab in children with severe Crohn disease (CD), the authors prospectively monitored 21 children aged 15 ± 2 years with severe CD who they treated with infliximab (5 mg/kg) on days 0, 15, and 45. One patient received only one injection. Eighteen patients were corticosteroid dependent, and 6 were receiving parenteral nutrition. Three patients were corticoid resistant (1 mg/kg/d >15 days). Sixteen had perianal disease.ResultsThe Harvey‐Bradshaw index (HB) decreased from 8 ± 3 on day 0 to 1 ± 2 on day 45 (P = 0.001). The inflammation factors decreased (P = 0.001), and albumin increased (P = 0.002). Nineteen children were in complete remission (HB < 4) on day 45, and 2 had improved (HB = −6 points). Tumor necrosis factor‐α (TNFα) in the stools (n = 16) decreased (P = 0.04). All perianal fistulas (n = 12) were closed by day 90. Fourteen of 21 patients had stopped taking steroids at 3 months, and all had stopped parenteral nutrition. Growth velocity was significantly greater after infliximab administration (Z score, +0.5) than before (−0.45;P = 0.004). Nineteen of 21 patients had relapsed (90%) at 1 year despite continued immunosuppressors. Seven had surgery because of an uncontrolled relapse (5), stenosis (1), or fistula (1). Six patients developed antinuclear antibodies (1/40–1/640e), and two had anti‐DNA antibodies. Epstein‐Barr virus (EBV) polymerase chain reaction (PCR) values increased (>100‐fold) in eight patients. One child developed an anaphylactic reaction to the medication, and one had a catheter‐related sepsis.ConclusionInfliximab produces spectacular results for children with severe CD and is well tolerated. However, its effect is transitory for many (90%), with frequent relapses despite continued immunosuppressors. Long‐term management with infliximab should be tested despite its worrying side effects.
The Complexity of Feeding Problems in 700 Infants and Young Children Presenting to a Tertiary Care Institution
Authors: Rommel Nathalie , De Meyer Anne‐Marie , Feenstra Louw , Veereman‐Wauters Gigi 
Published: January 2024

ABSTRACTBackground:Feeding problems are common in infants and young children. A multidisciplinary team approach contributes to better patient care. However, few quantitative data on multidisciplinary feeding assessment of children have been published.Objectives:The first aim of this study was to characterize the etiology of feeding difficulties in 700 children referred for assessment of severe feeding difficulty. The authors differentiated medical, oral, and behavioral categories. The second aim was to assess the prevalence of prematurity and dysmaturity in the patients and their relationship to the type of feeding problem.Methods:Clinical data from 700 children aged less than 10 years who were examined for severe feeding problems were analyzed.Results:Close to 50% of the children had a combined medical and oral condition underlying their feeding difficulties. More than half of the children were examined for gastrointestinal conditions, particularly gastroesophageal reflux disease. Behavioral problems were more frequently seen in children aged more than 2 years. The results indicate that oral sensory‐based feeding problems are related to past medical intervention. Children with feeding disorders had a significantly lower birthweight for gestational age. Preterm babies were overrepresented in this population.Conclusions:A multidisciplinary team approach is essential for assessment and management because combined medical and oral problems are the most frequent cause of pediatric feeding problems. A significant relationship was found between the type of feeding problem and age. Infants born preterm and/or with a birthweight below the tenth percentile for gestational age are at greater risk for developing feeding disorders. JPGN 37:75‐84, 2003.
Functional Outcomes of Pediatric Liver Transplantation
Authors: Alonso E. M. , Neighbors K. , Mattson C. , Sweet E. , Ruch‐Ross H. , Berry C. , Sinacore J. 
Published: January 2024

ABSTRACTThe functional status and health‐related quality of life (HRQOL) of children who survive liver transplantation (LT) have not been well documented. The purpose of this study was to determine the functional status and HRQOL in this population using a validated measure for children, the Child Health Questionnaire‐Parent Form 50 (CHQ‐PF50).MethodsThe CHQ‐PF50 instrument was completed by the parents of 55 children who agreed to participate in a mailing survey. Subscale scores for the sample were compared with those of a published normal population (n = 391).ResultsStudy sample characteristics were: 87% Caucasian, 54.5% female, mean age at survey was 9.6 years (range, 5–17 years). Responding caregivers were 95% biologic parents and 93% female. Compared with the normal population, LT recipients had lower subscale scores for general health perceptions (P < 0.0005), emotional impact on parents (<0.0005) and disruption of family activities (0.0005). The mean physical summary score of the LT recipients was lower than that of the normal population 48.1 ± 12.1 (P = 0.005), but the mean psychosocial summary score was similar 48.8 ± 11.9 (P = 0.156). Within the LT population, the original diagnosis (biliary atresia vs. other), type of LT (living donor vs. cadaveric), age at LT, z score for height, and hospital days did not significantly influence any of the subscale scores.ConclusionsChildren who have survived LT have functional outcomes in the physical domain that are lower than those of normal children. Self‐esteem and mental health in this group appeared normal. The parents in this sample experienced more emotional stress and disruption of family activities than did parents in a normal population.
Plasma Markers of Platelet Activation in Cystic Fibrosis Liver and Lung Disease
Authors: Schwarz Kathleen B. , Rosensweig Jeffrey , Sharma Savitri , Jones Lawrence , Durant Michael , Potter Carol , Narkewicz Michael R. 
Published: January 2024

ABSTRACTBackgroundTransforming growth factor beta 1 (TGFβ1) is a major fibrogenic cytokine, the expression of which is increased in the livers of children with cystic fibrosis liver disease (CFLD) and in the bronchoalveolar lavage fluid of patients with cystic fibrosis pulmonary disease (CFPD). The purpose of our study was to investigate the usefulness of plasma TGFβ1 as a noninvasive marker of CFLD and CFPD, or both and to investigate the contribution of platelet‐derived TGFβ1 to plasma TGFβ1 by correlating the latter with platelet factor 4 (PF4).MethodsThree groups of patients with cystic fibrosis were studied: 1) those with CFLD, 2) those with CF and no liver disease (CFNLD), and 3) those with CFPD. Controls were healthy adolescents and adults. Plasma TGFβ1 was assayed using the R and D Quantikine quantitative sandwich enzyme immunoassay technique and PF4 (American Bioproducts ELISA kit).ResultsPlasma TGFβ1 was markedly increased in CFPD (15 ± 3 ng/mL) versus healthy adults (1 ± 0 ng/mL; P < 0.004. The PF4 values followed a similar pattern: 105 ± 8 ng/mL in CFPD versus 12 ± 4 ng/mL (P < 0.0001). Plasma TGFβ1 values in CFLD did not differ from CFNLD patients of comparable age nor from values for healthy adolescents. Plasma TGFβ1 values were strongly correlated with values for PF4:r = 0.543, P < 0.0001.ConclusionsPlasma TGFβ1 is not a useful marker of CFLD. The increased plasma TGFβ1 and PF4 in CFPD patients are of interest both as possible noninvasive markers of pulmonary fibrosis and because the increased values suggest that platelet activation may play a pathogenetic role in CFPD.
Results of a New Technique in the Treatment of Severe Esophageal Stenosis in Children: Poliflex Stents
Authors: Broto Jesús , Asensio Marino , Vernet Jose Maria Gil 
Published: January 2024

ABSTRACTObjectivesThe authors present their experience in treating cases of severe esophageal stenosis resistant to conventional dilatation through the placement of silicone stents, describe a precise placement technique, and establish a standard protocol for the use of these devices.MethodsThe authors detail the placement procedure and follow‐up in a series of 10 patients (5 male, 5 female) between 1 and 23 years, in whom the technique was used. Nine patients had severe esophageal scarring caused by alkaline agents, and another had severe stenosis resulting from esophageal atresia; all were included in the dilatation program from the age of 6 months to 19 years. The method employs a self‐expanding biocompatible stent that is placed using a special applicator over the stenotic area after performing a dilatation. This is maintained long enough to guarantee esophageal scarring without stenosis, permitting oral feeding of the patient. In general, the children experienced nausea and some vomiting during periods lasting from 12 to 48 hours; these episodes were more common when the lesion was higher and longer. Treatment with midazolam and ondansetron relieved the symptoms. All patients received omeprazole while the stent was in place.ResultsFive patients received one stent, four others required two, and another needed three because of restenosis. In the five patients who required stent replacement, a low dose of steroids (0.3 mg/kg/24 h) was administered 2 weeks before and also during the placement time. Currently, five patients are completely cured, with follow‐up ranging from 4 to 19 months. Three patients now require stent replacement, and two others are under observation after having the stent removed. Long‐term tolerance to the stent has been excellent in all cases. The time the stent was in place varied from 20 to 133 days.ConclusionsThe siliconated polypropylene stents (Poliflex/Rüsch) appear to be a promising new tool in the conservative treatment of esophageal stenosis. The stents permit normal feeding during application, substantially reduce treatment time, and avoid repeated anesthesia sessions for dilatations.
Copper Deficiency And Excess In Infancy: Developing A Research Agenda
Authors: Araya Magdalena , Koletzko Berthold , Uauy Ricardo 
Published: January 2024

ABSTRACTCopper deficiency and excess have been recognized as potential health problems for infants and children worldwide. Clinical manifestations of copper deficiency and excess are well characterized but the precise sequence by which high copper intake interacts with genetic control systems, leading to liver damage in infants, is unknown. The possibility that genetic mutations or epigenetic factors related to the functional development of copper homeostasis, could make otherwise normal infants on normal copper intake more susceptible to copper toxicity has been an issue of concern. In January 2001 a group of pediatricians and researchers interested in this area met at Tegernsee, Bavaria, Germany, to reviewing the state of knowledge on the topic. They addressed six main issues: 1) The relevance of copper deficit and excess as health problems. 2) The appropriate biomarkers to identify and characterize copper status 3) The genetic variability in copper metabolism 4) The mechanisms of whole body copper homeostasis in early life and their changes with age 5) The development of experimental and animal models to address research questions on copper homeostasis in infants. 6) The safe upper and lower limits of copper intake/exposure from water and food. We present here the highlights of the discussions and the main conclusions of the meeting.
Bone Mineral Density of the Lumbar Spine in Children and Adolescents With Celiac Disease on a Gluten‐Free Diet in São Paulo, Brazil
Authors: Sdepanian Vera Lucia , de Miranda Carvalho Cecília Noronha , de Morais Mauro Batista , Colugnati Fernando Antonio Basile , Fagundes‐Neto Ulysses 
Published: January 2024

ABSTRACTBackgroundTo compare bone mineral density (BMD) in children and adolescents with celiac disease (CD) and control subjects and to evaluate diet adequacy and calcium metabolism in patients with CD.MethodsThirty patients with asymptomatic CD (17 children, 13 adolescents), on a gluten‐free diet, and 23 healthy subjects were studied. BMD of the lumbar spine (dual energy x‐ray absorptiometry) was performed on all patients and control subjects. In patients, food diaries for nine nonconsecutive days were obtained and analyzed. In patients, laboratory tests pertaining to calcium balance were obtained.ResultsThe mean weight and height of the adolescents with CD were lower than those of control subjects (weight: 45.8 ± 10.5 kg v 55.3 ± 10.5 kg, P = 0.037; height: 153.0 ± 11.0 cm v 167 ± 12.0 cm, P = 0.007). The mean BMD in adolescents with CD was significantly lower than that of the control subjects (0.917 ± 0.116 g/cm2v 1.060 ± 0.158 g/cm2, P = 0.015), whereas no significant difference was found between children with CD and control subjects (P = 0.595). A multiple‐regression model shows that increases in BMD relative to height were lower in adolescents with CD than in control subjects. The proportion of adolescents who had started a gluten‐free diet after 2 years of age was higher than that of children with CD (P < 0.001). High percentages of magnesium, calcium, and phosphorous deficiencies were present in CD patients' diets. The serum levels of ionized and total calcium and parathormone were normal.ConclusionsThe BMD of adolescents with CD was lower than that of the control subjects, whereas no difference was found between the BMD of children with CD and that of control subjects.
Are Proton Pump Inhibitors Indicated for the Treatment of Gastroesophageal Reflux in Infants and Children?
Authors: Rudolph Colin D. 
Published: January 2024

ABSTRACTRandomized controlled trials in the pediatric population that support the efficacy of proton pump inhibitors for the treatment of gastroesophageal reflux (GER) are lacking. Studies are needed to establish the safety of long‐term acid suppression in infants and children, as well as to confirm a causal relationship between GER and extraesophageal symptoms and signs.
Octreotide Therapy for Control of Acute Gastrointestinal Bleeding in Children
Authors: Eroglu Y. , Emerick K. M. , Whitingon P. F. , Alonso E. M. 
Published: January 2024

ABSTRACTObjectivesTo evaluate the outcomes of octreotide use in children with gastrointestinal bleeding with or without portal hypertension.MethodsThe medical charts of children who received continuous octreotide infusion for the indication of gastrointestinal bleeding from January 1995 to December 2000 were retrospectively reviewed.ResultsTwenty‐one children with portal hypertension received octreotide infusions (1–2 μg/kg/h) during 35 gastrointestinal bleeding episodes. The duration of infusion ranged from 19 hours to 7 days. Bleeding ceased in 71% of children. Post‐treatment rebleeding occurred in 52%, and the mortality was 19%. Response to octreotide, rebleeding, and mortality did not differ significantly between patients with or without intrinsic liver disease.Twelve children with gastrointestinal bleeding unrelated to portal hypertension received octreotide during 14 episodes. The duration of octreotide ranged from 3 hours to 36 days. Fifty percent of children had resolution of bleeding during the infusion. Post‐treatment rebleeding occurred in 29%, and the mortality was 50%. No major adverse events were observed in either group.ConclusionsA high rate of bleeding control was achieved with octreotide during acute gastrointestinal bleeding in children with portal hypertension. However, controlled studies are needed for more definitive description of its clinical effects. The role of octreotide is less clear in gastrointestinal bleeding unrelated to portal hypertension.
Gastrointestinal and Other Clinical Manifestations in 17 Children With Congenital Disorders of Glycosylation Type Ia, Ib, and Ic
Authors: Damen Gerard , de Klerk Hans , Huijmans Jan , den Hollander Jan , Sinaasappel Maarten 
Published: January 2024

ABSTRACTObjectivesThe typical signs and symptoms of congenital disorders of glycosylation (CDG) include dysmorphy, failure to thrive, and neurologic abnormalities. However, more and more children diagnosed at a young age are not dysmorphic and do not have neurologic involvement. The authors studied the gastrointestinal and other clinical manifestations of CDG type Ia, Ib, and Ic.MethodsAs of January 2003, 17 children were identified with CDG at the authors' institution. The medical records of the patients were reviewed.ResultsFive children had CDG Ia, three children CDG Ib, and nine children CDG Ic. Age at diagnosis ranged from 2 months to 15 years. Failure to thrive was present in 80% of patients with CDG Ia, in 66% of those with CDG Ib, and in 11% of those with CDG Ic. Five children had protein‐losing enteropathy (two CDG Ia, two CDG Ib, and one CDG Ic). Hepatomegaly was present in 40% of patients with CDG Ia, in 66% of those with CDG Ib, and in 11% of those with CDG Ic. In CDG Ic, hepatomegaly was transient. In CDG Ia, histologic analysis of the liver showed swollen hepatocytes, steatosis, and fibrosis. In CDG Ib, hamartomatous collections of bile ducts were seen. In one patient with CDG Ib, the clinical picture was restricted to congenital hepatic fibrosis for more than a decade.ConclusionsThe study confirms the heterogeneity of the clinical picture in children with CDG type Ia, Ib, and Ic. Children with protein‐losing enteropathy should be tested for CDG. Protein‐losing enteropathy can be caused, not only by CDG Ia and Ib, but also by type Ic. Children with congenital hepatic fibrosis should be tested for CDG, even in the absence of other symptoms. In CDG Ib, histologic analysis of the liver showed hamartomatous collections of bile ducts (Meyenburg complex).
Constipation and Intestinal Neuronal Dysplasia Type B: A Clinical Follow‐up Study
Authors: Schimpl Guenther , Uray Elisabeth , Ratschek Martin , Höllwarth Michael E. 
Published: January 2024

ABSTRACTObjectiveIntestinal neuronal dysplasia type B (IND B) is one of the gastrointestinal motility disorders with a defined malformation of the parasympathetic submucous and myenteric ganglia. The clinical presentation of IND B is variable, ranging from intestinal obstruction in the neonatal period to acute or chronic constipation in childhood.MethodsBetween 1993 and 1996, 105 patients (49 females and 56 males) were treated for constipation, and in all of them an IND type B was confirmed histopathologically. Twenty‐two neonates, 42 infants to 6 months of age (38% of them were premature, and 5% had additional malformations), and 41 patients to the age of 4 years were included in this study.All 105 patients had been treated conservatively. Treatment consisted of diet in all patients, cisapride in 70% of them, laxatives in 52%, and repeated anal dilatations in 12% of the patients. The mean duration of their treatment lasted from 3 months to 10 months (mean, 6 months).ResultsThe clinical follow‐up 5 to 9 years later in 89 of the 108 (85%) patients showed daily defecation in 80% of them and every second day in 14% of them. Only 5 (6%) patients experience recurrent constipation, which responds well to diet and laxatives.ConclusionsIn young patients, constipation related to IND B can be treated successfully by conservative treatment regimens, including diet, laxatives, and prokinetic drugs.
Effect of Baclofen on Emesis and 24‐Hour Esophageal pH in Neurologically Impaired Children With Gastroesophageal Reflux Disease
Authors: Kawai Masanobu , Kawahara Hisayoshi , Hirayama Satoru , Yoshimura Norikazu , Ida Shinobu 
Published: January 2024

ABSTRACTObjectivesGastroesophageal reflux disease (GERD) is difficult to control with medical therapy in neurologically impaired children. The gamma‐aminobutyric acid type B receptor agonist baclofen was recently reported to reduce reflux in adult patients with GERD by reducing the incidence of transient lower esophageal sphincter relaxations. The current study was undertaken to investigate the effects of baclofen on GERD in neurologically impaired children.MethodsEight neurologically impaired children with GERD between 2 months and 16 years were studied. Baclofen (0.7 mg/kg/day) was administered orally or via nasogastric tube in three divided doses 30 minutes before meals for 7 days. The frequency of emesis on and off baclofen were recorded as a measure of clinical impact. Twenty‐four–hour esophageal pH monitoring was conducted before and on the seventh day of the administration of baclofen.ResultsThe frequency of emesis was significantly decreased (P = 0.03). The total number of acid refluxes was significantly decreased both during the entire 24‐hour period (P = 0.01) and during the postprandial period (P = 0.049). The number of acid refluxes longer than 5 minutes was significantly decreased during the 24‐hour period (P = 0.02). The percentage total time of esophageal pH <4.0 and esophageal acid clearance time were not significantly different during the 24‐hour period or during the postprandial period. No adverse effects were observed, except for a slight reduction in muscle tone in one subject.ConclusionsIn this 1‐week trial, repetitive administration of baclofen reduced the frequency of emesis and the total number of acid refluxes in neurologically impaired children with GERD.
The Risk of Celiac Disease in 107 Families With at Least Two Affected Siblings
Authors: Gudjónsdóttir Audur H. , Nilsson Staffan , Ek Johan , Kristiansson Bengt , Ascher Henry 
Published: January 2024

ABSTRACTObjectivesScreening for celiac disease (CD) in the apparently healthy members of 107 nuclear families with two affected siblings (sib pair) and estimating the risk of CD in siblings and parents.MethodsOne hundred seven families from Sweden and southern Norway with at least two affected children were investigated. Frozen sera from 187 of the 192 healthy parents and from 94 of 95 siblings without diagnosed CD were examined for total immunoglobulin A (IgA) and anti‐endomysial antibodies (EMA). Individuals with positive antibody titers underwent small intestinal biopsy.ResultsPositive test for EMA was found in 6 of 94 (6.3%) siblings without previously diagnosed CD and in 8 of 189 (4.2%) parents. CD was confirmed by small intestinal biopsy in all siblings and seven parents. The estimated risk for CD in multiply affected families was 26.3% for siblings and 12.9% for parents. An unexpected male preponderance was found among the new CD cases (10 males, 3 females).ConclusionThe risk of CD in the members of nuclear families with two affected children is approximately three times higher than that when only one child is affected. The high male preponderance of new cases is unexpected and could not be explained fully by more silent disease in males as compared with females. Considering the high level of knowledge about CD in these families, the number of undiagnosed cases is surprisingly high. The authors suggest that serologic screening should be offered to all first‐degree relatives of patients with CD.