bluuebunny/crossref_metadata_2025_split huggingface dataset:
Natural History of Intestinal Failure, Investigated Through a National Network‐Based Approach
Authors: Guarino Alfredo , De Marco Giulio ,   Italian National Network for Pediatric Intestinal Failure
Published: January 2024

ABSTRACTObjectivesIntestinal failure (IF) is a condition whose treatment requires advanced knowledge and techniques and a multidisciplinary approach. Intestinal failure is the endpoint of many intestinal diseases and may result in full recovery, in life‐long parenteral nutrition, or in the death of the child. The aim of this study was to evaluate the natural history of IF in children using a national network of resources.MethodsItalian centers of pediatric gastroenterology merged in a national network, developing a collaborative management approach for children with IF. A consensus definition of IF was achieved. A database was set up to investigate the cause, epidemiologic factors, and natural history of IF.ResultsOne hundred nine children were enrolled in 5 years. The cause of IF was: short bowel syndrome (n = 48), disorders of motility (n = 16), structural enterocyte defects (n = 14), multiple food intolerance (n = 10), autoimmune enteropathy (n = 7), and others or unknown (n = 14). The eventual outcome was closely related to the primary cause, ranging from full and permanent intestinal sufficiency in children with multiple food intolerance to high death rate or total dependance upon parenteral nutrition in those with structural enterocyte defects. Intermediate outcomes were observed for the other causes. Four children received intestinal transplantation.ConclusionsThe network approach for IF provides an effective model to optimize resources and to investigate prospectively the natural history of IF. Based on the work on this series, a European network for IF could be an effective model for fulfilling the diagnostic and management needs, including intestinal transplantation.
Varicella in Pediatric Liver Transplant Patients: A Retrospective Analysis of Treatment and Outcome
Authors: Pacini‐Edelstein Susan J. , Mehra Mini , Ament Marvin E. , Vargas Jorge H. , Martin Martin G. , McDiarmid Sue V. 
Published: January 2024

ABSTRACTBackgroundVaricella is a common childhood disease that can cause morbidity and mortality among immunosuppressed patients. There have been few previous studies monitoring the course of pediatric liver transplant patients with acute varicella. The aim of this study was to evaluate the treatment, outcomes, and complications of pediatric liver transplant patients admitted with acute varicella infection.MethodsA retrospective chart review was carried out based on discharge diagnoses of orthotopic liver transplant and varicella among pediatric patients (age range, birth–18 years) admitted to the UCLA Medical Center between 1985 and 2001.ResultsFive hundred fifty‐six pediatric patients received liver transplantations between 1985 and 2001. Twenty‐two of these patients were admitted to the UCLA Medical Center with varicella (11 females, 11 males). No patients were treated on an outpatient basis. Mean age of the patients was 6 years (range, 1–16 years). None of these patients received the varicella vaccine before hospitalization. On admission, 5 of 22 patients (23%) had received varicella zoster immunoglobulin within 96 hours of exposure. The mean length of hospitalization was 6 days (range, 2–11 days). All immunosuppression dosages were reduced during the admissions. None of the patients had been treated with high‐dose corticosteroids for acute rejection before the onset of the varicella infection. Patients were treated until defervescence with intravenous acyclovir and until their varicella lesions crusted. Patients were discharged with oral acyclovir to complete a 10‐day course (including the intravenous treatment). No patients had complications from the varicella infection. A complication of an elevated serum creatinine for one patient was noted with the intravenous acyclovir treatment. This patient had associated headache and nausea that resolved when the creatinine level returned to normal.ConclusionsThere were no complications or dissemination of varicella infection among our pediatric liver transplant patients. Further prospective randomized trials are required to evaluate the management of pediatric liver transplant patients infected with varicella.
Autoantibodies in Mothers of Children with Neonatal Liver Disease
Authors: Burch Joanna M. , Sokol Ronald J. , Narkewicz Michael R. , Reichlin Morris , Frank Mark Barton , MacKenzie Todd , Lee Lela A. 
Published: January 2024

ABSTRACTObjectivesNeonatal lupus erythematosus (NLE) is associated with maternal anti‐Ro/La autoantibodies. It is characterized by heart block and/or cutaneous skin lesions, and occasionally liver disease. This study was performed to determine whether idiopathic neonatal cholestasis (INC) represents NLE without its cardiac or cutaneous findings.MethodsSera were obtained for autoantibody analysis from mothers of children with INC (N = 11), biliary atresia (N = 25), other liver disease excluding viral hepatitis (liver disease control subjects, N = 14), and healthy children (normal control subjects [NC], N = 22).ResultsThe characteristic serologic findings of NLE, high titer antibodies to Ro and/or La, were absent in mothers from all groups. An unexpected finding was the prevalence of autoantibodies in mothers of infants with liver disease of any type. The frequency of maternal antinuclear antibodies at ≥ 1:120 dilution was greater than the estimated frequency in the general population (22% vs. 9%, P = 0.044). The frequency of maternal low titer autoantibodies to 52 kD Ro detected by ELISA was significantly greater than in the NC group (31% vs. 5%, P = 0.014).ConclusionsThe majority of cases of INC do not represent NLE. The frequent presence of autoantibodies in mothers of infants in all neonatal liver disease groups raises the possibility that maternal serologic autoimmunity is associated with neonatal liver disease.
Adult Height and Weight of Breast‐Fed and Bottle‐Fed Israeli Infants
Authors: Zadik Zvi , Borondukov Ela , Zung Amnon , Reifen Ram 
Published: January 2024

ABSTRACTBackgroundBreast‐fed infants grow more slowly than bottle‐fed infants. This growth deceleration sometimes alarms health care personnel to the point of considering other forms of nutrition.ObjectivesTo evaluate the final adult anthropometric outcome associated with breast or formula feeding during infancy.DesignHeight and weight data were collected from eight well‐baby clinics representing various ethnic origins, lifestyles, and socioeconomic backgrounds. Children were measured every 1 to 2 months for the first 6 months, every 3 months until 2 years of age, and yearly thereafter, until they reached their final height. Longitudinal data were collected from 1960 healthy children (961 boys). Overall, 613 of the children were breast fed for 1 year and 218 for 6 months.ResultsThe magnitude of the decline in Z scores of breast‐fed vs. bottle‐fed infants, between birth and 1 year of age was not as great for height as for weight −0.2 and −0.3 respectively, and disappeared at 2 years of age. The weight for height decreased between birth and the end of the first year in breast‐fed children by 0.3 (Z score). Children switched to bottle feeding exhibited a growth spurt. However, there was no difference in the final heights or weights of breast‐fed children compared with bottle‐fed children 165.3 ± 6.2 (n = 134) versus 164.9 ± 6.4 (n = 195) in females, respectively, and 175.3 ± 6.8 (n = 122) versus 175.8 ± 7.1 (n = 162) in males, respectively. Adult obesity in this sample population (n = 637) was correlated with maternal obesity. Maternal BMI SD correlated with offspring BMI SD at 18 years of age (r = 0.873, P < 0.001) but not with breast feeding. Adult BMI was similar between the breast‐fed and bottle‐fed groups.ConclusionsDespite their slower growth rate, breast‐fed children reach the same final height as bottle‐fed children. Breast‐fed infants should be monitored according to specifically designed growth charts. Obesity in adult life is correlated with factors not related to breast feeding.
Nonsyndromic Paucity of Interlobular Bile Ducts: Report of 10 Patients
Authors: Yehezkely‐Schildkraut Vered , Munichor Mariana , Mandel Hanna , Berkowitz Drora , Hartman Corina , Eshach‐Adiv Orly , Shamir Raanan 
Published: January 2024

ABSTRACTObjectivesOnly a few reports of nonsyndromic paucity of interlobular bile ducts (NS‐PILBD) have been published. The authors' aim was to outline the clinical and laboratory profile of patients with NS‐PILBD diagnosed at a tertiary referral center.MethodsThe authors reviewed all the reports of pediatric liver biopsies performed between 1991 and 2000 at their institution. Upon diagnosis of NS‐PILBD, patients' records were examined for clinical, laboratory, and histologic data, and liver biopsy specimens were re‐evaluated.ResultsThree hundred biopsies were performed in children during the study period, of which 64 were in infants younger than 1 year. NS‐PILBD was diagnosed in 10 of 64 (16%) biopsy specimens. Mean age at presentation was 10 days (range, 1 day–6 weeks), and mean follow‐up was 4.5 years (range, 1–9 years). An underlying condition was identified in 70% of children with NS‐PILBD: namely congenital cytomegalovirus (n = 2), progressive familial intrahepatic cholestasis (PFIC, n = 2), mitochondrial DNA depletion (n = 1), Niemann‐Pick type C (n = 1), and arthrogryposis multiplex congenita, renal dysfunction, and cholestasis (ARC syndrome; n = 1). All children presented with jaundice. Four children had initially acholic stools. At their last follow‐up visit, failure to thrive was present in five children, and cholestasis in six children. Mortality was noted only in children with metabolic diseases (n = 2).ConclusionsIn the study, NS‐PILBD was common in young children undergoing liver biopsy. Although NS‐PILBD is nonspecific, a wide survey for inborn errors of metabolism should be included in the diagnostic work‐up of NS‐PILBD. In the authors' center, the association of certain metabolic diseases with NS‐PILBD carries a grave prognosis.
Fecal α1‐Antitrypsin Concentrations as a Measure of Enteric Protein Loss After Modified Fontan Operations
Authors: Fujii Tohru , Shimizu Toshiaki , Takahashi Ken , Kishiro Masahiko , Ohkubo Mataichi , Akimoto Katsumi , Yamashiro Yuichiro 
Published: January 2024

ABSTRACTBackgroundLittle is known about the enteric protein loss in patients after a modified Fontan operation before the appearance of overt symptoms or signs of protein‐losing enteropathy (PLE). The authors examined the possibility of using fecal α1‐antitrypsin concentration measurements for the early detection of postoperative PLE and in longer term postoperative monitoring of these patients.MethodsThe authors compared fecal α1‐antitrypsin concentrations in stool samples from 12 children 12.0 to 43.7 months after modified Fontan operations with those of 12 age‐matched control subjects and examined the relationship between the α1‐antitrypsin levels and time since operation. The authors also compared fecal α1‐antitrypsin concentrations of stools from the same patients obtained at two different time points after surgery with intervals between samples ranging from 14.7 to 19.8 months.ResultsNo significant differences in serum total protein and albumin levels were observed between patients after the modified Fontan operation and control subjects. The fecal concentrations of α1‐antitrypsin in patients after the Fontan operation were significantly (P < 0.01) higher than those in control subjects. There was no significant correlation between fecal α1‐antitrypsin concentrations and time elapsed after the Fontan operation. The fecal α1‐antitrypsin concentration increased significantly (P < 0.01) over periods of 14.7 to 19.8 months after the first measurement.ConclusionThe results show that enteric protein loss begins before the appearance of hypoproteinemia in patients after a modified Fontan operation, and that the measurement of fecal α1‐antitrypsin concentrations in random stool samples is useful as an early indicator. To watch for the development of PLE after Fontan operation, it may be important to perform longitudinal follow‐up examinations of enteric protein loss by measuring fecal α1‐antitrypsin concentrations early in the postoperative period.
Acid Secretion From Birth to Adulthood
Authors: Boyle John T. 
Published: January 2024

ABSTRACTAll infants, including premature infants as young as 24 weeks' gestational age, are able to maintain an intragastric pH below 4 from the first day of life. By 24 weeks, maximal acid output approaches that in adults. In adult studies, proton pump inhibitors (PPIs) are highly effective in relieving reflux symptoms and healing reflux esophagitis. Studies are needed to evaluate PPI efficacy and appropriate dosing in infants and children. Consequences such as hypergastrinemia, bacterial overgrowth, and other potential adverse events associated with long‐term PPI use also warrant evaluation in the pediatric population.
The Role of Lower Esophageal Sphincter Function and Dysmotility in Gastroesophageal Reflux in Premature Infants and in the First Year of Life
Authors: Davidson Geoffrey 
Published: January 2024

ABSTRACTTransient relaxations of the lower esophageal sphincter (tLESRs), but not delayed gastric emptying, are major mechanisms of gastroesophageal reflux in premature infants. These findings are similar to those seen in older children and adults with gastroesophageal reflux disease. Newer antireflux therapies should be developed that target the reduction of gastric acidity or reduction in the number of tLESRs.
Endoscopic Rectal Sparing in Children With Untreated Ulcerative Colitis
Authors: Rajwal Sanjay R. , Puntis John W. L. , McClean Patricia , Davison Suzanne M. , Newell Simon J. , Sugarman Ian , Stringer Mark D. 
Published: January 2024

ABSTRACTBackgroundUlcerative colitis (UC) typically is associated with a confluent proctitis, whereas rectal sparing may be seen in large bowel Crohn disease (CD). A few studies have reported rectal sparing in UC and suggested that this might indicate a more severe form of the disease. This study aimed to determine the prevalence and prognostic significance of rectal sparing in children with newly diagnosed, untreated UC.MethodsThe records of all children with untreated UC presenting to a regional pediatric gastroenterology service between January 1996 and December 2001 were retrospectively reviewed. Patients were divided into two groups according to the endoscopic appearance of the rectum: Group 1 (proctitis) and Group 2 (rectal sparing). Clinical features, intractability index (duration of active disease as a proportion of length of follow‐up), response to treatment, relapse index (number of recurrences per year), and the need for surgery were compared.ResultsThirty children with untreated UC were identified. Seven (23%) had rectal sparing at initial endoscopy, but disease distribution was otherwise similar in both groups. Presenting symptoms were similar in those with and without rectal sparing. In Group 1, 20 (87%) children achieved remission with initial medical treatment, compared with 3 (43%) in Group 2 (P < 0.05). The intractability index was higher in children with rectal sparing, but the difference was not statistically significant (P = 0.22). During a median follow‐up period of 2 years, one (4%) child in Group 1 and two (29%) children in Group 2 experienced primary sclerosing cholangitis, and two (29%) children with rectal sparing required colectomy, compared with none in Group 1.ConclusionsEndoscopic rectal sparing was seen in 23% of children with newly diagnosed, untreated UC, but this feature did not correlate with presenting symptoms. However, the presence of rectal sparing may indicate more aggressive disease that is less responsive to medical treatment.
Gluten‐Free Diet Has a Beneficial Effect on Chromosome Instability in Lymphocytes of Children With Coeliac Disease
Authors: Kolaček Sanja , Jadrešin Oleg , Petković Iskra , Mišak Zrinjka , Sonicki Zdenko , Booth Ian Westerby 
Published: January 2024

ABSTRACTObjectives.Children with coeliac disease (CD) have an increased number of chromosome aberrations in peripheral blood lymphocytes. Whether genetically determined or a secondary phenomenon in CD, chromosome abnormalities may be involved in the predisposition to cancer in CD patients. The aim of the study was to follow a group of children with CD in whom the initial frequency of chromosome aberrations at diagnosis was known and to measure the same variable after a minimum of 2 years on a gluten‐free diet.Methods.Chromosome aberrations in peripheral blood lymphocytes were determined in 17 patients with CD, before and after at least 24 months of a gluten free diet (mean, 33 months), and in 15 healthy children. The differences in the frequency of aberrations were analyzed by Mann‐Whitney U test and Wilcoxon matched‐pairs signed‐ranks test.Results.Twelve patients adhered to the diet and had a significantly lower frequency of chromosome aberrations than did 5 patients not following the diet (0.16%v 1.2%; P = 0.03), whereas at presentation there had been no difference (1.54%v 1.2%; P = 0.09). The frequency of aberrations at follow‐up in patients who were diet adherent was significantly lower than at presentation (1.54% v 0.16%; P = 0.02) and remained unchanged in patients who were not diet adherent (1.2%v 1.2%; P = 1). After at least 24 months of a gluten‐free diet, children with CD did not differ from healthy control subjects (0.16%v 0.27%; P = 0.54), whereas children not following the diet had an increased frequency of aberrations (1.2%v 0.27%; P = 0.05).Conclusions.The frequency of chromosome aberrations in peripheral blood lymphocytes of patients with CD decreased significantly on a gluten‐free diet. We conclude that genomic instability is a secondary phenomenon, possibly caused by chronic intestinal inflammation.
Serum Lipase After Secretin Stimulation Detects Mild Pancreatic Involvement in Cystic Fibrosis
Authors: Walkowiak Jaroslaw , Nousia‐Arvanitakis Sanda , Lisowska Aleksandra , Piotrowski Robert , Strzykala Krystyna , Bychowiec Bartosz , Songin Tomasz , Galli‐Tsinopoulou Assimina , Sands Dorota , Pidonia Ifigenia , Krawczynski Marian , Milanowski Andrzej , Herzig Karl‐Heinz 
Published: January 2024

ABSTRACTBackground:The assessment of severe pancreatic insufficiency in cystic fibrosis (CF) is not a diagnostic problem. However, identification of mild cases remains a challenge. The aim of this study was to assess the ability of serum lipase after secretin stimulation to identify mild pancreatic insufficiency in patients with CF.Material and Methods:Thirty patients with CF and pancreatic insufficiency (CF‐PI) and 30 patients with CF and pancreatic sufficiency (CF‐PS) were studied. Thirty healthy subjects with no known gastrointestinal disease served as controls. In all subjects, fecal fat excretion, fecal elastase‐1 (E1) concentration and basal and secretin‐stimulated serum lipase concentration were measured.Results:All patients with CF‐PI and 3 with CF‐PS had abnormally low fecal E1 concentrations. The remaining 27 CF‐PS patients and all controls had normal values. Basal and post‐stimulation lipase levels were extremely low in patients with CF‐PI. Mean basal and poststimulation serum lipase concentrations were significantly higher in CF‐PS who had normal fecal E1 concentrations but were still below those of controls (P < 0.001). Among the 27 CF‐PS patients with normal fecal elastase, high basal and poststimulation lipase values were found in 6 and 17 patients respectively.Conclusion:In patients with CF‐PS who have normal fecal elastase‐1 concentration, the measurement of basal or secretin‐stimulated lipase levels might be helpful in identifying the progression of the destructive process in the pancreas.
Biotherapeutic and Nutraceutical Agents: Working Group Report of the Second World Congress of Pediatric Gastroenterology, Hepatology, and Nutrition
Authors: Yamashiro Yuichiro , Castaneda Carlos , Davidson Geoff , Gibson Glenn , Penna Francisco José , Mack David , Splawski Judy 
Published: January 2024

Research
Characterize the taxonomy and features of candidate probiotics.
Determine the intestinal effects of probiotics: infectious diarrhea, necrotizing enterocolitis, inflammatory bowel diseases.
Determine extraintestinal effects: microflora manipulation, immunomodulation and allergy.
Intervention
Regulation and control of probiotic use as drugs through government agencies.
Application of good clinical practice guidelines to probiotic use.
Regulation and control of functional food and proper labelling.
Education
For physicians: what are probiotics and what are their specific indications.
For consumers: what is functional food and what can it do to improve health.
Celiac Disease and Other Immunologically Mediated Disorders of the Gastrointestinal Tract: Working Group Report of the Second World Congress of Pediatric Gastroenterology, Hepatology, and Nutrition
Authors: Troncone Riccardo , Bhatnagar Shinjini , Butzner Decker , Cameron Don , Hill Ivor , Hoffenberg Edward , Maki Markku , Mendez Virginia , de Jimenez Mabel Zacur 
Published: January 2024

Celiac Disease
Research

Treatment alternatives to the gluten‐free diet.
Definition of new diagnostic criteria.

Practical interventions to improve child health

Improved serologic testing.
Worldwide accepted definition of gluten‐free, labeling issues, availability and measures to monitor and assure compliance.

Educational needs and strategies to improve medical knowledge

Celiac disease awareness campaign

Food Allergy
Research

Identification of the whole spectrum of disorders due to food allergy.

Intervention

Identification and implementation of prevention strategies for food allergy.

Educational needs and strategies to improve medical knowledge

Diagnosis of food allergy, with a specific warning against non‐orthodox diagnostic tests.
Hepatic Failure and Liver Transplant: Working Group Report of the Second World Congress of Pediatric Gastroenterology, Hepatology, and Nutrition
Authors: Baker Alastair , Alonso M. Estella , Aw Marion Margaret , Ciocca Mirta , Porta Gilda , Rosenthal Philip 
Published: January 2024

Research
Definition(s) of acute liver failure in childhood.
Establish reliable predictive (prognostic) factors for acute liver failure.
Gain insight into the mechanisms that control proliferation of residual hepatocytes.
Intervention
Establish guidelines for the recognition of acute liver failure and optimal hospital management.
Establish an international committee to evaluate ethical aspects of obtaining materials and criteria to balance risk with benefit in treatment strategies devised to prevent liver transplantation.
Conduct randomized multicenter‐controlled trials to establish optimal use, indications and complications of liver support devices in acute liver failure.
Education
Provide primary health workers with guidelines for early recognition of acute liver failure and optimal hospital management including transfer to services with intensive care facilities and orthotopic liver transplantation.
Provide such guidelines with particular emphasis on treatments seen as harmless, and disseminate them to all health professionals likely to be involved with acute liver failure.