bluuebunny/crossref_metadata_2025 huggingface dataset:
Symptomatic Selenium Deficiency in a Child on Home Parenteral Nutrition
Authors: Kelly Deirdre A. , Coe Andrew W. , Shenkin Alan , Lake Brian D. , Walker‐Smith John A.
Published: January 2024

A 27‐month‐old girl with microvillous atrophy who was maintained on long‐term home parenteral nutrition presented with regression of walking skills. She was found to have a tender skeletal myopathy affecting both legs only. Plasma selenium level was <0.05 μmol/L (0.4 μg/dl) (normal 0.8–1.6 μmol/L: 6.3–12.6 μg/dl). red blood cell glutathione peroxidase was undetectable, and plasma glutathione peroxidase was 6 U/L (normal 90–350 U/L). Electromyography was abnormal, muscle biopsy showed a nonspecific myopathy, and muscle selenium was 0.23 μg/g dry weight (normal 0.45–1.05 μg/g dry weight). Following replacement with intravenous sodium selenite, there was a rapid improvement with complete disappearance of muscle pain and tenderness within 1 week. Crawling and walking skills were regained within 6 weeks, in dicating that selenium deficiency was the cause of her symptoms. It is suggested that selenium be monitored and supplemented in children on long‐term total parenteral nutrition particularly if cntcral absorption is deficient.
Pathophysiology of Gastroesophageal Reflux and Distal Esophageal Motility in Children with Gastroesophageal Reflux Disease
Authors: Cucchiara S. , Staiano A. , Di Lorenzo C. , De Luca G. , della Rocca A. , Auricchio S.
Published: January 2024

We investigated the mechanisms of gastroesophageal reflux (GER) and esophageal motility during endogenous esophageal acid exposure in 17 patients with reflux disease alone (age range 3–20 months) (group A) and in 10 patients with reflux disease complicated by esophagitis (age range 4–19 months) (group B), by simultaneous recording distal esophageal sphincter relaxation was the predominant mechanism of reflux in both groups of subjects; however, it was more frequent in group B patients (Bpts), whereas reflux episodes due to appropriate sphincter relaxation were delected more frequently in group A patients (Apts). During endogenous acid exposure, primary peristalsis was the most frequent esophageal motor event in all patients; furthermore, its amplitude was significantly higher in Apts as compared with Bpts. Primary peristalsis was more efficacious (rise of intraluminal pH by at least 0.5 unit) in patients with reflux disease alone, whereas nonspecific motor irregularities were more common in children with reflux esophagitis. It is concluded that the major mechanism of GER in patients with reflux esophagitis is an inappropriate sphincter relaxation; reflux due to appropriate sphincter relaxation is associated with less severe reflux disease; and patients with esophagitis exhibit a deranged esophageal motility during spontaneous acid exposure.
A Carbon‐13 Breath Test to Characterize Glucose Absorption and Utilization in Children
Authors: Lifschitz Carlos H. , Boutton Thomas W. , Carrazza Francisco , Beyreiss Klaus , Schmitz Jacques , Ricour Claude , Shulman Robert , Nichols Buford L.
Published: January 2024

After the administration of a 59% glucose‐water solution that contained tracer amounts of the stable non‐radioactive isotope 13C, breath samples were collected from five children with congenital glucose‐galactose malabsorption and five with severe small bowel villous atrophy and chronic diarrhea. The 13CO2, breath test curves of the children with the congenital malabsorption and chronic diarrhea were compared with each other and with those from three healthy children and four infants with severe malnutrition but no diarrhea. The breath test curves from the children with glucose‐galactose malabsorption and from those with diarrhea were significantly different from those of the other two groups, a finding consistent with impairment of glucose absorption. The [13C]glucose breath test clearly identified the children with severe glucose malabsorption. Further studies are required to determine whether less severe cases of carbohydrate malabsorption also can be identified using the parameters described in our study.
Anti‐reticulin Antibodies
Authors: Khoshoo V. , Bhan M. K. , Unsworth D. J. , Kumar R. , Walker Smith J. A.
Published: January 2024

R‐1 anti‐reticulin antibodies (ARA) were present in sera of 10 of the 12 children who subsequently fulfilled the ESPGAN diagnostic criteria for celiac disease (CD), hut was not found in any children in the age‐matched control groups, viz., severely malnourished (n = 20). acute gastroenteritis (n = 23), normally nourished asymptomatic (n = 38), and 42 children with protracted diarrhoea and failure to thrive due to nonceliac causes. ARA was a highly specific (100%) and sensitive (83%) assay for the early diagnosis of CD. A positive ARA assay with an initial subtotal villous atrophy was seen to always suggest CD, and these together would provide a useful basis for instituting gluten‐free diets in suspected cases of CD rather than histological findings alone, which may be often present in children in the third world with protracted diarrhoea and associated severe malnutrition.
Concentrations of IgA, Secretory IgA, IgM, Secretory IgM, IgD, and IgG in the Upper Jejunum of Children Without Gastrointestinal Disorders
Authors: Hjelt Karsten , Sorensen Christian Hjort , Nielsen Ole H. , Krasilnikoff Peter A.
Published: January 2024

Jejunal fluid was collected from 40 children aged 1–12.2 years and from 10 adults (age 20–25 years) who were without gastrointestinal disorders. They were intubated pernasally with a special tube system that made it possible to avoid contamination from the upper respiratory tract secretions. Quantitation of immunoglobulin A (IgA), secretory IgA (SIgA), IgM, SIgM, IgD, IgG, and albumin was performed by double sandwich enzyme‐linked immunosorbent assay. IgA was the dominating immunoglobulin in jejunal fluid, showing the highest concentrations, followed in order by IgM, IgG, and IgD. The IgD concentrations were about 1,000 times less than in plasma of age‐matched children. The immunoglobulin concentrations did not show any age‐related differences, except for IgG and for the SIgA/IgA ratio. The former declined with children's age (p < 0.02), whereas the latter was significantly higher in adults (interquartile range: 97–100%) than in young children aged l‐<5.5 years (interquartile range: 55–94%) (p < 0.001). This indicates an age‐related decline in leaking from gut mucosa. The concentrations of SIgA and SIgM showed a positive correlation (p < 0.001). The majority of IgA and IgM were estimated to be locally produced, whereas IgD and IgG were transudated from plasma.
Protein‐Losing Enteropathy Detected by 99mTc‐Labeled Human Serum Albumin Abdominal Scintigraphy
Authors: Lan Janet A. , Chervu L. Rao , Marans Zvi , Collins Janna C.
Published: January 2024

99mTc‐labeled human serum albumin (HSA) abdominal imaging is a new way of demonstrating gastrointestinal protein loss. We present two children with hypoalbuminemia of obscure etiology in whom albumin loss was localized in the gastrointestinal tract with 99mTc‐HSA scintigraphy and the loss correlated with abnormal fecal α1‐antitrypsin excretion. In the absence of gastrointestinal blood loss or contamination of the 99mTc‐HSA with free pertechnetate, significant activity accumulating in the gastrointestinal tract and moving with its contents is thought to represent 99mTc‐HSA leakage into bowel lumen. Abdominal imaging with 99mTc‐HSA has a low radiation burden to the patient and is readily available, relatively inexpensive, and easily performed. It can be used as a screening test for the detection of protein‐losing enteropathy. It also offers the potential of being able to localize the site of protein loss visually within the gastrointestinal tract.
A Controlled Trial on Utility of Oral Zinc Supplementation in Acute Dehydrating Diarrhea in Infants
Authors: Sachdev H. P. S. , Mittal N. K. , Mittal S. K. , Yadav H. S.
Published: January 2024

A controlled, randomized trial was conducted in 50 infants with acute dehydrating diarrhea to evaluate the effect of oral zinc supplementation in acute diarrhea. After completion of rehydration, 25 infants in Group A received oral zinc sulfate (20 mg elemental zinc twice daily) and an equal number in Group B were given placebo (glucose). Both groups were comparable with respect to various initial characteristics including nutritional status, diarrhea! disease, serum alkaline phosphatase, and serum and rectal mucosal zinc content. During therapy all the assessed parameters of zinc status (serum alkaline phosphatase and serum and rectal zinc) recorded significant elevation and reduction in Groups A and B, respectively. At recovery the zinc status of Group A was significantly better and was nearer that of healthy controls. The diarrheal duration and frequency in the zinc‐supplemented group were lower, but the differences were not significant (0.05 < p < 0.1). However, when only subjects with relatively severe initial zinc depletion (rectal zinc lower than the 15th percentile of healthy controls: 11 in Group A and 14 in Group B) were considered, the diarrheal duration and frequency were significantly (p < 0.05 and p < 0.01. respectively) lower in the zinc‐supplemented cases. Weight gain in both groups was similar. It is concluded that oral zinc administration in acute diarrhea can replenish body zinc status and this may shorten the diarrheal duration and frequency in children with relatively severe zinc depletion.
Comparison of Efficacy of a Glucose/Glycine/Glycylglycine Electrolyte Solution Versus the Standard WHO/ORS in Diarrheic Dehydrated Children
Authors: Pizarro Daniel , Posada Gloria , Mahalanabis Dilip , Sandi Leyla
Published: January 2024

It was hypothesized that a mixture of glucose and amino acids enhances sodium and water absorption and therefore diminishes the volume of oral rehydration solution, stool output, and duration of diarrhea. To investigate this hypothesis, the efficacies of two oral rehydration solutions (ORS) were compared, one containing (mmol/L): Na+ 90, K+ 20, Cl+80, citrate 10, glucose 67, glycine 53, and glycylglycine 30, yielding an osmolality of 350 mosmol/kg H2O, and the other, the standard ORS recommended by the World Health Organization, containing the same electrolyte concentration and only glucose 110 mmol/L, yielding 310 mosmol/kg H2O. The study group comprised 31 infants and small children for group A (receiving solution A, the glucose/glycine/glycylglycine‐based ORS) and 31 patients for group B (receiving solution B, the standard WHO/ORS). There were no significant differences between the groups in age, fluid loss, or dehydration, or between the groups with respect to clinical outcome, mean time to achieve rehydration, mean percent body weight gain, and serum electrolyte composition. The only statistically significant difference was the mean time between admission and the last diarrheic stool. The glycylglycine/glycine/glucose electrolyte solution was found to be suitable for rehydration, but not to have an advantage over the standard WHO/ORS.
Enhancement of Neonatal Somatic and Hepatic Growth by Orally Administered Epidermal Growth Factor in Rats
Authors: Berseth Carol Lynn , W. Go Vay Liang
Published: January 2024

Previous studies have shown that orally administered epidermal growth factor (EGF) enhances neonatal intestinal growth and that it may be absorbed in the intestine and bound by other organs. This study investigated whether the ingestion of EGF would be associated with growth of nonintestinal organs. In short‐term studies, 87 newborn rat pups were fed artificial formula containing various concentrations of EGF for 39 h. In long‐term studies, suckling newborn pups were fed EGF or an equal volume of distilled water for 5 days. The pups fed formula containing EGF for 39 h had a significant increase in hepatic incorporation of [3H]thymidine into DNA compared with pups not fed EGF. By 5 days of age, 13 pups fed EGF had heavier livers that contained more DNA and RNA compared with 14 control pups. Moreover, EGF‐fed pups had heavier hearts and kidneys than pups fed no EGF. These data suggested that ingestion of EGF is associated with the growth of nonintestinal organs.
Intestinal Duplication in a Patient with Neurofibromatosis
Authors: Berezin Stuart , Fakhry Joseph , Newman Leonard J. , Davidian Marianna , Slim Michael S.
Published: January 2024

A 5‐year‐old boy with neurofibromatosis is described, whose symptoms of abdominal pain and vomiting were due to an intestinal duplication. Intestinal duplication has not been previously reported as a cause of gastrointestinal symptoms in neurofibromatosis. The utilization of ultrasound and computerized tomography scan as useful modalities for the diagnosis of duplication of the bowel is described.
Wilson's Disease Associated with Pancreatitis
Authors: Weizman Zvi , Picard Elie , Barki Yehiel , Moses Shimon
Published: January 2024

A 12‐year‐old boy presented with a 2‐month history of abdominal pain and distention. A diagnosis of Wilson's disease was established, and D‐penicillamine therapy was initiated. An associated pancreatitis was diagnosed on presentation, based on elevated serum amylase and an enlarged pancreas ultrasonically. Subsequently, an 18‐month follow‐up disclosed no abdominal pain, with repeatedly normal serum amylase level and a normal pancreas on ultrasonography. Since abdominal pain is a common symptom in Wilson's disease on presentation, this possibility should be considered in untreated patients. It is concluded that pancreatitis may be associated with Wilson's disease, possibly because of copper deposition in the pancreas, and is probably responsive to copper chelation therapy.
The Impact of Nutrition in Cystic Fibrosis
Authors: Gaskin Kevin John
Published: January 2024

A high energy intake, compensating for mal‐absorption, and the energy cost of lung disease, lung infections, and the underlying metabolic abnormality, is required to ensure normal growth in patients with cystic fibrosis. This goal can be readily achieved by adherence to a high quantity, normally balanced diet (with 40% of the energy as triglycerides of long‐chain fatty acids). In contrast, this goal cannot be reached in the majority of patients adhering to low‐fat and thus low‐energy‐containing diets, which almost inevitably lead to malnutrition with wasting and stunting. The prevention of malnutrition may well have considerably enhanced the prognosis of patients at one clinic. Further work is needed to define the interrelationship of nutrition and lung disease, and to define the appropriate nutrient requirements induced by the lung disease per se, recurrent infections, and the underlying disease process.
Efficacy of Pancreatic Enzyme Supplementation in Children with Cystic Fibrosis
Authors: Ansaldi‐Balocco Nicoletta , Santini Bruna , Sarchi Claudio
Published: January 2024

Two studies were performed in children with cystic fibrosis (CF) to compare the efficacy and tolerability of pancreatic enzymes prepared as enteric‐coated microspheres with that of a conventional enzyme preparation. The parameters evaluated included the following: fecal fat excretion, coefficient of fat absorption, daily caloric intake, percent of diet as fats, proteins and carbohydrates, increase in height and weight, frequency and consistency of stools, palatability of the preparation, and patient compliance. The first study was an open randomized crossover trial of an enteric‐coated microsphere preparation (Pancrease) versus conventional pancreatin given alone or with cimetidine. With Pancrease compared to conventional pancreatin, a significant improvement was observed in all the digestive parameters in addition to better patient compliance. In the second study, the response of a group of CF patients given Pancrease for at least 3 months (3–67 months) was retrospectively compared with the response to conventional pancreatin, which had been given to the same patients from 10 months to 8 years earlier. In comparison to conventional pancreatin, Pancrease provided better digestive efficacy and greater increases in the growth rate of teenage patients. With Pancrease, the number of daily dosage units is decreased even when fat intake is increased. No adverse reactions were seen with either of the enzyme preparations used in these studies.
Nutritional Support for Pediatric Patients with Inflammatory Bowel Disease
Authors: Kleinman Ronald E. , Balistreri William F. , Heyman Melvin B. , Kirschner Barbara S. , Lake Alan M. , Motil Kathleen J. , Seidman Ernest , Udall John Nicholas
Published: January 2024

Pediatric patients with ulcerative colitis and Crohn's disease often suffer from malnutrition and growth failure. This is particularly true in pubertal children. Chronic insufficient nutrient intake is most often the cause of growth failure. Both parenteral nutrition and defined enteral formulas are available to rehabilitate patients with malnutrition and growth failure. Assessment of nutritional status and growth and the use of parenteral nutrition and defined enteral formulas to reverse malnutrition, growth failure, and inflammation in pediatric patients with inflammatory bowel disease are discussed.
Rapid versus Gradual Refeeding in Acute Gastroenteritis in Childhood
Authors: Hjelt Karsten , Paerregaard Anders , Petersen Werner , Christiansen Lene , Krasilnikoff Peter Andreas
Published: January 2024

Fifty‐two children aged 6–46 months (mean 19 months), hospitalized for acute gastroenteritis (GE), were randomized after oral rehydration to receive 7 days of either traditional gradual refeeding (GR) or rapid refeeding (RR), the latter consisting of a full‐strength lactose‐limited diet, including lactase‐treated whole milk. The study focused on the effect of a high energy intake, excluding possible negative effects of lactose. Both dietary regimens were well tolerated, the only difference in the clinical symptoms between the two regimens being a higher stool frequency within the RR group(p < 0.02). The total energy intake, as well as energy derived from fat and protein, was significantly higher in the RR than in the GR group (p < 0.0001). The mean daily energy intake of the latter group never reached recommended daily allowance (RDA) levels, while that of the RR group did on day 5. Moreover, during the whole period of dietary regimen, the RR group exceeded the RDA protein requirements (mean intake ranged 175–252%), while the GR group did not reach this RDA level until day 4. Milk was a major source of energy in the RR group, providing 47–59% of the daily energy intake. The short‐ and long‐term weight gains in the RR group were only a little higher than those of the GR group, the difference being insignificant. The chief asset of RR compared with GR was, therefore, psychological, in the sense that after rehydration the hospitalized child with mild to moderate GE can, by and large, be offered the quality and quantity of food and drink that he/she prefers without the fear of negative effects on the outcome.
Usefulness of Random Fecal α1‐Antitrypsin and Chymotrypsin Determinations in Children
Authors: Mbonda E. , Forget P. , Saye Z. , Leclercq‐Foucart J.
Published: January 2024

Random fecal chymotrypsin activity and fecal α1‐antitrypsin (FA‐1‐AT) concentrations were determined in 11 children with cystic fibrosis, 5 children with Crohn's disease, 9 children with chronic aspecific diarrhea, 85 children with acute gastroenteritis, and 54 control children. Cystic fibrosis patients showed only very low fecal chymotrypsin values that did not overlap with values obtained in patients with either acute or chronic diarrhea. When compared with our control group, a significant increase of FA‐1‐AT concentrations was found only in children with Crohn's disease. Normal values were found in all patients with either chronic aspecific diarrhea or cystic fibrosis, while 12 of 85 children with acute gastroenteritis showed FA‐1‐AT concentrations above the 95th percentile of control children. We conclude that diarrhea (either acute or chronic) does not significantly decrease the clinical usefulness of fecal chymotrypsin activity measurements in the diagnosis of pancreatic insufficiency, while acute (gastroenteritis) but not chronic (chronic aspecific diarrhea, cystic fibrosis) diarrhea can give rise to protein losing and FA‐1‐AT concentrations similar to those found in Crohn's disease.
Zinc Protoporphyrin Inhibits CO Production in Rats
Authors: Hamori Charles J. , Vreman Hendrik J. , Rodgers Pamela A. , Stevenson David K.
Published: January 2024

We studied the effect of zinc protoporphyrin (ZnPP) on in vivo total bilirubin production, as measured by the excretion rate of carbon monoxide (VeCO), in adult rats. A single subcutaneous dose of ZnPP (40 μmol/kg) suppressed the VeCO between 2–12 h posttreatment, with maximum suppression of approximately 20% by 4 h. The heme oxygenase activity in the liver and spleen of the ZnPP‐treated rats was significantly inhibited at 12 h compared with that of the controls. The in vitro inhibition of heme oxygenase activity by the addition of exogenous ZnPP to native rat liver and spleen tissue preparations was observed, as evidenced by suppressed CO production. This in vitro inhibition of heme oxygenase activity by ZnPP was further confirmed by finding decreased bilirubin formation, as measured directly by high‐performance liquid chromatography. This study demonstrates that ZnPP can inhibit in vivo liver and spleen heme oxygenase activity and suppress CO production in the rat, as well as inhibit in vitro heme oxygenase activity in native rat tissues.
Blastocystis hominis Gastroenteritis in a Hemophiliac with Acquired Immune Deficiency Syndrome
Authors: Narkewicz Michael R. , Janoff Edward N. , Sokol Ronald J. , Levin Myron J.
Published: January 2024

Blastocystis hominis is an enteric protozoan that has occasionally been associated with gastrointestinal illness in man but is not considered an opportunistic pathogen. We describe a 16‐year‐old hemophiliac with acquired immune deficiency‐related complex in whom upper gastrointestinal symptoms in conjunction with high densities of B. hominis in duodenal secretions and stool developed. Furazolidone treatment was associated with eradication of the organism and improvement in clinical symptoms. One month later, the patient had invasive Candida albicans esophagitis and died 2 weeks later of complications of acute pancreatitis. In immunocompetent individuals surveyed in Colorado the frequency of identification of B. hominis in stool concentrates was not different between asymptomatic and symptomatic persons. We suggest that B. hominis may be an opportunistic pathogen in immunocompromised patients and should be sought in duodenal secretions and feces when gastrointestinal symptoms are present.
Total Colonic Aganglionosis
Authors: Dykes Evelyn H. , Guiney E. J.
Published: January 2024

All cases of total colonic aganglionosis (TCA) diagnosed in Dublin, Ireland, from 1978 to 1986 are reviewed. Nine patients were found to have TCA, and eight have undergone definitive surgery. There have been no deaths, and all patients are continuing to thrive. The diagnostic and operative features of the disease are described.
Inhibition of Cholera‐Toxin‐Stimulated Intestinal Secretion by CGS 9343B in Rats
Authors: Fedorak Richard N. , Kotake Alvin , Douglas Frank , Chang Eugene B.
Published: January 2024

In this study, the effect of CGS 9343B on cholera‐toxin‐stimulated intestinal secretion in rats was determined using in vivo isolated loops. This recently developed compound is a potent and specific inhibitor of calmodulin, but not of protein kinase C. At a luminal dose of 15 mg/kg, CGS 9343B has little effect on basal intestinal absorption but completely inhibited the secretory effects of cholera toxin. The less specific calmodulin inhibitor, trifluoperazine, has a similar antisecretory effect, but unlike with CGS 9343B, severe toxicity was noted at luminal doses of 7.5 mg/kg. In animals where two intestinal loops were created, one with cholera toxin alone and the other with CGS 9343B and cholera toxin, significant inhibition of secretion was observed in both loops, consistent with a systemic effect of this compound. Finally, both CGS 9343B and trifluoperazine inhibited choleratoxin stimulated increases in mucosal cyclic AMP content, whereas basal levels were unaffected. We conclude that CGS 9343B significantly inhibits choleratoxin‐stimulated intestinal secretion, possibly by inhibition of calmodulin‐dependent adenylate cyclase activity. Its lack of major toxicity at therapeutic doses makes this compound potentially useful for the treatment of enterotoxigenic diarrheal diseases.
Pitfalls in Diagnosing Galactosemia
Authors: Sokol Ronald J. , McCabe Edward R. B. , Kotzer Ann Marie , Langendoerfer Sharon I.
Published: January 2024

Newborn galactosemia screening programs using the fluorescence spot test to detect red cell galactose‐1‐phosphate uridyltransferase activity are prone to inaccuracy if the screened infants have received blood transfusions. We describe an infant with galactosemia who received packed red cell transfusions in the first few days of life and was misdiagnosed after an initial positive screening test result. Although the patient was thought to have cytomegaloviral hepatitis, a percutaneous liver biopsy helped direct the evaluation toward identifying the galactosemia carrier state in both parents. This case report illustrates the need for careful consideration of the patient's history of transfusion of blood products when evaluating newborn screening results.
Pneumococcal Colitis
Authors: Brown Bruce P. , Sato Yutaka , Mock Donald , Mitros Frank , Franken E. A.
Published: January 2024

Gastrointestinal symptoms and signs have been reported in association with pneumococcal infection for many years. Only rarely has Streptococcus pneumoniae been reported as a bowel pathogen. We report the clinical, radiographic, colonoscopic, and pathologic features of a case of pneumococcal colitis in an otherwise healthy 3.5‐year‐old female.
The Effect of Aminophylline on Gastrointestinal Blood Flow and Oxygen Metabolism in the Conscious Newborn Lamb
Authors: Cronin C. M. G. , Canose J. , Buchanan D. , Holzman I. R.
Published: January 2024

Aminophylline, widely used in the neonatal period, has been reported to be associated with necrotizing enterocolitis. We hypothesized that aminophylline might alter gastrointestinal blood flow and oxygen delivery. The effects of this drug on gastrointestinal (GI) blood flow and oxygen delivery, consumption, and extraction were examined using the radioactive microsphere method in ten chronically catheterized, unanesthetized lambs aged 5–12 days. While cardiac output and systemic oxygen consumption did not change, we found statistically significant increases (p < 0.05) in small intestinal blood flow as a function of lamb weight and as a percentage of cardiac output. Splanchnic oxygen consumption also increased significantly. The oxygen reserve available to the GI tract can be compromised by aminophylline at levels similar to those used in the human neonate for treatment of apnea.
Enteropathy and Renal Involvement in an Infant with Evidence of Widespread Autoimmune Disturbance
Authors: Mitton S. G. , Mirakion R. , Larcher V. F. , Dillon M. J. , Walker‐Smith J. A.
Published: January 2024

An 8‐month‐old infant presented with a 1 month history of protracted diarrhea, vomiting, and weight loss. Small intestinal biopsy showed a flat mucosa and there was no clinical improvement with gluten, cow's milk protein, and disaccharidase‐free diet. Serial testing for autoantibodies revealed persistent autoantibodies to gut epithelial cells and to renal brush borders; on two occasions, atypical liver‐kidney microsomal antibodies were detected. Treatment with steroids produced clinical improvement but the patient finally succumbed with a combination of gut and renal dysfunction. The widespread nature of the antibodies, with clinical involvement of gut, liver, and kidney, suggest an underlying autoimmune mechanism for the pathogenesis of the condition. Serial autoantibody measurements may provide a means to monitor the disease progress and may be a guide to treatment.
Measurement of Plasma Prednisolone Level to Evaluate a Prednisone Treatment Failure in an Adolescent with Crohn's Disease
Authors: Milov David E. , Hill Malcolm , Andres Joel M. , Cynamon Harry A. , Hendeles Leslie
Published: January 2024

A 14‐year‐old boy experienced multiple hospitalizations because of symptoms due to Crohn's disease involving the stomach, duodenum, and ileum. He maintained that oral corticosteroids were not effective for control of his symptoms. However, i.v. corticosteroids always relieved his symptoms. To resolve the question of noncompliance versus altered corticosteroid absorption or metabolism, our patient underwent an oral prednisone absorption study. Prednisolone, the active metabolite of prednisone, was measured in his plasma using a high‐pressure liquid chromatography technique. The results led to the discovery of an elaborate deception by the patient and his subsequent need for psychotherapy. This report documents the importance of measuring plasma prednisolone concentrations to diagnose noncompliance, especially in adolescents who are overly concerned about their body image.
Effects of Two Levels of Intake of Chloride, Potassium, and Calcium on Mineral and Acid‐Base Metabolism in Premature Infants
Authors: Mosca F. , Manz F. , Primi D. , Marini A.
Published: January 2024

Growth (weight, length, mid‐upper arm circumference), acid‐base status, serum electrolyte levels, and selected parameters of urine (creatinine and electrolyte levels, urine pH, renal net acid excretion, levels of metabolites of aldosterone) were determined serially from the 10th to the 32nd days for 4 weeks in 21 premature infants (birth weight, 1,100–2,000 g) fed either formula A or formula B (formula B was formula A supplemented with chloride, potassium, and calcium). Premature infants fed formula B showed a higher weight gain (31 versus 28.2 g/day), a higher increment of middle‐upper arm circumference (0.31 versus 0.24 cm/week), and a decreased renal net acid excretion (1.24 versus 1.92 mEq/ kg/day). Almost all premature infants fed formula A and some with a high growth rate receiving formula B showed hypochloruria corresponding to chloride deficiency. Premature infants fed unsupplemented humanized formulas may have an inadequate intake of minerals.
Small Intestinal Development in Growth‐Retarded Fetal Sheep
Authors: Avila C. G. , Harding R. , Rees S. , Robinson P. M.
Published: January 2024

The functional maturity of the gastrointestinal system is essential for the survival of the neonate. The effects of reducing placental size and the ensuing fetal growth retardation on the development of the small intestine (SI) of the sheep fetus were investigated. At 140 days of gestation (term is 147 days), fetal body weight, gastrointestinal weight (from the abomasum to the rectum, i.e., the gut), SI weight, and SI length in the growth‐retarded fetuses (n = 6) were significantly reduced (p < 0.05) when compared with age‐ and breed‐matched control fetuses. The SI weight was disproportionately reduced as a fraction of the gut weight or SI length (p < 0.01). The proximal SI showed marked reductions in the thickness of the wall, muscularis externa, mucosa, villus height, and crypt depth (p < 0.003). Densities of villi and crypts were also reduced (p < 0.02). The crypt‐to‐villus ratio was maintained. Glycogen was accumulated basally in the villus epithelial cells, and their nuclei were located apically. These characteristics are comparable to those of much younger fetuses. In the distal SI, interanimal variation was large; however, the mean thickness of the wall, the muscularis externa, the mucosa, and the villus height were all reduced (p < 0.05). In this region, the density of villi and crypts, the pattern of glycogen accumulation, and the position of the nuclei did not markedly differ between growth‐retarded and control fetuses. Villus cell density was increased (p < 0.01). Thus, fetal growth retardation due to placental insufficiency affects the proximal and distal SI with different degrees of severity, with thinning of the SI wall, a reduction of the surface area available for absorption, reduced epithelial regenerative capacity, and possibly a retardation of normal epithelial maturation.
Electrogenic Sodium Transport in the Developing Rabbit Cecum
Authors: Potter Gregory D. , Burlingame Susan M. , Cragoe Edward J. , Seilin Joseph H.
Published: January 2024

The adult rabbit cecum absorbs Na by an electrogenic, C1‐independent process that is inhibited by the amiloride analogue phenamil. In the colon, this transport system is unique to the cecum. Because the developing colon exhibits many specialized functions, we have now examined the development of electrogenic Na transport in the newborn rabbit. Cecal tissue from animals between 7 and 38 days old was mounted in modified Üssing chambers for measurement of Na and C1 flux (J) and the short‐circuit current (Isc). At 7–10 days, the (Isc) was only 0.8 ± 0.18 μEq cm ‐2 h‐1, but by 35–38 days it had increased to 4.6 ± 0.79 μEq cm ‐2 h ‐1. The Na transport increased in parallel with Isc; JNanet = −0.49 ± 0.33 μEq cm‐2 h‐1 at 7–10 and 4.7 ± 1.6 (μEq cm‐2 h‐1 at 35–38 days. The Na transport was not inhibited by phenamil (10‐4 M) at 7–10 days, but by the 35–38‐day period, phenamil reduced the JNanet to 1.6 ± 0.37 μEq cm‐2 h‐1. The CI secretion was not stimulated by theophylline in the 14–16‐day‐old cecum, nor was Na absorption stimulated by epinephrine. The rabbit cecum does not demonstrate electrogenic Na absorption until after the 4th week of life.
Sulfasalazine‐Induced Hepatotoxicity in Children with Inflammatory Bowel Disease
Authors: Boyer Debra L. , Li B Ulysses K. , Fyda John N. , Friedman Roger A.
Published: January 2024

A case of severe sulfasalazine‐induced hepatotoxicity is reported in a 14‐year‐old girl with Crohn's disease. Fourteen days after beginning sulfasalazine, she developed a systemic reaction characterized by high fever, maculopapular rash, lymphadenopathy, abdominal pain, and malaise, with tender hepatomegaly and elevated liver functions, leukemoid reaction, with eosinophilia, and immune complexes. She responded promptly to high‐dose intravenous steroids with complete recovery. This case was compared to the three similar pediatrie cases in the literature. The latent period was 11–19 days between sulfasalazine therapy and the onset of high fever, generalized lymphadenopathy, and pruritic maculopapular rash that spared palms and soles and resolved with desquamation. All children had hepatotoxicity indicated by tender hepatomegaly, elevated liver enzymes, and histologie inflammation on liver biopsy. The similarity between these features and those of other sulfa‐induced hepatotoxic reactions suggests that the sulfapyridine moiety is the etiologic agent in these hypersensitivity reactions. Those children with circulating immune complexes responded well to steroids. Proper therapy for this rare but severe idiosyncratic reaction includes prompt recognition and discontinuation of sulfasalazine, and high‐dose cortico‐steroid therapy.
Milk Lactose Malabsorption in Gabon Measured by the Breath Hydrogen Test
Authors: Gendrel Dominique , Dupont Christophe , Richard‐Lenoble Dominique , Gendrel Christine , Nardou Marline , Chaussain Michèle
Published: January 2024

It is usual to consider that the greatest part of the black African population is lactose intolerant. Also, milk lactose malabsorption was studied by a breath hydrogen technique in 87 Gabonese children and 20 Gabonese adults (central Africa). The prevalence of malabsorption was 64.2% in rural schoolchildren, 65% in the urban hospitalized, and 60% in adults. Twelve children and six adults had clinical symptoms after a lactose load. All subjects were Bantus, with no tradition of consuming dairy products. These data must be considered in programs of nutritional support in Africa.
Determinants of Arm Muscle and Fat Accretion During the First Postnatal Month in Preterm Newborn Infants
Authors: Georgieff Michael K. , Amarnath Uma M. , Mills Marla M.
Published: January 2024

We measured arm muscle and fat areas in 22 preterm appropriate for gestational age infants at birth (mean + 1 SD birth weight: 1,640 + 484 g; gestational age: 31 + 2 weeks). Birth arm muscle and fat areas correlated significantly with gestational age (arm muscle: r = 0.86; p < 0.001; arm fat: r = 0.75; p < 0.001) and with birth weight. Deviations of birth weights from gestational age means (birth weight z‐scores) were related more to variations in arm muscle area (r = 0.69; p < 0.001) rather than arm fat area (r = 0.44; p = 0.04). Sixteen infants were followed over 4 weeks. They were most physiologically unstable (mean Physiologic Stability Index score = 5.3 + 3.5) during the first postnatal week when they also all lost weight. Their mean arm muscle area decreased significantly during the first week by > 10%, whereas the mean arm fat area remained unchanged. First week arm muscle losses were directly correlated with the lack of protein intake (r = 0.52; p < 0.05). The regression equation predicted a protein intake of 4.06 g/kg/day (95% confidence interval: 2.3–6.4) to prevent first week muscle loss. Enteral intake and weight gain were established after week I, accompanied by a significant reduction in physiologic instability (PSI score = 1.9 + 1.9; p < 0.001). Over the subsequent 3 weeks, arm muscle area increased more (138 + 58 mm2) than arm fat area (59 + 36 mm2; p < 0.001) and weekly rates of arm muscle and fat accretion became more dependent on energy intake and postnatal age, rather than protein intake or postconceptional age. Infants fed high‐fat, low‐protein own mother's milk (n = 7) did not have more fat accretion or less muscle accretion than infants fed significantly lower fat and higher protein content formulas (n = 9). If extrapolatable to total body stores, this study demonstrates that the major determinants of muscle and fat accretion in preterm infants are different at the time of birth, during periods of physiologic instability, and during the early period of postnatal growth.
Remodeling of the Rat Small Intestinal Mucosa During the Suckling Period
Authors: Trahair Jeffrey F.
Published: January 2024

The development of the suckling rat small intestine has been reexamined to investigate changes to cell kinetics and morphology more fully. In this period of rapid growth and reorganization, villi and crypts steadily increase in size in proximal regions; however, in distal regions a dramatic shortening of villi occurs at about 15 days. The result of this remodeling is that the normal adult proximodistal gradient of villus height is established. At 15 days, migration rates in both regions increase dramatically. In proximal regions, the extent of 24‐h migration of thymidine‐labeled enterocytes is dependent on villus height. In distal regions, the increase in migration rate can be explained by the shortening of the villi. The extent of 24‐h migration was not related to villus height. On the basis of these results, it is suggested that care must be taken in drawing conclusions relating any parameter to assumed distal region cell renewal patterns. This is because changes in the relative proportions of cell types present apparently can be brought about by villus deletion, independent of cell renewal.
Endocytosis and Processing of Protein by Isolated Villus and Crypt Cells of the Mouse Small Intestine
Authors: Heyman M. , Crain‐Denoyelle A. M. , Desjeux J. F.
Published: January 2024

A small but antigenically significant amount of intact food proteins is absorbed at the intestinal level. These proteins are transported by an endocytotic mechanism mostly in degraded form via the lysosomal pathway of the enterocytes, but those that escape lysosomal degradation appear to be a small fraction of potential food antigens that enter the body. To establish whether protein uptake and processing are similar between mature and immature enterocytes, villus and crypt cells were isolated from the small intestine of adult mice. In each fraction, we measured lysosomal cathepsin activities and cell binding, uptake, and degradation of horseradish peroxidase (HRP). The following results were observed: (a) mice exhibited an increasing villus‐to‐crypt gradient of cathepsins B and D; (b) binding, internalization and degradation of HRP were twice as high in crypt cells as in villus cells, indicating that the rate of endocytosis in crypt cells doubled; and (c) the percentage of intracellular intact and degraded HRP remained similar in both cell types, suggesting that lysosomal degradation was not the limiting step of endocytosis. Chloroquine and ammonia increased intracellular intact HRP, but monensin did not. These results suggest that intestinal villus and crypt cells do not transfer proteins at the same rate. This may have important biological consequences in physiopathological states in which the crypt/villus ratio is greatly increased.
Sulfasalazine Hypersensitivity with Hepatotoxicity, Thrombocytopenia, and Erythroid Hypoplasia
Authors: Gremse David A. , Bancroft John , Moyer M. Susan
Published: January 2024

Sulfasalazine, a commonly prescribed drug for the treatment of inflammatory bowel disease, can cause an allergic reaction with hepatotoxicity, lymphadenopathy, and rash. In addition, many hematologic complications have been observed with sulfasalazine, including aplastic anemia, thrombocytopenia, and erythroid hypoplasia. However, thrombocytopenia in association with sulfasalazine hypersensitivity has not been previously reported. We report a child who developed severe thrombocytopenia and anemia during a hypersensitivity reaction to sulfasalazine.
Expression of Class II MHC Antigens in the Intestinal Epithelium of Pediatric Celiac Disease
Authors: Bonamico M. , Mazzilli M. C. , Morellini M. , Vania A. , Carpino F. , Nicotra M. R. , Natali P. G.
Published: January 2024

Class II MHC antigen expression in the intestinal epithelium of 28 small bowel biopsies from 23 celiac patients were studied by means of indirect immunofluorescence and immunoperoxidase using monoclonal antibodies. Patients were divided on the basis of diet into two subgroups: 15 subjects on a gluten‐containing diet (GCD) and 13 on a gluten‐free diet (GFD). The control group included 10 pediatric subjects with normal intestinal mucosa who underwent intestinal biopsy for chronic diarrhea or short stature. DR antigens and invariant chain were expressed in all patients, regardless of the diet, as well as in the control subjects. DQ was found in one patient only on GCD. DP antigens were present in 12/15 patients on GCD, and in 2/13 on GFD (Fisher's exact test, p = 8.8 x 10‐4), as well as in 3/10 control subjects. In 4/5 celiac patients, DP antigens, which were undetectable on GFD, could be demonstrated after gluten challenge. The results of the study show that DR antigens are expressed by intestinal mucosa of celiac patients independently of their gluten exposure and that DQ antigens are consistently undetectable. Statistically significant differences in expression of DP antigens on enterocytes of celiac patients on GCD and their neoexpression after gluten challenge may represent a basis for further investigation.
Intestinal Permeability to Inert Sugars and Different‐Sized Polyethyleneglycols in Children with Celiac Disease
Authors: Stenhamma L. , Fälth‐Magnusson K. , Jansson G. , Magnusson K. ‐E. , Sundqvist Tommy
Published: January 2024

Intestinal permeability was measured in a total of 42 children, 29 of whom had celiac disease. The celiac children were studied at presentation, during gluten‐free diet, and/or at gluten challenge. The permeability was assessed by oral lactulose/L‐rhamnose in all 42 children and also by different‐sized polyethylene glycols (PEG) in 36 children. Results were compared with the findings of small intestinal biopsy. The mean of the permeability tests in children with enteropathy was significantly abnormal compared with the result in children with a normal mucosal morphology. The lactulose/L‐rhamnose test and the PEG test gave equivalent results in the same child. In the celiac children abnormal permeability properties at presentation normalized during gluten‐free diet and reappeared during gluten challenge. It is concluded that measurement of intestinal permeability may be a valuable tool in monitoring children with celiac disease, preferably when serial measurements are available in the same child.
Extent of Lactose Absorption in Children with Active Celiac Disease
Authors: Roggero P. , Ceccatelli M. P. , Volpe C. , Donattini T. , Giuliani M. G. , Lambri A. , Tavani E. , Careddu P.
Published: January 2024

We have estimated lactose absorption indirectly by the breath H test to see if disaccharide exclusion is necessary for untreated celiac children. Lactose at 2 g/kg body weight (maximum 50 g) was administered to 42 infants and children (ranging in age from 9 months to 12 years) with flat small intestinal mucosa. Later, different amounts of lactose were given to determine the quantities tolerated and absorbed. One hundred percent of patients expired hydrogen more than 20 ppm over the baseline after an oral lactose load of 2 g/kg (maximum 50 g). Thirty‐eight percent of them did not tolerate this quantity. Thirty‐seven subjects aged 0–6 years absorbed and tolerated 0.5–1.5 g/kg (5–12.5 g total), and five patients aged 6–12 years absorbed and tolerated 0.5–0.6 g/kg (12–16.2 g total). We conclude that in many subjects with untreated celiac disease, lactase activity is sufficient for absorption and tolerance of the amounts of lactose present in 250–300 ml cow's milk. Because of lactose's nutritional value, it should not be excluded unless necessary.
Lactose Malabsorption in Children with Symptomatic Giardia lamblia Infection
Authors: Mantovani M. Pettoello , Guandalini S. , Ecuba P. , Corvino C. , di Martino L.
Published: January 2024

An investigation was carried out on 61 children suffering from symptomatic giardiasis with the object of verifying the incidence and entity of lactose malabsorption. Furthermore, the possibility of a substitutive yogurt diet was verified in the lactose malabsorbers. The subjects, all children older than 1 year, were studied according to a schedule that included a lactose hydrogen breath test (BT) performed prior to therapy and a further BT 60 days following therapy. The subjects were divided in two groups: group A, 40 children, received a dose of 250 ml of cow's milk; group B, 21 children, received a stress dose of 2 g/kg lactose (max 50 g). Those subjects who were lactose malabsorbers at the 60 day follow‐up were also given a BT at 75 days, and in the case of persistent malabsorption, a further BT was performed after 24 h with the administration of yogurt (450 g containing 12.1 g of lactose). Furthermore, 40 subjects matched for age and sex but without any GI complaints served as controls. The results showed lactose malabsorption to be frequent in children with Giardia lamblia symptomatic infection. According to the BT with a standard lactose load, all patients were malabsorbers; when testing lactose absorption with 250 ml of cow's milk, 45% of patients were found to be malabsorbers. In the latter subjects, the oral load of yogurt was uniformly well tolerated and gave rise to no H2 increment on the BT. We conclude that the occurrence of lactose malabsorption of nutritional relevance is common in children suffering or having suffered from giardiasis. Such children, however, may benefit from the administration of yogurt, whose lactose appears to be well absorbed.