bluuebunny/crossref_metadata_2025_split huggingface dataset:
Assessment of the DQ Heterodimer Test in the Diagnosis of Celiac Disease in the Canary Islands (Spain)
Authors: Peña‐Quintana L. , Torres‐Galván M. J. , Déniz‐Naranjo M. C. , Ortigosa‐Castillo L. , Ramos‐Varela J. C. , Calvo‐Hernández F. , Fiuza‐Pérez M. D. , Rodríguez‐Gallego J. C. , Sánchez‐García F. 
Published: January 2024

ABSTRACTBackgroundCeliac disease is a multifactorial disorder of the proximal small intestine associated with a permanent intolerance to gluten. The HLA‐DQ(α1*0501, β1*02) heterodimer is strongly associated with this disease.Materials and MethodsThe authors studied a sample of 354 unrelated Caucasoid individuals: 118 patients with celiac disease and 236 control subjects. All patients and controls subjects were born in Gran Canaria (Canary Islands) at least two generations ago.The authors typed the HLA‐DQA1 and DQB1 genes by DNA methods. The positive and negative predictive values of the test were studied.ResultsThe mean age at diagnosis was 25.4 months, with a statistically significant proportion of females (64.4%, P < 0.002). For DQB1 gene, the susceptibility allele found was DQB1*02 (relative risk [RR] = 7.60, confidence interval [CI]: 5.35–10.78), whereas for the DQA1 gene, the susceptibility alleles found were DQA1*0501 (RR = 2.99, CI: 2.16–4.14) and DQA1*0201 (RR = 1.88, CI: 1.25–2.82). The presence of the DQ(α1*0501, β1*02) heterodimer was strongly associated with the disease (92.4% in the patients group vs. 21.6% in control subjects). HLA‐DQ8 heterodimer was absent in the authors' patients. DQB1*02 homozygous subjects presented a higher relative risk for celiac disease. There was no correlation of DQB1*02 dosage with age at onset below 12 years of age or with gender distribution. Sensitivity, specificity, and the positive and negative predictive values of the test were 92.4%, 78.4%, 68.1%, and 95.4%, respectively.ConclusionsThe presence of the DQ2 (DQA1*0501/DQB1*02) heterodimer is strongly associated with celiac disease in the population studied by the authors. The value of this test derives from its ability to exclude disease when a negative result occurs.
Tests to Assess Symptoms of Gastroesophageal Reflux in Infants and Children
Authors: Orenstein Susan R. 
Published: January 2024

ABSTRACTIn studying antireflux medications, we may use tests for assessing symptoms of gastroesophageal reflux to establish or confirm a diagnosis of reflux disease and to evaluate whether study subjects are improving with therapy. Symptom assessment questionnaires need to be validated, results need to be reproducible, and the outcome variables measured need to be clinically relevant. Other outcomes for clinical trials include measures of esophagitis: histologic (morphometric) measures in infants and endoscopic and histologic visualization in older children. Intraesophageal pH monitoring studies may be useful for obtaining pharmacodynamic data.
Current Regulatory Policies Regarding Pediatric Indications and Exclusivity
Authors: Rodriguez William J. , Roberts Rosemary , Murphy Dianne 
Published: January 2024

ABSTRACTPediatric studies have resulted in changes in the dose of many medications given to children and an increased awareness of safety issues. An additional 6 months of pediatric exclusivity legislated under the Food and Drug Administration (FDA) Modernization Act of 1997 for pediatric studies undertaken in response to written requests from the FDA have stimulated the conduct of a large number of studies.
Water and Electrolyte Salvage in an Animal Model of Dehydration and Malnutrition
Authors: Islam Sufia , Abély Michel , Alam N. Hoque , Dossou Flore , Chowdhury A. K. Azad , Desjeux Jehan‐François 
Published: January 2024

ABSTRACTBackground and aimRecently, a new oral rehydration solution (ORS) called Resomal has been designed specifically for children with severe malnutrition. The aim of this study was to assess the effect of malnutrition on renal and intestinal responses to dehydration, and to compare intestinal water and electrolyte absorption from Resomal and from the standard World Health Organization‐Oral Rehydration Solution (WHO‐ORS).MethodsMalnutrition was achieved in a rabbit model by feeding the animals daily for 30 days with half the amount of food that a well‐nourished group of control animals had consumed on the previous day. Dehydration was achieved by water deprivation for 46 hours in both control and malnourished rabbits. At 46 hours, dehydration was assessed by changes in body weight, urinary volume and osmolarity, and blood urea nitrogen concentration. At that time active colonic and jejunal mucosal electrolyte transport in Ussing chambers was also measured. Small intestinal absorption of water, sodium, and potassium was measured in vivo during intestinal perfusion of the two ORSs and in vitro by measurement of mucosal electrogenic glucose‐stimulated sodium absorption across intestinal patches.ResultsCompared to controls (C), well‐nourished but dehydrated (C+D) animals lost 12% of their body weight, with an 87% reduction in urine volume, a 110% increase in urine osmolality, and a 94% increase in blood urea nitrogen. In the colon of C+D animals, short‐circuit current (Isc) and net sodium transepithelial flux (JNa+ net) were increased. Almost identical results were obtained in malnourished and dehydrated (M+D) animals. In the jejunum, net in vivo absorption of water (JWater), sodium (JNa+), and potassium (JK+) were increased during standard ORS infusion in both dehydrated groups. During Resomal infusion, water absorption was the same as seen with WHO‐ORS, but sodium absorption was reduced, and potassium absorption was increased in both well‐nourished and malnourished dehydrated animals. In vitro, compared to controls, the glucose‐stimulated short‐circuit current (ΔIsc), JNa+ net and G were increased in both dehydrated groups.ConclusionDuring experimental dehydration, the kidney and large intestine salvage water and electrolytes, thus reducing the consequences of dehydration. These findings indicate that jejunal water absorption from Resomal and WHO‐ORS is increased during dehydration, but Resomal allows for less sodium and more potassium to be absorbed, both in well‐nourished and malnourished dehydrated rabbits.
Vitamin E Treatment in Pediatric Obesity‐Related Liver Disease: A Randomized Study
Authors: Vajro Pietro , Mandato Claudia , Franzese Adriana , Ciccimarra Edmondo , Lucariello Stefania , Savoia Marcella , Capuano Grazia , Migliaro Fiorella 
Published: January 2024

ABSTRACTObjectiveA beneficial role of antioxidants in hepatopathic obese individuals has hitherto been inferred only from uncontrolled pilot studies. The authors compared the effect of vitamin E and weight loss on transaminase values and on ultrasonographic bright liver in a controlled group of children with obesity‐related liver dysfunction.MethodsTwenty‐eight children with obesity‐related hypertransaminasemia and bright liver were randomly allocated to two single‐blind groups: group 1 (n = 14) treated with a low‐calorie diet associated with oral placebo for 5 months, and group 2 (n = 14) treated with a low‐calorie diet associated with oral vitamin E (400 mg/d × 2 months, 100 mg/d × 3 months). Transaminase values and ultrasonographic liver brightness along with weight loss and vitamin E levels were monitored.ResultsVariations in transaminase levels and percentage of patients with normalized transaminase values were comparable in the two groups. The disappearance of bright liver was observed only in patients who lost weight and was twice as common in patients from group 1. Two subgroups of patients with complete normalization of transaminase values emerged as a consequence of controlled adherence to diet alone (n = 6; significant decrease of percent overweight:P = 0.0019 ) and to vitamin E alone (n = 7; unmodified percent overweight and significant increase of vitamin E/cholesterol ratio:P < 0.0001). Changes in treatment‐induced alanine aminotransferase levels in these two subgroups were comparable at month 2, whereas values at month 5 were significantly lower in the subgroup adherent to diet alone (P = 0.04). In the subgroup adherent to vitamin E alone, after 2 months washout, transaminase remained stable in 5 patients and increased in 2; bright liver persisted in all.ConclusionsOral vitamin E warrants consideration in obesity‐related liver dysfunction for children unable to adhere to low‐calorie diets.
Functional Fecal Retention With Encopresis in Childhood
Authors: Loening‐Baucke Vera 
Published: January 2024

ABSTRACTObjectivesThe most common cause of encopresis in children is functional fecal retention (FFR). An international working team suggested that FFR be defined by the following criteria: a history of >12 weeks of passage of <2 large‐diameter bowel movements (BMs) per week, retentive posturing, and accompanying symptoms, such as fecal soiling. These criteria are usually referred to as the ROME II criteria. The aims of this study were to evaluate how well the ROME II criteria identify children with encopresis; to compare these patients to those identified as having FFR by historical symptoms or physical examination; to determine whether 1‐year treatment outcome varied depending on which definition for FFR was used; and to suggest improvements to the ROME II criteria, if necessary.MethodsData were reviewed from the history and physical examination of 213 children with encopresis. One‐year outcomes identified were failure, successful treatment, or full recovery.ResultsOnly 88 (41%) of the patients with encopresis fit the ROME II criteria for FFR, whereas 181 (85%) had symptoms of FFR by history or physical examination. Thirty‐two (15%) patients did not fit criteria for FFR, but only 6 (3%) appeared to have nonretentive fecal soiling. Rates of successful treatment (50%) and recovery (39%) were not significantly different in the two groups.ConclusionsThe ROME II criteria for FFR are too restrictive and do not identify many children with encopresis who have symptoms of FFR. The author suggests that the ROME II criteria for FFR could be improved by including the following additional items: a history of BMs that obstruct the toilet, a history of chronic abdominal pain relieved by enemas or laxatives, and the presence of an abdominal fecal mass or rectal fecal mass.
Lewis Antigen Expression in Gastric Mucosa of Children: Relationship With Helicobacter pylori Infection
Authors: Nogueira Ana Margarida , Marques Terezinha , Soares Paula Cristina M. , David Leonor , Reis Celso A. , Serpa Jacinta , Queiroz Dulciene M. , Rocha Gifone A. , Rocha Andréia C. 
Published: January 2024

ABSTRACTObjectiveLewis epithelial antigen expression has a role in Helicobacter pylori adherence, presumably mainly in cag A‐positive strains. The authors investigated whether Lewis antigen expression in children's gastric mucosa was associated with H. pylori infection, cag A status, patient age, or presence of duodenal ulcer (DU).MethodsThe expression of Lewis A (Lea), B (Leb), X (Lex), and Y (Ley) was detected by immunohistochemistry in the antral and oxyntic mucosae of 70 children. Children were divided in four age groups (<4 years; 4–8 years; 9–12 years; and 13–18 years).ResultsForty‐seven of the 70 children had H. pylori and 17 had DU. The cag A status was determined by polymerase chain reaction in 34 patients. Lea and Leb were expressed in 64% and 44% of the patients, respectively; Lex and Ley were expressed in the glands in all of the patients and in the superficial epithelium. Leb expression was more common among patients without H. pylori (15/23, 65%) than in those with H. pylori (16/47, 34%) (P = 0.03). In noninfected patients, Leb and superficial Ley expression were associated with increased age. Leb expression was more common in patients with chronic gastritis than in those with DU. Lex superficial expression was significantly associated with DU in patients with H. pylori.ConclusionIn children, the expression of Leb and Ley in the superficial gastric epithelium depends on age. Other receptors, such as Lex, may have a role in H. pylori colonization, especially in patients with DU. Studies assessing the expression of Lewis antigens in children may contribute to an understanding of the mechanisms of acquisition of H. pylori infection.
Gastric Histopathology, Iron Status and Iron Deficiency Anemia in Children with Helicobacter pylori Infection
Authors: Baysoy Gökhan , Ertem Deniz , Ademoğlu Evin , Kotiloğlu Esin , Keskin Sabiha , Pehlivanoğlu Ender 
Published: January 2024

ABSTRACTObjectivesHelicobacter pylori has been established as a major cause of gastritis and peptic ulcer disease in adults and children. H. pylori infection may also have a role in the development of some extragastrointestinal diseases, including iron deficiency anemia. The aim of this study is to investigate H. pylori‐related changes in gastric physiology and histology and the relationship of these changes to iron deficiency anemia in children.MethodsFifty‐two patients with gastrointestinal complaints were studied. Hematologic parameters, 3‐day vitamin C and iron consumption, serum gastrin levels, and gastric juice ascorbic acid levels were compared in patients with and without H. pylori infection. Dietary intake of vitamin C and iron, serum gastrin, gastric juice ascorbic acid content, and gastric histology were compared in patients with H. pylori infection and anemia and in patients with H pylori infection and no anemia. The CagA status of the H. pylori organisms was evaluated.ResultsTwenty‐eight of 52 patients had H. pylori. Thirty‐one patients had iron deficiency anemia. H. pylori infection was associated with low serum iron levels. H. pylori gastritis was associated with a decrease in the gastric juice ascorbic acid level. Infection with CagA‐positive strains was associated with a greater decrease in gastric juice ascorbic acid than infection with CagA‐negative strains. However, the gastric juice ascorbic acid levels of patients with H. pylori and anemia were not different from those of non‐anemic patients with H. pylori. Among patients with H. pylori infection, pangastritis was twice as common in those with anemia than in those without anemia.ConclusionsH. pylori infection was associated with a decrease in gastric juice ascorbic acid concentration, and this effect was more pronounced in patients with the CagA‐positive strain. Pangastritis was more common in patients whose H. pylori. infection was accompanied by anemia.
Low‐Dose Immunosuppression Reduces the Incidence of Post‐transplant Lymphoproliferative Disease in Pediatric Liver Graft Recipients
Authors: Ganschow Rainer , Schulz Tania , Meyer Thomas , Broering Dieter C. , Burdelski Martin 
Published: January 2024

ABSTRACTObjectivesIn pediatric solid organ transplantation, the Epstein‐Barr virus (EBV)‐related lymphoproliferative disorders (PTLD) still play a major role in post‐transplant morbidity and mortality. The aim of the study was to determine the incidence of PTLD in pediatric patients with liver transplant who receive low‐dose immunosuppression protocols.MethodsAll pediatric patients (n = 269) received a dual immunosuppression therapy consisting of cyclosporine A (initial trough levels, 170–200 μg/L; trough levels for maintenance immunosuppression after 1 year, 80–100 μg/L) and prednisolone (starting dose, 60 mg/m2). Steroids were reduced to 30 mg/m2 after 1 week, followed by a weekly tapering to 5 mg/m2. Seventy‐seven of 269 patients were switched to tacrolimus therapy. The authors evaluated the significance of EBV‐DNA monitoring by quantitative polymerase chain reaction in identifying patients at risk for PTLD.ResultsPatient survival was 90.3%; graft survival was 85.9%. Eight patients lost their grafts because of chronic rejection. The incidence of PTLD was low (0.7%), although a significant EBV viral load was found in 42.4% of the patients. One third of the patients with a viral load of 3,000 genomes/105 cells or greater had clinical signs of EBV infection.ConclusionsThe authors conclude that low‐dose immunosuppressive protocols significantly reduce the incidence of PTLD. In patients treated with that regimen, the monitoring of EBV viral load seems not to be helpful. It can be assumed that low‐dose immunosuppression does not suppress EBV‐specific cytotoxic CD8+ T cells, thus allowing the host to control EBV infection without the risk of PTLD. Our low‐dose immunosuppression protocol did not increase the risk of chronic rejection.
Irreversible Intestinal Failure
Authors: Goulet Olivier , Ruemmele Frank , Lacaille Florence , Colomb Virginie 
Published: January 2024

ABSTRACTIntestinal failure (IF) can be defined as the reduction of functional gut mass below the minimal amount necessary for digestion and absorption adequate to satisfy the nutrient and fluid requirements for maintenance in adults or growth in children. In developed countries, IF mainly includes individuals with the congenital or early onset of conditions requiring protracted or indefinite parenteral nutrition (PN). Short bowel syndrome was the first commonly recognized cause of protracted IF. The normal physiologic process of intestinal adaptation after extensive resection usually allows for recovery of sufficient intestinal function within weeks to months. During this time, patients can be sustained on parenteral nutrition. Only a few children have permanent intestinal insufficiency and life‐long dependency on PN. Non‐transplant surgery including small bowel tapering and lengthening may allow weaning from PN in some cases. Hormonal therapy with recombinant human growth hormone has produced poor results while therapy with glucagon‐like peptide‐2 holds promise. Congenital diseases of enterocyte development such as microvillus inclusion disease or intestinal epithelial dysplasia cause permanent IF for which no curative medical treatment is currently available. Severe and extensive motility disorders such as total or subtotal intestinal aganglionosis (long segment Hirschsprung disease) or chronic intestinal pseudo‐obstruction syndrome may also cause permanent IF. PN and home‐PN remain are the mainstays of therapy regardless of the cause of IF. Some patients develop complications while receiving long‐term PN for IF especially catheter related complications (thrombosis, sepsis) and liver disease. These patients may be candidates for intestinal transplantation. This review discusses the causes of irreversible IF and emphasizes the specific medico‐surgical strategies for prevention and treatment of these conditions at several stages of IF.
Administration of Oral Probiotic Bacteria to Pregnant Women Causes Temporary Infantile Colonization
Authors: Schultz Michael , Göttl Claudia , Young Rose J. , Iwen Peter , Vanderhoof Jon A. 
Published: January 2024

ABSTRACTBackgroundIt is difficult to permanently change the composition of the complex intestinal microflora of the adult. Orally administered probiotic bacteria produce only temporary colonization of the intestine in patients with a fully developed gut microflora. The gastrointestinal tract of a healthy fetus is sterile. During the birth process and rapidly thereafter, microbes from the mother and the surrounding environment colonize the gastrointestinal tract until a dense, complex microflora develops. Probiotic bacteria have been shown to beneficially influence the intestinal and systemic immune system and mediate protection against nosocomial infections affecting the neonate.ObjectivesThe purpose of this study was to determine whether oral administration of the probiotic micro‐organism Lactobacillus rhamnosus strain GG (L. GG) to the pregnant woman leads to colonization of the newborn infant.MethodsThe authors identified six women who were taking L. GG during late pregnancy. None of the children received L. GG after birth, and their mothers discontinued its consumption at the time of delivery. L. GG concentration in fecal samples was determined by colony morphology and molecular analysis.ResultsIn all four children delivered vaginally and in one of two children delivered by cesarean section, L. GG was present in fecal samples at 1 and 6 months of age. Three children remained colonized for at least 12 months, and in two children L. GG was detected in fecal samples at 24 months of age. Three mothers were tested 1 month post partum and no L. GG was present in fecal samples. No L. GG was found in one of these women 24 months post partum. There was no L. GG detectable in stools of the siblings of two children at the 2‐year and 3‐years after birth of the index child. L. GG was not isolated from the stools of children whose mothers were not taking L. GG.ConclusionsTemporary colonization of an infant with L. GG may be possible by colonizing the pregnant mother before delivery. Colonization is stable for as long as 6 months, and in unexplained circumstances may persist for as long as 24 months.
Impact of Sedation and Anesthesia on the Rectoanal Inhibitory Reflex in Children
Authors: Pfefferkorn Marian D. , Croffie Joseph M. , Corkins Mark R. , Gupta Sandeep K. , Fitzgerald Joseph F. 
Published: January 2024

ABSTRACTObjectiveThe study objective was to determine whether the rectoanal inhibitory reflex (RAIR) can be elicited during limited anorectal manometry (ARM) performed under general anesthesia (GA).MethodsIn this retrospective study, patients with intractable constipation who underwent ARM under GA from November 1999 to March 2002 were evaluated. Rectal biopsy specimens were examined for the presence of ganglion cells.ResultsEighty consecutive patients aged 5 months to 16 years were evaluated. Three patients with Hirschsprung disease were excluded from analysis. Ganglion cells were found in rectal biopsy specimens from 76 patients. RAIR was positive in 69 (90%) and negative in 8 (10%). Forty‐five patients received preoperative medications: midazolam (n = 36), midazolam + atropine (n = 3), atropine (n = 1), midazolam + glycopyrrolate (n = 3), and glycopyrrolate (n = 2). Different combinations of general anesthetic medications were used. Ten patients received neuromuscular blockers. The use of preoperative midazolam or atropine did not affect the presence or absence of RAIR; however, the proportion of patients with negative RAIR was higher in those receiving glycopyrrolate (P = 0.007) than in those receiving other medications. There was no significant difference in the effect of the general anesthetic agents or neuromuscular blockers used on the presence or absence of RAIR.ConclusionsThe rectoanal inhibitory reflex in children can be elicited even when anorectal manometry is performed under general anesthesia. Glycopyrrolate, an anticholinergic, appears to inhibit the demonstration of the RAIR.