bluuebunny/crossref_metadata_2025_split huggingface dataset:
Microvilli‐ and Desmosome‐Associated Bodies in Crohn's Disease and Other Disorders in Childhood
Authors: Lewis D. , Walker‐Smith J. A. , Phillips A. D. 
Published: February 2024

SummaryUltrastructural studies of the small and large intestine from children revealed five cases showing abnormal small electron‐dense bodies within enterocytes and goblet cells. The characteristic features of the bodies were: (a) they occurred within microvilli and within the apical part of the cell cytoplasm; (b) they showed an association with the most apically located desmosomes of the epithelial cells; (c) in the microvilli they were present within protrusions of the membrane, suggesting that they may bud out of the cell; and (d) they were not membrane‐bound within the cell but were found outside the cell as luminal, membrane‐bound particles. These bodies occurred in two children with Crohn's disease and in three children having other gastrointestinal disorders. Similar electron‐dense bodies which did not have all of the characteristics listed above were found in a sixth child who had Crohn's disease. These electron‐dense bodies do not resemble any known inclusion of intestinal epithelium, nor do they resemble any previously described microorganism, although their apparent release from the cell by a process of budding is suggestive of a microbial identity. The occurrence of these bodies in childhood Crohn's disease suggests that they may be disease‐related, but their precise nature and relevance to this or other gastrointestinal disorders remain unknown.
Effect of Exogenous Corticosteroid Therapy on Growth in Children with HBSAg‐Negative Chronic Aggressive Hepatitis
Authors: Clark Joseph H. , Fitzgerald Joseph F. 
Published: February 2024

SummaryThe effect of exogenous corticosteroid therapy on growth was studied in 12 patients with HBS Agnegative chronic aggressive hepatitis. All patients were less than 13 years of age at diagnosis and were free of cirrhosis. The patients' growth records were retrospectively reviewed for incremental growth changes while they were receiving daily prednisone and alternate‐day prednisone, as well as for 1 year after the cessation of therapy. These values were compared with age‐matched growth velocities in normal children. Daily steroid therapy was associated with a reduction in growth velocity (p < 0.01). There was an acceleration in growth velocity during the period of alternate‐day therapy, compared with predicted values (p < 0.03), which persisted during the 1st year after therapy was discontinued (p < 0.02). Nine of 12 patients crossed growth percentiles during steroid therapy. We have confirmed that daily steroid administration is associated with a reduction in linear growth, while alternate‐day therapy has an apparent positive effect on growth, presumably through control of the underlying disease. The mechanism of this supranormal growth rate is unclear.
Intestinal and Pulmonary Cryptosporidiosis in an Infant with Severe Combined Immune Deficiency
Authors: Kocoshis Samuel A. , Cibull Michael L. , Davis Thomas E. , Hinton Jeffrey T. , Seip Michael , Banwell John G. 
Published: February 2024

SummarySmall intestinal biopsy established the diagnosis of cryptosporidiosis in a 6‐month‐old male infant with severe combined immune deficiency. Scanning and transmission electron microscopy revealed the ultrastructural details of Cryptosporidium in several stages of development within the patient's small intestine. The organism was resistant to all antibiotics used, and the patient died in the 5th month of his illness, despite receiving parenteral alimentation and undergoing thymus trans‐plantation. His clinical course was similar to that of other patients with fatal immune deficiencies and cryptosporidiosis. Autopsy demonstrated organisms attacking the epithelium of the small intestine, pancreatic duct, and bronchioles. Multiple organ involvement in this patient negates the view that Cryptosporidium is site‐specific for a given host or is alimentary‐canal‐specific in humans.
Macromolecular Transport in Jejunal Mucosa of Children with Severe Malnutrition
Authors: Heyman Martine , Boudraa Ghazalia , Sarrut Suzanne , Giraud Maud , Evans Lynleigh , Touhami Mahmoud , Desjeux Jehan‐François 
Published: February 2024

SummarySmall intestinal permeability to horseradish peroxidase (HRP) was assessed in jejunal biopsies of malnourished children. Clinical examination, intestinal function (D‐xylose test), and the histological appearance of the mucosa were assessed in 14 children during the malnutrition phase and in eight children after 3 months of nutritional rehabilitation. Macromolecular permeability was evaluated by measuring transepithelial HRP fluxes from mucosa to serosa (J) using Ussing chambers. In‐tact HRP transport, i.e., nondegraded protein transport, was measured by enzyme assay, and tritiated HRP was concomitantly used to measure intact and degraded HRP fluxes (J). Electrical parameters (short‐circuit current, potential difference, and ionic conductance) were also recorded. Transepithelial HRP fluxes were significantly higher during malnutrition (J = 8.85 ± 1.44; J = 77.11 ± 10.66 pmol h−1 ± cm−2) than in the same children after 3 months of nutritional rehabilitation (J = 2.94 ± 1.10; J = 22.93 ± 8.15 pmol ± h−1 ± cm−2). The total ionic conductance, which is an index of paracellular permeability, did not change significantly. Calculated intratissue degradation of the protein did not appear to alter during malnutrition. These results point to a clinical situation in which malnutrition with diarrhea in children is associated with increased intestinal permeability to macromolecules by a mechanism not related to any paracellular leak of the protein, but rather to increased transcellular absorption.
Fatty Liver and Its Fibrous Changes Found in Simple Obesity of Children
Authors: Kinugasa Akihiko , Tsunamoto Kentaro , Furukawa Nobuaki , Sawada Tadashi , Kusunoki Tomoichi , Shimada Nobuo 
Published: February 2024

SummaryOf 299 obese children who visited our obesity clinics, 36 were found to have elevated levels of serum transaminases by routine laboratory examination. Liver biopsy was carried out in 11 children. Based on the criteria of Adler and Schaffner (1979), the biopsy specimens were studied histologically. As a result, fatty liver (Group I) was observed in three patients, fatty hepatitis (Group II) in two, fatty fibrosis (Group III) in five, and fatty cirrhosis (Group IV) in one. The duration of obesity, but not its degree, was considered to be related to the extent of fibrosis. Accordingly, we concluded that the fatty liver of simple obesity in children may progress to liver cirrhosis and that childhood obesity shoud be treated as early as possible.
Prognostic Factors Associated with Patient Survival During Nutritional Rehabilitation in Malnourished Children and Adolescents with Cystic Fibrosis
Authors: Levy L. , Durie P. , Pencharz P. , Corey M. 
Published: February 2024

SummaryNineteen children and adolescents with cystic fibrosis and malnutrition were given intensive nutritional support in an effort to reverse malnutrition. Standard techniques of enteral and parenteral feeding were used. As 10 of 19 patients died shortly after nutritional intervention began, we retrospectively analyzed patient data in order to discover whether or not any patient characteristics were associated with survival. Using a multivariate analysis, a linear discriminant function was derived employing average heart rate, the presence or absence of Pseudomonas cepacia in the sputum culture, PaCO2, and the patient's age at the time of intervention. This function correctly classified these 19 patients. A further 10 nonselected CF patients who also received nutritional support were similarly assessed using the function, and again, survival was accurately predicted. Therefore, this function can be used to predict the likelihood of patient survival during the provision of advanced nutritional support. It may be helpful in deciding whether or not advanced nutritional support is warranted in certain CF patients.
Safe Oral Rehydration of Hypertonic Dehydration
Authors: Blum D. , Brasseur D. , Kahn A. , Brachet E. 
Published: February 2024

Summary:Eighteen infants with severe hypernatremic dehydration secondary to acute gastroenteritis were rehydrated during the 1st day with an oral glucose electrolyte solution containing 60 mmol sodium/L at a mean rate of 120 ml/kg/24 h. These 18 children were safely treated with oral therapy alone. No convulsions were observed during treatment. The mean decrease in natremia was 0.32 mmol/L/h, which compared favorably with the mean fall in natremia of 26 other infants in similar initial conditions who were treated intravenously. The present study lends additional support to the opinion that a slow decrease in plasma sodium (<0.5 mmol/L/h) helps to avoid seizures during treatment. As no other untoward effects were observed, this study also confirms that oral solutions given at a slow rate can effectively replace intravenous fluids in the majority of such children.
Milk Versus No Milk in Rapid Refeeding After Acute Gastroenteritis
Authors: Isolauri E. , Vesikari T. , Saha P. , Viander M. 
Published: February 2024

Summary:Sixty‐five infants (mean age 14.7 months, range 6‐34 months), hospitalized for acute gastroenteritis, were treated with oral rehydration and rapid reintroduction of full feedings appropriate for age. Cow's milk and milk products were eliminated from the diet of 27 infants, whereas the remaining 38 children continued to receive their usual milk and milk products as parts of the mixed diet. There was no difference between the groups in the clinical recovery from diarrhea. No child had prolonged diarrhea. No new cases of clinical atopy were observed at 1‐month follow‐up, and there were no significant increases in the total or milk‐specific IgE levels. Serum IgG and IgA antibodies to β‐lactoglobulin and α‐casein were initially present in the majority of the children, but there were no appreciable changes in these cow's milk antibodies after gastroenteritis, regardless of the type of diet. It is concluded that cow's milk and milk products can be safely given in acute gastroenteritis as parts of the mixed diet for children over 6 months of age. Rapid reintroduction of feedings is beneficial for recovery from diarrhea, and there appears to be little need for dietary restrictions in this age group.
Functional Gastrointestinal Obstruction in a Child with Chronic Granulomatous Disease
Authors: Granot E. , Matoth I. , Korman S. H. , Ludomirsky A. , Lax E. 
Published: February 2024

Summary:A child with chronic granulomatous disease developed an antral‐pyloric obstruction, followed a month later by a postbulbar duodenal obstruction. At both areas, there was no evidence of an anatomical lesion, and some improvement in the passage of barium was observed following glucagon and metoclopramide administration. Presumably, symptoms have resulted from a functional disturbance of gastrointestinal motility.
Mesenchyme‐Dependent Differentiation of Epithelial Progenitor Cells in the Gut
Authors: Haffen K. , Kedinger M. , Simon‐Assmann P. 
Published: February 2024

SummaryThe digestive tract and the gut as a paradigm represents an attractive system for the study of mechanisms involved in the differentiation of two types of progenitor cells: the endodermal cells during embryonic life and the undifferentiated crypt cells during epithelial renewal of the adult intestine. The morphological and functional events that accompany the differentiation processes of progenitor cells into the polarized epithelial cell types characteristic of the intestine appear comparable in both situations (1,2). During organogenesis of the gut, histological observations underlined a close relationship between epithelial cells and their underlying mesenchymal cells (3,4). Developmental biologists have emphasized experimentally the importance of interactions between the endoderm and mesenchyme during organogenesis of the digestive tract. In the adult intestine, gastroenterologists have focused their attention on a specialized mesenchymal cell type (the pericryptal fibroblasts) that displays, like epithelial cells, proliferative activities and migrating properties. The aim of this review is to provide current knowledge on epithelial‐mesenchymal interactions during ontogenesis of the digestive tract and also to relate some experiments supporting the view of the perpetuation of epithelial‐mesenchymal interactions beyond embryonic life.
Long‐Term Home Parenteral Nutrition in Pediatrics
Authors: Vargas Jorge H. , Ament Marvin E. , Berquist William E. 
Published: February 2024

SummaryOne hundred two pediatric patients received all or part of their nutritional needs parenterally at home during the past decade. All received total parenteral nutrition (TPN) at night during an 8‐to 12‐h infusion. Patients with short bowel syndrome (33%), inflammatory bowel disease (23%), chronic intractable diarrhea (15%), chronic idiopathic intestinal pseudo‐obstruction syndrome (10%), and malignancy (10%) made up the largest groups. The mean duration of parenteral support was 735 days (range, 90–3650 days); the mean number of catheters per patient was 2.1 (range, 1–8). Twenty‐one patients continue to receive full or partial home TPN: four for more than 10 years and seven for more than 5 years. Fifty‐one no longer require it and have had healing of mucosa or bowel adaptation. Complications related to administration of fluid and electrolytes were quite rare. Biotin deficiency was recognized once. Thirty‐one have died, but only 13 deaths were related to TPN. Sepsis in nine and liver failure in two were the most common causes of death in the TPN‐related group. Three of 21 still on home TPN have graduated either from high school or college. All but one of the school age children attend regular school; one attends a school for the medically disabled, another attends a school for the mentally gifted.
In Vivo Intestinal Uptake of Immunoreactive Bovine Albumin in Piglet Enteritis
Authors: Keljo David J. , Bloch Kurt J. , Bloch Margot , Arighi Mimi , Hamilton J. R. 
Published: February 2024

SummaryWe examined the uptake of bovine serum albumin (BSA) from the intestine into the circulation of 3‐week‐old piglets infected with transmissible gastroenteritis virus. Transfer of immunoreactive bovine serum albumin (iBSA) from the intestinal lumen into the circulation was enhanced during both the early invasive phase of this viral enteritis (12‐h postinoculation) and the diarrheal phase (84‐h postinoculation). In some animals, enhanced uptake persisted into the recovery phase, 324 h after inoculation. Gel filtration studies suggested that iBSA had the molecular size characteristic of native BSA; no immunoreactive fragments of BSA were detected. Based on studies of two animal she half‐life of iBSA approximated that of porcine albumin. Further study is required to determine the immunological consequences of the enhanced uptake of protein occurring during viral infection of the intestine.
Congenital Diarrhea with Intestinal Inflammation and Epithelial Immaturity
Authors: Kanof Marjorie E. , Ranee Naomi E. , Hamilton Stanley R. , Luk Gordon D. , Lake Alan M. 
Published: February 2024

SummaryWe report an interesting case of congenital inflammatory bowel disease and intestinal epithelial immaturity that presented as secretory diarrhea. No infectious, metabolic, or anatomical basis for these findings was identified. As differentiated from previous reports of neonatal enteropathies, this infant demonstrated involvement of both the small and large intestine with histopathologic findings of acute and chronic inflammation, extensive submucosal fibrosis, and “flat” small intestine mucosa. In addition, this patient had a polyamine deficiency (a primary or secondary phenomenon), which may have contributed to delayed epithelial maturation. These findings suggest that the inflammatory bowel disease and altered epithelial maturation contributed to a fatal intractable diarrhea.
Lymphoblastic Stimulation Test with Food Proteins in Digestive Intolerance to Cow's Milk and in Infant Diarrheas
Authors: Baudon J. J. , Mougenot J. F. , Didry J. R. 
Published: February 2024

SummaryThe lymphoblastic stimulation test (LST) with cow's milk proteins was performed in 114 infants. In 42 infants, digestive intolerance to cow's milk proteins (CMI) was suspected; withdrawal‐rechallenge test confirmed intolerance in 34, and disproved it in the other eight patients. Of the other patients, 17 had acute gastroenteritis, 11 had postgastroenteritis subacute diarrhea, 12 had gluten intolerance, 14 had intractable diarrhea, and 18 had no digestive disorders. Of the 34 infants with CMI, 27 (79%) had a positive LST to one or more cow's milk proteins. Of the 34 positive LST patients, 12 also had soya intolerance; nine of these 12 infants (75%) had positive LST to soya. Of the eight infants who had a negative cow's milk rechallenge test, five (62%) had a positive LST. In the other groups, results were also positive in 12–27% of those having diarrhea of infectious origin or gluten intolerance, and in none of the infants without digestive disorders. Of the 14 cases of severe intractable diarrhea, 12 (86%) were also LST‐positive, but CMI could not be excluded. LST was positive, particularly in diarrheas of neonatal origin. Lymphoblastic stimulation was induced more frequently by casein than by β lactoglobulin, and least frequently by a lactalbutnin. In conclusion, LST is frequently positive in CMI, but is not sufficiently specific to be a reliable diagnostic examination.
Effects of Gliadin Peptides B1‐B4 in Celiac Disease.
Authors: Stallmach Andreas , Belitz Hans‐Dieter , Gellermann Barbara , Schäfer Hansjörg , Wieser Herbert , Stern Martin 
Published: February 2024

SummarySmall intestinal organ culture was used as an in vitro system to study the enterotoxic effects of gliadin peptides. Measurement of enterocyte height proved to be a reliable and reproducible way of assessing mucosal change during organ culture. Enterocyte height decreases nonspecifically in normal cultured mucosa, whereas the height of enterocytes of celiac mucosa increases in vitro in controls. All the gliadin peptide fractions (B1, B2, B3, B4) that had been prepared by peptic‐tryptic hydrolysis, ultrafiltration, and gel chromatography, equally inhibited the morphological increase of enterocyte height normally observed without gliadin in untreated celiac mucosa. Electrophoretic studies and amino acid analysis of B1‐B4 revealed similarity between gliadin fractions with quantitative differences in molecular weight distribution of the peptide components. Our studies suggest that organ culture assessed by morphometry is a suitable model for the investigation of toxic peptides of gliadin in celiac disease. In the future, pure gliadin peptides will have to be examined.
Intestinal Mucosa in Nephropathic Cystinosis
Authors: Iancu Theodore C. , Lerner Aaron , Shiloh Hanna 
Published: February 2024

SummaryThe major manifestations of nephropathic cystinosis are renal tubular acidosis, vitamin D‐resistant rickets, and dwarfism. Cystine crystals are deposited in a variety of cells, mainly phagocytic, including macro‐phages of the intestinal lamina propria. Previously, ultrastructural changes were suggested to occur in the absorptive epithelium as well, possibly as a result of local cystine toxicity. We report here on the light‐ and electron‐microscopic findings in the jejunal mucosa of two patients, aged 4 and 9 years with nephropathic cystinosis. Cystine crystals were easily identified in semithin sections of plastic‐embedded specimens as brick‐ and hexagon‐shaped spaces in macrophages. Electron microscopy showed that all crystals were in single‐membrane‐limited bodies (lysosomes), within phagocytic cells, and exclusively located in the lamina propria. In contrast to previous findings, the absorptive epithelium showed no abnormalities. We conclude that the growth failure in cystinosis is not a consequence of morphological toxic alterations in the intestinal epithelium, but is related to the known metabolic abnormalities of this condition. The use of rectal suction biopsy as a means of diagnosing cystinosis is also suggested as an alternative to other diagnostic methods.
Histochemical and Morphological Analysis of Colonic Epithelium from Children with Gardner's Syndrome and Adults Bearing Adenomatous Polyps
Authors: Lev Robert , Lebenthal Emanuel , Rossi Thomas , Lance Peter 
Published: February 2024

SummaryNormal and adenomatous colonic tissues from children with Gardner's syndrome were compared to analogous tissues from adults bearing adenomatous polyps using mucin histochemical and lectin‐binding techniques. Adenomatous tissue from children exhibited general morphological similarity to its adult homologue, but showed less dysplasia. Its goblet cells stained weaker for mucins and the lectins Dolichos biflorus agglutinin (DBA) and peanut agglutinin (PNA). This suggested un‐derglycosylation of side chains of mucins in these childhood adenomas. The weak DBA and relatively intense sulfomucin staining in these adenomas suggested that they arose from deep crypt cells. Adult adenomas shared certain histochemical properties with carcinomas, namely, increased affinity for periodic acid‐Schiff (PAS) and focally for PNA. There is evidence based on the effects of saponification and sialidase treatments that the weak initial PAS reaction in normal and adenomatous colonic goblet cells from both age groups results from sub‐stituents on sialic acid and, in the case of normal colon from children, on other monosaccharides as well. Finally, there was a frequent lack of parellelism between PAS and lectin staining suggesting that different groups within the sugars are responsible for reactivity with those compounds.
Development of Five Metabolic Activities Associated with the Intestinal Microflora of Healthy Infants
Authors: Midtvedt Ann‐Christine , Carlstedt‐Duke Bodil , Norin Karin Elisabeth , Saxerholt Henri , Midtvedt Tore 
Published: February 2024

Summary:The establishment of a functionally active intestinal flora was followed in 17 healthy Swedish children from birth up to 6 months of age. Utilizing gas chroma‐tography, spectrophotometry, and gel electrophoresis, feces were analyzed on certain biochemical markers that reflect the action of the intestinal flora in vivo. The establishment of the following five flora‐related functions was investigated: production of short chain fatty acids (SCFAs), degradation of mucin, conversion of bilirubin to urobilinogen and of cholesterol to coprostanol, and inactivation of fecal tryptic activity (FTA). Production of SCFAs was the first function to be established, followed by bilirubin conversion and mucin degradation. No child showed conversion of cholesterol. The values of FTA were lower than in adults. This study indicates that the establishment of a functionally active flora is a slow process and that some functions are almost fully established before other functions have started to develop. Environmental factors, such as the diet, seem to be of importance. In general, the functions seem to develop slower in those children receiving breast milk exclusively than in those receiving formula supplements.
Acute Obstructive Pancreatitis Secondary to a Duodenal Hematoma
Authors: Glassman Mark S. , Rodrigues Roy , Bussel James , Spivak William , Hilgartner Margaret 
Published: February 2024

Summary:An 18‐year‐old male with hemophilia presented with symptoms and signs of upper intestinal obstruction. Evaluation was consistent with an intramural duodenal hematoma and obstructive pancreatitis. As it is not possible to distinguish between these two disorders on a clinical basis, it is important to realize that pancreatitis may occur in such patients more often than is recognized.