bluuebunny/crossref_metadata_2025 huggingface dataset:
Disaccharidase Activities in Small Intestine of Rotavirus‐Infected Suckling Mice
Authors: Collins J. , Candy D. C. A. , Starkey W. G. , Spencer A. J. , Osborne M. P. , Stephen J.
Published: January 2024

A histochemical study of the time course of the appearance and location of lactase and α‐glucosidase (used to detect sucrase and maltase) activities was carried out on control and rotavirus‐infected mice from 7 to 14 days old. The overall pattern of enzyme activity was in agreement with previous quantitative studies on the activities of these enzymes. No evidence was obtained to support the idea that lactase deficiency was the result of repopulation of villi (denuded of lactase‐producing villus cells) with immature lactase‐negative cells. Low lactase activity was more likely to reflect profound changes in metabolically crippled cells, and recovery of lactase activity with recovery of normal metabolic functions. The location of enzyme activity to brush border regions rather than the cytoplasm of villus enterocytes enhances the significance of previous quantitative studies on these enzymes. The timing and duration of diminished lactase activities were such that they were unlikely to cause the induction or perpetuation of diarrhea in murine rotavirus diarrhea. The appearance in infected animals of α‐glucosidase 3 days earlier than normal indicates that, in addition to reversible changes seen with lactase, developmental changes were accelerated that affected both crypt and villus cells.
Neonatal Duodenal Perforation
Authors: Miller Barnett M. , Kumar Ashok
Published: January 2024

Duodenal perforation in neonates is uncommon, and has been described rarely in the third part of the duodenum. Gastrointestinal perforations without an obvious cause have been labeled as “spontaneous.” We report a case of perforation in the third part of the duodenum in a premature infant for which there was no obvious cause. In such “spontaneous” perforations, multi‐factorial etiology is likely and possible factors are discussed. The high mortality rate in such patients can be improved by early diagnosis and prompt resuscitation followed by surgery. Peritoneocentesis plays an important diagnostic role. It is also therapeutic in relieving the respiratory distress caused by free intraperitoneal air. The majority of duodenal perforations are amenable to primary closure at surgery and this should be the treatment of choice.
Endoscopic and Histologic Findings in the Upper Gastrointestinal Tract of Children with Crohn's Disease
Authors: Schmidt‐Sommerfeld Eberhard , Kirschner Barbara S. , Stephens Janet K.
Published: January 2024

In an attempt to define endoscopic and histologic changes suggestive of upper gastrointestinal Crohn's disease (CD), the medical histories, endoscopic reports, and biopsies were reviewed from 24 pediatric patients with CD and 28 age‐matched patients without CD in whom esophagogastroduodenoscopies were performed because of upper GI symptoms. No differences in the overall frequency of endoscopic abnormalities were found between the two groups. However, gastric erosions and ulcerations were more frequent in CD patients. Histological abnormalities in the stomach and duodenum were also more frequent in CD patients. Noncaseating granulomas were found in five patients with CD and in one patient without clinical, radiologic, or endoscopic evidence of CD. Focal inflammation in the stomach and duodenum occurred more frequently in CD patients. Two patients with CD had focal and deep chronic inflammatory infiltrates in the esophagus, which reached the submucosa. Abnormal histology was often seen in CD patients with normal endoscopic appearances. We conclude that superficial ulcerations seen during endoscopy and the histological finding of focal inflammation may represent upper GI CD in pediatric patients. Histological changes can be missed if biopsies are not taken from normal‐appearing mucosa during endoscopy.
Lactose Maldigestion
Authors: Wittenberg D. F. , Moosa A.
Published: January 2024

A study was performed to determine whether the age‐related prevalence of lactose maldigestion is increased in healthy institutionalized children. Previous malnutrition did not exclude children from study. The control children had no prior history of malnutrition or hospital admissions for diarrhea. The anthropometric comparison showed the institutionalized children to be shorter and lighter than the controls, but they were of normal weight by stature, suggesting a normal recent nutritional state. Fasting breath hydrogen tests were performed following an oral dose of lactose 1 g/kg, given as a 10% solution. A rise of breath hydrogen of ≥=20 ppm was considered to indicate lactose maldigestion. Twenty‐two of 44 controls and 39 of 49 study children were lactose maldigesters (p < 0.01). The control children showed an age‐related increase in the prevalence of lactose maldigestion, from 31.6% at age <5 years to 75% at age >9 years. The institutionalized children, by comparison had an increased prevalence of 78.8% in the group under 5 years of age (p = 0.002). Equally high prevalence rates were found at all ages tested. At follow‐up, lactose maldigestion was persistent in the majority of the study children. This had no nutritional impact despite a regular milk intake. The distinction between acquired and primary lactose maldigestion is of no practical significance in these children.
Kupffer Cell Engraftment Across the Major Histocompatibility Barrier in Mice
Authors: Paradis Khazal , Sharp Harvey L. , Vallera Daniel A. , Blazar Bruce R.
Published: January 2024

The source of population renewal for Kupffer cells (KC), the major antigen‐presenting cells of the liver, remains controversial. Using a well‐described murine bone marrow transplantation (BMT) model in which the donor and recipient are disparate at the major histocompatibility complex (MHC), we have studied (a) the source of KC renewal by genotypic analysis, cell surface (class II or la) antigens, and immune function assays; (b) the level of KC la expression post‐BMT in transplant recipients: and (c) the capacity of newly repopulating KC to present antigen to an la‐restricted T cell clone of donor la type. Kupffer cell engraftment, as assessed by each of these three methods, was noted to be predominantly of donor marrow origin by day 21 post‐BMT. Cell surface la expression was comparable to that of nontransplanted controls of the same strain as donor mice. Within 7 days post‐BMT, KC were mature antigen‐presenting cells. We conclude that KC rapidly repopulate the liver from donor bone marrow post‐BMT, and these macrophages are able to interact with T lymphocytes in an immunocompetent manner.
Intussusception of the Appendix in a Patient with Cystic Fibrosis
Authors: McIntosh J. Clarke , Mroczek Elizabeth C. , Baldwin Charles , Mestre Jose
Published: January 2024

This case report describes the clinical presentation and the radiographic, endoscopic, and pathologic findings in a patient with cystic fibrosis (CF) and intussusception of the appendix. This is the first time that intussusception of the appendix has been documented in a patient with CF. This disorder should be considered in the CF patient with cramping lower abdominal pain or rectal bleeding.
Chronic Pancreatitis in Muscular Cytochrome c Oxidase Deficiency
Authors: Kato Seiichi , Miyabayashi Shigeaki , Ohi Ryoji , Nakagawa Hiroshi , Abe Jun‐ichiro , Yamamoto Katsuya , Watanabe Shuichi , Tada Keiya
Published: January 2024

Chronic pancreatitis is described in a 10‐year‐old boy with mitochondrial myopathy due to cytochrome c oxidase (complex IV) deficiency. There have been few reports of chronic pancreatitis associated with congenital metabolic diseases. Marked pancreatic calcifications and pseudocysts of the pancreatic head and tail were demonstrated by computed tomography of the abdomen and by endoscopic retrograde pancreatography, which were thought to be useful methods for morphological examination of the pancreas. In the patient, no possible etiological factors were identified, including anomalous pancreatobiliary ductal junction or biochemical abnormalities. The association between cytochrome c oxidase deficiency and chronic pancreatitis remains to be established.
Pulse Oximetry and Upper Intestinal Endoscopy in Infants and Children
Authors: Bendig Donald W.
Published: January 2024

Infants and children undergoing upper intestinal endoscopy were monitored by both pulse oximetry and chest EKG. Fifty‐seven patients between the ages of 6 weeks and 36 months underwent 60 flexible upper intestinal endoscopies. All patients received parenteral sedation only. All procedures were successfully performed without significant complications. In 53 of the patients there was only transient mild oxygen desaturation with introduction of the endoscope into the pharynx and upper esophagus. In contrast, in seven patients, oxygen desaturation to less than 90% was noted following sedation but prior to insertion of the endoscope without overt clinical evidence of complications. Oxygen administered by nasal cannula resulted in a return of the oxygen saturation to at least the preprocedural level and allowed for safe completion of the studies. With improved monitoring, the use of smaller, more flexible endoscopes, and more experience, routine general anesthesia in children <3 years of age, as recommended in the past, may not be mandatory. Pulse oximetry may be particularly useful as an early indicator of poor oxygenation and may provide an objective means to assess the need for supplemental oxygen and to determine the degree of postprocedural observation.
Risk Factors for Fatal Diarrhea in Hospitalized Children in India
Authors: Sachdev H. P. S. , Kumar Shiv , Singh K. K. , Satyanarayana L. , Puri R. K.
Published: January 2024

There is scant information on the risk factors for diarrheal deaths in developing countries. A prospective evaluation was therefore conducted on 382 consecutive children (<5 years of age), who were hospitalized with diarrhea. A complete profile, including outcome, was available for 357 patients, 37 of whom died (10.4%). In the univariate analysis, four factors were significantly associated (p < 0.02) with death: associated major infection {pneumonia, septicemia, or meningitis), severe wasting (±50% weight for age), severe stunting (±85% height for age), and protraction of illness (>14 days). In the multivariate analysis, all four factors retained their significance. The adjusted odds ratios (95% confidence interval) were 4.7 (3.9, 5.6), 3.3 (2.7, 4.0), 1.9 (1.6, 2.3), and 1.5 (1.3, 1.8), respectively. In addition, in children <19 months of age (n = 241; 29 deaths) breast‐feeding had a significant (p < 0.001) protective effect (adjusted OR—2.3, 95% CI—1.9–2.8). It is concluded that even in a setting with high diarrheal fatality rates, high‐risk children can be identified and targeted for intensive intervention.
The Development of the Gastrointestinal System in Fetal Sheep in the Absence of Ingested Fluid
Authors: Avila C. G. , Harding R.
Published: January 2024

Our aim was to determine the effects of preventing the passage of ingested fluid on the development of the digestive tract in fetal sheep. The esophagus was fistulated and ligated in six fetuses at 90 days of gestation (term = 145 days); vascular catheters were implanted at day 120. Six control fetuses had vascular catheters implanted at day 120. At autopsy (day 135), although fetal body weights were similar in both groups, the abdominal girth and weights of the gastrointestinal tract, liver, and pancreas were reduced in experimental fetuses. In the gastric (abomasal) fundus and antrum, there was evidence of altered mucus composition in epithelial cells, a decrease in thickness of the muscularis externa, and an increase in thickness of the mucosa and its components. In the duodenum, there were significant changes in the thickness of most components of the wall; Brunner's glands were greatly reduced in size or were absent. Glandular cells contained less mucus in comparison to controls. In the proximal small intestine, there were significant reductions in the thickness of most components of the wall, and epithelial cell migration was retarded, resulting in a longer renewal time for villous cells. In the distal small intestine, the diameter of the intestine and submucosal and epithelial cell migration rate were significantly decreased in the experimental group. In summary, the absence of the passage of ingested fluid in fetal sheep restricts the growth and development of the gastrointestinal tract, liver, and pancreas.
Differences in Distribution and Severity of Helicobacter pylori Gastritis in Children and Adults with Duodenal Ulcer Disease
Authors: Queiroz Dulciene M. M. , Rocha Gifone A. , Mendes Edilberto N. , Carvalho Anfrisina S. T. , Barbosa Alfredo J. A. , Oliveira Celso A. , Lima Geraldo F.
Published: January 2024

The presence of Helicobacter pylori and the gastric mucosa histology were investigated in 15 children and 15 adults with duodenal ulcer. The microorganism was isolated from antral and oxyntic mucosa in 100% of patients, both adults and children. The results of Gram stain and preformed urease test were compared with those of culture and there was no difference in sensitivity among the tests. Antral chronic gastritis was observed in all patients. However, children presented oxyntic gastritis more frequently than adults. It was also observed that the endoscopic aspects were different in the two groups of patients. The results here observed strongly support the idea that, as well as in adults, H. pylori is the causative agent of the gastritis seen in children with duodenal ulceration. On the other hand, the histological findings of oxyntic mucosa of children with duodenal ulcer were different from those of adults.
Routine Measurements of Gluten Antibodies in Children of Short Stature
Authors: Knudtzon Jørgen , Fluge Gjermund , Aksnes Lage
Published: January 2024

Serum IgA and IgG gluten antibody levels were measured prospectively in 168 children without abdominal symptoms referred to the outpatient clinic for short stature. Elevated (>0.30 units) gluten IgA antibody levels (0.82 ± 0.22 units) were detected in five children (aged 8.5 ± 1.6 years, height standard deviation score −2.14 ± 0.26). They had probable celiac disease based on villus atrophy at duodenal biopsy, in vitro toxicity of wheat gluten, and disappearance of serum gluten antibodies after gluten withdrawal from the diet. Three of these children had elevated IgG antibody levels (>0.25 units). Elevated IgG antibody levels with normal IgA levels were demonstrated in 11 short children who had normal duodenal biopsy results. It is concluded that measurement of gluten antibodies, especially of the IgA type, should be included in the diagnostic evaluation of children with short stature.
Verotoxic Escherichia coli in Human Disease
Authors: Rosensweig Jeffrey N. , Gourley Glenn R.
Published: January 2024

Verotoxin‐producing E. coli (most frequently E. coli 0157) have been implicated in the pathogenesis of diarrhea, hemorrhagic colitis, hemolytic uremic syndrome and thrombotic thrombocytopenic purpura. Cattle, meat products, and other sources have been found to harbor these organisms. Isolation of E. coli 0157:H7 on MacConkey‐sorbitol agar is diagnostic, yet the bacteria are difficult to detect after the first week of infection. Enzyme linked immunosorbent assays (ELISAs) can detect verotoxin in fecal filtrates in the absence of viable bacteria. Serologic evidence has also been used to support the diagnosis of verotoxin‐associated infection. Evidence supporting the etiologic role of different verotox‐ins is reviewed. Treatment remains supportive since the use of antibiotics and antimotility agents can lead to poorer outcomes. Recommendations for prevention are presented.
Liver Function in Early Congenital Syphilis
Authors: Venter André , Pettifor John M. , Duursma June , Pudifin Dennis J. , Smyth Amelia , Becker Piet J.
Published: January 2024

In this prospective study, neonates with clinical congenital syphilis were investigated to determine if penicillin therapy caused a deterioration in liver function. The relationship between circulating immune complexes and liver involvement was monitored, and the efficacy of steroid therapy as an adjunct in the treatment of congenital syphilis was investigated. Thirty neonates with clinical congenital syphilis were randomly assigned into two groups: one group received penicillin therapy only, and the other group penicillin and prednisone as an adjunct. Twenty‐one infants who did not have clinical or serological syphilis, born to seropositive mothers, served as a “control” group. Liver function tests, full blood counts, and immunological studies were performed at various intervals up to 3 months of age. Although the symptomatic groups differed significantly from the asymptomatic group in most of the parameters measured, there were no significant differences noted between the two symptomatic groups at any time point. No direct relationship between penicillin therapy and either deteriorating liver function or the presence of circulating immune complexes could be demonstrated. Also, prednisone therapy did not modify any of the parameters studied.
Effect of Calcium/Phosphorus Ratio on Mineral Retention in Parenterally Fed Premature Infants
Authors: Pelegano James F. , Rowe Jonelle C. , Carey Dennis E. , LaBarre Donna J. , Edgren Karl W. , Lazar Ann Marie , Horak Eva
Published: January 2024

We hypothesized that retention of parenterally delivered calcium (Ca) and phosphorus (P) is affected by the ratio of the delivered minerals and that a 1.7:1 ratio would be optimal since this is the ratio of retention of these minerals by the fetus. Forty‐one very low birth weight (VLBW) infants were randomly assigned to one of three total parenteral nutrition (TPN) solutions that were different only in their Ca:P ratios: 2:1 (76 mg/kg/day Ca and 38 mg/kg/day of P), 1.7:1 (76 mg/kg/day Ca and 45 mg/kg/day P), and 1.3:1 (58 mg/kg/day of Ca and 45 mg/ kg/day of P). Serum levels of calcium, phosphorus, and alkaline phosphatase, retentions of calcium and phosphorus and urinary cyclic AMP levels were measured after 48 h on the assigned Ca to P ratio. Calcium retentions were higher with the 2:1 and 1.7:1 ratios and phosphorus retentions were higher with the 1.3:1 and 1.7:1 ratios. The 1.7:1 ratio allowed for the highest absolute retention of both minerals and was the closest to published in utero accretion of calcium and phosphorus. The serum and urine studies demonstrated no abnormalities on any of the three ratios. Cyclic AMPs were not different among groups and were not elevated compared to previous reports suggesting that none resulted in parathyroid hormone (PTH) stimulation. We conclude that the 1.7:1 ratio is better than higher or lower ratios for delivery of calcium and phosphorus in TPN solutions at the quantities studied.
Liver Transplantation for Cholesteryl Ester Storage Disease
Authors: Ferry George D. , Whisennand Hartwell H. , Finegold Milton J. , Alpert Elliot , Glombicki Alan
Published: January 2024

This case describes a patient with cholesteryl ester storage disease who underwent liver transplantation for progressive cirrhosis, portal hypertension, ascites, and uncontrollable gastrointestinal bleeding. Four and one‐half years posttransplant, her growth improved, cholesterol levels have returned to normal, and she is clinically well except for mild hypersplenism and an elevated blood urea nitrogen (BUN) and creatinine. Serum triglycerides remain elevated, but there have been no signs of progressive renal, intestinal, vascular, or pulmonary disease.
Development and Validation of a Pediatric Crohn's Disease Activity Index
Authors: Hyams Jeffrey S. , Ferry George D. , Mandel Francine S. , Gryboski Joyce D. , Kibort Phillip M. , Kirschner Barbara S. , Griffiths Anne M. , Katz Aubrey J. , Grand Richard J. , Boyle John T. , Michener William M. , Levy Joseph S. , Lesser Martin L.
Published: January 2024

Clinical and laboratory observations of 133 children and adolescents with Crohn's disease were used to validate an index of severity of illness previously developed by a group of senior pediatric gastroenterologists at a research forum in April 1990. This pediatric Crohn's disease activity index (PCDAI) included (a) subjective reporting of the degree of abdominal pain, stool pattern, and general well‐being; (b) presence of extraintestinal manifestations, such as fever, arthritis, rash, and uveitis; (c) physical examination findings; (d) weight and height; and (e) hematocrit, erythrocyte sedimentation rate, and serum albumin. Independent evaluation of each patient by two physician‐observers was performed at the time of a visit, and each physician completed a PCDAI index and a modified Harvey‐Bradshaw index and made a “global assessment” of disease activity as none, mild, moderate, or severe. Excellent interobserver agreement was noted for the PCDAI, modified Harvey‐Bradshaw index, and global assessment. There was a strong correlation between global assessment and both the PCDAI or modified Harvey‐Bradshaw. Increasing PCDAI scores were noted with increasing disease severity, and significant differences in scores were noted between the severity groups. We propose that the PCDAI could be used in multicenter projects to facilitate patient stratification by disease severity and that longitudinal PCDAI scores might provide a numerical measure of response to therapeutic regimens.
Serum Lipids in Celiac Children
Authors: Ciampolini Mario , Bini Stefania
Published: January 2024

The plasma lipid levels were investigated in 45 celiac children under 3 years of age and 49 over this age at diagnosis (untreated groups), after gluten‐free diet and in comparison with irritable bowel syndrome (IBS) groups matched for size, gender, and age. Total and HDL cholesterol levels were significantly lower and triglycerides higher in celiac untreated children than in IBS control children in both age groups. HDL cholesterol significantly increased in both age groups after some months of gluten‐free diet. Total and LDL cholesterol significantly increased and triglycerides decreased in the younger group of subjects after the period of gluten‐free diet. These results may be useful for diagnostic purposes and to motivate a strict gluten‐free diet in celiac children.
Affinity Chromatographic Identification and Quantitation of Blood Group A‐Active Oligosaccharides in Human Milk and Feces of Breast‐Fed Infants
Authors: Sabharwal Hemant , Sjöblad Sture , Lundblad Arne
Published: January 2024

The finding of large quantities of blood group A‐active oligosaccharides in the feces of a blood group A breast‐fed infant motivated a search for the origin of these compounds. Using an affinity chromatographic technique, the nature of A‐active oligosaccharides in human milk is demonstrated. The amounts of A‐active tetrasac‐charide (A‐tetra) and the Lewis b‐active lacto‐N‐difucohexaose I (LND‐I) varied between 19–375 mg/L for A‐tetra and 14–710 mg/L for LND‐I. Using the same technique, the amounts of A‐tetra and LND‐I in milk samples from five women of different blood groups were compared with those in the feces of their breast‐fed infants. The A‐tetra was present only in feces from infants of blood group A or AB mothers and the amount per 24 h corresponded roughly to that in a 1‐L portion of milk. One of the milk samples was also analyzed for the presence of larger A‐active oligosaccharides (A‐pentasaccha‐ride, A‐hexasaccharide, and A‐heptasaccharide). Their amounts were much less as compared to the amounts present in feces. These results indicate that milk is a possible source for the smallest A‐tetrasaccharide found in the feces of breast‐fed infants, while the larger A‐active oligosaccharides might be the result of an intestinal metabolic modification
Comparison of Nonabsorbable Markers Poly R‐478 and [14C]PEG‐4,000 for Use in Developmental Absorption Studies
Authors: Stahl Gary E. , Fayer Jane C. , Ling Saidee C. , Watkins John B.
Published: January 2024

To determine the utility of Poly R‐478, a stable, polymeric dye (MW, 40,000), as a nonabsorbable marker for studies in the developing small intestine, it was validated by comparison to carbon 14‐labeled polyethylene glycol, MW 4,000 ([14C]PEG‐4,000) in rats from 14 to 40 days of age. The recovery and quantification of Poly R‐478 from biological samples is simple and rapid compared to other nonradioactive nonabsorbable markers. In 40‐day‐old rats simultaneously given Poly R‐478 and [14C]PEG, total recoveries were similar, the percentages of the Poly R‐478 dose and the [14C]PEG dose recovered per segment were identical, and taurocholate absorption rates calculated using each marker were comparable. Recovery of Poly R‐478 from flushed intestinal segments, determined by a one‐step extraction, was equivalent to that of [14C]PEG in 40‐day‐old rats (96.5 ± 3.7% versus 102.7 ± 10.1%; NS) but was superior to that of [14C]PEG in 21‐day‐old rats (98.0 ± 6.2% versus 63.4 ± 5.5%; p < 0.001) and 14‐day‐old rats (97.8 ± 6.7% versus 56.7 ± 12.6%; p < 0.001). Within each age group, the distribution of Poly R‐478 within the intestine was similar to that of [14C]PEG. In addition, total taurocholate absorption in the presence of Poly R‐478 was comparable to that in the presence of [14C]PEG. Complete recovery of Poly R‐478 from a suspension of liver particles and from mixtures with two commercial infant formulas was demonstrated in vitro, suggesting that Poly R‐478 may be used when foods are present in the intestine. Poly R‐478 is an effective and easily measured nonabsorbable marker and allows for development absorption studies utilizing two radiolabeled substrates.
Esophageal pH Monitoring Data During Chest Physiotherapy
Authors: Vandenplas Yvan , Diericx Ann , Blecker Uwe , Lanciers Sophie , Deneyer Michel
Published: January 2024

Sixty‐three infants, aged from 1 to 4 months, were examined for gastroesophageal reflux (GER) using esophageal pH monitoring. Thirty were examined because of chronic vomiting, 21 were healthy controls examined for GER as part of a screening program for sudden infant death syndrome, and 12 had an acute respiratory disease (RD). The 24‐h pH monitoring data were within normal ranges in 26 infants (20 controls, 2 babies with emesis, and 4 with RD). Data were abnormal in 37 infants (1 control, 28 infants with emesis, and 8 with RD). All babies were submitted during a fasting awake period to a 30‐min chest physiotherapy session. In the three groups studied, the incidence of GER episodes detected by the pH probe was significantly higher during physiotherapy if compared (a) to the calculated mean incidence during a 30‐min period of the 24‐h investigation or (b) to the incidence during a fasting awake period such as that during which the physiotherapy was given (p < 0.001; Wilcoxon rank‐sum test). We conclude that chest physiotherapy significantly increases GER incidence. We therefore propose restricting chest physiotherapy to fasting periods. These data add to the confusion that already exists regarding the possible causal relationship between (acid) GER and respiratory disease.
The Curious Umbilicus
Authors: Hyman Neil , LeLeiko Neal S. , Dolgin Stephen
Published: January 2024

Omphalomesenteric duct derivatives cause an assortment of complications, most notably intestinal hemorrhage and obstruction. Intestinal obstruction is the most lethal complication and usually results from a diverticulum with an attachment to the umbilicus. This cause of intestinal obstruction is not generally recognized pre‐operatively. We report a teenager with episodic abdominal pain, acute small bowel obstruction, and a curious umbilical deformity with an underlying omphalomesenteric duct remnant. We believe that this physical finding suggests the diagnosis in patients with intestinal obstruction who have experienced unexplained episodes of abdominal pain.
Duodenojejunal Intussusception Secondary to Hamartomatous Polyp of Brunner's Glands
Authors: Chuang Jiin‐Haur , Chen Wei‐Jen
Published: January 2024

A case of duodenojejunal intussusception secondary to a large hamartomatous polyp of Brunner's glands was reported in a 15‐year‐old girl. Despite a great preponderance of ileocolic intussusception in childhood, this is the first reported case of duodenojejunal intussusception occurring in that age group. The diagnosis was not made by clinical examination; complete duodenal obstruction was shown in upper gastrointestinal series and by endoscopy. The intussusception was reduced manually at laparotomy, and the polyp was excised. Pathological examination revealed a preponderance of normal‐appearing Brunner's glands in the tumor, consistent with the diagnosis of Brunner's gland hamartoma.
Serum IgG and IgA Anti‐gliadin Antibodies as Markers of Mucosal Damage in Children with Suspected Celiac Disease upon Gluten Challenge
Authors: Wauters E. A. K. , Jansen J. , Houwen R. H. , Veenstra J. , Ockhuizen Th.
Published: January 2024

Serum anti‐gliadin antibodies (AGAs) of the IgG and IgA isotypes were determined in 17 children (mean age of 5.6 years) by means of an enzyme‐linked immunosorbent assay (ELISA). All children were suspected of celiac disease. They had been on dietary treatment for at least 10 months before they were challenged with gluten. Based on jejunal biopsy findings, 10 of the 17 children had to be considered positive. The sensitivity of the measurement of AGA at 6 weeks after gluten challenge was found to be 90% for IgG, 100% for IgA, and 100% for IgG/IgA combined. The specificity for IgG, IgA, and the IgG/IgA combination was 100, 71, and 100%, respectively. Twelve weeks after gluten challenge, the sensitivity as well as the specificity of AGA determination for IgG, IgA, and IgG/IgA were 100%. It is concluded that testing both IgG AGA and IgA AGA in children suspected of celiac disease is valuable in monitoring the course of the diagnostic provocation protocol and that jejunal biopsies can be abolished. This inexpensive tool can be useful in reducing the number of intestinal biopsies.
Coombs‐Positive Hemolytic Anemia Preceding Ulcerative Proctitis
Authors: Tyshkov Misha , Berezin Stuart , Jayabose Somasundaram , Bostwick Howard , Glassman Mark , Newman Leonard
Published: January 2024

A 12‐year‐old girl is described who developed rectal bleeding 5 months after being diagnosed as having a Coombs‐positive hemolytic anemia. Colonoscopy showed that the rectal bleeding was due to ulcerative proctitis. This is the first case report of Coombs‐positive hemolytic anemia preceding the onset of ulcerative proctitis in a child.
Hyperplastic Polyp of the Stomach Simulating Hypertrophic Pyloric Stenosis
Authors: Sanna Claudio M. , Loriga Piero , Dessi Enrico , Frau Gianna , Congiu Guido , Corrias Adriano , Corda Roberto
Published: January 2024

We describe a rare case of hyperplastic polyp of the stomach having its clinical origin in the neonatal period and initially stimulating hypertrophic stenosis of the pylorus. After this neoformation, which almost completely obstructed the pylorus, was cndoscopically removed, vomiting ceased and a rapid weight gain was observed. It is known that tumors are a rare cause of gastric outlet obstruction if compared with hypertrophic stenosis of the pylorus. However, our experience suggests that in the case of atypical symptoms, the investigations should be extended, especially to endoscopic examination of the upper digestive tract.
Chronic Inflammatory Bowel Disease in Children and Adolescents in Sweden
Authors: Hildebrand Hans , Fredrikzon Bo , Holmquist Lars , Kristiansson Bengt , Lindquist Bo
Published: January 2024

Summary: The incidence and prevalence of chronic inflammatory bowel disease (IBD) in children was established during 1984 and 1985 in a prospective study in Sweden. The patients with IBD were classified as having ulcerative colitis (UC), Crohn's disease (CD), probable Crohn's disease (PCD), and indeterminate colitis (IC) according to defined histopathologic, endoscopic, and radiologic criteria. The study covered 1.51 million children <16 years of age (93% of all children in Sweden). The incidence of IBD was 5.0 and 4.5 and the prevalence was 17.6 and 18.2 per 100,000 children during the 2 years, respectively. The mean prevalence of UC was 7.5 per 100,000 and of CD + PCD was 6.2 per 100,000. The prevalence of IC was 4.2 per 100,000, which corresponds to 23% of the children with IBD.
Evidence for a Role of Volatile Amines in the Development of Neonatal Hypergastrinemia
Authors: Lichtenberger Lenard M. , Gardner James W. , Barreto José C. , Morrissv Frank H.
Published: January 2024

We investigated the presence of volatile aliphatic amines by fluorescamine and gas chromato‐graphic‐head space analysis in human breast milk and amniotic fluid to assess their role in neonatal hypergastrinemia. These volatile nitrogenous amino acid metabolites have been previously demonstrated to stimulate gas‐trin release in in vivo and in vitro laboratory preparations. In the present study we demonstrated that these gastrin‐stimulatory volatile amines were present in significant concentrations in breast milk during the first several weeks after parturition and in amniotic fluid. The individual amines that were identified in both human milk and amniotic fluid samples were methylamine, dimethylamine, ethylamine, trimethylamine, propylamine, isobutylamine, and butylamine. This study provides indirect evidence to support the possibility that the hypergastrinemia measured in the fetus/neonate during the period immediately before and after birth may be attributable, in part, to the ingestion of fluid containing high concentrations of gastrin‐stimulating amines.
Influence of Breast Versus Formula Milk on Physiological Gastroesophageal Reflux in Healthy, Newborn Infants
Authors: Heacock Helen J. , Jeffery Heather E. , Baker Jennifer L. , Page Megan
Published: January 2024

The effect of milk type on physiological, gastroesophageal reflux (GER) was studied in 37 breast‐fed and 37 formula‐fed, healthy, term neonates aged 2–8 days. The neonates were randomly selected from the public maternity ward and studied for 4 h after their morning milk feed. GER was recorded by a pH microelectrode placed 6 cm above the gastroesophageal junction and analyzed in the third and fourth postprandial hours. Sleep state was accurately defined from the electroencephalogram, electrooculogram, electromyogram, breathing, and behavioral observations. Movement was recorded from a piezo‐electric transducer. In active sleep, the breast‐fed neonates demonstrated GER episodes of significantly shorter duration than the formula‐fed neonates. The means and 95% confidence intervals (CI) were 3.0 (1.6,5.2) compared with 8.3 (5.0,13.3) min/h of active sleep respectively (p < 0.05). This could not be explained by greater milk volume or increased movement before or during reflux in formula‐fed neonates. However, the lower median pH values for GER in breast‐fed neonates, 2.0 versus 2.5, were significantly different (p < 0.05). This difference may reflect more rapid gastric emptying. The lower esophageal pH is more likely to stimulate peristalsis and thus limit the duration of reflux (shorter episodes), in the breast‐fed neonates.
Maillard Reaction Products and Lysinoalanine
Authors: Langhendries J. P. , Hurrell R. F. , Furniss D. E. , Hischenhuber C. , Finot P. A. , Bernard A. , Battisti O. , Bertrand J. M. , Senterre J.
Published: January 2024

Heat processing is essential for the preservation of milk‐based infant formulas. Heating, however, induces a number of chemical changes during which lysine in the milk proteins reacts with reducing sugars to form Maillard reaction products (MRPs) and also reacts with the dehydroalanine resulting from cystine degradation to form lysinoalanine (LAL). Both products have been reported to induce histological changes in the straight portion of the proximal tubule in the rat kidney. This pilot study was made to investigate the urinary excretion by healthy preterm babies of MRPs and LAL contained in infant formula and to determine their influence on kidney function. Twelve healthy male preterm babies were first fed for 10 days with pooled human milk and then for 5 days with each of two experimental premature infant formulas in a cross‐over design. The infant formulas were sterilized either by ultra‐high temperature (UHT) treatment or by a conventional retort process to give products with low and high levels of MRPs and LAL, respectively. In total, some 15.6% of the initial lysine had been modified in the in‐can‐sterilized product, compared to 6.2% in the UHT product. Urinary excretion of MRP lactulosyllysine ranged from 1.3 to 3.9% of the ingested amount, whereas that of LAL ranged from 6.2 to 9.3%. The higher level of MRPs and LAL in the formulas compared to breast milk had no influence on creatinine clearance or electrolyte excretion. There was no evidence of tubular damage as determined by the urinary excretion of four kidney‐derived enzymes. Feeding of formula, however, did result in a general increase in urinary microprotein levels. This did not seem to be a reflection of tubular impairment and was probably due to a transient and normal adaptation to the higher protein content of the formulas, although an effect of MRPs and LAL could not be ruled out.
Infantile Refsum Disease
Authors: Mandel Hanna , Meiron Dan , Schutgens Ruud B. H. , Wanders Ronald J. A. , Berant Moshe
Published: January 2024

This article describes two siblings with infantile Refsum disease (IRD) whose initial presentation was that of malabsorption and mimicked a‐β‐ or homozygous hypo‐β‐lipoproteinemia. Failure to recognize IRD in the first‐born child precluded proper genetic counseling and prenatal diagnosis in subsequent pregnancies and also caused considerable delay in diagnosing IRD in the second child. The clinical heterogeneity of peroxisomal disorders constitutes a diagnostic challenge, which demands a high degree of awareness from the part of the clinician. This is particularly the case with IRD, where protracted diarrhea with low serum cholesterol levels appears to be a frequently occurring initial feature during the 1st months of life.
Contribution of Ultrasonography in the Diagnosis of Achalasia
Authors: Bergami G. L. , Fruhwirth R. , Di Mario M. , Fasanelli S.
Published: January 2024

We present a case of achalasia diagnosed with the aid of ultrasonography (US). After having examined the clinical‐radiological characteristics of the disease, and after having noted the diagnostic possibilities of manometry and endoscopy, we emphasize the use of US to clearly show the thickening of the muscular wall and the dilatation of the distal esophagus. In those of pediatric age, particularly, US permits differential diagnosis with the esophageal leiomyoma, which is difficult to obtain with other techniques. We suggest, therefore, including US in the study of patients with stenosis of the third distal section of the esophagus.
Vascular Compromise Prior to Intestinal Manifestations of Crohn's Disease in a 14‐Year‐Old Girl
Authors: Van Elburg R. M. , Henar E. L. , Bijleveld C. M. A. , Prins T. R. , Heymans H. S. A.
Published: January 2024

Vascular manifestations as extraintestinal symptoms of Crohn's disease are rare and only occasionally reported in children. A 14‐year‐old girl with vascular compromise prior to intestinal manifestations of Crohn's disease is described. The vascular symptoms were due to segmental narrowing of several major arteries as shown by angiography. This kind of vascular involvement in our patient is different from the pattern described in Crohn's disease and resembles Takayasu's disease. Recently, it has been suggested that Crohn's disease could be mediated by multifocal gastrointestinal infarction due to chronic focal mesenteric arteritis at the level of the muscularis propria of the gut. In Takayasu's disease, a granulomatous inflammation of the vasa vasorum of affected vessels is frequently found. An intramural arteritis, granulomatous in nature, could be the common pathway in both Crohn's and Takayasu's diseases. Until the etiologies of both diseases are uncovered, the interrelation between them will remain subject to speculation.
Pharmacokinetics and Safety of a New Solution of Vitamin K1(20) in Children with Cholestasis
Authors: Amédée‐Manesme Olivier , Lambert Willy E. , Alagille Daniel , De Leenheer André P.
Published: January 2024

In cholestatic diseases, the absorption of fat‐soluble compounds, including vitamin K1(20), is low and periodic administration of vitamin K1(20) is often necessary. Due to the low absorption of vitamin K1(20) from the Konakion formulation, late hemorrhagic disease of the newborn also occurs especially after oral vitamin K1(20) prophylaxis with Konakion. We investigated the pharmacokinetics and the safety of a new formulation of vitamin K1(20) in a mixed micelles (MM) solution. Compared to the old formulation (Konakion) using Cremophor EL as a solubilizer, the higher vitamin K1(20) levels (as measured by HPLC) in serum obtained after oral administration of the MM formulation clearly demonstrate a superiority of this new formulation. Additionally, the elimination of Cremophor EL as well as of propylene glycol from the formulation avoids possible adverse effects associated with intravenous or intramuscular administration. Furthermore, in most cases, the discomfort of parenteral injections can be overcome by simple oral administration even in children with severe cholestasis.
Intestinal Permeability to 51Cr‐EDTA and Orocecal Transit Time in Cystic Fibrosis
Authors: Escobar H. , Perdomo M. , Vasconez F. , Camarero C. , del Olmo M. T. , Suarez L.
Published: January 2024

Intestinal permeability was investigated in 16 children with cystic fibrosis and 14 age‐matched controls, making use of 51Cr‐EDTA as probe molecule. Orocecal transit time was also evaluated using the lactulose/ hydrogen breath test. There was an increase in permeability to 51Cr‐EDTA in patients with cystic fibrosis (mean 13.62%) compared with the control group (mean 1.08%). The orocecal transit time was prolonged in patients with cystic fibrosis compared with controls. Mean fasting breath hydrogen concentration was significantly greater in cystic fibrosis patients (13 ppm) compared with control patients (3 ppm). There was no correlation between the percentage of 51Cr‐EDTA excreted and the orocecal transit time in either control or cystic fibrosis patients.
Valproate Use Associated with Persistent Hyperammonemia and Mitochondrial Injury in a Child with Down's Syndrome
Authors: Kane Robert E. , Kotagel Suresh , Bacon Bruce R. , Vogler Carole A.
Published: January 2024

Valproate is a commonly prescribed anticonvulsant drug that may cause potentially fatal hepatotoxicity, bone‐marrow toxicity, and pancreatitis. Toxicity usually resolves, though, after discontinuation of the medication. We report a 9‐year‐old boy who had Down's syndrome and who developed valproate‐associated bone marrow toxicity, and hepatotoxicity that persisted > 2 years after discontinuation of valproate therapy. Three years after starting valproate, he developed erythrocyte aplasia with a severe, normochromic, macrocytic anemia requiring several blood transfusions. Several months later while still receiving valproate, he developed progressive hyperammonemia and decreased hepatic synthetic function. The macrocytic anemia resolved and hepatic synthetic function improved after discontinuation of valproate therapy. However, hyperammonemia, steatosis, mitochondrial injury, and marked hepatic iron accumulation persisted > 2 years after the valproate was discontinued. The persistent hyperammonemia was responsive to lactulose therapy. A decrease in hepatic iron content by serial phlebotomies did not result in any improvement in the hyperammonemia or hepatic synthetic function. This is the first report of persistent hyperammonemia and hepatic mitochondrial injury after valproic acid therapy.
Successful Late Kasai Portoenterostomy
Authors: Bielamowicz Anne , Weitzman Jordan J. , Alshak Najeeb S. , Rosenthal Philip
Published: January 2024

This article describes an infant with extrahe‐patic biliary atresia who underwent a successful hepato‐portoenterostomy at age 132 days in spite of a delay in diagnosis. Contributing to the delay in diagnosis of this case were the lack of appreciation of acholic stools, the presence of a gallbladder and distal common bile duct on ultrasound examination, and insufficient biopsy material for interpretation. This case emphasizes the variability in presentation and potential difficulties in diagnosis and the need for intraoperative cholangiogram and exploratory laparotomy in such cases. An approach to the patient with extrahepatic biliary atresia who is diagnosed late is discussed. A successful outcome can be achieved for such patients.
Reference Values of the Steatocrit and Its Modifications in Diarrheal Diseases
Authors: Guarino A. , Tarallo L. , Greco L. , Cesarano L. , Guandalini S. , Rubino A.
Published: January 2024

Conflicting results have been reported on the use of the steatocrit to measure fecal fat excretion. Aiming to assess the reliability of this method and its usefulness in the diagnosis of intestinal enteropathies, we measured the steatocrit in 747 healthy children and 442 children with diarrhea grouped according to diagnosis. The steatocrit was found to correlate strictly (r = 0.93) with the chemical measurement of fecal fat. Reference values and ranges were established. The maximal steatocrit was observed in neonates; afterwards, it progressively decreased to an undetectable level in children older than 2 years of age. A steatocrit abnormally high for age was found in 20% of patients with acute diarrhea and in 53% of those with chronic diarrhea. All celiac patients with a gluten‐containing diet showed a marked increase of steatocrit. We conclude that the steatocrit is a reliable and easy‐to‐perform test, which quickly provides valuable information in the diagnostic workup of the child with diarrhea.
Somatic Gene Therapy in Gastroenterology
Authors: Ledley Fred D.
Published: January 2024

Somatic gene therapy represents a new approach for treating a variety of genetic and acquired diseases and has many potential applications in gastroenterology. Somatic gene therapy is based on the ability to transfer recombinant genes efficiently into somatic tissues, such as the liver or intestine, and achieve expression of the recombinant gene product. Gene transfer could be used to replace genetically defective gene functions or prevent the progression of acquired or multifactorial diseases. The therapeutic application of gene transfer technologies requires development of methods for achieving efficient transfer and expression of recombinant gene products, research directed at developing strategies for altering the course of disease, and clinical research aimed at assessing both the technical feasibility and therapeutic efficacy of this approach.
Familial Microvillous Atrophy
Authors: Phillips A. D. , Schmitz J.
Published: January 2024

Twenty‐three cases of microvillous atrophy were reviewed to determine clinical and morphological characteristics of the disease. Congenital and late‐onset forms of presentation were clearly identified in which the late‐onset cases appeared to have a better prognosis. Three different, and distinctive, appearances of the proximal small intestinal mucosa were found. Careful orientation of mucosal samples allowed a temporal sequence of events to be delineated in which the first morphological abnormality to be detected in the epithelium was the accumulation of “secretory granules”; microvillous inclusions were seen in older cells in the upper villous region. It is suggested that, in familial microvillous atrophy, diarrhoea and disorganisation of the brush border assembly occur as a consequence of a more fundamental defect that affects the intracellular traffic of certain cell components, as indicated by the accumulation of “secretory granules.”