bluuebunny/crossref_metadata_2025 huggingface dataset:
Hypoxemia in Infants with Biliary Atresia
Authors: Seidenberg Jürgen , Kluge Eva , Rodeck Burkhard , Burdelski Martin , von der Hardt Horst
Published: January 2024

Hypoxemia in liver cirrhosis has been attributed to increased pulmonary perfusion; lung function abnormalities have rarely been found in adults. In infants, however, smaller airways and the disproportion in size between the enlarged liver and abdominal cavity leading to lung compression by elevated diaphragms may well suggest that ventilation disturbances play an important role in the development of hypoxemia. We examined lung functions, ventilation‐perfusion scans, chest radiographs, and blood gases in air and 80% oxygen in 19 infants with biliary atresia (mean age 14 months) and found maximum flows at functional residual capacity (VmaxFRC) markedly decreased [48%± 29% (mean± SD)] and thoracic gas volume (TGV) elevated (156%± 30.2%). Po2 was <9.3 kPa in seven of 19 patients, in whom TGV was higher compared with the other patients (182% vs. 141%, p < 0.005). However, the decrease in Po2, was much more closely correlated to the amount of shunting (r = 0.62, p < 0.05) than to the reduced airway patency (VmaxFRC/TGV, r = 0.41, p = 0.08). We conclude that airway narrowing probably by lung compression is present more frequently in infants than in adults with liver disease. We found some evidence that hyperinflation contributes to the observed low Po2 values, possibly aggravated by inadequate vasoconstriction to hypoxic stimuli. However, pulmonary shunting independent of ventilatory disturbances more readily explained hypoxemia already present in these infants.
Impact of Zinc Supplementation on Intestinal Permeability in Bangladeshi Children with Acute Diarrhoea and Persistent Diarrhoea Syndrome
Authors: Roy S. K. , Behrens R. H. , Haider R. , Akramuzzaman S. M. , Mahalanabis D. , Wahed M. A. , Tomkins A. M.
Published: January 2024

Zinc has been shown to enhance intestinal mucosal repair in patients suffering from acrodermatitis enteropathica; but the impact on mucosal integrity during acute (AD) or persistent (PD) diarrhoea is unknown. One hundred eleven children with AD and 190 with PD aged between 3 and 24 months received, randomly and blind to the investigators, either an elemental zinc supplement of 5 mg/kg body wt/day or placebo in multivitamin syrup for 2 weeks while intestinal permeability and, biochemical and anthropometric markers were serially monitored. The permeability test was administered as an oral dose of 5 g lactulose/1 g mannitol in a 20‐ml solution followed by a 5‐h urine collection. The ratio of the urinary probe sugars was correlated to clinical, biochemical, and microbiological parameters. At presentation, lactulose excretion was increased and mannitol excretion decreased in both AD and PD as compared with age‐matched asymptomatic children. The lactulose/mannitol ratio (L/M) was higher in subjects with mucosal invasive pathogens (rotavirus and enteropathogenic Escherichia coli) compared with children excreting Vibrio cholera and enterotoxigenic. coli.

Two‐week zinc supplementation significantly reduced lactulose excretion in both AD and PD, whereas the change in mannitol excretion and L/M was similar between study groups in both studies. Changes in lactulose excretion were significantly influenced by zinc supplementation in children with E. coli, Shigella sp., and Campylobacter jejuni stool isolates. The greatest reduction in total lactulose excretion was seen in supplemented children who on presentation were lighter (wt/age <80%), thinner (wt/ht <85%), and undernourished [middle upper arm circumference (MUAC) <12.5 cm] or with hypozincaemia (<14 μmlmol/L). The results suggest zinc supplementation improves intestinal permeability in certain groups of children with AD or PD syndrome and contributes to their recovery. This effect may indirectly reflect enhanced mucosal recovery. Further studies on the mechanisms of mucosal repair following zinc supplementation are recommended.
Postnatal Development of Liver and Exocrine Pancreas in Polycythemic Newborn Infants
Authors: Boehm G. , Delitzsch A. K. , Senger H. , DelSanto A. , Moro G. , Minoli I.
Published: January 2024

In 35 newborn infants appropriate for gestational age the influence of neonatal polycythemia (venous hematocrit >60% measured between the second and fourth hour of life) on development of enterohepatic circulation of bile acids, activities of pancreatic enzymes in duodenal juice, and the effects of hemodilution were studied during the second week of life. A significant correlation was found between the initial hematocrit and both the bile acid concentration in serum and lipase and trypsin activity in duodenal juice. Of 35 infants, 10 were not treated with hemodilution due to asymptomatic polycythemia; they had the highest concentration of serum bile acids associated with the lowest lipase and trypsin activity in duodenal juice. However, the 25 infants treated with hemodilution also showed serum bile acid concentrations and lipase and trypsin activity in duodenal juice out of the normal range when compared to normocythemic infants. These data indicate that, during the first days of life, polycythemia results in a delayed postnatal development of enterohepatic circulation of bile acids and exocrine pancreas functions independently from the occurrence of clinical symptoms. Thus, it can be concluded that, on the first day of life, all polycythemic infants should be treated with hemodilution. Moreover, the nutritional management of these infants must also account for the limited functional capacity of the gastrointestinal tract.
THE HEAT‐STABLE ENTEROTOXIN RECEPTOR
Authors: Cohen Mitchell B. , de Sauvage EJ , Camerato T R , Goeddel DV.
Published: January 2024

Heat‐stable enterotoxins (STa), a family of small polypeptides produced by Escherichia coli strains, are responsible for diarrheal disease in children and in travellers. STa binds to a receptor located on the enterocyte brush border membrane and, as a result, activates a membrane‐bound guanylate cyclase. These events begin a cascade that culminates in intestinal fluid secretion and diarrhea. This report, and a second study published simultaneously (Biochem Biophys Res Commun 1991; 179:1455–63), describe the molecular cloning and expression of cDNA encoding the receptor for STa in human intestine. The human cDNA was cloned by screening a cDNA library with a probe based on the sequence of the rat intestinal STa receptor (Cell 1990;63:941–8) and synthesized using the polymerase chain reaction. As in the rat, the human STa receptor contains an extracellular STa‐binding domain, a transmembrane region, and a cytoplasmic guanylate cyclase domain. Overall, the human receptor is 81% identical at the deduced amino acid level to the rat receptor for STa, although there is only 71% homology in the extracellular domain, de Sauvage et al. also transfected a human kidney cell line with a cDNA encoding for this receptor. The transfected cell line demonstrated both STa binding and STa‐mediated guanylate cyclase activation.
RETROVIRUS INFECTION OF ENTEROCYTES
Authors: Fantini J , Yaki N , Chermann J‐C.
Published: January 2024

Although the gastrointestinal tract is thought to be one of the major routes for infection with human immunodeficiency virus (HIV), controversy still exists concerning the ability of HIV to infect epithelial cells of the intestinal mucosa. In this paper, Fantini et al. detailed their studies of the ability of HIV to infect the human colon cancer cell line HT‐29‐D4. Differentiation of these epithelial cells can be regulated in vitro by modifying the culture medium concentration of glucose and by replacing glucose with galactose. Intestinal cells in culture were infected with three different strains of HIV—HIV‐1‐BRU, HIV‐1‐NDK (a highly cytopathic strain), and HIV‐2‐ROD. Exponentially growing HT‐29‐D4 cells were exposed for 16 h to HIV in serum‐supplemented medium. After washing, epithelial cells were harvested and subcultured. Virus replication in subcultured cells was determined by HIV‐specific DNA amplification using the polymerase chain reaction. Amplified products were analyzed for specificity by Southern blot hybridization with a radiolabeled DNA probe internal to the primer templates. The presence of CD4 molecules (i.e., receptors for cell binding of HIV) on intestinal epithelial cells was assessed using monoclonal antibodies and immunofluorescence labeling.

Fantini et al. found that undifferentiated HT‐29‐D4 cells express a CD4‐related antigen that lacks the epitope known to be involved in recognition of the HIV envelope binding protein referred to as gp120. Undifferentiated HT‐29‐D4 cells were infected by both HIV‐1 and HIV‐2 strains. However, the infection did not alter cell growth. Differentiated HT‐29‐D4 cells were also infected, either through the apical brush border or the basolateral membrane. Although the CD4‐like surface molecules were restricted to the basolateral domain of differentiated cells, antibodies against specific epitopes of the CD4 glycoprotein did not inhibit HIV infection. The authors conclude that intestinal epithelial cells can be infected by both HIV‐1 and HIV‐2 via either the apical membrane or the basolateral membrane. There was no evidence of viral replication by reverse transcriptase activity. However, following culture of HT‐29‐D4 cells with virus, HIV DNA could be recovered by gene amplification as long as 9 months after exposure of intestinal tissue culture cells to the virus. The authors speculate that this might provide an explanation for the prolonged seronegative state observed in HIV‐infected individuals.

These findings raise the possibility that infected lamina propria lymphocytes and macrophages could coinfect intestinal epithelial cells. In addition, HIV present in the intestinal lumen could bind to the apical surface and infect enterocytes.
Evaluation of Liquid or Powdered Fortification of Human Milk on Growth and Bone Mineralization Status of Preterm Infants
Authors: Moyer‐Mileur Laurie , Chan Gary M. , Gill Gurmail
Published: January 2024

Thirty‐five preterm (<1500 g) infants were fed preterm human milk (PHM) supplemented with either powdered fortifier (PF) or liquid supplement (LS). Bone mineral content (BMC) of the distal third radius was measured by photon absorptiometry. Biochemical indices of nutritional and bone status were obtained every 2 weeks. The initial BMC for both feeding regimens were similar. BMC did not change over the study period for infants fed LS. Infants fed PF had BMC values greater than LS infants at weeks 2 and 4 of study. Only infants fed PF had BMC values that demonstrated a consistent increase. Serum total protein and phosphorus values were greater for PF infants at week 4 than LS infants. Weight, length, occipital‐frontal circumference (OFC) gains, serum albumin, alkaline phosphatase, calcium, and vitamin D levels were similar in both groups. We conclude that products used to “enrich” PHM are adequate to meet the growth needs of the preterm infant. However, we found that infants fed the powdered fortified preterm human milk had higher bone mineralization than those fed the liquid supplemented human milk.
Plasma Vitamin E Concentrations of Older Infants Fed Cow's Milk or Infant Formula
Authors: Shank Jackie S. , Dorsey Jodee L. , Anderson Kathryn , Cooper William T. , Acosta Phyllis B.
Published: January 2024

Nutrition of older infants, though important for optimal brain development, is inadequately studied. The beverage choice markedly influences nutrient intake, but little is known regarding nutrition status of older infants, particularly for vitamin E. This study assessed vitamin E intakes and plasma tocopherol concentrations in two groups of healthy infants, 8 to 13 months of age, who had consumed either cow's milk (n = 45) or milk‐based formula (n = 55) for a minimum of the 3 preceding months. Mean (±SEM) vitamin E intake was significantly lower (p ± 0.001) by the infants who had consumed cow's milk (CMF) than by infants who had consumed formula (FF); 4.1 ± 0.25 mg/day and 10.9 ± 0.57 mg/day, respectively. Mean (±SEM) intake of linoleic plus linolenic acids was significantly lower (p ± 0.005) by CMF infants (3.4 ± 0.2 g) than by FF infants (9.9 ± 1.0 g), although mean (±SEM) dietary vitamin E to polyunsaturated fat ratio (E/PUFA ratio) was the same in both FF and CMF infants (1.3 ± 0.1). Plasma a‐tocopherol concentration (mean ± SD) was significantly lower (p ± 0.005) in CMF than in FF infants (0.86 ± 0.28 mg/dl vs. 1.14 ± 0.42 mg/dl, respectively). Dietary vitamin E intakes were positively correlated (p ± 0.05) with plasma a‐tocopherol concentrations. No correlations were found between plasma a‐tocopherol concentrations and total fat intake, dietary E/PUFA ratios, erythrocyte polyunsaturated fatty acids s≥C18:2, or number of hours postprandial that blood was drawn. The difference in the mean plasma a‐tocopherol concentration (0.28 mg/dl) between CMF and FF infants may be of importance to infants who have a high intake of polyunsaturated fatty acids.
Parasitic Infection of the Gut and Protein‐Losing Enteropathy
Authors: Sullivan Peter B. , Lunn Peter G. , Northrop‐Clewes Christine A. , Farthing Michael J. G.
Published: January 2024

Parasitic infection of the gut is highly prevalent in developing countries and contributes to the severe malnutrition associated with persistent diarrhoea in children. Using random faecal α1‐antitrypsin measurements as an index of protein‐losing enteropathy, we examined children with and without parasitic infection of the gut. Seventeen percent of children with persistent diarrhoea had evidence of protein‐losing enteropathy. This was found to be associated with hypoalbuminaemia in children infected with Strongyloides stercoralis. Only a minority (2 of 17) of children infected with Giardia lamblia had raised faecal α1‐antitrypsin excretion and this was not associated with hypoalbuminaemia. This study emphasises the need for rapid diagnosis and treatment of Strongyloides infection in children with persistent diarrhoea and severe nutrition.
Esophageal Stricture in Children
Authors: Dalzell A. M. , Shepherd R. W. , Cleghorn G. J. , Patrick M. K.
Published: January 2024

We made a retrospective analysis of the efficacy and complication rate of 268 esophageal dilatation procedures performed under fluoroscopic control using the fiber‐optic endoscope in 45 children with esophageal stricture. Antegrade and retrograde stricture dilatation was performed under general anesthetic, mainly as an outpatient procedure. Thirty‐six children had an esophageal stricture following tracheoesophageal fistula and/or esophageal atresia repair, and nine children had severe corrosive stricture of the esophagus following lye ingestion. The procedure was well tolerated and effective.
Rotavirus Induces a‐Interferon Release in Children with Gastroenteritis
Authors: De Boissieu D. , Lebon P. , Badoual J. , Bompard Y. , Dupont C.
Published: January 2024

α‐Interferon (IFN) blood levels were studied in 38 children hospitalized for rotavirus gastroenteritis. During rotavirus infection, a peak of systemic α‐IFN was observed within 2 days of onset of symptoms. There was a significant positive correlation between α‐IFN levels and the number of vomiting episodes (p = 0.0003) but not with duration of vomiting, maximal temperature, and duration of fever. α‐IFN levels were higher in children with peripheral cyanosis compared with children without peripheral cyanosis (p = 0.005) and tended to be higher in children with diarrhea of <3 days’ duration compared with those with diarrhea lasting >4 days (p = 0.06). α‐IFN could be partly responsible for the severity of such initial symptoms as vomiting and peripheral cyanosis in rotavirus gastroenteritis and could play a role in recovery from diarrhea.
Association Between HLA and Extrahepatic Biliary Atresia
Authors: Silveira Themis R. , Salzano Francisco M. , Donaldson Peter T. , Mieli‐Vergani Georgina , Howard Edward R. , Mowat Alex P.
Published: January 2024

The etiopathogenesis of extrahepatic biliary atresia (EHBA) remains undefined. There are clinical and pathological suggestions supporting the idea that EHBA could consist of at least two forms: the congenital (embryonic or fetal) and the acquired (perinatal) types. To test the hypothesis that susceptibility to this disease would be influenced by host genetic factors, we studied the human leukocyte antigen (HLA) system in 55 patients with and without major extrahepatic congenital anomalies. We found, especially in those without associated malformations, a significantly higher frequency of HLA‐B12, of haplotypes A9‐B5 and A28‐B35, and of their disequilibrium values, as compared with the 8th International Histocompatibility Workshop controls. This study suggests that immunogenetic factors may play a role in determining susceptibility to EHBA, and the different HLA frequencies in those with and without anomalies lend support to the hypothesis that biliary atresia may be an etiologically heterogeneous disorder.
A Morphological Study of β‐Lactoglobulin Absorption by Cultured Explants of the Human Duodenal Mucosa Using Immunocytochemical and Cytochemical Techniques
Authors: Wheeler Ennis E. , Challacombe David N. , Kerry Philip J. , Pearson Edwin C.
Published: January 2024

The absorption of (3‐lactoglobulin (BLG) by cultured explants of the normal adult duodenal mucosa has been studied in vitro using immunocytochemical techniques. Membrane filters (1 mm2) were soaked in medium containing BLG and placed on the mucosal surface. Explants were cultured for ≤60 min, and histological sections were immunostained for BLG. Increasing the BLG concentration in the filters and/or the exposure time increased the number and immunoreactivity of the immu‐nopositive epithelial cells present, suggesting increased BLG absorption. Increased BLG penetration of the duodenal mucosa in untreated coeliac disease was also shown using these techniques. In some studies horseradish peroxidase (HRP) was used as an alternative probe and pinpointed in epithelial cells by ultracytochemical techniques, confirming vesicular uptake of macromolecules. Colocalisation experiments using BLG and HRP and im‐munofluorocytochemical techniques suggested linkage between the mechanisms of absorption of both proteins into epithelial cells. Short‐term organ culture and protein challenge followed by immunolocalisation has been shown to be of value in studying the absorption of BLG and HRP by the upper small intestine and possibly of other protein macromolecules as well.
Distribution of Neuromuscular Junctions in the Bowel Affected by Hypoganglionosis
Authors: Yamataka A. , Miyano T. , Urao M. , Okazaki T.
Published: January 2024

Using monoclonal antibody 171B5 against synaptic vesicle proteins, neuromuscular junctions and synapses in the muscle layers of the bowel affected by hypoganglionosis were labeled and their distribution analysed. In the hypoganglionic bowel, there were less synapses in the myenteric plexus and few neuromuscular junctions compared to normal bowels. The bowel dysmotility of the patient with hypoganglionosis appears to be due to inadequate innervation between ganglion cells and smooth muscle cells.
Anderson's Disease
Authors: Strich David , Goldstein Robert , Phillips Alan , Shemer Ruth , Goldberg Yael , Razin Aharon , Freier Serem
Published: January 2024

We describe three patients with Anderson's disease who are members of one family; the father and mother are close relatives and three of seven children show symptoms of the disease. All patients suffered from diarrhea, failure to thrive, and recurrent infections during infancy. Although these symptoms disappeared later in life, biochemical disorders (such as low plasma levels of apolipoproteins A1 and B and cholesterol, resulting in avitaminosis E, plus failure to secrete chylomicrons after a fat meal) persisted. Electron microscopy of enterocytes of one of the patients showed accumulation of lipid vacuoles with no significant aberration of the Golgi apparatus itself. It is possible, therefore, that the disease reflects a defect in chylomicron assembly. We found that low levels of apolipoprotein (apo) B48 were present in the patients' plasma. This suggests that the processing of the B100 message resulting in apo B48 functions normally. The possibility that a mutation in the apo B gene results in an abnormal apo B48 protein is very unlikely since a variable number tandem repeat (VNTR) polymorphism probe mapped to chromosome 2 failed to show correspondence of the parent alleles with the disease. These observations confirm the suggestion that Anderson's disease is not linked to the apo B locus.
Upper Gastrointestinal Endoscopy in Recurrent Abdominal Pain of Childhood
Authors: Ashorn Merja , Mäki Markku , Ruuska Tarja , Karikoski‐Leo Riitta , Hällström Minna , Kokki Maarit , Miettinen Ari , Visakorpi Jarmo K.
Published: January 2024

Over a 2.5‐year period, 82 consecutive children complaining of recurrent abdominal pain underwent upper gastrointestinal endoscopy. Gastroscopy confirmed pathology in 48 of the children (58.5%). Four of the children, who also had undergone gastroscopy, had other diagnoses (lactose malabsorption, hydronephrosis, yersinosis), and 30 of the children (36.6%) retained the initial diagnosis of recurrent abdominal pain syndrome. Gastritis was found in 48 of the children, 18 of whom (37.5%) had positive test results for Helicobacter pylori, based on histology and/or culture. Of 16 H. pylori‐positive children tested, 12 (75%) also had an elevated concentration of IgG‐class antibodies to H. pylori in their sera. Three of the children had duodenal ulcer disease, all of whom were H. pylori positive. Esophagitis was found in eight of the children with gastritis, all of whom were found to have gastroesophageal reflux. Our data suggest that among the children with recurrent abdominal pain syndrome, organic pathology is more common than was previously thought. Altogether 22% of the children with recurrent abdominal pain syndrome were infected with H. pylori.
Bicarbonate and Citrate in Oral Rehydration Therapy
Authors: Elliott Elizabeth J. , Watson Alistair J. M. , Walker‐Smith John A. , Farthing Michael J. G.
Published: January 2024

In situ perfusion of the rat jejunum and ileum was used to study the effect of inclusion of bicarbonate or citrate on the ability of four oral rehydration solutions to promote small‐intestinal absorption of water and sodium. Solutions varied in their sodium (60–90m M) and glucose (111–140m M) content and osmolality (281–331 mosmol/kg). They were studied before and after exclusion of base both in normal intestine and in secreting intestine after exposure to cholera toxin. All solutions promoted net water absorption in the normal intestine and reversed net water secretion to absorption in the cholera toxin‐treated intestine to varying degrees. Net sodium movement was directly related to the sodium content of oral rehydration solutions. Inclusion of bicarbonate or citrate did not promote significantly greater absorption of water or sodium than did solutions without base, in normal or secreting intestine. In the secreting intestine, inclusion of bicarbonate in two solutions actually resulted in greater sodium secretion than did identical solutions from which bicarbonate was omitted. These studies suggest that the inclusion of base or base precursors in oral rehydration solutions to enhance water and sodium absorption is unjustified in both normal and secreting small intestine.
Induction of Exocrine Pancreas Maturation at Weaning in Young Developing Pigs
Authors: Pierzynowski S. G. , Weström B. R. , Erlanson‐Albertsson C. , Ahre'n B. , Svendsen J. , Karlsson B. W.
Published: January 2024

The influence of weaning, at either 4 or 6 weeks of age, on the maturation of the exocrine pancreas was studied in naturally reared Swedish Landrace pigs that had no access to solid food. The pigs were surgically fitted with chronic catheters at 3 weeks of age, permitting periodic sampling of pancreatic juice and blood in conscious animals ≤4 weeks after weaning. During the suckling period, pancreatic fluid and enzyme secretion remained low, both before (preprandial) and after (postprandial) milk ingestion. After weaning at 4 or 6 weeks of age, juice secretion, output of total protein, and levels of different hydrolases (amylase, trypsin, lipase, and carboxylester lipase) and the cofactor colipase all increased markedly postprandially. Moreover, after weaning, the plasma insulin level increased postprandially. This did not happen before weaning, although blood glucose levels always rose after feeding. The data showed a relationship between the time of weaning and the induction of exocrine pancreatic maturation in pigs. This finding implies that postnatal development of pancreatic function is triggered by the dietary change from sow milk to dry solid food. In contrast, the age of the pig appears to be of minor importance, since weaning at either 4 or 6 weeks of age gave a similar result.
Continuation of Breast‐Feeding in an Israeli Population
Authors: Birenbaum Emanuel , Vila Yael , Linder Nehama , Reichman Brian
Published: January 2024

Demographic and perinatal factors were analyzed in an Israeli urban community to determine the rates of continuation of breast‐feeding and the factors influencing these rates. For the purpose of this study, breast‐feeding was defined as occurring if infants received at least one breast‐feed during a 24‐h period. Among 633 mothers initiating breast‐feeding, 40.3% completed 3 months of breast‐feeding. The factors significantly associated with the continuation of breast‐feeding were maternal religious belief, high education level, high parity, and previous breast‐feeding success. Multivariate analysis by stepwise logistic regression showed that orthodox religious belief was the most significant associated factor. A third interview undertaken after 12 months among the 165 mothers who successfully completed 3 months of breast feeding showed declines of breastfeeding at the second (31.5%), third (53.1%), and fourth (60.4%) trimesters. Only 12.7% of this population completed 12 months of breast‐feeding. Again, the mothers' religious beliefs and education levels were significantly related to the continuation of breast‐feeding. Each specific community must be analyzed to find those groups of mothers at risk for early weaning from breast‐feeding.
Human Leukocyte Antigen Alleles and Haplotypes Associated with Selective Immunoglobulin A Deficiency in Spanish Pediatric Patients
Authors: Clerici Nora , Fernández Miguel , Saiz Isabel , Sainz Teofilo , Polanco Isabel
Published: January 2024

Class I and II human leukocyte antigens were determined by a standard microlymphocytotoxity test in a group of 45 pediatric patients with selective immuno‐globulin A deficiency (IgA‐D), 33 of them with frequent respiratory tract infections, allergic diseases, or gastrointestinal disorders (RTIAG), and 12 with celiac disease (CD). The results showed that the DR1 allele, and the A1,B8,Cw7,DR3,DQw2; B35,Cw4,DR1,DQw1; and B14,DR1,DQw1 haplotypes could be involved with IgA‐D susceptibility in RTIAG patients. Among the CD‐IgA‐D group, the B14 allele and A1,B8,Cw7,DR3,DQw2 haplotype were found to confer a high risk of developing IgA‐D. A possible protective role may be postulated for DR2 and DR4 in both types of IgA‐D patients. The present study confirms some of the previous findings in other white populations and describes new possible alleles and haplotypes that could be implicated with IgA‐D susceptibility and resistance.
The Effect of Colitis on Large‐Intestinal Electrolyte Transport in Early Childhood
Authors: Jenkins H. R. , Milla P. J.
Published: January 2024

The large intestine plays an important role in the salvage of electrolytes and water, although little is known of its function in early childhood in health or in disease. Using an in vitro voltage clamp technique, we have measured bidirectional sodium (Na) and chloride (C***l) fluxes (J), electrical potential difference (PD), short circuit current (Isc), and tissue resistance (R) in 10 pairs of healthy human infant left colon and four pairs of inflamed colon. In healthy tissue there was net Na and C***l absorption with JNa net approximating Isc. In contrast, inflamed colon exhibited significant reductions in PD, Isc, and R and increases in net serosa to mucosa flux of Na and Cl (JNasm and JClsm) resulting in net Na and Cl secretion. These results suggest that Na and Cl absorption in left colon is highly efficient in young children and that mucosal inflammation may seriously impair the colon's ability to conserve salt and water.
Saccharomyces boulardii for Clostridium difficile‐Associated Enteropathies in Infants
Authors: Buts Jean‐Paul , Corthier Gérard , Delmee Michel
Published: January 2024

Based on experimental evidence in animals showing that the oral administration of Saccharomyces boulardii is effective in reducing morbidity and mortality due to Clostridium difficile‐induced pseudomembranous colitis, we conducted an open trial to examine the effects of the living yeast, given as primary therapy, in a selected group of infants and children with persistent intestinal symptoms related to toxinogenic C. difficile overgrowth. Over a period of 10 consecutive months, we studied 19 eligible patients (median age 8 months) who presented with enteral symptoms lasting for > 15 days and who had solely C. difficile in stools with positive cytotoxin B assay. Serotyping of the strains and determination in vitro of production of toxins A and B were performed subsequently. The patients presented with persistent or protracted diarrhea, malabsorption, and failure to grow (n = 8), or with repeated attacks of colics, emesis, and hypermeteorism without diarrhea (n = 4), or with both entities (n = 7). Patients with chronic protracted diarrhea (n = 3) had‐depressed jejunal disaecharidase activities and ultrastructural changes of enterocytes, including sparce and shortened microvilli. None had evidence of colitis. All the strains of C. difficile tested (n = 17) belonged to pathogenic serotypes (A1, A8, C, F, G, H, and K) and produced in vitro high levels of toxins A (n = 16) and B (n = 17). S. boulardii was given orally in a lyophilized form over 15 days (250 mg two to four times per day according to age). Within 1 week of treatment, enteral symptoms and physical findings resolved in 18 patients (95%) with marked decreases (p < 0.001) in the number of stools, frequency of colic episodes, and total duration of colics per day. Clearing of toxin B was observed within 15 days of therapy in 16 cases (85%), whereas eradication of C. difficile from stools was complete after 1 month in 14 (73%). Also, the ultrastructural changes observed in patients with chronic protracted diarrhea (n = 3) had disappeared after 1 month. A clinical and bacteriological relapse occurred in two patients (11%), which resolved rapidly with a second 15‐day course of S. boulardii. These findings suggest that some toxinogenic strains of C. difficile may cause chronic enteropathies without colitis that may be improved by oral administration of S. boulardii.
Resting Energy Expenditure and Food‐Induced Thermogenesis in Obese Children
Authors: Tounian P. , Girardet J. Ph. , Carlier L. , Frelut M. L. , Veinberg F. , Fontaine J. L.
Published: January 2024

Nineteen overweight girls 14.54 ± 0.38 years of age were studied. Results were compared with those obtained in eight age‐matched (14.07 ± 0.51 years) and sex‐matched normal weight controls. Energy expenditure (EE) was determined using open‐circuit indirect calorimetry at rest, both after a 12‐h fast and after an oral sucrose load of 3 g/kg ideal body weight. Food‐induced thermogenesis (FIT) was evaluated by computing the area under the curve of the EE response above resting energy expenditure (REE) during the first 3 h after the sucrose load, REE (kcal/day) was higher in the overweight patients (1,818 ± 46 vs. 1,527 ± 67; p = 0.002); REE standardized by fat‐free mass (FFM) (kcal/kg FFM/day) was lower in obese children than in controls (35.2 ± 1.0 vs. 44.9 ± 1.9; p = 0.0001). A linear correlation between REE and FFM was evidenced in both controls and overweight subjects (r = 0.78 and 0.68, respectively; p = 0.05 and p < 0.001, respectively). Actual REE in the obese children was significantly lower than the value predicted by applying the regression equation of REE on FFM in controls to the actual FFM in obese children (paired t test; p = 0.003). FIT was identical in overweight and normal weight subjects, regardless of whether it was expressed in absolute value, as the percentage calorie intake, or standardized by FFM. However, obese children were assigned to two groups according to whether FIT was lower than the value in controls (group 1, <mean −1 SD; n = 9) or at least equal to the value in controls (group 2, mean ‐ 1 SD, n = 10); the only feature that discriminated between these two groups was the presence or absence of a family history for obesity (FHO): FIT was significantly higher in obese children with a negative FHO than in obese children with a positive FHO. Our results support the presence of an energy metabolism disorder in some obese children and suggest that its cause may be constitutional in obese children with a positive FHO.
The Nutritional Value of a Whey Hydrolysate Formula Compared with a Whey‐Predominant Formula in Healthy Infants
Authors: Vandenplas Yvan , Hauser Bruno , Blecker Uwe , Suys Bert , Peeters Stefaan , Keymolen Kathelijn , Loeb Helmuth
Published: January 2024

Forty‐five healthy infants were included in a double‐blind randomized prospective study comparing the nutritional value of two formulas. One group received a whey‐predominant formula (n = 20); the other group received a whey hydrolysate formula (n = 25). Four infants of the whey hydrolysate group were dropped because they refused the formula. Although the mean daily volume intake was smaller with the whey hydrolysate formula compared with the whey‐predominant formula (p < 0.001), the weight gain in the two groups after 13 weeks was identical (27.2 g/day in both groups; the mean difference in weight gain between the groups after 13 weeks was only 8 g). Length gain at 13 weeks was 10.4 cm in the whey‐predominant formula group and 10.8 cm in the whey hydrolysate formula group (p = NS). After 13 weeks, blood was sampled for hemoglobin, hematocrit, red blood cell count, white blood cell count, lymphocytes, glycemia, proteins, albumin, prealbumin, calcium, phosphorus, urea, creatinine, iron, iron‐binding capacity, zinc, and vitamins A and E. Except for the iron‐binding capacity, zinc, urea (in plasma as well as in urine) (all three were higher in the whey hydrolysate group), no significant differences were found. According to these results, exclusive feeding of the whey hydrolysate formula from birth to 3 months of age to healthy infants appears to result in an adequate nutritional status, as assessed at 3 months of age.
Gastrointestinal Tolerance, Fat Absorption, Plasma Ketone and Urinary Dicarboxylic Acid Levels in Low‐Birth‐Weight Infants Fed Different Amounts of Medium‐Chain Triglycerides in Formula
Authors: Wu Paul Y. K. , Edmond John , Morrow Jack W. , Auestad Nancy , Ponder Debra , Benson John
Published: January 2024

This study was conducted to evaluate the effect of medium‐chain triglycerides (MCT) in a formula for low‐birth‐weight (LBW) infants on gastrointestinal tolerance, fat absorption, plasma ketone levels, and urinary dicarboxylic acid (DCA) excretion. At the start of enteral feedings, 64 LBW infants (≤1500 g) were randomly assigned to one of four experimental formulas. The formulas contained either 0, 17, 34, or 50% of the total fat as MCT oil. The nonfat constituents of all four formulas were the same and identical to Similac Special Care 24 (SCF), Infants were studied from the start of enteral feeding until approximately 7 days after reaching full feeds. Growth and tolerance were assessed in all infants over the entire feeding period. A 48‐h balance study was conducted after enteral intake exceeded 100 kcal/kg/day for 3 days. Stool fat, plasma D‐(—)‐3‐hydroxybutyrate (3HB) and carnitine, serum glucose, and urinary DCA levels were determined. Groups did not differ in growth, formula intake, fat absorption (76–84%), serum glucose, or plasma carnitine levels. Gastrointestinal tolerance was excellent and did not differ among groups. Plasma 3HB was significantly different (p < 0.05) only between the 0 and 50% MCT groups, 50 ± 10 versus 120 ± 20 μM, respectively. The excretion of urinary DCAs increased with increasing amounts of MCT in the formula. In conclusion, fat absorption and gastrointestinal tolerance were not affected by different MCT levels (0 to 50% of the total fat), but higher levels of plasma 3HB and urinary DCAs were associated with higher levels of MCT in the LBW formulas studied.
Newborn Rabbit Gastric Smooth Muscle Cell Culture
Authors: Yuan Qi X. , McRoberts James A. , Lakshmanan J. , Yagi Hideki , Hyman Paul E.
Published: January 2024

Epidermal growth factor (EGF) and transforming growth factor‐alpha (TGF‐α) are two in a family of growth‐promoting peptides for many gastrointestinal epithelia. This study was designed to assess their mitogenic effect on cultured gastric myocytes and to characterize specific EGF receptors on these cells. Single myocytes were isolated from newborn rabbit gastric fundus and placed into tissue culture. The composition of the culture at confluence as assessed by immunostaining with smooth muscle actin‐specific monoclonal antibody (CGA7) was >95% myocytes. To assess the effect of putative growth factors, freshly isolated myocytes were incubated in Dulbecco's modified Eagle's (DME) medium containing 1% fetal bovine serum in the presence or absence of growth factors. After 6 days, cells were incubated in serum‐free medium with [3H]thymidine (1 μCi/ml) in the continued presence or absence of growth factors. After 24 h, EGF and TGF‐α but not insulin‐like growth factor I (IGF‐1) induced a dose‐dependent increase in [3H]thymidine incorporation. Ten nanomolar EGF or TGF‐α increased [3H]thymidine incorporation more than sixfold over control. EGF was more potent than was TGF‐α, with apparent median effective dose (ED50) values of 64 ± 14 pM and 166 ± 62 pM (p < 0.05), respectively. EGF bound to cultured myocytes with Kd = 7.6 ± 1.8 nM and Bmax = 27 ± 11 pmol/mg DNA or 440,000 receptors/cell. TGF‐α competed for binding at these receptors. Although IGF‐I did not stimulate thymidine incorporation, specific high‐affinity receptors for IGF‐I were detected on gastric myocytes. These results suggest that the EGF family may play a role in the growth of gastric smooth muscle.
Influence of Prenatal Corticosteroids on Bacterial Colonization in the Newborn Rat
Authors: Schiffrin E. J. , Carter E. A. , Walker W. A. , Frieberg E. , Benjamin J. , Israel E. J.
Published: January 2024

The interactions between bacteria and the host's intestinal barrier appear to be important regulators of bacterial colonization. In this study we investigated the effect of prenatal corticosteroids, known to accelerate the intestinal maturation of newborn rats, on bacterial colonization in the rat pup. Pregnant rats were treated with either cortisone acetate or normal saline on days 18–21 of gestation and were allowed to deliver spontaneously. The pups, after normal delivery, were sacrificed at different times during the first 10 days of life. The entire small intestine was removed, and each lumen was flushed to exclude nonadherent, transient organisms and homogenized. Tenfold dilutions were plated on horse‐blood agar (total bacteria) and MacConkey's medium (gram‐negatives). Quantitation and bacterial typification was determined after 24 h of incubation at 37°C. Total bacteria and gram‐negatives found in association with the mucosa were significantly lower in pups prenatally treated with steroids. These changes were not related to any changes in motility or intraluminal digestion. This suggests that the developmental condition of the host's intestinal barrier may be an important regulator of the bacterial micro‐environment of the newborn small intestinal mucosa
Ultrastructural Changes in the Duodenal Mucosa of HIV‐infected Children
Authors: Fontana M. , Boldorini R. , Zuin G. , Tosoni A. , Costanzi G. , Principi N.
Published: January 2024

We studied the ultrastructure of duodenal biopsy specimens from six HIV‐infected children with chronic gastrointestinal symptoms. A monomorphic pattern of microvillar damage (short and irregular microvilli, joined at their bases) was seen in all cases, even when the mucosa was normal on conventional histology. Among nine HIV antibody negative children, a similar pattern was seen only in three out of four celiac children with severely atrophic mucosa. No viral, bacterial, or protozoan pathogen was found. In HIV‐infected children tu‐buloreticular inclusions were also seen in endothelial cells. These ultrastructural changes could help to account for the gastrointestinal symptoms in HIV‐infected children.
Treatment of Cyclic Vomiting in Childhood with Erythromycin
Authors: Vanderhoof Jon A. , Young Rosemary , Kaufman Stuart S. , Ernst Laura
Published: January 2024

Cyclic vomiting syndrome is an unusual cause of episodic emesis in children. It manifests as intermittent episodes of severe vomiting, similar in time of onset and duration, with no symptoms during the intervening period. Dehydration necessitating intravenous fluid therapy may occur. Most therapeutic maneuvers have proven unsuccessful. We report the use of erythromycin as a prokinetic agent in the treatment of cyclic vomiting in 20 children (9 boys, 11 girls). Many patients had mild associated abdominal pain with their vomiting. Thirteen patients had previously been given metoclopramide, but none responded. Two patients were mildly developmentally delayed. Twenty patients were given oral erythromycin ethylsuccinate, approximately 20 mg/kg/day, in 2–4 divided doses for 7 days. This dosage was repeated as needed when symptoms reappeared. Thirteen of 20 patients reported total resolution of symptoms when reevaluated at 2 and 6 months. All males responded, 4 of 13 responders were female, and all seven with partial or no response to therapy were female. This uncontrolled trial suggests that erythromycin may be a useful prokinetic agent in the treatment of cyclic vomiting syndrome in childhood. As the study was uncontrolled, placebo effect cannot be excluded. Case‐controlled, double‐blinded prospective trials should be considered to evaluate the effectiveness of erythromycin in cyclic vomiting syndrome.
Contribution of Endoscopy to Early Diagnosis of Hypertrophic Pyloric Stenosis
Authors: De Backer A. , Bové T. , Vandenplas Y. , Peeters S. , Deconinck P.
Published: January 2024

The diagnostic accuracy of ultrasonography and gastrointestinal endoscopy was compared in 63 infants who were operated on for infantile hypertrophic pyloric stenosis. Endoscopy was far more accurate than ultrasonography, the diagnosis being made in 97 and 81% of the cases, respectively. The difference between the techniques was even more obvious in the younger patients and in those with a short history of vomiting. The ability to detect coexistent or other causes of vomiting with endoscopy appeared advantageous. The endoscopic procedure is easily done without general anesthesia and was without complications in our series. We recommend endoscopy as an important tool in very young patients with few clinical signs other than vomiting, allowing for appropriate treatment without delay.
Histologic Pathology of the Liver in Progressive Familial Intrahepatic Cholestasis
Authors: Alonso Estella M. , Snover Dale C. , Montag Anthony , Freese Deborah K. , Whitington Peter F.
Published: January 2024

SummaryThis work details the histologic findings in 84 liver biopsy specimens from 28 patients with progressive familial intrahepatic cholestasis (PFIC), who met the clinical criteria of early onset of chronic unremitting cholestasis, exclusion of any known metabolic or anatomic etiology, and low serum γ‐glutamyl transpeptidase (GGTP) values. Hepato‐canalicular cholestasis and disruption of the liver cell plate arrangement were early, uniform findings, and giant cell transformation was found in 56% of initial biopsies. Duct loss was a prominent finding; 70% of patients had ductal paucity, and many had abnormal bile duct epithelium, suggesting degeneration. Fibrosis was seen in the samples from 16 patients, including bridging fibrosis in specimens obtained from six patients during the first 2 years of life. Proliferating ductules at the margins of portal tracts increased as fibrosis progressed and were especially prominent in end‐stage histology. Cirrhosis developed in nine of these patients and had a characteristic histologic pattern, consisting of biliary cirrhosis with diffuse stellate lobular fibrosis associated with severe cholestasis and pseudoacinar transformation. Mallory hyalin and hepatocellular carcinoma were observed in materials from some patients with advanced cirrhosis. The constellation of histologic findings in PFIC forms a recognizable pattern, and the liver histology appears to have a predictable progression.
Abnormally Shaped Arteries in the Intestine of Children with Hirschsprung's Disease
Authors: Taguchi Tomoaki , Suita Sachiyo , Hirata Yuzo , Hirose Ryuichiro , Yamada Takaharu , Toyohara Toshimitsu
Published: January 2024

SummaryAbnormally shaped arteries were found in the intestines of 17 of 62 patients (27%) with Hirschsprung's disease. The histological characteristics of abnormal arteries included proliferation of collagen fibers and smooth muscle cells in the adventitia. The incidence of abnormal arteries in each type of aganglionosis was as follows: 25% (13 of 51) in short‐segment aganglionosis; 0% (0 of 6) in long‐segment aganglionosis; and 80% (4 of 5) in totalcolon or extensive aganglionosis. The older the patients were at the time of resection, the higher the incidence of abnormal arteries (<1 year old, 15%; 1–3 years old, 38%; >3 years old, 75%). The abnormally shaped arteries were mostly located in the histological transitional zone. These findings suggest the following possibilities: (a) the craniocaudal migration of ganglion cells was interrupted by intestinal ischemia in the presence of abnormal arteries in utero; (b) an ischemic episode caused both the disappearance of neural cells and dysplasia of the artery; (c) the ganglion cells were destroyed by mild ischemia caused by the abnormal arteries; or (d) the tension caused by mechanical expansion resulted in a change in the vascular walls in the transitional zone.
Resting Energy Expenditure in Infants with Cystic Fibrosis
Authors: Girardet J. P. , Tounian P. , Sardet A. , Veinberg F. , Grimfeld A. , Tournier G. , Fontaine J. L.
Published: January 2024

SummaryTo evaluate the contribution of energy expenditure to the energy imbalance seen in cystic fibrosis patients, resting energy expenditure was measured using open‐circuit indirect calorimetry in eight infants with cystic fibrosis, aged 2–7 months (mean, 4), without overt lung disease and in 10 healthy age‐matched controls. In both groups, we found close, significant, linear correlations between resting energy expenditure and body weight and between resting energy expenditure and fat‐free mass as measured by anthropometry. Cystic fibrosis patients had a 26% increase in resting energy expenditure per kilogram of fat‐free mass as compared with controls and a 32% increase in resting energy expenditure as compared with predicted values for fat‐free mass. These data from young infants free of clinical symptoms suggest a constitutional metabolic disorder in cystic fibrosis and support the need for early nutritional therapy in cystic fibrosis patients.