bluuebunny/crossref_metadata_2025_split huggingface dataset:
Abnormal Low Ratio of Cholic Acid to Chenodeoxycholic Acid in a Cholestatic Infant with Severe Hypoglycemia
Authors: Kimura Akihiko , Yuge Ken , Yukizane Shigenori , Kage Masayoshi , Nittono Hiroshi , Mahara Reijiro , Kurosawa Takao , Tohma Masahiko 
Published: January 2024

SummaryWe report a premature infant with severe hypoglycemia (serum glucose: 6 mg/dl) and cholestasis (serum total bile acids: 211.55 μmol/L) caused by hypoplasia of the interlobular bile ducts. This patient had developed intracranial hemorrhage and sepsis while undergoing treatment for hypoglycemia. As a result of endocrine evaluation, we made a diagnosis of idiopathic panhypopituitarism, congenital absence or hypoplasia of the pituitary gland. Moreover, we found abnormal bile acid profiles: The ratio of cholic acid to chenodeoxycholic acid was abnormally low in serum (0.04) and in biliary bile (0.33). However, 3α,7α,12α‐trihydroxy‐5β‐chole‐stan‐26‐oic acid and bile alcohols were not detected. We therefore suspected that the severe cholestasis and abnormal bile acid profiles in the serum and biliary bile in this patient were related to physiologic immaturity of the enterohepatic circulation of bile acids and immaturity of hepatic 12α‐hydroxylation.
Transient Gluten Hypersensitivity
Authors: Iacono G. , Nocerino A. , Guandalini S. , Carroccio A. , Cavataio F. , Balsamo V. 
Published: January 2024

SummaryWe report a case of transient gluten hypersensitivity, demonstrated by jejunal histology at diagnosis, normalization after gluten‐free diet, and acute clinical and histological relapse after a challenge with gluten powder at the age of 1 year, resembling that observed in cow's milk protein intolerance. Subsequent provocation tests did not show any alteration. Cases of supposed transient gluten hypersensitivity are rarely reported; our patient is characterized by the acute reaction to gluten challenge associated with a damaged histological picture and depressed levels of complement.
Alanine‐Based Oral Rehydration Solution
Authors: Sazawal S. , Bhatnagar S. , Bhan M. K. , Saxena S. K. , Arora N. K. , Aggarwal S. K. , Kashyap D. K. 
Published: January 2024

SummaryThis randomized, double‐blind trial determined whether adding 90 mmol/L of alanine with a reduction in glucose to 90 mmol/L (alanine ORS) improves the efficacy of the standard oral rehydration solution (WHO‐ORS). One hundred twenty‐nine males aged 3–48 months with weight for length ≥70% of NCHS, diarrheal duration ≤96 h, and clinical signs of mild to moderate dehydration were randomly allocated to either treatment group. During 0–6 h of treatment, ORS was offered at 120 ml/kg for rehydration without food or water. Beyond 6 h, ORS was offered as a volume‐to‐volume replacement for stool losses and a mixed diet of uniform composition was offered in amounts standardized for body weight. The most frequently isolated pathogens in alanine ORS and WHO‐ORS groups were rotavirus (42 and 48%, respectively) and enterotoxigenic Escherichia coli (15 and 12%, respectively). In the 0–6 h period when food was withheld, median urine output in ml/kg (8;5, p < 0.05) and percentage decrease in total serum solids (9:7%, p = 0.06) was significantly greater in alanine ORS than in WHO‐ORS; median ORS intake and stool output were marginally lower in the alanine group but the differences were statistically not significant. Between 0 h and recovery, although the median values for duration of diarrhea (56.5 and 65.0 h), ORS consumption (260 and 323 ml/kg), and stool output (188.4 and 216.3 g/kg) were lower in the alanine ORS group, these differences with the WHO‐ORS group were not statistically significant. Our findings are consistent with improved efficacy of WHO‐ORS following alanine fortification but the clinical benefits achieved in these mildly dehydrated cases fall short of what may be clinically meaningful and of relevance to public health
Serum Concentrations of Extracellular Matrix Components
Authors: Calçado Antonio , Trivedi Premila , Portmann Bernard , Mowat Alex P. 
Published: January 2024

SummaryLaminin, hyaluronic acid (HA), procollagen III amino‐terminal propeptide (PIIINP), and procollagen I carboxy‐terminal propeptide (PICP) concentrations were measured by radioimmunassay or radiometric assay in sera from 21 patients with glycogen storage disease (GSD). Laminin was increased in 16 of 29 samples from the six children with GSD I, 25/43 from the seven with GSD III, and 3/19 from the eight with GSD IX; laminin correlated with serum aspartate aminotransferase and γ‐glutamyltransferase but not with serum triglycerides, cholesterol, lactate, or urate. HA was increased in 20% of samples from GSD I, 58% from GSD III, and in none from GSD IX; HA correlated with serum lactate and urate but not with liver function tests, serum cholesterol, or triglycerides. Serum PIIINP was increased in only eight samples and PICP in only one; children with poor linear growth had low PIIINP and PICP. Immunostaining studies of nine liver biopsies taken at diagnosis showed increased laminin and PIIINP staining in portal tracts, fibrous septa, and around sinusoids in periportal regions; children with a greater degree of immunostaining did not always show significantly higher values of serum laminin or PIIINP. We speculate that raised serum laminin may reflect fibrogenesis (but not necessarily established fibrosis) in response to tissue damage in GSD, raised HA may reflect disturbed sinusoidal endothelial cell function, and low PIIINP and PICP impaired somatic growth rather than intrahepatic pathology.
Measurement of Colonic Transit Time in Children
Authors: Casasnovas A. Bautista , Cives R. Varela , Jeremias A. Villanueva , Castro‐Gago M. , Cadranel S. , Sierra R. Tojo 
Published: January 2024

SummaryTransit times through the whole colon and its segments were measured in 10 healthy children and 14 children suffering constipation secondary to myelomeningocele. The subjects ingested radio‐opaque markers on three successive days, and on the fourth a plain abdominal roentgenogram was taken using fast film. In the healthy children, total colonic transit time (mean ± SD) was 37.8 ± 6.2 h, with segmental times of 10.8 ± 3.5 h for the right colon, 12.2 ± 2.7 h for the left, and 14.7 ± 2.1 h for the rectosigmoid; upper normal limits of 17.8 h for the right colon, 17.6 h for the left, 19.1 h for the rectosigmoid, and 50.2 h for the total colonic transit time were established. In the constipated children, the total transit time was 59.9 ± 5.4 h, with segmental times of 15.9 ± 2.3 h for the right colon, 18.9 ± 2.3 h for the left, and 25.0 ± 2.6 h for the rectosigmoid. The technique described is simple, is easy to use in clinical practice, and involves a lower radiation dose than other methods. It may prove useful for measurement of colonic transit time in suitable patients.
Realimentation of Dehydrated Children with Acute Diarrhea
Authors: Römer Hans , Guerra Marisa , Piña José M. , Urrestarazu María I. , García Doris , Blanco María E. 
Published: January 2024

SummaryA randomized, prospective study was performed on 73 male children between 3 and 14 months of age with mild to moderate dehydration caused by acute diarrhea, and who were rehydrated with WHO‐ORS, and then at 4 h after admission were fed either cow's milk or an experimental diet based on chicken, plantain, and coconut oil. There were no statistically significant differences in the admission characteristics. The group of children fed cow's milk had a higher total volume of diarrheic evacuations (p < 0.05). The average duration of diarrhea was 20 h shorter in the group of infants on the milk‐free formula. A significant correlation was observed between the volume of diarrhea and the WHO‐ORS consumed during the first 48 h, but not to the volume of diet intake.
Evaluation of Growth and Changes in Body Composition Following Neonatal Diagnosis of Cystic Fibrosis
Authors: Greer Ristan , Shepherd Ross , Cleghorn Geoffrey , Bowling Francis G. , Holt Terence 
Published: January 2024

SummaryEarly deficits in nutritional status that might require specific treatment and early response to nutritional therapy were studied longitudinally in 25 infants with cystic fibrosis (CF) diagnosed by neonatal screening, using anthropometric and research body composition methodology, and evaluation of pancreatic function. At the time of confirmed diagnosis (mean 5.4 weeks), body mass, length, total body fat (TBF), and total body potassium (TBK) were all significantly reduced. Following diagnosis and commencement of therapy there was a normalization of weight, length, and TBK by 6–12 months of age, indicating catch‐up growth. But in some individuals the response was incomplete, and as a group, mean total body fat remained significantly lower than normal at 1 year of age. Seven of 25 (28%) were pancreatic sufficient at diagnosis, and all but one had evidence of declining pancreatic function requiring the institution of pancreatic enzyme therapy during the next 1–9 months. The median age of commencement of enzyme therapy was 10 weeks (range 5 weeks to 11 months). These longitudinal assessments emphasize the dynamic changes occurring in absorptive function, body composition, and nutritional status following neonatal diagnosis of cystic fibrosis and may reflect previously described abnormalities of energy metabolism in this age group. Abnormal body composition is evident in most CF infants following diagnosis by neonatal screening but pancreatic damage may still be evolving. We suggest that early active nutritional therapy and surveillance for changes in pancreatic function are warranted in CF infants diagnosed by neonatal screening.
Heart Rates of Breast‐Fed and Formula‐Fed Infants
Authors: Butte Nancy F. , O'Brian Smith E. , Garza Cutberto 
Published: January 2024

SummaryHeart rates of 46 breast‐fed and formula‐fed infants were monitored continuously for −18 h at 1 or 4 months of age. Heart rate differed significantly by age (1 month > 4 months; p < 0.001) and feeding mode (breastfed < formula‐fed; p < 0.001). Approximately 58% of the variability in heart rate could be attributed to feeding mode, sex, and age. Heart rate was correlated significantly with energy intake (r = 0.60; p < 0.001), but not sleeping metabolic rate or total daily energy expenditure. Energy intake accounted for none of the variability in heart rate beyond that explained by feeding mode, sex, and age.
Congenital Diabetes Mellitus and Fatal Secretory Diarrhea in Two Infants
Authors: Jonas Maureen M. , Bell Michael D. , Eidson Margaret S. , Koutouby Raghad , Hensley George T. 
Published: January 2024

SummaryTwo unrelated male infants presented with brittle insulin‐dependent diabetes mellitus in the first days of life. Subsequently they each developed severe secretory diarrhea, with stool volumes of more than 100 ml/ kg/day. Extensive biochemical and serological investigation failed to reveal the etiology of the diarrhea. The infants, cared for at different institutions, underwent therapeutic trials of various agents including loperamide, cholestyramine, prednisone, indomethacin, and somato‐statin analogue, without response. Both infants succumbed to septicemia and malnutrition related to diarrhea and poor control of glycemia. At autopsy, both were found to have absence of islets of Langerhans in the pancreas, and diffuse dysplastic changes in small and large intestinal mucosae. In particular, the entire alimentary tract in each case was lined by epithelia most typical of foregut mucosa: secretory‐type glands, absent crypts of Lieberkuhn, and absent villi. These cases are contrasted with previously‐reported infants with congenital diabetes mellitus, and the possible interrelation of these two highly unusual findings, congenital diabetes mellitus and diffuse intestinal dysplasia, is examined.
Button Gastrostomy Obstructing the Ileocecal Valve Removed by Colonoscopic Retrieval
Authors: Berman James H. , Radhakrishnan Jayant , Kraut Jerome R. 
Published: January 2024

SummaryA 16‐year‐old male with profound developmental disability acutely developed retching, irritability, and abdominal distension. He had undergone a fundopli‐cation and placement of a feeding gastrostomy 6 months earlier. A button gastrostomy tube was subsequently placed, but was noted to be missing from the stoma when the patient was awakened by his mother one morning. Within 48 hours he developed signs of abdominal obstruction. Abdominal radiography confirmed the presence of the gastrostomy button at the ileocecal valve. After a trial of decompression and medical management failed, the button was successfully removed endoscopically, resulting in prompt resolution of the obstruction. Gastrostomy buttons are a newer alternative to conventional catheters, but can become dislodged and migrate, as illustrated by this case. Refinements in the outer wing design may be needed.
Colostral Antioxidants
Authors: Buescher E. Stephen , McIlheran Sarah M. 
Published: January 2024

SummaryHuman colostrum contains several antioxidants which prevent the detection of human polymorpho‐nuclear leukocyte (PMN) respiratory burst products. Using column chromatography to fractionate colostrum, two peaks of antioxidant activity were resolved away from colostral proteins and further characterized. One peak contained both cytochrome c‐reducing activity and H2O2‐depleting activity. This peak had the chromatographic, spectral, and antioxidant characteristics of ascorbate, and by high performance liquid chromatography (HPLC) methods, was shown to contain ascorbate as well as at least four other materials. The antioxidant activity in this peak was totally ascorbate oxidase sensitive and partially uricase sensitive. The other peak contained only H2O2‐depleting activity and had the chromatographic, spectral, and antioxidant characteristics of uric acid. By HPLC, uric acid was the only component in this peak and its antioxidant activity was completely uricase sensitive and ascorbate oxidase resistant. Colostral uric acid levels were measured in eight postpartum women and found to be approximately one‐third of simultaneously determined serum uric acid levels. Colostrum contains at least two separate antioxidants, one of which is ascorbate‐like and the other is uric acid. We speculate that these antioxidants may function in human colostrum as traps for neutrophil‐generated reactive oxygen metabolites.
Macroamylasemia in a 5‐Year‐Old Girl
Authors: D'Avanzo Michele , Cobbaert Christa , Tolone Carlo , Toraldo Roberto , Canino Gianfranco , Vetrano Francesco , Santinelli Raffaele , Iafusco Ferdinando 
Published: January 2024

SummaryIn macroamylasemia, a macromolecular complex consisting of amylase linked to immunoglobulins circulates in the plasma and usually causes benign hyperamylasemia with low or normal amylasuria. Macroamylasemia is extremely rare in pediatric patients as it has been described in only four patients. We report herein the case of a 5‐year‐old girl with abdominal pain and macroamylasemia. To recognize macroamylase, we used agar gel electrophoresis, PEG precipitation, and fast protein liquid chromatography (FPLC). In our case, FPLC was found to be the most reliable method for the identification of the macromolecular complex. Macroamylasemia is merely a biochemical abnormality that is not associated with any kind of pathology. Its identification is therefore essential in order to avoid a wrong diagnosis, i.e., pancreatitis, with consequent inappropriate therapies.
Use of Intravenous Lipid and Hyperbilirubinemia in the First Week
Authors: Adamkin D. H. , Radmacher P. G. , Klingbeil R. L. 
Published: January 2024

SummarySerum triglycerides, free fatty acids, unconjugated bilirubin, and albumin were evaluated in 40 neonates receiving 0.5–3.5 g/kg/day of a 50/50 soybean‐safflower lipid emulsion infused during 18 h. The purpose of the study was to evaluate lipid tolerance and unconjugated hyperbilirubinemia according to our total parenteral nutrition protocol, which initiates lipid on postnatal day 4. Mean serum triglycerides and free fatty acids were within the range of prelipid infusion values at all dosages, and no statistically significant differences were noted between very‐low‐birth‐weight neonates and those > 1,500 g birth weight. Mean free fatty acid:albumin molar ratio was < 1.0 at all dosages and no individual patient values exceeded a ratio of 3.0. Mean peak serum unconjugated bilirubin of 5.8 mg/dl on postnatal day 3 was stable or fell the next 10 days of lipid‐inclusive total parenteral nutrition. Initiating intravenous lipid on the 4th postnatal day at 0.5 g/kg/day and increasing at 0.5 g/kg/day increments at the end of the 1st postnatal week appears to be tolerated well. However, 5% of serum triglyceride levels exceeded 200 mg/dl. Therefore, in view of the unpredictability of a given patient's tolerance to lipid infusion, there should be monitoring for lipemia.
Influence of Infant Diets on the Ecology of the Intestinal Tract of Human Flora‐Associated Mice
Authors: Hentges David J. , Marsh Wallace W. , Petschow Byron W. , Thai Wendy R. , Carter Melody K. 
Published: January 2024

SummaryThe effect of diet on intestinal ecology was studied in germ‐free mice that were inoculated orogastrically with predominant intestinal flora components isolated from the feces of breast‐fed human infants. The flora components colonized the intestines of mice and persisted at fixed population levels. Groups of flora‐ associated mice were fed either human milk, bovine milk, whey‐dominant formula, or formula modifications exclusively for 2 weeks, and then examined for changes in small intestinal and cecal flora composition, cecal pH, and resistance to intestinal colonization with Salmonella typhimurium. Dietary variations influenced the composition of the flora to a moderate degree but the differences were generally not statistically significant. However, the addition of bovine lactoferrin to the whey‐dominant formula resulted in significantly greater counts of Bifidobacterium, Bacteroides, Enterococcus and total aerobes in the small intestine when compared with mice fed unsup‐plemented formula. Bifidobacterium was present in large numbers in both the ceca and small intestines of mice fed the lactoferrin‐supplemented formula. Despite similarities in intestinal flora patterns among mice fed the various diets, human milk consumption resulted in a lower pH of cecal contents and a greater resistance to colonization by Salmonella typhimurium after orogastric challenge than the consumption of the other diets.
Type of Acidosis and Clinical Outcome in Infantile Gastroenteritis
Authors: Weizman Zvi , Houri Sion , Gradus Dorit Ben‐Ezer 
Published: January 2024

SummaryTo explore different types of metabolic acidosis in acute gastroenteritis of infancy, and to correlate it with nutritional status and clinical outcome, we examined prospectively 27 consecutive infants admitted with moderate to severe dehydration and arterial blood pH 7.20. Serum anion gap, creatinine, divalent cations (Ca, Mg), and the anionic contribution of total protein, lactate, and phosphate as well as ketones and urine pH, were determined on admission and compared with age‐matched controls. Twenty infants (Group A) presented with normal anion gap (13.1 ± 2.7) associated with hyperchloremia (115.8 ± 4.2 mmol/L, p < 0.01) and diminished bicarbonate (9.7 ± 2.4 mmol/L, p < 0.01), compared with controls. The other seven infants (group B) demonstrated an increased anion gap (26.7 ± 3.2, p < 0.01), associated with significant elevations of lactate (3.4 ± 0.7 mmol/L, p < 0.001), total protein (93.4 ± 12.3 g/L, p < 0.01), as well as phosphate (2.3 ± 0.2 mmol/L, p < 0.01) and creatinine (164.9 ± 45.1 μmol/L, p < 0.001), compared with controls. No significant deviations of blood divalent cations or ketones were noted in both groups. Urine pH was ± 5.5 in all subjects. Analysis of nutritional status and clinical outcome in both groups revealed significant differences. Infants in group B, compared with group A, were significantly more severely malnourished (7 of 7 versus 4 of 20, p < 0.001), were more likely to have prolonged diarrhea ( > 14 days) (5 of 7 versus 3 of 20, p < 0.001), and required longer durations of both intravenous therapy (6.7 ± 2.3 versus 2.2 ± 0.7 days, p < 0.001) and hospitalization (19.4 ± 4.6 versus 5.4 ± 1.6 days, p < 0.001). We conclude that some infants with acute diarrhea may develop severe dehydration and anion‐gap acidosis associated with lactic acidemia and renal dysfunction. These high‐risk infants more often demonstrate severe malnutrition, persistent course of diarrhea, and prolonged hospital stay. Early identification of such infants, and aggressive therapy, will probably improve outcome.
Persistent Diarrhea and Malnutrition—The Impact of Treatment on Small Bowel Structure and Permeability
Authors: Sullivan Peter B. , Lunn Peter G. , Northrop‐Clewes Christine , Crowe Peter T. , Marsh Michael N. , Neale Graham 
Published: January 2024

SummaryPrevious studies using dual sugar permeability tests suggested that damage to the small intestinal mucosa plays an important part in the development of persistent diarrhea in The Gambia. The present study has extended these findings by examining the effect of nutritional rehabilitation on intestinal permeability and mucosal morphology. Intestinal permeability, measured by lactulose:man‐nitol (L:M) absorption, and mucosal structure, measured by a quantitative, computerised morphologic technique, were evaluated in 20 children before and after such treatment. L:M ratios were high on admission, (0.66 ± 0.36) and, despite some temporary improvement, did not significantly improve (0.49 ± 0.30) following rehabilitation for one month. The changes in L:M ratio were largely due to an increase in lactulose absorption, showing that the small intestinal mucosa becomes more “leaky” as a result of nutritional rehabilitation. Although no correlation was found between measures of intestinal permeability and mucosal morphology, nutritional restitution was associated with a significant increase in size of the mucosal crypt cell compartment, but not in villous epithelial volumes during the same period. It is necessary to establish, by further prospective studies, the interval required for full restitution of small intestinal structure and function during treatment for persistent diarrhea.
Transient Protein‐Losing Hypertrophic Gastropathy Associated with Mycoplasma pneumoniae Infection in Childhood
Authors: Amitai D. Ben , Zahavi I. , Dinari G. , Garty B. Z. 
Published: January 2024

SummaryA 3‐year‐old boy with transient protein loss due to hypertrophic gastropathy is described. Serologic evidence of concomitant Mycoplasma pneumoniae infection was present. Although sometimes associated with infections, protein‐losing hypertrophic gastropathy has not been reported previously in association with Mycoplasma pneumoniae infection.