bluuebunny/crossref_metadata_2025_split huggingface dataset:
Early Prediction of Relapse During Gluten Challenge in Childhood Celiac Disease
Authors: Mayer Marina , Greco Luigi , Troncone Riccardo , Grimaldi Massimo , Pansa Giampiero 
Published: January 2024

SummaryThirty‐seven children, in whom celiac disease had been diagnosed because of flat mucosa on a gluten‐containing diet and recovery on a gluten‐free diet, were challenged with gluten powder, 10 g/day, in addition to an otherwise gluten‐free diet. A small intestinal biopsy was performed before the challenge; clinical symptoms, a 1‐h blood xylose test, and gliadin antibody measurement were used to establish the timing of the confirmatory biopsy. All but one case relapsed within 205 days (mode, 60 days). In no case was the relapse clinically evident. Raised levels of gliadin antibodies, a fall in xylose absorption, or both predicted the relapse in 37%, 7%, and 57% of cases, respectively. Evaluated individually, each test gave a considerable rate of false negative results. Discriminant coefficients produced for each test were used to compute a score that allowed the classification of patients into relapse/no relapse categories with a good degree of accuracy. The discriminant score rose sharply after only 15 days of challenge, indicating that it is possible to predict the relapse long before any clinical symptom appears.
The Effect of Short‐Term Refrigeration of Milk and Addition of Breast Milk Fortifier on the Delivery of Lipids During Tube Feeding
Authors: Lavine Margery , Clark Richard M. 
Published: January 2024

SummaryBoth storage of breast milk for 3 days at 4°C and the addition of breast milk fortifier containing lipid improved the delivery of lipids during continuous pump infusion. The percentage of the original lipid delivered after 8 h of pumping was 9, 25, 58, and 66% from the freshly collected milk, stored milk, freshly collected milk mixed with fortifier, and stored milk mixed with fortifier, respectively. Phospholipid and cholesterol levels also were measured. Their loss during delivery through the tube was similar to the loss of total lipid. While storage improved the delivery of lipids from breast milk, the additional lipid delivered from milk mixed with fortifier was primarily due to lipids from the fortifier.
Low Breast Milk Phosphorus Concentration in Familial Hypophosphatemia
Authors: Jonas Adam J. , Dominguez Barbara 
Published: January 2024

SummaryWe report breast milk mineral concentrations in a mother with familial hypophosphatemia that was untreated due to poor compliance with medical advice. Milk phosphorus content was extremely low despite normal maternal serum phosphorus concentrations. Milk calcium concentrations were only modestly decreased so that the ratio of calcium to phosphorus was greatly elevated. It appears that mothers with this disorder who breast feed should have their milk mineral content carefully monitored during lactation. If milk mineral content is abnormal despite maternal therapy with phosphorus and vitamin D, infants may require supplementation of mineral intake.
Do Esophageal pH Monitoring Data Depend on Recording Equipment and Probes?
Authors: Vandenplas Yvan , Goyvaerts Harry , Helven Rudy 
Published: January 2024

SummaryEsophageal pH monitoring data are influenced by numerous patient‐ and/or technical equipment‐related factors. In this study, data obtained with 10 pH recording devices of two different companies and 10 pH glassmicroelectrodes with an external reference electrode of one company, calibrated by different persons, were compared. Pearson correlation coefficients regarding the reflux index (the percentage of the investigation time with a pH < 4) ranged from 0.93 to 1.00. The lowest correlation coefficient (0.73) was obtained by comparing the number of episodes with a pH < 4 recorded with devices from different companies. These results suggest that differences in pH monitoring data recorded in comparable conditions with one type of pH electrode are irrelevant as far as clinical routine investigations are concerned. Data are not significantly influenced by the recording devices themselves, the age of the electrodes, or the skinpotential differences induced by the person calibrating the probes.
Sonographic Evaluation of Portal Hypertension in Children
Authors: Rabinowitz Simon S. , Norton Karen I. , Benkov Keith J. , Yeh Hsu‐Chong , Leleiko Neal S. 
Published: January 2024

SummaryPortal hypertension, an expected consequence of cirrhosis, often has an insidious course in children. A noninvasive technique using abdominal sonography has been previously employed by several investigators as a means of diagnosing this condition. Their technique involves sonographically measuring the diameter of the lesser omentum, which increases as a result of engorged collaterals. In this communication, the method is successfully employed in two children, an infant in whom cirrhosis developed who eventually died from acquired immunodeficiency syndrome, and one whose portal hypertension was relieved after orthotopic liver transplantation. Although successful in these two instances, the theoretical basis on which this technique is based is critically evaluated. Anatomical relationships are reviewed that would caution sonographers who attempt to duplicate these studies. Modifications of the technique that will minimize potential false positive results are also discussed.
Treatment of Duodenocutaneous Fistula with Somatostatin Analog in a Child with Dermatomyositis
Authors: Inamdar Sarla , Slim Michel S. , Bostwick Howard , Godine Louise 
Published: January 2024

SummaryA 6‐year‐old girl with juvenile dermatomyositis complicated by five duodenal perforations is described. The treatment consisted of suture closure of the perforations, reinforcement of the suture line with an omental graft, and adequate drainage of the closure site. The use of a somatostatin analog was successful in promptly arresting a leak from the duodenal perforations that had persisted for 3 months after the operation despite prolonged bowel rest and total parenteral nutrition. On follow‐up observation 2 months later, the patient had no abdominal complaints and was tolerating oral intake without problems.
Yersinia pseudotuberculosis
Authors: Rich Edward J. , McDonald Ruth A. , Christie Dennis L. 
Published: January 2024

Summary
Yersinia pseudotuberculosis is an uncommon gastrointestinal pathogen, recognized as a causative agent in some cases of mesenteric adenitis. We report the clinical and endoscopic features of a case of a 6‐year‐old girl with abdominal pain and bloody diarrhea. Colonoscopic examination revealed aphthous ulcers in the right colon, and mucosal biopsy culture grew Yersinia pseudotuberculosis. This case documents right colonic ulcers and bloody diarrhea with Yersinia pseudotuberculosis.
Impact on Iron Status of Introducing Cow's Milk in the Second Six Months of Life
Authors: Penrod James C. , Anderson Kathryn , Acosta Phyllis B. 
Published: January 2024

SummaryTo determine the impact on iron status of introducing cow's milk (CM) into the diet during the second 6 months of life, nutrient intake was assessed and iron status measured in 100 infants. Nutrient intake for 40 of the 45 infants, age 8 to 13 months, fed CM as the primary beverage for at least 3 months prior to the study and for 45 of 55 infants the same age fed a milk‐based infant formula (FF) as the primary beverage for at least 3 months were assessed. All infants in the study were healthy, and the majority were taking no medications or supplements other than vitamins or fluoride for 3 weeks prior to the assessment. Blood drawn by peripheral venipuncture was analyzed by Coulter Counter for complete blood count; plasma albumin, iron, ferritin, transferrin saturation, and total iron‐binding capacity were measured in all infants. CM‐fed infants had significantly lower mean iron and vitamin C intakes, plasma albumin, transferrin saturation, and ferritin than did FF infants. The frequency of low plasma iron, low transferrin saturation, and low plasma ferritin was significantly greater in CM‐fed than in FF infants. The percentage of subjects with three or more abnormal iron indices was more than twice as great in CM‐fed infants (58%) as in FF infants (23%). Feeding infants iron‐fortified formula to 12 months of age appears to deter iron deficiency.
Modifications of Intestinal Permeability During Food Provocation Procedures in Pediatric Irritable Bowel Syndrome
Authors: Barau Edith , Dupont Christophe 
Published: January 2024

SummaryAn intestinal permeability test analyzing the differential urinary elimination of lactulose and mannitol orally ingested at the same dosage was carried out first in fasting condition, then combined with specific food ingestion, in 17 children with clinical symptoms of irritable bowel syndrome (IBS). Foods were selected based on a suggestive clinical history or on a positive radioaller‐gosorbent or prick test. Comparison of the results with those of a control population reported in a previous study showed that in nine IBS patients, specific food ingestion was associated with a modification of intestinal permeability. The nine children all had a personal and/or familial history of allergy and/or raised total IgE. The symptoms disappeared in the nine patients after food exclusion either alone (seven patients) or together with further treatment by cromolyn (two patients). We conclude that, at least in some children, symptoms of IBS may be related to food hypersensitivity.
Anti‐Liver‐Kidney Microsome Antibody‐Positive Autoimmune Hepatitis Presenting as Fulminant Liver Failure
Authors: Porta Gilda , da Costa Gayotto Luis Carlos , Alvarez Fernando 
Published: January 2024

SummaryA 17‐month‐old girl presented with acute hepatitis, which took a fulminant course leading to death 2 months after onset. No known cause of fulminant liver failure could be identified. Postmortem examination of the liver showed massive multilobular necrosis and areas of severe piecemeal necrosis. A high level of total serum μ‐globulins raised the possibility of autoimmune hepatitis. Search for anti‐liver‐kidney microsome antibody in the patient's serum was positive by immunofluorescence and enzyme‐linked immunosorbent assay. Western blot analysis showed reactivity of the antibody with a 50‐kDa protein identical to that observed in children with autoimmune hepatitis. This patient's history strongly suggests that autoimmune hepatitis can present as fulminant liver failure in children. Early diagnosis in such a patient could lead to early immunosuppressive therapy.
A Study of the Relationship between Bile Salts, Bile Salt‐Stimulated Lipase, and Free Fatty Acids in Breast Milk
Authors: Forsyth J. S. , Donnet L. , Ross P. E. 
Published: January 2024

Summary:Breast milk jaundice has been reported to be associated with increased lipase activity and elevated free fatty acid (FFA) concentrations within breast milk. We have previously shown that bile salts are present in small concentrations in breast milk and the aim of this study was to examine the relationship of bile salt‐stimulated lipase (BSSL) activity, FFA concentration, and bile salt concentration in milks of normal infants and the milk of infants with breast milk jaundice. Mothers of healthy newborn infants were recruited in the early newborn period and 42 provided breast milk samples at 2 weeks, 30 at 6 weeks, 16 at 10 weeks, and 13 at 14 weeks postnatally. We initially studied the effect of lactation on bile salts and found there was a significant decline in both cholate and chenodeoxycholate levels with duration of lactation (p < 0.05). There was also a significant fall in BSSL activity with duration of lactation (p < 0.05), but no correlation was found between BSSL activity and bile salt concentration. FFA concentrations were similar throughout lactation and were not related to either BSSL activity or bile salt concentration. There was a significant increase in the concentration of cholate and the cholate‐to‐chenodeoxy‐cholate ratio in the milks of 12 infants with breast milk jaundice compared with normal milks, the BSSL activity was similar and contrary to previous reports, the FFA concentration was not increased in the milks of infants with breast milk jaundice.
Variation in Macronutrients in Human Bank Milk
Authors: Michaelsen Kim Fleischer , Skafte Lisbeth , Badsberg Jens Henrik , Jørgensen Merete 
Published: January 2024

Summary:Protein (P), fat (F), and carbohydrate (C) concentration in expressed human bank milk was determined by infrared analysis of 2,554 samples from 224 mothers. The mean contents of P, F, C, and energy (E, calculated from P, F, and C) were 9.0 g/L, 39.0 g/L, 71.9 g/L, and 696 kcal/L, respectively. There was a large variation in the concentration of energy‐yielding macronutrients. The contents of P. F, C, and E in the samples with the highest values (97.5 percentile) were 2.3‐, 4.8‐, 1.2‐, and 2.3‐fold, respectively, above the contents in the samples with the lowest values (2.5 percentile). The P content decreased exponentially during the 1st 8 months, followed by an increase during the following months. The F content decreased during the 1st 4 months, followed by an almost linear increase. The possible influence of different maternal characteristics on the macronutrient content of the milk was examined. The main results were as follows: the P and F contents increased slightly with increasing body mass index of the mother, the P content decreased with increasing amounts of milk delivered to the milk bank, and the F content was higher in mothers delivering large amounts of milk. By selecting incoming milk with a high P content, we have developed a “high‐protein” milk with a P content of about 12 g/L (true protein) and an E content of about 725 kcal/L. Thus, by continuous monitoring of macronutrient content in human bank milk it is possible to develop a “high‐protein” milk with sufficient P and E content to cover the needs of preterm infants with very low birth weights (< 1,500 g).
Prevention of Neonatal Necrotizing Enterocolitis
Authors: Carrion Vivien , Egan Edmund A. 
Published: January 2024

SummarySmall premature infants are often hypochlorhydric, and frequently their stomachs are colonized by enteric, gram‐negative bacteria. We tested a hypothesis that gastric pH affected the colonization of the stomach with enteric bacteria and that this colonization was causally related to the risk or severity of necrotizing enterocolitis. A prospective, double‐blind study was conducted that compared a group of infants supplemented with 0.01–0.02 ml of 1 N HCl/ml of milk to a group with a similar supplement of water. Gastric pH, gastric enteric bacteria counts, and the incidence and severity of necrotizing enterocolitis were monitored. The median gastric pH of the HCl‐supplemented group was lower (3.0) than controls (4.0) throughout the study (p < 0.001). The gastric enteric bacterial colonization rate and the quantitative bacterial counts were strongly correlated with gastric pH over 4 (p < 0.001). Somatic growth rates in infants in the HCl‐supplemented group were equal to, or exceeded, those in the control group. There was 1 case of necrotizing entero‐colitis among the 34 infants in the HCl‐supplemented group and 8 cases among the 34 in the control group (p = 0.02). It appears that acidifying the feedings of small premature infants to a pH low enough to inhibit bacterial proliferation in the stomach significantly lowers the risk of necrotizing enterocolitis.
Growth Acceleration and Final Height After Treatment for Delayed Diagnosis of Celiac Disease
Authors: Bosio L. , Barera G. , Mistura L. , Sassi G. , Bianchi C. 
Published: January 2024

SummaryThe only presenting clinical feature of diagnosing celiac disease (CD) late may be short stature. At the start of treatment with a gluten‐free diet (GFD), celiac children show an accelerated growth rate. The real duration of catch‐up growth and influence of diet on the final stature has not yet been defined. In order to evaluate the effect of a GFD on growth parameters, 24 children diagnosed late with CD were studied at our center. During the period of diagnosis, weight, height standard deviation score (HSDS), weight and height velocities (WV and HV), bone age (BA), and pubertal stage were recorded. Predicted height (PH) according to the Tanner method, parental height, and target height (TH) were also evaluated at diagnosis. All patients initially presented because of short stature or retarded growth (100% of patients with height <5th percentile). Patients showed an increased HV and WV during the first 3 years on a GFD, with maximum growth velocity occurring during the first year, but the catch‐up growth was incomplete over 3 years (mean HSDS ± SD, −1.77 ± 0.6). Puberty began in all patients at a normal age. The 12 patients who completed pubertal development reached their target height, whatever the duration of the GFD. The final height (between the 1st and 25th percentile) seemed influenced mainly by familial characteristics; height was below the 3rd percentile in 31% of parents examined.
Serum Carnitine and Nutritional Status in Children Treated with Continuous Ambulatory Peritoneal Dialysis
Authors: Murakami Ryusuke , Momota Tetsuya , Yoshiya Kunihiko , Yoshikawa Norishige , Nakamura Hajime , Honda Masataka , Ito Hiroshi 
Published: January 2024

SummaryThe serum carnitine (total carnitine), total protein, amino acid, and triglyceride levels were determined in children on continuous ambulatory peritoneal dialysis (CAPD). Compared with levels in controls, serum carnitine levels were significantly decreased in patients on CAPD for more than 4 months, while those of patients on CAPD for 1–3 months were not decreased. Patients on CAPD for more than 4 months also showed lower serum total protein levels than in normal controls. The mean triglyceride levels in patients on CAPD for both 1–3 months and more than 4 months were higher than those in normal controls. Among the amino acids, the serum levels of tryptophan, isoleucine, leucine, tyrosine, valine, serine, and aspargine were significantly lower in patients treated with CAPD than in normal controls, whereas the levels of other amino acids were either increased or not changed. Isoleucine and leucine levels showed a strong correlation with serum carnitine. Our data suggest that malnutrition plays a role in the decrease of serum carnitine levels in patients receiving CAPD.
Congenital Short Gut, Malrotation, and Dysmotility of the Small Bowel
Authors: Kern I. B. , Leece A. , Bohane T. 
Published: January 2024

SummaryA familial syndrome of congenital short small bowel, malrotation, and functional bowel obstruction is presented and illustrated in three male siblings with one long‐term survivor. Including the present three cases there have been 18 similar cases found in the literature. This disorder appears to be a sex‐linked recessive inheritance. Early barium meal and follow through in siblings of patients with this condition will detect malrotation that should be surgically corrected in order to prevent mid‐gut volvulus. Intravenous alimentation and early introduction of enteral feeding is important, not only to maintain nutrition but to encourage adaptation of the associated short and dysmotile bowel. These measures aim to improve the outcome of this frequently, but not invariably, fatal disorder.
Enterocolonic Fistula Due to Incarcerated Inguinal Hernia
Authors: Kneepkens C. M. F. , Bijleveld C. M. A. , Zwierstra R. P. 
Published: January 2024

SummaryA 2 1/2‐year‐old boy presented severely underweight and with an extremely distended abdomen. He was born prematurely and at age 6 months had an incarcerated inguinal hernia, which had been treated conservatively. Laboratory investigations at the time of presentation showed evidence of bacterial overgrowth. Barium studies revealed a fistula between the jejunum and sigmoid colon. Resection of the fistula was followed by complete recovery. We suggest that the incarcerated hernia underlies this fistula. Until now, this series of events has not been published.
Neonatal Hepatitis Syndrome with Paucity of Interlobular Bile Ducts in Cystic Fibrosis
Authors: Furuya Katryn N. , Roberts Eve A. , Canny Gerard J. , Phillips M. James 
Published: January 2024

SummaryA male infant presenting with neonatal hepatitis syndrome, characterized by conjugated hyperbilirubinemia and very mild liver function test abnormalities, at 2 weeks of age was found to have no excretion of radioisotope into the intestinal tract on hepatobiliary scan. Liver biopsy revealed severe interlobular bile duct paucity. Other features of Alagille's syndrome were not present; other conditions frequently associated with interlobular bile duct paucity were also excluded. Subsequently, the infant was found to have cystic fibrosis. Cystic fibrosis is thus another disease that may be associated with paucity of interlobular bile ducts presenting as neonatal hepatitis syndrome, and this represents a different pathogenesis of cholestatic jaundice in neonates with cystic fibrosis besides those previously recognized.
Improvement of Nutritional Status in Cholestatic Children with Supplemental Nocturnal Enteral Nutrition
Authors: Moreno Luis A. , Gottrand Frédéric , Hoden Stéphane , Turck Dominique , Loeuille Guy A. , Farriaux Jean‐Pierre 
Published: January 2024

SummaryProtein energy malnutrition is a common complication in cholestatic children in a hepatic transplant program, and may be detrimental to the postoperative outcome. Improvement of the nutritional status may be of obvious importance to improve the prognosis. This study compared oral nutrition with oral nutrition supplemented with nocturnal enteral feeding in children with prolonged cholestasis. In six children with prolonged cholestasis (conjugated bilirubin over 25 mg/L and/or GGT over 110 IU/L in infants aged less than 3 months or over 50 IU/L in older infants and/or alkaline phosphatase over 500 IU/L, for more than 3 months), we compared a 4 to 6 month period with oral nutrition and similar periods with 10 to 12 h nocturnal enteral feeding given at home as an energetic supplement. Energy intake during the second period was 180–200% of recommended dietary allowances. No ascites was found in the six patients during the study period. The Z scores of body weight, weight expressed as percent of ideal body weight (IBW), weight/height2, and arm circumference/head circumference were calculated at the beginning and at the end of each period. With only oral nutrition, a diminution in percentage of ideal W/H and a diminution in Z score for the body weight were observed in five of six patients. At the end of the second period, the average of all of the nutritional indexes was increased and the Z score for the body weight was also increased in four of six patients. Significant statistical differences (p < 0.05) were found in W as percentage of IBW and the Z score for log W/H2. Oral nutrition was insufficient to improve nutritional status in these children with prolonged cholestasis, whereas, despite complications of the underlying condition in two patients, nocturnal enteral feeding given as a complement to oral nutrition improved all of the nutritional indexes.