bluuebunny/crossref_metadata_2025 huggingface dataset:
The Role of Gastroesophageal Reflux in Pediatric Dysphagia
Authors: Catto‐Smith A. G. , Machida H. , Butzner J. D. , Gall D. G. , Scott R. B.
Published: January 2024

The history, physical examination, and the results of the upper gastrointestinal series, esophageal manometry, 24‐h pH recording, endoscopy, and biopsy are reviewed in 16 children (mean age of 10.6 years, range of 3 years 5 months to 15 years 3 months) who presented to the Alberta Children's Hospital with dysphagia (“food‐sticking”) without previously identified provocative disorders since January 1985. Of the 16 patients, 11 had had intermittent obstruction, and 7 had had intervention to relieve obstruction (2 Heimlich maneuvers, 1 intravenous glucagon, and 4 endoscopy after failure of intravenous glucagon). Although only five children had a recent history suggestive of gastroesophageal reflux, 12 had histologic evidence of reflux esophagitis (including 1 with a peptic stricture, 1 with “nutcracker” esophagus, and 1 with esophageal dysmotility characteristic of Down's syndrome) and all responded clinically to antireflux therapy. Of the remaining four patients, one had extrinsic esophageal compression from a vascular ring (right aortic arch with left ligamentum arteriosum), one had a single and another had recurrent episodes of food‐sticking without any identified abnormality, and one declined investigation. In childhood, dysphagia may be the presenting symptom of reflux esophagitis in the absence of a history suggestive of gastroesophageal reflux and without evidence of a peptic stricture.
Findings on Barium Swallow in Younger Siblings of Children with Hiatal Hernia (Partial Thoracic Stomach)
Authors: Thomas P. S. , Carré I. J.
Published: January 2024

The results are presented of an investigation by barium swallow of 271 younger siblings of children with hiatal hernia (HH). The incidence of HH among younger siblings with a history of repeated vomiting and regurgitation was 41% and in those without symptoms was 4.3%.
Functional Intestinal Obstruction in Henoch‐Schonlein Purpura
Authors: Sharief N. , Ward H. C. , Wood C. B. S.
Published: January 2024

Two patients with Henoch‐Schonlein purpura are described. Both developed prolonged intestinal obstruction requiring total parenteral nutrition. One child had transient partial duodenal obstruction on barium study and in the other extensive ecchymosis was confirmed at laparotomy.
Pancreatitis with Hypoglycemia‐Associated Convulsions Following Rotavirus Gastroenteritis
Authors: Nigro Giovanni
Published: January 2024

A case of acute pancreatitis and hypoglycemia‐associated convulsions following rotavirus gastroenteritis, occurring in a previously healthy 2‐year, 8‐month‐old girl, is reported. Rotavirus infection was demonstrated both by detection of virus particles in stools by electron microscopy and Rotazyme Abbott, and by detection of specific serum IgM and IgG antibodies. Pancreatitis was revealed by raised serum amylase and lipase levels and by ultrasonographic findings. Moreover, transient islet cell antibodies were found. No abnormalities were revealed by clinical and laboratory follow‐up studies. As suggested by this case report, further investigations on the possible pancreatic involvement by rotavirus may be helpful.
Anti‐Liver‐Kidney Micro some Antibody‐Positive Autoimmune Hepatitis Associated with Alopecia
Authors: Chaves Virginie , Paunier Luc , Berclaz Raymond , Delèze Guy , Abuaf Nisen , Belli Dominique C.
Published: January 2024

A 14‐year‐old girl presented with anti‐liver‐kidney microsome autoimmune hepatitis preceded by alopecia 3 years earlier. Both pathologies were greatly improved by immunosuppressive therapy. Alopecia is a newly reported extrahepatic manifestation of type 2 autoimmune hepatitis. Its appearance could alert the clinician to an increased risk of autoimmune hepatitis in children.
Comparison of Indium‐111 Scintigraphy and Colonoscopy with Histologic Study in Children for Evaluation of Colonic Chronic Inflammatory Bowel Disease
Authors: Tolia Vasundhara , Kuhns Lawrence R. , Chang Chung‐Ho , Slovis Thomas L.
Published: January 2024

Indium‐Ill leukocyte scanning and colonoscopy were performed in 19 children and adolescents with chronic inflammatory bowel disease to study the correlation of evaluation between these two diagnostic modalities in comparison to histologic study for colonic disease. Seven patients had ulcerative colitis, 10 had Crohn's disease, and two patients had no specific diagnosis after evaluation. The sensitivity of indium‐Ill scan was 18%, specificity was 62.5%, and accuracy for diagnosing colonic disease was only 37%. In comparison, sensitivity and specificity for colonoscopy were 100 and 57%, respectively. Furthermore, accuracy with colonoscopy was 84%. Our data suggest that the usefulness of scans is limited to patients in whom standard diagnostic procedures are contraindicated. In addition, it is essential to confirm the visual diagnostic impression on colonoscopy with histologic study.
Pancreatic Pseudocyst
Authors: Korman Stanley H. , Lebensart Pinchas , Martin Oz , Katz Schmuel , Tamir Israel
Published: January 2024

A pseudocyst of the pancreas in a 6‐year‐old girl persisted for 2 months despite bowel rest and nutritional support. Following percutaneous introduction of a catheter into the cyst under ultrasound guidance and external catheter drainage for 11 days, the pseudocyst resolved completely and permanently. Nonoperative percutaneous techniques for drainage of pancreatic pseudocysts in children may be an effective alternative to surgical intervention.
Complete Spontaneous Disruption of the Common Bile Duct
Authors: Dolgin Stephen E. , Levine Richard L. , Norton Karen I. , Marolda Joanne R. , Paries James G. , LeLeiko Neal S.
Published: January 2024

A 20‐month‐old infant had bile peritonitis from spontaneous complete avulsion of the distal common bile duct. The patient had recovered from extensive neonatal necrotizing enterocolitis that included the duodenum. We believe that this late unusual biliary catastrophe was a consequence of the neonatal necrotizing enterocolitis.
Sudden Death Caused by Unsuspected Pulmonary Arterial Hypertension, 10 Years After Surgery for Extrahepatic Biliary Atresia
Authors: Moscoso G. , Mieli‐Vergani G. , Mowat A. P. , Portmann B.
Published: January 2024

A case of extrahepatic biliary atresia presenting with an acute respiratory episode and rapid deterioration, 10 years after a successful portoenterostomy and a very active life, was the setting of unsuspected severe pulmonary arterial hypertension leading to sudden death. The pulmonary arteries showed widespread plexiform lesions, thickening of the muscular media, and subendothe‐lial proliferation. Occasionally, eccentric arterial obstructive lesions and fibrinous thrombi were observed. There was marked reduction of preacinar arterioles with a consequent increase in the alveolar/arterial ratio. The pulmonary veins showed arterialization of their walls. There was marked hypertrophy of myocardial fibres in the right ventricle together with foci of myocardial degeneration and fibrosis. Areas of endocardial thickening were observed in both ventricles. The absence of clinical indicators of pulmonary arterial hypertension at any but the terminal stages of the disease precluded any form of conservative management. Lung‐heart and presumably liver transplantation might have been the only option. Prospective assessment of pulmonary function and haemodynamic studies should be considered in cirrhotic patients with portal hypertension.
Hemorrhagic Colitis and Pseudomelanosis Coli in Ipecac Ingestion by Proxy
Authors: Johnson Joseph E. , Carpenter Becky L. M. , Benton Jane , Cross Robert , Eaton Lisle A. , Rhoads J. Marc
Published: January 2024

This report describes a toddler with chronic diarrhea, vomiting, and hypotonia due to surreptitious administration of syrup of ipecac by his mother (Munchausen's syndrome by proxy). Several features of this case distinguish it from previous reports of chronic ipecac ingestion in childhood: the development of grossly bloody stools; radiologic, endoscopic, and biopsy evidence of a chronic moderate colitis resembling ulcerative colitis; and the histologic finding of pseudomelanosis coli, providing an important clue to toxic ingestion. The significance and possible mechanism for genesis of pseudomelanosis coli is discussed. This case emphasizes the variability in presentation and difficulty in diagnosing long‐term ipecac ingestion by proxy. Ipecac toxicity should be considered in children with unexplained colitis and vomiting.
Effect of Dietary Fiber and Starch on Fecal Composition in Preschool Children Consuming Maize, Amaranth, or Cassava Flours
Authors: Hamaker Bruce R. , Rivera Kervan , Morales Enrique , Graham George G.
Published: January 2024

Metabolic balance studies were carried out in young children fed diets based on maize, amaranth, or cassava flours and in corresponding casein controls. Dietary fiber intakes were 22.2, 20.5, and 9.0 g/day for the maize, amaranth, and cassava groups, respectively. Fecal energy losses at least doubled in all test diets when compared with the corresponding controls and could generally be accounted for by recovered fiber in the feces. Fecal starch was also a significant contributor to fecal energy in the cassava group. All cassava fiber was recovered in the feces, whereas only 48.4 and 16.3% were recovered from ingested maize and amaranth. 2,6‐Diaminopimelic acid (DAPA), an indicator of bacterial mass, was highest in the cassava group. Expired breath hydrogen was highest for those consuming maize or cassava. Resistant starch may have been responsible for the high DAPA and breath hydrogen values in the cassava group.
Fatal Liver Failure in Two Children with Niemann‐Pick Disease Type B
Authors: Labrune P. , Bedossa P. , Huguet P. , Roset F. , Vanier M. T. , Odievre M.
Published: January 2024

We report on two young patients with Niemann‐Pick disease type B presenting with severe hepatic disease. Both children developed cirrhosis and died of intrahepatic block and mechanical hemolysis. Autopsy findings revealed complete obstruction of the sinusoids by lipid‐laden Kupffer's cells. These two cases illustrate a new hepatic lesion of Niemann‐Pick disease.
Erosion Through the Posterior Gastric Wall by a Pancreatic Pseudocyst Secondary to Gastric Duplication
Authors: Chawla Anupama , Gadaleta Dominick , Kenigsberg Kenneth , Kahn Ellen , Markowitz James
Published: January 2024

Gastric duplications are the rarest form of enteric duplication. They account for about 20% of all gastrointestinal duplications. Delayed and missed diagnosis leads to prolonged morbidity and mortality. We report a case of a child with a gastric duplication cyst in the body of the pancreas, associated with a pancreatic pseudocyst that ultimately eroded into the posterior wall of the stomach.
Selenium Absorption and Retention by Very‐Low‐Birth‐Weight Infants
Authors: Ehrenkranz Richard A. , Gettner Patricia A. , Nelli Catherine M. , Sherwonit Elaine A. , Williams Joanne E. , Ting Bill T. G. , Janghorbani Morteza
Published: January 2024

Measurements of dietary selenium absorption and retention were obtained after administration of a single dose of the extrinsic stable isotope tag 74Se in 20 appropriate for gestational age premature infants with birth weights between 720 and 1,630 g and gestational ages between 26 and 33 weeks. Infants were assigned randomly to receive a standard premature formula (1.34 μg of Se/dl) or a selenium‐supplemented version of that formula (2.03 μg of Se/dl). Each study consisted of one feeding that had been extrinsically labeled with 74Se (1.03 μg/kg) and a timed stool and urine collection. The percent 74Se absorption was 91.2 ± 5.4% (mean ± SD) from the standard formula and 86.2 ± 3.0% from the selenium‐supplemented formula (p < 0.05), but the percent of the absorbed 74Se retained was not different, i.e., 96.6 ± 2.1% and 95.0 ± 2.8%, respectively. The percent net absorption and net retention were also not different between the standard and selenium‐supplemented formulas; net absorption was 72.7 ± 18.1% vs. 67.8 ± 18.8% and net retention was 57.2 ± 17.6% vs. 53.3 ± 20.2%, respectively. The percent 74Se absorption and true selenium absorption were significantly correlated with the percent net selenium absorption and net selenium absorption, respectively. We conclude that an extrinsically administered dose of 74Se can be used to study selenium nutrition in growing premature infants.
Fat Absorption in Premature Infants
Authors: Hamosh Margit , Mehta Nitin R. , Fink Carol S. , Coleman Jane , Hamosh Paul
Published: January 2024

Fat absorption from two different premature infant formulas and one full‐term formula containing three different fat blends was investigated in two groups of premature infants. The first group of nine infants (gestational age, 29.1 ± 0.88 weeks; postnatal age, 3.13 ± 0.71 weeks) was fed alternately for 1 week each SMA preterm formula containing either high levels (50%) of medium‐chain triglycerides (MCT) (6:0, 8:0, and 10:0) or high levels (86%) of long‐chain triglycerides (LCT) (≥C12). Except for fat blends, the formulas were otherwise identical. The second group of 11 infants (gestational age, 30.5 ± 0.77 weeks, studied at a postnatal age of 4.33 ± 0.91 weeks) was fed for 1 week a full‐term infant formula, S‐26, containing 98% LCT. Fat absorption (studied during a 3‐day fat balance period) was similar irrespective of fat blend: 89.08 ± 2.37% during feeding of preterm SMA, 50% MCT; 87.0 ± 3.81% during feeding of preterm SMA, 86% LCT: and 83.00 ± 2.89% during feeding of S‐26, 98% LCT. Weight gain (grams per day) and increase in length (centimeters per day) were 23.2 ± 1.7, 21.20 ± 1.7, and 14.28 ± 2.9, and 0.17 ± 0.06, 0.16 ± 0.04, and 0.22 ± 0.07 during feeding of the three fat blends, respectively. Lipase activity levels in fasting gastric aspirates were higher during feeding of the LCT than the MCT formula. The possible stimulation of gastric lipase secretion secondary to long‐chain fatty acid stimulation of cholecystokinin secretion might be related to the efficient digestion of formula fat, irrespective of triglyceride‐fatty acid chain length. The study shows that preterm infants are able to digest and absorb efficiently formula fat containing as much as 98% long‐chain fatty acids.
Metabolites of Lactose Synthesis in Milk from Women During Established Lactation
Authors: Arthur Peter G. , Kent Jacqueline C. , Hartmann Peter E.
Published: January 2024

Summary: Metabolites in the lactose synthesis pathway were measured in human milk during established lactation. The mean concentrations (range) were: lactose, 198 m M (175–233); glucose, 1.5 m M (0.1–2.4); glucose 6‐phosphate, 11 μ M (4–23); glucose 1‐phosphate, 2.7 μ M (1.6–6.8); UDP‐glucose, 2.7 μ M (0.6–6.0); UDP‐galactose, 0.7 μ M (<0.1–2.5); inorganic phosphate, 2.2 m M (0.8–2.9). During storage of milk within the breast for 2 h, glucose 6‐phosphate concentration increased by 1.4 ± 0.5 μ M, while UDP‐glucose decreased by 1.0 ± 0.4 μ M. None of the other metabolites showed significant

changes in concentration during this period. Only glucose showed a circadian rhythm in concentration, with the highest concentrations between 1400 and 1800 h. The volumes of milk produced by the mothers ranged from 178 to 1,739 ml/24 h. Despite this variation, there were no significant relationships between the 24‐h average concentrations of glucose, glucose 6‐phosphate, glucose 1‐phosphate, UDP‐glucose, UDP‐galactose, or inorganic phosphate and the amount of lactose produced over 24 h.
Soluble Interleukin‐2 Receptor as a Marker of Lymphocyte Activation in Childhood Crohn's Disease
Authors: Proujansky Roy , Carpenter A. Betts
Published: January 2024

Summary: Serum concentrations of the soluble form of the interleukin‐2 receptor (sIL‐2R) were determined by an enzyme‐linked immunosorbent assay in a group of 39 pediatric and adolescent patients with Crohn's disease and in age‐matched ulcerative colitis patients and controls. sIL‐2R levels were found to be elevated in patients with Crohn's disease (p < 0.001), and increased sIL‐2R levels were detected in patients with clinically more severe disease. sIL‐2R levels correlated more closely with other laboratory markers of disease activity than with a disease activity score. A progressive increase in sIL‐2R levels was noted to correlate with endoscopic measurement of disease extent, while sIL‐2R levels did not correlate with other markers of systemic lymphocyte activation, suggesting possible local mucosal production. Sequential determinations in individual patients revealed a good correlation between sIL‐2R and clinical course. More important, elevated levels of sIL‐2R preceded clinical relapse of asymptomatic patients. We conclude that sIL‐2R measurement may be a useful adjunct to clinical assessment and routine laboratory testing in pediatric and adolescent patients with Crohn's disease and that serial levels may be predictive of clinical course and the response to therapy.
Crying Does Not Exacerbate Gastroesophageal Reflux in Infants
Authors: Orenstein Susan R.
Published: January 2024

The behavior and intraluminal esophageal pH of 48 infants (5–26 weeks old) were continuously recorded during the 120‐min period following their ingestion of a standard volume of apple juice (pH ∼ 4). To evaluate the effect of three basic behavior states on the frequency of infant gastroesophageal reflux, the data from all 19 of these infants who spent time in all three behavior states during the 120‐min study were analyzed for this study. Results were expressed as the frequency of gastroesophageal reflux episodes per hour of time spent crying, per hour of time awake without crying, and per hour of time asleep. Reflux frequency while crying was compared to reflux frequency while awake without crying; reflux frequency awake (both crying and not crying combined) was compared to reflux frequency asleep. The results indicate that, as has been shown previously, sleeping decreases reflux frequency [nine (1–48) episodes per hour awake vs. two (0–134) episodes per hour asleep, expressed as median (range), p = 0.025], but that, contrary to expectation, crying appears to decrease rather than increase both reflux frequency [11 (0–84) episodes per hour crying vs. 17 (0–213) episodes per hour awake without crying, p = 0.035] and total duration [21% (0–79) of crying time with pH < 4 vs. 41% (0–93) of noncrying awake time with pH < 4, p = 0.025], Crying does not exacerbate reflux in infants with gastroesophageal reflux disease.
Short‐Term Polycose Substitution for Lactose Reduces Calcium Absorption in Healthy Term Babies
Authors: Moya M. , Cortes E. , Ballester M. I. , Vento M. , Juste M.
Published: January 2024

A vitamin D‐independent paracellular and not saturable mechanism important for intestinal calcium absorption turned out to be less effective in lactose‐free diets. Twelve normal term babies with identical repletion levels of 25‐hydroxyvitamin D (25D) were fed sequentially with starting formulas that differed only in their carbohydrate sources: lactose (control) and polycose. A 3‐day test was performed after a 1‐week adjustment to the new diet. Fecal excretion of calcium was significantly higher in the lactose‐free formula period compared to the control one: 56.6 (13.9) versus 39.6 (12.8) mg/kg/day (p < 0.02); subsequently, the net retention was considerably higher and statistically significant (p < 0.01) for the control period versus the lactose‐free formula period: 99.4 (15.3) versus 78.1 (14.3). Plasma 25D values were 21.8 (9.1) and 23.7 (7.1) ng/ml at the beginning of the lactose and polycose periods, respectively, and 1,25‐dihydroxy‐vitamin‐D values were 64 (62) and 75 (45) pg/ml, not significant. Results suggest that formulas containing polycose as the sole carbohydrate source may reduce the intestinal absorption of calcium in term newborns, but determining long‐term biological significance requires further observation.
OKT3 Treatment of Steroid‐Resistant Rejection in Pediatric Liver Transplant Recipients
Authors: McDiarmid Sue V. , Busuttil Ronald W. , Terasaki Paul , Vargas Jorge H. , Ament Marvin E.
Published: January 2024

Of a total of 187 consecutive liver grafts in 149 pediatric recipients, 59 episodes of steroid‐resistant, biopsy‐proven rejection (32% of grafts) were treated with OKT3 monoclonal antibody. After 59 OKT3‐treated episodes, liver function at the end of treatment was normal in 40%, improved in 35%, and unchanged in 24%. Of 21 partial responses, 12 episodes eventually resolved to yield an overall complete response rate of 59%. CD3‐positive T‐cells >5% occurred during 61% of OKT3‐treated rejection episodes and was associated with impaired efficacy of OKT3 (30% complete response rate). Six grafts were re‐treated with OKT3 for rejection and in all CD3‐positive T‐cells could not be maintained <5%. Of the six grafts requiring repeat OKT3, five failed‐re‐treatment and required retransplantation. OKT3 antibodies of low titer were found prior to a second use of OKT3 in 65% of episodes. Patients treated with OKT3 after failing more than two preceding steroid courses had a significantly increased chance of graft loss (57%; p = 0.01). We conclude that this group of pediatric patients appeared less responsive to OKT3 compared to other series combining pediatric and adult recipients, possibly due to a more vigorous immune response in the child.
Effect of Oral Insulin on Lactase Activity, mRNA, and Posttranscriptional Processing in the Newborn Pig
Authors: Shulman Robert J. , Tivey David R. , Sunitha Iruvanti , Dudley Mary A. , Henning Susan J.
Published: January 2024

Insulin, found in human and pig colostrum and mature milk, appears to influence small intestinal growth and development. Ileal lactase activity is increased when porcine insulin is added to feedings administered to newborn piglets. We studied 2‐day‐old miniature piglets to determine whether the increase in lactase activity is accompanied by changes in enterocyte expression of lactase activity, steady‐state levels of lactase mRNA, and/or posttranscriptional changes in lactase processing. We randomized the piglets to receive bottle feedings of a swine‐weaning milk formula with (group F + I) or without (group F) the addition of 85 mU/ml of regular porcine insulin. The piglets were fed for 6 days (to 8 days of age), after which they were killed and the small intestine removed for analysis. Despite large differences between groups in enterocyte expression of lactase activity in the ileum, no differences were noted in the level of ileal lactase mRNA. The relative proportions of the 207, 210, and 230 kDa precursors of the 160 kDa mature lactase protein were similar between groups. These data indicate that the insulin‐induced increased expression of ileal lactase activity is not regulated at the level of its mRNA or at the level of processing of the polypeptide.
Levels and Molecular Forms of Immunoreactive Trypsin and Chymotrypsin in Amniotic Fluids from Normal and Cystic Fibrosis Fetus
Authors: Carrère J. , Muller F. , Boué A. , Guy‐Crotte O. , Figarella C.
Published: January 2024

We previously suggested that an activation defect of pancreatic proteolytic zymogens in newborns suffering from cystic fibrosis (CF) might contribute (by an adaptative‐like process) to the significant increase of the serum trypsin level observed in the disease at birth. To give support to this hypothesis we studied two pancreatic enzymes: trypsin 1 (IRT) and chymotrypsin A (IRChT) by noncompetitive enzyme immunoassays in amniotic fluids taken at 17–18 weeks of pregnancy. In normal fluids (102), the levels of the two enzymes were widely dispersed between 5 and 100 μ,g/L. A similar pattern was observed for the fluids with a 1 in 4 risk of CF with a normal outcome (24). In contrast, the levels of pancreatic enzymes in the fluids with affected fetus (40) were always below 45 μg/L for IRT and 55 μg/L for IRChT and most of them were under 20 μg/L for both enzymes. The molecular forms of IRT and IRChT in amniotic fluids were studied by gel filtration. In amniotic fluids with affected fetus, a major form of IRT was eluted in a position consistent with the elution of proteins around 25 kDa and two peaks of IRChT were eluted at 75 kDa and 25 kDa. These patterns are similar to those observed in normal serum when zymogens are present and are quite different from the patterns obtained by gel filtration of amniotic fluids with normal outcome. In normal fluids, the major form of IRT was eluted with a protein of higher molecular weight (–76,000) consistent with a complex of trypsin with α1‐proteinase inhibitor and an additional peak of IRChT (–14 kDa) was present, possibly corresponding to a degraded form of chymotrypsin. These data shows the absence of active pancreatic proteases in the intestinal fluid of CF affected fetus in opposite to their presence in normal fetus. This absence could lead to an increased synthesis of proteolytic enzymes at birth.
Serum Primary Bile Acids in the Course of Celiac Disease in Children
Authors: Ejderhamn J. , Samuelson K. , Strandvik B.
Published: January 2024

Scrum concentrations of primary bile acids (BAs) were determined in the course of celiac disease and in healthy age‐matched controls. Determinations of BAs were made by radioimmunoassays after an overnight fasting and for 4 h postprandially after intake of a standardized test meal. In untreated celiac disease presenting with subtotal villous atrophy, peak concentrations of primary BAs were significantly delayed and serum levels were significantly higher than in controls. Small but significant abnormalities persisted after a period of gluten‐free diet despite a normalization of mucosal morphology. Slightly pathological standard liver function tests in five patients were related to increased fasting levels but not to the postprandial pattern of BAs, indicating that the observed abnormalities postprandially were not related to liver disease. Further studies simultaneously focusing on deconjugation mechanisms, absorption, and pool sizes of BAs are required to explain the postprandial BA pattern under the course of celiac disease.
Solubility and Digestibility of Milk Proteins in Infant Formulas Exposed to Different Heat Treatments
Authors: Rudloff Silvia , Lönnerdal Bo
Published: January 2024

There are presently several fundamentally different technologies to produce infant formulas (IF), such as sterilization, spray‐drying, and treatment at ultrahigh temperature (UHT). The effects of heat treatment on milk proteins in IF were analyzed by column chromatography, gel electrophoresis, and Kjeldahl analysis, revealing strong protein‐protein and protein‐lipid interactions in processed milk. These interactions were more pronounced in conventionally (in‐can) sterilized than in spray‐dried (powdered) and UHT products confirming their temperature dependency. Analysis of raw materials, intermediate and end products of IF processing revealed that after homogenization the first indications of protein denaturation occurred, but that in‐can sterilization as the final heat treatment caused irreversible denaturation of proteins and strong protein‐lipid interactions. Lowering of the pH to 4–5, which is physiological for the stomach of young infants, enhanced the interactions. Support for an impairment of protein digestibility was given by in vitro analysis of protein digestibility, demonstrating significantly lower digestibility of in‐can sterilized IF compared to their spray‐dried and UHT counterparts. To investigate the effect of heat treatment on chemical reactions, i.e., occurrence of Maillard products, we showed by a fluorimetric assay that the amount of “available” lysine is lower in sterilized than in powdered IF. Our findings suggest that a more differentiated view regarding the protein quality of IF is needed.
Congenital Sodium Diarrhea with a Partial Defect in Jejunal Brush Border Membrane Sodium Transport, Normal Rectal Transport, and Resolving Diarrhea
Authors: Fell J. M. , Miller M. P. , Finkel Y. , Booth I. W.
Published: January 2024

Defective jejunal sodium/proton exchange causes severe, congenital secretory diarrhea. We report a boy who presented typically in utero, but in whom diarrhea resolved during the first year of life. Pregnancy was complicated by polyhydramnios, and an ultrasound at 31 weeks showed a distended fetal small intestine. The abdomen was grossly distended at birth, and profuse secretory diarrhea began immediately. He subsequently thrived on breast milk and electrolyte supplements. Studies of jejunal brush border sodium/proton exchange at 6 months showed a partial defect. Nonequilibrium rectal dialysis showed rectal sodium and potassium transport to be intact. Diarrhea lessened after 9 months, and the patient subsequently required occasional laxatives. These observations suggest that there is a spectrum of congenital abnormality in this exchanger, and that in children with incomplete defects normal colonic sodium salvage can subsequently mask net small intestinal secretion.
Eicosanoids in Hypoxic Insult to Neonatal Rabbit Bowel
Authors: Dollberg Shaul , Udassin Raphael , Ginat‐Israeli Talia , Weidenfeld Josef , Branski David
Published: January 2024

Eicosanoids, derivatives of arachidonic acid, play a role in several inflammatory diseases of the bowel. To determine whether prostaglandin E2 (PGE2), leukotriene B4 (LTB4), and leukotriene C4D4E4 (LTC4D4E4), have a role in hypoxic insult to the intestine, we examined the levels of these mediators in a hypoxic neonatal rabbit model. One group of animals underwent hypoxic insult postnatally, the second group did not undergo hypoxia and served as a control. The levels of PGE2, LTB4, and LTC4D4E4 were determined by radioimmunoassay. PGE2 in the hypoxic group was 1,779± 142 pg/mg protein (mean± SD) as opposed to 2,380± 197 pg/mg protein in the control group (p < 0.02). LTB4 level was 5,446± 3,492 pg/mg protein in the hypoxic rabbits and 3,362± 2,570 pg/mg protein in the control group (p < 0.03). There was no statistically significant difference in the level of LTC4D4E4 between the two groups. Our study shows that hypoxia shifts the arachidonic acid metabolism toward enhanced lipoxygenase activity with a resultant increase in LTB4 levels and a concomitant decrease in cyclooxygenase activity with reduced PGE2 levels in the bowel. The shift in the balance between these eicosanoids may play a role in the pathogenesis of ischemic‐hypoxic bowel diseases by enhancing the inflammatory response in the intestine, and simultaneously, diminishing cytoprotection.
Inhibition of Adhesion of S‐Fimbriated Escherichia coli to Epithelial Cells by Meconium and Feces of Breast‐Fed and Formula‐Fed Newborns
Authors: Schroten H. , Lethen A. , Hanisch F. G. , Plogmann R. , Hacker J. , Nobis‐Bosch R. , Wahn V.
Published: January 2024

We investigated the ability of meconium, feces from human milk‐fed (HMF) newborns, and feces from formula‐fed (FF) newborns to inhibit adhesion of S‐fimbriated E. coli to human buccal epithelial cells. S‐fimbriae are a common property of E. coli strains causing sepsis and meningitis in neonates. Meconium had the highest content of neuraminic acid and the strongest inhibitory effect on bacterial adhesion. HMF also exerted high inhibitory activity while FF was markedly less active: To achieve inhibitory effects comparable to HMF a sixfold amount of FF was required. Glycoproteins from excretions were separated by gel chromatography. Fractions obtained were analyzed for adhesion‐inhibiting activity. In all excretions analyzed, the mucin‐containing fraction could be identified as the major inhibitory component. Inhibition was probably mediated by specific interaction of this fraction with S‐fimbriae, as shown by binding of isolated fimbriae on Western blots after electrophoretic separation of glycoproteins. In conclusion, our data support the view that the mucin‐containing fraction from meconium and human milk exerts antibacterial functions by preventing adhesin‐mediated binding of pathogenic bacteria to mucosal epithelia.
ELISA Method for Quantitative Measurement of IgA and IgG Specific Anti‐gliadin Antibodies
Authors: Perticarari S. , Not T. , Cauci S. , Luchesi A. , Presani G.
Published: January 2024

In this study we describe the application of an enzyme‐linked immunosorbent assay (ELISA) for the measurement of anti‐α‐gliadin antibodies (AGA) in absolute units (μmlg protein/ml). Enriched samples of IgA and IgG AGA were obtained by means of protein A chromatography after immunoaffinity purification of pooled sera from untreated celiac patients. No cross‐reactivity with other food antigens (β‐lactalbumin, soya proteins, ovalbumin) was detected. The quantitative evaluation of protein content in IgA and IgG AGA samples obtained by immunoaffinity chromatography, was performed by means of ELISA sandwich method using a reference curve obtained with pure standard human immunoglobulins. Scalar concentrations of purified IgA and IgG were then used to obtain a reference standard curve by means of an ELISA method. Such standard curve was utilized for titrating AGA in 214 sample sera. The minimal detectable concentration of IgA and IgG AGA was 0.02 μmlg/ml. The reproducibility of within‐ and between‐assay resulted very good for IgA‐AGA and acceptable for IgG‐AGA. The method here described seems to be satisfactory not only for quantitative diagnostic purposes in routine screenings but also in epidemiological studies. Moreover, it can constitute a suitable way to solve practical problems of quality control of AGA ELISA assay.
Rectal Histopathology in Endemic Shigella and Salmonella Diarrhea
Authors: Sachdev H. P. S. , Chadha V. , Malhotra V. , Verghese A. , Puri R. K.
Published: January 2024

Rectal histopathology was evaluated in 34 cases (2 months‐12 yrs old) of endemic “invasive diarrhea” [>20 WBCs per high‐power field on stool microscopy with (RBC positive) or without (RBC negative) associated RBCs] where S. dysenteriae (n = 9), S. flexneri (n = 11), and nontyphoidal Salmonella were isolated as the sole identifiable enteropathogens. Persistent diarrhea (>14 days duration) was more common with Salmonella infection whereas RBC‐positive “invasive diarrhea” was more frequent with Shigella, particularly S. dysenteriae (all cases) infection. The histopathological profile was comparable to the earlier descriptions of infective colitis to a large extent and the nature of the infecting organism could not be determined on the basis of rectal histology alone. The other noteworthy features were as follows: (i) mild crypt distortion (26%) and branching (21%) in both Shigella and Salmonella infection; in Salmonella infection, dilation of the glands was significantly greater with persistent diarrhea; (ii) presence of chronic inflammatory cells either alone or in combination with neutrophils in 62%; a predominant neutrophilic response was significantly higher with S. dysenteriae infection and an acute presentation; (iii) pseudomembrane formation (six subjects; 18%) especially in S. dysenteriae (four cases); and (iv) a significant association of neutrophilic response, edema, and neutrophils within the vessels in the lamina propria and mucin depletion in the glands with RBC‐positive “invasive diarrhea.”
Urinary Growth Hormone in Growth‐Impaired Children with Chronic Inflammatory Bowel Disease
Authors: Braegger C. P. , Torresani T. , Murch S. H. , Savage M. O. , Walker‐Smith J. A. , MacDonald T. T.
Published: January 2024

Growth impairment of undefined aetiology occurs in ˜30% of children with chronic inflammatory bowel disease. We measured urinary growth hormone concentrations in 36 children with chronic inflammatory bowel disease and 51 normal controls. The median urinary concentration of growth hormone in the stunted children with chronic inflammatory bowel disease was 15.8 ng/g creatinine (range 4.3‐32.6), compared with 11.7 ng/g creatinine (range 4.1‐35.9) in those with normal growth. The difference was statistically not significant (p = 0.15). Moreover, there was no significant difference between the patients and the control group. One stunted patient had a urinary growth hormone (UGH) concentration below the normal range, whereas four patients with normal height were also below the normal range. Four of these five patients (80%) were on corticosteroid treatment at the time of urinary collection, whereas only 26% of the patients with normal UGH were also on corticosteroid treatment. We conclude that growth retardation in children with chronic inflammatory bowel disease is probably not related to growth hormone deficiency but must involve other mechanisms.
The Pathogenesis of Biliary Atresia
Authors: Ho Chi‐Whai , Shioda Kei , Shirasaki Kazuya , Takahashi Shigeki , Tokimatsu Shiyuji , Maeda Kazuichi
Published: January 2024

Specimens of the extrahepatic biliary trees and wedge liver biopsies were studied in 11 cases of biliary atresia. The hepatic arteries at the porta hepatis were examined with ultrasound in five of the 11 cases. The basic histopathological features were found to be epithelial damage and an inflammatory, sclerosing change of both the intrahepatic and the extrahepatic biliary trees, combined with an arteriopathy manifesting as hyperplasia and hypertrophy of the hepatic arteries in all cases. Based on the results of ultrasonography and histological examination, this arteriopathy was thought to affect the arteries from the trunk of the common hepatic artery to its peripheral branches supplying the entire biliary tree. To determine whether these morphologically abnormal hepatic arteries are related to the pathogenesis of biliary atresia in association with abnormal blood flow and pressure requires further investigation.
Assessment of Vitamin K Status of the Newborn Infant
Authors: von Kries Rüdiger , Greer Frank R. , Suttie John W.
Published: January 2024

This article reviews our current knowledge of the assessment of vitamin K status in the human infant, where a deficiency state has been described. Laboratory measures for the functional assessment of vitamin K‐dependent coagulation factors as well as the quantitative assay for vitamin K1 (phylloquinone) are reviewed. In addition, four different methods of measuring abnormal prothrombin, a protein induced by vitamin K absence (PIVKA‐II), and its use in clinical pediatrics are discussed. Finally, additional methods are briefly described for assessing vitamin K status, including serum osteocalcin, urinary excretion of γ‐carboxyglutamic acid, and deficiencies of the enzymes necessary for the regeneration of phylloquinone in the so‐called vitamin K cycle.
Postmenstrual Age Correlates to Indices of Protein Metabolism in Very Low Birth Weight Infants
Authors: Boehm G. , Räihä N. C. R.
Published: January 2024

In 14 infants who were normal in weight for gestational age and 14 infants who were small for gestational age, the plasma essential amino acid profiles and serum urea concentrations were studied between the 30th and 46th weeks of postmenstrual age. All infants were of very low birth weight (<1,500 g) and were fed with fresh human milk fortified with 6 g freeze‐dried human milk per 100 ml (mean protein intake 3.1 g/kg/day, mean energy intake 130 kcal/kg/day). With the exception of threonine, all measured plasma essential amino acid concentrations increased significantly with increasing postmenstrual age (appropriate for gestational age infants: r = 0.861, p < 0.01; small for gestational age infants: r = 0.772, p < 0.001). No differences in this increase could be found between the infants who were small or appropriate for gestational age. The serum urea concentrations also increased with increasing postmenstrual age without differences between the study groups (appropriate for gestational age infants: r = 0.658, p < 0.01; small for gestational age infants: r = 0.604, p < 0.05). The results indicate that very low birth weight infants of similar weights may have very different protein requirements, depending on their postmenstrual ages. Thus, postmenstrual age is of greater importance than birth weight when protein nutrition is planned for very low birth weight infants.
An Open Triple Crossover Study Comparing Water Absorption from Potable Water, Lucozade, and Dioralyte Using the Stable Isotope 18O
Authors: Mecrow I. K. , Miller V.
Published: January 2024

Nine volunteer children aged 6–9 years were randomised in a Latin square design to receive one of three oral solutions [Lucozade (Beechams), Dioralyte (Rorer Pharmaceuticals), and potable water] labeled with the stable isotope 18O. After administration, breath CO2 was collected using a face mask for a period of 120 min. Mass spectrometric analysis gave the enrichments of 18O in CO2 at parts per million levels. Analysis showed the accumulation of the tracer (and, by inference, absorption from the gut) to be quickest from Dioralyte and slowest from Lucozade. We hope to use this technique to investigate the absorption of water from oral rehydration solutions in infants with dehydration due to gastroenteritis.
Does the Bile Salt‐Stimulated Lipase of Human Milk Have a Role in the Use of the Milk Long‐Chain Polyunsaturated Fatty Acids?
Authors: Hernell Olle , Bläckberg Lars , Chen Qi , Sternby Berit , Nilsson Åke
Published: January 2024

Long‐chain polyunsaturated (LCP) fatty acids derived from linoleic (18:2 n‐6) and α‐linolenic (18:3 n‐3) acids are considered essential nutrients in preterm infants. The efficiency by which such fatty acids are released as absorbable products from triacylglycerol was explored in vitro using rat chylomicron triacylglycerol as substrate. When incubated with purified human pancreatic colipase‐dependent lipase and colipase, arachidonic acid (20:4 n‐6) was released less efficiently than linoleic acid from such triacylglycerol. This difference was not seen when purified human milk bile salt‐stimulated lipase (BSSL) was incubated with the triacylglycerol substrate, and it was almost abolished when colipase‐dependent lipase (with colipase) and BSSL acted simultaneously, as they do in breast‐fed infants. There was no difference in arachidonic acid and eicosapentaenoic acid (20:5 n‐3) release rates with either colipase‐dependent lipase or BSSL, albeit the release was more rapid with the milk enzyme than with colipase‐dependent lipase. Again, the most efficient release as absorbable free fatty acids was achieved when the two lipases operated together. The relative resistance to hydrolysis of arachidonic acid and eicosapentaenoic acid by colipase‐dependent lipase was best explained by the localization of the first double bond to the ***6–5 position of the respective fatty acid. The results obtained suggest that BSSL is of importance for the efficient use of human milk LCP fatty acids.
Effects of Cerulein and Epidermal Growth Factor on Pancreatic Growth in the Reserpinized Rat Model
Authors: Bérubé France‐Line , Benrezzak Ouhida , Vanier Micheline , Morisset Jean
Published: January 2024

This study was undertaken to determine the effect of reserpine on rat pancreatic growth, to evaluate if reserpine‐caused alterations can be prevented by epidermal growth factor (EGF) and/or cerulein treatment, to evaluate the time course of rat pancreas recovery after reserpine, and to determine if EGF and/or cerulein treatment can accelerate such a recovery. In the first experiment, three groups of male Sprague‐Dawley rats (250–265 g) were used. Ad libitum‐fed control animals received the reserpine vehicle, and one experimental group received reserpine (1 mg kg‐1 day‐1 for 7 days) while the other, pair‐fed group received the reserpine vehicle with a reduced amount of food to result in malnourishment. Rats from each of these three groups were also assigned to one of four treatments consisting of saline, EGF (10 μUg kg‐1), cerulein (1 μUg kg‐1), or a combination (same doses) twice a day for 7 days. In the morning of the 8th day, after an overnight fat, rats were killed. In the second experiment, rats were selected and treated with reserpine or the vehicle as described in experiment 1; after the 7‐day treatment, a first cohort of animals was allowed a 30‐day recovery period. Three other groups (an ad libitum‐fed control, a pair‐fed, and a reserpine group) were allowed a 6‐day recovery period during which they were treated subcutaneously, twice a day, with either saline, EGF (10 μUg kg‐1), cerulein (1 μUg kg‐1), or a combination (same doses). On the morning of the 31st or 7th day, after an overnight fat, rats were killed. After death, all pancreata were examined for weight and protein, amylase, chymotrypsinogen, RNA, and DNA content. In the ad libitumfed control group, EGF caused pancreatic hypertrophy, whereas cerulein was associated with hypertrophy and hyperplasia. In the pair‐fed malnourished group, the EGF effect was limited to slight increases in pancreatic weight and cell mass whereas cerulein caused hypertrophy; EGF plus cerulein caused pancreatic hyperplasia. In the reserpine group, EGF had no effect, whereas cerulein caused pancreatic hypertrophy and an increase in DNA content above the reserpine control. After 30 days of recovery, pancreata of pair‐fed animals and those of rescrpine‐treated animals were comparable with those of the ad libitum‐fed control rats with the exception of amylase levels, which remained reduced in the reserpine group. After 6 days of recovery, the pancreata of the reserpine rats did not improve, but chymotrypsinogen content returned to ad libitum‐fed control values. During the 6‐day recovery period, total regeneration of the pancreatic tissue was observed in response to cerulein whereas EGF had no effect. In conclusion, modifications and alterations of the pancreatic tissue caused by reserpine and associated malnutrition can be prevented by cerulein, and cerulein remains the most potent factor to promote regeneration of the pancreatic gland after reserpine treatment and an episode of malnutrition.
Is Histological Diagnosis of Neuronal Intestinal Dysplasia Related to Clinical and Manometric Findings in Constipated Children? Results of a Pilot Study
Authors: Koletzko Sibyllc , Ballauff Antje , Hadziselimovic Faruk , Enck Paul
Published: January 2024

Neuronal intestinal dysplasia (NH>) of the colon has been reported in adults and children with chronic constipation. However, it is unknown whether these histological abnormalities are related to the severity of symptomatology or the findings of anorectal manometry. We studied 57 children (2 weeks to 17 years old, 33 boys) who had chronic constipation and/or soiling or obstructive symptoms early in life and evaluated anamnestic data, symptoms, outcome after 6 months of conventional therapy, rectal biopsies for signs of dysganghonosis, and results of anorcctal manometry. In cooperative patients, defeciaion dynamics were recorded during manometry with simultaneous surface electromyogram of the external anal sphincter. In 30 older patients, severity of constipation was assessed by measurement of colonic transit time with radiopaque markers. Histology confirmed Hirschsprung's disease in nine (excluded from further analysis), hyperganglionosis of the plexus submucosus (classic NID) in six, heterotopic ganglion cells without hyperganglionosis (abortive NID) in 18, and no signs of dysganglionosis in 24 patients. The rectoanal inhibitory reflex was more often absent or abnormal in children with classic NID (six of six) and abortive NID (11 of 17) compared with children with normal histology (four of 22) (p < 0.001). All other manometric parameters analyzed, colonic transit times, and clinical outcomes were not related to histological diagnosis. In contrast, the severity of constipation and outcome was significantly worse in children with abnormal defecation dynamics. These results suggest that histological signs of NID in the submucous plexus are of uncertain value in assessing the clinical picture and should not influence decisions concerning further treatment, especially surgical interventions.