bluuebunny/crossref_metadata_2025_split huggingface dataset:
North American Indian Cirrhosis in Children: A Review of 30 Cases
Authors: Drouin Eric , Russo Pierre , Tuchweber Beatriz , Mitchell Grant , Rasquin‐Weber Andrée 
Published: January 2024

ABSTRACTBackgroundNorth American Indian childhood cirrhosis (NAIC) is a distinct, rapidly evolving form of familial cholestasis found in aboriginal children from northwestern Quebec. This is a retrospective review of the 30 patients treated in Quebec since the discovery of NAIC in 1970.MethodsThe clinical records and histologic samples from 30 patients were reviewed. Extensive metabolic, biochemical, viral, genetic, and radiologic studies were performed in most patients.ResultsGenetic analysis suggests autosomal recessive inheritance and a carrier frequency of 10% in this population. Gene mapping studies showed that the NAIC gene is located on chromosome 16q22. Typically, patients have neonatal cholestatic jaundice (70%) or hepatosplenomegaly (20%) with resolution of clinical jaundice by age 1 year but persistent direct hyperbilirubinemia. Portal hypertension was documented in 29 patients (91%). Variceal bleeding (15 patients, 50%) occurred as early as age 10 months. Surgical portosystemic shunting was performed in 13 of these 15 patients (87%); 4 (31%) rebled after 1 to 5 years. Fourteen patients died (47%). In 10 (71%), liver disease was the cause. Four children died of liver failure before liver transplantation became available. In transplanted livers, no recurrence of NAIC was observed after 1 to 10 years. Recognized infectious, metabolic, toxic, autoimmune, and obstructive causes of cirrhosis have been eliminated. The histologic features of NAIC show early bile duct proliferation and rapid development of portal fibrosis and biliary cirrhosis, suggesting a cholangiopathic phenomenon.ConclusionTogether with gene mapping studies showing that the NAIC gene is different from those of other familial cholestases, these observations suggest that NAIC is a distinct entity that could be classified as “progressive familial cholangiopathy.”
Measurement of Total Body Water in Children Using Bioelectrical Impedance: A Comparison of Several Prediction Equations
Authors: Beertema Wieske , van Hezewijk Marjan , Kester Arnold , Forget Pierre‐Philippe , van Kreel Bernard 
Published: January 2024

ABSTRACTBackgroundBody composition evaluation by bioelectrical impedance analysis in children makes use of different group‐specific population‐derived equations. The present study was conducted to attempt to validate the use of population‐independent physical model–derived equations in children.MethodsThe validity of bioelectrical impedance analysis for the measurement of total body water in children was evaluated by comparing results of two physical model–derived and two population‐derived equations with those of deuterium dilution as reference method in a group of 38 heterogeneous children.ResultsMeans ± standard deviation (in liters) for total body water measured with deuterium dilution and the physical model 1–derived equation were 18.4 ± 4.7 L and 18.1 ± 4.4 L, respectively. This difference is not significant, whereas significant differences were found for all other tested equations. Significant smaller absolute differences between the model 1 equation and deuterium reference results were found when compared with the results of the other three tested equations.ConclusionWhen compared with results of the reference deuterium method the physical model 1–derived equation was the only one that provided reliable total body water results by bioelectrical impedance analysis in children.
Smaller Volume, Thickened Formulas in the Management of Gastroesophageal Reflux in Thriving Infants
Authors: Khoshoo Vikram , Ross Gerald , Brown Shannan , Edell Dean 
Published: January 2024

ABSTRACTBackgroundThe effect of smaller volume, thickened formulas on gastroesophageal reflux is not clear.MethodsThe frequency of gastroesophageal reflux and duration of acid pH in the esophagus were determined in six thriving infants using extended esophageal pH monitoring.ResultsThere was a significant reduction in frequency of emesis and gastroesophageal reflux but not the duration of acid pH in the esophagus with the use of infant formula thickened with rice cereal to provide a nutritionally appropriate intake in a smaller volume.ConclusionsThickening of formula with rice cereal in a nutritionally balanced form and smaller volume may be an appropriate strategy for reducing frequency of emesis and gastroesophageal reflux in thriving infants.
Autoimmune Hepatitis as a Late Complication of Liver Transplantation
Authors: Hernandez Hector M. , Kovarik Paula , Whitington Peter F. , Alonso Estella M. 
Published: January 2024

ABSTRACTBackgroundThe development of de novo autoimmune hepatitis as a long‐term complication after liver transplantation has been recently reported. The authors describe five liver allograft recipients who developed chronic hepatitis associated with autoimmune features.MethodsFive of 155 liver transplant recipients at risk (2.5%) developed this particular form of graft dysfunction. The authors review the clinical records, liver histology, therapy, and outcome of these five patients.ResultsPatients included two boys and three girls. Median age at transplantation was 3.5 years (range, 0.5–14 years), median age at presentation was 9 years (range, 2–17 years), and median interval after transplantation was 5.1 years (range, 1.5–9 years). Indications for liver transplant included biliary atresia in four patients and primary sclerosing cholangitis in one patient. At the time of presentation, all patients were receiving cyclosporine as their primary immunosuppressive agent. Only one patient had a history of rejection, which had resolved. All patients presented with increased transaminase levels, and one had a mildly elevated conjugated bilirubin level. Only one patient had constitutional complaints. Acute and chronic rejection, viral hepatitis, vascular insufficiency, and biliary tract obstruction were excluded. Antinuclear antibody levels were elevated in four patients (titer range, 1:160–1:640), one of whom also had positive antismooth muscle antibody (titer 1:80) results. The fifth patient had an elevated serum total protein level. Histologic analysis of liver biopsy samples from the five patients showed findings consistent with chronic autoimmune hepatitis. All patients were treated with standard therapy for autoimmune hepatitis, which included daily steroids and azathioprine. Cyclosporine doses were reduced in three patients and eliminated in two. All patients responded with normalization (n = 2) or improvement (n = 3) of liver transaminases within the first 3 months of therapy. Histologic analysis of the 3‐month follow‐up liver biopsy was normal (n = 2) or showed improvement in inflammation (n = 2). Two patients developed acute allograft rejection within 6 to 12 months after discontinuation or reduction in cyclosporine.ConclusionsAutoimmune hepatitis occurs after liver transplantation in patients without a previous history of autoimmune hepatitis. The risk of developing autoimmune hepatitis appears to be greater in children after liver transplantation than in the general pediatric population. Standard therapy for autoimmune hepatitis is effective.
Helicobacter pylori and Meckel's Diverticula
Authors: Finn Laura S. , Christie Dennis L. 
Published: January 2024

ABSTRACTBackgroundHelicobacter pylori is known to infect only gastric mucosa and is strongly associated with gastroduodenal ulceration. The authors studied whether H. pylori colonizes the gastric mucosa of Meckel's diverticula, and determined its relationship to “gastritis” and bleeding.MethodsA 10‐year retrospective review identified 45 children with Meckel's diverticulum. Hematoxylin–eosin and Diff‐Quik stains were used to assess the presence and severity of gastritis, and to highlight organisms in the resected diverticula. Cases with organisms were then studied with antibodies specific for H. pylori using immunoperoxidase methods.ResultsTwenty‐eight children, 7 months to 12.6 years of age, had lower gastrointestinal hemorrhage caused by Meckel's diverticulum and had positive radionuclide scans. All had acid‐secreting mucosa in their diverticula, and ulceration. “Chronic gastritis” and eosinophilia were constant findings; “acute gastritis” was present in four patients. Twenty specimens exhibited lymphoid follicles in the gastric mucosa. Seventeen patients with Meckel's diverticula (age range, 1 month–14.7 years) who presented with acute abdominal pain associated with intussusception were used for comparison. Acid‐secreting gastric mucosa was seen in four patients. H. pylori was identified in only one of the 45 patients; this patient had ulceration and moderate “acute gastritis.”ConclusionsH. pylori does not colonize a substantial number of children who have ulcerated and bleeding Meckel's diverticulum in the presence of acid‐secreting mucosa. Although H. pylori is a notable cause of ulceration, the authors confirm that ulceration is possible in its absence, and alternative mechanisms of ulceration are important. The presence of lymphoid follicles in Meckel's diverticula, unlike gastric biopsies, is not associated with H. pylori.
Survival of Rotavirus Antibody Activity Derived From Bovine Colostrum After Passage Through the Human Gastrointestinal Tract
Authors: Pacyna Janina , Siwek Kasia , Terry Sandra J. , Roberton Elizabeth S. , Johnson Ronald B. , Davidson Geoffrey P. 
Published: January 2024

ABSTRACTBackgroundRotavirus is a major cause of infectious diarrhea in infants and young children. Several studies have shown that hyperimmune bovine colostrum, derived from cows immunized with rotavirus, can prevent rotavirus diarrhea when given passively. The objective of this study was to determine whether colostral antibody activity survived transit through the gut by measuring the level of rotavirus antibody activity in the feces.MethodsHyperimmune colostrum containing different levels of rotavirus antibody was administered to 105 children attending nine Adelaide childcare centres. Subjects were asked to drink 100 ml of whole milk supplemented with colostrum 3 times a day, for a period of 6 days. Stool samples were collected from the subjects before, during , and after consumption of the study product. Rotavirus activity was determined using a novel virus reduction enzyme‐linked immunosorbent assay (ELISA) and a virus neutralization assay.ResultsRotavirus antibody activity was detected in 521 (86%) of 602 fecal specimens obtained during the study using the virus reduction ELISA. The antibody activity was detected as early as 8 hours after ingestion of hyperimmune colostrum and up to 72 hours after consumption had ceased. There was a strong relation (r = 0.81) between the titer of rotavirus antibody administered to subjects and the level of antibody activity detected in the feces.ConclusionsThe results show that antirotavirus activity survived passage through the gut. Therefore, passive immunotherapy may be used to prevent or treat infectious diseases that affect the entire length of the gastrointestinal tract.
Successful Immune Response to a Recombinant Hepatitis B Vaccine in Children After Liver Transplantation
Authors: Duca Pablo , Del Pont Jose Marco , D'Agostino Daniel 
Published: January 2024

BackgroundPatients who undergo liver transplantation require multifaceted general care, and vaccination has a fundamental role before and after transplantation. The purpose of this study was to provide a prospective evaluation of the immunogenicity of the recombinant vaccine (RV) against Hepatitis B in pediatric patients with liver transplants (TxH) who for different reasons did not receive a pretransplantation vaccine.MethodsFrom June 1996 to December 1999, 47 pediatric patients with liver transplants were vaccinated with RV. Patients older than 1 year of age, in stable condition, and 6 months post‐transplantation were included. The vaccination scheme was 0–1 and 6 months, intramuscular 10 μg in less than 30 kg body weight, and 20 μg in more than 30 kg body weight. The nonresponder patients were vaccinated with a booster dose and a double dose 1 to 6 months after the last dose. Responders to titer HBs Ab enzyme immunoassay (EIA) <10 UI/ml were defined as nonresponders, to titer between 10–100 UI/ml as responders, and to higher titer of 100 UI/ml as high responders.ResultsThe following data were obtained from the 47 patients: mean ± standard deviation (SD) age at vaccination was 10.76 ± 5.96 years old and the mean ± SD post‐transplant time at the beginning of vaccination was 3.56 ± 2.19 years. Thirty‐three of 47 patients (70%) responded to doses according to body weight, and 14 (30%) did not respond, necessitating a booster dose after which 7 responded (50%). The global seroconversion was 85%. There was not a significant responder cyclosporine concentration dosage (154 vs. 150 ng/ml) difference between responders and nonresponders. Sixty‐six percent (8 of 12) of patients receiving a triple immunosuppressive scheme (cyclosporine, steroids, and Azathioprine) had a positive response, while 84% (16 of 19) receiving a double scheme (cyclosporine and steroids) and 100% (16 of 16) receiving monotherapy with cyclosporine had a positive response. By comparing a triple scheme with monotherapy, the Fisher exact test found a P < 0.01. Incidence of adverse effects (local pain) was 2.63%.ConclusionImmunization with RV was well‐tolerated with acceptable seroconversion and safety in recipients after liver transplantation, particularly in those undergoing cyclosporine monotherapy. The population studied showed better results than did other populations of immunosuppressed patients.
Fecal Calprotectin as a Measure of Disease Activity in Childhood Inflammatory Bowel Disease
Authors: Bunn Susan K. , Bisset W. Michael , Main Margaret J.C. , Golden Barbara E. 
Published: January 2024

ABSTRACTBackgroundCalprotectin is an abundant neutrophil protein that is extremely stable in feces. The aim of this study was to assess the effectiveness of fecal calprotectin as a noninvasive measure of disease activity in childhood inflammatory bowel disease (IBD) by comparison to a modified Lloyd‐Still and Green score and laboratory inflammatory indices.MethodsSpot fecal samples from 37 children with IBD and 31 control children were sent by ordinary mail to the laboratory. Fecal calprotectin concentration was measured by an in‐house enzyme linked immunosorbent assay (ELISA). A modified Lloyd‐Still & Green score (mLSS) was calculated for each child with IBD within 10 days of obtaining the fecal sample.ResultsCompared with control values (median, range) (2.1, 0.5–6.3 mg/L), fecal calprotectin was increased in 16 children with ulcerative colitis, (11.5, 0.6–272.5 mg/L, P < 0.001) and in 21 children with Crohn disease, (14.0, 0.7–59.7 mg/L, P < 0.001). Twelve “moderately affected” children (mLSS of 35–65) had higher fecal calprotectin concentrations (22.2, 2.7–141.7 mg/L) than 25 “mildly affected” children (mLSS > 65), (10.3, 0.6–272.5 mg/L, P = 0.002). For the total IBD group, fecal calprotectin concentration correlated negatively with the mLSS (r = ‐ 0.61, P < 0.001). It also correlated negatively with serum albumin concentration (r = ‐ 0.49, P = 0.002) and positively with erythrocyte sedimentation rate (r = 0.40, P = 0.01).ConclusionsFecal calprotectin seems to reflect bowel inflammation in children with IBD. As a simple, safe, noninvasive test, it has the potential to reduce the number of invasive investigations performed in these children.
Gastroesophageal Endoscopic Findings and Gastrointestinal Symptoms in Preterm Neonates With and Without Perinatal Indomethacin Exposure
Authors: Ojala Riitta , Ruuska Tarja , Karikoski Riitta , Ikonen R. Sami , Tammela Outi 
Published: January 2024

ABSTRACTBackgroundThe aim of this study was to investigate whether perinatal indomethacin treatment has effects on the development of esophageal and gastric lesions in preterm infants and to evaluate other potential etiologic factors behind these lesions.MethodsSixty‐nine infants were born at less than 33 weeks' gestation. Forty‐five of these infants underwent treatment with perinatal indomethacin (study group) and 24 did not (control group). All underwent upper gastrointestinal tract endoscopy and biopsy during the neonatal period. The correlation between gastrointestinal symptoms, abnormal endoscopic findings, and the factors correlating with the development of esophageal and gastric mucosal lesions was evaluated.ResultsAbnormal endoscopic findings were equally common in the study group (77.8%) and in controls (83.3%). There was no dependence between gastrointestinal symptoms and endoscopic findings because only 15 infants (21.7%) were symptomatic before endoscopy. The interval between endoscopy and the last perinatal indomethacin dose correlated significantly with abnormal esophageal findings and gastric mucosal lesions. Shorter duration of enteral feeding before endoscopy correlated with greater risk of abnormal esophageal findings. Older gestational age and need of ventilator treatment at the time of endoscopy remained the risk factors associated with abnormal gastric findings.ConclusionsEsophageal and gastric lesions diagnosed by endoscopy correlate poorly with the gastrointestinal symptoms of patients. Short duration of enteral feeding seems to be correlated with an increased risk of esophageal mucosal lesions, increasing gestational age and ventilator treatment with gastric mucosal lesions, and perinatal indomethacin with esophageal and gastric mucosal lesions in preterm infants. Ventilator‐treated preterm infants not receiving enteral nutrition and patients with indomethacin exposure might benefit from ulcer prophylaxis.
Assessment of Hepatic Function in Cystic Fibrosis by Lidocaine Metabolism
Authors: Gremse David A. , Sindel Lawrence J. , Hoff Charles J. , Wells David J. , Boerth Robert C. 
Published: January 2024

ABSTRACTBackgroundTo determine hepatic drug metabolism in patients with cystic fibrosis, as measured by monoethylglycinexylidide formation after lidocaine injection and indocyanine green (ICG) clearance.MethodsThe following study is a case–control study, which included 19 patients with cystic fibrosis and 13 control subjects. Serum monoethylglycinexylidide concentration was measured after intravenous injection of 1 mg/kg (maximum, 50 mg) lidocaine. Indocyanine green (0.5 mg/kg) was injected concomitantly, and absorbance (805 nm) of serum was measured over time to determine its volume of distribution, serum half‐life, and hepatic blood flow.ResultsMonoethylglycinexylidide formation was decreased in patients with cystic fibrosis compared with controls (39.4 ± 16.9 μg/L versus 70.3 ± 45.7 μg/L, mean ± SD , respectively, P < 0.02). Indocyanine green half‐life (4.6 ± 2.7 min versus 3.0 ± 1.0 min), volume of distribution (8.6 ± 5.5 L versus 8.3 ± 3.4 L), and hepatic blood flow (10.9 ± 5.9 ml · kg−1 · min−1 versus 7.4 ± 2.0 ml · kg−1 · min−1) were similar in both groups.ConclusionMonoethylglycinexylidide formation after lidocaine injection is impaired in patients with cystic fibrosis. This impairment may have clinical implications when using hepatically metabolized medications in patients with cystic fibrosis.
Saccharomyces boulardii Does Not Stimulate Mucosal Hyperplasia after Intestinal Resection in the Rat
Authors: Kollman Kathryn A. , Goulet Olivier , Vanderhoof Jon A. 
Published: January 2024

ABSTRACTBackgroundPolyamines have been shown to be important regulators of the intestinal adaptation process after massive bowel resection. Saccharomyces boulardii is yeast that has the ability to synthesize polyamines. Therefore, S. boulardii may be useful in the treatment of short bowel syndrome.MethodsTwenty 150‐g male Sprague‐Dawley rats were subjected to 80% jejunoileal resection. Another 20 animals received transection and closure and served as pair fed controls. One half of the resected rats and one half of the controls were given S. boulardii 25 mg/day.ResultsAfter 2 weeks, mucosal mass (mg/cm bowel) did not differ between treated and non‐treated animals despite the presence of a marked resection effect. Mucosal DNA, protein, and sucrase activity likewise did not differ. Subsequently, the experiment was repeated four times the original dose (100 mg/day) and found comparable results. In the proximal bowel, mucosal mass was 92 ± 6 mg/cm in treated animals versus 107 ± 8 mg/cm in untreated rats. In the distal small bowel, comparable values were 85 ± 5 mg/cm and 88 ± 4 mg/cm. Again, mucosal DNA, protein, and sucrase activity levels paralleled these results.ConclusionsAlthough S. boulardii may stimulate polyamine synthesis, it does not seem to be helpful in augmenting gut adaptation in this animal model of short bowel syndrome.
Delayed Gastric Emptying and Gastroesophageal Reflux: A Pathophysiologic Relationship
Authors: Estevão‐Costa José , Campos Miguel , Dias Jorge Amil , Trindade Eunice , Medina Ana Maria , Carvalho José Luis 
Published: January 2024

ABSTRACTBackgroundDelayed gastric emptying (DGE) is frequent in patients with gastroesophageal reflux (GER), but its pathophysiologic role has not yet been established. To identify a relationship between DGE and GER, we assessed whether DGE increases esophageal acid exposure and the related importance of possible mechanisms.MethodsThirty pediatric patients with pathological GER were divided according to gastric emptying scintigraphy into a DGE group (n = 14) and normal‐emptying group (n = 16). The esophageal pH‐monitoring parameters of the two groups were compared with respect to the individual variation between postprandial and fasting periods.ResultsPatients with DGE had less total acid exposure than did those with normal emptying, but patients in both groups had a pathological fraction of time when pH was below 4 in both the postprandial (median: 18 vs. 27.6;P = 0.49) and fasting (8.5 vs. 23.9;P = 0.01) periods. Patients in the normal‐emptying group had similar fraction of time when pH was below 4 in the postprandial and fasting periods. However, patients in the group with DGE had a fraction of time when pH was below 4 in the postprandial period that was almost double that presented in fasting period (postprandial to fasting ratio: 2.11:0.90;P = 0.002). The postprandial to fasting ratio for episodes per hour was similar in the two groups (1.81 vs. 1.79;P = 0.62). Patients with DGE had a significantly higher frequency of long episodes in the postprandial period than did those with normal emptying (62.5% vs. 38.2%;P = 0.04). The occurrence of the longest episode in the postprandial period was also significantly higher for patients with DGE (57.1% vs. 6.2%;P = 0.003).ConclusionsDGE seems to accentuate postprandial reflux by increasing the volume of refluxate per episode of reflux through an underlying incompetent lower esophageal sphincter.